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Sample records for congenital complete heart

  1. Remission of congenital complete heart block without anti-Ro/La antibodies: A case report

    Directory of Open Access Journals (Sweden)

    Souvik Mitra

    2013-01-01

    Full Text Available Anti-Ro/La negative congenital heart block (CHB is uncommon. We report one such case of CHB, with no associated structural heart disease or maternal autoantibodies. The heart block reverted to sinus rhythm spontaneously at two weeks of age, and the patient remains in sinus rhythm at a one year followup. Whether patients with antibody negative complete heart block have a different clinical course is conjectural.

  2. Exercise Performance in Children and Young Adults After Complete and Incomplete Repair of Congenital Heart Disease.

    Science.gov (United States)

    Rosenblum, Omer; Katz, Uriel; Reuveny, Ronen; Williams, Craig A; Dubnov-Raz, Gal

    2015-12-01

    Few previous studies have addressed exercise capacity in patients with corrected congenital heart disease (CHD) and significant anatomical residua. The aim of this study was to determine the aerobic fitness and peak cardiac function of patients with corrected CHD with complete or incomplete repairs, as determined by resting echocardiography. Children, adolescents and young adults (heart defect had decreased aerobic fitness, with evidence of impaired peak cardiac function and lower pulmonary perfusion. Patients that had undergone a complete repair had decreased aerobic fitness attributed only to deconditioning. These newly identified differences explain why in previous studies, the lowest fitness was seen in patients with the most hemodynamically significant heart malformations.

  3. Genotypic and phenotypic predictors of complete heart block and recovery of conduction after surgical repair of congenital heart disease.

    Science.gov (United States)

    Murray, Laura E; Smith, Andrew H; Flack, English C; Crum, Kim; Owen, Jill; Kannankeril, Prince J

    2017-03-01

    Complete heart block (CHB) is a major complication that occurs after congenital heart surgery. We hypothesized that genetic and clinical factors are associated with the development of postoperative CHB and recovery of atrioventricular (AV) conduction. The purpose of this study was to identify predictors of CHB and recovery after congenital heart surgery. Patients undergoing congenital heart surgery at our institution from September 2007 through June 2015 were prospectively enrolled in a parent study of postoperative arrhythmias. Patients with onset of CHB within 48 hours postoperatively were included in the study. Daily rhythm assessment was performed until demonstration of 1:1 conduction or pacemaker implantation. Of 1199 subjects enrolled, 56 (4.7%) developed postoperative CHB. In multivariate analysis, preoperative digoxin exposure (odds ratio [OR] 2.4, 95% confidence interval [CI] 1.3-4.4), aortic cross-clamp time (OR 1.08, 95% CI 1.04-1.11), ventricular septal defect closure (OR 2.2, 95% CI 1.2-4.1), and a common polymorphism in the gene encoding connexin-40 (GJA5 rs10465885 TT genotype; OR 2.1, 95% CI 1.2-3.8) were independently associated with postoperative CHB. Junctional acceleration (JA) (OR 4.0, 95% CI 1.1-15.1) and intermittent conduction noted during complete AV block (OR 9.1, 95% CI 1.0-80) were independently associated with 1:1 AV conduction recovery. Use of a multivariate model including both JA and intermittent conduction demonstrated good discrimination with a positive predictive value of 86% (95% CI 67%-96%) in predicting 1:1 conduction recovery. Preoperative factors, including a missense polymorphism in GJA5, are independently associated with increased risk for CHB. JA and intermittent conduction may prove useful in predicting recovery of AV conduction among patients with CHB after congenital heart surgery. Copyright © 2016 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  4. Congenital Heart Information Network

    Science.gov (United States)

    ... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

  5. Association of temporary complete AV block and junctional ectopic tachycardia after surgery for congenital heart disease.

    Science.gov (United States)

    Paech, Christian; Dähnert, Ingo; Kostelka, Martin; Mende, Meinhardt; Gebauer, Roman

    2015-01-01

    Junctional ectopic tachycardia (JET) is a postoperative complication with a mortality rate of up to 14% after surgery for congenital heart disease. This study evaluated the risk factors of JET and explored the association of postoperative temporary third degree atrioventricular (AV) block and the occurrence of JET. Data were collected retrospectively from 1158 patients who underwent surgery for congenital heart disease. The overall incidence of JET was 2.8%. Temporary third degree AV block occurred in 1.6% of cases. Permanent third degree AV block requiring pacemaker implantation occurred in 1% of cases. In all, 56% of patients with JET had temporary AV block (P < 0.001), whereas no case of postoperative JET was reported in patients with permanent AV block (P = 0.56). temporary third degree AV block did not suffer from JET. A correlation between temporary third degree AV block and postoperative JET could be observed. The risk factors identified for JET include younger age groups at the time of surgery, longer aortic cross clamping time and surgical procedures in proximity to the AV node.

  6. Association of temporary complete AV block and junctional ectopic tachycardia after surgery for congenital heart disease

    Directory of Open Access Journals (Sweden)

    Christian Paech

    2015-01-01

    Full Text Available Aim: Junctional ectopic tachycardia (JET is a postoperative complication with a mortality rate of up to 14% after surgery for congenital heart disease. This study evaluated the risk factors of JET and explored the association of postoperative temporary third degree atrioventricular (AV block and the occurrence of JET. Materials and Methods: Data were collected retrospectively from 1158 patients who underwent surgery for congenital heart disease. Results: The overall incidence of JET was 2.8%. Temporary third degree AV block occurred in 1.6% of cases. Permanent third degree AV block requiring pacemaker implantation occurred in 1% of cases. In all, 56% of patients with JET had temporary AV block (P < 0.001, whereas no case of postoperative JET was reported in patients with permanent AV block (P = 0.56. temporary third degree AV block did not suffer from JET. Conclusions: A correlation between temporary third degree AV block and postoperative JET could be observed. The risk factors identified for JET include younger age groups at the time of surgery, longer aortic cross clamping time and surgical procedures in proximity to the AV node.

  7. Techniques for transcatheter recanalization of completely occluded vessels and pathways in patients with congenital heart disease

    Directory of Open Access Journals (Sweden)

    Latson Larry

    2010-01-01

    Full Text Available Occlusions of major vessels in patients with congenital heart disease may occur due to a variety of factors. These occlusions are often felt to be best addressed surgically; however, we and others have been successful in recanalizing most of these vessels in the catheterization laboratory. Most of these patients will require multiple procedures in the catheterization laboratory to ensure vessel patency and to facilitate vessel growth. Physicians performing the procedure should have a thorough understanding of the anatomic considerations for the intended procedure and have access to a variety of devices and equipment to optimize the result of the procedure. In this article, we review some of the technical aspects that are vital for the success of the procedure.

  8. Congenital Heart Defects

    Science.gov (United States)

    ... of the heart. It is present at birth. Congenital heart defects are the most common type of birth defect. The defects can involve the ... and heart transplants. The treatment depends on the type of the defect, how ... and general health. NIH: National Heart, Lung, and Blood Institute

  9. Congenital heart disease

    Science.gov (United States)

    ... for acne, chemicals, alcohol, and infections (such as rubella ) during pregnancy can contribute to some congenital heart problems. Poorly ... medicines. Have a blood test early in your pregnancy to see if you are immune to rubella. If you are not immune, avoid any possible ...

  10. Isolated congenital complete heart block in a five-year-old seronegative girl born to a woman seropositive for human immunodeficiency virus: a case report.

    Science.gov (United States)

    Pallangyo, Pedro; Mawenya, Isaac; Nicholaus, Paulina; Mayala, Henry; Kalombola, Amida; Sharau, Godwin; Majani, Naiz; Janabi, Mohamed

    2016-10-19

    Congenital complete heart block is a life-threatening condition which is highly associated with autoimmune and connective tissue disorders. Presence of maternal autoantibodies for associated conditions increases the risk of delivering a child with congenital complete heart block, however, less than a half of all women with such antibodies are symptomatic even after delivery. Mortality rate is highest during the neonatal period (45 %) and about two-thirds of all cases will require permanent pacing at some point in their lives. We report a case of isolated complete heart block in a 5-year-old HIV-free girl of African descent born to an HIV-infected woman with no prior history of autoimmune disorders. She was referred to us with chief complaints of recurrent syncopal attacks and effort intolerance since birth. A physical examination was unremarkable except for her being small for her age (body mass index 16.3 kg/m2) and bradycardia. Her vital signs were within acceptable range with the exception of her pulse rate, which ranged between 22 and 34 beats/minute. An echocardiogram revealed a sinus bradycardia, otherwise a structurally normal heart. An electrocardiogram showed atrioventricular dissociation in keeping with third-degree atrioventricular block. The child underwent a permanent epicardial pacemaker insertion and has been symptom-free following pacing. Despite its infrequency and life-threatening potential, patients with congenital complete heart block have an excellent survival rate with timely diagnosis and intervention. An incidental detection of bradycardia in a fetus during routine obstetrical ultrasound examination should increase the index of suspicion for congenital complete heart block and warrant a screening for associated maternal autoantibodies.

  11. Case studies of the perceptions of women with high risk congenital heart disease successfully completing a pregnancy.

    Science.gov (United States)

    Ngu, Kylie; Hay, Margaret; Menahem, Samuel

    2014-09-01

    Women even with moderate to severe congenital heart disease (CHD) seek motherhood despite posing significant health risks to themselves and their infant. This study explored their motivations and perceptions and compared them to those of women with low risk CHD who conceived. Twenty women over 18 years with CHD who had a successful pregnancy were recruited, half of whom were identified as having a high risk cardiac abnormality. They completed a questionnaire and a semi-structured interview following which a thematic analysis was employed. Their medical records and clinical status were also reviewed and their current cardiac status graded by their attending cardiologist. Women with high risk (moderate to severe) CHD (n=10) appeared to have similar motivations for conceiving as women with low-risk (mild) CHD (n=10). Their decision to conceive seemed based on their own and at times unrealistic perceptions of the consequences of their CHD. Women with mild or more severe CHD had similar motivations to conceive tending to down play the seriousness of their CHD. Their drive for motherhood appeared to be stronger than the drive for self care. It behoves clinicians, both obstetricians and cardiologists caring for women with high risk CHD to be knowledgeable of the effects of the CHD on the pregnancy and the impact of the pregnancy on the cardiac status. Copyright © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  12. Congenital Heart Defects (For Parents)

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    ... and related health problems. Common Heart Defects Common types of congenital heart defects, which can affect any part of the heart ... circulation. Echocardiograms are the primary tool for diagnosing congenital ... is a specialized type of ultrasound that allows diagnosis of heart problems ...

  13. Isolated dextrocardia and congenital heart blocking

    OpenAIRE

    Khoury, Maurice; Harbieh, Bernard; Heriopian, Aline

    2015-01-01

    To our knowledge, isolated dextrocardia in association with congenital complete atrioventricular heart block has not been previously reported. We report such a case, elaborate on the possible pathogenesis, and present our experience with management.

  14. Congenital Heart Diseases associated with Identified Syndromes ...

    African Journals Online (AJOL)

    BACKGROUND: Congenital heart diseases are commonly associated with other extra cardiac congenital malformations. OBJECTIVE: To identify congenital heart diseases associated with identified syndromes and other extra cardiac congenital malformations in children in our hospital. METHODS: A prospective descriptive ...

  15. Anaesthetic management of emergency pacemaker implantation in a case of neonatal lupus erythematosus with complete congenital heart block & severe respiratory distress

    Directory of Open Access Journals (Sweden)

    Usha Kiran

    2007-01-01

    Full Text Available An 8-week old 3-kilogram male baby was brought to this tertiary care hospital with respiratory distress, marked tracheal tug, poor feeding and a heart rate of 46/minute. The child had been referred from a peripheral hospital as a case of neonatal lupus with complete congenital heart block. The mother was seropositive for systemic lupus erythematosus with a history of two abortions. Evaluation on admission revealed a heart rate between 40-60/ minute, respiratory rate 40-50/ minute, inspiratory stridor, bilateral crepitations, chest retrac-tion and a marked tracheal tug that improved with prone positioning. Electrocardiography and echocardiography confirmed complete congenital heart block with cardiomegaly and mild left ventricular dysfunction. Keeping in view the impending congestive heart failure, possible early cardiomyopathy and the bad obstetric history ur-gent pacemaker implantation was planned to allow early recovery of the child. The anaesthetic risk was high due to the heart block, ventricular dysfunction, laryngomalacia, severe tracheal tug and anticipated difficult weaning from controlled ventilation. General anaesthesia was administered with endotracheal tube and con-trolled ventilation using ketamine, rocuronium and sufentanil. For patient safety invasive monitoring was pro-vided and external pacing was kept standby. Epicardial pacemaker leads were implanted onto the left ventricu-lar wall through a left anterior 6th intercostal space thoracotomy. The child was electively ventilated for two post operative days. The tracheal tug and secretions gradually subsided over 2 weeks with oxygen, antibiotics, steroids, bronchodilators and physiotherapy. At the time of discharge from hospital 2 weeks after the implant the child was feeding well, tracheal tug was minimal and the lungs were clear.

  16. Congenital heart defect corrective surgeries

    Science.gov (United States)

    ... born with one or more heart defects has congenital heart disease . Surgery is needed if the defect could harm the child's long term health or well-being. Description There are many types of pediatric heart surgery . Patent ductus arteriosus (PDA) ...

  17. [Genetics of congenital heart diseases].

    Science.gov (United States)

    Bonnet, Damien

    2017-06-01

    Developmental genetics of congenital heart diseases has evolved from analysis of serial slices in embryos towards molecular genetics of cardiac morphogenesis with a dynamic view of cardiac development. Genetics of congenital heart diseases has also changed from formal genetic analysis of familial recurrences or population-based analysis to screening for mutations in candidates genes identified in animal models. Close cooperation between molecular embryologists, pathologists involved in heart development and pediatric cardiologists is crucial for further increase of knowledge in the field of cardiac morphogenesis and genetics of cardiac defects. The genetic model for congenital heart disease has to be revised to favor a polygenic origin rather than a monogenic one. The main mechanism is altered genic dosage that can account for heart diseases in chromosomal anomalies as well as in point mutations in syndromic and isolated congenital heart diseases. The use of big data grouping information from cardiac development, interactions between genes and proteins, epigenetic factors such as chromatin remodeling or DNA methylation is the current source for improving our knowledge in the field and to give clues for future therapies. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  18. Genetic Counseling for Congenital Heart Defects

    Science.gov (United States)

    ... Artery Disease Venous Thromboembolism Aortic Aneurysm More Genetic Counseling for Congenital Heart Defects Updated:Jan 19,2018 ... with congenital heart disease considers having children. Genetic counseling can help answer these questions and address your ...

  19. Bradyarrhythmias in Congenital Heart Disease.

    Science.gov (United States)

    Carlson, Steven K; Patel, Akash R; Chang, Philip M

    2017-06-01

    Bradyarrhythmias in adults with congenital heart disease (CHD) comprise a complex group of arrhythmia disorders with congenital and acquired origins, highly variable long-term sequelae, and complicated treatment options. They can develop across the spectrum of CHD defects and can be encountered at all ages. Although permanent pacing is effective in treating bradyarrhythmias, it is associated with many complications and morbidity, where it is often used early in life. This section discusses the incidence and prevalence of bradyarrhythmias in the CHD population, their timing of occurrence with respect to specific disease entities and interventions, and their short- and long-term clinical sequelae. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. Implantation of Total Artificial Heart in Congenital Heart Disease

    OpenAIRE

    Adachi, Iki; Morales, David S. L.

    2014-01-01

    In patients with end-stage heart failure (HF), a total artificial heart (TAH) may be implanted as a bridge to cardiac transplant. However, in congenital heart disease (CHD), the malformed heart presents a challenge to TAH implantation.

  1. In-Hospital Vital Status and Heart Transplants After Intervention for Congenital Heart Disease in the Pediatric Cardiac Care Consortium: Completeness of Ascertainment Using the National Death Index and United Network for Organ Sharing Datasets.

    Science.gov (United States)

    Spector, Logan G; Menk, Jeremiah S; Vinocur, Jeffrey M; Oster, Matthew E; Harvey, Brian A; St Louis, James D; Moller, James; Kochilas, Lazaros K

    2016-08-09

    The long-term outcomes of patients undergoing interventions for congenital heart disease (CHD) remain largely unknown. We linked the Pediatric Cardiac Care Consortium (PCCC) with the National Death Index (NDI) and the United Network for Organ Sharing Dataset (UNOS) registries to study mortality and transplant occurring up to 32 years postintervention. The objective of the current analysis was to determine the sensitivity of this linkage in identifying patients who are known to have died or undergone heart transplant. We used direct identifiers from 59 324 subjects registered in the PCCC between 1982 and 2003 to test for completeness of case ascertainment of subjects with known vital and heart transplant status by linkage with the NDI and UNOS registries. Of the 4612 in-hospital deaths, 3873 were identified by the NDI as "true" matches for a sensitivity of 84.0% (95% CI, 82.9-85.0). There was no difference in sensitivity across 25 congenital cardiovascular conditions after adjustment for age, sex, race, presence of first name, death year, and residence at death. Of 455 known heart transplants in the PCCC, there were 408 matches in the UNOS registry, for a sensitivity of 89.7% (95% CI, 86.9-92.3). An additional 4851 deaths and 363 transplants that occurred outside the PCCC were identified through 2014. The linkage of the PCCC with the NDI and UNOS national registries is feasible with a satisfactory sensitivity. This linkage provides a conservative estimate of the long-term death and heart transplant events in this cohort. © 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

  2. Predictors of pregnancy complications in women with congenital heart disease

    NARCIS (Netherlands)

    Drenthen, Willem; Boersma, Eric; Balci, Ali; Moons, Philip; Roos-Hesselink, Jolien W.; Mulder, Barbara J. M.; Vliegen, Hubert W.; van Dijk, Arie P. J.; Voors, Adriaan A.; Yap, Sing C.; van Veldhuisen, Dirk J.; Pieper, Petronella G.

    Aims Data regarding pregnancy outcome in women with congenital heart disease (CHD) are limited. Methods and results In 1802 women with CHD, 1302 completed pregnancies were observed. Independent predictors of cardiac, obstetric, and neonatal complications were calculated using logistic regression.

  3. Cyanotic congenital heart disease and atherosclerosis

    DEFF Research Database (Denmark)

    Tarp, Julie Bjerre; Jensen, Annette Schophuus; Engstrøm, Thomas

    2017-01-01

    Improved treatment options in paediatric cardiology and congenital heart surgery have resulted in an ageing population of patients with cyanotic congenital heart disease (CCHD). The risk of acquired heart disease such as atherosclerosis increases with age.Previous studies have speculated whether...

  4. Genetic Syndromes associated with Congenital Heart Disease

    OpenAIRE

    Ko, Jung Min

    2015-01-01

    Recent research has demonstrated that genetic alterations or variations contribute considerably to the development of congenital heart disease. Many kinds of genetic tests are commercially available, and more are currently under development. Congenital heart disease is frequently accompanied by genetic syndromes showing both cardiac and extra-cardiac anomalies. Congenital heart disease is the leading cause of birth defects, and is an important cause of morbidity and mortality during infancy a...

  5. Hyperuricaemia in congenital heart disease patients.

    Science.gov (United States)

    Martínez-Quintana, Efrén; Rodríguez-González, Fayna

    2015-01-01

    Hyperuricaemia is associated with traditional cardiovascular risk factors such as type 2 diabetes or dyslipidaemia and a higher mortality. Out of 528 congenital heart disease patients, 329 patients, including 190 male and 139 female patients, in whom uric acid determination was performed, were studied and followed up to determine survival. Male congenital heart disease patients with high serum uric acid concentrations (>7 mg/dl) showed significantly (p congenital heart disease patients with lower serum uric acid levels (≤7 mg/dl). Meanwhile, female congenital heart disease patients with higher serum uric acid concentrations (>5.7 mg/dl) were significantly (p congenital heart disease patients with lower serum uric acid concentrations (≤5.7 mg/dl). During a median follow-up of 90 months, 16 out of 528 congenital heart disease patients died - 14 patients of cardiac origin and two patients of non-cardiac origin - of whom 10 were hypoxaemic. Kaplan-Meier analysis showed no significant differences in mortality between male and female congenital heart disease patients with high and low serum uric acid level concentrations. Hypoxaemia, body mass index, and C-reactive protein concentrations are higher in hyperuricaemic congenital heart disease patients, although no significant differences were seen in mortality between congenital heart disease patients with high and low serum uric acid concentrations.

  6. History of the Congenital Heart Surgeons' Society.

    Science.gov (United States)

    Mavroudis, Constantine; Williams, William G

    2015-10-01

    The Congenital Heart Surgeons' Society is a group of over 100 pediatric heart surgeons representing 72 institutions that specialize in the treatment of patients with congenital heart defects. The Society began in 1972 and incorporated as a not-for-profit charitable organization in 2004. It has become the face and voice of congenital heart surgery in North America. In 1985, the Society established a data center for multicenter clinical research studies to encourage congenital heart professionals to participate in improving outcomes for our patients. The goals of the Congenital Heart Surgeons' Society are to stimulate the study of congenital cardiac physiology, pathology, and management options which are instantiated in data collection, multi-institutional studies, and scientific meetings. Honest and open discussion of problems with possible solutions to the challenges facing congenital heart professionals have been the strength of the Congenital Heart Surgeons' Society. It is imperative for the growth of an organization to know from where it came in order to know to where it is going. The purpose of this article is to review the history of the Congenital Heart Surgeons' Society. © The Author(s) 2015.

  7. The Danish Register of Congenital Heart Disease.

    Science.gov (United States)

    Olsen, Morten; Videbæk, Jørgen; Johnsen, Søren Paaske

    2011-07-01

    Congenital heart defects (CHD) constitute the largest group of congenital defects with a prevalence at birth of 5-11 per 1000 live births, and the population of adults with CHD is increasing. However, few population-based long-term outcome data exist. The Danish Register of Congenital Heart Disease holds data on patients diagnosed with CHD since 1963 and patients below 25 years of age with other types of heart disease. Overall and defect specific validation is ongoing. Together with other Danish registers, the Danish Register of Congenital Heart Disease provides extensive research possibilities.

  8. Heart transplantation in adult congenital heart disease.

    Science.gov (United States)

    Burchill, Luke J

    2016-12-01

    Heart failure (HF) in adult congenital heart disease (ACHD) is vastly different to that observed in acquired heart disease. Unlike acquired HF in which pharmacological strategies are the cornerstone for protecting and improving ventricular function, ACHD-related HF relies heavily upon structural and other interventions to achieve these aims. patients with ACHD constitute a small percentage of the total adult heart transplant population (∼3%), although the number of ACHD heart transplant recipients is growing rapidly with a 40% increase over the last two decades. The worldwide experience to date has confirmed heart transplantation as an effective life-extending treatment option in carefully selected patients with ACHD with end-stage cardiac disease. Opportunities for improving outcomes in patients with ACHD-related HF include (i) earlier recognition and referral to centres with combined expertise in ACHD and HF, (ii) increased awareness of arrhythmia and sudden cardiac death risk in this population, (iii) greater collaboration between HF and ACHD specialists at the time of heart transplant assessment, (iv) expert surgical planning to reduce ischaemic time and bleeding risk at the time of transplant, (v) tailored immunosuppression in the post-transplant period and (vi) development and validation of ACHD-specific risk scores to predict mortality and guide patient selection. The purpose of this article is to review current approaches to diagnosing and treating advanced HF in patients with ACHD including indications, contraindications and clinical outcomes after heart transplantation. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  9. Surgery in adults with congenital heart disease

    NARCIS (Netherlands)

    Zomer, A. C.; Verheugt, C. L.; Vaartjes, I.; Uiterwaal, C. S. P. M.; Langemeijer, M. M.; Koolbergen, D. R.; Hazekamp, M. G.; van Melle, J. P.; Konings, T. C.; Bellersen, L.; Grobbee, D. E.; Mulder, B. J. M.

    2011-01-01

    A significant proportion of patients with congenital heart disease require surgery in adulthood. We aimed to give an overview of the prevalence, distribution, and outcome of cardiovascular surgery for congenital heart disease. We specifically questioned whether the effects of surgical treatment on

  10. The changing epidemiology of congenital heart disease

    NARCIS (Netherlands)

    van der Bom, Teun; Zomer, A. Carla; Zwinderman, Aeilko H.; Meijboom, Folkert J.; Bouma, Berto J.; Mulder, Barbara J. M.

    2011-01-01

    Congenital heart disease is the most common congenital disorder in newborns. Advances in cardiovascular medicine and surgery have enabled most patients to reach adulthood. Unfortunately, prolonged survival has been achieved at a cost, as many patients suffer late complications, of which heart

  11. Nutritional treatment of congenital heart disease.

    OpenAIRE

    Bougle, D; Iselin, M; Kahyat, A; Duhamel, J F

    1986-01-01

    Twelve of 13 patients with congenital heart disease given continuous enteral nutrition displayed normal growth; cardiac function remained stable or improved in 10 in spite of the water load (146 +/- 22 ml/kg/day). This is safe treatment for malnutrition in congenital heart disease.

  12. Congenital heart defects in Kabuki syndrome.

    Science.gov (United States)

    Yuan, Shi-Min

    2013-01-01

    Kabuki syndrome (KS) is an entity of multiple congenital malformations with mental retardation with undetermined etiology. Congenital heart defects are one of the clinical manifestations of KS with insufficient elucidations. Literature of congenital heart defects associated with KS was comprehensively retrieved, collected and reviewed. The clinical features of the congenital heart defects in the patients with KS were summarized. Congenital heart defects were one of the clinical manifestations of KS with 90.6% of the patients being diagnosed prenatally or at an early age. Left-sided obstructions/aortic dilation and septal defects were the fi rst two types of anomalies, accounting up to 46.1% and 32.9%, respectively. The most common congenital heart defects were coarctation of the aorta, and atrial and ventricular septal defects. Fifteen (19.7%) patients received surgical repair of congenital heart defects at a mean age of 0.8 ± 1.3 years. Congenital heart defects are one of the clinical manifestations of KS with 90.6% of the patients being diagnosed prenatally or at an early age. About 20% of the patients warranted surgical repair of the heart defects. Patients with KS require close follow-up in terms of their etiology, clinical presentations and long-term prognosis.

  13. Mortality in adult congenital heart disease

    NARCIS (Netherlands)

    Verheugt, Carianne L.; Uiterwaal, Cuno S. P. M.; van der Velde, Enno T.; Meijboom, Folkert J.; Pieper, Petronella G.; van Dijk, Arie P. J.; Vliegen, Hubert W.; Grobbee, Diederick E.; Mulder, Barbara J. M.

    Mortality in adults with congenital heart disease is known to be increased, yet its extent and the major mortality risks are unclear. The Dutch CONCOR national registry for adult congenital heart disease was linked to the national mortality registry. Cox's regression was used to assess mortality

  14. Heart transplantation in adults with congenital heart disease.

    Science.gov (United States)

    Houyel, Lucile; To-Dumortier, Ngoc-Tram; Lepers, Yannick; Petit, Jérôme; Roussin, Régine; Ly, Mohamed; Lebret, Emmanuel; Fadel, Elie; Hörer, Jürgen; Hascoët, Sébastien

    2017-05-01

    With the advances in congenital cardiac surgery and postoperative care, an increasing number of children with complex congenital heart disease now reach adulthood. There are already more adults than children living with a congenital heart defect, including patients with complex congenital heart defects. Among these adults with congenital heart disease, a significant number will develop ventricular dysfunction over time. Heart failure accounts for 26-42% of deaths in adults with congenital heart defects. Heart transplantation, or heart-lung transplantation in Eisenmenger syndrome, then becomes the ultimate therapeutic possibility for these patients. This population is deemed to be at high risk of mortality after heart transplantation, although their long-term survival is similar to that of patients transplanted for other reasons. Indeed, heart transplantation in adults with congenital heart disease is often challenging, because of several potential problems: complex cardiac and vascular anatomy, multiple previous palliative and corrective surgeries, and effects on other organs (kidney, liver, lungs) of long-standing cardiac dysfunction or cyanosis, with frequent elevation of pulmonary vascular resistance. In this review, we focus on the specific problems relating to heart and heart-lung transplantation in this population, revisit the indications/contraindications, and update the long-term outcomes. Copyright © 2017. Published by Elsevier Masson SAS.

  15. Health in adults with congenital heart disease.

    Science.gov (United States)

    Cuypers, Judith A A E; Utens, Elisabeth M W J; Roos-Hesselink, Jolien W

    2016-09-01

    Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many adults with congenital heart disease encounter late complications. Late morbidity can be related to the congenital heart defect itself, but may also be the consequence of the surgical or medical treatment or longstanding alterations in hemodynamics, neurodevelopment and psychosocial development. This narrative review describes the cardiac and non-cardiac long-term morbidity in the adult population with congenital heart disease. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  16. Sleep in infants with congenital heart disease.

    Science.gov (United States)

    Ykeda, Daisy Satomi; Lorenzi-Filho, Geraldo; Lopes, Antonio A B; Alves, Rosana S C

    2009-01-01

    To investigate hypoxia and sleep disordered breathing in infants with congenital heart disease. Prospective study. In-hospital full polysomnography was performed on 14 infants with congenital heart disease, age 7 +/-1 months, and in 7 normal infants, age 10 +/-2 months. Congenital heart disease infants were classified as acyanotic (n=7) or cyanotic (n=7). Nutritional status, assessed by the Gomez classification and expressed as % weight for age, was 70 +/-7, 59 +/-11 and 94 +/-16 in the acyanotic, cyanotic congenital heart disease and control infants, respectively (pcongenital heart disease infants (11 out of 14) and only one control infant had an AHI >1 event/hour. The minimum oxygen saturation was 79% (74-82), 73% (57-74) and 90% (90-91) in the acyanotic, cyanotic congenital heart disease infants and controls, respectively (p congenital heart disease frequently present with sleep-disordered breathing associated with oxygen desaturations but not arousals. Therefore, sleep may represent a significant burden to infants with congenital heart disease.

  17. Adult congenital heart disease: a growing epidemic.

    Science.gov (United States)

    Ávila, Pablo; Mercier, Lise-Andrée; Dore, Annie; Marcotte, François; Mongeon, François-Pierre; Ibrahim, Reda; Asgar, Anita; Miro, Joaquim; Andelfinger, Gregor; Mondésert, Blandine; de Guise, Pierre; Poirier, Nancy; Khairy, Paul

    2014-12-01

    Medical and surgical breakthroughs in the care of children born with heart defects have generated a growing population of adult survivors and spawned a new subspecialty of cardiology: adult congenital heart disease. The prevalence of adult congenital heart disease is escalating at a rampant rate, outpacing the relatively static prevalence of pediatric congenital heart disease, because adults now surpass children in numbers by a ratio of 2:1. As such, congenital heart disease can no longer be considered primarily a pediatric specialty. Most congenital heart defects are not curable and require lifelong specialized care. Health care systems worldwide are challenged to meet the unique needs of this increasingly complex patient population, including the development of supraregional centres of excellence to provide comprehensive and multidisciplinary specialized care. In this review, we explore the incidence and prevalence of congenital heart disease and their changing patterns, address organization and delivery of care, highlight the importance of appropriate training and dedicated research, summarize the high burden of health care resource utilization, and provide an overview of common issues encountered in adults with congenital heart disease. Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  18. Transcription Factor Pathways and Congenital Heart Disease

    Science.gov (United States)

    McCulley, David J.; Black, Brian L.

    2013-01-01

    Congenital heart disease is a major cause of morbidity and mortality throughout life. Mutations in numerous transcription factors have been identified in patients and families with some of the most common forms of cardiac malformations and arrhythmias. This review discusses factor pathways known to be important for normal heart development and how abnormalities in these pathways have been linked to morphological and functional forms of congenital heart defects. A comprehensive, current list of known transcription factor mutations associated with congenital heart disease is provided, but the review focuses primarily on three key transcription factors, Nkx2-5, GATA4, and Tbx5, and their known biochemical and genetic partners. By understanding the interaction partners, transcriptional targets, and upstream activators of these core cardiac transcription factors, additional information about normal heart formation and further insight into genes and pathways affected in congenital heart disease should result. PMID:22449847

  19. DNA methylation abnormalities in congenital heart disease

    OpenAIRE

    Serra-Juh?, Clara; Cusc?, Ivon; Homs, A?da; Flores, Raquel; Tor?n, N?ria; P?rez-Jurado, Luis A

    2015-01-01

    Congenital heart defects represent the most common malformation at birth, occurring also in ∼50% of individuals with Down syndrome. Congenital heart defects are thought to have multifactorial etiology, but the main causes are largely unknown. We have explored the global methylation profile of fetal heart DNA in comparison to blood DNA from control subjects: an absolute correlation with the type of tissue was detected. Pathway analysis revealed a significant enrichment of differential methylat...

  20. Anesthetic drugs in congenital heart disease.

    Science.gov (United States)

    Friesen, Robert H

    2014-12-01

    The structural defects associated with the various forms of congenital heart disease lead to pathological and functional changes that place patients at risk for adverse events, and in fact the perioperative incidence of morbidity and mortality has been documented to be increased in children with congenital heart disease. Patients with congenital heart disease can present to the anesthesiologist in a relatively precarious state of balance of several hemodynamic factors, including preload, ventricular contractility, systemic vascular resistance, pulmonary vascular resistance, heart rate, and cardiac rhythm. Anesthetic drugs can affect each of these, and an ideal anesthetic drug for such patients does not exist. The purpose of this article is to review the hemodynamic effects of anesthetic drugs and how they may contribute to the occurrence of adverse events in children with congenital heart disease. © The Author(s) 2014.

  1. [Sex differences in congenital heart disease].

    Science.gov (United States)

    Aubry, P; Demian, H

    2016-12-01

    Gender influences the clinical presentation and the management of some acquired cardiovascular diseases, such as coronary artery disease, resulting in different outcomes. Differences between women and men are also noticed in congenital heart disease. They are mainly related to the prevalence and severity of some congenital heart defects at birth, and in adulthood to the prognosis, incidence of Eisenmenger syndrome and risks of pregnancy. The role of gender on the risk of operative mortality of congenital heart surgery remains debated. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  2. Cardiac Biomarkers in Adult Congenital Heart Disease

    NARCIS (Netherlands)

    J.A. Eindhoven (Jannet)

    2015-01-01

    markdownabstract__Abstract__ Congenital heart disease (ConHD) is the most common congenital abnormality in newborns, with a birth prevalence of 9 per 1000 live births.2 ConHD comprises a number of cardiac abnormalities with varying aetiology which can be divided into simple, moderate and

  3. DNA methylation abnormalities in congenital heart disease.

    Science.gov (United States)

    Serra-Juhé, Clara; Cuscó, Ivon; Homs, Aïda; Flores, Raquel; Torán, Núria; Pérez-Jurado, Luis A

    2015-01-01

    Congenital heart defects represent the most common malformation at birth, occurring also in ∼50% of individuals with Down syndrome. Congenital heart defects are thought to have multifactorial etiology, but the main causes are largely unknown. We have explored the global methylation profile of fetal heart DNA in comparison to blood DNA from control subjects: an absolute correlation with the type of tissue was detected. Pathway analysis revealed a significant enrichment of differential methylation at genes related to muscle contraction and cardiomyopathies in the developing heart DNA. We have also searched for abnormal methylation profiles on developing heart-tissue DNA of syndromic and non-syndromic congenital heart defects. On average, 3 regions with aberrant methylation were detected per sample and 18 regions were found differentially methylated between groups. Several epimutations were detected in candidate genes involved in growth regulation, apoptosis and folate pathway. A likely pathogenic hypermethylation of several intragenic sites at the MSX1 gene, involved in outflow tract morphogenesis, was found in a fetus with isolated heart malformation. In addition, hypermethylation of the GATA4 gene was present in fetuses with Down syndrome with or without congenital heart defects, as well as in fetuses with isolated heart malformations. Expression deregulation of the abnormally methylated genes was detected. Our data indicate that epigenetic alterations of relevant genes are present in developing heart DNA in fetuses with both isolated and syndromic heart malformations. These epimutations likely contribute to the pathogenesis of the malformation by cis-acting effects on gene expression.

  4. Congenital Heart Defects and Physical Activity

    Science.gov (United States)

    ... Thromboembolism Aortic Aneurysm More Congenital Heart Defects and Physical Activity Updated:Sep 12,2017 Exercise Is for Everyone ... almost all patients do some form of regular physical activity. There are a few exceptions, so it's good ...

  5. Epidemiology of congenital heart disease in Brazil

    OpenAIRE

    Pinto Júnior,Valdester Cavalcante; Branco,Klébia Magalhães P. Castello; Cavalcante,Rodrigo Cardoso; Carvalho Junior,Waldemiro; Lima,José Rubens Costa; Freitas,Sílvia Maria de; Fraga,Maria Nazaré de Oliveira; Souza,Nayana Maria Gomes de

    2015-01-01

    AbstractIntroduction:Congenital heart disease is an abnormality in the structure or cardiocirculatory function, occurring from birth, even if diagnosed later. It can result in intrauterine death in childhood or in adulthood. Accounted for 6% of infant deaths in Brazil in 2007.Objective:To estimate underreporting in the prevalence of congenital heart disease in Brazil and its subtypes.Methods:The calculations of prevalence were performed by applying coefficients, giving them function rates for...

  6. Endothelial function state following repair of cyanotic congenital heart diseases.

    Science.gov (United States)

    Sabri, Mohammad Reza; Daryoushi, Hooman; Gharipour, Mojgan

    2015-02-01

    Repairing cyanotic congenital heart disease may be associated with preserving endothelial function. The present study aimed to evaluate vascular endothelial function in patients with repaired cyanotic congenital heart disease. In a case-control study conducted in 2012 in Isfahan, Iran, 42 consecutive patients aged types of cyanotic congenital heart disease and had undergone complete repair of their congenital heart defect were assessed in regard to their endothelial function state by measuring flow-mediated dilatation and other cardiac function indices. They were paired with 42 sex- and age-matched healthy controls. The mean flow-mediated dilatation was lower in patients with repaired cyanotic congenital heart disease than in the controls [6.14±2.78 versus 8.16±1.49 respectively (pcongenital heart disease that was repaired after 2.5 years of age (mean age at repair 9±6.1 years). Early repair of a cyanotic defect can result in the protection of vascular endothelial function and prevent the occurrence of vascular accidents at an older age.

  7. Permanent complete heart block following surgical correction of ...

    African Journals Online (AJOL)

    Background: The risk of complete heart block (CHB) from congenital heart repairs in Ghana is unknown. This information is important for referring physicians and in pre-operative counselling of patients and facilitates the process of obtaining informed consent for such repairs. Objectives: This study was undertaken to ...

  8. Pregnancy in women with congenital heart disease

    NARCIS (Netherlands)

    Greutmann, Matthias; Pieper, Petronella G.

    2015-01-01

    Congenital heart defects are the most common birth defects. Major advances in open-heart surgery have led to rapidly evolving cohorts of adult survivors and the majority of affected women now survive to childbearing age. The risk of cardiovascular complications during pregnancy and peripartum

  9. [Congenital heart diseases in clinical practice].

    Science.gov (United States)

    Ratti, Carlo; Veronesi, Benedetta; Grassi, Laura; Bompani, Bruno

    2012-05-01

    Congenital heart diseases are abnormalities in the heart's structure that are present at birth. Some are known to be associated with genetic disorders. They affect 8 out of every 1,000 newborns. They range from simple defects with no symptoms to complex defects. They are divided in two types: cyanotic and not cyanotic.

  10. Anticoagulation in adults with congenital heart disease

    DEFF Research Database (Denmark)

    Jensen, A S; Idorn, L; Nørager, B

    2015-01-01

    Adults with congenital heart disease are a growing population. One of the major challenges in the care of these patients is to prevent thromboembolic episodes. Despite relative young age and no typical cardiovascular risk factors, this cohort has a high prevalence of thrombotic events....... It is difficult to use treatment algorithms from the general adult population with acquired heart disease in this heterogeneous population due to special conditions such as myocardial scarring after previous surgery, atypical atrial flutter, prothrombotic conditions and the presence of interatrial shunts....... Furthermore, there is a lack of scientific evidence regarding how to prevent thromboembolic events with anticoagulation in adults with congenital heart disease. The aim of this paper is to review the current literature pertaining to anticoagulation in adults with congenital heart disease and hence enable...

  11. Employment after heart transplantation among adults with congenital heart disease.

    Science.gov (United States)

    Tumin, Dmitry; Chou, Helen; Hayes, Don; Tobias, Joseph D; Galantowicz, Mark; McConnell, Patrick I

    2017-12-01

    Adults with congenital heart disease may require heart transplantation for end-stage heart failure. Whereas heart transplantation potentially allows adults with congenital heart disease to resume their usual activities, employment outcomes in this population are unknown. Therefore, we investigated the prevalence and predictors of work participation after heart transplantation for congenital heart disease. Retrospective review of a prospective registry. United Network for Organ Sharing registry of transplant recipients in the United States. Adult recipients of first-time heart transplantation with a primary diagnosis of congenital heart disease, performed between 2004 and 2015. None. Employment status reported by transplant centers at required follow-up intervals up to 5 y posttransplant. Among 470 patients included in the analysis (mean follow-up: 5 ± 3 y), 127 (27%) worked after transplant, 69 (15%) died before beginning or returning to work, and 274 (58%) survived until censoring, but did not participate in paid work. Multivariable competing-risks regression analysis examined characteristics associated with posttransplant employment, accounting for mortality as a competing outcome. In descriptive and multivariable analysis, pretransplant work participation was associated with a greater likelihood of posttransplant employment, while the use of Medicaid insurance at the time of transplant was associated with a significantly lower likelihood of working after transplant (subhazard ratio compared to private insurance: 0.55; 95% confidence interval: 0.32, 0.95; P = .032). Employment was rare after heart transplantation for congenital heart disease, and was significantly less common than in the broader population of adults with congenital heart disease. Differences in return to work were primarily related to pretransplant employment and the use of public insurance, rather than clinical characteristics. © 2017 Wiley Periodicals, Inc.

  12. Towards defining heart failure in adults with congenital heart disease.

    Science.gov (United States)

    Bolger, Aidan P; Gatzoulis, Michael A

    2004-12-01

    Injury to the myocardium disrupts geometric integrity and results in changes to intracardiac pressure, wall stress and tension, and the pattern of blood flow through the heart. Significant disruption to pump function results in heart failure which is defined in terms of symptoms: breathlessness and fatigue, signs of salt and water retention, and neurohormonal activation. This syndrome most commonly occurs in the context of injury due to ischaemic heart disease and dilated cardiomyopathy but because patients with congenital heart disease (CHD) are born with sometimes gross distortions of cardiac anatomy they too are subject to the forces that drive heart failure. This paper explores the available data relating to the clinical and neurohormonal manifestations of heart failure in patients with congenital heart disease and describes how, by additionally exploring events at a cellular level, we may be able to arrive at a definition of heart failure relevant to this population.

  13. Predictors of pregnancy complications in women with congenital heart disease

    NARCIS (Netherlands)

    W. Drenthen (Willem); H. Boersma (Eric); A. Balci (Ali); P. Moons (Philip); J.W. Roos-Hesselink (Jolien); B.J.M. Mulder (Barbara); H.W. Vliegen (Hubert); A.P.J. van Dijk (Arie); A.A. Voors (Adriaan); S-C. Yap (Sing-Chien); D.J. van Veldhuisen (Dirk); P.G. Pieper (Petronella)

    2010-01-01

    textabstractAims:Data regarding pregnancy outcome in women with congenital heart disease (CHD) are limited.Methods and results: In 1802 women with CHD, 1302 completed pregnancies were observed. Independent predictors of cardiac, obstetric, and neonatal complications were calculated using logistic

  14. Genetic Syndromes associated with Congenital Heart Disease.

    Science.gov (United States)

    Ko, Jung Min

    2015-09-01

    Recent research has demonstrated that genetic alterations or variations contribute considerably to the development of congenital heart disease. Many kinds of genetic tests are commercially available, and more are currently under development. Congenital heart disease is frequently accompanied by genetic syndromes showing both cardiac and extra-cardiac anomalies. Congenital heart disease is the leading cause of birth defects, and is an important cause of morbidity and mortality during infancy and childhood. This review introduces common genetic syndromes showing various types of congenital heart disease, including Down syndrome, Turner syndrome, 22q11 deletion syndrome, Williams syndrome, and Noonan syndrome. Although surgical techniques and perioperative care have improved substantially, patients with genetic syndromes may be at an increased risk of death or major complications associated with surgery. Therefore, risk management based on an accurate genetic diagnosis is necessary in order to effectively plan the surgical and medical management and follow-up for these patients. In addition, multidisciplinary approaches and care for the combined extra-cardiac anomalies may help to reduce mortality and morbidity accompanied with congenital heart disease.

  15. Heart Failure in Pediatric Patients With Congenital Heart Disease.

    Science.gov (United States)

    Hinton, Robert B; Ware, Stephanie M

    2017-03-17

    Heart failure (HF) is a complex clinical syndrome resulting from diverse primary and secondary causes and shared pathways of disease progression, correlating with substantial mortality, morbidity, and cost. HF in children is most commonly attributable to coexistent congenital heart disease, with different risks depending on the specific type of malformation. Current management and therapy for HF in children are extrapolated from treatment approaches in adults. This review discusses the causes, epidemiology, and manifestations of HF in children with congenital heart disease and presents the clinical, genetic, and molecular characteristics that are similar or distinct from adult HF. The objective of this review is to provide a framework for understanding rapidly increasing genetic and molecular information in the challenging context of detailed phenotyping. We review clinical and translational research studies of HF in congenital heart disease including at the genome, transcriptome, and epigenetic levels. Unresolved issues and directions for future study are presented. © 2017 American Heart Association, Inc.

  16. Congenital heart defects in children with oral clefts

    OpenAIRE

    Nahvi H.; Mollaeian M; Kazemian F; Hoseinpoor M; Keiani A; Khatami F; Khorgami Z; Goodarzi M; Ebrahim Soltani A; ahmadi J.

    2007-01-01

      Background: Oral clefts are among the most common congenital anomalies. Infants with oral clefts often have other associated congenital defects, especially congenital heart defects. The reported incidences and the types of associated malformations and congenital heart defects vary between different studies. The purpose of this study was to assess the incidence of associated congenital heart defects in children with oral clefts. Methods: All infants with cleft lip and palate referre...

  17. Cyanotic Congenital Heart Defects – literature review

    Directory of Open Access Journals (Sweden)

    Vlad Drăgoi

    2017-11-01

    Full Text Available Congenital heart defects are one of the most interesting and important chapters regarding abnormal fetal growth pathology. The objective of this article is to present a literature review for the main cyanotic congenital heart defects. The ones presented in the article are: tetralogy of Fallot, transposition of the great vessels, double outlet right ventricle, truncus arteriosus, total anomalous pulmonary venous circulation and additional information regarding very rare malformations such as pentalogy of Cantrell and Uhl anomaly. An early and precise identification of congenital heart defects is an important step in an accurate follow-up of a potential problematic pregnancy. Knowing the sonographic aspect, associated pathology and the current available treatment procedures are vital for the fetal outcome and for the physician to adapt the right management in every situation that might appear during the pregnancy and in the neonatal period.

  18. Epidemiology of congenital heart disease in Brazil

    Directory of Open Access Journals (Sweden)

    Valdester Cavalcante Pinto Júnior

    2015-04-01

    Full Text Available AbstractIntroduction:Congenital heart disease is an abnormality in the structure or cardiocirculatory function, occurring from birth, even if diagnosed later. It can result in intrauterine death in childhood or in adulthood. Accounted for 6% of infant deaths in Brazil in 2007.Objective:To estimate underreporting in the prevalence of congenital heart disease in Brazil and its subtypes.Methods:The calculations of prevalence were performed by applying coefficients, giving them function rates for calculations of health problems. The study makes an approach between the literature and the governmental registries. It was adopted an estimate of 9: 1000 births and prevalence rates for subtypes applied to births of 2010. Estimates of births with congenital heart disease were compared with the reports to the Ministry of Health and were studied by descriptive methods with the use of rates and coefficients represented in tables.Results:The incidence in Brazil is 25,757 new cases/year, distributed in: North 2,758; Northeast 7,570; Southeast 10,112; South 3,329; and Midwest 1,987. In 2010, were reported to System of Live Birth Information of Ministry of Health 1,377 cases of babies with congenital heart disease, representing 5.3% of the estimated for Brazil. In the same period, the most common subtypes were: ventricular septal defect (7,498; atrial septal defect (4,693; persistent ductus arteriosus (2,490; pulmonary stenosis (1,431; tetralogy of Fallot (973; coarctation of the aorta (973; transposition of the great arteries (887; and aortic stenosis 630. The prevalence of congenital heart disease, for the year of 2009, was 675,495 children and adolescents and 552,092 adults.Conclusion:In Brazil, there is underreporting in the prevalence of congenital heart disease, signaling the need for adjustments in the methodology of registration.

  19. Epidemiology of congenital heart disease in Brazil.

    Science.gov (United States)

    Pinto Júnior, Valdester Cavalcante; Branco, Klébia Magalhães P Castello; Cavalcante, Rodrigo Cardoso; Carvalho Junior, Waldemiro; Lima, José Rubens Costa; Freitas, Sílvia Maria de; Fraga, Maria Nazaré de Oliveira; Souza, Nayana Maria Gomes de

    2015-01-01

    Congenital heart disease is an abnormality in the structure or cardiocirculatory function, occurring from birth, even if diagnosed later. It can result in intrauterine death in childhood or in adulthood. Accounted for 6% of infant deaths in Brazil in 2007. To estimate underreporting in the prevalence of congenital heart disease in Brazil and its subtypes. The calculations of prevalence were performed by applying coefficients, giving them function rates for calculations of health problems. The study makes an approach between the literature and the governmental registries. It was adopted an estimate of 9: 1000 births and prevalence rates for subtypes applied to births of 2010. Estimates of births with congenital heart disease were compared with the reports to the Ministry of Health and were studied by descriptive methods with the use of rates and coefficients represented in tables. The incidence in Brazil is 25,757 new cases/year, distributed in: North 2,758; Northeast 7,570; Southeast 10,112; South 3,329; and Midwest 1,987. In 2010, were reported to System of Live Birth Information of Ministry of Health 1,377 cases of babies with congenital heart disease, representing 5.3% of the estimated for Brazil. In the same period, the most common subtypes were: ventricular septal defect (7,498); atrial septal defect (4,693); persistent ductus arteriosus (2,490); pulmonary stenosis (1,431); tetralogy of Fallot (973); coarctation of the aorta (973); transposition of the great arteries (887); and aortic stenosis 630. The prevalence of congenital heart disease, for the year of 2009, was 675,495 children and adolescents and 552,092 adults. In Brazil, there is underreporting in the prevalence of congenital heart disease, signaling the need for adjustments in the methodology of registration.

  20. Sports participation in adults with congenital heart disease

    NARCIS (Netherlands)

    P. Opic (Petra); E.M.W.J. Utens (Elisabeth); J.A.A.E. Cuypers (Judith); M. Witsenburg (Maarten); A.E. van den Bosch (Annemien); R.T. van Domburg (Ron); A.J.J.C. Bogers (Ad); H. Boersma (Eric); Pelliccia, A. (Antonio); J.W. Roos-Hesselink (Jolien)

    2015-01-01

    textabstractBackground: It is unclearwhether sports participation in adultswith repaired congenital heart disease is safe and has benefits. Methods: Congenital heart disease (ConHD) patients who underwent corrective surgery for Atrial Septal Defect, Ventricular Septal Defect, Pulmonary Stenosis,

  1. Current challenges in pediatric heart transplantation for congenital heart disease.

    Science.gov (United States)

    Kirklin, James K

    2015-10-01

    Pediatric heart transplantation is an established therapy for end-stage cardiac disease without suitable medical or surgical options. However, transplantation for congenital heart disease carries an incremental risk that challenges the pediatric transplant team on multiple levels. With improved outcomes following palliative and corrective congenital cardiac surgery, cardiac transplantation has decreased in recent years as a primary therapy. Nevertheless, congenital heart disease remains the most common indication for cardiac transplantation during infancy. Primary transplantation in infancy is selectively recommended for severe systemic ventricular dysfunction, severe atrioventricular valve insufficiency, and occlusive coronary artery anomalies, particularly with single ventricle physiology. Wait-list mortality remains highest for infants with prior palliative surgery and patients with failing Fontan physiology, both of whom have limited options for effective mechanical circulatory support. The sensitized patient carries an increased risk with prolonged wait times, although virtual cross-matches and single bead assays for donor-specific antigens have facilitated the transplant process. Early and late survival after transplantation for congenital heart disease remain inferior to cardiomyopathy, with prior Fontan procedure as a major risk factor. However, among survivors at 6 months, late outcomes are generally excellent. Major late causes of death include allograft vasculopathy, post-transplant lymphoproliferative disease, and acute rejection. Noncompliance with medications remains a major risk for teenage mortality. Despite the myriad of evolving challenges, pediatric heart transplantation for congenital heart disease enjoys routine short and long-term success at experienced centers for the vast majority of such patients without other options.

  2. Drug Therapy in Adult Congenital Heart Disease.

    Science.gov (United States)

    Contractor, Tahmeed; Levin, Vadim; Mandapati, Ravi

    2017-06-01

    Adults with congenital heart disease are at risk for atrial and ventricular arrhythmias that can lead to an increased morbidity as well as mortality. When catheter ablation is not an option or unsuccessful, antiarrhythmic drugs are the mainstay of treatment. There is limited data on the use of antiarrhythmics in this population. The purpose of this article is to discuss the practical aspects of the use of antiarrhythmics in adults with congenital heart disease. Several tables have been provided to provide clinicians a reference for daily use. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Changing Landscape of Congenital Heart Disease.

    Science.gov (United States)

    Bouma, Berto J; Mulder, Barbara J M

    2017-03-17

    Congenital heart disease is the most frequently occurring congenital disorder affecting ≈0.8% of live births. Thanks to great efforts and technical improvements, including the development of cardiopulmonary bypass in the 1950s, large-scale repair in these patients became possible, with subsequent dramatic reduction in morbidity and mortality. The ongoing search for progress and the growing understanding of the cardiovascular system and its pathophysiology refined all aspects of care for these patients. As a consequence, survival further increased over the past decades, and a new group of patients, those who survived congenital heart disease into adulthood, emerged. However, a large range of complications raised at the horizon as arrhythmias, endocarditis, pulmonary hypertension, and heart failure, and the need for additional treatment became clear. Technical solutions were sought in perfection and creation of new surgical techniques by developing catheter-based interventions, with elimination of open heart surgery and new electronic devices enabling, for example, multisite pacing and implantation of internal cardiac defibrillators to prevent sudden death. Over time, many pharmaceutical studies were conducted, changing clinical treatment slowly toward evidence-based care, although results were often limited by low numbers and clinical heterogeneity. More attention has been given to secondary issues like sports participation, pregnancy, work, and social-related difficulties. The relevance of these issues was already recognized in the 1970s when the need for specialized centers with multidisciplinary teams was proclaimed. Finally, research has become incorporated in care. Results of intervention studies and registries increased the knowledge on epidemiology of adults with congenital heart disease and their complications during life, and at the end, several guidelines became easily accessible, guiding physicians to deliver care appropriately. Over the past decades

  4. Congenital heart disease in young adulthood and beyond

    NARCIS (Netherlands)

    Verheugt, C.L.

    2009-01-01

    Over 95% of children with congenital heart defects now reach adulthood and the number of adults with congenital heart disease is estimated to be at least 1.2 million in Europe alone. Despite major developments in diagnostic methods and treatment of congenital heart disease, cure is rarely achieved.

  5. Employment in adults with congenital heart disease

    NARCIS (Netherlands)

    Kamphuis, M.; Vogels, T.; Ottenkamp, J.; Wall, E.E. van der; Verloove-Vanhorick, S.P.; Vliegen, H.W.

    2002-01-01

    Objective: To evaluate job participation, careerrelated problems, and actual job problems in adults with complex congenital heart disease (CHD) compared with adults with mild CHD and reference groups. Design: Cross-sectional study. Setting: Patients were randomly selected from the archives of the

  6. Employment in adults with congenital heart disease

    NARCIS (Netherlands)

    Kamphuis, Mascha; Vogels, Ton; Ottenkamp, Jaap; van der Wall, Ernst E.; Verloove-Vanhorick, S. Pauline; Vliegen, Hubert W.

    2002-01-01

    To evaluate job participation, career-related problems, and actual job problems in adults with complex congenital heart disease (CHD) compared with adults with mild CHD and reference groups. Cross-sectional study. Patients were randomly selected from the archives of the Department of Pediatric

  7. Impact of Congenital Heart Disease at Adulthood

    NARCIS (Netherlands)

    P. Opić (Petra)

    2013-01-01

    textabstractSince the first surgical techniques for patients with congenital heart disease (ConHD) became available some 55 years ago, virtually every area of patient care has evolved substantially. These improvements lead to an increased survival for patients with ConHD, with over 90% of infants

  8. Congenital Heart Disease and General Practice

    NARCIS (Netherlands)

    W.B. de Koning (Wilfred)

    2012-01-01

    textabstractThe treatment of patients with congenital heart disease (CHD) has progressed vastly over the last five decennia. In the Netherlands, around 200,000 children are born each year, around 1,800 of whom have a CHD. This incidence – 6 – 8 per thousand live births – is reported to be similar

  9. Systems biology approaches to heart development and congenital heart disease.

    Science.gov (United States)

    Sperling, Silke R

    2011-07-15

    Even though the foundation of systems biology approaches to cardiac function was led more than fifty years ago, there has been slow progression over the last few decades. Systems biology studies were mainly focused on lower organisms, frequently on yeast. With the boost of high-throughput technologies, systems level analyses, building one backbone of systems biology, started to complement the single-gene focus in the fields of heart development and congenital heart disease. A challenge is to bring together the many uncovered molecular components driving heart development and eventually to establish computational models describing this complex developmental process. Congenital heart diseases represent overlapping phenotypes, reflecting the modularity of heart development. The aetiology of the majority of congenital heart disease is still unknown, and it is suggestive that understanding the biological network underlying heart development will enhance our understanding for its alteration. This review provides an overview of the framework for systems biology approaches focusing on the developing heart and its pathology. Recent methodological developments building the basis for future studies are highlighted and the knowledge gained is specified.

  10. [Clinical features of early newborn infants with congenital heart disease].

    Science.gov (United States)

    Yu, Guoping; Mao, Liangyuan; Chen, Shaozhi

    2014-06-01

    To analyze the clinical feature of early newborn infants with congenital heart disease. We retrospectively analyzed the clinical features of 477 newborn infants with congenital heart disease born within seven days out of 28 050 live births in Shaoxing women and children hospital from October 1, 2009 to September 30, 2012. Infants with congenital heart disease were divided into single malformation group (240 cases), composite deformity group (199 cases) and multiple malformations group (38 cases). Differences of clinical feature were compared between the three groups. Atrial septal defect was the most malformation 91.6% (437/477) .Incidence of preterm birth was higher in newborn inants with congenital heart disease [512.23/10 000(134/2 616)] than infants without without congenital heart disease [134.86/10 000 (343/25 434) , P congenital heart disease groups was similar (P > 0.05) . The incidence of small for gestational age in congenital heart disease group (10.90%, 52/477) was also significantly higher than those without congenital heart disease group (5.91%, 1 630/27 573, P congenital heart disease of complex malformations, multiple malformations groups was higher than that in the single malformation group (P congenital heart disease. The incidence of preterm is higher in newborn infants with congenital heart disease. Complex and multiple malformations are linked with small for gestational age birth weight.

  11. [Relationship between congenital heart disease and bronchial dysplasia].

    Science.gov (United States)

    Zeng, Shuang-Lin; Li, Ya-Jun; Huang, Ting; Tan, Li-Hua; Mei, Xi-Long; Sun, Jian-Ning

    2011-11-01

    To study the relationship of the incidence of bronchial dysplasia (bronchial anomalous origin and bronchial stenosis) with congenital heart disease. A total of 185 children with congenital heart disease or bronchial dysplasia were enrolled. Bronchial dysplasia was identified by the 64-MSCT conventional scanning or thin slice scanning with three-dimensional reconstruction. Forty-five children (25.3%) had coexisting bronchial dysplasia and congenital heart disease. The incidence rate of bronchial dysplasia in children with congenital heart disease associated with ventricular septal defect was higher than in those without ventricular septal defect (33.7% vs 15.0%; Pincidence rate of bronchial dysplasia between the children with congenital heart disease who had a large vascular malformation and who did not. Bronchial dysplasia often occurs in children with congenital heart disease. It is necessary to perform a tracheobronchial CT scanning with three-dimensional reconstruction to identify tracheobronchial dysplasia in children with congenital heart disease, especially associated with ventricular septal defect.

  12. Implantation of total artificial heart in congenital heart disease.

    Science.gov (United States)

    Adachi, Iki; Morales, David S L

    2014-07-18

    In patients with end-stage heart failure (HF), a total artificial heart (TAH) may be implanted as a bridge to cardiac transplant. However, in congenital heart disease (CHD), the malformed heart presents a challenge to TAH implantation. In the case presented here, a 17 year-old patient with congenital transposition of the great arteries (CCTGA) experienced progressively worsening HF due to his congenital condition. He was hospitalized multiple times and received an implantable cardioverter defibrillator (ICD). However, his condition soon deteriorated to end-stage HF with multisystem organ failure. Due to the patient's grave clinical condition and the presence of complex cardiac lesions, the decision was made to proceed with a TAH. The abnormal arrangement of the patient's ventricles and great arteries required modifications to the TAH during implantation. With the TAH in place, the patient was able to return home and regain strength and physical well-being while awaiting a donor heart. He was successfully bridged to heart transplantation 5 months after receiving the device. This report highlights the TAH is feasible even in patients with structurally abnormal hearts, with technical modification.

  13. Congenital Heart Defects and CCHD

    Science.gov (United States)

    ... procedures with catheters (thin, flexible tubes) can fix medium and large ASDs. Heart surgery may be needed ... parts. Conditions in your everyday life (lifestyle and environment) Some things in your life and environment (where ...

  14. Tracheal quadrifurcation associated with congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Bhat, Venkatraman; Gadabanahalli, Karthik; Ahmad, Ozaire [Narayana Multispeciality Hospital and Mazumdar Shaw Cancer Center, Department of Radiology, Bangalore (India)

    2015-08-15

    Tracheal anomalies are known in association with congenital cardiac defects. Some of the well-described anomalies include accessory (displaced) tracheal bronchus with variants, tracheal trifurcation and accessory cardiac bronchus. Here we describe a case of tracheal quadrifurcation associated with complex congenital heart disease. Illustration of complex airway anatomy was simplified by the use of multidetector CT using a variety of image display options. Awareness of this complex anomaly will expand our knowledge of tracheal anomalies and equip the anesthesia and surgical team for better airway management. (orig.)

  15. Congenital Heart Disease and General Practice

    OpenAIRE

    Koning, Wilfred

    2012-01-01

    textabstractThe treatment of patients with congenital heart disease (CHD) has progressed vastly over the last five decennia. In the Netherlands, around 200,000 children are born each year, around 1,800 of whom have a CHD. This incidence – 6 – 8 per thousand live births – is reported to be similar round the world, making CHD the world’s commonest congenital anomaly. In the Netherlands, this high incidence and the improved treatment options both mean that the number of people surviving CHD is i...

  16. Junctional ectopic tachycardia evolving into complete heart block

    NARCIS (Netherlands)

    Henneveld, H; Hutter, P; Bink-Boelkens, M; Sreeram, N

    1998-01-01

    Transition from congenital junctional ectopic tachycardia to complete AV block was observed in an 8 month old girl, over a 36 hour period, during initial hospital admission. Two years later she had evidence of a rapidly increasing left ventricular end diastolic diameter, associated with lowest heart

  17. Congenital Heart Disease: Causes, Diagnosis, Symptoms, and Treatments.

    Science.gov (United States)

    Sun, RongRong; Liu, Min; Lu, Lei; Zheng, Yi; Zhang, Peiying

    2015-07-01

    The congenital heart disease includes abnormalities in heart structure that occur before birth. Such defects occur in the fetus while it is developing in the uterus during pregnancy. About 500,000 adults have congenital heart disease in USA (WebMD, Congenital heart defects medications, www.WebMD.com/heart-disease/tc/congenital-heart-defects-medications , 2014). 1 in every 100 children has defects in their heart due to genetic or chromosomal abnormalities, such as Down syndrome. The excessive alcohol consumption during pregnancy and use of medications, maternal viral infection, such as Rubella virus, measles (German), in the first trimester of pregnancy, all these are risk factors for congenital heart disease in children, and the risk increases if parent or sibling has a congenital heart defect. These are heart valves defects, atrial and ventricular septa defects, stenosis, the heart muscle abnormalities, and a hole inside wall of the heart which causes defect in blood circulation, heart failure, and eventual death. There are no particular symptoms of congenital heart disease, but shortness of breath and limited ability to do exercise, fatigue, abnormal sound of heart as heart murmur, which is diagnosed by a physician while listening to the heart beats. The echocardiogram or transesophageal echocardiogram, electrocardiogram, chest X-ray, cardiac catheterization, and MRI methods are used to detect congenital heart disease. Several medications are given depending on the severity of this disease, and catheter method and surgery are required for serious cases to repair heart valves or heart transplantation as in endocarditis. For genetic study, first DNA is extracted from blood followed by DNA sequence analysis and any defect in nucleotide sequence of DNA is determined. For congenital heart disease, genes in chromosome 1 show some defects in nucleotide sequence. In this review the causes, diagnosis, symptoms, and treatments of congenital heart disease are described.

  18. 3D Whole Heart Imaging for Congenital Heart Disease

    OpenAIRE

    Greil, Gerald; Tandon, Animesh (Aashoo); Silva Vieira, Miguel; Hussain, Tarique

    2017-01-01

    Three-dimensional (3D) whole heart techniques form a cornerstone in cardiovascular magnetic resonance imaging of congenital heart disease (CHD). It offers significant advantages over other CHD imaging modalities and techniques: no ionizing radiation; ability to be run free-breathing; ECG-gated dual-phase imaging for accurate measurements and tissue properties estimation; and higher signal-to-noise ratio and isotropic voxel resolution for multiplanar reformatting assessment. However, there are...

