Aortic coarctation surgery

Aortic coarctation Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S.[2], Cafer Zorkun, M.D., Ph.D. [3]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S.[4]

Overview

The treatment choice depends on the patients age of presentation, severity, the location of the coarctation and other associated anomalies. For children who present early, the role of medical management is for stablizing the patient for surgery. However, in older kids and adolescent presenting with hypertension treatment is guided towards correction of hypertension and other associated anomalies. Therapy/treatment is conservative if asymptomatic, but may require surgical resection of the narrow segment if there is arterial hypertension. The first operations to treat coarctation were carried out by Clarence Crafoord in Sweden in 1944. In some cases angioplasty can be performed to dilate the narrowed artery. If the coarctation is left untreated, arterial hypertension may become permanent due to irreversible changes in some organs (such as the kidney).

Re-coarctation may be treated with either percutaneous balloon dilation or percutaneous stent placement.

Surgery

General Considerations for Surgery

• Indications for surgery: Significant hypertension, congestive heart failure (CHF)
• Techniques available - Surgical relief of obstruction, balloon angioplasty and stents
• Symptomatic neonates and infants should undergo urgent intervention soon after the infant is stabilized.
• Asymptomatic patients - Undergo elective procedure
• Elective surgical or balloon therapy in children aged 2-5 years is suggested.
• There is lack of consensus about the best age for surgery and large randomized clinical trials are required in this direction. The rationale behind 2-5 years is that it gives aorta time to grow and to be larger than half the anticipated adult size at operation. However, issues of hypertension, left ventricle hypertrophy, and left venticular dysfunction favors earlier repair.
• Delaying surgery beyond 5 years is not advisable as residual hypertension stays if intervention is performed after age 5 years.
• The current surgical strategies are to aggressively and completely repair the coarctation of aorta and all other associated lesions.
• The mortality risk is high in neonates (surgery performed within 1 week of life), with associated cardiac anomalies, and low weight babies. This may be due to the poor presentation these infants have compared to the older child.
• Pseudo-coarctation (abnormal tortuosity of the aorta) does not require surgery as the tortuosity doesn't result in significant obstruction or hypertension.

Surgical Techniques

• The choice of technique depends on the patient's age at presentation, size, associated abnormalities, and anatomy of the coarctation.
• Surgical approach - median sternotomy is preferred over left lateral thoracotomy, in complex arch repairs.
• Subclavian flap aortoplasty is found to be the most commonly performed followed by resection in end-to-end anastomosis, patch aortoplasty, and bypass graft when the surgery is done during infancy. Whereas, in children and adolescent undergoing coarctation repair end-to-end anastomosis is commonest followed patch aortoplasty and subclavian flap aortoplasty.
• Patch aortoplasty are less frequently used these days because of concerns regarding the development of aortic aneurysm at the site of surgery.
• Available Techniques:
  • Resection and end-to-end anastomosis
  • Patch aortoplasty
  • Left subclavian flap aortoplasty
  • Tubular bypass grafts
  • Combination techniques

Indications for Surgery

• There is a lack of consensus for the indications and the time for surgery. Some groups of surgeon consider balloon angioplasty and stenting as the initial approach and reserving surgery for more complicated conditions or treatment failures. Whereas, others consider surgical repair as the first line of therapy.
• Surgery is the first choice in aortic coarctations involving:
  • Long length of aorta
  • Severe coarctations causing almost complete or complete obliteration of the aortic lumen.
  • Associated with other cardiac defects for e.g. large patent ductus arteriosus, ventricular septal defect.

Surgical Complications

• Aneurysms (associated with all repairs but commonest with prosthetic patch angioplasty)
• Aortic dissection
• Paradoxical hypertension
• Paraplegia
• Recoarctation (commoner in infants compared to older children)
• Vascular complications (common with subclavian flap repair)

Follow Up

• In post operative coarctation evaluate patients for:
  • Recoarctation: Echo may underestimate this
    • Aneurysm may develop at site of repair, most commonly after patch repair
    • Periodic imaging is mandatory
    • 2D- Doppler will miss aneurysm

• Follow up in patients with coarctation repair:
  • Annual follow up with meticulous control of BP at rest and exercise.
  • Screening for coronary artery disease and optimize risk factors.
  • Multi-modality imaging of entire aorta for ascending and coarctation site.
  • Cranial screening can be offered to the patients.

