A 45-year-old woman comes to the physician because of exertional dyspnea and fatigue. She has been gaining weight lately due to her sedentary lifestyle and started exercising with the help of a professional trainer. She becomes short of breath easily and attributes this to deconditioning. However, she had a near syncopal episode during her last exercise session. She denies dyspnea, fatigue at rest, cough, chest pain, morning headaches, nocturnal awakenings, snoring, or mood changes.
Her other medical problems include a history of pulmonary embolism (PE) 3 years ago after an abdominal hysterectomy for complex atypical endometrial hyperplasia. She received anticoagulation for six months and has been doing well since then. She quit smoking after the pulmonary embolism and denies using alcohol or illicit drugs. Her mother has a history of rheumatoid arthritis and her father died from an acute myocardial infarction.
Vitals signs and complete physical examination are within normal limits. Her BMI is 26 kg/m².
Complete blood count, liver function tests, and serum chemistries are within normal limits. Chest x-ray and high resolution CT scan of the chest without contrast showed no abnormalities. Echocardiogram shows normal left ventricular size and function, moderate right ventricular enlargement, and an estimated pulmonary artery systolic pressure of 60 mm Hg. Pulmonary function tests are within normal limits.
Which of the following is the most appropriate next step in management?

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Quizplus · Accepted Answer

11ec193a_98d0_c903_853d_ab3b83e8c8f9_MD0002_00 This patient's presentation is consistent with pulmonary hypertension (PH), defined as a mean pulmonary artery pressure > 25 mm Hg at rest. Pulmonary hypertension etiologies include drugs, connective tissue disease, HIV, sleep-related breathing disorders, chronic thromboembolic disease, left-sided heart disease, and COPD. Although she was a smoker, her normal pulmonary function tests exclude COPD. Echocardiography showed no evidence of left-sided heart disease. Chest CT did not reveal any interstitial lung disease. She does not appear to have obstructive sleep apnea. Given her otherwise normal tests and history of PE, chronic thromboembolic pulmonary hypertension (CTEPH) is the most likely etiology of her symptoms. Following a pulmonary embolic event, a patient can remain asymptomatic or minimally symptomatic for months to years. Progressive CTEPH can occur even without evidence of extensive pulmonary vascular obstruction, recurrent embolic events, or in situ thrombosis. Failure of plasma-mediated lysis of the initial clot followed by altered resistance in the peripheral pulmonary vascular bed may be responsible for disease progression. A V/Q scan is more sensitive than CT pulmonary angiogram in detecting CTEPH, and a normal V/Q scan virtually excludes CTEPH (Choice A). Surgical thromboendarterectomy is the only cure for CTEPH, and early referral should be made even if the disease severity is only mild. Also, all patients should receive life-long anticoagulation with a target INR of 2.0 to 3.0. (Choice B) Exercise echocardiogram can be helpful if the resting ECHO failed to identify pulmonary hypertension. This can also be used to evaluate for WHO functional class and help determine therapy. While this test may be performed during PH evaluation, a V/Q scan is more helpful at this time to identify the etiology of her symptoms. (Choice C) Polysomnography can diagnose obstructive sleep apnea, which can cause pulmonary hypertension. However, this patient has no symptoms (e.g., daytime sleepiness) of a sleep related disorder. (Choice D) Connective tissue disease and rheumatoid arthritis can lead to interstitial fibrosis with obliteration of the pulmonary vascular bed and pulmonary hypertension. However, this patient's normal joint examination, normal chest x-ray, and normal CT make this unlikely. Educational objective: V/Q scanning is superior to CT pulmonary angiogram in diagnosing chronic thromboembolic pulmonary hypertension (CTEPH). All patients should receive lifelong anticoagulation with a target INR of 2.0 to 3.0 and an early referral for surgical thromboendarterectomy. __________ References: Chronic thromboembolic pulmonary hypertension (http://www.ncbi.nlm.nih.gov/pubmed/20376164)

A 45-Year-Old Woman Comes to the Physician Because of Exertional