Alagic N, Elsayed M, Uygur B, Adarna LG, Chahwala JR, Bhagatwala K, Bulur S, Turaga NSN, Pudussery Kattalan JJ, Nanda NC
A 46-year-old female with shortness of breath on mild exertion. She had one episode of pulmonary edema in the recent past. There is no definite history of rheumatic fever. 2D TTE, 2D TEE and 3D TEE were done.
- What do movies 1–8 show?
- Rheumatic MS
- Lupus erythematosis
- Myxomatous MV
- Severe MV annular calcification producing stenosis
The mitral valve (arrow) shows diastolic dooming with a characteristic hockey stick appearance (
1, 4 and 5). Mitral valve (MV) area by Doppler pressure half time (PHT) method measured 0.94 cm2 consistent with severe stenosis. The most common etiology is rheumatic fever. The LA appendage appears clear on 2D TTE (
2) and this is confirmed by 2D TEE (
7). TR gradient measured 40 mm Hg by TEE suggestive of mild pulmonary hyper-tension (
6). 3D TEE (
8) shows a narrow MV orifice with commissural fusion indicative of severe stenosis.
The patient underwent successful percutaneous MV annuloplasty.
MOVIES 1 TO 8
Roomi AU, Shah A, Siddiqui LI, Elsayed M, Bulur S, Nanda NC
A young female with history of rheumatic heart disease in the past who is now presenting with shortness of breath on mild exertion. 2D TEE is done. (
9 and 10A and 10B and Figs. 2.1 and 2.2A and 2.2B).
- In a patient with mitral stenosis, all the following are contraindicated for balloon valvuloplasty except:
- Spontaneous echo contrast in LA and LAA
- Thrombus in LA or LAA
- Low velocities in LAA
- Marked thickening of mitral subvalvular apparatus
- Severe MR
Ans. (a) and (c)
9: TTE. Parasternal long axis view. Shows restricted motion of thickened MV leaflets presenting with typical hockey-stick appearance indicative of MS. LA is enlarged. Arrowhead shows thickened but mobile AV leaflets.
10A: TEE shows limited opening of the MV (arrow). Arrowhead points to spontaneous echo contrast (SEC) in the dilated LA;
10B: Transesophageal echocardiography. Arrow points to a thrombus in the LAA.
Fig. 2.1: TTE. Parasternal long axis view. Shows restricted motion of thickened MV leaflets presenting with typical hockey-stick appearance indicative of MS. LA is enlarged. Arrowhead shows thickened but mobile AV leaflets.
Figs. 2.2A and B: TEE. (A) Shows limited opening of the MV (arrow). Arrowhead points to spontaneous echo contrast (SEC) in the dilated LA; (B) Arrow points to a thrombus in the LAA.
Uygur B, Mohamed A, Gupta N, Elsayed M, Nanda NC
TEEs done in three different patients with mitral stenosis (MS) are shown. Mitral valve is only mildly thickened and LAA is clear in the first patient (
11 to 13). In the second patient, ASD created prior to balloon valvotomy is noted. LAA is clear. 3D TEE was also done showing a narrow mitral orifice (
14 to 17). CW Doppler displaying a flat MV diastolic slope is imaged in the third patient (Fig. 3.1).
- All of the following are causes of MS except:
- Mitral valve annular calcification (MAC)
- Rheumatic fever
- Systemic lupus erythematosis
- Turner syndrome
- Large vegetation/tumor
- All of the following are included in the Wilkin's score which is used to determine suitability for percutaneous MV balloon valvuloplasty except:
- MV thickening
- MV mobility
- MV calcification
- Subvalvular thickening
- Calcification of MV commissures
Calcification of mitral commissures, a contraindication for MV balloon valvuloplasty, is a significant omission in the Wilkin's criteria.
- Which of the following can be used to assess MS severity ?
- Continuity equation
- Pressure half time (PHT)
- Proximal isovelocity surface area
- Mean transmitral gradient
- Vena contracta
- All of the above
MOVIES 11 TO 17
Abdullah Al Shafi Majumder, AKM Monwarul Islam
GIANT LEFT ATRIUM
Giant left atrium (LA) is a rare entity with the decreasing incidence of rheumatic heart disease; the reported incidence varies from 0.3–0.6% in those suffering from rheumatic heart disease.1,2 It was traditionally defined as one where the LA touches the lateral chest wall on chest radiograph.1,3 With the advent of echocardiography, it has been re-defined variably as the LA having a cardio-thoracic ratio on chest radiograph of >0.7 combined with an LA diameter of >8 cm4 (or according to some authors >6.5 cm5) on two-dimensional transthoracic echocardiography (2D TTE).5 Giant LA is typically found in patients with rheumatic mitral valve disease, but has also been described in association with mitral valve prolapse, hypertrophic cardiomyopathy, cardiac amyloidosis, heart failure and chronic atrial fibrillation (AF). Giant LA commonly presents with its compression effects on adjacent structures, causing Ortner's syndrome due to left recurrent laryngeal nerve palsy, dysphagia due to esophageal compression, and dyspnea due to atelectasis. Giant LA, specially if accompanied by AF, may facilitate formation of thrombus, or spontaneous echo contrast (SEC), the latter may be the harbinger of the former.
Here, two cases of giant LA have been described, both were in association with predominant mitral stenosis (MS) of rheumatic origin; one showed a large LA mass, either thrombus or myxoma, and the other presented with profuse, dense SEC masking an underlying thrombus in the LA cavity.
A 26-year-old female presented with shortness of breath and cough for 6 months. The pulse was irregularly irregular, the 1st heart sound was of variable intensity, and there was a localized mid diastolic murmur in the apical area. ECG showed AF and right ventricular hypertrophy. 2D TTE as well as 2D transesophageal echocardiography (2D TEE) revealed features of severe MS, giant LA, measuring 10 cm, profuse, dense SEC in LA, but no definite thrombus (Figs. 4.1A to D and
18A to C). For management, initially percutaneous transvenous mitral commissurotomy (PTMC) was thought of, but considering probable technical difficulties in association with a hugely dilated LA, it was abandoned. Peroperatively, during open mitral commissurotomy (OMC), a large amount of thrombus was found in the LA cavity (Fig. 4.1E). Arrowheads in figures and
point to SEC.
