Atrasentan treatment of pulmonary vascular disease in piglets with increased pulmonary blood flow.

We studied the effect of chronic endothelin A receptor blockade by atrasentan on the pulmonary endothelin-1 system and vascular endothelial growth factor (VEGF) expression in piglets with high pulmonary blood flow. Twenty-five 4-week-old piglets with high pulmonary blood flow were randomized to three groups: sham operated (n = 8), placebo (water) (n = 7), or treatment with atrasentan (2 mg/kg per day) (n = 10). After 3 months, mean pulmonary arterial pressure (PAP) was higher in the placebo group than in the sham group [18 +/- 2 mm Hg versus 14 +/- 1 mm Hg; P < 0.

Pulmonary vascular changes in piglets with increased pulmonary blood flow and pressure.

In this model of pulmonary vascular disease, high pulmonary blood flow was created by an anastomosis between the left subclavian artery and the main pulmonary artery [Blalock-Taussig (BT) shunt] in 4-week-old piglets (n = 6). Additional ligation of the left pulmonary artery (LPA) was used to increase pulmonary artery pressure (n = 6). Seven piglets were sham-operated.

Late results after extended pulmonary artery reconstruction in the arterial switch operation.

Background: Pulmonary artery stenosis remains the most frequent late complication and cause of reintervention after the arterial switch operation for transposition of the great arteries. We investigated the influence of an extended pericardial patch augmentation of the neopulmonary root and pulmonary artery on late pulmonary artery stenosis development.

Methods: Augmentation of the neopulmonary root and pulmonary artery was achieved by reconstructing the posterior wall using a large glutaraldehyde-treated autologous pericardial patch.

Plasma L-arginine and metabolites of nitric oxide synthase in patients with left-to-right shunt after intracardiac repair.

Study Objective: Human plasma L-arginine serves as a substrate pool for endothelial-derived nitric oxide (NO) synthase. In this pilot study, we tested the hypothesis that plasma L-arginine and other metabolites of the L-arginine NO pathway could correlate with postoperative pulmonary hypertension after cardiopulmonary bypass (CPB).

Design: Forty-two patients (median age, 0.

Ventricularization of the atrialized chamber: a concept of Ebstein's anomaly repair.

Background: We report results of a technique of Ebstein's anomaly repair by creating a predominantly monocuspid valve with simultaneous ventricularization of the atrialized right ventricular (aRV) chamber.

Methods: Between March 1993 and April 2003, Ebstein's anomaly repair by valvuloplasty with combined ventricularization was performed in 23 patients aged 13.6 (4.

Pulmonary hypertension in infancy and childhood.

In this review, we discuss current concepts in the pathogenesis and management of pulmonary hypertension affecting infants and children, with special focus on left-to-right shunting, bronchopulmonary dysplasia, and primary pulmonary hypertension. In patients of these ages, functional aspects, such as an imbalance between vasoconstricting and vasodilating mechanisms, and morphological alterations of the vessel wall, contribute to the pulmonary hypertension. In the past decades, strategies have emerged for treatment that are targeted at the pathophysiological basis.

Long-term results after reconstruction of the left ventricular outflow tract by aortoventriculoplasty.

Background: Aortoventriculoplasty is an established method of reconstruction of complex left ventricular outflow tract (LVOT) obstruction by insertion of a mechanical valve prosthesis after patch enlargement of the LVOT. Little data exist with respect to long-term outcome.

Methods: Between March 1991 and June 2001, 24 patients with a median age of 10.