Sudarshan Kumar Vijay

Congenital Aorta Right Atrial Fistula: Successful Transcatheter Closure With the Amplatzer Occluder

A 12-year-old girl with a 4-year history of effort dyspnea and palpitations was referred to our institution for evaluation. Physical examination of the patient showed a continuous grade 3/6 heart murmur best heard along the right upper sternal border. A two-dimensional echocardiogram exhibited a small interatrial septal defect (diameter, 4 mm) with a bidirectional shunt and a high velocity jet in the dilated right atrium. A computed tomographic (CT) angiogram of the patient showed anomalous tortuous communication between the ascending aorta and the right atrium arising adjacent to the right coronary artery (Fig. 1). An ascending aortogram combined with selective angiography confirmed the presence of a large fistula with a broad origin (diameter, 5.6 mm) from the right aortic sinus anterior to the right coronary artery and a narrow termination into the posterior wall of the right atrium (Fig. 2). Cardiac catheterization showed the presence of a left-toright shunt with a pulmonary-to-systemic blood flow ratio (Qp:Qs) of 3:1. Coronary angiography demonstrated normal coronary arteries arising from the respective sinuses. Because the fistula had a separate anterior origin and a narrow terminal ending into the right atrium, a decision was made in favor of transcatheter device closure. The fistula was hooked antegradely using an Amplatzer right 1 (AR-1) catheter (Medtronic Inc., Minneapolis, MN, USA), and a standard percutaneous transluminal coronary angioplasty (PTCA) guidewire balance middle weight (BMW) was passed through the tortuosity of the fistula toward the right atrial end. The wire then was retrieved from the right atrial end with a snare and exteriorized from the right femoral vein. A long sheath available with the device then was passed through the right femoral venous route to the right atrium. An Amplatzer duct occluder (AGA Medical Corporation, Plymouth, MN, USA) size 8 9 6 mm was chosen for closure of the fistula and passed through the sheath with the help of the Amplatzer 1808 delivery system (Fig. 3). The fistula was successfully closed using the occluder with its large end placed through the fistula toward the aortic side. After deployment of the device, no residual flow through the fistula was demonstrated (Fig. 4).

Delineation of Anatomy of the Ruptured Sinus of Valsalva with Three-Dimensional Echocardiography: The Advantage of the Added Dimension

The rupture of sinus of Valsalva is a rare complication of infective endocarditis. Three‐dimensional (3D) echocardiography represents an important adjunctive tool to demonstrate the ruptured sinus of Valsalva with better delineation of its characteristics. We present an adult patient with rupture of right sinus of Valsalva aneurysm due to infective endocarditis of the aortic valve, in whom the two‐dimensional (2D) transthoracic echocardiogram erroneously localized the site of rupture into the right atrium. Whereas, 3D transthoracic echocardiogram accurately delineated the site of rupture into the right ventricle and it was confirmed on subsequent cardiac catheterization and angiogram. In addition, 3D echocardiography clearly showed the size and shape of the defect, which helped in successful transcatheter closure of the defect with amplatzer duct occluder device. (Echocardiography 2012;29:E148‐E151)

Aneurysmal Aorto-Right Ventricular Tunnel

A successful closure of an aneurysmal aorto-right ventricular tunnel (ARVT) in a 16-year-old male patient is reported here. An attempt at device closure had failed in this patient. Diagnosis was confirmed by Doppler echocardiography, 3-dimensional computed tomography, and cardiac catheterization. Surgical closure with a Dacron patch (W.L. Gore & Associates, Flagstaff, AZ) at the aortic end and direct closure at the ventricular end was done successfully with the patient under mild hypothermia. The postoperative echocardiogram showed a competent aortic valve with a closed ARVT.

Left Hemitruncus With Tetralogy of Fallot and Right Aortic Arch: Rare Survival Beyond the First Decade

Hemitruncus is an uncommon congenital anomaly that has been described in isolation or in association with other congenital cardiac malformations. This report describes a rare case of left hemitruncus with tetralogy of Fallot and a right-sided aortic arch. The patient presented to us in the early second decade. The diagnosis was suspected with echocardiography and confirmed with cardiac catheterization. The patient underwent successful surgical correction of the anomaly.

Giant Aorto–Right Ventricular Fistula With Single Coronary Artery

A 15-year-old boy with symptoms of dyspnea and fatigue since early childhood was referred to our institution for evaluation. Physical examination of the patient showed long, slender extremities and a pectus carinatum deformity of the chest (Figure 1A). His arm span–to-height ratio was 1.03, and his upper segment–to–lower segment ratio was 0.82. He had blood pressure of 126/70 mm Hg, with a heart rate of 96 bpm; a grade 4/6 continuous murmur was present in the right lower parasternal area. A 12-lead ECG showed right bundle-branch block (Figure 2), and chest radiography (posteroanterior view) revealed mild cardiomegaly. Radiography of the thoracolumbar spine of the patient disclosed a mild degree of scoliosis (inset, Figure 1A). Two-dimensional echocardiography (parasternal long-axis view) showed a large fistula (2 cm in diameter) arising from the right aortic sinus (Figure 1B; online-only Data Supplement Movie I). A tilted apical 4-chamber view showed a turbulent jet (velocity 3.5 m/s) at the free wall of the right ventricle (Figure 1C; online-only Data Supplement Movie II). Three-dimensional echocardiography in a tilted apical 5-chamber view showed a broad tunnel arising from the aorta (Figure 1D; online-only Data Supplement Movie III). Computed tomographic cardiac angiography revealed the presence of a …

Ratchet-traction effect: An underdiagnosed mechanism of pacing lead dislodgement

Pacing lead dislodgement contributes substantially, to the list of causes of early pacemaker failure. Reels syndrome is a rare cause of pacemaker failure, resulting from the dislodging of pacing electrodes by manipulation of the pulse generator by the patient. We describe here an intriguing case of pacemaker lead dislodgement by a novel and frequently underdiagnosed mechanism that mimicked the fluoroscopic diagnosis of reels syndrome.

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

Giant intrapericardial lipoma: an unusual cause of dyspnoea.

The pericardial lipomas are rare benign cardiac tumors. The clinical presentation of these cardiac neoplasms varies according to the site of growth. We describe here a case of massive intrapericardial lipoma leading to severe dyspnea, its diagnostic work up and briefly review the existing literature of this uncommon disorder. The patient underwent successful surgical resection of the tumor with complete alleviation of the symptoms.

Live three-dimensional transthoracic echocardiography of giant aorto-right ventricular tunnel

A 13-year-old male was referred to us for gradually progressive dyspnoea. The physical examination revealed a grade 4/6 continuous murmur with maximum intensity in the left parasternal area. A chest X-ray disclosed mild cardiomegaly and ECG was normal. The two-dimensional transthoracic echocardiogram (2D-TTE) in the apical five-chamber view showed a large tunnel-like structure arising from the right aortic sinus ( Panel A ; see Supplementary data online, Movie S1 ) and it …

Juvenile rheumatic mitral stenosis in association with perimembranous ventricular septal defect in a 3-year-old boy.

Rheumatic mitral stenosis (MS) is a well-known entity in the developing world. Though rarely seem, MS can present at a very young age with severe symptoms. We describe here an unusual association of a large perimembranous ventricular septal defect with juvenile rheumatic MS leading to severe pulmonary hypertension and heart failure in a very young child.