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Spontaneous Resolution of Hepatopulmonary Syndrome With the Subsequent Development of Portopulmonary Hypertension FREE TO VIEW

Radhika Zopey, MD; Tisha Wang, MD
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UCLA Medical Center, Los Angeles, CA

Chest. 2013;144(4_MeetingAbstracts):960A. doi:10.1378/chest.1698999
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are the two major pulmonary vascular complications of liver disease. While HPS is characterized by low pulmonary vascular resistance, PPHTN is defined by the presence of elevated pulmonary vascular resistance. Given these seemingly opposing pathophysiologic mechanisms, these conditions were traditionally felt to be mutually exclusive. In this case report, we present a patient with severe HPS who had spontaneous resolution of her severe HPS with the subsequent development of significant PPHTN.

CASE PRESENTATION: A 57 year-old female with a history of cirrhosis secondary to non-alcoholic steatohepatitis presented with dyspnea and progressive hypoxemia. An extensive work-up showed evidence of an intra-pulmonary shunt on contrast-enhanced echocardiography without other evidence of pulmonary disease to explain her profound hypoxemia. The patient was thus diagnosed with HPS and listed for liver transplant. Over the next three years, however, her dyspnea and hypoxia gradually improved spontaneously. Arterial blood gases showed improvement in the partial pressure of oxygen from an original value of 56 millimeters of mercury (mm Hg) to 83 mm Hg. A routine echocardiogram, however, was suggestive of pulmonary hypertension. She therefore underwent right heart catheterization, which showed a markedly elevated mean pulmonary artery pressure of 45 mmHg, consistent with PPHTN. The patient was subsequently taken off the liver transplant list until successful treatment of her PPHTN. Two additional similar cases have been seen in our institution.

DISCUSSION: Though HPS and PPHTN have distinct pathophysiologies, they can rarely occur in the same patient, either concurrently or in our case, with the sequential development of HPS followed by PPHTN. The dysregulation of the same vascular signaling pathway may lead to both pulmonary vascular dilatation and pulmonary arterial remodeling in the same patient. Though the overall mechanism remains unclear, one theory involves the possible differential binding of endothelin-1, a vasoactive signaling peptide, that induces vasoconstriction when bound to receptor A and vasodilation when bound to receptor B.

CONCLUSIONS: HPS and PPHTN can rarely occur in the same patient, either concurrently or sequentially. Although the mechanism for this remains unclear, it is important to be vigilant of this phenomenon as it may change the patient’s overall treatment plan, especially with regards to appropriateness and timing of liver transplant, which has been shown to resolve both pulmonary vascular conditions.

Reference #1: Singh C. Pulmonary Complications of Cirrhosis. Med Clin North Am, 93 (2009), 871-83.

Reference #2: Pham DM. Coexisting Hepatopulmonary Syndrome and Portopulmonary Hypertension. J Clin Gastroenterol, 44 (2010), e136-e140.

DISCLOSURE: The following authors have nothing to disclose: Radhika Zopey, Tisha Wang

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