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The Liver's Caprice: Porto-Pulmonary Hypertension and Hepato-Pulmonary Syndrome Together
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A6744 - The Liver's Caprice: Porto-Pulmonary Hypertension and Hepato-Pulmonary Syndrome Together
Author Block: P. J. Thacker1, A. Abichandani2, H. Grover1, A. Singh2; 1Abington Jefferson Health, Abington, PA, United States, 2St. Luke's University Health Network, Bethlehem, PA, United States.
Introduction:
Porto-pulmonary hypertension (POPH) and hepato-pulmonary syndrome (HPS) are both diseases that arise from liver cirrhosis. The reported prevalence of POPH in cirrhotics ranges from 0.5%- 3.5%, and that of HPS from 4%- 26%. Here we present an extremely rare case of both disease processes occurring in the same patient.
Case:
A 55-year-old Caucasian female presented with shortness of breath. She had a history significant for liver cirrhosis secondary to hepatitis C, and severe porto-pulmonary hypertension (POPH) on riociguat therapy - other causes of pulmonary arterial hypertension (PAH) had been previously ruled out. Her latest right heart catheterization- three months before her presentation- showed a mean pulmonary artery pressure of 45 mmHg, wedge pressure of 12 mmHg and pulmonary vascular resistance of 6.6 WU. On initial evaluation, she was hypoxemic and had significant clubbing. She was volume overloaded and received diuretics till euvolemic. Her hypoxemia persisted, and subsequent pulmonary function tests revealed an isolated DLCO reduction. An echocardiogram with agitated saline revealed neither an intracardiac shunt nor any signs of elevated right-sided pressures. A Tc99m-MAA lung perfusion scan was performed which demonstrated radiotracer activity in the kidneys indicating a right to left shunt. Conversion of POPH to HPS was suspected. Discontinuing the patient’s riociguat improved her oxygenation significantly, and she was discharged with plans for eventual liver transplant.
Discussion:
HPS and PPH are two distinct entities that result from the liver’s inability to clear vasoactive substances. The varying effect of these elements on pulmonary microvasculature results in the manifestation of both diseases. HPS is due to dilatation of pulmonary vasculature and POPH because of vasoconstriction. Our case is unique because the patient carried a confirmed diagnosis of POPH, and when she presented with worsening hypoxemia increasing her POPH therapy was considered, although she had no longer had evidence of PAH or shunt on her echocardiogram. The radionuclide scan revealed a shunt and HPS was diagnosed. Discontinuing POPH therapy confirmed the diagnosis. Our case illustrates the need to remember that both diseases can present with similar symptoms in similar patient populations. Also, while many therapies exist for the treatment of PAH, these are based on inducing pulmonary vasodilatation and may worsen symptoms of HPS as in our patient. Respiratory deterioration in a patient receiving treatment for POPH should alert the clinician to the development of HPS. More research is required to discover the actual prevalence of both diseases coexisting in cirrhotics.
Author Block: P. J. Thacker1, A. Abichandani2, H. Grover1, A. Singh2; 1Abington Jefferson Health, Abington, PA, United States, 2St. Luke's University Health Network, Bethlehem, PA, United States.
Introduction:
Porto-pulmonary hypertension (POPH) and hepato-pulmonary syndrome (HPS) are both diseases that arise from liver cirrhosis. The reported prevalence of POPH in cirrhotics ranges from 0.5%- 3.5%, and that of HPS from 4%- 26%. Here we present an extremely rare case of both disease processes occurring in the same patient.
Case:
A 55-year-old Caucasian female presented with shortness of breath. She had a history significant for liver cirrhosis secondary to hepatitis C, and severe porto-pulmonary hypertension (POPH) on riociguat therapy - other causes of pulmonary arterial hypertension (PAH) had been previously ruled out. Her latest right heart catheterization- three months before her presentation- showed a mean pulmonary artery pressure of 45 mmHg, wedge pressure of 12 mmHg and pulmonary vascular resistance of 6.6 WU. On initial evaluation, she was hypoxemic and had significant clubbing. She was volume overloaded and received diuretics till euvolemic. Her hypoxemia persisted, and subsequent pulmonary function tests revealed an isolated DLCO reduction. An echocardiogram with agitated saline revealed neither an intracardiac shunt nor any signs of elevated right-sided pressures. A Tc99m-MAA lung perfusion scan was performed which demonstrated radiotracer activity in the kidneys indicating a right to left shunt. Conversion of POPH to HPS was suspected. Discontinuing the patient’s riociguat improved her oxygenation significantly, and she was discharged with plans for eventual liver transplant.
Discussion:
HPS and PPH are two distinct entities that result from the liver’s inability to clear vasoactive substances. The varying effect of these elements on pulmonary microvasculature results in the manifestation of both diseases. HPS is due to dilatation of pulmonary vasculature and POPH because of vasoconstriction. Our case is unique because the patient carried a confirmed diagnosis of POPH, and when she presented with worsening hypoxemia increasing her POPH therapy was considered, although she had no longer had evidence of PAH or shunt on her echocardiogram. The radionuclide scan revealed a shunt and HPS was diagnosed. Discontinuing POPH therapy confirmed the diagnosis. Our case illustrates the need to remember that both diseases can present with similar symptoms in similar patient populations. Also, while many therapies exist for the treatment of PAH, these are based on inducing pulmonary vasodilatation and may worsen symptoms of HPS as in our patient. Respiratory deterioration in a patient receiving treatment for POPH should alert the clinician to the development of HPS. More research is required to discover the actual prevalence of both diseases coexisting in cirrhotics.
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