"Quality of life" analysis in the long-term follow-up after "Fontan" palliation for CHDs-a single-centre experience.

BACKGROUND: Complex CHDs are life threatening, and surgical treatment is needed for survival. Fontan palliation led to a significant increase in survival rates during the last decades. Consequently, quality of life became more essential. While a reduced quality of life compared to healthy children has been reported, detailed knowledge about individual quality of life and particular areas is lacking. Furthermore, the effect of different risk factors on quality of life is only rarely evaluated. METHOD AND RESULTS: Database of the department for pediatric cardiology, Heart Center Leipzig, was screened for children after total cavopulmonary connection palliation. n = 39 patients were included in the study, the outcome after total cavopulmonary connection was analysed in detail and quality of life data were collected and analysed using the standardised questionnaire "Pediatric quality of life inventory", version 4.0. We compared the total health score of our patients to the mean score of healthy children in the literature. The mean follow-up time was 6.4 ± 3.2 years, the overall survival was 100% after maximal follow-up time of 11.1 years. We could not find any age or gender dependence, nor an influence of age at total cavopulmonary connection on the later quality of life. Yet, patients with three-staged surgery exhibited a worse quality of life than patients with two-staged palliation. Late complications might influence quality of life, but patient number is too small, to find universal results. CONCLUSION: The total cavopulmonary connection palliation affects physical and psychological quality of life as well as cardiac health independently from age and gender. More patients and longer observation should be examined to confirm the results.

Long-Term Results Following Combined Repair of Atrioventricular Septal Defect with Tetralogy of Fallot.

Atrioventricular septal defect (AVSD) in association with tetralogy of Fallot (TOF) is a rare and complex congenital cardiac malformation. We report our institutional experience and outcomes following surgical correction over a 20-year period. Patients who underwent combined surgical AVSD and TOF correction between October 2001 and February 2020 were included for analysis. All patients underwent primary repair. The study data were prospectively collected and retrospectively analyzed. Primary outcomes were in-hospital mortality and long-term freedom from reoperation. During the study period, a total of 10 consecutive patients underwent combined surgical AVSD and TOF correction. Median age at operation was 307 days (IQR 228-457) and median weight was 7.7 kg (IQR 6.7-9.5). Down Syndrome was present in six of the patients. In-hospital mortality was 0%. One patient required re-exploration due to bleeding. Median follow-up was 11 years (IQR 11 months -16 years). There was one case of reoperation due to significant residual ventricular septal defect after 2 months. None of the patients died during follow-up. Combined primary AVSD and TOF repair can be performed with low early mortality and morbidity, as well as a high long-term freedom from reoperation.

Complex cases of acquired pulmonary vein stenosis after radiofrequency ablation: is surgical repair an option?

AIMS: Results of catheter based interventional treatment for pulmonary vein stenosis (PVS) following radiofrequency ablation (RFA) for atrial fibrillation remain suboptimal. Surgical repair may represent an alternative therapy, though long-term results have not been thoroughly investigated. METHODS AND RESULTS: We retrospectively assessed all patients in our centre undergoing surgical repair for radiofrequency-induced PVS. Data regarding surgical technique, clinical outcome, and rate of pulmonary vein (PV) restenosis were collected and analysed. Between 2004 and 2016, the rate for PVS resulting from RFA for atrial fibrillation in our institution was 0.79% (76/9633). During this period, five male patients with multiple PVS (3 ± 1) underwent surgical repair of a total of 13 symptomatic PVS. Surgery was performed in a standard setting under cardiopulmonary bypass. Stenotic veins were incised longitudinally followed by a patch augmentation plasty using either bovine pericard (n = 7) or polytetrafluoroethylene (PTFE) patches (n = 5). Localization of incision was on the anterior side of the PV only (n = 8) or on both the anterior and posterior sides (n = 4). In one PVS lesion, mechanical dilatation was sufficient. Long-term follow-up after 60 ± 69 months revealed an average restenosis rate of 38%. Restenosis was defined as narrowing >70%. All patients reported clinical improvement of symptoms at follow-up. CONCLUSION: Even in the era of wide circumferential lesions, PVS still occurs. While surgical PV patch plasty represents a valuable treatment option, restenosis remains an issue during follow-up. Nevertheless, surgical repair achieves highly acceptable long-term results for RFA-acquired PVS. Hence, it should be routinely discussed as a therapeutic option in cases with multiple PVS.

