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Sample records for adult congenital heart

  1. Adult congenital heart disease

    OpenAIRE

    Morphet, John AM

    2006-01-01

    One million people over the age of 20 suffer from congenital heart disease in the United States. These adult patients can slip through the cracks of our medical system; many are too old to be cared for in most pediatric institutions by pediatric cardiologists and, unfortunately, most adult cardiologists are not trained in congenital heart disease. Therefore, it is important to identify the common lesions in adult congenital heart disease and how they should be managed. Acyanotic congenital he...

  2. 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. PMID:27451323

  3. Gender differences in adult congenital heart disease

    NARCIS (Netherlands)

    P. Engelfriet; B.J.M. Mulder

    2009-01-01

    Objective. To assess gender differences in morbidity, mortality and patient management among adults born with a heart defect. Methods and results. The database of the European Heart Survey on adult congenital heart disease was explored. This contains data on 4110 patients with one of eight congenita

  4. 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 diff......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...

  5. The changing state of surgery for adult congenital heart disease

    OpenAIRE

    Monro, J

    2005-01-01

    The number of patients with adult congenital heart disease is expected to increase by 25% during this decade, so that by 2010 there will be more patients undergoing surgery for congenital heart disease over 16 years of age than under

  6. Evaluation of Adults With Congenital Heart Disease.

    Science.gov (United States)

    Graziani, Francesca; Delogu, Angelica Bibiana

    2016-03-01

    The clinical approach to adults with congenital heart diseases (ACHDs) is unique in cardiovascular medicine because these patients encompass a broad range of presentations. Each patient, despite having similar diagnosis, will be anatomically and physiologically unlike others within ACHD population, in relation to the type of repair, age at repair, associated defects, with specific long-term risk factors and complications. Furthermore, as many patients will not complain of symptoms, clinical evaluation and diagnostic testing must also be based on the underlying main diagnostic category, with complete standardized lesion-specific clinical protocols, investigating all known risk factors specific for each congenital heart disease and performed as part of screening for significant long-term complications. The first part of this review will focus on clinical history, physical examination, and the most important diagnostic testing in ACHD population. The second part of the article will focus on some clinical issues we have to face in our daily practice, such as heart failure, cyanosis, and pulmonary hypertension. Furthermore, as survival rates of ACHD population continue to improve and patients with this condition live longer, we will briefly report on a new clinical concern regarding the impact of acquired morbidities like coronary artery disease that appear to be of greater importance in defining outcome in older patients with ACHD. PMID:26957402

  7. Cardiac arrhythmias in adults with congenital heart disease

    NARCIS (Netherlands)

    Z. Koyak

    2016-01-01

    Arrhythmias are a major cause of hospital admissions and morbidity in adults with congenital heart disease (CHD). Furthermore, the leading cause of death in adults with CHD is sudden cardiac death (SCD) of presumed arrhythmic aetiology. The main objectives of this thesis were to identify risk factor

  8. 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.

  9. 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.

  10. [Adult patients with congenital heart disease].

    Science.gov (United States)

    Grabitz, R G; Kaemmerer, H; Mohr, F-W

    2013-01-01

    Unlike a few decades ago, today most patients with congenital heart disease reach adulthood after intervention or reparative surgery. As complete correction is generally not possible, a patient population with great complexity and a particular challenge to medical management is rising and a regular follow-up is mandatory. The aim of care is the timely recognition of residual or associated problems. Frequency and intensity of follow-up examinations depend on type and complexity of the lesion. The standard repertoire at follow-up consists of a specific history, clinical examination, ECG, Holter-monitoring, exercise tests, and echocardiography. Depending on the indication, cardio-MRI, CT scan, and sophisticated cardiac catheterization may become necessary. Long-term complications like rhythm disturbances, pulmonary hypertension, or heart failure are frequent, despite optimal care. Acute complications like arrhythmias, infective endocarditis, cerebral events, cerebral abscesses, aortic dissection, pulmonary embolism, and bleeding have to be recognized early and treated appropriately. Additional focus has to be placed on counseling and management of noncardiac disease and surgery, pregnancy and delivery, exercise at work and in private life, driving, and insurance issues. Training and certification of physicians as well as the establishment of specialized centers will help to ensure high quality health care for the affected patient population. PMID:23318541

  11. Advances in the Care of Adults With Congenital Heart Disease.

    Science.gov (United States)

    Nasr, Viviane G; Kussman, Barry D

    2015-09-01

    The significant decline in mortality among children and adolescents with congenital heart disease (CHD) is associated with an increasing prevalence of CHD in adults, particularly those with moderate to severe defects. As a significant percentage of adolescents and young adults are lost to follow-up in the transition from pediatric to adult care, they may present for elective procedures with substantial CHD-associated morbidity. In addition to the specific cardiac defect, the procedures performed, and the current pathophysiological status, several factors should be considered when managing the adult with CHD. These include the type of setting (adult vs pediatric institution); surgeon (pediatric vs adult cardiac surgeon); coexisting diseases associated with CHD, such as coronary artery disease, hepatic dysfunction, renal dysfunction, cerebrovascular accidents, myopathy, and coagulation disorders; acquired diseases of aging; pregnancy; and psychosocial functioning. The current status of the management of common and important congenital cardiac defects is also described. PMID:25542866

  12. 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); Domburg, R.V. (Ron Van); 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, Tet

  13. Clinical features of adult patients with Eisenmenger syndrome associated with different types of congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    陈果

    2013-01-01

    Objective To explore the clinical features and hemodynamics of adult patients with Eisenmenger syndrome in different types of congenital heart diseases (CHD) .Methods Patients with Eisenmenger syndrome with different types of CHD diagnosed by right heart

  14. 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.

  15. 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.

  16. Transfer to Adult Care--Experiences of Young Adults with Congenital Heart Disease.

    Science.gov (United States)

    Asp, Ann; Bratt, Ewa-Lena; Bramhagen, Ann-Cathrine

    2015-01-01

    More than 90% of children born with congenital heart disease survive into adulthood due to successes of cardiac surgery and medical management. Interviews with 16 young adults with congenital heart disease to explore their experiences of transfer from pediatric to adult care were performed. The analysis identified five themes; Feeling secure during the transfer process, Experiencing trust in the care, Expecting to be involved, Assuming responsibility for one's health is a process and Lack of knowledge leads to uncertainty. In conclusion; a structured and gradual transfer process was necessary to enable the informants to shoulder the responsibility for self-care.

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

    NARCIS (Netherlands)

    F. Meijboom; B.J.M. Mulder

    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 patien

  18. Physical performance, physical activity, body composition and exercise training in adults with congenital heart disease

    OpenAIRE

    Sandberg, Camilla

    2016-01-01

    Background Adults with congenital heart disease (CHD) is a growing population and related to advances in surgical and medical treatment, they now outnumber the children with corresponding lesions. Since a congenital heart lesion often results in reduced exercise capacity, this population is a potential target for physiotherapy. To what extent this reduction in exercise capacity is caused by abnormal cardiovascular anatomy and physiology or to what degree insufficient physical activity contrib...

  19. Adult congenital heart disease: A paradigm of epidemiological change.

    Science.gov (United States)

    Ntiloudi, Despοina; Giannakoulas, George; Parcharidou, Despοina; Panagiotidis, Theofilos; Gatzoulis, Michael A; Karvounis, Haralambos

    2016-09-01

    Increasing survival rates for patients with congenital heart disease (CHD) represent a major achievement of modern medicine. Despite incredible progress been made in diagnosis, follow-up, early treatment and management in adulthood, many patients are faced with long-term complications, such as arrhythmia, thromboembolism, heart failure, pulmonary hypertension, endocarditis and/or the need for reoperation. In parallel, half of the patients are female, most of childbearing age, and, thus warrant specialist reproductive counseling and appropriate obstetric care. It is not surprising therefore, that healthcare utilization has steadily increased for CHD in recent years. Furthermore, cardiology and other medical disciplines are now faced with new challenges, namely the provision of expert care and optimal, lifelong medical surveillance for these patients. PMID:27240150

  20. 3D Printing to Guide Ventricular Assist Device Placement in Adults With Congenital Heart Disease and Heart Failure.

    Science.gov (United States)

    Farooqi, Kanwal M; Saeed, Omar; Zaidi, Ali; Sanz, Javier; Nielsen, James C; Hsu, Daphne T; Jorde, Ulrich P

    2016-04-01

    As the population of adults with congenital heart disease continues to grow, so does the number of these patients with heart failure. Ventricular assist devices are underutilized in adults with congenital heart disease due to their complex anatomic arrangements and physiology. Advanced imaging techniques that may increase the utilization of mechanical circulatory support in this population must be explored. Three-dimensional printing offers individualized structural models that would enable pre-surgical planning of cannula and device placement in adults with congenital cardiac disease and heart failure who are candidates for such therapies. We present a review of relevant cardiac anomalies, cases in which such models could be utilized, and some background on the cost and procedure associated with this process. PMID:27033018

  1. Efficacy of Antiarrhythmic Drugs in Adults With Congenital Heart Disease and Supraventricular Tachycardias

    NARCIS (Netherlands)

    Koyak, Zeliha; Kroon, Bart; de Groot, Joris R.; Wagenaar, Lodewijk J.; van Dijk, Arie P.; Mulder, Bart A.; Van Gelder, Isabelle C.; Post, Marco C.; Mulder, Barbara J. M.; Bouma, Berto J.

    2013-01-01

    Supraventricular tachycardias (SVTs) are a major cause of morbidity in adults with congenital heart disease (CHD). Few data exist on safety and efficacy of antiarrhythmic drugs in this population. Our aim was to determine the efficacy of antiarrhythmic drugs in adults with CHD and first-onset SVT on

  2. Exercise prescription in adults with congenital heart disease: a long way to go

    OpenAIRE

    Swan, L.; Hillis, W

    2000-01-01

    OBJECTIVE—To determine if appropriate advice had been given to adults with congenital heart disease regarding safe and effective exercise, and to assess pre-existing misconceptions of the potential benefits and dangers of exercise.
DESIGN—An anonymous self assessment questionnaire.
SETTING—A tertiary referral clinic.
PATIENTS—99 adults (57 men, 42 women) with congenital heart disease, mean age 25.6 years.
MAIN OUTCOME MEASURES—The extent and nature of exercise advice given over previous years...

  3. Transitioning the young adult with congenital heart disease for life-long medical care.

    Science.gov (United States)

    Fernandes, Susan M; Landzberg, Michael J

    2004-12-01

    Guidelines for the successful orchestration of transitioning of the adolescent and young and older adult patient with congenital heart disease to a health care system appropriate for their long-term congenital heart disease care and counseling appear necessary to improve patient and family confidence, education, therapy, life quality, and survival outcomes. Schema for care organization and delivery for adult patients with congenital heart disease remain primitive and largely unimplemented. The presence of a strong central care oversight organization and the establishment of a multi-institutional database to assist in assessment of care outcomes and guidelines appears requisite to these needs and for the establishment of transitioning guidelines for these patients as they assume a greater and deeper shared control of their futures with their caregivers.

  4. Understanding age-based transition needs: Perspectives from adolescents and adults with congenital heart disease

    Science.gov (United States)

    The purpose of this study was to explore the transition process in congenital heart disease (CHD) care through the perceived needs and concerns of adolescents (pretransition) and the experiential insight from adults (post-transition), in order to inform future transition initiatives and information ...

  5. 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.

  6. Nationwide Hospitalization Trends in Adult Congenital Heart Disease Across 2003–2012

    OpenAIRE

    Agarwal, Shikhar; Sud, Karan; Menon, Venu

    2016-01-01

    Background We aimed to assess trends in hospitalization, outcomes, and resource utilization among patients admitted with adult congenital heart disease (ACHD). Methods and Results We used the 2003–2012 US Nationwide Inpatient Sample for this study. All admissions with an ACHD were identified using standard ICD codes. Resource utilization was assessed using length of stay, invasive procedure utilization, and cost of hospitalization. There was a significant increase in the number of both simple...

  7. Arrhythmias in Adult Congenital Heart Disease: Diagnosis and Management.

    Science.gov (United States)

    Kumar, Saurabh; Tedrow, Usha B; Triedman, John K

    2015-11-01

    Cardiac arrhythmias are a major source of morbidity and mortality in adults with CHD. A multidisciplinary approach in a center specializing in the care of ACHD is most likely to have the expertise needed provide this care. Knowledge of the underlying anatomy, mechanism of arrhythmia, and potential management strategies is critical, as well as access and expertise in the use of advanced imaging and ablative technologies. Future challenges in management include refining the underlying mechanism and putative ablation targets for catheter ablation of AF, an arrhythmia rapidly rising in prevalence in this population.

  8. Coronary artery problems and disease in adults with congenital heart disease: how to evaluate, how to prevent, how to treat.

    Science.gov (United States)

    Cataldo, S; Stuart, A G

    2014-10-01

    There are a wide variety of coronary artery anomalies and disease in adults with congenital heart disease (CHD). In fact, the increasing burden of acquired coronary artery disease (CAD) has to be considered in addition to congenital abnormalities of the coronary arteries, isolated or associated to other congenital diseases. This is largely a consequence of the increasing number of patients reaching older age. Due to complex underlying cardiac anatomy, previous surgery and comorbidities, treatment can be challenging. Individualized and multidisciplinary management involving congenital heart cardiologists, cardiac surgeons, coronary interventionists and imaging specialists is essential. This review gives an overview of coronary artery involvement in adults with CHD, summarizes the current literature and focuses on prevention, diagnosis and treatment. The potential role of cardiovascular risk factors for CAD is also discussed.

  9. Congenital heart disease

    Science.gov (United States)

    ... blood sugar level. Certain genes may play a role in congenital heart disease. Many family members may be affected. Talk to your provider about genetic counseling and screening if you have a family history ...

  10. Radiologic evaluation of coronary artery disease in adults with congenital heart disease.

    Science.gov (United States)

    Valenzuela, David M; Ordovas, Karen G

    2016-01-01

    Improved surgical and medical therapy have prolonged survival in patients with congenital heart disease (CHD) such that general medical conditions like coronary artery disease (CAD) are now the main determinants of mortality. A summary of the association of CAD with CHD, as well as a discussion of the radiologic evaluation of the coronary arteries in adults with CHD is described herein. Cross sectional imaging to evaluate CAD in adults with CHD should follow the same appropriateness criteria as gender and aged matched patients without CHD. Coronary CT imaging may be particularly valuable in evaluating the coronary arteries in this patient population as invasive coronary angiography may prove challenging secondary to complicated or unconventional anatomy of the coronary arteries. Further, typical methods for evaluating CAD such as stress or echocardiography may be impractical in adults with CHD. Finally, delineating the anatomic relationship of the coronary arteries and their relationship with the sternum, chest wall, conduits, grafts, and valves is highly recommended in patients with CHD prior to reintervention to avoid iatrogenic complications.

  11. Congenital heart defect corrective surgeries

    Science.gov (United States)

    ... open Arrhythmias Atrial septal defect (ASD) Breathing difficulty Cardiac catheterization Cardiovascular Coarctation of the aorta Congenital heart disease Heart failure - overview Heart transplant Hypoplastic left heart syndrome Patent ductus arteriosus Pediatric ...

  12. Congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    1997-01-01

    970296 Evaluating the degree of pulmonary vascularlesions in congenital heart disease with selective pul-monary angiography. PAN Shiwei(潘世伟), et al.Fuwai Hosp, CAMS & PUMC, Beijing, 100037. Chin JCardiol 1997; 25(1): 39-41. Objective: To evaluate the degree of pulmonary vas-

  13. Congenital Heart Information Network

    Science.gov (United States)

    ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright ©1996 - 2016 C.H.I.N. All rights reserved TX4-390-685 Original site design and HTML by Panoptic Communications

  14. Clinical countermeasures against congenital heart disease in adults%成人先天性心脏病临床对策

    Institute of Scientific and Technical Information of China (English)

    徐仲英

    2013-01-01

    Congenital heart disease is common among the newborns, and the incidence rate is about 0. 8%. Over the past 30 years,great progress has been made in the diagnosis and treatment of congenital heart disease in children. As a result,many children with such diseases now survive to adulthood. In the United States alone, the population of adults with congenital heart disease, either surgically corrected or uncorrected, is estimated to be increasing at a rate of about 5 percent every year; in 2008 there had be almost 1 million such patients. Currently in China, although there is no statistical data of incidence of adult congenital heart disease,the number of such patients must be huge. This article discusses congenital heart disease from two aspects: acyanotic and cyanotic;and clinical countermeasures against each type will be described.%先天性心脏病比较常见的,在新生儿中发生率约0.8%.在过去的30年,儿童先天性心脏病诊断和治疗取得了巨大进步,许多孩子因此可以存活到成年.仅在美国,经矫治或未矫治的先天性心脏病成人数,预计将以每年约5%的速度增长,2008已达近100万例患者.中国还没有成人先天性心脏病的统计数据,但患者数目必须是巨大的.文章从紫绀型和紫绀型2个方面阐述常见成人先天性心脏病的临床对策.

  15. Challenges Caring for Adults With Congenital Heart Disease in Pediatric Settings: How Nurses Can Aid in the Transition.

    Science.gov (United States)

    Anton, Kristin

    2016-08-01

    As surgery for complex congenital heart disease is becoming more advanced, an increasing number of patients are surviving into adulthood, yet many of these adult patients remain in the pediatric hospital system. Caring for adult patients is often a challenge for pediatric nurses, because the nurses have less experience and comfort with adult care, medications, comorbid conditions, and rehabilitation techniques. As these patients age, the increased risk of complications and comorbid conditions from their heart disease may complicate their care further. Although these patients are admitted on a pediatric unit, nurses can aid in promoting their independence and help prepare them to transition into the adult medical system. Nurses, the comprehensive medical teams, and patients' families can all effectively influence the process of preparing these patients for transition to adult care. PMID:27481810

  16. 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 depend

  17. Genetics Home Reference: critical congenital heart disease

    Science.gov (United States)

    ... Home Health Conditions critical congenital heart disease critical congenital heart disease Enable Javascript to view the expand/collapse boxes. Print All Open All Close All Description Critical congenital heart disease (CCHD) is a term that refers to a ...

  18. Perioperative Anaesthetic Management of a Patient of Gilbert’s Syndrome with Adult Congenital Heart Disease - A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Sambhunath Das

    2014-11-01

    Full Text Available Gilbert's syndrome is a hereditary condition with the genetic mutation of the enzyme uridine diphosphate glucuronosyltransferase, characterized by intermittent jaundice in the absence of hemolysis or underlying liver disease. These patients develop jaundice when subjected to fasting, stress and exercise. Majority of anaesthetics are metabolized by liver. Anaesthesia, surgery and cardiopulmonary bypass (CPB can act as triggers to hepatic injury. The successful perioperative management of an adult congenital heart disease patient for atrial septal defect closure under cardiopulmonary bypass was discussed in this report.

  19. A cardiac-specific health-related quality of life module for young adults with congenital heart disease: development and validation

    NARCIS (Netherlands)

    Kamphuis, M.; Zwinderman, K.A.H.; Vogels, T.; Vliegen, H.W.; Kamphuis, R.P.; Ottenkamp, J.; Verloove-Vanhorick, S.P.; Bruil, J.

    2004-01-01

    This study represents the development and validation of a cardiac-specific module of the generic health-related quality of life (HRQoL) instrument, the TAAQOL (TNO/AZL Adult Quality Of Life), for young adults with congenital heart disease (CHD). Items were selected based on literature, an explorativ

  20. The renin–angiotensin–aldosterone-system and right heart failure in congenital heart disease

    OpenAIRE

    Stine Andersen; Asger Andersen; Jens Erik Nielsen-Kudsk

    2016-01-01

    Adults with congenital heart disease represent a rapidly growing patient group. Dysfunction of the right ventricle is often present, and right heart failure constitutes the main cause of death. Heart failure therapies used in acquired left heart failure are often initiated in adults with right heart failure due to congenital heart disease, but the right ventricle differs substantially from the left ventricle, and the clinical evidence for this treatment strategy is lacking. In this review,...

