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2 results

  • Comparison of Doty and McGoon techniques for surgical reconstruction of congenital supravalvular aortic stenosis

  • Part of
  • Mehmet Biçer, Mehmet Dedemoğlu, Oktay Korun, Hüsnü F. Altın, Okan Yurdakök, Murat Çiçek, Fatih Özdemir, Arif Selçuk, Yasemin Altuntaş, Ilker K. Yücel, Ahmet Şaşmazel, Numan A. Aydemir
  • Journal:
    Cardiology in the Young / Volume 32 / Issue 9 / September 2022
    Published online by Cambridge University Press:
    08 November 2021, pp. 1483-1490
  • Background:

    We aimed to compare the results of two surgical methods for the treatment of congenital supravalvular aortic stenosis.

    Methods:

    From May 2004 to January 2020, 29 patients underwent surgical repair for supravalvular aportic stenosis in a single centre. The perioperative evaluation of the patients was retrospectively reviewed.

    Results:

    Fifteen (51.7%) and 14 (48.2%) patients were treated with the Doty and the McGoon methods, respectively. The median age of our cohort was 4.5 (3.0–9.9) years. Ten (34.5%) patients had Williams–Beuren syndrome, and pulmonary stenosis was observed in 12 (41.3%) patients. The median follow-up time was 2.5 (0.7–7.3) years. On follow-up, five patients had residual stenosis with the McGoon technique and one with the Doty technique (p = 0.05). One patient died early in the post-operative period in the Doty group, and three patients were re-operated on due to restenosis in the McGoon group. Freedom from re-operation in the Doty group at 1, 3, 5, and 10 years was 100%. In the McGoon group, freedom from re-operation rates at the 1-, 3-, and 7-year follow-up were 100, 88.9, and 44.4%, respectively (p = 0.08).

    Conclusion:

    Our results with both surgical techniques suggest that supravalvular aortic stenosis can be treated with good results. The Doty method provided better relief for the supravalvular aortic segment, considering the residual stenosis and the re-operation rates.

  • Chapter 17 - Supravalvular Aortic Stenosis

  • from Section 3 - Left-Sided Obstructive Lesions
  • Edited by Laura K. Berenstain, James P. Spaeth
  • Book:
    Congenital Cardiac Anesthesia
    Published online:
    09 September 2021
    Print publication:
    16 September 2021, pp 105-112

  • Summary

    Williams syndrome is a multisystem disorder with a variable clinical presentation resulting from a chromosome 7q11.23 microdeletion including the ELN gene coding for the protein elastin. Eighty percent of patients have cardiovascular abnormalities including supravalvar aortic stenosis, branch and peripheral pulmonary artery stenosis, and coronary artery obstructions. Patients are at high risk for cardiac arrest when undergoing sedation or general anesthesia due to an imbalance in myocardial oxygen supply and demand induced by anesthetic medications. Risk factors for cardiac arrest in the perioperative period are biventricular outflow tract obstruction and coronary artery obstruction by ostial or intraluminal stenosis. Hemodynamic goals include maintaining an age-appropriate heart rate, sinus rhythm, preload, contractility, and systemic vascular resistance. Sedation or general anesthesia for elective procedures should be performed at tertiary care medical centers with immediate access to cardiac specialists and the capability to provide extracorporeal membrane oxygenation support.