Figs. 4.1A to E: Transthoracic echocardiography apical 4-chamber (A), appendicular (B) and left parasternal long axis (C) views showing features of severe mitral stenosis, dilated left atrium and dense, spontaneous echo contrast. (D) Transesophageal echocardiography mid-esophageal view at 32° showing dilated left atrium, dense, spontaneous echo contrast, and appendage free of definite thrombus. (E) Thrombus extracted from left atrium during open mitral commissurotomy. (LA: Left atrium; RA: Right atrium; LV: Left ventricle; RV: Right ventricle; LAA: Left atrial appendage; MV: Mitral valve; TV: Tricuspid valve).
A 32-year-old female presented with shortness of breath and palpitations for 1 year. The pulse was irregularly irregular, the 1st heart sound was of variable intensity, and there was a localized mid-diastolic murmur in the apical area, similar to Case A. 2D TTE revealed features of severe MS, giant LA, measuring 11.2 cm × 12.1 cm, and a large, heterogeneous mass, measuring 6.7 cm × 6.3 cm and 33.1 cm2, attached to the LA wall, most likely a thrombus (Figs. 4.2A to D and
19A and B). For management, surgery was planned. Arrows in figures and
denote the thrombus.
Giant LA was first reported by Owen and Fenton as an autopsy finding in a patient with rheumatic fever in 1901.6 It is most commonly associated with severe, isolated or predominant mitral regurgitation (MR) of rheumatic etiology. In case of isolated MS, giant LA is unusual. Both the two cases presented here have severe MS without significant MR. For giant LA, echocardiography is the imaging technique of choice because of its precision in assessing the heart chambers. Nonetheless, in some cases, echocardiography may not be sufficient to define accurately the atria and adjacent structures; computed tomography and magnetic resonance imaging have been considered as additional techniques for this purpose, particularly in patients with a cardiothoracic ratio > 0.7.7
The enlarged LA is associated with blood stasis and thrombus formation and the presence of AF further increases the risk. The incidence of LA thrombus in patients with MS and AF is 7–38%; such an incidence is directly related to the size of LA.8-10 The prevalence of SEC in LA may be 54–65% in these patients.8,11 Dilated LA in association with predominant regurgitant lesion may actually be less prone to stasis in comparison to that resulting from predominant stenosis. Giant LA from underlying MS (with absence of significant MR) and in association with AF created an optimum milieu for the formation of thrombus in Case A and spontaneous echo contrast in Case B. Proper identification of SEC and LA thrombus is of crucial importance since the presence of the latter but not the former is a contraindication for cardioversion and PTMC. ‘Coumadin ridge’ is a ridge of atrial tissue separating the LA appendage from the left upper pulmonary vein. It can present as a linear structure or even sometimes as a nodular mass that protrudes into left atrium mimicking a thrombus or atrial myxoma. In the past, this structure was often mistaken for thrombus and resulted in the patient being mistakenly prescribed anticoagulation therapy with warfarin (coumadin) and it is because of this it derives its name. Occasionally, extensive SEC can mask a thrombus on 2D TEE as in Case A and in such a case live/real time three-dimensional (3D) TTE/TEE can be helpful in detecting it and also differentiating it from pectinate muscles.14 A disadvantage of both 2D TTE and TEE is that they provide only a thin slice or section of cardiac structures at any given time limiting their utility in comprehensively assessing the LA appendage. On the other hand, both 3D TTE and TEE can encompass the whole extent of the LA appendage in three-dimensions in the acquired data set, which can then be cropped and sectioned systematically at any desired angulation to more definitively look into the interior. Recently, cardiac computed tomography13 and cardiac magnetic resonance14 have been reported to be reliable alternatives to TEE for diagnosing a LA thrombus.
Figs. 4.2A to D: Transthoracic echocardiography off-axis (A) and apical 4-chamber (B, C, D) views showing features of severe mitral stenosis, dilated left atrium and a large, heterogeneous mass (arrow), measuring 6.7 cm × 6.3 cm (C) and 33.1 cm2 (D), attached to the LA wall. (LA: Left atrium; RA: Right atrium; LV: Left ventricle; RV: Right ventricle; MV: Mitral valve).
TEE assessment of LA appendage flow velocities by pulsed-wave Doppler examination can also provide incremental information about the risk of clot formation. Of the typical quadriphasic flow pattern thus derived, the late diastolic emptying velocity or LA appendage contraction flow occurring immediately after the P wave on the ECG is a significant predictor of thromboembolic risk. The contraction velocities of ≤ 20 cm/s (normal peak velocities 50–60 cm/s) have been found to be associated with increased risk of thromboembolism.
There may be more than one correct answer.
- Definition of a giant left atrium includes:
- Diameter of left atrium exceeds twice that of aorta
- The cardiac border touches the lateral chest wall on chest radiograph
- Diameter of left atrium is at least twice that of right atrium
- Cardiothoracic ratio >0.6 on a chest radiograph
- Diameter of left atrium exceeds 80 mm
Ans. (b) and (e)
- Regarding giant left atrium:
- Most commonly seen in rheumatic heart disease
- Isolated or predominant stenotic lesion of mitral valve is the usual cause
- Mitral valve prolapse may be a cause
- May lead to Ortner syndrome
- May harbour spontaneous echo contrast, but not thrombus
Ans. (a), (b) and (d)
- Spontaneous echo contrast:
- Always indicates presence of thrombus
- May be seen in the absence of atrial fibrillation
- More commonly found in mitral regurgitation than in mitral stenosis
- Cardiac CT scan is more useful than echocardiography for the diagnosis
- Is a contraindication for percutaneous transvenous mitral commissurotomy
- Regarding diagnosis of left atrial thrombus:
- Transthoracic echocardiography is highly sensitive but the specificity is only modest
- Transesophageal echocardiography has high sensitivity and specificity
- Pectinate muscles can produce confusion during transthoracic echocardiography
- Coumadin ridge is a differential diagnosis of an intracardiac mass
- 3D echo cannot differentiate a clot from pectinate muscles in the left atrial appendage
Ans. (b), (c) and (d)
- For detection of left atrial thrombus:
- Spontaneous echo contrast may hide thrombus
- Cardiac computed tomography and cardiac magnetic resonance may be useful
- Cardiac computed tomography is as sensitive and specific as transesophageal echocardio-graphy
- Cardiac magnetic resonance is the gold standard test
- Reduced left atrial appendage velocity may be a useful indicator
Ans. (a), (b), (c) and (e)
18A: Transthoracic 2D echocardiography apical 4-chamber view showing features of severe mitral stenosis, giant left atrium and profuse, dense spontaneous echo contrast in left atrium (arrowhead).
