HOLMES HEART IN THE ADULT - TRANSESOPHAGEAL ECHOCARDIOGRAPHIC FINDINGS AND LONG-TERM NATURAL SURVIVAL

We describe a 59-year-old patient with Holmes heart (single ventricle with normally related great arteries). To our knowledge, this is the o ldest patient in the literature with this rare congenital heart diseas e and the oldest one with single ventricle and unrestricted pulmonary blood flow who has developed Eisenmenger's physiology. A transesophage al echocardiographic examination showed a single large ventricle that was a morphologic left ventricle with a normally appearing aorta origi nating from this chamber and a normally related pulmonary artery arisi ng from a hypoplastic right infundibulum located anteriorly-superiorly and to the left of the left ventricle. The cardiac catheterization co nfirmed the echocardiographic findings and revealed a pulmonary artery pressure of 160/60 mmHg. There were no systolic gradients across the pulmonary and aortic outflow tracts. The estimated pulmonary vascular resistance was 2720 dynes-sec-cm(-5). Oximetry showed oxygen saturatio n in the left ventricle of 84% and femoral artery saturation of 77.7%. Our report further demonstrates that pulmonary stenosis is not the ul timate determinant of prolonged natural survival in patients with sing le ventricle. Since this patient is too old for cardiopulmonary transp lantation, we plan to follow him conservatively with medical therapy.