  1. Knowledge-based 3D reconstruction of the right ventricle : comparison with cardiac magnetic resonance in adults with congenital heart disease

    NARCIS (Netherlands)

    Trzebiatowska-Krzynska, Aleksandra; Driessen, Mieke; Sieswerda, GT; Wallby, Lars; Swahn, Eva; Meijboom, Folkert

    2015-01-01

    AIM: Assessment of right ventricular (RV) function is a challenge, especially in patients with congenital heart disease (CHD). The aim of the present study is to assess whether knowledge-based RV reconstruction, used in the everyday practice of an echo-lab for adult CHD in a tertiary referral center

  2. Prevalence and determinants of anemia in adults with complex congenital heart disease and ventricular dysfunction (subaortic right ventricle and single ventricle physiology).

    Science.gov (United States)

    Collins, Nicholas; Piran, Sanaz; Harrison, Jeanine; Azevedo, Eduardo; Oechslin, Erwin; Silversides, Candice K

    2008-09-01

    Anemia is well recognized as a marker of poor prognosis in patients with acquired heart disease and heart failure. Adults with complex congenital heart disease and ventricular dysfunction (subaortic right ventricle or single-ventricle physiology) represent a different population, because they are typically much younger and have less co-morbidity compared with patients with acquired forms of heart disease. The purpose of this study was to evaluate the prevalence and determinants of anemia in this population. Baseline hemoglobin levels were recorded at the time of the initial clinic visit, and final hemoglobin levels were those recorded before death or transplantation or at study completion. Anemia was defined as hemoglobin complex congenital heart disease and ventricular dysfunction, in particular those with Fontan physiology.

  3. Implementation of the American College of Cardiology/American Heart Association 2008 Guidelines for the Management of Adults With Congenital Heart Disease.

    Science.gov (United States)

    Goossens, Eva; Fernandes, Susan M; Landzberg, Michael J; Moons, Philip

    2015-08-01

    Although different guidelines on adult congenital heart disease (ACHD) care advocate for lifetime cardiac follow-up, a critical appraisal of the guideline implementation is lacking. We investigated the implementation of the American College of Cardiology/American Heart Association 2008 guidelines for ACHD follow-up by investigating the type of health care professional, care setting, and frequency of outpatient visits in young adults with CHD. Furthermore, correlates for care in line with the recommendations or untraceability were investigated. A cross-sectional observational study was conducted, including 306 patients with CHD who had a documented outpatient visit at pediatric cardiology before age 18 years. In all, 210 patients (68.6%) were in cardiac follow-up; 20 (6.5%) withdrew from follow-up and 76 (24.9%) were untraceable. Overall, 198 patients were followed up in tertiary care, 1/4 (n = 52) of which were seen at a formalized ACHD care program and 3/4 (n = 146) remained at pediatric cardiology. Of those followed in formalized ACHD and pediatric cardiology care, the recommended frequency was implemented in 94.2% and 89%, respectively (p = 0.412). No predictors for the implementation of the guidelines were identified. Risk factors for becoming untraceable were none or lower number of heart surgeries, health insurance issues, and nonwhite ethnicity. In conclusion, a significant number of adults continue to be cared for by pediatric cardiologists, indicating that transfer to adult-oriented care was not standard practice. Frequency of follow-up for most patients was in line with the ACC/AHA 2008 guidelines. A considerable proportion of young adults were untraceable in the system, which makes them vulnerable for discontinuation of care.

  4. Genetic Counseling for Congenital Heart Defects

    Science.gov (United States)

    ... High Blood Pressure Tools & Resources Stroke More Genetic Counseling for Congenital Heart Defects Updated:Oct 26,2015 ... with congenital heart disease considers having children. Genetic counseling can help answer these questions and address your ...

  5. Congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    1995-01-01

    950283 Surgical treatment of congenital coronaryartery fistula.CAO Qingheng(曹庆亨),et al.DeptCardiovasc Surg,Shanghai Chest Hosp,Shanghai,200030.Shanghai Med J 1995;18(1):10-12.From October 1957 through December 1990,twenty-five patients with congenital coronary artery fistula(CCAF),including 3 cases complicated with giantcoronary artery aneurysms,underwent surgical repair.The ages ranged from 4 to 47 years (mean 19.8years).CCAF originated from the right coronaryartery in 17 cases (68.0%) and terminated into RA,RV,pulmonary artery (PA) or LV,in 8 cases (32.

  6. Decentralization of care for adults with congenital heart disease in the United States: a geographic analysis of outpatient surgery.

    Directory of Open Access Journals (Sweden)

    Bryan G Maxwell

    Full Text Available BACKGROUND: Guidelines recommend that adults with congenital heart disease (CHD undergo noncardiac surgery in regionalized centers of expertise, but no studies have assessed whether this occurs in the United States. We hypothesized that adults with CHD are less likely than children to receive care at specialized CHD centers. METHODS: Using a comprehensive state ambulatory surgical registry (California Ambulatory Surgery Database, 2005-2011, we calculated the proportion of adult and pediatric patients with CHD who had surgery at a CHD center, distance to the nearest CHD center, and distance to the facility where surgery was performed. RESULTS: Patients with CHD accounted for a larger proportion of the pediatric population (n = 11,254, 1.0% than the adult population (n = 10,547, 0.07%. Only 2,741 (26.0% adults with CHD had surgery in a CHD center compared to 6,403 (56.9% children (p<0.0001. Adult CHD patients who had surgery at a non-specialty center (11.9 ± 15.4 miles away lived farther from the nearest CHD center (37.9 ± 43.0 miles than adult CHD patients who had surgery at a CHD center (23.2 ± 28.4 miles; p<0.0001. Pediatric CHD patients who had surgery at a non-specialty center (18.0 ± 20.7 miles away lived farther from the nearest CHD center (35.7 ± 45.2 miles than pediatric CHD patients who had surgery at a CHD center (22.4 ± 26.0 miles; p<0.0001. CONCLUSIONS: Unlike children with CHD, most adults with CHD (74% do not have outpatient surgery at a CHD center. For both adults and children with CHD, greater distance from a CHD center is associated with having surgery at a non-specialty center. These results have significant public health implications in that they suggest a failing to achieve adequate regional access to specialized ACHD care. Further studies will be required to evaluate potential strategies to more reliably direct this vulnerable population to centers of expertise.

  7. Risk Factors for Increased Hospital Resource Utilization and In-Hospital Mortality in Adults With Single Ventricle Congenital Heart Disease.

    Science.gov (United States)

    Collins, Ronnie Thomas; Doshi, Pratik; Onukwube, Jennifer; Fram, Ricki Y; Robbins, James M

    2016-08-01

    Most patients with single ventricle congenital heart disease are now expected to survive to adulthood. Co-morbid medical conditions (CMCs) are common. We sought to identify risk factors for increased hospital resource utilization and in-hospital mortality in adults with single ventricle. We analyzed data from the 2001 to 2011 Nationwide Inpatient Sample database in patients aged ≥18 years admitted to nonteaching general hospitals (NTGHs), TGHs, and pediatric hospitals (PHs) with either hypoplastic left heart syndrome, tricuspid atresia or common ventricle. National estimates of hospitalizations were calculated. Elixhauser CMCs were identified. Length of stay (LOS), total hospital costs, and effect of CMCs were determined. Age was greater in NTGH (41.5 ± 1.3 years) than in TGH (32.8 ± 0.5) and PH (25.0 ± 0.6; p <0.0001). Adjusted LOS was shorter in NTGH (5.6 days) than in PH (9.7 days; p <0.0001). Adjusted costs were higher in PH ($56,671) than in TGH ($31,934) and NTGH ($18,255; p <0.0001). CMCs are associated with increased LOS (p <0.0001) and costs (p <0.0001). Risk factors for in-hospital mortality included increasing age (odds ratio [OR] 5.250, CI 2.825 to 9.758 for 45- to 64-year old vs 18- to 30-year old), male gender (OR 2.72, CI 1.804 to 4.103]), and the presence of CMC (OR 4.55, CI 2.193 to 9.436) for 2 vs none). No differences in mortality were found among NTGH, TGH, and PH. Cardiovascular procedures were more common in PH hospitalizations and were associated with higher costs and LOS. CMCs increase costs and mortality. In-hospital mortality is increased with age, male gender, and the presence of hypoplastic left heart syndrome.

  8. Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan

    Directory of Open Access Journals (Sweden)

    Mark J Schuuring

    2010-08-01

    treatment for adults with CHD-PAH. This review focuses on bosentan in CHD-PAH. In particular, we discuss outcome of various clinical trials and compare efficacy and safety of bosentan to other advanced therapies.Keywords: pulmonary arterial hypertension, bosentan, endothelin-1 receptor antagonist, congenital heart disease

  9. Risk Factors for Increased Hospital Resource Utilization and In-Hospital Mortality in Adults With Single Ventricle Congenital Heart Disease.

    Science.gov (United States)

    Collins, Ronnie Thomas; Doshi, Pratik; Onukwube, Jennifer; Fram, Ricki Y; Robbins, James M

    2016-08-01

    Most patients with single ventricle congenital heart disease are now expected to survive to adulthood. Co-morbid medical conditions (CMCs) are common. We sought to identify risk factors for increased hospital resource utilization and in-hospital mortality in adults with single ventricle. We analyzed data from the 2001 to 2011 Nationwide Inpatient Sample database in patients aged ≥18 years admitted to nonteaching general hospitals (NTGHs), TGHs, and pediatric hospitals (PHs) with either hypoplastic left heart syndrome, tricuspid atresia or common ventricle. National estimates of hospitalizations were calculated. Elixhauser CMCs were identified. Length of stay (LOS), total hospital costs, and effect of CMCs were determined. Age was greater in NTGH (41.5 ± 1.3 years) than in TGH (32.8 ± 0.5) and PH (25.0 ± 0.6; p <0.0001). Adjusted LOS was shorter in NTGH (5.6 days) than in PH (9.7 days; p <0.0001). Adjusted costs were higher in PH ($56,671) than in TGH ($31,934) and NTGH ($18,255; p <0.0001). CMCs are associated with increased LOS (p <0.0001) and costs (p <0.0001). Risk factors for in-hospital mortality included increasing age (odds ratio [OR] 5.250, CI 2.825 to 9.758 for 45- to 64-year old vs 18- to 30-year old), male gender (OR 2.72, CI 1.804 to 4.103]), and the presence of CMC (OR 4.55, CI 2.193 to 9.436) for 2 vs none). No differences in mortality were found among NTGH, TGH, and PH. Cardiovascular procedures were more common in PH hospitalizations and were associated with higher costs and LOS. CMCs increase costs and mortality. In-hospital mortality is increased with age, male gender, and the presence of hypoplastic left heart syndrome. PMID:27291967

  10. Effects of iloprost combined with low dose tadalafil in adult congenital heart disease patients with severe pulmonary arterial hypertension: a single-center,open-label controlled study

    Institute of Scientific and Technical Information of China (English)

    张曹进

    2014-01-01

    Objective To evaluate the therapy efficacy of iloprost combined with low dose tadalafil in adult congenital heart disease(CHD)patients with severe pulmonary arterial hypertension(PAH).Methods Adult CHD patients with severe PAH were included and divided into the sequential combination therapy group[iloprost:10μg/inhalation,6 times per day for 6 months,and then add oral tadalafil(5 mg/d)till 12 months,n=32]and upfront combination therapy group[iloprost:10μg/inhalation,6 times per day combined with oral tadalafil(5 mg)

  11. The changing epidemiology of congenital heart disease

    NARCIS (Netherlands)

    T. van der Bom; A.C. Zomer; A.H. Zwinderman; F.J. Meijboom; B.J. Bouma; B.J.M. Mulder

    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 failur

  12. Catheter Ablation to Treat Supraventricular Arrhythmia in Children and Adults With Congenital Heart Disease: What We Know and Where We Are Going

    Science.gov (United States)

    Thomas, Patricia E.; Macicek, Scott L.

    2016-01-01

    Background: Catheter ablation has been used to manage supraventricular arrhythmia in children since 1990. This article reviews the history of catheter ablation used to treat arrhythmia in children and discusses new frontiers in the field. We also address ablation in adult patients with a history of congenital heart disease (CHD) that was diagnosed and initially treated in childhood. Methods: We conducted an evidence-based literature review to gather available data on ablation for supraventricular tachycardia in children and adult patients with CHD. Results: Ablations can be performed safely and effectively in children. Complication rates are higher in children arrhythmia. A paucity of data is available on ablation in adult patients with CHD. Conclusion: Electrophysiology for pediatric and adult patients with CHD is a rapidly growing and progressing field. We benefit from continuous development of ablation techniques for adults with structurally normal hearts and have the unique challenge and responsibility to ensure the safe and effective application of these techniques in the vulnerable population of pediatric and adult patients with CHD. PMID:27660579

  13. A cardiac-specific health-related quality of life module for young adults with congenital heart disease: development and validation.

    Science.gov (United States)

    Kamphuis, M; Zwinderman, K H; Vogels, T; Vliegen, H W; Kamphuis, R P; Ottenkamp, J; Verloove-Vanhorick, S P; Bruil, J

    2004-05-01

    This study represents the development and validation of a cardiac-specific module of the generic health-related quality of life (HRQoL) instrument, the TAAQOL (TNO/AZL Adult Quality Of Life), for young adults with congenital heart disease (CHD). Items were selected based on literature, an explorative previous study in CHD patients, interviews with patients, and the advice of experts. The newly developed Congenital Heart Disease-TNO/AZL Adult Quality of Life (CHD-TAAQOL) was tested in 156 patients with mild or complex CHD and consisted of three hypothesised subject scales: 'Symptoms' (9 items), 'Impact Cardiac Surveillance' (7 items), and 'Worries' (10 items). Cronbach's alpha for the three scales were 0.77, 0.78, and 0.82, respectively. Scale structure was confirmed by Principal Component Analysis, corrected item-scale and interscale correlations. Overall, 55% of reported health status problems were associated with negative emotions, which is an argument for assessing HRQoL as a concept distinct from health status. Convergent validity with validated generic instruments (TAAQOL and Short Form-36, SF-36) showed satisfactory coefficients. Discriminant validity was proven by significantly higher scores for mild CHD patients compared with those with complex CHD. In conclusion, the CHD-TAAQOL module together with the generic TAAQOL can be used to assess group differences for cardiac-specific HRQoL in young adults with CHD. Testing psychometric properties of the CHD-TAAQOL shows satisfactory results. However, to detect changes in HRQoL over time, further research is needed.

  14. Congenital Heart Defects and Physical Activity

    Science.gov (United States)

    ... Tools & Resources Stroke More Congenital Heart Defects and Physical Activity Updated:Apr 19,2016 Exercise Is for Everyone ... almost all patients do some form of regular physical activity. There are a few exceptions, so it's good ...

  15. Congenital bronchoesophageal fistula in adults

    Institute of Scientific and Technical Information of China (English)

    Bao-Shi Zhang; Nai-Kang Zhou; Chang-Hai Yu

    2011-01-01

    AIM: To study the clinical characteristics, diagnosis and surgical treatment of congenital bronchoesophageal fistulae in adults. METHODS: Eleven adult cases of congenital bronchoesophageal fistula diagnosed and treated in our hospital between May 1990 and August 2010 were reviewed. Its clinical presentations, diagnostic methods, anatomic type, treatment, and follow-up were recorded. RESULTS: Of the chief clinical presentations, nonspecific cough and sputum were found in 10 (90.9%), recurrent bouts of cough after drinking liquid food in 6 (54.6%), hemoptysis in 6 (54.6%), low fever in 4 (36.4%), and chest pain in 3 (27.3%) of the 11 cases, respectively. The duration of symptoms before diagnosis ranged 5-36.5 years. The diagnosis of congenital bronchoesophageal fistulae was established in 9 patients by barium esophagography, in 1 patient by esophagoscopy and in 1 patient by bronchoscopy, respectively. The congenital bronchoesophageal fistulae communicated with a segmental bronchus, a main bronchus, and an intermediate bronchus in 8, 2 and 1 patients, respectively. The treatment of congenital bronchoesophageal fistulae involved excision of the fistula in 10 patients or division and suturing in 1 patient. The associated lung lesion was removed in all patients. No long-term sequelae were found during the postoperative follow-up except in 1 patient with bronchial fistula who accepted reoperation before recovery. CONCLUSION: Congenital bronchoesophageal fistula is rare in adults. Its most useful diagnostic method is esophagography. It must be treated surgically as soon as the diagnosis is established.

  16. Energy expenditure in congenital heart disease.

    OpenAIRE

    Barton, J S; Hindmarsh, P C; Scrimgeour, C M; Rennie, M J; Preece, M A

    1994-01-01

    Growth failure is a well recognised consequence of severe congenital heart disease. Total daily energy expenditure (TDEE) was investigated in eight infants with severe congenital heart disease to determine whether an increase in this parameter is an important factor in their failure to thrive, and to estimate the energy intake that would be required to allow normal growth. The infants were studied over a seven day period before surgery using the doubly labelled water method. Growth failure wa...

  17. 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.

  18. 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.)

  19. Cardiac Embryology and Molecular Mechanisms of Congenital Heart Disease: A Primer for Anesthesiologists.

    Science.gov (United States)

    Kloesel, Benjamin; DiNardo, James A; Body, Simon C

    2016-09-01

    Congenital heart disease is diagnosed in 0.4% to 5% of live births and presents unique challenges to the pediatric anesthesiologist. Furthermore, advances in surgical management have led to improved survival of those patients, and many adult anesthesiologists now frequently take care of adolescents and adults who have previously undergone surgery to correct or palliate congenital heart lesions. Knowledge of abnormal heart development on the molecular and genetic level extends and improves the anesthesiologist's understanding of congenital heart disease. In this article, we aim to review current knowledge pertaining to genetic alterations and their cellular effects that are involved in the formation of congenital heart defects. Given that congenital heart disease can currently only occasionally be traced to a single genetic mutation, we highlight some of the difficulties that researchers face when trying to identify specific steps in the pathogenetic development of heart lesions. PMID:27541719

  20. 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

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

    OpenAIRE

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

    2015-01-01

    Background: 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. Methods: Exercise tests performed using a Bruce protocol and measurement of maximum oxygen consumpti...

  2. 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

    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 re

  3. Marital stability and congenital heart disease.

    Science.gov (United States)

    Silbert, A R; Newburger, J W; Fyler, D C

    1982-06-01

    The incidence of divorce or legal separation was studied in 438 families of children born with heart disease who entered the New England Regional Infant Cardiac Program between 1968 and 1973. The parents were interviewed when the children were 5 1/2 years old. The rate of divorce in 438 families of children with critical congenital heart disease was not significantly different from the rate in two comparison groups: (1) 25 families of children whose cardiac defect was spontaneously cured, and (2) 26 families of children catheterized in infancy for suspected cardiac defect but who were found to be free of heart disease. Rates of divorce or legal separation for the three groups were: critical congenital heart disease, 12.1%, spontaneously cured, 4.2% free of heart disease, 11.5% these rates were not significantly different. The average national divorce rate was 20.3% for the same period.

