MITRAL STENOSIS
Cause: Chronic rheumatic heart disease is the most common cause. It is rarely congenital.
Symptoms: Breathlessness, usually on exertion, palpitation and cough, may be hemoptysis, and weakness.
Signs: Pulse—low volume and jugular venous pressure (JVP)—normal (but raised in pulmonary hypertension).
In precordium:
- Inspection: Visible cardiac impulse in mitral area
- Palpation: Tapping apex beat. Diastolic apical thrill
- Auscultation:
- First heart sound—loud in all areas, more in mitral area. Second heart sound—normal in all the areas
- Mid-diastolic murmur (MDM) in mitral area, which is low-pitched, localized, rough, rumbling (LLRR), and best heard with the bell of stethoscope, in left lateral position with breathing hold after expiration, with presystolic accentuation
- Opening snap, just medial to the mitral area.
Investigations:
- X-ray of chest posteroanterior(P/A) view: It shows straightening of left border of heart and double border on right side
- Electrocardiogram (ECG) and color Doppler echocardiogram. Cardiac catheter in some cases.
Complications:
- Atrial fibrillation (AF)
- Pulmonary edema, pulmonary hypertension causing congestive cardiac failure (CCF), embolism and infarction
- Left atrial thrombus with systemic embolism
Treatment:
- Medical: Restriction of activity
- Low-dose diuretic (thiazide or frusemide)
- Anticoagulant (e.g. warfarin) to reduce the risk of embolism
- If AF—digoxin, beta-blocker and rate-limiting calcium antagonist (e.g. verapamil and diltiazem)
- If CCF—diuretics and digoxin.
- Surgical: Valvuloplasty, valvotomy and valve replacement.
MYXOMA OF HEART
It is the common primary tumor of heart, usually benign, attached by a pedicle to atrial septum. It occurs in 3rd–6th decade and is seen more in females.
Sites of origin: Left atrium (75%).
Clinical features: Three groups of manifestations—
- Obstructive features like mitral stenosis (MS), signs vary with posture. It may be syncope or vertigo
- Embolic features either systemic or pulmonary embolism
- Constitutional features: Fever, malaise, weakness, loss of weight, myalgia, arthralgia, clubbing, skin rash and Raynaud's phenomenon.
Investigations:
- Complete blood count (CBC) [Leukocytosis, polycythemia and high erythrocyte sedimentation rate (ESR)]
- Chest X-ray (may be similar to MS)
- 2D or transesophageal echocardiogram
- Computed tomography (CT) scan or magnetic resonance imaging (MRI) may be done.
Treatment: Surgical excision. Recurrence may occur.
MITRAL REGURGITATION
Causes:
- Chronic rheumatic heart disease
- Mitral valve prolapse (MVP)
- Papillary muscle dysfunction
- Infective endocarditis
- Trauma or mitral valvotomy
- Connective tissue diseases [rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Marfan syndrome and Ehlers–Danlos syndrome]
- Others: Ankylosing spondylitis, cardiomyopathy, and secondary to LV dilatation.
Symptoms: Breathlessness on exertion, palpitation, cough and weakness.
Signs: In precordium—
- Inspection: Visible cardiac impulse in mitral area
- Palpation: Apex beat is shifted, diffused and thrusting in character. Systolic thrill in mitral area
Investigations: X-ray of chest P/A view, ECG and color Doppler echocardiogram. Cardiac catheterization is done in some cases.
Complications: Acute left ventricular failure (LVF), infective endocarditis, embolism, arrhythmia (AF and ectopics) and CCF.
Treatment:
- In mild to moderate case:
- Diuretic (frusemide or thiazide). Vasodilator—angiotensin-converting enzyme (ACE) inhibitor (captopril, ramipril and lisinopril)
- If fast AF—digoxin. Anticoagulant—if AF or history of pulmonary embolism
- Prophylactic penicillin to prevent endocarditis. Follow up every 6 months by echocardiogram.
- In severe MR—replacement of valve.
MITRAL VALVE PROLAPSE
It is also called as Barlow's syndrome or floppy mitral valve. It can cause MR, may be congenital or due to degenerative myxomatous change. It is common in thin and young women, and may be familial.
Symptoms: Atypical chest pain in left submammary region, stabbing in quality, and confused with anginal pain. May be palpitation, dyspnea and fatigue.
Signs: Mid-systolic click is followed by late systolic murmur (cardinal sign). Later, signs of MR appear.
Investigation: Echocardiogram.
Treatment: If asymptomatic—reassurance and periodic echocardiography. If chest pain and palpitation–beta-blockers (propranolol, atenolol and metoprolol). Prophylactic penicillin is given to prevent infective endocarditis. If MR develops, treatment as in MR.
TRICUSPID REGURGITATION
Causes:
- Functional: Secondary to pulmonary hypertension, cor pulmonale and right heart failure (common cause)
- Chronic rheumatic heart disease
- Infective endocarditis (commonly involved in drug addicts)
- Others: Congenital heart disease (e.g. Ebstein's anomaly), carcinoid syndrome, right ventricular papillary muscle infarction, and trauma or steering wheel injury in chest.
Symptoms: May be asymptomatic. Symptoms of primary disease.
Signs: Pulse—normal, JVP—raised and giant “v” wave, oscillating up to ear lobule.
Precordium:
- Palpation: Left parasternal lift and epigastric pulsation [due to RVH (right ventricular hypertrophy)]
- Others: Liver may be enlarged, tender and pulsatile.
Investigations: Chest X-ray, ECG and color Doppler echocardiogram.
Complications: Right-sided heart failure and infective endocarditis.
Treatment: Treatment of primary cause. In severe organic tricuspid regurgitation (TR), valve replacement is done.
EBSTEIN'S ANOMALY
It is a congenital heart disease associated with downward displacement of tricuspid valve into the right ventricle. Hence, right atrium is large and right ventricle is small. Characteristically, multiple clicks occur due to asynchronous closure of tricuspid valve. Atrial septal defect (ASD) is commonly associated with this anomaly.
PULMONARY STENOSIS
Causes: Congenital (common). It is associated with carcinoid syndrome, Noonan syndrome and Fallot's tetralogy. May occur, if rubella in pregnancy. Three types—(1) valvular, (2) subvalvular, and (3) supravalvular.
Symptoms: May be asymptomatic. May be fatigue, weakness and effort syncope.
Signs: In precordium—
- Palpation: Left parasternal lift and epigastric pulsation (due to RVH). Systolic thrill in pulmonary area
- Auscultation:
- First heart sound—normal in all areas
- Second heart sound—P2 is soft in pulmonary area, and A2 is normal (wide splitting of second sound may be present).
- Ejection systolic murmur in pulmonary area radiates to neck
- Fourth heart sound may be present (due to right atrial contraction).
Investigations: ECG, chest X-ray and color Doppler echocardiogram.
Complications: Right heart failure and pulmonary embolism.
Treatment: In mild case, compatible with normal life. In severe symptomatic case, balloon valvuloplasty is done. Infective endocarditis is unusual in pulmonary stenosis (PS) and prophylactic antibiotic is unnecessary.
PULMONARY REGURGITATION
Causes:
- Dilatation of pulmonary valve secondary to pulmonary hypertension
- Secondary to MS
- Rarely rheumatic fever (RF) and carcinoid syndrome.
Symptoms: May be asymptomatic or symptoms of primary disease. Features of right heart failure.
Signs: Left parasternal lift and P2 may be soft. Early diastolic murmur in 3rd or 4th left intercostal space.
Treatment: Treatment of underlying cause. In severe regurgitation, valve replacement is done.
AORTIC STENOSIS
Causes:
- Chronic rheumatic heart disease
- Congenital bicuspid aortic valve (common in male)
- Others: Calcification in old age and congenital (in early age).
Symptoms:
- Breathlessness, mainly on exertion, palpitation and anginal chest pain
- Syncope (transient loss of consciousness) during effort
- Sudden death [probably due to ventricular fibrillation (VF)].
Signs: Pulse—low volume, slow rising, blood pressure (BP)—low systolic, normal diastolic and narrow pulse pressure.
In precordium:
- Inspection: Visible cardiac impulse in mitral area
- Palpation: Palpable apex beat and heaving. Systolic thrill in aortic area
- Auscultation:
- First heart sound—normal in all areas
- Second heart sound—A2 is soft in all the areas, and P2 is normal. May be reversed splitting of second heart sound
- Ejection systolic murmur in aortic area radiates to the neck.
Investigations: X-ray of chest, ECG and color Doppler echocardiogram. Cardiac catheterization is done in some cases.
Complications: Left ventricular failure, infective endocarditis (in 10%), sudden death due to VF, complete heart block (CHB) (in calcification of aortic valve), and systemic embolism.
Treatment: If aortic stenosis (AS) is asymptomatic, follow-up is done with periodic echocardiogram. Avoid strenuous activity or exercise.
- If symptomatic or syncopal attack or asymptomatic with severe AS—valve replacement
- If patient is unfit for surgery—percutaneous valvuloplasty
- In children, elderly or pregnancy—valvotomy.
AORTIC REGURGITATION
Causes:
- Chronic rheumatic heart disease
- Infective endocarditis
- Syphilitic aortitis
- Bicuspid aortic valve
- Others: Dissecting aneurysm of ascending aorta, Marfan's syndrome, seronegative arthritis (ankylosing spondylitis), RA, cystic medial necrosis and congenital.
Symptoms: It may be asymptomatic. Some of the other symptoms may be palpitation, breathlessness on exertion, cough and anginal pain.
Signs: Pulse—high volume, collapsing and dancing carotid pulse in neck (Corrigan's sign). BP—high systolic, low diastolic and wide pulse pressure.6
Precordium:
- Inspection: Visible cardiac impulse
- Palpation: Apex beat is shifted, thrusting. Diastolic thrill may be present in left parasternal area
- Auscultation:
- First heart sound—normal in all the areas. Second heart sound—A2 is absent, and P2 is normal
- Early diastolic murmur in left lower parasternal area with patient bending forward and breathing hold after expiration. Ejection systolic murmur may be present in aortic area due to increased flow.
Formula of 3 to diagnose aortic regurgitation (AR):
- 3 pulse: Collapsing (water hammer), dancing carotid and capillary pulsation
- 3 BP: Rise of systolic, fall of diastolic and wide pulse pressure
- 3 murmur: Early diastolic murmur, Austin Flint murmur and ejection systolic murmur.
Investigations: X-ray of chest, ECG and color Doppler echocardiogram. Cardiac catheterization is done in some cases.
Complications: Acute LVF, infective endocarditis and arrhythmia.