18B: Transthoracic 2D echocardiography appendicular view showing spontaneous echo contrast (arrowhead) in left atrium, but the appendage free of thrombus.
18C: Transesophageal echocardiography mid-esophageal view at 49° showing spontaneous echo contrast (arrowhead) in dilated left atrium, but the appendage free of thrombus.
19A: Transthoracic echocardiography off-axis view showing features of severe mitral stenosis, dilated left atrium and a large, heterogeneous mass (arrow), possibly attached to the left atrial wall.
19B: Transthoracic echocardiography apical 4-chamber view showing features of severe mitral stenosis, dilated left atrium and the thrombus in the left atrium.
- DeSanctis RW, Dean DC, Bland EF. Extreme left atrial enlargement. Some characteristic features. Circulation. 1964 Jan;29:14-23.
- El Maghraby A, Hajar R. Giant left atrium: a review. Heart Views. 2012 Apr;13(2):46-52.
- Hurst W. Memories of patients with a giant left atrium. Circulation. 2001;104:2630-1.
- Piccoli GP, Massini C, Di Eusanio G, Ballerini L, Tacobone G, Soro A, et al. Giant left atrium and mitral valve disease: Early and late results of surgical treatment in 40 cases. J Cardiovasc Surg. 1984;25:328–36.
- Oh JK. Echocardiographic evaluation of morphological and hemodynamic significance of giant left atrium. Circulation. 1992;86:328-30.
- Owen I, Fenton WJ. A case of extreme dilatation of the left auricle of the heart. Trans Clin Soc London. 1901;34:183-91.
- Moral S, Fernández-Friera L, Sanz J. Giant left atrium investigated by magnetic resonance imaging. Rev Esp Cardiol. 2011 Mar;64(3):232.
- Farman MT, Sial JA, Khan N, Rahu QA, Tasneem H, Ishaq H. Severe mitral stenosis with atrial fibrillation—a harbinger of thromboembolism. J Pak Med Assoc. 2010;60(6): 439-43.
- Apostolakis E, Shuhaiber JH: The surgical management of giant left atrium. Eur J Cardiothorac Surg. 2008, 33(2): 182-90.
- Di Eusanio G, Gregorini R, Mazzola A, Clementi G, Procaccini B, Cavarra F, et al. Giant left atrium and mitral valve replacement: risk factor analysis. Eur J Cardiothorac Surg. 1988;2(3):151-9.
- Saha GK, Ali MA, Hossain N, Zaher A, Miah MA, Zaman MA. Incidence of spontaneous echocontrast in left atrium in mitral stenosis detected by transoesophageal echo cardiography and related factors. Bangladesh Med Res Counc Bull. 2000 Aug;26(2):56-60.
- Kumar V, Nanda NC. Is it time to move on from two-dimensional transesophageal to three-dimensional transthoracic echocardiography for assessment of left atrial appendage? Review of existing literature. Echocardiography. 2012; 29(1):112-6.
- Romero J, Husain SA, Kelesidis I, et al. Detection of left atrial appendage thrombus by cardiac computed tomography in patients with atrial fibrillation: a meta-analysis. CircCardiovasc Imaging. 2013 Mar 1;6(2):185-94.
- Rathi VK, Reddy ST, Anreddy S, et al. Contrast-enhanced CMR is equally effective as TEE in the evaluation of left atrial appendage thrombus in patients with atrial fibrillation undergoing pulmonary vein isolation procedure. Heart Rhythm. 2013 Jul;10(7):1021-7.
Ahmad S Omran
RHEUMATIC SEVERE MITRAL STENOSIS PERCUTANEOUS MITRAL BALLOON VALVULOPLASTY (PMBV)
This 39-year-old male presented to our center with acute pulmonary edema. Transthoracic echocardiography (TTE) (Figs. 5.1A and B) showed mitral leaflet thickening, reduced motion of posterior mitral leaflet and typical “hockey stick appearance” of the anterior mitral valve leaflet consistent with rheumatic mitral stenosis (MS). Large pericardial effusion was noted in this study which is not uncommon in severe MS with pulmonary hypertension. This effusion was drained prior to percutaneous mitral valve balloon valvuloplasty (PMBV). Mitral valve area by pressure half time method (PHT) was calculated at less than 1.0 cm2 and mean gradient across the mitral valve was 15 mmHg. There was trace tricuspid regurgitation with estimated right ventricular systolic pressure of 60–70 mmHg. After initial stabilization of the patient, he underwent transesophageal echocardiography (TEE) for better visualization of the mitral valve and decision- making for PMBV. Transesophageal echocardiography showed marked spontaneous echo contrast (smoke) in the left atrium and appendage (LAA) but no obvious clot was noted. 3D TEE (Figs. 5.2A and B,
20 and 21) clearly showed severely stenotic mitral orifice with fusion of both commissures. Mitral valve area was re-assessed by 3D TEE direct planimetry at the level of the orifice and was repeated by QLAB software which came between 0.7–1.0 cm2 confirming TTE findings. Echocardiographic Wilkin's score of the mitral valve was calculated as 7–8. There was no contraindication for PMBV based on American and European guidelines for management of patients with valvular heart disease.1,2 Patient underwent PBMV under general anesthesia and 3D TEE guidance (Figs. 5.3A and B,
22 and 23). Successful PMBV was performed after 2 attempts of balloon inflation with PTMC-28 and 30 (Inoue balloons). 3D TEE en-face view of the mitral valve immediately after PMBV (Figs. 5.4A and B,
24) showed excellent result with mitral orifice area = 1.5 cm2, both commissures well split and only trace mitral regurgitation. Pre-discharge TTE showed mitral valve area (MVA) by PHT of 2.1 cm2 and mean gradient of 6–7 mmHg. Follow up TTE after one year showed MVA about 1.9 cm2 and patient is in good clinical condition.
