Paediatric aortic valve replacement using decellularized allografts: a multicentre update following 143 implantations and five-year mean follow-up.

OBJECTIVES: Decellularized aortic homografts (DAH) were introduced in 2008 as a further option for paediatric aortic valve replacement (AVR). METHODS: Prospective, multicentre follow-up of all paediatric patients receiving DAH for AVR in 8 European centres. RESULTS: A total of 143 DAH were implanted between February 2008 and February 2023 in 137 children (106 male, 74%) with a median age of 10.8 years (interquartile range 6.6-14.6). Eighty-four (59%) had undergone previous cardiac operations and 24 (17%) had undergone previous AVR. The median implanted DAH diameter was 21 mm (interquartile range 19-23). The median operation duration was 348 min (227-439) with a median cardiopulmonary bypass time of 212 min (171-257) and a median cross-clamp time of 135 min (113-164). After a median follow-up of 5.3 years (3.3-7.2, max. 15.2 years), the primary efficacy end-points peak gradient (median 14 mmHg, 9-28) and regurgitation (median 0.5, interquartile range 0-1, grade 0-3) showed good results but an increase over time. Freedom from death/explantation/endocarditis/bleeding/thromboembolism at 5 years were 97.8 ± 1.2/88.7 ± 3.3/99.1 ± 0.9/100 and 99.2 ± 0.8%, respectively. Freedom from death/explantation/endocarditis/bleeding/thromboembolism at 10 years were 96.3 ± 1.9/67.1 ± 8.0/93.6 ± 3.9/98.6 ± 1.4 and 86.9 ± 11.6%, respectively. In total, 21 DAH were explanted. Seven were replaced by a mechanical AVR, 1 Ross operation was performed and a re-do DAH was implanted in 13 patients with no redo mortality. The calculated expected adverse events were lower for DAH compared to cryopreserved homograft patients (mean age 8.4 years), and in the same range as for Ross patients (9.2 years) and mechanical AVR (13.0 years). CONCLUSIONS: This large-scale prospective analysis demonstrates excellent mid-term survival using DAH with adverse event rates comparable to paediatric Ross procedures.

Matched comparison of decellularized homografts and bovine jugular vein conduits for pulmonary valve replacement in congenital heart disease.

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.

Simultaneous Aortic and Pulmonary Valve Replacement in Repaired Congenital Heart Disease.

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. CONCLUSIONS: 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.

Idiopathic Aneurysm of the Aortic Arch in an Infant.

Congenital aortic aneurysms are rare disorders, usually associated with genetic aortic syndromes. Here, we describe the case of an idiopathic aortic arch aneurysm which had been diagnosed prenatally by fetal echocardiography. The diagnosis was confirmed after birth in the neonatal period and successful surgical resection of the aneurysm was performed at the age of 3 months. The idiopathic etiology of the aneurysm, its localization, and the early surgical resection render this case very unusual.

4D-flow cardiac magnetic resonance imaging after aortic root replacement with long-valved decellularized aortic homografts: comparison to valve-sparing aortic root replacement and healthy controls.

OBJECTIVES: Long-valved decellularized aortic homografts (DAH) may be used in young patients to treat aortic valve disease associated with aortic root dilatation, thereby eliminating the need for prosthetic material and anticoagulation. METHODS: Thirty-three male subjects in 3 equally sized cohorts were compared: patients following DAH implantation with a median age of 29 years [interquartile range (IQR) 27.5-37.5], patients post-valve-sparing aortic root replacement (VSARR), median 44 years (IQR 31.5-49) and healthy controls, median 33 years (IQR 28-40, P = 0.228). Time-resolved three-dimensional phase-contrast cardiac magnetic resonance imaging was performed to assess maximum blood flow velocity, pulse wave velocity, mechanical energy loss (EL), wall shear stress and flow patterns (vorticity, eccentricity, helicity) in 5 different planes of the aorta. RESULTS: The mean time between surgery and cardiovascular magnetic resonance was 2.56 ± 2.0 years in DAH vs 2.67 ± 2.1 in VSARR, P = 0.500. No significant differences in maximum velocity and pulse wave velocity were found between healthy controls and DAH across all planes. Velocity in the proximal aorta was significantly higher in VSARR (182.91 ± 53.91 cm/s, P = 0.032) compared with healthy controls. EL was significantly higher in VSARR in the proximal aorta with 1.85 mW (IQR 1.39-2.95) compared with healthy controls, 1.06 mW (0.91-1.22, P = 0.016), as well as in the entire thoracic aorta. In contrast, there was no significant EL in DAH in the proximal, 1.27 m/W (0.92-1.53, P = 0.296), as well as in the thoracic aorta, 7.7 m/W (5.25-9.90, P = 0.114), compared with healthy controls. There were no significant differences in wall shear stress parameters for all 5 regions of the thoracic aorta between the 3 groups. DAH patients, however, showed more vorticity, helicity and eccentricity in the ascending aorta compared with healthy controls (P < 0.019). CONCLUSIONS: Decellularized long aortic homografts exhibit near to normal haemodynamic parameters 2.5 years postoperatively compared with healthy controls and VSARR.

