Transition From Pulmonary to Gastrointestinal Hemorrhage: A Rare Case of Hematemesis in an Adolescent With Chronic Pulmonary Hypertension and Valvular Regurgitation.

Pulmonary hypertension is a chronic cardiopulmonary disease with increased pulmonary arterial pressure and right-sided heart overload, and early symptoms often include dyspnea with hemoptysis. Hematemesis, as the presentation of gastrointestinal bleeding, is a remarkably unusual, underappreciated form of the disease, especially in children, along with valvular heart disease. We present a 15-year-old boy with preexisting chronic pulmonary hypertension and a history of pulmonary/aortic valvular regurgitation, who reported copious hematemesis after episodes of hemoptysis and epistaxis. At presentation, he was hemodynamically compromised with anemia, hypoxia and features of right-sided heart strain. Initial work-up was consistent with an upper gastrointestinal bleed, but further evaluation revealed severe pulmonary hypertension with right ventricular pressure overload and persistent pulmonary and aortic valvular regurgitation on Doppler echocardiography, confirmed radiographically with cardiomegaly and pulmonary vascular congestion. A cardiopulmonary source of bleeding was considered based on the clinical presentation and cardiopulmonary findings, although a gastrointestinal source could not be definitively excluded. The diagnosis was made based on clinical evidence in addition to imaging and echocardiography, consistent with pediatric pulmonary hypertension criteria for the diagnosis. Conservative measures were adopted for the patient, including hemodynamic stabilization, blood transfusion, proton pump inhibitor (PPI) therapy and antifibrinolytics, along with supportive management leading to cessation of hemorrhage and clinical stabilization. This case highlights the need to consider a cardiopulmonary source of bleeding in patients with pulmonary hypertension and hematemesis. Early identification of this unusual presentation is important to prevent misdiagnosis, inappropriate invasive gastrointestinal procedures and timely management with cardiopulmonary resuscitation.