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Aim: This study evaluates the risk factors associated with right ventricular (RV) dilation and dysfunction leading to pulmonary valve replacement (PVR) or adverse cardiac events in repaired Tetralogy of Fallot (rToF) patients., Methods: Data from all rToF patients who underwent magnetic resonance imaging (MRI) evaluation at our hospital between February 2007 and September 2020 were collected., Results: Three hundred and forty-two patients (60% males, 42% older than 18 years), with a median age of 16 years (IQR 13-24) at the time of MRI, were included. All patients underwent complete repair at a median age of 8 months (IQR 5-16), while palliation was performed in 56 patients (16%). One hundred and forty-four patients (42%) subsequently received pulmonary valve replacement (PVR). At the multivariate analysis, male gender was an independent predictor for significant RV dilation, RV and left ventricular (LV) dysfunction. Transventricular ventricular septal defect (VSD) closure and previous palliation significantly affected LV function and RV size, respectively. Male gender and the transventricular VSD closure were independent predictors for PVR., Conclusions: Male gender and surgical history (palliation, VSD closure approach) significantly affected the long-term outcomes in rToF patients and should be taken into consideration in the follow-up management and in PVR timing in this patient population.

Objectives: The aim of this study was to identify the predictors of death and of reintervention after mitral valve replacement (MVR) in children., Methods: A single-centre retrospective study was performed including 115 patients under the age of 18 undergoing MVR between 1982 and 2019. For all patients, the ratio of prosthetic valve size (diameter in mm) to weight (kg) at surgery was calculated and long-term result was assessed. The primary outcome was freedom from mitral valve (MV) re-replacement. The composite secondary outcome was freedom from death or transplant., Results: Fifty-four patients had a previous surgical attempt to MV repair. The median age at surgery was 5.5 years (interquartile range 1.21-9.87). Death/transplant-free survival was 77 ± 4% at 5 years and 72 ± 5% at 10 years. Univariate analysis showed a size/weight ratio higher than 2 and age <2 years as significant risk factors for death or transplant. Freedom from MV re-replacement at 5 and 10 years was 90 ± 3% and 72 ± 6%, respectively. Biological prosthesis implanted at first replacement (P = 0.007) and size/weight ratio higher than 2 (P = 0.048) were predictors of reoperation. Significant upsizing (P < 0.0001) of mitral prosthesis was observed at re-replacement., Conclusions: MVR is a viable strategy in children with unrepairable MV disease. Mortality can be predicted based on size/weight ratio and age <2 years. MV re-replacement can be performed with low morbidity and mortality and a larger-size prosthesis can often be placed at the time of redo., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes <15 mm. Based on successful experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, the prosthesis has been modified for surgical valve replacement in pediatric patients with atrioventricular dysfunction with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9-24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P<0.001) with no significant increase in grade of regurgitation. Twelve months after implantation, cumulative incidence analysis indicated that 55% of the patients would be expected to be free from death, heart transplantation, structural valve deterioration, or valve replacement. Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.

We present the surgical implantation in mitral position of the Edwards Sapien 3 valve for prosthetic valve endocarditis in a severely ill child after multiple valve replacements. The procedure was safely performed and provided adequate hemodynamic results in the short term. This report highlights the excellent potential of Sapien 3 valve for the treatment of mitral valve disease in children with a mitral valve annulus of appropriate size. Of note, the use of the Sapien 3 valve should be considered when a bioprosthesis is required at the time of valve re-replacement., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background and Aim of the Study: The study aim was to analyze the safety and longevity of cryopreserved homografts used for primary right ventricular outflow tract reconstruction (RVOTR), and to compare the outcome using either standard or bicuspidalized allografts., Methods: Between February 2000 and September 2014, a total of 53 patients underwent primary RVOTR using either a standard (n = 40) or a bicuspidalized (n = 13) cryopreserved homograft. The median age at surgery was 15.5 months (range: 1-419.06 months), and bodyweight was 8.5 kg (range: 4.1-71 kg). The median standard homograft size was 17.5 mm (range: 10-25 mm), while the median bicuspidalized homograft size was 16 mm (range: 14-22 mm). Follow up was complete in 91.4% of patients, with a median duration of 30.11 months (range: 0.26-161.26 months). Data analysis included primary diagnosis, type of surgery, age at surgery, size of conduit, need for reintervention, and survival. Predetermined primary outcomes were represented by survival and freedom from conduit reintervention., Results: Two patients with standard cryopreserved homograft died. during the early postoperative period (3.7%). No deaths were conduit-related. The five-year survival rate and ten-year freedom from reintervention were 91% (range: 74.7-97.2%) and 53.6% (range: 97-33.2%), respectively. RV-PA conduit replacement was performed in 14 patients (26.4%) at a median interval of 44.5 months (range: 14.93-162.46 months). Among these patients, four children (30.7%) received bicuspidalized homografts, and 10 (25%) received standard homografts. Causes of reintervention were conduit stenosis in six cases (43%), severe homograft valve regurgitation in two (14.2%), conduit stenosis and homograft valve regurgitation in two (14.2%), and stenosis of distal anastomosis involving pulmonary bifurcation in four (28.6%). Univariate analyses showed a longer freedom from reintervention for bicuspidalized compared to standard homograft (p = 0.03)., Conclusion: The results obtained suggested that bicuspidalized homograft performance compares well with that of standard allografts in terms of freedom from reintervention. Bicuspidalized homograft use is strongly indicated for primary RVOTR in small children, when a standard homograft of appropriate size is not available.

