Many patients with congenital heart disease and systemic-to-pulmonary shunts develop pulmonary arterial hypertension (PAH), particularly if the cardiac defect is left unrepaired. A persistent increase in pulmonary blood flow may lead to obstructive arteriopathy and increased pulmonary vascular resistance, a condition that can lead to reversal of shunt and cyanosis (Eisenmenger syndrome). Cardia...

Primary cardiac tumor in infancy is an uncommon condition which is rarely accompanied with congenital heart disease. Although these tumors are generally benign, the complication associated with it, such as arrhythmia, outflow tract obstruction, and heart failure, may result in early mortality, when combined with congenital heart disease. Early surgical treatment may reduce complication risk and...

IFE expectancy and the degree of invalidism have been considered a function of the type of cardiac malformation in patients with congenital heart disease. When the abnormality involves a large communication between the ventricles or great vessels, however, the pulmonary vascular bed plays an important role in governing prognosis. The following group of congenital malformations are involved in t...

We report a mother and daughter with features of the velocardiofacial (VCF) syndrome and monosomy for 22q11 identified using molecular techniques. The mother had surgery as a child for a cleft palate and a congenital heart defect, and her facial features were consistent with the diagnosis. The daughter had developmental delay, absent speech, scoliosis, and similar facial features, but no cleft ...

We report the case of a 43-day-old boy with branchio-oculo-facial syndrome (BOFS) and congenital heart defect. On clinical examination, he presented growth retardation, epicanthal folds, small palpebral fissures, telecanthus, broadened nasal bridge, lip pseudocleft, micrognathia, dysplastic and posteriorly-rotated ears, branchial clefts, short and webbed neck, supernumerary nipple, hypotonia an...

Congenital heart defects are a major congenital abnormality and are assuming increasing importance. A study was undertaken to estimate the incidence of congenital heart defects in Northern Ireland over a five year period (1974-1978), to determine the age at diagnosis and to assess the risk of recurrence in sibs. An incidence rate of 7.3 per 1000 total births was found. This reduced to 3.1 per 1...