The clinical and anatomical findings in two patients with isolated ventricular inversion and situs solitus are described. The other 4 previously published cases are reviewed. The 6 patients with this malformation, all without pulmonary stenosis, presented a clinical picture of cyanotic congenital heart disease, associated with increased pulmonary blood flow (hypoxaemia and cardiac failure). The...

Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly due to a malformation of the sixth aortic arch of the affected side during embryogenesis. It can occur in isolation or may be associated with other congenital cardiovascular malformations. The diagnosis is usually set at adolescence, however it can remain asymptomatic and late diagnosis is possible. Recurrent pulmonary inf...

Among certain patients with cardiovascular malformations, a common phenomenon is a ventricular septal defect and absence of anatomic origin of the pulmonary arterial supply from the heart. In this situation, true pulmonary arteries may be present or absent. In the latter case, the pulmonary arterial supply comes through bronchial arteries. When true pulmonary arteries are present, the left and ...

Pulmonary arteriovenous malformations (PAVMs) are vascular anomalies of the lung and carry the risk of cerebral thromboembolism, brain abscess, or pulmonary hemorrhage. We describe a 64-year-old male with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) who presented with a five-year history of progressive effort dyspnea and a PAVM in the right upper lobe successfully treated ...

Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by a triad of cutaneous vascular nevi, soft tissue or bony hypertrophy, and varicose veins or venous malformations involving one or more extremities. An incidence of venous thromboembolism of up to 22% has been reported in this disorder. Also reported is the development of chronic thromboembolic pulmonary hypertension (CTEP...

Hereditary Haemorrhagic Telangiectasia (HHT), or Osler-Weber-Rendu syndrome is an uncommon autosomal dominant multi-organ condition of vascular dysplasias. We describe a 19 year old Indian female who presented with cerebral abscess secondary to paradoxical emboli from pulmonary arteriovenous malformations (PAVMs) associated with HHT. Cerebral, pulmonary, hepatic and gastrointestinal involvement...

The results of banding of the pulmonary artery in 111 children with cardiac malformations associated with excessive pulmonary blood flow are presented. Thirty-three of these children died, eleven either of late band complications or after a corrective operation. Patients with ventricular septal defect represent the largest group of malformation among the 111 patients. The overall mortality for ...

The results of banding of the pulmonary artery in 111 children with cardiac malformations associated with excessive pulmonary blood flow are presented. Thirty-three of these children died, eleven either of late band complications or after a corrective operation. Patients with ventricular septal defect represent the largest group of malformation among the 111 patients. The overall mortality for ...

correlation between sacral ratio and bowel function as well as fecal continence has been well documented in patients with anorectal malformations (arms). one hundred and twenty children with arms were investigated in this study. sacral ratio (sr) was measured from pelvic x-rays of patients. among these, 52 patients (43%) had no pelvic x-ray and were excluded from this study. sr was measured by ...

Tetralogy of Fallot (TOF) is one of the most common congenital heart malformations comprising a ventricular septal defect, right ventricular outflow tract obstruction, right ventricular hypertrophy, and overriding aorta. A rare variant includes pulmonary atresia and major aortopulmonary collateral arteries. Altered hemodynamics within the functional single-ventricle results in turbulent flow an...