  19. Congenital complete atrioventricular block in the early pediatric population

    Directory of Open Access Journals (Sweden)

    Riccardo Cappato

    2010-05-01

    Full Text Available Congenital complete atrioventricular block (CCAVB is a rare potentially lethal disease with an estimated incidence of 1 every 15.000 to 20.000 live born infants. In structurally normal hearts two kinds of congenital heart block can be identified: one usually diagnosed in utero associated with the circulating maternal anti-SS-A/Ro and anti-SS-B/La antibodies, the other kind is detected later in the neonatal period or during the infancy or childhood and present no clear relation with maternal antibodies. Nowadays, the diagnosis can be made in utero as early as between week 16 and 28 of gestation by foetal echocardiography. The mortality in isolated CCAVB is estimated between 8 and 16% and between 4 and 8% in children and adults. The mortality and morbidity of patients diagnosed outside the neonatal period is significantly lower than those with a in utero diagnosis. Risk factors for worse outcome in CCAVB are the foetal diagnosis, the presence of hydrops fetalis, delivery at

  20. Initial validation of a healthcare needs scale for young people with congenital heart disease.

    Science.gov (United States)

    Chen, Chi-Wen; Ho, Ciao-Lin; Su, Wen-Jen; Wang, Jou-Kou; Chung, Hung-Tao; Lee, Pi-Chang; Lu, Chun-Wei; Hwang, Be-Tau

    2018-01-01

    To validate the initial psychometric properties of a Healthcare Needs Scale for Youth with Congenital Heart Disease. As the number of patients with congenital heart disease surviving to adulthood increases, the transitional healthcare needs for adolescents and young adults with congenital heart disease require investigation. However, few tools comprehensively identify the healthcare needs of youth with congenital heart disease. A cross-sectional study was employed to examine the psychometric properties of the Healthcare Needs Scale for Youth with Congenital Heart Disease. The sample consisted of 500 patients with congenital heart disease, aged 15-24 years, from paediatric cardiology departments and covered the period from March-August 2015. The patients completed the 25-item Healthcare Needs Scale for Youth with Congenital Heart Disease, the questionnaire on health needs for adolescents and the WHO Quality of Life-BREF. Reliability and construct, concurrent, predictive and known-group validity were examined. The Healthcare Needs Scale for Youth with Congenital Heart Disease includes three dimensions, namely health management, health policy and individual and interpersonal relationships, which consist of 25 items. It demonstrated excellent internal consistency and sound construct, concurrent, predictive and known-group validity. The Healthcare Needs Scale for Youth with Congenital Heart Disease is a psychometrically robust measure of the healthcare needs of youth with congenital heart disease. It has the potential to provide nurses with a means to assess and identify the concerns of youth with congenital heart disease and to help them achieve a successful transition to adult care. © 2017 John Wiley & Sons Ltd.

  1. Managing congenital heart disease and comorbidities – opening a ...

    African Journals Online (AJOL)

    prognosis? Congenital heart disease and comorbidities. The birth incidence of congenital heart disease (CHD) is just less than 1%.1 Of these children, approximately 50 - 60% will require surgery. Between 25% and 30%2 of children with CHD will have some form of additional congenital lesion, a comorbidity or structural.

  2. Postnatal Cardiac Autonomic Nervous Control in Pediatric Congenital Heart Disease

    NARCIS (Netherlands)

    Nederend, I.; Jongbloed, M.R.M.; de Geus, J.C.N.; Blom, N.A.; ten Harkel, A.D.J.

    2016-01-01

    Congenital heart disease is the most common congenital defect. During childhood, survival is generally good but, in adulthood, late complications are not uncommon. Abnormal autonomic control in children with congenital heart disease may contribute considerably to the pathophysiology of these long

  3. Facts about Congenital Heart Defects

    Science.gov (United States)

    ... no signs or symptoms. Others might cause a baby to have the following symptoms: Blue-tinted nails or lips Fast or troubled breathing Tiredness when feeding Sleepiness Diagnosis ... of the heart of the developing baby. However, some CHDs are not detected until after ...

  4. Nutrition in neonatal congenital heart disease

    OpenAIRE

    Morgan CT; Shine AM; McMahon CJ

    2013-01-01

    Conall T Morgan,1 Anne Marie Shine,2 Colin J McMahon1 1Department of Pediatric Cardiology, 2Department of Clinical Nutrition and Dietetics, Our Lady's Children's Hospital Crumlin, Dublin, Republic of Ireland Abstract: There are 40,000 infants born in the USA with congenital heart disease annually. Achievement of adequate oral nutrition is difficult in this population. Malnutrition is common. Single ventricle physiology, the risk of necrotizing enterocolitis, and cardiopulmona...

  5. The global burden of congenital heart disease

    OpenAIRE

    Hoffman, Julien IE

    2013-01-01

    Abstract Although the incidence of congenital heart disease (CHD) is similar worldwide, the burden of supporting these patients falls more heavily on countries with high fertility rates. In a country with a fertility rate of about eight per woman, the population has to support four times as many children with CHD as in a country with a fertility rate of two. Countries with the highest fertility rates tend to have the lowest incomes per capita, thus accentuating the disparity. Countries with h...

  6. Genetics of Congenital Heart Disease: Past and Present.

    Science.gov (United States)

    Muntean, Iolanda; Togănel, Rodica; Benedek, Theodora

    2017-04-01

    Congenital heart disease is the most common congenital anomaly, representing an important cause of infant morbidity and mortality. Congenital heart disease represents a group of heart anomalies that include septal defects, valve defects, and outflow tract anomalies. The exact genetic, epigenetic, or environmental basis of congenital heart disease remains poorly understood, although the exact mechanism is likely multifactorial. However, the development of new technologies including copy number variants, single-nucleotide polymorphism, next-generation sequencing are accelerating the detection of genetic causes of heart anomalies. Recent studies suggest a role of small non-coding RNAs, micro RNA, in congenital heart disease. The recently described epigenetic factors have also been found to contribute to cardiac morphogenesis. In this review, we present past and recent genetic discoveries in congenital heart disease.

  7. Virtual Surgery in Congenital Heart Disease

    DEFF Research Database (Denmark)

    Sørensen, Thomas Sangild; Mosegaard, Jesper; Kislinskiy, Stefan

    2014-01-01

     Teaching, diagnosing, and planning of therapy in patients with complex structural cardiovascular heart disease require profound understanding of the three-dimensional (3D) nature of cardiovascular structures in these patients. To obtain such understanding, modern imaging modalities provide high...... et al., Cardiol Young 13:451–460, 2003). In combination with the availability of virtual models of congenital heart disease (CHD), techniques for computer- based simulation of cardiac interventions have enabled early clinical exploration of the emerging concept of virtual surgery (Sorensen et al...

  8. Early extubation after congenital heart surgery

    Directory of Open Access Journals (Sweden)

    Mirza Halimić

    2014-12-01

    Full Text Available Introduction: Despite recent advances in anesthesia, cardiopulmonary bypass and surgical techniques, children undergoing congenital heart surgery require postoperativemechanical ventilation. Early extubation was definedas ventilation shorter than 12 hours. Aim of this study is to identify factors associated with successful early extubation after pediatric cardiac surgery.Methods: The study was performed during period from January 2006 to January 2011 at Pediatric Clinic and Heart Center University Clinical center Sarajevo. One hundred children up to 5 years of age, who have had congenital heart disease, with left–right shunt and obstructive heart disease were included in the study. Patients were divided into two groups: Group I - patients extubated within 12 hours after surgery and Group II - patients extubated 12 or more hours after surgery. Results: The most frequently encountered preoperative variables were age with odds ratio 4% 95%CI (1-7%, Down's syndrome 8.5 95%CI (1.6-43.15, failure to thrive 4.3 95%CI( 1-18. Statistically significant postoperative data included lung disease (reactive airways, pneumonia, atelectasis, pneumothorax and with odds ratio 35.1 95 %CI (4-286 and blood transfusion with odds ratio 4.6 95%CI (2-12. Blood transfusion (p=0.002 (Wald=9.2 95%CI (2-12, during as well as after operation procedure has statistically significant influence on prediction time of extubation. Proven markers were age with cut of 21.5 months (sensitivity 74% and specificity 70% and extracorporeal circulation (ECC with cut-of 45.5 minutes (sensitivity 71% and specificity 65%.Conclusion: Early extubation is possible in many children undergoing congenital heart surgery. Younger age and prolonged ECC time are markers associated with prolonged mechanical ventilation.

  9. Adult Congenital Heart Disease: Scope of the Problem.

    Science.gov (United States)

    Mazor Dray, Efrat; Marelli, Ariane J

    2015-11-01

    This article reviews the changing epidemiology of congenital heart disease summarizing its impact on the demographics of the congenital heart disease population and the progress made in order to improve outcomes in this patient population. Birth prevalence of congenital heart disease can be modified by many factors. As a result of decreasing mortality and increasing survival in all forms of congenital heart disease, the median age of patients has increased and adults now compose two-thirds of patients with congenital heart disease. Disease burden and resulting health services utilization increase significantly across the lifespan. Bridging the gap between policy and quality of care can be improved by referral to specialized adult congenital heart disease centers and planning delivery of specialized services that are commensurate with population needs, program accreditation criteria and certified training of designated workforce. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Association of congenital neuroblastoma and congenital heart disease. Is there a common embryologic basis

    Energy Technology Data Exchange (ETDEWEB)

    Bellah, R.; D' Andrea, A.; Darillis, E.; Fellows, K.E.

    1989-01-01

    Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific wellknown associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma.

  11. Congenital heart disease and chromossomopathies detected by the karyotype

    OpenAIRE

    Trevisan, Patrícia; Rosa, Rafael Fabiano M.; Koshiyama, Dayane Bohn; Zen, Tatiana Diehl; Paskulin, Giorgio Adriano; Zen, Paulo Ricardo G.

    2014-01-01

    OBJECTIVE: To review the relationship between congenital heart defects and chromosomal abnormalities detected by the karyotype.DATA SOURCES: Scientific articles were searched in MEDLINE database, using the descriptors "karyotype" OR "chromosomal" OR "chromosome" AND "heart defects, congenital". The research was limited to articles published in English from 1980 on.DATA SYNTHESIS: Congenital heart disease is characterized by an etiologically heterogeneous and not well understood group of lesio...

  12. Ocular pathology in congenital heart disease.

    Science.gov (United States)

    Mansour, A M; Bitar, F F; Traboulsi, E I; Kassak, K M; Obeid, M Y; Megarbane, A; Salti, H I

    2005-01-01

    To describe the ocular findings in subjects with congenital heart disease (CHD). In a prospective study, the same observer examined 240 consecutive patients with CHD admitted to the medical centre. Two independent geneticists performed identification of syndromes. The commonest anatomic cardiac anomalies were ventricular or atrial septal defects (62), tetralogy of Fallot (39), pulmonary stenosis (25), and transposition of the great arteries (24). The heart lesions were divided physiologically into volume overload (90), cyanotic (87), and obstructive (63). In all, 105 syndromic subjects included the velocardiofacial syndrome (18), Down's syndrome (17), CHARGE association (6), DiGeorge syndrome (5), Williams syndrome (3), Edwards syndrome (3), Noonan syndrome (3), VACTERL association (2), and Patau syndrome (trisomy 13) (2). The paediatric team recognized 51 patients as syndromic. Two independent geneticists recognized additional 54 patients as syndromic. Positive eye findings were present in 55% (132) and included retinal vascular tortuosity (46), optic disc hypoplasia (30), trichomegaly (15), congenital ptosis (12), strabismus (11), retinal haemorrhages (8), prominent eyes (7), and congenital cataract (6). There was a strong correlation between the retinal vascular tortuosity and both a low haematocrit (P=0.000) and a low arterial oxygen saturation (P=0.002). Patients with CHD are at a high risk for ocular pathology and need screening for various ocular abnormalities.

  13. Variation in congenital heart surgery costs across hospitals

    National Research Council Canada - National Science Library

    Pasquali, Sara K; Jacobs, Marshall L; He, Xia; Shah, Samir S; Peterson, Eric D; Hall, Matthew; Gaynor, J William; Hill, Kevin D; Mayer, John E; Jacobs, Jeffrey P; Li, Jennifer S

    2014-01-01

    A better understanding of costs associated with common and resource-intense conditions such as congenital heart disease has become increasingly important as children's hospitals face growing pressure...

  14. Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome.

    Science.gov (United States)

    Krieger, Eric V; Leary, Peter J; Opotowsky, Alexander R

    2015-11-01

    Patients with adult congenital heart disease have an increased risk of developing pulmonary hypertension. There are several mechanisms of pulmonary hypertension in patients with adult congenital heart disease, and understanding them requires a systematic approach to define the patient's hemodynamics and physiology. This article reviews the updated classification of pulmonary hypertension in patients with adult congenital heart disease with a focus on pathophysiology, diagnostics, and the evaluation of pulmonary hypertension in special adult congenital heart disease populations. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Associated noncardiac congenital anomalies among cases with congenital heart defects.

    Science.gov (United States)

    Stoll, Claude; Dott, Beatrice; Alembik, Yves; Roth, Marie-Paule

    2015-02-01

    Cases with congenital heart defects (CHD) often have other associated anomalies. The purpose of this investigation was to assess the prevalence and the types of associated anomalies in CHD in a defined population. The anomalies associated with CHD were collected in all live births, stillbirths and terminations of pregnancy during 26 years in 346,831 consecutive pregnancies of known outcome in the area covered by our population based registry of congenital anomalies. Of the 4005 cases with CHD born during this period (total prevalence of 115.5 per 10,000), 1055 (26.3%) had associated major anomalies. There were 354 (8.8%) cases with chromosomal abnormalities including 218 trisomies 21, and 99 (2.5%) nonchromosomal recognized dysmorphic conditions. There were no predominant recognized dysmorphic conditions, but VACTERL association. However, other recognized dysmorphic conditions were registered including Noonan syndrome, fetal alcohol syndrome, and skeletal dysplasias. Six hundred and two (15.0%) of the cases had non syndromic, non chromosomal multiple congenital anomalies (MCA). Anomalies in the urinary tract, the musculoskeletal, the digestive, and the central nervous systems were the most common other anomalies. Prenatal diagnosis was obtained in 18.7% of the pregnancies. In conclusion the overall prevalence of associated anomalies, which was one in four infants, emphasizes the need for a thorough investigation of cases with CHD. A routine screening for other anomalies may be considered in infants and in fetuses with CHD. One should be aware that the anomalies associated with CHD can be classified into a recognizable anomaly, syndrome or pattern in one out of nine cases with CHD. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  16. Sequential segmental classification of feline congenital heart disease.

    Science.gov (United States)

    Scansen, Brian A; Schneider, Matthias; Bonagura, John D

    2015-12-01

    Feline congenital heart disease is less commonly encountered in veterinary medicine than acquired feline heart diseases such as cardiomyopathy. Understanding the wide spectrum of congenital cardiovascular disease demands a familiarity with a variety of lesions, occurring both in isolation and in combination, along with an appreciation of complex nomenclature and variable classification schemes. This review begins with an overview of congenital heart disease in the cat, including proposed etiologies and prevalence, examination approaches, and principles of therapy. Specific congenital defects are presented and organized by a sequential segmental classification with respect to their morphologic lesions. Highlights of diagnosis, treatment options, and prognosis are offered. It is hoped that this review will provide a framework for approaching congenital heart disease in the cat, and more broadly in other animal species based on the sequential segmental approach, which represents an adaptation of the common methodology used in children and adults with congenital heart disease. Copyright © 2015 Elsevier B.V. All rights reserved.

  17. Cyanotic congenital heart disease and atherosclerosis.

    Science.gov (United States)

    Tarp, Julie Bjerre; Jensen, Annette Schophuus; Engstrøm, Thomas; Holstein-Rathlou, Niels-Henrik; Søndergaard, Lars

    2017-06-01

    Improved treatment options in paediatric cardiology and congenital heart surgery have resulted in an ageing population of patients with cyanotic congenital heart disease (CCHD). The risk of acquired heart disease such as atherosclerosis increases with age.Previous studies have speculated whether patients with CCHD are protected against atherosclerosis. Results have shown that the coronary arteries of patients with CCHD are free from plaques and stenosis. Decreased carotid intima-media thickness and low total plasma cholesterol may indicate a reduced risk of later development of atherosclerosis. However, the evidence is still sparse and questionable, and a reasonable explanation for the decreased risk of developing atherosclerosis in patients with CCHD is still missing.This review provides an overview of what is known about the prevalence and potential causes of the reduced risk of atherosclerosis in patients with CCHD. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  18. Acceptance and psychological impact of implantable defibrillators amongst adults with congenital heart disease.

    Science.gov (United States)

    Bedair, Radwa; Babu-Narayan, Sonya V; Dimopoulos, Konstantinos; Quyam, Sadia; Doyle, Anne-Marie; Swan, Lorna; Gatzoulis, Michael A; Wong, Tom

    2015-02-15

    The psychological impact of implantable cardioverter defibrillators (ICDs) in adults with congenital heart disease (ACHD) has not been established. To compare device acceptance, quality of life, anxiety and depression between ACHD patients with ICDs (ICD-Congenital), with pacemakers (PPM-Congenital), with no devices (No Device-Congenital) and non-ACHD patients with ICDs (ICD-Non-Congenital). A total of 147 ACHD and 46 non-ACHD patients (age 45.0±14.7 years, 56.5% males) completed the Florida Patient Acceptance Survey (FPAS), the 36-item Short Form Health Survey (SF-36) and Hospital Anxiety & Depression Scale (HADS). ICD-Congenital patients (n=59) showed lower device acceptance compared to PPM-Congenital patients (n=41), p=0.04, and reported worse quality of life (p=0.001) and higher prevalence of depression (p=0.009) when compared to No Device-Congenital (n=47) patients. ICD-Congenital and ICD-Non-Congenital patients (n=46) showed similar mental and physical health, device acceptance, anxiety and depression. Within ICD-Congenital, patients with poorest device acceptance (FPAS congenital heart disease who receive an ICD than those who receive pacemakers. Appropriate screening for anxiety and depression may be warranted for ACHD patients considered for ICD implantation or already living with ICDs. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  19. Dextrocardia in Adults with Congenital Heart Disease.

    Science.gov (United States)

    Offen, Sophie; Jackson, Dan; Canniffe, Carla; Choudhary, Preeti; Celermajer, David S

    2016-04-01

    Dextrocardia is rare in the general population, and may be associated with significant additional cardiac malformations. We aimed to identify the prevalence and patterns of additional cardiac defects, as well as the associated long-term morbidity and mortality, in adult patients with dextrocardia, in a specialised Adult Congenital Heart Disease (ACHD) service. A retrospective study of patients with dextrocardia managed by our tertiary ACHD service, since January 2000, was performed. Medical records were reviewed and the National Death Index was consulted to confirm survival status. Of 3698 adults in our ACHD Service, 19 (0.5%) had dextrocardia. Mean follow-up duration was 7±7.5 years. The mean age at last review was 36.8±10.5 years (range 20-63 years). Situs was solitus in 14 (74%) and inversus in five (26%). Eleven patients (58%) had functional single ventricles, of whom five had atrioventricular (AV)-ventriculoarterial (VA) discordance and two had VA discordance only. Four patients with two ventricles had AV-VA discordance. All patients had at least one additional cardiac malformation. Fourteen patients (74%) required surgical intervention. Eleven patients (58%) underwent a Fontan-type operation. Five patients (26%) required ablation procedures for arrhythmia. One patient had infective endocarditis and two deaths occurred, both in patients who also had AV-VA discordance. Dextrocardia remains a rare finding in adults, even in a highly select group of patients with known congenital heart disease. Those with associated congenital heart abnormalities are likely to have complex lesions, which may require multiple surgical and medical interventions. Despite this, our series demonstrated that patients surviving to adulthood and then managed in an ACHD centre may have good medium-term survival. Copyright © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by

  20. Myocardial Fibrosis in Congenital Heart Disease.

    Science.gov (United States)

    Rathod, Rahul H; Powell, Andrew J; Geva, Tal

    2016-05-25

    Myocardial fibrosis is common in patients with congenital heart disease (CHD) and has been associated with arrhythmias, decreased functional status, and adverse ventricular mechanics. There are multiple types of myocardial fibrosis that occur in response to different pathophysiologic stimuli. Recent advances in imaging technology have made detection and quantification of the types of myocardial fibrosis possible. In this review, we describe the pathophysiology of myocardial fibrosis, examine the imaging techniques used to evaluate fibrosis, and discuss the relationship between myocardial fibrosis and clinical outcomes in CHD. (Circ J 2016; 80: 1300-1307).

  1. Therapeutic Cardiac Catheterizations for Children with Congenital Heart Disease

    Science.gov (United States)

    Therapeutic Cardiac Catheterizations for Children with Congenital Heart Disease Introduction A therapeutic cardiac catheterization is a procedure performed to treat your child’s heart defect. A doctor will use special techniques and ...

  2. Longitudinal echocardiographic follow-up in children with congenital complete atrioventricular block

    NARCIS (Netherlands)

    Beaufort-Krol, Gertie C. M.; Leeuwen, Miek J. M. Schasfoort-Van; Stienstra, Ymkje; Bink-Boelkens, Margreet Th. E.

    2007-01-01

    Background: Due to a low heart rate (HR) in children with congenital complete atrioventricular block (CCAVB), a larger stroke volume of the left ventricle (LV) may be expected. If so, end-diastolic (LVEDD) and end-systolic (LVESD) diameters may be enlarged and even dilated cardiomyopathy (DCM) may

  3. Preattentive processing of heart cues and the perception of heart symptoms in congenital heart disease

    NARCIS (Netherlands)

    Karsdorp, Petra A.; Kindt, Merel; Everaerd, Walter; Mulder, Barbara J. M.

    2007-01-01

    The present study was aimed at clarifying whether preattentive processing of heart cues results in biased perception of heart sensations in patients with congenital heart disease (ConHD) who are also highly trait anxious. Twenty-six patients with ConHD and 22 healthy participants categorized

  4. Nutrition in neonatal congenital heart disease

    Directory of Open Access Journals (Sweden)

    Morgan CT

    2013-09-01

    Full Text Available Conall T Morgan,1 Anne Marie Shine,2 Colin J McMahon1 1Department of Pediatric Cardiology, 2Department of Clinical Nutrition and Dietetics, Our Lady's Children's Hospital Crumlin, Dublin, Republic of Ireland Abstract: There are 40,000 infants born in the USA with congenital heart disease annually. Achievement of adequate oral nutrition is difficult in this population. Malnutrition is common. Single ventricle physiology, the risk of necrotizing enterocolitis, and cardiopulmonary bypass prevent the establishment of normal oral feeding patterns. Improved nutrition results in improved surgical outcomes, lower mortality, and shorter hospital stay. In this review, we discuss the challenges this population faces. Keywords: necrotizing enterocolitis, malnutrition, growth failure, hypoplastic left heart

  5. Keeping children with congenital heart disease healthy.

    Science.gov (United States)

    Woodward, Cathy S

    2011-01-01

    Keeping children with congenital heart disease healthy is vital to their long-term survival and quality of life. Nurse practitioners are in an excellent position to keep these sometimes fragile children healthy before, between, and after their cardiac surgeries. Primary care visits should address developmental morbidity. Referral for in-depth evaluations and intervention should be initiated for children with hemodynamically significant heart disease. Infants may also experience poor feeding. Nutritional guidance may include fortifying formulas or enteral tube feedings. Attention to immunization status and prevention of winter illnesses and endocarditis may reduce complications in this high-risk group of children. Copyright © 2011 National Association of Pediatric Nurse Practitioners. Published by Mosby, Inc. All rights reserved.

  6. Repair of congenital heart defects associated with single pulmonary artery.

    Science.gov (United States)

    Bockeria, Leo A; Makhachev, Osman A; Khiriev, Titalav Kh; Podzolkov, Vladimir P; Zelenikin, Mikhail A; Kim, Aleksey I; Zaets, Sergey B

    2015-02-01

    Experience with complete repair of congenital heart defects associated with unilateral absence of a pulmonary artery is limited. The aim of this retrospective study was to present our surgical experience of this complex category of patients, to analyze immediate results of surgical interventions, and to suggest a rational surgical strategy. Of 37 patients with a single pulmonary artery who underwent complete repair of associated heart defects, the left or right pulmonary artery was absent in 32 and 5, respectively. The most frequent heart defects were tetralogy of Fallot (n = 25) and ventricular septal defect (n = 8). The median age of these patients was 7.1 years. Preoperative examinations included echocardiography, cardiac catheterization and angiocardiography, with quantitative assessment of the single pulmonary artery. In-hospital parameters of surgical outcome were analyzed. Recorded hospital mortality was 2.7% (1/37). The single death was in a patient with tetralogy of Fallot, agenesis of the left pulmonary artery, and a small diameter of the contralateral pulmonary artery (Nakata index 174 mm(2)·m(-2)). The right-to-left ventricular systolic pressure ratio after complete tetralogy of Fallot repair in patients who survived the operation was 0.58 ± 0.11. Complete repair of congenital heart defects in patients with unilateral absence of a pulmonary artery is associated with a relatively low risk. If the hilar artery is of adequate size, surgical intervention should attempt restoration of the communication between the disconnected hilar artery and the pulmonary trunk, in addition to repairing the heart defects. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  7. Adult Congenital Heart Disease with Focus on Pregnancy

    NARCIS (Netherlands)

    T.P.E. Ruys (Titia)

    2013-01-01

    textabstractThe prevalence of Congenital Heart Disease (CHD) has been described to be 8,2 per 1000 live births in European countries.(1) Congenital heart disease is a collective term for a large number of different diagnoses with different anatomical substrate, complexity and prognosis. The most

  8. A systematic review of trends and patterns of congenital heart ...

    African Journals Online (AJOL)

    Objectives: To determine the patterns of congenital heart disease in children in Nigeria and examine trends in the occurrence ... Results: The commonest congenital heart diseases in Nigeria are ventricular septal defect (40.6%), patent ductus arteriosus. (18.4%) ...... Milgram E. High prevalence of muscular ventricular sep-.

  9. The trace elements in congenital cyanotic heart disease

    Directory of Open Access Journals (Sweden)

    Mohamed Ali Hegazi

    2014-03-01

    Conclusion: Congenital cyanotic heart disease were associated with a highly significant decrease in the mean serum selenium and zinc levels, when compared with control group and non significant increase the mean serum copper levels. Changes in these trace elements suggested to play an important role in the pathogenesis of myocardial damage in congenital cyanotic heart disease.

  10. Emotional Problems in Adolescents with Congenital Heart Diseases

    Directory of Open Access Journals (Sweden)

    Beena Johnson

    2014-10-01

    Full Text Available Chronic diseases of childhood and the associated emotional problems are receiving major attention in the current pediatric practice. Adolescents with congenital heart diseases have significantly higher emotional problems compared to those without chronic illnesses. Early detection and management of emotional problems is essential to improve the quality of life of the adolescents with congenital heart diseases.

  11. Nutrition care for newborns with congenital heart disease.

    Science.gov (United States)

    Steltzer, Michelle; Rudd, Nancy; Pick, Barbara

    2005-12-01

    Those health care professionals entrusted with the care of infants with congenital heart disease require an understanding of the unique nutritional needs of this population. This article defines the congenital, physiologic, and nutritional variables encountered in this population. The nutritional needs, multi-factorial sources of undernutrition, and consequences of inadequate nutrition in infants with congenital heart disease are discussed, as well as medical and nutritional management strategies intended to optimize growth and reduce morbidity.

  12. Congenital heart disease in the newborn requiring early intervention

    Directory of Open Access Journals (Sweden)

    Sin Weon Yun

    2011-05-01

    Full Text Available Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA, critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS, obstructed total anomalous pulmonary venous return (TAPVR, which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA is opened widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

  13. Social burden and lifestyle in adults with congenital heart disease.

    Science.gov (United States)

    Zomer, A Carla; Vaartjes, Ilonca; Uiterwaal, Cuno S P; van der Velde, Enno T; Sieswerda, Gert-Jan T; Wajon, Elly M C; Plomp, Koos; van Bergen, Paul F M; Verheugt, Carianne L; Krivka, Eva; de Vries, Cees J; Lok, Dirk J A; Grobbee, Diederick E; Mulder, Barbara J M

    2012-06-01

    We aimed to evaluate how the presence and severity of congenital heart disease (CHD) influence social life and lifestyle in adult patients. A random sample (n = 1,496) from the CONgenital CORvitia (n = 11,047), the Dutch national registry of adult patients with CHD, completed a questionnaire on educational attainment, employment and marital statuses, and lifestyle (response 76%). The Utrecht Health Project provided a large reference group (n = 6,810) of unaffected subjects. Logistic regression models were used for subgroup analyses and to adjust for age, gender, and socioeconomic status where appropriate. Of all patients 51.5% were men (median age 39 years, interquartile range 29 to 51) with mild (46%), moderate (44%), and severe (10%) CHD. Young (sports participation (adjusted OR 1.2, p lifestyles compared to the reference group. Copyright © 2012 Elsevier Inc. All rights reserved.

  14. Xenopus: An Emerging Model for Studying Congenital Heart Disease

    Science.gov (United States)

    Kaltenbrun, Erin; Tandon, Panna; Amin, Nirav M.; Waldron, Lauren; Showell, Chris; Conlon, Frank L.

    2011-01-01

    Congenital heart defects affect nearly 1% of all newborns and are a significant cause of infant death. Clinical studies have identified a number of congenital heart syndromes associated with mutations in genes that are involved in the complex process of cardiogenesis. The African clawed frog, Xenopus, has been instrumental in studies of vertebrate heart development and provides a valuable tool to investigate the molecular mechanisms underlying human congenital heart diseases. In this review, we discuss the methodologies that make Xenopus an ideal model system to investigate heart development and disease. We also outline congenital heart conditions linked to cardiac genes that have been well-studied in Xenopus and describe some emerging technologies that will further aid in the study of these complex syndromes. PMID:21538812

  15. [Sports in children with congenital heart diseases].

    Science.gov (United States)

    Bosser, Gilles; Moulin-Zinsch, Anne; Fischer-Atalla, Reem

    2017-05-01

    The practice of physical activity is one of the essential elements for health in general but also for the well-being and the quality of life. It is highly desirable to encourage physical activities in children with congenital heart diseases, taking into account all the benefits associated with this practice (quality of life, life expectancy) and this especially since these children often have limited capacity (due to their heart disease but also often by relative deconditioning). While there is a transient increase in risk of cardiac complications during intense activity, it would nevertheless be inappropriate to contra-indicate physical activities considering the well-known benefits in the medium and long term. The risks associated with the practice of physical activity must be assessed, on one hand, in terms of the severity of the heart disease, and on the other hand, on the nature and intensity of the activity. The stress test is here an essential tool because it helps to assess the physical capacity and cardiorespiratory adaptations to exercise. The international recommendations for competitive sports generally give an appropriate advice for a specific situation but the practice of moderate activity or leisure sports which are highly desirable should not be neglected and be strongly encouraged. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  16. Postnatal Cardiac Autonomic Nervous Control in Pediatric Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Ineke Nederend

    2016-04-01

    Full Text Available Congenital heart disease is the most common congenital defect. During childhood, survival is generally good but, in adulthood, late complications are not uncommon. Abnormal autonomic control in children with congenital heart disease may contribute considerably to the pathophysiology of these long term sequelae. This narrative review of 34 studies aims to summarize current knowledge on function of the autonomic nervous system in children with a congenital heart defect. Large scale studies that measure both branches of the nervous system for prolonged periods of time in well-defined patient cohorts in various phases of childhood and adolescence are currently lacking. Pending such studies, there is not yet a good grasp on the extent and direction of sympathetic and parasympathetic autonomic function in pediatric congenital heart disease. Longitudinal studies in homogenous patient groups linking autonomic nervous system function and clinical outcome are warranted.

  17. Postnatal Cardiac Autonomic Nervous Control in Pediatric Congenital Heart Disease.

    Science.gov (United States)

    Nederend, Ineke; Jongbloed, Monique R M; de Geus, Eco J C; Blom, Nico A; Ten Harkel, Arend D J

    2016-04-15

    Congenital heart disease is the most common congenital defect. During childhood, survival is generally good but, in adulthood, late complications are not uncommon. Abnormal autonomic control in children with congenital heart disease may contribute considerably to the pathophysiology of these long term sequelae. This narrative review of 34 studies aims to summarize current knowledge on function of the autonomic nervous system in children with a congenital heart defect. Large scale studies that measure both branches of the nervous system for prolonged periods of time in well-defined patient cohorts in various phases of childhood and adolescence are currently lacking. Pending such studies, there is not yet a good grasp on the extent and direction of sympathetic and parasympathetic autonomic function in pediatric congenital heart disease. Longitudinal studies in homogenous patient groups linking autonomic nervous system function and clinical outcome are warranted.

  18. Chance of surgery in adult congenital heart disease.

    Science.gov (United States)

    Verheugt, Carianne L; Uiterwaal, Cuno Spm; Vaartjes, Ilonca; van der Velde, Enno T; Zomer, A C; Meijboom, Folkert J; Pieper, Petronella G; Post, Marco C; Vliegen, Hubert W; Hazekamp, Mark G; Grobbee, Diederick E; Mulder, Barbara Jm

    2017-08-01

    Background Young patients with congenital heart disease reaching adulthood face mandatory transition to adult cardiology. Their new cardiologist needs to assess the chances of major future events such as surgery. Using a large national registry, we assessed if patient characteristics at the age of 18 years could predict the chance of congenital heart surgery in adulthood. Design and methods Of 10,300 patients from the CONCOR national registry, we used general patient characteristics at age 18 years, underlying congenital heart defect, history of complications, and interventions in childhood as potential predictors of congenital heart surgery occurring from age 18 years up to age 40 and 60 years. Cox regression was used to calculate hazard ratios with 95% confidence intervals. Analyses were performed separately for all congenital heart surgery and for valvular surgery alone. Results Altogether 2427 patients underwent congenital heart surgery after age 18 years, 1389 of whom underwent valvular surgery. Underlying heart defect, male sex, multiple defects, childhood endocarditis, supraventricular arrhythmia, aortic complications and paediatric cardiovascular surgery, independently predicted adult congenital heart surgery. The mean chance of congenital heart surgery was 22% up to age 40 and 43% up to age 60 years; individual chances spanned from 9-68% up to age 40 and from 19-93% up to age 60 years. Conclusion At the time of transition from paediatric to adult cardiology, an easily obtainable set of characteristics of patients with congenital heart disease can meaningfully inform cardiologists about the patient's individual chance of surgery in adulthood. Our findings warrant validation in other cohorts.

  19. Caries treatment under general anesthesia of congenital heart disease patients before heart surgery : Case reports of two patients

    OpenAIRE

    角田, 初恵; 浅川, 麻美; 曽根, 信哉; 松本, 弘紀; 田中, 光郎; カクタ, ハツエ; アサカワ, アサミ; ソネ, シンヤ; マツモト, ヒロキ; タナカ, ミツロウ; Hatsue, KAKUTA; Asami, ASAKAWA; Shinya, SONE; Hiroki, MATSUMOTO; Mitsuro, TANAKA

    2005-01-01

    Two child patients who have congenital heart disease were treated under general anesthesia for severe dental caries in order to prevent infectious endocarditis after the heart operation. Although both the caries treatment and the subsequent heart operation of these two cases were satisfactorily completed, the necessity of early and periodical oral management for these kinds of patients was strongly suggested. The pediatric dentistry department of a general hospital should play a role in build...

  20. Junctional ectopic tachycardia after congenital heart surgery.

    Science.gov (United States)

    Cools, E; Missant, C

    2014-01-01

    In this literature review, we try to give anesthesiologists a better understanding about Junctional Ectopic Tachycardia (JET), a narrow complex tachycardia that frequently occurs during and after surgery for congenital heart disease. Information was found in the databases of Pubmed, Science Direct, Medline and the Cochrane Library, by using the mesh terms "Tachycardia, Ectopic Junctional", combined with "Diagnosis", "Etiology", "Physiopathology", "Complications" and "Therapy". The publication date of the articles ranged from 1990 to 2012. Risk factors for the development JET are surgery near the AV node, a duration of cardiopulmonary bypass longer than 90 minutes, young age, the use of inotropic drugs and hypomagnesaemia. The diagnosis of Junctional Ectopic Tachycardia can be made on a 12-lead ECG, demonstrating a narrow-complex tachycardia with inverted P-waves and VA dissociation. Adenosine administration and an atrial electrocardiogram can help to confirm the diagnosis. If JET has a minimal impact on the hemodynamic status of the patient, risk factors should be avoided and the adrenergic tonus should be reduced. Hemodynamic unstable JET can be treated by amiodarone, hypothermia and pacing. Extracorporeal membrane oxygenation (ECMO) and radiofrequency or cryoablation are treatment options for life-threatening and resistant JET. JET is the most frequent arrhythmia during and after congenital cardiac surgery. The ECG is the only available method to diagnose JET, demonstrating inverted P-waves and VA-dissociation. Amiodarone seems to be the most effective treatment option, because it can restore sinus rhythm and reduces the JET rate.

  1. Relationship between TBX20 gene polymorphism and congenital heart disease.

    Science.gov (United States)

    Yang, X F; Zhang, Y F; Zhao, C F; Liu, M M; Si, J P; Fang, Y F; Xing, W W; Wang, F L

    2016-06-02

    Congenital heart disease in children is a type of birth defect. Previous studies have suggested that the transcription factor, TBX20, is involved in the occurrence and development of congenital heart disease in children; however, the specific regulatory mechanisms are yet to be evaluated. Hence, this study aimed to evaluate the relationship between the TBX20 polymorphism and the occurrence and development of congenital heart disease. The TBX20 gene sequence was obtained from the NCBI database and the polymorphic locus candidate was predicted. Thereafter, the specific gene primers were designed for the restriction fragment length polymorphism-polymerase chain reaction (RFLP-PCR) of DNA extracted from the blood of 80 patients with congenital heart disease and 80 controls. The results of the PCR were subjected to correlation analysis to identify the differences between the amplicons and to determine the relationship between the TBX20 gene polymorphism and congenital heart disease. One of the single nucleotide polymorphic locus was found to be rs3999950: c.774T>C (Ala265Ala). The TC genotype frequency in the patients was higher than that in the controls, similar to that for the C locus. The odds ratio of the TC genotypes was above 1, indicating that the presence of the TC genotype increases the incidence of congenital heart diseases. Thus, rs3999950 may be associated with congenital heart disease, and TBX20 may predispose children to the defect.

  2. Epidemiology of Congenital Heart Disease in India.

    Science.gov (United States)

    Bhardwaj, Ritu; Rai, Sunil Kumar; Yadav, Abhishek Kumar; Lakhotia, Siddharth; Agrawal, Damyanti; Kumar, Ashok; Mohapatra, Bhagyalaxmi

    2015-01-01

    Congenital heart defects (CHDs) affect a large number of newborns and account for a high proportion of infant mortality worldwide. There are regional differences in the prevalence and distribution pattern of CHDs. The aim of this study is to estimate the distribution pattern and prevalence of CHDs among the population of north-central India and to compare the results with studies in other regions of the country to get an overview of prevalence of CHDs in India. We carried out a prospective study in the outpatient department of a tertiary care referral center in north-central India. This study was carried out from January 2011 to April 2014, with 34 517 individuals being recruited for the study. All patients were examined by chest x-ray, electrocardiogram, and 2D echocardiography. Prevalence rate per 1000 individuals examined was calculated. Relative frequencies of individual CHD types as a proportion of total CHDs were also calculated. Out of 34 517 individuals examined, 661 were diagnosed with CHDs, giving a prevalence of 19.14 per 1000 individuals. The most common defect was ventricular septal defect (33%), followed by atrial septal defect (19%) and tetralogy of Fallot (16%). The majority of CHD cases (58%) diagnosed were between 0 and 5 years of age. The prevalence of CHDs in adults was 2.4 per 1000 individuals in this cohort, with atrial septal defect (44.5%) being the most frequent defect. The prevalence of CHDs in our cohort was high, possibly because of the power of the diagnostic methods we used and the inclusion of all age groups. Adults with CHDs may significantly contribute to the prevalence of CHDs in the next generation, and this needs to be considered when estimating prevalence rates. Although several small regional studies have been carried out in India, there is an urgent need to establish a nationwide registry/database for congenital heart defects. © 2014 Wiley Periodicals, Inc.

  3. [Clinical study of congenital heart disease accompanied by hypospadias].

    Science.gov (United States)

    Lin, Yun-Hua; Xiao, Qian; Wang, Jun-Sheng; Jiang, Yong-Guang

    2014-02-01

    To study the concurrence of congenital heart disease and hypospadias and the relationship between the two diseases. We investigated the incidence and types of congenital heart disease accompanied by hypospadias in male children received in our hospital from January 2002 to December 2012, compared them with those in the general population, and analyzed the correlation of different types of heart disease with the incidence rate of hypospadias. Of the 7 385 male children with congenital heart disease, 134 (1.81%) were found with hypospadias, with a significantly higher morbidity than in the general population (0.33% -0.40%) (P congenital heart abnormalities (21/972, 2.16%) than in the atrial septal defect (10/1 015, 0.99%) and patent ductus arteriosus (6/565, 1.06%) groups (P type of hypospadias among different heart disease groups (P > 0.05). Hypospadias is a common concurrent condition in male children with congenital heart disease. The incidence rate of hypospadias is related with the type of congenital heart disease, and the two conditions may have some common pathogenic or susceptive factors.

  4. Ivabradine for junctional ectopic tachycardia in post congenital heart surgery.

    Science.gov (United States)

    Kumar, Vivek; Kumar, Gaurav; Joshi, Sajan; Sharma, Vipul

    We report two cases of malignant junctional ectopic tachycardia (JET), in infants following congenital heart surgery. After the failure of conventional therapy the arrhythmia was controlled by oral Ivabradine, a drug which is routinely used to lower heart rate in angina and heart failure in adult practice. Copyright © 2017 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  5. Increased arterial stiffness in children with congenital heart disease.

    Science.gov (United States)

    Häcker, Anna-Luisa; Reiner, Barbara; Oberhoffer, Renate; Hager, Alfred; Ewert, Peter; Müller, Jan

    2018-01-01

    Objective Central systolic blood pressure (SBP) is a measure of arterial stiffness and strongly associated with atherosclerosis and end-organ damage. It is a stronger predictor of cardiovascular events and all-cause mortality than peripheral SBP. In particular, for children with congenital heart disease, a higher central SBP might impose a greater threat of cardiac damage. The aim of the study was to analyse and compare central SBP in children with congenital heart disease and in healthy counterparts. Patients and methods Central SBP was measured using an oscillometric method in 417 children (38.9% girls, 13.0 ± 3.2 years) with various congenital heart diseases between July 2014 and February 2017. The test results were compared with a recent healthy reference cohort of 1466 children (49.5% girls, 12.9 ± 2.5 years). Results After correction for several covariates in a general linear model, central SBP of children with congenital heart disease was significantly increased (congenital heart disease: 102.1 ± 10.2 vs. healthy reference cohort: 100.4 ± 8.6, p heart disease subgroups revealed higher central SBP in children with left heart obstructions (mean difference: 3.6 mmHg, p hearts after total cavopulmonary connection (mean difference: 2.1 mmHg, p = .015) compared with the reference. Conclusion Children with congenital heart disease have significantly higher central SBP compared with healthy peers, predisposing them to premature heart failure. Screening and long-term observations of central SBP in children with congenital heart disease seems warranted in order to evaluate the need for treatment.

  6. Fetal cardiac axis and congenital heart defects in early gestation.

    Science.gov (United States)

    Sinkovskaya, Elena S; Chaoui, Rabih; Karl, Katrin; Andreeva, Elena; Zhuchenko, Ludmila; Abuhamad, Alfred Z

    2015-02-01

    To investigate the association between cardiac axis and fetal congenital heart defects to demonstrate the potential clinical applicability of cardiac axis measurement for detection of congenital heart defect in early gestation. This case-control study was undertaken in three tertiary centers with expertise in fetal imaging in early gestation. Fetal cardiac axis was evaluated between 11 0/7 and 14 6/7 weeks of gestation in 197 fetuses with confirmed congenital heart defects. A control group was selected by matching each fetus with a congenital heart defect with two fetuses in the control group with similar crown-rump length (± 5 mm) and date of study (± 2 months). Cardiac axis was measured on the four-chamber view as the angle between the line that traces the long axis of the heart and the line that bisects the thorax in an anteroposterior direction. In the control group, mean cardiac axis was 44.5 ± 7.4°. The cardiac axis did not significantly change in early pregnancy. In the congenital heart defect group, 25.9% of fetuses had cardiac axis measurements within normal limits. In 74.1%, the cardiac axis was abnormal including 110 fetuses in the case group with left deviation (cardiac axis > 97.5th percentile), 19 fetuses in the case group with right deviation (cardiac axis axis. The performance of cardiac axis measurement in detection of major congenital heart defect was significantly better than enlarged nuchal translucency, tricuspid regurgitation, or reversed A-wave in ductus venosus used alone or in combination. Abnormal cardiac axis is present in two-thirds of fetuses with congenital heart defect in early gestation. Adding cardiac axis assessment to the nuchal translucency measurement is helpful in defining a population at risk for fetal congenital heart defect.

  7. Congenital heart disease and chromossomopathies detected by the karyotype.

    Science.gov (United States)

    Trevisan, Patrícia; Rosa, Rafael Fabiano M; Koshiyama, Dayane Bohn; Zen, Tatiana Diehl; Paskulin, Giorgio Adriano; Zen, Paulo Ricardo G

    2014-06-01

    To review the relationship between congenital heart defects and chromosomal abnormalities detected by the karyotype. Scientific articles were searched in MEDLINE database, using the descriptors "karyotype" OR "chromosomal" OR "chromosome" AND "heart defects, congenital". The research was limited to articles published in English from 1980 on. Congenital heart disease is characterized by an etiologically heterogeneous and not well understood group of lesions. Several researchers have evaluated the presence of chromosomal abnormalities detected by the karyotype in patients with congenital heart disease. However, most of the articles were retrospective studies developed in Europe and only some of the studied patients had a karyotype exam. In this review, only one study was conducted in Latin America, in Brazil. It is known that chromosomal abnormalities are frequent, being present in about one in every ten patients with congenital heart disease. Among the karyotype alterations in these patients, the most important is the trisomy 21 (Down syndrome). These patients often have associated extra-cardiac malformations, with a higher risk of morbidity and mortality, which makes heart surgery even more risky. Despite all the progress made in recent decades in the field of cytogenetic, the karyotype remains an essential tool in order to evaluate patients with congenital heart disease. The detailed dysmorphological physical examination is of great importance to indicate the need of a karyotype.

  8. Evaluation of swallowing in infants with congenital heart defect.

    Science.gov (United States)

    Pereira, Karine da Rosa; Firpo, Cora; Gasparin, Marisa; Teixeira, Adriane Ribeiro; Dornelles, Silvia; Bacaltchuk, Tzvi; Levy, Deborah Salle

    2015-01-01

    Introduction Surgical repair of congenital heart disease in the first years of life compromises the coordination of the suction, breathing, and swallowing functions. Objective To describe the alterations in swallowing found in infants with congenital heart defect during their hospitalization. Methods Prospective, cross-sectional study in a reference hospital for heart disease. The sample consisted of 19 postsurgical patients who underwent an evaluation of swallowing. The infants included were younger than 7 months and had a diagnosis of congenital heart defect and suspected swallowing difficulties. Results Of the 19 infants with congenital heart defect, the median age was 3.2 months. A significant association was found between suction rhythm and dysphagia (p = 0.036) and between oral-motor oral feeding readiness and dysphagia (p = 0.014). Conclusions The data suggest that dysphagia often occurs after surgery in infants with congenital heart defect. Infants with congenital heart defect had very similar behavior to preterm infants in terms of oral feeding readiness.

  9. Evaluation of Swallowing in Infants with Congenital Heart Defect

    Directory of Open Access Journals (Sweden)

    Pereira, Karine da Rosa

    2014-11-01

    Full Text Available Introduction Surgical repair of congenital heart disease in the first years of life compromises the coordination of the suction, breathing, and swallowing functions. Objective To describe the alterations in swallowing found in infants with congenital heart defect during their hospitalization. Methods Prospective, cross-sectional study in a reference hospital for heart disease. The sample consisted of 19 postsurgical patients who underwent an evaluation of swallowing. The infants included were younger than 7 months and had a diagnosis of congenital heart defect and suspected swallowing difficulties. Results Of the 19 infants with congenital heart defect, the median age was 3.2 months. A significant association was found between suction rhythm and dysphagia (p = 0.036 and between oral-motor oral feeding readiness and dysphagia (p = 0.014. Conclusions The data suggest that dysphagia often occurs after surgery in infants with congenital heart defect. Infants with congenital heart defect had very similar behavior to preterm infants in terms of oral feeding readiness.

  10. Congenital heart disease and chromossomopathies detected by the karyotype

    Directory of Open Access Journals (Sweden)

    Patrícia Trevisan

    2014-06-01

    Full Text Available OBJECTIVE: To review the relationship between congenital heart defects and chromosomal abnormalities detected by the karyotype.DATA SOURCES: Scientific articles were searched in MEDLINE database, using the descriptors "karyotype" OR "chromosomal" OR "chromosome" AND "heart defects, congenital". The research was limited to articles published in English from 1980 on.DATA SYNTHESIS: Congenital heart disease is characterized by an etiologically heterogeneous and not well understood group of lesions. Several researchers have evaluated the presence of chromosomal abnormalities detected by the karyotype in patients with congenital heart disease. However, most of the articles were retrospective studies developed in Europe and only some of the studied patients had a karyotype exam. In this review, only one study was conducted in Latin America, in Brazil. It is known that chromosomal abnormalities are frequent, being present in about one in every ten patients with congenital heart disease. Among the karyotype alterations in these patients, the most important is the trisomy 21 (Down syndrome. These patients often have associated extra-cardiac malformations, with a higher risk of morbidity and mortality, which makes heart surgery even more risky.CONCLUSIONS: Despite all the progress made in recent decades in the field of cytogenetic, the karyotype remains an essential tool in order to evaluate patients with congenital heart disease. The detailed dysmorphological physical examination is of great importance to indicate the need of a karyotype.

  11. Genetics of Congenital Heart Malformations: Clinical and Molecular Studies

    NARCIS (Netherlands)

    M.W. Wessels (Marja)

    2009-01-01

    markdownabstract__Abstract__ Congenital heart malformations (CHM) are among the most common congenital defects, occurring in 8 out of 1000 live-births. In the past decade significant progress has been made in the identification of genes implicated in the signaling pathways involved in

  12. Clinical profile of cyanotic congenital heart disease in neonatal ...

    African Journals Online (AJOL)

    Ashraf Abou-Taleb

    2016-02-01

    % of congenital heart defects and has a high morbidity and mortality in neonates. Objective: The aim of this work was to study clinical profile and available therapeutic modalities used in the management of CCHD. Subjects ...

  13. Evaluation of the General Practice Research Database congenital heart defects prevalence: comparison to United Kingdom national systems.

    Science.gov (United States)

    Wurst, Keele E; Ephross, Sara A; Loehr, James; Clark, Douglas W; Guess, Harry A

    2007-04-01

    As part of an effort to validate the General Practice Research Database (GPRD) for future studies of medication use in pregnancy, this study examined whether the rates of all, and specific types of, congenital heart defects obtained from the GPRD are similar to those obtained from UK national systems. The prevalence rates of heart defects for 2001-2003 were determined from the GPRD and compared with both the National Congenital Anomaly System (NCAS) and the European Concerted Action of Congenital Anomalies and Twins (EUROCAT). Rate ratios (RRs) and 95% CIs were calculated comparing the prevalence of all congenital heart defects as well as specific types of heart defects in the three data sources. In addition, the effect of the child's age on the frequency of heart defects in the GPRD was determined. The prevalence of heart defects in the GPRD was more than twice as high as in the NCAS and slightly higher than in the EUROCAT. All differences were statistically significant. The prevalence of specific heart defects varied across the GPRD, NCAS, and EUROCAT. The measured prevalence of congenital heart defects in the GPRD was higher if calculated including children up to age 6. The comparisons of the GPRD prevalence rates to national prevalence estimates demonstrate that the GPRD can serve as a more complete source of background prevalence for the most commonly occurring congenital heart defects, which is essential to properly assess possible associations between maternal exposures and congenital heart defects.

  14. Guidelines for the outpatient management of complex congenital heart disease.

    Science.gov (United States)

    Wernovsky, Gil; Rome, Jonathan J; Tabbutt, Sarah; Rychik, Jack; Cohen, Meryl S; Paridon, Stephen M; Webb, Gary; Dodds, Kathryn M; Gallagher, Maureen A; Fleck, Desiree A; Spray, Thomas L; Vetter, Victoria L; Gleason, Marie M

    2006-01-01

    An increasingly complex group of children is now being followed as outpatients after surgery for congenital heart disease. A variety of complications and physiologic perturbations, both expected and unexpected, may present during follow-up, and should be anticipated by the practitioner and discussed with the patient and family. The purpose of this position article is to provide a framework for outpatient follow-up of complex congenital heart disease, based on a review of current literature and the experience of the authors.

  15. Nutritional status of children with congenital heart disease

    OpenAIRE

    Monteiro,Flávia Paula Magalhães; Araujo,Thelma Leite de; Lopes,Marcos Venícios de Oliveira; Chaves,Daniel Bruno Resende; Beltrão,Beatriz Amorim; Costa,Alice Gabrielle de Sousa

    2012-01-01

    OBJECTIVE: to characterize nutritional status and variables that predict nutritional changes in children with congenital heart disease. METHOD: a cross-sectional study undertaken in two health institutions between January and June 2009, using a questionnaire with questions about nutrition, applied to 132 children under two years of age who had congenital heart disease. Children who had additional serious illnesses were excluded. RESULT: the predominant percentile values and Z scores were conc...

  16. Nutritional Approach of Pediatric Patients Diagnosed with Congenital Heart Disease

    OpenAIRE

    Togănel Rodica

    2013-01-01

    Congenital heart defects are among the most frequent anomalies present at birth, representing a heterogeneous group of malformations, both in terms of pathogenesis and clinical significance of the lesion. Failure to grow is well documented in infants with complex congenital heart defects; the presence of associated chromosomal abnormalities, cyanosis, and cardiac failure adds to the complexity and challenge. Malnutrition etiology can be grouped into the following three categories: inadequate ...

  17. Proteinuria in Congenital Heart Disease: Is It a Real Problem?

    OpenAIRE

    Majid Maleki; Shamsi Ghaffari; Mohammad Reza Ghaffari; Mahmood Samadi; Bahman Rastkar; Pooya Maleki; Sahar Behnam

    2011-01-01

    The relationship between congenital heart disease and nephropathy has been known for a long time although its mechanism has not been understood thoroughly. Furthermore such studies have been performed in older populations. 74 children aged between two months to 168 months (20 normal as control group, 20 cyanotic and 34 acyanotic patients with congenital heart disease were investigated for their renal function and protein excretion. The data were analyzed using SPSS (version 16....

  18. Genetic assembly of the heart: implications for congenital heart disease.

    Science.gov (United States)

    Srivastava, D

    2001-01-01

    More children die from congenital heart defects (CHD) each year than are diagnosed with childhood cancer, yet the causes remain unknown. The remarkable conservation of genetic pathways regulating cardiac development in species ranging from flies to humans provides an opportunity to experimentally dissect the role of critical cardiogenic factors. Utilization of model biological systems has resulted in a molecular framework in which to consider the etiology of CHD. As whole genome sequencing and single nucleotide polymorphism data become available, identification of genetic mutations predisposing to CHD may allow preventive measures by modulation of secondary genetic or environmental factors. In this review, genetic pathways regulating cardiogenesis revealed by cross-species studies are reviewed and correlated with human CHD.

  19. Congenital Anorectal Malformation Severity Does Not Predict Severity of Congenital Heart Defects

    NARCIS (Netherlands)

    Jonker, Jara E.; Liem, Eryn T.; Elzenga, Nynke J.; Molenbuur, Bouwe; Trzpis, Monika; Broens, Paul M. A.

    2016-01-01

    Objective To determine the prevalence of congenital heart defects (CHDs) in patients with mild or severe congenital anorectal malformations (CARMs), and whether all patients with CARM need pediatric cardiology screening. Study design We included 129 patients with CARM born between 2004 and 2013, and

  20. Burden and impact of congenital syndromes and comorbidities among adults with congenital heart disease.

    Science.gov (United States)

    Bracher, Isabelle; Padrutt, Maria; Bonassin, Francesca; Santos Lopes, Bruno; Gruner, Christiane; Stämpfli, Simon F; Oxenius, Angela; De Pasquale, Gabriella; Seeliger, Theresa; Lüscher, Thomas F; Attenhofer Jost, Christine; Greutmann, Matthias

    2017-08-01

    Our aim was to assess the overall burden of congenital syndromes and non-cardiac comorbidities among adults with congenital heart disease and to assess their impact on circumstances of living and outcomes. Within a cohort of 1725 adults with congenital heart defects (65% defects of moderate or great complexity) followed at a single tertiary care center, congenital syndromes and comorbidities were identified by chart review. Their association with arrhythmias, circumstances of living and survival was analyzed. Within the study cohort, 232 patients (13%) had a genetic syndrome, 51% at least one comorbidity and 23% ≥2 comorbidities. Most prevalent comorbidities were systemic arterial hypertension (11%), thyroid dysfunction (9%), psychiatric disorders (9%), neurologic disorders (7%), chronic lung disease (7%), and previous stroke (6%). In contrast to higher congenital heart defect complexity, the presence of comorbidities had no impact on living circumstances but patients with comorbidities were less likely to work full-time. Atrial arrhythmias were more common among patients with moderate/great disease complexity and those with comorbidities but were less common among patients with congenital syndromes (pCongenital syndromes and comorbidities are highly prevalent in adults with congenital heart disease followed at specialist centers and add to the overall complexity of care. The presence of these additional factors has an impact on living circumstances, is associated with arrhythmias and needs to be further explored as prognostic markers. Copyright © 2017 Elsevier B.V. All rights reserved.

  1. Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report

    OpenAIRE

    Nakao, Atsushi; Takeda, Tomohiro; Hisaeda, Yoshiya; Hirota, Atsushi; Amagata, Syusuke; Sakurai, Yuko; Kawakami, Tadashi

    2013-01-01

    Pancreatic agenesis is a rare disease that causes neonatal diabetes mellitus and exocrine pancreatic insufficiency. We report the case of a very low birth weight infant with congenital diaphragmatic hernia, and congenital heart disease (ventricular septal defect and patent ductus arteriosus). Failure to gain weight, despite well-managed respiratory and heart failure, was improved by infusion of subcutaneous insulin, administration of pancreatic enzyme, and nutrition of medium-chain-triglyceri...

  2. Atrial tachyarrhythmia in adult congenital heart disease

    Science.gov (United States)

    Karbassi, Arsha; Nair, Krishnakumar; Harris, Louise; Wald, Rachel M; Roche, S Lucy

    2017-01-01

    The adult congenital heart disease (ACHD) population continues to grow and most cardiologists, emergency room physicians and family doctors will intermittently come into contact with these patients. Oftentimes this may be in the setting of a presentation with atrial tachyarrhythmia; one of the commonest late complications of ACHD and problem with potentially serious implications. Providing appropriate initial care and ongoing management of atrial tachyarrhythmia in ACHD patients requires a degree of specialist knowledge and an awareness of certain key issues. In ACHD, atrial tachyarrhythmia is usually related to the abnormal anatomy of the underlying heart defect and often occurs as a result of surgical scar or a consequence of residual hemodynamic or electrical disturbances. Arrhythmias significantly increase mortality and morbidity in ACHD and are the most frequent reason for ACHD hospitalization. Intra-atrial reentrant tachycardia and atrial fibrillation are the most prevalent type of arrhythmia in this patient group. In hemodynamically unstable patients, urgent cardioversion is required. Acute management of the stable patient includes anticoagulation, rate control, and electrical or pharmacological cardioversion. In ACHD, rhythm control is the preferred management strategy and can often be achieved. However, in the long-term, medication side-effects can prove problematic. Electrophysiology studies and catheter ablation are important treatments modalities and in certain cases, surgical or percutaneous treatment of the underlying cardiac defect has a role. ACHD patients, especially those with complex CHD, are at increased risk of thromboembolic events and anticoagulation is usually required. Female ACHD patients of child bearing age may wish to pursue pregnancies. The risk of atrial arrhythmias is increased during pregnancy and management of atrial tachyarrhythmia during pregnancy needs specific consideration. PMID:28706585

  3. Perioperative infections in congenital heart disease.

    Science.gov (United States)

    Murni, Indah K; MacLaren, Graeme; Morrow, Debra; Iyer, Parvathi; Duke, Trevor

    2017-12-01

    Perioperative infections have significant consequences for children with congenital heart disease (CHD), which can manifest as acute or chronic infection followed by poor growth and progressive cardiac failure. The consequences include delayed or higher-risk surgery, and increased postoperative morbidity and mortality. A systematic search for studies evaluating the burden and interventions to reduce perioperative infections in children with CHD was undertaken using PubMed. Limited studies conducted in low- to middle-income countries demonstrated the large burden of perioperative infections among children with CHD. Most studies focussed on infections after surgery. Few studies evaluated strategies to prevent preoperative infection or the impact of infection on decision-making around the timing of surgery. Children with CHD have multiple risk factors for infections including delayed presentation, inadequate treatment of cardiac failure, and poor nutrition. The burden of perioperative infections is high among children with CHD, and studies evaluating the effectiveness of interventions to reduce these infections are lacking. As good nutrition, early corrective surgery, and measures to reduce nosocomial infection are likely to play a role, practical steps can be taken to make surgery safer.

  4. Genomic imbalances in syndromic congenital heart disease.

    Science.gov (United States)

    Molck, Miriam Coelho; Simioni, Milena; Paiva Vieira, Társis; Sgardioli, Ilária Cristina; Paoli Monteiro, Fabíola; Souza, Josiane; Fett-Conte, Agnes Cristina; Félix, Têmis Maria; Lopes Monlléo, Isabella; Gil-da-Silva-Lopes, Vera Lúcia

    To identify pathogenic genomic imbalances in patients presenting congenital heart disease (CHD) with extra cardiac anomalies and exclusion of 22q11.2 deletion syndrome (22q11.2 DS). 78 patients negative for the 22q11.2 deletion, previously screened by fluorescence in situ hybridization (FISH) and/or multiplex ligation probe amplification (MLPA) were tested by chromosomal microarray analysis (CMA). Clinically significant copy number variations (CNVs ≥300kb) were identified in 10% (8/78) of cases. In addition, potentially relevant CNVs were detected in two cases (993kb duplication in 15q21.1 and 706kb duplication in 2p22.3). Genes inside the CNV regions found in this study, such as IRX4, BMPR1A, SORBS2, ID2, ROCK2, E2F6, GATA4, SOX7, SEMAD6D, FBN1, and LTPB1 are known to participate in cardiac development and could be candidate genes for CHD. These data showed that patients presenting CHD with extra cardiac anomalies and exclusion of 22q11.2 DS should be investigated by CMA. The present study emphasizes the possible role of CNVs in CHD. Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  5. Potential for stem cell use in congenital heart disease.

    Science.gov (United States)

    Pincott, Emma Siân; Burch, Michael

    2012-03-01

    This article reports on the evolving field of stem cell therapy and its impact on the management of cardiac pathology, in particular congenital heart disease. To date, stem cell therapy has focused on cardiomyoplasty for heart muscle disease, stem cell therapies are already in clinical use for these disorders. Research is now also supporting the potential role of stem cell therapy for congenital heart disease. In the future it may be possible to use stem cells to create cellular grafts and structures that may be surgically implanted into the disordered heart using bioengineering technology. Different types of stem cells have been evaluated and the identification of specific cardiac stem cells offers great potential. Preliminary animal studies investigating fetal cardiac therapies are also underway. These new directions for stem cell research provide exciting potential for the future management of congenital heart disease.

  6. 2013 update on congenital heart disease, clinical cardiology, heart failure, and heart transplant.

    Science.gov (United States)

    Subirana, M Teresa; Barón-Esquivias, Gonzalo; Manito, Nicolás; Oliver, José M; Ripoll, Tomás; Lambert, Jose Luis; Zunzunegui, José L; Bover, Ramon; García-Pinilla, José Manuel

    2014-03-01

    This article presents the most relevant developments in 2013 in 3 key areas of cardiology: congenital heart disease, clinical cardiology, and heart failure and transplant. Within the area of congenital heart disease, we reviewed contributions related to sudden death in adult congenital heart disease, the importance of specific echocardiographic parameters in assessing the systemic right ventricle, problems in patients with repaired tetralogy of Fallot and indication for pulmonary valve replacement, and confirmation of the role of specific factors in the selection of candidates for Fontan surgery. The most recent publications in clinical cardiology include a study by a European working group on correct diagnostic work-up in cardiomyopathies, studies on the cost-effectiveness of percutaneous aortic valve implantation, a consensus document on the management of type B aortic dissection, and guidelines on aortic valve and ascending aortic disease. The most noteworthy developments in heart failure and transplantation include new American guidelines on heart failure, therapeutic advances in acute heart failure (serelaxin), the management of comorbidities such as iron deficiency, risk assessment using new biomarkers, and advances in ventricular assist devices. Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

  7. Neurocognitive functioning in adults with congenital heart disease.

    Science.gov (United States)

    Ilardi, Dawn; Ono, Kim E; McCartney, Rebecca; Book, Wendy; Stringer, Anthony Y

    2017-03-01

    Adults with congenital heart disease (CHD) are at increased risk of psychological disorders and cognitive deficiencies due to structural/acquired neurological abnormalities and neurodevelopmental disorders as children. However, limited information is known about the neuropsychological functioning of adults with CHD. This study screened neuropsychological abilities and explored group differences related to cardiac disease severity and neurological risk factors in adults with CHD. Participants completed brief neuropsychological testing. Information about neurobehavioral and psychological symptoms, employment, education, and disability were also collected from the patient and a family member. Forty-eight participants with adult CHD completed neuropsychological testing. Visuospatial skills and working memory were worse than expected compared to the typical population. Frequency of neurological comorbidities (e.g., stroke, seizures) was higher in those with more severe heart disease (e.g., single ventricle or cyanotic disease), and executive functioning was weaker in those with neurological comorbidities. Those with more severe heart disease were more likely to be unemployed and to receive disability benefits, but educational attainment did not differ. Those who received disability performed worse on tasks of executive functioning. Findings suggest concerns about neuropsychological functioning that need to be more comprehensively assessed in adults with CHD. Understanding the cognitive limitations of this aging population can help guide access to resources, transition of care, and medical care engagement, thus improving quality of care and quality of life. © 2016 Wiley Periodicals, Inc.

