Clinical Practice Algorithm For the Follow-Up of Repaired Coarctation of the Aorta

Feb 16, 2023   |  Majd Makhoul, MD, FACC; Dor Markush, MD; Carissa Marrie Baker-Smith, MD, FACC; Jonathan Edelson, MD, FACC; Roni M. Jacobsen, MD, FACC; Ali N. Zaidi, MD, FACC; Christopher Statile, MD, FACC; Ritu Sachdeva, MBBS, FACC; Anitha Parthiban, MBBS, FACC

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Quick Takes

  • Older children with repaired coarctation of the aorta (CoA) with mild or no residual lesions may be followed less frequently (up to every 2 years) with echocardiography.
  • Cardiac computed tomography and cardiac magnetic resonance imaging are useful adjuncts to echocardiography in the serial evaluation of older children and adults with repaired CoA.
  • After CoA repair, patients should be monitored for systemic and masked hypertension.

Surgical or catheter-based intervention for coarctation of the aorta (CoA) generally results in excellent outcomes; however, residual structural and functional vascular abnormalities may remain.1 Secondary cardiovascular (CV) risk factors that play a critical role in the lifelong CV well-being of these patients may be under-recognized. Timely and comprehensive evaluation along with appropriate ancillary testing are essential to optimize care and management of these patients. However, evidence-based guidelines for the care of these patients are lacking and significant practice variation exists.

Goals and Details of the Algorithm

The goal of the congenital heart disease (CHD) Clinical Practice Algorithm for Repaired CoA is to provide an age-based decision-support tool that can be used in the follow-up and surveillance of patients with isolated repaired CoA throughout their lifespans. To allow for a standard evaluation and management approach, these recommendations are not intended for pregnant women or for those with unrepaired CoA, complex arch repair (bypass grafting), associated significant CHD, significant dilation of the aortic sinuses and/or ascending aorta, or significant aortic valve disease (moderate or greater aortic stenosis and/or regurgitation). These recommendations may be applicable to patients with chromosomal abnormalities and bicuspid aortic valves with no greater than mild stenosis or regurgitation.

Three age-based subalgorithms for CoA are included, incorporating the differing management considerations for patients in these groups (<1 year of age, 1-18 years of age, and ≥18 years of age). Each pathway provides a suggested guide to the frequency of clinic visits and type of testing, with younger age and greater-than-mild residual lesions meriting closer follow-up.2,3 Thresholds to prompt possible further evaluation or reintervention (upper- to lower-extremity blood pressure [BP] cuff gradient and/or arch echocardiographic mean systolic gradient ≥20 mm Hg) are based on available literature and expert consensus.4-7 The role of cross-sectional imaging as an adjunct to echocardiography is emphasized and best practice recommendations for appropriate use of these modalities are provided based on existing guidelines.4 This is particularly important in older children and adults, for whom echocardiographic images of the arch are not optimal because of difficult acoustic windows. Baseline cardiac computed tomography scanning or cardiac magnetic resonance imaging in the teenage years followed by surveillance every 3-5 years is incorporated into the 1-18 years of age and adult subalgorithms.

Patients with repaired CoA can continue to have or develop new hypertension even after early repair and with optimal surgical results.5 Hypertension can also be a sign of recoarctation; therefore, upper- and lower-extremity BPs should be obtained with each visit. Routine office BP monitoring may be insufficient because masked hypertension can occur in up to 45% of pediatric patients after repair of CoA;5 therefore, ambulatory BP monitoring every 1-2 years when available is reasonable. Exercise testing may also be useful in detecting abnormal BP response.6 The Quality Working Group of the American College of Cardiology (ACC) Adult Congenital and Pediatric Cardiology (ACPC) member section incorporated assessment and counseling for CV risk factors into the clinical algorithm based on current evidence for risk of vascular dysfunction in CoA.

By using this tool, a standard approach for frequency of follow-up and testing, including echocardiography and cross-sectional imaging, for patients with isolated repaired CoA from birth to adulthood may be achieved. The algorithm may be used as a reference but is not intended to replace clinical decision making in individual cases, especially in the presence of comorbidities that fall outside its scope.

