An infant with pulmonary hypertension due to a congenital porto-caval shunt

Abstract

A 20-month-old male child died during an episode of acute bronchitis. The autopsy revealed massive hypertrophy and acute dilatation of the right heart which was caused by pulmonary hypertension exhibiting plexogenic arteriopathy and necrotizing arteriitis of small lung vessels. Further examination revealed complete old fibrotic occlusion of the extrahepatic portal vein and a large porto-systemic shunt. Esophageal varices indicating portal hypertension were not present. Histology showed enlarged hepatic arteries within the portal tracts while branches of the portal vein were lacking. The placenta had been examined at the time of birth and multiple chorangiomas reported. Furthermore, multiple old and florid occlusions of fetal vessels had been detected together with focal lymphoplasmocytic inflammation, which may indicate that intrauterine infection and vascular compromise were the cause of the complete closure of the portal vein around birth. The association of pulmonary hypertension with liver disease is well known, and portal hypertension has been considered a key factor in the pathogenesis of pulmonary hypertension. However, this case illustrates that portal hypertension is not a requisite for the development of pulmonary hypertension. It rather suggests that plexogenic arteriopathy of the lungs can be caused by porto-caval shunting independent of liver damage and portal hypertension. Toxic metabolites of nutrients or residual activity of pancreatic enzymes reaching the pulmonary vascular bed may be involved in the pathogenesis.

Pulmonary Hypertension in Patients With Congenital Portosystemic Venous Shunt: A Previously Unrecognized Association

“…To the best of our knowledge, only a few patients with CPSVS have so far been reported to be associated with PAH, 3,16-21 and 4 of them developed PAH during childhood. 3,17,19,21 We herein describe a follow-up evaluation of 9 patients with CPSVS. We determined that PAH is 1 of the major complications of CPSVS.…”

Role of per-rectal portal scintigraphy in long-term follow-up of congenital portosystemic shunt

“…An intrahepatic CPSS is tightly surrounded by liver parenchyma and may be under pressure during growth, perhaps leading to spontaneous closure. One case report of a child with intrahepatic CPSS who died of pulmonary hypertension described an enlarged portal tract with multiple thin-walled angiomatous vessels; these may be a feature of shunts that tend to close or regress under pressure (5). By contrast, an extrahepatic CPSS is under less pressure from the surrounding tissues and thus may retain its size, or enlarge, as the patient grows.…”

“…c ongenital portosystemic shunt (CPSS), which is a major cause of neonatal hypergalactosemia without galactosemetabolizing-enzyme deficiency (1), causes brain manganese deposition, pulmonary hypertension, and hyperammonemia leading to hepatic encephalopathy (2)(3)(4)(5)(6)(7)(8). CPSS is generally suspected if serum total bile acid (TBA) and ammonia levels are elevated, and it is diagnosed by using color Doppler ultrasonography, dynamic contrast-enhanced computed tomography (CT), and per-rectal portal scintigraphy (PRPS) (7,(9)(10)(11)(12).…”

Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review

“…Although recognition of the shunt can be incidental in a patient free of symptoms, most CPSSs are diagnosed when secondary complications become clinically evident: hypoxia owing to hepatopulmonary syndrome, dyspnea caused by pulmonary arterial hypertension, encephalopathy, neonatal cholestasis, or liver nodules [6]. Focal nodular hyperplasia, adenoma, hyperplastic nodules, diffuse nodular regenerative hyperplasia, hepatocellular carcinoma, and hepatic sarcoma have been described in association with CPSS [7].…”