A 35-year-old African American woman comes to the clinic due to exertional shortness of breath. The patient's exercise tolerance a year ago was walking 7 blocks but now she walks less than a block before becoming short of breath. She also feels lightheaded when she forces herself "to go on." The patient denies any episodes of syncope, chest pain, cough, wheezing, orthopnea, nausea, or lower extremity edema. She has been treated for Raynaud phenomenon and fingertip ulcerations for the last 2 years. She also suffers from severe heartburn controlled with high-dose pantoprazole. Family history is not significant. She does not smoke cigarettes or consume alcohol. Blood pressure is 112/72 mm Hg and pulse is 94/min. Pulse oximetry shows 95% on room air. BMI is 23 kg/m².
Which of the following would be the most expected finding on physical examination?

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11ec25c8_d4cb_de29_bc81_414f521f228c_MD0003_00 This patient most likely has CREST syndrome (calcinosis cutis, Raynaud phenomenon, esophageal dysmotility with reflux, sclerodactyly, telangiectasia), which is strongly associated with limited cutaneous systemic sclerosis (SS). African American patients are most commonly affected by SS, and vascular dysfunction is a prominent component of the disease. Raynaud phenomenon is present in approximately 90% of patients and typically precedes other disease manifestations by several years. Pulmonary arterial hypertension (PAH) is also common, especially in the limited cutaneous subtype of SS. limited cutaneous PAH results from intimal hyperplasia of the pulmonary arteries and typically presents with progressive dyspnea. Patients may also experience exertional syncope or presyncope (eg, lightheadedness) due to right ventricular (RV) failure and inadequate venous return to the left atrium. Physical examination should demonstrate RV heave (or parasternal heave), which is an impulse palpated immediately to the left of the sternum that suggests RV enlargement. A loud pulmonary component of the second heart sound and signs of right heart failure (eg, peripheral edema, hepatomegaly) may also be present. (Choice A) Aortic coarctation causes increased blood flow to the upper extremities and decreased blood flow to the lower extremities, resulting in brachial-femoral pulse delay. Absence of upper extremity hypertension and the presence of CREST syndrome features make aortic coarctation less likely. (Choice B) Aortic stenosis can cause a systolic ejection murmur with radiation to the carotids but would be unlikely in a young patient. Hypertrophic cardiomyopathy (HCM) is characterized by a systolic ejection murmur without radiation to the carotids, and African American patients are most commonly affected. However, the absence of a family history makes HCM less likely. (Choice D) This patient's PAH may lead to RV failure, which can demonstrate a right-sided third heart sound (S₃). However, inspiration increases the volume of blood in the right side of the heart; therefore, an S₃ due to RV volume overload is best heard at the left lower sternal border on end-inspiration. An S₃ heard at the apex on end-expiration is consistent with left ventricular failure rather than RV failure. (Choice E) Wide and fixed splitting of the second heart sound occurs in an atrial septal defect (ASD). An uncorrected ASD can eventually lead to right-sided heart failure (and dyspnea) in adults, but PAH due to SS is more likely in this patient with a history of Raynaud phenomenon. Educational objective: Pulmonary arterial hypertension is a common manifestation of systemic sclerosis that typically presents with progressive dyspnea and often leads to right ventricular enlargement and right-sided heart failure. References: Systemic sclerosis-associated pulmonary arterial hypertension. (http://www.ncbi.nlm.nih.gov/pubmed/24081346)

A 35-Year-Old African American Woman Comes to the Clinic Due