Congenital Heart Surgery

Guest Editorial by Sasha Agati, MD. Co-Chair of Congenital Heart Academy   Dear Colleagues,   First, I would like to thank Prof. Varun Aggarwal for the invitation and the opportunity to share my experience and point of view, at the same time I very proud to announce a stable collaboration between “The Congenital Heart International Professionals (CHiP) Network” and “Congenital Heart Academy”. The “editorial opinion”, I personally think, that represents the best option for express and stimulate comments and pro vs con: so today I am feeling very lucky. In the last 3 years, it was so difficult upgrade our “experience” based knowledge due to pandemic status, but that was time to review our data, our activities, our outcomes. We experienced with the growth of web based educational platform like Congenital Heart Academy, that there was the amazing opportunity to receive “one-way” information and why not education too. Yes, one-way because web-based seminars make the teacher/speaker in a real “honorary” position and all information came as a “Review” done every time from different super-experts. For sure this creates a new world, a new way of perception of science and probably a new attitude for clinical practices.   During these years, several webinars were focused on “Transposition of Great Arteries” and we have learnt a lot. For example, the extraordinary testimony about the coronary translocation story done by Prof. Marcelo Jatene, very difficult to know about this from other sources. But, what I think, make more rumors on the web was the position about Rashkind procedure and Lecompte maneuver in the field Transposition of Great Arteries physiology and long-term results.   In this editorial, I will comment about Rashkind using a selection of web-limited world strategies (see Gil Wernowky series YouTube Congenital Heart Academy Channel: https://www.youtube.com/watch?v=lCTUp9nPBgU&t=3434s). Most still believe that Rashkind is a life-saving procedure, but in the current practices it is increasingly used as a “routine” procedure to stabilize the patient (rather than for desaturation patients with Transposition of Great Arteries – cardiologist view – Rashkind for all!!!) in most of the cases done directly in cardiac intensive care bed. Percutaneous balloon atrial septostomy (PBAS) has historically been applied in neonates with transposition of the great arteries only with inadequate mixing at the atrial level in order to enlarge or create an interatrial septal defect. Many reports have emphasized that Rashkind procedure is not without complications. Cogo et all [1] demonstrated that Rashkind procedure should be used for those patients with significant hypoxemia, hemodynamic instability, or both. After procedure, removal of catheter from the vein both femoral or umbilical and again bleeding control can be obtained only by clot formation [3]. Now, in transposition of great arteries 80 to 90% of cardiac output is “recirculating” venous return so if a clot form anywhere in the baby by central line or peripherally inserted venous catheter this will easily cross the aortic valve causing stroke (2% to 5% reported incidence). Rashkind procedure was not associated with an increased risk of necrotic enterocolitis but was associated with twice the risk of stroke [2-3].   The selective use of Rashkind procedure for those patients with significant hypoxemia, hemodynamic instability or both still represents the correct strategy for the optimization of underdevelopment outcome of neonates with Transposition of Great Arteries [4]   On the other side, prostaglandin infusion “loved” by most of surgeons make the patient so “stable” than and you don’t need to run in theater, but, causing heart failure as basic complication and characterized by wide pulse pressure accompanied by tachycardia. Apnea occupies the first adverse effect with intubation necessity [5].   Both strategies are not “free of charge”, in some place Rashkind is done under general anesthesia (less than before) and prostaglandin infusion requires stable vascular access most likely a central or a peripherally inserted central line, caffeine and what about inflammation. So, “do what you do best “…. but let think about: if only a small percentage of “real symptomatic” newborn need Rashkind procedure and few of them require continuous infusion of prostaglandin are we able to “switch” the child without additional procedure? Let’s think about this and wait for your comment.   