Figs. 5.1A and B: Transthoracic echocardiography (TTE). (A) TTE in parasternal long-axis view shows mitral valve thickening, reduced motion of posterior mitral leaflet and typical “hockey stick appearance” of the anterior mitral valve leaflet (AMVL) consistent with rheumatic mitral stenosis (MS). Large pericardial effusion is noted (PE). (B) Apical 4-chamber view showing diastolic doming of the mitral leaflets. (LV: Left ventricle; LA: Left atrium).
Percutaneous mitral balloon valvuloplasty (PMBV) was first described by Inoue in 1984. Since its introduction, PMBV has demonstrated good immediate and midterm results and has replaced surgical mitral commissurotomy as the preferred treatment of rheumatic mitral stenosis in appropriate candidates.
Figs. 5.2A and B: 3D transesophageal echocardiography (3D TEE). (A) 3D TEE zoom mode acquisition of the mitral valve in surgical view shows a severely stenotic mitral valve. Marked “smoke” can be seen in the left atrial appendage (LAA) and left atrium (LA). 3D grid is seen (5 mm) which can be calibrated to calculate mitral orifice area from LA or LV side. (B) 3D TEE zoom mode from left ventricular (LV) perspective showing fish-mouth appearance of the stenotic mitral valve.
Figs. 5.3A and B: 3D TEE guidance during percutaneous mitral balloon valvuloplasty (PMBV). Patient is under general anesthesia and TEE was done in supine position. (A) Live 3D TEE surgical view of the mitral valve showing full inflation of the balloon within the mitral orifice. (B) Corresponding view by fluoroscopy demonstrating the first attempt by a PTMC-28 Inoue balloon. (LA: Left atrium; LAA: Left atrial appendage; RA: Right atrium).
- In echocardiographic assessment of the mitral valve (MV) for balloon valvuloplasty (PMBV) which of the following statements is correct?
- A Wilkin score about 10-12 is associated with a successful result
- In a patient with mitral valve area more than 1.5 cm2, PMBV is contraindicated
- PMBV is indicated in a patient with severe MS and less than moderate mitral regurgitation
- Based on ACC/AHA guideline, PMBV is reasonable (indication IIa) for asymptomatic patients with severe MS (MVA ≤1.5 cm2, stage C) and favorable valve morphology who have new onset of AF in the absence of contraindications
Figs. 5.4A and B: 3D TEE zoom mode of the mitral valve from left ventricular (LV) aspect. (A) Preoperative study showing 3D direct planimetry of the mitral orifice which was calculated as 0.7 cm2. (B) Immediate post PMBV study in same view using the same method shows mitral orifice area of 1.5 cm2. This method of calculation of the mitral valve area by 3D is not yet validated by a large study but is very useful in cath lab setting during PMBV where pressure half time (PHT) method is not very accurate. However, one recent study done in our center showed 3D planimetry underestimates mitral valve area compared to the PHT method.
20: 3D TEE zoom mode acquisition of the mitral valve from left atrial perspective.
21: 3D TEE zoom mode acquisition of the mitral valve from left ventricular perspective.
22: Live 3D TEE zoom mode to guide the interventionist to enter the orifice of the mitral valve.
23: Live 3D TEE zoom mode showing full inflation of the Inoue balloon within the mitral orifice.
24: Live 3D TEE zoom mode immediately after PMBV showing mitral valve from left ventricular side with split commissures and acceptable orifice opening. No signs of leaflets avulsion are seen.
- Vahanian A, Alfieri O, Andreotti F, et al. Guidelines on the management of valvular heart disease (version 2012). European Heart Journal 2012; 33, 2451-96.
- Nishimura RA, Otto CM, Bonow, et al. 2014 AHA/ACC Guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. JACC, March 4, 2014.
Adarna LG, Elsayed M, Alagic N, Uygur B, Chahwala JR, Bhagatwala K, Bulur S, Turaga NSN, Pudussery Kattalan JJ, Nanda NC
Elderly patient complaining of shortness of breath. There is no history of prior cardiac surgery. 2D TTE was done.
- What do the25–31 show?
- Rheumatic MS
- Congenital MS
- MS produced by degenerative calcific disease
- Mitral annuloplasty ring
Mitral annular calcification (MAC, arrow) is easily recognized initially in the parasternal long axis view by high echogenicity posteriorly at the LV-LA junction. This calcification may extend to involve the basal portions of MV leaflets but generally does not produce MV stenosis. Rarely, calcification may extend to the body and tips of the leaflets resulting in stenosis as in this patient. MVA by Doppler pressure half time (PHT) method was 1.38 cm2 consistent with MV obstruction. Peak and mean MV gradients were also high at 26 and 13 mmHg, respectively (
29 and 30). Calcification may also involve MV chordae and papillary muscles. This is different from rheumatic MS in which commissures are fused. This patient also has degenerative calcific AV disease with mild AR (#1,
31). #2 in
31 represents high velocity mitral inflow visualized by color Doppler.
32 shows shadowing (arrow) produced by MV annular calcification. The structures underneath the calcification are obliterated.
