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Grown-up congenital heart disease

István Hartyánszky, Gábor Bogáts
INTRODUCTION: Congenital heart defects are frequently present in patients with Down syndrome. AIM: The authors analyzed the impact of changing approach in surgical management of congenital heart defect on the life expectancy of patients with Down syndrome. METHOD: Between 1974 and 1997 the data of 359 children with Down syndrome were collected. Among them 255 patients had no surgery and the mortality in this group was 25.9%, whereas the mortality in the group of 104 patients who underwent palliative surgery was 8...
October 2016: Orvosi Hetilap
Sachin Talwar, Manikala V Kumar, Vishnubhatla Sreenivas, Shiv K Choudhary, Manoj Sahu, Balram Airan
BACKGROUND: The number of grown ups with congenital heart diseases (GUCHs) is steadily increasing. AIMS: To analyze factors predicting early cardiac morbidity following cardiac surgery in GUCH at a tertiary care center. SETTING AND DESIGN: Retrospective study at a multispeciality tertiary referral center. METHODS: Between January 2004 and December 2014, 1432 patients ≥13 years of age (acyanotic defects: 843, cyanotic defects: 589) underwent surgery for congenital heart defects...
September 2016: Annals of Pediatric Cardiology
M Athar, S Ali, S M Ahmed, R Mazahir
Tetralogy of fallot (TOF) is one of the most common congenital heart disease (CHD) in children. With the development of pediatric surgery and intensive care units, increasing number of grown-up CHD patients are presenting for non-cardiac surgeries. Non-operated TOF patients suffer from chronic hypoxia and decreased pulmonary blood flow resulting in considerable alteration in the physiology. The optimal management of these patients, therefore, require a thorough understanding of the pathophysiology of the uncorrected TOF...
May 24, 2016: Revista Española de Anestesiología y Reanimación
Peter Zartner
Pacemaker therapy (PMT) in grown-up congenital heart disease (GUCH) must meet the demands of a young, dynamic and heterogeneous group of patients. The duration of the therapy has to be planned for several decades and should be accompanied by an invasive procedure at the very least. Most of the patients enter adulthood with their pacemaker (PM) already implanted; for others the indications can be derived from the published guidelines for GUCH and PMT, but need to be adjusted to the individual situation of the patient...
June 2016: Herzschrittmachertherapie & Elektrophysiologie
Ireneusz Haponiuk, Katarzyna Gierat-Haponiuk, Dominika Szalewska, Piotr Niedoszytko, Stanisław Bakuła, Maciej Chojnicki
Congenital heart defects (CHD) are the cause of reduced physical performance. The presence of congenital abnormalities in the heart of grown-up patients contributes to excessive hypo-kinesia. We present endurance parameters and a personalized comprehensive cardiac rehabilitation program before an extreme mountain climbing of a 27-year-old patient with an uncorrected ventricular septal defect (VSD). A 26-year-old female patient with an uncorrected congenital VSD was admitted to the department of cardiac rehabilitation before the planned high-mountain expedition...
March 2016: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
Raffaella Marzullo, Roberto Bordese, Antonia Bassignana, Gaetana Ferraro, Giovanni Dall'Orto, Lorena Ferrarotti, Daniela Libertucci, Laura Rissone, Giuseppe Amoroso, Guglielmo Actis Dato, Carlo Albera, Laura Bergamasco, Gabriella Agnoletti
BACKGROUND: Adults with congenital heart disease are a relatively new population that progressively increases in size and complexity. In Italy, there are no accurate data concerning the distribution of congenital defects and the long-term outcome relating to both congenital heart disease per se and comorbidities, due to the aging process. METHODS: The Piedmont Adult Congenital Heart Disease Registry has been designed to investigate these aspects and to support a high quality healthcare development for grown-up congenital heart patients...