Symptomatic myocardial bridging: a frequently occurring coronary variation can cause severe myocardial ischaemia in affected children with underlying cardiac conditions.

Myocardial bridging is a congenital coronary artery anomaly in which the coronary artery has a partly "tunnelled" intramyocardial course. This tunnelling leads to compression of the affected vessel segment during ventricular systole. It is considered to be a benign variation of the norm in about 25% of the population caused by an aberrancy of embryologic coronary development. The bridging is also thought to cause severe cardiac conditions in a few of those affected. The series of six young patients presented here is the largest series so far to report on symptomatic myocardial bridging in children with different underlying heart diseases. All patients recently presented to our centre with signs of myocardial ischaemia. They subsequently underwent coronary angiography, which revealed myocardial bridging of the ramus interventricularis anterior. In all patients, therapy with ß blockers was started to reduce heart rate and myocardial contractility. ß Blocker treatment was also given in order to prolong diastole and improve coronary artery blood flow. Two patients underwent surgical exposure of the involved coronary segment: a 2-year-old boy because of recurrent, severe myocardial ischaemia in combination with a reduction of general health, changes in ST-segments, and the presence of a dilative cardiomyopathy; and a 13-year-old girl because of evidence of myocardial ischaemia during exercise testing after surviving sudden cardiac death. Surgery was successful and recovery was complete and uneventful. The presented series shows that myocardial bridging can be symptomatic and may require urgent treatment and even surgical intervention in early childhood in rare cases.

First successful repair of an aortico-to-right ventricular tunnel (ARVT) in d-transposition of the great arteries with aortic valve atresia and ventricular septal defect.

The presented case reports on the first successful complex biventricular repair in a neonate with an aortico-to-right ventricular tunnel and dextrotransposition of the great arteries complicated by aortic atresia.

Long-term outcomes following mitral valve replacement in children at heart center Leipzig: a 20-year analysis.

BACKGROUND: Although mitral valve repair is the preferred surgical strategy in children with mitral valve disease, there are cases of irreparable severe dysplastic valves that require mitral valve replacement. The aim of this study is to analyze long-term outcomes following mitral valve replacement in children in a tertiary referral center. METHODS: A total of 41 consecutive patients underwent mitral valve replacement between February 2001 and February 2021. The study data was prospectively collected and retrospectively analyzed. Primary outcomes were in-hospital mortality, long-term survival, and long-term freedom from reoperation. RESULTS: Median age at operation was 23 months (IQR 5-93), median weight was 11.3 kg (IQR 4.8-19.4 kg). One (2.4%) patient died within the first 30 postoperative days. In-hospital mortality was 4.9%. Four (9.8%) patients required re-exploration for bleeding, and 2 (4.9%) patients needed extracorporeal life support. Median follow-up was 11 years (IQR 11 months - 16 years). Long-term freedom from re-operation after 1, 5, 10 and 15 years was 97.1%, 93.7%, 61.8% and 42.5%, respectively. Long-term survival after 1, 5, 10 and 15 years was 89.9%, 87%, 87% and 80.8%, respectively. CONCLUSION: If MV repair is not feasible, MV replacement offers a good surgical alternative for pediatric patients with MV disease. It provides good early- and long-term outcomes.

Early and long-term outcomes following redo mitral valve surgery in patients with prior minimally invasive mitral valve surgery.