Methods: Algorithm Development

The CHD Clinical Practice Algorithm for Repaired CoA was written by the Quality Working Group of the ACC ACPC member section. This is a multidisciplinary group of experienced pediatric and adult congenital cardiologists with diverse training and practice backgrounds. A subset of this group authored the first algorithm (for secundum atrial septal defects [ASDs]) of a series for mildly complex CHD.8 The same quality-driven approach used for the secundum ASD algorithm was used for the development of this CoA algorithm to translate current evidence and best practice recommendations into an easily used and practical clinical tool. The algorithm is evidence-based and existing guidelines were used when applicable.4,9-11 When differences were noted in guidelines (American Heart Association [AHA] vs. European Society of Cardiology [ESC]), a practical approach was adapted by the group. The CoA algorithm was peer reviewed for consensus.

Conclusions

Clinicians, practices, and centers may use this algorithm to create internal quality improvement initiatives. The hope is that a standardized approach for the appropriate patient will improve resource use and quality outcomes, as well as facilitate multicenter studies.

References

  1. McElhinney DB, Yang SG, Hogarty AN, et al. Recurrent arch obstruction after repair of isolated coarctation of the aorta in neonates and young infants: is low weight a risk factor? J Thorac Cardiovasc Surg 2001;122:883-90.
  2. Marx GR, Allen HD. Accuracy and pitfalls of Doppler evaluation of the pressure gradient in aortic coarctation. J Am Coll Cardiol 1986;7:1379-85.
  3. Aldousany AW, DiSessa TG, Alpert BS, Birnbaum SE, Willey ES. Significance of the Doppler-derived gradient across a residual aortic coarctation. Pediatr Cardiol 1990;11:8-14.
  4. Sachdeva R, Valente AM, Armstrong AK, et al. ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 appropriate use criteria for multimodality imaging during the follow-up care of patients with congenital heart disease: a report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography. J Am Coll Cardiol 2020;75:657-703.
  5. Flynn JT, Kaelber DC, Baker-Smith CM, et al.; Subcommittee on Screening and Management of High Blood Pressure in Children. Clinical practice guideline for screening and management of high blood pressure in children and adolescents. Pediatrics 2017;Aug 21:[Epub ahead of print].
  6. Luitingh TL, Lee MGY, Jones B, et al. A cross-sectional study of the prevalence of exercise-induced hypertension in childhood following repair of coarctation of the aorta. Heart Lung Circ 2019;28:792-9.
  7. Clarke MM, Zannino D, Stewart NP, et al. Normative blood pressure response to exercise stress testing in children and adolescents. Open Heart 2021;8:e001807.
  8. Plummer S, Parthiban A, Sachdeva R, Zaidi A, Statile C. Clinical Practice Algorithm for the Follow-up of Unrepaired and Repaired Secundum Atrial Septal Defects. http://www.acc.org. Mar 08, 2022. Accessed 01/06/22. https://www.acc.org/Latest-in-Cardiology/Articles/2022/03/08/19/34/Clinical-Practice-Algorithm-For-the-Follow-up-of-Unrepaired-and-Repaired-SASD.
  9. Stout KK, Daniels CJ, Aboulhosn JA, et al. 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. J Am Coll Cardiol 2019;73:e81-e192.
  10. Baumgartner H, De Backer J, Babu-Narayan SV, et al.; ESC Scientific Document Group. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J 2021;42:563-645.
  11. Feltes TF, Bacha E, Beekman RH 3rd, et al.; American Heart Association Congenital Cardiac Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Clinical Cardiology, Council on Cardiovascular Radiology and Intervention, American Heart Association. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation 2011;123:2607-52.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Noninvasive Imaging, Prevention, Valvular Heart Disease, Vascular Medicine, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Echocardiography/Ultrasound, Hypertension

Keywords: Aortic Coarctation, Masked Hypertension, Cardiologists, Pregnant Women, Dilatation, Longevity, Aorta, Thoracic, Follow-Up Studies, Bicuspid Aortic Valve Disease, Echocardiography, Algorithms, Ambulatory Care, Catheters


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