Use of the pressure wire method for measuring pulmonary arterial pressures in patients with pulmonary atresia.

OBJECTIVE: The objective of the study was to analyse the use of the pressure wire for the acquisition of intravascular pulmonary pressures in the presence of pulmonary atresia and systemic-dependent pulmonary blood flow. METHODS: In this study, we included patients with pulmonary atresia and systemic-dependent pulmonary circulation referred for diagnostic catheterisation for evaluation of pulmonary pressures during the period from April, 2012 to April, 2013. The systemic-pulmonary collateral arteries were selectively catheterised, and in the absence of a critical stenosis angiographically determined; the pressure wire was introduced in these arteries to reach the main pulmonary artery, and/or lobar, and segmental branches. Aortic and pulmonary pressures were simultaneously obtained. We evaluated the feasibility and safety of the method. RESULTS: We studied 10 patients (age 21 days to 11 years). In all of them, the pressures of pulmonary circulation - main artery, and/or lobar, and segmental branches - were successfully measured with the pressure wire. Of eight patients with indication for Rastelli surgery, the pulmonary pressures were considered normal in five, and slightly increased in three. In two patients requiring univentricular correction - total cavopulmonary anastomosis - the diastolic pressure was increased (20 mmHg). All procedures were performed without haemodynamic instability, cardiac arrhythmia, systemic saturation reduction, death, or any other complication. CONCLUSION: Measurement of pulmonary vascular pressures using the pressure wire in small patients with pulmonary atresia is safe and effective. It allows the acquisition of reliable pressure curves, even in the presence of small vessels, bending and tortuosity, without the risk usually associated with the use of conventional diagnostic catheters.

Atresia congênita do óstio da artéria coronária esquerda. Dificuldade diagnóstica e revascularizaçäo cirúrgica bem sucedida em dois pacientes / Congenital atresia of the ostium of the left coronary artery. Diagnostic difficulty and successful surgical revascularization in two patients

We report two cases of congenital atresia of the ostium of the left coronary artery. Case 1: a six-month-old infant presenting with serious cardiac insufficiency. A noninvasive diagnosis of dilated myocardiopathy was established and the clinical picture was pharmacologically compensated. When the patient was nine months of age, a hemodynamic study was performed that revealed congenital atresia of the ostium of the left coronary artery; the infant immediately underwent a successful anastomosis of the internal mammary artery with the left coronary artery. Case 2: an eleven-year-old asymptomatic boy with a history of heart murmur from the age of six months on, was refered for surgery with a diagnosis of anomalous origin of the left coronary artery from pulmonary trunk. A definitive diagnosis of atresia of the left coronary ostium was only established during surgery. Successful surgical revascularization with the left internal mammary artery, and left ventricular aneurysmectomy were performed.

Hipoplasia congênita isolada do ventrículo direito / Congenital isolated hypoplasia of the right ventricle

Two cases of congenital trabecular hypoplasia of the right ventricle are reported. In the first, the neonatal diagnosis was missed and the child did well until the 13th month of life when a modified Blalock-Taussig shunt was done because of increasing cyanosis. Outcome was good until the 4th year of life when symptomatic atrioventricular block was detected in an emergency situation. A bidirectional Glenn anastomosis and pacemaker implantation were successfully carried out after clinical establization and the child is doing well up to now. The second case presents the disease with its worst features: severe cyanosis and acidosis in the first day of life. A modified Blalock-Taussig shunt was performed and death occurred soon after the operation.