Nationwide Registry-Based Analysis of Infective Endocarditis Risk After Pulmonary Valve Replacement.

Background Infective endocarditis (IE) after pulmonary valve replacements in congenital heart disease is a significant concern. This study aimed to identify specific long-term risk factors for IE after percutaneous pulmonary valve implantation or surgical pulmonary valve replacement. Methods and Results All patients with congenital heart disease from the National Register for Congenital Heart Defects with at least 1 pulmonary valve replacement before January 2018 were included. A total of 1170 patients (56.3% men, median age at study inclusion 12 [interquartile range {Q1-Q3} 5-20 years]) received 1598 pulmonary valve replacements. IE occurred in 4.8% of patients during a follow-up of total 9397 patient-years (median 10 [Q1-Q3, 6-10] years per patient). After homograft implantation 7 of 558 (1.3%) patients developed IE, after heterograft implantation 31 of 723 (4.3%) patients, and after Melody valve implantation 18 of 241 (7.5%) patients. Edwards Sapien and mechanical valves were used less frequently and remained without IE. The incidence of IE in heterografts excluding Contegra valves was 7 of 278 (2.5%), whereas the incidence of IE in Contegra valves was 24 of 445 (5.4%). The risk of IE was not increased compared with homografts if Contegra valves were excluded from the heterografts (hazard ratio [HR], 2.60; P=0.075). The risk of IE was increased for bovine jugular vein valves, Contegra valves (HR, 6.72; P<0.001), and Melody valves (HR, 5.49; P<0.001), but did not differ between Melody valves and Contegra valves (HR, 1.01; P=0.978). Conclusions Bovine jugular vein valves have the highest risk of IE, irrespective of the mode of deployment, either surgical or percutaneous.

Pulmonary valve prostheses: patient's lifetime procedure load and durability. Evaluation of the German National Register for Congenital Heart Defects.

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.

Ventricular assist devices in paediatric cardiomyopathy and congenital heart disease: An analysis of the German National Register for Congenital Heart Defects.

BACKGROUND: Ventricular assist devices (VAD) are increasingly used in patients with end-stage heart failure due to acquired heart disease. Limited data exists on the use and outcome of this technology in children. METHODS: All children (<18 years of age) with VAD support included in the German National Register for Congenital Heart Defects were identified and data on demographics, underlying cardiac defect, previous surgery, associated conditions, type of procedure, complications and outcome were collected. RESULTS: Overall, 64 patients (median age 2.1 years; 45.3% female) receiving a VAD between 1999 and 2015 at 8 German centres were included in the analysis. The underlying diagnosis was congenital heart disease (CHD) in 25 and cardiomyopathy in 39 children. The number of reported VAD implantations increased from 13 in the time period 2000-2004 to 27 implantations in the time period 2010-2014. During a median duration of VAD support of 54 days, 28.1% of patients experienced bleeding complications (6.3% intracerebral bleeding), 14.1% thrombotic (10.9% VAD thrombosis) and 23.4% thromboembolic complications (including cerebral infarction in 18.8% of patients). Children with cardiomyopathy were more likely to receive a cardiac transplantation (79.5% vs. 28.0%) compared to CHD patients. Survival of cardiomyopathy patients was significantly better compared to the CHD cohort (p < 0.0001). Multivariate Cox-proportional analysis revealed a diagnosis of CHD (hazard ratio [HR] 4.04, p = 0.001), age at VAD implantation (HR 1.09/year, p = 0.04) and the need for pre-VAD extracorporeal membrane oxygenation (ECMO) support (HR 3.23, p = 0.03) as independent predictors of mortality. CONCLUSIONS: The uptake of VAD therapy in children is increasing. Morbidity and mortality remain high, especially in patients with congenital heart disease and those requiring ECMO before VAD implantation.

Education of Children With Cyanotic Congenital Heart Disease After Neonatal Cardiac Surgery.

BACKGROUND: The aim of the study was to evaluate the educational achievement of patients diagnosed with univentricular heart physiology (UVHP) or transposition of the great arteries (TGA) after neonatal cardiac surgery. METHODS: An exploratory online survey was performed with patients registered with the National Register for Congenital Heart Defects in Germany. For this publication, a subgroup analysis was conducted among patients diagnosed with TGA (n = 173; 36.3%) and UVHP (n = 304; 63.7%). RESULTS: Median age of the sample at school enrollment was 6 years (range, 5-8 years). The majority were enrolled at a standard elementary school (n = 368 of 477; 77.1%), although patients with UVHP were enrolled significantly more often at a special needs school (n = 52 of 304; 17.1%, TGA patients n = 11/ of 173; 6.4%, P < .001). A total of 45.8% (n = 66 of 144) of the patients graduated with a high school diploma. A substantial number of patients had been diagnosed with behavioral or learning disorders (TGA patients n = 63 of 173 [36.4%], UVHP patients n = 148 of 304 [48.7%]) and received early supportive therapy or remedial teaching before (TGA patients n = 89 of 173 [51.4%], UVHP patients n = 209 of 304 [68.8%]) and/or during their school careers (TGA patients n = 54 of 173 [31.2%], UVHP patients n = 120 of 304 [39.5%]). CONCLUSIONS: A large proportion of patients who underwent neonatal cardiac surgery graduated with a high school diploma. These results are of great importance to congenital heart defect patients, affected families, and treating physicians. Nevertheless, study participants, especially patients with UVHP, face some academic challenges. We conclude that long-term follow-up examinations and regular developmental assessments may be beneficial.

