Fellow Cleveland Clinic Children's Cleveland Clinic Children's Hospital Cleveland , Ohio, United States
Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare anomaly with time varying age at diagnosis, depending on the presence or absence of associated lesions. Depending on the time of diagnosis and associated lesions, therapies are directed either towards anatomic repair or physiologic repair with the right ventricle remaining as the systemic ventricle. In the absence of associated lesions some are managed expectantly. Cardiac MRI (CMR) is the gold standard for assessment of ventricular and valvar function. We sought to assess the systemic ventricular function and AV valve regurgitation in these different groups by CMR in a large cohort of patients followed at our institution.
Objective: To assess degree of systemic ventricle function and systemic AV valve regurgitation (SAVVR) in a large cohort of patients, with the hypothesis that patients undergoing anatomic repair would have the least amount of SAVVR at last follow up
Design/Methods: A retrospective single center chart review of patients (pts) with ccTGA was performed. Pts without CMR and pts who underwent single ventricle palliation and heart transplant were excluded from the study.
Results: Of 240 patients with ccTGA, 38 patients had CMR studies available for review. 20 patients had been followed medically while 8 had undergone anatomic repair with double switch procedure, and 10 patients had physiologic repair. Mild systemic ventricular dysfunction was seen in all 3 groups of patients, being most common in the physiologic repair group (60%). Higher degree of systemic AV valve regurgitation (SAVVR) was seen in the physiologic repair group with 70% of patients having ≥ moderate degree of SAVVR, compared to 55% of patients in the medical group. Greater degree of systemic ventricular dilation was seen in the physiologic repair and medical groups (40% in each subgroup having ≥ moderate dilation). Conclusion(s): Patients in both the medical and physiologic repair groups had higher prevalence of SAVVR and higher degree of systemic ventricular dilation. Systemic ventricular dysfunction was more common in the physiologic repair group.
Authors/Institutions: Salima Bhimani, Cleveland Clinic Children's Hospital, Cleveland, Ohio, United States; Neha Chellu, Cleveland Clinic Children's Hospital, Beachwood, Ohio, United States; Moses A. Anabila, Cleveland Clinic, Cleveland, Ohio, United States; Tara Karamlou, Cleveland Clinic Children's Hospital, Beachwood, Ohio, United States; Rukmini Komarlu, Cleveland Clinic Children's Hospital, Cleveland, Ohio, United States