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Acute endocarditis
4%
10/252
Extra-cardiac left-to-right shunting
5%
12/252
Mitral regurgitation
8%
21/252
Mitral stenosis
12%
30/252
Reversal of left-to-right shunting
63%
159/252
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The patient in this vignette presents with fixed splitting of the second heart sound suggestive of an atrial septal defect (ASD). Untreated ASDs can lead to Eisenmenger syndrome which is characterized by reversal of left-to-right cardiac shunting, right ventricular hypertrophy, and pulmonary hypertension. Eisenmenger syndrome is a long-term sequela of untreated septal defects and extracardiac left-to-right shunts. This most commonly develops before puberty but can also present later in life. In Eisenmenger syndrome, long-term left-to-right shunting causes increased right heart pressure and pulmonary blood flow. This ultimately leads to the triad of pulmonary hypertension, right ventricular hypertrophy, and reversal of left-to-right shunting due to a change in pressure gradient between the left and right heart. Clinically, this may present with exertional dyspnea, fatigue, syncope, and systemic hypoxia (e.g., central cyanosis and clubbing). Incorrect Answers: Answer 1: Acute endocarditis refers to inflammation of a heart valve often due to underlying infection. Risk factors for acute endocarditis include intravenous drug use and damaged heart valves (e.g., mitral valve prolapse). Untreated septal defects are not associated with acute endocarditis. Answer 2: Extra-cardiac left-to-right shunting can occur due to a patent ductus arteriosus and anomalous pulmonary venous return. Similar to intra-cardiac left-to-right shunts, extra-cardiac shunts can lead to Eisenmenger syndrome. Extra-cardiac shunts are not consequences of a persistent atrial septal defect. Answer 3: Mitral regurgitation is characterized by a holosystolic murmur at the cardiac apex and can be caused by rheumatic fever and chordae tendonae rupture. Mitral regurgitation is not a consequence of a persistent atrial septal defect. Answer 4: Mitral stenosis is characterized by a diastolic decrescendo murmur and an opening snap on auscultation. Rheumatic fever is the primary cause of mitral stenosis. Mitral stenosis is not a consequence of a persistent atrial septal defect. Bullet Summary: Eisenmenger syndrome can result from an untreated septal defect and is characterized by the triad of pulmonary hypertension, right ventricular hypertrophy, and reversal of left-to-right shunting.
3.3
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