  8. Congenital heart defects in children with oral clefts

    Directory of Open Access Journals (Sweden)

    Nahvi H.

    2007-09-01

    Full Text Available   Background: Oral clefts are among the most common congenital anomalies. Infants with oral clefts often have other associated congenital defects, especially congenital heart defects. The reported incidences and the types of associated malformations and congenital heart defects vary between different studies. The purpose of this study was to assess the incidence of associated congenital heart defects in children with oral clefts. Methods: All infants with cleft lip and palate referred to the Children's Medical Center and Bahramy; the teaching Hospitals of the Tehran University of Medical Sciences from 1991 to 2005 were prospectively enrolled in this study group. All patients were examined and noted by an academic cleft team contain; a pediatrician and a pediatric surgeon, and received cardiac consultation and echocardiography by a pediatric cardiologist. non cardiac associated anomalies, still born and patients without echocardiography were excluded from the study.  Data including age, gender, exposure to contagions and high risk elements ,consanguinity and familial history of oral cleft, type of oral cleft, results of cardiac consultation and echocardiography and associated cardiac anomalies were cumulated and analyzed by SSPS version 13.5Results: Among the 284 infants with oral clefts, 162 were male (57% and 122 were female (43%. Seventy-nine patients (27.8% had cleft lip, 84 (29.5% had cleft palate and 121 (42.6% had both cleft lip and palate. Of all the patients, 21.1% had congenital heart defects. the most common type Of these congenital heart defects(28.3%  was atrial septal defect.Conclusions: For patients with cleft lip and palate, we recommend preoperative cardiac consultation, careful examination and routine echocardiography for associated cardiac anomalies, as well as appropriate management and prophylactic antibiotic therapy for those with associated congenital heart anomaly.

  9. Pattern and Diagnosis of Congenital Heart Disease in Patients ...

    African Journals Online (AJOL)

    Objective: To study the pattern of Congenital Heart Diseases (CHD) in children referred to Ahmed Gasim Cardiac Center) in Khartoum. Methods: This is a prospective cross-sectional, clinic based study conducted over a six months period. The children were referred to the Cardiac Centre because of suspected heart ...

  10. Serum zinc values in children with congenital heart disease | Sadoh ...

    African Journals Online (AJOL)

    Background: Some children with congenital heart diseases (CHD) may have increased pulmonary blood flow that causes recurrent bronchopneumonia and congestive heart failure. Serum zinc is reduced in children with pneumonia and patients on diuretics. Objective: To evaluate the serum zinc level of children with CHD ...

  11. Underlying congenital heart disease in Nigerian children with ...

    African Journals Online (AJOL)

    EB

    2013-09-03

    Sep 3, 2013 ... Keyword: pneumonia, congenital heart disease, congestive heart failure. African Health Sciences 2013; 13(3): 607 - 612 ... study of acute respiratory infections among children in Northern Nigeria, the rate of pneumonia .... stenosis involved valvular and supravalvular membrane. Of the 14 patients with CHD, ...

  12. Heartbeat sensitivity in adults with congenital heart disease

    NARCIS (Netherlands)

    Rietveld, Simon; Karsdorp, Petra A.; Mulder, Barbara J. M.

    2004-01-01

    This study tested the hypothesis that patients with a congenital heart disease are sensitive regarding heartbeat perception, reflected in enhanced attention for heartbeat, estimation of own heart rate, and a vulnerability to become anxious by listening to heartbeat sounds. Twenty adults with a

  13. The Spectrum of Paediatric Congenital Heart Disease at The ...

    African Journals Online (AJOL)

    Background Congenital Heart Disease (CHD) is a significant cause of morbidity and mortality amongst infants and children globally. Complex heart lesions are more costly to manage than simple lesions. Geographical differences in the spectrum of CHD have been reported; knowledge of the spectrum of CHD provides a ...

  14. Retrospective review of congenital heart disease in 976 dogs.

    Science.gov (United States)

    Oliveira, P; Domenech, O; Silva, J; Vannini, S; Bussadori, R; Bussadori, C

    2011-01-01

    Knowledge of epidemiology is important for recognition of cardiovascular malformations. Review the incidence of congenital heart defects in dogs in Italy and assess breed and sex predispositions. Nine hundred and seventy-six dogs diagnosed with congenital heart disease (CHD) of 4,480 dogs presented to Clinica Veterinaria Gran Sasso for cardiovascular examination from 1997 to 2010. A retrospective analysis of medical records regarding signalment, history, clinical examination, radiography, electrocardiography, echocardiography, angiography, and postmortem examination was performed. Breed and sex predisposition were assessed with the odds ratio test. CHD was observed in 21.7% of cases. A total of 1,132 defects were observed with single defects in 832 cases (85%), 2 concurrent defects in 132 cases (14%), and 3 concurrent defects in 12 cases (1%). The most common defects were pulmonic stenosis (PS; 32.1%), subaortic stenosis (SAS; 21.3%), and patent ductus arteriosus (20.9%), followed by ventricular septal defect (VSD; 7.5%), valvular aortic stenosis (AS; 5.7%), and tricuspid dysplasia (3.1%). SAS, PS, and VSD frequently were associated with other defects. Several breed and sex predispositions were identified. The results of this study are in accordance with previous studies, with slight differences. The breed and sex predilections identified may be of value for the diagnosis and screening of CHD in dogs. Additionally, the relatively high percentage of concurrent heart defects emphasizes the importance of accurate and complete examinations for identification. Because these data are from a cardiology referral center, a bias may exist. Copyright © 2011 by the American College of Veterinary Internal Medicine.

  15. Cumulative radiation exposure in pediatric patients with congenital heart disease.

    Science.gov (United States)

    Walsh, Mark A; Noga, Michelle; Rutledge, Jennifer

    2015-02-01

    Certain pediatric patients undergoing surgery for the most severe forms of congenital heart disease are exposed to high doses of ionizing radiation. The amount of cumulative radiation exposure from all modalities has not yet been evaluated. The purpose of our study was to evaluate the cumulative radiation exposure in a contemporary cohort of patients with congenital heart disease undergoing single-ventricle palliation. This is a single-center, retrospective study of pediatric patients undergoing Fontan completion between May 2005 and May 2010. Radiation exposure from all procedures including cardiac catheterizations, computed tomography (CT) scans, plain film radiography, and nuclear medicine scans was evaluated. Radiation dose was calculated as the dose area product (μGy m(2)) and was measured in all cardiac catheterizations, CT scans, and other imaging modalities. Seventy patients who underwent Fontan completion at a mean age of 3.6 ± 1.5 years (range 1.4-8 years) were included in the study. Mean number of chest X-rays was 32 ± 8 (range 10-285) with a mean cumulative total exposure of 1,320 μGy m(2) (range 480-12,960) per patient. Mean number of cardiac catheterizations was 2.45 ± 1.3 (range 1-8), and mean fluoroscopy and cine angiography exposures per case were 1,103 ± 245 and 1,412 ± 273 μGy m(2) giving a mean cumulative exposure of 9,054 μGy m(2) (range 2,515-201,200) per patient for all catheterizations. Mean number of CT scans performed was 0.44 ± 0.4 (0-11), and the mean exposure was 352 μGy m(2), giving a mean cumulative total of 154 μGy m(2) (range 0-3,872) per person. A total of five lung perfusion scans were carried out. Radiation exposure in patients with congenital heart disease undergoing single-ventricle palliation is quite variable. Most of the exposure to ionizing radiation occurs during cardiac catheterization. Strategies to utilize other imaging modalities such as MRI would decrease exposure in this particular group of patients who

  16. The effect of a resilience improvement program for adolescents with complex congenital heart disease.

    Science.gov (United States)

    Lee, Sunhee; Lee, Junga; Choi, Jae Young

    2017-04-01

    Adolescents with congenital heart disease need to increase their resilience in the face of challenges in order to preserve their health and quality of life. This study aimed to develop a resilience improvement program for adolescents with congenital heart disease and also to evaluate any change in resilience and quality of life as a measure of the effectiveness of the resilience improvement programs. A nonequivalent control group pretest-posttest study was designed. Twenty-five adolescents who attended the first resilience improvement program were included in the experimental group, and 31 adolescents who took part in the second program were placed in the control group. Adolescents with congenital heart disease completed a self-report questionnaire on three separate occasions: the pretest, the first posttest and the second posttest. The self-report questionnaire included general characteristics and instruments to measure resilience and quality of life. For the longitudinal analysis, generalized estimating equations were used to evaluate the difference in the estimated average trajectories of resilience and quality of life changes. Independent predictors of resilience improvement in adolescents with congenital heart disease were the experimental group ( p=0.02) and middle and high school students ( p=0.02). Quality of life was not associated with membership in the experimental group. However, males scored higher than females on quality of life measures ( p=0.02). It is essential for healthcare providers to apply various programs, including those targeted at accepting illness, improving autonomy and independently managing disease, to adolescents with congenital heart disease.

  17. Increasing mortality burden among adults with complex congenital heart disease.

    Science.gov (United States)

    Greutmann, Matthias; Tobler, Daniel; Kovacs, Adrienne H; Greutmann-Yantiri, Mehtap; Haile, Sarah R; Held, Leonhard; Ivanov, Joan; Williams, William G; Oechslin, Erwin N; Silversides, Candice K; Colman, Jack M

    2015-01-01

    Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease. Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029. Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot. Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease. © 2014 The Authors. Congenital Heart Disease Published by Wiley Periodicals, Inc.

  18. Magnetic resonance imaging of congenital heart disease at 0.3 T

    Energy Technology Data Exchange (ETDEWEB)

    Malmgren, N.

    1995-11-01

    The aim of this study was to systematically evaluate the potential of static MR imaging in congenital heart disease. The diagnosis of vascular rings, causing significant respiratory morbidity, previously required invasive methods for diagnosis. In aberrant left pulmonary artery, an uncommon anomaly, MR imaging at 0.3 T provided complete anatomic information. Complex congenital heart malformation is a diagnostic challenge. MR imaging at 0.3 T was efficient in defining most of the components of complex disease. It is especially valuable for imaging of extracardiac structures, which can be difficult to evaluate using echocardiography. MR imaging is an important complement to echocardiography. Improved equipment with sophisticated dynamic applications using high magnetic field strength is now available. The anatomical evaluation of congenital heart anomalies is however still based on static images, and low field magnets will certainly be used even in the future. 141 refs, 11 figs, 8 tabs.

  19. Consanguinity and the risk of congenital heart disease.

    Science.gov (United States)

    Shieh, Joseph T C; Bittles, Alan H; Hudgins, Louanne

    2012-05-01

    Consanguineous unions have been associated with an increased susceptibility to various forms of inherited disease. Although consanguinity is known to contribute to recessive diseases, the potential role of consanguinity in certain common birth defects is less clear, particularly since the disease pathophysiology may involve genetic and environmental/epigenetic factors. In this study, we ask whether consanguinity affects one of the most common birth defects, congenital heart disease, and identify areas for further research into these birth defects, since consanguinity may now impact health on a near-global basis. A systematic review of consanguinity in congenital heart disease was performed, focusing on non-syndromic disease, with the methodologies and results from studies of different ethnic populations compared. The risks for congenital heart disease have been assessed and summarized collectively and by individual lesion. The majority of studies support the view that consanguinity increases the prevalence of congenital heart disease, however, the study designs differed dramatically. Only a few (n = 3) population-based studies that controlled for potential sociodemographic confounding were identified, and data on individual cardiac lesions were limited by case numbers. Overall the results suggest that the risk for congenital heart disease is increased in consanguineous unions in the studied populations, principally at first-cousin level and closer, a factor that should be considered in empiric risk estimates in genetic counseling. However, for more precise risk estimates a better understanding of the underlying disease factors is needed. Copyright © 2012 Wiley Periodicals, Inc.

  20. A flatline Lead I in a neonate with suspected congenital heart disease.

    Science.gov (United States)

    Riniker, Kristy; Rigales, Luis; Hoffman, Irwin

    In a neonate with bradycardia and hypoxia, and clinical concern for critical congenital heart disease (CCHD), highly unusual ECG findings were present. These included a flatline Lead 1, and identical Leads 2, 3, and AVF. These abnormalities were due to bilateral arm to leg lead exchanges and not to heart disease. Complete cardiac workup including echocardiogram was normal. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Echocardiographic evaluation of coronary arteries in congenital heart disease.

    Science.gov (United States)

    Freire, Grace; Miller, Michelle S

    2015-12-01

    Among populations of patients with the congenital heart disease, there is considerable diversity in the anatomy of the coronary arteries. Understanding these anatomical differences is vitally important in directing interventions and surgical repair. In this report, the authors describe the echocardiographic evaluation of the variants of coronary artery anatomy in the following lesions: transposition of the great arteries, congenitally corrected transposition of the great arteries, double-inlet left ventricle, common arterial trunk, tetralogy of Fallot, and double-outlet right ventricle.

  2. The incidence of congenital heart disease: Previous findings and perspectives

    OpenAIRE

    Miranović Vesna

    2014-01-01

    Congenital heart defects (CHD) are the most common of all congenital anomalies, and represent a significant global health problem. Involvement of medical professionals of different profiles has led to drastic changes in survival and quality of life of children with CHD. The motivation for the implementation of the first large population studies on this subject was not only to obtain answers to the question on the level of incidence of CHD, but the harmoniza...

  3. Evaluation of canine congenital heart disease using an echocardiographic algorithm.

    Science.gov (United States)

    Oyama, M A; Sisson, D D

    2001-01-01

    Evaluation of canine congenital heart disease presents a diagnostic challenge to many ultrasonographers. To assist clinicians attempting to examine these patients, an echocardiographic algorithm containing the six most common canine congenital heart diseases (i.e., patent ductus arteriosus, subaortic stenosis, pulmonic stenosis, ventricular septal defect, tricuspid dysplasia, and tetralogy of Fallot) is presented. The algorithm focuses on the underlying disease pathogenesis and the resultant changes in cardiac structure and function that can be readily identified during echocardiographic examination. Use of this algorithm provides a framework from which the ultrasonographer gains both experience and confidence in diagnosing congenital heart disease via echocardiography. This algorithm is supported by a number of still figures within the article as well as real-time echocardiographic images available for viewing at AAHA's website, www.aahanet.org.

  4. AIR TRAVEL FOR INFANTS WITH CONGENITAL HEART DISEASE

    Directory of Open Access Journals (Sweden)

    Adarsh Patil

    2015-01-01

    Full Text Available INTRODUCTION : It is well documented that, healthy person can tolerate the cabin environment of a commercial airline which is pressurized to the level of 5000 ft. however this environment brings profound physiological changes in patients with cardiovascular dise a se. With rise in number of patients travelling internationally for treatment of cardiac problems especially infants travelling by air for congenital heart disease treatment has increased in the recent time and is evident by the reports of medical incidents involving infants with congenital heart disease onboard . (1 Though the IATA medical manual mentions that adult patients with Esenminger Syndrome should not undertake air travel . (2 This article examines the case for infants with congenital heart diseases as there have been no previous studies reported

  5. Adult congenital heart disease: surgical advances and options.

    Science.gov (United States)

    Guleserian, Kristine J

    2011-01-01

    The number of adults with congenital heart disease (CHD) has steadily increased as medical and surgical treatment of congenital heart lesions--whether simple or complex--continues to improve. Over the past half century advances in surgical technique have continued with the evolution of traditional surgical repair and introduction of new surgical procedures for complex lesions previously considered to be irreparable. This article describes the rich history of surgical repair, important surgical considerations specific to the adult undergoing primary or reoperative cardiac repair or palliation, the most common types congenital heart lesions and associated cardiac procedures (including cardiac transplantation) performed in the adult population, as well as considerations regarding the optimal surgical environment and current surgical training and education. Copyright © 2011 Elsevier Inc. All rights reserved.

  6. Do All Children with Congenital Complete Atrioventricular Block Require Permanent Pacing ?

    Directory of Open Access Journals (Sweden)

    Christian Balmer

    2003-07-01

    Full Text Available With an incidence of 1 in 20’000 live born infants1, congenital complete atrioventricular block (CCAVB is a rare disease. The aetiology is not completely understood. However, CCAVB may be isolated or combined with congenital heart diseases in up to 53% of affected individuals2. Isolated CCAVB is in up to 98% of the children associated with positive autoimmune antibodies in the maternal serum (anti-Ro/SS-A and anti-LA/SS-B3,4. Interestingly, these antibodies are not specifically directed against the conduction system but also against normal myocardial cells and may cause myocarditis5,6. Affection of the conduction system can occur at different levels7. Histologically, the atrioventricular node tissue may be replaced by fibrous fatty tissue with variable involvement of the distal conduction system8.

  7. Determinants of early dilated cardiomyopathy in neonates with congenital complete atrioventricular block.

    Science.gov (United States)

    Silvetti, Massimo Stefano; Drago, Fabrizio; Ravà, Lucilla

    2010-09-01

    Dilated cardiomyopathy (DCM) can occur in infants with congenital complete atrioventricular block (CCAVB) treated by permanent pacemaker (PM), even without other congenital heart defects. The objective is to find the risk factors of this complication. Retrospective analysis of a single-centre experience. Since 1992, 25 patients, aged 25 (1-355) days [median (range)], with normal ejection fraction (EF), underwent PM implantation (13 DDD, 12 VVIR) with an RV-pacing site. Follow-up was 4 (0.3-16) years. DCM occurred after 4 (3-23) months in eight patients (32%). Univariate analysis identified the following risk factors: younger age at implantation [5 (1-85) days vs. 90 (1-355) P = 0.007], a broad QRS (50 vs. 18% P = 0.03), prolonged QTc at implantation (63 vs. 0%, P = 0.001), and greater duration of heart rate >160 bpm during the first month after implantation (18 vs. 2%, P = 0.03). By multivariate analysis prolonged QTc was the only significant risk factor for DCM (hazard ratio: 23, P mode to allow predominant narrow QRS junctional rhythm (one patient each). Neonates with CCAVB without other congenital heart defects and prolonged QTc are at high risk for DCM possibly due to electromechanical dyssynchrony induced by high-rate RV pacing. In patients in whom RV pacing was discontinued, EF became normal.

  8. Introduction to the Congenital Heart Defects: Anatomy of the Conduction System.

    Science.gov (United States)

    Moore, Jeremy P; Aboulhosn, Jamil A

    2017-06-01

    The position and course of the conduction system in congenital heart disease are intricately tied to the underlying congenital malformation. Although only subtle differences exist between the anatomy of the conduction axis for simple congenital heart lesions and normal anatomy, almost every patient with congenital heart disease harbors some important anatomic variation. This article summarizes the body of literature by retaining original classical concepts and by attempting to translate the available knowledge into useful points for the congenital heart disease specialist. This discussion spans the entire spectrum of simple to complex congenital heart disease. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. Advances in cardiac magnetic resonance imaging of congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Driessen, Mieke M.P. [University of Utrecht, University Medical Center Utrecht, Department of Radiology, PO Box 85500, Utrecht (Netherlands); University of Utrecht, University Medical Center Utrecht, Department of Cardiology, PO Box 85500, Utrecht (Netherlands); The Interuniversity Cardiology Institute of the Netherlands (ICIN) - Netherlands Heart Institute, PO Box 19258, Utrecht (Netherlands); Breur, Johannes M.P.J. [Wilhelmina Children' s Hospital, University Medical Center Utrecht, Department of Pediatric Cardiology, PO Box 85500, Utrecht (Netherlands); Budde, Ricardo P.J.; Oorschot, Joep W.M. van; Leiner, Tim [University of Utrecht, University Medical Center Utrecht, Department of Radiology, PO Box 85500, Utrecht (Netherlands); Kimmenade, Roland R.J. van; Sieswerda, Gertjan Tj [University of Utrecht, University Medical Center Utrecht, Department of Cardiology, PO Box 85500, Utrecht (Netherlands); Meijboom, Folkert J. [University of Utrecht, University Medical Center Utrecht, Department of Cardiology, PO Box 85500, Utrecht (Netherlands); Wilhelmina Children' s Hospital, University Medical Center Utrecht, Department of Pediatric Cardiology, PO Box 85500, Utrecht (Netherlands)

    2015-01-01

    Due to advances in cardiac surgery, survival of patients with congenital heart disease has increased considerably during the past decades. Many of these patients require repeated cardiovascular magnetic resonance imaging to assess cardiac anatomy and function. In the past decade, technological advances have enabled faster and more robust cardiovascular magnetic resonance with improved image quality and spatial as well as temporal resolution. This review aims to provide an overview of advances in cardiovascular magnetic resonance hardware and acquisition techniques relevant to both pediatric and adult patients with congenital heart disease and discusses the techniques used to assess function, anatomy, flow and tissue characterization. (orig.)

  10. A review of heart failure in adults with congenital heart disease.

    Science.gov (United States)

    Parekh, Dhaval R

    2011-01-01

    The nearly one-million estimated adult congenital heart disease (ACHD) patients in the United States now outnumber children with congenital heart disease (CHD). With continued improvement in survival due to surgical and medical management of patients born with CHD, there is an overall shift in the burden of care from childhood to adulthood. Due to this transitioning population, the probability of heart failure continues to increase with age and represents nearly one-quarter of all mortality in ACHD. Despite these sobering figures adult cardiologist and fellows continue to have limited exposure in the care of patients with congenital heart disease. The syndrome of heart failure represents a complex derangement of neurohormones, natriuretic peptides, and cytokines leading to progressive symptoms of exercise intolerance, dyspnea, and fatigue. Congenital heart patients represent a unique challenge in both categorization and protocol management of heart failure (HF). It remains unclear if the current four-stage ACC/AHA guidelines for diagnosis and treatment of HF in adults can serve as a meaningful framework for congenital heart patients. Additionally, widely used conventional HF therapy of beta-blockers and angiotensin converting enzyme inhibitors (ACE-I) have not demonstrated clear survival benefit in this population. Unfortunately, adequately powered and controlled randomized studies are grossly lacking and remain challenging to conduct. Nonetheless, a review of heart failure associated with ACHD is provided.

  11. Prevalence of Congenital Heart Diseases in Children with Congenital Hypothyroidism

    Directory of Open Access Journals (Sweden)

    Mohamed Abdel Megied Abo El-Magd

    2013-04-01

    Full Text Available The aim of the work was to assess the prevalence of cardiac anomalies in primary congenital hypothyroidism (PCH patients. Fifty patients with PCH recruited after diagnosis by ultrasonography or scintigraphy (64% Dysgenesis, 36% Dyshormonogenesis. The prevalence of cardiac anomalies was 18%, with renal anomalies being 8%. There was no significant difference in the longitudinal follow-up of growth and sexual maturation between a hypothyroid with and without anomalies. Statistically significant difference was found with replacement therapy of both groups. Hence, echocardiography should be done to screen this birth defect as soon as possible so as to prevent or delay the possible complications. [Arch Clin Exp Surg 2013; 2(2.000: 85-91

  12. Neurodevelopmental Outcomes in Congenital Heart Disease

    Science.gov (United States)

    ... that are most sensitive to oxygen and nutrient deprivation. Some types of CHD allow blood clots to ... American Heart Association, Inc. All rights reserved. Unauthorized use prohibited. The American Heart Association is a qualified ...

  13. Pancreatic agenesis with congenital diaphragmatic hernia and congenital heart disease: a case report.

    Science.gov (United States)

    Nakao, Atsushi; Takeda, Tomohiro; Hisaeda, Yoshiya; Hirota, Atsushi; Amagata, Syusuke; Sakurai, Yuko; Kawakami, Tadashi

    2013-10-01

    Pancreatic agenesis is a rare disease that causes neonatal diabetes mellitus and exocrine pancreatic insufficiency. We report the case of a very low birth weight infant with congenital diaphragmatic hernia, and congenital heart disease (ventricular septal defect and patent ductus arteriosus). Failure to gain weight, despite well-managed respiratory and heart failure, was improved by infusion of subcutaneous insulin, administration of pancreatic enzyme, and nutrition of medium-chain-triglyceride -enriched formula. This is the first case of pancreatic agenesis with both malformations where the patient is discharged from the hospital. Early diagnosis and adequate treatments to compensate pancreatic function may prevent mortality and improve growth.

  14. Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Atsushi Nakao

    2013-10-01

    Full Text Available Pancreatic agenesis is a rare disease that causes neonatal diabetes mellitus and exocrine pancreatic insufficiency. We report the case of a very low birth weight infant with congenital diaphragmatic hernia, and congenital heart disease (ventricular septal defect and patent ductus arteriosus. Failure to gain weight, despite well-managed respiratory and heart failure, was improved by infusion of subcutaneous insulin, administration of pancreatic enzyme, and nutrition of medium-chain-triglyceride -enriched formula. This is the first case of pancreatic agenesis with both malformations where the patient is discharged from the hospital. Early diagnosis and adequate treatments to compensate pancreatic function may prevent mortality and improve growth.

  15. Intraventricular Hemorrhage in Moderate to Severe Congenital Heart Disease.

    Science.gov (United States)

    Ortinau, Cynthia M; Anadkat, Jagruti S; Smyser, Christopher D; Eghtesady, Pirooz

    2018-01-01

    Determine the prevalence of intraventricular hemorrhage in infants with moderate to severe congenital heart disease, investigate the impact of gestational age, cardiac diagnosis, and cardiac intervention on intraventricular hemorrhage, and compare intraventricular hemorrhage rates in preterm infants with and without congenital heart disease. A single-center retrospective review. A tertiary care children's hospital. All infants admitted to St. Louis Children's Hospital from 2007 to 2012 with moderate to severe congenital heart disease requiring cardiac intervention in the first 90 days of life and all preterm infants without congenital heart disease or congenital anomalies/known genetic diagnoses admitted during the same time period. None. Cranial ultrasound data were reviewed for presence/severity of intraventricular hemorrhage. Head CT and brain MRI data were also reviewed in the congenital heart disease infants. Univariate analyses were undertaken to determine associations with intraventricular hemorrhage, and a final multivariate logistic regression model was performed. There were 339 infants with congenital heart disease who met inclusion criteria and 25.4% were born preterm. Intraventricular hemorrhage was identified on cranial ultrasound in 13.3% of infants, with the majority of intraventricular hemorrhage being low-grade (grade I/II). The incidence increased as gestational age decreased such that intraventricular hemorrhage was present in 8.7% of term infants, 19.2% of late preterm infants, 26.3% of moderately preterm infants, and 53.3% of very preterm infants. There was no difference in intraventricular hemorrhage rates between cardiac diagnoses. Additionally, the rate of intraventricular hemorrhage did not increase after cardiac intervention, with only three infants demonstrating new/worsening high-grade (grade III/IV) intraventricular hemorrhage after surgery. In a multivariate model, only gestational age at birth and African-American race were predictors

  16. Acquired heart conditions in adults with congenital heart disease: a growing problem.

    Science.gov (United States)

    Tutarel, Oktay

    2014-09-01

    The number of adults with congenital heart disease is increasing due to the great achievements in the field of paediatric cardiology, congenital heart surgery and intensive care medicine over the last decades. Mortality has shifted away from the infant and childhood period towards adulthood. As congenital heart disease patients get older, a high prevalence of cardiovascular risk factors is encountered similar to the general population. Consequently, the contribution of acquired morbidities, especially acquired heart conditions to patient outcome, is becoming increasingly important. Therefore, to continue the success story of the last decades in the treatment of congenital heart disease and to further improve the outcome of these patients, more attention has to be given to the prevention, detection and adequate therapy of acquired heart conditions. The aim of this review is to give an overview about acquired heart conditions that may be encountered in adults with congenital heart disease. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  17. Hemodynamics in CHD: mechanical regulation of congenital heart defects

    OpenAIRE

    Yalçın, Hüseyin Çağatay

    2011-01-01

    This is the periodic report for the HEMODYNAMICS IN CHD project, which received funding under the Seventh Framework Programme (FP7). The project will be applying the techniques developed in to a well established animal model for a severe CHD, hypoplastic left heart syndrome, to dissect the contribution of blood flow related forces on this disease. The report includes images generated to study embryonic development of congenital heart defects.

  18. Junctional ectopic tachycardia following repair of congenital heart ...

    African Journals Online (AJOL)

    Background: Postoperative junctional ectopic tachycardia (JET) is a rare and transient phenomenon occurring after repair of congenital heart defects. Report on this arrhythmia in the subregion is rare. We set out to determine the incidence of this arrhythmia and review the treatment and outcomes of treatment in our centre.

  19. Pattern of congenital heart defects in children with Down syndrome ...

    African Journals Online (AJOL)

    It is thus important to diagnose and intervene early to improve the quality of life. Few studies have been done on Down syndrome patients in Nigeria. Objective: The objective was to find out the incidence and types of congenital heart defects in Down syndrome patients seen in the Paediatric cardiology unit of this tertiary ...

  20. Epidemiology of adult congenital heart disease: demographic variations worldwide

    NARCIS (Netherlands)

    Mulder, B. J. M.

    2012-01-01

    The population of adults with a congenital heart defect (CHD) is increasing, due to improved survival after cardiac surgery. To accommodate the specialised care for these patients, a profound interest in the epidemiology of CHD is required. The exact size of the current population of adults with CHD

  1. Spectrum of congenital heart diseases in children with Down ...

    African Journals Online (AJOL)

    Objective: Congenital heart diseases (CHD) frequently occur in children with Down syndrome. A few studies in Nigeria have highlighted the pattern of CHD in such patients, but results are variable with no data from the study area for comparison. This study aims to determine the pattern of CHD among children with Down ...

  2. Genetics and inheritance issues in congenital heart disease

    NARCIS (Netherlands)

    van Engelen, K.

    2013-01-01

    Congenital heart disease (CHD) is among the most common birth defects, occurring in approximately 8 per 1,000 live births. It leads to significant morbidity and mortality in children as well as adults. Due to improvements in cardiac surgery and medical care, nowadays approximately 90% of CHD

  3. Changing demographics of pulmonary arterial hypertension in congenital heart disease

    NARCIS (Netherlands)

    Mulder, B. J. M.

    2010-01-01

    Pulmonary arterial hypertension (PAH) is a serious complication of congenital heart disease (CHD). Without early surgical repair, around one-third of paediatric CHD patients develop significant PAH. Recent data from the Netherlands suggest that >4% of adult CHD patients have PAH, with higher rates

  4. Underlying congenital heart disease in Nigerian children with ...

    African Journals Online (AJOL)

    Background: Pneumonia is a common cause of childhood morbidity and mortality globally. Some congenital heart disease(CHD) may predispose their sufferer to bronchopneumonia. Objective: To evaluate the contribution of CHD to pneumonia in children seen in a tertiary hospital. Methods: Over a year, consecutive ...

  5. Profile of congenital heart defects among children at Aminu Kano ...

    African Journals Online (AJOL)

    Background: The pattern of congenital heart diseases (CHD) varies from region to region even in the same country. Echocardiography has been available in Aminu Kano Teaching Hospital for about 10 years now but problems then were non.available of appropriate probes for children making it difficult to make some ...

  6. A systematic review of trends and patterns of congenital heart ...

    African Journals Online (AJOL)

    Method: We searched PubMed database, Google scholar, TRIP database, World Health Organisation libraries and reference lists of selected articles for studies on patterns of congenital heart disease among children in Nigeria between 1964 and 2015. Two researchers reviewed the papers independently and extracted the ...

  7. Chance of surgery in adult congenital heart disease

    NARCIS (Netherlands)

    Verheugt, Carianne L.; Uiterwaal, Cuno S. P. M.; Vaartjes, Ilonca; van der Velde, Enno T.; Zomer, A. C.; Meijboom, Folkert J.; Pieper, Petronella G.; Post, Marco C.; Vliegen, Hubert W.; Hazekamp, Mark G.; Grobbee, Diederick E.; Mulder, Barbara J. M.

    Background: Young patients with congenital heart disease reaching adulthood face mandatory transition to adult cardiology. Their new cardiologist needs to assess the chances of major future events such as surgery. Using a large national registry, we assessed if patient characteristics at the age of

  8. Chance of surgery in adult congenital heart disease

    NARCIS (Netherlands)

    Verheugt, Carianne L.; Uiterwaal, Cuno S.P.M.|info:eu-repo/dai/nl/136603947; Vaartjes, Ilonca|info:eu-repo/dai/nl/304812102; Van Der Velde, Enno T.; Zomer, A. C.|info:eu-repo/dai/nl/142296120; Meijboom, Folkert J.|info:eu-repo/dai/nl/136537367; Pieper, Petronella G; Post, Marco C.; Vliegen, Hubert W; Hazekamp, Mark G.; Grobbee, Diederick E.|info:eu-repo/dai/nl/071889256; Mulder, Barbara J M

    2017-01-01

    Background Young patients with congenital heart disease reaching adulthood face mandatory transition to adult cardiology. Their new cardiologist needs to assess the chances of major future events such as surgery. Using a large national registry, we assessed if patient characteristics at the age of

  9. Chance of surgery in adult congenital heart disease

    NARCIS (Netherlands)

    Verheugt, Carianne L.; Uiterwaal, Cuno Spm; Vaartjes, Ilonca; van der Velde, Enno T.; Zomer, A. C.; Meijboom, Folkert J.; Pieper, Petronella G.; Post, Marco C.; Vliegen, Hubert W.; Hazekamp, Mark G.; Grobbee, Diederick E.; Mulder, Barbara Jm

    2017-01-01

    Background: Young patients with congenital heart disease reaching adulthood face mandatory transition to adult cardiology. Their new cardiologist needs to assess the chances of major future events such as surgery. Using a large national registry, we assessed if patient characteristics at the age of

  10. Spectrum of congenital heart diseases in children with Down

    African Journals Online (AJOL)

    ABEOLUGBENGAS

    clinic and had echocardiography diagnosis of congenital heart diseases. Results: Of the 41 cases of DS seen during the study period, 32 (78.0%) children aged 2 weeks to 22 months (Mean +SD = 5.6 ± 4.0 months) were diagnosed with CHD. Male to female ratio was 2.2:1. Atrioventricular septal defect (AVSD) was the ...

  11. Occupational challenges of young adult patients with congenital heart disease

    NARCIS (Netherlands)

    Sluman, M. A.; de Man, S.; Mulder, B. J. M.; Sluiter, J. K.

    2014-01-01

    Background Despite improved survival of adults with congenital heart disease (CHD), higher rates of unemployment and work-related problems are seen, especially among younger adults. This study was performed to gain insight into current barriers and facilitating experiences at work among young adult

  12. Coping Behaviors of Parents with Children with Congenital Heart Disease.

    Science.gov (United States)

    Strobino, Jane

    The study addresses parental coping patterns of children with congenital heart disease in the state of Hawaii. Attention was given to geography and ethnicity as well as parental and child characteristics as factors impacting on the coping pattern. Telephone interviews with parents (N=32) obtained data concerning parent characteristics, their…

  13. Advances in cardiac magnetic resonance imaging of congenital heart disease

    NARCIS (Netherlands)

    Driessen, Mieke M P; Breur, Johannes M. P. J.; Budde, Ricardo P J; van Oorschot, Joep W M; van Kimmenade, Roland R J; Sieswerda, Gertjan Tj.; Meijboom, Folkert J; Leiner, Tim

    Due to advances in cardiac surgery, survival of patients with congenital heart disease has increased considerably during the past decades. Many of these patients require repeated cardiovascular magnetic resonance imaging to assess cardiac anatomy and function. In the past decade, technological

  14. Advances in cardiac magnetic resonance imaging of congenital heart disease

    NARCIS (Netherlands)

    Driessen, M.M.; Breur, J.M.; Budde, R.P.; Oorschot, J.W. van; Kimmenade, R.R. van; Sieswerda, G.T.; Meijboom, F.J.; Leiner, T.

    2015-01-01

    Due to advances in cardiac surgery, survival of patients with congenital heart disease has increased considerably during the past decades. Many of these patients require repeated cardiovascular magnetic resonance imaging to assess cardiac anatomy and function. In the past decade, technological

  15. Nutritional Approach of Pediatric Patients Diagnosed with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Togănel Rodica

    2013-04-01

    Full Text Available Congenital heart defects are among the most frequent anomalies present at birth, representing a heterogeneous group of malformations, both in terms of pathogenesis and clinical significance of the lesion. Failure to grow is well documented in infants with complex congenital heart defects; the presence of associated chromosomal abnormalities, cyanosis, and cardiac failure adds to the complexity and challenge. Malnutrition etiology can be grouped into the following three categories: inadequate intake, inefficient absorption and utilization, and/or increased energy needs. The consequences of malnutrition are both short and long term, timely nutritional intervention being necessary in order to maintain an adequate nutritional state. Because there are several types of congenital heart defects and multiple mechanisms by which they produce failure to thrive, no single strategy will be adequate to treat all cases. Medical complications such as chylotorax, necrotizing enterocolitis, laryngeal and neurological dysfunction play a major role in the requisite nutrition therapy in infants with congenital heart defect; limited access to human milk and parenteral concerns, as well as stress about feeding are also factors that can contribute to poor outcomes concerning nutrition and growth. Protocols are being considered and designed, and a systematic approach is always needed. The quality of life for patient and family, as well as getting the child back on track for age-appropriate development are always at the fore-front of each care plan.

  16. Cerebrovascular accidents in adult patients with congenital heart disease

    NARCIS (Netherlands)

    Hoffmann, A.; Chockalingam, P.; Balint, O. H.; Dadashev, A.; Dimopoulos, K.; Engel, R.; Schmid, M.; Schwerzmann, M.; Gatzoulis, M. A.; Mulder, B.; Oechslin, E.

    2010-01-01

    To investigate the prevalence and characteristics of cerebrovascular accidents (CVA) in a large population of adults with congenital heart disease (CHD). In a retrospective analysis of aggregated European and Canadian databases a total population of 23 153 patients with CHD was followed up to the

  17. Cerebrovascular accidents in adult patients with congenital heart disease

    NARCIS (Netherlands)

    Hoffmann, A.; Chockalingam, P.; Balint, O.H.; Dadashev, A.; Dimopoulos, K.; Engel, R.; Schmid, M.; Schwerzmann, M.; Gatzoulis, M.A.; Mulder, B.J.M.; Oechslin, E.

    2010-01-01

    Objective To investigate the prevalence and characteristics of cerebrovascular accidents (CVA) in a large population of adults with congenital heart disease (CHD). Methods and results In a retrospective analysis of aggregated European and Canadian databases a total population of 23 153 patients with

  18. Congenital malformation of the systemic heart of Sepia officinalis l ...

    African Journals Online (AJOL)

    Numerous preparations of the circulatory system of Sepia officinalis L. caught from the Bay of Arcachon (Atlantic Coast of France) in 1989 and 1996 showed an obvious congenital malformation of the systemic heart complex. The malformation consisted of a cord- or truncus-like structure at the left cranio-apical ventricle.

  19. Pregnancy in women with congenital heart disease : Complications and mechanisms

    NARCIS (Netherlands)

    Kampman, Marlies Aleida Maria

    2016-01-01

    In part I of this thesis we described the incidence of cardiovascular complications, new predictors of cardiovascular complications during and after pregnancy, and we assessed the impact of pregnancy on cardiac function and remodeling in women with congenital heart disease (CHD). We showed that

  20. Congenital Heart Diseases in Adults: A Review of Echocardiogram ...

    African Journals Online (AJOL)

    Background: Congenital abnormalities of the heart and cardiovascular system are reported in almost 1% of live births, and about half of these children need medical or surgical help in infancy. In the first decade, a further 25% require surgery to maintain or improve their life. Only 10% survive to adolescence without treatment ...

  1. Spectrum of congenital heart diseases in children with Down

    African Journals Online (AJOL)

    ABEOLUGBENGAS

    Abstract. Objective: Congenital heart diseases (CHD) frequently occur in children with Down syndrome. A few studies in Nigeria have highlighted the pattern of CHD in such patients, but results are variable with no data from the study area for comparison. This study aims to determine the pattern of CHD among children.

  2. Left ventricular non-compaction: prevalence in congenital heart disease.

    Science.gov (United States)

    Stähli, Barbara E; Gebhard, Catherine; Biaggi, Patric; Klaassen, Sabine; Valsangiacomo Buechel, Emanuela; Attenhofer Jost, Christine H; Jenni, Rolf; Tanner, Felix C; Greutmann, Matthias

    2013-09-10

    Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy, originally described as an isolated disease without other structural cardiac abnormalities. The aim of this study was to explore the prevalence of LVNC among adults with different types of congenital heart disease. From our databases we identified adults with congenital heart disease who fulfilled diagnostic criteria for LVNC. We report frequencies of associated congenital cardiac defects and the prevalence of LVNC among patients with different congenital heart defects. From a total of 202 patients with LVNC, 24 patients (12%; mean age 32 ± 11 years, 19 males) had additional congenital cardiac defects. Associated defects were left ventricular outflow tract abnormalities in 11 patients (46%), including 7 uni- or bicuspid aortic valves; two aortic coarctations; one diffuse aortic hypoplasia and one subaortic stenosis, Ebstein anomaly in 6 patients (25%), tetralogy of Fallot in two (8%), and double outlet right ventricle in one patient (4%). In our cohort, the prevalence of LVNC was highest among patients with Ebstein anomaly (6/40, 15%), followed by aortic coarctation (2/60, 3%), tetralogy of Fallot (3/129, 2%) and uni- or bicuspid aortic valves (7/963, 1%). In adults, various forms of congenital heart disease are associated with LVNC, particularly stenotic lesions of the left ventricular outflow tract, Ebstein anomaly, and tetralogy of Fallot. In the future, studying these patients in more depth may provide a better understanding of the interplay between genetic and hemodynamic factors that lead to the phenotype of LVNC. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  3. Readmissions after adult congenital heart surgery: Frequency and risk factors.

    Science.gov (United States)

    Kim, Yuli Y; He, Wei; MacGillivray, Thomas E; Benavidez, Oscar J

    2017-03-01

    Despite their clinical importance, 30-day readmission after adult congenital heart surgery has been understudied. They sought to determine the frequency of unplanned readmissions after adult congenital heart surgery and to identify any potential associated risk factors. Retrospective cohort study using State Inpatient Databases for Washington, New York, Florida, and California from 2009 to 2011. Federal and nonfederal acute care hospitals. Admissions of patients age 18-49 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating adult congenital heart surgery. Readmission was defined as any nonelective hospitalization for a given patient ≤30 days of discharge from the index congenital heart surgery admission. Of 9863 admissions, there were 8912 patients discharged home, of which 1419 were readmitted (14.2%). Unadjusted mortality rate was 2.6%. Most common indications for readmission were cardiac (pericardial disease, atrial fibrillation, heart failure) and infectious (postoperative infection, endocarditis). On multivariable analysis, female gender (adjusted odds ratio [AOR] 1.1; P = .05), black race (AOR 1.2; P = .05), median income Heart Surgery-1 (RACHS-1) 3 complexity (AOR 1.3; P = .04), and emergent admissions (AOR 1.5 P heart surgery hospitalizations results in unplanned readmission. Female gender, lower income status, black race, government-sponsored insurance, renal failure, unscheduled index admission, and RACHS-1 three surgical procedures are risk factors for subsequent unplanned 30-day readmission. These risk factors may serve as potential quality improvement targets to reduce readmissions. © 2016 Wiley Periodicals, Inc.

  4. Outcome after cardiopulmonary resuscitation in patients with congenital heart disease.

    Science.gov (United States)

    Van Puyvelde, Tim; Ameloot, Koen; Roggen, Mieke; Troost, Els; Gewillig, Marc; Budts, Werner; Van De Bruaene, Alexander

    2017-03-01

    Outcome after cardiopulmonary resuscitation (CPR) in patients with underlying congenital heart disease is uncertain. This study aimed at evaluating outcome after CPR in patients with underlying congenital heart disease, factors related to worse outcome after CPR and whether survivors of sudden cardiac death (SCD) have a worse outcome when compared to an age, gender and disease-matched control population. Between 1984 and 2015, all patients with congenital heart disease who received in or out-of-hospital CPR were identified from the database of congenital heart disease from the University Hospitals Leuven. Postoperative and neonatal (heart defect were included in the study. Thirty-eight patients (66% men; median age 25 years (interquartile range 9-40); 68% out-of-hospital) were identified, of which 27 (66%) survived the event. The main cause of SCD was ventricular tachycardia or fibrillation ( n=21). Heart defect complexity (odds ratio (OR) 5.1; 95% confidence interval (CI) 1.2-21.9; P=0.027), pulmonary hypertension (OR 13.8; 95% CI 2.1-89.5; P=0.006) and time to return of spontaneous circulation (OR 1.1; 95% CI 1.0-1.1; P=0.046) were related to worse outcome. Survivors of SCD had a worse prognosis when compared to an age, gender and disease-matched control group (5-year survival 76% vs. 98%; P=0.002). The complexity of underlying heart defect, pulmonary hypertension and time to return of spontaneous circulation are related to worse outcome in the case of CPR. Survivors of SCD have a worse outcome when compared to matched controls, indicating the need for adequate implantable cardioverter defibrillator indication assessment and for stringent follow-up of patients with worsening haemodynamics.

  5. Complete congenital sternal cleft in an adult: repair by autogenous tissues.

    Science.gov (United States)

    Suri, R K; Sharma, R K; Jha, N K; Sharma, B K

    1996-08-01

    We are reporting here a rare case of congenital complete sternal cleft in an adult of 25 years of age, with absent anterior pericardium and "diastasis recti." Successful surgical repair was achieved along with the preservation of the already existing anatomic and mechanical sanctity of thoracic cage, sternal reconstruction, and protection of the underlying heart and great vessels with a good cosmetic result using autogenous tissue i.e., iliac bone and "V-Y" myoplasty of pectoralis major muscles with their functional preservation and correction of associated anomalies.

  6. False Heart Rate Feedback and the Perception of Heart Symptoms in Patients with Congenital Heart Disease and Anxiety

    NARCIS (Netherlands)

    Karsdorp, Petra A.; Kindt, Merel; Rietveld, Simon; Everaerd, Walter; Mulder, Barbara J. M.

    2009-01-01

    Background Little is known about the mechanisms explaining an increased perception of heart symptoms in congenital heart disease (ConHD). In the present study, it was suggested that a combination of high trait anxiety and disease history increases the perception of heart symptoms. Purpose It was

  7. False heart rate feedback and the perception of heart symptoms in patients with congenital heart disease and anxiety

    NARCIS (Netherlands)

    Karsdorp, P.A.; Kindt, M.; Rietveld, S.; Everaerd, W.; Mulder, B.J.M.

    2009-01-01

    Background: Little is known about the mechanisms explaining an increased perception of heart symptoms in congenital heart disease (ConHD). In the present study, it was suggested that a combination of high trait anxiety and disease history increases the perception of heart symptoms. Purpose: It was

  8. Living with a Congenital Heart Defect

    Science.gov (United States)

    ... Asha's Story Shandler's story Nicholas' Story Ken's Story William's Story William’s Story I was born with a heart defect, ... we moved to Scotland where I began a Master’s program. Thirteen years after the open-heart surgery, ...

  9. [Pediatric cardiology and congenital heart disease: from fetus to adult].

    Science.gov (United States)

    Subirana, M Teresa; Oliver, José M; Sáez, José M; Zunzunegui, José L

    2012-01-01

    This article contains a review of some of the most important publications on congenital heart disease and pediatric cardiology that appeared in 2010 and up until September 2011. Of particular interest were studies on demographic changes reported in this patient population and on the need to manage the patients' transition from the pediatric to the adult cardiology department. This transition has given rise to the appearance of new areas of interest: for example, pregnancy in women with congenital heart disease, and the effect of genetic factors on the etiology and transmission of particular anomalies. In addition, this review considers some publications on fetal cardiology from the perspective of early diagnosis and, if possible, treatment. There follows a discussion on new contributions to Eisenmenger's syndrome and arrhythmias, as well as on imaging techniques, interventional catheterization and heart transplantation. Finally, there is an overview of the new version of clinical practice guidelines on the management of adult patients with congenital heart disease and of recently published guidelines on pregnancy in women with heart disease, both produced by the European Society of Cardiology. Copyright © 2012 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

  10. Understanding congenital heart defects through abdominal fetal electrocardiography: case reports and clinical implications.

    Science.gov (United States)

    Velayo, Clarissa; Sato, Naoaki; Ito, Takuya; Chisaka, Hiroshi; Yaegashi, Nobuo; Okamura, Kunihiro; Kimura, Yoshitaka

    2011-05-01

    Congenital heart defects are the most common fetal structural anomalies of which a significant number remain unrecognized during postnatal life. Fetal electrocardiography (FECG) is an ideal clinical tool to complement ultrasonography for the screening and management of these cases where early and accurate diagnoses would allow definite rather than palliative treatment. The objective of this report was to correlate the particular FECG results found with the different types of congenital heart defects involved and to further demonstrate the usefulness of FECG in clinical settings. This is a report of four cases of prenatally diagnosed congenital heart defects seen at a university hospital in Sendai, Japan. Their complete and thorough evaluation included, among other tests, abdominal FECG analysis. The presence of premature ventricular contractions, a prolonged pre-ejection period (PEP > 75 msec), and prolonged QTc intervals (QTc > 440 msec) served as markers of hemodynamic alteration but were unlikely determinants of disease severity precluding further investigation. In practice, similar findings found on FECG should raise the index of suspicion for the presence of congenital heart disease and prompt a targeted ultrasound scan. © 2011 The Authors. Journal of Obstetrics and Gynaecology Research © 2011 Japan Society of Obstetrics and Gynecology.

  11. [Transcatheter treatment of combined congenital heart anomalies in an adult cyanotic patient].

    Science.gov (United States)

    Yalonetsky, Sergey; Khoury, Asaad; Schwartz, Yitzhack; Lorber, Avraham

    2006-09-01

    Contemporary catheterization techniques allow effective percutaneous transcatheter treatment of congenital heart defects as an attractive alternative to open heart surgery. This case report describes transcatheter treatment of congenital pulmonary valve stenosis and secundum atrial septal defect which were diagnosed in a 31 year old woman who presented with cyanosis. This illustrates difficulties in the diagnosis of congenital heart disease in adult patients and demonstrates that transcatheter techniques in the treatment of these congenital anomalies are effective.

  12. Hyperkalemia-induced complete heart block

    Directory of Open Access Journals (Sweden)

    Alireza Baratloo

    2015-05-01

    Full Text Available Background: Potassium, as an extracellular ion, plays an important role in the electrophysiologic function of the myocardium and any change in extracellular concentration of this ion might have a marked impression upon myocyte electrophysiologic gain. High serum potassium levels are thought to impair pulse conduction in Purkinje fibers and ventricles more than that in the Atrioventricular (AV node. Therefore, although complete AV block can occur, it is a rare initial presentation. Case Report: We describe a 62-year-old man with a history of diabetes mellitus, ischemic heart disease and previous Coronary Artery Bypass Graft (CABG, who came to our emergency department due to generalized weakness starting 2 days before admission. The patient also had decreased force in lower limbs, exacerbating from the morning, and was finally diagnosed as a hyperkalemia-induced Complete Heart Block (CHB. It should also be noted that the patient responded dramatically to the administration of 10 mL of 10% calcium gluconate along with external pacing until potassium level correction became effective. Conclusion: In spite of the fact that Hyperkalemia can be associated with frequent Electrocardiogram (ECG abnormality, advanced heart blocks (second- and third-degree AV blocks are usually found only in patients with pre-existing heart failure, conduction abnormalities, or other cardiac diseases. Institution of effective treatment rapidly and forgiveness of traditional non-effective, time consumptive and sometimes risking full-adjustment modalities, such as sodium bicarbonate infusion or exchange resins that prevent their use in the emergent phase, can help minimize patient morbidity and mortality.

  13. Five Facts about Congenital Heart Defects

    Science.gov (United States)

    ... involved, but of everyone? In the United States, hospital costs for people with a heart defect were about $1.4 billion in one year. Families and the government share the burden of these costs, which means that all taxpayers ...

  14. Stress-induced heart symptoms and perceptual biases in patients with congenital heart disease

    NARCIS (Netherlands)

    Karsdorp, Petra A.; Kindt, Merel; Rietveld, Simon; Everaerd, Walter; Mulder, Barbara J. M.

    2007-01-01

    BACKGROUND: The aim of the present study is to clarify whether biased symptom perception towards heart symptoms may explain a reduced quality of life in patients with congenital heart disease (ConHD). The present study tested the hypothesis that the combination of ConHD and high trait anxiety

  15. Prenatal chromosomal microarray analysis in fetuses with congenital heart disease: a prospective cohort study.

    Science.gov (United States)

    Wang, Yan; Cao, Li; Liang, Dong; Meng, Lulu; Wu, Yun; Qiao, Fengchang; Ji, Xiuqing; Luo, Chunyu; Zhang, Jingjing; Xu, Tianhui; Yu, Bin; Wang, Leilei; Wang, Ting; Pan, Qiong; Ma, Dingyuan; Hu, Ping; Xu, Zhengfeng

    2018-02-01

    Currently, chromosomal microarray analysis is considered the first-tier test in pediatric care and prenatal diagnosis. However, the diagnostic yield of chromosomal microarray analysis for prenatal diagnosis of congenital heart disease has not been evaluated based on a large cohort. Our aim was to evaluate the clinical utility of chromosomal microarray as the first-tier test for chromosomal abnormalities in fetuses with congenital heart disease. In this prospective study, 602 prenatal cases of congenital heart disease were investigated using single nucleotide polymorphism array over a 5-year period. Overall, pathogenic chromosomal abnormalities were identified in 125 (20.8%) of 602 prenatal cases of congenital heart disease, with 52.0% of them being numerical chromosomal abnormalities. The detection rates of likely pathogenic copy number variations and variants of uncertain significance were 1.3% and 6.0%, respectively. The detection rate of pathogenic chromosomal abnormalities in congenital heart disease plus additional structural anomalies (48.9% vs 14.3%, P heart disease group. Additionally, the detection rate in congenital heart disease with additional structural anomalies group was significantly higher than that in congenital heart disease with soft markers group (48.9% vs 19.8%, P heart disease with additional structural anomalies and congenital heart disease with intrauterine growth retardation groups (48.9% vs 50.0%), congenital heart disease with soft markers and congenital heart disease with intrauterine growth retardation groups (19.8% vs 50.0%), or congenital heart disease with soft markers and isolated congenital heart disease groups (19.8% vs 14.3%). The detection rate in fetuses with congenital heart disease plus mild ventriculomegaly was significantly higher than in those with other types of soft markers (50.0% vs 15.6%, P heart disease in clinical practice. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Hematologic Abnormalities in Cyanotic Congenital Heart Disease Patients

    Directory of Open Access Journals (Sweden)

    Soheila Chamanian

    2015-01-01

    Full Text Available Introduction: Patients with cyanotic heart disease may have an acceptable quality of life. However, they are invariably prone to several complications. The aim of this study is search about hematologic abnormalities in cyanotic congenital heart disease patients. Materials and Methods:  In this cross sectional study every cyanotic congenital heart disease patients who was referred to the adult congenital heart disease clinic was selected and asked of any possible hyperviscosity symptoms, gingival bleeding, Epistaxis, hemoptysis, hypermenorrhagia and gouty arthritis irrespective of their age, gender and primary diagnosis in a six-month period. In this regard, 02 saturation was obtained via pulse oximetry, an abdominal ultrasound was done in order to discover any gallstones and lab tests including CBC, coagulation parameters (bleeding time(BT,clotting time(CT, prothrombin time(PT,international ratio( INR, Ferritin, blood urea nitrogen (BUN and creatinine (Cr were provided as well. Results:  A total of 69 patients were enrolled in the present study. The mean age of the patients was 22.44±5.72 with a minimum of 15 and the maximum of 46 years old. Twenty two (34.4% of them were female and 45(65.6% were male. Conclusion: Our patients had less hyperuricemia, there is no correlation between hyperviscosity symptoms and haematocrit level and an inverse correlation between the Ferritin level and hyperviscosity symptoms were seen.  

  17. Improving pacemaker therapy in congenital heart disease: contractility and resynchronization.

    Science.gov (United States)

    Karpawich, Peter P

    2015-01-01

    Designed as effective therapy for patients with symptomatic bradycardia, implantable cardiac pacemakers initially served to improve symptoms and survival. With initial applications to the elderly and those with severe myocardial disease, extended longevity was not a major concern. However, with design technology advances in leads and generators since the 1980s, pacemaker therapy is now readily applicable to all age patients, including children with congenital heart defects. As a result, emphasis and clinical interests have advanced beyond simply quantity to quality of life. Adverse cardiac effects of pacing from right ventricular apical or epicardial sites with resultant left bundle branch QRS configurations have been recognized. As a result, and with the introduction of newer catheter-delivered pacing leads, more recent studies have focused on alternative or select pacing sites such as septal, outflow tract, and para-bundle of His. This is especially important in dealing with pacemaker therapy among younger patients and those with congenital heart disease, with expected decades of artificial cardiac stimulation, in which adverse myocellular changes secondary to pacing itself have been reported. As a correlate to these alternate or select pacing sites, applications of left ventricular pacing, either via the coronary sinus, intraseptal or epicardial, alone or in combination with right ventricular pacing, have gained interest for patients with heart failure. Although cardiac resynchronization pacing has, to date, had limited clinical applications among patients with congenital heart disease, the few published reports do indicate potential benefits as a bridge to cardiac transplant. Copyright © 2015 Elsevier Inc. All rights reserved.

  18. [Plasma aminogram in infants operated on complex congenital heart disease].

    Science.gov (United States)

    Villares, J M Moreno; Leal, L Oliveros; Díaz, I Sánchez; González, P Gómez

    2008-01-01

    Post-surgical morbidity of congenital heart disease operated at early ages still is high. Both pre-surgical malnourishment and the repercussions of the systemic response to the aggression, including extracorporeal circulation contribute to it. The metabolism of proteins has been little studied in these infants and toddlers, as well as its repercussion on clinical outcomes. The aim of this study was to assess the effect of the surgery on the plasma aminogram of infants early operated for complex congenital heart disease. We prospectively gathered the anthropometrical and analytical data of 55 children heart surgery at the day of intervention and at days +1 (n = 53), +3 (n = 39), and +7 (n = 19). The data are presented as mean and standard deviation. The comparison between the variables through time was done by one-tailed analysis of variance for repeated samples. It was considered to be statistically significant with a p value congenital heart disease was within the normal range before the surgery, irrespective of the nutritional status. 2. After the surgery, a significant decrease is observed for plasma levels of most of the amino acids, with a trend towards normalization, which is slower for certain branched amino acids, particularly for glutamine. 3. The clinical significance of these findings deserves further studies.

  19. Somatic growth following congenital heart surgery in economically underprivileged children.

    Science.gov (United States)

    Kasar, Pankajkumar A; Suresh Kumar, Raghavan Nair; Raman Kutty, Vellappillil

    2011-01-01

    To assess the impact of congenital heart surgery on anthropometric scores of growth in economically disadvantaged children. A cohort of 100 economically disadvantaged children was followed up after cardiac surgery for their nutritional recovery. Weight, height and body mass index for age were measured just before surgery and at a median period of 48.1 months (range 9-59.9 months) after surgery. Z scores of the age-adjusted variables were computed and McNemar OR was calculated for odds of improvement. The mean weight for age of the cohort increased from 14.74±5.76 to 23.83±7.83 kg. In malnourished children (weight for age Z score ≤-2) the mean weight changed from -3.01 to -1.6 (pcongenital heart disease or associated syndromes. Congenital heart surgery resulted in a salutary improvement in the growth of children from economically underprivileged backgrounds. Residual malnutrition was likely to be associated with extreme economic backwardness, surgery for cyanotic congenital heart disease or coincidental syndromes.

  20. Behaviour Problems in Children with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Beena Johnson

    2015-01-01

    Full Text Available As more children survive with congenital heart diseases, management of their behavioural problems are becoming increasingly important. In this article we aim to review the current status of knowledge on this aspect. Children with congenital heart diseases have more behavioural problems compared to children without chronic illnesses. Behavioural problems in children can be classified into externalizing behaviours and internalizing behaviours. Externalizing behaviours are marked by defiance, impulsivity, hyperactivity, disruptiveness, aggression and antisocial features. Internalizing behaviours are evidenced by withdrawal, dysphoria and anxiety. Boys with congenital heart diseases have more externalizing problems compared to girls. Preoperative hypoxia as well as peri and postoperative cardiocirculatory insufficiency can lead to internalizing and externalizing behavioral problems in future. High treatment intensity and palliative interventions are associated with poor behavioral outcomes. Children who underwent open heart surgery at very young age are prone to develop attention-deficit hyperactivity disorder on reaching school age. A comprehensive approach in this field is essential, so that effective early interventions and guidance can be planned.

  1. Experiences Of Mothers Of Children With Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Joana Angélica Marques Pinheiro

    2017-04-01

    Full Text Available Introduction: This article discusses the experience of mothers during the process of falling ill of their children with congenital heart disease. Method: This is a descriptive research with qualitative approach conducted with nine mothers who had their babies admitted to a pediatric cardiology unit in a reference center for treatment of congenital heart disease in the North and Northeast regions in Brazil located in Fortaleza, Ceará. Information and reports on the experience of these mothers emerging from the diagnosis process, surgery, treatment and hospitalization were collected in semi-structured individual interviews. After interviews, the testimonies of mothers were transcribed in full-length, identifying the speeches with the codes M1, M2,...M9, thereby ensuring anonymity. We used the thematic analysis technique of Minayo (2012 for treatment of information and design of the content of this article. This article is part of the research project: Breastfeeding babies with heart disease, approved by the Research Ethics Committee of the Messejana Hospital, under Opinion No 1,285,784. Results: The study demonstrated that infants with congenital heart disease require specific and specialized care and that mothers undertake a long and sometimes painful journey in the search for the healing of their child. Conclusion: Based on the collected speeches, we learned that mothers also need to be assisted by professionals of the health team. They must support the mother's empowerment in the child care and strengthen the relationship of the mother-infant binomial, and help in coping and adapting to the reality in the process of falling ill and healing of their child.   Key words: Congenital heart disease. Experience of mothers

  2. Overweight predicts poorer exercise capacity in congenital heart disease patients.

    Science.gov (United States)

    Kuehl, Karen; Tucker, Alicia; Khan, Munziba; Goldberg, Paula; Anne Greene, E; Smith, Megan

    2015-12-07

    Overweight (OW) and obesity (OB) are endemic in the United States and affect adolescents and adults with congenital heart disease (ACHD). Defining the burden of excess weight on the cardiovascular system in ACHD is the goal of this study. Limitation of exercise capacity due to overweight or obesity might be reversible with weight loss and improve quality of life for ACHD adults. Exercise tests performed using a Bruce protocol and measurement of maximum oxygen consumption were retrospectively reviewed on 418 CHD patients. OW and OB were defined as the 85-95 or > 95 percentile respectively for age and gender or by adult criteria. Severity of CHD was assigned based on criteria published in standard guidelines. 63 patients had mild, 198 moderate, and 157 severe heart disease. Each ACHD group was 32 to 34% OW or OB. Measured exercise time (ET) of CHD patients with moderate or severe heart disease was less than that of controls in each weight categories. However, OB or OW people have shorter ET than their normal weight peers with CHD. Multiple regression using ET as the dependent variable finds that female sex, relative BMI, and VE/VCO2 at peak exercise are all associated with lesser ET with high significance. Peak heart rate is associated with greater ET, with borderline significance. Severity of heart disease is not independently associated with ET. OW and OB are strongly associated with reduced ET in persons with congenital heart disease. Losing weight may improve exercise capacity in ACHD.

  3. Determination of the prevalence of congenital heart disease in the patients admitted to the heart clinic

    OpenAIRE

    Shokoufeh Ahmadipour; Behzad Mohammadpour Ahranjani; Sara Daeichin; Zahra Mirbeig Sabzevari

    2015-01-01

    Objective: To investigate the prevalence of congenital heart disease (CHD) among the patients who refferred to the heart clinic so as to make an early and correct diagnosis. Methods: In this descriptive-cross sectional study, all the patients admitted to the heart clinic who had symptoms or signs of CHD were included. The data were collected in one year based on the medical records. The main variables consisted of age, gender, history of folic acid consumption by the mother in ...

  4. Down-Klinefelter syndrome (48,XXY,+21) in a neonate associated with congenital heart disease.

    Science.gov (United States)

    Rodrigues, M A; Morgade, L F; Dias, L F A; Moreira, R V; Maia, P D; Sales, A F H; Ribeiro, P D

    2017-09-27

    Double aneuploidy is considered a rare phenomenon. Herein, we describe a case of double aneuploidy 48,XXY,+21 in a neonate with congenital heart defects. The 28-day-old neonate male (23-year-old mother and 24-year-old father) was admitted to a neonatal intensive care unit owing to congenital heart disease. Echocardiography showed a complete atrioventricular septal defect with Rastelli type B and significant left ventricular failure, moderate atrioventricular valve regurgitation, right-sided heart failure, and preserved systolic function. Cytogenetic analysis of the newborn showed double aneuploidy 48,XXY,+21. The maternal karyotype was 46,XX,inv(9)(p11q13) and the paternal was 46,XY. Characteristics associated with Down syndrome are observed in newborns; on the other hand, children under 10 months of age and neonates may show little or no signs of the Klinefelter syndrome. According to this study, there seem to be differences between the frequency of congenital heart disease among patients with Down-Klinefelter and Down syndrome. At about 11 months of age, the child died after undergoing heart surgeries. The early cytogenetic study is important for better diagnosis and management of the disease.

