Ductus arteriosus: more than just the patent ductus arteriosus.

The ductus arteriosus is important to fetal circulation. Failure to close at birth is a common event. In this educational pictorial essay, we illustrate the association of the ductus arteriosus with a variety of congenital cardiac, vascular and pulmonary lesions. These lesions can impact the systemic circulation, the pulmonary circulation or the airway and include coarctation of the aorta, ductal origin of the pulmonary artery and vascular rings.

First antenatally confirmed case of arterial tortuosity syndrome.

Arterial tortuosity syndrome (ATS) is a rare, autosomal recessive, connective tissue disorder. It predominantly involves the arterial tree with clinical features reflecting the systems involved. There have been few cases of ATS suspected during antenatal screening ultrasound in high-risk families, but none confirmed. We present the first case of ATS confirmed antenatally in the fetus with cascade testing, detecting the disease in the mother as well.

A case of horseshoe lung and complex congenital heart disease in a term newborn.

We describe and illustrate a rare association of hypoplastic left heart syndrome, absent hilar left pulmonary artery, and an unusual bronchopulmonary malformation. This case highlights the utility of combination imaging of echocardiography and CT angiography in diagnosing a cyanotic newborn with a chest radiograph that is suspicious for lung hypoplasia.

Clipped tube fenestration after extracardiac Fontan allows for simple transcatheter coil occlusion.

BACKGROUND: Expensive devices are increasingly used to close a patent fenestration after a modified Fontan operation. We report our 5-year institutional experience of clipped tube fenestration after extracardiac Fontan operation, which allows for simple transcatheter coil occlusion. METHODS: We retrospectively reviewed 30 children, median age of 4.0 years (range, 2.4 to 8.8 years) who underwent extracardiac Fontan operation between May 1996 and May 2001, and were fenestrated using a 4- to 8-mm diameter clipped tube graft. RESULTS: Ten children had a patent fenestration occluded by transcatheter placement of 15 detachable coils (5- to 8-mm diameter). Aortic oxygen saturations increased on average by 5.5% (2% to 14%) and mean pressures in the Fontan circuit by 2.5 mm Hg (0 to 3 mm Hg). Four had immediate complete occlusion angiographically and 6 had trivial residual shunt, but complete occlusion by echocardiography at follow-up. There have been no immediate complications, late coil embolizations, thromboembolic events, or documented hemolysis within a follow-up after coil implantation of 1.7 years (0.4 to 4.5 years). Spontaneous fenestration closure was documented in 8 patients at cardiac catheterization and 9 patients by echocardiography with consistent improvement in resting transcutaneous oxygen saturation. Two children with a patent fenestration have been considered inappropriate for closure, and there was one early surgical death. There have been no complications related to the tube fenestration modification within a follow-up postoperation of 2.6 years (0.1 to 5.5 years). CONCLUSIONS: Clipped tube fenestration after extracardiac Fontan operation is a useful surgical modification that allows for simple transcatheter coil occlusion.