Stenting

Primary Percutaneous Treatment of Native Coarctation

Balloon dilation can be used to treat unrepaired native coarctation, but it is generally avoided in the first 6 to 12 months of life due to potential vascular complications and a high rate of re-coarctation. As primary treatment, there is a high risk of aortic rupture compared to recoarctation in which case there is scar tissue.. Aortic aneurysm formation is another potential complication following balloon dilation or surgery. Despite the thickening in the area of coarctation, these patients have abnormally weak aortas and may have cystic medial necrosis in the areas surrounding the coarctation.

Percutaneous Treatment of Coarctation Following Initial Repair

Percuataneous balloon dilation is associated with lower morbidity than surgical repair and is viewed as the primary mode of treating re-coarctation. Although the risk of aortic rupture is lower than in primary balloon dilation of coarctation, there is a low but finite risk of balloon rupture.

Stent implantation has been advocated as a means to reduce the risk of aortic rupture, aortic dissection, recoil, and subsequent aortic aneurysm formation associated with balloon dilation. Complications include inability to fully dilated the stent and retroperitoneal bleeding. Stenting reduced the incidence of hypertension from 71% before the procedure to 26% following the procedure in one study. The incidence of late aortic aneurysm formation is not known.

2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines

Therapeutic Recommendations for Coarctation of the Aorta

Class I

1. Surgical repair or catheter-based stenting is recommended for adults with hypertension and significant native or recurrent coarctation of the aorta. (Level of Evidence: B-NR)

2. GDMT is recommended for treatment of hypertension in patients with coarctation of the aorta. (Level of Evidence: C-EO)

Class IIb

1. Balloon angioplasty for adults with native and recurrent coarctation of the aorta may be considered if stent placement is not feasible and surgical intervention is not an option. (Level of Evidence: B-NR)

2008 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)

Recommendations for Interventional and Surgical Treatment of Coarctation of the Aorta in Adults (DO NOT EDIT)

Class I

"1. Intervention for coarctation is recommended in the following circumstances:"

"a. Peak-to-peak coarctation gradient greater than or equal to 20 mm Hg. (Level of Evidence: C)"

"b. Peak-to-peak coarctation gradient less than 20 mm Hg in the presence of anatomic imaging evidence of significant coarctation with radiological evidence of significant collateral flow. (Level of Evidence: C)"

"2. Choice of percutaneous catheter intervention versus surgical repair of native discrete coarctation should be determined by consultation with a team of ACHD cardiologists, interventionalists, and surgeons at an ACHD center. (Level of Evidence: C)"

"3. Percutaneous catheter intervention is indicated for recurrent, discrete coarctation and a peak-to-peak gradient of at least 20 mm Hg. (Level of Evidence: B)"

"4. Surgeons with training and expertise in CHD should perform operations for previously repaired coarctation and the following indications:"

"a. Long recoarctation segment. (Level of Evidence: B)"

"b. Concomitant hypoplasia of the aortic arch. (Level of Evidence: B)"

Class IIb

"1. Stent placement for long-segment coarctation may be considered, but the usefulness is not well established, and the long-term efficacy and safety are unknown.(Level of Evidence: C) "

Recommendations for Key Issues to Evaluate and Follow-Up (DO NOT EDIT)

Class I

"1. Lifelong cardiology follow-up is recommended for all patients with aortic coarctation (repaired or not), including an evaluation by or consultation with a cardiologist with expertise in ACHD. (Level of Evidence: C)"

"2. Patients who have had surgical repair of coarctation at the aorta or percutaneous intervention for coarctation of the aorta should have at least yearly follow-up.(Level of Evidence: C)"

"3. Even if the coarctation repair appears to be satisfactory, late postoperative thoracic aortic imaging should be performed to assess for aortic dilatation or aneurysm formation. (Level of Evidence: B)"

"4. Patients should be observed closely for the appearance or reappearance of resting or exercise-induced systemic arterial hypertension, which should be treated aggressively after recoarctation is excluded. (Level of Evidence: B)"

"5. Evaluation of the coarctation repair site by MRI/CT should be performed at intervals of 5 years or less, depending on the specific anatomic findings before and after repair. (Level of Evidence: C) "

Class IIb

"1. Routine exercise testing may be performed at intervals determined by consultation with the regional ACHD center. (Level of Evidence: C) "