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      Primary repair of symptomatic neonates with tetralogy of Fallot with or without pulmonary atresia

      review-article
      , MD , , MD, , MD
      Korean Journal of Pediatrics
      The Korean Pediatric Society
      Tetralogy of Fallot, Neonate, Early primary repair, Palliation

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          Abstract

          Recently, surgical outcomes of repair of tetralogy of Fallot (TOF) have improved. For patients with TOF older than 3 months, primary repair has been advocated regardless of symptoms. However, a surgical approach to symptomatic TOF in neonates or very young infants remains elusive. Traditionally, there have been two surgical options for these patients: primary repair versus an initial aortopulmonary shunt followed by repair. Early primary repair provides several advantages, including avoidance of shunt-related complications, early relief of hypoxia, promotion of normal lung development, avoidance of ventricular hypertrophy and fibrosis, and psychological comfort to the family. Because of advances in cardiopulmonary bypass techniques and accumulated experience in neonatal cardiac surgery, primary repair in neonates with TOF has been performed with excellent early outcomes (early mortality<5%), which may be superior to the outcomes of aortopulmonary shunting. A remaining question regarding surgical options is whether shunts can preserve the pulmonary valve annulus for TOF neonates with pulmonary stenosis. Symptomatic neonates and older infants have different anatomies of right ventricular outflow tract (RVOT) obstructions, which in neonates are nearly always caused by a hypoplastic pulmonary valve annulus instead of infundibular obstruction. Therefore, a shunt is less likely to preserve the pulmonary valve annulus than is primary repair. Primary repair of TOF can be performed safely in most symptomatic neonates. Patients who have had primary repair should be closely followed up to evaluate the RVOT pathology and right ventricular function.

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          Most cited references56

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          A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow.

          A new angiographic method for quantitative standardization of cross-sectional area of bilateral pulmonary arteries, the PA-index, and retrospective analysis of the PA-index in different types of operative procedures are presented. This study included 40 subjects in the normal control group, 46 patients in the tetralogy group, 26 patients in the Rastelli group, and 15 patients in the Fontan group. The normal value of the PA-index was 330 +/- 30 mm2/BSA and was consistent in a wide range of body surface areas from infancy to adolescence. The PA-index in the tetralogy and Rastelli groups ranged from 100 to 400 mm2/BSA. There were no early deaths in the tetralogy group, but the incidence of low cardiac output was higher in patients with a smaller PA-index, especially when the PA-index was less than 150 mm2/BSA. Low cardiac output was more severe in the Rastelli group. The operative mortality was significantly affected by the PA-index. In the Rastelli group, all of the patients with a PA-index of less than 200 mm2/BSA died, whereas the mortality rate in patients with a PA-index of more than 200 was only 6% (p less than 0.01). The mortality rate was not influenced by any other factors, such as aortic cross-clamp time or age at operation. In the Fontan group, two patients with a PA-index of less than 250 mm2/BSA died of severe heart failure, and 12 of 13 patients with a PA-index of more than 250 survived (p less than 0.01). Our results indicated the validity of the PA-index in predicting the postoperative prognosis of the various entities. In tetralogy, all patients with a PA-index over 100 mm2/BSA can undergo correction safely; in Rastelli operation, those with a PA-index under 200 should have a palliative procedure first, whereas those with a PA-index over 250 can be considered good candidates for the Fontan procedure. The PA-index may also serve a useful guide in comparing surgical results from different institutions with patients having anomalies of varying severity.
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            Repair of tetralogy of Fallot in neonates and young infants.

            The timing of repair of tetralogy of Fallot (TOF) remains controversial. Advantages to early complete repair include removal of right ventricular outflow tract obstruction, alleviation of systemic hypoxia, and avoidance of palliation with an arteriopulmonary shunt. This is a retrospective review of 99 children with TOF pulmonary stenosis (TOF/PS) or TOF pulmonary atresia (TOF/PA) who were <90 days of age undergoing early complete repair. Fifty-nine were prostaglandin E dependent, and 91% of neonates were symptomatic at the time of repair. Univariate and multivariate analyses of patient characteristics, anatomic features, and operative management showed the diagnosis of TOF/PA and smaller body surface area to be the only independent risk factors for death. Early mortality was 3% (3 of 99), and actuarial survival rates were 94% at 1 year and 91.6% at 5 years. Freedom from catheterization was 86% at 1 year and 73% at 5 years. Patients repaired for TOF/PA had a significantly lower freedom from reoperation than did those repaired for TOF/PS. Early complete TOF repair can be accomplished with a low mortality. Children with TOF/PA repaired had a lower freedom from reoperation that did those with TOF/PS. Longer follow-up, with emphasis on arrhythmias and right ventricular function, is required to define the long-term benefits of early repair.
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              Complete repair of tetralogy of Fallot in the neonate: results in the modern era.

