Varun Shankar Narain

Ruptured Anterior Mitral Leaflet Aneurysm in Aortic Valve Infective Endocarditis—Evaluation by Three‐Dimensional Echocardiography

Ruptured aneurysm of the anterior mitral leaflet is a rare but a devastating complication secondary to aortic valve infective endocarditis. We report a case of 30‐year‐old male with native aortic valve endocarditis who was referred to us for evaluation of worsening of heart failure after an initial period of responsiveness to antibiotics. Detailed evaluation with two‐dimensional and three‐dimensional transthoracic echocardiography revealed ruptured anterior mitral leaflet aneurysm with severe eccentric mitral regurgitation along with a large vegetation on the aortic valve. The patient underwent successful surgical closure of the defect along with aortic valve replacement.

Multimodality imaging of submitral left ventricular aneurysm.

Submitral left ventricular aneurysms are a rare nonischemic aneurysm thought to be developmental in origin. These aneurysms invariably occur at the site of posterior mitral annulus and lead to mitral valve incompetence. We hereby describe a case of a 30‐year‐old adult with a rare form of submitral left ventricular aneurysm involving whole of the posterior mitral annulus. Multimodality imaging with two‐dimensional echocardiography, three‐dimensional echocardiography, computed tomography and invasive left ventriculography helped the patient to undergo successful resection of the aneurysm along with mitral valve replacement.

Classical triad of Kearns-Sayre syndrome

We describe the images of a 27-year-old man presenting with recurrent syncope due to complete heart block (figure 1). The patient, on further examination, showed ophthalmoplegia, bilateral ptosis (figure 2) and pigmentary retinopathy (figure 3). Kearns-Sayre syndrome (KSS) is a rare mitochondrial genetic disorder with multisystem involvement. KSS usually presents before the age of 20u2005years. The triad of external opthalmoplegia, pigmentary retinopathy, and cardiac conduction defects are sufficient to make a confident diagnosis of …

Right‐Sided Endomyocardial Fibrosis with a Right Atrial Thrombus: Three‐Dimensional Transthoracic Echocardiographic Evaluation

Endomyocardial fibrosis is a form of restrictive cardiomyopathy mainly affecting poor children and young adults in geographically restricted areas of Latin America, Africa, and Southeast Asia. The pathophysiological hallmark of the disease is focal or diffuse endocardial thickening involving mainly the inflow, the apices, and the subvalvular region leading to valvular regurgitation, diastolic dysfunction and obliteration of the ventricular apex. Advanced right‐sided disease has slow flow of blood through chambers with propensity of thrombus formation especially in the right atria. Although two‐dimensional transthoracic echocardiography remains the cornerstone for the diagnosis of this disease, the case presented here shows how three‐dimensional transthoracic echocardiography can add substantial information regarding the region of involvement of the right ventricle as well as the various characteristics of the right atrial thrombus.

Real time three-dimensional transthoracic echocardiography of ruptured left sinus of Valsalva aneurysm to left ventricle.

Aneurysms arising from the sinus of Valsalva are a rare cardiac defect that can present with various signs and symptoms, and if not diagnosed and treated rapidly can lead to fatal outcomes. Unruptured aneurysms are usually asymptomatic and found incidentally during diagnostic studies. More commonly, aneurysm of sinus of Valsalva is detected after the occurrence of rupture. Echocardiography has become the investigative tool of choice for this condition, not only for diagnosis but also for quantification of severity. We hereby report a rare case of a 15‐year‐old patient presenting with complaints of effort dyspnea and palpitations. Two‐dimensional transthoracic echocardiography (TTE) showed aneurysmal dilatation of left sinus of Valsalva which had ruptured into the left ventricle. Also, there was an intimal flap within the sinus of Valsalva aneurysm. The anatomical relationship between the aorta, aneurysm, and the left ventricle as well as the intimal flap within the aneurysm was clearly delineated with the help of three‐dimensional TTE. After confirmation of the diagnosis with multidetector computed tomography, patient underwent successful surgical repair of the defect.

Cleft anterior mitral leaflet with supravalvular aortic stenosis a rare association

A 20 year old female was referred to us for evaluation of effort dyspnoea of NYHA class II with feeble left common carotid, left brachial and left radial artery as compared to other sides. Detail evaluation with two dimensional (2D) transthoracic echocardiographic, 2D transesophageal echocardiography and multidetector computed tomography (MDCT) delineated Cleft AML and diffuse type of supravalvular aortic stenosis. To the best of our knowledge, no such case have been described in the literature where these anomalies co-existed in the same patient.

