CORRECTED TRANSPOSITION OF THE GREAT-ARTERIES WITHOUT ASSOCIATED DEFECTS IN ADULT PATIENTS - CLINICAL PROFILE AND FOLLOW-UP

Objective-To assess the clinical course of adult patients with correct ed transposition of the great arteries without associated anomalies. D esign-All patients with corrected transposition of the great arteries without associated anomalies were reviewed with complete clinical and echocardiographic assessment. The complications were evaluated in each decade. Setting-Tertiary centre with a specific unit dealing with ''g rown-up'' adolescent and adult congenital heart disease, designated as a quaternary centre and a general hospital with a referral centre for ''grown-up'' congenital heart disease. Patients-18 patients (nine mal e and nine female) aged 16-61 years followed for 1-30 years (mean 10 y ears). Results-There were no deaths. Six patients had a worsening abil ity index during follow up. Complications were: (a) complete heart blo ck in seven, three of whom required pacemaker insertion; (b) significa nt left atrioventricular valve regurgitation in 50%, appearing only in the third decade (12%), with increasing frequency thereafter. Infecti ve endocarditis was responsible for increasing left atrioventricular v alve regurgitation in only one patient; (c) supraventricular arrhythmi a appeared in the fifth decade, and occurred in all patients over the age of 60 years. One patient aged 61 had recurrent sustained ventricul ar tachycardia; and (d) congestive heart failure developed only after 50 years in 66%. One patient had severe left atrioventricular valve re gurgitation; the function of the systemic ventricle was only moderatel y reduced in the other three. Three of the nine women had seven uneven tful pregnancies. Conclusions Patients with corrected transposition of the great arteries without associated defects may remain undiagnosed until adult life. Symptoms occur rarely before the fourth and fifth de cades, when rhythm disturbance, left atrioventricular valve regurgitat ion, and moderately impaired systemic ventricular function cause conge stive cardiac failure. The role of pacemaker insertion or surgery for left atrioventricular valve regurgitation needs further assessment.