  5. Cardiac CT angiography in children with congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Siripornpitak, Suvipaporn, E-mail: ssiripornpitak@yahoo.com [Division of Diagnostic Radiology, Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok (Thailand); Pornkul, Ratanaporn [Division of Diagnostic Radiology, Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok (Thailand); Khowsathit, Pongsak [Pediatric Cardiac Unit, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok (Thailand); Layangool, Thanarat; Promphan, Worakan [Pediatric Cardiology Unit, Queen Sirikit National Institute of Child Health, Bangkok (Thailand); Pongpanich, Boonchob [Pediatric Cardiac Unit, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok (Thailand)

    2013-07-15

    Cardiac imaging plays an important role in both congenital and acquired heart diseases. Cardiac computed tomography (angiography) cCT(A) is a non-invasive, increasingly popular, complementary modality to echocardiography in evaluation of congenital heart diseases (CHD) in children. Despite radiation exposure, cCT(A) is now commonly used for evaluation of the complex CHD, giving information of both intra-cardiac and extra-cardiac anatomy, coronary arteries, and vascular structures. This review article will focus on the fundamentals and essentials for performing cCT(A) in children, including radiation dose awareness, basic techniques, and strengths and weaknesses of cCT(A) compared with cardiac magnetic resonance imaging (cMRI), and applications. The limitations of this modality will also be discussed, including the CHD for which cMRI may be substituted.

  6. Assessing the influence of consanguinity on congenital heart disease

    Directory of Open Access Journals (Sweden)

    Alan H Bittles

    2011-01-01

    Full Text Available Numerous articles have been published linking consanguineous marriage to an elevated prevalence of congenital heart disease, with ventricular septal defects and atrial septal defects the most commonly cited disorders. While initially persuasive, on closer examination many of these studies have fundamental shortcomings in their design and in the recruitment of study subjects and controls. Improved matching of cases and controls, to include recognition of the long-established community boundaries within which most marriages are contracted, and the assessment of consanguinity within specific levels and types of marital union would improve and help to focus the study outcomes. At the same time, major discrepancies between studies in their reported prevalence and types of congenital heart disease suggest an urgent need for greater standardization in the classification and reporting of these disorders.

  7. Nutritional status of children with congenital heart disease.

    Science.gov (United States)

    Monteiro, Flávia Paula Magalhães; de Araujo, Thelma Leite; Lopes, Marcos Venícios de Oliveira; Chaves, Daniel Bruno Resende; Beltrão, Beatriz Amorim; Costa, Alice Gabrielle de Sousa

    2012-01-01

    to characterize nutritional status and variables that predict nutritional changes in children with congenital heart disease. a cross-sectional study undertaken in two health institutions between January and June 2009, using a questionnaire with questions about nutrition, applied to 132 children under two years of age who had congenital heart disease. Children who had additional serious illnesses were excluded. the predominant percentile values and Z scores were concentrated within the range of normal levels. The Z scores, however, presented negative variations with a deviation to the left. In the analysis of predictive factors, the occurrence of immediate and acute malnutrition was related to a decrease in skinfold thickness (decrease in subscapular skinfold thickness, while immediate malnutrition was related to a high Apgar score. Chronic malnutrition was related to female children with higher ages. it is evidenced that it is necessary to carry out nutritional strategies which improve prognosis, so as to widen the nursing care directed at these children.

  8. Histone methylations in heart development, congenital and adult heart diseases

    OpenAIRE

    Zhang, Qing-Jun; Liu, Zhi-Ping

    2015-01-01

    Heart development comprises myocyte specification, differentiation and cardiac morphogenesis. These processes are regulated by a group of core cardiac transcription factors in a coordinated temporal and spatial manner. Histone methylation is an emerging epigenetic mechanism for regulating gene transcription. Interplay among cardiac transcription factors and histone lysine modifiers plays important role in heart development. Aberrant expression and mutation of the histone lysine modifiers duri...

  9. Nutritional Management of Children with Congenital Heart Disease

    OpenAIRE

    Wheat, Jeffrey C.

    2002-01-01

    Children and infants with congenital heart disease (CHD) often have disturbances in growth and development. Recent research suggests that this is due mainly to inadequate caloric intake and increased energy expenditure as compared to normal children (1). This presents a significant problem because if left untreated these children can develop permanent disabilities and poorer outcomes after corrective surgery. In order to treat this problem, a systematic approach must be used to identify the s...

  10. Assessing the influence of consanguinity on congenital heart disease

    OpenAIRE

    Alan H Bittles

    2011-01-01

    Numerous articles have been published linking consanguineous marriage to an elevated prevalence of congenital heart disease, with ventricular septal defects and atrial septal defects the most commonly cited disorders. While initially persuasive, on closer examination many of these studies have fundamental shortcomings in their design and in the recruitment of study subjects and controls. Improved matching of cases and controls, to include recognition of the long-established community boundari...

  11. DNA content in children with congenital heart disease

    OpenAIRE

    Hegazi, Mohamed Ali; Shalaby, Rokaya Hussen

    2014-01-01

    CHD represents the most important component of pediatric cardiovascular diseases. It has a high risk of morbidity and mortality in newborns and infants. were considered as multifactorial diseases. However genetic factors were considered as a corner stone of their etiology. The DNA content represents the nuclear genomic concentration. It is affected by several multifactors. Objective: The aim of our study was to determine the DNA content in particular common congenital heart disease. To det...

  12. Consanguinity and the Risk of Congenital Heart Disease

    OpenAIRE

    Shieh, Joseph T. C.; Bittles, Alan H.; Hudgins, Louanne

    2012-01-01

    Consanguineous unions have been associated with an increased susceptibility to various forms of inherited disease. Although consanguinity is known to contribute to recessive diseases, the potential role of consanguinity in certain common birth defects is less clear, particularly since the disease pathophysiology may involve genetic and environmental/epigenetic factors. In this study we ask whether consanguinity affects one of the most common birth defects, congenital heart disease, and identi...

  13. A review of the economics of adult congenital heart disease.

    Science.gov (United States)

    Seckeler, Michael D; Thomas, Ian D; Andrews, Jennifer; Joiner, Keith; Klewer, Scott E

    2016-01-01

    Adults living with congenital heart disease (CHD) now outnumber children with the disease. Thanks to medical advances over the past 75 years, many of these fatal childhood heart problems have changed to chronic medical conditions. As the population of adults with CHD increases, they will require increasingly complex medical, surgical and catheter-based therapies. In addition, social burdens including education, employment and insurability, which increase the societal costs of adult CHD, are now being recognized for adults living with CHD. This review summarizes the available literature on the economics of adult CHD.

  14. Prevalence of Dyslipidemia in Children with Congenital Heart Disease

    Energy Technology Data Exchange (ETDEWEB)

    Fuenmayor, Gabriela; Redondo, Ana Carolina Costa; Shiraishi, Karen Saori [Hospital do Coração - Associação do Sanatório Sírio, São Paulo, SP (Brazil); Souza, Rogerio [Hospital do Coração - Associação do Sanatório Sírio, São Paulo, SP (Brazil); Instituto do Coração, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP (Brazil); Elias, Patrícia Figueiredo; Jatene, Ieda Biscegli, E-mail: ijatene@hcor.com.br [Hospital do Coração - Associação do Sanatório Sírio, São Paulo, SP (Brazil)

    2013-09-15

    Dyslipidemia is one of the main risk factors associated with cardiovascular diseases. Few data on the impacts of congenital heart diseases are available with regard to the prevalence of dyslipidemia in children. Our study evaluated the lipid profile in children with congenital heart disease at a referral center. From January 2011 to July 2012, 52 pediatric patients had their lipid, metabolic and clinical profiles traced. The mean age was 10.4 ± 2.8 years and male/female rate of 1.38:1. Our population had 53.8% patients with high levels of total cholesterol and 13.4% (CI 95 %, from 6.6 to 25.2%) of them also presenting LDL levels ≥ 130 mg/dL, which characterizes dyslipidemia. The group of dyslipidemic patients presented only two obese individuals. Our data show that the presence of congenital heart disease does not lead to higher risk associated with the prevalence of dyslipidemia. Therefore, the screening of this specific population should follow the regular pediatric guidelines, which are also independent of the nutritional status of the children tested.

  15. Complex congenital heart disease in a complicated and precious pregnancy.

    Science.gov (United States)

    Elsherif, Zeinelabdien; Mahmood, Naveed; Jamil, Shazia; Wagas, Hadeel

    2015-07-01

    A single ventricle is a rare congenital heart disease that accounts for less than 1% of all congenital heart diseases. A woman was assessed in our obstetric clinic for the first time at the gestational age of 28 weeks and found to have placental bleeding. She also had complex congenital heart disease and atrial fibrillation requiring anticoagulation. Echocardiography revealed double-inlet single ventricle with right and left atrioventricular valves entering into this chamber and levo-transposition of the great arteries. After an extensive discussion with the patient regarding the risks and benefits of anticoagulation including risk of stroke, the agreed plan was to start her on intravenous heparin with close observation and to continue pregnancy for at least 32 weeks in order to reduce the postpartum risk for the fetus. The pregnancy progressed without any further complications and the patient had elective caesarean section at 33 weeks of gestation and delivered a healthy baby boy. 2015 BMJ Publishing Group Ltd.

  16. Gluten Sensitivity among Egyptian Infants with Congenital Heart Disease.

    Science.gov (United States)

    El-Alameey, Inas R; Ahmed, Hanaa H; Tawfik, Sawsan M; Hassaballa, Fawzia; Gawad, Ayman M Abdel; Eltahlawy, Eman

    2017-03-15

    Gastrointestinal symptoms are a common feature in infants with congenital heart disease. This study was designed to evaluate age-dependent serum levels of antigliadin antibodies among malnourished Egyptian infants with congenital heart disease (CHD) and gastrointestinal symptoms. This case-control study conducted on 60 infants with established congenital heart disease. They were subdivided into cyanotic and acyanotic groups, and each group includes 30 patients compared with thirty apparently healthy infants of matched age, sex, and social class. Serum antigliadin antibodies levels were measured using ELISA. The mean age of introduction of cereals in the diet and appearance of gastrointestinal symptoms were six months. On comparison with controls, patients showed highly significant higher serum levels of antigliadin antibodies (P < 0.000). On analysing risk factors using odds ratio, the age at onset of GIT symptoms, diarrhoea, abdominal pain, and distension had been found to be significantly associated with high serum antigliadin antibodies among malnourished CHD infants with a prediction of 95%. Serum IgA, IgM, and IgG class antibodies to gliadin play a significant role in the pathogenesis of malnutrition in infants with CHD. Gluten containing foods should never be introduced before the end of the six months.

  17. Prevalence of dyslipidemia in children with congenital heart disease.

    Science.gov (United States)

    Fuenmayor, Gabriela; Redondo, Ana Carolina Costa; Shiraishi, Karen Saori; Souza, Rogerio; Elias, Patrícia Figueiredo; Jatene, Ieda Biscegli

    2013-09-01

    Dyslipidemia is one of the main risk factors associated with cardiovascular diseases. Few data on the impacts of congenital heart diseases are available with regard to the prevalence of dyslipidemia in children. Our study evaluated the lipid profile in children with congenital heart disease at a referral center. From January 2011 to July 2012, 52 pediatric patients had their lipid, metabolic and clinical profiles traced. The mean age was 10.4 ± 2.8 years and male/female rate of 1.38:1. Our population had 53.8% patients with high levels of total cholesterol and 13.4% (CI 95 %, from 6.6 to 25.2%) of them also presenting LDL levels ≥ 130 mg/dL, which characterizes dyslipidemia. The group of dyslipidemic patients presented only two obese individuals. Our data show that the presence of congenital heart disease does not lead to higher risk associated with the prevalence of dyslipidemia. Therefore, the screening of this specific population should follow the regular pediatric guidelines, which are also independent of the nutritional status of the children tested.

  18. The Society of Thoracic Surgeons Congenital Heart Surgery Database Mortality Risk Model: Part 1-Statistical Methodology

    National Research Council Canada - National Science Library

    O'Brien, Sean M; Jacobs, Jeffrey P; Pasquali, Sara K; Gaynor, J William; Karamlou, Tara; Welke, Karl F; Filardo, Giovanni; Han, Jane M; Kim, Sunghee; Shahian, David M; Jacobs, Marshall L

    2015-01-01

    ... after congenital cardiac operations. Included were patients of all ages undergoing cardiac operations, with or without cardiopulmonary bypass, at centers participating in The Society of Thoracic Surgeons Congenital Heart Surgery Data...

  19. Prenatal detection of congenital heart disease - Results of a national screening programme

    NARCIS (Netherlands)

    Van Velzen, C. L.; Clur, S. A.; Rijlaarsdam, M. E B; Bax, C. J.; Pajkrt, E.; Heymans, M. W.; Bekker, M. N.; Hruda, J.; De Groot, C. J M; Blom, N. A.; Haak, M. C.

    2016-01-01

    Objective Congenital heart disease (CHD) is the most common congenital malformation and causes major morbidity and mortality. Prenatal detection improves the neonatal condition before surgery, resulting in less morbidity and mortality. In the Netherlands a national prenatal screening programme was

  20. Malnutrition in hospitalized children with congenital heart disease.

    Science.gov (United States)

    Cameron, J W; Rosenthal, A; Olson, A D

    1995-10-01

    To determine the prevalence of malnutrition among hospitalized children with congenital heart disease by age, disease process, and clinical status. Cross-sectional, retrospective chart review. Pediatric cardiology units at a 150-bed tertiary care teaching hospital in Ann Arbor, Mich. Patients (n = 160) were randomly selected from consecutive admissions to the Pediatric Cardiology and Thoracic Surgery Services during a 1-year period. None. Acute and chronic malnutrition, assessed by comparing the patients' weight and height with established means. Acute and chronic malnutrition occurred in 33% and 64% of the patients, respectively. Age, diagnostic category, and symptoms were associated with malnutrition. Eighty percent of infants presented with acute malnutrition compared with 18% of patients of other ages (P heart disease, and no patients with primary rhythm disturbances. Acute malnutrition affected 11% and chronic malnutrition affected 50% of patients with left-sided heart obstruction. Acute or chronic malnutrition occurred in 70% or more of patients with cyanosis and/or congestive heart failure but in only 30% of patients with neither (P congenital heart disease remains common, highlighting the importance of nutritional screening and intervention.

  1. The Congenital Heart Disease Genetic Network Study: Cohort description.

    Science.gov (United States)

    Hoang, Thanh T; Goldmuntz, Elizabeth; Roberts, Amy E; Chung, Wendy K; Kline, Jennie K; Deanfield, John E; Giardini, Alessandro; Aleman, Adolfo; Gelb, Bruce D; Mac Neal, Meghan; Porter, George A; Kim, Richard; Brueckner, Martina; Lifton, Richard P; Edman, Sharon; Woyciechowski, Stacy; Mitchell, Laura E; Agopian, A J

    2018-01-01

    The Pediatric Cardiac Genomics Consortium (PCGC) designed the Congenital Heart Disease Genetic Network Study to provide phenotype and genotype data for a large congenital heart defects (CHDs) cohort. This article describes the PCGC cohort, overall and by major types of CHDs (e.g., conotruncal defects) and subtypes of conotrucal heart defects (e.g., tetralogy of Fallot) and left ventricular outflow tract obstructions (e.g., hypoplastic left heart syndrome). Cases with CHDs were recruited through ten sites, 2010-2014. Information on cases (N = 9,727) and their parents was collected through interviews and medical record abstraction. Four case characteristics, eleven parental characteristics, and thirteen parent-reported neurodevelopment outcomes were summarized using counts and frequencies and compared across CHD types and subtypes. Eleven percent of cases had a genetic diagnosis. Among cases without a genetic diagnosis, the majority had conotruncal heart defects (40%) or left ventricular outflow tract obstruction (21%). Across CHD types, there were significant differences (ptypes and subtypes, provides a reference work for investigators who are interested in collaborating with or using publically available resources from the PCGC.

  2. CONGENITAL HEART DISEASE—Changing Concepts in the Surgical Treatment

    Science.gov (United States)

    Shumway, Norman E.; Lower, Richard R.; Hurley, Edward J.; Dong, Eugene; Stofer, Raymond C.

    1962-01-01

    Probably the most important continuing advance in the treatment of congenital heart disease is the ever-diminishing risk of operations on the open heart. The uncomplicated septal defect or valvular stenosis is now corrected under direct vision with essentially the same risk as that which attends the routine operation for patent ductus arteriosus. Perfusion systems, and corrective heart operations, are now available for any patient who weighs 10 kilograms or more; palliative operations are often prescribed for critically ill patients weighing less than 10 kilograms. With respect to the future, successful removal and replantation of the heart in dogs opens the door for imaginative approaches to many states now considered inoperable. Still more inspiring is the realization that cardiac homotransplantation is surgically feasible and immunologically possible, if specific transplantation antigens can be isolated. PMID:13912411

  3. Congenital and Acquired Valvular Heart Disease in Pregnancy.

    Science.gov (United States)

    Goldstein, Sarah A; Ward, Cary C

    2017-08-24

    The number of pregnancies complicated by valvular heart disease is increasing. This review describes the hemodynamic effects of clinically important valvular abnormalities during pregnancy and reviews current guideline-driven management strategies. Valvular heart disease in women of childbearing age is most commonly caused by congenital abnormalities and rheumatic heart disease. Regurgitant lesions are well tolerated, while stenotic lesions are associated with a higher risk of pregnancy-related complications. Management of symptomatic disease during pregnancy is primarily medical, with percutaneous interventions considered for refractory symptoms. Most guidelines addressing the management of valvular heart disease during pregnancy are based on case reports and observational studies. Additional investigation is required to further advance the care of this growing patient population.

  4. Genetics of Congenital Heart Defects: The NKX2-5 Gene, a Key Player

    Directory of Open Access Journals (Sweden)

    Ill-Min Chung

    2016-01-01

    Full Text Available Congenital heart defects (CHDs represent the biggest fraction of morbid congenital anomalies worldwide. Owing to their complex inheritance patterns and multifactorial etiologies, these defects are difficult to identify before complete manifestation. Research over the past two decades has established firmly the role of genetics in the development of these congenital defects. While syndromic CHDs are more straightforward, non-syndromic CHDs are usually characterized by multiple mutations that affect intricate inter-connected developmental pathways. Knock-out and gene expression studies in mice and other genetic models have been performed to elucidate the roles of these implicated genes. Functional analysis has not been able to resolve the complete picture, as increasingly more downstream effects are continuously being assigned to CHD mutant factors. NKX2-5, a cardiac transcription factor, has received much attention for its role in cardiac dysmorphogenesis. Approximately 50 different mutations in this gene have been identified to date, and only a few have been functionally characterized. The mutant NKX2-5 factor can regulate a number of off-targets downstream to facilitate CHD development. This review summarizes the genetic etiology of congenital heart defects and emphasizes the need for NKX2-5 mutation screening.

  5. Role of catheter and surgical ablation in congenital heart disease.

    Science.gov (United States)

    Hebe, Joachim

    2002-08-01

    The role of surgery and radiofrequency current ablation for the treatment of tachycardias in patients with congenital heart disease The use of radiofrequency current application as a treatment strategy has stimulated a revolution in our understanding of tachycardia mechanisms. The extension of its use to patients with congenital heart defects and tachyarrhythmias has opened the door to new treatments with known success rates and known risks for mortality and morbidity. Antiarrhythmic surgery aims to dissect or excavate a responsible substrate and is especially worth considering if cardiac surgery is being undertaken for other reasons. With suitable surgical skill and interest, and with strong electrophysiologic support, high success rates have been documented. Antiarrhythmic surgical incisions have the advantage of being visually controllable regarding the extent and location of damage to myocardial tissue. In other situations, radiofrequency current ablation is preferred because of its less-invasive character, its use of local anesthesia, and the avoidance of surgical trauma. Both surgery and catheter ablation require precise clarification of the tachycardia mechanism and precise localization of the underlying substrate. The importation of such techniques into the realm of open chest surgery would be difficult in light of the need for multiple intracardiac catheters and repeated fluoroscopically guided catheter positioning. Electrophysiologic studies performed during the antiarrhythmic surgical procedure cannot provide complete information, and their use is thus restricted to the arrhythmogenic myocardial target only [32,45]. In contrast, catheter-mediated electrophysiologic studies offer the option of exact diagnosis, precise substrate localization, and interventional treatment in a single session. Moreover, validation of the linear lesion's completeness has become a reliable predictor for mid- and long-term success in avoiding recurrences. As a result, the

  6. Single dose myocardial protection technique utilizing del Nido cardioplegia solution during congenital heart surgery procedures.

    Science.gov (United States)

    Charette, K; Gerrah, R; Quaegebeur, J; Chen, J; Riley, D; Mongero, L; Corda, R; Bacha, E

    2012-03-01

    The single dose cardioplegia technique for myocardial protection during congenital heart surgery is a viable alternative to multidose protocols. Thirty-four pediatric patients with aortic cross clamp times greater than 90 minutes were grouped by modified adult (MA) multidose solution or del Nido (dN) single dose solution. Also, data from eight patients where the cross clamp times were greater than two hours on one dose of dN solution were included. In the 90-minute plus arm of the study, there were no significant differences between the groups when comparing the risk adjustment for congenital heart surgery (RACHS) (p=0.6), cardiopulmonary bypass times (CPB) (p=0.5), aortic cross camp times (p=0.5), weights (p=0.7) and number of intraoperative exogenous blood units (p=0.5). There were significant differences between the groups (p<0.05) in the number of cardioplegia doses and with perioperative glucose levels. In the greater than two hours group, the incidence of complete heart block (CHB) was 0.125% and there were no deaths or mechanical circulatory support (MCS) devices used. del Nido cardioplegia solution is a reasonable tool for myocardial protection during congenital heart surgery that significantly decreased the number of cardioplegic interventions and perioperative glucose values in our study groups.

  7. Lung transplantation and repair of complex congenital heart lesions in patients with pulmonary hypertension.

    Science.gov (United States)

    Mendeloff, E N; Huddleston, C B

    1998-04-01

    Pulmonary vascular disease in conjunction with either a previously repaired or an unrepaired congenital heart defect is the third most common indication for lung transplantation in the pediatric age range. Because scarcity of donor organs remains a critical issue and heart-lung donor blocks are becoming diminishingly available, efforts must be directed towards other options such as combining lung transplantation with correction of the underlying congenital heart defect. Certain defects like congenital pulmonary vein stenosis are eradicated by removal of the diseased lungs, whereas others such as complete atrioventricular canal and pulmonary atresia with ventricular septal defect require cardioplegic arrest of the heart and intracardiac repair in conjunction with the lung transplantation. A breakdown of this patient population into subgroups may be helpful both in thinking about the pathophysiology and in determining appropriate indications and timing of transplantation. Earlier studies from our center showed the high-risk nature and formidable undertaking of caring for this complex group of patients. Through continued experience, there has been gradual improvement in early outcomes. As with all other groups of lung transplantation patients, obliterative bronchiolitis remains the major deterrent to long-term survival.

  8. Challenging pacemaker implantation in a patient with acquired dextrocardia after pneumonectomy, skoliosis and complete heart block.

    Science.gov (United States)

    Hamm, Karsten; Haghi, Dariush; Borggrefe, Martin; Kuschyk, Jürgen

    2010-09-01

    Pacemaker implantation after pneumonectomy is rare and there have been no previously reported cases of acquired dextrocardia after implantation. The authors report the case of a pacemaker implantation in a patient with complete heart block, impaired left ventricular function, sclerosis of heart valves and radiation induced vasculopathy resulting in ostial stenosis of the right coronary artery 30 years after radiochemotherapy in childhood. Acquired dextrocardia after right pneumonectomy for mucoepidermoid carcinoma made implantation a challenge due to and poor fluoroscopic visualization of the heart and increased radio-opacity of the right chest when compared to congenital dextrocardia.

  9. Emerging Research Directions in Adult Congenital Heart Disease: A Report from a National Heart, Lung, and Blood Institute/Adult Congenital Heart Association Working Group

    Science.gov (United States)

    Gurvitz, Michelle; Burns, Kristin M.; Brindis, Ralph; Broberg, Craig S.; Daniels, Curt J.; Fuller, Stephanie M.P.N.; Honein, Margaret A.; Khairy, Paul; Kuehl, Karen S.; Landzberg, Michael J.; Mahle, William T.; Mann, Douglas L.; Marelli, Ariane; Newburger, Jane W.; Pearson, Gail D.; Starling, Randall C.; Tringali, Glenn R.; Valente, Anne Marie; Wu, Joseph C.; Califf, Robert M.

    2016-01-01

    Congenital heart disease (CHD) is the most common birth defect, affecting about 0.8% of live births. Advances in recent decades have allowed >85% of children with CHD to survive to adulthood, creating a growing population of adults with CHD. Little information exists regarding survival, demographics, late outcomes, and comorbidities in this emerging group, and multiple barriers impede research in adult CHD (ACHD). The National Heart, Lung, and Blood Institute and the Adult Congenital Heart Association convened a multidisciplinary Working Group to identify high-impact research questions in ACHD. This report summarizes the meeting discussions in the broad areas of CHD-related heart failure, vascular disease and multisystem complications. High-priority subtopics identified included heart failure in tetralogy of Fallot, mechanical circulatory support/transplantation, sudden cardiac death, vascular outcomes in coarctation of the aorta, late outcomes in single ventricle disease, cognitive and psychiatric issues, and pregnancy. PMID:27102511

  10. The genetics of congenital heart disease… understanding and improving long-term outcomes in congenital heart disease: a review for the general cardiologist and primary care physician.

    Science.gov (United States)

    Simmons, M Abigail; Brueckner, Martina

    2017-10-01

    This review has two purposes: to provide an updated review of the genetic causes of congenital heart disease (CHD) and the clinical implications of these genetic mutations, and to provide a clinical algorithm for clinicians considering a genetics evaluation of a CHD patient. A large portion of congenital heart disease is thought to have a significant genetic contribution, and at this time a genetic cause can be identified in approximately 35% of patients. Through the advances made possible by next generation sequencing, many of the comorbidities that are frequently seen in patients with genetic congenital heart disease patients can be attributed to the genetic mutation that caused the congenital heart disease. These comorbidities are both cardiac and noncardiac and include: neurodevelopmental disability, pulmonary disease, heart failure, renal dysfunction, arrhythmia and an increased risk of malignancy. Identification of the genetic cause of congenital heart disease helps reduce patient morbidity and mortality by improving preventive and early intervention therapies to address these comorbidities. Through an understanding of the clinical implications of the genetic underpinning of congenital heart disease, clinicians can provide care tailored to an individual patient and continue to improve the outcomes of congenital heart disease patients.

  11. A positive perspective of knowledge, attitude, and practices for health-promoting behaviors of adolescents with congenital heart disease.

    Science.gov (United States)

    Huang, Hui-Ru; Chen, Chi-Wen; Chen, Chin-Mi; Yang, Hsiao-Ling; Su, Wen-Jen; Wang, Jou-Kou; Tsai, Pei-Kwei

    2017-08-01

    Health-promoting behaviors could serve as a major strategy to optimize long-term outcomes for adolescents with congenital heart disease. The associations assessed from a positive perspective of knowledge, attitudes, and practice model would potentially cultivate health-promoting behaviors during adolescence. The purpose of this study was to examine the relationships between disease knowledge, resilience, family functioning, and health-promoting behaviors in adolescents with congenital heart disease. A total of 320 adolescents with congenital heart disease who were aged 12-18 years were recruited from pediatric cardiology outpatient departments, and participated in a cross-sectional survey. The participants completed the Leuven Knowledge Questionnaire for Congenital Heart Disease; Haase Adolescent Resilience in Illness Scale; Family Adaptability, Partnership, Growth, Affection, and Resolve; and Adolescent Health Promotion scales. The collected data were analyzed using descriptive statistics and three multiple regression models. Greater knowledge of prevention of complications and higher resilience had a more powerful effect in enhancing health-promoting behaviors. Having symptoms and moderate or severe family dysfunction were significantly more negatively predictive of health-promoting behaviors than not having symptoms and positive family function. The third model explained 40% of the variance in engaging in health-promoting behaviors among adolescents with congenital heart disease. The findings of this study provide new insights into the role of disease knowledge, resilience, and family functioning in the health-promoting behavior of adolescents with congenital heart disease. Continued efforts are required to plan family care programs that promote the acquisition of sufficient disease knowledge and the development of resilience for adolescents with congenital heart disease.

  12. Report of the National Heart, Lung, and Blood Institute Working Group: An Integrated Network for Congenital Heart Disease Research.

    Science.gov (United States)

    Pasquali, Sara K; Jacobs, Jeffrey P; Farber, Gregory K; Bertoch, David; Blume, Elizabeth D; Burns, Kristin M; Campbell, Robert; Chang, Anthony C; Chung, Wendy K; Riehle-Colarusso, Tiffany; Curtis, Lesley H; Forrest, Christopher B; Gaynor, William J; Gaies, Michael G; Go, Alan S; Henchey, Paul; Martin, Gerard R; Pearson, Gail; Pemberton, Victoria L; Schwartz, Steven M; Vincent, Robert; Kaltman, Jonathan R

    2016-04-05

    The National Heart, Lung, and Blood Institute convened a working group in January 2015 to explore issues related to an integrated data network for congenital heart disease research. The overall goal was to develop a common vision for how the rapidly increasing volumes of data captured across numerous sources can be managed, integrated, and analyzed to improve care and outcomes. This report summarizes the current landscape of congenital heart disease data, data integration methodologies used across other fields, key considerations for data integration models in congenital heart disease, and the short- and long-term vision and recommendations made by the working group. © 2016 American Heart Association, Inc.

  13. Spectrum of Congenital Heart Diseases in Eastern Nepal: A tertiary care hospital experience

    OpenAIRE

    Prashant Shah; Kunjang Sherpa; Naveen Kumar Pandey; Bhawani Manandhar; Sahadeb Prasad Dhungana

    2017-01-01

    Background & Objectives: Congenital heart diseases are neglected especially in world’s poorest nations and appear to be ignored and unexplored dimension of health. The exact prevalence and spectrum of congenital heart diseases in Nepal is largely unknown. The aim of this study was to describe the local experience on the magnitude and the pattern of congenital heart disease in order to increase the awareness of the public and health policy makers on its burden in Nepal.Materials & Meth...

  14. Compliance with Adult Congenital Heart Disease Guidelines: Are We Following the Recommendations?

    Science.gov (United States)

    Gerardin, Jennifer F; Menk, Jeremiah S; Pyles, Lee A; Martin, Cindy M; Lohr, Jamie L

    2016-05-01

    As the adult congenital heart disease population increases, poor transition from pediatric to adult care can lead to suboptimal quality of care and an increase in individual and institutional costs. In 2008, the American College of Cardiology and American Heart Association updated the adult congenital heart disease practice guidelines and in 2011, the American Heart Association recommended transition guidelines to standardize and encourage appropriate timing of transition to adult cardiac services. The objective of this study was to evaluate if patient age or complexity of congenital heart disease influences pediatric cardiologists' decision to transfer care to adult providers and to evaluate the compliance of different types of cardiology providers with current adult congenital heart disease treatment guidelines. A single-center retrospective review of 991 adult congenital heart disease patients identified by ICD-9 code from 2010 to 2012. Academic and community outpatient cardiology clinics. Nine hundred ninety-one patients who are 18 years and older with congenital heart disease. None. The compliance with health maintenance and transfer of care recommendations in the outpatient setting. For patients seen by pediatric cardiologists, only 20% had transfer of care discussions documented, most often in younger simple patients. Significant differences in compliance with preventative health guidelines were found between cardiology provider types. Even though a significant number of adults with congenital heart disease are lost to appropriate follow-up in their third and fourth decades of life, pediatric cardiologists discussed transfer of care with moderate and complex congenital heart disease patients less frequently. Appropriate transfer of adults with congenital heart disease to an adult congenital cardiologist provides an opportunity to reinforce the importance of regular follow-up in adulthood and may improve outcomes as adult congenital cardiologists followed the

  15. Re-entry using anatomically determined isthmuses: a curable ventricular tachycardia in repaired congenital heart disease.

    Science.gov (United States)

    Kapel, Gijsbert F L; Reichlin, Tobias; Wijnmaalen, Adrianus P; Piers, Sebastiaan R D; Holman, Eduard R; Tedrow, Usha B; Schalij, Martin J; Stevenson, William G; Zeppenfeld, Katja

    2015-02-01

    Ventricular tachycardia (VT) is an important cause of late morbidity and mortality in repaired congenital heart disease. The substrate often includes anatomic isthmuses that can be transected by radiofrequency catheter ablation similar to isthmus block for atrial flutter. This study evaluates the long-term efficacy of isthmus block for treatment of re-entry VT in adults with repaired congenital heart disease. Thirty-four patients (49±13 years; 74% male) with repaired congenital heart disease who underwent radiofrequency catheter ablation of VT in 2 centers were included. Twenty-two (65%) had a preserved left and right ventricular function. Patients were inducible for 1 (interquartile range, 1-2) VT, median cycle length: 295 ms (interquartile range, 242-346). Ablation aimed to transect anatomic isthmuses containing VT re-entry circuit isthmuses. Procedural success was defined as noninducibility of any VT and transection of the anatomic isthmus and was achieved in 25 (74%) patients. During long-term follow-up (46±29 months), all patients with procedural success (18/25 with internal cardiac defibrillators) were free of VT recurrence but 7 of 18 experienced internal cardiac defibrillator-related complications. One patient with procedural success and depressed cardiac function received an internal cardiac defibrillator shock for ventricular fibrillation. None of the 18 patients (12/18 with internal cardiac defibrillators) with complete success and preserved cardiac function experienced any ventricular arrhythmia. In contrast, VT recurred in 4 of 9 patients without procedural success. Four patients died from nonarrhythmic causes. In patients with repaired congenital heart disease with preserved ventricular function and isthmus-dependent re-entry, VT isthmus ablation can be curative. © 2014 American Heart Association, Inc.

  16. The usefulness of brain natriuretic peptide in complex congenital heart disease: a systematic review.

    Science.gov (United States)

    Eindhoven, Jannet A; van den Bosch, Annemien E; Jansen, Philip R; Boersma, Eric; Roos-Hesselink, Jolien W

    2012-11-20

    Brain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide (NT-proBNP) are well-established markers for heart failure in the general population. However, the value of BNP as a diagnostic and prognostic marker for patients with structural congenital heart disease (CHD) is still unclear. Therefore, the purpose of this study was to evaluate the clinical utility of BNP in patients with CHD. We executed a PubMed literature search and included 49 articles that focused on complex congenital heart defects such as tetralogy of Fallot, systemic right ventricle, and univentricular hearts. Data on BNP measurements and cardiac function parameters were extracted. In all patients after correction for tetralogy of Fallot, BNP levels were elevated and correlated significantly with right ventricular end-diastolic dimensions and severity of pulmonary valve regurgitation. Patients with a systemic right ventricle had elevated BNP levels, and positive correlations between BNP and right ventricular function were seen. In patients with a univentricular heart, elevated BNP levels were observed before completion of the Fontan circulation or when patients were symptomatic; a clear association between BNP and New York Heart Association functional class was demonstrated. In conclusion, this review shows an overall increase in BNP values in complex CHD, although differences between types of congenital heart anomaly are present. As BNP values differ widely, conclusions for individual patients should be drawn with caution. Further investigation with sequential BNP measurement in a large, prospective study is warranted to elucidate the prognostic value of BNP assessment in patients with CHD. Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  17. Robotics and imaging in congenital heart surgery

    Science.gov (United States)

    Vasilyev, Nikolay V; Dupont, Pierre E; del Nido, Pedro J

    2012-01-01

    The initial success seen in adult cardiac surgery with the application of available robotic systems has not been realized as broadly in pediatric cardiac surgery. The main obstacles include extended set-up time and complexity of the procedures, as well as the large size of the instruments with respect to the size of the child. Moreover, while the main advantage of robotic systems is the ability to minimize incision size, for intracardiac repairs, cardiopulmonary bypass is still required. Catheter-based interventions, on the other hand, have expanded rapidly in both application as well as the complexity of procedures and lesions being treated. However, despite the development of sophisticated devices, robotic systems to aid catheter procedures have not been commonly applied in children. In this article, we describe new catheter-like robotic delivery platforms, which facilitate safe navigation and enable complex repairs, such as tissue approximation and fixation, and tissue removal, inside the beating heart. Additional features including the tracking of rapidly moving tissue targets and novel imaging approaches are described, along with a discussion of future prospects for steerable robotic systems. PMID:22413986

  18. Contraceptive Practices of Women With Complex Congenital Heart Disease.

    Science.gov (United States)

    Miner, Pamela D; Canobbio, Mary M; Pearson, Dorothy D; Schlater, Mary; Balon, Yvonne; Junge, Kathryn J; Bhatt, Ami; Barber, Deena; Nickolaus, Michelle J; Kovacs, Adrienne H; Moons, Philip; Shaw, Kate; Fernandes, Susan M

    2017-03-15

    Understanding the contraceptive practices of women with complex congenital heart disease (CHD) and providing them individualized contraception counseling may prevent adverse events and unplanned high-risk pregnancies. Given this, we sought to examine the contraceptive practices in women with CHD, describe adverse events associated with contraceptive use, and describe the provision of contraception counseling. Women >18 years were recruited from 2011 to 2014 from 9 adult CHD (ACHD) centers throughout North America. Subjects completed a 48-item questionnaire regarding contraceptive use and perceptions of contraception counseling, and a medical record review was performed. Of 505 subjects, median age was 33 (interquartile range 26 to 44) and 81% had CHD of moderate or great complexity. The majority (86%, 435 of 505) of the cohort had used contraception. The types included barrier methods (87%), oral contraception (OC) 84%, intrauterine device (18%), Depo-Provera (15%), vaginal ring (7%), patch (6%), hormonal implant (2%), Plan B (19%), and sterilization (16%). Overall OC use was not significantly different by CHD complexity. Women with CHD of great complexity were more likely to report a thrombotic event while taking OC than those with less complex CHD (9% vs 1%, p = 0.003). Contraception counseling by the ACHD team was noted by 43% of subjects. Unplanned pregnancy was reported by 25% with no statistical difference by CHD complexity. In conclusion, contraceptive practices of women with complex CHD are highly variable, and the prevalence of blood clots while taking OC is not insignificant while provision of contraception counseling by ACHD providers appears lacking. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. Functional basis of sinus bradycardia in congenital heart block.

    Science.gov (United States)

    Hu, Keli; Qu, Yongxia; Yue, Yuankun; Boutjdir, Mohamed

    2004-03-05

    Congenital heart block (CHB) is a conduction abnormality characterized by complete atrioventricular (AV) block. CHB affects fetuses and/or newborn of mothers with autoantibodies reactive with ribonucleoproteins 48-kDa SSB/La, 52-kDa SSA/Ro, and 60-kDa SSA/Ro. We recently established animal models of CHB and reported, for the first time, significant sinus bradycardia preceding AV block. This unexpected observation implies that the spectrum of conduction abnormalities extends beyond the AV node to also affect the SA node. To test this hypothesis, we investigated the functional basis of this sinus bradycardia by characterizing the effects of antibodies from mothers with CHB children (positive IgG) on ionic currents that are known to significantly contribute to spontaneous pacing in SA node cells. We recorded L- (I(Ca.L)) and T- (I(Ca.T)) type Ca2+, delayed rectifier K+ (I(K)), hyperpolarization-activated (I(f)) currents, and action potentials (APs) from young rabbit SA node cells. We demonstrated that positive IgG significantly inhibited both I(Ca.T) and I(Ca.L) and induced sinus bradycardia but did not affect I(f) and I(K). Normal IgG from mothers with healthy children did not affect all the currents studied and APs. These results establish that IgG from mothers with CHB children causes substantial inhibition of I(Ca.T) and I(Ca.L), two important pacemaker currents in rabbit SA node cells and point to both I(Ca.T) and I(Ca.L) as major players in the ionic mechanism by which maternal antibodies induce sinus bradycardia in CHB. These novel findings have important clinical significance and suggest that sinus bradycardia may be a potential marker in the detection and prevention of CHB. The full text of this article is available online at http://circres.ahajournals.org

  20. Segmental analysis of congenital heart disease: putting the "puzzle" together with computed tomography.

    Science.gov (United States)

    Bierhals, Andrew J; Rossini, Sebastian; Woodard, Pamela K; Javidan-Nejad, Cylen; Billadello, Joseph J; Bhalla, Sanjeev; Gutierrez, Fernando R

    2014-08-01

    Advances in surgical and medical treatment for congenital heart disease have resulted in greater life expectancy. As a result, there has been an increase in the utilization of cross-sectional imaging for diagnosis and management of complex congenital heart disease. This manuscript describes a morphological and sequential segmental approach to deciphering the code of complex congenital heart defects in cross-sectional imaging, mostly computed tomography. This manuscript will review approaches to differentiate types of transposition, the anatomic relationships of cardiac structures, and the application of these relationships in the description of complex congenital heart disease.

  1. Angiopoietin-2 in adults with congenital heart disease and heart failure.

    Directory of Open Access Journals (Sweden)

    Alexander Lukasz

    Full Text Available BACKGROUND: Chronic heart failure is an important cause for morbidity and mortality in adults with congenital heart disease (ACHD. While NT-proBNP is an established biomarker for heart failure of non-congenital origin, its application in ACHD has limitations. The angiogenic factors Angiopoietin-1 and -2 (Ang-1, Ang-2, vascular endothelial growth factor (VEGF, and soluble receptor tyrosine kinase of the Tie family (sTie2 correlate with disease severity in heart failure of non-congenital origin. Their role in ACHD has not been studied. METHODS: In 91 patients Ang-2 and NT-proBNP were measured and related to New York Heart Association class, systemic ventricular function and parameters of cardiopulmonary exercise testing. Ang-1, VEGF, and sTie2 were also measured. RESULTS: Ang-2 correlates with NYHA class and ventricular dysfunction comparable to NT-proBNP. Further, Ang-2 showed a good correlation with parameters of cardiopulmonary exercise testing. Both, Ang-2 and NT-proBNP identified patients with severely limited cardiopulmonary exercise capacity. Additionally, Ang-2 is elevated in patients with a single ventricle physiology in contrast to NT-proBNP. VEGF, Ang-1, and sTie2 were not correlated with any clinical parameter. CONCLUSION: The performance of Ang-2 as a biomarker for heart failure in ACHD is comparable to NT-proBNP. Its significant elevation in patients with single ventricle physiology indicates potential in this patient group and warrants further studies.

  2. Autonomic regulation in fetuses with congenital heart disease.

    Science.gov (United States)

    Siddiqui, Saira; Wilpers, Abigail; Myers, Michael; Nugent, J David; Fifer, William P; Williams, Ismée A

    2015-03-01

    Exposure to antenatal stressors affects autonomic regulation in fetuses. Whether the presence of congenital heart disease (CHD) alters the developmental trajectory of autonomic regulation is not known. This prospective observational cohort study aimed to further characterize autonomic regulation in fetuses with CHD; specifically hypoplastic left heart syndrome (HLHS), transposition of the great arteries (TGA), and tetralogy of Fallot (TOF). From 11/2010 to 11/2012, 92 fetuses were enrolled: 41 controls and 51 with CHD consisting of 19 with HLHS, 12 with TGA, and 20 with TOF. Maternal abdominal fetal electrocardiogram (ECG) recordings were obtained at 3 gestational ages: 19-27 weeks (F1), 28-33 weeks (F2), and 34-38 weeks (F3). Fetal ECG was analyzed for mean heart rate along with 3 measures of autonomic variability of the fetal heart rate: interquartile range, standard deviation, and root mean square of the standard deviation of the heart rate (RMSSD), a measure of parasympathetic activity. During F1 and F2 periods, HLHS fetuses demonstrated significantly lower mean HR than controls (pHeart rate variability at F3, as measured by standard deviation, interquartile range, and RMSSD was lower in HLHS than controls (p<0.05). Other CHD subgroups showed a similar, though non-significant trend towards lower variability. Autonomic regulation in CHD fetuses differs from controls, with HLHS fetuses most markedly affected. Copyright © 2015 Elsevier Ltd. All rights reserved.

  3. Congenital Heart Disease In Pediatric Patients: Recognizing The Undiagnosed And Managing Complications In The Emergency Department.

    Science.gov (United States)

    Judge, Pavan; Meckler Mshs, Garth

    2016-05-01

    Congenital heart disease is the most common form of all congenital malformations and, despite advances in prenatal and newborn screening, it may present undiagnosed to the emergency department. Signs and symptoms of congenital heart disease are variable and often nonspecific, making recognition and treatment challenging. Patient presentations can range from life-threatening shock or cyanosis in a neonate to respiratory distress or failure to thrive in infants. Advances in surgical techniques have improved short- and long-term survival of infants and children with congenital heart disease, but these children are at risk for a variety of complications related to the underlying or surgical anatomy and physiology. This review focuses on the recognition and initial management of patients with undiagnosed congenital heart disease presenting to the ED and touches on considerations for postoperative infants and children with complex congenital heart disease.

  4. Ablation of Atrial Fibrillation in Patients with Congenital Heart Disease.

    Science.gov (United States)

    Refaat, Marwan M; Ballout, Jad; Mansour, Moussa

    2017-12-01

    With improved surgical techniques and medical management for patients with congenital heart diseases, more patients are living longer and well into adulthood. This improved survival comes with a price of increased morbidity, mainly secondary to increased risk of tachyarrhythmias. One of the major arrhythmias commonly encountered in this subset of cardiac patients is AF. Similar to the general population, the risk of AF increases with advancing age, and is mainly secondary to the abnormal anatomy, abnormal pressure and volume parameters in the hearts of these patients and to the increased scarring and inflammation seen in the left atrium following multiple surgical procedures. Catheter ablation for AF has been shown to be a very effective treatment modality in patients with refractory AF. However, data and guidelines regarding catheter ablation in patients with congenital heart disease are not well established. This review will shed light on the procedural techniques, success rates and complications of AF catheter ablation in patients with different types of CHD, including atrial septal defects, tetralogy of Fallot, persistent left superior vena cava, heterotaxy syndrome and atrial isomerism, and Ebstein anomaly.

  5. Sports participation in adults with congenital heart disease.

    Science.gov (United States)

    Opić, Petra; Utens, Elisabeth M W J; Cuypers, Judith A A E; Witsenburg, Maarten; van den Bosch, Annemien; van Domburg, Ron; Bogers, Ad J J C; Boersma, Eric; Pelliccia, Antonio; Roos-Hesselink, Jolien W

    2015-01-01

    It is unclear whether sports participation in adults with repaired congenital heart disease is safe and has benefits. Congenital heart disease (ConHD) patients who underwent corrective surgery for Atrial Septal Defect, Ventricular Septal Defect, Pulmonary Stenosis, Tetralogy of Fallot or Transposition of the Great Arteries in our center between 1968 and 1980 were included, and participated in our longitudinal follow-up study with serial evaluations in 2001 and 2011. At both time points patients filled in questionnaires on sports participation, subjective physical functioning and quality of life. Exercise testing, echocardiogram and 24-hour continuous ambulatory ECG-monitoring were performed in both 2001 and 2011. All clinical events (re-intervention, arrhythmia, heart failure) were prospectively recorded. No relationship was found between practicing sports and the occurrence of sudden death, PVCs or SVTs. Patients with moderate/complex forms of ConHD practiced fewer hours of sports compared with the general Dutch normative population. Patients with both simple and moderate/complex ConHD who practiced sports showed a higher exercise capacity. More favorable subjective physical functioning was found for moderate/complex patients who practiced sports. Adults with repaired ConHD are less often involved in sports than the Dutch general population. The patients that were engaged in sports show a higher exercise capacity than those who did not. Sports participation in patients with ConHD was not associated with an increased incidence of adverse cardiac events. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  6. Ambient air pollution and congenital heart defects in Lanzhou, China

    Science.gov (United States)

    Jin, Lan; Qiu, Jie; Zhang, Yaqun; Qiu, Weitao; He, Xiaochun; Wang, Yixuan; Sun, Qingmei; Li, Min; Zhao, Nan; Cui, Hongmei; Liu, Sufen; Tang, Zhongfeng; Chen, Ya; Yue, Li; Da, Zhenqiang; Xu, Xiaoying; Huang, Huang; Liu, Qing; Bell, Michelle L.; Zhang, Yawei

    2015-07-01

    Congenital heart defects are the most prevalent type of birth defects. The association of air pollution with congenital heart defects is not well understood. We investigated a cohort of 8969 singleton live births in Lanzhou, China during 2010-2012. Using inverse distance weighting, maternal exposures to particulate matter with diameters ≤10 μm (PM10), nitrogen dioxide (NO2), and sulfur dioxide (SO2) were estimated as a combination of monitoring station levels for time spent at home and in a work location. We used logistic regression to estimate the associations, adjusting for maternal age, education, income, BMI, disease, folic acid intake and therapeutic drug use, and smoking; season of conception, fuel used for cooking and temperature. We found significant positive associations of Patent Ductus Arteriosus (PDA) with PM10 during the 1st trimester, 2nd trimester and the entire pregnancy (OR 1st trimester = 3.96, 95% confidence interval (CI): 1.36, 11.53; OR 2nd trimester = 3.59, 95% CI: 1.57, 8.22; OR entire pregnancy = 2.09, 95% CI: 1.21, 3.62, per interquartile range (IQR) increment for PM10 (IQR = 71.2, 61.6, and 27.4 μg m-3, respectively)), and associations with NO2 during 2nd trimester and the entire pregnancy (OR 2nd trimester = 1.92, 95% CI: 1.11, 3.34; OR entire pregnancy = 2.32, 95% Cl: 1.14, 4.71, per IQR increment for NO2 (IQR = 13.4 and 10.9 μg m-3, respectively)). The associations for congenital malformations of the great arteries and pooled cases showed consistent patterns. We also found positive associations for congenital malformations of cardiac septa with PM10 exposures in the 2nd trimester and the entire pregnancy, and SO2 exposures in the entire pregnancy. Results indicate a health burden from maternal exposures to air pollution, with increased risk of congenital heart defects.

  7. The impact of an infant's severe congenital heart disease on the family: a prospective cohort study.

    Science.gov (United States)

    Werner, Helene; Latal, Beatrice; Valsangiacomo Buechel, Emanuela; Beck, Ingrid; Landolt, Markus A

    2014-01-01

    The aim of this article is to investigate the impact of a child's severe congenital heart disease on the family and to prospectively examine the influence of disease specific and psychosocial factors on the family. A prospective cohort study. Parents of 104 infants who had undergone cardiopulmonary bypass surgery before the age of 12 months for congenital heart disease were included. None. Parents completed the generic Impact on Family scale and a social support questionnaire; a large number of medical data were extracted from the patients' hospital records. Parents most frequently reported that they were thinking about not having more children and living on a "roller coaster." No difference was found in the total Impact on Family scale score between fathers and mothers. The presence of a genetic disorder in the child and lower levels of perceived social support was significantly associated with a greater impact on the family. The impact of an infant's congenital heart disease on the family is determined both by child's medical condition and family's psychosocial factors. Families with poorer social support network may have the greatest need for professional interventions, especially if their child has an underlying genetic disorder. © 2013 Wiley Periodicals, Inc.

  8. Stem Cell Therapy for Congenital Heart Disease: A Systematic Review.

    Science.gov (United States)

    Tsilimigras, Diamantis I; Oikonomou, Evangelos K; Moris, Demetrios; Schizas, Dimitrios; Economopoulos, Konstantinos P; Mylonas, Konstantinos S

    2017-12-12

    Congenital heart disease (CHD) constitutes the most prevalent and heterogeneous group of congenital anomalies. Although surgery remains the gold standard treatment modality, stem cell therapy has been gaining ground as a complimentary or alternative treatment option in certain types of CHD. The aim of this study was to present the existing published evidence and ongoing research efforts on the implementation of stem cell-based therapeutic strategies in CHD. A systematic review was conducted by searching Medline, ClinicalTrials.gov, and the Cochrane library, along with reference lists of the included studies through April 23, 2017. Nineteen studies were included in this review (8 preclinical, 6 clinical, and 5 ongoing trials). Various routes of cardiac stem cell delivery have been reported, including intracoronary, intramyocardial, intravenous, and epicardial. Depending on their origin and level of differentiation at which they are harvested, stem cells may exhibit different properties. Preclinical studies have mostly focused on modeling right ventricle dysfunction or failure and pulmonary artery hypertension by using pressure or volume overload in vitro or in vivo. Only a limited number of clinical trials on patients with CHD exist, and these primarily focus on hypoplastic left heart syndrome. Cell-based tissue engineering has recently been introduced, and research currently is focusing on developing cell-seeded grafts and patches that could potentially grow in parallel with whole body growth once implanted in the heart. It seems that stem cell delivery to the diseased heart as an adjunct to surgical palliation may provide some benefits over surgery alone in terms of cardiac function, somatic growth, and quality of life. Despite encouraging preliminary results, stem cell therapies for patients with CHD should only be considered in the setting of well-designed clinical trials. More wet laboratory research experience is needed, and translation of promising findings

  9. Transition to Adult Congenital Heart Disease Care: A Review.

    Science.gov (United States)

    Hays, Laura

    2015-01-01

    The population of adults with congenital heart disease (ACHD) has grown due to recent advances in surgical procedures. The survival rate to adulthood is now more than 95%. This review identifies current recommendations and status of ACHD management and treatment in the United States by examining comprehensive guidelines for management and transition and comparing them to the current state of the science. Successful transition from pediatric to adult care begins during the adolescent years, and prepares patients for management at an ACHD regional center utilizing multidisciplinary teams of ACHD specialists. Advocacy and research needs for the ACHD population persist. Copyright © 2015 Elsevier Inc. All rights reserved.

  10. Arrhythmias in Adults with Congenital Heart Disease: What Are Risk Factors for Specific Arrhythmias?

    Science.gov (United States)

    Loomba, Rohit S; Buelow, Matthew W; Aggarwal, Saurabh; Arora, Rohit R; Kovach, Joshua; Ginde, Salil

    2017-04-01

    An increasing number of patients with congenital heart disease are now surviving into adulthood. This has also led to the emergence of complications from the underlying congenital heart disease, related surgical interventions, and associated combordities. While the prevalence of particular arrhythmias with specific congenital heart disease has been previously described, a detailed analysis of all lesions and a large number of comorbidities has not been previously published. Admissions with congenital heart disease were identified in the National Inpatient Sample. Associated comorbidities were also identified for these patients. Univariate analysis was done to compare those risk factors associated with specific arrhythmias in the setting of congenital heart disease. Next, regression analysis was done to identify what patient characteristics and comorbidities were associated with increased risk of specific arrhythmias. A total of 52,725,227 admissions were included in the analysis. Of these, 109,168 (0.21%) had congenital heart disease. Of those with congenital heart disease, 27,088 (25%) had an arrhythmia at some point. The most common arrhythmia in those with congenital heart disease was atrial fibrillation, which was noted in 86% of those with arrhythmia followed by atrial flutter which was noted in 20% of those with congenital heart disease. The largest burden of arrhythmia was found to be in those with tricuspid atresia with a 51% prevalence of arrhythmia in this group followed by Ebstein anomaly which had an arrhythmia prevalence of 39%. Increasing age, male gender, double outlet right ventricle, atrioventricular septal defect, heart failure, obstructive sleep apnea, transposition of the great arteries, congenitally corrected transposition, and tetralogy of Fallot were frequently noted to be independent risk factors of specific arrhythmias. Approximately, 25% of adult admissions with congenital heart disease are associated with arrhythmia. The burden of

  11. Adherence to guidelines in the clinical care for adults with congenital heart disease: the Euro Heart Survey on adult congenital heart disease

    NARCIS (Netherlands)

    Engelfriet, Peter; Tijssen, Jan; Kaemmerer, Harald; Gatzoulis, Michael A.; Boersma, Eric; Oechslin, Erwin; Thaulow, Erik; Popelová, Jana; Moons, Philip; Meijboom, Folkert; Daliento, Luciano; Hirsch, Rafael; Laforest, Valérie; Thilén, Ulf; Mulder, Barbara

    2006-01-01

    AIMS: To investigate the role of guidelines in structuring the clinical care for adult patients with congenital heart disease (CHD), and to assess adherence to the guidelines in Europe. METHODS AND RESULTS: A selected number of current guidelines were chosen pertaining to operative procedures,

  12. Hematuria and a complex congenital heart defect in a newborn foal

    Science.gov (United States)

    Spiro, Illana

    2002-01-01

    A 16-hour-old foal was referred for evaluation of hematuria. A pansystolic heart murmur and left thoracic thrill were detected; laboratory diagnostics and ultrasonography identified a complex congenital heart defect. The diagnosis was confirmed at postmortem. PMID:12001506

  13. Intrapartum electrocardiogram alteration in fetuses with congenital heart disease: a case-control study.

    Science.gov (United States)

    Gay, Estelle; Bornallet, Géraldine; Gaucherand, Pascal; Doret, Muriel

    2015-11-01

    To assess if the fetal electrocardiogram especially ST segment is modified by congenital heart diseases: modifications in frequencies of the different ST events and modifications in signal quality. A retrospective case-control study, comparing frequencies of the different ST events and the quality of the signal between fetuses with congenital heart diseases and fetuses without congenital heart disease. From 2000 to 2011, fifty-eight fetuses with congenital heart disease had their heart rate recording using a STAN device during labor. Control group was fetuses who were born just before a case and had a STAN as a second line for intrapartum surveillance. Cases and controls were matched on parity, gestational age at birth, presence of growth restriction and umbilical artery pH. Frequencies of the different ST event and quality of the signal were first analyzed for the global labor recording, and then separately for the first and the second phase of labor. No statistically significant difference in ST event frequencies between fetuses with congenital heart disease and the control group was found. Regarding the quality of the signal, 11.49% (±18.82) of recording time is a signal loss for fetus with congenital heart disease whereas only 5.18% (±10.67) for the control group (p=0.028). This is the first study investigating for intrapartum electrocardiogram modification in fetus with congenital heart disease. Congenital heart diseases do not modify frequencies of ST events. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  14. The epidemiology of heart failure in adults with congenital heart disease.

    Science.gov (United States)

    Rodriguez, Fred H; Marelli, Ariane J

    2014-01-01

    The impact of lifelong exposure to myocardial dysfunction in populations with congenital heart disease (CHD) is becoming increasingly recognized. Most children born with CHD now reach adulthood and the long-term sequelae of treatment are contributing to substantial comorbidity. The combination of structural changes present at birth with changes resulting from cardiac surgery can result in heart failure. This article reports on the current state of knowledge on the epidemiology of heart failure in this patient population. Copyright © 2014 Elsevier Inc. All rights reserved.

  15. Congenital heart malformations induced by hemodynamic altering surgical interventions

    Directory of Open Access Journals (Sweden)

    Madeline eMidgett

    2014-08-01

    Full Text Available Embryonic heart formation results from a dynamic interplay between genetic and environmental factors. Blood flow during early embryonic stages plays a critical role in heart development, as interactions between flow and cardiac tissues generate biomechanical forces that modulate cardiac growth and remodeling. Normal hemodynamic conditions are essential for proper cardiac development, while altered blood flow induced by surgical manipulations in animal models result in heart defects similar to those seen in humans with congenital heart disease. This review compares the altered hemodynamics, changes in tissue properties, and cardiac defects reported after common surgical interventions that alter hemodynamics in the early chick embryo, and shows that interventions produce a wide spectrum of cardiac defects. Vitelline vein ligation and left atrial ligation decrease blood pressure and flow; and outflow tract banding increases blood pressure and flow velocities. These three surgical interventions result in many of the same cardiac defects, which indicate that the altered hemodynamics interfere with common looping, septation and valve formation processes that occur after intervention and that shape the four-chambered heart. While many similar defects develop after the interventions, the varying degrees of hemodynamic load alteration among the three interventions also result in varying incidence and severity of cardiac defects, indicating that the hemodynamic modulation of cardiac developmental processes is strongly dependent on hemodynamic load.

  16. Stem cell therapy and tissue engineering for correction of congenital heart disease

    OpenAIRE

    Avolio, Elisa; Caputo, Massimo; Madeddu, Paolo

    2015-01-01

    This review article reports on the new field of stem cell therapy and tissue engineering and its potential on the management of congenital heart disease. To date, stem cell therapy has mainly focused on treatment of ischemic heart disease and heart failure, with initial indication of safety and mild-to-moderate efficacy. Preclinical studies and initial clinical trials suggest that the approach could be uniquely suited for the correction of congenital defects of the heart. The basic concept is...

  17. The Rate of Addiction in Parents of Children with Congenital Heart Disease Compared with Healthy Children

    Directory of Open Access Journals (Sweden)

    Tahereh Boryri

    2017-03-01

    Full Text Available BackgroundCongenital heart diseases (CHD are the most common congenital anomaly in children and also the leading cause of mortality from congenital anomalies. Various factors including smoking, drinking alcohol and addiction play role in development of congenital heart diseases. This study was conducted with the aim of investigation of the prevalence of addiction in parents of children with congenital heart disease compared with healthy children.Materials and MethodsThis was a case-control study conducted on 320 children with congenital heart disease aged 6 months to 16 years and 320 healthy children as control group. Children referring to Ali Asghar hospital or who were hospitalized in Imam Ali Hospital were included in the study and their demographic characteristics and their parents were collected. Data were analyzed using SPSS 20.ResultsAverage age of diseased and healthy children was 4.08 ± 4.11 and 3.59 ± 2.36, respectively. The rate of addiction of father, mother and parents of children with congenital heart disease was higher than those of children in control group. The most common congenital heart disease was ventricular septal defect (VSD.ConclusionIn overall, this study showed addiction rate of parents in children with congenital heart disease was higher.

  18. Current diagnosis and treatments for critical congenital heart defects.

    Science.gov (United States)

    Zeng, Zhandong; Zhang, Hongwei; Liu, Fengli; Zhang, Ning

    2016-05-01

    Congenital heart defects (CHD) affect approximately 7% of infants, and account for 3% of all infant deaths. CHD is most often caused by the defects associated with ductus arteriosus, which is a vessel that usually closes shortly after birth. The types of CHD include tetralogy of fallot, hypoplastic left heart syndrome, pulmonary atresia, total anomalous pulmonary venous return, transposition of great arteries, tricuspid atresia and truncus arteriosus. There are some risk factors that can increase the chance of a fetus developing CHD such as prematurity, an existing CHD in a first-degree relative, genetic syndromes, infections in utero, maternal drug consumptions and disorders. CHD is diagnosed is through different techniques including pulse oximetry, echocardiograms and physical exams. In this review, we examined the current incidence of CHD, the risk factors associated with CHD, the current methods of diagnosis and surgical options used to repair the defects.

  19. Growth and nutritional status of children with congenital heart disease.

    Science.gov (United States)

    da Silva, Viviane Martins; de Oliveira Lopes, Marcos Venícios; de Araujo, Thelma Leite

    2007-01-01

    Factors predictive of growth deficit and nutritional status in children with congenital heart disease remain unclear. The objective of this study was to characterize the growth and nutritional status of children with congenital heart disease based on anthropometric measurements and z scores. One hundred and thirty-five children 1 year or younger, who had not undergone surgical correction, were evaluated. The variables studied were sex; age; type of heart disease; length, weight; z scores (length-for-age, weight-for-age, weight-for-length); abdominal, thoracic, and cephalic circumferences; triceps and subscapular skinfold thickness; and birth weight and birth length. The mean age of children in this study was 4.75 +/- 3.75 months and most (66.7%) were male. Mean anthropometric measurements were birth length, 48.6 +/- 2.34 cm; birth weight, 3.11 +/- 0.63 kg; cephalic circumference, 38.51 +/- 3.28 cm; thoracic circumference, 38.65 +/- 3.76 cm; abdominal circumference, 37.96 +/- 3.27 cm; triceps skinfold thickness, 3.69 +/- 1.57 mm; subscapular skinfold thickness, 3.22 +/- 1.34 mm; current length, 57.54 +/- 7.87 cm; and current weight, 4.46 +/- 1.49 kg. Variables significant for malnutrition in logistic regression models were sex, type of heart disease, birth weight, birth length, subscapular thickness, triceps thickness, and cephalic circumference. Nutritional defects were more evident in the case of the weight-for-age index. Boys had greater deterioration in the weight-for-age index, possibly indicating acute malnutrition, and girls had worse values for the height-for-age index, indicating a risk of chronic malnutrition.

  20. Incidence of Congenital Heart Disease: The 9-Year Experience of the Guangdong Registry of Congenital Heart Disease, China

    OpenAIRE

    Yanji Qu; Xiaoqing Liu; Jian Zhuang; Guanchun Chen; Jinzhuang Mai; Xiaoling Guo; Yanqiu Ou; Jimei Chen; Wei Gong; Xiangmin Gao; Yong Wu; Zhiqiang Nie

    2016-01-01

    There are 16.5 million newborns in China annually. However, the incidence of congenital heart disease (CHD) has not been evaluated. In 2004, we launched an active province-wide hospital-based CHD registry in the Guangdong Province of southern China. In this study, we examined the incidence of CHD and its subtypes from 2004 to 2012 and compared our findings to the literature. Our results indicate there is an increasing trend of CHD incidence. The increase in incidence occurred mainly for singl...

  1. Educational achievement among long-term survivors of congenital heart defects: a Danish population-based follow-up study

    DEFF Research Database (Denmark)

    Olsen, Morten; Hjortdal, Vibeke E.; Mortensen, Laust Hvas

    2011-01-01

    Congenital heart defect patients may experience neurodevelopmental impairment. We investigated their educational attainments from basic schooling to higher education.......Congenital heart defect patients may experience neurodevelopmental impairment. We investigated their educational attainments from basic schooling to higher education....

  2. Management of newborns with duct-dependent congenital heart disease with prostaglandin E1 – Slovenian guidelines

    Directory of Open Access Journals (Sweden)

    Mojca Grošelj Grenc

    2012-02-01

    Conclusions: It is critical to start with PGE1 infusion as soon as we clinically suspect ductdependent congenital heart disease. With this approach, morbidity and mortality of newborns with congenital heart disease is significantly reduced.

  3. Diagnosis of complex congenital heart disease: morphologic-anatomic method and terminology.

    Science.gov (United States)

    Van Praagh, R

    1984-01-01

    A summary and brief illustration of the morphologic-anatomic method of diagnosis of congenital heart disease is presented. The principles of scientific neologizing are considered and exemplified. Scientific freedom of speech and expression is commented upon. It is suggested that unnecessary renaming of numerous cardiac anatomic structures and many forms of congenital heart disease be discontinued, and that terminology be de-emphasized.

