Arteriohepatic dysplasia: Familial pulmonary arterial stenosis with neonatal liver disease

The clinical features and course of five children with the recently described syndrome of arteriohepatic dysplasia are presented. All had bilateral pulmonary arterial stenosis, proven at cardiac catheterization, as well as associated liver disease of varying severity. In one of the fatal cases, a hitherto undescribed anomaly was found—stenosis of the right coronary artery ostium. A viral etiology, eg, the congenital rubella syndrome, was considered most likely, but detailed investigations proved to be negative.

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Letters to the Editor

PEDIATRICS ◽

10.1542/peds.68.3.464a ◽

1981 ◽

Vol 68 (3) ◽

pp. 464-465

Author(s):

Solomon E. Levin

Keyword(s):

Cardiac Catheterization ◽

Clinical Evidence ◽

Pulmonary Stenosis ◽

Arterial Stenosis ◽

Letters To The Editor ◽

Genetic Origin ◽

Arteriohepatic Dysplasia ◽

Pulmonary Arterial ◽

Pulmonary Arterial Stenosis ◽

Peripheral Pulmonary Stenosis

The comments of Drs Riely, Rosenfield, and Cotlier are interesting, especially in relation to the abnormal eye findings and additional skeletal anomalies they have reported in cases of arteriohepatic dysplasia.1 Their contention that the syndrome is likely to be of genetic origin receives support from my own observations on two families seen subsequent to submission of our article for publication.2 In one family there were two siblings, a boy aged 5 years 3 months with significant pulmonary arterial stenosis documented at cardiac catheterization, and his younger sister, with clinical evidence of mild peripheral pulmonary stenosis.

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Experimental studies on the implantation of intravascular stents in puppies—potential use for aortic coarctation and pulmonary arterial stenosis

Cardiology in the Young ◽

10.1017/s1047951100002353 ◽

1994 ◽

Vol 4 (4) ◽

pp. 366-372 ◽

Cited By ~ 2

Author(s):

Toshihiro Ino ◽

Shinjiro Shimazaki ◽

Keijiro Yabuta ◽

Ryozo Okada

Keyword(s):

Aortic Coarctation ◽

Experimental Studies ◽

Pulmonary Arteries ◽

Arterial Stenosis ◽

Inferior Caval Vein ◽

Major Disadvantage ◽

Intravascular Stents ◽

Pulmonary Arterial ◽

Pulmonary Arterial Stenosis

AbstractWe have investigated angiographically and histologically the vascular response to implantation of intravascular stents in normal puppies. Eight balloon-expandable stents were placed in six mongrel puppies (weight 6.4–10.5 kg) under anesthesia. Four stents were successfully placed in the pulmonary arteries and three in the aorta. In one case, the strut of the stent was hooked by a leaflet of the tricuspid valve at the time of placement and eventually had to be implanted in the inferior caval vein. Two puppies died, one from bleeding from the femoral artery and the other from thrombotic obstruction at the superior mesenteric artery, both after successful implantation. The former was due to repeat arterial puncture and the latter wasdue to failure to use aspirin. Angiographic studies showed satisfactory opacities of the branch arteries in all but one puppy. The diameter of the arteries prior to implantation was 7.5±1.5 mm. The diameters at the middle of the stents immediately after implantation and during the period of follow-up were 6.9±1.7mm and 6.9±2.1 mm, respectively. Histologic studies revealed that neogenic endothelial layers of 30 to 40 pm in thickness had formed over the struts of the stent without formation of thrombusthree weeks after implantation. Scanning electron microscopy revealed that the endothelial cells of the neoendothelial layer had almost identical characteristics to those of normal arteries. These results indicate that clinical application of this stent is feasible but that use of anticoagulants is mandatory during and after implantation in the treatment of selected patients with aortic coarctation or pulmonary arterial stenosis. Relative progression of stenosis may occur, however, and the inability for re-expansion is a major disadvantage of this stent.

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