Pulmonary hypertension in Portugal: first data from a nationwide registry.

Introduction: Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical.

Methods: We conducted a prospective, observational, and multicenter registry with a joint collaboration from five centers from Portugal and included adult incident patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH).

Results: Of the 79 patients enrolled in this study, 46 (58.

Long-term effect of bosentan in pulmonary hypertension associated with complex congenital heart disease.

Background: Bosentan is recommended for symptomatic patients with Eisenmenger syndrome due to simple congenital lesions such as atrial and ventricular septal defects (VSD). However, its long-term efficacy and safety in patients with pulmonary arterial hypertension (PAH) associated with complex congenital heart disease (CHD) is unknown.

Objectives: We examined the short- and long-term effects and safety profile of bosentan in patients with PAH and complex CHD.

Ascending aortic aneurysm and patent foramen ovale: a rare cause of platypnea-orthodeoxia.

Platypnea-orthodeoxia is a rare syndrome characterized by dyspnea and hypoxia induced by the upright position and relieved by the supine position. Several factors related to atrial anatomy can facilitate shunting through an atrial septal defect; in many cases, the syndrome is associated with patent foramen ovale and right-to-left shunt, and has also been linked to aortic aneurysm. We present a case of platypnea-orthodeoxia syndrome in a 61-year-old woman with patent foramen ovale and ascending aortic aneurysm.

[Guidelines for the management of pulmonary hypertension patients].

Pulmonary hypertension (PH) was until recently an obscure pathology which frequently went unidentified as it lacked a precise diagnostic strategy. Recent years have seen advances in the knowledge of the pathogenesis and mechanisms of vascular lesion of PH. This has led to the scientific community's growing interest in this area, an interest manifested in appreciable progress in the pathology's clinical characterisation, diagnostic strategies and the development of effective drugs.

Pulmonary veno-occlusive disease: a case report.

The authors present the case of a 22-year-old female patient, white, referred to the cardiovascular outpatient clinic in November 2006 for pulmonary arterial hypertension. Complementary diagnostic exams revealed elevated pulmonary arterial pressure, normal capillary wedge pressure and a reduced cardiac index on invasive hemodynamic study. A high-resolution pulmonary CT scan identified a diffuse ground-glass opacity with a centrilobular pattern, and a marked decrease in CO diffusion on respiratory function assessment.

Patent ductus arteriosus--neonatal intensive care unit registry.

Objectives: This study took place at the Neonatal Intensive Care Unit (NICU) of the Dr Daniel de Matos Maternity Hospital and evaluated the incidence of patent ductus arteriosus (PDA), the clinical evolution of the newborns affected, the treatment prescribed, and associated morbidity and mortality.

Methods: We carried out a retrospective medical chart review of newborns admitted to the NICU between January 2001 and December 2005.

Results: PDA was found in 69 newborns.

Percutaneous retrieval of foreign bodies from the cardiovascular system.

We report our experience of seven patients referred to our hospital with foreign bodies embolized in the cardiovascular system, namely fragmented catheters and devices used in interventional techniques, which were retrieved by a percutaneous approach. The patients' ages ranged from 2 to 29 years, with a mean age of 17. The majority (57%) were male.

Ectopic origin of the right coronary artery--case report.

Coronary artery anomalies can occur in isolation or in association with other congenital heart defects. Clinical presentation ranges from asymptomatic forms to sudden cardiac death. The authors report a case of anomalous origin of the right coronary artery from the left sinus of Valsalva, diagnosed with multidetector computed tomography coronary angiography.

Cardiac tumors in the pediatric age group--case reports.

We present two case reports of primary cardiac tumors diagnosed in the pediatric age group, with different clinical evolution and therapeutic approach.