              To review more than a decade of experience with complete repair of tetralogy of Fallot (TOF) in neonates at the University of Michigan; to assess early and late survival, perioperative complications, and the incidence of reoperation; and to analyze patient, procedural, and morphologic risk factors to determine their effects on outcome. Palliation of TOF with systemic-to-pulmonary artery shunts has been the accepted standard for symptomatic neonates and infants. Complete repair has traditionally been reserved for infants older than 6 months of age because of the perception that younger and smaller infants face an unacceptably high surgical risk. A retrospective review from August 1988 to November 1999 consisted of 61 consecutive symptomatic neonates with TOF who underwent complete repair. Thirty-one patients had TOF with pulmonary stenosis, 24 had TOF with pulmonary atresia, and 6 had TOF with nonconfluent pulmonary arteries. The mean age at repair was 16 +/- 13 days, and the mean weight was 3.2 +/- 0.7 kg. Before surgery, 36 patients were receiving an infusion of prostaglandin, 26 were mechanically ventilated, and 11 required inotropic support. Right ventricular outflow tract obstruction was managed with a transannular patch in 49 patients and a right ventricle-to-pulmonary artery conduit in 12. Cardiopulmonary bypass time averaged 71 +/- 26 minutes. Hypothermic circulatory arrest was used in 52 patients (mean 38 +/- 12 minutes). After cardiopulmonary bypass, the average intraoperative right/left ventricular pressure ratio was 55% +/- 13%. There were no new clinically apparent neurologic sequelae after repair. The postoperative intensive care unit stay was 9.1 +/- 8 days, with 6.8 +/- 7 days of mechanical ventilation. There was one hospital death from postoperative necrotizing enterocolitis on postoperative day 71 and four late deaths, only one of which was cardiac-related. Actuarial survival was 93% at 5 years. Follow-up was available for all 60 hospital survivors and averaged 62 months (range 1-141 months). Twenty-two patients required a total of 24 reoperations at an average interval of 26 months after repair. Indications for reoperation included right ventricular outflow tract obstruction (19), branch pulmonary artery stenosis (11), severe pulmonary insufficiency (4), and residual ventricular septal defect (1). The 1-month, 1-year, and 5-year freedom from reoperation rates were 100%, 89%, and 58%, respectively. Complete repair of TOF in the neonate is associated with excellent intermediate-term survival. Although the reoperation rate is significant, this is to be expected with the complex right ventricular outflow tract and pulmonary artery anatomy seen in symptomatic neonates and the need for conduit replacement in patients with TOF with pulmonary atresia.
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                Author and article information

                Journal
                Korean J Pediatr
                Korean J Pediatr
                KJP
                Korean Journal of Pediatrics
                The Korean Pediatric Society
                1738-1061
                2092-7258
                January 2014
                31 January 2014
                : 57
                : 1
                : 19-25
                Affiliations
                Department of Thoracic Cardiovascular Surgery, Sejong General Hospital, Bucheon, Korea.
                Author notes
                Corresponding author: Chang-Ha Lee, MD. Department of Thoracic Cardiovascular Surgery, Sejong General Hospital, 28 Hohyeon-ro 489beon-gil, Sosa-gu, Bucheon, Korea. Tel: +82-32-340-1483, Fax: +82-32-340-1236, leechha@ 123456sejongh.co.kr
                Article
                10.3345/kjp.2014.57.1.19
                3935108
                24578712
                aadcae47-9b12-45df-bc16-707d491111c9
                Copyright © 2014 by The Korean Pediatric Society

                This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 24 June 2013
                : 10 September 2013
                Categories
                Review Article

                Pediatrics
                tetralogy of fallot,early primary repair,palliation,neonate
                Pediatrics
                tetralogy of fallot, early primary repair, palliation, neonate

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