OBJECTIVES: The frequency of minimally invasive mitral valve surgery (MVS) has steadily increased over the last decades and therefore surgeons are now encountering an increasing number of patients requiring mitral valve (MV) reoperations post-minimally invasive MVS. The aim of this study was to analyse the early postoperative outcomes and the long-term survival in patients who undergo reoperative MVS following previous minimally invasive surgery. METHODS: Patients who underwent redo MVS following prior minimally invasive MVS between January 2002 and December 2021 were included in our analysis. Study data were prospectively collected and retrospectively analysed. The primary outcomes were 30-day mortality and long-term survival. RESULTS: Among the 187 included patients, 34 (18.2%) underwent repeat MV repair and 153 (81.8%) MV replacement. The median age was 66 years (interquartile range 56-74) and 80 (42.8%) patients were female. Redo MVS was performed through median sternotomy in 169 patients (90.4%). A total of 77 (41.2%) patients had additional concomitant procedures. The median intensive care unit stay was 1 day (1-5). The 30-day mortality was 6.4% (12/187). Estimated survival at 5 and 12 years was 61.8% and 38.3%, respectively. Preoperative stroke (hazard ratio 3.28, 95% confidence interval 1.37-7.85, P = 0.007) as well as infective endocarditis (hazard ratio 1.85; 95% confidence interval 1.09-3.11, P = 0.021) were independent predictors of long-term mortality. CONCLUSIONS: Redo MVS following prior minimally invasive MVS can be performed safely with low early perioperative mortality and acceptable long-term survival. Preoperative stroke, infective endocarditis and concomitant tricuspid valve surgery are independent predictors of long-term mortality.

Gender differences in mitral valve surgery.

BACKGROUND: Knowledge regarding gender-specific mitral valve (MV) pathology and postoperative outcome is rare. We herein describe a single-center experience focusing on gender differences in MV surgery. MATERIALS AND METHODS: A total of 3,761 patients underwent minimal invasive MV surgery at our institution between 1999 and 2011. Demographic data, pre-, intra-, and postoperative characteristics have been collected, including details on MV pathology and surgical technique. Patient data have been analyzed with consideration of gender-specific differences. RESULTS: The cohort consisted of 2,124 male (56.5%; 58.8 ± 12.5 years) and 1,637 female (43.5%; 64.5 ± 13 years) patients. Mitral regurgitation was observed equally in women (91.3%) and men (92.4%). Additional MV stenosis has been diagnosed in 2.7% of men but in 13.9% of women (p < 0.001). Calcification of the posterior MV leaflet showed a similar trend: 20.1% in women compared with 6.5% in men. Prolapse of the posterior leaflet was present predominantly in men with 63.1 versus 35.7% in women (p < 0.001). Distinct MV repair differences were retrospectively detected between genders: posterior mitral leaflet resection was performed in 17.9% of men versus 10.1% of women; posterior mitral leaflet chordae replacement was performed in 39.3% of men compared with 20.4% of women. Prosthetic MV replacement was necessary in 26.8% of women compared with only 10.7% of men. Concomitant tricuspid valve surgery was mostly performed in women (14.4 versus 8.2%). Male patients showed a significant better postoperative long-term survival than females, with 96, 89, and 72% compared with 92, 82, and 58% after 1, 5, and 10 years, respectively (p < 0.0001). CONCLUSION: Substantial gender-specific differences regarding MV pathology, operative strategy, and long-term outcome are present that need to be addressed in clinical practice.

Long-term results following atrioventricular septal defect repair.

BACKGROUND: Atrioventricular septal defects (AVSD) represent 4-7% of congenital cardiac malformations. Definitive early repair is favored over prior pulmonary artery banding and delayed definitive repair in many centers. The aim of this study was to analyze long-term outcomes following AVSD repair over a 21-year period. METHODS: A total of 202 consecutive patients underwent surgical AVSD correction between June 1999 and December 2020. Surgery was performed using the double-patch technique. The study data were prospectively collected and retrospectively analyzed. Primary outcomes were In-hospital mortality and overall long-term freedom from reoperation. RESULTS: Median age at operation was 120 days (IQR 94-150), median weight was 5.0 kg (4.2-5.3). None of the patients died within the first 30 postoperative days. In-hospital mortality was 0.5% (1/202 patients). Median follow-up was 57 months (11-121). Overall freedom from reoperation at 5, 10 and 15 years was 91.8%, 86.9% and 86.9%, respectively. CONCLUSION: AVSD repair with the double-patch technique is a safe and effective procedure with good early postoperative outcomes and low long-term reoperation rates.

Anti-oxidative or anti-inflammatory additives reduce ischemia/reperfusions injury in an animal model of cardiopulmonary bypass.

