Congenital Heart Surgery

Outcomes in Anomalous Aortic Origin of a Coronary Artery Following a Prospective Standardized Approach. Molossi S, Agrawal H, Mery CM, Krishnamurthy R, Masand P, Sexson Tejtel SK, Noel CV, Qureshi AM, Jadhav SP, McKenzie ED, Fraser CD Jr.Circ Cardiovasc Interv. 2020 Feb;13(2):e008445. doi: 10.1161/CIRCINTERVENTIONS.119.008445. Epub 2020 Feb 13.PMID: 32069111   Take Home Points: Surgical unroofing of anomalous aortic origin of a coronary artery cannot be offered to all patients and alternatives are needed. Transection and reimplantation of anomalous aortic origin of a coronary artery offer good results at a 4-year follow-up. After surgical repair, ˜ 90% of patients with anomalous aortic origin of a coronary artery can be cleared for exercise.     Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch: Summary: Surgical correction of anomalous aortic origin of a coronary artery is usually offered to symptomatic patients and high-risk asymptomatic patients in order to correct or prevent ischemia, and to prevent sudden death. The creation of an unobstructed coronary blood inflow from the appropriate sinus of Valsalva is the goal. Unroofing of the intramural and interarterial course of the coronary artery is the most common surgical procedure performed for such anomaly. However, it is not always possible to perform it due to anatomic variants, and it is not devoted of postoperative risks including coronary events, coronary reinterventions, and aortic insufficiency. Bonilla-Ramirez et al. compared their results with a transection and reimplantation technique in 16 patients to 45 unroofings at Texas Children’s Hospital. Preoperatively, about 45% had exertional symptoms and 30% nonexertional symptoms (leaving another 25% asymptomatic). Objective ischemia was demonstrated by stress cardiac MRI in 46%. Compared to unroofing, the transection and reimplantation technique required longer cardiopulmonary and aortic cross-clamp time by about one third. One patient with the transection and reimplantation technique had recurrent ischemia mandating CABG at a median follow-up of 4 years (compared to none among unroofing). One patient with unroofing had recurrent sudden cardiac arrest due to a previously unrecognized myocardial bridge. This patient had a redo with the transection and reimplantation technique and did well thereafter according to the authors. Over 90% of patients were cleared for exercise in both groups after surgical repair and postoperative non-invasive assessment. The transection and reimplantation led to the demonstration of less postoperative ischemia than the unroofing.   Comment: Surgeons dealing with anomalous aortic origin of a coronary artery know that unroofing —although favored— is not always possible. This paper from Bonilla-Ramirez et al. (along others such as Gaillard M. et al. featured in November 2020), is reassuring as it states that other ways of creating an unobstructed coronary inflow in such anomalies can result in good mid-term outcomes. The authors describe their decision not to perform unroofing for 3 categories of patients: 1) the course of the coronary artery is below the commissure, 2) when unroofing does not relocate the ostium to the appropriate sinus, and 3) when unroofing results in compression by the intercoronary pillar. Stating their rationale, they also express that they do not use osteoplasty techniques and that they do not create aortic buttons. The fact that one patient with unroofing and recurrent symptoms was treated by the alternative technique of transection and reimplantation highlights the facts that not all patients have the same baseline anatomy and that likely the repair should be tailored to the individual problem rather than trying to fit every anatomical variant with a standard technique. Interestingly, the failure of a post-reimplantation technique leading to CABG highlights that surgical manipulation of coronary origins is not devoted of potential complications. It makes us wonder if creating an aortic button may have prevented such complication. As the authors suggest, postoperative assessment by provocative myocardial ischemia testing should be performed before allowing exercise to prevent complications. As most studies have a limited follow-up (about 5 years), further studies with longer follow-up will be needed to better define the freedom from ischemic events among repaired anomalous aortic origin of a coronary artery. No question, we need large multicentric studies with long follow-up to better understand what technique should be applied to what anatomical variant and what should be expected or prevented in these patients down the road.    

Subcoronary Ross/Ross-Konno operation in children and young adults: initial single-centre experience. Murin P, Weixler VHM, Moulla-Zeghouane J, Romanchenko O, Schleiger A, Lorenzen V, Sinzobahamvya N, Zacek P, Photiadis J, Cho MY.Eur J Cardiothorac Surg. 2021 Jan 4;59(1):226-233. doi: 10.1093/ejcts/ezaa307.PMID: 33141218   Take Home Points: Subcoronary Ross/Ross-Konno operation is a choice for children who are not indicated for graft-enforced Ross procedure. Size mismatch between aortic and pulmonary annulus is not a problem as the aortic annulus and LVOT can be enlarged with Konno incision +/- additional patch in the non-coronary sinus.     Commentary from Dr. Yasuhiro Kotani (Okayama, Japan), section editor of Congenital Heart Surgery Journal Watch: Summary: A total of 50 patients with median age of 6.3 years who underwent subcoronary Ross/Ross-Konno operation was reviewed. After the harvest, the autograft was trimmed just to leave some tissues for the implantation. The annulus was enlarged when necessary. Konno incision was performed in 42 (84%) patients, including 24 patients with LVOTO and/or annular hypoplasia and 18 patients with a size mismatch between aortic and pulmonary annulus. An additional patch for non-coronary sinus was used in 17 (34%) patients. The autograft rotation was decided based on the orientation of the coronary artery ostium. Overall survival was 95%. Freedom from aortic valve regurgitation more than mild was 97% at 5years and only 2 (4%) patients required aortic valve replacement. No aortic root dilation was seen in the mid-term follow-up. Significance: Aortic valve surgery in children and young adults has several options, such as Ross procedure and aortic valve replacement with or without Konno, however, it is still in debate what procedure to be selected as the reconstructed aortic valve and the root should be grown. Subcoronary Ross/Ross-Konno procedure may be the answer that overcomes the disadvantages of aortic valve replacement and Ross procedure. Comment: No standard care was shown in the surgical treatment of the aortic valve in children and young adults. These patients are at age of somatic growth and this makes the choice of surgery difficult. Aortic valve replacement with or without Konno promises a longevity unless adverse events related to the prosthetic valve happen. Patients who are athletes and female may not be suitable as it requires warfarin in lifetime. In contrast, Ross procedure has a benefit for a potential growth of the autograft and no need for anticoagulation, however it creates 2-valve diseases and the dilatation of the implanted autograft remains an issue, especially for patients with a bicuspid valve. As originally described by Dr. Ross in 1967, subcoronary Ross should be taken into reconsideration. There are certainly some technical tips but it might be theoretically better option for children and young adults for potential growth without root dilatation. Size mismatch between aortic and pulmonary root can be always addressed by additional Konno-incision and patch for non-coronary sinus and fits for small children before school age.    

Survival Outcomes of Two-Stage Intracardiac Repair in Large Ventricular Septal Defect and Trisomy 18. Nakai R, Fujioka T, Okamura K, Suzuki T, Nakao A, Kobayashi J, Tsuchiya K. Pediatr Cardiol. 2021 Jan 29. doi: 10.1007/s00246-021-02546-9. Online ahead of print. PMID: 33515091   Take Home Messages: Patients with Trisomy 18 and congenital heart disease are offered surgical repair. Cardiac surgical repair improves survival in patients with Trisomy 18. For patients with Trisomy 18 and VSD, PAB followed by intracardiac repair (two-stage approach) offers improved survival.   Commentary from Dr. Luis Quinonez (Boston, MA, USA), section editor of Congenital Heart Surgery Journal Watch: Summary: The authors present a retrospective review of their experience treating 18 patients (2 male, 16 female) with Trisomy 18 and VSD using a two-stage approach (2005-2019). Their approach was placing a PAB as a neonate or infant, followed months later by a complete intracardiac repair with PAB takedown. The gestational age and birth weight were 38 ± 2 weeks and 1861 ± 325 g. Pulmonary artery banding was placed after a median duration of 19.5 (range 6–194) days of life. The age and body weight at the time of intracardiac repair were 18.2 (7.6–50.7) months and 6.0 ± 1.0 kg. There was one hospital death.   Following intracardiac repair, CPR was performed in two patients. Other complications included pneumonia (n = 1), chylothorax (n = 1), pleural effusion (n = 1), and pneumothorax (n = 1). Two patients needed temporary peritoneal dialysis following VSD closure. Follow-up was 24.9 (3.9–173.0) months. The 1- and 5-year survival rates after VSD repair were 88.5% (95% confidence interval, CI 61.4–97.0) and 77.5% (95% CI 42.8–92.8). This survival was significantly better than a comparison group who was palliated with a PAB only. The authors provide details of 3 other cases who underwent complete repair as their first operation, and all died. The authors conclude that “two-stage ICR for large VSDs in patients with T18 improves their life expectancy. Offering palliative surgery during the early neonatal period followed by complete repair after sufficient weight gain is safe and improves survival.”   Comment: Many institutions offer cardiac surgical repair to patients with Trisomy 18. Any ethical discussion surrounding this practice is beyond the scope of this commentary. Neonates with Trisomy 18 and congenital heart disease pose many challenges related to associated comorbidities. They are often small for gestational age; may be premature; and may have associated airway disease, just to name a relevant few. The authors of the paper advocate for a two-stage repair of VSDs beginning with a PAB, followed later by complete intracardiac repair. The justification for such a practice seems mostly related the low weight of the patients in their cohort and the poor experience with complete repair (although only in 3 patients).   The authors have good follow-up survival outcomes using the two-stage approach, although morbidity was significant. Other experienced centers may advocate performing complete intracardiac repair from the outset, arguing that complete repair is feasible in a low weight neonate/infant and the morbidity and mortality of the single procedure is similar or less than a staged approach. The differences in approach (staged vs. complete repair) between centers will most likely be related to the comfort level of the surgeon in performing an intracardiac repair in a low birthweight neonate and the experience of the center in managing these small patients with multiple comorbidities. Regardless of the approach taken, the post-operative care of patients with Trisomy 18 can be challenging. Post-operative morbidities are significant, including the need for tracheostomy and gastrostomy. Whatever approach is taken, staged or not, the treating institution needs to commit expertise and resources to manage patients with Trisomy 18.