Pediatric urgent heart transplantation with age or weight mismatched donors: Reducing waiting time by enlarging donor criteria.

BACKGROUND: Despite considerable progress in heart transplantation, pediatric waiting list mortality is still high, and often patients do not have enough time to wait. We hypothesized that extending the donor criteria regarding age and weight mismatch does not significantly affect the early follow-up. METHODS: We retrospectively analyzed our pediatric heart transplantation patients operated on from 2014 to 2020 for high (>3.0) or low (<0.6) donor-recipient weight ratio (DRWR) or chronological age mismatches (donor organ >5 years older than recipient age). This patient cohort constituted "mismatched heart transplantations" (mHTX). We compared mHTX preoperative status, postoperative course, 1-year survival, and early clinical follow-up to standard pediatric heart transplantations (sHTX). RESULTS: We performed 20 pediatric heart transplantations-10 mHTX and 10 sHTX. The minimum DRWR was 0.44, the maximum was 5.60, and the maximum age mismatch was 42.6 years. Median days in the intensive care unit (p = .436) and time-to-first-rejection episode (p = .925) were comparable. Nine patients in each group were alive after 1 year, two patients were operated within 1 year of follow-up. One mHTX patient developed cardiac allograft vasculopathy after 15 months and died 648 days after transplantation (p = .237). All other patients were alive at the end of follow-up and in good clinical conditions (median follow-up for mHTX was 732.5 days, 1149.5 days for sHTX). CONCLUSION: Postoperative course and early follow-up after mHTX were comparable to sHTX. In urgent clinical situations, extended donor criteria may be considered an additional option for pediatric heart transplantation.

Valve-sparing aortic root replacement in adult patients with congenital heart disease.

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.

Three-step preoperative sequential planning for pulmonary valve replacement in repaired tetralogy of Fallot using computed tomography.

OBJECTIVES: Our goal was to compare results between a standard computed tomography (CT)-based strategy, the 'three-step preoperative sequential planning' (3-step PSP), for pulmonary valve replacement in repaired tetralogy of Fallot versus a conventional planning approach. METHODS: We carried out a retrospective study with unmatched and matched groups. The 3-step PSP comprised the planning of mediastinal re-entry, cannulation for cardiopulmonary bypass (CPB) and the main procedure, using standard 3-dimensional videos. Operative times (skin incision to CPB, CPB time, end of CPB to skin closure and cross-clamp time) as well as postoperative length of stay and in-hospital mortality were compared. RESULTS: Eighty-two patients (49% classical tetralogy of Fallot) underwent an operation (85% with pulmonary homograft) with 1.22% in-hospital mortality. The 3-step PSP (n = 14) and the conventional planning (n = 68) groups were compared. There were no statistically significant differences in the preoperative characteristics. Differences were observed in the total operative time (P = 0.009), skin incision to CPB (P = 0.034) and cross-clamp times (74 ± 33 vs 108 ± 47 min; P = 0.006), favouring the 3-step PSP group. Eight matched pairs were compared showing differences in the total operative time (263 ± 44 vs 360 ± 66 min; P = 0.008), CPB time (123 ± 34 vs 190 ± 43 min; P = 0.008) and postoperative length of stay (P = 0.031), favouring the 3-step PSP group. CONCLUSIONS: In patients with repaired tetralogy of Fallot undergoing pulmonary valve replacement, preoperative planning using a standard CT-based strategy, the 3-step PSP, is associated with shorter operative times and shorter postoperative length of stay.

