Title: A Case Series of Porto Pulmonary Hypertension with Hepatopulmonary Syndrome

Author(s): Sayeda Ali MD; Rasik Neupane MD; Molly Howsare, DO; Niraj Niraula MD

Email: Sali@mercy.com

Introduction:  Porto pulmonary arterial hypertension (PoPAH) is a form of pulmonary arterial hypertension associated with or without chronic liver disease. Right heart catheterization (RHC) can differentiate PoPAH and assesses the hepatic hemodynamics. PoPAH is defined as mPAP ≥ 25 mmHg, a pulmonary vascular resistance >3 Woods Unit (WU), and pulmonary capillary wedge pressure ≤ 15 mmHg. Hepatopulmonary syndrome (HPS) results from pulmonary vascular dilatations, resulting in shunt and oxygen desaturation. PoPAH is a pulmonary arterial vasoconstriction. PoPAH along with hepatopulmonary syndrome, shows the extremes of a spectrum of pulmonary vascular changes.

Case 1:  A 40-year-old female with past medical hx of Nonalcoholic steatohepatitis cirrhosis, recurrent ascites, and hypothyroidism, presented to the ED with c/c of dizziness, back pain, and increased dyspnea. She was admitted to the ICU due to respiratory distress and altered mental status. The patient was icteric, had anasarca, and hypoxemic. Lab findings showed hyponatremia, and increased A-a gradient of 77.5mmHg, ECHO revealed right ventricular systolic pressure (RSVP) of 125 mmHg. RHC findings were consistent with severe PoPAH and moderate HPS.

Case 2:  A 52-year-old female with a past medical history of Lupus, CKD, and Liver cirrhosis with portal hypertension presented with chest pain, dyspnea, and syncope. RHC 3 years prior revealed severe pulmonary hypertension with PVR of 9 WU. Lab findings showed increased Cr and A-a gradient to 34.2mmHg. ECHO showed RSVP of 67 mmHg. Right heart catheterization findings showed severe pulmonary hypertension WHO class I with Porto-pulmonary hypertension and Hepatopulmonary syndrome.

In both cases Treprostinil was initiated.

Discussion:  Liver disease and portal hypertension can be associated with pulmonary vascular complications. On average HPS affects one quarter of patients with severe chronic liver disease. Both cases presented with decreased oxygenation with manifestation of insidious onset dyspnea with platypnea and orthodeoxia. Oximetry and arterial blood gas analysis along with transthoracic contrast echocardiography (TTCE) in those with evidence of impaired oxygenations should be performed (3). An elevated A-a oxygen gradient ≥ 15 mmHg is more sensitive which is increased in both cases. RHC findings were consistent for both HPS and PoPAH. Awareness of evaluation and management algorithms for POPAH and HPS are critical for the optimization of outcomes in patients with these conditions (4). HPS and PoPAH can be coexistent in the same individual as per our cases. Diagnostic accuracy and management can guide clinicians for more detection and proper disease prognostication in future.