  4. Recurrent congenital fibrosarcoma with heart metastases.

    Science.gov (United States)

    Lohi, Olli; Vornanen, Martine; Kähkönen, Marketta; Vettenranta, Kim; Parto, Katriina; Arola, Mikko

    2012-07-01

    Congenital fibrosarcomas are malignant tumors that arise in soft tissues. In infants this unique tumor does not commonly metastasize, even though there may be local recurrences. We report here a boy who had congenital fibrosarcoma in his right foot, which was completely excised at the age of 3 days. Four months later, a solitary encapsulated metastasis emerged in thoracic chest wall, which was operated. During adjuvant chemotherapy he developed histologically confirmed fibrosarcoma metastases in the heart. After extended treatment with cyclophosphamide/topotecan and gemcitabine/docetaxel, the heart tumors disappeared and he has been in complete remission for 3 years. PMID:22217490

  5. Paternal age and offspring congenital heart defects

    DEFF Research Database (Denmark)

    Su, Xiu Juan; Yuan, Wei; Huang, Guo Ying;

    2015-01-01

    Paternal age has been associated with offspring congenital heart defects (CHDs), which might be caused by increased mutations in the germ cell line because of cumulated cell replications. Empirical evidences, however, remain inconclusive. Furthermore, it is unknown whether all subtypes of CHDs are.......17-2.43). We observed similar results when subanalyses were restricted to children born to mothers of 27-30 years old. After taking into consideration of maternal age, our data suggested that advanced paternal age was associated with an increased prevalence of one subtype of offspring congenital heart defects...

  6. Diagnosis and treatment of congenital heart defects

    International Nuclear Information System (INIS)

    The Clinic for Congenital Heart Defects in Bad Oeynhausen, Germany, specializes in the interdisciplinary care of children and adolescents with congenital heart defects. The available expertise covers all aspects of diagnosis and treatment, as well as clinical research at the highest level. The clinic has a complete array of imaging systems, including a Philips Integris BV5000 Biplane cath lab system, Philips Gyroscan Intera CV MR system, ultrasound and PET systems. The range of available equipment makes it possible to select the most appropriate imaging modality for each situation, with particular reference to the need to minimize the overall radiation exposure. (orig.)

  7. Phase-contrast MRI and applications in congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Goldberg, A., E-mail: adgoldberg@geisinger.edu [Department of Radiology, Geisinger Health System, Danville, PA (United States); Jha, S. [Department of Radiology, Hospital of the University of Pennsylvania, Philadelphia, PA (United States)

    2012-05-15

    A review of phase-contrast magnetic resonance imaging techniques, with specific application to congenital heart disease, is presented. Theory, pitfalls, advantages, and specific examples of multiple, well-described congenital heart disease presentations are discussed.

  8. Congenital Heart Disease: Vascular Risk Factors and Medication

    NARCIS (Netherlands)

    H.P.M. Smedts (Dineke)

    2011-01-01

    textabstractCongenital heart disease (CHD) is among the most common congenital abnormalities and involves structural anomalies of the heart and/or related major blood vessels. Congenital heart disease arises in the fi rst trimester of pregnancy, occurring often and in many forms. The reported CHD bi

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

    OpenAIRE

    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 in those with septal defects. A spectrum of cardiac defects is associated with PAH-CHD, although most cases develop as a consequence of large systemic-to-pulmonary shunts. Eisenmenger's syndrome, ch...

  10. Types of Congenital Heart Defects

    Science.gov (United States)

    ... Thus, the heart has to work harder to pump blood through the valve. Atresia (ah-TRE-ze-AH). ... right ventricle can get very overworked trying to pump blood to the pulmonary artery. These infants may have ...

  11. 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.)

  12. Echocardiographic evaluation of right ventricular function in congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    Yiu-fai Cheung

    2014-01-01

    Objective This review aims to provide an overview of conventional and novel indices used in clinical and research arenas for evaluation of right ventricular (RV) function in congenital heart diseases with a dual-chambered circulation.Data sources Articles cited in this review were selected using PubMed search of publications in English with no date limits.The search terms included "echocardiography","right ventricle","RV function","cardiac function",and "congenital heart disease".Key references were also searched for additional publications.Study selection Articles related to description of echocardiographic techniques in the evaluation of subpulmonary or systemic RV function and their applications in congenital cardiac malformations were retrieved and reviewed.Results Three approaches have been used to evaluate subpulmonary and systemic RV function:(1) assessment of changes in RV size in the cardiac cycle,(2) determination of Doppler-derived velocities and systolic and diastolic time intervals,and (3) quantification of myocardial velocities and deformation.Conclusions Conventional and novel echocardiographic techniques enable the evaluation of subpulmonary and systemic RV function.Novel echocardiographic techniques have further allowed quantification of RV volumes and direct interrogation of myocardial deformation.These new techniques show promise in a more comprehensive evaluation beyond "eye-bailing" of RV function in the growing population of adolescent and adult congenital heart patients.

  13. 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.  

  14. 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 cardiopulmonary bypass...

  15. Nutrition in neonatal congenital heart disease

    OpenAIRE

    McMahon, Colin

    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...

  16. Evaluation of neonates with congenital heart disease

    OpenAIRE

    Tokel, Kürşad; Saygılı, Arda; Mercan, Şükrü; Varan, Birgül; Gürakan, Berkan; Aşlamacı, Sait

    2001-01-01

    From April 1995 to June 1999 190 neonates with congenital heart disease underwent medical interventional and surgical treatment in our institution The symptoms physical findings and cardiac diagnoses of patients were retrospectively analysed in four age groups according to age at admission: before 3 days 3 14 days 14 28 days and 28 42 days The results of interventional catheterization medical and surgical treatment and complications were also investigated The mean age at admission was 19 2 ± ...

  17. Recurrent congenital heart block in neonatal lupus.

    Science.gov (United States)

    Escobar, Maria C; Gómez-Puerta, José A; Albert, Dimpna; Ferrer, Queralt; Girona, Josep

    2007-07-01

    Congenital heart block (CHB) is the main complication of neonatal lupus (NL) and is strongly associated with the presence of anti-SSA/Ro and anti-SSB/La antibodies. The recurrence of CHB in subsequent pregnancies in mothers with these antibodies is uncommon, occurring in approximately 15% of cases. We describe here a case of recurrent CHB in a previously asymptomatic mother with Sjögren syndrome and discuss the current strategies for the prevention and treatment of CHB in NL.

  18. 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...

  19. Consecutive percutaneous valve-in-valve replacement late after Ross procedure: A novel approach in an adult with congenital heart disease.

    Science.gov (United States)

    Wiktor, Dominik M; Kay, Joseph D; Kim, Michael S

    2015-11-15

    The emergence of transcatheter valve technology over the last decade has made significant impact on the treatment of patients with valvular heart disease. There has been increasing experience with both native and valve-in-valve indications with promising results. We present the case of a young woman with congenital heart disease who underwent the Ross procedure for bicuspid aortic valve endocarditis with subsequent reoperation and surgical aortic valve replacement for neo-aortic root dilation who experienced worsening symptoms related to both pulmonary and aortic valve dysfunction. She was successfully treated with percutaneous pulmonary and aortic valve replacement with excellent early term technical results and marked improvement in symptoms.

  20. 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.

  1. Congenital heart disease and chromossomopathies detected by the karyotype

    OpenAIRE

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

    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 les...

  2. [Real time 3D echocardiography in congenital heart disease].

    Science.gov (United States)

    Acar, P; Dulac, Y; Taktak, A; Villacèque, M

    2004-05-01

    The introduction of the 3D mode in echocardiography has led to its use in everyday clinical practice. One hundred and fifty real time 3D echocardiographic examinations were performed in 20 foetus, 110 children and 20 adults with various congenital heart lesions (shunts, valvular lesions, aortic diseases). The 4x matricial probe enables the instantaneous acquisition of transthoracic volumes. Four modes of 3D imaging were used: real time, total volume, colour Doppler and biplane. Quantitative measurements were performed at an outlying station. The feasibility of the method in the foetus, the child and the adult was respectively 90%, 99% and 85%. Real time 3D echocardiography did not affect the diagnoses made by standard echocardiography. The 3D imaging gave a more accurate description of atrial septal defects and congenital valvular lesions. Biplane imaging was decisive in the quantitative approach to aortic dilatation of Marfan's syndrome and in segmental analysis of the foetal heart. 3D colour Doppler imaging has been disappointing but the possibilities of volumic quantification of blood flow are very promising. The present limitations of the method are the inadequate resolution in the small child and the absence of quantitative measurement on the echograph. The facility of utilisation of the matricial probe should lead to routine usage of 3D echocardiography as with 2D and the Doppler modes. Its value should be decisive in many congenital cardiac lesions requiring surgery or interventional catheterisation. PMID:15214550

  3. 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 (Sparacino, 19

  4. 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 ...

  5. 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

  6. Risk factors predisposing to congenital heart defects

    Science.gov (United States)

    Ul Haq, Faheem; Jalil, Fatima; Hashmi, Saman; Jumani, Maliha Iqbal; Imdad, Aamer; Jabeen, Mehnaz; Hashmi, Javad Tauseef; Irfan, Furqan Bin; Imran, Muhammad; Atiq, Mehnaz

    2011-01-01

    Introduction: Congenital heart disease (CHD) is associated with multiple risk factors, consanguinity may be one such significant factor. The role of consanguinity in the etiology of CHD is supported by inbreeding studies, which demonstrate an autosomal recessive pattern of inheritance of some congenital heart defects. This study was done to find out the risk factors for CHD. Methods: A case-control study was done on pediatric patients at a tertiary care hospital, Aga Khan University Hospital, located in Karachi, Pakistan. A total of 500 patients, 250 cases and 250 controls were included in the study. Results: Amongst the 250 cases (i.e. those diagnosed with CHD), 122 patients (48.8%) were born of consanguineous marriages while in the controls (i.e. non-CHD) only 72 patients (28.9%) showed a consanguinity amongst parents. On multivariate analysis, consanguinity emerged as an independent risk factor for CHD; adjusted odds ratio 2.59 (95% C. I. 1.73 - 3.87). Other risk factors included low birth weight, maternal co-morbidities, family history of CHD and first born child. On the other hand, medications used by the mother during the index pregnancy, maternal age and gender of the child did not significantly increase the risk of developing CHD. Conclusions: Analyses of our results show that parental consanguinity, family history of CHD, maternal co-morbidities, first born child and low birth weight are independent risk factors for CHD. PMID:21976868

  7. Risk factors predisposing to congenital heart defects

    International Nuclear Information System (INIS)

    Congenital heart disease (CHD) is associated with multiple risk factors, consanguinity may be one such significant factor. The role of consanguinity in the etiology of CHD is supported by inbreeding studies, which demonstrate an autosomal recessive pattern of inheritance of some congenital heart defects. This study was done to find out the risk factors for CHD. A case-control study was done on pediatric patients at a tertiary care hospital, Aga Khan University Hospital, located in Karachi, Pakistan. A total of 500 patients, 250 cases and 250 controls were included in the study. Amongst the 250 cases (i.e. those diagnosed with CHD), 122 patients (48.8%) were born of consanguineous marriages while in the controls (i.e. non-CHD) only 72 patients (28.9%) showed a consanguinity amongst parents. On multivariate analysis, consanguinity emerged as an independent risk factor for CHD; adjusted odds ratio 2.59 (95% C. I. 1.73 - 3.87). Other risk factors included low birth weight, maternal co-morbidities, family history of CHD and first born child. On the other hand, medications used by the mother during the index pregnancy, maternal age and gender of the child did not significantly increase the risk of developing CHD. Analyses of our results show that parental consanguinity, family history of CHD, maternal co-morbidities, first born child and low birth weight are independent risk factors for CHD

  8. Aortopathy associated with congenital heart disease: a current literature review

    OpenAIRE

    Katrien Francois

    2015-01-01

    In patients born with congenital heart disease, dilatation of the aorta is a frequent feature at presentation and during follow-up after surgical intervention. This review provides an overview of the pathologies associated with aortopathy, and discusses the current knowledge on pathophysiology, evolution, and treatment guidelines of the aortic disease associated with congenital heart defects.

  9. Incidence of congenital heart disease in Beijing, China

    Institute of Scientific and Technical Information of China (English)

    YANG Xue-yong; LI Xiao-feng; L(U) Xiao-dong; LIU Ying-long

    2009-01-01

    Background The incidence of congenital heart disease has been studied in developed countries for many years, but rarely in the mainland of China. Fetal echocardiographic screening for congenital heart disease was first performed in Beijing in the early 2000s, but the impact was not clear. The current study was undertaken to determine the incidence of congenital heart disease in Beijing, China and to estimate the impact of fetal echocardiography on the incidence of liveborn congenital heart disease.Methods The study involved all infants with congenital heart disease among the 84 062 total births in Beijing during the period of January 1 and December 31, 2007. An echocardiographic examination was performed on every baby suspected to have congenital heart disease, prenatally or/and postnatally.Results A total of 686 infants were shown to have congenital heart disease among 84 062 total births. The overall incidence was 8.2/1000 total births. Mothers of 128 of 151 babies diagnosed prenatally were chosen to terminate the pregnancy. Two of the 151 infants died in utero. A specific lesion was identified for each infant and the frequencies of lesions were determined for each class of infants (total births, stillbirths and live births). The incidence of congenital heart disease in stillbirths and live births was 168.8/1000 and 6.7/1000, respectively. The difference between the incidence of total birth and the incidence of live birth was statistically significant (P<0.001).Conclusions The incidence of liveborn congenital heart disease in Beijing is within the range reported in developed countries. Fetal echocardiography reduce significantly the incidence of livebom congenital heart disease.

  10. 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.

  11. Pregnant women with congenital heart disease: cardiac, anesthetic and obstetrical implications.

    Science.gov (United States)

    Fernandes, Susan M; Arendt, Katherine W; Landzberg, Michael J; Economy, Katherine E; Khairy, Paul

    2010-03-01

    Remarkable advances in surgical and clinical management have resulted in survival to adulthood in the large majority of patients with congenital heart malformations, even with the most complex disease. Over 1 million adults with congenital heart disease currently live in the USA, approximately half of whom are women of childbearing age. Collectively, congenital malformations are the most common form of heart disease in pregnant women. Indeed, in North America, congenital defects are now the leading cause of maternal morbidity and mortality from heart disease. This article begins with a summary of cardiovascular changes during pregnancy and highlights key features in pre-pregnancy counseling, maternal cardiac and obstetric risk, and neonatal complications. Management issues regarding pregnancy and delivery are elaborated, including anesthesia considerations. While it is beyond the scope of this article to discuss particulars related to all forms of congenital heart disease, selected subtypes are detailed at greater length. In the absence of clinical trial evidence to inform the care of pregnant women with congenital heart disease, this article is inspired by the premise that knowledgeable multidisciplinary assessment and management provides the best opportunity to substantially improve outcomes for mother and baby. PMID:20222821

  12. Three dimensional echocardiography in congenital heart defects

    Directory of Open Access Journals (Sweden)

    Shirali Girish

    2008-01-01

    Full Text Available Three dimensional echocardiography (3DE is a new, rapidly evolving modality for cardiac imaging. Important technological advances have heralded an era where practical 3DE scanning is becoming a mainstream modality. We review the modes of 3DE that can be used. The literature has been reviewed for articles that examine the applicability of 3DE to congenital heart defects to visualize anatomy in a spectrum of defects ranging from atrioventricular septal defects to mitral valve abnormalities and Ebstein′s anomaly. The use of 3DE color flow to obtain echocardiographic angiograms is illustrated. The state of the science in quantitating right and left ventricular volumetrics is reviewed. Examples of novel applications including 3DE transesophageal echocardiography and image-guided interventions are provided. We also list the limitations of the technique, and discuss potential future developments in the field.

  13. 3 tesla magnetic resonance imaging in children and adults with congenital heart disease; 3-Tesla-Magnetresonanztomographie zur Untersuchung von Kindern und Erwachsenen mit angeborenen Herzfehlern

    Energy Technology Data Exchange (ETDEWEB)

    Voges, I.; Hart, C.; Kramer, H.H.; Rickers, C. [Universitaetsklinikum Schleswig-Holstein, Klinik fuer angeborene Herzfehler und Kinderkardiologie, Kiel (Germany); Jerosch-Herold, M. [Harvard Medical School, Department of Radiology, Brigham and Women' s Hospital, Boston (United States); Helle, M. [Universitaetsklinikum Schleswig-Holstein, Institut fuer Neuroradiologie, Kiel (Germany)

    2010-09-15

    Cardiovascular magnetic resonance imaging (CMR) has become a routinely used imaging modality for congenital heart disease. A CMR examination allows the assessment of thoracic anatomy, global and regional cardiac function, blood flow in the great vessels and myocardial viability and perfusion. In the clinical routine cardiovascular MRI is mostly performed at field strengths of 1.5 Tesla (T). Recently, magnetic resonance systems operating at a field strengths of 3 T became clinically available and can also be used for cardiovascular MRI. The main advantage of CMR at 3 T is the gain in the signal-to-noise ratio resulting in improved image quality and/or allowing higher acquisition speed. Several further differences compared to MRI systems with lower field strengths have to be considered for practical applications. This article describes the impact of CMR at 3 T in patients with congenital heart disease by meanings of methodical considerations and case studies. (orig.) [German] Die kardiovaskulaere Magnetresonanztomographie (MRT) hat sich zu einer etablierten bildgebenden Methode zur Untersuchung von Patienten mit angeborenen Herzfehlern entwickelt. Sie erlaubt in einer einzigen Untersuchung die exakte Beurteilung von Anatomie, globaler und regionaler Funktion, Blutfluessen sowie der myokardialen Perfusion und Vitalitaet. In der klinischen Routine erfolgen die Untersuchungen zumeist bei einer Feldstaerke von 1,5 Tesla (T), mittlerweile gibt es jedoch Geraete und Bildgebungstechniken, die die kardiovaskulaere MRT auch bei 3 T ermoeglichen. Der wesentliche Vorteil der MRT bei 3 T ist das hoehere Signal-zu-Rausch-Verhaeltnis, das sowohl zu einer Verbesserung der Bildqualitaet als auch zu einer Verkuerzung der Untersuchungszeit genutzt werden kann. Darueber hinaus bestehen verschiedene andere Unterschiede gegenueber Systemen mit niedriger Feldstaerke, die im praktischen Einsatz beachtet werden muessen. Dieser Artikel beschreibt die Erfahrungen der 3-T-MRT fuer die

  14. 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.

  15. 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.

  16. 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.

  17. 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.

  18. Cancer risk among patients with congenital heart defects

    DEFF Research Database (Denmark)

    Olsen, Morten; Garne, Ester; Sværke, Claus;

    2013-01-01

    OBJECTIVE: We aimed to assess cancer risk in congenital heart defect patients, with and without Down's syndrome, compared with the general population. METHODS: We identified all patients born and diagnosed with congenital heart defects from 1977 to 2008 using the Danish National Registry of...... Patients, covering all Danish hospitals. We compared cancer incidence in the congenital heart defect cohort with that expected in the general population (∼5.5 million) using the Danish Cancer Registry, and computed age- and gender-standardised incidence ratios. RESULTS: We identified 15,905 congenital...... heart defect patients, contributing a total of 151,172 person-years at risk; the maximum length of follow-up was 31 years (median 8 years). In all, 53 patients were diagnosed with cancer, including 30 female and 23 male patients (standardised incidence ratio = 1.63; 95% confidence interval: 1...

  19. Hyperuricemia in Congenital Heart Diseases: True or Numbers

    OpenAIRE

    A Zavarehi; ATabib; M Meraji; P Nakhostin Davari; MY Arabi Mghadam; AA Shahmohamadi; SR Miri

    2006-01-01

    Background: Uric acid has been shown to be increased in congenital heart diseases (CHD) and Eisenmenger syndrome. The present study was undertaken to investigate the incidence of hyperuricemia, renal dysfunction and the exacerebrating factors in congenital heart diseases (CHD). Methods: We studied prospective cross sectionaly 500 patients, aged one month to 18 years. Blood for serum uric acid, hemoglobin (Hb), hamatocrit (Hct), arterial blood gas (ABG), blood urea nitrogen (BUN), creatinine (...

