Type Ia tricuspid atresia, with extensive coronary artery abnormalities, is identified in the oldest living patient with this condition, a 22 year old woman. Clinical characteristics include severe cyanosis, effort dyspnea, myocardial infarction in the past and persistent angina pectoris. "Ideal" pulmonary flow and adequate left ventricular function, despite an akinetic apical segment, are substantive factors for this exceptional longevity. Coronary abnormalities consist of: 1) total proximal occlusion of the left anterior descending coronary artery; and 2) partial diversion of coronary artery flow to a segmental pulmonary artery branch. Nonvisualization of the coronary sinus is also noted. Factors other than atherosclerosis may account for total proximal occlusion of the left anterior descending coronary artery. Survival is threatened by adverse effects of ongoing ischemic coronary events.
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