Double orifice mitral valve with bicuspid aortic valve: evaluation by three-dimensional echocardiography.

![Figure][1] nn[![Graphic][3] ][3][![Graphic][4] ][4][![Graphic][5] ][5][![Graphic][6] ][6]nnnnA 15-year-old male presented to us with dyspnea on exertion of New York Heart Association functional class II. Cardiac auscultation revealed a pansystolic murmur at the apex. Two-

Pulmonary Artery Stenosis due to Lung Carcinoma: A Rare Cause of Dyspnea.

Acquired bilateral pulmonary artery stenosis in adults due to lung malignancy is infrequently reported. We describe an adult male who presented to us with chief complaints of dyspnea on exertion and one episode of hemoptysis. Two dimensional transthoracic echocardiography with color Doppler showed presence of an extra cardiac mass causing severe extrinsic compression of both the right and left pulmonary artery leading to high pressure severe tricuspid regurgitation and extension of the mass into the left atrium. Three dimensional transthoracic echocardiography clearly delineated the anatomy of the left atrial mass and its surrounding anatomical relationship. The diagnosis of non small cell lung carcinoma was confirmed by multidetector computed tomography (MDCT) and with MDCT guided biopsy with histopathology. Patient succumbed one month later due to an episode of massive hemoptysis.

Multimodality Imaging of Holmes Heart with Parachute Mitral Valve

Case Report: A 10-year-old boy was admitted to our center with complaints of dyspnea on exertion of New York Heart Association functional class II and easy fatigueability. On general examination pulse was 90/min, blood pressure was 100/70 mmHg, normal jugular venous pressure and there was central cyanosis with clubbing. Cardiac auscultation revealed a mid-diastolic murmur and a pansystolic murmur at the apex. Evaluation by two-dimensional (2D) transthoracic echocardiography (TTE) Figure 1. Two-dimensional transthoracic echocardiography with color Doppler. Apical four-chamber view A, B. showing presence of double inlet left ventricular type of single ventricle. Apical long-axis and papillary muscle level parasternal short-axis view showing parachute type mitral valve with a single papillary muscle (indicated by arrow in D, E.) resulting into severe mitral stenosis C. and moderate to severe mitral regurgitation F. LA = left atrium; RA = right atrium; SV = single ventricle.

Calcified Aorto–Right Ventricular Tunnel in a Patient With Multiple Lentigines Syndrome

From the *Department of Cardiology, C.S.M. Medical University (King George’s Medical College), Lucknow, Uttar Pradesh, India; and the †Department of Radiodiagnosis, C.S.M. Medical University (King George’s Medical College), Lucknow, Uttar Pradesh, India. Manuscript received June 5, 2012; revised manuscript received June 14, 2012, accepted June 26, 2012. A12-year old boy with multiple pigmented nevi and lentigines (A) presented to us with symptoms of effort dyspnea and recent onset cyanosis. He had mild thoracolumbar scoliosis, unilateral sensorineural hearing loss, and growth retardation. The 2-dimensional transthoracic echocardiogram revealed a circular echolucency lateral to the tricuspid annulus (B, Online Video 1) (LA left atrium; LV left ventricle; RA right atrium; RV right ventricle), and color Doppler echocardiogram showed a tunnel-like structure with turbulent jet filling the right ventricle (C, Online Video 2). Two-dimensional echocardiogram with continuous-wave Doppler showed severe valvular pulmonic stenosis (D), and contrast echocardiogram revealed right to left shunt across the foramen ovale (Online Video 3). Cardiac catheterization showed spiral calcification and catheter passage from the aorta to the RV (E, Online Video 4), and the aortogram revealed flow across the tunnel (Online Video 5). Sixty-four slice computed tomography angiography showed a dilated aortic root (DA) with a tortuous aorto–right ventricular tunnel (F). Computed tomography volume-rendered imaging showed a giant tunnel and single left coronary artery giving branches (Bs) to the RV (G), with massive dilation of the aortic root and presence of calcified bands at the origin of the tunnel (H). LAD left anterior descending artery; MPA main pulmonary artery; PA pulmonary artery; RAA right atrial appendage. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jacc.2012.06.066