Pulmonary veno-occlusive disease (PVOD), a rare form of pulmonary arterial hypertension (PAH) characterized by a progressive obstruction of small pulmonary veins, is both difficult to diagnose and treat effectively. We reported a case of limited cutaneous systemic sclerosis complicated with PAH in which early definitive diagnosis of PVOD was made by surgical lung biopsy. A 53-year-old Japanese ...

Lone (or primary), pulmonary hypertension (P.H.T.) may be defined as a condition of high pressure in the pulmonary artery associated with right ventricular hypertrophy for which no cause can be found outside the pulmonary vessels. The diagnosis is one which is made by exclusion of other diseases which are known to be potential or predisposing causes of P.H.T. The most important of these are chr...

OBJECTIVES This study reports our clinical experience with transcatheter closure of secundum atrial septal defects (ASDs) in children, using the Amplatzer, a new occlusion device. BACKGROUND None of the devices previously used for transcatheter closure of interatrial communications has gained wide acceptance. METHODS We examined the efficacy and safety of the Amplatzer, a new self-centering...

A 22 year-old male patient was admitted with supracardiac, nonobstructive, total anomalous pulmonary venous connection and large atrial septal defect. Cardiac catheterization revealed severe pulmonary hypertension due to pulmonary overflow (Qp/Qs 6, pulmonary vascular resistance = 1.6 Woods/m(2)). Complete repair was performed through median sternotomy and cardiopulmonary bypass. The patient wa...

Gamillscheg et al. report on a single patient who had a large pulmonary arteriovenous malformation with significant right-to-left shunting and a cerebrovascular accident secondary to presumed paradoxic embolism. The accompanying still-frame cineangiogram demonstrates a large arteriovenous malformation with a 10-mm feeding artery and no obvious narrowing, making it difficult to provide endovascu...

Holt-Oram syndrome is an autosomal dominant disorder, characterised by skeletal abnormalities of the upper limb associated with congenital heart defect, mainly atrial and ventricular septal defects. Skeletal defects exclusively affect the upper limbs in the preaxial radial ray distribution and are bilateral and asymmetrical. They range from clinodactyly, absent or digitalised thumb, hypoplastic...

Anomalies of the pulmonary venous return are uncommon, particularly malformations with total drainage of the pulmonary veins directly into the right atrium. Brody (1942) reviewed the literature on anomalous pulmonary venous drainage, dealing with 106 cases, including four personal ones. Further necropsy reports have since been presented by Vass and Mack (1949) and Smith (1951), the latter also ...

BACKGROUND The complexity of ventricular septal defects in early infancy led to development of new mini-invasive techniques based on collaboration of cardiac surgeons with interventional cardiologists, called hybrid procedures. Hybrid therapies aim to combine the advantages of surgical and interventional techniques in an effort to reduce the invasiveness. The aim of this study was to present ou...