Treatment: In mild asymptomatic case, follow-up.
- In asymptomatic, moderate-to-severe AR with normal left ventricular (LV) function, conservative treatment. Systolic BP should be controlled with vasodilator drugs (nifedipine or ACE inhibitors)
- In severe case—valve replacement.
HEART FAILURE
It is the failure of heart to maintain adequate cardiac output to meet the demand of tissue or can do so only at the expense of an elevated filling pressure. Three types—(1) left-sided, (2) right-sided, and (3) biventricular failure.
Acute left ventricular failure: It is the failure of left ventricle to propel blood in systemic circulation. As a result, there is accumulation of blood in pulmonary circulation, causing pulmonary edema.
Causes of LVF (or pulmonary edema):
- Systemic hypertension
- Acute myocardial infarction (MI)
- Aortic valvular disease (stenosis and regurgitation)
- Mitral regurgitation (MR)
- Others: Cardiomyopathy, coarctation of aorta, and rapid or excess infusion of fluid or blood or plasma.
Symptoms: Breathlessness, may be orthopnea. Cough with frothy sputum, palpitation, restlessness, sweating and oliguria.
Signs: Patient looks dyspneic with propped up position, and cyanosis. Pulse—tachycardia, may be pulsus alternans. BP—low but may be high, if the patient is hypertensive.
In precordium:
- Apex beat: It may be shifted, thrusting or heaving in character
- Signs of primary cause may be present.
Investigations: Chest X-ray shows pulmonary edema (perihilar bat wing appearance), ECG and echocardiogram.
Treatment of acute LVF (pulmonary edema):
- Bed rest. Propped up position. High flow O2 inhalation
- Diuretic: Injection frusemide IV, 80–120 mg. It may be repeated
- Morphine [if no contraindications such as bronchial asthma, chronic obstructive Pulmonary disease (COPD), emphysema and chronic bronchitis]: 10–20 mg intravenous (IV) slowly with antiemetic metoclopramide or cyclizine
- ACE inhibitors: Ramipril, captopril and enalapril
- If there is no response, inotropic agents like dopamine and dobutamine are given
- Treatment of primary cause. Anti-arrhythmic drug is given, if arrhythmia occurs.
CONGESTIVE CARDIAC FAILURE
It means right heart failure. There is inability of right ventricle to propel blood resulting in backflow of blood to systemic veins, causing engorged vein, enlarged liver and dependent edema.
Causes:
- Secondary to left-sided heart failure (common cause)
- Mitral stenosis with pulmonary hypertension
- Chronic cor pulmonale due to any cause
- Pulmonary hypertension
- Others: PS or regurgitation, TR, shunt anomaly [ASD, ventricular septal defect (VSD)], reversal of shunt (Eisenmenger syndrome), cardiomyopathy, and right ventricular MI.
Symptoms:
- Breathlessness on exertion, cough with mucoid sputum, and palpitation
- Pain in right upper abdomen (due to hepatomegaly) and swelling of legs
- Others: Weakness, weight loss, anorexia, nausea, vomiting, oliguria and nocturia.
Signs: Pulse (low volume), BP (low) and JVP (engorged and pulsatile). Dependent pitting edema in legs.
In precordium:
- Inspection: Visible cardiac impulse
- Palpation: Apex beat may be shifted. Thrill—absent or present according cause
- Auscultation: Heart sounds and murmur according to the vulvular lesion.
Abdomen: Liver is enlarged and tender.
Three cardinal signs of CCF: (1) engorged and pulsatile neck veins, (2) enlarged and tender liver, and (3) dependent pitting edema.
Investigations: X-ray of chest, ECG, and echocardiogram. Others—CBC, ESR, urea, creatinine and electrolytes.
Treatment:
- Complete rest. Restriction of fluid and salt
- Vasodilator [ACE inhibitor or angiotensin II receptor blockers (ARB)]
- Beta-blocker (bisoprolol 1.25 mg daily, gradually increases the dose over 12 weeks up to 10 mg daily)
- Digoxin (helpful in CCF with AF)
- Treatment of arrhythmia (e.g. amiodarone) and treatment of underlying cause
- Heart transplantation—if all above measures fail.
Causes of biventricular failure: Dilated cardiomyopathy, myocarditis, extensive MI, right heart failure secondary to left heart failure (MR, AS or AR), hyperdynamic circulation (in severe anemia, thyrotoxicosis, arteriovenous shunt and beriberi), myxedema and multiple vulvular diseases.
HYPERTENSION
It means persistent rise of arterial BP above the arbitrarily normal range. If systolic BP is >140 mm Hg and diastolic BP is >90 mm Hg, the patient is diagnosed as hypertensive.
Causes:
- Primary or essential hypertension (95%)—cause unknown
- Secondary (5%):
- Renal (common): Chronic glomerulonephritis, chronic pyelonephritis, diabetic nephropathy, adult polycystic kidney disease and renal artery stenosis
- Endocrine: Cushing's syndrome, Conn's syndrome (primary aldosteronism), pheochromocytoma, congenital adrenal hyperplasia, hyperparathyroidism, primary hypothyroidism, hyperthyroidism, and acromegaly
- Drugs: Alcohol, oral contraceptive pill, steroid and erythropoietin
- Others: Preeclampsia and eclampsia (toxemia of pregnancy), pregnancy-induced hypertension, coarctation of aorta, and cerebral tumor.
Clinical features: It may be asymptomatic and is detected in routine examination. Symptoms are headache, dizziness, giddiness, insomnia and blurring of vision. Features of complication such as heart failure, cerebrovascular disease (CVD), and renal failure.
Complications of hypertension:
- Cardiovascular: Ischemic heart disease (IHD), acute LVF, and dissecting aneurysm
- Renal: Renal failure
- Ocular: Retinopathy
- Neurological: CVD (intracerebral or subarachnoid hemorrhage), and hypertensive encephalopathy.
Note: Following points are important—
- A single reading is not sufficient. At least three readings in different times should be taken to label as hypertensive
- BP should be measured at least 5 minutes after the patient has taken rest comfortably in sitting or supine position
White coat hypertension: When BP is recorded in the doctor's chamber, there may be transient rise in BP in a normal individual. This is called white coat hypertension.
Grades of hypertension:
Grade | Systolic | Diastolic |
|---|---|---|
Grade 1 (mild) | 140–159 mm Hg and/or | 90–99 mm Hg |
Grade 2 (moderate) | 160–179 mm Hg and/or | 100–109 mm Hg |
Grade 3 (severe) | ≥180 mm Hg and/or | ≥110 mm Hg |
Hypertensive encephalopathy: It is characterized by very high BP with neurological abnormalities such as severe headache, loss of consciousness, convulsion, paresthesia, transient disturbance of speech or vision, and retinopathy.
Malignant hypertension: It is characterized by severe hypertension with diastolic BP >130 mm Hg, associated with grade III or IV retinopathy (retinal hemorrhage or exudates and papilledema) and renal failure or encephalopathy. If untreated, death occurs within months.
Treatment: Complete rest. Slow and controlled reduction of BP by oral antihypertensive over 24–48 hours is ideal (rapid reduction is avoided as it reduces tissue perfusion, can cause cerebral damage, and may even precipitate coronary or renal insufficiency). Sometimes IV or intramuscular (IM) labetalol, IV glyceryl trinitrate, and IM hydralazine may be used.
Refractory hypertension: When there is no response to antihypertensive drugs, it is called refractory hypertension. Causes are irregular and inadequate drug therapy (common cause) or undiagnosed cause (renal artery stenosis or pheochromocytoma).
Resistant hypertension: It means failure to control BP with full dose of appropriate three drugs including a diuretic.
Following things should be seen:
- Improper BP measurement
- Volume overload due to excess sodium intake, renal disease or inadequate diuretic therapy
- Inadequate dose and improper combination of drugs or noncompliance
- Patient may take nonsteroidal anti-inflammatory drugs (NSAIDs), steroid, oral contraceptive pills, cyclosporine and erythropoietin
- Other secondary causes of hypertension and associated obesity, and excess alcohol intake.
Investigations in hypertension: History, physical examination and laboratory investigation should be done.
Laboratory investigations:
- Routine: Urine examination [routine/microbial (R/M)], blood urea, creatinine, serum electrolytes, blood sugar, lipid profile. X-ray of chest P/A view, ECG and echocardiogram
Treatment of hypertension:
- General measures (nondrug treatment):
- Salt restriction, smoking should be stopped, weight reduction if obese, dietary modification (low fat, increase fruits and vegetables), avoid anxiety and tension, and restriction of tea, coffee and alcohol
- Regular exercise (at least 30 minutes daily)
- Control of diabetes mellitus (DM) and other modifiable risk factors.
- Drug treatment:
- Diuretic: Thiazide
- ACE inhibitors: Enalapril, lisinopril and ramipril
- ARB: Losartan, valsartan and irbesartan
- Calcium channel blockers: Amlodipine, cilnidipine, nifedipine, diltiazem and verapamil
- Beta-blockers: Atenolol, metoprolol, and bisoprolol
- Combined alpha and beta-blocker: Labetalol and carvedilol
- Alpha-blocker: Prazosin
- Others: Methyldopa (used in pregnancy)
- Management of primary cause, if any.
How to start drug in treatment of hypertension: Single drug is started (diuretic or beta-blocker or calcium channel blocker or ACE inhibitor). If no response, combination therapy.
Contraindications of beta-blocker:
- Respiratory: Bronchial asthma, COPD, emphysema and chronic bronchitis
- Cardiovascular system (CVS): Bradycardia, partial or CHB, CCF, peripheral vascular disease (Raynaud's phenomenon)
- Endocrine: Pheochromocytoma (beta-blocker alone is avoided), DM receiving insulin (masks the features of hypoglycemia).
Treatment of hypertension in specific conditions
Hypertension in bronchial asthma: Avoid β-blockers. May be used—diuretics, calcium channel blocker, ARB, and ACE inhibitor (it may cause cough).
Hypertension in chronic kidney disease (Target BP is <130/80 mm Hg):
- Angiotensin-converting enzyme inhibitors and ARB may delay progression of kidney disease (if creatinine is >2.5 mmol/L, these should be avoided)
- Calcium channel blockers may be used
- Loop diuretic (frusemide).
Hypertension in pregnancy:
- Methyldopa or labetalol
- Calcium channel blocker (nifedipine) may be used. Beta-blocker may be used (avoid in first trimester)
- Angiotensin-converting enzyme inhibitor is contraindicated. Diuretic is also avoided
- Severe hypertension or eclampsia may be treated with intravenous hydralazine.
