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- W4206755364 abstract "Dr Vincent Tam (Fort Worth, Tex). Thank you for the privilege of discussing this research. Thank you for a very nice presentation on a complex subject. I enjoyed reading your manuscript as well. I have at least 1 major criticism, and that is that it is really not a fair comparison between these 2 groups of patients. One group is kids who obviously had relatively favorable anatomy to undergo primary biventricular (BiV) repair, and then the second group had a much larger proportion of kids who had noncommitted ventricular septal defects (VSDs), heterotaxies, and atrioventricular canal. So these are not really valid comparisons to compare 1 group to the other. What is the likelihood of failure in your palliative procedures to achieve candidacy for BiV repair? In other words, are there patients who are palliated that never reach BiV repair? Dr Olubunmi Oladunjoye (Boston, Mass). Thank you, Dr Tam, first for taking the time to review the manuscript and also for your question. It is true that the 2 groups are different in terms of their anatomy and demographic characteristics. The point we were trying to bring about here is to say that despite the fact that those patients are complex and have undergone the staged BiV conversion, they still have outcomes that are similar to those who seemed favorable for BiV repair. Regarding your question about patients who did not reach BiV conversion, this study focused on patients who underwent BiV conversion. There are indeed patients who have had staged procedures, and are awaiting BiV conversion. In this group, we have had no mortality. Dr Tam. In the subgroup of patients with heterotaxy syndrome, atroventricular canal, and double outlet right ventricle, to me, this is a very complex and different group of patients. So given these data, is it your recommendation that these patients undergo palliation and staged repair rather than simple straightforward repair? Dr Oladunjoye. Thank you very much for your question. Given the complexity of their anatomy, a complex reconstruction with long crossclamp time and high risk in a young child is not preferable. Given the findings presented here, we would propose staged BiV conversion as an alternative to either primary BiV repair or single-ventricle palliation. Dr Tam. I think it will be, to me, much more interesting in looking at this subgroup of patients when the authors believe it is appropriate to proceed with perhaps even more than 1 stage of palliation in preparation for complete repair. Dr Tae Jin Yun (Seoul, South Korea). Congratulations on this excellent outcome. I have 2 questions. When you commented on 1.5-ventricle physiology, did it mean that you took down the bidirectional Glenn to reanastomose the superior vena cava to the right atrium in patients with previous bidirectional Glenn procedure or the Fontan operation? Dr Oladunjoye. Yes, we excluded the patients with a 1.5 ventricle in this study to allow for a better comparison between the BiV group and the staged BiV conversion. In most of our patients the left ventricle was actually of adequate size, so 1.5-ventricle repair would only be performed to reduce the complexity of BiV reconstruction. We do not think that it would provide physiological benefit except in patients with compromise of right heart structures. Dr Yun. In my experience, for patients with complex double outlet right ventricle with remote VSD the right ventricular volume may be compromised by intracardiac baffling, and one-and-a-half ventricular repair strategy may be beneficial in this setting. The second question is, there were 11 patients with noncommitted VSD in your primary repair group, and primary repair was done in the patients as young as age 1.5 months. That might have been quite technically demanding. Were there any contraindications for left ventricle to aorta rerouting, such as tricuspid valve interposition between the VSD and the aortic valve, straddling of the mitral valve or tricuspid valve, or restricted VSD in this subset? If there were any risk factors, were they attributable to the high mortality in this subgroup? Dr Oladunjoye. The high mortality in the group may be due to the complexity of the anatomy at young age. These patients tend to have a noncompliant ventricle after such a large operation, and higher probability of residual disease (eg, VSD, valve compromise, or outflow obstruction), combinations of which can be associated with high mortality. Discussant (Beijing, China). Very nice presentation. From your data for noncommitted double outlet right ventricle you seem to have a large number for single-ventricle repair palliation. How is your strategy for noncommitted VSD double outlet right ventricle? So how was the treatment strategy, I mean, how you did the decision making to do a single-ventricle repair or BiV repair, because in our institution we presented several years ago at this meeting, and probably one-fifth of patients needed a single-ventricle repair but the rest of the patients we can do a BiV repair. I was just curious about your decision making. Dr Oladunjoye. Thank you very much for your question. So for the noncommitted VSD group, we do know that this is a very complex group and many studies have also shown that these are complex cases. We would take into consideration the size and function of ventricles, need to enlarge VSD, and valvular function. Age is also an important factor. Before the staged approach, we would be forced to commit a patient to either single ventricle or high-risk primary BiV repair. But now we are more likely to complete the repair in stages to mitigate risk. Dr Sitaram Emani (Boston, Mass). May I just add that our approach has evolved over time. Early in our experience, we were reticent about taking on very complex anatomies because of risk of mortality, particularly in patients with unfavorable anatomy, including atrioventricular valve regurgitation, abnormal atrioventricular valve attachments, long distance between the left ventricle and aorta, dextrocardia, and pulmonary vein abnormalities. The single-ventricle pathway seemed to have less upfront risk in such patients. But what we found over time is that if you distribute the complete repair into multiple operations, each of them can be performed safer than trying to do it all at once, yet you can ultimately get to the point where you can have a successful BiV repair. So now our approach is actually to ask the question, why not stage the repair rather than take the risk of a primary repair in an infant. Although this approach might require extra operations, the end result is that the patients have ventricles that have been better prepared with fewer residual lesions, and I think have better long-term and short-term outcomes. Discussant. I have a question about the left ventricular outflow tract obstruction (LVOTO) after the repair. Did you stratify the different groups, which group of patients that developed LVOTO more often than another group? Dr Oladunjoye. So when we did try to stratify, we looked at the risk factors for LVOTO and found out that the type of VSD was not a risk factor for LVOTO. Dr Emani. We certainly expected to find that age was going to be a risk factor because the patch would not be expected to grow over time. We also believed that perhaps the type of material would affect risk of LVOTO. Neither turned out to be risk factors. Upon further analysis of patients who developed LVOTO, we believe it is turbulence from an abnormal outflow tract that leads to subaortic membrane. In a few patients who did not have adequate VSD enlargement at the time of the original operation, LVOTO develops at the level of the VSD. We have moved toward more aggressive VSD enlargement for this reason. Discussant. Thank you. Just a little comment. We are now trying to do a more aggressive BiV repair with double outlet right ventricle, especially in noncommitted VSD. Now our strategy is the echocardiogram to screen the patient, and if it is a noncommitted we do 3-dimensional printing to simulate their situation. But sometimes we still explore the heart and sometimes we found, even though this 3-dimensional printing didn't indicate we can do a BiV repair, we still can try that. So in the past 2 years, I can't remember the exact number, but very rare patient we do a single-ventricle palliation. For most we can do a BiV repair. Although the tricuspid is in the way, we can resect the tricuspid valve and do the baffle and then put back the tricuspid valve, and all these patients are doing well. Dr Emani. I agree that most patients can undergo a BiV repair. The question is how you get there. To do a complex operation with multiple components in an infant or neonate may be risky from the standpoint of survival and residual disease. A staged approach in such patients is feasible and may be safer. The only downside is the need for multiple surgeries." @default.
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- W4206755364 title "Discussion" @default.
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