5206SICD registry in European paediatric and adult patients with congenital heart defects: preliminary results of the SIDECAR project

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M S Silvetti ◽  
L Kornyei ◽  
J Kwiatkowska ◽  
I Tamburri ◽  
F A Saputo ◽  
...  
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Young Patients ◽  
Adult Patients ◽  
Icd Implantation ◽  
Preliminary Results ◽  
Inappropriate Shocks ◽  
European Paediatric

Abstract Aim Use of the subcutaneous implantable cardioverter-defibrillator (S-ICD) to prevent sudden cardiac death is increasing. Few data exist on S-ICD in young patients. We report preliminary data from a multicenter European registry of paediatric and young adult patients who underwent S-ICD implantation Methods Observational, prospective, non-randomized, standard-of-care study on S-ICD implantation/follow-up in young patients with inherited arrhythmias (IA), cardiomyopathies, and congenital heart defects (CHD). Results 29 patients (12 CHD, 14 Cardiomyopathies, and 3 IA), mean age 17±6 years, 13 of them <18 years, with body mass index (BMI) 23±4, underwent S-ICD implantation (primary prevention 66%). The first 8 patients underwent a three-incision procedure, the following 21 (72%) a two-incision procedure. No intraoperative complications occurred. Over 19 months median follow-up (25th–75th percentiles, 5–37) 5 patients (17%) received appropriate and 2 (7%) inappropriate shocks. Four patients (14%) had device-related complications requiring surgical intervention: three skin erosions at the superior parasternal incision, one pocket infection. A higher risk of complications was seen in patients who underwent standard procedures [hazard ratio (HR) 14.7, 95% confidence interval (CI) 2.34 to 93.03; P=0.001] and those with BMI <20 (HR 11.06, 95% CI 1.01–121.07; P=0.008). Conclusion These preliminary results of a multicenter European paediatric registry suggest that S-ICD is safe and effective with low rates of inappropriate shocks. Improvement of implantation techniques seems associated with better outcome.

scholarly journals Perspectives in the management of congenital heart defects in adult patients

2015 ◽  
Vol 156 (3) ◽  
pp. 92-97
Author(s):  
István Hartyánszky ◽  
Sándor Varga ◽  
Kálmán Havasi ◽  
Barna Babik ◽  
Márta Katona ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Adult Patients ◽  
Univentricular Heart ◽  
Transposition Of Great Arteries ◽  
Number Of Patients

Due to improving results in congenital heart surgery, the number of adult patients with congenital heart defect is increasing. The question is: what kind of problems can be managed in this patient-group? The authors review the different problems of management of congenital heart defects in adults based on national and international literature data. Simple defects recognised in adults, postoperative residual problems, changing of small grafts and valves, correction of primary or operated coarctation aortae can be usually managed without problems. A very close follow-up is necessary to establish the correct period for heart transplantation in patients with transposition of great arteries with Senning/Mustard operation, and univentricular heart corrected with “Fontan-circulation” type surgical procedure. The authors conclude that although the number of patients increases, only a few congenital heart diseases may cause problems. It seems important (1) to monitor asymptomatic patient who underwent operation (Fallot-IV, Ross procedure, etc.), (2) follow up regularly patients who underwent Senning/Mustard procedure (magnetic resonance imaging, echocardiography, brain natriuretic peptide measurement), (3) define the proper period of preparation for heart transplantation of patients with a univentricular heart, with special attention to the possibility of multiorgan (lung, liver, etc.) failure. Due to the improvement of foetal diagnosis of congenital heart defects, the number of patients with complex congenital heart defects is decreasing. The standard management of these patients could be primary heart transplantation in infancy. Orv. Hetil., 2015, 156(3), 92–97.

Surgical management of congenital heart defects in adolescent and adult patients, between years 2001–2008

2009 ◽  
Vol 150 (37) ◽  
pp. 1739-1743 ◽  
Author(s):  
István Hartyánszky ◽  
Andrea Székely ◽  
László Király ◽  
Zsolt Prodán ◽  
Sándor Mihályi ◽  
...  
Keyword(s):  
Surgical Management ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Adult Patients

A felnőttkorban operált veleszületett szívhibák között vezetnek az I. rekonstrukciós beavatkozások: a) frissen felismert betegségek, b) megelőzően inoperábilisnak ítélt kórképek, c) pulmonalis hypertonia, jobbkamra-elégtelenség miatt „elkésett” műtétek. Növekszik a II. REDO műtétek száma: a) residuumok korrigálása, b) kinőtt, diszfunkciós homograftok cseréje, c) műtéti/intervenciós korrigálás utáni recoarctatio (aneurysma, dissectio) sebészete, d) aorta valvulotomia/valvuloplastica, illetve társvitiumok (TGA) korrigálásának következményeként Ross-műtét, műbillentyű-beültetés . Betegek, eredmények: A 2001–2008 között végzett 4496 műtét közül 166 volt fiatal-felnőtt korú (16–52, átlagéletkor: 28 év) (Ia: 77, Ib: 15, Ic: 4, IIa: 11, IIb: 22, IIc: 9, IId: 28). Műtéti mortalitás nem volt, 1 beteg pulmonalis hypertoniás krízisben, 1 jobbkamra-elégtelenség miatti malignus ritmuszavarban, 2 többszerv-elégtelenségben halt meg. Konklúzió: A rizikófaktorokat a pulmonalis hypertonia és a jobbkamra-elégtelenség jelenti. A bonyolult sebészi megoldások a „congenitalis szívsebész” számára nem jelentenek problémát, de koraibb diagnózisok, terápiában az extracorporalis membránoxigenátor használata az eredményeket javíthatja.

