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Ann Thorac Surg 1997;63:1216-1217
© 1997 The Society of Thoracic Surgeons
Department of Cardiovascular Diseases, King Faisal Specialist Hospital, Po Box 3354, Riyadh 11211, Saudi Arabia, e-mail: alhalees{at}kfshrc.edu.sa
To the Editor:
We reviewed with great interest the article by Godart and associates [1]. They conclude that surgical treatment of tetralogy of Fallot with absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. Although they alluded to attempts at initial palliation [2, 3] to improve symptoms before total repair, their choice remains primary total correction. Their overall in-hospital mortality rate was 8%.
We, like others, were distressed with the high mortality rate in primary total correction of severely symptomatic infants with tetralogy of Fallot and absent pulmonary valve (33% in our own series). The prospect of initial palliation appeared attractive, and since 1991 we have adopted a selective approach of palliating severely symptomatic infants by pulmonary artery banding followed by elective total repair at a later stage. Our initial experience was encouraging, and we actually demonstrated regression of pulmonary artery size after pulmonary artery banding by angiographic measurement [4].
Eight severely symptomatic infants with tetralogy of Fallot and absent pulmonary valve (3 on mechanical ventilation) were managed by initial pulmonary artery banding (mean age, 5 months; mean weight, 6.5 kg). All patients recovered without major complications; 2 patients continued to have recurrent episodes of chest infection requiring management in the hospital. Six patients underwent total repair after a mean of 22 months without mortality, and 2 are still awaiting total repair.
Only 1 patient required pulmonary artery plication. Comparing this experience with our earlier experience and the experience of Godart and associates, it was clear that adopting a selective two-stage repair of tetralogy of Fallot with absent pulmonary valve had significantly reduced the need for pulmonary artery plication and operative mortality.
References
Pediatric Cardiac Surgery, Hospital Marie Lannelongue, 133, Ave de la Résistance, 92350 Le Plessis Robinson, France
To the Editor:
We read with interest the letter by Al-Halees and associates because it highlights the problems of dealing with tetralogy of Fallot and absent pulmonary valve, especially in severely ill infants. We are pleased with results obtained to rescue such patients. Al-Halees and associates recommended a two-stage approach because of the high mortality rate with primary total correction. They proposed pulmonary artery banding at initial palliation and total repair at a later age. They noticed a regression of the pulmonary artery size after banding, and only 1 of the 6 patients who were reoperated on required pulmonary artery plication.
In fact, it may be difficult to draw out rules and obtain worldwide consensus in a quite rare pathology. Infants who do not survive primary complete correction may be a special group. It has been pointed out that intrinsic histologic change in the pulmonary artery might be the determining factor of this syndrome. The resultant effect is aneurysmal dilatation of the pulmonary artery, which is already observed in utero in humans. Momma and colleagues [1], in an experimentally created model of tetralogy of Fallot with absent pulmonary valve in fetal rats, noticed an inverse correlation between the size of the enlarged pulmonary artery and the size of adjacent bronchi. In fact, the compression of intrapulmonary bronchi with consequent distal bronchomalacia appears to be the consequence of the former dilatation of the pulmonary artery. Thus, impairment of bronchial and alveolar multiplication has been reported with, for example, dysplastic or completely absent bronchial cartilages and resultant loss of bronchial wall support [2, 3]. So patients presenting in early infancy are those with severe lung impairment. For these patients, it should be of interest to perform preoperative bronchoscopy to establish and quantify the bronchial deformities. In contrast, the second group, including usually older patients with mild symptoms and a better life expectancy, is the group with less lung impairment.
Reduction in size of the pulmonary artery after banding, as reported by Al-Halees and associates, is not always the rule. What they might suggest is that pulmonary artery banding could decrease pulmonary artery enlargement by reducing pulmonary overflow. But the left-to-right shunt at ventricular level is usually not significant. It is of interest to note that 2 patients in their series remained symptomatic after palliation, necessitating hospital management, and 1 required pulmonary arterioplasty at complete repair. Instead of pulmonary artery banding, others have proposed complete ligation of the pulmonary trunk and aortopulmonary shunt to eliminate pulmonary regurgitation and control and the pulmonary blood flow in these infants [4]. In fact, both techniques are old-fashioned and the results are not reliable. Only 5 of 8 infants who had undergone pulmonary artery banding survived in two different series [5, 6].
On the other hand, 2 patients in our series had persistent aneurysmal dilatation of the pulmonary artery with bronchial compression, many years after initial complete repair without pulmonary arterioplasty. One of them had a complete obstruction of the left main bronchus and the other is awaiting-since publication-plasty for reduction of the right enlarged pulmonary artery (45 mm). Moreover, this was observed despite pulmonary valve insertion. So the decrease in pulmonary artery size cannot be anticipated, and this may rely on the initial degree of pulmonary artery enlargement. All these data are clearly in favor of pulmonary arterioplasty at primary correction, which remains our procedure of choice.
Furthermore, there is no consensus whether or not a pulmonary valve should be inserted at primary repair. The role of pulmonary regurgitation has been emphasized, and some authors have clearly recommended insertion of a pulmonary valve [3, 7]. A homograft is probably the most suitable material for these infants, accepting the need for reoperation in future, similar to what is usually performed in patients with truncus arteriosus. We endorse this view, especially in infants with elevated pulmonary artery pressure, and this may be helpful in early extubation and survival. Nevertheless, in our experience, primary complete repair with pulmonary arterioplasty and usually no need for pulmonary valve insertion was performed with a low mortality rate, very similar to that reported by Watterson and associates [8]. We believe that such an approach is clearly recommended in severely symptomatic infants with tetralogy of Fallot and absent pulmonary valve.
References
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