  4. Aortic valve prosthesis-patient mismatch and exercise capacity in adult patients with congenital heart disease

    NARCIS (Netherlands)

    van Slooten, Ymkje J.; Melle, van Joost P.; Freling, Hendrik G.; Bouma, Berto J.; van Dijk, Arie P. J.; Jongbloed, Monique R. M.; Post, Martijn C.; Sieswerda, Gertjan T.; in 't Veld, Anna Huis; Ebels, Tjark; Voors, Adriaan A.; Pieper, Petronella G.

    Objectives To report the prevalence of aortic valve prosthesis patient mismatch (PPM) in an adult population with congenital heart disease (CHD) and its impact on exercise capacity. Adults with congenital heart disease (ACHD) with a history of aortic valve replacement may outgrow their prosthesis

  5. Congenital heart defects in europe: prevalence and perinatal mortality, 2000 to 2005

    DEFF Research Database (Denmark)

    Dolk, Helen; Loane, Maria; Garne, Ester

    2011-01-01

    This study determines the prevalence of Congenital Heart Defects (CHD), diagnosed prenatally or in infancy, and fetal and perinatal mortality associated with CHD in Europe.......This study determines the prevalence of Congenital Heart Defects (CHD), diagnosed prenatally or in infancy, and fetal and perinatal mortality associated with CHD in Europe....

  6. Increased prevalence of congenital heart defects in monozygotic and dizygotic twins

    DEFF Research Database (Denmark)

    Herskind, Anne Maria; Almind Pedersen, Dorthe; Christensen, Kaare

    2013-01-01

    Smaller studies and many case series reports indicate that congenital heart defects may be more common in monochorionic twins than in dichorionic twins and singletons.......Smaller studies and many case series reports indicate that congenital heart defects may be more common in monochorionic twins than in dichorionic twins and singletons....

  7. Congenital Heart Disease: Guidelines of Care for Children with Special Health Care Needs.

    Science.gov (United States)

    Minnesota State Dept. of Health, Minneapolis. Services for Children with Handicaps.

    These guidelines were written to help families coordinate the health care that may be needed by a child with congenital heart disease. The booklet begins with general information about congenital heart disease. It then discusses the goals of health care, the health care team, the importance of periodic health care, and record keeping procedures.…

  8. Problems in the organization of care for patients with adult congenital heart disease

    NARCIS (Netherlands)

    Meijboom, Folkert; Mulder, Barbara

    2010-01-01

    The prevalence of congenital heart disease among adults in Europe, or in any country in Europe, is not known. This is due to a lack of agreement on the incidence of congenital heart disease, with estimations varying from four per 1000 births to 50 per 1000 births, and it is not known how many

  9. School Children with Congenital Heart Disease: Quality of Life and Policy Implications

    Science.gov (United States)

    Roberts, Jillian; Massie, Kendra; Mortimer, Tamara; Maxwell, Lani

    2005-01-01

    Congenital heart disease is one of the most common pediatric chronic illnesses that can have an impact on the lives of affected children and their families. Despite the growing number of school-age children who live with congenital heart disease, few researchers have investigated the lives and well-being of these children and their families. The…

  10. Turning 18 with congenital heart disease : prediction of infective endocarditis based on a large population

    NARCIS (Netherlands)

    Verheugt, Carianne L.; Uiterwaal, Cuno S. P. M.; van der Velde, Enno T.; Meijboom, Folkert J.; Pieper, Petronella G.; Veen, Gerrit; Stappers, Jan L. M.; Grobbee, Diederick E.; Mulder, Barbara J. M.

    Aims The risk of infective endocarditis (IE) in adults with congenital heart disease is known to be increased, yet empirical risk estimates are lacking. We sought to predict the occurrence of IE in patients with congenital heart disease at the transition from childhood into adulthood. Methods and

  11. Turning 18 with congenital heart disease: prediction of infective endocarditis based on a large population

    NARCIS (Netherlands)

    Verheugt, Carianne L.; Uiterwaal, Cuno S. P. M.; van der Velde, Enno T.; Meijboom, Folkert J.; Pieper, Petronella G.; Veen, Gerrit; Stappers, Jan L. M.; Grobbee, Diederick E.; Mulder, Barbara J. M.

    2011-01-01

    The risk of infective endocarditis (IE) in adults with congenital heart disease is known to be increased, yet empirical risk estimates are lacking. We sought to predict the occurrence of IE in patients with congenital heart disease at the transition from childhood into adulthood. We identified

  12. Pulmonary arterial hypertension associated with congenital heart disease

    Directory of Open Access Journals (Sweden)

    Michele D'Alto

    2012-12-01

    Full Text Available Pulmonary arterial hypertension (PAH is a common complication of congenital heart disease (CHD, with most cases occurring in patients with congenital cardiac shunts. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodelling and dysfunction, resulting in a progressive rise in pulmonary vascular resistance and increased pressures in the right heart. Eventually, reversal of the shunt may arise, with the development of Eisenmenger's syndrome, the most advanced form of PAH-CHD. The prevalence of PAH-CHD has fallen in developed countries over recent years and the number of patients surviving into adulthood has increased markedly. Today, the majority of PAH-CHD patients seen in clinical practice are adults, and many of these individuals have complex disease or received a late diagnosis of their defect. While there have been advances in the management and therapy in recent years, PAH-CHD is a heterogeneous condition and some subgroups, such as those with Down's syndrome, present particular challenges. This article gives an overview of the demographics, pathophysiology and treatment of PAH-CHD and focuses on individuals with Down's syndrome as an important and challenging patient group.

  13. Adolescents and Adults with Congenital Heart Diseases in Oman

    Directory of Open Access Journals (Sweden)

    Asim Al-Balushi

    2015-01-01

    Full Text Available Objectives: The aim of our study was to examine the spectrum, demographics, and mortality rate among adolescents and adults with congenital heart diseases (CHD in Oman. Methods: Data was collected retrospectively from the Royal Hospital, Muscat, electronic health records for all patients with a diagnosis of CHD aged 13 years and above. Data was analyzed according to the type of CHD and in-hospital mortality was assessed using Kaplan-Meier survival analysis. Results: A total of 600 patients with CHD were identified, among them 145 (24% were aged 18 years or below. The median age was 24 years. The majority of patients had a simple form of CHD. Atrial and ventricular septal defects together constituted 62.8% of congenital heart diseases. Most patients were clustered in Muscat (32% and the Batinah regions (31.1% of Oman. Patients with tetralogy of Fallot and Fontan had shorter survival time than recorded in the published literature. Conclusion: Mostly simple forms of CHD in younger patients was observed. The survival rate was significantly shortened in more complex lesions compared to simple lesions. A national data registry for CHD is needed to address the morbidities and mortality associated with the disease.

  14. Intravascular and intracardiac stents used in congenital heart disease.

    Science.gov (United States)

    Okubo, M; Benson, L N

    2001-03-01

    Intravascular or intracardiac stenoses occur in many forms of congenital heart disease or after attempted surgical repair. Although balloon dilation is one option for management, restenosis can occur due to elastic recoil immediately after the procedure. To address to such stenotic lesions, many reports support implanting endovascular stents to provide a framework for vessel expansion. Both balloon-expandable fixed tubular mesh stainless steel devices, and self-expandable stents have had an extensive clinical application. In pediatric patients, stents are used for a variety of stenoses, such as systemic venous obstruction pathways (eg, Mustard, Fontan baffle, or bidirectional cavopulmonary connections), pulmonary artery, right ventricular to pulmonary conduits, aortic coarctation, the arterial duct, aorticopulmonary collaterals, or postoperative systemic to pulmonary shunts. Because of improvements in device profile, implantation rates have increased. Complications such as stent fracture, migration, aneurysm formation, and in-stent restenosis occur but only rarely. This latter event may be because of intimal hyperplasia and/or continued vessel (and patient) growth related to the stent diameter. As such, some instances require redilation to manage the acquired lesion. Stent application has importantly altered management algorithms in congenital heart disease.

  15. Computational modeling and engineering in pediatric and congenital heart disease.

    Science.gov (United States)

    Marsden, Alison L; Feinstein, Jeffrey A

    2015-10-01

    Recent methodological advances in computational simulations are enabling increasingly realistic simulations of hemodynamics and physiology, driving increased clinical utility. We review recent developments in the use of computational simulations in pediatric and congenital heart disease, describe the clinical impact in modeling in single-ventricle patients, and provide an overview of emerging areas. Multiscale modeling combining patient-specific hemodynamics with reduced order (i.e., mathematically and computationally simplified) circulatory models has become the de-facto standard for modeling local hemodynamics and 'global' circulatory physiology. We review recent advances that have enabled faster solutions, discuss new methods (e.g., fluid structure interaction and uncertainty quantification), which lend realism both computationally and clinically to results, highlight novel computationally derived surgical methods for single-ventricle patients, and discuss areas in which modeling has begun to exert its influence including Kawasaki disease, fetal circulation, tetralogy of Fallot (and pulmonary tree), and circulatory support. Computational modeling is emerging as a crucial tool for clinical decision-making and evaluation of novel surgical methods and interventions in pediatric cardiology and beyond. Continued development of modeling methods, with an eye towards clinical needs, will enable clinical adoption in a wide range of pediatric and congenital heart diseases.

  16. [Interventional treatment of tachyarrhythmia in children with congenital heart disease].

    Science.gov (United States)

    Hebe, Joachim; Nürnberg, Jan-Hendrik; Langes, Klaus

    2014-09-01

    In children and adolescents with congenital heart disease (CHD) tachyarrhythmia occurs more frequently compared to patients with otherwise normal hearts. Arrhythmia substrates may be a natural part of certain congenital cardiac malformations or may result from long lasting myocardial deterioration as a result of CHD and/or cardiac surgery. Treatment of tachycardia is more frequently required even in early childhood, as the impact on quality of life, morbidity and mortality is higher due to an often reduced hemodynamic tolerance. Over the past 20 years interventional electrophysiology has been established as the therapy of choice for the majority of chronic or chronically recurrent tachycardia even in children with CHD. The success and risks of treatment are predominantly influenced by the individual expression of the cardiac anomaly and, if surgery has been performed, the highly variant postoperative anatomy. Introduction of 3D electroanatomical mapping systems together with modern cardiac imaging tools have significantly contributed to an improved understanding, particularly in postoperative tachycardia. Despite such progress, success rates are lower and recurrences are more frequent compared to patients without CHD. Complex and often multiple tachycardia courses account for the still limited performance as well as a frequently insufficient lesion formation with the use of radiofrequency current in the hypertrophic and fibrotic myocardium. Electrophysiology in children and adolescents, particularly if CHD is present, represents a highly specialized discipline requiring a high expertise in CHD, CHD surgery and cardiac electrophysiology and is ideally imbedded within an interdisciplinary cardiological and cardiosurgical setting.

  17. Employment characteristics of a complex adult congenital heart disease cohort.

    Science.gov (United States)

    Pickup, L; Gaffey, T; Clift, P; Bowater, S; Thorne, S; Hudsmith, L

    2017-08-01

    Due to advances in surgical techniques and subsequent management, there have been remarkable improvements in the survival of patients with congenital heart disease. In particular, larger numbers of patients with complex disease are now living into adulthood and are entering the workforce. To establish the types of employment complex adult congenital heart disease (ACHD) patients are engaged in, based on the largest cohort of patients with a single-ventricle circulation in the UK. Records of all patients with a univentricular (Fontan) circulation at the Queen Elizabeth Hospital were reviewed. Employment status was categorized according to the Standard Occupational Classification criteria (2010). A total of 210 patient records were reviewed. There was the same proportion of professionals in our cohort compared to the rest of the UK (20% versus 20%). There were greater proportions working in the caring, leisure and other service occupations (15% versus 9%), the elementary occupations (17% versus 11%), sales and customer service occupations (14% versus 8%) and administrative and secretarial occupations (12% versus 11%). The reverse trend was observed for associate professions and technical occupations (7% versus 14%), skilled trades (10% versus 11%), process, plant and machine operatives (3% versus 6%) and managers, directors and senior officials (2% versus 10%). The data show that ACHD patients with a single ventricle are engaged in a diverse range of occupations. It is essential that early education and employment advice are given to this cohort to maximize future employment potential.

  18. Somatic Development in Children with Congenital Heart Defects.

    Science.gov (United States)

    Poryo, Martin; Paes, Laura Antonia; Pickardt, Thomas; Bauer, Ulrike M M; Meyer, Sascha; Wagenpfeil, Stefan; Abdul-Khaliq, Hashim

    2018-01-01

    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. 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. 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. 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. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. [Congenital heart disease mortality in Spain during a 10 year period (2003-2012)].

    Science.gov (United States)

    Pérez-Lescure Picarzo, Javier; Mosquera González, Margarita; Latasa Zamalloa, Pello; Crespo Marcos, David

    2017-07-12

    Congenital heart disease is a major cause of infant mortality in developed countries. In Spain, there are no publications at national level on mortality due to congenital heart disease. The aim of this study is to analyse mortality in infants with congenital heart disease, lethality of different types of congenital heart disease, and their variation over a ten-year period. A retrospective observational study was performed to evaluate mortality rate of children under one year old with congenital heart disease, using the minimum basic data set, from 2003 to 2012. Mortality rate and relative risk of mortality were estimated by Poisson regression. There were 2,970 (4.58%) infant deaths in a population of 64,831 patients with congenital heart disease, with 73.8% of deaths occurring during first week of life. Infant mortality rate in patients with congenital heart disease was 6.23 per 10,000 live births, and remained constant during the ten-year period of the study, representing 18% of total infant mortality rate in Spain. The congenital heart diseases with highest mortality rates were hypoplastic left heart syndrome (41.4%), interruption of aortic arch (20%), and total anomalous pulmonary drainage (16.8%). Atrial septal defect (1%) and pulmonary stenosis (1.1%) showed the lowest mortality rate. Congenital heart disease was a major cause of infant mortality with no variations during the study period. The proportion of infants who died in our study was similar to other similar countries. In spite of current medical advances, some forms of congenital heart disease show very high mortality rates. Copyright © 2017. Publicado por Elsevier España, S.L.U.

  20. Ventricular assist device use in congenital heart disease with a comparison to heart transplant.

    Science.gov (United States)

    Miller, Jacob R; Eghtesady, Pirooz

    2014-09-01

    Despite advances in medical and surgical therapies, some children with congenital heart disease (CHD) are not able to be adequately treated or palliated, leading them to develop progressive heart failure. As these patients progress to end-stage heart failure they pose a unique set of challenges. Heart transplant remains the standard of care; the donor pool, however, remains limited. Following the experience from the adult realm, the pediatric ventricular assist device (VAD) has emerged as a valid treatment option as a bridge to transplant. Due to the infrequent necessity and the uniqueness of each case, the pediatric VAD in the CHD population remains a topic with limited information. Given the experience in the adult realm, we were tasked with reviewing pediatric VADs and their use in patients with CHD and comparing this therapy to heart transplantation when possible.

  1. Genetic counseling in the adult with congenital heart disease: what is the role?

    Science.gov (United States)

    Burchill, Luke; Greenway, Steven; Silversides, Candice K; Mital, Seema

    2011-08-01

    New discoveries using high-resolution methods for detecting genetic aberrations indicate that the genetic contribution to congenital heart disease has been significantly underestimated in the past. DNA diagnostics have become more accessible and genetic test results are increasingly being used to guide clinical management. Adult congenital heart disease specialists seeking to counsel adults with congenital heart disease about the genetic aspects of their condition face the challenge of keeping abreast of new genetic techniques and discoveries. The emphasis of this review is on the genetic basis of structural cardiovascular defects. A framework for identifying adult congenital heart disease patients most likely to benefit from genetic testing is suggested, along with a summary of current techniques for genetic testing. The clinical and ethical challenges associated with genetic counseling are highlighted. Finally, emerging technologies and future directions in genetics and adult congenital heart disease are discussed.

  2. Illustrated Imaging Essay on Congenital Heart Diseases: Multimodality Approach Part III: Cyanotic Heart Diseases and Complex Congenital Anomalies.

    Science.gov (United States)

    Bhat, Venkatraman; Belaval, Vinay; Gadabanahalli, Karthik; Raj, Vimal; Shah, Sejal

    2016-07-01

    From the stand point of radiographic analysis most of the complex cyanotic congenital heart diseases (CHD), can be divided into those associated with decreased or increased pulmonary vascularity. Combination of a specific cardiac configuration and status of lung vasculature in a clinical context allows plain film diagnosis to be predicted in some CHD. Correlation of the position of the cardiac apex in relation to the visceral situs is an important information that can be obtained from the plain film. This information helps in gathering information about the atrio-ventricular, ventricular arterial concordance or discordance. Categorization of the cyanotic heart disease based on vascularity is presented below. Thorough understanding of cardiac anatomy by different imaging methods is essential in understanding and interpreting complex cardiac disease. Basic anatomical details and background for interpretation are provided in the previous parts of this presentation.

  3. Illustrated Imaging Essay on Congenital Heart Diseases: Multimodality Approach Part II: Acyanotic Congenital Heart Disease and Extracardiac Abnormalities.

    Science.gov (United States)

    Bhat, Venkatraman; Belaval, Vinay; Gadabanahalli, Karthik; Raj, Vimal; Shah, Sejal

    2016-06-01

    Acyanotic heart disease constitutes a significant majority of patient who may present with non-cardiac symptoms. Either they are detected incidentally or present with respiratory complaints. Equipped with knowledge of anatomy by echocardiography and radiographic methods described in previous part of this presentation, diagnosis may be confidently attempted. On plain radiography acyanotic congenital heart diseases have variable appearance depending upon severity of disease. Cardiac size, chamber enlargement and pulmonary vascular pattern are key elements. Typically left to right shunts with large volume flow are associated with pulmonary plethora. Plain radiography has an important role in detecting manifestation of pulmonary arterial hypertension. Severe stenosis of pulmonary valve is associated with pulmonary oligemia. Small intra-cardiac shunts and anomalies of coronary arteries generally present with normal cardiac size and pulmonary arterial pattern. Disease spectrum presented in this illustration demands thorough scrutiny of pulmonary, osseous and abdominal abnormalities. This section illustrates some commonly encountered spectrum of acyanotic cardiac disease.

  4. [Perspectives in the management of congenital heart defects in adult patients].

    Science.gov (United States)

    Hartyánszky, István; Varga, Sándor; Havasi, Kálmán; Babik, Barna; Katona, Márta; Bogáts, Gábor

    2015-01-18

    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.

  5. Spectrum of Congenital Heart Diseases in Eastern Nepal: A tertiary care hospital experience

    Directory of Open Access Journals (Sweden)

    Prashant Shah

    2017-01-01

    Full Text Available Background & Objectives: Congenital heart diseases are neglected especially in world’s poorest nations and appear to be ignored and unexplored dimension of health. The exact prevalence and spectrum of congenital heart diseases in Nepal is largely unknown. The aim of this study was to describe the local experience on the magnitude and the pattern of congenital heart disease in order to increase the awareness of the public and health policy makers on its burden in Nepal.Materials & Methods: This is an observational hospital based study carried out in a tertiary care hospital in Eastern Nepal. The duration of this study was from April 2015 to July 2016. The echocardiography reports of all patients clinically suspected of having congenital heart disease were retrieved, and their diagnostic details were extracted. Only patients of day one of life to 14 years of age were included. Congenital heart diseases like bicuspid aortic valve, mitral valve prolapse and various inherited cardiomyopathies were excluded.Results: A total of 330 echocardiograms were performed for clinically suspected congenital heart disease.  The mean age of study population was 22.31±34.08 months with male to female ratio of 1.2:1. 23% of clinically suspected congenital heart disease cases turned out to have normal echocardiography. Acyanotic congenital heart disease was most common (81.5% followed by cyanotic congenital heart disease (14.2% and obstructive congenital heart disease (4.3%. Atrial septal defect was found to be the most common form of acyanotic congenital heart disease (52% which was followed by ventricular septal defect (28.8% and patent ductus arteriosus (14.8%. Tetralogy of Fallot and double outlet right ventricle were the most common form of cyanotic CHD representing 44.4% of all cyanotic patients. Pulmonary stenosis was the most common obstructive congenital heart disease observed in this study population (63.6%. Rarer entities, like d-transposition of great

  6. Association of maternal chronic disease with risk of congenital heart disease in offspring

    Science.gov (United States)

    Chou, Hsin-Hsu; Chiou, Meng-Jiun; Liang, Fu-Wen; Chen, Lea-Hua; Lu, Tsung-Hsueh; Li, Chung-Yi

    2016-01-01

    Background: Information about known risk factors for congenital heart disease is scarce. In this population-based study, we aimed to investigate the relation between maternal chronic disease and congenital heart disease in offspring. Methods: The study cohort consisted of 1 387 650 live births from 2004 to 2010. We identified chronic disease in mothers and mild and severe forms of congenital heart disease in their offspring from Taiwan’s National Health Insurance medical claims. We used multivariable logistic regression analysis to assess the associations of all cases and specific types of congenital heart disease with various maternal chronic diseases. Results: For mothers with the following chronic diseases, the overall prevalence of congenital heart disease in their children was significantly higher than for mothers without these diseases: diabetes mellitus type 1 (adjusted odds ratio [OR] 2.32, 95% confidence interval [CI] 1.66–3.25), diabetes mellitus type 2 (adjusted OR 2.85, 95% CI 2.60–3.12), hypertension (adjusted OR 1.87, 95% CI 1.69–2.07), congenital heart defects (adjusted OR 3.05, 95% CI 2.45–3.80), anemia (adjusted OR 1.31, 95% CI 1.25–1.38), connective tissue disorders (adjusted OR 1.39, 95% CI 1.19–1.62), epilepsy (adjusted OR 1.37, 95% CI 1.08–1.74) and mood disorders (adjusted OR 1.25, 95% CI 1.11–1.41). The same pattern held for mild forms of congenital heart disease. A higher prevalence of severe congenital heart disease was seen only among offspring of mothers with congenital heart defects or type 2 diabetes. Interpretation: The children of women with several kinds of chronic disease appear to be at risk for congenital heart disease. Preconception counselling and optimum treatment of pregnant women with chronic disease would seem prudent. PMID:27729382

  7. Association of maternal chronic disease with risk of congenital heart disease in offspring.

    Science.gov (United States)

    Chou, Hsin-Hsu; Chiou, Meng-Jiun; Liang, Fu-Wen; Chen, Lea-Hua; Lu, Tsung-Hsueh; Li, Chung-Yi

    2016-12-06

    Information about known risk factors for congenital heart disease is scarce. In this population-based study, we aimed to investigate the relation between maternal chronic disease and congenital heart disease in offspring. The study cohort consisted of 1 387 650 live births from 2004 to 2010. We identified chronic disease in mothers and mild and severe forms of congenital heart disease in their offspring from Taiwan's National Health Insurance medical claims. We used multivariable logistic regression analysis to assess the associations of all cases and specific types of congenital heart disease with various maternal chronic diseases. For mothers with the following chronic diseases, the overall prevalence of congenital heart disease in their children was significantly higher than for mothers without these diseases: diabetes mellitus type 1 (adjusted odds ratio [OR] 2.32, 95% confidence interval [CI] 1.66-3.25), diabetes mellitus type 2 (adjusted OR 2.85, 95% CI 2.60-3.12), hypertension (adjusted OR 1.87, 95% CI 1.69-2.07), congenital heart defects (adjusted OR 3.05, 95% CI 2.45-3.80), anemia (adjusted OR 1.31, 95% CI 1.25-1.38), connective tissue disorders (adjusted OR 1.39, 95% CI 1.19-1.62), epilepsy (adjusted OR 1.37, 95% CI 1.08-1.74) and mood disorders (adjusted OR 1.25, 95% CI 1.11-1.41). The same pattern held for mild forms of congenital heart disease. A higher prevalence of severe congenital heart disease was seen only among offspring of mothers with congenital heart defects or type 2 diabetes. The children of women with several kinds of chronic disease appear to be at risk for congenital heart disease. Preconception counselling and optimum treatment of pregnant women with chronic disease would seem prudent. © 2016 Canadian Medical Association or its licensors.

  8. Air travel and adults with cyanotic congenital heart disease.

    Science.gov (United States)

    Harinck, E; Hutter, P A; Hoorntje, T M; Simons, M; Benatar, A A; Fischer, J C; de Bruijn, D; Meijboom, E J

    1996-01-15

    Concern has been expressed that a reduction of partial oxygen pressure during flight in commercial aircraft may induce dangerous hypoxemia in patients with cyanotic congenital heart disease. To evaluate the validity of this concern, the transcutaneous SaO2 was measured in 12 adults with this type of heart disease and 27 control subjects during simulated commercial flights of 1.5 and 7 hours in a hypobaric chamber. Ten of those patients and 6 control subjects also were evaluated during two actual flights of approximately 2.5 hours in a DC-10 and an A-310, respectively. During the prolonged simulated and actual flights, the capillary blood pH, gases, and lactic acid were analyzed in the patients and during one of the actual flights also in the control subjects. During the simulated flights the SaO2 was at all times lower in the patients than in the control subjects. However, the maximal mean actual percentage decrease, as compared with sea level values, did not exceed 8.8% in either patients or control subjects. During the actual flights, this maximal decrease in the patients was 6%. In-flight reduction of the capillary PO2 was considerable in the control subjects but not in the patients. It is our hypothesis that the lack of a significant decrease of the PO2 in the patients might possibly be due to a high concentration of 2.3 diphosphoglycerate in the red cells. The flights had no influence on the capillary blood pH, PCO2, bicarbonate, or lactic acid levels in either patients or control subjects. Atmospheric pressure changes during commercial air travel do not appear to be detrimental to patients with cyanotic congenital heart disease.

  9. Chylothorax in children with congenital heart disease: incidence of thrombosis.

    Science.gov (United States)

    Bauman, M E; Moher, Conrad; Bruce, A K; Kuhle, S; Kaur, Satvinder; Massicotte, M P

    2013-08-01

    Postoperative chylothorax is a frequently encountered pathology occurring in up to 4% of patients undergoing surgery for repair of congenital heart disease. Symptomatic thrombosis is associated with chylothorax and may contribute to its severity and duration. Furthermore, vessel thrombosis resulting in persistent vessel occlusion may impede future treatments, diagnostic studies and cardio-surgical interventions. The objective of this study was to determine the incidence of upper system thrombosis in pediatric congenital heart patients with confirmed chylothorax with ultrasound screening of all patients diagnosed with chylothorax. All pediatric patients with confirmed with chylothorax underwent doppler ultrasound of the upper venous system as per hospital standard. This retrospective cohort study enrolled all children between February 1, 2010-August 2012, post cardiac surgery with confirmed chylothorax to determine the incidence of all thrombosis. There were 1396 children who underwent 1396 cardiac surgical procedures during the study time with 760 undergoing cardiopulmonary bypass. Development of chylothorax occurred in 54 of 1396, 3.9% (95%CI 3.0;5.0) procedures in all children. In those children with chylothorax, 28 of 54 episodes, 51.8% (95%CI 38.9;64.6) had confirmed VTE. The 51.8% incidence in this study demonstrates a 2.6 fold increase in risk of thrombosis compared to 20% in children with heart disease and central venous lines and may result in serious clinical consequences. The contribution of upper venous system thrombosis to chylothorax is unknown. Often, clinical suspicion of chylothorax exists, however the lack of a standardized approach to objective diagnosis results in delayed confirmation. Approaches to therapy either treatment of confirmed thrombosis or prevention of thrombosis in patients with chylothorax require formal evaluation. Future studies are urgently needed. Copyright © 2013 Elsevier Ltd. All rights reserved.

  10. Adult congenital heart disease: experience with the surgical approach

    Directory of Open Access Journals (Sweden)

    Fernando Tadeu Vasconcelos Amaral

    2013-09-01

    Full Text Available OBJECTIVE: To report the institution experience with the surgical treatment of adults with congenital heart disease due to the increasing number of these patients and the need for a better discussion of the subject. METHODS: Retrospective analysis describing demographic data, risk factors and results. RESULTS: 191 patients between 16 and 74 years old were operated on. Primary correction was done in 171 cases, 93 (55% for atrial septal defect repair. Among 20 (12% reoperations, pulmonary valve replacement was done in six cases. The mean intensive care and hospital stay were 2.7 and 8.5 days respectively, significantly greater for the reoperated cases (P=0.001. The mean bypass and clamping times were 68.6 and 44.7 minutes respectively, greater for the reoperated cases (P<0.0001 and P=0.0003 respectively. Hospital mortality was 4.2% and male sex, functional class III-IV and older age at operation were predictive risk factors. Significant complications were more frequent in the reoperated cases (P<0.003, mainly atrial flutter and fibrillation. Among 183 patients discharged, 149 (82% are being followed and atrial flutter and fibrillation are common. The mean functional class value improved significantly after operation (1.66 to 1.11; P<0.0001. The estimated survival was 96.2% in six years. CONCLUSION: Heart surgery in adults with congenital heart disease can be accomplished with low mortality and functional class improvement. Immediate and late complications are frequent. Multicenter studies are important to better characterize this patient population in the country.

  11. Association between perioperative dexmedetomidine and arrhythmias after surgery for congenital heart disease.

    Science.gov (United States)

    Shuplock, Jacqueline M; Smith, Andrew H; Owen, Jill; Van Driest, Sara L; Marshall, Matt; Saville, Benjamin; Xu, Meng; Radbill, Andrew E; Fish, Frank A; Kannankeril, Prince J

    2015-06-01

    Dexmedetomidine is commonly used after congenital heart surgery and may be associated with a decreased incidence of postoperative tachyarrhythmias. Using a large cohort of patients undergoing congenital heart surgery, we examined for an association between dexmedetomidine use in the immediate postoperative period and subsequent arrhythmia development. A total of 1593 surgical procedures for congenital heart disease were performed. Dexmedetomidine was administered in the immediate postoperative period after 468 (29%) surgical procedures. When compared with 1125 controls, the group receiving dexmedetomidine demonstrated significantly fewer tachyarrhythmias (29% versus 38%; Pheart surgery, it may be associated with increased odds of bradyarrhythmias. © 2015 American Heart Association, Inc.

  12. Improving heart disease knowledge and research participation in adults with congenital heart disease (the Health, Education and Access Research Trial: HEART-ACHD).

    Science.gov (United States)

    Valente, Anne Marie; Landzberg, Michael J; Gianola, Ann; Harmon, Amy J; Cook, Stephen; Ting, Jennifer G; Stout, Karen; Kuehl, Karen; Khairy, Paul; Kay, Joseph D; Earing, Michael; Houser, Linda; Broberg, Craig; Milliren, Carly; Opotowsky, Alexander R; Webb, Gary; Verstappen, Amy; Gurvitz, Michelle

    2013-10-09

    The objective of this prospective multi-center study was to evaluate heart disease knowledge within the adult congenital heart disease (ACHD) population, pilot an educational intervention and assess interest in research participation among new patients at ACHD clinics. Many adults with congenital heart disease lack knowledge about their heart condition that may contribute to undesirable outcomes. Patients ≥18 years of age were recruited upon their first presentation to an ACHD clinic and underwent an educational intervention consisting of creation of a personal health information 'passport' and an introduction to web-based resources. Subjects were asked to complete initial and follow-up surveys documenting their perceived knowledge. Nine hundred twenty-two subjects were recruited from 12 ACHD centers, and 520 (57%) completed follow-up surveys. Patients who completed the follow-up survey were more likely to be women, have more education, and have mild heart disease. At follow-up, the ability of the subjects to name their heart condition improved (78% to 83%, p=0.002). Improvements were seen in mean Likert items regarding perceived knowledge of appropriate exercise (pheart rhythm problems or endocarditis (p<0.0001), reasons for cardiac tests (p<0.007), and birth control options and pregnancy safety (p<0.0001). On follow-up, subjects reported a better understanding of medical research (p<0.01), and higher interest in research participation (p<0.003). This joint clinician-patient pilot program will help inform future efforts toward patient education and participation in research with a focus on standardization of protocols for life-long longitudinal follow-up and continued multi-center collaboration in the ACHD population. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  13. Klebsiella oxytoca Endocarditis With Complete Heart Block

    Directory of Open Access Journals (Sweden)

    Saad Ullah MD

    2016-08-01

    Full Text Available Gram-negative bacterial endocarditis causes 5% of all bacterial endocarditis. Among gram-negative bacteria, Klebsiella species are rare causes of native valve endocarditis. Klebsiella oxytoca is an extremely rare subspecies that can infrequently cause endocarditis and is associated with poor outcome. We report a case of Klebsiella oxytoca endocarditis in an elderly man who initially presented with stroke but later developed sepsis and heart block secondary to endocarditis.

  14. The Total Artificial Heart in End-Stage Congenital Heart Disease.

    Science.gov (United States)

    Villa, Chet R; Morales, David L S

    2017-01-01

    The development of durable ventricular assist devices (VADs) has improved mortality rates and quality of life in patients with end stage heart failure. While the use of VADs has increased dramatically in recent years, there is limited experience with VAD implantation in patients with complex congenital heart disease (CHD), despite the fact that the number of patients with end stage CHD has grown due to improvements in surgical and medical care. VAD use has been limited in patients with CHD and end stage heart failure due to anatomic (systemic right ventricle, single ventricle, surgically altered anatomy, valve dysfunction, etc.) and physiologic constraints (diastolic dysfunction). The total artificial heart (TAH), which has right and left sided pumps that can be arranged in a variety of orientations, can accommodate the anatomic variation present in CHD patients. This review provides an overview of the potential use of the TAH in patients with CHD.

  15. The Total Artificial Heart in End-Stage Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Chet R. Villa

    2017-05-01

    Full Text Available The development of durable ventricular assist devices (VADs has improved mortality rates and quality of life in patients with end stage heart failure. While the use of VADs has increased dramatically in recent years, there is limited experience with VAD implantation in patients with complex congenital heart disease (CHD, despite the fact that the number of patients with end stage CHD has grown due to improvements in surgical and medical care. VAD use has been limited in patients with CHD and end stage heart failure due to anatomic (systemic right ventricle, single ventricle, surgically altered anatomy, valve dysfunction, etc. and physiologic constraints (diastolic dysfunction. The total artificial heart (TAH, which has right and left sided pumps that can be arranged in a variety of orientations, can accommodate the anatomic variation present in CHD patients. This review provides an overview of the potential use of the TAH in patients with CHD.

  16. Craniofacial characteristics in unilateral complete cleft lip and palate patients with congenitally missing teeth.

    Science.gov (United States)

    Wu, Ting-Ting; Ko, Ellen Wen-Ching; Chen, Philip Kuo-Ting; Huang, Chiung-Shing

    2013-09-01

    Congenitally missing permanent teeth are common in patients with clefts. This retrospective study was conducted to evaluate the craniofacial characteristics in patients with unilateral complete cleft lip and palate with congenitally missing permanent teeth. A series of 73 consecutive patients with nonsyndromic unilateral complete cleft lip and palate were enrolled. Evaluation of congenitally missing permanent teeth was based on the panoramic films taken from 7 to 11 years of age. The cephalometric films taken around 9 years of age were used to compare the craniofacial morphology in patients with no congenitally missing permanent teeth (n = 20) and 1 (n = 25), 2 (n = 18), and 3 (n = 10) congenitally missing permanent teeth. The Spearman correlation coefficient was used to assess the association of increased numbers of congenitally missing permanent teeth with each cephalometric parameter. Anterior facial height, distance from the maxillary incisor and first molar to the palatal plane, and overjet decreased as the number of congenitally missing permanent teeth increased in patients with unilateral cleft lip and palate. Unilateral cleft lip and palate patients with congenitally missing permanent teeth have a unique craniofacial morphology with a reduced vertical dimension. Copyright © 2013 American Association of Orthodontists. Published by Mosby, Inc. All rights reserved.

  17. Transcriptional atlas of cardiogenesis maps congenital heart disease interactome.

    Science.gov (United States)

    Li, Xing; Martinez-Fernandez, Almudena; Hartjes, Katherine A; Kocher, Jean-Pierre A; Olson, Timothy M; Terzic, Andre; Nelson, Timothy J

    2014-07-01

    Mammalian heart development is built on highly conserved molecular mechanisms with polygenetic perturbations resulting in a spectrum of congenital heart diseases (CHD). However, knowledge of cardiogenic ontogeny that regulates proper cardiogenesis remains largely based on candidate-gene approaches. Mapping the dynamic transcriptional landscape of cardiogenesis from a genomic perspective is essential to integrate the knowledge of heart development into translational applications that accelerate disease discovery efforts toward mechanistic-based treatment strategies. Herein, we designed a time-course transcriptome analysis to investigate the genome-wide dynamic expression landscape of innate murine cardiogenesis ranging from embryonic stem cells to adult cardiac structures. This comprehensive analysis generated temporal and spatial expression profiles, revealed stage-specific gene functions, and mapped the dynamic transcriptome of cardiogenesis to curated pathways. Reconciling known genetic underpinnings of CHD, we deconstructed a disease-centric dynamic interactome encoded within this cardiogenic atlas to identify stage-specific developmental disturbances clustered on regulation of epithelial-to-mesenchymal transition (EMT), BMP signaling, NF-AT signaling, TGFb-dependent EMT, and Notch signaling. Collectively, this cardiogenic transcriptional landscape defines the time-dependent expression of cardiac ontogeny and prioritizes regulatory networks at the interface between health and disease. Copyright © 2014 the American Physiological Society.

  18. Myocardial Factor Revisited: The Importance of Myocardial Fibrosis in Adults with Congenital Heart Disease

    OpenAIRE

    Broberg, Craig S.; Burchill, Luke J.

    2015-01-01

    Pioneers in congenital heart surgery observed that exercise capacity did not return to normal levels despite successful surgical repair, leading some to cite a “myocardial factor” playing a role. They conjectured that residual alterations in myocardial function would be significant for patients’ long-term outlook. In fulfillment of their early observations, today’s adult congenital heart disease (ACHD) population shows well-recognized features of heart failure, even among patients without cle...

  19. Long-Term Outcome of Catheter-Related Arterial Thrombosis in Infants with Congenital Heart Disease.

    Science.gov (United States)

    Rizzi, Mattia; Kroiss, Sabine; Kretschmar, Oliver; Forster, Ishilde; Brotschi, Barbara; Albisetti, Manuela

    2016-03-01

    To investigate the long-term outcome of catheter-related arterial thrombosis in children. Data from clinical and radiologic long-term follow-up of infants with congenital heart disease developing arterial thrombosis following femoral catheterization are presented. Ninety-five infants with radiologically proven arterial thrombosis because of cardiac catheter (n = 52; 55%) or indwelling arterial catheter (n = 43; 45%) were followed for a median time of 23.5 months (IQR 13.3-47.3). Overall, radiologic complete thrombus resolution was observed in 64 (67%), partial resolution in 8 (9%), and no resolution in 23 (24%) infants. Complete resolution was significantly more frequent in infants with indwelling arterial catheter-related thrombosis compared with cardiac catheter-related thrombosis (P = .001). Patients with complete resolution had a significantly lower blood pressure difference and increased ankle-ankle index compared with patients with partial or no resolution (P < .0001). However, symptoms of claudication were present only in 1 case and clinical significant legs growth retardation (≥ 15 mm) was present in 1%. A significant percentage of persistent occlusion is present in children with arterial catheter-related thrombosis on long-term follow-up. In these children, the magnitude of leg growth retardation is small and possibly not clinically relevant. However, in children with congenital heart disease, the high prevalence of persistent arterial occlusion may hamper future diagnostic and/or interventional catheterization. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. Anaesthetic management of patients with congenital heart disease presenting for non-cardiac surgery.

    Directory of Open Access Journals (Sweden)

    Mohindra R

    2002-01-01

    Full Text Available The incidence of congenital heart disease is about one percent of all live births in the United States. Treatment is being performed at a younger age and these children are showing improved survival. It is not unusual for children with congenital heart disease to present for non-cardiac surgery. Their management depends on their age, type of lesion, extent of corrective procedure, the presence of complications and other congenital anomalies. Each patient needs a detailed pre-operative evaluation to understand the abnormal anatomy and physiology, and related anaesthetic implications. No anaesthetic agent is an absolute contraindication, although drugs beneficial for one lesion may be detrimental for another. Regional anaesthesia has also been safely used in children with congenital heart disease. However the anaesthesiologist must have a detailed understanding of the pathophysiology of the lesion and the pharmacology of drugs being used to be able to provide safe anaesthesia for children with congenital heart disease.

  1. [Clinical experiences on correction of congenital heart diseases with robotic technology: a report of 160 cases].

    Science.gov (United States)

    Yang, Ming; Gao, Chang-qing; Xiao, Cang-song; Wang, Gang; Wang, Jia-li; Wu, Yang

    2012-08-28

    To summarize the clinical experiences on correction of congenital heart disease with robotic technology at a single center. Between January 2007 and May 2012, this retrospective study recruited 160 consecutive patients undergoing robotic surgery for congenital heart diseases. There were 74 males and 86 females with a median age of 35 years (range: 11-62). The procedures included secundum-type atrial septal defect repair (n = 130), ostium primum defect repair (n = 1), perimembranous ventricular septal defect repair (n = 21), mitral valve repair for anterior leaflet cleft (n = 7) and mitral valve repair plus left atrial myxoma resection (n = 1). Cardiopulmonary bypass graft was established through cannulation of right femoral artery, vein and right internal jugular vein under the guidance of transesophageal ultrasound. Myocardial protection was performed with cold blood cardioplegic solution or HTK solution and a transthoracic Chitwood clamp was used to occlude ascending aorta. Via three 8-mm ports and one 15-mm port in right chest, the microscopic instruments were manipulated to complete defect closure or mitral valve plasty, utilizing da Vinci S or da Vinci SI robotic system. Echocardiography was performed intraoperatively and at pre-discharge. Routine follow-ups were conducted. The clinical data of operating time, cardiopulmonary bypass time and follow-up examinations were retrospectively analyzed. All cases were treated successfully without a conversion into median sternotomy. No operative mortality or severe surgical complications were observed. Seventy-six cases of secundum-type atrial septal defect were completed on beating heart. The learning curves were noted for operating time of beating heart group and cross clamp time of arrest heart group. No residual shunt, malignant arrhythmia or mitral valve regurgitation was detected on intraoperative or postoperative echocardiography and during a median follow-up period of (29.1 ± 16.3) months. Robotic minimal

  2. Urine β 2-Microglobolin in the Patients with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Noor Mohammad Noori

    2013-06-01

    Full Text Available Background: This study aimed to evaluate the renal tubular function in the patients with congenital heart disease using β2-microglobulin. Methods: In this case-control study, based on oxymetry, the patients with congenital heart disease were divided into two groups of cyanotic (n=20 and acyanotic (n=20. Congenital heart disease was diagnosed by echocardiography. Healthy individuals within the same age and sex groups were used as controls. Na+, β2-micro globulin, creatinine (Cr, and β2-microglobulin/Cr ratio were measured in random urine samples and the results were compared to the same parameters in the control group using Tukey, One-Way ANOVA, and X2 tests. Results: Based on the study results, urine sodium in the patients with cyanotic heart disease was significantly different from that of the controls (P=0.023. The results also revealed a significant difference between the two groups with congenital heart disease regarding urine β2-microglobulin (P=0.045. In addition, the patients with cyanotic heart disease were significantly different from those with acyanotic heart disease and the controls regarding urine β2-micro globulin/Cr ratio (P=0.012 and P=0.026, respectively. Conclusions: The results of this study demonstrated that renal tubular dysfunction began in the patients with congenital heart disease, especially in those with cyanotic congenital heart disease. Besides, early diagnosis before cardiac surgery leads to better control of renal tubular disease.

  3. Pheochromocytoma complicated by cyanotic congenital heart disease: a case report.

    Science.gov (United States)

    Yamamoto, Keiko; Namba, Noriyuki; Kubota, Takuo; Usui, Takeshi; Takahashi, Kunihiko; Kitaoka, Taichi; Fujiwara, Makoto; Hori, Yumiko; Kogaki, Shigetoyo; Oue, Takaharu; Morii, Eiichi; Ozono, Keiichi

    2016-04-01

    Coincidental cyanotic congenital heart disease and pheochromocytoma is uncommon, although some cases have been reported. We describe a girl aged 15 yr and 11 mo with pheochromocytoma and tricuspid atresia treated by performing the Fontan surgery. The patient did not have any specific symptoms of syndrome related to pheochromoytoma or a family history of pheochromocytoma. During cardiac catheterization, her blood pressure increased markedly, and an α-blocker was administered. Catecholamine hypersecretion was observed in the blood and urine, and abdominal computed tomography revealed a tumor in the right adrenal gland. Scintigraphy showed marked accumulation of (123)I-metaiodobenzylguanidine in the tumor, which led to a diagnosis of pheochromocytoma. We did not detect any germline mutations in the RET, VHL, SDHB, SDHD, TMEM127, or MAX genes. This patient had experienced mild systemic hypoxia since birth, which may have contributed to the development of pheochromocytoma.

  4. Mechanical Concepts Applied in Congenital Heart Disease and Cardiac Surgery.

    Science.gov (United States)

    Gerrah, Rabin; Haller, Stephen J; George, Isaac

    2017-06-01

    All biological processes are governed by principles of physics that dictate the pathophysiology and even the treatment of congenital heart diseases. In this review, basic concepts such as flow, pressure, resistance, and velocity are introduced, followed by more complex laws that describe the relationship between these variables and the disease processes. Finally, physical phenomena such as turbulence, steal and runoff phenomenon, and energy loss are discussed. By application of these principles, one can accurately quantify modifications undertaken to treat diseases, for example, the size of a patch that augments a vessel and the angle of an anastomosis to allow a certain flow. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  5. Dental health and management for children with congenital heart disease.

    LENUS (Irish Health Repository)

    FitzGerald, Kirsten

    2010-01-01

    Congenital heart disease (CHD) is one of the most common developmental anomalies. Children with CHD are at increased risk of developing oral disease, and are at increased risk from the systemic effects of oral disease. Recent changes in guidelines related to prophylaxis against infective endocarditis have highlighted the importance of establishing and maintaining oral health for this group of patients. The management of children with CHD can be complex and, unfortunately, many of these children do not receive the care they require. The challenges that these children pose are discussed, and suggestions are made for the appropriate management of these patients and the key role that all those working in primary dental care have to play.

  6. Haploinsufficiency of TAB2 causes congenital heart defects in humans

    DEFF Research Database (Denmark)

    Thienpont, Bernard; Zhang, Litu; Postma, Alex V

    2010-01-01

    Congenital heart defects (CHDs) are the most common major developmental anomalies and the most frequent cause for perinatal mortality, but their etiology remains often obscure. We identified a locus for CHDs on 6q24-q25. Genotype-phenotype correlations in 12 patients carrying a chromosomal deletion...... on 6q delineated a critical 850 kb region on 6q25.1 harboring five genes. Bioinformatics prioritization of candidate genes in this locus for a role in CHDs identified the TGF-beta-activated kinase 1/MAP3K7 binding protein 2 gene (TAB2) as the top-ranking candidate gene. A role for this candidate gene....... To definitively confirm the role of this candidate gene in CHDs, we performed mutation analysis of TAB2 in 402 patients with a CHD, which revealed two evolutionarily conserved missense mutations. Finally, a balanced translocation was identified, cosegregating with familial CHD. Mapping of the breakpoints...

  7. Prepregnancy Diabetes and Offspring Risk of Congenital Heart Disease

    DEFF Research Database (Denmark)

    Øyen, Nina; Diaz, Lars Jorge; Leirgul, Elisabeth

    2016-01-01

    BACKGROUND: Maternal diabetes mellitus is associated with an increased risk of offspring congenital heart defects (CHD); however, the causal mechanism is poorly understood. We further investigated this association in a Danish nationwide cohort. METHODS AND RESULTS: In a national cohort study, we...... pharmacies. Persons with CHD (n=16 325) were assigned to embryologically related cardiac phenotypes. The CHD prevalence in the offspring of mothers with pregestational diabetes mellitus was 318 per 10 000 live births (n=232) in comparison with a baseline risk of 80 per 10 000; the adjusted relative risk...... for CHD was 4.00 (95% confidence interval, 3.51-4.53). The association was not modified by year of birth, maternal age at diabetes onset, or diabetes duration, and CHD risks associated with type 1 (insulin-dependent) and type 2 (insulin-independent) diabetes mellitus did not differ significantly. Persons...

  8. How Do We Define Congenital Heart Defects for Scientific Studies?

    DEFF Research Database (Denmark)

    Garne, Ester; Olsen, Morten Smærup; Johnsen, Søren Paaske

    2011-01-01

    Estimates of the prevalence of congenital heart defects (CHD) have been published over many years and from many regions. As they are based on different definitions of which cases to include in the CHD prevalence, published prevalence estimates vary substantially. With the increasing use...... of echocardiography in neonatal intensive care, a patent ductus arteriosus (PDA) or flow over the atrial septum will often be visible. These findings may be coded as CHD at discharge and in this way falsely increase the CHD prevalence in the population. There are several purposes for which population-based data...... on CHD may be used: etiology, planning of treatment, or obtain information on outcome, including mortality. For etiology studies, it is important to include terminations of pregnancy as well as all births with CHD. For mortality studies in live births, inclusion of preterm born infants with PDA...

  9. Pacing and Defibrillators in Complex Congenital Heart Disease

    Science.gov (United States)

    Chubb, Henry; O’Neill, Mark; Rosenthal, Eric

    2016-01-01

    Device therapy in the complex congenital heart disease (CHD) population is a challenging field. There is a myriad of devices available, but none designed specifically for the CHD patient group, and a scarcity of prospective studies to guide best practice. Baseline cardiac anatomy, prior surgical and interventional procedures, existing tachyarrhythmias and the requirement for future intervention all play a substantial role in decision making. For both pacing systems and implantable cardioverter defibrillators, numerous factors impact on the merits of system location (endovascular versus non-endovascular), lead positioning, device selection and device programming. For those with Fontan circulation and following the atrial switch procedure there are also very specific considerations regarding access and potential complications. This review discusses the published guidelines, device indications and the best available evidence for guidance of device implantation in the complex CHD population. PMID:27403295

  10. Congenital heart disease in adults and its problems

    Directory of Open Access Journals (Sweden)

    Teddy Ontoseno

    2001-10-01

    Full Text Available There were 40 adult congenital heart disease (CHD patients seen in the Cardiology Division during 1 year (February 1993 - February 1994. The most frequently seen defect was atrial septal defect; however there were also cases with patent ductus arteriosus, pulmonary stenosis, ventricular septal defect, and tetralogy of Fallot. Hemodynamic disorder, serious hindrance to education achievement, and occupational threat due to limited physical capabilities as well as malnutrition are some of prominent issues to be closely anticipated. In general the older the patients the more serious hemodynamic disorder they suffer due CHD. It is worth thinking how to improve the quality of life of CHD patients who succeed to live their adult lives and minimize any possible fatal complication risks.

  11. Chromosomal abnormalities in patients with congenital heart disease.

    Science.gov (United States)

    Trevisan, Patrícia; Zen, Tatiana Diehl; Rosa, Rafael Fabiano Machado; Silva, Juliane Nascimento da; Koshiyama, Dayane Bohn; Paskulin, Giorgio Adriano; Zen, Paulo Ricardo Gazzola

    2013-12-01

    Chromosomal abnormalities (CAs) are an important cause of congenital heart disease (CHD). Determine the frequency, types and clinical characteristics of CAs identified in a sample of prospective and consecutive patients with CHD. Our sample consisted of patients with CHD evaluated during their first hospitalization in a cardiac intensive care unit of a pediatric referral hospital in Southern Brazil. All patients underwent clinical and cytogenetic assessment through high-resolution karyotype. CHDs were classified according to Botto et al. Chi-square, Fisher exact test and odds ratio were used in the statistical analysis (p defect. CAs detected through karyotyping are frequent in patients with CHD. Thus, professionals, especially those working in Pediatric Cardiology Services, must be aware of the implications that performing the karyotype can bring to the diagnosis, treatment and prognosis and for genetic counseling of patients and families.

  12. Congenital heart defect in sibs with discordant karyotypes.

    Science.gov (United States)

    Digilio, M C; Marino, B; Canepa, S A; Borzaga, U; Giannotti, A; Dallapiccola, B

    1998-11-02

    Congenital heart defects (CHDs) are genetically heterogeneous, associated with a variety of genetic conditions. Familial aggregation of CHD in patients with and without Down syndrome is rare. We report on the occurrence of concordant CHD in three sets of sibs with discordant karyotypes. In the first family, atrioventricular canal (AVC) was diagnosed in a chromosomally normal child and in his brother with Down syndrome. In the second family, AVC was associated with trisomy 21 in one sib and with trisomy 18 in the other. In the third family, tetralogy of Fallot was present in one patient with Down syndrome and in his nonsyndromic sister. Although the genetic heterogeneity of Down and non-Down CHD is not disputed, a susceptibility to both euploid and aneuploid CHDs could exist, and common predisposing factors could play a role in both conditions.

  13. Dental health and management for children with congenital heart disease.

    LENUS (Irish Health Repository)

    FitzGerald, Kirsten

    2012-02-01

    Congenital heart disease (CHD) is one of the most common developmental anomalies. Children with CHD are at increased risk of developing oral disease, and are at increased risk from the systemic effects of oral disease. Recent changes in guidelines related to prophylaxis against infective endocarditis have highlighted the importance of establishing and maintaining oral health for this group of patients. The management of children with CHD can be complex and, unfortunately, many of these children do not receive the care they require. The challenges that these children pose are discussed, and suggestions are made for the appropriate management of these patients and the key role that all those working in primary dental care have to play.

  14. Correlation between NFATC1 gene polymorphisms and congenital heart disease in children.

    Science.gov (United States)

    Li, C-L; Niu, L; Fu, M-Y; Tian, J; Wang, Q-W; An, X-J

    2017-08-01

    To analyze the links between NFATC1 gene polymorphism and congenital heart disease in children. In the present study, we selected 85 children patients with congenital heart disease who were hospitalized from February 2013 to February 2015 as research subjects (observation group), and 92 healthy subjects as control group. Restriction fragment length polymorphism (RFLP) was used for analysis of NFATC1 gene in samples from each group. The distribution of NFATC1 genotype and allele between the observation group (children with congenital heart disease) and the control group showed no significant difference (p >0.05), but AA, GG genotypes, and allele frequency between pathological samples of children with congenital heart disease and the control group displayed significant difference (p congenital heart disease in observation group also showed a difference, i.e., homozygote (AA, GG) ratio in children with severe congenital heart disease is relatively high. There is a correlation between NFATC1 genes and the incidence of congenital heart disease in children, and a correlation between different genotypes and allele frequency and the incidence of the disease.

  15. The World Society for Pediatric and Congenital Heart Surgery: its mission and history.

    Science.gov (United States)

    Tchervenkov, Christo I; Stellin, Giovanni; Kurosawa, Hiromi; Jacobs, Jeffrey P; Mavroudis, Constantine; Bernier, Pierre-Luc; Maruszewski, Bohdan; Kreutzer, Christian; Cicek, Sertac; Kinsley, Robin H; Nunn, Graham R; Jonas, Richard A

    2009-01-01

    The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) was established in 2006 to assemble pediatric and congenital heart surgeons from all continents and regions of the world and their colleagues from related specialties dealing with pediatric and congenital heart disease. Since its birth, it has held a highly successful inaugural scientific meeting in 2007 in Washington, DC, and a World Summit on Pediatric and Congenital Heart Surgery Services, Education, and Cardiac Care for Children and Adults with Congenital Heart Disease in 2008 in Montreal. It currently has 549 members from 71 countries and in a short period of time has become the largest organization in the world of pediatric and congenital heart surgeons. Its brief history already seems to be a guarantee of a promising future. Projects in the areas of research, training and education, patient care, and community service will allow the Society to reach its goals. By bringing together professionals from every region of the world, the WSPCHS should play a significant role in the improvement of care for children and adults with congenital heart disease around the world.

  16. Congenital heart defects in molecularly proven Kabuki syndrome patients.

    Science.gov (United States)

    Digilio, Maria Cristina; Gnazzo, Maria; Lepri, Francesca; Dentici, Maria Lisa; Pisaneschi, Elisa; Baban, Anwar; Passarelli, Chiara; Capolino, Rossella; Angioni, Adriano; Novelli, Antonio; Marino, Bruno; Dallapiccola, Bruno

    2017-11-01

    The prevalence of congenital heart defects (CHD) in Kabuki syndrome ranges from 28% to 80%. Between January 2012 and December 2015, 28 patients had a molecularly proven diagnosis of Kabuki syndrome. Pathogenic variants in KMT2D (MLL2) were detected in 27 patients, and in KDM6A gene in one. CHD was diagnosed in 19/27 (70%) patients with KMT2D (MLL2) variant, while the single patient with KDM6A change had a normal heart. The anatomic types among patients with CHD included aortic coarctation (4/19 = 21%) alone or associated with an additional CHD, bicuspid aortic valve (4/19 = 21%) alone or associated with an additional CHD, perimembranous subaortic ventricular septal defect (3/19 = 16%), atrial septal defect ostium secundum type (3/19 = 16%), conotruncal heart defects (3/19 = 16%). Additional CHDs diagnosed in single patients included aortic dilatation with mitral anomaly and hypoplastic left heart syndrome. We also reviewed CHDs in patients with a molecular diagnosis of Kabuki syndrome reported in the literature. In conclusion, a CHD is detected in 70% of patients with KMT2D (MLL2) pathogenic variants, most commonly left-sided obstructive lesions, including multiple left-sided obstructions similar to those observed in the spectrum of the Shone complex, and septal defects. Clinical management of Kabuki syndrome should include echocardiogram at the time of diagnosis, with particular attention to left-sided obstructive lesions and mitral anomalies, and annual monitoring for aortic arch dilatation. © 2017 Wiley Periodicals, Inc.

  17. prevalence and types of congenital heart disease in babies born in the city of Khorramabad (2007- 2011)

    OpenAIRE

    azam Mohsenzadeh; sasan saket; shokofeh Ahmadipour; babak Baharvand

    2014-01-01

    Background: Congenital heart disease is the most common congenital malformations in newborns. The aim of this study was to determine the prevalence and types of congenital heart disease in babies born in the city of Khorramabad. Materials and Methods: The present study was a descriptive cross-sectional. Sampling method was census. All babies born in the city of Khorramabad from 2007 to 2011, whom their first echocardiogram was diagnosed with congenital heart disease, were studied. The stud...

  18. Relationship between regulation of signaling pathways in heart development and congenital heart diseases

    Directory of Open Access Journals (Sweden)

    Li WANG

    2013-11-01

    Full Text Available The cardiac lineage arises from the cardiogenic area located in anterior lateral plate mesoderm during gastrula stage. Differentiation of cells in cardiac mesoderm into cardiac progenitor cells was regulated by complex signaling networks involving specific cytokines, inducing signal proteins and the core cardiac transcription factors. Any link of signaling networks influenced by mutation in genetics and changes in environment would lead to a series of congenital heart defects. Therefore, studies of signal transduction mechanism in heart development will give rise to a series of significant theoretical and clinical contributions to the mechanism of development of congenital heart diseases. Recently, it has been proved that a group of early transcription factors, including Nkx2.5, GATA4, Tbx5 and Isl-1, were involved in the complicated signaling networks during heart development. However, the mechanism of most signaling pathways in the networks remains unclear. In the present paper, the recent progresses concerning cardiac progenitor cells, a group of transcription factors, including Nkx2.5, GATA4, Tbx5, Isl-1 and Apelin/APJ pathways, were discussed. DOI: 10.11855/j.issn.0577-7402.2013.11.019

  19. Utilisation of three-dimensional printed heart models for operative planning of complex congenital heart defects.

    Science.gov (United States)

    Olejník, Peter; Nosal, Matej; Havran, Tomas; Furdova, Adriana; Cizmar, Maros; Slabej, Michal; Thurzo, Andrej; Vitovic, Pavol; Klvac, Martin; Acel, Tibor; Masura, Jozef

    2017-01-01

    To evaluate the accuracy of the three-dimensional (3D) printing of cardiovascular structures. To explore whether utilisation of 3D printed heart replicas can improve surgical and catheter interventional planning in patients with complex congenital heart defects. Between December 2014 and November 2015 we fabricated eight cardiovascular models based on computed tomography data in patients with complex spatial anatomical relationships of cardiovascular structures. A Bland-Altman analysis was used to assess the accuracy of 3D printing by comparing dimension measurements at analogous anatomical locations between the printed models and digital imagery data, as well as between printed models and in vivo surgical findings. The contribution of 3D printed heart models for perioperative planning improvement was evaluated in the four most representative patients. Bland-Altman analysis confirmed the high accuracy of 3D cardiovascular printing. Each printed model offered an improved spatial anatomical orientation of cardiovascular structures. Current 3D printers can produce authentic copies of patients` cardiovascular systems from computed tomography data. The use of 3D printed models can facilitate surgical or catheter interventional procedures in patients with complex congenital heart defects due to better preoperative planning and intraoperative orientation.

  20. Heart Transplantation in Congenital Heart Disease: In Whom to Consider and When?

    Science.gov (United States)

    Attenhofer Jost, Christine H.; Schmidt, Dörthe; Huebler, Michael; Balmer, Christian; Noll, Georg; Caduff, Rosmarie; Greutmann, Matthias

    2013-01-01

    Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger's syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future. PMID:23577237

  1. Forkhead box transcription factors in embryonic heart development and congenital heart disease.

    Science.gov (United States)

    Zhu, Hong

    2016-01-01

    Embryonic heart development is a very complicated process regulated precisely by a network composed of many genes and signaling pathways in time and space. Forkhead box (Fox, FOX) proteins are a family of transcription factors characterized by the presence of an evolutionary conserved "forkhead"or "winged-helix" DNA-binding domain and able to organize temporal and spatial gene expression during development. They are involved in a wide variety of cellular processes, such as cell cycle progression, proliferation, differentiation, migration, metabolism and DNA damage response. An abundance of studies in model organisms and systems has established that Foxa2, Foxc1/c2, Foxh1 and Foxm1, Foxos and Foxps are important components of the signaling pathways that instruct cardiogenesis and embryonic heart development, playing paramount roles in heart development. The previous studies also have demonstrated that mutations in some of the forkhead box genes and the aberrant expression of forkhead box gene are heavily implicated in the congenital heart disease (CHD) of humans. This review primarily focuses on the current understanding of heart development regulated by forkhead box transcription factors and molecular genetic mechanisms by which forkhead box factors modulate heart development during embryogenesis and organogenesis. This review also summarizes human CHD related mutations in forkhead box genes as well as the abnormal expression of forkhead box gene, and discusses additional possible regulatory mechanisms of the forkhead box genes during embryonic heart development that warrant further investigation. Copyright © 2015 Elsevier Inc. All rights reserved.

  2. [Prevalence of congenitial kidney and urinary tract anomalies in neonates and infants with congenital heart disease].

    Science.gov (United States)

    Czarniak, Piotr; Kosiak, Wojciech; Chojnicki, Maciej; Król, Ewa; Zurowska, Aleksandra

    2006-01-01

    Congenital heart diseases are the most frequent anomalies and one of the most important reasons of mortality and morbidity in neonates and infants. Coincidence of additional malformation aggravates prognosis. The frequency of incidence of other anomalies accompanying congenital hart diseases is defined as 11 to 45%. Estimation of the frequency of a coincidence of congenital kidney anomalies on the basis of the ultrasound examination (US) in children with congenital heart disease in first year of life. Abdominal US were done in 350 neonates and infants hospitalized in Department of Pediatric Cardiology Medical University of Gdansk in the first twenty-four hours after diagnosis of congenital heart disease. Examinations were performed in 187 male (53%) and 163 female (47%) in average age 54.9 days (range from 1 to 349 days). In examined population congenital kidney anomalies were found in 4.9% of children from which 18% represent hipoplasia, and another 18% dysplasia. 15.3% of examined patients were diagnosed by dilatation of collecting system, whereof 3% mild, 7% moderate, and 5% severe degree. 1. The frequency of incidence of kidney anomalies and collecting system dilatation in the examined population of neonates and infants with congenital heart disease accounts for 20.2%. 2. Abdominal ultrasound screening with carefully examination of urinary tract should be performed in neonates and infants with congenital heart disease.

  3. Home-based interval training increases endurance capacity in adults with complex congenital heart disease.

    Science.gov (United States)

    Sandberg, Camilla; Hedström, Magnus; Wadell, Karin; Dellborg, Mikael; Ahnfelt, Anders; Zetterström, Anna-Klara; Öhrn, Amanda; Johansson, Bengt

    2017-12-04

    The beneficial effects of exercise training in acquired heart failure and coronary artery disease are well known and have been implemented in current treatment guidelines. Knowledge on appropriate exercise training regimes for adults with congenital heart disease is limited, thus further studies are needed. The aim of this study was to examine the effect of home-based interval exercise training on maximal endurance capacity and peak exercise capacity. Randomized controlled trial. Twenty-six adults with complex congenital heart disease were recruited from specialized units for adult congenital heart disease. Patients were randomized to either an intervention group-12 weeks of home-based interval exercise training on a cycle ergometer (n = 16), or a control group (n = 10). The latter was instructed to maintain their habitual physical activities. An incremental cardiopulmonary exercise test and a constant work rate cardiopulmonary exercise test at 75% of peak workload were performed preintervention and postintervention. Twenty-three patients completed the protocol and were followed (intervention n = 13, control n = 10). Postintervention exercise time at constant work rate cardiopulmonary exercise test increased in the intervention group compared to controls (median[range] 12[-4 to 52]min vs 0[-4 to 5]min, P = .001). At incremental cardiopulmonary exercise test, peak VO2 increased 15% within the intervention group (P = .019) compared to 2% within the control group (P = .8). However, in comparison between the groups no difference was found (285[-200 to 535] ml/min vs 17[-380 to 306] ml/min, P = .10). In addition, peak workload at incremental cardiopulmonary exercise test increased in the intervention group compared to controls (20[-10 to 70]W vs 0[-20 to 15]W, P = .003). Home-based interval exercise training increased endurance capacity and peak exercise capacity in adults with complex congenital heart disease. Aerobic endurance might

  4. Calcineurin as a marker of myocardial hypertrophy in children with valvular congenital heart diseases

    Directory of Open Access Journals (Sweden)

    A. V. Kamenshchyk

    2015-06-01

    Full Text Available Aim. To identify interrelations between the calcineurin levels in children with valvular congenital heart diseases without heart failure and the echocardiography parameters of myocardial hypertrophy. Results: It was established the significantly decreased calcineurin level in congenial valvular heart diseases in children as well as an absence of correlations of the left ventricle myocardial mass and index with negative ones to the dimensions of right ventricle in comparison to healthy children of corresponding age. Conclusion: The obtained data testifies the importance of calcineurin system activity in the formation of pathologic myocardial hypertrophy in children with valvular congenital heart diseases and without manifestation of heart failure.

