Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: pulmonary hypertension and pulmonary vascular dilatation

Abstract

HPS and PPHTN are unusual and challenging pulmonary manifestations of liver disease. We report two pediatric cases in association with heterotaxy polysplenia syndrome and congenital absence of the portal vein. Both patients were symptomatic and hemodynamically compromised and required aggressive medical therapy. One patient with PPHTN alone achieved a successful liver transplant. The second child presented with combined HPS and PPHTN and exhibited a different evolution of pulmonary vascular disease. These cases illustrate associations that must be entertained in the setting of heterotaxy syndrome, cyanosis, or pulmonary hypertension and how strategic medical combined with surgical management can provide a good outcome.

Fig. 1

Selective right pulmonary arteriogram in case 1 shows diffuse dilated intraparenchymal pulmonary vessels with rapid return of contrast to left atrium, in less than two beats. The left pulmonary arteriogram had identical findings.

Fig. 2

Selective injection in the hepatic artery (upper) in case 1 with venous return demonstrating the large collateral draining to the azygous vein below the diaphragm (lower). The portal vein is not seen.

Fig. 3

Venous phase in Case 1 from injection into the hepatic artery with simultaneous balloon occlusion of the venous collateral. A portal venous stump was not seen on the liver side.