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Condino, A. A.1; O’Connor, J. A.1; Narkewicz, M. R.1; Claussen, L.2; Doran, A.2; Ivy, D. D.2; Sokol, R. J.1
1Pediatrics, 2Cardiology, Univ of Colorado and The Children’s Hospital, Denver, United States
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Introduction: Pulmonary hypertension (PHTN), a rare complication of portal hypertension (PH),is known as portopulmonary hypertension (PPHTN). PPHTN is defined by an elevated pulmonary artery (PA) pressure, increased pulmonary vascular resistance, and a normal pulmonary wedge pressure with underlying portal hypertension. The aim of this study was to investigate the clinical presentation and manifestation of PPHTN in a group of pediatric patients.
Methods: A chart review identified 6 patients with PPHTN. The clinical presentation and evaluation for both PH and PHTN were reviewed.
Results: The causes of PH in our 6 patients included primary sclerosing cholangitis (PSC), cryptogenic cirrhosis (CC), portal vein thrombosis (PVT) in 2, and biliary atresia (BA) in 2 (Table). The median time from the diagnosis of PH to PHTN was 12.1 years. One patient presented with PHTN prior to PH. Five patients presented with a new heart murmur or prominent P2. Three had syncope pre and 1 had syncope post-diagnosis. Three patients had normal EKG’s and two normal chest x-rays. The mean baseline right ventricular systemic pressure (RVSP) by echocardiogram (ECHO) was 72.1 mmHg. Three patients had cardiac catheterizations with an average mean PA pressure (MPAP) of 69 mmHg. PPHTN was refractory to standard and experimental medical management. Two patients died (pt.3-at 20.3 yrs, pt.4-at 13.8yrs)and postmortem lung tissue showed plexiform lesions and pulmonary arteriopathy.
Conclusion: PPHTN was diagosed 12 years after recognition of PH, compared to 4–7 years in adult series. Symptoms of PPHTN may be subtle and overlooked. Pediatric patients with PH who present with a new heart murmur, prominent P2, dyspnea, or syncope, require evaluation. From our experience, EKG and chest x-ray are insensitive screens for PPHTN. ECHO and cardiology referral are essential for this diagnosis.
© 2004 Lippincott Williams & Wilkins, Inc.
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