Multiple arterial anomalies characterized by tortuosity and rolling of the pulmonary arteries and aorta were diagnosed on echocardiography in an asymptomatic newborn infant with a phenotype suggesting Ehlers-Danlos syndrome. These changes were later confirmed on angiography, which also showed peripheral vascular abnormalities. The electrocardiogram showed a probable hemiblock of the left anterosuperior branch, and the chest x-ray showed an excavated pulmonary trunk with normal pulmonary flow.

It has been reported that schistosomiasis-associated PAH (Sch-PAH) has a more benign clinical course compared with idiopathic PAH (IPAH). We therefore hypothesized that Sch-PAH subjects would present with less impaired cardiopulmonary and metabolic responses to exercise than IPAH patients, even with similar resting pulmonary hemodynamic abnormalities. The aim of this study was to contrast physiologic responses to incremental exercise on cycle ergometer between subjects with Sch-PAH and IPAH. We performed incremental cardiopulmonary exercise tests (CPET) in subjects newly diagnosed with IPAH (n = 9) and Sch-PAH (n = 8), within 1 month of the hemodynamic study and before the initiation of specific therapy for PAH. There were no significant between-group differences in cardiac index, pulmonary vascular resistance or mean pulmonary artery pressure. However, mean peak oxygen uptake (VO2) was greater in Sch-PAH than IPAH patients (75.5±21.4 vs 54.1±16.1% predicted, p = 0.016), as well as the ratio of increase in VO2 to work rate (8.2±1.0 vs 6.8±1.8 mL/min/W, p = 0.03). Additionally, the slope of the ventilatory response as a function of CO2 output was lower in Sch-PAH (40.3±3.9 vs 55.6±19.8; p = 0.04), and the heart rate response for a given change in VO2 was also diminished in Sch-PAH compared to IPAH (80.1±20.6 vs 123.0±39.2 beats/L/min; p = 0.02). In conclusion, Sch-PAH patients had less impaired physiological responses to exercise than IPAH subjects with similar resting hemodynamic dysfunction. Our data suggest a more preserved cardiopulmonary response to exercise in Sch-PAH which might be related to its better clinical course compared to IPAH.

The VTIpv correlated well with the PVR. The measurement of this index before and after oxygen inhalation may become a useful noninvasive test for differentiating persistent vascular disease from dynamic and flow-related pulmonary hypertension.

ObjectiveThis study aims to investigate the incidence of postnatal diagnosis of congenital heart disease (CHD) and the predictive factors for hospital mortality.MethodsThis retrospective cohort study was conducted at a Brazilian tertiary center, and data were collected from medical records with inclusion criteria defined as any newborn with CHD diagnosed in the postnatal period delivered between 2015 and 2017. Univariate and multivariate analyses were performed to determine the potential risk factors for mortality.ResultsDuring the 3-year period, 119 (5.3%) children of the 2215 children delivered at our institution were diagnosed with CHD. We considered birth weight (P=0.005), 1st min Apgar score (P=0.001), and CHD complexity (P=0.013) as independent risk factors for in-hospital mortality. The most common CHD was ventricular septal defect. Indeed, 60.5% cases were considered as "complex" or "significant" CHDs. Heart surgeries were performed on 38.9% children, 15 of whom had "complex" or "significant" CHD. A mortality rate of 42% was observed in this cohort, with 28% occurring within the initial 24 h after delivery and 38% occurring in patients admitted for heart surgery.ConclusionThe postnatal incidence of CHD at our service was 5.3%. Low 1st min Apgar score, low birth weight, and CHD complexity were the independent factors that affected the hospital outcome.

Coronary artery fistula is a rare disease accounting for approximately 0.2% to 0.4% of all congenital cardiac defects 1 . It was first described by Krause in 1865 and, since then, approximately 400 cases have been reported 2 with a large variation in regard to the site of origin and drainage in the cardiac chambers. Congestive heart failure in the neonatal period resulting from coronary artery fistula is extremely infrequent, and most of the time it is diagnosed in a routine examination because of the presence of a continuous murmur.With the appearance of Doppler echocardiography, the diagnosis could be established in a noninvasive way 3 , leaving the invasive procedures for confirmation and treatment of the disease.We report a case of right coronary artery fistula with congestive heart failure in a neonate diagnosed through Doppler echocardiography and confirmed with angiography. The fistula was closed in the catheterization room. Case ReportThe patient is a full-term male neonate weighing 3,900g. He was born from a normal delivery, the first child, and from the São Paulo area. No abnormalities in regard to the gestational antecedents were reported. The main complaint was dyspnea on feeding since the first day of life. On the third day of life, the patient was admitted to the hospital because of dyspnea and jaundice.The patient was transferred to our service at the age of 40 days in regular condition, pale (+/4+), hydrated, anicteric, acyanotic, afebrile, tachycardic, tachypneic, active, and reactive.On physical examination, the liver was palpated 3cm from the right costal margin, the spleen was unpalpable, and lung auscultation revealed symmetric respiratory sounds, without other noises. Diastolic thrill could be heard on the lower left sternal margin and cardiac auscultation showed rhythmic beats of normal intensity with a continuous murmur (3+/4+), more intense in the lower left parasternal region irradiating to the base of the back. Hemoglobin and hematocrit values were 10.5g/dL and 31%, respectively.The electrocardiogram showed a sinus rhythm, heart rate of 160 bpm, biventricular hypertrophy, with predominance of the left ventricle, and diffuse alterations in the ventricular repolarization (primary inversion of the T wave in some leads, without necrotic areas). Chest X-ray showed global cardiomegaly (2+/4+) with bilateral increase in pulmonary flow.The patient received furosemide, spironolactone, and Cedilanid-D. Doppler echocardiography was then performed showing situs solidus, atrioventricular and ventriculoarterial concordance, intact interatrial and interventricular septa, moderate dilation of the right ventricle, and significant reduction in its systolic function (hypokinesia of