Congenital heart disease and pulmonary arterial hypertension in South America (2013 Grover Conference series).

South America is a territory of 17,819,100 km(2), where ∼388 million people live in 13 countries. In the region, access to medical assistance (e.g.

ALDH1A2 (RALDH2) genetic variation in human congenital heart disease.

Background: Signaling by the vitamin A-derived morphogen retinoic acid (RA) is required at multiple steps of cardiac development. Since conversion of retinaldehyde to RA by retinaldehyde dehydrogenase type II (ALDH1A2, a.k.

Platelet protease-activated receptor 1 and membrane expression of P-selectin in pulmonary arterial hypertension.

Introduction: Pulmonary arterial hypertension (PAH) is frequently associated with thrombotic events, particularly involving the pulmonary microcirculation at sites of vascular injury. We therefore decided to analyse protease-activated receptor 1 (PAR1), a key element in the activation of human platelets by thrombin, in PAH patients in stable clinical condition.

Methods: Using flow cytometry, we analyzed platelet PAR1 density, PAR1-mediated exposure of P-selectin and the formation of platelet-leukocyte aggregates in 30 PAH patients aged 11 to 78 years (median 50.

Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension.

Purpose: In patients with pulmonary hypertension, extrinsic compression of the left main coronary artery by a dilated pulmonary trunk may cause angina, left ventricular ischemia, and sudden death. We assessed coronary artery compression in relation to pulmonary trunk diameter and other demographic, echocardiographic, hemodynamic, and scintigraphic variables.

Methods: Thirty-six patients (aged 15 to 86 years) with pulmonary hypertension, either idiopathic or associated with congenital heart disease, were enrolled.

Arterial hypertension in a child with Williams-Beuren syndrome (7q11.23 chromosomal deletion).

We report the case of a 7-year-old male child diagnosed with Williams-Beuren syndrome and arterial hypertension refractory to clinical treatment. The diagnosis was confirmed by genetic study. Narrowing of the descending aorta and stenosis of the renal arteries were also diagnosed.