Hypertension in diabetes mellitus:
- Angiotensin-converting enzyme inhibitor, ARB, and calcium channel blocker may be used
Hypertension in peripheral vascular disease:
- Calcium channel blocker, alpha-blocker and ACE inhibitor
- Avoid beta-blocker.
Hypertension in dyslipidemia:
- Alpha-blocker, ACE inhibitor, ARB, and calcium channel blocker
- Avoid beta-blocker and diuretic (which worsen lipid profile).
Hypertension in psoriasis: Calcium channel blocker may be used. Avoid β-blocker and ACE inhibitor (which aggravates).
Hypertension in angina: Beta-blocker, calcium channel blocker and nitrate.
RHEUMATIC FEVER
It is a multisystem disorder that occurs as a sequela to pharyngitis by group A beta-hemolytic streptococcus. It is common in children and young adults, 5–15 years of age.
Diagnostic criteria of RF: Diagnosed revised by Jones criteria. After Streptococcus pharyngitis, usually a latent period of 1–3 weeks.
- Major criteria (5 criterions):
- Shifting or migrating polyarthritis involving big joints (knee, elbow, ankle and wrist)
- Carditis
- Rheumatic chorea
- Erythema marginatum
- Subcutaneous nodule.
- Minor criteria: Fever, arthralgia, previous history of RF, high ESR or C-reactive protein (CRP), leukocytosis, and first- or second-degree AV block in ECG
- Supportive evidence of previous streptococcal infection—such as recent streptococcal sore throat, scarlet fever, high antistreptolysin O (ASO) (>200) or other streptococcal antibody titer (anti-DNAse or antihyaluronidase) or positive throat swab culture.
Diagnosis is made by two or more major criteria, or one major and two or more minor criteria plus supportive evidence of streptococcal infection.
Signs of carditis: RF can cause carditis involving all the layers of heart (endocardium, myocardium and pericardium), called pancarditis.
- Signs of endocarditis: Soft heart sounds, pansystolic murmur (due to MR), mild diastolic murmur (Carey Coombs murmur), and early diastolic murmur (due to AR)
- Signs of myocarditis: Tachycardia, soft heart sounds, S3 gallop, cardiomegaly and heart failure
- Signs of pericarditis: Pericardial rub and pericardial effusion may develop.
Investigations:
- CBC and ESR (high ESR and leukocytosis)
- CRP: High ASO titer—high (in adult >200, in children >300)
- Throat swab culture (to find Streptococcus beta-hemolyticus)
- Chest X-ray, ECG and echocardiography.
Complications:
- Later, rheumatic heart disease causing vulvular stenosis and regurgitation.
Treatment of acute RF:
- Complete bed rest
- Oral phenoxymethyl penicillin—250 mg 6 hourly for 10 days or single injection benzathine penicillin 1.2 million units, deep IM in buttock. Erythromycin may be given, if allergic to penicillin
- Analgesic (for pain). Aspirin 60 mg/kg/day in divided doses. Higher dose may be required
- Other treatment: If carditis or severe arthritis, prednisolone 1–2 mg/kg daily. If chorea, diazepam for mild case or haloperidol in severe case.
- Treatment of complications like cardiac failure, valvular lesion, heart block, arrhythmia, etc., if needed.
Prophylactic of RF: To prevent recurrence, oral phenoxymethyl penicillin 250 mg 12 hourly or injection benzathine penicillin, 1.2 million units deep IM in buttock every 4 weeks should be given. In penicillin-sensitive cases, erythromycin (250 mg 12 hourly) may be used. It should be continued up to 21 years of age or 5 years after the last attack (recurrence after 5 years is rare), whichever comes last.
Antibiotic prophylaxis should be given for dental or surgical procedure.
Note: Following points are important—
- Skin infection with Streptococci is not associated with RF
- Streptococcal sore throat may not be present in some cases
- More than 50% patients of RF with carditis will develop chronic valvular disease after 10–20 years. All the cardiac valves may be involved, but commonly mitral valve is affected (90%). Also aortic valve may be involved. Involvement of tricuspid and pulmonary valves is rare (5%)
- In chronic rheumatic heart disease, may not be history of RF in 50–60% cases
- Arthritis in RF recovers completely without any residual change (RF licks the joints, kills the heart).
CONGENITAL HEART DISEASES
It may be cyanotic and acyanotic.
Cyanotic: Tetralogy of Fallot (TOF), transposition of great vessels, truncus arteriosus, pulmonary atresia, tricuspid atresia and Ebstein's anomaly.
Acyanotic: Left to right shunt [ASD, VSD and patent ductus arteriosus (PDA)], obstructive lesion (coarctation of aorta, AS and PS), and abnormal position of heart (dextrocardia).
VENTRICULAR SEPTAL DEFECT
Causes: Commonly congenital. Also acquired due to rupture of interventricular septum after acute MI, rarely trauma.
Three types according to size:
- Small: It is asymptomatic, closes spontaneously
- Moderate: Patient presents with fatigue and dyspnea
- Large: Murmur is soft.
Symptoms: May be asymptomatic. Symptoms are breathlessness, palpitation, fatigue and weakness.
Signs: In precordium—
- Inspection: Visible cardiac impulse in left parasternal area
- Palpation: Systolic thrill in left parasternal area in 4th or 5th intercostal space
- Auscultation: Pansystolic murmur in left parasternal area in 4th or 5th intercostal space.
Complications: Infective endocarditis (common in small VSD), pulmonary hypertension with reversal of shunt (Eisenmenger syndrome), and heart failure.
Investigations: ECG, X-ray of chest, color Doppler echocardiography, cardiac catheterization. CMR (cardiac magnetic resonance angiography) may be helpful.
Treatment:
- Small VSD: No surgery. Prophylactic penicillin for subacute bacterial endocarditis (SBE)
- Moderate to large VSD: Surgical correction
- When Eisenmenger syndrome develops: Surgery is contraindicated, as it aggravates right-sided heart failure. Following treatments are given—
- Diuretic and digoxin in some cases. Venesection, if polycythemia
- Heart lung transplantation may be done.
ATRIAL SEPTAL DEFECT
It is common in female. It is of two types:
- Ostium primum (15%): Due to atrioventricular (AV) defect in septum
- Ostium secundum (75%): Defect at fossa ovalis in atrial mid septum.
Symptoms: May be asymptomatic. Symptoms may be breathlessness on exertion, palpitation and weakness.
Signs: In precordium—
- On auscultation:
- First heart sound is normal. Wide and fixed splitting of second heart sound
- Ejection systolic murmur in left 2nd and 3rd intercostal space. High-pitched MDM in tricuspid area.
Note: When there is reversal of shunt, features of Eisenmenger syndrome will be found.
Investigations: X-ray of chest, ECG and color Doppler echocardiogram. Cardiac catheterization or CMR may be done.
Complications: Pulmonary hypertension with reversal of shunt (Eisenmenger syndrome), AF (common), embolism (pulmonary and systemic) and brain abscess.
Treatment:
- No surgery in small ASD. Surgical closure in moderate to large ASD
- Angiographic closure is possible with transcatheter clamshell device
PATENT DUCTUS ARTERIOSUS
Common in female, M:F = 1:3. Probable causes are maternal rubella in first trimester, birth at high altitude, prematurity.
Symptoms: May be asymptomatic. May be breathlessness on exertion, palpitation, weakness, anorexia.
Signs: Pulse—may be high volume, BP—wide pulse pressure, JVP—normal.
In precordium:
- Palpation: Apex beat is thrusting or heaving. Systolic thrill in pulmonary area
- Auscultation: Continuous murmur in left 2nd and 3rd intercostal space, called machinery murmur like “train in a tunnel”.
Complications: Pulmonary hypertension with reversal of shunt (Eisenmenger syndrome), CCF, infective endocarditis, and AF. Duct may rupture or calcify.
Investigations: ECG, X-ray of chest and color Doppler echocardiogram. Cardiac catheterization, MRI or CMR may be done.
Treatment:
- Small PDA can be closed during cardiac catheterization by using implantable occlusive device
- Large PDA: surgical closure
- Prophylactic penicillin for infective endocarditis
- If Eisenmenger syndrome develops, surgery is contraindicated (see Eisenmenger syndrome).
EISENMENGER SYNDROME
Pulmonary hypertension with reversal of shunt is called Eisenmenger syndrome. Causes are ventricular septal defect (VSD), ASF, and patent ductus arteriosus (PDA). Persistently raised pulmonary flow (due to left to right shunt) causes high pulmonary resistance, pulmonary hypertension, and high pressure in right ventricle with reversal of shunt (from right to left side).
Symptoms: Dyspnea, fatigue, syncope, angina, hemoptysis and features of CCF.
Signs:
- Central cyanosis. In PDA, differential cyanosis occurs (cyanosis in toes, not in the hands)
- Clubbing. In PDA, differential clubbing occurs(clubbing in toes, not in the hands)
- Pulse: Low volume. Prominent “a” wave in JVP
- Other signs of pulmonary hypertension: Palpable P2, left parasternal lift, and epigastric pulsation
- Tricuspid regurgitation (TR) may occur. Original murmur of VSD, ASD or PDA decrease in intensity, may disappear.
Treatment:
- Heart lung transplantation may be done
- Surgery is contraindicated in Eisenmenger syndrome, as it aggravates right-sided heart failure.
TETRALOGY OF FALLOT
It is a cyanotic congenital heart disease consisting of four components:
- Pulmonary stenosis (right ventricular outflow tract obstruction)
- Overriding and dextroposition of aorta (aortic origin two-thirds from left ventricle and one-third from right ventricle)
- Right ventricular hypertrophy
- VSD.
Symptoms:
- Breathlessness, weakness, cough, chest pain and palpitation
- Bluish discoloration of lips and fingers during exertion
- Young child usually presents with cyanotic spell (Fallot's spell) during exertion, feeding or crying. The child becomes apneic and unconscious. Squatting relieves cyanosis
- Growth retardation. Syncope, seizure, cerebrovascular events or even sudden death.
Signs:
- Short stature and cyanosis (both central and peripheral). Generalized clubbing involving fingers and toes
- Pulse—low volume, BP—low, JVP—prominent “a” wave (due to RVH).
Precordium:
- Inspection: Visible cardiac impulse in apical and epigastric region
- Palpation: Left parasternal lift and epigastric pulsation (due to RVH), systolic thrill in pulmonary area
- Auscultation: First heart sound is normal in all areas, second heart sound is soft (or absent) in pulmonary area, and A2 is normal. Ejection systolic murmur in pulmonary area, radiates to neck.
