Neonatal enlargement of the aortic arch roof without cardiopulmonary bypass using ductal patency for lower body perfusion: impact on long-term growth, function and shape of the aortic arch†

Abstract

Objectives: This study evaluates the long-term outcome of neonatal aortic arch roof enlargement using ductal patency in the context of coarctation associated with aortic arch hypoplasia.

Methods: Retrospective single-centre analysis of children undergoing roof enlargement of the distal arch (left common carotid artery-left subclavian artery) without cardiopulmonary bypass (utilizing ductal patency for lower body perfusion); followed by resection and extended end-to-end anastomosis, through a left posterior thoracotomy. This study evaluates the long-term outcome with emphasis on arch growth and shape.

Results: Thirty consecutive patients were included (2006-24). Median age and weight were 6.0 [interquartile range: 4.0-7.8)] days and 3.1 (2.7-3.5) kg, respectively. Simple congenital heart disease with simple intracardiac shunts (n = 17) and complex congenital heart disease (Complete Atrioventricular Septal Defect (AVSD), interrupted aortic arch and univentricular hearts) (n = 13) constituted the cohort. Non-ischaemic clamp time for roof enlargement was 43 (36-50) min. Ischaemic clamp time for coarctation resection was 23 (21-25) min. Pulmonary artery banding was performed in 19 (63.3%) patients. Twenty-seven (90%) successfully underwent staged repair at 6.1 (4.5-8.2) months age. Follow-up was complete at a median duration of 46.9 (21.7-159.9) months. All patients survived the operation and are in good health at follow-up. Median ventilation time, ICU and hospital stay were 1 (1-2), 3 (2-5) and 23.5 (14-40) days, respectively. No patient developed any neurological complication. Three developed left subclavian artery thrombosis, one requiring surgical revision. With one unrelated late accidental death 14 years after neonatal repair, Kaplan-Meier survival was 90.9 [50.8-98.7]% at 15 years. Two patients underwent arch re-enlargement at the inner curvature to accommodate the DKS during stage 2, resulting in freedom from reoperation of 93.3[75.9-98.3]% at 10 years. All survivors enjoy subjective normal exercise tolerance with no relevant gradient. No patient is on anti-hypertensive medication. Median Z value of the proximal, transverse and distal arch was normalized to -0.88 (-2.19 to -0.12), -0.66(-1.33 to 0.08) and 0.34 (-0.10 to 1.33), respectively, at the last follow-up. Twenty-three (76.7%) arches achieved Romanesque shape at follow-up.

Conclusions: Long-term results of this minimally invasive approach show proportional growth without relevant gradient, freedom from hypertension and a Roman arch form, thus making it the preferred approach.

Keywords: Roman arch; aortic arch hypoplasia; aortic arch repair; coarctation; hypertension.

Graphical abstract

Figure 1:

(A) (Ia-Ib) CoA and severe arch hypoplasia. (IIa, IIb) Aortic arch enlargement while maintaining ductal perfusion to lower torso. (III) Reperfusion. (IV, V) Resection of coarctation and extended end-to-end anastomosis. (B) Cerebral and somatic intraoperative NIRS in 17 patients (since the introduction of NIRS) showing stable NIRS during the non-ischaemic aortic clamping and expected drop during ischaemic clamping

Figure 2:

(A) KM freedom from reoperation. (B) KM freedom from catheter reintervention

Figure 3:

(A) Preoperative echo picture of hypoplastic arch plus isthmus. (B) Remodelling of roof enlarged arch at follow-up