Double semilunar valve replacement in complex congenital heart disease using decellularized homografts.

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.

First results of HeartWare left ventricular assist device implantation with tunnelling of the outflow graft through the transverse sinus.

OBJECTIVES: The number of left ventricular assist device (LVAD) implants for the treatment of advanced heart failure is increasing tremendously. The main therapeutic goal of this operation is to provide a bridge to transplant for patients awaiting a donor heart. In 2011, we developed a novel, minimally invasive surgical technique for LVAD implantation. To avoid possible outflow graft injuries during redo sternotomies as well as to provide a more physiological outflow towards the aortic arch, a further modification of this approach was made with outflow graft tunnelling through the transverse sinus. METHODS: More than 500 LVADs were implanted at Hannover Medical School between 2008 and 2015. From September 2012 to December 2015, we used this novel technique in 17 consecutive bridge-to-transplant patients and analysed their clinical outcomes retrospectively. Baseline characteristics were obtained for all patients, and outcome data were collected from a review of electronic medical records. Subsequently, we compared the results of a data analysis of a group of 86 patients with a minimally invasive left thoracotomy LVAD implantation with the results from patients in a control group receiving a conventional outflow graft placement between May 2009 and January 2015. RESULTS: Our data demonstrate that the outcomes and adverse events of the operated group were comparable to those of the control group. Three patients of the study group died within the first year (3 of 17, 18%); survival to 3 years was 84%. The adverse events were similar in both groups. The study group had 3 ischaemic strokes (18%) and 1 LVAD thrombosis (6%). Five patients had LVAD thrombosis (5 of 86, 6%) and 6 in the control group had ischaemic strokes (6 of 86, 7%). The average in-hospital stay was 35.4 days for the study group and 27.4 days for the control group. Three patients from the study group and 5 from the control group had cardiac transplants. The average time until cannulation and start of extracorporeal circulation was 56 min in the study group and 96 min in the control group. Re-thoracotomy was necessary in 2 patients from the control group, whereas none was necessary in the study group. CONCLUSIONS: LVAD implantation with outflow graft tunnelling through the transverse sinus is an innovative technique to prevent outflow graft damage in case of cardiac resternotomy. The results of this study show that there are no significant differences in pump speed or flow, adverse events or patient outcomes compared with the standard implant techniques. The theoretical benefits of this novel technique are the reduced risk in redo cases and the physiological direction of blood flow. Consequently, this procedure might be particularly suitable for younger patients who received an LVAD as a bridge-to-transplant option.

Prevalence of Intellectual Disabilities and Epilepsy in Different Forms of Spastic Cerebral Palsy in Adults.

BACKGROUND: Spastic cerebral palsy may be interconnected with other neurodevelopmental disorders such as intellectual disabilities, and epilepsy. Brain synaptic plasticity and successful restorative rehabilitation may also contribute to diminish neurological deficit of patients having cerebral palsy. The aim of this study was to investigate the prevalence of intellectual disabilities and epilepsy in adult patients with different forms of spastic cerebral palsy and to find out correlation between the severity level of intellectual disabilities and epilepsy. SUBJECTS AND METHODS: Adults diagnosed with different forms of spastic cerebral palsy were analyzed during a three-month period. The investigated features were: gender and age; form of cerebral palsy; the prevalence of intellectual disabilities and epilepsy. Intellectual disabilities were divided into 4 severity levels. The correlation between the severity level of intellectual disabilities and epilepsy was statistically analyzed. RESULTS: Intellectual disability was present in 55% of patients diagnosed with spastic cerebral palsy. Epilepsy was present in 36% of such patients. It was recorded in 51.1% of quadriplegic, 21.9% of diplegic, and 19.2% of hemiplegic patients. Intellectual disability was present in 73.8% of quadriplegic, 31.3% of diplegic, and 53.8% of hemiplegic patients. The statistically significant correlation existed between the severe intellectual disability and epilepsy. CONCLUSIONS: Intellectual disabilities and epilepsy most frequently occurred in patients with most severe forms of spastic cerebral palsy. Epilepsy is strongly correlated to the severity level of intellectual disability. Such patients require additional special modes of treatment and restorative rehabilitation to improve the functional outcome.