Abstract
The patient, an 11-year-old boy, visited our hospital with chief complaints of hematemesis and melena. Endoscopy revealed R-C sign-positive esophageal varices, and angiography revealed pylemphraxis in front of the liver and spontaneous splenorenal shunt, pediatric portal hypertension being diagnosed. Moreover, X-ray of the chest revealed a projection of the left second arch and ECG revealed hypertrophy of the right ventricle. Conclusive diagnosis of the patient as having pulmonary hypertension was achieved by right heart catheterization. Transection of the esophagus through the chest and secondary resection of the gastric varices and the spleen were performed.
Twelve cases of pediatric portal hypertension complicating pulmonary hypertension have been reported including this case Eight of the 12 cases had a past history of undergoing a shunt procedure while two cases had spontaneous port-systemic shunt, suggesting that the latter is related to the cause of pulmonary hypertension. The direct activity of a specific substance in the portal blood carried to the lung through the shunt, rather than through the liver, would cause pulmonary hypertension.