  5. Analysis of regional congenital cardiac surgical outcomes in Florida using the Society of Thoracic Surgeons Congenital Heart Surgery Database.

    Science.gov (United States)

    Jacobs, Jeffrey P; Quintessenza, James A; Burke, Redmond P; Bleiweis, Mark S; Byrne, Barry J; Ceithaml, Eric L; Decampli, William M; Giroud, Jorge M; Perryman, Richard A; Rosenkranz, Eliot R; Wolff, Grace; Posner, Vicki; Steverson, Sue; Blanchard, William B; Schiebler, Gerry L

    2009-08-01

    Florida is the fourth largest state in the United States of America. In 2004, 218,045 live babies were born in Florida, accounting for approximately 1744 new cases of congenital heart disease. We review the initial experience of The Society of Thoracic Surgeons Congenital Heart Surgery Database with a regional outcomes report, namely the Society of Thoracic Surgeons Florida Regional Report. Eight centres in Florida provide services for congenital cardiac surgery. The Children's Medical Services of Florida provide a framework for quality improvement collaboration between centres. All congenital cardiac surgical centres in Florida have voluntarily agreed to submit data to the Society of Thoracic Surgeons Database. The Society of Thoracic Surgeons and Duke Clinical Research Institute prepared a Florida Regional Report to allow detailed regional analysis of outcomes for congenital cardiac surgery. The report of 2007 from the Society of Thoracic Surgeons Congenital Heart Surgery Database includes details of 61,014 operations performed during the 4 year data harvest window, which extended from 2003 through 2006. Of these operations, 6,385 (10.5%) were performed in Florida. Discharge mortality in the data from Florida overall, and from each Florida site, with 95% confidence intervals, is not different from cumulative data from the entire Society of Thoracic Surgeons Database, both for all patients and for patients stratified by complexity. A regional consortium of congenital heart surgery centres in Florida under the framework of the Children's Medical Services has allowed for inter-institutional collaboration with the goal of quality improvement. This experience demonstrates, first, that the database maintained by the Society of Thoracic Surgeons can provide the framework for regional analysis of outcomes, and second, that voluntary regional collaborative efforts permit the pooling of data for such analysis.

  6. The World Database for Pediatric and Congenital Heart Surgery: The Dawn of a New Era of Global Communication and Quality Improvement in Congenital Heart Disease.

    Science.gov (United States)

    St Louis, James D; Kurosawa, Hiromi; Jonas, Richard A; Sandoval, Nestor; Cervantes, Jorge; Tchervenkov, Christo I; Jacobs, Jeffery P; Sakamoto, Kisaburo; Stellin, Giovanni; Kirklin, James K

    2017-09-01

    The World Society for Pediatric and Congenital Heart Surgery was founded with the mission to "promote the highest quality comprehensive cardiac care to all patients with congenital heart disease, from the fetus to the adult, regardless of the patient's economic means, with an emphasis on excellence in teaching, research, and community service." Early on, the Society's members realized that a crucial step in meeting this goal was to establish a global database that would collect vital information, allowing cardiac surgical centers worldwide to benchmark their outcomes and improve the quality of congenital heart disease care. With tireless efforts from all corners of the globe and utilizing the vast experience and invaluable input of multiple international experts, such a platform of global information exchange was created: The World Database for Pediatric and Congenital Heart Disease went live on January 1, 2017. This database has been thoughtfully designed to produce meaningful performance and quality analyses of surgical outcomes extending beyond immediate hospital survival, allowing capture of important morbidities and mortalities for up to 1 year postoperatively. In order to advance the societal mission, this quality improvement program is available free of charge to WSPCHS members. In establishing the World Database, the Society has taken an essential step to further the process of global improvement in care for children with congenital heart disease.

  7. Orthotropic heart transplantation for adult congenital heart disease: a case with heterotaxy and dextrocardia.

    Science.gov (United States)

    Matsuda, Hikaru; Fukushima, Norihide; Ichikawa, Hajime; Sawa, Yoshiki

    2017-01-01

    A 41-year-old male with heterotaxy (left isomerism) and dextrocardia composed by single ventricle, absent inferior vena cava, bilateral superior vena cava (SVC), common atrioventricular valve has received orthotopic heart transplantation (HTx) after long waiting period as Status-1. Reconstructions of bilateral SVC and hepatic vein route were successful without use of prosthetic material, and the donor heart was placed in the left mediastinum. In spite of satisfactory early recovery, the patient expired 4 months after transplantation mainly from fungal infection which developed following humoral rejection. HTx for adult patients with complex congenital heart disease is demanding in technical as well as pre- and post-transplant management, and indication should be critically determined.

  8. Congenital heart disease and rheumatic heart disease in Africa: recent advances and current priorities.

    Science.gov (United States)

    Zühlke, Liesl; Mirabel, Mariana; Marijon, Eloi

    2013-11-01

    Africa has one of the highest prevalence of heart diseases in children and young adults, including congenital heart disease (CHD) and rheumatic heart disease (RHD). We present here an extensive review of recent data from the African continent highlighting key studies and information regarding progress in CHD and RHD since 2005. Main findings include evidence that the CHD burden is underestimated mainly due to the poor outcome of African children with CHD. The interest in primary prevention for RHD has been recently re-emphasised, and new data are available regarding echocardiographic screening for subclinical RHD and initiation of secondary prevention. There is an urgent need for comprehensive service frameworks to improve access and level of care and services for patients, educational programmes to reinforce the importance of prevention and early diagnosis and a relevant research agenda focusing on the African context.

  9. Growth curves in Down syndrome with congenital heart disease

    Directory of Open Access Journals (Sweden)

    Caroline D’Azevedo Sica

    Full Text Available SUMMARY Introduction: To assess dietary habits, nutritional status and food frequency in children and adolescents with Down syndrome (DS and congenital heart disease (CHD. Additionally, we attempted to compare body mass index (BMI classifications according to the World Health Organization (WHO curves and curves developed for individuals with DS. Method: Cross-sectional study including individuals with DS and CHD treated at a referral center for cardiology, aged 2 to 18 years. Weight, height, BMI, total energy and food frequency were measured. Nutritional status was assessed using BMI for age and gender, using curves for evaluation of patients with DS and those set by the WHO. Results: 68 subjects with DS and CHD were evaluated. Atrioventricular septal defect (AVSD was the most common heart disease (52.9%. There were differences in BMI classification between the curves proposed for patients with DS and those proposed by the WHO. There was an association between consumption of vitamin E and polyunsaturated fatty acids. Conclusion: Results showed that individuals with DS are mostly considered normal weight for age, when evaluated using specific curves for DS. Reviews on specific curves for DS would be the recommended practice for health professionals so as to avoid precipitated diagnosis of overweight and/or obesity in this population.

  10. Nutritional challenges and outcomes after surgery for congenital heart disease.

    Science.gov (United States)

    Cabrera, Antonio G; Prodhan, Parthak; Bhutta, Adnan T

    2010-03-01

    Adequate nutrition is crucial in children after surgery for congenital heart disease. We address selected nutritional and caloric requirements for children in the perioperative period before and after cardiac surgery and explore nutritional interdependence with other system functions. Recommendations based on our current practice are made at the end of each section. Early identification of deficient oropharyngeal motor skills and vocal cord dysfunction is crucial to establish enteral nutrition safely and has been demonstrated to improve clinical outcomes. The use of prealbumin as a marker of nutritional state should be accompanied by C-reactive protein given the influence of inflammation on its levels. Insulin infusions may improve outcomes in patients with postoperative hyperglycemia. Trace element abnormalities and early identification of immune-compromised states can aid in reducing morbidity in children after cardiac surgery. Use of feeding protocols and a home surveillance system for hypoplastic left heart syndrome improves outcomes. Adequate nutritional support in children after cardiac surgery is a challenge. Attention to lesion-specific feeding problems, supplementation of trace elements and minerals, and an organized approach to pace, timing, and type of feeding are beneficial.

  11. Growth curves in Down syndrome with congenital heart disease.

    Science.gov (United States)

    Sica, Caroline D'Azevedo; Cesa, Claudia Ciceri; Pellanda, Lucia Campos

    2016-01-01

    To assess dietary habits, nutritional status and food frequency in children and adolescents with Down syndrome (DS) and congenital heart disease (CHD). Additionally, we attempted to compare body mass index (BMI) classifications according to the World Health Organization (WHO) curves and curves developed for individuals with DS. Cross-sectional study including individuals with DS and CHD treated at a referral center for cardiology, aged 2 to 18 years. Weight, height, BMI, total energy and food frequency were measured. Nutritional status was assessed using BMI for age and gender, using curves for evaluation of patients with DS and those set by the WHO. 68 subjects with DS and CHD were evaluated. Atrioventricular septal defect (AVSD) was the most common heart disease (52.9%). There were differences in BMI classification between the curves proposed for patients with DS and those proposed by the WHO. There was an association between consumption of vitamin E and polyunsaturated fatty acids. Results showed that individuals with DS are mostly considered normal weight for age, when evaluated using specific curves for DS. Reviews on specific curves for DS would be the recommended practice for health professionals so as to avoid precipitated diagnosis of overweight and/or obesity in this population.

  12. Role of virtual reality in congenital heart disease.

    Science.gov (United States)

    Ong, Chin Siang; Krishnan, Aravind; Huang, Chen Yu; Spevak, Philip; Vricella, Luca; Hibino, Narutoshi; Garcia, Juan R; Gaur, Lasya

    2018-02-05

    New platforms for patient imaging present opportunities for improved surgical planning in complex congenital heart disease (CHD). Virtual reality (VR) allows for interactive manipulation of high-resolution representations of patient-specific imaging data, as a supplement to traditional 2D visualizations and 3D printed heart models. We present the novel use of VR for the presurgical planning of cardiac surgery in two infants with complex CHD to demonstrate interactive real-time views of complex intra and extracardiac anatomy. The use of VR for cardiac presurgical planning is feasible using existing imaging data. The software was evaluated by both pediatric cardiac surgeons and pediatric cardiologists, and felt to be reliable and operated with a very short learning curve. VR with controller-based interactive capability allows for interactive viewing of 3D models with complex intra and extracardiac anatomy. This serves as a useful complement to traditional preoperative planning methods in terms of its potential for group based collaborative discussion, user defined illustrative views, cost-effectiveness, and facility of use. © 2018 Wiley Periodicals, Inc.

  13. White Matter Volume Predicts Language Development in Congenital Heart Disease.

    Science.gov (United States)

    Rollins, Caitlin K; Asaro, Lisa A; Akhondi-Asl, Alireza; Kussman, Barry D; Rivkin, Michael J; Bellinger, David C; Warfield, Simon K; Wypij, David; Newburger, Jane W; Soul, Janet S

    2017-02-01

    To determine whether brain volume is reduced at 1 year of age and whether these volumes are associated with neurodevelopment in biventricular congenital heart disease (CHD) repaired in infancy. Infants with biventricular CHD (n = 48) underwent brain magnetic resonance imaging (MRI) and neurodevelopmental testing with the Bayley Scales of Infant Development-II and the MacArthur-Bates Communicative Development Inventories at 1 year of age. A multitemplate based probabilistic segmentation algorithm was applied to volumetric MRI data. We compared volumes with those of 13 healthy control infants of comparable ages. In the group with CHD, we measured Spearman correlations between neurodevelopmental outcomes and the residuals from linear regression of the volumes on corrected chronological age at MRI and sex. Compared with controls, infants with CHD had reductions of 54 mL in total brain (P = .009), 40 mL in cerebral white matter (P Development-II scores but did correlate positively with MacArthur-Bates Communicative Development Inventory language development. Infants with biventricular CHD show total brain volume reductions at 1 year of age, driven by differences in cerebral white matter. White matter volume correlates with language development, but not broader developmental indices. These findings suggest that abnormalities in white matter development detected months after corrective heart surgery may contribute to language impairment. ClinicalTrials.gov: NCT00006183. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Hypoalbuminaemia predicts outcome in adult patients with congenital heart disease

    Science.gov (United States)

    Kempny, Aleksander; Diller, Gerhard-Paul; Alonso-Gonzalez, Rafael; Uebing, Anselm; Rafiq, Isma; Li, Wei; Swan, Lorna; Hooper, James; Donovan, Jackie; Wort, Stephen J; Gatzoulis, Michael A; Dimopoulos, Konstantinos

    2015-01-01

    Background In patients with acquired heart failure, hypoalbuminaemia is associated with increased risk of death. The prevalence of hypoproteinaemia and hypoalbuminaemia and their relation to outcome in adult patients with congenital heart disease (ACHD) remains, however, unknown. Methods Data on patients with ACHD who underwent blood testing in our centre within the last 14 years were collected. The relation between laboratory, clinical or demographic parameters at baseline and mortality was assessed using Cox proportional hazards regression analysis. Results A total of 2886 patients with ACHD were included. Mean age was 33.3 years (23.6–44.7) and 50.1% patients were men. Median plasma albumin concentration was 41.0 g/L (38.0–44.0), whereas hypoalbuminaemia (disease complexity, hypoalbuminaemia remained a significant predictor of death. Conclusions Hypoalbuminaemia is common in patients with ACHD and is associated with a threefold increased risk of risk of death. Hypoalbuminaemia, therefore, should be included in risk-stratification algorithms as it may assist management decisions and timing of interventions in the growing ACHD population. PMID:25736048

  15. Prognosis of the Newborns with Congenital Heart Diseases

    Directory of Open Access Journals (Sweden)

    Akbar Molaei

    2015-07-01

    Full Text Available Background: Congenital heart disease (CHD is defined as problems affecting the heart of the fetus. According to previous studies, the incidence rate varies from 4-12 in 1000 live births. This study aimed to evaluate the demographic characteristics, clinical presentations, and findings that have impact on the prognosis of newborns hospitalized in Tabriz children Hospital, northwest of Iran. Methods: This cross-sectional study was conducted on 82 neonates with CHD admitted in Tabriz children Hospital, northwest of Iran from December 2011 to December 2012. Demographic characteristics, main complaints, symptoms, clinical presentations, associated anomalies, diagnoses, and also the correlation between the treatment process and mortality were evaluated. All data were analyzed using SPSS™ version 16 statistical software. Results: A total of 82 neonates with CHD were enrolled. The mean age and birth weight were 8.54 days and 2828.17 grams, respectively. The most common chief complaint was respiratory distress (58 cases, 70.73%. The most common diagnosis was d-transposition of great arteries and the most frequent therapeutic procedure was medical therapy in combination with mechanical ventilation (31 cases, 37.8%. 10 neonates (12.1% had complications while seizure was the most common complication of the study (4 case, 4.87%. Of 82 newborns in this study, 30 patients (36.6% died of which 13 cases (43.33% had DTGA; the most common therapeutic procedure was mechanical ventilation plus medical therapy which was performed in 22 patients (73.33%. In our study, no significant correlation could be observed between age, sex or weight of neonates and final outcome. Conclusion: Mortality of neonates with critical CHD is high. Also, the neonates treated with more invasive methods have higher mortality rates. It is obvious that both early detection and timely management affect ultimate prognosis of these patients. Hence, prenatal (fetal echocardiography and

  16. Heart transplantation in the setting of complex congenital heart disease and physiologic single lung.

    Science.gov (United States)

    Zuckerman, Warren A; Richmond, Marc E; Lee, Teresa M; Bacha, Emile A; Chai, Paul J; Chen, Jonathan M; Addonizio, Linda J

    2015-12-01

    To highlight the success of heart transplantation in patients with complex congenital heart disease and physiologic single lung by providing an update on the world's largest reported cohort. Demographic, perioperative, postoperative, and outcomes data were collected retrospectively on all patients undergoing heart transplant to single lung at Columbia University Medical Center since 1992, and compared with all other patients undergoing transplants performed for single ventricle or tetralogy of Fallot during that time. Twenty-two patients (mean age, 20.6 years; range, 5 months-47 years) underwent heart transplant to single lung. Compared with controls (n = 67), the single lung group had more male patients and a greater proportion of tetralogy compared with single ventricle patients, although the single lung group had fewer post-Fontan patients. Age, weight, and body surface area were similar between the groups as were use of mechanical circulatory support and mechanical ventilation before transplant. Median time to extubation, time on inotropes, and length of stay were similar. There were 3 perioperative deaths, including a patient who died during postoperative day 1 from primary graft failure, likely related to a combination of elevated pulmonary vascular resistance and volume load. There were 5 additional mortalities during intermediate- and long-term follow-up, none of which were related to single-lung physiology. There was no significant survival difference between the groups. In patients with complex congenital heart disease and single lung physiology, heart transplant alone remains an excellent option, with comparable outcomes to patients undergoing transplant with similar cardiac anatomy and dual lung physiology. Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  17. Risk Factors of Congenital Heart Diseases: A Case-Control Study in Northwest Iran

    Directory of Open Access Journals (Sweden)

    Naghavi-Behzad Mohammad

    2013-03-01

    Full Text Available Introduction: Congenital heart diseases are of immense importance and also a high prevalence. Contributing factors to developing these defects have not been abundantly studied. Therefore, the current study was conducted aiming at determining the effective factors on Congenital Heart Disease (CHD in newborn infants of Northwest Iran. Methods: A case-control study was carried out in North-West of Iran from 2002 to 2012 and a total of 473 infants entered the study. Required data were obtained through check lists completed by the information of hospital records and interview with mothers of 267 newborn infants with CHD together with medical records of mothers as the case group, and 206 medical records of healthy infants at the same period all together with those of their mothers as the control group. The obtained data were statistically analyzed using descriptive statistical methods, T-test, Spearman’s correlation coefficient, and Multi-variable Logistic Regression Model (OR with 95% CI, using SPSS.19. In the present study, P value less than 0.05 was considered statistically significant. Results: Based on the results of univariable analyses, the number of previous cesarean sections, past medical history of diseases, gestational age (GA, fetal weight at birth, diastolic blood pressure, fetal heart rate, pulse rate, fetal hemoglobin and hematocrit levels, and fetal head circumference at birth have significant relationship with incidence of congenital abnormalities (P<0.05. Family history, past cesarean sections history, past medical history and GA had significant relationship with CHD incidence. Conclusion: Based on the results of present study, in order to control and reduce the cases of CHD, it is crucial to make proper decisions and implement policies for reducing cesarean cases, lowering consanguineous marriages, providing proper pre-marriage counseling, prompt treatment of mothers’ illnesses, improving pregnancy health care and mothers

  18. Prenatal detection of major congenital heart disease - optimising resources to improve outcomes.

    Science.gov (United States)

    Corcoran, Siobhan; Briggs, Kaleigh; O' Connor, Hugh; Mullers, Sieglinde; Monteith, Cathy; Donnelly, Jennifer; Dicker, Patrick; Franklin, Orla; Malone, Fergal D; Breathnach, Fionnuala M

    2016-08-01

    Congenital heart disease (CHD) is the most common major structural fetal abnormality and the benefits of prenatal detection are well described. The objective of this study was to evaluate the precision of prenatal diagnosis at a single tertiary referral unit over two three year periods (2006, 2007, 2008 and 2010, 2011, 2012), before and after a prenatal screening protocol for CHD was developed to include extended cardiac views, mandatory recall for suboptimal views, and a multidisciplinary Fetal Cardiac clinic was established. There exists a single national centre for paediatric cardiothoracic surgery in Ireland, a situation which facilitates near complete case ascertainment. Surgery records of the National Children's Cardiac Centre were interrogated for all cases of major congenital heart defects requiring surgical intervention in the first six months of life. Minor procedures such as ligation of a patent ductus arteriosus and isolated atrial septal defect repairs were excluded. Analyses of the Fetal Medicine database at the Rotunda Hospital (a stand-alone tertiary level perinatology centre with 8500 deliveries per year) and the mortality data at the Perinatal Pathology department were conducted. The Cochrane-Armitage trend test was used to determine statistical significance in prenatal detection rates over time. 51,822 women delivered during the study period, and the incidence of major congenital heart disease either that underwent surgical intervention or that resulted in perinatal mortality, was 238/51,822 (0.5%). Prenatal detection of major CHD increased from 31% to 91% (p<0.001). Detection of critical duct-dependant lesions rose from 19% to 100%. We attribute the dramatic improvement in prenatal detection rates to the multifaceted changes introduced during the study period. Improved prenatal detection for births that are geographically remote from the National Paediatric Cardiac Centre will require local replication of this prenatal programme. Copyright

  19. Turning 18 with congenital heart disease: prediction of infective endocarditis based on a large population.

    Science.gov (United States)

    Verheugt, Carianne L; Uiterwaal, Cuno S P M; van der Velde, Enno T; Meijboom, Folkert J; Pieper, Petronella G; Veen, Gerrit; Stappers, Jan L M; Grobbee, Diederick E; Mulder, Barbara J M

    2011-08-01

    The risk of infective endocarditis (IE) in adults with congenital heart disease is known to be increased, yet empirical risk estimates are lacking. We sought to predict the occurrence of IE in patients with congenital heart disease at the transition from childhood into adulthood. We identified patients from the CONCOR national registry for adults with congenital heart disease. Potential predictors included patient characteristics, and complications and interventions in childhood. The outcome measure was the occurrence of IE up to the age of 40 and 60. A prediction model was derived using the Cox proportional hazards model and bootstrapping techniques. The model was transformed into a clinically applicable risk score. Of 10 210 patients, 233 (2.3%) developed adult-onset IE during 220 688 patient-years. Predictors of IE were gender, main congenital heart defect, multiple heart defects, and three types of complications in childhood. Up to the age of 40, patients with a low predicted risk (congenital heart disease, the use of six simple clinical parameters can accurately predict patients at relatively low or high risk of IE. After confirmation in other cohorts, application of the prediction model may lead to individually tailored medical surveillance and educational counselling, thus averting IE or enabling timely detection in adult patients with congenital heart disease.

  20. Circulating matrix metalloproteinases and tissue inhibitors of metalloproteinases levels in pediatric patients with congenital heart disease: Relationship to cardiac functions.

    Science.gov (United States)

    Kiliç, Zübeyir; Uçar, Birsen; Özdemir, Gökmen; Colak, Ömer; Bal, Cengiz; Ertuğrul, Türkan

    2014-09-01

    The pathological effects of matrix metalloproteinases and their tissue inhibitors in cardiovascular diseases are of considerable interest. In our study, we aimed to determine and evaluate the potential significance of circulating matrix metalloproteinases-2 and 9, tissue inhibitors of matrix metalloproteinases-1 and 2 levels in four patient subgroup of pediatric cardiology field and expose pathophysiologic differences between these groups. Eighty-seven patients with the diagnosis of congenital heart disease and 47 healthy controls were enrolled in the study. The study group was stratified to 4 subgroups; 14 patients with right ventricular volume overload, 30 patients with left ventricular volume overload, 19 patients with left to right shunt who developed pulmonary hypertension and 24 patients with cyanotic congenital heart disease. For evaluation of the relationships between serum matrix metalloproteinases and their tissue inhibitors levels with cardiac structures and functions; complete blood count, arterial oxygen saturation, detailed echocardiographic measurements (including tissue Doppler) in all patients and hemodynamic parameters of the patients who went to cardiac catheterization were recorded. Serum matrix metalloproteinase levels were determined by ELISA test. Statistical evaluations were performed with SPSS 16.0. For parameters showing normal distribution, comparisons were made with t-test and ANOVA test. However, for parameters without normal distribution, groups were compared with Mann-Whitney U test and Kruskal-Wallis test. We demonstrated that serum tissue inhibitors of matrix metalloproteinases-1 levels of patients with pulmonary hypertension secondary to congenital heart diseases were significantly higher than the patients with left to right shunt without pulmonary hypertension and controls (p<0.01). Although serum matrix metalloproteinases and their tissue inhibitors levels in patients with cyanotic congenital heart diseases and patients with

  1. Personality traits, quality of life, and perceived health in adolescents with congenital heart disease

    NARCIS (Netherlands)

    Rassart, J.; Luyckx, K.; Goossens, E.; Apers, S.; Klimstra, T.A.; Moons, P.

    2013-01-01

    This study investigated how the Big Five personality traits were related to quality of life and perceived health in adolescents with congenital heart disease (CHD). Adolescents with CHD were selected from the database of paediatric and congenital cardiology of the University Hospitals Leuven. A

  2. Prenatal detection of congenital heart diseaseresults of a national screening programme

    NARCIS (Netherlands)

    van Velzen, C. L.; Clur, S. A.; Rijlaarsdam, Meb; Bax, C. J.; Pajkrt, E.; Heymans, M. W.; Bekker, M. N.; Hruda, J.; de Groot, Cjm; Blom, N. A.; Haak, M. C.

    2016-01-01

    ObjectiveCongenital heart disease (CHD) is the most common congenital malformation and causes major morbidity and mortality. Prenatal detection improves the neonatal condition before surgery, resulting in less morbidity and mortality. In the Netherlands a national prenatal screening programme was

  3. The incidence of congenital heart disease: previous findings and perspectives.

    Science.gov (United States)

    Miranović, Vesna

    2014-01-01

    Congenital heart defects (CHD) are the most common of all congenital anomalies, and represent a significant global health problem. Involvement of medical professionals of different profiles has led to drastic changes in survival and quality of life of children with CHD. The motivation for the implementation of the first large population studies on this subject was not only to obtain answers to the question on the level of incidence of CHD, but the harmonization of criteria and protocols for monitoring and treatment of certain defects as well as the planning of medical staff dealing with children with CHD. Data on the incidence varies from 4-10/1000 live births. Fetal echocardiography can have potential impact on decrease of CHD incidence. The increase in incidence may be due to the possibility that children with CHD will grow up and have offsprings. Owing to the progress that has been made, an increasing number of patients experiences adulthood, creating an entirely new and growing population of patients: patients with "adult" CHD. Survivors suffer morbidity resulting from their circulatory abnormalities as well as from medical and surgical therapies they have been subjected to. Application of the achievements of human genome projects will in time lead to drastic changes in the approach to the patients with CHD. Until the time when it is possible, the goal will be further improvement of the existing system of service: networking in a unique, multicenter clinical registry of patients with CHD, as well as upgrading of technical and non-technical conditions for the treatment of patients with CHD. We are in an unprecedented time of change, but are actually at the end of the beginning of making pediatric cardiac care a highly reliable institution.

  4. The incidence of congenital heart disease: Previous findings and perspectives

    Directory of Open Access Journals (Sweden)

    Miranović Vesna

    2014-01-01

    Full Text Available Congenital heart defects (CHD are the most common of all congenital anomalies, and represent a significant global health problem. Involvement of medical professionals of different profiles has led to drastic changes in survival and quality of life of children with CHD. The motivation for the implementation of the first large population studies on this subject was not only to obtain answers to the question on the level of incidence of CHD, but the harmonization of criteria and protocols for monitoring and treatment of certain defects as well as the planning of medical staff dealing with children with CHD. Data on the incidence varies from 4-10/1000 live births. Fetal echocardiography can have potential impact on decrease of CHD incidence. The increase in incidence may be due to the possibility that children with CHD will grow up and have offsprings. Owing to the progress that has been made, an increasing number of patients experiences adulthood, creating an entirely new and growing population of patients: patients with “adult” CHD. Survivors suffer morbidity resulting from their circulatory abnormalities as well as from medical and surgical therapies they have been subjected to. Application of the achievements of human genome projects will in time lead to drastic changes in the approach to the patients with CHD. Until the time when it is possible, the goal will be further improvement of the existing system of service: networking in a unique, multicenter clinical registry of patients with CHD, as well as upgrading of technical and non-technical conditions for the treatment of patients with CHD. We are in an unprecedented time of change, but are actually at the end of the beginning of making pediatric cardiac care a highly reliable institution.

  5. Myocardial Architecture, Mechanics, and Fibrosis in Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Sarah Ghonim

    2017-05-01

    Full Text Available Congenital heart disease (CHD is the most common category of birth defect, affecting 1% of the population and requiring cardiovascular surgery in the first months of life in many patients. Due to advances in congenital cardiovascular surgery and patient management, most children with CHD now survive into adulthood. However, residual and postoperative defects are common resulting in abnormal hemodynamics, which may interact further with scar formation related to surgical procedures. Cardiovascular magnetic resonance (CMR has become an important diagnostic imaging modality in the long-term management of CHD patients. It is the gold standard technique to assess ventricular volumes and systolic function. Besides this, advanced CMR techniques allow the acquisition of more detailed information about myocardial architecture, ventricular mechanics, and fibrosis. The left ventricle (LV and right ventricle have unique myocardial architecture that underpins their mechanics; however, this becomes disorganized under conditions of volume and pressure overload. CMR diffusion tensor imaging is able to interrogate non-invasively the principal alignments of microstructures in the left ventricular wall. Myocardial tissue tagging (displacement encoding using stimulated echoes and feature tracking are CMR techniques that can be used to examine the deformation and strain of the myocardium in CHD, whereas 3D feature tracking can assess the twisting motion of the LV chamber. Late gadolinium enhancement imaging and more recently T1 mapping can help in detecting fibrotic myocardial changes and evolve our understanding of the pathophysiology of CHD patients. This review not only gives an overview about available or emerging CMR techniques for assessing myocardial mechanics and fibrosis but it also describes their clinical value and how they can be used to detect abnormalities in myocardial architecture and mechanics in CHD patients.

  6. Adults with congenital heart disease: patients' knowledge and concerns about inheritance

    NARCIS (Netherlands)

    van Engelen, Klaartje; Baars, Marieke J. H.; van Rongen, Lotte T.; van der Velde, Enno T.; Mulder, Barbara J. M.; Smets, Ellen M. A.

    2011-01-01

    With recent advances in medical and surgical management, most patients with congenital heart disease (CHD) survive to reproductive age. Current guidelines recommend counseling about inheritance and transmission of CHD to offspring. We evaluated whether adult CHD patients recalled having received

  7. Sonocubic fine: new three-dimensional ultrasound software to the screening of congenital heart diseases

    Directory of Open Access Journals (Sweden)

    Edward Araujo Júnior

    2014-09-01

    Full Text Available Congenital heart disease is the most common fetal congenital malformations; however, the prenatal rate detection still is low. The two-dimensional echocardiography is the "gold standard" exam to screening and diagnosis of congenital heart disease during the prenatal; however, this exam is operator-depending and it is realized only in high risk pregnancies. Spatio-temporal image correlation is a three-dimensional ultrasound software that analyses the fetal heart and your connections in the multiplanar and rendering modes; however, spatio-temporal image correlation too is operator-depending and time-consuming. We presenting a new three-dimensional software named Sonocubic fine to the screening of congenital heart disease. This software applies intelligent navigation technology to spatio-temporal image correlation volume datasets to automatically generate nine fetal echocardiography standard views. Thus, this new software tends to be less operator-depending and time-consuming.

  8. Uteroplacental Blood Flow, Cardiac Function, and Pregnancy Outcome in Women With Congenital Heart Disease

    NARCIS (Netherlands)

    Pieper, Petronella G.; Balci, Ali; Aarnoudse, Jan G.; Kampman, Marlies A. M.; Sollie, Krystyna M.; Groen, Henk; Mulder, Barbara J. M.; Oudijk, Martijn A.; Roos-Hesselink, Jolien W.; Cornette, Jerome; van Dijk, Arie P. J.; Spaanderman, Marc E.; Drenthen, Willem; van Veldhuisen, Dirk J.

    2013-01-01

    Background Pregnant women with congenital heart disease (CHD) are susceptible to cardiovascular, obstetric, and offspring complications. In women with CHD, cardiac dysfunction may compromise uteroplacental flow and contribute to the increased incidence of obstetric and offspring events. Methods and

  9. Effects of eHealth physical activity encouragement in adolescents with complex congenital heart disease

    DEFF Research Database (Denmark)

    Klausen, Susanne Hwiid; Andersen, Lars L; Søndergaard, Lars

    2016-01-01

    OBJECTIVE: To assess benefit and harms of adding an eHealth intervention to health education and individual counseling in adolescents with congenital heart disease. DESIGN: Randomized clinical trial. SETTING: Denmark. PATIENTS: A total of 158 adolescents aged 13-16years with no physical activity...... restrictions after repaired complex congenital heart disease. INTERVENTIONS: PReVaiL consisted of individually tailored eHealth encouragement physical activity for 52weeks. All patients received 45min of group-based health education and 15min of individual counseling involving patients' parents. OUTCOMES...... counseling did not affect outcomes among adolescents with congenital heart disease. Our results do not support the use of this eHealth intervention in adolescents with complex congenital heart disease. TRIAL REGISTRATION: Clinical trials.gov identifier: NCT01189981....

  10. Fibrinogen function is impaired in whole blood from patients with cyanotic congenital heart disease

    DEFF Research Database (Denmark)

    Jensen, A S; Johansson, Pär I.; Bochsen, Louise

    2013-01-01

    BACKGROUND: Patients with cyanotic congenital heart disease (CCHD) have haemostatic abnormities associated with bleeding and thrombo-embolic events. The haemostatic abnormalities are not fully understood, but recent studies indicate that elevated haematocrit and fibrinogen function may...

  11. Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease.

    Science.gov (United States)

    Myers, Patrick O; Tissot, Cécile; Beghetti, Maurice

    2014-01-01

    Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease, and is now predominantly among patients with uncorrected left-to-right shunts. A growing population is characterized by persistent or recurrent PAH after surgical or interventional correction of left-to-right shunts; the latter having a worse prognosis than other forms of PAH associated with congenital heart disease. New treatments for PAH have been shown to be effective in improving PAH exercise capacity and hemodynamics, raising the hope for making previously inoperable congenital heart defects operable and shifting the framework for the assessment of operability. This review focuses on current methods for assessing operability in PAH associated with congenital heart disease, and the possibility of "treat-and-repair" vs. "repair-and-treat" strategies for patients with inoperable or borderline PAH.

  12. Can Pediatric Electrophysiologists Safely Perform Electrophysiology Studies on Adults with Congenital Heart Disease?

    OpenAIRE

    Snyder, Christopher S.; Cannon, Bryan; Rosenthal, Geoffrey L.; Moltedo, Jose; Fenrich, Arnold L.

    2007-01-01

    Introduction: Adults with congenital heart disease (ACHD) are known to have arrhythmias. The purpose of this study was to review the safety and efficacy of a pediatric electrophysiologist performing electrophysiology studies (EPS) on ACHD.

  13. Pulse Oximetry During the First 24 Hours as a Screening Tool For Congenital Heart Defects

    Directory of Open Access Journals (Sweden)

    Patriciu Mihaela

    2017-02-01

    Full Text Available Introduction: Although screening for congenital heart defects (CHD relies mainly on antenatal ultrasonography and clinical examination after birth, life-threatening cardiac malformations are often not diagnosed before the patient is discharged.

  14. Improving assessment of congenital heart disease through rapid patient specific modeling.

    Science.gov (United States)

    Gilbert, Kathleen; Farrar, Genevieve; Cowan, Brett; Suinesiaputra, Avan; Occleshaw, Christopher; Pontre, Beau; Perry, James; Hegde, Sanjeet; Omens, Jeffrey; McCulloch, Andrew; Young, Alistair

    2016-08-01

    Congenital heart disease is the most common birth defect, with an incidence of 75 in every 1000 births. As a result of improved interventions, 90% of people with congenital heart disease now survive to adulthood. They must undergo regular imaging to assess their biventricular (left and right ventricular) function. Analysis of the images is problematic due to the large variety of shapes and complex geometry. In this paper we extend a biventricular modeling method to improve the analysis of MR images from congenital heart disease patients. We used a subdivision surface method to create three customizable exemplars, representing common manifestations of anatomy, and incorporated these as priors into an interactive biventricular customization procedure. The CHD-specific priors were tested on 60 cases representing a variety of congenital heart diseases for which the gold standard manual contours were available. The introduction of multiple priors showed a significant decrease in analysis time while maintaining good correlation between the two methods (R2 >.82).

  15. MTRR 66A>G polymorphism in relation to congenital heart defects.

    NARCIS (Netherlands)

    Beijnum, I.M. van; Kouwenberg, M.; Kapusta, L.; Heijer, M. den; Linden, I.J. van der; Daniels, O.; Blom, H.J.

    2006-01-01

    BACKGROUND: Evidence is accumulating that periconceptional folic acid supplementation may prevent congenital heart defects (CHD). The methionine synthase reductase (MTRR) enzyme restores methionine synthase (MTR) enzyme activity and therefore plays an essential role in the folate- and vitamin

  16. A case of successfully managed pregnancy in a patient with complex cyanotic congenital heart disease.

    Science.gov (United States)

    Liu, J Y; Tan, W K; Tan, E L; Tan, J L; Tan, L K

    2017-06-01

    Medical advances have increased survival of patients with congenital heart disease. However, cardiac disease in pregnancy carries significant maternal and fetal risks, posing enormous challenges to obstetricians. Cyanotic congenital heart disease is associated with maternal complications such as arrhythmias, thromboembolic events and death. Fetal complications include small for gestational age, miscarriage and prematurity. Cyanotic congenital heart disease patients who continue their pregnancies require holistic multidisciplinary team care with early and coordinated planning for delivery. Management of such patients include early counseling regarding pregnancy-associated risks, close monitoring of their cardiac function and regular scanning for fetal assessment. Choice of anesthesia for these patients requires meticulous planning to achieve a favorable balance between systemic and pulmonary vascular resistance, ensuring minimal change in right-to-left shunting. We report a case of a successfully managed pregnancy in a patient with complex congenital heart disease and a single ventricle of left ventricle morphology.

  17. Theory of mind deficit in adult patients with congenital heart disease.

    Science.gov (United States)

    Chiavarino, Claudia; Bianchino, Claudia; Brach-Prever, Silvia; Riggi, Chiara; Palumbo, Luigi; Bara, Bruno G; Bosco, Francesca M

    2015-10-01

    This article provides the first assessment of theory of mind, that is, the ability to reason about mental states, in adult patients with congenital heart disease. Patients with congenital heart disease and matched healthy controls were administered classical theory of mind tasks and a semi-structured interview which provides a multidimensional evaluation of theory of mind (Theory of Mind Assessment Scale). The patients with congenital heart disease performed worse than the controls on the Theory of Mind Assessment Scale, whereas they did as well as the control group on the classical theory-of-mind tasks. These findings provide the first evidence that adults with congenital heart disease may display specific impairments in theory of mind. © The Author(s) 2013.

  18. How often is congenital heart disease recognized as a significant comorbidity among hospitalized adults with congenital heart disease?

    Science.gov (United States)

    Robbins, James M; Onukwube, Jennifer; Goudie, Anthony; Collins, R Thomas

    2017-05-15

    Despite frequent life-long hemodynamic and electrophysiologic abnormalities, adults with congenital heart defects (CHDs) are often lost to medical follow-up. Using a cohort of adults with CHD receiving hospital care in Arkansas, we sought to determine how often a CHD is recognized and coded during hospital admissions. Data for this study come from the Agency for Healthcare Research and Quality's Arkansas State Inpatient Database (SID) for years 2004 to 2012. Using unique identifiers that link patients across hospitalizations, we created a cohort of 3973 patients≥18years old with an ICD-9 code for a CHD diagnosis noted at discharge during any hospitalization. These 3973 patients had 19,638 hospitalizations. A CHD was listed as the principal diagnosis in 3% of hospitalizations, a secondary diagnosis in 22%, and no CHD was listed in 75% of hospitalizations. Among patients with a critical CHD, no critical CHD was noted in 69% of hospitalizations. Cardiovascular events (heart failure, arrhythmias, cerebrovascular accidents, embolic event, or death) occurred in 60% of hospitalizations of critical CHD patients wherein no critical CHD was recorded. CHDs are rarely acknowledged during hospitalizations of adults with a known CHD even when cardiovascular events occur. Improved awareness, disclosure and attention to comorbid CHDs among patients and providers may improve hospital management and outcomes of cardiovascular events. Copyright © 2017 Elsevier Ireland Ltd. All rights reserved.

  19. Chronic kidney disease in congenital heart disease patients: a narrative review of evidence.

    Science.gov (United States)

    Morgan, Catherine; Al-Aklabi, Mohammed; Garcia Guerra, Gonzalo

    2015-01-01

    Patients with congenital heart disease have a number of risk factors for the development of chronic kidney disease (CKD). It is well known that CKD has a large negative impact on health outcomes. It is important therefore to consider that patients with congenital heart disease represent a population in whom long-term primary and secondary prevention strategies to reduce CKD occurrence and progression could be instituted and significantly change outcomes. There are currently no clear guidelines for clinicians in terms of renal assessment in the long-term follow up of patients with congenital heart disease. Consolidation of knowledge is critical for generating such guidelines, and hence is the purpose of this view. This review will summarize current knowledge related to CKD in patients with congenital heart disease, to highlight important work that has been done to date and set the stage for further investigation, development of prevention strategies, and re-evaluation of appropriate renal follow-up in patients with congenital heart disease. The literature search was conducted using PubMed and Google Scholar. Current epidemiological evidence suggests that CKD occurs in patients with congenital heart disease at a higher frequency than the general population and is detectable early in follow-up (i.e. during childhood). Best evidence suggests that approximately 30 to 50 % of adult patients with congenital heart disease have significantly impaired renal function. The risk of CKD is higher with cyanotic congenital heart disease but it is also present with non-cyanotic congenital heart disease. Although significant knowledge gaps exist, the sum of the data suggests that patients with congenital heart disease should be followed from an early age for the development of CKD. There is an opportunity to mitigate CKD progression and negative renal outcomes by instituting interventions such as stringent blood pressure control and reduction of proteinuria. There is a need to

  20. Linking the congenital heart surgery databases of the Society of Thoracic Surgeons and the Congenital Heart Surgeons' Society: part 2--lessons learned and implications.

    Science.gov (United States)

    Jacobs, Jeffrey P; Pasquali, Sara K; Austin, Erle; Gaynor, J William; Backer, Carl; Hirsch-Romano, Jennifer C; Williams, William G; Caldarone, Christopher A; McCrindle, Brian W; Graham, Karen E; Dokholyan, Rachel S; Shook, Gregory J; Poteat, Jennifer; Baxi, Maulik V; Karamlou, Tara; Blackstone, Eugene H; Mavroudis, Constantine; Mayer, John E; Jonas, Richard A; Jacobs, Marshall L

    2014-04-01

    A link has been created between the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the Congenital Heart Surgeons' Society Database (CHSS-D). Five matrices have been created that facilitate the automated identification of patients who are potentially eligible for the five active CHSS studies using the STS-CHSD. These matrices are now used to (1) estimate the denominator of patients eligible for CHSS studies and (2) compare "eligible and enrolled patients" to "potentially eligible and not enrolled patients" to assess the generalizability of CHSS studies. The matrices were applied to 40 consenting institutions that participate in both the STS-CHSD and the CHSS to (1) estimate the denominator of patients that are potentially eligible for CHSS studies, (2) estimate the completeness of enrollment of patients eligible for CHSS studies among all CHSS sites, (3) estimate the completeness of enrollment of patients eligible for CHSS studies among those CHSS institutions participating in each CHSS cohort study, and (4) compare "eligible and enrolled patients" to "potentially eligible and not enrolled patients" to assess the generalizability of CHSS studies. The matrices were applied to all participants in the STS-CHSD to identify patients who underwent frequently performed operations and compare "eligible and enrolled patients" to "potentially eligible and not enrolled patients" in following five domains: (1) age at surgery, (2) gender, (3) race, (4) discharge mortality, and (5) postoperative length of stay. Completeness of enrollment was defined as the number of actually enrolled patients divided by the number of patients identified as being potentially eligible for enrollment. For the CHSS Critical Left Ventricular Outflow Tract Study (LVOTO) study, for the Norwood procedure, completeness of enrollment at centers actively participating in the LVOTO study was 34%. For the Norwood operation, discharge mortality was 15% among 227 enrolled patients

  1. Pbx/Meis Deficiencies Demonstrate Multigenetic Origins of Congenital Heart Disease

    OpenAIRE

    Stankunas, Kryn; Shang, Ching; Twu, Karen Y.; Kao, Shih-Chu; Jenkins, Nancy A.; Copeland, Neal G.; Sanyal, Mrinmoy; Selleri, Licia; Cleary, Michael L.; Chang, Ching-Pin

    2008-01-01

    Congenital heart diseases are traditionally considered to be multifactorial in pathogenesis resulting from environmental and genetic interactions that determine penetrance and expressivity within a genetically predisposed family. Recent evidence suggests that genetic contributions have been significantly underestimated. However, single gene defects occur only in a minority of cases, and multigenetic causes of congenital heart diseases have not been fully demonstrated. Here, we show that inter...

  2. Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease

    OpenAIRE

    Myers, Patrick Olivier; Tissot-Daguette, Cécile; Beghetti, Maurice

    2013-01-01

    Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease, and is now predominantly among patients with uncorrected left-to-right shunts. A growing population is characterized by persistent or recurrent PAH after surgical or interventional correction of left-to-right shunts; the latter having a worse prognosis than other forms of PAH associated with congenital heart disease. New treatments for PAH have been shown to be effective in improving PAH exercise capac...

  3. Vascular endothelial growth factor in children with cyanotic and acyanotic and congenital heart disease

    OpenAIRE

    Baghdady, Yasser; Hussein, Yasser; Shehata, Mohamed

    2010-01-01

    Introduction Vascular endothelial growth factor is a potent stimulator of angiogenesis. Children with cyanotic congenital heart disease often experience the development of widespread formation of collateral blood vessels, which may represent a form of abnormal angiogenesis resulting in increased morbidity and mortality. We undertook the present study to determine whether children with cyanotic congenital heart disease have elevated serum levels of vascular endothelial growth factor compared t...

  4. Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease

    Science.gov (United States)

    2013-01-01

    Cardiovascular magnetic resonance (CMR) has taken on an increasingly important role in the diagnostic evaluation and pre-procedural planning for patients with congenital heart disease. This article provides guidelines for the performance of CMR in children and adults with congenital heart disease. The first portion addresses preparation for the examination and safety issues, the second describes the primary techniques used in an examination, and the third provides disease-specific protocols. Variations in practice are highlighted and expert consensus recommendations are provided. Indications and appropriate use criteria for CMR examination are not specifically addressed. PMID:23763839

  5. Bioengineering and Stem Cell Technology in the Treatment of Congenital Heart Disease

    Science.gov (United States)

    Bosman, Alexis; Edel, Michael J.; Blue, Gillian; Dilley, Rodney J.; Harvey, Richard P.; Winlaw, David S.

    2015-01-01

    Congenital heart disease places a significant burden on the individual, family and community despite significant advances in our understanding of aetiology and treatment. Early research in ischaemic heart disease has paved the way for stem cell technology and bioengineering, which promises to improve both structural and functional aspects of disease. Stem cell therapy has demonstrated significant improvements in cardiac function in adults with ischaemic heart disease. This finding, together with promising case studies in the paediatric setting, demonstrates the potential for this treatment in congenital heart disease. Furthermore, induced pluripotent stems cell technology, provides a unique opportunity to address aetiological, as well as therapeutic, aspects of disease. PMID:26239354

  6. Pregnancy in young women with congenital heart disease: Lesion-specific considerations.

    Science.gov (United States)

    Wald, Rachel M; Sermer, Mathew; Colman, Jack M

    2011-05-01

    Young women with heart disease are increasingly being seen in obstetrical referral centres owing, in large part, to the dramatic improvements in survival of young adults with congenital heart disease in recent years. Although pregnancies in most women with heart disease result in favourable outcomes, there are important exceptions that must be recognized. These exceptions pose a significant mortality risk to the mother and/or the fetus. The present article provides a general framework for the classification of congenital heart lesions in pregnant women as well as a detailed lesion-specific review.

  7. Improving the quality of transition and transfer of care in young adults with congenital heart disease.

    Science.gov (United States)

    Everitt, Ian K; Gerardin, Jennifer F; Rodriguez, Fred H; Book, Wendy M

    2017-05-01

    The transition and transfer from pediatric to adult care is becoming increasingly important as improvements in the diagnosis and management of congenital heart disease allow patients to live longer. Transition is a complex and continuous process that requires careful planning. Inadequate transition has adverse effects on patients, their families and healthcare delivery systems. Currently, significant gaps exist in patient care as adolescents transfer to adult care and there are little data to drive the informed management of transition and transfer of care in adolescent congenital heart disease patients. Appropriate congenital heart disease care has been shown to decrease mortality in the adult population. This paper reviews the transition and transfer of care processes and outlines current congenital heart disease specific guidelines in the United States and compares these recommendations to Canadian and European guidelines. It then reviews perceived and real barriers to successful transition and identifies predictors of success during transfer to adult congenital heart disease care. Lastly, it explores how disease-specific markers of outcomes and quality indicators are being utilized to guide transition and transfer of care in other chronic childhood illnesses, and identifies existing knowledge gaps and structural impediments to improving the management of transition and transfer among congenital heart disease patients. © 2017 Wiley Periodicals, Inc.

  8. Addressing sexual health in congenital heart disease: when being the same isn't the same.

    Science.gov (United States)

    Loomba, Rohit S; Aggarwal, Saurabh; Pelech, Andrew N

    2015-01-01

    A larger number of individuals born with congenital heart disease is living into adolescence and young adulthood. With this comes the responsibility to counsel these patients regarding their sexual and reproductive health. This study utilizes representative data from the National Health and Nutrition Examination Survey to compare sexual measures including percentage of that sexually active, age of first sexual activity, number of sexual partners, condom use, and history of sexually transmitted diseases in those with and without congenital heart disease. A total of 1086 patients (1057 without congenital heart disease and 29 with congenital heart disease) were included in this study. Likelihood of being sexually active, age of first sexual intercourse, and condom use did not differ significantly between the two groups after multivariate analysis. Incidence of sexually transmitted disease did not differ between the two groups after multivariate analysis except for genital warts. There are no major differences in sexual measures between those with and without congenital heart disease. The absence of significant differences in sexual measures in those with congenital heart disease compared with the general population places this group of individuals at increased health risk known to occur with pregnancy. © 2014 Wiley Periodicals, Inc.

  9. Heart transplantation in pediatric and congenital heart disease: a single-center experience.

    Science.gov (United States)

    Rungan, Santuri; Finucane, Kirsten; Gentles, Tom; Gibbs, Helen C; Hu, Rong; Ruygrok, Peter N

    2014-04-01

    To describe the indications and outcomes for pediatric patients and patients with congenital heart disease (CHD) undergoing heart transplantation (HT) in New Zealand. A retrospective audit of 253 patients who underwent HT from 1987 to end 2012 was undertaken. Thirty-seven patients were subdivided into two groups, those aged pediatric heart disease (PHD) and those with CHD. Six patients aged heart disease in 3. At follow-up, 11 patients had died. Actuarial survival was 91% at one year and 79% at five years. Of the four patients with a mechanical assist device to bridge, three were transplanted and alive at follow-up. The CHD group comprised 21 (8.3%) patients, median age 25 years (range 6-48) and 19 (90%) were male. At follow-up, three patients had died. Actuarial survival was 95% at one year, 94% at five years, and 85% at ten years. All five patients with pre-HT Fontan circulation were alive a median of eight years following HT. Heart transplantation for carefully selected pediatric patients and patients with CHD can be successfully performed with favorable outcomes in a geographically isolated unit.

  10. Timing of diagnosis affects mortality in critical congenital heart disease.

    Science.gov (United States)

    Eckersley, Luke; Sadler, Lynn; Parry, Emma; Finucane, Kirsten; Gentles, Thomas L

    2016-06-01

    Screening for critical congenital heart disease (CHD) with prenatal ultrasound or postnatal pulse oximetry has the potential to improve outcome. To guide screening recommendations, this study aimed to identify the proportion and outcome of major CHD diagnosed before (early) or after (late) postnatal discharge prior to the introduction of postnatal oximetry screening. A retrospective, population-based review of all major CHD in New Zealand from 2006 to 2010. The timing of diagnosis relative to discharge and to intervention in critical and non-critical cases with intention to treat was determined, as was the relationship of diagnostic timing to mortality at 1 year of age. Late diagnosis occurred in 20% of critical and 51% of non-critical cases. Mortality occurred in 18% of critical vs 8% of non-critical cases. Mortality was lower with an early diagnosis of critical CHD (early diagnosis 16% vs late diagnosis 27%, p=0.04). Isolated critical CHD benefited most from early diagnosis (mortality, early diagnosis 12% vs late diagnosis 29%, p=0.002). Early diagnosis occurred in >90% critical complex CHD and hypoplastic left heart syndrome, 85% d-transposition of the great arteries (d-TGA) and 53% critical left ventricular outflow tract obstruction (LVOTO). Deaths in d-TGA and LVOTO primarily occurred prior to intervention and for d-TGA most often when birth was distant from the cardiac centre. Excess mortality occurs following late diagnosis of critical CHD, and for d-TGA even with early diagnosis if intervention is not immediately available. Antenatal detection retains an important role in reducing mortality related to critical CHD. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  11. Congenital heart disease in Nigerian children: a multicenter echocardiographic study.

    Science.gov (United States)

    Sadoh, Wilson E; Uzodimma, Chinyere C; Daniels, Queennette

    2013-04-01

    Congenital heart disease (CHD) is among the leading causes of morbidity and mortality in childhood. We report on the spectrum of echocardiographically diagnosed CHD from three different centers across Nigeria. Over a period of 42 months, children who were referred for echocardiographic evaluation in the centers located in three large metropolitan cities were consecutively recruited if they were confirmed to have identifiable CHD. Data were collected on age, gender, and types of CHD and analyzed using SPSS 16 (Chicago, Illinois,). A total of 605 children were recruited, their mean age was 2.1 ± 3.5 (range 0-17) years, and 296 (48.9%) were males. Nearly half (42.5%) had echocardiographic diagnosis of their CHD within the first year of life. Only 17% of the diagnoses were made in the neonatal age group. Acyanotic CHDs were more common than the cyanotic heart diseases (82.8% vs 17.2%). The most common CHD was ventricular septal defect (VSD; 46.6%) followed by patent ductus arteriosus (12.1%), atrial septal defect (8.7%), atrioventricular septal defect (8.2%), and tetralogy of Fallot (7.8%). More than half of the VSDs were perimembranous (55.1%) followed by outlet VSD (23.8%), muscular (10.7%), and inlet VSD (9.6%). Only 42 (6.9%) of the entire study population had definitive intervention. The VSD is the most prevalent CHD in Nigerian children. There is increasing awareness, availability, and use of diagnostic facilities as mirrored in the age distribution of the children. However, access to definitive surgery is poor and draws attention to the urgent need for affordable surgical facilities in the country.

  12. Contemporary cardiac surgery for adults with congenital heart disease.

    Science.gov (United States)

    Beurtheret, Sylvain; Tutarel, Oktay; Diller, Gerhard Paul; West, Cathy; Ntalarizou, Evangelia; Resseguier, Noémie; Papaioannou, Vasileios; Jabbour, Richard; Simpkin, Victoria; Bastin, Anthony J; Babu-Narayan, Sonya V; Bonello, Beatrice; Li, Wei; Sethia, Babulal; Uemura, Hideki; Gatzoulis, Michael A; Shore, Darryl

    2017-08-01

    Advances in early management of congenital heart disease (CHD) have led to an exponential growth in adults with CHD (ACHD). Many of these patients require cardiac surgery. This study sought to examine outcome and its predictors for ACHD cardiac surgery. This is an observational cohort study of prospectively collected data on 1090 consecutive adult patients with CHD, undergoing 1130 cardiac operations for CHD at the Royal Brompton Hospital between 2002 and 2011. Early mortality was the primary outcome measure. Midterm to longer-term survival, cumulative incidence of reoperation, other interventions and/or new-onset arrhythmia were secondary outcome measures. Predictors of early/total mortality were identified. Age at surgery was 35±15 years, 53% male, 52.3% were in New York Heart Association (NYHA) class I, 37.2% in class II and 10.4% in class III/IV. Early mortality was 1.77% with independent predictors NYHA class ≥ III, tricuspid annular plane systolic excursion (TAPSE) <15 mm and female gender. Over a mean follow-up of 2.8±2.6 years, 46 patients died. Baseline predictors of total mortality were NYHA class ≥ III, TAPSE <15 mm and non-elective surgery. The number of sternotomies was not independently associated with neither early nor total mortality. At 10 years, probability of survival was 94%. NYHA class among survivors was significantly improved, compared with baseline. Contemporary cardiac surgery for ACHD performed at a single, tertiary reference centre with a multidisciplinary approach is associated with low mortality and improved functional status. Also, our findings emphasise the point that surgery should not be delayed because of reluctance to reoperate only. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  13. Congenital Complete Atrioventricular Block : Clinical and Experimental Studies

    NARCIS (Netherlands)

    Blank, A.C.

    2014-01-01

    Complete atrioventricular block (CAVB) is a cardiac conduction defect wherein the trans¬mission of the normal electrical impulse from the atria to the ventricle is interrupted due to structural or functional impairment of the atrioventricular (AV) conduction system. If CAVB is diagnosed in utero or

  14. Operative techniques in association with arrhythmia surgery in patients with congenital heart disease.

    Science.gov (United States)

    Mavroudis, Constantine; Deal, Barbara; Backer, Carl L; Stewart, Robert D

    2013-01-01

    Arrhythmia surgery in patients with congenital disease is challenged by the range of anatomic variants, arrhythmia types, and intramyocardial scar location. Experimental and clinical studies have elucidated the mechanisms of arrhythmias for accessory connections, atrial fibrillation, atrial reentry tachycardia, nodal reentry tachycardia, focal or automatic atrial tachycardia, and ventricular tachycardia. The surgical and transcatheter possibilities are numerous, and the congenital heart surgeon should have a comprehensive understanding of all arrhythmia types and potential methods of ablation. The purpose of this article is to introduce resternotomy techniques for safe mediastinal reentry and to review operative techniques of arrhythmia surgery in association with congenital heart disease.

  15. Arrhythmias in adult congenital heart disease: the year in review.

    Science.gov (United States)

    Mondésert, Blandine; Abadir, Sylvia; Khairy, Paul

    2013-05-01

    Management of arrhythmias is an integral component of care for adults with congenital heart disease (CHD). Our objective was to highlight the important advances from the year 2012 regarding arrhythmias in adult CHD, with a focus on diagnostic considerations, acute management, catheter ablation, and device therapy. During the course of 2012, Holter studies suggested that routine screening was helpful in guiding the clinical decisions for certain patient subgroups, such as adults with tetralogy of Fallot. Supportive evidence was provided for the common practice of anticoagulation and/or screening for intracardiac thrombosis by transesophageal echocardiography prior to electrically cardioverting atrial tachyarrhythmias. Advances in catheter ablation, particularly robotic magnetic navigation, offer new hope for patients in whom access to arrhythmia substrates is not feasible by standard means. The subcutaneous defibrillator emerged as an innovative solution of great interest to the patients at risk of sudden death in whom transvenous lead implantation is unachievable or contraindicated. Finally, 2012 ended with a major milestone: the establishment of physician certification in adult CHD by the American Board of Medical Specialties. The year 2012 witnessed important advances in the diagnosis and management of arrhythmias in adults with CHD.

  16. The importance of copy number variation in congenital heart disease

    Science.gov (United States)

    Costain, Gregory; Silversides, Candice K; Bassett, Anne S

    2016-01-01

    Congenital heart disease (CHD) is the most common class of major malformations in humans. The historical association with large chromosomal abnormalities foreshadowed the role of submicroscopic rare copy number variations (CNVs) as important genetic causes of CHD. Recent studies have provided robust evidence for these structural variants as genome-wide contributors to all forms of CHD, including CHD that appears isolated without extra-cardiac features. Overall, a CNV-related molecular diagnosis can be made in up to one in eight patients with CHD. These include de novo and inherited variants at established (chromosome 22q11.2), emerging (chromosome 1q21.1), and novel loci across the genome. Variable expression of rare CNVs provides support for the notion of a genetic spectrum of CHD that crosses traditional anatomic classification boundaries. Clinical genetic testing using genome-wide technologies (e.g., chromosomal microarray analysis) is increasingly employed in prenatal, paediatric and adult settings. CNV discoveries in CHD have translated to changes to clinical management, prognostication and genetic counselling. The convergence of findings at individual gene and at pathway levels is shedding light on the mechanisms that govern human cardiac morphogenesis. These clinical and research advances are helping to inform whole-genome sequencing, the next logical step in delineating the genetic architecture of CHD. PMID:28706735

  17. Genetic testing in congenital heart disease: A clinical approach

    Science.gov (United States)

    Chaix, Marie A; Andelfinger, Gregor; Khairy, Paul

    2016-01-01

    Congenital heart disease (CHD) is the most common type of birth defect. Traditionally, a polygenic model defined by the interaction of multiple genes and environmental factors was hypothesized to account for different forms of CHD. It is now understood that the contribution of genetics to CHD extends beyond a single unified paradigm. For example, monogenic models and chromosomal abnormalities have been associated with various syndromic and non-syndromic forms of CHD. In such instances, genetic investigation and testing may potentially play an important role in clinical care. A family tree with a detailed phenotypic description serves as the initial screening tool to identify potentially inherited defects and to guide further genetic investigation. The selection of a genetic test is contingent upon the particular diagnostic hypothesis generated by clinical examination. Genetic investigation in CHD may carry the potential to improve prognosis by yielding valuable information with regards to personalized medical care, confidence in the clinical diagnosis, and/or targeted patient follow-up. Moreover, genetic assessment may serve as a tool to predict recurrence risk, define the pattern of inheritance within a family, and evaluate the need for further family screening. In some circumstances, prenatal or preimplantation genetic screening could identify fetuses or embryos at high risk for CHD. Although genetics may appear to constitute a highly specialized sector of cardiology, basic knowledge regarding inheritance patterns, recurrence risks, and available screening and diagnostic tools, including their strengths and limitations, could assist the treating physician in providing sound counsel. PMID:26981213

  18. Congenital heart disease in the 21st century.

    Science.gov (United States)

    Winlaw, David

    2007-09-01

    Paediatric cardiac surgery is now a mature specialty, yielding good results for those born with congenital heart disease (CHD). The current status of this surgery is considered, highlighting progress in genetic studies, improvements in intensive care management, and contemporary management of the low cardiac output syndrome. Emerging issues include the neurodevelopmental status of patients undergoing cardiac surgery, and known issues with the Fontan circulation. A framework for considering adults with CHD is described, including a review of common procedures and expected outcomes. Similarities and differences between routine adult cardiac intensive care and care of adults with CHD are also discussed. Some patients classed as the "successes" of paediatric cardiac programs, as well as those with known persisting problems, need close follow-up in adult facilities. There is every indication that significant numbers of patients with complex disease are now entering a phase of life when late complications may present. Some overlap in experience between paediatric and adult care settings is invaluable in providing optimal care.

  19. Congenital heart disease: A primer for the pediatric neuropsychologist.

    Science.gov (United States)

    Cassidy, Adam R; Ilardi, Dawn; Bowen, Susan R; Hampton, Lyla E; Heinrich, Kimberley P; Loman, Michelle M; Sanz, Jacqueline H; Wolfe, Kelly R

    2017-09-06

    Congenital heart disease (CHD) affects millions of people worldwide, including over one million children in the United States. Approximately 25% of children born with CHD require intensive surgical intervention within the first year of life. Despite improved rates of survival into adulthood - rates that exceed 90% in the modern era - children and adolescents with CHD remain at risk for neurological injury and a range of neurobehavioral and psychosocial challenges that pose a threat to quality of life across the lifespan. Consequently, as experts in both clinical psychology and brain development, neuropsychologists are becoming increasingly involved in cardiac follow-up and monitoring to promote optimal developmental outcomes. The primary objective of this paper is to provide an evidence-based, clinically-oriented primer on CHD for pediatric neuropsychologists working with this growing population of survivors. Following an introduction to current standard-of-care guidelines for managing children and adolescents with CHD, we present an overview of brain development within the context of CHD, review neuropsychological outcomes, examine factors influencing variability in outcomes, and discuss implications and strategies for clinical assessment.

  20. Critical Congenital Heart Disease Newborn Screening Implementation: Lessons Learned.

    Science.gov (United States)

    McClain, Monica R; Hokanson, John S; Grazel, Regina; Van Naarden Braun, Kim; Garg, Lorraine F; Morris, Michelle R; Moline, Kathleen; Urquhart, Keri; Nance, Amy; Randall, Harper; Sontag, Marci K

    2017-06-01

    Introduction The purpose of this article is to present the collective experiences of six federally-funded critical congenital heart disease (CCHD) newborn screening implementation projects to assist federal and state policy makers and public health to implement CCHD screening. Methods A qualitative assessment and summary from six demonstration project grantees and other state representatives involved in the implementation of CCHD screening programs are presented in the following areas: legislation, provider and family education, screening algorithms and interpretation, data collection and quality improvement, telemedicine, home and rural births, and neonatal intensive care unit populations. Results The most common challenges to implementation include: lack of uniform legislative and statutory mandates for screening programs, lack of funding/resources, difficulty in screening algorithm interpretation, limited availability of pediatric echocardiography, and integrating data collection and reporting with existing newborn screening systems. Identified solutions include: programs should consider integrating third party insurers and other partners early in the legislative/statutory process; development of visual tools and language modification to assist in the interpretation of algorithms, training programs for adult sonographers to perform neonatal echocardiography, building upon existing newborn screening systems, and using automated data transfer mechanisms. Discussion Continued and expanded surveillance, research, prevention and education efforts are needed to inform screening programs, with an aim to reduce morbidity, mortality and other adverse consequences for individuals and families affected by CCHD.

