Abstract
The interdependence between the heart and liver in maintaining hemodynamic stability during the perioperative period of either orthotopic heart (OHT) or liver (OLT) transplantation is important. The pre-transplant hemodynamic changes that occur in patients with end-stage liver disease (ESLD) can include decreased systemic vascular resistance, poor ventricular response to stress and increased cardiac output (CO). Concomitant pulmonary disorders are often present in ESLD. Portopulmonary hypertension (PoPHTN) is an important marker for increased mortality in liver transplant patients. The pathophysiologic mechanisms specific to PoPHTN have been compared with other known forms of pulmonary hypertension, including primary pulmonary hypertension, and has been found to fall within a spectrum of disorders related to factors both due to intrinsic liver failure [with resultant portal hypertension and hepatopulmonary syndrome (HPS)] as well as pulmonary vascular remodeling. We present a 47-year-old Caucasian female with ESLD secondary to non-alcoholic steatohepatitis and HPS. Our current case demonstrates the difficulty in managing patients with acute pulmonary hypertension after OLT. Review of the contemporary literature demonstrated a total of eight case reports of post-transplant severe pulmonary hypertension thought to be due to a combination of either HPS or PoPHTN. This case highlights the complexities of patient management in the acute setting after OLT. Furthermore, it demonstrates the intricate role of careful preoperative evaluation and screening in patients undergoing workup for solid organ transplantation.