eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiothoracic Surgery

Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment: Follow-up

Author: Theodore C Koutlas, MD, Assistant Professor, Department of Surgery, Division of Cardiothoracic Surgery, Pitt County Memorial Hospital
Coauthor(s): David M Maziarz, MD, Fellow, Department of Surgery, Wake Forest University Baptist Medical Center; Clifton C Reade, MD, Fellow, Department of Cardiothoracic Surgery, University of Pennsylvania; Katie Love, MD, Resident Physician, Department of Surgery, Pitt County Memorial Hospital, Brody School of Medicine at East Carolina University
Contributor Information and Disclosures

Updated: Jul 21, 2009

Outcome and Prognosis

Outcomes from coarctation repair depend on several factors, most notably the patient's age at the time of operation, the method of repair, and the presence of associated anomalies.

The recoarctation rate with the end-to-end repair has historically approached 60%, with somewhat lower rates for subclavian-flap aortoplasty and prosthetic graft repair. However, recent studies have demonstrated that end-to-end repair favorably compares with other techniques, with recoarctation rates of only 5-10%.6

Some suggested reasons for this dramatic change are improved neonatal ICUs and advances in vascular surgical technique and suture materials. Improved preoperative conditioning and increased use of prostaglandins have also contributed to lowered mortality rates. Circumferential suture lines were initially blamed for high rates of recoarctation; however, because this technique was perfected with the arterial switch operation, they should likely prove effective for coarctation as well. At this time, the current authors know of no prospective randomized trials that are being conducted to compare the repair techniques.

Overall mortality rates are still 2-10% in neonates, which includes those with intracardiac anomalies as well as those with coarctation.

A 1998 retrospective study of 176 patients by Seirafi and associates demonstrated an overall mortality rate of 7.4%.21 Nine of 13 deaths were in patients with associated complex intracardiac anomalies. No mortality occurred in the 113 patients with isolated coarctation. A 15% incidence of residual or recurrent coarctation was reported, although neither the patient's age at operation nor the type of repair appeared to influence recurrence. The other major complication was persistent hypertension, which was associated with repair in patients older than 1 year (27% vs 4.2%).

In 1998, Backer and associates reported a series of 55 infants who underwent repair by using extended resection with end-to-end reanastomosis.22 They reported a 5.4% mortality rate and a 3.6% rate of recurrence. The median age at surgery was 21 days. A total of 62% of surgeries performed through a left thoracotomy and the others through a median sternotomy with concurrent repair of associated anomalies. About 47% of patients had isolated coarctation, and 53% had at least 1 associated intracardiac anomaly, with ventricular septal defect (VSD) comprising 69% of those anomalies. Total resection of all ductal tissue was achieved.

In 2000, Allen and associates described a modified subclavian patch aortoplasty.23 They demonstrated excellent results in 53 infants with no mortality and only a 4% incidence of clinically significant recoarctation. Of these instances, 49% were associated with cardiac anomalies. As with all techniques, the key features to this technique are a tension-free repair and a long aortotomy to help prevent restenosis.

Despite some excellent outcomes, controversy remains over the use of flap aortoplasty in infants younger than 3 months. Potential pitfalls are associated with early use of this technique, most notably concern over the loss of the major vascular supply to the left upper extremity. Complications are rare (the left subclavian was historically divided for pulmonary systemic shunts, with complications occurring rarely); however, decreased growth and rare reports of ischemia and gangrene have been documented.24 In addition, neonates most often have associated arch hypoplasia, which is better addressed with resection and end-to-end anastomosis than with other methods. Complete hypoplasia of the aortic arch is best addressed by means of arch reconstruction performed through a median sternotomy.

The prosthetic-path onlay technique has the advantages of a relatively short operating time, minimal dissection, maximal augmentation of the stenosed area, and maintenance of normal vascular anatomy. However, this technique has been associated with an aneurysmal-development rate of as much as 35%, and repeat operation is required in almost 20% of patients.18

Future and Controversies

Balloon angioplasty has been performed for more than 20 years; however, its use in coarctation remains controversial because of the formation of pseudoaneurysms and concerns about long-term patency. The surgical literature shows a high restenosis rate for neonatal (<40 d) balloon angioplasty; 57% need surgical intervention. However, in the population with surgically treated restenosis (18%), secondary angioplasty is the treatment of choice, and no repeat interventions were necessary.25

A long-term randomized study of angioplasty versus surgery in children (3-10 y) showed that only 50% of patients treated with balloon angioplasty remained free of both aneurysmal formation and repeat intervention compared with 87.5% of surgically treated subjects.26

At present, the optimal timing and mode of treatment for patients with coarctation remains controversial. In neonates and young children, surgical intervention remains the criterion standard. It is well-tolerated, with excellent long-term efficacy due to an 88-96% 5 years freedom from reintervention. Balloon angioplasty carries a higher risk of complication and suboptimal outcome in as many as 19% of native coarctation cases. Stents overcome some of the problems inherent to simple dilation but are not without serious complications related to materials and biomechanics.

