Abstract
BACKGROUND Pulmonary arterial hypertension (PAH), which is caused by increased pulmonary artery pressure, results in right-heart failure and presents with shortness of breath, chest pain, and syncope. PAH has idiopathic, heritable, and drug/toxin causes and is accompanied by other conditions, including connective tissue disease, congenital heart disease, and portal hypertension. Rarely, portal hypertension causes a type of PAH called portopulmonary hypertension (POPH). Portal hypertension can be triggered by liver cirrhosis, which can result from non-alcoholic steatohepatitis (NASH), a metabolic syndrome caused by hypopituitarism. Although an association between hypopituitarism and POPH has been suggested, few reports have described this relationship. CASE REPORT A 43-year-old woman with hypopituitarism received hormone replacement therapy after partial hypothalamic resection at age 4 years. At age 32 years, she developed liver cirrhosis from NASH due to adult growth hormone (GH) deficiency. Despite restarting GH replacement therapy, she refused the required GH doses for economic reasons. She was hospitalized with abdominal pain and dyspnea and was found to have severe POPH. She received PAH-specific therapies, including endothelin receptor antagonist and prostacyclin analog. Pulmonary hypertension improved on day 3 of hospitalization while the cardiac index increased gradually. On day 12, her respiratory status rapidly worsened and percutaneous cardiopulmonary support was applied. On day 18, she died of multiple organ failure and disseminated intravascular coagulation despite intensive care management. CONCLUSIONS Severe PAH, particularly POPH, remains incurable despite the use of PAH-specific therapies and intensive care management. For hypopituitarism patients, careful observation, including of the cardiopulmonary system, can improve the prognosis after completing hormone replacement therapy.