Investigations: CBC and ESR (may be polycythemia, ESR may be low). Chest X-ray (boot-shaped heart), ECG. 2D and color Doppler echoardiogram. Cardiac catheterization may be done.
Complications: Infective endocarditis (common), paradoxical emboli, cerebral abscess (10% cases), polycythemia (due to hypoxemia, may cause CVD and MI), and coagulation abnormality.
Acyanotic Fallot: When TOF is associated with infundibular PS. Outflow obstruction is mild, no cyanosis.
Treatment: Total surgical correction should be done before 5 years of age.
- If pulmonary artery is hypoplastic or anatomy is unfavorable, temporarily palliative surgery called Blalock–Taussig shunt is done. Corrective surgery is done later
- Prophylactic penicillin to prevent infective endocarditis.
Blalock–Taussig shunt: It is the anastomosis between left subclavian artery with left pulmonary artery. It improves pulmonary blood flow and pulmonary artery development, and may help definitive surgery later on.16
COARCTATION OF AORTA
It is the narrowing of aorta.
Causes: Congenital. Rarely, acquired in trauma, Takayasu disease. It is of two types:
- Postductal (adult type): Below the origin of left subclavian artery, where ductus arteriosus joins the aorta
- Preductal (infantile type, 2%): Above the origin of left subclavian artery.
Symptoms: More common in male. May be asymptomatic. Symptoms are headache, nose bleeding and claudication of lower limbs and cold legs (due to poor blood flow in lower limbs).
Signs: BP—High in upper limb, low in lower limb. Pulse—Normal in upper limb, feeble in lower limb with radiofemoral delay.
Precordium:
- Inspection: Visible cardiac impulse. Visible dilated tortuous artery around the scapula, anterior axilla and over the left sternal border (due to collateral vessels)
- Palpation: Heaving apex beat and thrill over the collateral vessels
- Auscultation: Both first and second heart sounds are normal. Systolic murmur is audible near sternum, better heard in 4th intercostal space posteriorly (site of coarctation).
Investigations:
- X-ray of chest P/A view—shows heart is enlarged, rib notching, and figure of '3' sign (constriction at coarctation, prestenotic and poststenotic dilatation)
- ECG [LV hypertrophy (LVH)] and echocardiogram. CT scan and CMR may be done.
Complications:
- Hypertension and its complication [LVF and cerebrovascular accident (CVA)]
- Infective endocarditis
- Rupture at the coarctation site
- Dissecting aneurysm and aneurysm of aorta
- Subarachnoid hemorrhage (rupture of Berry aneurysm or circle of Willis).
Treatment: Surgery as early as possible, before 5 years of age.
ISCHEMIC HEART DISEASE
It is due to reduced coronary blood flow to myocardium, when there is imbalance between supply of oxygen and myocardial demand. IHD includes angina pectoris and myocardial infarction.
Causes of IHD: Coronary blood flow may be reduced due to atherosclerosis (most common), thrombosis, spasm, embolus, coronary ostial stenosis and coronary arteritis.
Common presentations of IHD: Angina pectoris, acute MI, arrhythmia, heart failure and sudden death.
Nonmodifiable:
- Age: Common in elderly
- Sex: More in male. After menopause in female, incidence is same
- Family history: More common, if there is family history of IHD
- Genetic factors: A number of genetic factors have been linked with coronary artery disease.
Modifiable:
- Smoking: IHD is more in smokers
- Alcohol: Moderate alcohol consumption is associated with less risk but high intake increases the risk
- Diet: High fats are associated with IHD. Diet low in fresh fruits, vegetables and polyunsaturated fatty acids are associated with increased risk
- Obesity: Overweight has an increased risk
- Exercise: More in sedentary workers
- Others: Hypertension, DM, hyperlipidemia, psychosocial factors (stress, depression, and anxiety).
ANGINA PECTORIS
It is defined as paroxysmal precordial pain of short duration due to transient myocardial ischemia.
Symptoms: Main symptom is pain, which has the following characters—
- Site: Central, retrosternal chest
- Character: Stabbing or squeezing or constricting
- Radiation: Lower jaw, neck and inner side of left arm up to the finger
- Precipitated by exertion, eating or emotion (3 E)
- Relieved by rest and nitroglycerine
- Duration: 5–10 minutes (less than half hour).
Signs: Usually no definitive physical sign.
Investigations:
- ECG is often normal. During attack—ST depression, T inversion
- Chest X-ray, echocardiography, exercise tolerance test (ETT) and coronary arteriography
- Other stress testing: Myocardial perfusion scan, stress echocardiography and transthoracic echocardiography
- For risk factor: Fasting lipid profile and blood sugar.
Treatment:
During acute attack: Sublingual glyceryl trinitrate (GTN) as aerosol or tablet under the tongue. It will relieve attack of angina in 2–3 minutes. If no response, it can be repeated. But if still no response, myocardial infarction should be excluded.
Prevention of further attack:
- Antiplatelet therapy: Low-dose (75–150 mg) aspirin and clopidogrel (75 mg daily)
- Antianginal drugs:
- To prevent angina pain: Oral nitrates such as isosorbide dinitrate (10–20 mg, 8 hourly), isosorbide mononitrate (20–60 mg, once or twice a day) can be given by mouth
- If recurrent or persistent pain: Coronary angiogram should be done. If coronary artery blockage, then stenting or percutaneous transluminal coronary angioplasty (PTCA) or coronary artery bypass surgery (CABG) may be required.
- Risk factors should be controlled
- Lifestyle modification. No smoking, and avoid alcohol. Avoid anxiety, tension and depression
- Reduction of weight if obese. Regular exercise, at least 30 minutes daily
- Control of hypertension and DM. Lipid-lowering drugs—atorvastatin and rosuvastatin.
Treatment of unstable angina:
- Hospitalization. Complete bed rest, oxygen and sedation, if needed
- Aspirin or clopidogrel or combined. Nitroglycerine, beta-blocker and calcium channel blocker
- Heparin: Low-molecular-weight (LMW) heparin [enoxaparin subcutaneous (SC)] for 5–7 days
- If pain persists, nitroglycerine infusion
- If all fail, urgent coronary angiography and revascularization, if necessary.
MYOCARDIAL INFARCTION
It is defined as myocardial necrosis, which occurs as result of critical imbalance between coronary blood flow and myocardial demand due to occlusion of coronary artery by thrombus.
Types:
- Q wave (Transmural) or ST-elevated MI
- Non-Q wave or non-ST-elevated (NSTEMI) or subendocardial MI.
Symptoms:
- Central chest pain which is severe, stabbing or squeezing or constricting, radiates to lower jaw, neck and inner side of left arm up to finger, and not relieved by rest or nitroglycerine, persist for >30 minutes
- Sweating and fear of impending death. Nausea and vomiting (more in inferior MI) and breathlessness
- Collapse or cardiogenic shock.
Signs:
- The patient is restless with pallor, sweating and tachycardia (bradycardia in inferior MI)
- Hypotension, oliguria and cold peripheries
- Signs of complications such as cardiogenic shock, acute LVF, arrhythmia, mitral regurgitation and pericarditis.
Investigations:
- ECG: It shows ST elevation (with upward convexity), pathological Q wave, and T inversion
- Enzymatic changes in acute MI:
- Troponin I: rise in 2–4 hours, may persist up to 7 days
- Serum glutamic oxaloacetic transaminase (SGOT): increases after 12 hours, peak in 24 hours, returns to normal in 3–4 days
- LDH: rises after 12 hours, peak in 3–4 days, and normal after 7–10 days.
- Others: Chest x-ray and echocardiography may be done.
Complications of MI: May be early and late.
- Early complications:
- Arrhythmia: Ventricular ectopics (more common), VF, ventricular tachycardia, sinus bradycardia (common in inferior MI), sinus tachycardia, AF and heart block
- Cardiogenic shock
- Cardiac failure (LVF and biventricular failure)
- Acute pericarditis (common in 2nd or 3rd day)
- Thromboembolism (systemic and pulmonary)
- Rupture of papillary muscle or chordae tendineae resulting in MR, rupture of interventricular septum causing VSD, rupture of ventricular wall.
- Late complications: Ventricular aneurysm (10%), post-myocardial infarction syndrome (Dressler's syndrome), frozen shoulder and postinfarction angina.
Treatment of acute myocardial infarction:
- Admission in coronary care unit (CCU) and complete bed rest. High flow O2 inhalation (60%) 2–4 L/min by nasal cannula
- To relieve pain: Injection morphine (5–10 mg) or dimorphine (2.5–5 mg) plus antiemetic (cyclizine or metoclopramide) IV. May be repeated, if necessary
- Chewable aspirin 300 mg and clopidogrel 300 mg
- Primary percutaneous coronary intervention (PCI), if available. Should be performed as early as possible
- Or thrombolytic therapy: Streptokinase, if no contraindication
- Other therapy: β-blocker (if no contraindication), IV bolus atenolol 5–10 mg or metoprolol 5–15 mg slowly over 5 minutes. Oral atenolol 25–50 mg BD, or bisoprolol 5 mg daily or metoprolol 25–50 mg BD or TDS may be given
- Sublingual nitroglycerin—0.3–1 mg
- Anticoagulants: Heparin 5,000 IU, IV, bolus, and then 0.25 U/kg/hour. Or low-molecular-weight heparin (S/C enoxaparin 1 mg/kg body weight 12 hourly)
- Angiotensin-converting enzyme inhibitor.
Follow-up after acute MI:
- Patient should be reviewed after 6–8 weeks.
- Risk factors should be reviewed and modified accordingly:
- Lifestyle modification (avoid stress and heavy work). Regular exercise. Weight control, if obese
- Smoking should be stopped. Avoid fatty and oily food
- Control of hypertension and DM
- Antiplatelet (aspirin or clopidogrel) should be continued indefinitely
- ACE inhibitor should be continued indefinitely in patient with persistent LV dysfunction (EF <40%)
- Beta-blocker and lipid-lowering agents
VENTRICULAR ANEURYSM
If in ECG, ST remains elevated after few months of acute MI, the diagnosis is ventricular aneurysm. X-ray of chest shows enlarged heart, bulge or rounded protrusion from LV wall, and calcification may occur at the wall of aneurysm. Confirmed by echocardiogram.
ACUTE PERICARDITIS
It is the acute inflammation of pericardium.
Causes:
- Viral (coxsackie B virus and echovirus)—common cause
- Acute RF
- After acute MI (in 2nd or 3rd day)
- Bacterial (Staphaylococcus aureus and Hemophilus influenza), tuberculous and fungal (histoplasmosis, and coccidioidomycosis).
