We describe the clinicopathological features of a case of total anomalous pulmonary vein drainage (TAPVD) associated with atresia of the common pulmonary vein (ACPV). A male Japanese infant born at 37 weeks of gestation demonstrated apnea and severe respiratory acidosis immediately after delivery. The patient died of hypoxemic respiratory failure 6 days after birth despite the initiation of art...

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...

Changes in the radiological appearances of the hilar region of the lungs may indicate that the pulmonary blood supply is not normal. In atresia of the pulmonary valve, the pulmonary arteries may be absent, but more often they are small and may be supplied or supplemented by anomalous vessels. Then blood reaches the lungs mainly by the bronchial arteries or by other collaterals and there is litt...

We present a case of pulmonary vein (PV) stenosis after radio-frequency (RF) ablation, in which a hemodynamic change in the pulmonary artery was similar to that of congenital PV atresia on time-resolved contrast-enhanced magnetic resonance angiography (TR-MRA). A 48-year-old man underwent RF ablation due to atrial fibrillation. The patient subsequently complained of hemoptysis, dyspnea on exert...

BACKGROUND Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients. METHODS AND RESULTS Since 19...

Two patients with isolated atresia of the pulmonary veins are described. One patient presented with haemoptysis and the other with recurrent unilateral chest infections. The diagnosis was confirmed in both instances by cardiac catheterisation and pulmonary angiography. One patient died one year after the diagnosis was made and the second child has been stable during a follow up period of three ...

Three patients with normal hearts and no pulmonary abnormality had neonatal tricuspid regurgitation causing cardiorespiratory distress and cyanosis. The signs of tricuspid regurgitation resolved over a few weeks. In the acute phase echocardiography showed gross dilatation of the right atrium and ventricle. The interatrial septum bulged into the left atrium during the whole cardiac cycle. Dopple...

We present two cases of individual pulmonary vein atresia without vestige of an involved pulmonary vein. On CT, we noted the absence or interruption of normal pulmonary venous structures, and the presence of abnormal vascular structures that represented collaterals for the involved lung parenchyma. On angiography, the atretic pulmonary vein was found to drain into the other ipsilateral pulmonar...

There is still disagreement concerning the precise nature of the anterior ventricular chamber in "tricuspid atresia". Some argue that it is a right ventricle, while our own previous studies have suggested it is comparable to the outlet chamber seen in classical "single ventricle". We have compared the morphology of the anterior ventricular chambers in 48 examples of tricuspid atresia (absent ri...

The structural effect of prostaglandin E1 on the pulmonary circulation in pulmonary atresia has been studied by applying quantitative morphometric techniques to the injected and inflated lungs of eight babies who had received prostaglandin E1 for between 30 hours and 12 days. The most striking effect was on the pulmonary arterial smooth muscle. Relative arterial medial thickness was reduced and...