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Objectives:  Patients with congenital heart disease frequently require surgical or percutaneous interventional valve replacement after initial congenital heart defect (CHD) repair. In some of these patients, simultaneous replacement of both semilunar valves is necessary, resulting in increased procedural complexity, morbidity, and mortality. In this study, we analyze the outcomes of simultaneous aortic and pulmonary valve replacements following multiple surgical interventions for CHD., Methods:  This was a retrospective study of 24 patients who after initial repair of CHD underwent single-stage aortic and pulmonary valve replacement at our institution between 2003 and 2021., Results:  The mean age of the patients was 28 ± 13 years; the mean time since the last surgery was 15 ± 11 years. Decellularized valved homografts (DVHs) were used in nine patients, and mechanical valves were implanted in seven others. In eight patients, DVHs, biological, and mechanical valves were implanted in various combinations. The mean cardiopulmonary bypass time was 303 ± 104 minutes, and aortic cross-clamp time was 152 ± 73 minutes. Two patients died at 12 and 16 days postoperatively. At a maximum follow-up time of 17 years (mean 7 ± 5 years), 95% of the surviving patients were categorized as New York Heart Association heart failure class I., Conclusion:  Single-stage aortic and pulmonary valve replacement after initial repair of CHD remains challenging with substantial perioperative mortality (8.3%). Nevertheless, long-term survival and clinical status at the latest follow-up were excellent. The valve type had no relevant impact on the postoperative course. The selection of the valves for implantation should take into account operation-specific factors-in particular reoperability-as well as the patients' wishes., Competing Interests: A.H. holds shares in Corlife oHG, the company providing the patented service of processing decellularized allografts used in this study. All other authors declared no potential conflict of interest with respect to the study, authorship, and publication of this article., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

For decades, bovine jugular vein conduits (BJV) and classic cryopreserved homografts have been the two most widely used options for pulmonary valve replacement (PVR) in congenital heart disease. More recently, decellularized pulmonary homografts (DPH) have provided an alternative avenue for PVR. Matched comparison of patients who received DPH for PVR with patients who received bovine jugular vein conduits (BJV) considering patient age group, type of heart defect, and previous procedures. 319 DPH patients were matched to 319 BJV patients; the mean age of BJV patients was 15.3 (SD 9.5) years versus 19.1 (12.4) years in DPH patients (p = 0.001). The mean conduit diameter was 24.5 (3.5) mm for DPH and 20.3 (2.5) mm for BJV (p < 0.001). There was no difference in survival rates between the two groups after 10 years (97.0 vs. 98.1%, p = 0.45). The rate of freedom from endocarditis was significantly lower for BJV patients (87.1 vs. 96.5%, p = 0.006). Freedom from explantation was significantly lower for BJV at 10 years (81.7 vs. 95.5%, p = 0.001) as well as freedom from any significant degeneration at 10 years (39.6 vs. 65.4%, p < 0.001). 140 Patients, matched for age, heart defect type, prior procedures, and conduit sizes of 20-22 mm (± 2 mm), were compared separately; mean age BJV 8.7 (4.9) and DPH 9.5 (7.3) years (p = n.s.). DPH showed 20% higher freedom from explantation and degeneration in this subgroup (p = 0.232). Decellularized pulmonary homografts exhibit superior 10-year results to bovine jugular vein conduits in PVR., (© 2023. The Author(s).)

