Introduction
Primary intracardiac repair during the neonatal or early infantile period is generally the treatment of choice for coarctation of the aorta with ventricular septal defect. However, in low-birth-weight or premature infants, alternative treatment strategies, including staged repair, are necessary due to the high risks associated with cardiopulmonary bypass.
Herein, we report a case of staged repair for coarctation of the aorta with a ventricular septal defect, using stent angioplasty followed by surgical repair, in an extremely low-birth-weight premature infant.
Case
A female infant, born at 32 weeks and 1 day of gestational age through caesarean section as the second child of a monochorionic diamniotic twin pregnancy and weighing 1060 g, was diagnosed with coarctation of the aorta, ventricular septal defect, and patent ductus arteriosus. She was transferred to our institute while receiving intravenous prostaglandin E1. Pulmonary overcirculation progressed, with lower extremity perfusion dependent on the patent ductus arteriosus. On day 3 (body weight: 1020 g), stent angioplasty for coarctation of the aorta was performed via puncture of the right common carotid artery using an Ultimaster Tansei® 4.0*9.0 mm (Terumo, Tokyo, Japan) (Figure S1). On day 5, pulmonary artery banding and patent ductus arteriosus ligation were performed to address the pulmonary overcirculation. As she grew, the peak velocity at the stent site increased, accompanied by signs of cardiac failure. Therefore, on day 35 (body weight: 1294 g), balloon dilation of the stent was performed. During inflation, the balloon (5.5 mm in diameter) accidentally ruptured. The peak velocity decreased from 2.7 to 2.0 m/s. At the age of 5 months, with a body weight of approximately 3 kg, intracardiac repair was performed. Enhanced CT revealed that the stent had shortened to 7.2 mm in length due to balloon dilation (Figure 1a).
Following a standard median full sternotomy, cardiopulmonary bypass was established via cannulation of the brachiocephalic artery through a graft and the superior and inferior caval veins. The banding tape around the pulmonary artery trunk was removed, and the patent ductus arteriosus was divided. The aorta was divided just proximal to the stent. After clamping, the descending aorta distal to the stent, which was completely covered with neointima, was excised along with the aortic wall (Figure 2a). Extended end-to-end anastomosis without patch material was successfully performed following sufficient dissection of the aorta (Figure 2b). The ventricular septal defect was closed via a right atriotomy using an extended polytetrafluoroethylene patch. Weaning from cardiopulmonary bypass was smooth. The cardiopulmonary bypass time, aortic cross-clamp time, and lower body circulatory arrest time were 188, 82, and 30 minutes, respectively. Her postoperative course was uneventful except for the left pulmonary artery stenosis due to haematoma-induced compression and hoarseness. She was transferred back to the referring hospital on postoperative day 29 and was discharged shortly thereafter. Postoperative CT revealed no residual coarctation (Figure 1b). One year postoperatively, she remains well, with no symptoms or signs of recoarctation.
Posterior view of 3D computed tomography. ( a ) Preoperative image. The white arrow indicates the stent. ( b ) Postoperative image. No residual coarctation was observed.
Intraoperative views. ( a ) The stent was completely covered with neointima. The white arrow indicates the stent. ( b ) Extended end-to-end anastomosis of the aortic arch.
Discussion
Residual or recurrent coarctation must be avoided during repair of coarctation of the aorta. Therefore, we perform the repair by marking an incision along the lesser curvature of the aortic arch, extending towards the opposite side of the brachiocephalic artery, via a median full sternotomy with cardiopulmonary bypass, to ensure adequate reconstruction of the aortic arch. However, in low-birth-weight or premature infants, cardiopulmonary bypass is challenging because of its associated risks. In such patients, alternative treatment strategies to primary intracardiac repair should be considered. Although end-to-end anastomosis or subclavian flap repair combined with main pulmonary artery banding via left thoracotomy may be treatment options, we do not employ these techniques for coarctation repair, as noted above.
Bilateral pulmonary artery banding with continuous prostaglandin E1 infusion may be considered as an alternative technique. However, adjusting the banding can be challenging in such low-birth-weight infants. Moreover, intestinal blood flow may become unstable due to bidirectional or diminished diastolic flow through the patent ductus arteriosus. Considering these disadvantages, we opted for stent angioplasty for coarctation. Reference Radtke, Waller, Hebra and Bradley1
We treated four cases with stenting for coarctation followed by surgical repair, three of which (excluding the present case) involved simple coarctation. During surgery, patch augmentation or graft interposition was required for aortic arch reconstruction. In contrast, in our patient with ventricular septal defect, direct anastomosis was achievable. This was likely because post-stenotic dilatation of the pulmonary artery widened the subaortic space and positioned the stent relatively higher (Figure S2). Grieshaber et al. reported 26 cases of stent angioplasty for coarctation, representing the largest study in neonates to date. In their report, the use of patch material for aortic repair was more frequent in the stented coarctation group than in the native coarctation group, Reference Grieshaber, Merbecks and Jaschinski2 consistent with our cases. Interestingly, in the stented coarctation group, the freedom from re-intervention rate was higher when patch material was not used. The age at intracardiac repair tends to be older in staged repair than in primary repair, which may influence the re-intervention-free rate.
Gorenflo et al. reported 11 cases of surgical correction following stent angioplasty for coarctation. Reference Gorenflo, Boshoff and Heying3 In that report, a 1.5 kg infant with coarctation and a ventricular septal defect underwent surgical repair after stenting and, to the best of our knowledge, represented the smallest reported surgical case involving coarctation complex after stenting. In comparison, our patient weighed 1020 g at the time of stent angioplasty, making this the smallest reported surgical case to date.
In conclusion, we successfully performed a staged repair for coarctation with a ventricular septal defect using stent angioplasty in an extremely low-birth-weight infant. Direct anastomosis was achieved during surgical repair. Our staged approach appears to be a feasible treatment strategy for low-birth-weight or premature infants.
Supplementary material
The supplementary material for this article can be found at https://doi.org/10.1017/S104795112510961X.
Acknowledgements
None.
Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Competing of interest
None.
Ethical standards
The authors assert that all procedures contributing to this work comply with the ethical standards of the relevant national guidelines on human experimentation and with the Helsinki Declaration of 1975, as revised in 2008, and has been approved by the institutional committees (Mt. Fuji Shizuoka Children’s Hospital).