  1. Energy metabolism in infants with congenital heart disease.

    Science.gov (United States)

    Nydegger, Andreas; Bines, Julie E

    2006-01-01

    Failure to thrive is common in children with congenital heart disease and influences the metabolic response to injury and outcome after corrective cardiac surgery. Energy imbalance is a major contributing factor. However, the published literature is difficult to interpret as studies generally involve small patient numbers with a diverse range of types and severity of cardiac lesions and genetic and/or prenatal factors. The age and time of corrective surgery affects the potential for nutritional recovery. Although the immediate postoperative period is characterized by a hypermetabolic state, low total and resting energy expenditure are reported within 24 h of surgery. After 5 d, resting energy expenditure returns to preoperative levels. Significant improvements in weight and growth occur within months after corrective surgery. However, limited postoperative recovery in nutritional status and growth occurs in infants with a low birth weight, intellectual deficit, or residual malformation. Further studies are needed to inform the timing of corrective cardiac surgery to maximize nutritional outcomes and to identify those infants who may benefit from aggressive preoperative nutrition support.

  2. Risk factors of different congenital heart defects in Guangdong, China.

    Science.gov (United States)

    Ou, Yanqiu; Mai, Jinzhuang; Zhuang, Jian; Liu, Xiaoqing; Wu, Yong; Gao, Xiangmin; Nie, Zhiqiang; Qu, Yanji; Chen, Jimei; Kielb, Christine; Lauper, Ursula; Lin, Shao

    2016-04-01

    Limited studies have evaluated the risk factors for congenital heart defects (CHDs) in China and compared them for different types of CHDs. This study examined risk factors between isolated and multiple CHDs as well as among CHDs subtypes in Guangdong, Southern China. This population-based case-control study included 4,034 pairs of case and control infants enrolled in the Guangdong Registry of CHD study, 2004-2013. Multivariate logistic regression was used to compute adjusted odds ratios (ORs) while simultaneously controlling for confounders. Multiple maternal environmental exposures, including living in newly renovated rooms, residential proximity to main traffic, paternal smoking, and maternal occupation as manual worker, were significantly associated with CHDs with ORs ranging 1.30-9.43. Maternal perinatal diseases (including maternal fever, diabetes, influenza, and threatened abortion), maternal medication use (antibiotic use), advanced maternal age, low socioeconomic status, and paternal alcohol intake were also significantly associated with CHDs, with ORs ranging 1.60-3.96. Isolated CHDs and multiple defects have different profiles of risk factors, while subtype of CHD shares common risk factors. These results suggest that maternal environmental exposures/occupation and perinatal diseases/medication use were dominant risk factors associated with CHDs in Southern China. Isolated and multiple CHDs may have different etiologic factors.

  3. Muscle function in adults with congenital heart disease.

    Science.gov (United States)

    Kröönström, Linda Ashman; Johansson, Linda; Zetterström, Anna-Klara; Dellborg, Mikael; Eriksson, Peter; Cider, Åsa

    2014-01-01

    The aim was to assess muscle function in a sample of Swedish adult men and women with congenital heart disease (ACHD) and to compare the results with published reference values in healthy adults. From April 2009 to December 2010, 762 adult outpatients were assessed for their suitability and individual need for tests of physical fitness. The patients performed five muscle function tests, two isotonic tests and three isometric tests. Of the 762 patients, 315 (41.3%) patients performed the tests. Patients with ACHD had lower isotonic muscle function compared to healthy reference values. In the heel lift test, men with ACHD performed at 63% and women at 58% of the healthy reference values and in the shoulder flexion test the corresponding performance level was 60% for men with ACHD and 85% for the women. Multiple regression analyses showed that NYHA class II-IV was a significant predictor for a lower isotonic muscle function i.e. heel lift in women (pfunction in a broad and unselected group of patients with ACHD. Our data shows that patients with ACHD have lower isotonic muscle function. The impacts of low muscle function in activities of daily living and the question of whether muscle function could be improved with exercise training need further investigation. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

  4. Congenital Heart Disease in Adolescents With Gluteal Muscle Contracture

    Science.gov (United States)

    You, Tian; Zhang, Xin-tao; Zha, Zhen-gang; Zhang, Wen-tao

    2015-01-01

    Abstract Gluteal muscle contracture (GMC), presented with hip abduction and external rotation when crouching, is common in several ethnicities, particularly in Chinese. It remains unclear that the reasons why these children are weak and have no choice to accept repeated intramuscular injection. Here, we found some unique cases which may be useful to explain this question. We describe a series of special GMC patients, who are accompanied with congenital heart disease (CHD). These cases were first observed in preoperative examinations of a patient with atrial septal defect (ASD), which was proved by chest X-ray and cardiac ultrasound. From then on, we gradually identified additional 3 GMC patients with CHD. The original patient with ASD was sent to cardiosurgery department to repair atrial septal first and received arthroscopic surgery later. While the other 3 were cured postoperative of ventricular septal defect (VSD), tetralogy of fallot (TOF), patent ductus arteriosus (PDA), respectively, and had surgery directly. The study gives us 3 proposals: (1) as to CHD children, it is essential to decrease the use of intramuscular injection, (2) paying more attention to cardiac examination especially cardiac ultrasound in perioperative period, and (3) taking 3D-CT to reconstruct gluteal muscles for observing contracture bands clearly in preoperation. However, more larger series of patients are called for to confirm these findings. PMID:25654394

  5. Congenital heart disease at Laos Children's Hospital: Two year experience.

    Science.gov (United States)

    Hwang, In-Chang; Sisavanh, Malouny; Billamay, Somxay; Phangmanixay, Sommanikhone; Oudavong, Bounleua; Kang, Jeehoon; Kwon, Bo Sang; Kim, Gi Beom; Bae, Eun Jung; Noh, Chung Il; Choi, Jung Yun

    2017-03-01

    Management of congenital heart disease (CHD) in developing countries is challenging because of limited access to health-care facilities and socioeconomic limitations. The aim of this study was to describe the recent experience with CHD at Children's Hospital, Vientiane, Laos, the only pediatric referral hospital in the country. From July 2013 to November 2015, 1009 echocardiograms were carried out in 797 individuals who visited Children's Hospital, in whom CHD was identified in 213. Demographic characteristics, echocardiography and age at first CHD diagnosis on echocardiogram were compared by residential area. Among the 213 patients, the most frequent anomalies, in descending order, were ventricular septal defect, atrial septal defect, patent ductus arteriosus, and tetralogy of Fallot. Moderate or severe CHD requiring surgery or intervention was detected in 137 patients; median age at initial diagnosis was 6.0 months (IQR, 1.5-29.8). Among those with moderate or severe CHD, 89 patients were from rural areas and 48 from the capital area. The proportion of patients from rural areas older than 1 and 3 years at diagnosis was 46.1% and 32.6%, respectively. In contrast, patients from the capital area were diagnosed at a significantly earlier age: 16.7% at age >1 year and 6.2% at age >3 years (both P Laos, especially in rural areas. Better education and training of local medical staff, and greater access to medical advice are required to improve CHD care in Laos. © 2016 Japan Pediatric Society.

  6. Congenital heart disease affects cerebral size but not brain growth.

    Science.gov (United States)

    Ortinau, Cynthia; Inder, Terrie; Lambeth, Jennifer; Wallendorf, Michael; Finucane, Kirsten; Beca, John

    2012-10-01

    Infants with congenital heart disease (CHD) have delayed brain maturation and alterations in brain volume. Brain metrics is a simple measurement technique that can be used to evaluate brain growth. This study used brain metrics to test the hypothesis that alterations in brain size persist at 3 months of age and that infants with CHD have slower rates of brain growth than control infants. Fifty-seven infants with CHD underwent serial brain magnetic resonance imaging (MRI). To evaluate brain growth across the first 3 months of life, brain metrics were undertaken using 19 tissue and fluid spaces shown on MRIs performed before surgery and again at 3 months of age. Before surgery, infants with CHD have smaller frontal, parietal, cerebellar, and brain stem measures (p < 0.001). At 3 months of age, alterations persisted in all measures except the cerebellum. There was no difference between control and CHD infants in brain growth. However, the cerebellum trended toward greater growth in infants with CHD. Somatic growth was the primary factor that related to brain growth. Presence of focal white matter lesions before and after surgery did not relate to alterations in brain size or growth. Although infants with CHD have persistent alterations in brain size at 3 months of age, rates of brain growth are similar to that of healthy term infants. Somatic growth was the primary predictor of brain growth, emphasizing the importance of optimal weight gain in this population.

  7. Mortality Trends in Pediatric and Congenital Heart Surgery: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery Database.

    Science.gov (United States)

    Jacobs, Jeffrey P; He, Xia; Mayer, John E; Austin, Erle H; Quintessenza, James A; Karl, Tom R; Vricella, Luca; Mavroudis, Constantine; O'Brien, Sean M; Pasquali, Sara K; Hill, Kevin D; Husain, S Adil; Overman, David M; St Louis, James D; Han, Jane M; Shahian, David M; Cameron, Duke; Jacobs, Marshall L

    2016-10-01

    Previous analyses of The Society of Thoracic Surgeons (STS) Adult Cardiac Surgery Database have demonstrated a reduction over time of risk-adjusted operative mortality after coronary artery bypass grafting. The STS Congenital Heart Surgery Database (STS CHSD) was queried to assess multiinstitutional trends over time in discharge mortality and postoperative length of stay (PLOS). Since 2009, operations in the STS CHSD have been classified according to STAT (The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery) Congenital Heart Surgery Mortality Categories. The five STAT Mortality Categories were chosen to be optimal with respect to minimizing variation within categories and maximizing variation between categories. For this study, all index cardiac operations from 1998 to 2014, inclusive, were grouped by STAT Mortality Category (exclusions: patent ductus arteriosus ligation in patients weighing less than or equal to 2.5 kg and operations that could not be assigned to a STAT Mortality Category). End points were discharge mortality and PLOS in survivors for the entire period and for 4-year epochs. The Cochran-Armitage trend test was used to test the null hypothesis that the mortality was the same across epochs, by STAT Mortality Category. The analysis encompassed 202,895 index operations at 118 centers. The number of centers participating in STS CHSD increased in each epoch. Overall discharge mortality was 3.4% (6,959 of 202,895) for 1998 to 2014 and 3.1% (2,308 of 75,337) for 2011 to 2014. Statistically significant improvement in discharge mortality was seen in STAT Mortality Categories 2, 3, 4, and 5 (p values for STAT Mortality Categories 1 through 5 are 0.060, <0.001, 0.015, <0.001, and <0.001, respectively). PLOS in survivors was relatively unchanged over the same time intervals. Sensitivity analyses reveal that the finding of declining risk-stratified rates of discharge mortality over time is not simply attributable to the addition

  8. Correlates of posttraumatic stress disorder in adults with congenital heart disease.

    Science.gov (United States)

    Eslami, Bahareh

    2017-05-01

    The aims of this study were to compare the level of posttraumatic stress disorder between adults with and without congenital heart disease, and to examine the correlates of posttraumatic stress disorder (e.g., sociodemographics). Cross-sectional. Two university-affiliated heart hospitals in Tehran, Iran. A sample of 347 adults with congenital heart disease aged 18-64 years (52% women), and 353 adults without congenital heart disease matched by sex and age (±2 years) was recruited. The PTSD Scale: Self-report version was used to assess the diagnosis and severity of posttraumatic stress disorder. Hierarchical multivariate logistic regression analyses were performed to explore correlates of likely posttraumatic stress disorder diagnosis among each group of participants. The posttraumatic stress disorder in the patients was comparable to those of the control group, except for increased arousal (P = .027) which was scored higher among the patients. Over 52% of adults with congenital heart disease met the criteria for a likely posttraumatic stress disorder diagnosis compared with 48% of adults without congenital heart disease. The regression analyses among patients revealed that elevated depressive symptoms (OR = 1.27) and a positive history of cardiac surgery (OR = 2.02) were significantly associated with posttraumatic stress disorder. The model could explain 29% of the variance in posttraumatic stress disorder. The high and comparable prevalence of posttraumatic stress disorder among patients and nonpatients highlight the significance of the context in which adults with congenital heart disease may face other/additional stressors than disease-related ones, an issue that clinicians need also take into account. Furthermore, the association of posttraumatic stress disorder with elevated depressive symptoms warrant a comprehensive psychological assessment and management of adults with congenital heart disease, in particular among those with a history of

  9. Types and distribution of congenital heart defects associated with trisomy 21 in Singapore.

    Science.gov (United States)

    Tan, Monica; Xu, Cunzhi; Sim, Sarah K R; Seow, Adeline L H; Tan, Teng Hong; Quek, Swee Chye

    2013-03-01

    Atrioventricular septal defect (AVSD) is widely accepted as the most common type of congenital heart defect in trisomy 21. Most of these studies, however, were conducted in Caucasian communities. The few Asian studies that had been conducted on this subject yielded different results. In the largest study of its kind in Asia, we described the distribution of types of congenital heart defects associated with trisomy 21 in Singapore. Five hundred and eighty-eight patients with trisomy 21 born in 1996-2010, and confirmed by karyotyping, were included in the study. The diagnosis of congenital heart defects were made on echocardiography. Variables extracted for analysis were demographics (race and gender) and the types of congenital heart defects. Except for complex cyanotic heart defects, haemodynamically significant lesions were accounted for separately in cases where more than one type of congenital heart defect coexisted in a patient. Ventricular septal defect (VSD) (39.2%) was the most common congenital heart defect associated with trisomy 21 in our study, followed by patent ductus arteriosus (34.3%), secundum atrial septal defect (23.4%) and AVSD (15.6%). This study validates previous smaller Asian studies identifying VSD as the most common cardiac lesion associated with trisomy 21. A high proportion (25.0%) of trisomy 21 patients with tetralogy of Fallot also had AVSDs. Coarctation of the aorta was uncommon. VSD was the most common congenital heart defect seen in trisomy 21 in our study. A high proportion (25.0%) of trisomy 21 patients with tetralogy of Fallot also had AVSDs. © 2013 The Authors. Journal of Paediatrics and Child Health © 2013 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  10. Utilizing Hybrid Techniques to Maximize Clinical Outcomes in Congenital Heart Disease.

    Science.gov (United States)

    Bearl, David W; Fleming, Gregory A

    2017-08-01

    The past couple of decades have brought tremendous advances to the field of pediatric and adult congenital cardiology. Recent collaborations between interventional cardiologists and cardiac surgeons have sparked novel innovative hybrid procedures that provide potentially safer, faster, and less invasive alternatives to the management of many diseases. This paper will review the most recent advances in hybrid interventions in the field of pediatric and adult congenital cardiology. The earliest experiences with hybrid interventions included intraoperative stenting of pulmonary arteries and perventricular device closure of ventricular septal defects. Newer hybrid interventions have focused on a hybrid approach to stage 1 palliation for infants with hypoplastic left heart syndrome and hybrid approaches to transcatheter pulmonary valve replacement in patients of all ages. Hybrid approaches to complex congenital heart disease are important in the management of congenital heart disease offering a less invasive approach, and novel hybrid procedures will likely be a focus of future research in this field.

  11. Medico-legal aspects of congenital heart diseases in buying and selling of pets

    Directory of Open Access Journals (Sweden)

    Annamaria Passantino

    2017-01-01

    Full Text Available Aim: The veterinarian should be able to assess congenital and inherited malformations such as heart defects because they may be object of legal disputes. In this study, the authors report some cases of congenital heart defects in pets (dogs and cats to clarify whether or not they may be considered a redhibitory defect. Materials and Methods: A total of 28 medical records of pets referred with suspected congenital heart disease were examined. All patients aged between 3 and 24 months underwent clinical examination, chest X-ray examination, electrocardiogram, and echocardiography and angiocardiography when necessary. Results: Congenital heart diseases or associated cardiac malformations were confirmed. Considering the above congenital diseases as redhibitory defect and the rights of the owners from a strictly legal viewpoint, 9 owners demanded an estimatory action and 11 a redhibitory action; 1 owner decided to demand the reimbursement of veterinary expenses because the animal died; 7 owners took no legal action but requested surgical intervention. Conclusions: Until more appropriate and detailed legislation on the buying and selling of pet animals is put in place; the authors propose to include in the contract a temporal extension of the guarantee relating to congenital heart disease, which can often become evident later.

  12. Are the children and adolescents with congenital heart disease living in Southwestern Ontario really overweight and obese?

    Science.gov (United States)

    Welisch, Eva; Rauch, Ralf; Seabrook, Jamie A; Filler, Guido; Norozi, Kambiz

    2014-10-01

    To assess the prevalence of overweight and obesity in children with congenital heart disease and compare them with age-matched healthy children in Southwestern Ontario, Canada. We compared the Center of Disease Control weight and body mass index z-scores of 1080 children, aged 2 to 18 years, who presented to our paediatric cardiology outpatient clinic from 2008 to 2010 for congenital heart disease with 1083 healthy controls. In all, 18.2% of the children with congenital heart disease and 20.8% of healthy children were identified to be either overweight or obese. Overall, the weight category distribution had been similar between the congenital heart disease and healthy control groups, as well as between the congenital heart disease subgroups. There was no difference in normal weight and overweight/obese categories between children with congenital heart disease and healthy children. The underweight category, however, showed a significantly higher prevalence in congenital heart disease compared with healthy children (6.8 and 4.5%, respectively, p = 0.03). The prevalence of overweight/obesity did not differ in children with congenital heart disease compared with age-matched healthy children; however, it is still high (18.2%). Obesity may represent an additional risk factor for the long-term cardiovascular health of congenital heart disease patients aside from the underlying heart defect.

  13. Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: introduction.

    Science.gov (United States)

    Marelli, Ariane; Beauchesne, Luc; Mital, Seema; Therrien, Judith; Silversides, Candice K

    2010-03-01

    With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society consensus conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. The present introductory section is a summary of the epidemiology and scope of adult CHD in Canada, the structure of the Canadian health care system and adult congenital cardiac health services in Canada. The recommendations for antibiotic prophylaxis and genetic evaluation in this population are included. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology, including sections on genetics, outcomes, diagnostic workups, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy and contraception risks, and follow-up recommendations. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

  14. Advancing Prenatal Detection of Congenital Heart Disease: A Novel Screening Protocol Improves Early Diagnosis of Complex Congenital Heart Disease.

    Science.gov (United States)

    Letourneau, Karen M; Horne, David; Soni, Reeni N; McDonald, Keith R; Karlicki, Fern C; Fransoo, Randy R

    2017-10-13

    Prenatal diagnosis of complex congenital heart disease (CHD) during routine obstetric ultrasound (US) examinations improves postnatal outcomes, but sensitivity is low (introduction of a novel prenatal screening protocol. The intervention was done in October 2004, emphasizing screening and not diagnosis of complex CHD. It involved education, practical scanning tips, a checklist, and feedback on cases. We also assessed the effect of the intervention in different screening settings: community hospitals, tertiary hospitals, and fetal assessment units. The sensitivity for detecting complex CHD increased from 29.8% to 88.3% (P < .0001), while the positive predictive value remained high. The largest improvement in detection was found for US units in community hospitals (52.4% higher; P < .0001), followed by tertiary hospitals (39.9%; P = .0004), and fetal assessment units (7.2%; P = .16). Additionally, there was a significant decrease in the presentation of neonates in critical condition from before to after the implementation (24.3% to 13.1%, respectively; P = .0165). Implementing a focused routine prenatal screening protocol can vastly improve detection rates of critical cardiac abnormalities. The improvement in community hospitals was particularly important because early detection ensured that the birth was planned to take place in an appropriate facility. Our novel protocol can be performed by all sonographers, regardless of experience, equipment used, and hospital setting. © 2017 by the American Institute of Ultrasound in Medicine.

  15. Comparison of Echocardiography and 64-Multislice Spiral Computed Tomography for the Diagnosis of Pediatric Congenital Heart Disease

    OpenAIRE

    Li, Aiyin; Peng, Zhenpeng; Zhang, Chengqi

    2017-01-01

    Background The goals of this study were: to compare echocardiogram and 64-multislice spiral computed tomography (64-MSCT) in diagnosing pediatric congenital heart disease; to determine the significance of ECHO for diagnosing congenital heart disease; and to identify the appropriate diagnosis for congenital heart disease through combined use of 64-MSCT and ECHO. Material/Methods Thirty patients underwent both ECHO and 64-MSCT diagnoses before their surgeries. Imaging from ECHO and 64-MSCT were...

  16. CLINICAL AND INSTRUMENTAL DATA COMPLEX AGGREGATIVE IN FATAL OUTCOME RISK PROGNOSIS IN NEWBORN WITN CONGENITAL HEART DISEASES

    OpenAIRE

    Елена Глебовна Цой; Людмила Николаевна Игишева; Ольга Владимировна Куренкова; Сергей Алексеевич Максимов; Любовь Михайловна Казакова

    2017-01-01

    Tactical issues of newborn monitoring optimization with congenital heart diseases require a decision. Objective – a predicative model creation of high fatal outcome risk in newborn with congenital heart disease in neonatal period, a received model approbation in practices. Materials and methods. Clinical and instrumental data analyzing was carried out in 98 newborn with critical congenital heart diseases on the third day of life. 45 criteria were outlined among all clinical and instru...

  17. Pattern and clinical aspects of congenital heart diseases and their management in Cameroon.

    Science.gov (United States)

    Tantchou Tchoumi, J C; Ambassa, J C; Chelo, D; Djimegne, F Kamdem; Giamberti, A; Cirri, S; Kingue, S; Butera, G

    2011-02-01

    The aim of the study was to investigate the occurrence, pattern and clinical aspects of congenital heart diseases and their management in Cameroon. In this multicentred retrospective study from January 2006 till November 2009, out of 51,636 consulting in three referral centres, 505 were recruited. All the patients presented with the following symptoms: cyanosis, clubbing, frequent respiratory tract infections, failure to thrive, growth retardation, precordial murmur and dyspnoea. Patients were sent for the screening of congenital heart disease. After the comprehensive Doppler echocardiography, the recruited patients were diagnosed with congenital (67.2%) and in few, acquired heart disease. Heart murmur, dyspnoea and growth retardation was the triad mostly encountered. The occurrence of congenital heart diseases in Cameroon is 9.87%. In Douala, isolated ventricular septal defect, interatrial septal defect and isolated pulmonary valve stenosis were more diagnosed than in Shisong (P abroad; 9% in the cardiac centre. Our data show that congenital heart diseases are represented in Cameroon as in the literature; isolated ventricular septal defect is the predominant pathology.

  18. Prevalence of congenital heart defects in patients with Down syndrome in the municipality of Pelotas, Brazil.

    Science.gov (United States)

    Vilas Boas, Luciana T; Albernaz, Elaine P; Costa, Rafaéla Gonçalves

    2009-01-01

    To determine the prevalence of congenital heart defects in patients with Down syndrome in the municipality of Pelotas, Brazil, describing the most frequent types and assessing the associated factors. Cross-sectional study including children with Down syndrome who were born and lived in Pelotas from January 2000 to December 2005. Data were collected by means of home interviews with mothers or guardians. Univariate and bivariate analyses were carried out to analyze the factors related to congenital heart defect. Forty-seven mothers of patients with Down syndrome were interviewed. Twenty-two (46.8%) of the patients had a diagnosis of congenital heart defect. Of them, 28% had early cardiac evaluation before 3 months of life. The most frequent heart defect was interatrial communication (17%); atrioventricular septal defect affected five patients. Bivariate analysis between the outcome congenital heart defect and the predicting factors maternal age, paternal age, parents' and child's skin color, presence of other malformations and child's sex showed that the associations were not statistically significant. The prevalence of Down syndrome and congenital heart defects in our region is similar to the rates found by other authors; therefore, we highlight the importance of diagnostic suspicion and early referral by pediatricians to cardiac evaluation. Another relevant aspect is the small number of patients who underwent karyotype testing. In addition, the number of associated malformations was lower than that found by other authors.

  19. The usefulness of brain natriuretic peptide in simple congenital heart disease-a systematic review

    NARCIS (Netherlands)

    J.A. Eindhoven (Jannet); A.E. van den Bosch (Annemien); H. Boersma (Eric); J.W. Roos-Hesselink (Jolien)

    2013-01-01

    textabstractBrain natriuretic peptide and N-terminal pro-brain natriuretic peptide are two well-established markers for cardiac failure in acquired heart disease. Nevertheless, the clinical utility of these markers in patients with congenital heart disease remains unclear. Therefore, the aim of this

  20. Dilated convolutional neural networks for cardiovascular MR segmentation in congenital heart disease

    NARCIS (Netherlands)

    Wolterink, Jelmer M.|info:eu-repo/dai/nl/413994112; Leiner, Tim|info:eu-repo/dai/nl/238322467; Viergever, Max A.|info:eu-repo/dai/nl/108781828; Išgum, Ivana|info:eu-repo/dai/nl/31484984X

    2017-01-01

    We propose an automatic method using dilated convolutional neural networks (CNNs) for segmentation of the myocardium and blood pool in cardiovascular MR (CMR) of patients with congenital heart disease (CHD). Ten training and ten test CMR scans cropped to an ROI around the heart were provided in the

  1. Mobile health in adults with congenital heart disease: Current use and future needs

    NARCIS (Netherlands)

    Schuuring, M.J.; A. Backx (Ad); Zwart, R.; Veelenturf, A.H.; D. Robbers-Visser (Daniëlle); M. Groenink (Maarten); A. Abu-Hanna (Ameen); N. Bruining (Nico); M.P. Schijven; B.J.M. Mulder (Barbara); B.J. Bouma (Berto)

    2016-01-01

    textabstractObjective Many adults with congenital heart disease (CHD) are affected lifelong by cardiac events, particularly arrhythmias and heart failure. Despite the care provided, the cardiac event rate remains high. Mobile health (mHealth) brings opportunities to enhance daily monitoring and

  2. Resynchronization therapy after congenital heart surgery to improve left ventricular function

    NARCIS (Netherlands)

    Roofthooft, Marcus T. R.; Blom, Nico A.; Rijlaarsdam, Marry E. B.; Bökenkamp, Regina; Ottenkamp, Jaap; Schalij, Martin J.; Bax, Jeroen J.; Hazekamp, Mark G.

    2003-01-01

    This report describes the mid-term beneficial hemodynamic effect of biventricular pacing in an infant with congestive heart failure after congenital heart surgery, due to resynchronization of the left and right ventricle, optimization of the AV delay, and (partial) correction of the LV dyssynchrony

  3. The usefulness of brain natriuretic peptide in complex congenital heart disease: A systematic review

    NARCIS (Netherlands)

    J.A. Eindhoven (Jannet); A.E. van den Bosch (Annemien); P.R. Jansen (Philip); H. Boersma (Eric); J.W. Roos-Hesselink (Jolien)

    2012-01-01

    textabstractBrain natriuretic peptide (BNP) and N-terminal pro-brain natriuretic peptide (NT-proBNP) are well-established markers for heart failure in the general population. However, the value of BNP as a diagnostic and prognostic marker for patients with structural congenital heart disease (CHD)

  4. Peri-operative care in adults with congenital heart disease : Room for improvement in after care

    NARCIS (Netherlands)

    Schoormans, D.; Smets, E.M.A.; Zwart, R.; Sprangers, M.A.G.; Veelenturg, T.H.; de Mol, B.A.; Hazekamp, M.G.; Koolbergen, D.R.; Sojak, V.; Bouma, B.J.; Groenink, M.; Boekholdt, M.S.; Backx, A.P.; Mulder, B.J.

    2013-01-01

    Patient satisfaction with care has received little attention within the field of congenital heart disease. Our objective was to examine patient satisfaction with the care received when undergoing open-heart surgery in order to identify the best and worst aspects of peri-operative care. Moreover, we

  5. Congenital heart disease and its journey from dental plaque to arterial plaque

    Directory of Open Access Journals (Sweden)

    Vinathi Reddy Kankara

    2016-01-01

    Full Text Available Congenital heart disease is mostly found in children, approximately around 7–10% from overall heart diseases. The etiology is multifactorial but reported associations include untreated maternal diabetes, phenylketonuria, intake of retinoic acid last but not least is oral pathogens present in periodontopathic bacteria. The main objective of this article is to explain about different mechanisms by which it is associated with dental, periodontal manifestations. It also explains about two patients who reported to our hospital with congenital heart disease and their dental and periodontal management.

  6. [Early rehabilitation and cardiorespiratory response in children after correction of congenital heart defects].

    Science.gov (United States)

    Petronić, Ivana; Milincić, Zeljka; Nikolić, Dejan; Cirović, Dragana; Ilić, Slobodan; Parezanović, Vojislav; Radlović, Vladimir; Knezević, Tatjana; Pavićević, Polina

    2008-01-01

    Significant improvement in survival and quality of life in patients after the correction of congenital heart defects was enabled by the introduction of surgical procedures. Due to increased vulnerability in the period after defect correction, early rehabilitation have found its place in recovery. Various studies suggest good effects of a rehabilitation programme especially in the early rehabilitation period. In our study we wanted to stress the importance of early rehabilitation as well as its effects of implementation. We evaluated the children treated at the University Children's Hospital during a four-year period. All participants were divided into two groups according to age: the first group included children up to the age of one year, and the second group included those from the first till the third year of life. Patients were divided into two groups according to the presence of complications. We monitored children with 4 types of congenital heart defects: Tetralogy of Fallot, Transposition of the Great Arteries, Coarctation of the Aorta and Stenosis of the Aorta. All exercises were implemented twice daily. Out of the pulmonary system parameters we monitored saturation of oxygen during the entire programme. Other parameters that were evaluated were: heart rate, ECG and breathing frequency. In the children with corrected defects, the most frequent complication was athelectasis especially in the group of patients till the first year of life. The patients with the onset of complications had a longer rehabilitation programme. Saturation of oxygen at the end of the programme was significantly elevated. From the above-mentioned it can be concluded that individual rehabilitation assessment for every patient is needed. Early rehabilitation measures give sustained effects of well-being after completion of the programme.

  7. Early rehabilitation and cardiorespiratory response in children after correction of congenital heart defects

    Directory of Open Access Journals (Sweden)

    Petronić Ivana

    2008-01-01

    Full Text Available INTRODUCTION Significant improvement in survival and quality of life in patients after the correction of congenital heart defects was enabled by the introduction of surgical procedures. Due to increased vulnerability in the period after defect correction, early rehabilitation have found its place in recovery. Various studies suggest good effects of a rehabilitation programme especially in the early rehabilitation period. OBJECTIVE In our study we wanted to stress the importance of early rehabilitation as well as its effects of implementation. METHOD We evaluated the children treated at the University Children's Hospital during a four-year period. All participants were divided into two groups according to age: the first group included children up to the age of one year, and the second group included those from the first till the third year of life. Patients were divided into two groups according to the presence of complications. We monitored children with 4 types of congenital heart defects: Tetralogy of Fallot, Transposition of the Great Arteries, Coarctation of the Aorta and Stenosis of the Aorta. All exercises were implemented twice daily. Out of the pulmonary system parameters we monitored saturation of oxygen during the entire programme. Other parameters that were evaluated were: heart rate, ECG and breathing frequency. RESULTS In the children with corrected defects, the most frequent complication was athelectasis especially in the group of patients till the first year of life. The patients with the onset of complications had a longer rehabilitation programme. Saturation of oxygen at the end of the programme was significantly elevated. CONCLUSION From the above-mentioned it can be concluded that individual rehabilitation assessment for every patient is needed. Early rehabilitation measures give sustained effects of well-being after completion of the programme.

  8. [Successful outcome of a pregnancy with an extremely low fetal heart rate (34 bpm) due to isolated complete heart block--case report].

    Science.gov (United States)

    Hamela-Olkowska, Anita; Dangel, Joanna; Miszczak-Knecht, Maria

    2009-09-01

    Isolated complete congenital heart block (CHB) in the majority of cases is associated with the presence of autoantibodies to SSA (Ro) and SSB (La) antigens in the maternal serum. The prognosis is less favorable in fetuses with a ventricular rate bpm. We have reported a case of a fetus with an isolated non-autoimmune CHB with an extremely low ventricular rate (34bpm) in which the outcome was favorable. In the neonate the non-compaction of the myocardium was diagnosed.

  9. Scoliosis Surgery in Children With Congenital Heart Disease.

    Science.gov (United States)

    Kadhim, Muayad; Spurrier, Ellen; Thacker, Deepika; Pizarro, Christian; Mackenzie, William G

    2013-10-22

    Study Design. Retrospective cohort study.Objective. To describe preoperative evaluation, anesthetic and peri-operative management and complications in patients with congenital heart disease (CHD) who underwent surgery to correct a spine deformity.Summary of Background Data. Patients with surgically palliated or repaired CHD may have nearly normal circulation or may have important residual abnormalities that affect the planning and conduct of surgery to correct a spine deformity.Methods. We examined the records of 21 patients with spine deformity who had previous surgical intervention for CHD. Three types of spine surgery and instrumentation were examined, posterior fusion with instrumentation (PSFI), growing rod instrumentation (GR), and vertical expandable prosthetic titanium rib instrumentation (VEPTR).To objectify the degree of preoperative cardiac physiologic derangement, patients were classified into three groups: single ventricle physiology and Fontan circulation (S), two ventricles with no residual abnormal cardiac physiology condition (2N), and two ventricles with residual cardiac physiology problem(2R).Results. Subjects were 8 boys and 13 girls with mean age of 11.1±5.2 years. Sixteen patients underwent surgery to correct scoliosis, one to correct kyphosis, and 4 did not undergo surgery. Total number of surgeries was 23 (16 PSFI, 5 GR and 2 VEPTR). Based on cardiac physiology, two patients were 2N, 11 were 2R and 8 were Group S. Mean estimated blood loss was 1685 ml during PSFI, 515 ml during GR and 150 ml during VEPTR. Mean volume of blood transfusion was 44 ml/kg for PSFI, 19 ml/kg for GR, while no transfusion was administered during VEPTR. Median ICU stay was 2 days ranging from hours to 78 days. Median hospital length of stay was 7 days ranging from 3 to 93 days. There were no deaths.Conclusion. Given meticulous multidisciplinary planning and execution, major spine surgery can be safely and successfully performed in patients with significant residua

  10. Prepregnancy Diabetes and Offspring Risk of Congenital Heart Disease

    Science.gov (United States)

    Diaz, Lars J.; Leirgul, Elisabeth; Boyd, Heather A.; Priest, James; Mathiesen, Elisabeth R.; Quertermous, Thomas; Wohlfahrt, Jan; Melbye, Mads

    2016-01-01

    Background— Maternal diabetes mellitus is associated with an increased risk of offspring congenital heart defects (CHD); however, the causal mechanism is poorly understood. We further investigated this association in a Danish nationwide cohort. Methods and Results— In a national cohort study, we identified 2 025 727 persons born from 1978 to 2011; among them were 7296 (0.36%) persons exposed to maternal pregestational diabetes mellitus. Pregestational diabetes mellitus was identified by using the National Patient Register and individual-level information on all prescriptions filled in Danish pharmacies. Persons with CHD (n=16 325) were assigned to embryologically related cardiac phenotypes. The CHD prevalence in the offspring of mothers with pregestational diabetes mellitus was 318 per 10 000 live births (n=232) in comparison with a baseline risk of 80 per 10 000; the adjusted relative risk for CHD was 4.00 (95% confidence interval, 3.51–4.53). The association was not modified by year of birth, maternal age at diabetes onset, or diabetes duration, and CHD risks associated with type 1 (insulin-dependent) and type 2 (insulin-independent) diabetes mellitus did not differ significantly. Persons born to women with previous acute diabetes complications had a higher CHD risk than those exposed to maternal diabetes mellitus without complications (relative risk, 7.62; 95% confidence interval, 5.23–10.6, and relative risk, 3.49; 95% confidence interval, 2.91–4.13, respectively; P=0.0004). All specific CHD phenotypes were associated with maternal pregestational diabetes mellitus (relative risk range, 2.74–13.8). Conclusions— The profoundly increased CHD risk conferred by maternal pregestational diabetes mellitus neither changed over time nor differed by diabetes subtype. The association with acute pregestational diabetes complications was particularly strong, suggesting a role for glucose in the causal pathway. PMID:27166384

  11. [Social concern and independence in adults with congenital heart disease].

    Science.gov (United States)

    Niwa, Koichiro; Tateno, Shigeru; Tatebe, Shunsuke; Fujita, Kanako; Sugita, Katsuo; Terai, Masaru; Aotsuka, Hiroyuki; Takahashi, Osahiro

    2002-05-01

    Recent advances in medical and surgical treatment have led to the survival of increasing numbers of adults with congenital heart disease (CHD). However, the social status of these patients remains unknown. This survey investigated the social prospects for adults with CHD, and the limiting factors for social independence. A written questionnaire on patient characteristics, education, employability, marital status and insurability was designed to define the characteristics of social independence in adults with CHD. Randomly selected adults with CHD were enrolled: 13 patients with cyanotic unrepaired CHD (4 males, 9 females, mean age: 29.8 +/- 10 years, range: 18-56 years) and 102 patients with other CHDs (48 males, 54 females, mean age: 29.5 +/- 10 years, range: 18-74 years). University of California at Los Angeles functional class I-II was found in 94% of patients, medication in 46%, and hospitalization in 51%. Compared with the data from Japanese general population, study patients had a lower ratio of high school graduates (86% vs 94%), life insurability (51% vs 71%), marital status (31% vs 32%) and employability (82% vs 80%). Patients with unrepaired cyanotic CHD had significantly lower ratio than those with other CHDs (marital status 15%, p = 0.19; employability 40%, p = 0.0003; high school graduates 69%, p = 0.06; life insurability 18%, p = 0.02, respectively). Factors affecting social independence in adults with CHD were severity of disease, continuing medication, lower level of education, lower self-esteem, and unknown natural history of CHD. To improve social independence in these patients, further development of medical and surgical therapy and more detailed knowledge of the patients, caretakers and society in this field are needed.

  12. Body mass index in adult congenital heart disease.

    Science.gov (United States)

    Brida, Margarita; Dimopoulos, Konstantinos; Kempny, Alexander; Liodakis, Emmanouil; Alonso-Gonzalez, Rafael; Swan, Lorna; Uebing, Anselm; Baumgartner, Helmut; Gatzoulis, Michael A; Diller, Gerhard-Paul

    2017-08-01

    Abnormal body mass index (BMI) is associated with higher mortality in various cardiovascular cohorts. The prognostic implications of BMI in adults with congenital heart disease (ACHD) are unknown. We aim to assess the distribution of BMI and its association with symptoms and survival in the ACHD population. We included 3069 ACHD patients (median age 32.6 years) under follow-up at our institution between 2001 and 2015. Patients were classified based on BMI as underweight (30), and symptoms, exercise capacity and mortality were assessed. Overall, 6.2% of patients were underweight, 51.1% had normal weight, 28.2% were overweight and 14.6% were obese. Higher BMI values were associated with lower all-cause and cardiac mortality on univariable Cox analysis, and this effect persisted after adjustment for age, defect complexity, cyanosis and objective exercise capacity. Higher BMI was especially associated with better prognosis in symptomatic ACHD patients (HR 0.94 (95% CI 0.90 to 0.98), p=0.002) and those with complex underlying cardiac defects (HR 0.96 (95% CI 0.91 to 0.997), p=0.048) In patients with a complex cardiac defect who had repeated weight measurements, weight loss was also associated with a worse survival (HR 1.82 (95% CI 1.02 to 3.24), p=0.04). ACHD patients with a higher BMI had a lower mortality. The association between BMI and mortality was especially pronounced in symptomatic patients with complex underlying cardiac defects, suggesting that cardiac cachexia may play a role. Indeed, weight loss in complex ACHD patients was linked to an even higher mortality. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  13. Scoliosis surgery in children with congenital heart disease.

    Science.gov (United States)

    Kadhim, Muayad; Spurrier, Ellen; Thacker, Deepika; Pizarro, Christian; Mackenzie, William G

    2014-02-01

    Retrospective cohort study. To describe preoperative evaluation, anesthetic and perioperative management, and complications in patients with congenital heart disease (CHD) who underwent surgery to correct a spine deformity. Patients with surgically palliated or repaired CHD may have nearly normal circulation or may have important residual abnormalities that affect the planning and conduct of surgery to correct a spine deformity. We examined the records of 21 patients with spine deformity who had previous surgical intervention for CHD. Three types of spine surgery and instrumentation were examined, posterior spinal fusion with instrumentation (PSFI), growing rod (GR) instrumentation, and vertical expandable prosthetic titanium rib instrumentation (VEPTR). To objectify the degree of preoperative cardiac physiological derangement, patients were classified into 3 groups: single ventricle physiology and Fontan circulation (S), two ventricles with no residual abnormal cardiac physiology condition (2N), and two ventricles with residual cardiac physiology problem (2R). Subjects were 8 boys and 13 girls with mean age of 11.1 ± 5.2 years. Sixteen patients underwent surgery to correct scoliosis, 1 to correct kyphosis, and 4 did not undergo surgery. Total number of surgical procedures was 23 (16 PSFI, 5 GR, and 2 VEPTR). On the basis of cardiac physiology, 2 patients belonged 2N, 11 were 2R, and 8 were group S. Mean estimated blood loss was 1685 mL during PSFI, 515 mL during GR, and 150 mL during VEPTR. Mean volume of blood transfusion was 44 mL/kg for PSFI, 19 mL/kg for GR, whereas no transfusion was administered during VEPTR. Median intensive care unit stay was 2 days ranging from hours to 78 days. Median hospital length of stay was 7 days ranging from 3 to 93 days. There were no deaths. Given meticulous multidisciplinary planning and execution, major spine surgery can be safely and successfully performed in patients with significant residua of CHD. 4.

  14. Cerebrovascular accidents in adult patients with congenital heart disease.

    Science.gov (United States)

    Hoffmann, A; Chockalingam, P; Balint, O H; Dadashev, A; Dimopoulos, K; Engel, R; Schmid, M; Schwerzmann, M; Gatzoulis, M A; Mulder, B; Oechslin, E

    2010-08-01

    To investigate the prevalence and characteristics of cerebrovascular accidents (CVA) in a large population of adults with congenital heart disease (CHD). In a retrospective analysis of aggregated European and Canadian databases a total population of 23 153 patients with CHD was followed up to the age of 16-91 years (mean 36.4 years). Among them, 458 patients (2.0%) had one or more CVA, with an estimated event rate of 0.05% per patient-year. Permanent neurological sequelae were noted in 116 patients (25.3%). The prevalence of CVA in selected diagnostic categories was as follows: open atrial septal defect 93/2351 (4.0%); closed atrial or ventricular septal defect 57/4035 (1.4%); corrected tetralogy of Fallot 52/2196 (2.4%); Eisenmenger physiology 24/467 (5.1%); other cyanotic 50/215 (23.3%); mechanical prostheses (29/882 (3.3%). Associated conditions in patients with CVA were absence of sinus rhythm (25%), transvenous pacemakers (7%), endocarditis (2%), cardiac surgery (11%) and catheter intervention (2%), but with the exception of absent sinus rhythm these were not significantly more prevalent in patients with CVA. CVA are a major contributor to morbidity in this young population despite absence of classical cardiovascular risk factors. Although the prevalence of CVA in patients with CHD appears low, it is 10-100 times higher than expected in control populations of comparable age. Residua occur in a strong minority of patients. The subjects at highest risk are those patients with CHD with cyanotic lesions, in whom the prevalence is over 10-fold above the average.

  15. Anthropometric profiles of children with congenital heart disease

    Directory of Open Access Journals (Sweden)

    Damayanti R. Sjarif

    2011-02-01

    Full Text Available Background: Undernutrition is a common cause of morbidity in children with CHD. Previous data from developing country showed prevalence of preoperative undernutrition in children with CHD was up to 45%. The aim of this study are to determine the anthropometric profiles and prevalence of undernutrition in children with CHD by using the anthropometric measurement.Methods: A cross-sectional study was carried out in children aged 0-2 years old with CHD in Cipto Mangunkusumo hospital. All patients underwent an anthropometric evaluation (weight, length and head circumference at presentation. Undernutrition, failure to thrive /FTT, short stature and microcephaly were determined according to WHO, weight-for-length, weight-for-age at 2 points, length-for-age, head circumference-for-age z-score < -2SD accordingly.Results: We had total of 95 patients, 73 patients with acyanotic and 22 patients with cyanotic lesions. Prevalence of undernutrition in CHD was 51.1%, with 22.3% severe undernutrition. FTT was found in 64.9%, short stature in 49.5% and microcephaly in  37% patients. FTT was found higher in acyanotic (72.2% compared to cyanotic lesions (42.9%. In acyanotic, weight was affected more than length (72.2% vs 49.3%. In cyanotic, weight and length affected equally (42.9% vs 54.5%. Diet counseling were done in patients with undernutrition. Medicines, transcatheter or surgery intervention were indicated in selected patients.Conclusions: Prevalence of FTT was higher than undernutrition in children with CHD. FTT was found higher in acyanotic lesions. In acyanotic, weight was affected more than length. In cyanotic, weight and length affected equally. (Med J Indones 2011; 20:40-5Keywords: congenital heart disease, failure to thrive, short stature, undernutrition

  16. Chromosome 5q33 deletions associated with congenital heart defects.

    Science.gov (United States)

    Starkovich, Molly; Lalani, Seema R; Mercer, Catherine L; Scott, Daryl A

    2016-12-01

    Congenital heart defects (CHD) are present in over 1% of all newborns and are the leading cause of birth-defect-related deaths in the United States. We describe two male subjects with CHD, one with an atrial septal defect, a ventricular septal defect, and pulmonary artery stenosis; and the other with tetralogy of Fallot and a right aortic arch, who carry partially overlapping, de novo deletions of chromosome 5q33. The maximum region of overlap between these deletions encompasses HAND1 and SAP30L, two genes that have previously been shown to play a role in cardiac development. HAND1 encodes a basic helix-loop-helix transcription factor. Cardiac-specific ablation of Hand1 in mice causes septal, valvular, and outflow tract defects. SAP30L, its paralog SAP30, and other SAP proteins form part of a multi-subunit complex involved in transcriptional regulation via histone deacetylation. Morpholino knockdown of sap30L in zebrafish, which do not have a distinct sap30 gene, leads to cardiac hypoplasia and cardiac insufficiency. We subsequently identified two other individuals with chromosomal deletions involving HAND1 and SAP30L in whom cardiac-related medical problems were not described. These observations suggest that haploinsufficiency of HAND1 and/or SAP30L may contribute to the development of CHD, although the contribution of other genes on chromosome 5q33 cannot be excluded. Our findings also suggest that the penetrance of CHD associated with 5q33 deletions is incomplete and may be influenced by other genetic, environmental or stochastic factors. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  17. Congenital Heart Defects and Receipt of Special Education Services.

    Science.gov (United States)

    Riehle-Colarusso, Tiffany; Autry, Andrew; Razzaghi, Hilda; Boyle, Coleen A; Mahle, William T; Van Naarden Braun, Kim; Correa, Adolfo

    2015-09-01

    We investigated the prevalence of receipt of special education services among children with congenital heart defects (CHDs) compared with children without birth defects. Children born from 1982 to 2004 in metropolitan Atlanta with CHDs (n = 3744) were identified from a population-based birth defect surveillance program; children without birth defects (n = 860 715) were identified from birth certificates. Cohorts were linked to special education files for the 1992-2012 school years to identify special education services. Children with noncardiac defects or genetic syndromes were excluded; children with CHDs were classified by presence or absence of critical CHDs (ie, CHDs requiring intervention by age one year). We evaluated the prevalence of receipt of special education services and prevalence rate ratios using children without birth defects as a reference. Compared with children without birth defects, children with CHDs were 50% more likely to receive special education services overall (adjusted prevalence rate ratio [aPRR] = 1.5; 95% confidence interval [CI]: 1.4-1.7). Specifically, they had higher prevalence of several special education categories including: intellectual disability (aPRR = 3.8; 95% CI: 2.8-5.1), sensory impairment (aPRR = 3.0; 95% CI: 1.8-5.0), other health impairment (aPRR = 2.8; 95% CI: 2.2-3.5), significant developmental delay (aPRR = 1.9; 95% CI: 1.3-2.8), and specific learning disability (aPRR = 1.4; 95% CI: 1.1-1.7). For most special education services, the excess prevalence did not vary by presence of critical CHDs. Children with CHDs received special education services more often than children without birth defects. These findings highlight the need for special education services and the importance of developmental screening for all children with CHDs. Copyright © 2015 by the American Academy of Pediatrics.

  18. Congenital heart defects and parental occupational exposure to chemicals.

    Science.gov (United States)

    Snijder, Claudia A; Vlot, Ingrid J; Burdorf, Alex; Obermann-Borst, Sylvia A; Helbing, Willem A; Wildhagen, Mark F; Steegers, Eric A P; Steegers-Theunissen, Régine P M

    2012-05-01

    Congenital heart defects (CHDs) are the most common major malformations in newborns. In this study we examined the associations between the occurrence of CHDs in children and periconceptional occupational parental exposures to chemicals. In an age-matched case-control study with standardized data collection at c. 15 months after birth, 424 mothers and 421 fathers of a child with CHD and 480 mothers and 477 fathers of a non-malformed child, filled out questionnaires on periconceptional general and job characteristics. A job exposure matrix, which links the information on job title and a description of work tasks to an expert judgement on exposure to chemicals in the workplace, was used. The overall prevalence of occupational exposure to chemicals was 5.0 in cases and 6.2% in controls for mothers [odds ratio (OR) adjusted = 0.92; 95% confidence interval (CI): 0.26-3.25], while 22.3 and 15.9% for fathers, respectively (OR adjusted = 1.23; 95% CI: 0.39-3.91). No association of maternal occupational exposure to chemicals with risk of CHDs was found. Paternal exposure to phthalates was associated with a higher incidence of CHDs in general (OR adjusted = 2.08; 95% CI: 1.27-3.40). Paternal exposure to phthalates was associated with perimembranous ventricular septal defect (OR adjusted = 2.84; 95% CI: 1.37-5.92), to polychlorinated compounds with atrioventricular septal defect (OR adjusted = 4.22; 95% CI: 1.23-14.42) and to alkylphenolic compounds with coarctation of the aorta (OR adjusted = 3.85; 95% CI: 1.17-12.67). Periconceptional paternal (but not maternal) occupational exposure to certain chemicals is associated with an increased risk of CHDs in children. The results, however, must be interpreted cautiously as exposure probabilities are a crude measure of exposure.

  19. Physical activity evaluation in children with congenital heart disease.

    Science.gov (United States)

    Voss, Christine; Harris, Kevin C

    2017-09-01

    Significant advances in the management of children with congenital heart disease (CHD) have resulted in marked improvements in survival and life expectancy. Thus, there is an increased emphasis on promoting physical activity to optimise healthy development and long-term cardiovascular health. Evaluation of physical activity levels as part of ongoing clinical care is recommended to facilitate physical activity counselling and/or exercise prescription. Physical activity is a complex health behaviour that is challenging to evaluate. We provide an overview of techniques for measuring physical activity in children with CHD with a focus on how to do this in the clinical context. Accelerometers are devices that objectively assess intensity and duration of physical activity under free living conditions. They enable evaluation against physical activity guidelines, but are costly and require advanced technical expertise. Pedometers are a simple-to-use and cost-effective alternative, but an outcome metric of daily step count limits classification against guidelines. Commercial wearable activity trackers offer an appealing user experience and can provide valid estimates in children. Furthermore, activity trackers enable remote monitoring of physical activity levels, which may facilitate exercise prescription and activity counselling. Questionnaires are the most cost-effective and time-effective method, but recall error in younger children is a consideration. Routine exercise testing in children with CHD provides important insight into functional status but should not be viewed as a proxy measure of habitual physical activity. Understanding the spectrum and role of physical activity measurement tools is important for clinicians focused on optimising cardiovascular health in children with CHD. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  20. Creating Opportunities for Optimal Nutritional Experiences for Infants With Complex Congenital Heart Disease.

    Science.gov (United States)

    Steltzer, Michelle M; Sussman-Karten, Karen; Kuzdeba, Hillary Bishop; Mott, Sandra; Connor, Jean Anne

    To our knowledge, successful breastfeeding in the population with single ventricle congenital heart disease has not been reported in the literature, particularly during the interstage period. A retrospective case study including inpatient nutrition and a complete history of daily logs with the home surveillance monitoring program was performed. Successful full breastfeeding (exceeding prescribed weight growth goals) after Stage I surgery was achieved during the interstage period. The infant was discharged at 3.41 kg, not consistently breastfeeding, and progressed to 7.05 kg at 5 months of age, fully breastfeeding. Supporting breastfeeding for infants who have undergone repairs for single ventricle anatomy can be challenging but can be accomplished. It requires a concerted team effort, clear communication, and collaboration among caregivers, the mother, and her supporters. Copyright © 2016 National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved.

  1. [Fibular transfer in a nine-week-old infant for complete congenital absence of the tibia].

    Science.gov (United States)

    Karakurt, Lokman; Yilmaz, Erhan; Avci, Mustafa; Serin, Erhan

    2002-01-01

    The treatment of complete congenital absence of the tibia is controversial. The goal of the treatment is to construct a satisfactorily functioning knee joint, for which fibular transfer was described as an appropriate method. We performed reconstructive surgery using a modified Browns procedure in a nine-week-old boy with Jones type 1 congenital longitudinal deficiency of the tibia. In his final control at three years of age a good outcome was obtained according to the criteria by Epps et al. To our knowledge, this is the youngest patient to receive fibular transfer in the literature, to which we ascribed the satisfactory outcome.

  2. Adult congenital heart surgery: adult or pediatric facility? Adult or pediatric surgeon?

    Science.gov (United States)

    Kogon, Brian E; Plattner, Courtney; Leong, Traci; Kirshbom, Paul M; Kanter, Kirk R; McConnell, Mike; Book, Wendy

    2009-03-01

    One of the current controversies in the field of adult congenital heart disease is whether patients should be cared for at an adult or pediatric facility and by an adult or pediatric heart surgeon. After transitioning our program from the children's hospital to the adult hospital, we analyzed our experience with each system. Between 2000 and 2007, 303 operations were performed on adults (age >or= 18 years) with congenital heart disease. One hundred eighty-five operations were performed in an adult hospital and 118 in a pediatric hospital. Forty-six operations were performed by an adult heart surgeon and 257 by a congenital heart surgeon. Mean age, coexisting medical problems, and preoperative risk factors were higher in both the adult hospital group and adult surgeon group compared with the respective pediatric groups. Mortality was similar at the adult and pediatric hospitals (4.3% versus 5.1%), but was markedly higher in the adult surgeon group compared with the pediatric surgeon group (15.2% versus 2.7%; p = 0.0008). By multivariate analysis, risk factors for mortality included older age at the time of surgery (p = 0.028), surgery performed at a children's hospital (p = 0.013), and surgery performed by an adult heart surgeon (p = 0.0004). Congenital heart surgery can be performed in adults with reasonable morbidity and mortality. Caring for an anticipated aging adult congenital population with increasingly numerous coexisting medical problems and risk factors is best facilitated in an adult hospital setting. Also, when surgery becomes necessary, these adult patients are best served by a congenital heart surgeon.

  3. Style of coping and its determinants in adults with congenital heart disease in a developing country.

    Science.gov (United States)

    Eslami, Bahareh; Macassa, Gloria; Sundin, Örjan; Khankeh, Hamid Reza; Soares, Joaquim J F

    2014-01-01

    The objective of this study is to compare coping strategies between adults with and without congenital heart disease and to scrutinize the associations between different available resources (e.g., social support) and adoption of certain coping strategies. The study has a cross-sectional case-control design. The study was conducted in two university-affiliated heart hospitals in Tehran, Iran. The participants comprised 347 persons (18-64 years) with and 353 individuals without congenital heart disease, matched by gender and age. Coping strategies, assessed with the Utrecht Coping List-short form, were compared between both groups. Block-wise multiple regression analyses were conducted to scrutinize the associations between different independent variables (e.g., demographic/socioeconomic statuses) and adoption of certain styles of coping (dependent variables) among all participants and separately for each group. The styles of coping in the patients were comparable with those of the control group. Multivariate analyses revealed that congenital heart disease per se was not associated with style of coping except for palliative reaction pattern. The active problem-solving coping style was associated with never married marital status, parenthood, unemployment, higher level of anxiety/somatic symptoms, lower level of depressive symptoms, and better social support. The avoidance behavior style was associated with having a low income, whereas the expression of emotion style was associated with higher anxiety symptoms, experience of financial strain, and income. None of the adopted coping strategies was related to the heart disease variables. The adults with congenital heart disease coped as well as adults without congenital heart disease. Marital status, parenthood, annual income, financial strain, psychological adjustment, and perceived social support were important explanatory factors in adopting a certain style of coping among adults with congenital heart disease. However

  4. Characteristics and outcomes of heart failure-related hospitalization in adults with congenital heart disease.

    Science.gov (United States)

    Moussa, Nidhal Ben; Karsenty, Clement; Pontnau, Florence; Malekzadeh-Milani, Sophie; Boudjemline, Younes; Legendre, Antoine; Bonnet, Damien; Iserin, Laurence; Ladouceur, Magalie

    2017-05-01

    Heart failure (HF) is the main cause of death in adult congenital heart disease (ACHD). We aimed to characterize HF-related hospitalization of patients with ACHD, and to determine HF risk factors and prognosis in this population. We prospectively included 471 patients with ACHD admitted to our unit over 24 months. Clinical and biological data and HF management were recorded. Major cardiovascular events were recorded for ACHD with HF. HF was the main reason for hospitalization in 13% of cases (76/583 hospitalizations). Patients with HF were significantly older (median age 44±14 years vs. 37±15 years; Pcongenital heart disease (P=0.04). In the multivariable analysis, pulmonary arterial hypertension (odds ratio [OR] 6.2, 95% confidence interval [CI] 3.5-10.7), history of HF (OR 9.8, 95% CI 5.7-16.8) and history of atrial arrhythmia (OR 3.6, 95% CI 2.2-5.9) were significant risk factors for HF-related admissions (Pheart transplantation during the median follow-up of 18 months (95% CI 14-20 months). The risk of cardiovascular events was 19-fold higher after HF-related hospitalization. HF is emerging as a leading cause of morbidity and mortality in the ACHD population. Earlier diagnosis and more active management are required to improve outcomes of HF in ACHD. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  5. Determination of the prevalence of congenital heart disease in the patients admitted to the heart clinic

    Directory of Open Access Journals (Sweden)

    Shokoufeh Ahmadipour

    2015-11-01

    Full Text Available Objective: To investigate the prevalence of congenital heart disease (CHD among the patients who refferred to the heart clinic so as to make an early and correct diagnosis. Methods: In this descriptive-cross sectional study, all the patients admitted to the heart clinic who had symptoms or signs of CHD were included. The data were collected in one year based on the medical records. The main variables consisted of age, gender, history of folic acid consumption by the mother in pregnancy, clinical signs, symptoms and so on. Results: Among the 763 admitted patients, 498 were males and the rest were females. Infants were the most common group and teenagers were the least one. The most common findings for which the patients had been referred were chest pain and a murmur heard during a normal physical examination. Based on the echocardiography findings, ventricular and atrial septal defects were the most common ones. The history of folic acid consumption was negative in 168 mothers within their pregnancy. Conclusions: Since the causes and risk factors in the incidence of CHD in children are numerous, we recommended that the information about these diseases should be given to the community and strengthen the referral system, design registration system of CHD set up in the country round.

  6. Heart failure treatment in adults with congenital heart disease: where do we stand in 2014?

    Science.gov (United States)

    Krieger, Eric V; Valente, Anne Marie

    2014-09-01

    Heart failure (HF) is the leading cause of death in adults with repaired congenital heart disease (CHD). However there is currently little evidence to guide treatment strategies in this growing group of patients. Unlike the majority of HF, which is usually caused by LV systolic or diastolic dysfunction, CHD-HF is more often a consequence of RV disease, valve dysfunction, shunting or pulmonary hypertension. It is therefore not appropriate to extrapolate from the acquired HF literature and apply it to this heterogeneous population of CHD patients. Additionally, patients with CHD have been excluded from most large trials of medical or device therapy of HF, which has resulted in small retrospective and underpowered studies in the CHD population. This article critically reviews the current knowledge about CHD-HF, paying particular attention to medical therapy in different CHD populations, cardiac resynchronisation therapy and implantable cardiac defibrillators, and the challenges of heart transplantation and mechanical circulatory support in CHD patients. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  7. Nutritional Condition Characterization of Children Under 5 Years Old with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Yan González Ramos

    2017-09-01

    Full Text Available Foundation: congenital heart disease is the most frequent anomaly at birth and causes 20 % of neonatal deaths. The haemodynamic alterations presented in these cases affect their nutritional condition and cause complications related to postoperative survival. Objective: to characterize the nutritional condition of children under 5 with congenital heart disease. Method: a descriptive, correlational and cross - sectional study was carried out in patients younger than 5 years old with congenital heart disease at the Cienfuegos cardiopathy clinic. The following variables were used: age, sex, type of heart disease and nutritional evaluation. The information was processed using the statistical package SPSS 19, proceeding to the analysis and discussion of those results that were shown in tables, graphs and percentages. Results: acyanotic heart disease was the most frequent, representing 2/3 of the total. Males and children older than 1 year were those with the greatest nutritional problems. 10.5 % of the studied patients had low birth weight. 97.4 % of the patients with low height were in the stage of homeorhesis associated with acyano- genic cardiopathies with increased pulmonary flow. The biochemical parameters did not present great affectation. Conclusion: nutritional condition is frequently affected in children with congenital heart disease, which has declined in recent years thanks to prenatal diagnosis of critical heart disease, where survival is minimal.

  8. Study of prevalence & risk factors of congenital heart defect (Review Article

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    ali dehghani

    2017-09-01

    Full Text Available Abstract Background: Congenital heart defects are known as the state that comes from birth and influences on structure and function of baby's heart, The different types of defects can range from mild (e.g., a small hole between the heart chambers to hard (like a flaw or weakness in a part of the heart. Method: This article is a review article in which the articles published in Farsi and English that the bases valid as Medline, Google Scholar, Pubmed, Springer, SID index has been used, as well as for the study of keywords associated with the use of MESH keywords in identifying and no time limit listed in the databases were searched. Result The prevalence of congenital heart defect, in general, less than one per cent in newborn. The ventricular wall abnormalities defect (VSD, atrial septal defect of (ASD, patent ductus arteriosus (PDA and tetralogy of Fallot (TOF Top among the most types of congenital heart anomalies. Factors such as the age of the parents at conception, maternal risk of diabetes, influenza and febrile illness during pregnancy, drug use during pregnancy and taking a multivitamin before and during the Pregnancy were influenced of newborns with congenital heart defects. Conclusion: According to studies need to be conducted in the presence of multiple risk factors for these disorders, seem to have a detailed plan to Study of More about the factors that affect the risk of developing these disorders, as well as interventions to reduce risk factors identified particularly during pregnancy.

  9. Pregnancy in young women with congenital heart disease: Lesion-specific considerations

    OpenAIRE

    Wald, Rachel M; Sermer, Mathew; Colman, Jack M

    2011-01-01

    Young women with heart disease are increasingly being seen in obstetrical referral centres owing, in large part, to the dramatic improvements in survival of young adults with congenital heart disease in recent years. Although pregnancies in most women with heart disease result in favourable outcomes, there are important exceptions that must be recognized. These exceptions pose a significant mortality risk to the mother and/or the fetus. The present article provides a general framework for the...

  10. Origins and consequences of congenital heart defects affecting the right ventricle.

    Science.gov (United States)

    Woudstra, Odilia I; Ahuja, Suchit; Bokma, Jouke P; Bouma, Berto J; Mulder, Barbara J M; Christoffels, Vincent M

    2017-10-01

    Congenital heart disease is a major health issue, accounting for a third of all congenital defects. Improved early surgical management has led to a growing population of adults with congenital heart disease, including patients with defects affecting the right ventricle, which are often classified as severe. Defects affecting the right ventricle often cause right ventricular volume or pressure overload and affected patients are at high risk for complications such as heart failure and sudden death. Recent insights into the developmental mechanisms and distinct developmental origins of the left ventricle, right ventricle, and the outflow tract have shed light on the common features and distinct problems arising in specific defects. Here, we provide a comprehensive overview of the current knowledge on the development into the normal and congenitally malformed right heart and the clinical consequences of several congenital heart defects affecting the right ventricle. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

  11. Quality of Life and Congenital Heart Disease in Childhood and Adolescence

    Energy Technology Data Exchange (ETDEWEB)

    Bertoletti, Juliana [Instituto de Cardiologia/Fundação Universitária de Cardiologia - IC/FUC, Porto Alegre, RS (Brazil); Marx, Giovana Caroline; Hattge, Sérgio Pedro Júnior [Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS (Brazil); Pellanda, Lucia Campos, E-mail: lupellanda@gmail.com [Instituto de Cardiologia/Fundação Universitária de Cardiologia - IC/FUC, Porto Alegre, RS (Brazil); Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS (Brazil)

    2014-02-15

    Advances in cardiac surgery techniques and early diagnosis have enabled the increased survival of individuals with congenital heart disease. The investigation of the quality of life in children and adolescents with congenital heart disease provides complementary information to clinical data that can assist in decision making on the part of health professionals. Although many studies have been conducted to investigate the quality of life of children and adolescents with congenital heart disease, the results prove to be contradictory; while some studies show that congenital heart disease can impact the quality of life, others describe a better perception of quality of life among children and adolescents who suffer from the disease when compared with healthy control subjects. The purpose of this study is to review the literature on the assessment of health related quality of life in children and adolescents with congenital heart disease, in order to systematize the existing knowledge on this topic today. It is observed that research seeks to investigate aspects of personality in cardiac patients, their coping strategies used and perceived social support, aiming at better understanding the association of these variables with the level of quality of life in this population.

  12. Pbx/Meis deficiencies demonstrate multigenetic origins of congenital heart disease.

    Science.gov (United States)

    Stankunas, Kryn; Shang, Ching; Twu, Karen Y; Kao, Shih-Chu; Jenkins, Nancy A; Copeland, Neal G; Sanyal, Mrinmoy; Selleri, Licia; Cleary, Michael L; Chang, Ching-Pin

    2008-09-26

    Congenital heart diseases are traditionally considered to be multifactorial in pathogenesis resulting from environmental and genetic interactions that determine penetrance and expressivity within a genetically predisposed family. Recent evidence suggests that genetic contributions have been significantly underestimated. However, single gene defects occur only in a minority of cases, and multigenetic causes of congenital heart diseases have not been fully demonstrated. Here, we show that interactions between alleles of 3 Pbx genes, which encode homeodomain transcription factors, are sufficient to determine the phenotypic presentation of congenital heart diseases in mice. A major role is served by Pbx1, whose inactivation results in persistent truncus arteriosus. Reduction or absence of Pbx2 or Pbx3 leads to Pbx1 haploinsufficiency and specific malformations that resemble tetralogy of Fallot, overriding aorta with ventricular septal defect, and bicuspid aortic valves. Disruption of Meis1, which encodes a Pbx DNA-binding partner, results in cardiac anomalies that resemble those caused by Pbx mutations. Each of the observed cardiac defects represents developmental abnormalities affecting distinct stages of cardiac outflow tract development and corresponds to specific types of human congenital heart disease. Thus, varied deficiencies in the Pbx gene family produce a full spectrum of cardiac defects involving the outflow tract, providing a framework for determining multigenetic causes of congenital heart anomalies.

