INTRODUCTION:Hepatopulmonary Syndrome (HPS) and Portopulmonary Hypertension (POPH) are clinically and pathophysiologically distinct entities which occur independently in 5-10% of liver transplant candidates. We describe a case in which HPS is diagnosed and then spontaneously resolves with the subsequent appearance of POPH.
CASE PRESENTATION:A 48 year old man, abstinent for 4 years, presented with alcoholic cirrhosis to the Liver Transplant clinic for evaluation. Dyspnea and hypoxia were noted on initial evaluation. On room air, upright arterial blood gas showed PaO2= 50, with supine PaO2= 62, demonstrating orthodeoxia. On 100% FiO2, supine PaO2= 402. Nuclear lung perfusion scan demonstrated quantitative shunt index= 23.7%, representing intrapulmonary vascular dilatation. Echocardiography (echo) with agitated saline contrast injection showed contrast in the left heart after 8-9 cardiac cycles, illustrating intrapulmonary shunting. Right ventricular systolic pressure (RVSP) on echo= 21mmHg. The diagnosis of severe hepatopulmonary syndrome was made. The patient was listed for transplant. After nine months, repeat nuclear perfusion scan showed a decreased shunt index= 12.6%. Three months later, shunt index had decreased to 8.6%, repeat upright PaO2 increased to 63, and RVSP= 28mmHg. Fourteen months after initial evaluation, oxygenation had markedly improved, with mild persistent orthodeoxia, and perfusion scan had normalized, but echo showed pulmonary hypertension, with elevated RVSP= 54 mmHg. Eight months later, the patient presented complaining of progressive dyspnea with minimal exertion, a new symptom, and syncopal episodes preceded by cough. Echo now showed RVSP= 78, with new RVH and RV systolic dysfunction, and EKG showed new right axis deviation. Cardiac catheterization was done, demonstrating severe pulmonary hypertension, with PA pressures= 85/36/53 mmHg, cardiac output= 4.27 L/min, PAOP= 34 mmHg, and PVR= 356 dyne*sec/cm5. CT angiogram was negative for PE, whether chronic or acute. This diagnosis of POPH was made and the patient was started on inhaled iloprost. Liver transplant is deferred until improvement in POPH is documented.
DISCUSSIONS:Due to the success of orthotopic liver transplantation, there has been increasing interest in the diagnosis and therapeutic options for the pulmonary vascular complications of hepatic disease. These pulmonary vascular complications range from the hepatopulmonary syndrome, which is characterized by intrapulmonary vascular dilatations, to portopulmonary hypertension, which is characterized by an elevated pulmonary vascular resistance as a consequence of obstruction to pulmonary arterial blood flow. POPH and HPS are two distinct diseases, with completely different mechanisms of action. It is rare for both diseases to occur in the same patient.
CONCLUSION:This case highlights the spontaneous resolution of severe HPS subsequently followed by the evolution of severe POPH over a several month period. Such a scenario has significant implications for liver transplant candidates.
DISCLOSURE:Jason Golbin, No Financial Disclosure Information; No Product/Research Disclosure Information