  20. Cardiac biomarkers in children with congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    Masaya Sugimoto; Seiko Kuwata; Clara Kurishima; Jeong Hye Kim; Yoich Iwamoto; Hideaki Senzaki

    2015-01-01

    Background: Most congenital heart diseases (CHDs) have specific hemodynamics, including volume and pressure overload, as well as cyanosis and pulmonary hypertension, associated with anatomical abnormalities. Such hemodynamic abnormalities can cause activation of neurohormones, inflammatory cytokines, fibroblasts, and vascular endothelial cells, which in turn contribute to the development of pathologic conditions such as cardiac hypertrophy,fi brosis, and cardiac cell damages and death. Measuring biomarker levels facilitates the prediction of these pathological changes, and provides information about the stress placed on the myocardial cells, the severity of the damage, the responses of neurohumoral factors, and the remodeling of the ventricle. Compared to the ample information on cardiac biomarkers in adult heart diseases, data from children with CHD are still limited. Data sources: We reviewed cardiac biomarkers-specifi cally focusing on troponin as a biomarker of myocardial damage, amino-terminal procollagen type III peptide (PIIIP) as a biomarker of myocardialfi brosis and stromal remodeling, and B-type natriuretic peptide (BNP)/N-terminal proBNP as biomarkers of cardiac load and heart failure, by introducing relevant publications, including our own, on pediatric CHD patients as well as adults. Results: Levels of highly sensitive troponin I are elevated in patients with atrial septal defects (ASDs) and ventricular septal defects (VSDs). PIIIP levels are also elevated in patients with ASD, VSD, pulmonary stenosis, and Tetralogy of Fallot. Measurement of BNP and N-terminal proBNP levels shows good correlation with heart failure score in children. Conclusions: In the treatment of children with CHD requiring delicate care, it is vital to know the specifi c degree of myocardial damage and severity of heart failure. Cardiac biomarkers are useful tools for ascertaining the condition of CHDs with ease and are likely to be useful in determining the appropriate care of

  1. Long-term tricuspid valve prosthesis-related complications in patients with congenital heart disease

    NARCIS (Netherlands)

    van Slooten, Ymkje J.; Freling, Hendrik G.; van Melle, Joost P.; Mulder, Barbara J. M.; Jongbloed, Monique R. M.; Ebels, Tjark; Voors, Adriaan A.; Pieper, Petronella G.

    2014-01-01

    OBJECTIVES: In patients with acquired valvar disease, morbidity and mortality rates after tricuspid valve replacement (TVR) are high. However, in adult patients with congenital heart disease, though data concerning outcome after TVR are scarce, even poorer results are suggested in patients with Ebst

  2. Familial congenital heart disease in Bandung, Indonesia

    Directory of Open Access Journals (Sweden)

    Sri Endah Rahayuningsih

    2013-01-01

    Full Text Available Background Congenital heart disease (CHD may occur in several members of a family. Studies have shown that familial genetic factor play a role in CHD.Objective To identify familial recurrences of CHD in families with at least one member treated for CHD in Dr. Hasan Sadikin Hospital, Bandung Indonesia.Methods In this descriptive study, subjects were CHD patients hospitalized or treated from January 2005 to December 2011. We constructed family pedigrees for five families.Results During the study period, there were 1,779 patients with CHD. We found 5 families with 12 familial CHD cases, consisting of 8 boys and 4 girls. Defects observed in these 12 patients were tetralogy of Fallot, transposition of the great arteries, persistent ductus arteriosus, ventricular septal defect, tricuspid atresia, pulmonary stenosis, and dilated cardiomyopathy. Persistent ductus arteriosus was the most frequently observed defect (4 out of 12 subjects. None of the families had a history of consanguinity. The recurrence risk of CHD among siblings was calculated to be 0.67%, and the recurrence risk of CHD among cousins was 0.16%.Conclusion Familial CHD may indicate the need for genetic counseling and further pedigree analysis.

  3. Familial congenital heart disease in Bandung, Indonesia

    Directory of Open Access Journals (Sweden)

    Sri Endah Rahayuningsih

    2013-03-01

    Full Text Available Background Congenital heart disease (CHD may occur in several members of a family. Studies have shown that familial genetic factor play a role in CHD. Objective To identify familial recurrences of CHD in families with at least one member treated for CHD in Dr. Hasan Sadikin Hospital, Bandung Indonesia. Methods In this descriptive study, subjects were CHD patients hospitalized or treated from January 2005 to December 2011. We constructed family pedigrees for five families. Results During the study period, there were 1,779 patients with CHD. We found 5 families with 12 familial CHD cases, consisting of 8 boys and 4 girls. Defects observed in these 12 patients were tetralogy of Fallot, transposition of the great arteries, persistent ductus arteriosus, ventricular septal defect, tricuspid atresia, pulmonary stenosis, and dilated cardiomyopathy. Persistent ductus arteriosus was the most frequently observed defect (4 out of 12 subjects. None of the families had a history of consanguinity. The recurrence risk of CHD among siblings was calculated to be 0.67%, and the recurrence risk of CHD among cousins was 0.16%. Conclusion Familial CHD may indicate the need for genetic counseling and further pedigree analysis. [Paediatr Indones. 2013;53:173-6.

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

    OpenAIRE

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

    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...

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

    Directory of Open Access Journals (Sweden)

    B.J.M. Mulder

    2010-12-01

    Full Text Available 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 in those with septal defects. A spectrum of cardiac defects is associated with PAH-CHD, although most cases develop as a consequence of large systemic-to-pulmonary shunts. Eisenmenger's syndrome, characterised by reversed pulmonary-to-systemic (right-to-left shunt, represents the most advanced form of PAH-CHD and affects as many as 50% of those with PAH and left-to-right shunts. It is associated with the poorest outcome among patients with PAH-CHD. 40 yrs ago, ∼50% of children with CHD requiring intervention died within the first year, and <15% survived to adulthood. Subsequent advances in paediatric cardiology have seen most patients with CHD survive to adulthood, with resulting shifts in the demographics of CHD and PAH-CHD. The number of adults presenting with CHD is increasing and, although mortality is decreasing, morbidity is increasing as older patients are at increased risk of arrhythmia, heart failure, valve regurgitation and PAH. Data show that probability of PAH increases with age in patients with cardiac defects.

  6. 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.

  7. Early complications of stenting in patients with congenital heart disease : a multicentre study

    NARCIS (Netherlands)

    van Gameren, Menno; Witsenburg, Maarten; Takkenberg, Johanna J. M.; Boshoff, Derize; Mertens, Luc; van Oort, Anton M.; de Wolf, DanieL; Freund, Matthias; Sreeram, Narayanswani; Bokenkamp, Regina; Talsma, Melle D.; Gewillig, Marc

    2006-01-01

    Aims Stenting has become an established interventional cardiology procedure for congenital heart disease. Although most stent procedures are completed successfully, complications may occur. This multicentre study evaluated early complications after stenting in patients with congenital heart disease,

  8. 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

  9. Congenital Heart Disease and Impacts on Child Development

    Directory of Open Access Journals (Sweden)

    Mariana Alievi Mari

    2016-02-01

    Full Text Available Abstract Objective: To evaluate the child development and evaluate a possible association with the commitment by biopsychosocial factors of children with and without congenital heart disease. Methods: Observational study of case-control with three groups: Group 1 - children with congenital heart disease without surgical correction; Group 2 - children with congenital heart disease who underwent surgery; and Group 3 - healthy children. Children were assessed by socio-demographic and clinical questionnaire and the Denver II Screening Test. Results: One hundred and twenty eight children were evaluated, 29 in Group 1, 43 in Group 2 and 56 in Group 3. Of the total, 51.56% are girls and ages ranged from two months to six years (median 24.5 months. Regarding the Denver II, the children with heart disease had more "suspicious" and "suspect/abnormal" ratings and in the group of healthy children 53.6% were considered with "normal" development (P≤0.0001. The biopsychosocial variables that were related to a possible developmental delay were gender (P=0.042, child's age (P=0.001 and income per capita (P=0.019. Conclusion: The results suggest that children with congenital heart disease are likely to have a developmental delay with significant difference between children who have undergone surgery and those awaiting surgery under clinical follow-up.

  10. A Child with Lung Hypoplasia, Congenital Heart Disease, Hemifacial Microsomia, and Inguinal Hernia: Ipsilateral Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Chengming Fan

    2015-01-01

    Full Text Available A 3-year-old Chinese boy was diagnosed with ipsilateral congenital malformations: right lung hypoplasia, dextroversion of heart, atrial septal defect, hepatic vein drainage directly into the right atrium, facial asymmetry, right microtia and congenital deafness, and indirect inguinal hernia. He underwent indirect inguinal hernia repair at the age of 2. Although without any facial plastic surgery performed, he underwent a repair of atrial septal defect and recovered uneventfully. At 6-month follow-up, the patient was free from any symptom of dyspnea; his heart function returned to the first grade.

  11. 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.

  12. Diagnostic Value of Fetal Echocardiography for Congenital Heart Disease

    OpenAIRE

    Zhang, Ya-Fei; Zeng, Xian-Ling; Zhao, En-Fa; Lu, Hong-Wei

    2015-01-01

    Abstract Prenatal diagnosis of fetal congenital heart disease (CHD) has been shown to have a significant effect on prenatal and postnatal management and outcomes. However, the factors influencing the diagnostic accuracy and which pregnant trimester is the most adaptive for fetal heart disease remain uncertain despite of extensive researches. The aim of the present study was to evaluate the accuracy of echocardiography for detecting CHD and potential influence factors. We searched Chinese Biom...

  13. Accuracy of Pulse Oximetry in Children with Congenital Heart Disease.

    OpenAIRE

    Amoozgar, Hamid; Abbasi, Reza; Ajami, Gholam Hossein; BORZEE, Mohammad; Cheriki, Sirous; SOLTANI, Manuchehr

    2010-01-01

    Background: Detection of arterial oxygen saturation in pediatric patients with congenital heart disease (CHD) can be helpful for their management. Methods: We compared oxygen saturation and heart rate detected by pulse oximeter with the data of cardiac catheterization in order to determine the reliability and pitfalls of pulse oximetry (PO) in pediatric patients with CHD. 110 patients with different cyanotic and acyanotic CHD who were referred for right and left sided cardiac catheterizat...

  14. 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

    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 advan

  15. 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…

  16. 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...

  17. [Anti-Ro/SSA antibodies in congenital heart block].

    Science.gov (United States)

    Barrios Prieto, Ernesto; Martínez Ceccopieri, David Alejandro; Panduro Barón, J Guadalupe; Fajardo Dueñas, Sergio

    2012-06-01

    Describe a case of a female patient having anti-Ro/SSA antibodies without any other risk factor or collagen disease. In her first pregnancy a congenital heart block and hydrops in the fetus were diagnosed, and these caused stillbirth. In a second pregnancy an in utero treatment resulted in the succesful delivery of a normal child.

  18. Haploinsufficiency of TAB2 Causes Congenital Heart Defects in Humans

    NARCIS (Netherlands)

    B. Thienpont; L. Zhang; A.V. Postma; J. Breckpot; L.C. Tranchevent; P. van Loo; K. Møllgård; N. Tommerup; I. Bache; Z. Tümer; K. van Engelen; B. Menten; G. Mortier; D. Waggoner; M. Gewillig; Y. Moreau; K. Devriendt; L.A. Larsen

    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

  19. 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.

  20. Cardiac Hemodynamics in the Pathogenesis of Congenital Heart Disease and Aortic Valve Calcification

    Science.gov (United States)

    Nigam, Vishal

    2011-11-01

    An improved understanding of the roles of hemodynamic forces play in cardiac development and the pathogenesis of cardiac disease will have significant scientific and clinical impact. I will focus on the role of fluid dynamics in congenital heart disease and aortic valve calcification. Congenital heart defects are the most common form of birth defect. Aortic valve calcification/stenosis is the third leading cause of adult heart disease and the most common form of acquired valvular disease in developed countries. Given the high incidence of these diseases and their associated morbidity and mortality, the potential translational impact of an improved understanding of cardiac hemodynamic forces is very large. Division of Pediatric Cardiology, Rady Children's Hospital, San Diego

  1. A cluster randomized trial of a transition intervention for adolescents with congenital heart disease: rationale and design of the CHAPTER 2 study

    OpenAIRE

    Mackie, Andrew S; Rempel, Gwen R; Kovacs, Adrienne H.; Kaufman, Miriam; Rankin, Kathryn N.; Jelen, Ahlexxi; Manlhiot, Cedric; Anthony, Samantha J.; Magill-Evans, Joyce; Nicholas, David; Sananes, Renee; Oechslin, Erwin; Dragieva, Dimi; Mustafa, Sonila; Williams, Elina

    2016-01-01

    Background The population of adolescents and young adults with congenital heart disease (CHD) is growing exponentially. These survivors are at risk of late cardiac complications and require lifelong cardiology care. However, there is a paucity of data on how to prepare adolescents to assume responsibility for their health and function within the adult health care system. Evidence-based transition strategies are required. Methods The Congenital Heart Adolescents Participating in Transition Eva...

  2. Complete congenital heart block in autoimmune hepatitis (SLA-positive).

    Science.gov (United States)

    Knolle, P; Mayet, W; Lohse, A W; Treichel, U; Meyer zum Büschenfelde, K H; Gerken, G

    1994-08-01

    Complete congenital heart block is a serious complication of neonatal lupus erythematosus which most often occurs in children of mothers suffering from connective tissue disease. We report the occurrence of complete congenital heart block associated with autoimmune hepatitis (SLA-positive). A 32-year-old woman was treated for more than 10 years for autoimmune hepatitis (SLA-/ANA-positive) and remained in clinical remission under immunosuppressive therapy. She showed an MHC-haplotype typical for autoimmune hepatitis (A1, B8, DR3). After a normal first pregnancy, an emergency caesarean section was performed in the 32nd week of her second pregnancy because of fetal bradycardia. The child died a few hours after delivery of complete congenital AV-block. Retrospective analysis of the maternal serum showed the emergence of SS-A/Ro-antibodies prior to the second pregnancy. The maternal serum antibodies were reactive with the 52 kD SS-A/Ro-antigen, as demonstrated by immunoblot employing recombinant SSA/Ro-antigen. The occurrence of complete congenital heart block has been shown to be associated with the presence of SS-A/Ro antibodies as well as the MHC-haplotype DR3. With respect to this genetic linkage, pregnant patients with autoimmune hepatitis and the MHC-haplotype DR3 should be examined for the presence of SS-A/Ro-antibodies. They should be closely followed during pregnancy to enable early detection of the development of congenital heart block, as prevention by plasmapheresis plus dexamethasone may be possible at an early stage.

  3. 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.

  4. Radionuclide angiocardiography in the diagnosis of congenital heart disorders

    Energy Technology Data Exchange (ETDEWEB)

    Jones, R.H.; Austin, E.H.; Peter, C.A.; Sabiston, D.C. Jr.

    1981-06-01

    Radionuclide angiocardiography provides a noninvasive assessment of cardiac function and blood flow through the heart and lungs. During the past three years, this procedure has been used at the Duke University Medical Center for evaluation of 343 patients with congenital heart disorders. A review of this experience shows tat the resulting data were frequently useful in the surgical management of these patients. In patients with abnormal blood flow patterns, noninvasive imaging of blood flow was useful before and after operative correction. Radionuclide measurements of left-to-right intracardiac shunts were sufficiently accurate for use in the initial evaluation of patients with murmurs and to document the absence of shunt after operative closure of intracardiac septal defects. Moreover, measurements of right-to-left cardiac shunts were of benefit in the management of children with cyanotic heart disease. Measurements of left ventricular function obtained during rest and exercise were most useful in patients with origin of the left coronary artery from the pulmonary artery and in patients with congenital valvular insufficiency. This experience demonstrates that radionuclide angiocardiography provides important measurements of central hemodynamics and cardiac function which are useful in the management of patients with congenital heart disorders.

  5. A congenital malformation of the systemic heart complex in Sepia officinalis L. (Cephalopoda)

    Science.gov (United States)

    Schipp, R.; von Boletzky, S.; Jakobs, P.; Labourg, P. J.

    1998-03-01

    In semi-adult Sepia officinalis L. (Cephalopoda) from the Bay of Arcachon (France) a congenital malformation of the systemic heart is described by macro-and microscopical methods. It concerns an atypical doubling of the site of insertion at the cephalic aorta at the apical ventricle. Its comparison with the paired anlagen of the systemic heart complex in normal embryogenesis and the central circulatory system of Nautilus gives rise to interpret it as a form of atavism. The possible causal role of mutagenic antifoulings is discussed.

  6. A method for accurate, non-destructive diagnosis of congenital heart defects from heart specimens

    OpenAIRE

    Schleich, Jean-Marc; Abdulla, Tariq; Houyel, Lucile; Paul, Jean-François; Summers, Ron; Dillenseger, Jean-Louis

    2013-01-01

    International audience The accurate analysis of congenital heart defect (CHD) specimens is often difficult and up to now required the opening of the heart. The objective of this study is to define a non-destructive method that allows for the precise analysis of each specimen and its different cardiac components in order to improve classification of the defect and thus provide an indication of underpinning causal mechanisms. We propose a method in which the heart volume is acquired by a CT ...

  7. Living with a Congenital Heart Defect

    Science.gov (United States)

    ... for people with CHD. I have always loved cycling; and in 2005, I completed my first endurance ... important to talk with a healthcare provider about diet and nutrition. Medications Some children and adults with ...

  8. Computer-assisted instruction; MR imaging of congenital heart disease

    International Nuclear Information System (INIS)

    To develop a software program for computer-assisted instruction on MR imaging of congenital heart disease for medical students and residents to achieve repetitive and effective self-learning. We used a film scanner(Scan Maker 35t) and IBM-PC(486 DX-2, 60 MHz) for acquisition and storage of image data. The accessories attached to the main processor were CD-ROM drive(Sony), sound card(Soundblaster-Pro), and speaker. We used software of Adobe Photoshop(v 3.0) and paint shop-pro(v 3.0) for preprocessing image data, and paintbrush from microsoft windows 3.1 for labelling. The language used for programming was visual basic(v 3.0) from microsoft corporation. We developed a software program for computer-assisted instruction on MR imaging of congenital heart disease as an effective educational tool

  9. 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.

  10. 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.

  11. Assessing the influence of consanguinity on congenital heart disease

    International Nuclear Information System (INIS)

    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

  12. 10.7.Congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    1993-01-01

    930270 Clinical analysis of rupture of aorticsinus aneurysm in 30 cases.ZHANG Yuwei (张玉威),et al.Instit Cardiovasc Dis,Dept Med,Shenyang Milit General Hosp,PLA.Chin Cir J1993;8(1):30—31.Rupture of aortic sinus aneurysm is a rarecongenital heart disease.It is sometimes misdi-agnosed and treated because of no typical symp-toms and signs.30 cases with rupture of a-neurysm of aortic sinus were studied.Clinicaldata suggested that the disease could be firstlynoticed when the healthy male aged 20~40years developed suddenly chest pain or extreme-ly uncomfortable feeling with gradually increas-ing heart dysfunction low—frequency harsh andsuperficial continuous precordial murmurs.Chest X-ray exhibited enlargement of heartand asymmetry of pulmonary plethora.ECG

  13. Role of Pulmonary Angiography in Congenital Heart Diseases among Children

    Directory of Open Access Journals (Sweden)

    Kavita U Vaishnav

    2013-08-01

    Full Text Available CT is a useful imaging modality for the morphologic evaluation of CHD. CT is useful in identifying different types of congenital anomalies of the cardiovascular system in children. It is essentially useful in the evaluation of vascular anomalies located outside the heart and in the assessment of complications that occur post operatively. [Natl J Med Res 2013; 3(4.000: 332-337

  14. Containing Costs in the Treatment of Congenital Heart Disease

    OpenAIRE

    Waldman, J. Deane; George, Lily; Lamberti, John J.; Lodge, Frederick A.; Pappelbaum, Stanley J.; Turner, Searle W.; Mathewson, James W.; Kirkpatrick, Stanley E.

