Asian Cardiovasc Thorac Ann 2002;10:310-313
© 2002 Asia Publishing EXchange Pte Ltd
Coarctation of the Aorta in Neonates And Young Infants: Surgical Experience
Narasinga Rao Pantula, MCh,
Raghavan Nair Suresh Kumar, DM1,
Anil Kumar Dharmapuram, MCh,
Mohmoud Hassan Mohamed, MCh,
Sushil Chandran, MCh,
Achal Kumar Dhir, FRCA2,
Dileep Kumar Saxena, MD2,
Sivan Pillay Azhagappan, MD2,
Velayudhan Ramakrishna Pillai, MD1,
Venkitachalam Chokkanathapuram Gopalakrishnan, MD1,
Mohamed Amin Fikree, FRCP1,
Yousuph Abdul Nazer, MCh,
Timothy Boyd Cartmill, FRACS,
Mrutyunjaya Rao Ivatury, MCh
Department of Cardiac Surgery
1 Department of Cardiology
2 Department of Cardiac Anaesthesia Al Mafraq Hospital Abu Dhabi UAE
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For reprint information contact: Ivatury Mrutyunjaya Rao, MCh Tel: 971 2 582 3100 Fax: 971 2 582 1549 email: imrao{at}emirates.net.ae Department of Cardiac Surgery, Al Mafraq Hospital, P.O. Box 2951, Abu Dhabi, UAE.
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ABSTRACT
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A retrospective analysis of repair of aortic coarctation in young infants was conducted. Between April 1997 and December 2000, 21 patients under 4 months of age underwent repair of coarctation. Their mean age and weight were 41 ± 42 days (range, 2 to 120 days) and 3.6 ± 0.7 kg (range, 2.6 to 4.9 kg). The indications for surgery were congestive heart failure and/or shock. Diagnosis was made by 2-dimensional echocardiography with Doppler color flow imaging. Preoperative gradients ranged from 25 to 100 mm Hg. Aortic arch hypoplasia was present in 8 patients; 7 patients also had ventricular septal defect. Wide excision of the coarctation segment with extended end-to-end anastomosis was performed in 20 patients, while 1 required a Gore-Tex interposition graft between the left common carotid artery and the descending aorta. Subclavian angioplasty was performed to augment the anastomosis in 1 patient. There was no early mortality. One patient died 2 months after surgery. Follow-up examination revealed recoarctation in 5 patients (23.8%), all of whom underwent successful balloon dilatation. In conclusion, wider excision of the coarctation with extended end-to-end anastomosis reduces the chances of recoarctation. Percutaneous balloon angioplasty for treating recoarctation is effective in immediately reducing pressure gradients.
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INTRODUCTION
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The surgical repair of neonatal coarctation has evolved considerably since it was first performed in the 1950s. However, considerable debate continues on the ideal timing of operation, optimal technique of reconstructing the arch, and whether associated intracardiac defects should be repaired in the same operation. Irrespective of the technique used, operative repair in the first year of life, especially in the neonatal period, carries a significant risk of restenosis.1 Fortunately, the majority of recoarctations can be dilated using modern techniques of balloon angioplasty with no added morbidity. We report our experience with coarctation below the age of 4 months.
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PATIENTS AND METHODS
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In this study, the aortic arch is defined as the portion lying between the brachiocephalic artery and the ductus arteriosus, and it comprises the transverse arch and the isthmus. The transverse arch lies between the brachiocephalic artery and the left subclavian artery, and the isthmus continues from there to the ductus (or ligamentum) arteriosus. The aortic arch was considered hypoplastic when a 5-mm segment measured less than 50% of the diameter of the descending aorta above the level of the diaphragm.2 Any heart defect having a surgical indication other than a ventricular septal defect (VSD) is considered a complex intracardiac lesion. Recoarctation is defined as any postoperative stenosis in the repair area giving rise to a resting peak pressure gradient above 20 mm Hg.1 It is considered as residual coarctation when the gradient was detected during postoperative hospital stay or as recurrent coarctation when the gradient was detected after hospital discharge. Early mortality is death that occurred within the first 30 postoperative days or before hospital discharge.