MOVIES 25 TO 32
Ronald B Williams, Valentyna Ivanova, Sirikarn Napan, June A Yamrozik, Moneal Shah, Robert WW Biederman
A MASS BY ANY OTHER NAME
A 79-year-old Caucasian female was referred for an echocardiogram after having a pre-op chest X-ray for hip replacement surgery. The patient's X-ray had showed a left atrial calcified mass that had increased in size from a 2011 chest X-ray at the time of a right knee replacement. Her past medical history was significant for hypertension, osteoarthritis, dyslipidemia, and family history of coronary artery disease. She was asymptomatic at the time of presentation.
A transthoracic echo was performed which showed a large mass involving the posterior mitral annulus and posterior mitral valve leaflet extending into the left atrium and left ventricle (Figs. 7.1A to D). The mass demonstrated an uneven, irregular surface with peripheral calcifications causing mildly elevated gradients without frank mitral stenosis with a mean 5 mm Hg gradient and 1–2+ mitral regurgitation. The LV was normal in size and systolic function with grade one diastolic dysfunction. The atria bilaterally were dilated. The differential diagnosis was MAC (caseous mitral annular calcification), fibroma, or fibroelastoma of the posterior mitral leaflet.
Figs. 7.1A to D: The echo images show the location, calcification, but it is unclear as to whether the extent of the mass is involving the atrium and/or ventricle.
Figs. 7.2A to F: The 2XIR, TIR, SSFP images show the round, well-demarcated mass at the posterior mitral annulus.
With the broad differential, the patient underwent cardiac magnetic resonance (CMR) imaging. Utilizing Steady State Free Precession (SSFP; dynamic sequence), the patient was imaged in the VLA/HLA views of the heart for structure and function. Black blood (2XIR) images were acquired for anatomical/morphological orientation of great vessels and heart. With the T1W, SSFP, and Triple Inversion (TIR) sequences the mass appeared round, markedly hypointense and well demarcated at the posterior mitral annulus, measuring 36 mm × 29 mm × 37 mm (Figs. 7.2A to F). On first pass perfusion imaging, there was no enhancement noted. On the postgadolinium (late gadolinium enhancement (LGE)) images, the mass shows peripheral ring enhancement without central enhancement (Figs. 7.3A to E). Thus, based on the tissue characteristic and contrast kinetics, the mass was not perfused, had extensive calcification and central proteinaceous material. In the correct setting and location within the posterior wall of the LV, this is pathognomonic for caseous calcification of the mitral annulus, also known as calcific amorphous transformation (CAT), (
33 and 34: Echos: HLA, LVOT, SA, HLA (4 = chamber) CMRI, LVOT (3 = chamber) CMRI, SA CMRI).
With the results from the CMR study, her cardiologist placed the patient on aspirin, since there was no evidence supporting systemic anticoagulation in a patient with normal sinus rhythm and mild mitral stenosis. This patient was scheduled for her hip replacement without change in her medical or surgical therapy.
Mitral annular calcification (MAC) typically presents as a large, round mass in the region of the atrioventricular groove. Caseous MAC or CAT is a rare variant, typically larger, rounded mass with central necrosis, and when incised, has a cream-like mixture with a toothpaste-like texture, consisting of amorphous, acellular and calcific material with a chronic inflammatory reaction. These masses can be several centimeters in size, displacing the mitral valve leaflets, causing valve dysfunction, stenosis, and regurgitation. CMR imaging shows low signal intensity on T1W/T2W images, with rim/ring enhancement on the contrast images.
Figs. 7.3A to E: CMR perfusion image showing no enhancement; LGE imaging showing ring/rim enhancement typical of caseous MAC.
Caseous MAC, which is associated with increased risk of CVA/TIA, likely due to associated comorbidities, is typically a benign condition with no intervention or medical therapy required.
In the presence of asymptomatic MAC, specific medical treatment other than aspirin therapy, is not required. Some studies suggest there is a strong correlation between MAC and atherosclerosis, valvular heart disease, stroke, renal disease and other vascular diseases. As such, appropriate medical management of cardiovascular risk factors is recommended without specific therapy aimed at MAC (or CAT).
- In comparison with CMR, TTE imaging has what general limitations?
- Limited image quality due to patient's body habitus
- Limited depth of image window
- Reduced spatial resolution
- None of the above
- (a) and (b)
- (a), (b) and (c)
- The temporal resolution (TR) is typically higher or lower in echocardiography as related to CMR, leading to better or lower ability to resolve highly mobile masses.
- Echo has higher TR but lower ability to resolve mobile masses
- Echo has higher TR and higher ability to resolve mobile masses
- CMR has lower TR and higher ability to resolve mobile masses
- MAC and/or CAT can be recognized on TTE but confirmation that it does not represent a tumor via tissue characterization by CMR (or CT) is advantageous.
- True but only by MRI, not CT
- True for both MRI and CT
- False for both
- Calcium is not well represented on MRI from a physical principle but due to its capability to define both T1 and T2, calcium can be inferred.
- Hounsfield units via CT accurately and with histological confirmation define calcium
- (b), (d) and (e)
33A: Echo: Apical four chamber view.
33B: Echo: Parasternal long axis view.
33C: Echo: Parasternal short axis view.
34A: SSFP HLA (4 = chamber) CMRI
34B: SSFP LVOT (3 = chamber) CMRI
Chahwala JR, Elsayed M, Alagic N, Adarna LG, Bhagatwala K, Bulur S, Turaga NSN, Nanda NC
Adult patient with MR. 2D TTE was done.
