A 53-year-old woman comes to the emergency department after coughing up blood while at work. She has had no prior episodes but gives a lengthy history of shortness of breath, fatigue, palpitations, and cough while lying down. She has not seen a physician in many years. Her blood pressure is 122/70 mm Hg, pulse is 98/min, and respirations are 23/min. Her body mass index is 20 kg/m². The patient appears anxious and fatigued. Jugular venous pressure is estimated at 9 cm H₂O. Cardiac examination shows normal apical impulse with loud 1st heart sound and short apical low-pitched diastolic rumbling murmur. Bilateral crackles are heard. Electrocardiogram shows a broad-notched P wave in lead II as well as right axis deviation. Chest radiograph shows pulmonary edema, prominent pulmonary arteries at the hilum, elevation of the left mainstem bronchus, and left atrial enlargement with a flattening of the left heart border. Which of the following is the most likely diagnosis?

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Explanation: 11ec2b69_c11b_399a_8f63_71dbc11148d4_MD0005_00 This patient's presentation suggests likely mitral stenosis (MS) due to rheumatic heart disease. There is often a latency period of 10-20 years between the initial episode of rheumatic fever and symptomatic MS, with most patients manifesting during the 4th or 5th decade of life. Common symptoms include dyspnea (70% of patients), fatigue, atrial fibrillation, and thromboembolism (eg, stroke). Hemoptysis (from pulmonary edema or pulmonary apoplexy) and hoarseness (due to compression of recurrent laryngeal nerve by enlarged left atrium) may occur. Pregnant women with MS are at risk for atrial fibrillation and pulmonary edema due to physiologic hypervolemia and increased left atrial and pulmonary venous pressure. Advanced disease may lead to right-sided heart failure (eg, edema, pleural effusions, hepatic congestion). Cardiac auscultation may show the classic MS murmur, a low-pitched diastolic rumble most prominent at the apex. Other findings include loud 1st heart sound (S1), loud pulmonic (P2) component of second heart sound (due to pulmonary hypertension), and normal apical impulse. MS diagnosis is based on clinical presentation and typical demographics; it is further confirmed by classic findings from physical examination and diagnostic evaluation (eg, chest x-ray, echocardiogram). (Choice A) Patients with atrial septal defect and moderate-to-large left-to-right shunt have increased flow through the pulmonic valve. This produces a mid-systolic ejection murmur resulting from increased flow across the pulmonic valve. Another characteristic finding is the presence of wide and fixed splitting of the second heart sound. (Choice B) Cardiac auscultation in patients with mitral valve prolapse typically shows single or multiple non-ejection clicks and/or mid-to-late systolic murmur of mitral regurgitation. The apical impulse is displaced in patients with chronic mitral regurgitation. (Choice C) Noonan syndrome is an autosomal dominant disorder characterized by short stature, facial dysmorphism, and a spectrum of congenital heart defects. Cardiac involvement is seen in up to 90% of patients and includes pulmonic stenosis, atrial septal defects, and hypertrophic cardiomyopathy. (Choice D) Restrictive cardiomyopathy presents with signs and symptoms of congestive heart failure (eg, jugular venous distension, bibasilar crackles, pulmonary vascular congestion), biatrial enlargement, and pulmonary artery hypertension. First and second heart sounds are usually normal. However, this patient's presentation (loud S1 and apical rumbling murmur) is most suggestive of mitral stenosis. Educational objective: The most common cause of mitral stenosis is rheumatic heart disease, with symptoms presenting 10-20 years after initial rheumatic fever. Patients often experience dyspnea, orthopnea, and paroxysmal nocturnal dyspnea. Cardiac auscultation shows loud first heart sound (S1) and mid-diastolic rumble best heard at the cardiac apex. References: Mitral stenosis. (http://www.ncbi.nlm.nih.gov/pubmed/19747723)

A 53-Year-Old Woman Comes to the Emergency Department After Coughing