No evidence suggests superiority of an endovascular therapy over surgery for treatment of primary coarctation.27 However, for patients with hemodynamic instability or for those who have undergone surgery for restenosis, angioplasty can be a palliative and potentially curative procedure. Al-Ata et al studied a series of infants with complex aortic coarctations and relative contraindications to surgery who underwent palliative stent implantation as a nondefinitive procedure.28 Three of the four improved and eventually underwent definitive repair. 

Stent placement in infants has also been studied, although (to the authors' knowledge) no long-term results have been reported. A report published in 2000 by Thanopoulos et al suggests that stent implantation can be performed with low morbidity and mortality rates and that no recoarctation was noted in any of the 17 patients who were examined at the age of 33 months.4 Peak systolic gradients were reduced to just 2.1 mm Hg from 50 mm Hg. A report of retrospective study by Marshall et al in 2000 suggested that, although stent implantation was a feasible option for challenging cases, it was not without a risk of serious complications.29

Stent placement is still under investigation in adolescents and adults, and long-term data is still being released. A nonrandomized selection-biased case series describes initial and 5-year outcomes for stent placement to treat adult aortic coarctation. Complications included a single incidence of stent migration requiring percutaneous intervention and a femoral pseudoaneurysm requiring surgical correction. Complete relief of coarctation was achieved with no recoarctation.30 A retrospective review of endovascular stent placement to treat thoracic aortic aneurysm following repair of aortic coarctation in a select few patients revealed promising, yet nondefinitive results.31

The future of aortic coarctation repair may include direct genetic intervention or in utero therapy, which may decrease the growth of ductal tissue into the main aorta or which may help alter neonatal blood flow in such a way to prevent coarctation completely. Until that time, surgical techniques will continue to improve, and the use of angioplasty should continue as studies continue to find the subset of patients in whom it will prove most useful.

 


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Workup: Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment
Treatment: Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment
Follow-up: Coarctation of the Aorta and Interrupted Aortic Arch, Surgical Treatment
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References
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References

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Further Reading

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Keywords

coarctation of the aorta and interrupted aortic arch, CoA, coarctation tubular hypoplasia, IAA, interrupted aortic arch, infantile coarctation, adult coarctation, preductal coarctation, postductal coarctation, juxtaductal coarctation, recoarctation, tubular hypoplasia, pseudocoarctation, DiGeorge syndrome, DiGeorge's syndrome, ventricular septal defect, VSD, Turner syndrome, Turner's syndrome, aortopulmonary window, left ventricular outflow tract obstruction, double-outlet right ventricle, truncus arteriosus, bicuspid aortic valve, hypertension, treatment, diagnosis

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Contributor Information and Disclosures

Author

Theodore C Koutlas, MD, Assistant Professor, Department of Surgery, Division of Cardiothoracic Surgery, Pitt County Memorial Hospital
Theodore C Koutlas, MD is a member of the following medical societies: American College of Surgeons, Society of Thoracic Surgeons, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Coauthor(s)

David M Maziarz, MD, Fellow, Department of Surgery, Wake Forest University Baptist Medical Center
David M Maziarz, MD is a member of the following medical societies: American College of Surgeons
Disclosure: Nothing to disclose.

Clifton C Reade, MD, Fellow, Department of Cardiothoracic Surgery, University of Pennsylvania
Clifton C Reade, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, Society of Thoracic Surgeons, and Southeastern Surgical Congress
Disclosure: Nothing to disclose.

Katie Love, MD, Resident Physician, Department of Surgery, Pitt County Memorial Hospital, Brody School of Medicine at East Carolina University
Katie Love, MD is a member of the following medical societies: American College of Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Daniel S Schwartz, MD, FACS, Assistant Clinical Professor of Cardiothoracic Surgery, Mount Sinai School of Medicine; Chief of Thoracic Surgery, Huntington Hospital
Daniel S Schwartz, MD, FACS is a member of the following medical societies: American College of Chest Physicians, American College of Surgeons, Society of Thoracic Surgeons, and Western Thoracic Surgical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons, and Southern Surgical Association
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting

Chief Editor

John Kupferschmid, MD, Director of Congenital Heart Surgery, Department of Surgery, Methodist Children's Hospital at San Antonio
John Kupferschmid, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, Society of Thoracic Surgeons, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

 
 
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