Successful repair of congenitally corrected transposition with ventricular septal defect and pulmonary stenosis presenting with heart failure in the fifth decade of life is described. This is the oldest patient to undergo this surgery, as per existing literature.
Pulmonary atresia with ventricular septal defect and coronary-dependent pulmonary circulation arising from both major coronary arteries is rare. Dependence of pulmonary blood flow on the coronaries and the risk of early development of pulmonary vascular obstructive disease warrant early surgical repair in these patients. We report a case of a ten-month-old infant with pulmonary atresia with ventricular septal defect and coronary artery-to-main pulmonary artery connections who was successfully managed with ligation of the coronary fistulas and intracardiac repair.
OBJECTIVES
The study objective was to evaluate the cardiac, neurodevelopmental, psycho-social and health-related quality of life (HRQOL) outcomes of children who underwent an arterial switch operation (ASO).
METHODS
Children who underwent ASO were evaluated on follow-up at 3–5 years with cardiovascular, neurodevelopmental and HRQOL assessment using validated tools. Children with developmental delay, attention-deficit hyperactivity disorder, autism spectrum disorder, neuromotor and speech and language impairment were considered to have neurodevelopmental disorder (NDD). The impact of socioeconomic status (Kuppuswamy classification), perioperative cardiac, nutritional and psycho-social factors on outcomes was analysed.
RESULTS
There were 61 (89.7%) survivors at a mean follow-up of 50.9 ± 7.6 months. The median age at surgery was 41 days (22–74.5). One-third of patients had growth restriction. Two children had residual cardiovascular lesions requiring intervention. The mean HRQOL score was >90 in all scales of the Paediatric Quality of Life Inventory™ 3.0 Cardiac Module. Neurological abnormalities were seen in 19 patients (31.1%) of whom 17 (27.9%) patients had NDD and 12 had developmental delay. Speech and language impairment, attention-deficit hyperactivity disorder, and neuromotor impairment were found in 16.4%, 3.3% and 6.7% patients, respectively. On multivariate analysis, increasing time to lactate normalization and low socioeconomic status were associated with developmental delay after ASO.
CONCLUSIONS
While intermediate-term cardiac outcomes and HRQOL after ASO were fairly satisfactory, NDD was identified in one-fourth of these children. Increasing time to lactate normalization after ASO and low socioeconomic status were associated with suboptimal intermediate neurodevelopment outcomes after ASO.
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