This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Background: Portopulmonary hypertension (PPHTN) and hepatopulmonary syndrome (HPS) are distinct, rare, and potentially fatal complications of portal hypertension. PPHTN is a syndrome of abnormal pulmonary vasoconstriction leading to pulmonary arterial hypertension. HPS is a state of abnormal pulmonary vasodilatation resulting in intrapulmonary vascular shunting and abnormal gas exchange. The pathogenesis of these conditions is unknown but they may be caused by elevated circulating levels vasoactive molecules. Without early diagnosis and treatment, complications of hypoxia and heart failure can progress with subtle or no symptoms. Both conditions are associated with high rates of morbidity and mortality. The reported prevalence of PPHTN and HPS among adults with cirrhosis or end stage liver disease referred for liver transplantation is 2%-20% and 4%-19% respectively. The prevalence in children is unknown and patients are not routinely screened for these conditions.
Aim : To determine the prevalence of PPHTN and HPS in children with portal hypertension. To determine what clinical signs and symptoms can be used as predictors of PPHTN or HPS. Methods: A cross-sectional study of patients followed at The Children's Hospital Pediatric Liver Center will be conducted. Fifty patients, less than 25 years old, with known portal hypertension will undergo an echocardiogram, abdominal ultrasound, chest x-ray, electrocardiogram and pulse oximetry to screen for PPHTN and HPS. Analysis of Data: The prevalence of PPHTN and HPS will be determined in this population and correlated with clinical signs and symptoms to identify early and unrecognized predictors of these syndromes.
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