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Ann Thorac Surg 2005;79:1824
© 2005 The Society of Thoracic Surgeons
Department of Pediatric Cardiac Surgery, Giovanni XXIII Hospital, Via Lorenzo D'Agostino 1/A, Bari 70124, Italy
detroise{at}libero.it
We read with interest the article by Napoleone and colleagues [1] regarding successful treatment of a patient with a double outlet right ventricle (DORV), intact ventricular septum, and incompetent mitral valve. Surgical treatment included left ventricle exclusion through avulsion of the mitral valve.
In the article, a reference was made to the treatment of a similar case, which was ours [2] concerning the option we adopted to achieve left ventricular exclusion by patching the mitral valve remnants, judging our choice as suboptimal due to the risks of ventricular dysfunction "similar to what is believed to be the case of pulmonary atresia and intact ventricular septum."
As we pointed out in our article, the fate that we had to assign to the left ventricle in our case was strongly debated preoperatively, and the final decision was taken, bearing in mind the anatomical reference models (ie, all 13 cases of DORV and intact ventricular septum), reported in the literature [3–8].
In these reports, we found that all those cases with malposition of the great vessels [2] had a clear hypoplastic left ventricle with a highly restrictive or atresic left atrioventricular junction, and apart from 3 patients that died from untreated critical associated anomalies (restrictive interatrial communication in 2 and critical pulmonary stenosis in 1), all other patients lived beyond neonatal age. Most of these infants, despite the limited surgical and perioperative technology available at that time, survived early infancy, which is when bi-directional cavopulmonary anastomosis would have been performed. These observations led us to speculate that a vestigial cavity may be well tolerated in this unusual pattern.
Looking at our particular case, we realized that we were treating the only exception with the respect to the cases described in the literature that had most likely escaped in late fetal life from the hypoplasia, due to an incompetent valve that, altering the volume of the left ventricle with a negative effect on the mass/volume ratio, masked a "vestigial" cavity.
Therefore, in our "decision making," we decided to complete the aborted "involvement" of the left ventricle, usually associated in this anatomical pattern, through the total restriction of ventricular inflow, and in the meantime, decompressing the left atrium, we attempted to allow the pulmonary outflow tract to work as an ideal source of accessory pulmonary blood flow, once a bidirectional cavopulmonary anastomosis was accomplished.
At a further 27 months of follow-up, the child is in a superb condition without any medication.
At the last outpatient evaluation, the left ventricle was found to be extremely flat and no bi-ventricular impairment was detected.
Anyhow, in our opinion, we believe the rarity of this anatomical situation does not allow an estimate to be made of the hemodynamic consequences of whatever surgical option is adopted.
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