Introduction: Double inlet left ventricle (DILV) accounts for 1% of congenital cardiac anomalies.(1) DILV with L-looped ventricles and anterior and leftward aorta is the most common type of univentricular connection with a left-sided subaortic hypoplastic RV and right-sided morphologic LV making up 63-74% of cases.(2-3) Approximately 1.5% of patients with congenital heart disease (CHD) are born with double outlet right ventricle (DORV).(4) Interrupted aortic arch (IAA) comprises 1.3 to 2.9% of CHD cases, with type A interruption distal to the left subclavian artery accounting for 30-37% of cases. Although rare, there is known association of IAA with DILV, DORV and TGA.(5-6)  It is exceedingly rare for these to occur together, and even more uncommon for an infant to present with this combination late in the setting of ductal dependent systemic blood flow. Without surgical palliation, patients rarely survive infancy. However, survival due to an anatomically protected pulmonary vascular bed with development of Eisenmenger syndrome has been described.(7)

Case Description: Presented here is a unique case of a 5-month-old male, born full-term in Kenya, requiring oxygen for the first week of life until discharge home. He returned to hospital 1 week later in the setting of emesis presumed secondary to infection, necessitating transfer to another hospital where a CT scan demonstrated CHD. Furosemide was started for diuresis. Given no available surgical option in his home country, his father moved him to the United States seeking surgical repair. On presentation, he weighed <1st percentile, tolerating formula feeds and enteral diuretics. Echocardiography confirmed his cardiac segmental anatomy of {S,L,L}, situs solitus, levocardia, DILV with superior-inferior L-looped ventricles, DORV with L-malposed great vessels arising primarily from the rudimentary left-sided RV and hypoplastic aortic valve, root, and ascending aorta with IAA Type A and patent ductal communication to the descending aorta. There was trivial AV valve regurgitation and normal LV systolic function. By catheterization, Qp:Qs was 2.4:1, with this measurement limited by the systemic saturation differential in the upper and lower extremity and an assumption of a normal pulmonary vein saturation. The LVEDP was 10 mmHg with a transpulmonary gradient of 35 mmHg and PVRi 4.02 WUi. He underwent a Norwood procedure with a 4-mm Blalock-Taussig-Thomas shunt, Damus-Kaye-Stansel, atrial septectomy, and IAA repair. Coronary artery flow profile by intraoperative epicardial echocardiography was not suggestive of obstruction. LV dysfunction necessitated VA-ECMO for postoperative cardiogenic shock. He was decannulated on postoperative day 2 and underwent delayed sternal closure on postoperative day 5. He remained in the CTICU for 14 days and discharged with good weight gain on oral diuresis and afterload reduction.

Conclusion: This is a rare case of late-presenting, unrepaired single ventricle CHD with IAA and systemic circulation provided by a PDA, until successful stage 1 surgical palliation was performed on another continent at 5 months of life. This constellation of lesions, combined with late presentation for repair, is rarely observed. This case highlights the variability of congenital cardiac care on an international level.

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