- Others: Acute renal failure, trauma, radiation, drugs (doxorubicin and cyclophosphamide), and collagen disease (SLE and scleroderma)
Symptoms: Chest pain, which is retrosternal, sharp or stabbing in nature, aggravated by movement, lying down and deep breathing, exercise and swallowing. Pain may be relieved by sitting or bending forward. Other symptoms are according to cause [e.g. low grade evening rise of temperature, night sweat and weight loss in tuberculosis (TB)].
Signs: Pericardial rub.
Investigations:
- ECG: ST elevated with upward concavity (chair shaped or saddle shaped)
- Chest X-ray P/A view and echocardiography. CT and cardiac MRI may be done in some cases
- Others: According to suspicion of cause.
Treatment:
- If pain—NSAID (indomethacin or ibuprofen). In severe or recurrent pain, steroid is given
- If no response to steroid: azathioprine or colchicine may be added
- If recurrence with no response to medical treatment—pericardiotomy may be done
- Treatment of primary cause—antibiotic, if bacterial infection. Anti-Koch's, if tuberculosis is suspected.
PERICARDIAL EFFUSION
Accumulation of fluid in the space between parietal and visceral pericardium.
Causes:
- After acute pericarditis (bacterial and viral), and tuberculosis (the most common)
- Collagen diseases (SLE and RA)
- Others: Myxedema, lymphoma, secondary metastasis, renal failure and dialysis, after radiotherapy.
Signs:
- Pulse—low volume, tachycardia, there may be pulsus paradoxus (indicating cardiac tamponade)
- JVP—raised, Kussmaul's sign positive (rise of JVP during inspiration)
- Blood pressure—low systolic, normal diastolic and narrow pulse pressure
- Liver is enlarged and tender.
In precordium:
- Area of cardiac dullness is increased (on percussion)
- Apex beat is difficult to palpate
- Heart sounds are muffled or distant
- Bronchial sound at the left inferior angle of scapula (Ewart sign).
Investigations:
- Chest X-ray (heart is globular and lung fields are oligemic), CBC, ECG, and echocardiogram (shows echo-free zone)
- Paracentesis—to see color, analysis of pericardial fluid [Gram staining, cytology, biochemistry, AFB, culture/sensitivity (C/S), and ADA]
- MRI—very helpful
- Other investigations according to the suspicion of causes.
Confirmation of diagnosis: By echocardiogram (shows the echo free zone). Paracentesis is definitive.
Treatment: According to cause—
- If tuberculosis, antituberculosis drug plus prednisolone
- If bacterial cause is suspected—broad-spectrum antibiotic
- Other treatment of primary cause (e.g. hypothyroidism, SLE, RA and lymphoma)
- Paracentesis, if cardiac temponade develops.
CARDIAC TAMPONADE
It is a state of compression of heart in rapidly developing pericardial effusion. It interferes with diastolic filling of heart and the patient develops features of shock.
Causes:
- Trauma or cardiac surgery (causing hemopericardium)
- Malignancy (repeated effusion may occur)
- Myocardial rupture
- Dissecting aortic aneurysm
- Any cause of pericardial effusion can cause.
Symptoms: Heaviness and compression in chest, dyspnea and features of shock.
Signs: See above in pericardial effusion.
Treatment: Immediate pericardiocentesis and treatment of primary cause.
CHRONIC CONSTRICTIVE PERICARDITIS
It is a disease characterized by progressive thickening, fibrosis and calcification of pericardium.22
Causes:
- Infection: TB, coxsackie B virus and histoplasmosis
- Hemopericardium (due to trauma and myocardial rupture after infarction or dissecting aneurysm)
- Collagen disease (RA)
- Others: Cardiac operation, mediastinal irradiation and idiopathic, rarely after acute purulent pericarditis.
Symptoms: Cough, breathlessness on exertion, may be orthopnea and paroxysmal nocturnal dyspnea. Weakness, dizziness, giddiness, anorexia, nausea and vomiting. Abdominal swelling and later ankle swelling.
Signs:
- Tachycardia and low-volume pulse. Pulsus paradoxus may be present
- JVP—raised, fall of Y descent (Friedreich's sign). Kussmaul's sign is positive (raised JVP on inspiration)
- Pericardial knock (a third heart sound due to rapid ventricular filling)
- Enlarged tender liver and ascites
- Peripheral edema later on.
Complications: Atrial fibrillation (in 30% cases), ascites and myocardial fibrosis.
Investigations:
- Chest X-ray (pericardial calcification in 50% cases) and ECG
- Echocardiogram, CT scan or CMR. Cardiac catheterization is done in some cases
- Other investigations according to suspicion of cause [Mantoux test (MT), RA and antinuclear antibodies (ANA)].
Treatment: Complete resection of pericardium. Treatment of primary cause should be done.
MYOCARDITIS
It is the inflammation of the myocardium of heart.
Causes:
- Infection: Viral (coxsackie A and B and influenza A and B), bacterial (Streptococcus, Pneumococcus, and Lyme disease), protozoal (Chagas disease), and fungal (Candida and Actinomyces)
- Rheumatic fever
- Diphtheritic myocarditis
- Others: Drugs and toxins (doxorubicin and lithium), radiation and autoimmune (SLE and RA).
Symptoms: Palpitation, breathlessness and chest pain. Features of cardiac failure or primary disease, if any.
Signs:
- Pulse—low volume and tachycardia
- Apex beat may be displaced downward and outward due to cardiomegaly
- First and second heart sounds may be soft. Third or fourth heart sound may be present
- Features of heart failure and arrhythmia may be present.
Investigations:
- Chest X-ray, ECG, echocardiography and MRI
Treatment: Complete bed rest and low-salt diet. Diuretics and ACE inhibitors may be helpful. Treatment of arrhythmia. Treatment of primary cause, if any.
ENDOCARDITIS
It is the infection of endocardium, mainly the lining of chamber or heart valve or in congenital anomaly, usually occurs at the site of preexisting heart disease or septal defect. Infection with virulent organism may cause acute endocarditis in normal heart (e.g. by Staphylococcus aureus).
Acute Endocarditis
It is usually caused by highly virulent and invasive organism (S. aureus, Streptococcus and Pneumococcus). It can affect damaged as well as normal heart. Vegetations are usually very large and valve destruction is more than in subacute endocarditis.
Symptoms: Fever, usually very high with chill and rigor. Headache, bodyache, malaise, weakness, chest pain and breathlessness.
Signs: Patient looks toxic with very high temperature. Prominent and changing heart murmur. Stigmata of subacute or chronic endocarditis are usually absent.
Treatment: Vancomycin (1 g twice daily IV) and gentamicin (1 mg/kg twice daily IV).
Subacute Bacterial Endocarditis
It is usually caused by organisms of low virulence, affecting rheumatic or congenitally abnormal valves.
Predisposing factors or causes of SBE:
- Rheumatic valve lesion (e.g. AR and MR)
- Congenital heart disease (VSD, PDA, bicuspid aortic valve, coarctation of aorta and TOF)
- Prosthetic valve
- Dental extraction
- Instrumentation (IV cannula, central venous (CV) line, and cardiac catheterization)
- Cardiac surgery
- IV drug abuse (right-sided endocarditis is more common, especially tricuspid valve).
Organisms causing infective endocarditis:
- Subacute bacterial endocarditis:
- Streptococcus viridans: Most common (35–50%)
- Enterococcus faecalis and Enterococcus faecium
- Streptococcus bovis (associated with large bowel carcinoma), Streptococcus milleri and other Streptococci
- Staphylococcus aureus or epidermidis
- HACEK organisms (Haemophilus, Actinobacillus, Cardiobacterium hominis, Eikenella and Kingella).
- Acute bacterial endocarditis: S. aureus (common). Others—Pseudomonas, Candida, Streptococcus pneumoniae, and Neisseria gonorrhoeae
Symptoms of SBE:
- Fever, usually low grade and continuous, is persistent and does not respond to usual antibiotics
- Chest pain and palpitation, difficulty in breathing. Anorexia, weight loss, malaise, weakness, night sweat and arthralgia
- Symptoms of embolism according to involvement like brain (CVD), kidney (renal infarction), and lung (pulmonary infarction).
Signs:
General examination:
- Appearance: Ill-looking, emaciated and toxic, and anemia
- In hands:
- Clubbing involving all fingers and toes
- Osler's node (small painful violaceous raised nodule, present on the tip of the fingers)
- Janeway lesion (large painless erythematous macules on the palm and sole)
- Infarction at the tip of fingers or toes, petechiae on the dorsum or other parts
- Splinter hemorrage (subungual)
- Infarction due to embolism.
- Pulse—tachycardia, BP—may be low
- Precordium
- Signs of previous heart disease (AR, MR, ASD, VSD and PDA)
- Murmur—appearance of new murmur or changing character of previous murmur.
- Abdomen: Splenomegaly may be present
- Fundoscopy: Roth's spot (white-centered retinal hemorrhage).
Investigations:
- CBC, ESR (high) and CRP (high)
- Blood C/S (both aerobic and anaerobic): Three samples from different sites at 1 hour apart
- Echocardiography (preferably transesophageal echocardiography) to see vegetation, valvular lesion or congenital anomaly
- Urine examination (R/M) (hematuria and proteinuria may be present). Serum urea and creatinine
- Chest X-ray (shows cardiomegaly) and ECG.
Complications of SBE:
- Heart failure (LVF is a common cause of death), valve destruction, regurgitation and obstruction
- Aortic root abscess and systemic embolism
- Right-sided endocarditis involves the pulmonary valve and may cause septic pulmonary emboli, occasionally infarction and lung abscess.
Causes of noninfective endocarditis:
- Libman–Sacks (nonbacterial verrucous endocarditis in SLE)
- Marantic endocarditis (nonbacterial thrombotic or verrucous endocarditis found in malignancy, such as bronchial carcinoma).
- For Viridans streptococci: Benzyl penicillin 1.2 g IV 4 hourly and gentamycin 1 mg/kg IV 8 hourly for 4 weeks or ceftriaxone 2 g once daily IV or vancomycin 15 mg/kg IV 12 hourly for 4 weeks
- In penicillin allergy or methicillin resistant Staphylococcus aureus (MRSA): Triple therapy with vancomycin, gentamycin with oral rifampicin. Or another regimen—Vancomycin 1 g 12 hourly IV with ceftriaxone 2 g every 24 hours
- In prosthetic valve endocarditis: IV penicillin 6 weeks and IV gentamicin 2 weeks
- For HACEK organisms: Ceftriaxone 2 g IV once daily for 4 weeks. If prosthetic valve is involved, then treatment should be given for 6 weeks.