Severe inborn cardiac malformations are typically corrected in cardioplegia, with a cardio-pulmonary bypass (CPB) taking over body circulation. During the operation the arrested hearts are subjected to a global ischemia/reperfusion injury. Although the applied cardioplegic solutions have a certain protective effect, application of additional substances to reduce cardiac damage are of interest.18 domestic piglets (10-15 kg) were subjected to a 90 min CPB and a 120 min reperfusion phase without or with the application of epigallocatechin-3-gallate (10 mg/kg body weight) or minocycline (4 mg/kg body weight), with both drugs given before and after CPB. 18 additional sham-operated piglets without or with epigallocatechin-3-gallate or minocycline served as controls. In total 36 piglets were analyzed (3 CPB-groups and 3 control groups without or with epigallocatechin-3-gallate or minocycline respectively; 6 piglets per group). Hemodynamic and blood parameters and ATP-measurements were assessed. Moreover, a histological evaluation of the heart muscle was performed. RESULTS: Piglets of the CPB-group needed more catecholamine support to achieve sufficient blood pressure. Ejection fraction and cardiac output were not different between the 6 groups. However, cardiac ATP-levels and blood lactate were significantly lower and creatine kinase was significantly higher in the three CPB-groups. Markers of apoptosis, hypoxia, nitrosative and oxidative stress were significantly elevated in hearts of the CPB-group. Nevertheless, addition of epigallocatechin-3-gallate or minocycline significantly reduced markers of myocardial damage. Noteworthy, EGCG was more effective in reducing markers of hypoxia, whereas minocycline more efficiently decreased inflammation. CONCLUSIONS: While epigallocatechin-3-gallate or minocycline did not improve cardiac hemodynamics, markers of myocardial damage were significantly lower in the CPB-groups with epigallocatechin-3-gallate or minocycline supplementation.

Rescue surgery for neonate with huge rhabdomyoma and left outflow obstruction.

We describe a rescue operation in a neonate with a large left ventricular tumor obstructing the left ventricular outflow tract. Surgical resection was performed through an aortotomy and transseptal approach. We excised the main part of the tumor, which was obstructing the outflow tract, leaving a portion that was strongly attached to posterior wall of the left ventricle and mitral valve annulus, which was not feasible to remove. Histological examination showed a rhabdomyoma with benign features. The girl was doing well after 3 months, with no residual tumor growth and no signs of obstruction of the left ventricular outflow tract.

The role of the heart team in complicated transcatheter aortic valve implantation: a 7-year single-centre experience.

OBJECTIVES: European guidelines recommend to perform transcatheter aortic valve implantation (TAVI) within a multidisciplinary heart team. However, there is a strong drive--despite existing guidelines--to perform TAVI outside of specialized centres. The aim of this study was to clarify the necessity of on-site cardiac surgery by providing a clear insight into the complications during/after TAVI that needed surgical management. METHODS: A total of 2287 (1523 transfemoral, 752 transapical and 12 transaortic) patients, with a mean age of 84.5 ± 5.3 years, and a mean log EuroSCORE of 21.7 ± 16.3, of which 205 were female (84%), underwent TAVI since February 2006 at our institution. All procedure-related complications that required surgical interventions, whether immediate or delayed but within the initial hospital stay, were recorded and retrospectively analysed. RESULTS: Out of this cohort, 245 (10.7%) patients required surgical treatment due to major complications. A total of 42 patients (1.8%) underwent conversion to full sternotomy and 27 (1.2%) were dependent on the short-term use of the heart-lung machine. Vascular complications with surgical intervention were seen in 85 patients (3.7%), 54 patients (2.4%) had to have a rethoracotomy within their initial stay and 15 (0.7%) required a cardiac reoperation. CONCLUSIONS: Severe complications during TAVI that can only be resolved surgically will continue to occur. Therefore, each TAVI procedure should be conducted or accompanied by a cardiac surgeon and an experienced team within a specialized centre.

Four-dimensional modelling of the mitral valve by real-time 3D transoesophageal echocardiography: proof of concept.

