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?
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.
intractable left ventricular failure
intractable respiratory failure
massive PE
intractable right ventricular failure or sudden death
Respiratory