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Department of Pediatric Cardiovascular Surgery, Childrens Research Hospital, Kyoto Prefectural University of Medicine, Kyoto 602-8566, Japan
(Email: newriver{at}koto.kpu-m.ac.jp).
We thank Dr Troise and colleagues [1] for their interest in our article [2].
A number of previous reports indicate that morphological problems in the pulmonary artery (PA) may produce diminished and unbalanced pulmonary perfusion, and may also result in future PA maldevelopment, which adversely affects the prognosis of the patient. One of the morphological problems that we often encounter is pulmonary arterial stenosis at the ductal attachment (pulmonary coarctation). As our article indicated, it seems that PA reconstruction and augmentation in early infancy for the patients with pulmonary coarctation may prevent PA maldevelopment and may provide sufficient and balanced pulmonary arterial development, similar to the excellent results of one-stage unifocalization in the early days of life for patients with major aortopulmonary collateral arteries.
We do not have data that indicates the incidence of pulmonary coarctation; however, a few reports suggest a higher incidence of pulmonary coarctation than we believed [3]. We now believe that we should perform an operation once the pulmonary coarctation becomes evident. The surgical intervention can include PA augmentation with modified Blalock-Taussig shunt, PA augmentation with right ventricle–PA conduit, or one-stage definitive repair with PA augmentation. We prefer a staged strategy with PA augmentation by fresh autologous pericardium and a Blalock-Taussig shunt. We also believe that a catheter intervention for pulmonary coarctation may have higher risk and that stent placement in the PA may prevent future growth of the PA itself.
We recommend complete removal of the ductal tissue from the PA with surgical intervention. However, patients suspected of residual ductal tissue in the PA (eg, patients with large or torturous ductus) should have frequent postoperative follow-up evaluations to prevent postoperative PA maldevelopment, as described by Troise and colleagues [1]. Once postoperative PA stenosis or distortion is detected, we recommend aggressive surgical intervention to correct the problem. In our series, most of the patients achieved sufficient and balanced pulmonary development, but 5 of 15 patients required reinterventions to the PA at the next staged operation or as an isolated operation.
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