Objective: Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared with adults. In order to define the outcome in the second decade after Ross operation in children, a nationwide review of 23 years of experience was undertaken., Methods: 305 children underwent Ross operation in 11 paediatric units between 1990 and 2012. Age at surgery was 9.4±5.7 years, indication aortic stenosis in 103 patients, regurgitation in 109 and mixed lesion in 93. 116 (38%) patients had prior procedures. Root replacement was performed in 201 patients, inclusion cylinder in 14, subcoronary grafting in 17 and Ross-Konno in 73., Results: There were 10 (3.3%) hospital and 12 late deaths (median follow-up 8.7 years). Survival was 93±2% and 89±3% and freedom from any reoperation was 76±3% and 67±6% at 10 and 15 years. 34 children had autograft 37 reoperations (25 replacement, 12 repair): three required transplantation after reoperation. Freedom from autograft reoperation was 86±3% and 75±6% at 10 and 15 years. 32 children had right heart redo procedures, and only 25 (78%) conduit replacements (15-year freedom from replacement, 89±4%). Prior operation (p=0.031), subcoronary implant (p=0.025) and concomitant surgical procedure (p=0.004) were risk factors for left heart reoperation, while infant age (p=0.015) was for right heart. The majority (87%) of late survivors were in NYHA class I, 68% free from medication and six women had pregnancies., Conclusions: Despite low hospital risk and satisfactory late survival, paediatric Ross operation bears substantial valve-related morbidity in the first two decades. Contrary to expectation, autograft reoperation is more common than homograft., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

Objective: This study reviews a single-center experience with the Ross procedure in infants and young children., Methods: From November 1993 to March 2012, 55 children aged less than 17 years underwent a Ross procedure. The patients ranged in age from 2 days to 17 years (median, 5.9 years). Thirteen patients were infants, and 18 patients were preschool children. The predominant indication for the Ross procedure was aortic stenosis. Twenty-seven patients (49%) with left ventricular outflow tract obstruction underwent a modified Ross-Konno procedure. Twenty-five patients (45%) had undergone 40 previous cardiac procedures. Preoperatively, 3 patients showed severe left ventricular dysfunction, with 2 of the patients requiring intubation and inotropic support. Concomitant procedures were performed in 11 patients (20%). Nine patients underwent mitral valve surgery, and 2 patients underwent subaortic membrane resection., Results: Patients were followed up for a median of 66 months (range, 3 months to 17 years). Overall survival at 1, 2, 5, and 10 years was 84.9%. Hospital mortality rate was 13% (7/55 patients). All deaths occurred in neonates or infants, except 1 who was aged less than 4 years. Freedom from reoperation for autograft failure was 100% at 1 year, 96.7% at 5 years, and 73.7% at 10 years. During follow-up, 7 patients required a reoperation on the autograft for dilatation and severe aortic insufficiency. Freedom from reoperation for the right ventricular outflow tract replacement was 56.1% at 10 years., Conclusions: The low rate of autograft failure demonstrates that the Ross procedure is an attractive option for the management of aortic valve disease and complex left ventricular outflow tract obstruction in the pediatric population. However, alternative options must be considered in adolescents and young adults., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

The aim of the study was to evaluate the medium-term results of aortic valve neocuspidalization according to Ozaki compared to Ross procedure for treatment of isolated aortic valve disease in pediatric age. Thirty-eight consecutive patients with congenital or acquired aortic valve disease underwent either Ozaki (n = 22) or Ross (n = 16) operation between 01/2015 and 05/2020. The primary outcome was progression of aortic valve disease and aortic ring and root dimension, whereas secondary outcome was freedom from reintervention or death by type of operation. Median age was 12.4 (8.8–15.8) years and the prevailing lesion was stenosis in 20 cases (52%) and incompetence in 18 (48%). One death occurred in the Ross group in the early postoperative period, while there were no deaths in the Ozaki group. Effective treatment of aortic valve stenosis or regurgitation occurred in both groups and remained stable over a median follow-up of 18.2 (5–32) months. In Ozaki group, 3 patients required aortic valve replacement at 4.9, 3.5, and 33 months, respectively. In Ross group, 1 patient required Melody pulmonary valve replacement, whereas none required aortic valve surgery. Finally, significantly higher aortic transvalvular gradient at follow-up was recorded in Ozaki group compared to Ross group. Overall, there was no significant difference in freedom from reoperation or death between the two groups. The medium-term outcome of Ozaki and Ross in pediatric patients is similar, despite an increased tendency of the former to develop aortic transvalvular gradient in the follow-up. Future larger multicenter studies with longer follow-up are warranted to confirm these results. [ABSTRACT FROM AUTHOR]