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Anomalous papillary muscle in hypertrophic cardiomyopathy
  1. falfonso{at}

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Hypertrophic cardiomyopathy may cause congestive heart failure. Failure to recognise its predominant pathophysiologic mechanism may have adverse consequences. A 75 year old women was admitted for progressive dyspnoea and new onset atrial fibrillation. Propafenone was unsuccessful in restoring sinus rhythm. Digoxin and diuretics were started without clinical improvement, and captopril was added. A two dimensional (cross sectional) echocardiogram revealed asymmetric septal hypertrophy (ante- rior septum 25 mm) and a dynamic intraven- tricular gradient of 85 mm Hg. Digoxin and captopril were halted and oral verapamil was administered with care. However, progressive hypotension and oliguria ensued and eventually the patient experienced cardiac arrest and died. Pathological examination revealed a hypertrophied left ventricle with anomalous insertion of papillary muscle directly into the anterior mitral leaflet (below). The left ventricular cavity was reduced by the anterior displacement of the hypertrophied papillary muscle. The ventricular aspect of the septum, facing the anomalous papillary muscle, showed a thin, whitish cap, suggestive of dynamic mid-cavity apposition of both structures. Severe disarray was recognised on histology.  In some patients with hypertrophic cardiomyopathy, direct continuity between a hypertrophied papillary muscle and the mitral valve is detected. This may induce severe left ventricular outflow obstruction. When this rare congenital malformation is recognised mitral valve replacement rather than myotomy–myectomy has been advocated. Unfortunately, in our patient the diagnosis of hypertrophic cardiomyopathy was performed too late, and the change in medical treatment was unable to halt the downhill course of her cardiac failure. Our findings underscore the importance of keeping a high degree of suspicion for the diagnosis of this disease in patients unresponsive to conventional management. Likewise, special care is required (including off-axis echocardiographic views) to identify correctly diverse abnormalities of the mitral apparatus causing intraventricular gradients.