Arch Augmentation via Median Sternotomy for Coarctation of Aorta With Proximal Arch Hypoplasia

Coarctation of the aorta can be associated with hypoplasia of the proximal transverse aortic arch. One approach to manage this condition is via left thoracotomy and extended end-to-end anastomosis with the expectation that the proximal arch will grow over time. Our preferred approach is to augment the aorta via midline sternotomy. We hypothesized that this approach is safe, durable, and allows reliable growth of the aorta.

We identified the records of patients with biventricular anatomy who had coarctation of the aorta, hypoplasia of the proximal transverse arch, and no other cardiac lesion that would mandate cardiopulmonary bypass use and midline sternotomy. The records of 62 such patients operated on between 2005 and 2016 were retrospectively reviewed. Patient demographics, clinical variables and outcome data were collected and analyzed using SAS 9.4. Data are presented as median (interquartile range [IQR]).

Sixty-two patients (23 girls [37%]) underwent repair at 10 (IQR, 5 to 21) days of life. Forty-nine (79%) patients were on prostaglandin infusion to maintain ductal patency. Fifteen (24%) patients presented in shock with end organ dysfunction, 17 (27%) were on inotropes, and 26 (42%) were mechanically ventilated. The proximal transverse arch was 41% (IQR, 34% to 47%) of the size of ascending aorta as measured by echocardiography (z-score, -5 [IQR, -5.8 to -4.3]). Following median sternotomy, repair was carried out on cardiopulmonary bypass (41 [IQR, 37 to 47] minutes). The arch was reconstructed with (n = 26 [42%]) or without (n = 36 [58%]) coarctectomy usually using homograft patch aortoplasty (n = 58 [94%]). In all but 2 patients, repair was undertaken with circulatory arrest (27 [IQR, 22 to 31] minutes). Patients were extubated 4 (IQR, 3 to 5) days later and discharged home in 12 (IQR, 8 to 18) days. There was no mortality, and 8 morbidity events (3 recurrent nerve injury, 2 chylothorax, 1 phrenic nerve injury, 1 seizure, and 1 superficial wound infection) in 7 (11%) patients. All patients are alive at 41 (IQR, 11 to 64) months of follow-up. Reintervention was required in 6 (10%) patients (5 catheter based and 3 surgical) for recurrent distal coarctation. Reintervention-free survival at 1, 3, and 5 years was 87%. Only 1 child was currently on antihypertensive therapy, and all were in New York Heart Association functional class I symptoms. At last echocardiogram, the proximal transverse arch was 97% (IQR, 84% to 103%) of the diameter of the ascending aorta (z-score, 0.8 (IQR, 0.3 to 1.3]), ejection fraction was 70% (IQR, 60% to 76%), and only 2 patients had significant left ventricular hypertrophy.

Arch augmentation via median sternotomy is a safe and effective procedure that can be accomplished with low morbidity and mortality. The reconstructed arch retains excellent growth potential resulting in a very favorable physiologic outcome.