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Grown-up congenital heart disease

Jung Bin Park, Jae Gun Kwak, Hong-Gook Lim, Woong-Han Kim, Jeong Ryul Lee, Yong Jin Kim
BACKGROUND AND OBJECTIVES: Mechanical circulatory support with extracorporeal membrane oxygenation (ECMO) and ventricular assist device has always been the optimal choice for treating the majority of medically intractable low cardiac output case. We retrospectively investigated our institution's outcomes and variables associated with a high risk of mortality. SUBJECTS AND METHODS: From 1999 to 2014, 86 patients who were of pediatric age or had grown-up congenital heart disease underwent mechanical circulatory support for medically intractable low cardiac output in our pediatric intensive care unit...
July 2017: Korean Circulation Journal
Lilia Oreto, Claudia Parrella, Roberto Ricci, Marcello Chinali
No abstract text is available yet for this article.
June 8, 2017: Journal of Thoracic and Cardiovascular Surgery
Carlo N De Cecco, Giuseppe Muscogiuri, José M Madrid Pérez, Marwen Eid, Pal Suranyi, Virginia W Lesslie, Gorka Bastarrika
The survival rate of patients with congenital heart disease (CHD) has dramatically improved over the last 2 decades because of technological and surgical advances in diagnosis and treatment, respectively. The vast majority of CHD patients are, in fact, amenable to treatment by either device closure or surgery. Considering the wide spectrum of surgical procedures and complex native and derived anatomy, continuous and detailed follow-up is of paramount importance. Cardiac magnetic resonance and cardiac computed tomography angiography are the cornerstones of diagnosis and follow-up of CHD, allowing for comprehensive noninvasive assessment of the heart, coronary tree, and intrathoracic great vessels, along with both morphological and functional evaluation...
July 2017: Journal of Thoracic Imaging
Laura Iop, Adolfo Paolin, Paola Aguiari, Diletta Trojan, Elisa Cogliati, Gino Gerosa
Cryopreserved allogeneic conduits are the elective biocompatible choice among currently available substitutes for surgical replacement in end-stage valvulopathy. However, degeneration occurs in 15 years in adults or faster in children, due to recipient's immunological reactions to donor's antigens. Here, human aortic valves were decellularized by TRICOL, based on Triton X-100 and sodium cholate, and submitted to standard cryopreservation (TRICOL-human aortic valves (hAVs)). Tissue samples were analyzed to study the effects of the combined procedure on original valve architecture and donor's cell removal...
March 9, 2017: Journal of Cardiovascular Translational Research
Syed Shahabuddin, Shiraz Hashmi, Syed Ezze Rakhshan, Jamal Kabeer Khan, Shahid Ahmed Sami, Muneer Amanullah
In the current era grown up congenital heart disease (GUCH) patients undergoing surgical interventions are increasing. Most of the interventions in the developed countries are either complex or redo-operations in patients who had previously undergone repair, palliation or correction. However, in the developing countries most of the interventions are primary and corrective. This descriptive retrospective study comprised GUCH patients who underwent surgical intervention for congenital heart disease (CHD) at Aga Khan University Hospital, Karachi, from January 2006 to December 2015...
October 2016: JPMA. the Journal of the Pakistan Medical Association
Fortunato Iacovelli, Martino Pepe, Gaetano Contegiacomo, Vito Alberotanza, Filippo Masi, Alessandro Santo Bortone, Stefano Favale
[This corrects the article DOI: 10.1155/2016/5482578.].
2016: Case Reports in Cardiology
Marco Astengo, Caroline Berntsson, Åse A Johnsson, Peter Eriksson, Mikael Dellborg
OBJECTIVE: Coarctation of the aorta (CoA) is a common condition. Adult patients with newly diagnosed CoA and patients with recurring or residual CoA require evaluation of the severity of aortic obstruction. Cardiac catheterization is considered the gold standard for the evaluation of hemodynamically significant CoA. The European Society of Cardiology (ESC) Guidelines for the management of grown-up congenital heart disease (GUCH) include noninvasive criteria for identifying significant CoA...
October 25, 2016: 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...
November 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
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