Membranous septal aneurysm in a child with Noonan syndrome and hypertrophic cardiomyopathy.

Unlabelled: A 4-year-old boy was diagnosed with hypertrophic cardiomyopathy with moderate degree of obstruction of the left ventricular outflow tract, secondary to asymmetric septal hypertrophy during infancy. Echocardiogram on follow-up showed increase in left ventricular outflow tract obstruction and appearance of an aneurysm in the membranous portion of interventricular septum. The appearance of the membranous septal aneurysm may be related to the direction of jet arising from the point of contact of the anterior mitral leaflet with the hypertrophied basal septum and hitting this part of the interventricular septum.

Efficacy, safety and tolerability of bosentan as an adjuvant to sildenafil and sildenafil alone in persistant pulmonary hypertension of newborn (PPHN).

Background: Pulmonary Arterial Hypertension (PAH) carries a poor prognosis in both adult and pediatric patients. It is a life-threatening condition in newborns. Current recommendations advocate the use of targeted monotherapy as a first-line approach for the treatment of Persistent Pulmonary Hypertension of the Newborn (PPHN).

Unguarded left atrioventricular orifice: An unusual cause of hypoplastic left ventricle and double-outlet right ventricle with intact ventricular septum.

An unguarded atrioventricular orifice is an extremely rare congenital anomaly characterized by the absence of the atrioventricular valve in varying proportions. While atresia of the mitral or aortic valves are usually described as causes for hypoplastic left heart, our case highlights the role of free atrioventricular valve regurgitation and consequent volume loss of the left heart, giving rise to a small left ventricle. There was an associated double-outlet right ventricle and Type B aortic interruption.

Three-dimensional transesophageal echocardiography-guided transcathetar closure of ruptured noncoronary sinus of valsalva aneurysm.

Sinus of Valsalva aneurysm accounts for only 1% of congenital cardiac anomalies. Sinus of Valsalva aneurysm can cause aortic insufficiency, coronary artery flow compromise, cardiac arrhythmia, or aneurysm rupture. Three-dimensional transesophageal echocardiography (3DTEE) represents an adjunctive tool to demonstrate the ruptured sinus of Valsalva with better delineation.

Clinical, angiographic profile and percutaneous endovascular management of Takayasu's arteritis - A single centre experience.

Objective: Aim of the study was to evaluate clinical, angiographic profile and percutaneous endovascular management of Takayasu's arteritis.

Background: Takayasu's arteritis is a chronic inflammatory vasculitis affecting the aorta and its major branches. Although it is more prevalent in Asia, the distribution of the disease is worldwide with different vascular involvement patterns and clinical manifestations.

Angiographic profile and endovascular interventions in Takayasu's arteritis.

Background: Takayasu's arteritis (TA) is a rare inflammatory, granulomatous vasculitis primarily involving the aorta and its major branches. Clinical and angiographic manifestations of the disease differ in various parts of the world.

Objectives: To analyze the clinical features, angiographic profile, and endovascular interventions in TA patients (pts).

Ebstein's anomaly with subpulmonary obstruction--a rare association.

Ebstein's anomaly comprises a wide spectrum of congenital cardiac abnormalities involving the tricuspid valve (TV) apparatus and the architecture of the morphological right ventricle (RV). We report a case of Ebstein's anomaly in a 3-year-old boy having a unique association with severe subpulmonic obstruction caused by tethered and dysplastic anterior tricuspid leaflet (ATL), and a large ventricular septal defect. In view of poorly functioning RV, the baby was planned for a bidirectional Glenn shunt with subsequent Fontan completion.

Percutaneous transcatheter device closure of coronary arteriovenous fistula through persistent left superior vena cava.

Congenital coronary artery fistula is a rare malformation that may involve any or all coronary artery branches and any cardiac chamber. Elective closure of coronary artery fistula by surgery or percutaneous transcatheter technique is generally accepted in the presence of symptoms. Controversies exist in the management of asymptomatic patients.

Percutaneous recanalization of an occluded hepatic vein in a difficult subset of pediatric Budd-Chiari syndrome.

We present a 3-year-old child with Budd Chiari syndrome having idiopathic complete occlusion of all three major hepatic veins and small hepatic venules. Adequate antegrade flow in right hepatic vein was established by transfemoral balloon angioplasty followed by stenting of the same. Long term antiplatelet therapy was instituted.

Retrograde approach for closure of ruptured sinus of Valsalva.

Though ruptured sinuses of Valsalva have been traditionally managed surgically, they are amenable to transcatheter closure. Various devices have been used for closure of these defects. We describe a novel technique of closure of a ruptured right sinus of Valsalva into the right ventricular outflow tract.