OBJECTIVES: The complexity of the mitral valve (MV) anatomy and function is not yet fully understood. Assessing the dynamic movement and interaction of MV components to define MV physiology during the complete cardiac cycle remains a challenge. We herein describe a novel semi-automated 4D MV model. METHODS: The model applies quantitative analysis of the MV over a complete cardiac cycle based on real-time 3D transoesophageal echocardiography (RT3DE) data. RT3DE data of MVs were acquired for 18 patients. The MV annulus and leaflets were semi-automatically reconstructed. Dimensions of the mitral annulus (anteroposterior and anterolateral-posteromedial diameter, annular circumference, annular area) and leaflets (MV orifice area, intercommissural distance) were acquired. Variability and reproducibility (intraclass correlation coefficient, ICC) for interobserver and intraobserver comparison were quantified at 4 time points during the cardiac cycle (mid-systole, end-systole, mid-diastole and end-diastole). RESULTS: Mitral annular dimensions provided highly reliable and reproducible measurements throughout the cardiac cycle for interobserver (variability range, 0.5-1.5%; ICC range, 0.895-0.987) and intraobserver (variability range, 0.5-1.6%; ICC range, 0.827-0.980) comparison, respectively. MV leaflet parameters showed a high reliability in the diastolic phase (variability range, 0.6-9.1%; ICC range, 0.750-0.986), whereas MV leaflet dimensions showed a high variability and lower correlation in the systolic phase (variability range, 0.6-22.4%; ICC range, 0.446-0.915) compared with the diastolic phase. CONCLUSIONS: This 4D model provides detailed morphological reconstruction as well as sophisticated quantification of the complex MV structure and dynamics throughout the cardiac cycle with a precision not yet described.

Protective effects of pulsatile flow during cardiopulmonary bypass.

BACKGROUND: Children with congenital heart disease are often operated at a very young age. Cardiopulmonary bypass (CPB) has become indispensable for these sometimes very complex operations, but one cannot neglect a possible negative impact of CPB on organ function. Traditionally, CPB was developed with non-pulsatile flow but there are clinical observations that pulsatile flow might be superior with improved patient outcomes. Therefore, we attempted to elucidate whether CPB with pulsatile flow preserves organ integrity compared with nonpulsatile flow. METHODS: We studied 27 piglets of 4 weeks age and divided them into 3 experimental groups: control group (no CPB); non-pulsatile and pulsatile-CBP with 90-minutes CPB and 120-minutes recovery and reperfusion. Thereafter, histology of kidney, liver, and hippocampus was performed. Moreover, we measured adenosine triphosphate (ATP) content in these organs. RESULTS: Histologic evaluation revealed that laminar flow produced significant cellular edema in the kidney and hippocampus. Additionally, markers for hypoxia, apoptosis, and nitrosative stress were elevated but predominately in the hippocampus and proximal tubules of the kidney. Most of these alterations were reduced to or near control levels with pulsatile CPB. Moreover, ATP content of all 3 organs examined was higher and kidney and liver enzymes were lower in the pulsatile group compared with the non-pulsatile CPB. With regard to histologic changes, the liver seemed to be a less sensitive organ. CONCLUSIONS: In our study during pulsatile CPB, organ damage was significantly attenuated as compared with non-pulsatile CPB. Therefore, in pediatric patients pulsatile CPB may improve clinical outcomes.

Hippocampal Neuroprotection by Minocycline and Epigallo-Catechin-3-Gallate Against Cardiopulmonary Bypass-Associated Injury.

Surgical correction of congenital cardiac malformations mostly implies the use of cardiopulmonary bypass (CPB). However, a possible negative impact of CPB on cerebral structures like the hippocampus cannot be neglected. Therefore, we investigated the effect of CPB on hippocampus CA1 and CA3 regions without or with the addition of epigallocatechin-3-gallate (EGCG) or minocycline. We studied 42 piglets and divided them into six experimental groups: control without or with EGCG or minocycline, CPB without or with EGCG or minocycline. The piglets underwent 90 minutes CPB and subsequently, a 120-minute recovery and reperfusion phase. Thereafter, histology of the hippocampus was performed and the adenosine triphosphate (ATP) content was measured. Histologic evaluation revealed that CPB produced a significant peri-cellular edema in both CA regions. Moreover, we found an increased number of cells stained with markers for hypoxia, apoptosis and nitrosative stress. Most of these alterations were significantly reduced to or near to control levels by application of EGCG or minocycline. ATP content was significantly reduced within the hippocampus after CPB. This reduction could not be antagonized by EGCG or minocycline. In conclusion, CPB had a significant negative impact on the integrity of hippocampal neural cells. This cellular damage could be significantly attenuated by addition of EGCG or minocycline.

