Chronic Thromboembolic Pulmonary Hypertension: Medical Therapy as a Viable Option in a Limited Resource Environment

Pulmonary endarterectomy is the only potentially curable treatment of choice for chronic thromboembolic pulmonary hypertension, while balloon pulmonary angioplasty has been advocated for peripheral (distal) chronic thromboembolic pulmonary hypertension. In an environment where experts for surgical interventions are not readily available, oral medications should be considered early, especially when the distal pulmonary vessels are affected. We present a 43-year-old male diagnosed with elephantiasis of the left leg at 20 years of age. He presented at the emergency room with a 6-month history of progressive dyspnea, easy fatigability, cough with hemoptysis, and World Health Organization functional class IV. Electrocardiogram findings were consistent with pulmonary embolism. He had transthoracic echocardiography and also chest computed tomography pulmonary angiography, which confirmed peripheral chronic thromboembolic pulmonary hypertension. He was commenced on oral medications with significant improvement in prognostic variables such as 6-minute walk test, World Health Organization functional class, and oxygen saturation even a year after commencement.