    1984-01-01

    In the Congenital Heart Program at San Diego Children's Hospital, alterations in medical practice have reduced costs without impairing quality or access. Pediatric cardiac catheterization was done in 483 consecutive elective patients without overnight hospital stay. Hospital readmission was required in one patient for psoas tendinitis. Avoiding overnight hospital stay minimized attendant risks of hospital care, lessened psychosocial trauma and reduced the average hospital bill by $493 (29%). ...

  15. Implantable cardioverter-defibrillators in congenital heart disease

    OpenAIRE

    Chubb, H; Rosenthal, E

    2016-01-01

    Implantable cardioverter-defibrillators (ICD) have an important role in reducing sudden cardiac death in patients with congenital heart disease (CHD); however, the benefit of ICDs needs to be weighed up against both short-term and long-term adverse effects, which are difficult to evaluate in the heterogeneous CHD population. A tailored approach, taking into account risk stratification and patient-specific factors, is needed to select the most appropriate strategy. This review discusses primar...

  16. 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.

  17. Prevalence of Dyslipidemia in Children with Congenital Heart Disease

    International Nuclear Information System (INIS)

    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. Unknown syndrome: abnormal facies, congenital heart defects, hypothyroidism, and severe retardation.

    OpenAIRE

    Young, I D; Simpson, K.

    1987-01-01

    We present a female infant with (1) abnormal facies: microcephaly, blepharophimosis, small, low set, posteriorly rotated ears, bulbous nose, carp shaped mouth, micrognathia; (2) congenital heart abnormalities: large atrial and ventricular septal defects; (3) congenital hypothyroidism; (4) severe global retardation.

  19. 77. Ultrasonography assessment of congenital renal anomalies in children with congenital heart diseases

    OpenAIRE

    M.H. Hamadah; Kabbani, M; M.A. Faraji

    2016-01-01

    Ultrasound (US) assessment of renal anomalies in children requiring pediatric cardiac surgery is not a standard practice. This study aimed to study the role of bedside US performed by intensivists to detect occult renal anomalies associated with congenital heart disease (CHD). Prospective descriptive study for 100 consecutive children with CHD admitted to Pediatric Cardiac Intensive Care Unit (PCICU) from Januarry 1st, 2015 through June, July 2015. Ultrasound of kidneys was performed initiall...

  20. 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.

  1. Congenital Heart Disease: How to Care for Your Baby and Yourself

    Science.gov (United States)

    MENU Return to Web version Congenital Heart Disease | How to Care for Your Baby and Yourself I was depressed when I found out my baby has a heart problem. ... a sick baby. Source Caring for Infants with Congenital Heart Disease and Their Families by RB Saenz, M.D., ...

  2. Use of an Administrative Database to Determine Clinical Management and Outcomes in Congenital Heart Disease

    OpenAIRE

    Gutgesell, Howard P.; Hillman, Diane G.; McHugh, Kimberly E.; Dean, Peter; Matherne, G. Paul

    2011-01-01

    We review our 16-year experience using the large, multi-institutional database of the University HealthSystem Consortium to study management and outcomes in congenital heart surgery for hypoplastic left heart syndrome, transposition of the great arteries, and neonatal coarctation. The advantages, limitations, and use of administrative databases by others to study congenital heart surgery are reviewed.

  3. Haemodynamic findings on cardiac CT in children with congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo [University of Ulsan College of Medicine, Department of Radiology and Research Institute of Radiology, Asan Medical Center, Seoul (Korea, Republic of)

    2011-02-15

    In patients with congenital heart disease, haemodynamic findings demonstrated on cardiac CT might provide useful hints for understanding the haemodynamics of cardiac defects. In contrast to morphological features depicted on cardiac CT, such haemodynamic findings on cardiac CT have not been comprehensively reviewed in patients with congenital heart disease. This article describes normal haemodynamic phenomena of cardiovascular structures and various abnormal haemodynamic findings with their mechanisms and clinical significance on cardiac CT in patients with congenital heart disease. (orig.)

  4. Families of children with congenital heart disease: A literature review.

    Science.gov (United States)

    Wei, Holly; Roscigno, Cecelia I; Hanson, Cherissa C; Swanson, Kristen M

    2015-01-01

    In 2000 and 2002, the National Heart, Lung, and Blood Institute launched two initiatives to encourage treatment innovations and research on children with heart disease and their families. Since then, no systematic reviews have examined the evidence regarding the impacts of having a child with congenital heart disease (CHD) on families. This review synthesized key findings regarding families of children with CHD, critiqued research methods, described what has been done, and provided recommendations for future inquiry. Databases searched included PubMed, CINAHL, Family & Society Studies Worldwide, Women's Studies International, and PsycINFO. The literature search followed the PRISMA guidelines. As a result, ninety-four articles were reviewed. Four major themes were derived: parents' psychological health, family life, parenting challenges, and family-focused interventions. In conclusion, while they found parents having psychological symptoms, researchers did not explore parents' appraisals of what led to their symptoms. Research is needed to explore parents' experiences and expectations. PMID:26404115

  5. Spironolactone therapy in infants with congestive heart failure secondary to congenital heart disease.

    OpenAIRE

    Hobbins, S M; Fowler, R. S.; Rowe, R D; Korey, A G

    1981-01-01

    The efficacy of treatment with spironolactone for congestive heart failure secondary to congenital heart disease was studied in 21 infants under 1 year of age. All received digoxin and chlorothiazide. In addition, group A (n = 10) was given supplements of potassium and group B (n = 11) received spironolactone. Daily clinical observations of vital signs, weight, hepatomegaly, and vomiting were recorded. Paired t test analysis showed significant reduction in liver size and weight (P less than 0...

  6. Osteogenesis Imperfecta Type II with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Sona Khangare

    2008-05-01

    Full Text Available Objective: Osteogenesis imperfect (OI is an inherited disorder of type1 collagen synthesis with varied complication. OI type II is a perinatally lethal variety, characterized by short limbs, broad long bones, radiologic evidence of severe osseous fragility and defective ossification. These patient usually are stillborn or die in early infancy of respiratory failure. It has a wide range of phenotypic expressions, but cardiovascular anomalies tend to be rare association. When they do occur, they usually consist of aortic or mitral valve disease. Case Presentation: Here we come across a rare case of OI type II in a neonate with acyanotic congenital heart disease. Echocardiography revealed moderate size ostium secundum atrial septal defect without pulmonary hypertension. The patient expired after 6 hour of life. Conclusion: Any case of OI should be screened for congenital cardiovascular defect and another abnormality.

  7. Autoimmune congenital heart block: complex and unusual situations.

    Science.gov (United States)

    Brito-Zerón, P; Izmirly, P M; Ramos-Casals, M; Buyon, J P; Khamashta, M A

    2016-02-01

    Autoimmune congenital heart block (ACHB) is an immune-mediated cardiac disease included among the manifestations collectively referred to as neonatal lupus. The placental transference of maternal Ro/La autoantibodies may damage the conduction tissues during fetal development leading to blocking of signal conduction at the atrioventricular (AV) node in an otherwise structurally normal heart. Irreversible complete AV block is the main cardiac manifestation of ACHB, but some babies may develop endocardial fibroelastosis, valvular insufficiency, and/or frank cardiomyopathies with significantly reduced cardiac function requiring transplant. The severity of ACHB is illustrated by a global mortality rate of 20% and pacemaker rates of at least 64%, often within the first year of life. This review analyses the main complex and/or unusual clinical situations associated with ACHB, including unusual maternal immunological profiles, infrequent maternal autoimmune diseases, cardiac damage unrelated to AV block, fetal invasive management, late complications after birth, risk of congenital heart block (CHB) in ovodonation and in vitro fertilization techniques, the role of maternal features other than autoimmunity, the influence of the birth order or the risk of CHB in twins and triplets. PMID:26762645

  8. Cine magnetic resonance imaging in congenital heart disease

    International Nuclear Information System (INIS)

    Cine magnetic resonance imaging (MRI) was performed in 33 patients aged 19 days to 18 years (mean 5.1 years), who had congenital heart disease comfirmed at echocardiography or angiography. Prior to cine MRI, gated MRI with spin echo (SE) sequence was perfomed to evaluate cardiac structure. Cine MRI was demonstrated by fast low fip angle shot imaging technique with a 30 deg flip angle, 15 msec echo time, 30∼40 msec pulse repetition time, and 128X128 acquisition matrix. Abnormalities of cardiac structure were extremely well defined in all patients. Intracardiac and intravasucular blood flow were visualized with high signal intensity area, whereas ventricular filling flow and left to right shunt flow through ventricular septal defect and atrial septal defect were visualized with low signal intensity area. However, in the patients who had severe congestive heart failure or respiratory arrhythmia, the good recording of cine MRI was not obtained because of artifacts. Gated MRI with SE sequence provides excellent visualization of fine structures, and cine MRI can provide high spatial resolution imaging of flow dynamic in a variety of congenital heart disease, noninvasively. (author)

  9. 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.

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

    International Nuclear Information System (INIS)

    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. (letter)

  11. Fetal echocardiographic screening in twins for congenital heart diseases

    Institute of Scientific and Technical Information of China (English)

    LI Hui; MENG Tao; SHANG Tao; GUAN Yun-ping; ZHOU Wei-wei; YANG Guang; BI Li-hua

    2007-01-01

    Background Congenital heart disease (CHD) is the most common congenital disorder at birth. Yagel and colleagues's method of heart examination has been proved valuable in finding CHD prenatally in single pregnancies. The aim of this study was to analyze the frequency of CHD in twin pregnancies and the sensitivity of the method.Methods A total of 1103 pregnant women with twins were enrolled in this study, including 127 cases with high-risk for CHD. Five transverse ultrasound measurements were used for fetal heart examination, including the upper abdomen view, four-chamber view, five-chamber view, pulmonary artery bifurcation view, and three-vessel view. In the fetuses who were diagnosed with CHD and whose parents requested termination of the pregnancy, autopsy of the fetal heart was performed after an abortion, and a blood sample was collected from the heart for chromosome evaluation. In the other fetuses, a close follow-up was conducted by echocardiography within one year after birth.Results Antenatally, CHD was found in 12 twins, of which 4 were from the high-risk group (3.15%), and 8 from the low-risk group (0.82%). In 2 pairs of the twins, the two fetuses had a same kind of CHD (one pair had tetralogy of Fallot (TOF), another pair had rhabdomyoma). Another pair had different types of anomaly (one fetus had TOF, and the other duodenal atresia with a normal heart). Termination of pregnancy was performed in these three pairs and the autopsy of the fetal heart confirmed the ultrasound findings. In the other 9 pairs, CHD was detected in one fetus, and a normal heart in the others. In the cases who received chromosome evaluation, 2 had abnormal chromosomes. During the follow-up after birth, heart examinations confirmed the prenatal diagnosis in 7 of the 9. The diagnosis of CHD was missed antenatally in 2 pairs of twins. In both the cases, one fetus was normal, and the other was confirmed as having CHD after birth (small ventricle septum defect in one, and persistent

  12. Congenital meningocoele presenting in an adult

    Directory of Open Access Journals (Sweden)

    Raghvendra V Ramdasi

    2014-01-01

    Full Text Available A 53-year-old male patient presented with low back pain radiating to both lower limbs for 2 years and urinary incontinence for 2 months. He had swelled over his lower back since birth. The neurological examination revealed a decreased perianal sensation. Local examination of the lumbar swelling showed a brilliantly transilluminant, cystic midline swelling in the lumbar area with underlying spina bifida. Magnetic resonance imaging revealed a low-lying conus at L3 level associated with spina bifida at L5 and a meningocoele sac. The patient underwent excision of the meningocoele and detethering of cord by extirpation of hypertrophied filum terminale. The patient had an immediate relief of his symptoms. At 3 month follow-up the lumbar wound had healed well, and there was a significant improvement in the perianal hypoesthesia. The adult presentation of a congenital meningocoele and spinal dysraphism is rare, especially in the fifth decade. The possible causes of this delayed presentation are analyzed, and the relevant literature on the subject is presented.

  13. Adult duodenal intussusception associated with congenital malrotation

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Enteroenteric intussusception is a condition in which full-thickness bowel wall becomes telescoped into the lumen of distal bowel. In adults, there is usually an abnormality acting as a lead point, usually a Meckels' diverticulum, a hamartoma or a tumour. Duodenoduodenal intussusception is exceptionally rare because the retroperitoneal situation fixes the duodenal wall.The aim of this report is to describe the first published case of this condition. A patient with duodeno-duodenal intussusception secondary to an ampullary lesion is reported. A 66 year-old lady presented with intermittent abdominal pain, weight loss and anaemia. Ultrasound scanning showed dilated bile and pancreatic ducts.CT scanning revealed intussusception involving the full-thickness duodenal wall. The lead point was an ampullary villous adenoma. Congenital partial (type Ⅱ)malrotation was found at operation and this abnormality permitted excessive mobility of the duodenal wall such that intussusception was possible. This condition can be diagnosed using enhanced CT. Intussusception can be complicated by bowel obstruction, ischaemia or bleeding,and therefore the underlying cause should be treated as soon as possible.

  14. Haploinsufficiency of TAB2 Causes Congenital Heart Defects in Humans

    OpenAIRE

    Thienpont, Bernard; Zhang, Litu; Postma, Alex V; Breckpot, Jeroen; Tranchevent, Léon-Charles; Van Loo, Peter; Møllgård, Kjeld; Tommerup, Niels; Bache, Iben; Tümer, Zeynep; van Engelen, Klaartje; Menten, Björn; Mortier, Geert; Waggoner, Darrel; Gewillig, Marc

    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-β-activated kinase 1/M...

  15. Congenital Heart Disease in an Infant with 49,XXXXY Syndrome

    Directory of Open Access Journals (Sweden)

    Mustafa Argun

    2015-04-01

    Full Text Available 49,XXXXY syndrome which is characterized with the addition of three extra X chromosomes to 46,XY is the rarest sex chromosome aneuploidy syndrome. Its classical findings were defined as a triad of mental retardation, hypogonadism and radioulnar synostosis. In 49,XXXXY syndrome, congenital heart defects like patent ductus arteriosus, atrial septal defect, ventricular septal defect, pulmonary stenosis, Fallot’s tetralogy have been reported. We present a case diagnosed in the newborn stage with low birth weight, short stature, dysmorphic craniofacial findings and hypoplastic male genitalia who was found to have severe pulmonary hypertension and medium patent ductus arteriosus when admitted at 4 months of age with heart failure and who underwent transcathater ductus closure with Amplatzer Duct Occluder I. To our knowledge, our case is the first reported 49,XXXXY syndrome with patent ductus arteriosus closed with the transcathater route.

  16. Studies of Genes Involved in Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Tushar K. Ghosh

    2014-05-01

    Full Text Available Congenital heart disease (CHD affects the intricate structure and function of the heart and is one of the leading causes of death in newborns. The genetic basis of CHD is beginning to emerge. Our laboratory has been engaged in identifying mutations in genes linked to CHD both in families and in sporadic cases. Over the last two decades, we have employed linkage analysis, targeted gene sequencing and genome wide association studies to identify genes involved in CHDs. Cardiac specific genes that encode transcription factors and sarcomeric proteins have been identified and linked to CHD. Functional analysis of the relevant mutant proteins has established the molecular mechanisms of CHDs in our studies.

  17. Haploinsufficiency of TAB2 causes congenital heart defects in humans

    DEFF Research Database (Denmark)

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

    2010-01-01

    in cardiac development was further supported by its conserved expression in the developing human and zebrafish heart. Moreover, a critical, dosage-sensitive role during development was demonstrated by the cardiac defects observed upon titrated knockdown of tab2 expression in zebrafish embryos......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...... demonstrated that this translocation disrupts TAB2. Taken together, these data clearly demonstrate a role for TAB2 in human cardiac development....

  18. Epidemiological study of congenital heart defects in children and adolescents: analysis of 4,538 cases

    Directory of Open Access Journals (Sweden)

    Nelson Itiro Miyague

    2003-03-01

    Full Text Available OBJECTIVE: To analyze the frequency and prevalence of congenital heart defects in a tertiary care center for children with heart diseases. METHODS: We carried out an epidemiological assessment of the first medical visit of 4,538 children in a pediatric hospital from January 1995 to December 1997. All patients with congenital heart defects had their diagnoses confirmed at least on echocardiography. The frequency and prevalence of the anomalies were computed according to the classification of sequential analysis. Age, weight, and sex were compared between the groups of healthy individuals and those with congenital heart defects after distribution according to the age group. RESULTS: Of all the children assessed, 2,017 (44.4% were diagnosed with congenital heart disease, 201 (4.4% with acquired heart disease, 52 (1.2% with arrhythmias, and 2,268 (50% were healthy children. Congenital heart diseases predominated in neonates and infants, corresponding to 71.5% of the cases. Weight and age were significantly lower in children with congenital heart defects. Ventricular septal defect was the most frequent acyanotic anomaly, and tetralogy of Fallot was the most frequent cyanotic anomaly. CONCLUSION: Children with congenital heart defects are mainly referred during the neonatal period and infancy with impairment in gaining weight. Ventricular septal defect is the most frequent heart defect.

  19. 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…

  20. 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.…

  1. Lactacidosis in the neonate is minimized by prenatal detection of congenital heart disease

    NARCIS (Netherlands)

    Verheijen, PM; Lisowski, LA; Stoutenbeek, P; Hitchcock, JF; Bennink, GBWE; Meijboom, EJ

    2002-01-01

    Objectives To investigate the impact of prenatal detection of congenital heart disease on preventing severe preoperative lactacidosis. Design Patients operated upon for congenital heart disease during the first 31 days of life (n=209) were studied retrospectively, 21 were diagnosed prenatally and 18

  2. 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....

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

    OpenAIRE

    Mohindra R; Beebe David; Belani Kumar

    2002-01-01

    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 evaluat...

  4. 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. PMID:27504381

  5. Cardiovascular MRI in congenital heart disease. An imaging atlas

    Energy Technology Data Exchange (ETDEWEB)

    Sridharan, Shankar; Price, Gemma; Tann, Oliver; Hughes, Marina; Muthurangu, Vivek; Taylor, Andrew M. [UCL Institute of Child Health and Great Ormond Street Hospital for Children, London (United Kingdom). Centre for Cardiovascular MR

    2010-07-01

    This new and unique clinical resource offers rapid access to high-quality images covering a broad spectrum of paediatric and adult cardiac pathologies visualized using MRI and CT. Key images of each condition and a clear interpretation of their MR appearances allow for greater understanding of the pathology. Focus is given to the planning of imaging planes, techniques and sequences to obtain the best images and improve MR assessment. This text would benefit all health professionals involved in imaging congenital cardiac disease. (orig.)

  6. The Role of Cardiovascular Magnetic Resonance in Pediatric Congenital Heart Disease

    OpenAIRE

    Taylor Andrew M; Hughes Marina L; Ntsinjana Hopewell N

    2011-01-01

    Abstract Cardiovascular magnetic resonance (CMR) has expanded its role in the diagnosis and management of congenital heart disease (CHD) and acquired heart disease in pediatric patients. Ongoing technological advancements in both data acquisition and data presentation have enabled CMR to be integrated into clinical practice with increasing understanding of the advantages and limitations of the technique by pediatric cardiologists and congenital heart surgeons. Importantly, the combination of ...

