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A6752 - Pentoxifylline Reduces Intrapulmonary Shunt in a Patient with Hepatopulmonary Syndrome
Author Block: N. Alipanah1, N. Juul2, V. Mohindra3, A. A. Gohil3; 1Internal Medicine, Santa Clara Valley Medical Center, San Jose, CA, United States, 2Pulmonary and Critical Care Medicine, Stanford University, Palo Alto, CA, United States, 3Pulmonary and Critical Care Medicine, Santa Clara Valley Medical Center, San Jose, CA, United States.
Introduction:The only definitive treatment for hepatopulmonary syndrome (HPS) is liver transplantation. Pentoxifylline reduces TNF-alpha in rat models of cirrhosis. A few case reports on patients with HPS have demonstrated similar findings and improvement in PaO2 with pentoxifylline over a three-month period. Here, we report a case of HPS treated with pentoxifylline with near complete resolution of intrapulmonary shunt over nine months. Description of case: A 56-year-old woman with history of asthma and alcoholic cirrhosis presented to the hospital with progressively worsening exertional dyspnea of 2 years not relieved with inhalers. Exam was notable for oxygen saturation (SaO2) 78% on room air requiring up to 8 L supplemental O2 via nasal cannula (NC) to maintain SaO2 at 90%, clubbing, and absence of wheezing. Pulmonary function testing showed a severely reduced DLCO (39%) in the absence of significant restriction or obstruction. Transthoracic echocardiogram (TTE) with agitated saline contrast study revealed an intrapulmonary shunt. High resolution CT scan found no evidence of an interstitial lung disease. She subsequently underwent quantitative Technetium 99m (99m Tc)-labeled macroaggregated albumin (MAA) scanning which showed 56% intrapulmonary shunt. Based on her high shunt percentage, she was deemed not to be a transplant candidate. She was subsequently discharged home on 6L NC at rest and 10L facemask with activity to maintain SaO2 > 88%. At follow up in clinic, she was started on thrice daily pentoxifylline (400 mg). Three months later, her resting SaO2 had improved to 93% on 4 L NC. At six months follow up, she was maintaining SaO2 at 97% with ambulation on 5 L NC. At 9 months follow up, she was able to maintain SaO2 at 100% on 2 L NC. Repeat transthoracic echocardiogram with contrast showed significant reduction of intrapulmonary shunt. Repeat quantitative MAA scanning showed remarkable decline in right to left shunt percentage to 8.93%. Discussion: We report the first case of prolonged use of pentoxifylline associated with near resolution of intrapulmonary shunt on TTE and MAA in a patient with severe HPS. Our patient tolerated pentoxifylline without any adverse effects and remains on this treatment. More studies are needed to elucidate the ideal dose and duration of treatment necessary to maintain shunt reduction. Pentoxifylline may be ideal as a bridge to lung transplantation or as chronic treatment for those not undergoing transplantation.