Diagnosis and treatment in a series of patients with cardiac corrected transposition of the great arteries (double discordance)

Jesús De Rubens-Figueroa, Servicio de Cardiología Pediátrica, Instituto Nacional de Pediatría, Ciudad de México, México
Paulina Villamar-García, Servicio de Pediatría, Instituto Nacional de Pediatría, Ciudad de México, México
Diana G. Ruiz-Meléndez, Servicio de Cardiología, Instituto Nacional de Pediatría, Ciudad de México, México
Carlos González-Rebeles-Guerrero, Servicio de Cardiología Pediátrica, Instituto Nacional de Pediatría, Ciudad de México, México
Alexis Palacios-Macedo, Servicio de Cirugía Cardiaca, Instituto Nacional de Pediatría, Ciudad de México, México

Objective: The atrio-ventricular and ventricle-arterial double discordance (DD) or corrected transposition of the great arteries is a rare heart disease, it occurs in 0.02-0.07 of every 1,000 live newborns. The objective of the study is to describe the diagnosis, treatment and evolution of a series of patients with DD. Method: A retrospective and descriptive study was carried out, reviewing the records of patients diagnosed with DD in the last 22 years. Descriptive statistics were performed. Numrical variables were obtained using means and standard deviation and categorical variables using frequencies and percentages. Results: Thirty patients were studied in 22 years with a ratio of 1.5:1 for men, with a mean age of 20 months. The situs was solitus in 24/29 patients (82.7%). Ventricular septal defect was the most frequent lesion in 25/29 (86.2%) Tricuspid insufficiency in 70%. Four patients diagnosed with pulmonary atrial hypertension. With atrio-ventricular block 20%. One with Wolff-Parkinson-White syndrome. Surgical treatment was carried out in 70% of patients. Eight with Glenn procedure (26.6%) and 4 with Fontan surgery (13.3%). Follow-up ranged from 1 month to 17 years. Five died (16.6%). Of the 25 patients in follow-up, 18 patients (72%) had normal ventricular function, 5 with Grade II Ross classification (20%) and 2 in Grade III (8%). Conclusions: The quality of life of these patients is improving and there is still controversy in the literature about the ideal time to perform the most appropriate surgical procedure.

Keywords: Congenital heart disease. Congenitally corrected transposition of the great arteries. Ventricular inversion. Double discordance.