aly characterized by the absence of the pulmonary valve. The absence of the pulmonary valve, along with the hypoplasia of the pulmonary annulus, leads to aneurysmal dilatation of the pulmonary artery and its branches. Other consequences include pulmonary stenosis and pulmonary insufficiency (1). The 3-day-old baby presented with cyanosis and tachypnea. He had to and fro murmurs at the upper lef...

BACKGROUND Lung hypoplasia, pulmonary persistent hypertension of the newborn and its morphological changes are the main features in congenital diaphragmatic hernia (CDH). This study was undertaken to investigate if antenatal use of sildenafil and/or bosentan attenuates vascular remodeling, promotes branching, and improves alveolarization in experimental nitrofeninduced CDH. METHODS Nitrofen (...

Pulmonary hypoplasia is common in the perinatal period and is a significant cause of death in newborn infants, and oligohydramnios is one of the most commonly associated abnormalities.1 Oligohydramnios may retard fetal lung growth and can result in pulmonary hypoplasia in experimental animals and human fetuses with prolonged rupture of membranes.2,3 Neonates exposed to oligohydramnios caused by...

BACKGROUND Hydrops foetalis is defined as excessive fluid accumulation within the foetal extravascular compartments and body cavities. It has been described in human and veterinary medicine, but despite several descriptive studies its aetiology is still not fully clarified. Pulmonary hypoplasia and anasarca (PHA) syndrome is a rare congenital abnormality in cattle that is characterised by hydro...

BACKGROUND We hypothesized that gene transfer of vascular endothelial growth factor (VEGF) mediated by an adenovirus vector might induce pulmonary artery angiogenesis in a lamb model of pulmonary artery hypoplasia. METHODS Thirteen fetal lambs had left pulmonary artery banding at 106 days of gestation. Following birth, 3 groups were divided: VEGF group (n = 5) and beta-GAL group (n = 4) recei...

The patient is a 41-year-old woman who was referred to our hospital because of an abnormal chest radiograph. On admission, she was asymptomatic, but her cardiac silhouette was shifted to the right on chest radiograph. From contrast-enhanced chest computed tomography (CT), we suspected that the right upper pulmonary vein connected to the inferior vena cava. The CT also revealed hypoplasia of the...

A 10-year old girl underwent a CT angiogram to investigate right lung hypoplasia. This showed a normal bronchial tree, lung parenchyma and pulmonary venous drainage, but an absent right pulmonary artery. Cardiac catheterization with pulmonary vein wedge injections identified a disconnected hypoplastic right pulmonary artery system supplied by an occluded right-sided ductus arteriosus. Transcath...

T etralogy of Fallot is the most common cyanotic congenital heart lesion. Its anatomical features were first described by Stenson in 1672. In 1888 Fallot published his clinical observations, linking the four elements he considered to be the main anatomical abnormalities (interventricular septal defect, stenosis of the right ventricular outflow tract, right ventricular hypertrophy and an aorta t...