  7. Prognostic Value of Serum B-Type Natriuretic Peptide in Early Mortality and Morbidity of Children with Congenital Heart Disease after Open Heart Surgery

    Directory of Open Access Journals (Sweden)

    Nozar Givtaj

    2009-06-01

    Full Text Available Background: Brain type natriuretic peptide (BNP is a cardiac hormone that is secreted mainly by the ventricles in response to volume expansion and pressure load. It can predict post-operative complications after heart surgery in adults. We sought to investigate the prognostic value of BNP in children after heart surgery. Methods: We measured the BNP serum levels in 96 children with congenital heart diseases before, immediately after, and 12 hours after open heart surgery. We studied the ability of the post-operative BNP serum level variations to predict mortality and morbidity in children. Results: In total, 96 patients, comprising 40 (41.7% females and 56 (58.3% males with a mean age of 4.1 years (range: 1 month to 17 years, with various congenital heart diseases were studied. The rise in the serum BNP level 12 hours post surgery was directly related to mortality before discharge from hospital (P value=0.004, congestive heart failure after surgery (P value<0.001 , patients' cyanosis (P value=0.045, duration of ICU stay (r=0.342, P value=0.004, and post-operative need for inotropic drugs (P value<0.001. Conclusion: The rise in the BNP serum level 12 hours after heart surgery is a good marker for predicting mortality, morbidity, and early diagnosis of heart failure in children.

  8. 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.

  9. Mutations of connexin43 in fetuses with congenital heart malformations

    Institute of Scientific and Technical Information of China (English)

    CHEN Ping; XIE Li-jian; HUANG Guo-ying; ZHAO Xiao-qing; CHANG Cai

    2005-01-01

    Background Gap junction channels formed by connexin43 (Cx43) protein are important in cardiac morphogenesis, and Cx43 gene is thought to be associated with congenital heart malformation (CHM). This study was undertaken to detect the mutations of Cx43 in fetuses with CHM.Methods Cx43 extron DNA was amplified by PCR from 16 fetuses with a variety of CHM. The PCR products were analyzed by SSCP and DNA sequencing. Thirty children who had no CHM were selected as controls. Results Eight homozygous mutations of Cx43 were observed in a fetus with double outlet right ventricule (DORV), five of the 8 mutations were missense mutations including Arg239Trp, Ser251Thr, Ala253Pro, Pro283Leu and Thr290Asn, and the remaining 3 were silent polymorphisms including Gly252Gly, Pro256Pro and Thr275Thr. No mutations were found in other fetuses and the control group.Conclusions Mutations of Cx43 may be associated with congenital conotruncal anomalies. PCR-SSCP is an effective method for screening the mutations of Cx43.

  10. Genetics of congenital heart disease: the glass half empty.

    Science.gov (United States)

    Fahed, Akl C; Gelb, Bruce D; Seidman, J G; Seidman, Christine E

    2013-02-15

    Congenital heart disease (CHD) is the most common congenital anomaly in newborn babies. Cardiac malformations have been produced in multiple experimental animal models, by perturbing selected molecules that function in the developmental pathways involved in myocyte specification, differentiation, or cardiac morphogenesis. In contrast, the precise genetic, epigenetic, or environmental basis for these perturbations in humans remains poorly understood. Over the past few decades, researchers have tried to bridge this knowledge gap through conventional genome-wide analyses of rare Mendelian CHD families, and by sequencing candidate genes in CHD cohorts. Although yielding few, usually highly penetrant, disease gene mutations, these discoveries provided 3 notable insights. First, human CHD mutations impact a heterogeneous set of molecules that orchestrate cardiac development. Second, CHD mutations often alter gene/protein dosage. Third, identical pathogenic CHD mutations cause a variety of distinct malformations, implying that higher order interactions account for particular CHD phenotypes. The advent of contemporary genomic technologies including single nucleotide polymorphism arrays, next-generation sequencing, and copy number variant platforms are accelerating the discovery of genetic causes of CHD. Importantly, these approaches enable study of sporadic cases, the most common presentation of CHD. Emerging results from ongoing genomic efforts have validated earlier observations learned from the monogenic CHD families. In this review, we explore how continued use of these technologies and integration of systems biology is expected to expand our understanding of the genetic architecture of CHD. PMID:23410880

  11. 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.

  12. 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.

  13. 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. PMID:27212797

  14. Pregnancy after fontan repair of complex congenital heart disease.

    Science.gov (United States)

    Hoare, J V; Radford, D

    2001-11-01

    We describe four successful pregnancies in three women who had previously had a Fontan repair for congenital heart disease. Each pregnancy resulted in a live birth and there was no maternal mortality The infants were premature, being delivered at 26, 30 and 35 weeks, and weighing 1,020, 1,333 and 1,930 g respectively The fourth infant was born at 32 weeks and no birthweight is available. Maternal complications occurred and were those anticipated after a Fontan repair. Two mothers required treatment for supraventricular arrhythmias (atrial flutter and fibrillation). Ventricular failure was present in two mothers and required ongoing drug treatment. Raised systemic venous pressures caused peripheral oedema in two mothers and hepatomegaly and ascites in one mother. The physiology, potential complications, anaesthetic concerns and drug treatment in pregnancy after Fontan repair are discussed.

  15. The team approach to pregnancy and congenital heart disease.

    Science.gov (United States)

    Kafka, Henryk; Johnson, Mark R; Gatzoulis, Michael A

    2006-11-01

    The successful pediatric management of congenital heart disease has resulted in increasing numbers of these patients in the reproductive age group and increasing clinical challenges for their physicians. These challenges can be met successfully, with improved results for mother and child, through a concerted comprehensive team approach that relies on a thorough understanding of the patient's underlying cardiac pathology and its anticipated interaction with the pregnancy, and ongoing close evaluation and communication with a team of trained and experienced specialist, including (but not limited to) cardiologist, obstetricians, anesthetists, pediatricians, clinical nurse specialists, and clinical geneticists. Such teams are not always available locally and it will be necessary to refer medium- and high-risk patients to a specialized tertiary care center.

  16. Genetic link between renal birth defects and congenital heart disease.

    Science.gov (United States)

    San Agustin, Jovenal T; Klena, Nikolai; Granath, Kristi; Panigrahy, Ashok; Stewart, Eileen; Devine, William; Strittmatter, Lara; Jonassen, Julie A; Liu, Xiaoqin; Lo, Cecilia W; Pazour, Gregory J

    2016-01-01

    Structural birth defects in the kidney and urinary tract are observed in 0.5% of live births and are a major cause of end-stage renal disease, but their genetic aetiology is not well understood. Here we analyse 135 lines of mice identified in large-scale mouse mutagenesis screen and show that 29% of mutations causing congenital heart disease (CHD) also cause renal anomalies. The renal anomalies included duplex and multiplex kidneys, renal agenesis, hydronephrosis and cystic kidney disease. To assess the clinical relevance of these findings, we examined patients with CHD and observed a 30% co-occurrence of renal anomalies of a similar spectrum. Together, these findings demonstrate a common shared genetic aetiology for CHD and renal anomalies, indicating that CHD patients are at increased risk for complications from renal anomalies. This collection of mutant mouse models provides a resource for further studies to elucidate the developmental link between renal anomalies and CHD. PMID:27002738

  17. Oral contraceptive use and conotruncal congenital heart disease

    Directory of Open Access Journals (Sweden)

    Mars Nashrah Abdullah

    2014-11-01

    Full Text Available Background Congenital heart disease (CHD represents some of the more prevalent malformations among live births and remains the leading cause of death from congenital malformations. Conotruncal anomalies comprise a diverse group of CHD involving the outflow tracts of the heart and the great vessels. Oral contraceptive exposure before pregnancy may be one of the risk factors for conotruncal CHD. Objective To evaluate the effect of oral contraceptive use before pregnancy on the risk of conotruncal CHD in children. Methods A case-control study was conducted from July 2010 until July 2011 in Haji Adam Malik Hospital, Medan. Subjects with CHD were divided into two groups: conotruncal CHD as the case group and non-conotruncal CHD as the control group. Both groups had mothers with and without histories of oral contraceptive use before pregnancy. Parents were interviewed using questionnaires. Statistical analyses were performed using Chi-square test, student’s T-test, and Mann Whitney test. Results A total of 80 subjects were eligible, with 40 subjects in each group. The percentages of subjects whose mothers used oral contraceptives were 62% of the conotruncal CHD group and 60% of the non-conotruncal CHD group (OR 0.82; 95%CI 0.33 to 1.98. The mean duration of maternal oral contraceptive use before pregnancy was 19.1 months for the case group and 18.8 months for the control group (P=0.87. Conclusion In children with CHD, maternal oral contraceptive use before pregnancy does not appear to increase the risk of conotruncal CHD. [Paediatr Indones. 2014;54:309-13.].

  18. 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.

  19. 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.

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

    Directory of Open Access Journals (Sweden)

    Christine H. Attenhofer Jost

    2013-01-01

    Full Text Available 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.

  1. CLINICAL STUDY OF CONGENITAL HEART DISEASE IN INFANTS IN TERTIARY CARE HOSPITAL

    OpenAIRE

    Sandeep V.Harshangi; Laxmi Nagaraj Itagi; Venkatesh Patil; Vijayanath.V

    2013-01-01

    Congenital heart disease are primarily disease of neonates, infants and children. The burden of CHD in India is likely to be enormous due to very high birth rate. The reported incidence is 8-10/ 1000 live births. Objective was to know the pattern of clinical presentation of various congenital heart disease in 0-1 year age group. To study the different form of cyanotic and acyanotic congenital heart disease in present cases. 50 cases of CHD proven by 2D echocardiography were studied for 2 ye...

  2. I point my heart with the tip of my fingers - Biometry for the diagnosis of congenital heart defects

    OpenAIRE

    Abdulla, Tariq; Dillenseger, Jean-Louis; Summers, Ron; Paul, Jean-François; Houyel, Lucile; Schleich, Jean-Marc

    2013-01-01

    International audience Congenital heart defects are characterized by abnormal positioning of some anatomical structures relative to a normal heart. Classically the classification of the disease or the stage of the disease is based on the measurement of the relative position of these structures by the cardiac morphologist. We propose a method in which the heart volume is acquired by a CT scanner. In this paper we present a tool which allows us to define landmarks interactively in this heart...

  3. Can folic acid protect against congenital heart defects in Down syndrome?

    NARCIS (Netherlands)

    Meijer, Willemijn M.; Werler, Martha M.; Louik, Carol; Hernandez-Diaz, Sonia; de Jong-van den Berg, Lolkje T. W.; Mitchell, Allen A.

    2006-01-01

    BACKGROUND: Several studies have suggested a protective effect of folic acid (FA) on congenital heart anomalies. Down syndrome (DS) infants are known to have a high frequency of heart anomalies. Not all children with DS suffer from heart anomalies, which raises the question whether maternal factors

  4. Congenital Heart Defects in Europe Prevalence and Perinatal Mortality, 2000 to 2005

    NARCIS (Netherlands)

    Dolk, Helen; Loane, Maria; Garne, Ester

    2011-01-01

    Background-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. Methods and Results-Data were extracted from the European Surveillance of Congenital Anomalies central database for

  5. Homocysteine related Nutritional and Genetic Risk Factors for Human Congenital Heart Defects

    NARCIS (Netherlands)

    A.C. Verkleij-Hagoort (Anna)

    2007-01-01

    textabstractCongenital heart defects (CHDs) belong to the most common group of major congenital malformations in newborns. Most CHDs are considered complex diseases with a multifactorial aetiology, which are thought to result from interactions between genetic and environmental factors. This thesis p

  6. The origin of congenital heart defects and the epigenetic programming of the healthy child

    NARCIS (Netherlands)

    S.A. Obermann-Borst (Sylvia)

    2013-01-01

    textabstractBirth defects are a global burden affecting 7% of births worldwide. Congenital heart defects (CHD) are the most common congenital malformation with approximately 1 million children born each year. It is not only the most frequent group of birth defects in human, but also the leading caus

  7. Pregnancy-related Health Behavior of Women with Congenital Heart Disease : Room for Behavioral Change Interventions

    NARCIS (Netherlands)

    Moons, Philip; Budts, Werner; Costermans, Els; Huyghe, Els; Pieper, Petronella G.; Drenthen, Wim

    2009-01-01

    Background. Pregnancy in women with congenital heart disease is associated with maternal and neonatal complications. In order to reduce risks for unfavorable outcomes, pregnant women need to adopt specific health behaviors. We investigated the pregnancy-related health behavior of women with congenit

  8. Congenital lobar emphysema in an adult

    Directory of Open Access Journals (Sweden)

    Mir Sadaqat

    2011-01-01

    Full Text Available Congenital lobar emphysema (CLE is a clinico-radiological diagnosis, seen usually by four-six weeks of age (50% of patients and rarely (<5% of patients after the age of six months. Here, we report a young male with gradual onset of mild exertional breathlessness and physical examination revealing the features of right sided pneumothorax. X-ray of chest, with subsequent CT of chest, leads to the diagnosis of CLE. The pulmonary function tests, bronchoscopic examination and a1 -antitrypsin level are normal. Patient is managed conservatively.

  9. State-of-the-Art CT Imaging Techniques for Congenital Heart Disease

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo [Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    2010-02-15

    CT is increasingly being used for evaluating the cardiovascular structures and airways in the patients with congenital heart disease. Multi-slice CT has traditionally been used for the evaluation of the extracardiac vascular and airway abnormalities because of its inherent high spatial resolution and excellent air-tissue contrast. Recent developments in CT technology primarily by reducing the cardiac motion and the radiation dose usage in congenital heart disease evaluation have helped expand the indications for CT usage. Tracheobronchomalacia associated with congenital heart disease can be evaluated with cine CT. Intravenous contrast injection should be tailored to unequivocally demonstrate cardiovascular abnormalities. Knowledge of the state-of-the-art CT imaging techniques that are used for evaluating congenital heart disease is helpful not only for planning and performing CT examinations, but also for interpreting and presenting the CT image findings that consequently guide the proper medical and surgical management.

  10. De novo mutations in Congenital Heart Disease with Neurodevelopmental and Other Birth Defects

    OpenAIRE

    Homsy, Jason; Zaidi, Samir; Shen, Yufeng; Ware, James S.; Samocha, Kaitlin E; Karczewski, Konrad J.; DePalma, Steven R.; McKean, David; Wakimoto, Hiroko; Gorham, Josh; Jin, Sheng Chih; Deanfield, John; Giardini, Alessandro; Porter, George A.; Kim, Richard

    2015-01-01

    Congenital heart disease (CHD) patients have increased prevalence of extra-cardiac congenital anomalies (CA) and risk of neurodevelopmental disabilities (NDD). Exome sequencing of 1,213 CHD parent-offspring trios identified an excess of protein-damaging de novo mutations, especially in genes highly expressed in developing heart and brain. These mutations accounted for 20% of patients with CHD, NDD and CA but only 2% with isolated CHD. Mutations altered genes involved in morphogenesis, chromat...

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

    OpenAIRE

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

    2015-01-01

    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...

  12. Heart transplantation for congenital heart disease in the first year of life.

    Science.gov (United States)

    Chinnock, Richard E; Bailey, Leonard L

    2011-05-01

    Successful infant heart transplantation has now been performed for over 25 years. Assessment of long term outcomes is now possible. We report clinical outcomes for322 patients who received their heart transplant during infancy. Actuarial graft survival for newborn recipients is 59% at 25 years. Survival has improved in the most recent era. Cardiac allograft vasculopathy is the most important late cause of death with an actuarial incidence at 25 years of 35%. Post-transplant lymphoma is estimated to occur in 20% of infant recipients by25 years. Chronic kidney disease grade 3 or worse is present in 31% of survivors. The epidemiology of infant heart transplantation has changed through the years as the results for staged repair improved and donor resources remained stagnant. Most centers now employ staged repair for hypoplastic left heart syndrome and similar extreme forms of congenital heart disease. Techniques for staged repair, including the hybrid procedure, are described. The lack of donors is described with particular note regarding decreased donors due to newer programs for appropriate infant sleep positioning and infant car seats. ABO incompatible donors are a newer resource for maximizing donor resources, as is donation after circulatory determination of death and techniques to properly utilize more donors by expanding the criteria for what is an acceptable donor. An immunological advantage for the youngest recipients has long been postulated, and evaluation of this phenomenon may provide clues to the development of accommodation and/or tolerance. PMID:22548030

  13. 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.

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

    Science.gov (United States)

    Mitra, Souvik; Saha, Anindya Kumar; Sardar, Syamal Kumar; Singh, Arun Kumarendu

    2013-07-01

    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.

  15. New York Heart Association class assessment by cardiologists and outpatients with congenital cardiac disease: a head-to-head comparison of three patient-based versions

    NARCIS (Netherlands)

    D. Schoormans; Y.L. Mager; F.J. Oort; M.A.G. Sprangers; B.J.M. Mulder

    2012-01-01

    Background: The objective of this study was to compare three patient-based New York Heart Association assessments with cardiologist assessments in outpatients with congenital cardiac disease. Methods: Consecutive adult outpatients completed three questionnaires in a random order: a patient-based tra

  16. APN plan improves outcome for pregnant patient with congenital heart disease.

    Science.gov (United States)

    Haynes, Annette; Frederick, Andrea; Chirkoff, Andrea

    2012-01-01

    Advanced practice nurses work in many roles to support delivery of safe patient care. Eighty-five percent of children born with congenital heart disease (CHD) live to adulthood. The pregnant adult with CHD presents challenges for nursing across many care-delivery systems. Progression of care delivery across these systems requires innovative planning and organization. This article describes the plan developed by advanced practice nurses in a CHD clinic and in inpatient coronary care and obstetric units to support a pregnant patient with CHD. The plan focused on collaboration and communication among interdisciplinary teams. The goal was to address multidisciplinary communication, leadership, and staff education. The result was a successful high-risk delivery with organized education and patient care across systems. PMID:22543487

  17. 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 be of...... importance. The aim of this study was to characterise the haemostatic profile and examine the potential role of haematocrit on clot formation and strength in CCHD patients. Furthermore to examine whether CCHD patients with history of haemoptysis have diminished fibrinogen function compared to those without...... haemoptysis. METHODS: In a prospective study 75 adult CCHD patients had haematocrit, platelet count, and plasma fibrinogen concentration examined. Furthermore thrombelastography(TEG) as well as TEG Functional Fibrinogen(TEG FF) assay evaluating fibrinogen function(FLEV) was performed. Data were compared with...

  18. Progressive pulmonary hypertension in cyanotic congenital heart disease with severe pulmonary stenosis

    OpenAIRE

    Cheung, YF; Leung, MP; Wang, EP

    1996-01-01

    We report the progressive development of pulmonary hypertension despite the presence of severe pulmonary stenosis in three patients with cyanotic congenital heart disease. The associated intracardiac lesions were complete transposition with a ventricular septal defect, double outlet right ventricle, and a heart with univentricular atrioventricular connection, respectively. Serial measurements on hemodynamics andhistological findings documented the progression of pulmonary hypertension and pul...