March 2016: Giornale Italiano di Cardiologia
Giordano Zampi, Amedeo Pergolini, Andrea Celestini, Fabrizio Benvissuto, Maria Denitza Tinti, Mariano Ortenzi, Luigi Sommariva
We report the case of an elderly woman with persistent unrepaired atrial septal defect and moderate pulmonary stenosis. The diagnostic work-up and the echocardiographic findings of such a rare case are reported, along with a brief description of heart failure pathophysiology in this grown-up congenital heart disease.To the best of our knowledge, this is the first case with the greater longevity in an elderly patient with unrepaired atrial septal defect and pulmonary stenosis ever reported in the literature.
January 2016: Giornale Italiano di Cardiologia
Fortunato Iacovelli, Martino Pepe, Gaetano Contegiacomo, Vito Alberotanza, Filippo Masi, Alessandro Santo Bortone, Stefano Favale
Left ventricular noncompaction (LVNC) is a myocardial disorder probably due to the arrest of normal embryogenesis of the left ventricle. It could be isolated or associated with other extracardiac and cardiac abnormalities, including coronary artery anomalies. Despite the continuous improvement of imaging resolution quality, this cardiomyopathy still remains frequently misdiagnosed, especially if associated with other heart diseases. We report a case of LVNC association with both malposition of the great arteries and a very original coronary artery pattern...
2016: Case Reports in Cardiology
Werner Budts, Jolien Roos-Hesselink, Tanja Rädle-Hurst, Andreas Eicken, Theresa A McDonagh, Ekaterini Lambrinou, Maria G Crespo-Leiro, Fiona Walker, Alexandra A Frogoudaki
No abstract text is available yet for this article.
May 7, 2016: European Heart Journal
Yusuke Tsuboko, Yasuyuki Shiraishi, Akihiro Yamada, Tomoyuki Yambe, Satoshi Matsuo, Yoshikatsu Saiki, Masaaki Yamagishi
Pulmonary conduit valves are used as one of the surgical treatment methods of congenital heart diseases. We have been designing a sophisticated pulmonary conduit valve for the right ventricular outflow tract reconstruction in pediatric patients. In this study, two types of polyester grafts with or without bulging structures for the conduit valves were used and evaluated from the hemodynamic point of view focusing on the application of these conduit valves in the grown-up congenital heart failure patients. We examined valvular function in the originally developed pulmonary mock circulatory system, which consisted of a pneumatic driven right ventricular model, a pulmonary valve chamber, and an elastic pulmonary compliance model with peripheral vascular resistance units...
2015: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
Cecilia Vecoli, Andrea Borghini, Ilenia Foffa, Lamia Ait-Ali, Eugenio Picano, Maria Grazia Andreassi
BACKGROUND/OBJECTIVES: Children with congenital heart disease are exposed by repeated imaging to ionizing radiation, which may have important implications for lifetime health risks. Leukocyte telomere length (LTL), a reliable biomarker of genomic instability, is associated with increased risk of cancer and cardiovascular disease. We investigated LTL in grown-up patients with CHD (GUCHs) and a positive history of medical radiation exposure as well as the influence of functional polymorphisms of genes involved in DNA repair...
February 1, 2016: International Journal of Cardiology
J-T Sieweke, M Westhoff-Bleck, L C Napp, M Avsar, J Vogel-Claussen, A Schäfer, J Bauersachs, M Brehm
No abstract text is available yet for this article.
January 15, 2016: International Journal of Cardiology
Kunio Kusajima, Takaya Hoashi, Koji Kagisaki, Hideo Ohuchi, Isao Shiraishi, Hajime Ichikawa
OBJECTIVE: Surgical experiences of the reoperative double ventricular outflow tract reconstruction long after the successful repair of conotruncal anomalies were reviewed. METHODS: Ten adult patients with conotruncal anomalies (6 females, 22.9 ± 5.5 years old) underwent the reoperative double ventricular outflow tract reconstruction. Primary diagnosis was pulmonary atresia with ventricular septal defect in 6 patients, truncus arteriosus in 3, and double-outlet right ventricle in 1...
