A 38-year-old woman comes to the physician with progressive exertional shortness of breath over the last 6 months. She thinks it began with a cold followed by a cough that eventually subsided. However, the shortness of breath has persisted. The patient can barely walk 2 blocks on level ground without becoming short of breath. She has a 15-pack-year smoking history.
Her blood pressure is 142/78 mm Hg, pulse is 90/min, and oxygen saturation is 94% on room air. Her BMI is 27 kg/m². Cardiac examination reveals a 2/6 holosystolic murmur at the lower sternal border that increases with inspiration. Lungs are clear to auscultation. There is trace bilateral lower-extremity edema.
Pulmonary function test results are as follows:

Which of the following is the most likely diagnosis?

Question

A 38-year-old woman comes to the physician with progressive exertional shortness of breath over the last 6 months. She thinks it began with a cold followed by a cough that eventually subsided. However, the shortness of breath has persisted. The patient can barely walk 2 blocks on level ground without becoming short of breath. She has a 15-pack-year smoking history.
Her blood pressure is 142/78 mm Hg, pulse is 90/min, and oxygen saturation is 94% on room air. Her BMI is 27 kg/m². Cardiac examination reveals a 2/6 holosystolic murmur at the lower sternal border that increases with inspiration. Lungs are clear to auscultation. There is trace bilateral lower-extremity edema.
Pulmonary function test results are as follows:

Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec193a_98da_6602_853d_31fd4a9a6e82_MD0002_00 This patient's presentation of progressive shortness of breath, holosystolic murmur increased with inspiration (tricuspid regurgitation), and peripheral edema is likely from pulmonary hypertension (PH). Patients most commonly present with exertional dyspnea, fatigue, and syncope that reflects an inability to increase cardiac output during activity. Typical angina is due to pulmonary artery stretching or right ventricular ischemia despite normal coronary arteries. PH is often due to idiopathic (primary) causes, chronic hypoxemia, obstructive or restrictive lung disease, thromboembolic disease, or left-sided heart disease. However, PH is more commonly seen in severe interstitial lung disease with lung volumes < 50% predicted or severe obstructive lung disease. Pulmonary function tests may be normal or slightly abnormal (mild to moderate decreased FEV₁ and FVC) as PH itself may cause some of the abnormalities. However, diffusion capacity for carbon monoxide (DLCO) is usually significantly decreased in most PH patients. Pulmonary function tests This patient's BMI of < 30 kg/m² makes obstructive sleep apnea/obesity hypoventilation unlikely (Choice C). The FEV₁/FVC of > 80% makes obstructive lung disease less likely (Choice A). The FVC of > 80% makes restrictive lung disease unlikely (Choices B and E). This patient's pulmonary function tests (except DLCO) are essentially normal, making chronic thromboembolic disease, left-sided heart disease, and idiopathic primary PH the likely possibilities. The low DLCO also favors idiopathic primary PH. Educational objective: Pulmonary hypertension may present with nonspecific symptoms such as dyspnea, lethargy, or fatigue. Pulmonary function tests in primary pulmonary hypertension are usually normal or mildly abnormal with decreased diffusion capacity for carbon monoxide. __________ References: Pulmonary arterial hypertension: an update on diagnosis and treatment. (http://www.ncbi.nlm.nih.gov/pubmed/20704168) Diagnosis and treatment of pulmonary hypertension. (http://www.ncbi.nlm.nih.gov/pubmed/11352291)

A 38-Year-Old Woman Comes to the Physician with Progressive Exertional