  19. IS LOW BIRTH WEIGHT ASSOCIATED WITH CONGENITAL HEART DISEASE?

    Directory of Open Access Journals (Sweden)

    Nutan Nalini

    2016-03-01

    Full Text Available BACKGROUND Daily we see lots of still birth cases and the agony associated with it after carrying the fetus for so many months with the negative outcome it is quite disheartening. Malnutrition is quite rampant in the country as far as the females are concerned and in the name of the religion and rituals, it complicates the issue further. If the mother is malnourished, the chance of low birth weight baby is high. OBJECTIVES To correlate the prevalence of cardiac anomalies in low birth weight fetuses. MATERIAL AND METHOD The study was carried out in 40 stillbirth fetuses with detailed account of nutritional status of the mother, consanguinity, history of previous pregnancies, miscarriages, socio-economic status and exposure to drugs/radiation. The number of cases with fetus having cardiac anomalies was quite significant. RESULTS Out of total 40 stillbirth fetuses, 29 (72.5% stillbirths were from less than 2.5kg and 11 (27.5% were from more than 2.5kg. Cardiac anomalies were present in 16 and 02 cases respectively. Fused heart-01, mesocardia-01, Dextrocardia with CORV-02, Interrupted aortic arch-02, Abnormal origin of right subclavian artery-01, Tetralogy of Fallot-01, VSD-07, ASD-01, transposition of great vessels-01, persistent truncus arteriosus with tricuspid atresia, elongated hypertrophied abnormally positioned Rt. Atrium, rudimentary Rt. Ventricle-01, mal-attachment of ductus arteriosus-03, double superior vena cava-01, dilated caval system-01. CONCLUSION Considering the high incidence of cardiac anomalies in fetuses of low birth weight, we must try to create awareness and support the expectant mothers to avoid the low birth weight babies and thus the congenital anomalies.

  20. 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

  1. Adult duodenal intussusception associated with congenital malrotation

    OpenAIRE

    Gardner-Thorpe, J; Hardwick, RH; Carroll, NR; Gibbs, P.; Jamieson, NV; Praseedom, RK

    2007-01-01

    Enteroenteric intussusception is a condition in which full-thickness bowel wall becomes telescoped into the lumen of distal bowel. In adults, there is usually an abnormality acting as a lead point, usually a Meckels' diverticulum, a hamartoma or a tumour. Duodeno-duodenal intussusception is exceptionally rare because the retroperitoneal situation fixes the duodenal wall. The aim of this report is to describe the first published case of this condition. A patient with duodeno-duodenal intussusc...

  2. Report from the international society for nomenclature of paediatric and congenital heart disease: creation of a visual encyclopedia illustrating the terms and definitions of the international pediatric and congenital cardiac code.

    Science.gov (United States)

    Giroud, Jorge M; Jacobs, Jeffrey P; Spicer, Diane; Backer, Carl; Martin, Gerard R; Franklin, Rodney C G; Béland, Marie J; Krogmann, Otto N; Aiello, Vera D; Colan, Steven D; Everett, Allen D; William Gaynor, J; Kurosawa, Hiromi; Maruszewski, Bohdan; Stellin, Giovanni; Tchervenkov, Christo I; Walters, Henry L; Weinberg, Paul; Anderson, Robert H; Elliott, Martin J

    2010-10-01

    Tremendous progress has been made in the field of pediatric heart disease over the past 30 years. Although survival after heart surgery in children has improved dramatically, complications still occur, and optimization of outcomes for all patients remains a challenge. To improve outcomes, collaborative efforts are required and ultimately depend on the possibility of using a common language when discussing pediatric and congenital heart disease. Such a universal language has been developed and named the International Pediatric and Congenital Cardiac Code (IPCCC). To make the IPCCC more universally understood, efforts are under way to link the IPCCC to pictures and videos. The Archiving Working Group is an organization composed of leaders within the international pediatric cardiac medical community and part of the International Society for Nomenclature of Paediatric and Congenital Heart Disease (www.ipccc.net). Its purpose is to illustrate, with representative images of all types and formats, the pertinent aspects of cardiac diseases that affect neonates, infants, children, and adults with congenital heart disease, using the codes and definitions associated with the IPCCC as the organizational backbone. The Archiving Working Group certifies and links images and videos to the appropriate term and definition in the IPCCC. These images and videos are then displayed in an electronic format on the Internet. The purpose of this publication is to report the recent progress made by the Archiving Working Group in establishing an Internet-based, image encyclopedia that is based on the standards of the IPCCC.

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

    Institute of Scientific and Technical Information of China (English)

    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 ofCHDwere 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 ofCHD 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 ofCHD set up in the country round.

  4. The registry of anomalous aortic origin of the coronary artery of the Congenital Heart Surgeons' Society.

    Science.gov (United States)

    Brothers, Julie A; Gaynor, J William; Jacobs, Jeffrey P; Caldarone, Christopher; Jegatheeswaran, Anusha; Jacobs, Marshall L

    2010-12-01

    The anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva with interarterial, intramural, and/or intraconal course is a rare congenital anomaly that is associated with a high risk of sudden death in children. The Congenital Heart Surgeons' Society established the Registry of Anomalous Aortic Origin of the Coronary Artery to help determine the outcome of children and young adults managed with surgical intervention versus observation and to test the hypothesis that subsets of patients with anomalous aortic origin of a coronary artery can be identified in whom the risk of intervention is less than the risk of observation. All institutional members of the Congenital Heart Surgeons' Society were recruited for participation. The registry consists of a retrospective cohort of patients diagnosed between 1 January, 1998 and 20 January, 2009 and a prospective, population-based cohort of patients newly diagnosed from 21 January, 2009 onwards. Baseline demographics, diagnoses, and results of tests will be obtained through a review of the medical records. Annual follow-up data will be collected. Data will be analysed for different factors of risk at diagnosis, different strategies of treatment, and the impact of both on the outcomes of the patients. As of June 2010, 28 institutions had applied for approval from their institutional review board and 16 institutions had received approval from their institutional review board. Seventy-four patients have enrolled to date. We hope to use the established Pediatric Cardiomyopathy Registry as a guide to successful implementation, with a cooperative effort between institutions. The overall purpose of the Registry of Anomalous Aortic Origin of the Coronary Artery is to determine the outcome of surgical intervention versus observation in children and young adults with anomalous aortic origin of a coronary artery, and to describe the natural and "unnatural" history of these patients over the course of their lifetime

  5. Successful heart transplantation from a donor with Ullrich congenital muscular dystrophy

    OpenAIRE

    Plonka, Caitlyn; Wearden, Peter D.; Morell, Victor O.; Miller, Steven A.; Webber, Steven A.; Feingold, Brian

    2013-01-01

    Heart transplantation is the most effective therapy for children with end-stage heart disease; however, its use is limited by the number of donor organs available. This shortage may be further compounded by concerns about organ quality, leading to refusal of potential donor organ offers. We report on the successful transplantation and 5 year follow-up of a heart from a donor with Ullrich congenital muscular dystrophy (UCMD). The candidate was critically ill at the time of th...

  6. 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.

  7. Common variation in ISL1 confers genetic susceptibility for human congenital heart disease.

    Directory of Open Access Journals (Sweden)

    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.

  8. Quality of Life and Congenital Heart Disease in Childhood and Adolescence

    International Nuclear Information System (INIS)

    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

  9. Management considerations for the adult with congenital adrenal hyperplasia.

    Science.gov (United States)

    Auchus, Richard J

    2015-06-15

    The congenital adrenal hyperplasias (CAH) are a group of genetic defects in cortisol biosynthesis, most commonly steroid 21-hydroxylase deficiency (21OHD). With the advent of cortisone therapy in the 1960s and newborn screening in the 1990s, most children with 21OHD now reach adulthood. The needs and concerns of adults with 21OHD overlap with those of children, but the focus and approach shift as these patients reach adulthood. Cohort studies suggest that adults with 21OHD experience significant health concerns such as infertility, obesity, short stature, neoplasia, and bone loss, as well as reduced quality of life. Nevertheless, the spectrum of health status and disease severity is broad, but only some of the reasons for these disparities are known. This review will summarize the current state of knowledge and suggested approaches to management adults with classic 21OHD, plus a few major considerations for adults with nonclassic 21OHD.

  10. Congenital Heart Diseases in the Newborns of Diabetic Mothers: an Echocardiographic Study

    OpenAIRE

    S Rahimpour; V Modarresi; Behjati, M; MA Behjati

    2011-01-01

    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 classifie...

  11. A Retrospective Evaluation of the Patients with Congenital Heart Disease in Neonatal Intensive Care Unit

    OpenAIRE

    Emine Yurdakul Ertürk; Şükrü Küçüködük; Kemal Baysal; Pelin Ayyıldız; Ayşegül Yılmaz; Gönül Oğur

    2016-01-01

    Introduction: Congenital heart disease (CHD) is the most common congenital anomaly in newborns. In this study it was aimed to investigate the demographic features of the patients with CHD diagnosed in neonatal intensive care unit (NICU) to determine the frequency of the anomalies and to elucidate the etiologic risk factors. Materials and Methods: The files of 361 newborns who were diagnosed with CHD and hospitalized at Ondokuz Mayıs University NICU were retrospectively examined from 2006 t...

  12. Adverse effect left congenital diaphragmatic hernia to position and function of the fetal heart

    OpenAIRE

    Adhi Pribadi; Johanes Cornelius Mose

    2015-01-01

    Background: There many heart problems in patients with CDH. Methods: Between January 2012 and July 2015, three pregnancies (1:2153) performed ultrasound examination to investigate suspected CDH. All symptoms displayed descriptively in table. Results: In prenatal life, ultrasonography has a high sensitivity for detection of congenital diaphragmatic hernia. The definite ultrasonographic diagnosis of fetal congenital diaphragmatic hernia lies on the visualization of abdominal organs in th...

  13. 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

  14. Relationship Between Polymorphism of Methylenetetrahydrofolate Dehydrogenase and Congenital Heart Defect

    Institute of Scientific and Technical Information of China (English)

    JUN CHENG; WEN-LI ZHU; JING-JING DAO; SHU-QING LI; YONG LI

    2005-01-01

    Objective To investigate the relationship between G1958A gene polymorphism of methylenetetrahydrofolate dehydrogenase (MTHFD) and occurrence of congenital heart disease (CHD) in North China. Methods One hundred and ninety-two CHD patients and their parents were included in this study as case group in Liaoning Province by birth defect registration cards, and 124 healthy subjects (age and gender matched) and their parents were simultaneously selected from the same geographic area as control. Their gene polymorphism of MTHFD G1958A locus was examined with PCR-RFLP, and serum folic acid and homocysteine (Hcy) levels were tested with radio-immunoassay and fluorescence polarization immunoassay (FPIA). Results There existed gene polymorphism at MTHFD G1958A locus in healthy subjects living in North China. The percentages of GG, GA, and AA genotype were 57.98%, 35.57%, and 6.45% respectively, and the A allele frequency was 24.23%, which was significantly different from Western population. No difference was observed when comparing genotype distribution and allele frequency between the case and control groups, so was the result from the comparison between genders. The A allele frequency of arterial septal defect patients' mothers (10.87%) was significantly lower than that of controls (28.15%) (P=0.014), with OR=0.31 (95% CI: 0.09-0.84), and no difference in the other subgroups. The percentage of at least one parent carrying A allele in arterial septal defect subgroup (43.48%) was significantly lower than that in controls (69.64%) (P=0.017), with OR=0.34 (95% CI: 0.12-0.92). The analysis of genetic transmission indicated that there was no transmission disequillibrium in CHD nuclear families. Their serum folic acid level was significantly higher than that of controls (P=0.000), and Hcy level of the former was higher than that of the latter with no statistical significance (P>0.05). Serum Hcy and folic acid levels of mothers with gene mutation were lower than those of mothers

  15. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary arterial hypertension associated with congenital heart disease

    Directory of Open Access Journals (Sweden)

    Antonio Lopes

    2014-01-01

    Full Text Available Congenital heart disease (CHD with intracardiac/extracardiac shunts is an important etiology of pulmonary arterial hypertension (PAH. The majority of children with congenital cardiac shunts do not develop advanced pulmonary vasculopathy, as surgical repair of the anomalies is now performed early in life. However, if not repaired early, some defects will inevitably lead to pulmonary vascular disease (truncus arteriosus, transposition of the great arteries associated with a ventricular septal defect (VSD, atrioventricular septal defects remarkably in Down syndrome, large, nonrestrictive VSDs, patent ductus arteriosus and related anomalies. The majority of patients are now assigned to surgery based on noninvasive evaluation only. PAH becomes a concern (requiring advanced diagnostic procedures in about 2-10% of them. In adults with CHD, the prevalence of advanced pulmonary vasculopathy (Eisenmenger syndrome is around 4-12%. [1] This article will discuss the diagnostic and management approach for PAH associated with CHD (PAH-CHD.

  16. DISTRIBUTION OF CONGENITAL HEART DISEASES AT TERTIARY CARE CENTER: SINGLE CENTER EXPERIENCE

    Directory of Open Access Journals (Sweden)

    Vaishali

    2016-02-01

    Full Text Available BACKGROUND The current study was undertaken at a tertiary care center, Bhopal, MP, India, with the objective of establishing frequency of occurrence of congenital heart diseases by echocardiography. MATERIALS AND METHOD 10,000 consecutive cases undergoing Echo Color Doppler in the Cardiology Department Hamidia Hospital, Bhopal, between 1st Jan 2009 and July 2011 were analysed. Echo CD was performed by consultant cardiologist using Acuson Aspen Color Doppler machine following the ASE guidelines. RESULTS AND CONCLUSIONS In the present study out of 10,000consecutive cases undergoing echo 648 were identified to having congenital heart diseases. Isolated VSD (30.5%, isolated ASD (23.6% PDA (9% and TOF (8.3% were commonest defect observed. Most common congenital heart disease found in the present study is VSD and is most prevalent in males and is highest among 0-5 yrs.

  17. The present and future of interventional catheterization for congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    @@Over the past decade, the focus of the pediatric catheterization laboratory has changed dramatically from its primary function of providing diagnosis to offering treatment.Since Rashkind first described the technique for atrial septostomy in the setting of complete transposition of the great arteries in 1966, therapeutic catheterization techniques have replaced conventional surgery, including both corrective and palliative surgeries, for many lesions. Interventional catheterization has become an important modality in the treatment of congenital heart disease. In addition, in the staged management of complex congenital heart disease, better outcomes have been acquired by combined surgical and interventional catheterization strategies. Although great advances have been made in the therapeutic catheterization for congenital heart disease in some Chinese medical centers, there is considerable difference in both clinical applications and basic researches between China and other developed countries. Thus, it is very important to expand this advanced technique aggressively but carefully.

  18. Ultrasonographic views for the screening of congenital heart defects in the first level of care

    International Nuclear Information System (INIS)

    Congenital heart diseases are the main cause of infant mortality for congenital malformations in our country and they are the defects that more usually escape diagnosis in ultrasonographic screening, especially if we consider that associated risk factors call for a fetal echocardiogram are not identified in most pregnant women with fetuses affected with a heart disease. With this paper, we intend to bring within reach of both the specialists dedicated to this activity in primary care and the Masters in Genetic Counseling a review article about the principal aspects to be evaluated in each of the three echocardiography views that are used in Cuba as part of screening these defects, as well as the main signs of suspicion of congenital heart diseases that give reason for having a pregnant woman referred to the immediately higher level of care

  19. 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.

  20. Down syndrome: Molecular mapping of the congenital heart disease and duodenal stenosis

    Energy Technology Data Exchange (ETDEWEB)

    Korenburg, J.R. (University of California, Los Angeles (United States)); Bradley, C.; Disteche, C.M. (University of Washington, Seattle (United States))

    1992-02-01

    Down syndrome (DS) is a major cause of congenital heart and gut disease and mental retardation. DS individuals also have characteristic facies, hands, and dermatoglyphics, in addition to abnormalities of the immune system, and increased risk of leukemia, and an Alzheimer-like dementia. Although their molecular basis is unknown, recent work on patients with DS and partial duplications of chromosome 21 has suggested small chromosomal regions located in band q22 that are likely to contain the genes for some of these features. The authors now extend these analyses to define molecular markers for the congenital heart disease, the duodenal stenosis, and an 'overlap' region for the facial and some of the skeletal features. They report the clinical, cytogenetic, and molecular analysis of two patients. These studies provide the molecular basis for the construction of a DS phenotypic map and focus the search for genes responsible for the physical features, congenital heart disease, and duodenal stenosis of DS.

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

    International Nuclear Information System (INIS)

    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

  2. Introduction: December 2015 HeartWeek Issue of Cardiology in the Young - Highlights of HeartWeek 2015: Challenges and Dilemmas of Pediatric Cardiac Care including Heart Failure in Children and Congenital Abnormalities of the Coronary Arteries.

    Science.gov (United States)

    Jacobs, Jeffrey P

    2015-12-01

    This December Issue of Cardiology in the Young represents the 13th annual publication in Cardiology in the Young generated from the two meetings that composed "HeartWeek in Florida". "HeartWeek in Florida", the joint collaborative project sponsored by the Cardiac Centre at the Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, together with Johns Hopkins All Children's Heart Institute of Saint Petersburg, Florida, averages over 1000 attendees every year and is now recognised as one of the major planks of continuing medical and nursing education for those working in the fields of diagnosis and treatment of cardiac disease in the foetus, neonate, infant, child, and adult. "HeartWeek in Florida" combines the International Symposium on Congenital Heart Disease, organised by All Children's Hospital and Johns Hopkins Medicine, and entering its 16th year, with the Annual Postgraduate Course in Paediatric Cardiovascular Disease, organised by The Children's Hospital of Philadelphia entering its 19th year. This December 2015 Issue of Cardiology in the Young features highlights of the two meetings that compose HeartWeek. Johns Hopkins All Children's Heart Institute's 15th Annual International Symposium on Congenital Heart Disease was held at the Renaissance Vinoy Resort & Golf Club, Saint Petersburg, Florida, from Friday, 6 February, 2015, to Monday, 9 February, 2015. This Symposium was co-sponsored by The American Association for Thoracic Surgery and its special focus was "Congenital Abnormalities of the Coronary Arteries". The Children's Hospital of Philadelphia's annual meeting - Cardiology 2015, the 18th Annual Update on Paediatric and Congenital Cardiovascular Disease: "Challenges and Dilemmas" - was held at the Hyatt Regency Scottsdale Resort and Spa at Gainey Ranch, Scottsdale, Arizona, from Wednesday, 11 February, 2015, to Sunday, 15 February, 2015. We would like to acknowledge the tremendous contributions made to paediatric and congenital cardiac care

  3. Cardiac surgery in grown-up congenital heart patients. Will the surgical workload increase?

    DEFF Research Database (Denmark)

    Klcovansky, J.; Søndergård, Lars; Helvind, M.;

    2008-01-01

    The number of patients with grown-up congenital heart (GUCH) disease is steadily increasing. Although there is agreement that the medical service for GUCH patients should be expanded in coming years, it is still unknown whether this should also include the surgical service. In an attempt to eluci......The number of patients with grown-up congenital heart (GUCH) disease is steadily increasing. Although there is agreement that the medical service for GUCH patients should be expanded in coming years, it is still unknown whether this should also include the surgical service. In an attempt...

  4. Evaluation of the grading and disorder assessment of congenital heart disease with pulmonary arterial hypertension

    International Nuclear Information System (INIS)

    Pulmonary arterial hypertension is one of the most common and serious complications in congenital heart disease. Identification of whether the pulmonary, arterial hypertension is dynamic or resistance remains as the great importance for deciding to transfer for surgery, intervention or conservative therapy and directly concerning with the prognosis and choice of treatment. This review mainly deals with the problems such as grading, staging, pathophysiology and the correlative mechanism with clinical assessment of pulmonary. arterial hypertension in congenital heart disease and furthermore providing comprehensive informations for clinical diagnosis and treatment. (authors)

  5. 1st and 2nd Trimester Headsize in Fetuses with Congenital Heart Disease: A Cohort Study

    DEFF Research Database (Denmark)

    Lauridsen, Mette Høj; Petersen, Olav Bjørn; Vestergaard, Else Marie;

    2014-01-01

    Background: Congenital heart disease (CHD) is associated with neuro-developmental disorders. The influence of CHD on the brain may be present in the fetus. We hypothesize that fetal cerebral growth is impaired as early as 2nd trimester. Aim: To investigate if fetal cerebral growth is associated...... and screening for fetal malformations is carried out. Our cohort includes all fetuses in Western Denmark (2.9 million inhabitants) screened in between January 1st 2012 and December 31st 2013, diagnosed with any structural, non-syndromic congenital heart disease either during pregnancy or up to 6...

