Successful outcomes following living donor liver transplantation for portopulmonary hypertension

Abstract

Pulmonary arterial hypertension (PAH) associated with portal hypertension [portopulmonary hypertension (PPHTN)] occurs in 2% to 10% of patients with advanced liver disease and carries a very poor prognosis without treatment. Most hepatic transplantation centers consider moderate to severe PPHTN to be a contraindication to liver transplantation because of the high rate of perioperative complications. We present 3 patients with PPHTN who were managed with intravenous prostacyclin therapy followed by living donor liver transplantation (LDLT). These individuals demonstrated subsequent resolution of their pulmonary hypertension and were weaned off all PAH-specific medical therapy. We present their demographics, clinical courses, and hemodynamics. We discuss the potential indications for LDLT and risks with respect to this patient population. Pulmonary arterial hypertension (PAH) associated with portal hypertension (with or without cirrhosis) has been termed portopulmonary hypertension (PPHTN). 1 PPHTN occurs in 2% to 10% of patients with advanced liver disease, with about 1% of patients demonstrating clinically severe pulmonary hypertension. 2,3Diagnostic criteria for PPHTN include (1) the presence of clinical portal hypertension, (2) a mean pulmonary artery pressure (mPAP) greater than 25 mm Hg, (3) a pulmonary artery occlusion pressure (PAOP) less than 15 mm Hg, and (4) Liver transplantation (LT) is a potential option for patients with PPHTN; however, patients with moderate to severe PPHTN (mPAP ! 35 mm Hg) and a PVR greater than 250 dyne Á second/cm 5 have a particularly poor prognosis and unacceptably high posttransplant mortality.5 As a result, most LT centers consider Abbreviations: DDLT, deceased donor liver transplantation; DLCO/VA, diffusing capacity for carbon monoxide corrected for alveolar volume; FEV1, forced expiratory volume in the first second; FVC, forced vital capacity; LDLT, living donor liver transplantation; LT, liver transplantation; MELD, Model for End-Stage Liver Disease; MESSAGE, MELD Exceptional Case Study Group; mPAP, mean pulmonary artery pressure; PAH, pulmonary arterial hypertension; PAOP, pulmonary artery occlusion pressure; PAP, pulmonary artery pressure; PPHTN, portopulmonary hypertension; PVR, pulmonary vascular resistance; RAP, right atrial pressure; RHC, right heart catheterization; RV, right ventricular; RVSP, right ventricular systolic pressure.The current address for Richard B. Freeman is

Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview

“…Initial experience indicated that post-transplant improvement in ''POPH'' was limited to patients with PH secondary to an increased cardiac output (elevated mean Ppa but PVR ,240 dyn?s?cm -5 ). Accumulating evidence from different transplant centres is now manifesting post-transplant improvement in selected POPH patients, some of them being able to wean-off their PAH-specific therapy a few months after LTx (probably ,50% of those surviving the early months after LTx) [33,40,54,56]. Although these are encouraging results, evidence derives from retrospective analyses of transplant databases or case series.…”

Assessment of Biometal Profile in Children with Autism Spectrum Disorder, with Attention Deficit Hyperactivity Disorder, or with Other Psychiatric Diagnoses: A Comparative Outpatient Study

“…A mean PAP of 50 mmHg is contraindication to liver transplantation [3]. An aim of PAP of <35 mmHg is necessary if liver transplant is contemplated in cirrhotic patients [37]. The 5-year survival of POPH is 14-45% of those receiving medical treatment.…”

Portopulmonary hypertension

“…Assuming up to 8.5% of LT candidates have PPH, at any point in time there may be ∼1300 PPH-LT candidates [27]. The outcome of PPH following LT remains unpredictable despite screening, careful patient selection, higher priority for LT, and advances in single and combination PAH-specific therapies (Table 19.4) [69][70][71][72][73][74][75][76][77][78]. Current policy adopted by the American Association for the Study of Liver Diseases (AASLD) calls for screening TTE to detect pulmonary hypertension in every patient considered for LT in the United States [79].…”