Background: Porto pulmonary hypertension (PoPH) is combination of portal hypertension and pre capillary pulmonary arterial hypertension (PAH). Prognosis is poor in pediatric patients. To evaluate incidence, clinical profile, investigational profile and outcome of pediatric PoPH, the following study was done.

Method: The children (age 3 – 12 years) who had portal hypertension and respiratory distress were admitted in pediatric gastro enterology ward of PGIMER – Chandigarh from July 1993 to June 2003. History and clinical examination (Ascites, GI Bleed, Jaundice, dyspnea, fatigue, chest pain, syncope etc.), investigations (USG whole abdomen, LFT, UGI Endoscopy, pulse oxymetry, echocardiography, contrast echocardiography, chest X-ray etc.) were noted.

Simplified Bernoulli equation is used to detect PAP by echocardiography. Contrast echocardiography is done to rule out hepato pulmonary syndrome.

Results: Out of 10 cases of portal hypertension, 6 were male, 4 were female. Age was 3 – 12 years (mean 8 year). Out of 10 cases, 6 were cirrhosis, 4 were non cirrhotic portal hypertension. Invasive procedure like cardiac catheterization was not done. PAP (measured by echocardiography) was 40 – 55 mm Hg. 4 patients who had non cirrhotic portal hypertension improved after portal systemic shunt and reduction of portal hypertension. 6 patients who had cirrhosis and pulmonary hypertension could not survive. Symptomatic treatment of pulmonary hypertension was done. (IV prostaglandins etc.)

Conclusion: Porto pulmonary hypertension is a rare but serious problem in children. Research is still going on to get best management.

Keyword: Porto Pulmonary Syndrome in Children.

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Sujay Chaudhuri

Division of Pediatric Gastroenterology, Department of Gastroenterology, PGIMER Chandigarh