Surgery during pregnancy - results of a German questionnaire.

OBJECTIVES: Worldwide, not only the number of female medical students, but also of female surgeons increases. Simultaneously, younger generations take a closer look to their work-life balance. With this in mind, it seems necessary to evaluate the expectations of female surgeons in particular with respect to pregnancy during their surgical career. METHODS: Therefore, a nationwide survey was conducted in Germany from July to December 2016 under the auspices of the German Society of Surgery as well as the Professional Board of German Surgeons. The questionnaire involved 2,294 female surgeons and 1,843 complete records were evaluated. RESULTS: Of the analyzed answers, 62% of the women (n=781) were operating during pregnancy. The joy of surgery (91.6%), followed by team spirit (57.1%), were the main motivations to perform operations while pregnant. Operative activity decreased from 30.8% in the first 3 months of pregnancy to 21.5% during the last three months. Regarding the possible complaints, e.g., leg edema, back pain, premature labor and vaginal bleeding, there were no significant differences between the women with or without activity in the operating room. Sick leave due to pregnancy (1-10 days) was stated by 40.4% of respondents. CONCLUSION: Despite strong legal regulations for pregnant surgeons, the survey showed that most female surgeons are eager to operate despite their pregnancy. The results also demonstrate no significant differences regarding complications during pregnancy- or pregnant-dependent absence from work. Hospitals and surgical departments are asked to establish proper working conditions for pregnant surgeons and pregnancy should not be an obstacle for a career in surgery.

Somatic Development in Children with Congenital Heart Defects.

OBJECTIVES: Somatic development is impaired in children with congenital heart defects (CHDs), and head circumference seems to be a strong predictor of neurodevelopmental prognosis. The aim of this study was to generate up-to-date reference values for the somatic development (head circumference, body weight, and length/height) of children with CHDs. STUDY DESIGN: Our study population consisted of all patients included in the PAN study (Prävalenz angeborener Herzfehler bei Neugeborenen in Deutschland), which was conducted prospectively over a 3-year study period by the Competence Network for Congenital Heart Defects. All children with mild, moderate, and severe CHDs born in 2006-2009 in Germany were enrolled. For computing of z-scores, only children with the following characteristics were included: appropriate for gestational age, nonsyndromic disease, term or post-term delivery, and no cardiac surgery. RESULTS: There were 2818 patients included. New z-scores for the described somatic measures of children with mild, moderate, and severe CHDs were computed. Comparisons with the KiGGS study (Gesundheit von Kindern und Jugendlichen in Deutschland) and the Berlin Longitudinal Study revealed significantly lower measurements for all measures-most notably in children with severe CHDs and/or cardiac surgery. In our cohort, no catch-up growth was seen after cardiac surgery. CONCLUSION: Children with severe CHDs demonstrated the most abnormal pattern in growth, including head circumference before and after cardiac surgery, which is indicative of accompanying brain pathology unrelated to operative injury.

A Biobank for Long-term and Sustainable Research in the Field of Congenital Heart Disease in Germany.

Congenital heart disease (CHD) is the most frequent birth defect (0.8%-1% of all live births). Due to the advance in prenatal and postnatal early diagnosis and treatment, more than 90% of these patients survive into adulthood today. However, several mid- and long-term morbidities are dominating the follow-up of these patients. Due to the rarity and heterogeneity of the phenotypes of CHD, multicenter registry-based studies are required. The CHD-Biobank was established in 2009 with the aim to collect DNA from patients and their parents (trios) or from affected families, as well as cardiovascular tissues from patients undergoing corrective heart surgery for cardiovascular malformations. Clinical/phenotype data are matched to the International Paediatric and Congenital Cardiac Code (IPCCC) and the International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10). The DNA collection currently comprises samples from approximately 4200 participants with a wide range of CHD phenotypes. The collection covers about 430 trios and 120 families with more than one affected member. The cardiac tissue collection comprises 1143 tissue samples from 556 patients after open heart surgery. The CHD-Biobank provides a comprehensive basis for research in the field of CHD with high standards of data privacy, IT management, and sample logistics.

Three parties, one direction: Research priorities in adults with congenital heart disease. What do professionals, patients and relatives want to know?

BACKGROUND: Patients, their relatives, as well as medical specialists from the fields of paediatric cardiology, cardiology and cardiac surgery were surveyed to investigate the current research needs in the field of congenital heart disease (CHD) focussing specifically on the needs of those affected. MATERIAL AND METHODS: An online survey including four groups of patients with CHD (Fontan circulation, transposition of the great arteries [TGA] after atrial switch [AS] and after arterial switch operation [ASO], tetralogy of Fallot [TOF]) was performed. Each questionnaire comprised twelve topics. Persons affected (patients and relatives) were surveyed by means of CHD group specific questionnaires. Participants were recruited through the German National Register for Congenital Heart Defects. RESULTS: N=596 affected persons (Fontan circulation: n=189; TGA after AS: n=64; TGA after ASO: n=90; TOF: n=253) and 75 physicians (57.3% paediatric cardiologists, 28.0% cardiologists, 10.7% cardiac surgeons, 4.0% other) participated. In general, those affected assume a greater need for research than physicians. Regarding the CHD related topics to be the focus of future research, those affected largely agreed with the participating physicians, although with a different ranking of research topics. CONCLUSIONS: Based on the results of our study the challenges immanent in routine care for the CHD patient groups investigated can be identified. Accordingly, these topics should be prioritized in the research of the coming years.