Chronic thromboembolic pulmonary hypertension in pediatric patients.

Objective: Chronic thromboembolic pulmonary hypertension is a rare form of pulmonary hypertension that can lead to progressive right heart failure and death. Pulmonary thromboendarterectomy surgery is the treatment of choice resulting in significant improvements in functional status, cardiopulmonary hemodynamics, and survival. This study reports the largest case series of pediatric patients with chronic thromboembolic pulmonary hypertension who underwent pulmonary thromboendarterectomy surgery at one institution.

Successful surgical closure of an arterial duct in 18 children in a third world country.

Objectives: To perform surgical closure of a clinically significant arterial duct on children in a third world country.

Background: An arterial duct is one of the most common congenital cardiac defects. Large arterial ducts can cause significant pulmonary overcirculation, causing symptoms of congestive cardiac failure, ultimately resulting in premature death.

Mitral/systemic atrioventricular valve repair in congenital heart disease.

Because there is no ideal substitute for the mitral or the systemic atrioventricular valve (SAVV) in a patient of any age, repair is the optimal treatment for important congenital or acquired mitral/SAVV disease. Valve repair techniques have evolved to the point where early repair may be offered to asymptomatic patients with favorable anatomy. The indications for operation depend on the presence or absence of symptoms and the physiologic consequences of mitral/SAVV pathology.

Bone morphogenetic proteins induce apoptosis in human pulmonary vascular smooth muscle cells.

Pulmonary vascular medial hypertrophy in primary pulmonary hypertension (PPH) is mainly caused by increased proliferation and decreased apoptosis in pulmonary artery smooth muscle cells (PASMCs). Mutations of the bone morphogenetic protein (BMP) receptor type II (BMP-RII) gene have been implicated in patients with familial and sporadic PPH. The objective of this study was to elucidate the apoptotic effects of BMPs on normal human PASMCs and to examine whether BMP-induced effects are altered in PASMCs from PPH patients.

Increased expression of endoarterial vascular cell adhesion molecule-1 mRNA in an experimental model of lung transplant rejection: diagnosis by pulmonary arterial biopsy.

Background: Early detection of rejection after lung transplantation may prevent allograft failure. This study determines if mRNA from the cell adhesion molecules intercellular adhesion molecule-1, vascular cell adhesion molecule (VCAM)-1, and E-selectin in pulmonary endovascular tissue samples could be markers of early rejection.

Methods: Single left lung transplants were performed in five dogs.