Objectives: We evaluated 4384 procedures performed between 1957 and 2018, collected in the National Register for Congenital Heart Defects, conducted on 997 patients with 1823 pulmonary valve replacements (PVRs), including 226 implanted via catheter [transcatheter valve (TCV)]. Main study targets are as follows: TCV benefit, valve type durability, decade-wise treatment changes and procedure frequencies over the lifetime of a PVR patient., Methods: We studied TCV impact on surgical valve replacement (via Kaplan-Meier); pulmonary valve type-specific performance (Kaplan-Meier and Cox regressions with age group as stratification or ordinary variable); procedure interval changes over the decades (Kaplan-Meier); procedure load, i.e. frequency of any procedure/surgical PVR/interventional or surgical PVR by patient age (multistate analyses)., Results: TCV performance was equivalent to surgical PVRs and extended durability significantly. Homografts were most durable; Contegras lasted comparably less in older; and Hancock devices lasted less in younger patients. Matrix P-valves showed poorer performance. Age group stratification improves the precision of valve-specific explantation hazard estimations. The current median interval between procedures is 2.6 years; it became significantly shorter in most age groups below 40 years. At 30 years, 80% of patients had undergone ≥3 procedures, 20% ≥3 surgical PVRs and 42% ≥3 surgical or interventional PVRs., Conclusions: TCVs doubled freedom from explantation of conventional valves. Homografts' age group-specific explantation hazard ratio was lowest; Matrix P's hazard ratio was highest. Age-stratified Cox regressions improve the precision of prosthesis durability evaluations. The median time between procedures for PVR patients shortened significantly to 2.6 years. At 30 years, 42% had ≥3 PVRs., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Aortic root dilatation is frequently observed in patients with congenital heart defects (CHD), but has received little attention in terms of developing a best practice approach for treatment. In this study, we analysed our experience with aortic valve-sparing root replacement in patients following previous operations to repair CHD., Methods: In this study, we included 7 patients with a history of previous surgery for CHD who underwent aortic valve-sparing operations. The underlying initial defects were tetralogy of Fallot (n = 3), transposition of great arteries (n = 2), coarctation of the aorta (n = 1), and pulmonary atresia with ventricle septum defect (n = 1). The patients' age ranged from 20 to 40 years (mean age 31 ± 6 years)., Results: David reimplantation was performed in 6 patients and a Yacoub remodelling procedure was performed in 1 patient. Four patients underwent simultaneous pulmonary valve replacement. The mean interval between the corrective procedure for CHD and the aortic valve-sparing surgery was 26 ± 3 years. There was no operative or late mortality. The patient with transposition of great arteries following an arterial switch operation was re-operated 25 months after the valve-sparing procedure due to severe aortic regurgitation. In all other patients, the aortic valve regurgitation was mild or negligible at the latest follow-up (mean 8.7 years, range 2.1-15.1 years)., Conclusions: Valve-sparing aortic root replacement resulted in good aortic valve function during the first decade of observation in 6 of 7 patients. This approach can offer a viable alternative to root replacement with mechanical or biological prostheses in selected patients following CHD repair., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Decellularized homograft valves (DHVs) have shown promising clinical results, particularly in the treatment of congenital heart disease. However, DHV appears to elicit an immune response in a subset of young patients, indicated by early valve degeneration. As the decellularization process is quality controlled for each DHV, we hypothesized that there may be residual immunogenicity within the extracellular matrix of DHV., Methods: A semi-quantitative dot blot analysis was established to screen for preformed recipient antibodies using secondary anti-human antibodies. Fifteen DHV samples (7 aortic, 8 pulmonary) were solubilized and exposed to serum from 20 healthy controls., Results: The sera from young controls (n = 10, 18-25 years) showed significantly stronger binding of preformed antibodies than sera from older individuals (n = 10, 48-73 years). The difference between the means of arbitrary units was 15.1 ± 6.5 (P = 0.0315). There was high intraindividual variance in the mean amounts of arbitrary units of antibody binding with some healthy controls showing >10 times higher antibody binding towards 2 different DHV. The amount of preformed antibodies bound to DHVs was higher in aortic than in pulmonary DHVs. The mean number of antibody binding (in arbitrary units) was 17.2 ± 4.5 in aortic and 14.5 ± 4.7 in pulmonary DHV (P = 0.27). The amount of preformed antibodies bound to pulmonary DHVs was statistically significantly higher in the sera of healthy males (n = 