Real-world, long-term survival of incident patients with pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive, fatal disease. Long-term outcomes data are scarce in Portugal. We aimed to estimate survival of newly diagnosed PAH at a Portuguese referral center in the modern management era. METHODS: Between January 2009 and November 2015 all incident PAH cases were consecutively enrolled in a prospective cohort study. Sixty-five patients were followed up for a median of 3.1 [interquartile range 1.7-5.4] years. Kaplan-Meier survival analysis was used to estimate 1-, 3-, and 5-year survival and to compare it with a historical PAH survival estimated from the NIH cohort. RESULTS: Mean age was 48±19 years with female preponderance (68%). The most common PAH subgroup was congenital heart disease (PAH-CHD) (n=31; 48%), followed by connective tissue disease (PAH-CTD) (n=16; 25%), idiopathic (IPAH) (n=8; 12%) and hereditary (HPAP) (n=1; 1.5%). BNP values (hazard ratio [HR] 2.07; 95%CI 1.34-3.22; P=0.001) and male gender [HR 4.34 (1.44-13.09); P=0.009] were predictors of death. Survival rates at 1-, 3- and 5-years were 95%, 77% and 71%. Survival was not statistically different between PAH etiologies (Log-rank P=0.7). However, PAH-CHD was associated with a decreased risk of the combined endpoint of all-cause mortality and admission for decompensated heart failure [HR 0.36 (0.15-0.85); P=0.02]. We found a non-significant numerically higher survival of incident IPAH, HPAH and DPAH patients in comparison with the historical NIH cohort. CONCLUSIONS: In this cohort of incident PAH patients, PAH-CHD patients had better overall prognosis. Higher BNP values and male gender were associated with higher mortality.

[Angioplasty in aortic coarctation and recoarctation in children and adolescents]. / Angioplastia na coartação e recoartação da aorta em crianças e adolescentes.

STUDY OBJECTIVE: To determine the efficacy of percutaneous balloon angioplasty in children and adolescents with Coarctation and Recoarctation of the Aorta. DESIGN AND SETTING: We dilated native coarctation and recoarctation after a complete hemodynamic and angiographic assessment of the situation in a young population referred to a university hospital. PATIENTS AND INTERVENTION: In 22 patients with coarctation or recoarctation of the aorta, the systolic pressure gradient and the internal diameter of the stenotic area were measured before and after dilation, with a balloon catheter with an insufflation diameter no greater than the aortic diameter measured at the level of the diagram muscle. No infants were included nor patients with a rare aortic anatomy. RESULTS: A significant gradient reduction from 36.59 +/- 12.03 mm Hg to 10.45 +/- 7.52 mm Hg, as well an increase from 4.54 +/- 2.04 mm to 8.05 +/- 2.82 mm in aortic internal diameter, were achieved with no complications. CONCLUSIONS: Similar results were obtained both in native coarctation and recoarctation without significant complications. Age selection, aortic anatomy and balloon size were the major determinants of success.

True double aortic lumen in tetralogy of Fallot.

In an infant with tetralogy of Fallot submitted to angiographic study, a double aortic lumen was found. Although no particular complaints were related to the additional abnormality, we report this case to emphasize the possibility of occurrence of persistent embryonic fifth aortic arch.

[Right coronary fistula to the right atrium. Diagnosis using Doppler echocardiography with color coding]. / Fístula coronária direita para a aurícula direita. Diagnóstico por Eco Doppler com codificação em cor.

A case of right coronary fistula draining to the right atrium which was diagnosed by Echo Doppler and colour flow mapping is presented. A patent ductus arterious was initially diagnosed clinically on this child and he was been waiting for 2D Echo and Doppler registration. The patient is now on waiting list for corrective surgery and a cardiac catheterization confirmed totally our diagnosis obtained by non invasive methods.