Organ-protective effects on the liver and kidney by minocycline in small piglets undergoing cardiopulonary bypass.

Cardiopulmonary bypass (CPB) often is required for the operative correction of congenital heart defects in small infants. Unfortunately, CPB is associated with injury of inner organs such as the brain, kidney, lung, and liver. Renal failure and increase in liver enzymes are typical side effects observed after CPB. Here, we investigate whether organ protection of the kidney and liver can be achieved with the application of minocycline, which is known-besides its anti-infective effects-to act as a poly-ADP-ribose-polymerase inhibitor. Twenty-nine 4-week-old Angler Sattelschwein-piglets (8-15 kg) were divided into four groups: control group (n = 8), CPB group (n = 9), minocycline-control group (n = 6), and the minocycline-CPB group (n = 6). CPB groups were thoracotomized and underwent CPB for 120 min (cross-clamp, 90 min; reperfusion, 30 min) followed by a 90-min recovery time. The control groups also were thoracotomized but not connected to CPB. The minocycline group received 4 mg/kg minocycline before and 2 mg/kg after CPB. In the kidneys, CPB histologically resulted in widening of Bowman's capsule, and-mainly in tubules-formation of poly-ADP-ribose, nitrosylation of tyrosine-residues, nuclear translocation of hypoxia-induced factor HIF-1α, and of apoptosis-inducing factor (AIF). In addition, we found significantly less ATP in the kidney and significantly increased plasma urea and creatinine. Similar but gradually attenuated changes were found in the liver together with significantly elevated de-Ritis coefficient. These changes in the kidney and liver were significantly diminished by minocycline (except AIF in the liver which was similar in all groups). In conclusion, CPB causes damage in the kidney and-to a lower degree-in the liver, which can be attenuated by minocycline.

Biventricular amputation for biventricular pulsatile assist device implantation in patients with severe ventricular septal perforation.

Complete ischaemic destruction of the cardiac apex as well as the majority of the ventricular septum is usually a lethal condition with no alternatives except mechanical circulation support and highly urgent heart transplantation. Depending on the mass of the necrotic and destructed myocardium, implantation of a ventricular assist device can be challenging due to insufficient tissue quality. We describe here a new approach to pulsatile Thoratec (Thoratec Corporation, Pleasanton, CA, USA) extracorporeal biventricular assist device implantation.

Mitral Valve Regurgitation: A Severe Complication following Left Ventricular Biopsy 15 Years after Heart Transplantation.

A 71-year-old male patient underwent orthotopic heart transplantation in 1995. Due to left heart catheterization 15 years later, biopsy from the left ventricular apex was performed for rejection screening. Two days later, echocardiography revealed severe mitral valve regurgitation requiring mitral valve replacement. This is a very rare case showing that left heart biopsy may lead to severe hemodynamic complications with the need for surgical intervention.

Cooling after successful resuscitation in cardiac surgery patients.

BACKGROUND: Despite many years of intensive research sudden cardiac death is one of the most common causes of death all over the world. The European Resuscitation Council (ERC) recommends the use of moderate therapeutic hypothermia for 12-24 hours to improve neurological outcome. However, the beneficial effect of this therapy on outcomes for cardiac surgery patients with In- Hospital- Resuscitation (IHR) has not been well studied.The purpose of this single center analysis was to investigate our first experience in a non - selected IHR population, where hypothermia was induced independent from initial heart rhythm disturbance. METHOD: A total of 20 resuscitated patients who were treated in our institution between January 2010 and December 2011 formed the study cohort. RESULTS: In all patients post- resuscitation course was significantly prolonged with severe low cardiac output syndrome in six patients (30%). Overall four patients (20%) sustained septicemia with the need for high dose inotropic support. The 30 day mortality was 30% (six of twenty). However, stroke with severe neurological impairment appeared in only four patients (20%) after resuscitation with subsequent therapeutic hypothermia. CONCLUSION: With our observation study we could demonstrate the benefits for neurological outcome due to therapeutic hypothermia in cardiac surgery patients after successful resuscitation. However post- resuscitation treatment should focus on sufficient therapeutic strategies to avoid the distinctive short term morbidity and mortality.