10) than in the sera of healthy females (n = 10). The mean number of arbitrary units was 17.2 ± 4.2 in male and 11.7 ± 5.3 in female sera (P = 0.036). Antibody binding to aortic DHV was also higher in males, but not significant (18.8 ± 5.0 vs 15.6 ± 4.0). Blood group (ABO) incompatibility between the serum from controls and DHV showed no impact on antibody binding, and there was no age-related impact among DHV donors., Conclusions: Residual immunogenicity of decellularized homografts appears to exist despite almost complete cell removal. The established dot blot method allows a semi-quantitative assessment of the individual immune response towards extracellular DHV components and potentially the possibility of preoperative homograft matching., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Objectives: Patients with complex congenital heart disease often require multiple reoperations, resulting in increased rates of operative morbidity and mortality. Decellularized heart valves (DHVs) have led to reduced reoperation rates compared with current other valve substitutes when used for pulmonary valve replacement and have also shown very auspicious early results in aortic valve replacement. The aim of the work was to analyse the outcome of a single-stage decellularized valve implantation in the aortic and pulmonary position., Methods: A prospective follow-up of all patients who received a single-stage double semilunar valve replacement using DHV at our institution., Results: Since 2011, 5 patients underwent combined semilunar valve replacement with DHV at our institution: two following a Ross procedure (31-year-old man and 38-year-old woman) and 3 after repair of the truncus arteriosus communis (2-year-old boy and 11-year-old and 16-year-old girls). All patients had undergone previous surgery. The Ross patients had preceding valve procedures, and the patients with truncus arteriosus communis had undergone 1 repair and subsequent operative procedures. Despite challenging operations (median bypass time 346 min, range 275-477 min; median cross-clamp time 229 min, range 140-307 min), there was no perioperative mortality or reoperations. Four of the patients were extubated within 24 h, and the other patient was extubated on postoperative day 2. During follow-up, a good semilunar valve and biventricular heart function was present in all 5 patients, and the New York Heart Association functional class was I for all the patients at the time of their latest follow-up (median 31 months, range 8-82 months). The mean echocardiographic gradient of decellularized aortic homografts was 5.4 ± 3.2 mmHg and 11.6 ± 4.2 mmHg for the decellularized pulmonary homografts. Valvular regurgitation was 0 or 0-I for all DHVs., Conclusions: A single-stage double semilunar valve replacement with DHV has shown promising early results in these 5 very complex cases, providing an additional surgical option after multiple preceding valve procedures in young patients.

Background: This study we evaluate our results for surgical treatment of thoracic aortic aneurysms in patients with congenital heart disease., Patients and Methods: Fifty patients aged between 12 and 71 years were treated for 51 thoracic aortic aneurysms. Forty-four of the patients developed aneurysms in the ascending aorta and seven developed aneurysms in the descending aorta. The underlying diseases for ascending aortic aneurysms included 16 congenital aortic stenosis, 16 bicuspid aortic valves, 4 transposition of the great arteries, 3 tetralogy of Fallot, 2 truncus arteriosus communis, and 3 other diseases. Patients with connective tissue disorders were excluded. All descending aortic aneurysms developed late after coarctation repair. The time interval between the correction and aneurysm operations was 15 years in ascending aneurysms and 28 years in descending aneurysms., Results: The operative procedures for 44 ascending aortic aneurysms included 38 ascending aortic replacements, with or without aortic valve replacement (including 31 conduits), and 6 David operations. Graft replacement was undertaken for the seven descending aortic aneurysms. There were two hospital deaths. Forty-eight hospital survivors were in New York Heart Association functional class II or less at follow-up, which was conducted up to a maximum of 8 years after the procedure. All patients were free from reoperation and thromboembolic events. Aortic valve function was good in all six patients after the valve-sparing operation., Conclusion: Thoracic aortic aneurysms in patients with congenital heart disease is highly associated with bicuspid aortic valve and aortic coarctation. For ascending aortic aneurysms, conduit replacement is the method of choice. The David procedure provides good results in selected patients. For descending aortic aneurysms, graft replacement is the preferred procedure. The relatively late development of thoracic aortic aneurysms indicates that long-term follow-up in patients with congenital heart disease, especially in patients with bicuspid aortic valve and aortic coarctation, is essential., (Georg Thieme Verlag KG Stuttgart · New York.)