Percutaneous device closure of persistent left superior Vena Cava connecting to the left atrium with intact coronary sinus : a rare entity

We report a 4 year old child referred for routine cardiology evaluation as part of evaluation of murmur with scoliosis. On examination, there was mild duskiness. Echocardiography revealed bilateral superior vena cavae, a small innominate vein, a left superior vena cava to the left atrium and an intact coronary sinus. The findings were confirmed on computed tomography angiography. Percutaneous closure of the left superior vena cava by an Amplatzer vascular plug was done successfully. Although the patient was apparently asymptomatic, the procedure was done to prevent risk of cerebral emboli.peer-reviewe

Persistence of pulmonary arterial hypertension after relief of left sided obstructive lesions in small infants : our experience

Background Infants with critical left sided obstructive lesions usually present with left ventricle dysfunction and pulmonary arterial hypertension (PAH). Left ventricular dysfunction and pulmonary artery pressures usually normalize after relief of obstruction.In some,PAH persists despite adequate relief of obstruction. Patients and Methods We retrospectively reviewed records of infants (less than 3 months) who underwent intervention for left sided obstruction (n=50) over four years (May 2012 –May 2016). Result We report four infants who had persistent of PAH despite relief of left sided obstruction. The causes of PAH were found to be high left ventricular end diastolic pressure(n=1), severe hypoplasia of pulmonary veins (n=1), stenosis of the lower branch of the right pulmonary artery (n=1) and nonregression of pulmonary vascular resistance (n=1). Conclusion That persistence of PAH after relief of obstruction should be investigated and other, less common causes should be sought.peer-reviewe

Isolated Biventricular Noncompaction in an adult with severe pulmonary hypertension : an association reviewed.

Biventricular noncompaction is a recently recognized rare form of cardiomyopathy. It is characterized by altered structure of myocardial wall as a result of intrauterine arrest of compaction of the myocardial fibers in absence of coexisting congenital lesion. Left ventricle is the most affected site for noncompaction, but right ventricular involvement has been reported in a few cases. Diagnosis is made with 2-dimensional echocardiography or cardiac magnetic resonance imaging. While major clinical manifestations are heart failure, arrhythmias and embolic events,pulmonary artery hypertension ( PAH)has not been well elaborated in the literature. We present a 13- year old boy who had Biventricular noncompaction complicated by severe pulmonary hypertension. Pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic left ventricular dysfunction secondary to noncompaction. This article reviews the literature particularly with reference to PAH in the context of this case.peer-reviewe

Dysphagia in an adult tetralogy of fallot with double aortic arch

Double aortic arch (DAA) is a common vascular ring. It may occur in isolation or coexist with various types of congenital heart disease . The anomaly usually presents in early infancy. This reports a 23yr old male presenting with dysphagia, who was found to have a double aortic arch and tetralogy of Fallot .Both lesions were successfully corrected surgically.peer-reviewe

Rapidly progressing giant aneurysm of right ventricular outflow tract with severe conduit obstruction : report of two cases

Pseudoaneurysm of the right ventricular outflow tract (RVOT) has been reported as a rare complication of RVOT reconstruction performed using conduit replacement or patch repair. The RVOT needs to be followed up on echocardiography and if aneurysm a large aneurysm forms, it needs to be operated. Usually progression of aneurysm is slow. Rarely, it may present with symptoms secondary to the compression of adjoining mediastinal structures or thrombus formation inside the aneurysm . We report two cases of rapidly progressing massive RVOT aneurysm needing early surgical intervention. Both patients had ventricular septal defect (VSD) closure and right ventricle to pulmonary artery conduit placement. In both patients Contegra pulmonary valved conduit (Medtronic) had been used.peer-reviewe

Mycotic aneurysm : a lesser known complication of coarctation repair in a child, managed by endovascular aneurysm repair

We report a 12-year-old child diagnosed with mycotic aneurysm at the site of coarctation of aorta (CoA) repair. Surgical intervention, though very high risk, is the mainstay of management. In selected cases endovascular stenting is being reported in adults. Here we report the use of an endovascular stent in a child.peer-reviewe

Symptomatic giant left atrial aneurysm in a child : a rare entity

Isolated left atrial aneurysms are rare entities in clinical practice. Usually the condition is diagnosed in the second to fourth decades of life. The presence of such lesions in the pediatric age group is scantily described. We present a 2 year boy who presented with complaints of excessive irritability, respiratory distress and swelling of the feet. On examination, child was tachypnoeic with irregularly irregular rhythm. Echo showed a huge aneurysmal LA appendage with severe left ventricle dysfunction. The child underwent surgical resection for same. Findings were confirmed intraoperatively but he continued to have low cardiac output state after the surgery, with frequent arrhythmias and expired on day 7 of surgery. The case is reviewed and compared with the available English literature.peer-reviewe

Ductal aneurysm masquerading as nonresolving pneumonia: A challenging differential!

We report here, the case of a six-and-a-half-month-old boy investigated for persistent respiratory distress and homogeneous opacity in the left upper lobe. Echocardiography revealed a giant ductal aneurysm compressing the left pulmonary artery and upper lobe division of the left bronchus. Computerized tomography angiogram delineated the exact anatomy and prompt surgical resection provided a successful cure to this lesser known entity