- Factors that need to be taken into account when semi-quantitatively assessing MR severity during echocardiographic examination are all of the following except: (35–38)
- Nyquist limit
- Color gain
- Color M-mode to check whether MR is pansystolic or not
- Family history
- Multiple plane examination
- Blood pressure
- LA and LV dilatation
- Eccentricity of MR jet
Nyquist limit is best standardized around 50 cm/sec since a lower Nyquist limit such as 23 cm/sec will increase MR jet size and produce artifacts extending beyond the cardiac borders (
38). Very high Nyquist limit will reduce the jet size and result in underestimation of severity. Changes in Nyquist limit also change the color wall filter and that is the reason for changes in jet size. Color gain is also an important factor. In
37, the color gain was reduced from 67 to 46 and the MR jet shows a considerable decrease in size. When the color gain was further decreased to 27, severe MR completely disappeared. Color gain should be gradually increased till speckles and artifacts appear and then it should be gradually decreased till they just disappear. MR should also be assessed using multiple planes and the maximum jet size taken.
- What does the arrow in39 point to?
Ans. (d)This patient also has a hypertrophied papillary muscle.
- Leading edge of a thrombus
- Vestigeal membrane
- False tendon
MOVIES 35 TO 39
Alagic N, Adarna LG, Elsayed M, Chahwala JR, Bhagatwala K, Bulur S, Turaga NSN, Mohamed A, Gupta N, Nanda NC
This is an adult patient with a murmur and shortness of breath. 2D TTE was done (
- What is the abnormality (40–42)?
- Prolapse of anterior MV leaflet
- Prolapse of posterior MV leaflet
- Prolapse of both MV leaflets
- MV is redundant but does not prolapse beyond MV annulus
There is marked prolapse of both MV leaflets (arrow in
40) with severe MR. The MV chordae also appear to be redundant.
MOVIES 40 TO 42
Naveen Garg, Kanwal K Kapur
EVALUATION OF MITRAL REGURGITATION
A 51-year-old male, a known case of diabetes mellitus, systemic hypertension and triple vessel disease (CT- angiography done a few days ago showed LAD 90%, LCx 100% and RCA 100% blocked) reported with complaints of dyspnea on exertion. Troponin-T was negative. He came for echocardiography evaluation before CABG.
Preoperative analysis revealed posteriorly directed MR jet with dense CW-Doppler signals, color Doppler flow signals from MR reaching the roof of LA (Figs. 10.1A to E).
- Is severe MR the cause of patient's symptoms?
- Does this patient need MV replacement/MV repair along with CABG?
Further hemodynamic analysis shows in Table 10.1.
Figs. 10.1A to E: (A) Jet of MR VTI = 79.3 cm; (B) MR jet touching roof of LA; (C) Mitral Inflow velocity; (D) LVOT VTI = 16.9 cm; (E) MV inflow VTI = 13.1 cm
Mitral inflow (Total SV) = 0.785 × 2.52 × 2.51 × 13.1 = 64.78 ml
Forward Stroke Volume = 0.785 × 2.09 × 2.09 × 16.9 = 57.94 ml
MR volume and fraction = 6.84 ml and 10.56% respectively.
Thus, in spite of color Doppler and CW-Doppler findings indicative of severe MR, MR volume and fraction done by the stroke volume method suggest only mild MR precluding the need for associated MV replacement or repair.
Patient underwent off pump CABG and was followed up by a color Doppler study after 6 months……(
44 POST CABG) (Figs. 10.2A to C).
LVOT VTI = 14.8 cm; MV VTI= 10.6 cm
Forward SV= 50.7 ml; Mitral Inflow (Total SV) = 52.4 ml MR volume and fraction = 1.72 ml and 3.2% respectively.
- The symptoms in this patient were due to three vessel coronary artery disease which considerably improved after CABG. The significant reduction in MR jet area was likely due to amelioration of papillary muscle ischemia.
- Color flow imaging and CW-Doppler signal density are widely used indices in the assessment of MR severity. However occasionally, as was the case in this patient, significant errors could occur if additional quantitative parameters are not performed.
- However, quantitative estimation involves diameter assessment across two valves and this could lead to significant interobserver variability and also significant errors in estimation of MR volume.
Although the estimated regurgitant volume by the Doppler calculation is negligible (only 6.8 ml), the semi quantitative estimation by jet area method shows that this regurgitation could actually be moderate to severe. The small LA size in this patient who never had pulmonary edema is consistent with relatively mild MR. This case therefore illustrates the limitations in the Doppler computation (diameter assessment errors for LVOT and mitral annulus) as well as the possible overestimations by jet area method. This case however does show that decision to avoid mitral valve repair procedure during CABG was justified as the MR practically disappeared after the revascularization procedure.
Editor's note: Prominent flow acceleration (PISA) and the large vena contracta also suggest that MR was significant and might have improved after CABG. LA volume is normal in this patient and that can provide a clue that MR, if it was chronic, was less severe. MR volume of only 6.84 ml calculated by the output method is definitely grossly erroneous.
MOVIES 43 AND 44
Bulur S, Nanda NC
This is a 70-year-old male with known murmur of MR for several years who came for a follow-up examination. 2D TTE was done.
- (Fig. 11.1 and45): How would you grade the severity of MR?
- Severe because the VC dimension is 0.88 cm (more than 0.7 cm) and the MR jet occupies more than 40% of LA
MR does appear severe in this apical four chamber view.
- (Figs. 11.2 and 11.3): MR is found only in mid to late systole using color M-mode with minimal MR occurring very early in systole due to the closing motion of MV (Fig. 11.2). Also, the LA is not enlarged (Fig. 11.3). Would these findings make you reconsider the severity of MR?
Both of these findings are important when considering MR severity specially in patients with classical mid to late systolic MV prolapse in whom the MR may not be pan-systolic. Also, LA would be expected to be enlarged in patients with chronic (but not acute) severe MR. Arrow in Figure 11.3 points to posterior MV leaflet prolapse.
- What is the best method to calculate MR volume in this patient?