- Q fever endocarditis: Prolonged treatment with doxycycline and rifampicin or ciprofloxacin.
Prevention during dental procedure: Antibiotic is given in high risk cases, such as prosthetic heart valve, previous infective endocarditis, and congenital heart disease.
Drugs used for prophylaxis:
- Amoxicillin 2 g 1 hour before procedure
- If penicillin allergy: Clindamycin 600 mg or cephalexin 2 g or azithromycin or clarithromycin 500 mg 1 hour before procedure.
CARDIOMYOPATHY
Cardiomyopathies are a group of diseases involving the heart muscle and not due to congenital, valvular, hypertension and coronary arterial or pericardial abnormalities.
Three types: (1) Hypertrophic, (2) dilated (ischemic), and (3) restrictive cardiomyopathy.
Hypertrophic cardiomyopathy (HCM): It is a disease of heart muscle characterized by hypertrophy of cardiac muscle with misalignment of cardiac fibers. Hypertrophy may be generalized or localized to the interventricular septum (asymmetrical septal hypertrophy) or other regions (apical hypertrophic cardiomyopathy).
Types are:
- Asymmetrical septal hypertrophy (70%)
- Basal septal hypertrophy (15–20%)
- Concentric (8–10%)
- Apical or lateral wall (<2%).
Symptoms: May be asymptomatic. May be angina or breathlessness or presyncope or syncope on exertion, palpitation and sudden death.
Signs: Pulse—Carotid pulse is jerky. BP—low systolic, normal diastolic and narrow pulse pressure.
Precordium:
- Palpation: Apex beat—heaving (may be double apical impulse). Systolic thrill may be palpable at apex
- Auscultation: Ejection systolic murmur at left lower sternal border. Pansystolic murmur at the apex due to mitral regurgitation.
Treatment:
- In nonobstructive case: Beta-blocker and rate-limiting calcium channel blocker (verapamil and diltiazem). Amiodarone may be helpful in arrhythmia
- In left ventricular outflow obstruction: Dual chamber pacing may be needed. Partial surgical resection (myectomy) may be needed
- Other treatment: Implantable cardioverter-defibrillator (ICD) (if clinical risk factors for sudden death). Cardiac transplantation may be needed in congestive heart failure (CHF) not responding to treatment. Infective endocarditis prophylaxis may be needed.
Advice to be given in HCM:
- Vigorous exercise and dehydration should be avoided
- Genetic counseling, as in 50% cases, may be inherited as autosomal dominant
- First-degree family members should be screened by echocardiogram.
Drugs to be avoided in HCM: Digoxin, vasodilators, diuretics, nitrates, dihydropyridine calcium channel blockers, and alcohol (may cause vasodilatation).
Dilated cardiomyopathy (DCM): It is characterized by dilatation and impaired contraction of left and sometimes right ventricle causing progressive left-sided and later right-sided heart failure. Functional mitral or TR may occur.
Causes:
- Alcohol
- 25% cases are inherited as autosomal dominant trait
- Autoimmune reaction to viral myocarditis
- Ischemic heart disease
- Nutritional—thiamine (Vitamin B1) deficiency
- Others: Muscular dystrophies (Duchenne or Becker thyrotoxicosis), pregnancy, infiltrative disease (hemochromatosis and sarcoidosis), and idiopathic in many cases.
Symptoms: Breathlessness on exertion. Features of heart failure (palpitation, swelling of legs and fatigue), sporadic chest pain and sudden death.
Signs: Signs of cardiac failure (left or right or biventricular) and arrhythmia.
Investigations: ECG, chest X-ray and echocardiography.
Treatment: Mainly of heart failure.
- Rest, salt and fluid restriction, and avoid exercise
- Medical therapy: β-blockers, ACE inhibitors or angiotensin receptor blocker, diuretics and nitrates. Anti-arrhythmic drugs, if arrhythmia (amiodarone)
- In some patients—implantation of ICD
- Treatment of primary cause, if any
- Cardiac transplantation may be indicated.
Restrictive cardiomyopathy (RCM): Rare, ventricular filling is impaired, as ventricles are “stiff”. This leads to high atrial pressures with atrial hypertrophy, dilatation and later AF.
Treatment: Symptomatic but prognosis is usually poor and transplantation may be indicated.
Postpartum cardiomyopathy: If any patient develops cardiac failure in last trimester of pregnancy or within 6 months after delivery in absence of previous heart disease, it is called postpartum or peripartum cardiomyopathy. It is a type of dilated cardiomyopathy. Cause is unknown. Immune and viral causes are postulated. Other factors are advanced age, multiple pregnancy, multiparity, and hypertension in pregnancy. Commonly occurs immediately after or in the month before delivery (peripartum).
Symptoms: Common in multipara, age above 30 years. Respiratory distress, orthopnea and heart failure. Cough with frothy sputum due to pulmonary edema.
Signs: Signs of heart failure. AF or other arrhythmia may occur.
Diagnostic criteria (four criteria):
- Presentation in last month of pregnancy or within 6 months of delivery
- Absence of an obvious cause for heart failure
- Previously normal cardiac status
- Echocardiographic evidence of systolic LV dysfunction.
Treatment: Symptomatic for heart failure (diuretics, ACE inhibitor, and digoxin). Beta-blocker may be helpful in some cases. Inotropic agent may be given.
ARRHYTHMIA
It is defined as the disorder of rate, rhythm and conduction of cardiac impulse.
Types:
- Impulse arising from sinoatrial (SA) node: Sinus arrhythmia, sinus tachycardia, sinus bradycardia, and sick sinus syndrome
- Impulse arising from atria: AF, atrial flutter, atrial tachycardia and atrial ectopics
- Impulse arising from AV junction or nodal: Nodal or junctional rhythm, nodal tachycardia, and nodal ectopics
- Impulse arising from ventricles: Ventricular tachycardia, VF and ventricular ectopics
- Heart block: SA block, AV block, and bundle branch block [left bundle branch block (LBBB) and right bundle branch block (RBBB)].
Sinus arrhythmia: It is an arrhythmia in which heart rate increases in inspiration and decreases in expiration. It is a benign condition, common in children and young adults, sometimes in healthy old person. ECG findings—PP or RR interval (Short in inspiration and long in expiration).
Sinus tachycardia: When heart rate is more than 100/min in sinus rhythm. Causes:
- Physiological: Anxiety, emotion, exercise, pain and pregnancy
- Pathological: Anemia, fever, thyrotoxicosis, shock (except vasovagal attack, in which bradycardia is present), heart failure, bleeding, hypovolemia, chronic constrictive pericarditis, acute anterior MI, and drugs (salbutamol, propantheline, and thyroxine).
Treatment: If symptomatic—beta-blockers (propranolol and atenolol) or calcium channel blocker (verapamil). Treatment of primary cause if any.
Sinus bradycardia: When heart rate is less than 60/min in sinus rhythm. Causes:
- Physiological (due to increased vagal tone): Athlete and sleep. Occasionally, healthy elderly
- Pathological: Hypothyroidism, hypothermia, raised intracranial pressure, drugs (digoxin, β-blockers and verapamil), acute inferior MI, obstructive jaundice, and hypokalemia.
Causes of bradycardia:
- Sinus bradycardia due to any cause
- Second-degree heart block (Mobitz type II) and CHB
- Nodal rhythm, idioventricular rhythm and drugs (β-blocker, digoxin).
Treatment: Treatment of primary cause.
SICK SINUS SYNDROME
It is the dysfunction of SA node characterized by sinus bradycardia, sinus arrest or junctional rhythm which may lead to dizziness or syncope, followed by episodes of paroxysmal tachycardia, so called tachy-brady syndrome. It is due to fibrosis, degenerative changes or ischemia of SA node. Probable causes are elderly, IHD, drug (digoxin), cardiomyopathy, rheumatic heart disease and idiopathic.
Symptoms: May be asymptomatic. May be dizziness, syncope and palpitation.
Signs: Pulse (bradycardia or tachycardia or drop beat). Features of primary disease.
Investigations: ECG and Holter monitoring (single ECG may sometimes be normal).
Treatment: If asymptomatic—no specific therapy. Follow-up. If symptomatic—permanent dual chamber pacemaker. Anti-arrhythmic drug may be required.
Supraventricular tachycardia (SVT): It is a type of tachycardia that occurs due to reentry or rapidly firing ectopic focus in atria or AV node.
Causes:
- Physiological: Anxiety, tension, tea, coffee and alcohol
- Pathological: Thyrotoxicosis, IHD, Wolff–Parkinson–White (WPW) syndrome and digitalis toxicity.
Symptoms: Palpitation, dizziness, syncope, breathlessness, and chest pain. Polyuria after the attack.
Complications: Short diastolic filling time, so less stroke volume and precipitate heart failure.
Treatment:
- Rest. Carotid sinus massage or Valsalva maneuver. It acts by increasing the vagal tone
- If no response:
- Or IV verapamil 10 mg slowly over 5–10 minutes (verapamil should be avoided if QRS >0.12 second or history of WPW syndrome or if the patient is on β-blocker).
- Other drugs: β-blocker or digoxin may be used
- If patient is hemodynamically unstable (hypotension and pulmonary edema): Direct current (DC) shock
- If attack is frequent: Prophylactic oral β-blocker, verapamil or digoxin may be given
- In WPW syndrome: Transvenous radiofrequency catheter ablation is the treatment of choice
- In some cases—antitachycardia pacing is done (overdrive atrial pacing).
WPW SYNDROME
It is a syndrome in which there is an accessory pathway that bypasses AV node and connects the atrium and ventricle (by bundles of Kent).
It is of two types:
- Type A: Accessory pathway on left side (in ECG, tall R in V1 and V2)
- Type B: Accessory pathway on right side (in ECG, deep Q in V1 and V2).
Clinical features: May be asymptomatic. May present with palpitation, paroxysmal attack of atrial or SVT, and AF. Syncope and sudden death (due to AF). Rarely, features of ventricular tachycardia and VF.
Investigations: ECG shows short PR interval (<0.12 second) and wide QRS. Delta wave in the upstroke of QRS (slurred QRS). Q wave may be present in lead II, III, and a VF (confused with inferior MI). Electrophysiological study may be done.
Treatment: If asymptomatic—no treatment is required. If symptomatic, transvenous radiofrequency catheter ablation of accessory pathway is the specific treatment. If not available, prophylactic anti-arrhythmic drug should be given (β-blocker and amiodarone). Surgical resection of accessory pathway may be done.
