A 44-year-old woman has been complaining of a 4-year history of increasing dyspnea and fatigue. Physical examination reveals increased JVP and a reduced carotid pulse. Precordial examination reveals a left parasternal lift, loud P2, and right-sided S3 and S4. There are no audible murmurs. CXR reveals clear lung fields and an ECG shows evidence of right ventricular hypertrophy. Pulmonary function tests show a slight restrictive pattern. A diagnosis of primary pulmonary hypertension (PPH) is made. Which of the following is the most likely cause of death in this condition?
Correct Answer: intractable right ventricular failure or sudden death
Description: Death is usually the result of either intractable right heart failure or sudden death. The natural history of the disease is unclear because the disease is asymptomatic for a long period. Survival from diagnosis is dependent on the functional class of the patient. Functional class IV dyspnea suggests a mean survival of only 6 months. Patients with PPH are also at increased risk of veno-thromboembolic disease and current guidelines recommend chronic anticoagulation with warfarin. Left ventricular failure and myocardial infarction are not usual causes of death in patients with PPH.
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