ELSEVIER International Journal of Cardiology 56 (1996) 301-305 International Journal of cardiology Holmes heart in the adult: transesophageal echocardiographic findings and long-term natural survival Antonio Vitarelli” , Fulvio Gabbarini Cardiac Department, ‘LA Sapienza’ Universiq, Rome, Italy Received 28 May 1996; revised 23 July 1996: accepted 23 July 1996 Abstract We describe a 59-year-old patient with Holmes heart (single ventricle with normally related great arteries). To our knowledge, this is the oldest patient in the literature with this rare congenital heart diseaseand the oldest one with single ventricle and unrestricted pulmonary blood flow who has developed Eisenmenger’s physiology. A transesophageal echocardiographic examination showed a single large ventricle that was a morphologic left ventricle with a normally appearing aorta originating from this chamber and a normally related pulmonary artery arising from a hypoplastic right infundibulum located anteriorly-superiorly and to the left of the left ventricle. The cardiac catheterization confirmed the echocardiographic findings and revealed a pulmonary artery pressureof 160/60 mmHg. There were no systolic gradients across the pulmonary and aortic outflow tracts. The estimated pulmonary vascular resistance was 2720 dynes-set-cm- ‘. Oximetry showed oxygen saturation in the left ventricle of 84% and femoral artery saturation of 77.7%. Our report further demonstrates that pulmonary stenosis is not the ultimate determinant of prolonged natural survival in patients with single ventricle. Since this patient is too old for cardiopulmonary transplantation, we plan to follow him conservatively with medical therapy. Keywords: Congenital heart disease;Single ventricle; Transesophageal echocardiography 1. Introduction The development of new surgical approaches to single ventricle (car triloculare biatriatum) has made it important to know the long-term outcome in unoperated patients. Although this congenital heart disease may result in death in infancy or childhood, a selected group of patients reach adulthood [1,2] and *Corresponding author, via Lima 35 00198 Rome Italy. Tel: 06/85301427; fax: 06/8841926; e mail: AMtarelli@agora.stm.it in fact single ventricle is one of the commoner complex malformations seen in adults. Most often it is associated with transposed great arteries. To our knowledge, the oldest patients described with single ventricle and transposition of the great arteries were 62 years of age with associated pulmonary stenosis [3] and 31 years of age with no appreciable pulmonic stenosis [4]. A more rare variety of single ventricle is the anomaly usually called ‘Holmes heart’, [S] in which the great arteries are normally related. We recently studied a man with Holmes heart and 0167.5273/96/$15.00 0 1996 Elsevier Science Ireland Ltd. All rights reserved PII SO167-5273(96)02783-O