Drugs to be avoided in WPW syndrome: Digoxin, and IV verapamil. These shorten refractory period of accessory pathway.
ATRIAL FIBRILLATION
It is an arrhythmia where atria beat rapidly, chaotically, and ineffectively, while the ventricles respond at irregular intervals, producing the characteristic irregularly irregular pulse.
Clinically, five types:
- First detected—not diagnosed previously, irrespective of duration or severity of symptoms
- Paroxysmal: Self-limiting and stops spontaneously within 7 days
- Persistent—continuous >7 days
- Long-standing persistent—continuous >1 year
- Permanent—continuous, with a joint decision between the patient and the physician to cease further attempts to regain sinus rhythm.
According to heart rate, two types:
- Fast AF: Heart rate >100 beats/min
Causes:
- Chronic rheumatic heart disease with valvular lesions, commonly MS
- Coronary artery disease (commonly, acute myocardial infarction)
- Thyrotoxicosis
- Hypertension
- Lone AF (idiopathic in 10%)
- Others: ASD, chronic constrictive pericarditis, acute pericarditis, cardiomyopathy, myocarditis, sick sinus syndrome, coronary bypass surgery, pneumonia, thoracic surgery, electrolyte imbalance (hypokalemia and hyponatremia), alcohol, and pulmonary embolism.
Complications: Systemic and pulmonary embolism (systemic from left atrium and pulmonary from right atrium) and heart failure.
Symptoms: Palpitation, breathlessness and weakness.
Signs:
- Pulse: Irregularly irregular (irregular in rhythm and volume)
- Examination of heart (heart rate to see pulsus deficit, mitral valvular or other cardiac disease)
- Thyroid status (warm sweaty hands, tremor, tachycardia, exophthalmos and thyroid gland size)
- Check BP in hypertensive case.
Investigations:
- ECG shows absent P wave (replaced by fibrillary “f” wave) with irregularly irregular (R-R interval is irregular)
- Chest X-ray and echocardiography. Thyroid function test, if thyrotoxicosis is suspected.
Treatment:
Aims of treatment: Control of heart rate, restoration of sinus rhythm, prevention of recurrence, and treatment of primary cause.
- To control rate—digoxin, β-blocker or calcium channel blocker (verapamil or diltiazem) may be given
- If no response, cardioversion (medical or DC shock) should be done as follows:
- Intravenous anti-arrhythmic drug such as flecainide, propafenone, vernakalant or amiodarone. Oral flecainide or propafenone may be given
- If no response—DC shock should be given.
- Long-term treatment (according to types):
- Paroxysmal AF:
- If asymptomatic: No treatment. Treatment of primary cause, regular follow-up
- If symptomatic: β-blocker. If no response, flecainide or propafenone may be given. Amiodarone is effective in prevention. Low-dose aspirin to prevent thromboembolism
- If bradycardia (SA disease): Permanent overdrive atrial pacing
- In intractable cases: Radiofrequency ablation may be done, if no structural heart disease.
- Persistent AF:
- To control rhythm: DC cardioversion. May be repeated, if relapse
- β-blocker or amiodarone may be used to prevent recurrence
- If no response—transvenous radiofrequency ablation may be done.
Lone AF: It means AF without any cause. Usually life span is normal.
ATRIAL FLUTTER
It is characterized by rapid atrial rate associated with 2:1, 3:1, 4:1 or more AV block.
Causes: These causes are like atrial fibrillation (AF). AF and flutter may be present together, it is called flutter-fibrillation.
Symptoms: Palpitation, breathlessness, fatigue, weakness, light headedness, dizziness and even syncope.
ECG findings: Saw-tooth appearance of P wave (normal P is replaced by flutter or F wave). RR is regular (may be irregular, when there is variable block).
Treatment: To control heart rate, digoxin, β-blocker or verapamil. β-blocker or amiodarone can be used to prevent recurrence. If no response, DC cardioversion or atrial overdrive pacing may be done. In persistent or troublesome symptoms, radiofrequency catheter ablation.
Ectopics: Ectopic beat or extrasystole is a premature beat which arises from other than SA node and comes earlier than normal beat. It arises from abnormal focus such as atria, AV node or ventricle.
Types: Atrial, nodal, and ventricular.
Atrial ectopics: May occur in normal people. Other causes are, excess tea, coffee and smoking. Any organic heart disease (myocarditis and cardiomyopathy), electrolyte imbalance, and COPD.
ECG findings: P (small or inverted), PR interval (short) and PP interval (irregular).
Ventricular ectopics: It may be unifocal, multifocal, bigeminy, trigeminy, and quadrigeminy.
Causes:
- Normally in young adults, also in anxiety, excess caffeine, and alcohol
- Myocarditis and cardiomyopathy
- Valvular heart disease and MVP
- Others: Hypertensive heart disease, hypokalemia, digoxin toxicity and hypoxemia.
Treatment: In absence of any heart disease and asymptomatic case, no treatment is necessary. β-blocker may be used. If organic heart disease, treatment of primary cause. Anti-arrhythmic drug does not improve, may even worsen the prognosis.
Ventricular tachycardia (VT): It is defined as three or more consecutive ectopic beats and heart rate usually 140–220 beats/min with regular rhythm. It may be sustained or nonsustained.
Causes:
- Acute MI
- Chronic IHD (especially with poor LV function)
- Others: Ventricular aneurysm, MVP, hypokalemia, hypomagnesemia, and idiopathic.
Symptoms: Palpitation, dyspnea, dizziness and giddiness.
Treatment:
- If patient is hemodynamically unstable (such as hypotension, systolic BP <90 mm Hg or heart failure): DC shock. If hemodynamically stable, IV amiodarone bolus followed by IV infusion. If fails, DC shock should be done
- To prevent recurrence: β-blocker and oral amiodarone may be used. Correction of hypokalemia, hypomagnesemia, hypoxemia and acidosis should be done
- If all fail: Implantable cardioverter-defibrillator or radiofrequency ablation of focus.
Ventricular fibrillation: It is characterized by rapid, irregular, ineffective, and uncoordinated ventricular activation with no mechanical effect. There is chaotic electrical disturbance of ventricles, with impulse occurring irregularly at 300–500/min. Cardiac output falls to zero. It is the most common cause of sudden death. It may occur as a primary arrhythmia or as a complication in acute MI.
Causes: Acute MI, and electrolyte imbalance (hypokalemia and hypomagnesemia). Others—Electrocution, drowning, and drug overdose (digitalis, adrenaline and isoprenaline).
Signs: Patient is unconscious. Pulse is absent and BP is not recordable. Respiration is absent. Pupils are dilated and less or no reaction to light. Heart sounds are absent.
Treatment:
- Immediate defibrillation—200 Joules. If no response, another shock with 200 Joules. If still no response, another shock with 360 Joules. If three shocks unsuccessful—adrenaline IV, followed by cardiopulmonary resuscitation
- If defibrillator is not available—cardiopulmonary resuscitation should be given
- The patient who survives from VF in absence of any cause is at high risk of sudden death. It is treated with ICD.
HEART BLOCK
It is defined as defect in either initiation or conduction of cardiac impulse.
Sites: SA node, AV node, bundle of His and branches of bundle of His (left and right).
Types:
- SA block
- Atrioventricular block. It is of three types:
- First-degree AV block
- Second-degree AV block. Two types: (i) Mobitz type I (Wenckebach's phenomenon) and (ii) Mobitz type II
- Complete heart block or third-degree heart block.
Hemiblock: It means when there is block involving one of the fascicles of left bundle branch.
There are two types:
- When left axis deviation, it is called left anterior hemiblock
- When right axis deviation, it is called left posterior hemiblock.
Note: There may be two or three blocks. In such case, it is called bifascicular or trifascicular block.
Sinoatrial block: Failure to initiate an impulse from SA node.
Causes: Degenerative changes in elderly, IHD (involving SA node), drugs (digoxin), and increased vagal tone.
Symptoms: May be asymptomatic. May be dizziness and giddiness.
Signs: Drop beat and no heart sound at the time of drop beat.
Investigations: ECG and Holter monitoring may show the block.
Treatment: No treatment, if asymptomatic. Withdrawal of offensive drug, if any. If syncopal attack or sick sinus syndrome, permanent pacemaker should be given.
First-degree AV Block: It is the prolongation of PR >0.22 second
Causes: Normally in athlete (due to increased vagal tone), drugs (digitalis toxicity), acute MI (common in inferior MI), acute rheumatic carditis, in elderly (atherosclerosis), and hyperkalemia.
Symptoms: Usually asymptomatic. ECG shows prolonged PR interval, >0.22 second.
Treatment: No specific treatment is necessary.
Second-degree AV Block: Three types: (1) Mobitz type I (Wenckebach phenomenon), (2) Mobitz type II, and (3) 2:1 or 3:1 heart block.
Mobitz type I (Wenckebach phenomenon): Progressive prolongation of PR interval followed by drop beat.
Site of block: Higher area of AV node (proximal to bundle of His).
Causes: Physiological—in athlete, during rest, sleep (due to increased vagal tone), digoxin toxicity and acute MI (commonly inferior).
Symptoms: Usually asymptomatic, may be features of primary disease.
Sign: Pulse is irregular (drop beat occurs).
ECG findings: Progressive lengthening of PR interval followed by absent QRS complex.
Treatment: No treatment is necessary. Primary cause should be treated.
Mobitz type II: Site of lesion is in His-Purkinje system.
Cause: Acute anterior MI.
ECG: Some P waves are not followed by QRS, PR and PP interval is constant, and wide QRS.
(In 2:1 AV block, alternate P wave is conducted. It may be 3:1, 4:1). This type of AV block is rare and more severe. It is generally a sign of severe conduction system disease.
Treatment:
- If due to anterior MI: Temporary pacing followed by permanent pacemaker.
COMPLETE HEART BLOCK
No impulse from atria transmitted to the ventricles. So ventricles generate their own rhythm.
Causes:
- Acute CHB: Acute MI (commonly inferior)
- Chronic CHB:
- Progressive fibrosis of distal His-Purkinje system (Lev's disease) in elderly
- Progressive fibrosis of proximal His-Purkinje system (Lenegre's disease) in younger
- Other causes: Cardiomyopathy, myocarditis, drugs (digoxin, β-blocker, amiodarone), cardiac surgery (aortic valve replacement, VSD repair), radiofrequency AV node ablation, infiltrative disease (sarcoidosis, amyloidosis, and hemochromatosis), infection (infective endocarditis, Chagas disease, Lyme disease), collagen disease (SLE and RA), congenital CHB [common in child of mother with SLE due to transplacental transfer of anti-Ro antibody/anti-Sjögren's-syndrome-related antigen A (SSA)].
