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Congenital Heart Surgery Service, Texas Children's Hospital, Baylor College of Medicine, 6221 Fannin St, Mail Code: WT 19345-H, Houston, TX 77030-2399
(Email: charlesf{at}bcm.tmc.edu).
Is there controversy remaining in the surgical treatment of tetralogy of Fallot (TOF)? Can we continue to modify our approach to these patients and improve their short-term and long-term prognosis? This report of 28 years of experience with TOF with atrioventricular septal defect (AVSD) underscores the fact that there is still much room for refinement [1].
Tetralogy of Fallot with AVSD is a complex constellation of lesions, historically associated with challenging operative correction and variable, but significant perioperative risk. In many ways, this association is the "ultimate" form of TOF. Operative risk is exacerbated by the very large ventricular septal defect patch required for the malaligned ventricular septal defect component, as well as atrioventricular valve reconstruction, and right ventricular outflow tract (RVOT) resection with or without pulmonary valvotomy and transannular incision. Unnecessary or avoidable compromise of the reconstructed right heart will leave the patient with a suboptimal long-term prognosis. The right heart will ultimately suffer the ill effects of pulmonary and tricuspid insufficiency, infundibular dilation, scarring, and dysrhythmia. These observations are quite familiar to any practitioner faced with the challenge of caring for the remotely repaired adult patient with TOF presenting with right heart failure. In this regard, the present article provides additional, important information to be considered by the surgeon responsible for repairing a child presenting with TOF with or without AVSD.
This report documents that a surgical management strategy focused on optimizing the individual patient's right heart structure offers the potential of not only outstanding perioperative survival, but important lack of long-term right heart dysfunction. By avoiding a transmural infundibulotomy, the authors have demonstrated the minimal need for right heart reoperation. Of particular note, despite a very high incidence of pulmonary valvotomy and minitransannular incision (<3 mm through the pulmonary annulus), this late echocardiographic study demonstrates little, if any pulmonary insufficiency. Many have opined that this is one of the principal benefits of the transatrial–transpulmonary approach. By avoiding a transmural RVOT incision, the infundibulum remains contractile rather than becoming a dysfunctional, subvalvar capacitance chamber. Is this why the present study has observed a lack of pulmonic insufficiency in spite of the known presence of very abnormal pulmonary valves in patients with TOF/AVSD? Surely many of the patients in this series, as others have observed, will have the potential for pulmonic insuffiency, yet this is somehow nullified by the presence of a working infundibulum.
Another issue that continues to prompt debate is the timing of complete repair. Certainly, advocates of complete neonatal correction have demonstrated that acceptable operative mortality rates can be achieved with this approach. However, the question remains as to whether a complex neonatal repair of TOF/AVSD will afford the patient the best right heart function during what will hopefully be another 70 plus years of life. At this point in the evolution of congenital cardiac surgery, these outcome data are simply not yet available. However, in this author's view the present article offers an additional glimpse into what the unfolding story of how to optimally protect a child's right heart is with the ultimate goal of cardiac structure and function capable of sustaining a normal life span. By performing non-neonatal, infant complete repair with uniform avoidance of an RVOT incision (including the limited use of palliative shunts in neonates where necessary), the authors have achieved outstanding long-term patient outcome with minimal need for reoperation. This achievement in the more complex variation of TOF with the addition of AVSD is noteworthy of consideration and has broader implications to all patients undergoing repair of TOF. The surgeon must, of course, be primarily concerned with operative survival, but of critical secondary concern is the effect of the type and timing of repair on the patient's heart function over time.
This generation of congenital heart surgery is blessed by the enormous improvements that have occurred over the first 50 years of TOF repair. Yet, we must accept the reality that few, if any of these repaired patients faces the prospect of a normal lifespan free of cardiac reintervention. Critical assessment of technique and outcome data such as those provided in this article are important adjuncts to our ongoing refinement of methodology for the long-term benefit of our patients.
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