scholarly journals Impact of 3D Printing on Short-Term Outcomes of Biventricular Conversion From Single Ventricular Palliation for the Complex Congenital Heart Defects

2021 ◽  
Vol 8 ◽  
Author(s):  
Bozhong Shi ◽  
Yanjun Pan ◽  
Weiru Luo ◽  
Kai Luo ◽  
Qi Sun ◽  
...  
Keyword(s):  
3D Printing ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Vena Cava ◽  
Left Ventricular ◽  
Long Term Results ◽  
Short Term ◽  
The Mean

Background: Although Fontan palliation seems to be inevitable for many patients with complex congenital heart defects (CHDs), candidates with appropriate conditions could be selected for biventricular conversion. We aimed to summarize our single-center experience in patient selection, surgical strategies, and early outcomes in biventricular conversion for the complex CHD.Methods: From April 2017 to June 2021, we reviewed 23 cases with complex CHD who underwent biventricular conversion. Patients were divided into two groups according to the development of the ventricles: balanced ventricular group (15 cases) and imbalanced ventricular group (8 cases). Early and short-term outcomes during the 30.2 months (range, 4.2–49.8 months) follow-up period were compared.Results: The overall mortality rate was 4.3% with one death case. In the balanced ventricular group, 6 cases received 3D printing for pre-operational evaluation. One case died because of heart failure in the early postoperative period. One case received reoperation due to the obstruction of the superior vena cava. In the imbalanced ventricular group, the mean left ventricular end-diastolic volume was (33.6 ± 2.1) ml/m2, the mean left ventricular end-diastolic pressure was 9.1 ± 1.9 mmHg, and 4 cases received 3D printing. No death occurred while one case implanted a pacemaker due to a third-degree atrioventricular block. The pre-operational evaluation and surgery simulation with a 3D printing model helped to reduce bypass time in the balanced group (p &lt; 0.05), and reduced both bypass and aorta clamp time in the imbalanced group (p &lt; 0.05). All patients presented great cardiac function in the follow-up period.Conclusion: Comprehensive evaluation, especially 3D printing technique, was conducive to finding the appropriate cases for biventricular conversion and significantly reduced surgery time. Biventricular conversion in selected patients led to promising clinical outcomes, albeit unverified long-term results.

Disease-specific knowledge and information preferences of young patients with congenital heart disease

2013 ◽  
Vol 24 (2) ◽  
pp. 321-330 ◽  
Author(s):  
Wiebke Lesch ◽  
Katharina Specht ◽  
Anke Lux ◽  
Michael Frey ◽  
Elisabeth Utens ◽  
...  
Keyword(s):  
Congenital Heart Defects ◽  
Information Needs ◽  
Congenital Heart ◽  
Heart Defects ◽  
Young Patients ◽  
Transition Phase ◽  
Sources Of Information ◽  
Specific Knowledge ◽  
Information Preferences ◽  
Disease Specific

AbstractAimsPersons suffering from congenital heart defects require lifelong specialist medical care. Failure to attend cardiological follow-up examinations and risky health behaviour in the transition phase may cause severe medical complications. A good level of disease-specific knowledge enhances compliance. Therefore, the study's aim was to investigate: (a) the level of disease-specific knowledge, (b) information preferences, and (c) sources of information for children, adolescents, and young adults regarding their illness.Methods and resultsIn all, 596 patients, aged 10–30 years, participated in this cross-sectional survey study (response rate: 53%). All patients were already enrolled in the German National Register for Congenital Heart Defects. The main outcome measures included disease-specific knowledge, information preferences, and information sources regarding patients’ individual cardiac condition. The patients demonstrated a major knowledge gap concerning their illness and how to live with it. For all three age groups, patients’ information needs were unmet on nearly half of the topics of interest. Children's information needs were comparable to those of adolescents and adults concerning several important topics, for example, work/career, sports. Information preferences varied according to age and gender, rather than disease severity. The most important sources of information were physicians (71.0%), family and friends (58.2%), and the Internet (37.5%).ConclusionThe study revealed substantial knowledge gaps, indicating a need for structured multidisciplinary patient education interventions. These interventions should start as early as in childhood and help patients manage their condition and assume responsibility for their own health, so that the transition phase runs smoothly.

Congenital Heart Defects in Neonates: Determining the Incidence of Genetic Testing and Follow-up Consultation at UCLA

2015 ◽  
Vol 208 (6) ◽  
pp. 359
Author(s):  
Sibel Kantarci ◽  
Claire Baldauf ◽  
Elena Minakova ◽  
Joanna Parga ◽  
Isabell Purdy ◽  
...  
Keyword(s):  
Genetic Testing ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects
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