  13. Common variation in ISL1 confers genetic susceptibility for human congenital heart disease.

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    Kristen N Stevens

    Full Text Available Congenital heart disease (CHD is the most common birth abnormality and the etiology is unknown in the overwhelming majority of cases. ISLET1 (ISL1 is a transcription factor that marks cardiac progenitor cells and generates diverse multipotent cardiovascular cell lineages. The fundamental role of ISL1 in cardiac morphogenesis makes this an exceptional candidate gene to consider as a cause of complex congenital heart disease. We evaluated whether genetic variation in ISL1 fits the common variant-common disease hypothesis. A 2-stage case-control study examined 27 polymorphisms mapping to the ISL1 locus in 300 patients with complex congenital heart disease and 2,201 healthy pediatric controls. Eight genic and flanking ISL1 SNPs were significantly associated with complex congenital heart disease. A replication study analyzed these candidate SNPs in 1,044 new cases and 3,934 independent controls and confirmed that genetic variation in ISL1 is associated with risk of non-syndromic congenital heart disease. Our results demonstrate that two different ISL1 haplotypes contribute to risk of CHD in white and black/African American populations.

  14. Restrictive lung function in pediatric patients with structural congenital heart disease.

    Science.gov (United States)

    Hawkins, Stephen M M; Taylor, Amy L; Sillau, Stefan H; Mitchell, Max B; Rausch, Christopher M

    2014-07-01

    We sought to describe the prevalence of restrictive lung function in structural congenital heart disease and to determine the effect of cardiothoracic surgical intervention. The data from a retrospective review of the spirometry findings from pediatric patients with structural congenital heart disease were compared with the data from 220 matched controls. Restrictive lung function was defined as a forced vital capacity of 80%. Of the children with congenital heart disease, 20% met the criteria for restrictive lung function compared with 13.2% of the controls (P = .03). The prevalence in those with congenital heart disease without a surgical history was similar to that of the controls (odds ratio, 0.62; 95% confidence interval, 0.34-1.13). Restrictive lung function was more likely if surgical intervention had occurred within the first year of life (odds ratio, 1.96; 95% confidence interval, 1.08-3.55; P congenital heart disease after cardiothoracic surgical intervention than in the controls or patients without surgical intervention. The prevalence was also greater with surgical intervention at an earlier age. The risk was equivalent when sternotomy alone was compared with thoracotomy alone but was significantly greater when both sternotomy and thoracotomy were performed. The risk increased with each additional surgery performed. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  15. Accuracy of the Masimo SET® LNCS neo peripheral pulse oximeter in cyanotic congenital heart disease.

    Science.gov (United States)

    Griksaitis, Michael J; Scrimgeour, Gemma E; Pappachan, John V; Baldock, Andrew J

    2016-08-01

    Introduction Non-invasive peripheral pulse oximeters are routinely used to measure oxyhaemoglobin saturation (SpO2) in cyanotic congenital heart disease. These probes are calibrated in healthy adult volunteers between arterial saturations of ~75 and 100%, using the gold standard of co-oximetry on arterial blood samples. There are little data to attest their accuracy in cyanotic congenital heart disease. Aims We aimed to assess the accuracy of a commonly used probe in children with cyanotic congenital heart disease. Children with cyanotic congenital heart disease admitted to the Paediatric Intensive Care Unit with an arterial line in situ were included to our study. Prospective simultaneous recordings of SpO2, measured by the Masimo SET® LNCS Neo peripheral probe, and co-oximeter saturations (SaO2) measured by arterial blood gas analysis were recorded. A total of 527 paired measurements of SpO2 and SaO2 (using an ABL800 FLEX analyser) in 25 children were obtained. The mean bias of the pulse oximeter for all SaO2 readings was +4.7±13.8%. The wide standard deviation indicates poor precision. This mean bias increased to +7.0±13.7% at SaO2 recordings congenital heart disease. This may have serious implications for clinical decisions.

  16. Frequency of Congenital Heart Diseases in Prelingual Sensory-Neural Deaf Children

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    Masoud Motasaddi Zarandy

    2016-03-01

    Full Text Available Introduction: Hearing impairment is the most frequent sensorial congenital defect in newborns and has increased to 2–4 cases per 1,000 live births. Sensory-neural hearing loss (SNHL accounts for more than 90% of all hearing loss. This disorder is associated with other congenital disorders such as renal, skeletal, ocular, and cardiac disorders. Given that congenital heart diseases are life-threatening, we decided to study the frequency of congenital heart diseases in children with congenital sensory-neural deafness.  Materials and Methods: All children who had undergone cochlear implantation surgery due to SNHL and who had attended our hospital for speech therapy during 2008–2011 were evaluated by Doppler echocardiography.  Results: Thirty-one children (15 boys and 16 girls with a mean age of 55.70 months were examined, and underwent electrocardiography (ECG and echocardiography. None of the children had any signs of heart problems in their medical records. Most of their heart examinations were normal, one patient had expiratory wheeze, four (12% had mid-systolic click, and four (12% had an intensified S1 sound. In echocardiography, 15 children (46% had mitral valve prolapse (MVP and two (6% had minimal mitral regurgitation (MR. Mean ejection fraction (EF was 69% and the mean fractional shortening (FS was 38%.  Conclusion:  This study indicates the need for echocardiography and heart examinations in children with SNHL.

  17. First-cousin matings and congenital heart disease in Saudi Arabia.

    Science.gov (United States)

    Becker, S; Al Halees, Z

    1999-01-01

    Consanguinity may be a risk factor that contributes to congenital heart disease in an inbred population, particularly among first cousins; with high rates of intermarriage between relatives, consanguinity is associated with congenital heart disease particularly among first cousins. This study examines a group of 1,028 consecutive congenital heart disease (CHD) patients identified through the Congenital Heart Disease Registry at King Faisal Specialist Hospital in Riyadh, Saudi Arabia. Families were interviewed by an Arabic-speaking data collector for information on consanguinity and demographic data. After exclusions for nonavailability, data were collected on 949 cases and the proportions of first-cousin consanguinity in the study sample were compared to national population data. A z test of proportions was utilized to test the hypothesis that there are higher proportions of first-cousin consanguinity in the study sample of congenital heart patients than in the general population. Data indicate that the proportion of first-cousin matings among CHD patients is significantly higher than that of first-cousin intermarriages reported in the general population (p < 0.001). Regional breakdowns of the data substantiate significant differences (p < 0.001) in the proportions of first-cousin consanguinity in the study sample compared to the general population. In a population with a high degree of inbreeding, consanguinity may exacerbate underlying genetic risk factors, particularly for offspring of first-cousin matings. This finding has public health implications for genetic counseling and prevention of some cardiac malformations. Copyright 2000 S. Karger AG, Basel

  18. Isolation and characterization of cardiogenic, stem-like cardiac precursors from heart samples of patients with congenital heart disease.

    Science.gov (United States)

    Ghazizadeh, Zaniar; Vahdat, Sadaf; Fattahi, Faranak; Fonoudi, Hananeh; Omrani, Gholamreza; Gholampour, Maziar; Aghdami, Nasser

    2015-09-15

    Regenerative therapies based on resident human cardiac progenitor cells (hCPCs) are a promising alternative to medical treatments for patients with myocardial infarction. However, hCPCs are rare in human heart and finding efficient source and proper surface marker for isolation of these cells would make them a good candidate for therapy. We have isolated 5.34∗10(6)±2.04∗10(5)/g viable cells from 35 heart tissue samples of 23 patients with congenital heart disease obtained during their heart surgery along with 6 samples from 3 normal subjects during cardiac biopsy. According to FACS analysis, younger ages, atrial specimen and disease with increased pulmonary vascular resistance were associated with higher percentage of c-kit(+) (CD117) hCPCs. Analysis for other stemness markers revealed increased CD133(+) cells in the hearts of patients with congenital heart disease. By using both immune-labeling and PCR, we demonstrated that these cells express key cardiac lineage and endothelial transcription factors and structural proteins during in vitro differentiation and do express stemness transcription factors in undifferentiated state. Another novel datum of potentially relevant interest is their ability in promoting greater myocardial regeneration and better survival in rat model of myocardial infarction following transplantation. Our results could provide evidence for conditions associated with enriched hCPCs in patients with congenital heart disease. Moreover, we showed presence of a significant number of CD133 expressing cardiogenic stem-like cardiac precursors in the heart of patients with congenital heart disease, which could be isolated and stored for future regenerative therapies in these patients. Copyright © 2015 Elsevier Inc. All rights reserved.

  19. Hypertension, obesity, and coronary artery disease in the survivors of congenital heart disease.

    Science.gov (United States)

    Roche, S Lucy; Silversides, Candice K

    2013-07-01

    Obesity, hypertension, and coronary artery disease are prevalent in the general population and well recognized as contributors to cardiac morbidity and mortality. With surgical and medical advances, there is a growing and aging population with congenital heart disease who are also at risk of developing these comorbidities. In addition, some congenital cardiac lesions predispose patients to conditions such as hypertension or coronary artery disease. The effect of these comorbidities on the structurally abnormal heart is not well understood, but might be very important, especially in those with residual abnormalities. Thus, in addition to surveillance for and treatment of late complications it is important for the congenital cardiologist to consider and aggressively manage acquired comorbidities. In this review we explore the prevalence of hypertension, obesity, and coronary artery disease, discuss congenital lesions that predispose to these conditions and review management strategies for this unique population. Copyright © 2013 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  20. Incidence of Congenital Heart Disease: The 9-Year Experience of the Guangdong Registry of Congenital Heart Disease, China

    Science.gov (United States)

    Zhuang, Jian; Chen, Guanchun; Mai, Jinzhuang; Guo, Xiaoling; Ou, Yanqiu; Chen, Jimei; Gong, Wei; Gao, Xiangmin; Wu, Yong; Nie, Zhiqiang

    2016-01-01

    There are 16.5 million newborns in China annually. However, the incidence of congenital heart disease (CHD) has not been evaluated. In 2004, we launched an active province-wide hospital-based CHD registry in the Guangdong Province of southern China. In this study, we examined the incidence of CHD and its subtypes from 2004 to 2012 and compared our findings to the literature. Our results indicate there is an increasing trend of CHD incidence. The increase in incidence occurred mainly for single lesion and the most common subtypes (e.g., ventricular or atrial septal defect, patent ductus arteriosus). There were no increases found for multiple lesions or more complex subtypes. The proportion of CHD cases that were detected early (e.g., 1 week) increased over time. The incidence of CHD stabilized in 2010–2012 with the average cumulative incidences of 9.7, 9.9, and 11.1 per 1,000 live births at 1 week, 1 month, and 1 year, respectively. The incidences of CHD subtypes were comparable with recent international results. The data did not support previous reports that Asian children have a higher incidence of pulmonary outflow obstructions and lower incidence of transposition of the great arteries. However, there was a lower incidence of left ventricular outflow tract obstructions observed in our series. The increase in CHD incidence observed over time was due to improved detection and diagnosis. The true incidence of CHD in China was approximately 11.1 per 1,000 live births, which is higher than previously reported. PMID:27409588

  1. Incidence of Congenital Heart Disease: The 9-Year Experience of the Guangdong Registry of Congenital Heart Disease, China.

    Directory of Open Access Journals (Sweden)

    Yanji Qu

    Full Text Available There are 16.5 million newborns in China annually. However, the incidence of congenital heart disease (CHD has not been evaluated. In 2004, we launched an active province-wide hospital-based CHD registry in the Guangdong Province of southern China. In this study, we examined the incidence of CHD and its subtypes from 2004 to 2012 and compared our findings to the literature. Our results indicate there is an increasing trend of CHD incidence. The increase in incidence occurred mainly for single lesion and the most common subtypes (e.g., ventricular or atrial septal defect, patent ductus arteriosus. There were no increases found for multiple lesions or more complex subtypes. The proportion of CHD cases that were detected early (e.g., 1 week increased over time. The incidence of CHD stabilized in 2010-2012 with the average cumulative incidences of 9.7, 9.9, and 11.1 per 1,000 live births at 1 week, 1 month, and 1 year, respectively. The incidences of CHD subtypes were comparable with recent international results. The data did not support previous reports that Asian children have a higher incidence of pulmonary outflow obstructions and lower incidence of transposition of the great arteries. However, there was a lower incidence of left ventricular outflow tract obstructions observed in our series. The increase in CHD incidence observed over time was due to improved detection and diagnosis. The true incidence of CHD in China was approximately 11.1 per 1,000 live births, which is higher than previously reported.

  2. Incidence of Congenital Heart Disease: The 9-Year Experience of the Guangdong Registry of Congenital Heart Disease, China.

    Science.gov (United States)

    Qu, Yanji; Liu, Xiaoqing; Zhuang, Jian; Chen, Guanchun; Mai, Jinzhuang; Guo, Xiaoling; Ou, Yanqiu; Chen, Jimei; Gong, Wei; Gao, Xiangmin; Wu, Yong; Nie, Zhiqiang

    2016-01-01

    There are 16.5 million newborns in China annually. However, the incidence of congenital heart disease (CHD) has not been evaluated. In 2004, we launched an active province-wide hospital-based CHD registry in the Guangdong Province of southern China. In this study, we examined the incidence of CHD and its subtypes from 2004 to 2012 and compared our findings to the literature. Our results indicate there is an increasing trend of CHD incidence. The increase in incidence occurred mainly for single lesion and the most common subtypes (e.g., ventricular or atrial septal defect, patent ductus arteriosus). There were no increases found for multiple lesions or more complex subtypes. The proportion of CHD cases that were detected early (e.g., 1 week) increased over time. The incidence of CHD stabilized in 2010-2012 with the average cumulative incidences of 9.7, 9.9, and 11.1 per 1,000 live births at 1 week, 1 month, and 1 year, respectively. The incidences of CHD subtypes were comparable with recent international results. The data did not support previous reports that Asian children have a higher incidence of pulmonary outflow obstructions and lower incidence of transposition of the great arteries. However, there was a lower incidence of left ventricular outflow tract obstructions observed in our series. The increase in CHD incidence observed over time was due to improved detection and diagnosis. The true incidence of CHD in China was approximately 11.1 per 1,000 live births, which is higher than previously reported.

  3. Adolescents with congenital heart disease: the importance of perceived parenting for psychosocial and health outcomes.

    Science.gov (United States)

    Luyckx, Koen; Goossens, Eva; Missotten, Lies; Moons, Philip

    2011-11-01

    : Little is known about how parenting relates to psychosocial functioning and health behavior in adolescents with congenital heart disease (CHD). Different parenting styles were identified through relying on adolescent perceptions of multiple dimensions (regulation, responsiveness, and psychological control). The degree to which parents were perceived as consistent in their rearing style was assessed. : Adolescents with CHD were selected from the database of pediatric and congenital cardiology of the University Hospitals Leuven; control individuals were recruited at secondary schools. A total of 429 adolescents (14-18 years) with CHD participated; 403 were matched on gender and age with control individuals. Adolescents completed questionnaires on maternal and paternal regulation, psychological control, and responsiveness. Main outcome measures were depressive symptoms, loneliness, quality of life, health status, alcohol, cigarette, and drug use. : No significant differences emerged between adolescents with CHD and controls in perceived parenting styles. Democratic parenting was accompanied by the most optimal pattern of outcomes in adolescents with CHD, whereas psychologically controlling parenting by the least optimal pattern. Overprotective parenting was related to high patient substance use. Perceiving both parents as democratic turned out most favorably for psychosocial functioning and quality of life, whereas parental consistency was unrelated to substance use in adolescents with CHD. : By building bridges between the fields of adolescent medicine and family studies, the present study generated important information on the role of parents in psychosocial and behavioral functioning of adolescents with CHD. Future longitudinal studies could inform family-based interventions for this population.

  4. Personality traits, quality of life, and perceived health in adolescents with congenital heart disease

    OpenAIRE

    Rassart, Jessica; Luyckx, Koen; Goossens, Eva; Apers, Silke; Klimstra, Theo; Moons, Philip

    2013-01-01

    The present study investigated how the Big Five personality traits were related to quality of life and perceived health in adolescents with congenital heart disease (CHD). Adolescents with CHD were selected from the database of pediatric and congenital cardiology of the University Hospitals Leuven. A total of 366 adolescents (15-20 years) participated; 364 were matched on sex and age with community controls. Adolescents’ personality was assessed using the Quick Big Five, quality of life was m...

  5. Transhepatic Central Venous Catheters in Pediatric Patients With Congenital Heart Disease.

    Science.gov (United States)

    Boe, Brian A; Zampi, Jeffrey D; Yu, Sunkyung; Donohue, Janet E; Aiyagari, Ranjit

    2015-10-01

    Patients with congenital heart disease may have limited venous access routes as a result of multiple central venous catheters, surgical interventions, and catheterization procedures. Unconventional venous access includes transhepatic central venous catheter. We evaluated transhepatic central venous catheter placed in patients with congenital heart disease and risk factors associated with complications and outcomes. Demographic, procedural, and complication data were retrospectively collected on all patients who underwent transhepatic central venous catheter placement at our center over the past 10 years. This study was completed in a tertiary congenital heart center. A total of 92 transhepatic central venous catheters were placed in 54 patients (63% male patients). The median age and weight of the patient population was 5.7 months and 5.5 kg, respectively. Placement of a transhepatic central venous catheter. Successful catheter placement occurred in 96% of cases with median procedure time of 54 minutes with a procedural complication rate of 14%. A total of 86 complications occurred in 54 catheters placed during 2,166 catheter-days (39.7 complications per 1,000 catheter-days). Individual complication rates per 1,000 catheter-days included catheter dysfunction (14.8), dislodgement (8.8), systemic infection (5.1), thrombosis (4.2), local infection (3.7), and bleeding (3.2). Two complications contributed to patient deaths. Factors associated with developing complications included polyurethane central venous catheters (p = 0.03) and catheter duration at least 21 days (p = 0.004). The overall mortality in this population was 50% with median length of hospitalization of 49 days (interquartile range, 33-97). Transhepatic central venous catheters represent a viable option for patients with limited access. Polyurethane catheters and catheter duration at least 21 days are associated with increased transhepatic central venous catheter complications. Although complication

  6. Objectively measured physical activity levels of young children with congenital heart disease.

    Science.gov (United States)

    Stone, Nicola; Obeid, Joyce; Dillenburg, Rejane; Milenkovic, Jovana; MacDonald, Maureen J; Timmons, Brian W

    2015-03-01

    Physical activity tends to be lower in school-age children with congenital heart disease than in healthy controls. To the best of our knowledge, objectively measured physical activity levels of preschool-age children with congenital heart disease have not been studied. A total of 10 children with either coarctation of the aorta (n=6; age 3.8±0.9) or tetralogy of Fallot (n=4, age 4.3±0.9) were recruited from the cardiology unit of McMaster Children's Hospital. Height (103.7±8.2 cm) and weight (17.3±2.7 kg) measurements were recorded, and physical activity was determined using accelerometry over 7 consecutive days. Patients were compared with age-, sex-, and season of data acquisition-matched controls. Parents completed a questionnaire regarding the child's physical activity and sedentary behaviours. Patients spent on average 219.4±39.9 minutes engaged in total physical activity per day at the following intensities: light, 147.5±22.3; moderate, 44.0±11.8; moderate-to-vigorous, 71.9±22.6; and vigorous, 27.9±11.7. No significant differences were observed between patients and controls for total physical activity (p=0.80) or any of the intensities (p=0.71, 0.46, 0.43, and 0.45, respectively). Only 40% of patients and controls met the new Canadian Physical Activity Guidelines for the Early Years of at least 180 minutes of physical activity at any intensity every day. Of the patients' parents, 90% believed that their child was as active, if not more active, than his/her siblings, and 80% of parents reported their child spending 1-3 hours in screen time activities daily. Children aged 3-5 years old with congenital heart disease have comparable physical activity levels to age-, sex-, and season-matched controls, and many do not meet Canadian Physical Activity Guidelines.

  7. The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2017 Update on Outcomes and Quality.

    Science.gov (United States)

    Jacobs, Jeffrey P; Mayer, John E; Mavroudis, Constantine; O'Brien, Sean M; Austin, Erle H; Pasquali, Sara K; Hill, Kevin D; Overman, David M; St Louis, James D; Karamlou, Tara; Pizarro, Christian; Hirsch-Romano, Jennifer C; McDonald, Donna; Han, Jane M; Becker, Susan; Tchervenkov, Christo I; Lacour-Gayet, Francois; Backer, Carl L; Fraser, Charles D; Tweddell, James S; Elliott, Martin J; Walters, Hal; Jonas, Richard A; Prager, Richard L; Shahian, David M; Jacobs, Marshall L

    2017-03-01

    The Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital and pediatric cardiac surgical clinical data registry in the world. It is the platform for all activities of The Society of Thoracic Surgeons related to the analysis of outcomes and the improvement of quality in this subspecialty. This report summarizes current aggregate national outcomes in congenital and pediatric cardiac surgery and reviews related activities in the areas of quality measurement, performance improvement, and transparency. The reported data about aggregate national outcomes are exemplified by an analysis of 10 benchmark operations performed from January 2012 to December 2015. This analysis documents the overall aggregate operative mortality (interquartile range among all participating programs) for the following procedural groups: off-bypass coarctation repair, 1.3% (0.0% to 1.8%); ventricular septal defect repair, 0.6% (0.0% to 0.9%); tetralogy of Fallot repair, 1.1% (0.0% to 1.4%); complete atrioventricular canal repair, 3.0% (0.0% to 4.7%); arterial switch operation, 2.7% (0.0% to 4.1%); arterial switch operation and ventricular septal defect repair, 5.3% (0.0% to 6.7%); Glenn/hemi-Fontan, 2.5% (0.0% to 4.5%); Fontan operation, 1.2% (0.0% to 1.2%); truncus arteriosus repair, 9.4% (0.0% to 16.7%); and Norwood procedure, 15.7% (8.9% to 25.0%). Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  8. Evaluation of Complications of Heart Surgery in Children With Congenital Heart Disease at Dena Hospital of Shiraz

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    Mirzaei, Maryam; Mirzaei, Samaneh; Sepahvand, Elham; Koshkaki, Afifeh Rahmanian; Jahromi, Marzieh Kargar

    2015-01-01

    Introduction: Today, with progress in the field of congenital heart surgery, different complicated actions are done in children. These actions may be associated with several complications, especially open heart surgery in which the cardiopulmonary bypass (CPB) is used. Serious complications can be caused high morbidity and mortality rates. Present study has been performed to determine the incidence of morbidity and mortality in cardiac surgery in children. Method: In a cross-sectional retrosp...

  9. Sleep-Disordered Breathing in Patients with Pulmonary Valve Incompetence Complicating Congenital Heart Disease.

    Science.gov (United States)

    Miles, Susan; Ahmad, Waheed; Bailey, Amy; Hatton, Rachael; Boyle, Andrew; Collins, Nicholas

    2016-12-01

    Long standing pulmonary regurgitation results in deleterious effects on right heart size and function with late consequences of right heart volume overload including ventricular dilatation, propensity to arrhythmia and right heart failure. As sleep disordered breathing may predispose to elevations in pulmonary vascular resistance and associated negative effects on right ventricular function, we sought to assess this in patients with underlying congenital heart disease. We performed a pilot study to evaluate the incidence of sleep-disordered breathing in a patient population with a history of long standing pulmonary valve incompetence in patients with congenital heart disease using overnight oximetry. Patients with a background of tetralogy of Fallot repair or residual pulmonary incompetence following previous pulmonary valve intervention for congenital pulmonary stenosis were included. Twenty-two patients underwent overnight oximetry. The mean age of the cohort was 34.3 ± 15.2 years with no patients observed to have severe underlying pulmonary hypertension. Abnormal overnight oximetry was seen in 13/22 patients (59.1%) with 2/22 (9.1%) patients considered to have severe abnormalities. An important proportion of patients with a background of pulmonary incompetence complicating congenital heart disease are prone to the development of sleep-disordered breathing as assessed by overnight oximetry. Further study into the prevalence and mechanisms of sleep-disordered breathing in a larger cohort are warranted. © 2016 Wiley Periodicals, Inc.

  10. Myocardial Factor Revisited: The Importance of Myocardial Fibrosis in Adults with Congenital Heart Disease

    Science.gov (United States)

    Broberg, Craig S.; Burchill, Luke J.

    2015-01-01

    Pioneers in congenital heart surgery observed that exercise capacity did not return to normal levels despite successful surgical repair, leading some to cite a “myocardial factor” playing a role. They conjectured that residual alterations in myocardial function would be significant for patients’ long-term outlook. In fulfillment of their early observations, today’s adult congenital heart disease (ACHD) population shows well-recognized features of heart failure, even among patients without clear residual anatomic or hemodynamic abnormalities, demonstrating the vital role of the myocardium in their morbidity and mortality. Whereas the ‘myocardial factor’ was an elusive concept in the early history of congenital heart care, we now have imaging techniques to detect and quantify one such factor – myocardial fibrosis. Understanding the importance of myocardial fibrosis as a final common pathway in a variety of congenital lesions provides a framework for both the study and treatment of clinical heart failure in this context. While typical heart failure pharmacology should reduce or attenuate fibrogenesis, efforts to show meaningful improvements with standard pharmacotherapy in ACHD repeatedly fall short. This paper considers the importance of myocardial fibrosis and function, the current body of evidence for myocardial fibrosis in ACHD, and its implications for research and treatment. PMID:25897907

  11. Myocardial factor revisited: The importance of myocardial fibrosis in adults with congenital heart disease.

    Science.gov (United States)

    Broberg, Craig S; Burchill, Luke J

    2015-06-15

    Pioneers in congenital heart surgery observed that exercise capacity did not return to normal levels despite successful surgical repair, leading some to cite a "myocardial factor" playing a role. They conjectured that residual alterations in myocardial function would be significant for patients' long-term outlook. In fulfillment of their early observations, today's adult congenital heart disease (ACHD) population shows well-recognized features of heart failure, even among patients without clear residual anatomic or hemodynamic abnormalities, demonstrating the vital role of the myocardium in their morbidity and mortality. Whereas the 'myocardial factor' was an elusive concept in the early history of congenital heart care, we now have imaging techniques to detect and quantify one such factor--myocardial fibrosis. Understanding the importance of myocardial fibrosis as a final common pathway in a variety of congenital lesions provides a framework for both the study and treatment of clinical heart failure in this context. While typical heart failure pharmacology should reduce or attenuate fibrogenesis, efforts to show meaningful improvements with standard pharmacotherapy in ACHD repeatedly fall short. This paper considers the importance of myocardial fibrosis and function, the current body of evidence for myocardial fibrosis in ACHD, and its implications for research and treatment. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  12. A systematic review of prenatal screening for congenital heart disease by fetal electrocardiography.

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    Verdurmen, Kim M J; Eijsvoogel, Noortje B; Lempersz, Carlijn; Vullings, Rik; Schroer, Christian; van Laar, Judith O E H; Oei, S Guid

    2016-11-01

    Congenital heart disease (CHD) is the most common severe congenital anomaly worldwide. Diagnosis early in pregnancy is important, but the detection rate by two-dimensional ultrasonography is only 65%-81%. To evaluate existing data on CHD and noninvasive abdominal fetal electrocardiography (ECG). A systematic review was performed through a search of the Cochrane Library, PubMed, and Embase for studies published up to April 2016 using the terms "congenital heart disease," "fetal electrocardiogram," and other similar keywords. Primary articles that described changes in fetal ECG among fetuses with CHD published in English were included. Outcomes of interest were changes in fetal ECG parameters observed for fetuses with congenital heart disease. Findings were reported descriptively. Only five studies described changes observed in the fetal electrocardiogram for fetuses with CHD, including heart rate, heart rate variability, and PR, QRS, and QT intervals. Fetal ECG reflects the intimate relationship between the cardiac nerve conduction system and the structural morphology of the heart. It seems particularly helpful in detecting the electrophysiological effects of cardiac anatomic defects (e.g. hypotrophy, hypertrophy, and conduction interruption). Fetal ECG might be a promising clinical tool to complement ultrasonography in the screening program for CHD. Copyright © 2016 International Federation of Gynecology and Obstetrics. Published by Elsevier Ireland Ltd. All rights reserved.

  13. Deletion of a single-copy DAAM1 gene in congenital heart defect: a case report

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    Bao Bihui

    2012-08-01

    Full Text Available Abstract Background With an increasing incidence of congenital heart defects (CHDs in recent years, genotype-phenotype correlation and array-based methods have contributed to the genome-wide analysis and understanding of genetic variations in the CHD population. Here, we report a copy number deletion of chromosomal 14q23.1 in a female fetus with complex congenital heart defects. This is the first description of DAAM1 gene deletion associated with congenital heart anomalies. Case Presentation Compared with the control population, one CHD fetus showed a unique copy number deletion of 14q23.1, a region that harbored DAAM1 and KIAA0666 genes. Conclusions Results suggest that the copy number deletion on chromosome 14q23.1 may be critical for cardiogenesis. However, the exact relationship and mechanism of how DAAM1 and KIAA0666 deletion contributes to the onset of CHD is yet to be determined.

  14. NORMOTHERMIA FOR PEDIATRIC AND CONGENITAL HEART SURGERY: AN EXPANDED HORIZON

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    Ahmad Mahir eShamsuddin

    2015-04-01

    Full Text Available Cardiopulmonary bypass (CPB in pediatric cardiac surgery is generally performed with hypothermia, flow reduction and hemodilution. From 10/2013 to 12/2014 55 patients, median age 6 years (range 2 months to 52 years, median weight 18.5kg (range 3.2 to 57 kg, underwent surgery with normothermic high flow CPB in a new unit.There were no early or late deaths. Fifty patients (90.9% were extubated within 3 hrs, 3 (5.5% within 24 hrs and 2 (3.6% within 48 hrs. Twentyfour patients (43.6% didn’t require inotropic support, 31 (56.4% received dopamine or dobutamine: 21 ≤5 mcg/kg/min, 8 5-10 mcg/kg/min, and 2 >10 mcg/kg/min. Two patients (6.5% required noradrenaline 0.05-0.1 mcg/kg/min.On arrival to ICU and after 3 and 6 hrs and 8:00 am the next morning, mean lactate levels were 1.9±09, 2.0±1.2, 1.6±0.8 and 1.4±0.7 mmol/L (0.6-5.2 mmol/L respectively. From arrival to ICU to 8:00 am the next morning mean urine output was 3.8±1.5 mL/kg/hr (0.7-7.6 mL/kg/hr, and mean chest drainage was 0.6±0.5 mL/kg/hr (0.1-2.3 mL/kg/hr. Mean ICU and hospital stay were 2.7±1.4 days (2-8 days and 7.2±2.2 days (4-15 days respectively. In conclusion, normothermic high flow CPB allows pediatric and congenital heart surgery with favorable outcomes even in a new unit. The immediate post-operative period is characterized by low requirement for inotropic and respiratory support, low lactate production, adequate urine output, minimal drainage from the chest drains, shorter ICU and hospital stay.

  15. Late Detection of Critical Congenital Heart Disease Among US Infants

    Science.gov (United States)

    Peterson, Cora; Ailes, Elizabeth; Riehle-Colarusso, Tiffany; Oster, Matthew E.; Olney, Richard S.; Cassell, Cynthia H.; Fixler, David E.; Carmichael, Suzan L.; Shaw, Gary M.; Gilboa, Suzanne M.

    2015-01-01

    IMPORTANCE Critical congenital heart disease (CCHD) was added to the Recommended Uniform Screening Panel for Newborns in the United States in 2011. Many states have recently adopted or are considering requirements for universal CCHD screening through pulse oximetry in birth hospitals. Limited previous research is directly applicable to the question of how many US infants with CCHD might be identified through screening. OBJECTIVES To estimate the proportion of US infants with late detection of CCHD (>3 days after birth) based on existing clinical practice and to investigate factors associated with late detection. DESIGN, SETTING, AND PARTICIPANTS Descriptive and multivariable analysis. Data were obtained from a multisite population-based study of birth defects in the United States, the National Birth Defects Prevention Study (NBDPS). We included all live-born infants with estimated dates of delivery from January 1, 1998, through December 31, 2007, and nonsyndromic, clinically verified CCHD conditions potentially detectable through screening via pulse oximetry. MAIN OUTCOMES AND MEASURES The main outcome measure was the proportion of infants with late detection of CCHD through echocardiography or at autopsy under the assumption that universal screening at birth hospitals might reduce the number of such late diagnoses. Secondary outcome measures included prevalence ratios for associations between selected demographic and clinical factors and late detection of CCHD. RESULTS Of 3746 live-born infants with nonsyndromic CCHD, late detection occurred in 1106 (29.5% [95%CI, 28.1%–31.0%]), including 6 (0.2%) (0.1% –0.4%) first receiving a diagnosis at autopsy more than 3 days after birth. Late detection varied by CCHD type from 9 of 120 infants (7.5%[95%CI, 3.5%–13.8%]) with pulmonary atresia to 497 of 801 (62.0% [58.7%–65.4%]) with coarctation of the aorta. In multivariable analysis, late detection varied significantly by CCHD type and study site, and infants with

  16. THE CONGENITAL MALFORMATIONS OF THE HEART FROM THE RURAL ENVIRONMENT OF BACĂU COUNTY

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    Aura Manuela David

    2006-08-01

    Full Text Available : During 1998 – 2003 it has been realized a study on a lot of 27,623 subjects represented by children from Bacu county. There were found 128 cases of congenital malformations of the heart, from which 82 cases are coming from the rural environment. The cases of congenital malformations of the heart are found more at boys (45 than at girls (37. The children found with congenital malformations of the heart are of different ages as following: 0-1 year – 55 cases, 1-3 years – 13 cases, 3-5 years – 3 cases, 5-7 years – 3 cases, 7-10 years – 3 cases and over 10 years – 6 cases. From the cases found in the rural environment, 18 deceases have been registered (14 boys and 4 girls who were under 1 year old. The most frequent congenital malformations of the heart found and confirmed are defects of auricular and ventricular septum, the transposition of the great blood vessels, the auricular and ventricular hypoplasia, coarctation of aorta, the congenital aortic insufficiency, the tetralogy of Fallot, hypoplasic aortic stenosis, the persistence of Botall orifice and the heart’s position right part of the body. A number of 16 children have benefited of surgical treatment of reparation in special clinics from Iai, Bucureti and Târgu Mure.

  17. [Percutaneous intervention in the correction of congenital heart deffects (DCC): experience in as UMAE].

    Science.gov (United States)

    Campos-García, Vicente; Ordóñez-Toquero, Guillermo; Monjaraz-Rodríguez, Sarain; Gómez-Conde, Eduardo

    Congenital heart defects are common in infants and adults, affecting quality of life if not corrected. Unlike open surgery, percutaneous intervention allows correction with a high success rate and speedy recovery. In Mexico, there are not enough studies to describe their efficacy and safety. A cohort study was conducted in the Hospital "Manuel Avila Camacho", in Puebla, Mexico, including 149 patients with congenital heart defects repaired by percutaneous intervention, recording data from clinical records. The following were documented: post-guided fluoroscopy, hemodynamic changes, cardiac catheterization drilling anatomical changes, and complications six months later such as infection or bleeding at the puncture site, device migration, endocarditis, or death. SPSS was used, using descriptive and inferential statistics. The patients' congenital heart defects treated were ductus arteriosus, atrial septal defect, and aortic coarctation, with ductus arteriosus being recorded as the most frequent congenital heart defect. Primary angioplasties were performed in 75% and stenting in the rest. Anatomical corrections of congenital defects were successful in 96.4% of patients (p < 0.01), with minimal adverse effects (p < 0.01). We conclude that our hospital has good efficacy and safety in percutaneous intervention, comparable to published reports.

  18. Noninvasive Screening for Pulmonary Hypertension by Exercise Testing in Congenital Heart Disease.

    Science.gov (United States)

    Müller, Jan; Heck, Pinar Bambul; Ewert, Peter; Hager, Alfred

    2017-05-01

    Patients with congenital heart disease and native or palliated conditions are at risk to develop pulmonary hypertension (PH) in later life. Screening for PH is currently performed by regular echocardiographic follow-up, which appears to be difficult in several congenital conditions. This study evaluated the screening for PH in congenital heart disease by cardiopulmonary exercise testing (CPET). We analyzed our database including all patients with congenital heart disease referred for CPET in our institution from June 2001 to September 2013 and identified 683 patients who had an accompanied heart catheterization less than 6 month after CPET. Those 130 patients with proven PH were compared with the other 563 patients with congenital heart disease but without PH. Peak oxygen uptake was the most discriminative variable, showing two thresholds at 16.3 mL/min per kg and 25.2 mL/min per kg. The highest specificity of 95% for PH was found in patients with a peak oxygen uptake of 16.3 mL/min per kg or less and a breathing reserve of 37.4% or less. In patients with a peak oxygen uptake exceeding 16.3 mL/min per kg, there was a high specificity of 86.3% but a low sensitivity of 53.1%. With 25.2 mL/min per kg as the threshold, the sensitivity for PH was only 10.0%. Detection of PH in patients with congenital heart disease by CPET is difficult because of many falsely positive tests. However, a peak oxygen uptake higher than 25.2 mL/min per kg makes the diagnosis of PH unlikely. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  19. Geriatric congenital heart disease: burden of disease and predictors of mortality.

    Science.gov (United States)

    Afilalo, Jonathan; Therrien, Judith; Pilote, Louise; Ionescu-Ittu, Raluca; Martucci, Giuseppe; Marelli, Ariane J

    2011-09-27

    The study sought to measure the prevalence, disease burden, and determinants of mortality in geriatric adults with congenital heart disease (ACHD). The population of ACHD is increasing and aging. The geriatric ACHD population has yet to be characterized. Population-based cohort study using the Quebec Congenital Heart Disease Database of all patients with congenital heart disease coming into contact with the Quebec healthcare system between 1983 and 2005. Subjects with specific diagnoses of congenital heart disease and age 65 years at time of entry into the cohort were followed for up to 15 years. The primary outcome was all-cause mortality. The geriatric ACHD cohort consisted of 3,239 patients. From 1990 to 2005, the prevalence of ACHD in older adults remained constant from 3.8 to 3.7 per 1,000 indexed to the general population (prevalence odds ratio: 0.98; 95% confidence interval [CI]: 0.93 to 1.03). The age-stratified population prevalence of ACHD was similar in older and younger adults. The most common types of congenital heart disease lesions in older adults were shunt lesions (60%), followed by valvular lesions (37%) and severe congenital heart lesions (3%). Type of ACHD and ACHD-related complications had a minor impact on mortality, which was predominantly driven by acquired comorbid conditions. The most powerful predictors of mortality in the Cox proportional hazards model were: dementia (hazard ratio [HR]: 3.24; 95% CI: 1.53 to 6.85), gastrointestinal bleed (HR: 2.79; 95% CI: 1.66 to 4.69), and chronic kidney disease (HR: 2.50; 95% CI: 1.72 to 3.65). The prevalence of geriatric ACHD is substantial, although severe lesions remain uncommon. ACHD patients that live long enough acquire general medical comorbidities, which are the pre-eminent determinants of their mortality. Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  20. Chronic Hypoxemia in Children With Congenital Heart Defect Impairs Airway Epithelial Sodium Transport.

    Science.gov (United States)

    Kaskinen, Anu K; Helve, Otto; Andersson, Sture; Kirjavainen, Turkka; Martelius, Laura; Mattila, Ilkka P; Rautiainen, Paula; Pitkänen, Olli M

    2016-01-01

    Ambient hypoxia impairs the airway epithelial Na transport, which is crucial in lung edema reabsorption. Whether chronic systemic hypoxemia affects airway Na transport has remained largely unknown. We have therefore investigated whether chronic systemic hypoxemia in children with congenital heart defect affects airway epithelial Na transport, Na transporter-gene expression, and short-term lung edema accumulation. Prospective, observational study. Tertiary care medical center responsible for nationwide pediatric cardiac surgery. Ninety-nine children with congenital heart defect or acquired heart disease (age range, 6 d to 14.8 yr) were divided into three groups based on their level of preoperative systemic hypoxemia: 1) normoxemic patients (SpO2% ≥ 95%; n = 44), 2) patients with cyanotic congenital heart defect and moderate hypoxemia (SpO2 86-94%; n = 16), and 3) patients with cyanotic congenital heart defect and profound systemic hypoxemia (SpO2 ≤ 85%; n = 39). Nasal transepithelial potential difference served as a surrogate measure for epithelial Na transport of the respiratory tract. Profoundly hypoxemic patients had 29% lower basal nasal transepithelial potential difference (p = 0.02) and 55% lower amiloride-sensitive nasal transepithelial potential difference (p = 0.0003) than normoxemic patients. In profoundly hypoxemic patients, nasal epithelial messenger RNA expressions of two airway Na transporters (amiloride-sensitive epithelial Na channel and β1- Na-K-ATPase) were not attenuated, but instead α1-Na-K-ATPase messenger RNA levels were higher (p = 0.03) than in the normoxemic patients, indicating that posttranscriptional factors may impair airway Na transport. The chest radiograph lung edema score increased after congenital cardiac surgery in profoundly hypoxemic patients (p = 0.0004) but not in patients with normoxemia or moderate hypoxemia. The impaired airway epithelial amiloride-sensitive Na transport activity in profoundly hypoxemic children with

  1. Identification of adults with congenital heart disease of moderate or great complexity from administrative data.

    Science.gov (United States)

    Steiner, Jill M; Kirkpatrick, James N; Heckbert, Susan R; Habib, Asma; Sibley, James; Lober, William; Randall Curtis, J

    2018-01-01

    There is relatively sparse literature on the use of administrative datasets for research in patients with adult congenital heart disease (ACHD). The goal of this analysis is to examine the accuracy of administrative data for identifying patients with ACHD who died. A list of the International Classification of Diseases codes representing ACHD of moderate- or great-complexity was created. A search for these codes in the electronic health record of adults who received care in 2010-2016 was performed, and used state death records to identify patients who died during this period. Manual record review was completed to evaluate performance of this search strategy. Identified patients were also compared with a list of patients with moderate- or great-complexity ACHD known to have died. About 134 patients were identified, of which 72 had moderate- or great-complexity ACHD confirmed by manual review, yielding a positive predictive value of 0.54 (95% CI 0.45, 0.62). Twenty six patients had a mild ACHD diagnosis. Thirty six patients had no identified ACHD on record review. Misidentifications were attributed to coding error for 19 patients (53%), and to acquired ventricular septal defects for 11 patients (31%). Diagnostic codes incorrect more than 50% of the time were those for congenitally corrected transposition, endocardial cushion defect, and hypoplastic left heart syndrome. Only 1 of 21 patients known to have died was not identified by the search, yielding a sensitivity of 0.95 (0.76, 0.99). Use of administrative data to identify patients with ACHD of moderate or great complexity who have died had good sensitivity but suboptimal positive predictive value. Strategies to improve accuracy are needed. Administrative data is not ideal for identification of patients in this group, and manual record review is necessary to confirm these diagnoses. © 2017 Wiley Periodicals, Inc.

  2. Caloric intake during the perioperative period and growth failure in infants with congenital heart disease.

    Science.gov (United States)

    Nicholson, George T; Clabby, Martha L; Kanter, Kirk R; Mahle, William T

    2013-02-01

    Infants with congenital heart disease have impaired weight gain during the first several months of life. Efforts have focused on improving weight gain and nutritional status during the first months of life. Close examination of the data suggests that the immediate postoperative period is problematic. Etiology of this early growth failure should be identified to develop effective interventions. This is a retrospective study of neonates who underwent modified systemic-to-pulmonary artery shunt, including Norwood palliation, at Children's Healthcare of Atlanta between January 2009 and July 2011. We analyzed growth from time of surgical intervention to hospital discharge. Measures of calculated weight-for-age Z-score (WAZ score) were performed using the World Health Organization's Anthro Software (version 3.2.2, January 2011; WHO, Geneva, Switzerland). Seventy-three patients were identified. Eight patients did not meet inclusion criteria. Complete data were collected on the remaining 65 patients. Median caloric intake patients received was 50.4 [interquartile range (IQR) 41.6 to 63.6] calories/kg/day while exclusively on parental nutrition. At hospital discharge, the median WAZ score was -2.0 (IQR -2.7 to -1.2) representing an overall median WAZ score decrease of -1.3 (IQR -1.7 to -0.7) from time of shunt palliation to hospital discharge. Despite studies showing poor weight gain in infants with congenital heart disease after neonatal palliation, this study reports the impact of hospital-based nutritional practices on weight gain in infants during the immediate postoperative period. Our data demonstrate that actual caloric intake during the cardiac intensive care unit stay is substantially below what is recommended.

  3. Early postoperative arrhythmias after open heart surgery of pediatric congenital heart diseas

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    Hee Joung Choi

    2010-04-01

    Full Text Available Purpose : Early postoperative arrhythmias are a major cause of mortality and morbidity after open heart surgery in the pediatric population. We evaluated the incidence and risk factors of early postoperative arrhythmias after surgery of congenital heart disease. Methods : From January 2002 to December 2008, we retrospectively reviewed the medical records of the 561 patients who underwent cardiac surgery in Kyungpook National University Hospital. We analyzed patients' age and weight, occurrence and type of arrhythmia, cardiopulmonary bypass (CPB time, aortic cross clamp (ACC time, and postoperative electrolyte levels. Results : Arrhythmias occurred in 42 of 578 (7.3% cases of the pediatric cardiac surgery. The most common types of arrhythmia were junctional ectopic tachycardia (JET and accelerated idioventricular rhythm (AIVR, which occurred in 17 and 13 cases, respectively. The arterial switch operation (ASO of transposition of the great arteries (TGA had the highest incidence of arrhythmia (36.4%. Most cases of cardiac arrhythmia showed good response to management. Patients with early postoperative arrhythmias had significantly lower body weight, younger age, and prolonged CPB and ACC times (P &lt;0.05 than patients without arrhythmia. Although the mean duration of ventilator care and intensive care unit stay were significantly longer (P &lt;0.05, the mortality rate was not significantly different among the 2 groups. Conclusion : Early postoperative arrhythmias are a major complication after pediatric cardiac surgery; however, aggressive and immediate management can reduce mortality and morbidity.

  4. Between invisible defects and visible impact: the life experiences of adolescents and young adults with congenital heart disease.

    Science.gov (United States)

    Chiang, Yueh-Tao; Chen, Chi-Wen; Su, Wen-Jen; Wang, Jou-Kou; Lu, Chun-Wei; Li, Yuh-Fen; Moons, Philip

    2015-03-01

    To describe the life experiences of adolescents and young adults with congenital heart disease. Owing to medical advances, most children with congenital heart disease are expected to survive into adulthood. The transitional development from adolescence to adult is the critical period for fostering self-care. Descriptive phenomenological study. Thirty-five patients of 15-24 years old with congenital heart disease were recruited from paediatric cardiology clinics by purposive sampling. They were individually interviewed between October 2012-February 2013 using a semi-structured interview guideline and joined adult congenital heart disease clinics at two medical centres in northern Taiwan. The data were analysed using descriptive phenomenological method developed by Giorgi. The essence of the life experience of adolescents and young adults with congenital heart disease involves a dynamic process of moving between invisible defects and coexistence with the disease. Six themes emerged: (1) invisible defects: the existence of imperfect understanding; (2) conflict: interpersonal frustrations; (3) imbalance: the loss of self-balance; (4) suffering: increasing anxiety; (5) encounters: meeting needs; and (6) coexistence: positive coping strategies. As patients with congenital heart disease transition from adolescence into adulthood, they must learn about their disease, overcome frustration and anxiety and develop self-care strategies for coexisting with congenital heart disease. Results of this study may serve as clinical care guidelines for adolescents and young adults with congenital heart disease and give a reference for developing transitional intervention strategies. © 2014 John Wiley & Sons Ltd.

  5. Antenatal diagnosis of congenital heart disease and Down's syndrome: the potential effect on the practice of paediatric cardiology.

    Science.gov (United States)

    Abu-Harb, M.; Wyllie, J.; Hey, E.; Richmond, S.; Wren, C.

    1995-01-01

    OBJECTIVE--To predict the effect of antenatal ultrasound screening for congenital heart disease and maternal serum screening of Down's syndrome on the practice of paediatric cardiology and paediatric cardiac surgery. DESIGN--A retrospective and prospective ascertainment of all congenital heart disease diagnosed in infancy in 1985-1991. SETTING--One English health region. PATIENTS--All congenital heart disease diagnosed in infancy by echocardiography, cardiac catheterisation, surgery, or necropsy was classified as "complex", "significant", or "minor" and as "detectable" or "not detectable" on a routine antenatal ultrasound scan. RESULTS--1347 infants had congenital heart disease which was "complex" in 13%, "significant" in 55%, and "minor" in 32%. 15% of cases were "detectable" on routine antenatal ultrasound. Assuming 20% detection and termination of 67% of affected pregnancies, liveborn congenital heart disease would be reduced by 2%, infant mortality from congenital heart disease by 5%, and paediatric cardiac surgical activity by 3%. Maternal screening for Down's syndrome, assuming 75% uptake, 60% detection, and termination of all affected pregnancies, would reduce liveborn cases of Down's syndrome by 45%, liveborn cases of congenital heart disease by 3.5%, and cardiac surgery by 2.6%. CONCLUSIONS--Screening for congenital heart disease using the four chamber view in routine obstetric examinations and maternal serum screening for Down's syndrome is likely to have only a small effect on the requirements for paediatric cardiology services and paediatric cardiac surgery. PMID:7547001

  6. Psychosocial aspects of congenital heart disease in adulthood: A longitudinal cohort study of 20-33 years follow-up

    NARCIS (Netherlands)

    E.H.M. van Rijen (Susan)

    2003-01-01

    markdownabstract__Abstract__ Adults with congenital heart disease form a rather new phenomenon. Improvements in surgical techniques over the last decades have lead to lower mortality rates for children born with a congenital heart disease, enabling more of them to grow into adulthood

  7. The role of cardiac surgery in adult patients with congenital heart disease.

    Science.gov (United States)

    Padalino, Massimo A; Vida, Vladimiro L; Lo Rito, Mauro; Daliento, Luciano; Stellin, Giovanni

    2013-05-01

    The number of adults with congenital heart disease (CHD) has constantly increased as medical and surgical treatment of CHD - either simple or complex - continues to improve. Over the past half century, advances in surgical techniques have continued with the evolution of traditional surgical repair and introduction of new surgical procedures for complex lesions which were previously considered to be irreparable. We sought to analyze the current role of cardiac surgery in the treatment of congenital anomalies of the heart in those patients who have reached adulthood with or without surgical repair or palliation, with particular attention to future directions and perspectives.

  8. Thrombotic and Thromboembolic Complications in Patients with Adult Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Behnood Bikdeli

    2017-02-01

    Full Text Available Improved medical and surgical interventions have increased the longevity of patients with congenital heart defects and most such patients live into adulthood. Thrombotic and thromboembolic complications constitute a major cause of mortality and morbidity in patients with adult congenital heart disease (ACHD. Such events include acute coronary syndromes, ischemic stroke (cardioembolic due to thrombus formation in the systemic ventricle, secondary to atrial arrhythmias, or due to paradoxical embolism, and venous thromboembolism. Some thrombotic phenomena are also specific to patients with ACHD, such as those related to Fontan circulation. We provide a succinct overview of thrombotic and thromboembolic complications in patients with ACHD, focusing on stroke and venous thromboembolic events.

  9. Congenital Heart Disease Requiring Maintenance of Ductus Arteriosus in Critically Ill Newborns Admitted at A Tertiary Neonatal Intensive Care Unit

    Directory of Open Access Journals (Sweden)

    Cucerea Manuela

    2016-10-01

    Full Text Available Introduction: Congenital heart diseases (CHD have been reported to be responsible for 30 to 50% of infant mortality caused by congenital disabilities. In critical cases, survival of newborns with CHD depends on the patency of the ductus arteriosus (PDA, for maintaining the systemic or pulmonary circulation. The aim of the study was to assess the efficacy and side effects of PGE (prostaglandin E administration in newborns with critical congenital heart disease requiring maintenance of the ductus arteriosus.

  10. Congenital Heart Disease Requiring Maintenance of Ductus Arteriosus in Critically Ill Newborns Admitted at A Tertiary Neonatal Intensive Care Unit

    OpenAIRE

    Cucerea Manuela; Simon Marta; Moldovan Elena; Ungureanu Marcela; Marian Raluca; Suciu Laura

    2016-01-01

    Introduction: Congenital heart diseases (CHD) have been reported to be responsible for 30 to 50% of infant mortality caused by congenital disabilities. In critical cases, survival of newborns with CHD depends on the patency of the ductus arteriosus (PDA), for maintaining the systemic or pulmonary circulation. The aim of the study was to assess the efficacy and side effects of PGE (prostaglandin E) administration in newborns with critical congenital heart disease requiring maintenance of the d...

  11. The evolving role of the total artificial heart in the management of end-stage congenital heart disease and adolescents.

    Science.gov (United States)

    Ryan, Thomas D; Jefferies, John L; Zafar, Farhan; Lorts, Angela; Morales, David L S

    2015-01-01

    Advances in medical therapies have yielded improvement in morbidity and a decrease in mortality for patients with congenital heart disease, both surgically palliated and uncorrected. An unintended consequence is a cohort of adolescent and adult patients with heart failure who require alternative therapies. One intriguing option is placement of a total artificial heart (TAH) either as a bridge to transplant or as a destination therapy. Of the 1091 Jarvik-7 type TAH (Symbion, CardioWest and SynCardia) placed between 1985 and 2012, only 24 have been performed in patients with congenital heart disease, and a total of 51 were placed in patients younger than 21. At our institution, the SynCardia TAH was implanted in a 19-year-old patient with cardiac allograft failure because of chronic rejection and related multisystem organ failure including need for hemodialysis. Over the next year, she was nutritionally and physically rehabilitated, as were her end organs, allowing her to come off dialysis, achieve normal renal function and eventually be successfully transplanted. Given the continued growth of adolescent and adult congenital heart disease populations with end-stage heart failure, the TAH may offer therapeutic options where previously there were few. In addition, smaller devices such as the SynCardia 50/50 will open the door for applications in smaller children. The Freedom Driver offers the chance for patients to leave the hospital with a TAH, as does the AbioCor, which is a fully implantable TAH option. In this report, we review the history of the TAH and potential applications in adolescent patients and congenital heart disease.

  12. Why should we care about ethical and policy challenges in congenital heart disease?

    Science.gov (United States)

    Kirkpatrick, James N; Kaufman, Beth

    2013-01-01

    Congenital heart disease (CHD) affects 1% of infants worldwide, and approximately 90% of children with serious CHD who have access to surgery survive to adulthood. Particularly as this population ages, there are unique ethical and policy challenges pertaining to this diverse population of children and adults, which also serve as a paradigm for other chronic diseases. A unique forum to discuss these issues occurred at the University of Pennsylvania in Philadelphia on March 16 to 17, 2012, and was entitled "Ethics of the Heart: Ethical and Policy Challenges in Adult and Pediatric Congenital Heart Disease." The conference convened a multidisciplinary panel of nationally known experts in the fields of Pediatric Congenital Heart Disease, Adult Congenital Heart Disease, and Bioethics to identify and discuss the most important ethical issues in CHD through talks, panel discussions, and one-on-one interviews in six topic areas: genetic testing, transitions of care from pediatric to adult CHD, transplantation and mechanical circulatory support, research and development in CHD, the social and personal costs of success in treating CHD, and end-of-life considerations. This article is an introduction to the topics discussed.

  13. Repair of congenital heart disease with associated pulmonary hypertension in children: what are the minimal investigative procedures? Consensus statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI).

    Science.gov (United States)

    Lopes, Antonio Augusto; Barst, Robyn J; Haworth, Sheila Glennis; Rabinovitch, Marlene; Al Dabbagh, Maha; Del Cerro, Maria Jesus; Ivy, Dunbar; Kashour, Tarek; Kumar, Krishna; Harikrishnan, S; D'Alto, Michele; Thomaz, Ana Maria; Zorzanelli, Leína; Aiello, Vera D; Mocumbi, Ana Olga; Santana, Maria Virginia T; Galal, Ahmed Nasser; Banjar, Hanaa; Tamimi, Omar; Heath, Alexandra; Flores, Patricia C; Diaz, Gabriel; Sandoval, Julio; Kothari, Shyam; Moledina, Shahin; Gonçalves, Rilvani C; Barreto, Alessandra C; Binotto, Maria Angélica; Maia, Margarida; Al Habshan, Fahad; Adatia, Ian

    2014-06-01

    Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

  14. A survey to evaluate the management of orthodontic patients with a history of rheumatic fever or congenital heart disease.

    Science.gov (United States)

    Gaidry, D; Kudlick, E M; Hutton, J G; Russell, D M

    1985-04-01

    A survey concerning the orthodontic management of patients with a history of rheumatic fever or congenital heart disease was mailed to a random sample of 500 orthodontists throughout the United States. Two hundred thirty-two surveys were completed and returned. Analysis revealed that 88% of the respondents were aware that they should screen for patients with a history of rheumatic fever or congenital heart disease, only 11% used the antibiotic regimen recommended in 1977 by the American Heart Association (see Fig. 2), one half of the respondents thought it was necessary to provide antibiotic coverage during banding, and 38% deemed it necessary for debanding procedures which were likely to cause some degree of bleeding from the gingival sulcus. Only one half of the respondents would discontinue treatment if a condition of gingivitis developed which did not show signs of improvement, and only 70% provided medication for orthodontic procedures that could result in bacteremia. Furthermore, the results of this survey indicated that there was a great variation in the antibiotic regimen of orthodontic treatment for patients at risk of developing endocarditis and that there was a need to make the orthodontist more aware of the 1977 recommendations of the American Heart Association. Finally, a comparison between the year of graduation from an orthodontic program and the number of respondents who did not screen for or did not premedicate at-risk patients revealed a direct relationship. The earlier the education, the smaller the number of respondents who screened or premedicated.(ABSTRACT TRUNCATED AT 250 WORDS)

  15. Evaluation of Complications of Heart Surgery in Children With Congenital Heart Disease at Dena Hospital of Shiraz.

    Science.gov (United States)

    Mirzaei, Maryam; Mirzaei, Samaneh; Sepahvand, Elham; Rahmanian Koshkaki, Afifeh; Kargar Jahromi, Marzieh

    2015-08-23

    Today, with progress in the field of congenital heart surgery, different complicated actions are done in children. These actions may be associated with several complications, especially open heart surgery in which the cardiopulmonary bypass (CPB) is used. Serious complications can be caused high morbidity and mortality rates. Present study has been performed to determine the incidence of morbidity and mortality in cardiac surgery in children. In a cross-sectional retrospective, records of 203 patients undergoing surgery for congenital heart disease in Dena hospital during 2013-2015 were reviewed for incidence of complications. Data was analyzed by using descriptive and analytical statistics and using SPSS version 18. The mean age of samples was 3/65±4/47 years. The majority of samples (73/8%) were undergoing open surgery. The overall adverse cardiovascular complications were respectively, renal complications (44/3%), lung (40/3%), anemia (35/9%), heart (34/4%), gastrointestinal (17/2%), brain (14/2%), need for re-intubation of the trachea 11/3%), infection (7/8%) required reoperation (5/9%) and vascular complications (1/4%). High incidence of complications after congenital heart surgery makes necessary attention to complications and their treatment after surgery. It is necessary to apply the measures and careful monitoring of patients to minimize these effects.

  16. Incidence of congenital heart disease in newborns after pulse oximetry screening introduction.

    Science.gov (United States)

    Methlouthi, Jihene; Mahdhaoui, Nabiha; Bellaleh, Manel; Guith, Aida; Zouari, Douha; Ayech, Hedia; Nouri, Sonia; Séboui, Hassen

    2016-03-01

    Congenital heart defects are the most common congenital malformations in the newborn with an estimated incidence ranging between 6-9 ‰. In Tunisia, this incidence reaches 1.9 ‰. This can be explained by misdiagnosed cases. In fact, the diagnosis is based mainly on clinical examination which allows making the diagnosis only in 50 in 75 % of the cases. Several studies showed the interest of pulse oximetry oximeter in the screening of cyanogenic and duct dependent congenital heart diseases. The Objective of our work is to determine the incidence of congenital heart diseases screened by physical examination coupled with the measure of transcutaneous saturation Methods:  forward-looking and descriptive Study, concerning all the births of the central maternity of Sousse, during a period of 12 months. We have measured transcutaneous saturation of all the newborn children in association to routine physical, examination. This study aimed to screen congenital heart disease and then calculate their incidence. during the period of the study 10447 newborn children had been born in the central maternity of Sousse. Among whom, 26 had a congenital heart defect. The incidence of the neonatal cardiac malformations in our current study was found in 2.77 ‰ of alive births. It was about 1.1 ‰ of newborns in a similar study practised in 2009 (Chi2=76.2 et pheart disorder was diagnosed following the grip taking of the only percutaneous saturation in 5 newborn children and following the clinical examination in 21. the measure of the transcutaneous saturation in the first day of life is a little expensive and specific method for the early screening of the duct dependent cardiac malformations.

  17. Congenital Heart Diseases in the Newborns of Diabetic Mothers: an Echocardiographic Study

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    S Rahimpour

    2011-10-01

    Full Text Available Introduction: Despite the discovery of insulin and current improvement in diabetics care, congenital malformations in diabetics are still more frequent than in the general population. The aim of this study was to identify congenital heart dieases (CHD in the newborns of diabetic mothers (IDMS. Methods: In our prospective study, color doppler echocardiography was performed in 75 consecutive full- term newborns of diabetic mothers by GE Vivid3 echocardiographic device. Newborns were classified into two subgroups according to the type of the mothers’ diabetes: pre-gestational and gestational. They were also those were classified into three subgroups according to their birth weight: appropriate, large and small for gestational age. Data analysis was made by Fisher exact test and Chi-Square test. Results: Forty nine (65% and thirty six (35% of subjects were infants of gestational (IGDM and pre-gestational diabetic mothers (IPDM, respectively. Fifty five Newborns (73% were apropriate, fourteen (19% were large and six (8% were small for gestational age. The most common echocardiographic findings included: patent ductus arteriosus (PDA: 54.7%, hypertrophic cardiomyopathy (HCMP: 24%, ventricular septal defect (VSD: 4%, atrial septal defect (ASD: 2.7%, transposition of great arteries (TGA: 1.3% and coarctation of the Aorta (COA: 1.3%. Overall incidence of congenital heart diseases was 9.3 after exclusion of PDA and HCMP cases. The incidence of congenital heart diseases was higher in macrosomic than nonmacrosomic infants of diabetic mothers (P<0.001. Congenital heart diseases were more common in infants of pre-gestational than gestational diabetic mothers (P=0.004. Conclusion: Our results showed that diabetic mothers are at increased risk of giving birth to a newborn with congenital heart disease, and transthoracic echocardiography is recommended for all infants of diabetic mothers.

  18. Complex congenital heart defect, heterotaxy, imperforate anus, and other congenital anomalies in a 27-week infant: a case study.

    Science.gov (United States)

    Koerner, Angela

    2014-01-01

    According to multiple researchers and studies, congenital heart disease (CHD) occurs in approximately 4.8-12.0 of 1,000 live births in the general population, and 2.4 per 1,000 cases are serious enough to require surgery or cardiac catheterization in the first year of life.1 Historically, it has been assumed that the earlier the gestational age with CHD, the poorer the outcome; however, with continued improvements in neonatal care, this hypothesis should be looked at more closely. This case illustrates the challenges associated with prematurity, complex cardiac defects, intraventricular hemorrhage (IVH), and other congenital anomalies that increase the risk of infection and/or surgical intervention. It will discuss the hospital course of a twin, born at 27 weeks gestation, who was found to have all of these diagnoses, yet, despite the complexity of his case, he had a predominantly uncomplicated hospital course.

  19. Left-right asymmetry and cardiac looping: implications for cardiac development and congenital heart disease.

    Science.gov (United States)

    Kathiriya, I S; Srivastava, D

    2000-01-01

    Proper morphogenesis and positioning of internal organs requires delivery and interpretation of precise signals along the anterior-posterior, dorsal-ventral, and left-right axes. An elegant signaling cascade determines left- versus right-sided identity in visceral organs in a concordant fashion, resulting in a predictable left-right (LR) organ asymmetry in all vertebrates. The complex morphogenesis of the heart and its connections to the vasculature are particularly dependent upon coordinated LR signaling pathways. Disorganization of LR signals can result in myriad congenital heart defects that are a consequence of abnormal looping and remodeling of the primitive heart tube into a multi-chambered organ. A framework for understanding how LR asymmetric signals contribute to normal organogenesis has emerged and begins to explain the basis of many human diseases of LR asymmetry. Here we review the impact of LR signaling pathways on cardiac development and congenital heart disease.

  20. Prediction of posthypoxic cardiopathy of newborns of mothers with congenital heart disease

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    E. V. Kostousova

    2015-01-01

    Full Text Available Neonatal infants of women with congenital heart disease underwent clinical and instrumental examinations to predict the types of adaptation of the cardiovascular system in the early neonatal period. A study group comprised 125 newborns of mothers with CHD; a control group consisted of 50 babies of somatically healthy mothers. The paper describes different types of adaptation, their characteristics and frequency in the infants born to mothers with heart diseases depending on whether maternal on-line defect correction is made. The newborns of mothers with congenital heart diseases are shown to be a high risk group for heart diseases in the early neonatal period. At the same time, blood pressure and echocardiographic parameters and the level of vasoregulators are of high informative value in diagnosing the types of adaptation of the cardiovascular system at the preclinical stage.