Between April 1997 and December 2000, a total of 21 patients below 4 months of age underwent surgical repair of aortic coarctation either as an isolated procedure or in association with pulmonary artery (PA) banding for VSD. Cases where coarctation repair was part of the surgical management of complex major intracardiac anomalies were excluded. There were 10 males and 11 females. Their mean age and weight were 41 ± 42 days (range, 2 to 120 days) and 3.6 ± 0.7 kg (range, 2.6 to 4.9 kg), with 17 patients under 1 month old. Clinical features (Table 1
) included feeble or absent femoral pulses, congestive heart failure, and basal ejection systolic murmur. Eight infants presented with shock, acidosis, and renal failure. Preoperatively, 7 infants were ventilated, 8 required inotropic support, and 10 had prostaglandin E1 (PGE1) infusion.
A neonatologist or pediatrician conducted a thorough physical examination with special attention to any dysmorphic features. Four neonates had dysmorphic features, which included 2 with Turner’s syndrome; 1 with ambiguous genitalia, imperforate anus, microphthalmia, and hydrocephalus; and another with dextrocardia and hypoplastic lung. Noninvasive measurement of arterial pressure was made in all limbs using the oscillometric method. Diagnosis was made clinically and confirmed by 2-dimensional echocardiography with Doppler color flow imaging. Pressure gradients were estimated using the modified Bernoulli formula, and they ranged from 25 to 100 mm Hg (mean, 49 ± 16 mm Hg). The cardiologist at the time of the diagnosis and the surgeon at the time of operation carefully evaluated the anatomic features of the aortic arch. Aortic arch hypoplasia was present in 8 patients. Associated cardiac defects included bicuspid aortic valve in 1 and VSD in 7 patients.
Through a left posterolateral thoracotomy via the 3rd intercostal space in neonates or the 4th intercostal space in older patients, the aorta was mobilized up to the origin of the innominate artery and down to the distal aorta. The ductus arteriosus was divided between 2 ligatures. The coarctation segment was widely resected (Figure 1A), an incision on the concavity of the arch was extended from the point of transection distally to beyond the origin of the left common carotid artery proximally (Figure 1B), and an oblique end-to-end anastomosis (Figure 1C) was made using a continuous 7/0 polypropylene suture.
During the postoperative period, resting blood pressure was measured invasively using arterial monitoring lines in the right arm and leg, and the gradients were recorded. Two-dimensional echocardiography with color Doppler was performed immediately after operation, at discharge, 3 monthly during the first year of follow-up, and at least yearly thereafter.
Continuous data are presented as mean ± standard deviation.
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RESULTS
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Primary resection of the coarctation with extended end-to-end anastomosis was performed in 20 patients, while 1 patient with severe hypoplasia of the entire transverse arch required a 6-mm Gore-Tex interposition graft (WL Gore & Associates, Flagstaff, AZ, USA) between the left common carotid artery and the descending aorta. Subclavian angioplasty was also required to augment the aortic arch in 1 patient.
There were no operative deaths or major complications related to the surgical procedure. The infant who had an interposition graft along with PA banding died 2 months following hospital discharge. The cause of death was not known.
The patients were followed up for a mean of 976 ± 466 days (range, 270 to 1,700 days). Five patients (23.8%) developed significant Doppler gradients ranging from 49 to 60 mm Hg (mean, 54.2 ± 4.2 mm Hg) at a mean follow-up period of 452 ± 193 days (range, 275 to 820 days). All 5 underwent balloon dilatation successfully and were in New York Heart Association functional class I.
Of the 7 patients with associated VSD, 3 had large defects and required concomitant PA banding to prevent pulmonary congestion. Another patient required VSD closure during the same hospital stay because of persistent congestive heart failure after coarctation repair. One patient underwent elective transatrial VSD closure 1.5 years later. The remaining 2 patients had small muscular VSDs, which were shown on follow-up echocardiograms becoming progressively smaller, obviating the need for any intervention. The patients who underwent surgical closure of VSD had no morbidity related to the procedure.
