During the next 6 years, the dyspnea slowly but progressively worsened and, in 1996 at age 59, he underwent evaluation by a cardiologist. Cardiac catheterization disclosed angiographically normal epicardial coronary arteries and a left ventricular ejection fraction of approximately 25%. Echocardiogram disclosed left ventricular dimensions of 6.3 cm in end diastole and 5.7 cm in peak systole. A diagnosis of idiopathic dilated cardiomyopathy was made, and appropriate therapy was instituted. A dual-chamber transvenous pacemaker was inserted because of complete heart block, which apparently had been present for decades.

In March 1999, the patient was reevaluated because of further worsening of symptoms of heart failure. At that time, his pulmonary arterial pressure was 55/22 mg Hg; mean pulmonary artery wedge pressure, 15 mm Hg; body mass index, 30 kg/m2; and blood pressure, 135/90 mm Hg. On cardiopulmonary stress test, he was able to exercise for only 2 minutes and 14 seconds on a Naughton protocol, achieving a peak exercise oxygen consumption of 10 cc/kg/min. At baseline, his heart rate was 64, and at peak exercise, it was still only 64 beats a minute. The minute ventilation went from 15 to 46 L per minute (79% of predicted), despite the fact that the heart rate did not change. The room air blood gases disclosed a pH of 7.46; partial pressure of carbon dioxide, 39; partial pressure of oxygen, 58; forced expiratory volume in 1 second, 1.7; and forced vital capacity, 2.71. At this time, the patient was on enalapril, hydrochlorothiazide, allopurinol, spironolactone, digoxin, and aspirin. He had had systemic hypertension for many years before being on any hypertensive therapy. He smoked 20 cigarettes daily for about 40 years.

Because of progressive worsening of the heart failure, cardiac transplantation was performed. The procedure went smoothly. The excised heart weighed 707 g, and a number of adhesions were present on the epicardial surface. Both ventricular cavities were quite dilated (Figure). A small scar was present in the anterior wall of the right ventricle, and a large transmural scar was present in the most basal portion of the ventricular septum in its central portion (Figure). There was no scarring in the left ventricular free wall. The 4 cardiac valves were normal. The epicardial coronary arteries were large and virtually devoid of atherosclerotic plaques.

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The unusual finding in the heart was the large transmural scar in the ventricular septum and a smaller scar in the right ventricular free wall. The stab wound to the heart 40 years earlier led to a 2-day delay of hemopericardium and an emergency cardiac operation. The details of the operation 40 years ago are not available, but no sutures or prosthetic material was present in the ventricular septum, indicating that no procedure was done within the heart. It is likely, however, that the knife penetrated not only the right ventricular free wall but also the ventricular septum, producing a ventricular septal defect in the muscular portion of the ventricular septum. The ventricular septal defect later closed, because the ventricular septum was intact and no precordial murmur had ever been heard. The heart failure was probably a combination of 40 years of complete heart block, causing considerable dilatation of both ventricular cavities with superimposed systemic hypertension for many years. The area of the atrioventricular bundle was extensively scarred.

In summary, we describe a patient who underwent cardiac transplantation because of heart failure and was found to have a large scar in his ventricular septum and fibrosis of his atrioventricular nodal area, suggesting that he had complete heart block for 40 years. The ventricular dilatation likely was the result of both complete heart block and superimposed systemic hypertension.