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 (58.2%) were classified as PAH and 33 patients (41.8%) as CTEPH. PAH patients had a mean age of 43.4 ± 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 ± 5.9 mmHg) and mean pulmonary vascular resistance (11.4 ± 6.5 Wood units), with a low cardiac index (2.7 ± 1.1 L·min(-1)·m(-2)); no patient was under selective pulmonary vasodilators; however, at follow-up, most patients were on single (50%), double (28%), or triple (9%) combination vasodilator therapy. One-year survival was 93.5%, similar to CTEPH patients (93.9%), that were older (60.0 ± 12.5 years) and had higher RAP (11.0 ± 5.2 mmHg, P = 0.015). CONCLUSIONS: We describe for the first time nationwide data on the diagnosis, management, and prognosis of PAH and CTEPH patients in Portugal. Clinical presentation and outcomes are comparable with those reported on other national registries.

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. METHODS: We followed 14 patients with PAH and complex CHD for a mean of four years. Demographic parameters, exercise capacity assessed by the six-minute walking test (6MWT) and oxygen saturation were assessed at baseline, six months and at follow-up. RESULTS: Mean age was 37.1 ± 11.7 years; 90% were in WHO class III or IV. The most common diagnosis was pulmonary atresia with VSD (35.7%), followed by truncus arteriosus (28.6%), patent ductus arteriosus (21.4%) and transposition of the great arteries (14.3%). After six months of treatment, six-minute walking distance (6MWD) increased from 371.9 to 428.4 m (p=0.005) and functional class was improved (p=0.005). After four years, one patient discontinued bosentan due to side effects and four patients were started on sildenafil, after a mean 38 months of bosentan treatment. Mean 6MWD for patients on bosentan monotherapy (n=8) was 440.1 ± 103.8 m, whereas for patients on bosentan-sildenafil combination therapy (n=4) it was 428.8 ± 96.9 m, after four years of therapy. Two patients died during follow-up. CONCLUSIONS: Bosentan was safe and was associated with improved exercise capacity in patients with PAH and complex CHD. This improvement was sustained for up to four years and the safety profile was similar to simple CHD patients.

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]. / Recomendações para a abordagem clínica do doente com hipertensão pulmonar.

INTRODUCTION: 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. All of this together has been fundamental in changing the previously unfavourable prognosis of this disease. This evolution implies the need to rationalise the use of available resources through organisation of healthcare services, defining the role of each level of care, and developing norms for good clinical management practices in keeping with best medical practice guidelines. These twin aspects have attracted the interest of the scientific community, as shown by the wealth of literature, and have led healthcare authorities to introduce regulatory mechanisms. In order to improve clinical practice, the Pulmonary Vascular Disease Study Group (NEDVP) of the Portuguese Society of Internal Medicine (SPMI), the Pulmonary Hypertension Study Group (GEHTP) of the Portuguese Society of Cardiology and the boards of the Portuguese Societies of Pulmonology and Paediatric Cardiology created an interdisciplinary working group. The group's remit was to draft this document, "Guidelines for the management of pulmonary hypertension patients", based on a review of the literature and the authors' clinical expertise. These guidelines aim to present all the relevant evidence on the diagnostic and treatment strategy of PH and the definition of requirements for referral centres. The organisation of care is fundamental for an appropriate and rational use of the available resources and for the better care of the patient.

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. Median gestational age (GA) was 28 weeks and birth weight (BW) 1100 grams. The diagnosis of PDA was established, on average, between the fifth and sixth day of life. Of the 53 newborns with criteria for closure, 49 had indomethacin therapy, with a success rate of 88%; surgical ligation was subsequently necessary in six of these. Thirty-eight newborns presented associated comorbidities, and eight died. CONCLUSIONS: The results obtained in this study are in agreement with the literature. In cases with GA of less than 30 weeks and/or BW below 1500g, the need for treatment for PDA closure is greater than in cases with higher GA/BW, and a more aggressive approach is required, with presymptomatic prolonged indomethacin. The results obtained in this work led us to conclude that echocardiography should be performed to screen for PDA in all newborns of less than 30 weeks or with BW below 1500g. We intend in the near future to answer a question that emerged from this study: will earlier diagnosis lead to improved outcomes?

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. The retrieval equipment used included a pigtail catheter, multipurpose catheter with hand-prepared snare, Amplatz gooseneck snare, nitinol multisnare set and basket catheter. The foreign bodies were successfully removed percutaneously in all cases, with no complications.

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.