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DISCUSSION
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The outcome of neonatal coarctation repair has improved significantly since it was first performed as a result of early noninvasive diagnosis and aggressive preoperative stabilization of sick neonates with ventilation, inotropic support, and PGE1 infusion.1,3,4 Furthermore, advances in surgical and anesthetic techniques and intensive care management have lowered mortality. However, controversy continues as to the ideal way of management. Surgical therapy remains the treatment of choice for native coarctation of the aorta.5 Commonly performed surgical techniques include resection with end-to-end anastomosis,6,7 subclavian flap angioplasty,8 extended end-to-end anastomosis,9–12 and prosthetic patch angioplasty.13
Irrespective of the surgical procedure, recoarctation is more likely in neonates and small infants.14 Some degree of arch hypoplasia is often present in neonates with aortic coarctation.9–12 Early diagnosis and institution of PGE1 therapy may have contributed to more children with coarctation and severe arch hypoplasia making it to surgery, who in former years would not have survived that long.11 Recoarctation was mainly attributed to failure of the anastomotic line to grow or to inadequate resection of ductal tissue.3,4,8,15
Recoarctation rates of 10% to 47% in this age group have been documented, varying with the type of surgical repair performed.6–8,11,14,16 Patch angioplasty reportedly has the highest rates of recurrence, particularly when performed in infancy.16,17 It is also associated with late aneurysm formation.18 Pathological studies have shown that the ductal tissue in patients with coarctation extends much further into the aortic wall than normal, both circumferentially and longitudinally.15 Recoarctation is more likely in neonates and infants following subclavian angioplasty in which ductal tissue is retained.7,8 Moreover, left upper limb ischemia7 and subclavian steal remain potential complications in these patients. However, subclavian angioplasty may still have a place in a subset of patients with particular anatomical arrangements.
Wider resection with extended end-to-end anastomosis usually provides an adequate neonatal coarctation repair.9–12 Resection of the entire coarctation segment along with removal of all the ductal tissue and extending the incision to the concavity of the arch beyond the origin of the left common carotid artery will make recurrence less likely.11
In our series, 5 patients developed recurrent coarctation at a mean follow-up period of 15 months. All of them had been operated in the neonatal period. Analysis of these 5 patients revealed that 3 had significant transverse arch hypoplasia, with one of them requiring additional subclavian angioplasty to augment the arch and another having a residual gradient of 30 mm Hg early following coarctation repair. Both the remaining 2 patients with normal arch anatomy had residual gradients. In one of them, technical difficulties during surgery necessitated revision of the anastomosis, leaving a mild residual gradient. We consider the 3 cases with residual gradients as imperfect technical repairs leading to residual coarctation. The other 2 had good surgical repairs with no residual gradients at the time of discharge. They were regarded as having recurrent coarctation, presumably due to inadequate growth of the anastomotic site. All 5 cases of recoarctation were treated successfully by balloon dilatation.
Percutaneous balloon angioplasty for treating recoarctation is effective in immediately reducing pressure gradients, with good long-term results in most cases. Long-term results are less impressive in the presence of transverse arch hypoplasia.2 However, it may be safely said that the availability of this treatment even for small babies reduces the concern of leaving mild residual gradient at initial surgery. In fact, we propose that sequential surgery and balloon dilatation might be considered as planned therapy for some patients.
In conclusion, early referral, establishing diagnosis with careful details of arch anatomy and additional intracardiac defects, aggressive preoperative stabilization when needed with PGE1 infusion, inotropes, and ventilatory support have been major factors in reducing mortality. Adequate surgical repair with wider excision of the coarctation segment along with removal of all visible ductal tissue, the incision extended to the concavity of the arch beyond the origin of the left common carotid artery, and an oblique end-to-end anastomosis reduces the chances of recoarctation. Recurrence can almost always be successfully addressed by balloon angioplasty with no added morbidity, and this would be the preferred next line of management in these patients, with secondary surgery reserved for selected patients nearer to maturity of growth.
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ABSTRACT
INTRODUCTION
