Complex heart disease with interrupted aortic arch: Echocardiography diagnosis with successful single-stage repair in a low-resource setting

Abstract

Background: Congenital heart disease (CHD) is the most common of all birth defects. Interrupted aortic arch (IAA) is rare, attributing to 1% of all CHD and exhibits left sidedness in most cases. It usually presents in the first 2 weeks of life with heart failure and shock. Early recognition, preoperative stabilization with prostaglandin infusion, and timely surgery are necessary to prevent mortality. Single-stage repair has proven to have good outcomes. However, resource constraints in low- and middle-income countries can hinder diagnosis and intervention by single-stage repair due to a paucity of state of the art centers and inventory. Case Description: We present the experience of a low-resource center with two children with rare complex forms of IAA, both of whom underwent successful single-stage cardiac surgeries; a 6-week-old infant was admitted with difficulty in feeding and heart failure signs. Moreover, a 2-year-old boy was referred with failure to thrive, chronic respiratory problems, and feeding problems since early infancy. Both children were diagnosed with IAA with associated defects including patent ductus arteriosus by transthoracic echocardiography. Management: After stabilization with anticongestive medication, single-stage repair was attempted in both. The postoperative course was uneventful, and the postsurgical echocardiogram showed no residual defect. Both cases were discharged. They are under follow-up, are symptom free, have gained weight, and have not shown any need for reintervention yet. Conclusion: If preparative diagnosis is made correctly and surgery planned meticulously, the immediate and short-term outcome of single-stage repair can be favorable.

Keywords

• aortic arch syndromes
• echocardiography
• heart disease
• infant