Symptoms: Weakness, dizziness, giddiness, syncope (Stokes Adams attack), and breathlessness on exertion.
Signs:
- Pulse: Bradycardia, 20–40 beats/min (<40 beats/min), high volume, does not increase by exercise or injection atropine
- BP: High systolic, normal diastolic, and high pulse pressure
- Neck vein: Cannon waves (large “a” wave) may be present
- Heart sounds: Variable intensity of first heart sound
- Murmur: Systolic flow murmur.
ECG criteria: Atrial rate more, ventricular rate <40. PP interval is constant. No relationship between P wave and QRS complex (PR looks variable).
Treatment: If the patient is symptomatic, permanent pacemaker should be given. In congenital CHB, pulse rate is high, and no pacemaker is necessary.
Stokes–Adam attack: Syncope or blackout in patient with CHB due to ventricular asystole.
Symptoms and signs:
- Syncope or blackout with or without preceding dizziness. During attack, the patient is unconscious, looks pale and may have convulsion. If asystole persists, there may be cyanosis, absent pulse, fixed and dilated pupil, and incontinence of urine. Plantar is extensor
- Usually consciousness recovers rapidly followed by flushing.
Treatment: Permanent pacemaker.
RIGHT BUNDLE BRANCH BLOCK
ECG criteria: RSR in V1 and V2 (M pattern). QRS (wide, >0.12 second).
Causes:
- Normal variant (common)
- Atrial septal defect
- Others: Right ventricular hypertrophy, chronic cor pulmonale, pulmonary embolism, cardiomyopathy, and conduction system fibrosis.
Fascicular block (hemiblock): Two types—
- Right bundle branch block with left anterior hemiblock (block in anterior fascicle). ECG shows RBBB with left axis deviation
- Right bundle branch block with left posterior hemiblock (block in posterior fascicle). ECG shows RBBB with right axis deviation.
LEFT BUNDLE BRANCH BLOCK
Causes: Severe coronary artery disease, acute MI, cardiomyopathy, myocarditis, aortic valve disease (stenosis or regurgitation), LV hypertrophy, and hypertension.
Symptoms: Features of primary disease.
Signs: On auscultation, there is reverse splitting of second heart sound.
Treatment: Treatment of primary cause. In acute MI, if new LBBB occurs and temporary pacemaker is indicated.
TAKAYASU'S DISEASE
It is a chronic, inflammatory, and granulomatous panarteritis of unknown cause involving the elastic arteries commonly aorta and its major branches such as carotid, ulnar, brachial, radial, and axillary. Occasionally, it may involve pulmonary artery, rarely abdominal aorta, and renal artery resulting in obstruction. It is also called pulseless disease or aortic arch syndrome.
Types: There are four types—
- Type 1: Involves aortic arch and its major branches
- Type 2: Involves descending aorta and abdominal aorta
- Type 3: Involves both type 1 and type 2. This may be complicated by aortic regurgitation
- Type 4: Involves the pulmonary arteries.
Clinical features: Common in young female, 25–30 years (F:M ratio is 8:1), more in Asians.
- In acute stage—may present with fever, malaise, weight loss, arthralgia, myalgia and high ESR
- In chronic case—dizziness, giddiness, headache, blurring of vision, syncope and claudication of upper limb. There may be features of aortic regurgitation, renal artery stenosis or anginal pain, and hypertension.
Signs: All pulses of upper limbs are absent, but present in lower limbs. BP is undetectable in upper limb and normal or high in lower limb. Bruit may be present over the carotid, also renal bruit. Fundoscopy (shows wreath-like anastomosis around the optic disk).
Investigations:
- Complete blood count (high ESR and normocytic normochromic anemia), and CRP (high)
- Chest X-ray shows cardiomegaly and widening of aorta
- CT angiography—helpful to detect stenosis, occlusion and condition of arteries
- Aortography of aortic arch and its branches (shows narrowing, coarctation and aneurysmal dilatation)
- Serum immunoglobulin—high.
Treatment:
- Prednisolone 40–60 mg daily or 1–2 mg/kg. If difficult to taper, or in refractory case, methotrexate 25 mg/week may be given with prednisolone. Or methotrexate, mycophenolate mofetil or azathioprine may be added with prednisolone
- Cyclophosphamide may be used in resistant case
- Anti-TNF agents such as etanercept and infliximab may be given in relapse
- Reconstructive vascular surgery in selected case
- Angioplasty, stenting or bypass surgery may be done, if vascular complication occurs
- Treatment of hypertension.
Complications: Heart failure, stroke, seizure, organ failure, retinopathy and renovascular hypertension.
PACEMAKER
It is an artificial device used to electrically stimulate the heart. It is composed of two parts—battery powered generator and wire electrode (attached to the heart chamber to be stimulated, atrium or ventricle or both).
Pacemaker may be single chamber or dual chamber. Two types—Temporary and permanent.
Indications of temporary pacemaker:
- Acute inferior MI with AV block or severe bradycardia with hemodynamic change
- Acute extensive anterior MI with second- or third-degree AV block or new bifascicular block
- Atrioventricular reentry tachycardia and ventricular tachycardia can be terminated by overdrive pacing.
Indications of permanent pacemaker: Commonly CHB with syncope or Stokes Adams syndrome and sick sinus syndrome.
ECG findings (atrial pacing): There is a spike followed by P wave.
ECG findings (ventricular pacing): There is a spike followed by wide QRS (looks like LBBB).
Complications of pacemaker:
- Early: Pneumothorax, infection, lead displacement, cardiac tamponade and pocket hematoma
- Late: Infection, erosion of generator or lead, chronic pain at implant site, lead fracture, malfunction, perforation of ventricular wall, ventricular arrhythmia [premature ventricular contraction (PVC)], electromagnetic interference, pacemaker failure, pacemaker-mediated tachycardia (by dual chamber pacing), and pacemaker syndrome (by single-chamber pacing).37
PULMONARY HYPERTENSION
Mean pulmonary arterial pressure >25 mm Hg at rest and >30 mm Hg during exercise.
Types:
- Primary or idiopathic pulmonary hypertension—no underlying cause. May have genetic predisposition
- Secondary pulmonary hypertension—more common than idiopathic pulmonary hypertension.
Causes:
- Respiratory: COPD, emphysema, chronic bronchitis, diffuse parenchymal lung disease (DPLD), and pulmonary thromboembolism
- Cardiac: Left-sided heart failure, MS and reversal of shunt (ASD, VSD and PDA)
- Connective tissue disorders: Scleroderma and SLE
- Sleep apnea syndrome and other sleep disorders
- Drugs: Cocaine.
Symptoms: Asymptomatic. May be shortness of breath (orthopnea or paroxysmal nocturnal dyspnea), chest pain, palpitations, cough, and hemoptysis (rarely). Fatigue, ankle swelling, dizziness and syncope.
Signs:
- Epigastric pulsation (indicates RVH)
- Palpable P2, prominent “a” wave in JVP, and left parasternal heave (indicates RVH)
- Loud P2 on auscultation, and early diastolic murmur [Graham Steel murmur due to pulmonary regurgitation (PR)].
Complications: Right-sided heart failure and cor pulmonale, and arrhythmia.
Investigations:
- CBC and ESR
- Chest X-ray P/A view (shows enlargement of pulmonary arteries) and CT scan of chest
- ECG [shows RVH and right atrial hypertrophy (RAH)], and Doppler echocardiography
- Pulmonary function test and ventilation-perfusion (V/Q) scan
- Open lung biopsy and genetic tests
- Other investigations according to suspicion of cause.
Treatment: Reduction of weight, avoid heavy exercise and smoking must be stopped. Oxygen inhalation.
- Diuretics, digoxin, and vasodilator drugs (ACE inhibitors, sildenafil and tadalafil)
- Other drugs: Endothelin receptor antagonists (bosentan and ambrisentan), calcium channel blockers (amlodipine and nifedipine), and anticoagulants (warfarin)
- Surgery: Atrial septostomy, heart-lung transplantation in selected cases, and pulmonary thromboendarterectomy.
CARDIAC ARREST
Causes:
- Ventricular fibrillation (most common cause)
- Ventricular tachycardia (pulseless)
- Asystole
- Electromechanical dissociation
- Others: Myocardial rupture, cardiac tamponade, respiratory arrest (loss of breathing function), massive pulmonary embolism, tension pneumothorax, electrocution and drowning.
Clinical features: Sudden collapse and loss of consciousness. No pulse, no BP, and no breathing. Permanent brain damage and death can occur unless the flow of blood to the brain is restored within 5 minutes.
Treatment:
- A: Airway restoration
- B: Breathing should be ensured (mouth to mouth breathing)
- C: Circulation [cardiopulmonary resuscitation (CPR) should be started, 15 compression—2 breaths]
- Precordial thump
- Defibrillation: In ventricular fibrillation
- Other supportive therapy: Injection adrenaline and transvenous pacemaker.
MARFAN'S SYNDROME
It is a connective tissue disorder, inherited as autosomal dominant. Male and female are equally affected. It is characterized by triad of eye, skeletal, and cardiac abnormalities.
- Eye: Blue sclera, subluxation or dislocation of lens (ectopia lentis), iridodonesis (tremor of iris), heterochromia iris (various color of iris), myopia, retinal detachment and glaucoma
- Skeletal: Tall, lean and thin, arachnodactyly, hyperextensibility of joints, high arch palate, kyphosis, scoliosis or both, and pes planus. Pectus excavatum or carinatum or asymmetry of chest. Arm span > height. Lower segment > upper segment
- CVS: AR (due to aortic root dilatation and secondary to cystic medial necrosis involving aorta), MR (with MVP).
Complications: Dissecting aneurysm, infective endocarditis and heart failure.
Investigations: X-ray of chest, ECG, echocardiogram, and CT or CMR (to see aortic dilatation).
Treatment:
- Medical treatment:
- β-blocker: It reduces aortic dilatation and prevents the risk of aortic rupture or dissecting aneurysm. Atenolol is more preferable
- ACE receptor blocker: It prevents aortic root dilatation
- Prophylaxis for infective endocarditis.
- Surgery: Replacement of ascending aorta and aortic valve if progressive dilatation of aorta (>5 cm)
- Advice to patient: Avoid strenuous exercise to prevent aortic dissection. Genetic counselling and orthopedic measures. Regular checkup and echocardiography should be done annually.