- Multiplying 2D TTE VC (taking 0.88 cm as the diameter and assuming the VC to be circular or elliptical) by MR VTI obtained by continuous wave Doppler
- Using the standard PISA method
- Multiplying the VC obtained by 3D TTE and MR VTI assessed by continuous wave Doppler
- Using the volume method by subtracting the stroke volumes obtained from LVO and RVO using PW Doppler
The exact size of the VC which is necessary to calculate MR volume, can be assessed by 3D TTE (or 3D TEE). It cannot be generally obtained reliably by 2D TTE (or 2D TEE) because at any given time the 2D provides only a thin slice-like view of the MV and MR. On the other hand, the full extent of the MV and MR VC is encompassed in the 3D data-set facilitating accurate viewing of the shape and size of VC. The PISA method is less reliable because it assumes the PISA to be hemispherical which is not the case in most patients. The volume method is also unreliable because of errors in correctly measuring the LVO and RVO diameters. Also, associated regurgitation of other valves will affect the results. In this patient, MR volume was calculated as 29.8 ml consistent with only moderate MR (Figs. 11.4 and 11.5 and
Fig. 11.2: Color M-mode examination. Left arrow points to minimal MR resulting from closing movement of the MV. Right arrow shows mid to late systolic MR. (PLX: parasternal long axis view).
Fig. 11.4: Vena contracta (VC) area (A1) of the mid to late systolic MR by 3DTTE measured 0.31 cm2. VC of early systolic MR produced by the closing movement of MV was negligible.
Fig. 11.5: Shows calculation of MR volume (VOL) by multiplying the combined velocity time integrals (VTI) of the two MR jets by the vena contracta (VC, 0.31 cm2) of MR obtained by 3DTTE. Left arrow shows measurement of VTI (14 cm) of MR produced by closing movement of the MV and right arrow VTI (82 cm) of the mid to late systolic MR. Total MR volume measured 29.8 ml.
MOVIES 45 AND 46
Alagic N, Elsayed M, Adarna LG, Chahwala JR, Bhagatwala K, Bulur S, Turaga NSN, Mohamed A, Gupta N, Pudussery Kattalan JJ, Nanda NC
- What is the severity of MR in this patient? (47)
MR appears moderate only with a PVC otherwise it appears mild. It is important to take into account the presence of arrhythmia when evaluating valve regurgitation severity.
Adarna LG, Chahwala JR, Elsayed M, Alagic N, Bhagatwala K, Bulur S, Turaga NSN, Mohamed A, Gupta N, Nanda NC
- What does the arrow point to in this adult patient with MR (48)?
- Minimal lower velocity MR occurring during MV closure before real MR.
- Artifact produced by CW Doppler.
The peak MR velocity is very high reflecting the gradient between LV and LA pressures during systole.
Ahmad S Omran
FLAIL ANTERIOR MITRAL VALVE LEAFLET
This 61-year-old woman presented to our ER with acute coronary syndrome. Emergency coronary intervention was done for LAD stenosis. Transthoracic echocardiography (Figs. 14.1A and B
49, 50) showed flail anterior mitral valve leaflet and severe eccentric posteriorly directed jet of mitral regurgitation (MR). Preoperative transesophageal echocardiography (TEE) confirmed the diagnosis of myxomatous mitral valve with at least two ruptured chordae of the anterior leaflet and severe MR (Figs. 14.2A and B
51, 52).1,2 Patient underwent cardiac surgery for mitral valve repair (MV repair). Ruptured chordae tendineae were replaced with two pairs of artificial Gore-Tex sutures (Figs. 14.3A and B). An annuloplasty ring was used to stabilize the repair and improve leaflet coaptation (size 30 Physio ring). Intraoperative TEE immediately after repair showed an excellent result with no evidence of mitral stenosis or regurgitation. Patient was discharged home and follow up echocardiography 2 years after MV repair showed no residual MR.
Figs. 14.1A and B: Transthoracic echocardiography (TTE). (A) TTE in apical 4-chamber view shows anterior mitral valve leaflet (AMVL) with flail tip. (B) Same view with color Doppler demonstrates severe eccentric posteriorly directed jet of mitral regurgitation (MR) with flow signals swirling around the left atrium. (LV: Left ventricle; LA: Left atrium; RA: Right atrium; RV: Right ventricle).
Figs. 14.2A and B: 3D Transesophageal echocardiography (3D TEE). (A) 3D TEE zoom mode acquisition of the mitral valve in surgical view shows middle segment of the anterior mitral leaflet (A2) with 2 ruptured chordae. (B) Same view with full volume color Doppler acquisition demonstrates severe eccentric posteriorly directed jet of mitral regurgitation (MR). (AoV: Aortic valve; LAA: Left atrial appendage).
Figs. 14.3A and B: Surgical exploration of the mitral valve via the left atrium. (A) Two chordae of the middle segment of the anterior mitral valve leaflet were ruptured. (B) Ruptured chordae (Rup. Chor) were replaced with Gore-Tex sutures. A size 30 complete ring (Physio ring) was used for mitral annuloplasty. (AMVL: Anterior mitral valve leaflet).
- In echocardiographic assessment of the mitral valve which of the following statements is correct?
- Anterior mitral valve leaflet consists of 3 scallops with distinct indentation between the scallops.
- Ruptured chordae in myxomatous mitral valve are more common in the female.
- Bileaflet prolapse may cause central MR. Degree of MR can be underestimated in this pathology.
- Interrogation of left upper pulmonary vein inflow is adequate for assessment of MR severity.
49: Intraoperative TEE in 4-chamber view showing flail anterior mitral leaflet.
50: Same view of previous movie demonstrates severe posteriorly directed jet of MR.
51: 3D TEE surgical view of the mitral valve showing 2 ruptured chordae tendineae.
52: Postoperative 3D TEE showing Physio ring annuloplasty.
- Tsang W, Lang RM. Is 3-Dimensional echocardiography essential for intraoperative Assessment? Circulation, Aug 2013; 643-52.
- Faletra FF, Demertzis S, Pedrazzini G, et al. Three-Dimensional transesophageal echocardiography in degenerative mitral regurgitation. J Am Soc Echocardiogr 2015;28: 437-48.
