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Division of Cardiac Surgery, Children's National Medical Center, 111 Michigan Ave NW, Washington, DC 20010
(Email: rjonas{at}cnmc.org).
Supravalvar aortic stenosis is a rare lesion. In the early years of congenital cardiac surgery, it was managed in the same way as any circumferential stenosis by using a simple single-patch plasty technique. Doty contributed an important advance with the introduction of the inverted bifurcated patch. Although Doty's technique effectively enlarges the circumference of the sinotubular ridge, which is usually the site of most severe stenosis, it does not provide a symmetric reconstruction or enlargement of the left coronary sinus.
Recognizing these deficiencies as well as the importance of restriction of coronary blood flow into the left coronary sinus has led many centers during the last 10 to 15 years to begin applying the symmetric 3-patch technique originally described by Brom. The authors of the article published in this issue [1] are correct in stating that there is little information available to clarify the advantages or disadvantages of the Brom technique relative to the more traditional Doty technique.
After their analysis, the authors strongly endorse the 3- patch technique. Their conclusions are similar to those drawn from a series of 75 patients managed at Children's Hospital Boston during a 41-year experience. Multiple sinus reconstruction appears to result in superior hemodynamics and is associated with reduction in both mortality rate and need for reoperation.
One limitation of the article by Metton and colleagues [1] is the same senior surgeon did most of the Brom operations. It is also not clear which criteria were used to select the 1- vs 2- vs 3-patch technique. The limitations statement of the article suggests that this may have been mainly surgeon-dependent, which considerably weakens the findings of the study. However, a subsequent analysis undertaken by the authors looking only at 25 patients operated on by the same surgeon also suggests superiority of the Brom repair.
Considerable progress has been made in understanding the genetic basis of the elastin arteriopathy that causes supravalvar aortic stenosis. This lesion is deceptively complex and involves abnormalities of coronary perfusion as well as left ventricular hypertension and hypertrophy. Nevertheless the long-term results of surgical repair are surprisingly satisfactory. The article by Metton and colleagues [1] provides further evidence that symmetric reconstruction with 3 patches is likely to be the optimal method of repair for most patients.
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