Primary pulmonary hypertension presenting as portal hypertension
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This case study details a child with portal hypertension originating from primary pulmonary hypertension. The unique presentation lacked prior cardiopulmonary symptoms and underlying liver disease.
Area of Science:
- Pediatric Medicine
- Cardiology
- Gastroenterology
Background:
- Portal hypertension presents with symptoms like hematemesis, hepatosplenomegaly, and ascites.
- Pulmonary hypertension can be a severe condition affecting the heart and lungs.
Observation:
- A two-and-a-half-year-old child exhibited signs of portal hypertension.
- The source of the elevated pressure was identified within the pulmonary arterial system.
Findings:
- Autopsy revealed severe primary pulmonary hypertension as the cause.
- This occurred without preceding cardiopulmonary symptoms or known risk factors like liver disease or portal vein thrombosis.
Implications:
- Highlights an unusual etiology of portal hypertension in pediatric cases.
- Suggests pulmonary vascular disease should be considered in unexplained portal hypertension.
Abstract:
A two and a half-year old child is described who presented with signs of portal hypertension (hematemesis, hepatosplenomegaly, ascites). Her subsequent work-up revealed that the "pressure-head" originated within the pulmonary arterial bed. Indeed, severe changes of primary pulmonary hypertension were found at autopsy. What is unique about this case is the absence of cardiopulmonary symptoms prior to the development of suprahepatic portal hypertension. In addition, the pulmonary disease developed in the absence of underlying chronic hepatic disease or extrahepatic portal vein thrombosis which, reportedly, can lead to pulmonary hypertension.