References: 1. Balloon atrial septostomy and pre-operative brain injury in neonates with trasposition of the great arteries: a systemic review and a meta-analysis. A. Polito, Z. Ricci, T. Fragasso, P. E. Cogo. Cariology in the Young (2012) 2. Analysis of 8681 neonates with transposition of great arteries: outcomes with and without Rashkind balloon atrial septostomy. D. Mukherjee, M. Lindsay, Y. Zhang, T. Lardaro, H. Osen, D. C. Chang, J. I. Brenner, F. Abdullah. Cardiologi in the Young (2010). 3. Thrombus formation in the heart following balloon atrial septostomy in transposition of great arteries. S. Talwar, S. Ramakrishnan, P. Gharde, S. K. Choudhary. IJTCS (2022) 4. Preoperative brain injury in transposition of the great arteries is associated with oxygenation and time to surgery, not balloon atrial septostomy. Petit CJ, Rome JJ, Wernovsky G et al. Circulation (2009) 5. Congenital Heart disease: The State-of-the-Art on Its Pharmacological Therapeutics. Carlos Daniel Varela-Chinchilla, D. Edith Sánchez-Mejía 1 and Plinio A. Trinidad-Calderón. JCDD (2022)

Outcomes of aortic valve repair in children resulting in bicuspid anatomy: Is there a need for tricuspidization?   Schulz A, Buratto E, Wallace FRO, Fulkoski N, Weintraub RG, Brizard CP, Konstantinov IE.J Thorac Cardiovasc Surg. 2022 Jul;164(1):186-196.e2. doi: 10.1016/j.jtcvs.2022.01.022. Epub 2022 Jan 26.PMID: 35227498   Take Home Points: Outcomes of aortic valve repair in small children are excellent. Tricuspidization of bicuspid valve is inferior compared to bicuspid repair in terms of chance of reoperation.     Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), chief section editor of Congenital Heart Surgery Journal Watch:      Summary:   Single center retrospective study included 127 patients who underwent aortic valve repair with creation/preservation of bicuspid aortic valve anatomy or tricuspidization of bicuspid valves between 1980 and 2016. Median age was 0.73 years (interquartile range (IQR, 0.1-8.9), and median weight was 8.15 kg (IQR, 3.9-31.7). The cohort included 29 neonates (22.8%), 36 infants (28.3%), and 62 children (48.8%). Repair was performed without a patch in 69 patients (54.3%). Survival at 10 years was 94.8%. Freedom from aortic valve reoperation at 5 and 10 years was 79.9% and 65.6%, respectively. Re-repair was undertaken in 53.7% (22/41). Freedom from aortic valve replacement at 5 and 10 years was 90.3% and 75.8%, respectively. Risk factors for reoperation were age less than 1 year, unicuspid valve, and the presence of Shone complex and concomitant aortic arch repair. There were 107 patients (107/127, 84.25%) with preoperative bicuspid aortic valve morphology that was preserved. They were compared with a separate cohort of 44 patients who underwent tricuspidization of bicuspid aortic valve during the same period. There was no difference in survival or freedom from aortic valve reoperation. However, freedom from aortic valve replacement was lower after tricuspidization with 49.7% versus 75.8% after 10 years (P=0.0118).   Comment:   This is a retrospective study for aortic valve repair done at Royal Children’s Hospital, Melbourne. They have a large experience of surgical aortic valve for patients with aortic stenosis. Their outcomes are outstanding with freedom from aortic valve replacement after 10 years is 75.8 % even a half of their patients are neonates and infants. This study showed age less than 1 year, unicuspid valve, and concomitant arch procedure was a risk for reoperation. More importantly, they identified bicuspid repair was superior to tricuspidization regarding with a chance of reoperation. This makes sense when looking at figures and video in the manuscript that their techniques for aortic valve repair are excellent. Term of “aortic valve repair” is difficult to interpret as different surgeons use different techniques. So, it is often tricky to only look at numbers of reoperation rate, etc. in the paper. Excellence of this manuscript is that they have beautiful figures to show exactly how they do for aortic valve repair (Figure 2&3). Also, they provide a video that every surgeon can understand their technique visually, not by the description. When looking at these figures and video, you can appreciate that their techniques of aortic valve repair support their excellent results that not all surgeons can reproduce same outcomes. Hence, I understand that their institutional preference to treat patients with aortic stenosis is surgical aortic valve repair.