November 2015: General Thoracic and Cardiovascular Surgery
Fernando Tadeu Vasconcelos Amaral, Paulo Henrique Manso, André Schmidt, Ricardo Nilson Sgarbieri, Walter Villela de Andrade Vicente, Clovis Carbone Junior, Jane Somerville
During the last decades, advances in diagnosis and treatment of congenital heart disease have allowed many individuals to reach adulthood. Due mainly to the great diagnostic diversity and to the co-morbidities usually present in this age group, these patients demand assistance in a multidisciplinary facility if an adequate attention is aimed. In this paper we reviewed, based in the international literature and also on the authors' experience, the structural conditions that should be available for these patients...
July 2015: Revista Brasileira de Cirurgia Cardiovascular
Kálmán Havasi, Anita Kalapos, Krisztina Berek, Péter Domsik, Gábor Kovács, Gábor Bogáts, István Hartyánszky, Erzsébet Kertész, Márta Katona, Katalin Rácz, Miklós Csanády, Tamás Forster, Attila Nemes
Improvements in surgical techniques and technical advancements have made possible for several patients with congenital heart disease to grow up to adulthood. It has been decided to create a registry for their more precise treatment. This registry now includes 2770 patients with data on 3043 operations, with almost 30 different diagnoses. The purpose of this paper is to review the facts and the basics leading to the establishment of this registry.
May 17, 2015: Orvosi Hetilap
Martin Schweiger, Volkmar Falk, Mirko Biry, Michael Hübler, Markus J Wilhelm
INTRODUCTION: Miniaturization and surgical progress in the field of ventricular assist device (VAD) therapy will lead to an increasing use in grown-up patients with congenital heart disease (GUCH). VAD implantation in such patients, especially biventricular VAD (BVAD) placement, can be very challenging, potentially associated with high morbidity and mortality. METHODS AND RESULTS: We report on a 47-year-old male patient in terminal biventricular failure with pulmonary hypertension 40 years after a Mustard procedure...
April 2015: International Journal of Artificial Organs
A Haddad, R Bourezak, M Aouiche, R Ait Mohand, A Hamzaoui, S E Bourezak
INTRODUCTION: With advances in recent decades in the field of congenital heart disease both for imaging in medical therapy, a large number of heart disease is diagnosed before birth. Many of them benefit from surgery and reach adulthood, they do not require further action. Some of them develop later in their lives other problems requiring reoperation in adulthood. This sparked the birth of a subspecialty within the department of congenital heart disease: GUCH Unit "grown up congenital heart disease"...
September 2015: Annales de Cardiologie et D'angéiologie
Mariangela Peruzzi, Giuseppe Biondi-Zoccai, Antonino G M Marullo, Giacomo Frati
No abstract text is available yet for this article.
April 2015: Journal of Cardiothoracic and Vascular Anesthesia
Fabrizio Gandolfo, Fabrizio De Rita, Asif Hasan, Massimo Griselli
There is no reliable published data on the overall prevalence or incidence of heart failure (HF) in children. However, the success of mechanical circulatory support (MCS) in management of HF has raised the prospect of a previously unavailable treatment modality. Orthotopic heart transplant (OHTx) remains the gold standard treatment, but the number of patients requiring this treatment far outweighs the donor availability. It is therefore not surprising to see the popularity of various MCS modalities, with different devices ranging from veno-arterial extra corporeal membrane oxygenation (VA-ECMO) to ventricular assist devices (VADs), which are either para-corporeal or intra-corporeal, with pulsatile or continuous flow...
September 2014: Annals of Cardiothoracic Surgery
Antonio Hernández-Madrid, Mélèze Hocini, Jian Chen, Tatjana Potpara, Laurent Pison, Carina Blomström-Lundqvist
The aim of this survey was to provide insight into current practice regarding the management of paediatric arrhythmias in Europe. The survey was based on a questionnaire sent via the Internet to the European Heart Rhythm Association (EHRA) electrophysiology research network centres. The following topics were explored: patient and treatment selection, techniques and equipment, treatment outcomes and complications. The vast majority of paediatric arrhythmias concerns children older than 1 year and patients with grown-up congenital heart disease...
December 2014: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
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