Pulmonary hypertension in patient with end stage pulmonary sarcoidosis: challenges in treatment

Abstract

Introduction and aim Pulmonary hypertension (PH) is recognised as a serious complication of pulmonary sarcoidosis. The frequency of PH largely depends on the severity of the disease and is associated with poor outcomes. Current sarcoidosis associated pulmonary hypertension (SAPH) treatment recommendations suggest optimisation of underlying lung disease and use of oxygen when hypoxemia is present, however, there is still insufficient evidence to recommend advanced therapies. Here we present challenges in treatment of a woman with end stage pulmonary sarcoidosis and severe SAPH to demonstrate the effect of oral vasodilator therapy while on the list for urgent lung transplantation. Case report A 40-year-old lady with history of sarcoidosis presented to our clinic due to worsening respiratory insufficiency. Physical exam was notable for cachexia, tachypnoe, cyanosis, peripheral edema, and ascites. On presentation the patient was put on supplemental oxygen and diuretic therapy. Due to severe dyspnoe 6 minute walk test could not be performed. Following a thoracic CT scan, transthoracic echocardiography, and right heart catheterization (Table 1 A,B) she was determined to have stage IV sarcoidosis with severe pulmonary fibrosis and cystic degeneration, SAPH, and associated right ventricular failure. Due to poor functional status (NYHA IV stage), severe pulmonary fibrosis, as well as severe PH, she was determined to have a bad prognosis according to available scientific data. The patient was included to the Urgent Lung Transplant list and Sildenafil was started increasing dosage from 10 to 20 mg x 3 times a day alongside optimal pharmaceutical heart failure treatment. The clinical improvement was noticed immediately after the initiation of specific PH therapy and has remained stable for a year. 2D echocardiographic parameters didn`t change significantly. The patient is doing better from a clinical stand-point, the object[...].