Extrahepatic portal vein obstruction (EHPVO) is present in 20-40% of patients with portal hypertension in India(1,2). EHPVO may be (i) secondary to oomphalitis, umbilical vein catheterization, intraabdominal sepsis and trauma or (ii) idiopathic. Congenital cardiac anomalies have been reported more with EHPVO of idiopathic group than in nonidiopathic group(3). Congenital anomalies reported are atrial septal defect, ventricular septal defect, mitral valve prolapse, tetralogy of Fallot etc. In our recent analysis of 157 children with EHPVO we had 3 cases with cardiac anomalies. One of which we have already reported(4). We report here 2 more cases.

Case 1: A 6-year-old boy presented with 3 episodes of melena without hematemesis. There was no significant birth or family history. On examination patient was pale, had a wide fixed split second heart sound in left 2nd intercostal space and a 4 cm palpable spleen. Laboratory investigations revealed hemoglobin of 8.5 g/ dl with normal platelet and leukocyte count. Liver function tests were normal. Ultrasonography showed a portal cavernoma with patent splenic vein. Liver echotexture was normal. Upper gastrointestinal scopy showed 2 grade III esophageal varices. Echocardiography showed ostium secundum type of atrial septal defect. Endoscopic sclerotheray was done with 1 % polidocanol. Patient required 4 sessions of sclerotherapy to eradicate va- rices. Patient was well at 9 months of followup.

Case 2: A 10-month-old male child, a case of tetralogy of Fallot, presented with two episodes of hematemesis and melena. There was no significant birth or family history. On examination, child was malnourished, pale and acyanotic with both liver and spleen being 1 cm palpable. Laboratory investigations revealed hemoglobin of 4 g/ dl, normal leukocyte and platelet count. Liver function tests were normal. Abdominal sonography revealed portal cavernoma with patent splenic vein and mild splenomegaly. Liver was normal in size and echotexture. Echocardiography re- vealed a large sub aortic ventricular septal defect with 50% of aortic override with predominantly valvular and mild infundibular pulmonary stenosis. Upper gastrointestinal scopy revealed 4 grade IV esophageal varices which were thrombosed using cyanoacrylate glue in two sessions. We used cyanoacrylate glue as thrombosis is achieved in fewer sessions.

The most common abnormalilty in EHPVO consists of a thrombus surrounded by a cavernous leash of collateral vessels.  Portal vein thrombosis secondary to umbilical vein sepsis or oomphalitis is the widely held hypothesis for the development of portal vein occlusion. However in a majority of EHPVO patients, the cause is unknown.

Various congenital cardiac anomalies are commonly described with EHPVO(2,3). We have earlier published a case report of EHPVO with atrial septal defect(4). We found congenital cardiac anomalies in 3 of 157 children (1.9%) with EHPVO. Anomalies that were present included atrial septal defect (secundum type) in two patients and Fallot's tetralogy in one. Various other studies also demonstrated this association. Dilawari et al. found congenital anomalies like mitral valve prolapse and ventricular septal defect in 4 (2%) out of 213 patients with EHPVO(2).

An awareness of the possibility of associated cardiac anomalies with EHPVO has practical implications. Cardiac anomalies might contraindicate any major surgical procedure to be performed for the control of variceal hemorrhage. Endoscopic sclerotherapy is the first line of treatment in the management of variceal hemorrhage reserving shunt surgeries for those in whom endoscopic treatment has failed or is unacceptable. Also in the event of variceal hemorrhage, associated cardiac anomalies may increase morbidity and mortality.

Anirudha M. Gopanpallikar, Pravin M. Rathi,
Prabha Sawant,
Rajiv Gupta,
Sheetal D. Dhadphale,
Viral N. Patrawala,
Department of Gastroenterology, L.T.M. Medical College and
L.T.M. General Hospital,
Sion, Mumbai 400 022, Itidia.