Ahmad S Omran
FLAIL POSTERIOR MITRAL VALVE LEAFLET
This 45-year-old male presented to our center with severe shortness of breath. Transthoracic echocardiography (Figs. 15.1A and B) showed flail posterior mitral valve leaflet at middle scallop (P2) with multiple ruptured chordae. Severe eccentric anteriorly directed jet of mitral regurgitation (MR) was noted. Preoperative transesophageal echocardiography (TEE) confirmed the diagnosis of myxomatous mitral valve with at least two ruptured chordae of the posterior leaflet and severe MR (Figs. 15.2A and B,
53–56).1,2 Patient underwent cardiac surgery for mitral valve repair (MV repair). Posterior leaflet had quadrangular resection at P2 and sliding plasty of medial and lateral scallops (P3 and P1). An annuloplasty ring (C-E Physio II ring size 32) was used to stabilize the repair and improve leaflet coaptation (Figs. 15.3A and B). Intraoperative TEE immediately after repair showed excellent result with no signs of mitral stenosis, residual MR or mitral systolic anterior motion (SAM). Patient was discharged home and follow up echocardiography 2 years after MV repair showed no residual MR.
Figs. 15.1A and B: : Transthoracic echocardiography (TTE). (A) TTE in parasternal long-axis view shows posterior mitral valve leaflet (PMVL) with flail middle scallop (P2). (B) Same view with color Doppler demonstrates severe eccentric anteriorly directed jet of mitral regurgitation (MR). (LV: Left ventricle; LA: Left atrium).
Figs. 15.2A and B: 3D Transesophageal echocardiography (3D TEE). (A) 3D TEE zoom mode acquisition of the mitral valve in surgical view shows middle scallop of the posterior mitral leaflet (P2) with multiple ruptured chordae. (B) Same view with full volume color acquisition demonstrates severe eccentric anteriorly directed jet of mitral regurgitation (MR). (LAA: Left atrial appendage).
Figs. 15.3A and B: Surgical exploration of the mitral valve via the left atrium. (A) At least two chordae of the middle segment of the posterior mitral valve leaflet were ruptured (Rup. chord). (B) Quadrangular resection of the P2 (Quad. Resec) was performed with sliding plasty of other scallops to prevent postoperative SAM (systolic anterior motion). A size 32 complete ring (Physio II ring) was used for mitral annuloplasty.
- In echocardiographic assessment of the mitral valve (MV) which of the following statement is correct?
- Accuracy of 2D TEE in identifying MV segments is about 90-97%.
- In 3D TEE with surgical view of the mitral valve, anterior mitral leaflet is adjacent to the right coronary cusp of the aortic valve.
- 3D TEE zoom mode acquisition provides the image with the highest frame rate for assessment of the mitral valve.
- In surgical view of the mitral valve by 3D TEE, aortic valve should be positioned at the bottom of the image and left atrial appendage to the right of the image.
53: Intraoperative TEE in 4-chamber view showing flail middle scallop of posterior mitral leaflet (P2).
54: Same view of previous movie demonstrates severe anteriorly directed jet of MR.
55: 3D TEE surgical view of the mitral valve showing multiple ruptured chordae tendineae.
56: Same view of previous movie with full volume color acquisition showing severe anteriorly directed jet of mitral regurgitation (MR).
- Omran AS, Woo A, David, et al. Intraoperative transesophageal echocardiography accurately predicts mitral valve anatomy and suitability for repair. J Am Soc Echocardiogr. 2002;15(9):950-7.
- Lang RM, Badano LP, Tasang W, et al. EAE/ASE Recommendations for image acquisition and display using three-dimensional echocardiography. J Am Soc Echocardiogr. 2012;25: 3-46.
Nilda Espinola-Zavaleta, Salomon Rivera-Peralta, Hugo Rodriguez-Zanella, Jose Antonio Arias-Godinez, Maria Eugenia Ruiz-Esparza, Nydia Avila-Vanzzini, Juan Francisco Fritche-Salazar, Angel Romero-Cardenas
A 39-year-old male with history of blunt chest trauma and sudden onset of dyspnea. Physical exam revealed an apical holosystolic murmur grade IV/VI radiating to the axilla. 2D and 3D echocardiography was done (Figs. 16.1, 16.2 and
- The most likely diagnosis is:
- Flail MV
Mitral regurgitation can be explained by multiple causes such as endocarditis, ischemia, tumor, myxomatous disease, trauma, hypertrophic cardiomyopathy, among others. However, blunt chest trauma rarely causes mitral regurgitation. In this clinical scenario, it is caused by papillary muscle, chordae tendineae and/or MV rupture.1 The most frequent reason is rupture of the anterior mitral leaflet, followed by rupture of chordae tendineae. The latter structures are thinner and more fragile, making them susceptible to rupTure.2 In the present case, blunt trauma resulted in a flail posterior MV leaflet with severe MR as evidenced by 2D TTE and 3D TEE.
Fig. 16.1: Zoomed view of the mitral valve in four chamber view, showing a flail posterior mitral leaflet (arrow).
57: Zoomed view of the mitral valve in four chamber view, showing a flail posterior mitral leaflet.
58: 2D TTE and color flow four chamber view with severe mitral regurgitation.
59: 3D TEE showing enface view of the flail posterior MV leaflet.
- Hajime K, Yoshiharu H, Shinji H, Norimasa M, Taira K. Mitral valve plasty for Mitral regurgitation after blunt chest trauma. Ann Thorac Cardiovasc Surg. 2001;7:175–9.
- Selzer A, Kelly JJ, Vannitamby M, Walker P, Gerbode F, Kerth WJ. The syndrome of mitral insufficiency due to isolated rupture of the chordae tendineae. Am J Med. 1967;43: 822–36.
Mohamed A, Gupta N, Elsayed M, Nanda NC
This patient is a 48-year-old male referred for a murmur. 2D TTE showed mitral valve prolapse with moderate MR.
- What does the arrow in60 show?
- Membrane in LV
- MV chordae rupture
- Redundant MV chordae
- Membranous cyst in LV
Redundant MV chordae are more often seen in patients with myxomatous MV and prolapse as in this case. They are occasionally seen as an isolated finding.