H epatopulmonary syndrome (HPS) is a wellknown consequence of chronic liver disease. 1 HPS is defined as a clinical triad of chronic liver disease, increased alveolar-arterial gradient while breathing room air, and evidence of intrapulmonary vascular dilations. 1 Most patients present with symptoms related to their liver disease (e.g., ascites or gastrointestinal bleeding), although some patients present with respiratory symptoms (e.g., dyspnea). 2 Typically, HPS occurs in patients with chronic liver disease resulting in cirrhosis, but it also has occurred in patients with noncirrhotic portal hypertension (HTN). 2,3 Orthotopic liver transplantation (OLT) has become a therapeutic option for patients with HPS, and patients often realize a regression of intrapulmonary shunts after OLT. 4 Recurrent HPS after OLT recently was reported in a patient who underwent OLT for severe HPS associated with nonalcoholic steatohepatitis. 5 HPS resolved after OLT, but the patient again experienced cirrhosis and subsequently developed HPS. We report a patient who developed severe HPS several years post-OLT, but unlike the typical presentation of HPS, our patient did not have cirrhosis or evidence of portal HTN.
Case ReportA 9-year-old boy was referred for evaluation of progressive hypoxia. He was born with biliary atresia and underwent a Kasai procedure without success. He underwent OLT at the age of 7 months, which was complicated early by severe pneumonia caused by Pneumocystis carinii requiring mechanical ventilatory support, and later (4 years), by Epstein-Barr virusassociated lymphoproliferative disorder. This was treated with antiviral medications and a reduction in immunosuppression. One year later, a diagnosis of Hodgkin's lymphoma was made. His treatment consisted of splenectomy, radiotherapy to mediastinal lymph nodes (with lung shielding), and chemotherapy (courses of mechlorethamine, vincristine, procarbazine, and prednisone alternating with doxorubicin, bleomycin, vinblastine, and dacarbazine for 8 months).He was evaluated for respiratory symptoms at the age of 7 years. He had a nonproductive cough that worsened with exercise. Spirometry results were interpreted as normal, and room air arterial PO 2 was 63 mm Hg (alveolar-arterial gradient, 50 mm Hg). The favored diagnoses at that time included radiation and bleomycin pneumonitis, and the patient was treated with oral steroid therapy. Exertional dyspnea and arterial oxygen desaturation persisted, but remained stable.He was referred at 9 years for evaluation of malaise and progressive dyspnea with marked exercise intolerance. Room air arterial PO 2 was 55 mm Hg (alveolar-arterial gradient, 57.5 mm Hg), and he showed orthodeoxia. Arterial oxygen on a fraction of inspired oxygen of 1.0 was 428 mm Hg. Spirometry showed a forced vital capacity of 1.04 L (59% predicted), but with an expiratory time of only 0.5 seconds; previous test results had been normal, and this test also was interpreted as likely to be normal.Chest radiography showed a mild increase in perihilar interst...
