Video clip is available online.We report an infant with heterotaxy-dextrocardia who underwent heart transplantation using an atrial switch procedure.
CLINICAL SUMMARYThe institutional review board of the University of Iowa approved this report with a waiver of consent (IRB 202103110, March 11, 2021). The male patient was prenatally diagnosed with congenital heart disease. He was born at 34 weeks with a birth weight of 2364 g. A postnatal echocardiogram confirmed the diagnosis of left atrial isomerism, dextrocardia, common atrium, hypoplastic left ventricle with mitral and aortic atresia, hypoplastic aortic arch, ipsilateral pulmonary veins (PV), left-sided single superior vena cava (SVC), and interrupted left-sided inferior vena cava (IVC) with hemiazygos connection to the SVC. Both proximal coronary arteries were patent; however, systolic flow reversal was detected with coronary fistulas to the right ventricle. On day 2 of life, he developed persistent lactic acidosis with ST segment changes on electrocardiogram. An urgent bilateral pulmonary artery banding was performed to stabilize the patient on prostaglandin infusion. Cardiac catheterization and ductal stenting were planned on day 24 of life. He developed significant ST segment changes during the procedure and the intervention was aborted. Considering the atypical clinical course and From the Divisions of
ObjectiveSurgical algorithms for aortic root disease are established in adults. The root of children needs to have a potential for somatic growth, therefore a spectrum of techniques can be used to reconstruct the root. This study reviews a national experience in 50 consecutive paediatric patients requiring aortic root repair, and evaluates the efficacy of our treatment algorithm in this age group.MethodsAll aortic root procedures at our institution from 2001–2014 were reviewed. Follow-up was 100% complete. Patients undergoing aortic valve repair only (n = 7) or Ross/Konno procedures (n = 5) were excluded.ResultsThere were 27 male patients. Mean age at surgery was 14.5 years (range: 4 weeks-18 years); mean weight was 46.1 kg (range: 3.5–105 kg). The predominant pathology was mixed disease of aortic stenosis (AS) / aortic incompetence (AI) in 28/50, (AI) in 13/50 and (AS) in 1/50. Aortic root dilatation (6 Marfan’s + 2 Loeys-Dietz syndrome) was the dominant pathology in 8/50 patients. 30/50 patients underwent a Ross procedure; 12/50 patients underwent aortic root replacement (ARR) with aortic homograft/biocomposite graft; 8/50 patients underwent David valve-sparing aortic root replacement (VSARR). There was 1 perioperative death (2%) with a 5 year actuarial survival of 98%. Mean follow up was 3.96 years (range: 0.1–11.6 years). There were no late mortalities.Re-intervention was required in 4/50 (8%); 1 Ross patient required (VSARR) for autograft dilatation, 2 required redo (ARR) with a composite graft for acute homograft failure, and 1 (VSARR) patient required mechanical AVR for AI.ConclusionAortic root surgery in children can be accomplished with low rates of early-mid term re-intervention and mortality. Our experience confirms the applicability of adult treatment principles, and highlights the need for versatility in surgical technique in children.
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