Preduodenal Portal Vein Associated with Complex Congenital Heart Disease and Heterotaxy Syndrome: An Unexpected Cause of Common Complaint

Abstract

We read with great interest the article on preduodenal portal vein (PDPV) by Weber et al. that was published in The American Surgeon.1 We have also encountered two cases of children with PDPV who had complex congenital heart disease (CHD) associated with heterotaxy syndrome (HS). Both children presented common symptoms related to PDPV, such as fatigue, chest or abdominal discomfort, and headache. To identify PDPV as the cause of the common symptoms was challenging because they were confounded with those related to their CHD.2 Case 1: A 12-year-old boy who had been diagnosed with complete atrioventricular septal defect, malposed great arteries, pulmonary atresia, bilateral superior caval veins, and right single coronary artery complained of general fatigue, headache, nausea, and postprandial abdominal discomfort. The patient was born at 41 weeks’ gestational age, with a birth weight of 2868 g. He underwent placement of a modified Blalock-Taussig shunt at four months of age followed by bilateral bidirectional cavopulmonary shunt at seven months of age. The patient underwent an extra cardiac total cavopulmonary connection at two years of age. Repeated physical examinations, X-rays, and ultrasonography failed to reveal the causes of his symptoms. Because we suspected that the symptoms might be due to failing Fontan circulation,2 we performed cardiac catheterization at 10 years of age. Central venous pressure, cardiac index, and pulmonary arterial resistance were 13 mmHg, 3.1 L/min/m, 0.32 Wood units · m, respectively. Bosentan, a pulmonary vasodilator, was administrated but was ineffective. Further investigations, including abdominal ultrasonography and contrast-enhanced CT, were performed at 11 years of age. The examinations showed that the duodenum was obstructed owing to PDPV (Fig. 1 A). A gastrojejunostomy was performed at 12 years of age. The laparotomy revealed the absence of a spleen, a left-sided liver with cirrhosis, and a midline gallbladder. The portal vein was observed running anteriorly across the duodenum, compressing it (Fig. 1 B). Intestinal malrotation was also identified. A gastrojejunostomy reconstructed with Roux-en Y anastomosis was performed in a retrocolic and peristaltic manner. The patient had an uneventful postoperative course, and the common complaints disappeared completely.