  6. MR evaluation of cardiovascular physiology in congenital heart disease: flow and function.

    Science.gov (United States)

    Weber, Oliver M; Higgins, Charles B

    2006-01-01

    Cardiovascular magnetic resonance (CMR) has become the method of choice in the evaluation of a number of questions in congenital heart disease. In addition to morphology, modern CMR techniques allow the visualization of function and flow in a temporally resolved manner. Among the pathologies where these methods play a major role are shunts, septal defects, aortic coarctation, anomalies of the pulmonary arteries, and valvular regurgitation. This paper explains the basics of functional and flow encoded CMR and discusses their application in the assessment of several types of congenital heart disease.

  7. Treacher Collins syndrome with multiple congenital heart defects after paroxetine exposure: case report.

    Science.gov (United States)

    Dinlen, N; Zenciroğlu, A; Dilli, D; Aydin, B; Beken, S; Okumuş, N

    2014-01-01

    Treacher Collins syndrome is an autosomal dominant disorder of craniofacial development with an incidence of I in 40,000 to in 70,000 live births. It is characterized by abnormalities of the pinnae which are frequently associated with atresia of the external auditory canals and anomalies of the middle ear ossicles. Rarely congenital heart defects can be present. Prenatal paroxetine exposure may enhance the risks of major malformation, particularly cardiac defects. This article reports a newborn, whose mother used paroxetine during pregnancy, presenting with multiple congenital heart defects associated to typical physical characteristics of Treacher Collins syndrome.

  8. Double Aneuploidy 48,XXY,+21 Associated with a Congenital Heart Defect in a Neonate.

    Science.gov (United States)

    Shu, X; Zou, C; Shen, Z

    2013-12-01

    A neonate with a double aneuploidy associated with congenital heart defect (CHD) suffered from cyanosis after birth. He had typical features of Down syndrome (DS) including hypertelorism, slightly lowset ears with protruding pinna. Doppler echocardiography indicated complex congenital heart disease with an ostium secundum atrial septal defect, enlarged right ventricle, and mild tricuspid valve regurgitation. Further chromosomal analysis showed a karyotype of 48,XXY,+21: a double aneuploidy of DS and Klinefelter syndrome (KS). Until now, only seven cases of double aneuploidy associated with CHD defect have been reported. PMID:24778570

  9. Two congenital coronary abnormalities affecting heart function: anomalous origin of the left coronary artery from the pulmonary artery and congenital left main coronary artery atresia

    Institute of Scientific and Technical Information of China (English)

    Xiao Yanyan; Jin Mei; Han Ling; Ding Wenhong; Zheng Jianyong; Sun Chufan; Lyu Zhenyu

    2014-01-01

    Background The anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) and congenital left main coronary artery atresia (CLMCA-A) are two kinds of very rare coronary heart diseases which affect heart function profoundly.This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients.Methods From April 1984 to July 2012,in Beijing Anzhen Hospital,23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A.We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation,diagnosis,and treatments of these two kinds of congenital coronary abnormalities.Results The 23 patients (13 males and 10 females,aged ranging from 2.5 months to 65 years) identified with ALCAPA were classified into infantile type (age of onset younger than 12 months,16 cases) and adult type (age of onset older than 12 months,7 cases).Four patients were diagnosed with CLMCA-A (three males and one female,aged ranging from 3 months to 2 years).The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection,heart failure,dyspnea,feeding intolerance,diaphoresis,and failure to thrive.And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis,dilated cardiomyopathy,and acute myocardial infarction.As for the adult-type ALCAPA,cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease,myocarditis,or patent ductus arteriosus.In ECG examination:Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I,avL,and V4-V6,especially in lead avL.However,ECG of adult-type ALCAPA lacked distinct features.In chest radiography:pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A,while pulmonary artery segment dilation was more common in

  10. Living With Half a Heart - Experiences of Young Adults With Single Ventricle Physiology

    DEFF Research Database (Denmark)

    Overgaard, Dorthe; King, Catriona; Christensen, Rie F;

    2013-01-01

    Research (UCSF), Rigshospitalet, Copenhagen, Denmark. Abstract BACKGROUND AND RESEARCH OBJECTIVE: Approximately 3% of children with congenital heart disease born in Denmark have single ventricle physiology (SVP). In previous decades, these children did not survive into adulthood. However, because of new......J Cardiovasc Nurs 2013;28(2):187-96 Living With Half a Heart-Experiences of Young Adults With Single Ventricle Physiology: A Qualitative Study. Overgaard D, King C, Christensen RF, Schrader AM, Adamsen L. SourceDorthe Overgaard, PhD, RN Researcher, The Heart Centre, Copenhagen University Hospital...

  11. Congenital Heart Diseases in the Newborns of Diabetic Mothers: an Echocardiographic Study

    Directory of Open Access Journals (Sweden)

    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.

  12. Noteworthy Literature in 2015: Anesthesia for Congenital Heart Disease.

    Science.gov (United States)

    Twite, Mark; Ing, Richard J

    2016-03-01

    This article is a review of the literature published during the 12 months of 2015, which is of interest to the congenital cardiac anesthesiologist. While the review is not exhaustive, it identifies 7 themes in the literature for 2015 and cites 78 peer-reviewed publications. PMID:26783264

  13. Pulmonary vascular remodeling in congenital heart disease : Enhanced expression of heat shock proteins

    NARCIS (Netherlands)

    Geiger, Ralph; Sharma, Hari S.; Mooi, Wolter J.; Berger, Rolf M. F.

    2009-01-01

    In congenital heart disease (CHD), mechanical wall stress by increased pulmonary artery pressure and pulmonary blood flow is believed to play a pivotal role in the pathogenesis of pulmonary plexogenic arteriopathy (PPA). The pathogenesis of this disease that involves significant pulmonary arterial r

  14. Outcome of pregnancy in women with congenital heart disease - A literature review

    NARCIS (Netherlands)

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

    2007-01-01

    A search of peer-reviewed literature was conducted to identify reports that provide data on complications associated with pregnancy in women with structural congenital heart disease (CHD). This review describes the outcome of 2,491 pregnancies, including 377 miscarriages (15%) and 114 elective abort

  15. Awareness of infective endocarditis prophylaxis in parents of children with congenital heart disease: A prospective survey

    International Nuclear Information System (INIS)

    A prospective survey of parents of the children with congenital heart disesease was conducted to determine their awareness as regards the importance of oral hygiene and prophylaxis against infective endocarditis (IE). The results of this study demonstrated that only 8% of the parents were aware of the importance of good oro-dental hygiene and need for IE prophylaxis

  16. Explanatory value of the ability index as assessed by cardiologists and patients with congenital heart disease

    DEFF Research Database (Denmark)

    Overgaard, Dorthe; Schrader, Anne-Marie; Lisby, Karen Holst;

    2012-01-01

    The Ability Index was developed to classify patients with congenital heart disease into four functional classes. Functional class is typically determined by the cardiologist, based on data from a clinical interview. The validity of the Ability Index as assessed by the patient has never been...

  17. Emotional and Behavioural Problems in Children and Adolescents with Congenital Heart Disease

    Science.gov (United States)

    Johnson, Beena; Francis, Johnson

    2005-01-01

    Major physical illnesses usually have an impact on the psychological well-being of any individual. An illness of early onset, with necessity of frequent diagnostic and therapeutic interventions can adversely affect the emotional balance and behavioural adaptation of children and adolescents. This is applicable for congenital heart disease,…

  18. Explanatory Value of the Ability Index as Assessed by Cardiologists and Patients with Congenital Heart Disease

    DEFF Research Database (Denmark)

    Schrader, Anne-Marie Voss; Lisby, Karen H; King, Catriona;

    2012-01-01

    Introduction. The Ability Index was developed to classify patients with congenital heart disease into four functional classes. Functional class is typically determined by the cardiologist, based on data from a clinical interview. The validity of the Ability Index as assessed by the patient has...

  19. Physical Activity and Sport Participation in Youth with Congenital Heart Disease: Perceptions of Children and Parents

    Science.gov (United States)

    Moola, Fiona; Faulkner, Guy E. J.; Kirsh, Joel A.; Kilburn, Jennifer

    2008-01-01

    This study explored perceptions toward physical activity and sport in the lives of youth with congenital heart disease. Thirteen cardiac participants were interviewed in the presence of their parents, and a process of inductive analysis was conducted. Sport was not considered a valued pursuit despite the belief that it is essential for the…

  20. Menstrual cycle and its disorders in women with congenital heart disease.

    NARCIS (Netherlands)

    Drenthen, W.; Hoendermis, E.S.; Moons, P.; Heida, K.Y.; Roos-Hesselink, J.W.; Mulder, B.J.M.; Dijk, A.P.J. van; Vliegen, H.W.; Sollie, K.M.; Berger, R.M.; Lely, A.T.; Canobbio, M.M.; Pieper, P.G.

    2008-01-01

    OBJECTIVES: To investigate the age at menarche, the prevalence of menstrual cycle (interval) disorders, and determinants in women with congenital heart disease (CHD). DESIGN: Using two CHD registries, 1802 (82%) of the 2196 women with CHD contacted (aged 18-58 years) provided written informed consen

  1. Risk Factors for Gross Motor Dysfunction in Infants with Congenital Heart Disease

    Science.gov (United States)

    Long, Suzanne H.; Eldridge, Bev J.; Galea, Mary P.; Harris, Susan R.

    2011-01-01

    Infants with congenital heart disease (CHD) that is severe enough to require early surgery are at risk for cognitive and motor delays, as well as musculoskeletal impairments, and are best managed by an interdisciplinary team during their hospital stay and after discharge. The purpose of this article is to review some of the risk factors associated…

  2. Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease

    NARCIS (Netherlands)

    Zijlstra, Willemijn M H; Douwes, Johannes M; Ploegstra, Mark-Jan; Krishnan, Usha; Roofthooft, Marcel; Hillege, Hans L; Ivy, D Dunbar; Rosenzweig, Erika B; Berger, Rolf M F

    2016-01-01

    Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore

  3. Dietary intake of B-vitamins in mothers born a child with a congenital heart defect

    NARCIS (Netherlands)

    Verkleij-Hagoort, A.C.; Vries, de J.H.M.; Ursem, N.T.C.; Jonge, de R.; Hop, W.C.J.; Steegers-Theunissen, R.P.M.

    2006-01-01

    BACKGROUND: Periconceptional use of multivitamins reduces the risk of a child with a congenital heart defect (CHD). Data on the impact of maternal diet, however, are lacking. AIM OF THE STUDY: We investigated the association between the maternal dietary intake of B-vitamins and having a child with a

  4. The haematocrit – an important factor causing impaired haemostasis in patients with cyanotic congenital heart disease

    DEFF Research Database (Denmark)

    Jensen, A S; Johansson, P I; Idorn, L;

    2013-01-01

    BACKGROUND: Patients with cyanotic congenital heart disease(CCHD) have haemostatic abnormalities, which result in an increased risk of bleeding. The cause is unknown, but recent studies have indicated that an elevated haematocrit, which is present in cyanotic patients, could be an important factor...

  5. Awareness of infective endocarditis prophylaxis in parents of children with congenital heart disease: A prospective survey

    Directory of Open Access Journals (Sweden)

    Nath Parrimala

    2008-01-01

    Full Text Available A prospective survey of parents of the children with congenital heart disesease was conducted to determine their awareness as regards the importance of oral hygiene and prophylaxis against infective endocarditis (IE. The results of this study demonstrated that only 8% of the parents were aware of the importance of good oro-dental hygiene and need for IE prophylaxis.

  6. Awareness of infective endocarditis prophylaxis in parents of children with congenital heart disease: A prospective survey

    OpenAIRE

    Nath Parrimala; Kiran V; Maheshwari Sunita

    2008-01-01

    A prospective survey of parents of the children with congenital heart disesease was conducted to determine their awareness as regards the importance of oral hygiene and prophylaxis against infective endocarditis (IE). The results of this study demonstrated that only 8% of the parents were aware of the importance of good oro-dental hygiene and need for IE prophylaxis.

  7. PETER’S TYPE II ANOMALY ASSOCIATED WITH CONGENITAL HEART DEFECT: RARE CASE REPORT

    OpenAIRE

    Darshan Kumar; Sunil G

    2015-01-01

    PURPOSE: Reporting a case of Peters’ type II with congenital heart defect a rare form of anterior segment dysgenesis in which abnormal cleavage of the anterior chamber occurs. It is characterized by central corneal opacification secondary to defects in a posterior corneal stroma, Descemet's membrane, and endothelium. Majority of cases are sporadic, bilateral , and associated with other systemic malformations. MATERIAL AND METHOD: A...

  8. Screening of 99 Danish patients with congenital heart disease for GATA4 mutations

    DEFF Research Database (Denmark)

    Zhang, Litu; Tümer, Zeynep; Jacobsen, Joes Ramsøe;

    2006-01-01

    Congenital heart disease (CHD) affects nearly 1% of the population, but only few genes involved in human CHD are presently known. Germ-line mutations in the zinc finger transcription factor GATA4 have been associated with familial cases of atrial and ventricular septal defects and pulmonary steno...

  9. Recreational scuba diving in patients with congenital heart disease: Time for new guidelines.

    Science.gov (United States)

    Schleich, Jean-Marc; Schnell, Frédéric; Brouant, Benoît; Phan, Gerald; Lafay, Vincent; Bonnemains, Laurent; Bédossa, Marc

    2016-01-01

    The number of recreational scuba divers is steadily increasing. In its latest recommendations, the French Federation of Undersea Studies and Sports listed congenital heart disease as a formal and final contraindication to scuba diving. On the other hand, with the progress made in their management, the prognosis and quality of life of patients with congenital heart diseases have improved considerably, enabling them to engage in physical and sports endeavours, which are known to confer general health and psychological benefits. As a consequence, the ability of these patients to dive has become a regular and recurrent issue. We review the various types of scuba diving, the physical performance required for its practice, its effects on cardiovascular function and the elements that need to be considered before recommending whether it can be practiced safely at various levels of difficulty. Because of the diversity and broad heterogeneity of congenital heart diseases, a detailed evaluation of each patient's performance based on clinical criteria common to all congenital heart diseases is recommended. PMID:27364729

  10. Time Course of Atrial Fibrillation in Patients With Congenital Heart Defects

    NARCIS (Netherlands)

    Teuwen, C.P.; Ramdjan, T.T.; Gotte, M.; Brundel, B.J.; Evertz, R.; Vriend, J.W.; Molhoek, S.G.; Dorman, H.G.; Opstal, J.M. van; Konings, T.C.; Voort, P. van der; Delacretaz, E.; Houck, C.; Yaksh, A.; Jansz, L.J.; Witsenburg, M.; Roos-Hesselink, J.W.; Triedman, J.K.; Bogers, A.J.; Groot, N.M. de

    2015-01-01

    BACKGROUND: The incidence of atrial fibrillation (AF) is rising in the aging patients with congenital heart defects (CHD). However, studies reporting on AF in patients with CHD are scarce. The aim of this multicenter study was to examine in a large cohort of patients with a variety of CHD: (1) the a

  11. Time Course of Atrial Fibrillation in Patients With Congenital Heart Defects

    NARCIS (Netherlands)

    Teuwen, Christophe P.; Ramdjan, Tanwier T. T. K.; Gotte, Marco; Brundel, Bianca J. J. M.; Evertz, Reinder; Vriend, Joris W. J.; Molhoek, Sander G.; Dorman, Henderikus G. R.; van Opstal, Jurren M.; Konings, Thelma C.; van der Voort, Pepijn; Delacretaz, Etienne; Houck, Charlotte; Yaksh, Ameeta; Jansz, Luca. J.; Witsenburg, Maarten; Roos-Hesselink, Jolien W.; Triedman, John K.; Bogers, Ad J. J. C.; de Groot, Natasja M. S.

    2015-01-01

    Background The incidence of atrial fibrillation (AF) is rising in the aging patients with congenital heart defects (CHD). However, studies reporting on AF in patients with CHD are scarce. The aim of this multicenter study was to examine in a large cohort of patients with a variety of CHD: (1) the ag

  12. Time Course of Atrial Fibrillation in Patients with Congenital Heart Defects

    NARCIS (Netherlands)

    Teuwen, Christophe P; Ramdjan, Tanwier T T K; Götte, Marco; Brundel, Bianca J J M; Evertz, Reinder; Vriend, Joris W J; Molhoek, Sander G; Dorman, H G Reinhart; van Opstal, Jurren M; Konings, Thelma C; van der Voort, Pepijn; Delacrétaz, Etienne; Houck, Charlotte; Yaksh, Ameeta; Jansz, Luca J; Witsenburg, Maarten; Roos-Hesselink, Jolien W; Triedman, John K; Bogers, Ad J J C; de Groot, Natasja M S

    2015-01-01

    BACKGROUND: -The incidence of atrial fibrillation (AF) is rising in the aging patients with congenital heart disease (CHD). However, studies reporting on AF in CHD patients are scarce. The aim of this multicenter study was to examine in a large cohort of patients with a variety of CHD 1) the age of

  13. Are Grown-ups with Congenital Heart Disease Willing to Participate in an Exercise Program?

    NARCIS (Netherlands)

    Dontje, Manon L.; Feenstra, Marlies; de Greef, Mathieu H. G.; Nieuwland, Wybe; Hoendermis, Elke S.

    2014-01-01

    ObjectiveTo examine the willingness of grown-ups with congenital heart disease (GUCH) to participate in the GUCH Training Program-Individualised (GTI), an exercise program specifically designed for GUCH, and to identify factors affecting their willingness to participate. Design and SettingIn this cr

  14. Unusual type of congenital heart disease associated with the Holt-Oram-Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Ruzic, B.; Bosnar, B.; Beleznay, O.

    1981-06-01

    Case report of a very rare case of Holt-Oram-Syndrome (in a seven months old baby) associated with tricuspid atresia (itself a rare condition of isolated congenital heart desease) and anomalous return of pulmonary vein into the right atrium. According to the classification based on anatomy, our case corresponds to type Ia. The diagnosis was confirmed clinically, electrocardiographically, radiologically and angiographically.

  15. New clinical molecular diagnostic methods for congenital and inherited heart disease

    NARCIS (Netherlands)

    Jongbloed, Jan Dh; Pósafalvi, Anna; Kerstjens-Frederikse, Wilhelmina S; Sinke, Richard J; van Tintelen, J Peter

    2011-01-01

    IMPORTANCE OF THE FIELD: For patients with congenital and inherited heart disorders, causative mutations are often not identified owing to limitations of current screening techniques. Identifying the mutation is of major importance for genetic counseling of patients and families, facilitating the di

  16. Virtual cardiotomy based on 3-D MRI for preoperative planning in congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Soerensen, Thomas Sangild [University of Aarhus, Department of Computer Science, Aarhus N (Denmark); University of Aarhus, Institute of Clinical Medicine, Aarhus (Denmark); Beerbaum, Philipp; Razavi, Reza; Greil, Gerald Franz [King' s College London School of Medicine, Division of Imaging Sciences, London (United Kingdom); Guy' s and St. Thomas' Hospital, Department of Paediatric Cardiology, London (United Kingdom); Mosegaard, Jesper [Alexandra Institute, Aarhus (Denmark); Rasmusson, Allan [University of Aarhus, Department of Computer Science, