Congenital Heart Surgery

Kawashima Procedure: The Impact of Age. Ivanov Y, Buratto E, Ishigami S, Chowdhuri KR, Schulz A, Brizard CP, Konstantinov IE.Ann Thorac Surg. 2023 Aug;116(2):366-372. doi: 10.1016/j.athoracsur.2022.10.015. Epub 2022 Oct 23.PMID: 36288759 Take Home Points: Kawashima operation can be performed with low operative risk and acceptable long-term outcomes CAVV procedure carries a risk for mortality. Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch:  This retrospective, single-center study looked at surgical outcomes of patients who underwent the Kawashima procedure from March 1990 to November 2020 at Royal Children´s Hospital, Melbourne Australia. Thirty patients underwent Kawashima procedure at a median age of 11.7 months (IQR, 4.4-27.4) and a median weight of 8.1 kg (IQR, 5.3-11). Concomitant procedures were done in 13 (43.3%) of 30 patients. Six patients (20%) had antegrade flow after the Kawashima procedure; in 4 of them, it was left intact; 1 patient underwent partial main pulmonary artery closure; another patient underwent tightening of pulmonary artery band. Early mortality was 0%. There were 2 take-downs. Median follow-up was 9.7 years (range, 2 months to 25.8 years). In 53%(n=16), pulmonary arteriovenous malformation (PAVM) developed in a mean time of 3.6 ± 2.4 years after Kawashima procedure; in all these patients, antegrade pulmonary blood flow was absent. Fontan completion was achieved in 25 of 30 patients (86.2%) at a median time of 3.6 years (IQR, 2.5-6.6) after Kawashima. There were 2 deaths (6.8%) after Kawashima procedure but before Fontan completion. Both patients needed AV valve replacement and died of heart failure. Overall freedom from death and transplantation at 5,10, and 20 years after Kawashima was 93.2%, 89.5%, and 67% respectively. Freedom from death and transplantation at 10 years was 100% for children 3 to 6 months of age at the time of Kawashima. During a median follow-up of 9.7 years, patients (23 - 88.5%) were in New York Heart Association functional class I-II; 2 patients (7.7%) were in functional class III; 1 patient (3.8%) was in functional class IV. Comment: This single-center study included 30 patients who underwent Kawashima at a median age of 11.7 months (IQR, 4.4-27.4). In this cohort, 90% of the patients were associated with left isomerisms and 77% had Common Atrioventricular valve (CAVV). Five patients needed CAVV repair at the time of Kawashima, and 2 patients who needed take-down after Kawashima needed AVV replacement. Overall freedom from death and transplantation among patients who underwent AVV repair concomitant to Kawashima procedure was worse than those who did not (p=0.04). Two patients needed a Fontan revision because of the development of PAVM. The Kawashima procedure is indicated for patients with functional single ventricles and azygos/hemi-azygos continuation. This pathology is frequently associated with heterotaxy syndrome UAVSD, bilateral SVC, and DORV. Problematic complications included the progression of CAVV regurgitation after the initial systemic-to-pulmonary shunt was problematic before and after the Kawashima procedure as well as the development of PAVM after the Kawashima procedure. This paper suggests that early Kawashima procedure between 3 to 6 months of age had better results. A longer duration of shunt-dependent physiology might increase the degree of CAVV and trigger systemic ventricular dysfunction. To preserve the systemic ventricular function, early Kawashima procedure is recommended. As patients after Kawashima procedure frequently develop PAVM, early Fontan completion is also recommended. Fontan completion in this study was at a median of 3.6 years. It is younger than previous studies. Many centers now agree that Kawashima procedure can safely be performed in infants. Fontan completion at 2-3 years old might be an optimal strategy. As for the ante-grade PBF, we adopted the strategy of closure of all ante-grade flow at the time of Kawashima and performing swift Fontan completion. It might be better to leave some ante-grade flow if patients have risks for early Fontan completion.

Kawashima Procedure: The Impact of Age. Ivanov Y, Buratto E, Ishigami S, Chowdhuri KR, Schulz A, Brizard CP, Konstantinov IE.Ann Thorac Surg. 2023 Aug;116(2):366-372. doi: 10.1016/j.athoracsur.2022.10.015. Epub 2022 Oct 23.PMID: 36288759 Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch:  Take Home Points: Kawashima operation can be performed with low operative risk and acceptable long-term outcomes CAVV procedure carries a risk for mortality. Summary: This retrospective, single-center study looked at surgical outcomes of patients who underwent the Kawashima procedure from March 1990 to November 2020 at Royal Children´s Hospital. A total of 30 patients underwent Kawashima procedure at a median age of 11.7 months (IQR, 4.4-27.4) and a median weight of 8.1 kg (IQR, 5.3-11). Concomitant procedures were done in 13 (43.3%) of 30 patients. There were 6 of 30 patients (20%) with antegrade flow after the Kawashima procedure; in 4 of them, it was left intact; 1 patient underwent partial main pulmonary artery closure; another patient underwent tightening of pulmonary artery band. Early mortality was 0%. There were 2 takedowns. Median follow-up was 9.7 years (range, 2 months to 25.8 years). In 16 of 30 patients (53%), PAVM developed in a mean time of 3.6 ± 2.4 years after Kawashima procedure; in all these patients, antegrade pulmonary blood flow was absent. Fontan completion was achieved in 25 of 30 patients (86.2%) at a median time of 3.6 years (IQR, 2.5-6.6) after Kawashima. There were 2 deaths (6.8%) after Kawashima procedure but before Fontan completion. Both patients needed AV valve replacement and died of heart failure. Overall freedom from death and transplantation at 5,10, and 20 years after Kawashima was 93.2%, 89.5%, and 67%. Freedom from death and transplantation at 10 years was 100% for children 3 to 6 months of age at the time of Kawashima. During a median follow-up of 9.7 years, most of the patients (23; 88.5%) were in New York Heart Association functional class I-II; 2 patients (7.7%) were in functional class III; 1 patient (3.8%) was in functional class IV. Comment: This single-center study included 30 patients who underwent Kawashima at a median age of 11.7 months (IQR, 4.4-27.4). In this cohort, 90% of the patients were associated with left isomerism and 77% had CAVV. Five patients needed CAVV repair at the time of Kawashima, and 2 patients who needed take-down after Kawashima needed AVV replacement. Overall freedom from death and transplantation among patients who underwent AVV repair concomitant to Kawashima procedure was worse than those who did not (p=0.04). Two patients needed a Fontan revision because of the development of PAVM. As previous studies presented, Kawashima procedure was indicated for patients with functional single ventricles and azygos/hemi-azygos continuation. The patients are frequently associated with heterotaxy syndrome UAVSD, bilateral SVC, and DORV. Progression of CAVV regurgitation after the initial systemic-to-pulmonary shunt was the issue before and after the Kawashima procedure, and the development of PAVM after the Kawashima procedure was another issue. This article augmented that early Kawashima procedure between 3 to 6 months of age had better results. We agree with the authors. Long-duration of shunt-dependent physiology might increase the degree of CAVV and trigger systemic ventricular dysfunction. To preserve the systemic ventricular function, early Kawashima procedure is recommended. As patients after Kawashima procedure frequently develop PAVM, early Fontan completion is also recommended. Fontan completion in this study was at a median of 3.6 years. It is younger than previous studies. We think that Kawashima procedure can be nowadays safely performed in infants. Fontan completion at 2-3 years old might be an optimal strategy. As for the ante-grade PBF, we adopted the strategy of closure of all ante-grade flow at the time of Kawashima and performing swift Fontan completion. It might be better to leave some ante-grade flow if patients have risks for early Fontan completion.

Timing of Balloon Atrial Septostomy in Patients with d-TGA and Association with Birth Location and Patient Outcomes. Thomas C, Yu S, Lowery R, Zampi JD. Pediatr Cardiol. 2023 Aug;44(6):1333-1341. doi: 10.1007/s00246-022-03079-5. Epub 2022 Dec 24.PMID: 36565310 Take Home Points:  Patients with d-TGA transported from an outside institution had a longer median time time from birth to the BAS as compared to those born the authors’ tertiary care center (4.0 hours vs. 14.1 hours). Patients delivered at outside institutions had lower minimum PaO2 and greater frequency of inotropic support, as compared to those born at the authors’ tertiary care center. Neither time to BAS nor location of birth was associated with age at time of arterial switch operation, however a longer time from birth to BAS was associated with longer ICU LOS and total hospital LOS. Commentary from Dr. Arash Salavitabar (Columbus, OH, USA), section editor of Congenital Heart Disease Interventions Journal Watch: Commentary:  The authors report on a retrospective study aimed at evaluating their single-center experience with outcomes in infants with d-transposition of the great arteries (d-TGA) as they relate to timing of balloon atrial septostomy (BAS) and institution of birth. The primary outcome was time from birth to BAS and secondary outcomes included mortality, hospital and ICU length of stay, duration of mechanical ventilation, evidence of pulmonary hypertension, and neurologic abnormalities. Of 217 patients with d-TGA physiology, 96 (44%) patients required emergent or urgent BAS, 69 (72%) of which were prenatally diagnosed. Patients born at outside institutions had a median travel distance of 99 miles (IQR 56, 115) and transport time of 10.3 hours (6.8, 12.0), with a shorter transport time for prenatally diagnosed patients vs. postnatally diagnosed patients born at outside institutions (6.4h vs. 10.7h). The median time from birth to BAS was 4.0 hours in those born at the authors’ center and 14.1 hours when transported from an outside institution. Importantly, patients delivered at outside institutions had lower minimum PaO2 (28.2 mmHg vs. 31.9 mmHg, p=0.02) and greater frequency of inotropic support (48.3% vs. 7.5%, p <0.0001) as compared to those born at the authors’ center. Neither time to BAS nor location of birth was associated with age at time of arterial switch operation, however a longer time from birth to BAS was associated with longer ICU LOS and total hospital LOS. There were no differences in frequency of seizures or abnormal neuroimaging based on time to BAS or birth location. The authors stressed the importance of patients with prenatally diagnosed d-TGA being delivered at a tertiary care center with access to interventional cardiology services to decrease the time to urgent or emergent BAS. Based on this data, this is certainly an important conclusion that has implications on both patient stability and oxygenation, as well as healthcare resource utilization. As the authors admit, more data is needed on neurological sequelae of late BAS in these patients, however the above data has been previously correlated with adverse neurologic outcomes, stressing its significance.

Aortic valve neocuspidization using the Ozaki technique: A meta-analysis of reconstructed patient-level data  Konstantinos S. Mylonas, MD a,#, Panagiotis T. Tasoudis, MS b,#, Dionysios Pavlopoulos, MD, PhD a, Meletios Kanakis, MD, PhD c, George T. Stavridis, MD, FETCS a, and Dimitrios V. Avgerinos, MD, PhD, FACS, FACC Take Home Points: The midterm outcomes of the Ozaki procedure are excellent in terms of hemodynamics, survival, and freedom from reoperation. Commentary from Dr. Shuhua Luo (Chengdu, Sichuan, China), section editor of Congenital Cardiac Surgery Journal Watch: Summary: A total of 22 studies reporting on 1,891 patients that underwent Ozaki reconstruction were included in this meta-analysis. Mean age at the time of surgery was 43.2 ± 24.5 years (65 ± 12.3 years for adult patients and 12.3 ± 3.8 years for pediatric patients). The most common indication was aortic stenosis (46.4%, 95% CI 34.1-58.6). At latest follow-up, peak gradient was 15.7 ± 7.4 mm Hg and only 0.25% (95% CI 0-2.3) had moderate aortic insufficiency. In-hospital mortality was 0.7% (95% CI 0.1-1.7). Late mortality was 1.9% during a mean follow-up of 38.1 ± 23.8 months. One-year, 3-year, and 5-year freedom from reoperation rates were 98.0 %, 97.0 % and 96.5%, respectively. More than half of the reoperations were due to infective endocarditis (51.5%, 95% CI 18.3-84.0). Comment:      Ozaki’s procedure is a promising method to independently reconstruct the three leaflets of the aortic valve using autologous pericardium treated with glutaraldehyde. Most current data on this technique arise from single-center experiences, which are based on small patient populations. The present systematic review includes a total of 1,891 patients making it the largest and most comprehensive data synthesis to date on the Ozaki procedure. Although the results showed promising midterm outcomes of the Ozaki procedure, several questions remained to be answered in the future study. (1) Aortic valve disease can be complicated at multiple levels such as left ventricular outflow tract obstruction(LVOT), aortic stenosis or regurgitation, aortic annulus stenosis or dilatation, and ascending aortic dilatation. In addition, many underlying causes can lead to aortic valve disease including infective endocarditis, rheumatic disease, or auto-immune pathologies. It is unknown whether the Ozaki procedure is a reasonable choice for these different clinical scenarios, as the current meta-analysis showed the majority of patients who underwent the Ozaki procedure had aortic stenosis, and the concomitant procedures such as LVOT muscle resection, aortic annular enlargement, or replacement of ascending aorta were not analyzed in the current study. (2) The results of the Ozaki procedure in pediatric patients were particularly interesting. The mean age and body weight of pediatric patients was 12.3 ± 3.8 years and 42.1 ± 17.9 kg respectively, indicating that the size of patients was near to adult when undergoing Ozaki procedure. The aortic valve disease in young infants or small children posed a significant clinical challenge as the machinal prosthesis replacement was not usually an option due to the small size of the aortic annulus, and the Ross procedure was associated with high mortality. It will be interesting to look at the outcome of the Ozaki procedure in young children. (3) Although the original Ozaki procedure included the tricuspid leaflets reconstruction, some patients only need a single-cusp reconstruction using the Ozaki principle. It remains to know that the percentage of patients who only received a single-cusp reconstruction in the current meta-analysis, may have the potential impact on the midterm outcome.      In summary, the Ozaki procedure is an attractive alternative for aortic valve disease in patients. We are looking forward to more data shed light on the surgical decision-making in various clinical scenarios, including patients’ size, individual detailed anatomy, and underlying causes.

Preliminary Results With a Novel Expanded Polytetrafluoroethylene-based Pulmonary Valved Conduit Christopher W. Baird, Mariana Chavez, Carl L. Backer, Mark E. Galantowicz, and Pedro J. Del Nido Take Home Points: A novel expanded polytetrafluoroethylene-based pulmonary valved conduit was tested with a prospective, multicenter, single-arm study. This preliminary evaluation suggested promising valve function with no thromboembolic, no infectious complications, no valve-related re-interventions, no valve-related adverse events or unexpected findings. Improved right ventricular volumes and encouraging hemodynamic performance were proved by CMRI and through current follow-up. Commentary from Dr. Masamichi Ono (Munich, Germany), section editor of Congenital Heart Surgery Journal Watch:  Summary: This is a prospective, multicenter, single-arm study from centers in the USA, reporting results after the implantation of a novel expanded polytetrafluoroethylene (ePTFE)-based pulmonary valved conduit. A total of 17 patients (52.9% male; median age, 12 years; range, 6 to 17 years) were included. One patient had left coronary compression after the implantation of this conduit and was replaced with a biological valved conduit in the operating room. There has been no mortality and 100% freedom from device-related re-interventions. During follow-up, no evidence of worsening valvular insufficiency/stenosis was observed. No patient developed thrombus, calcification, or endocarditis. Six-month postoperative cardiac magnetic resonance imaging in 11 patients suggests favorable right ventricular remodeling with no significant change in ejection fraction. Comment: Reconstruction of the RVOT and replacement of pulmonary valves in pediatric and adolescent patients with congenital heart diseases have been ongoing challenges, especially given the lack of a durable prosthesis that is adjustable to the patient’s growth needs and is free of infection risks. Current RVOT reconstruction is limited to cryopreserved homografts, decellularized homografts, bioprosthetic valved conduits, bioprosthetic valves, bovine jugular conduits, and hand-sutured ePTFE grafts. All current conduits pose fundamental limitations. ePTFE has good biocompatibility, and its microporous structure impedes cellular penetration and subsequent calcification. Yamagishi, et al reported that hand-made ePTFE valve conduits have shown improved durability and significantly reduced infection rates. This novel ePTFE-based valved conduit was industry-manufactured. The study demonstrated the safety and feasibility of this conduit. In this study, only a 20 mm-sized valved conduit was tested. Further studies with other (smaller or larger) sizes are mandatory to prove the usefulness of this conduit. Long-term durability should be also proved in further follow-up studies. Yamagishi et al developed an ePTFE valved conduit with a fan-shaped configuration consisting of a 0.1-mm-thick ePTFE membrane and bulging sinuses to improve valve mobility. They demonstrated the excellent results of the freedom from reoperation was 98.8% at 5 years and 93.8% at 10 years with a median follow-up period of 4.9 years. This conduit has been developed based on their experiences. Therefore, the preliminary results with a novel ePTFE pulmonary valve conduit are quite promising. Continued surveillance of these patients will be performed to assess the performance of this valve over time, and further studies with small-sized conduits are desirable.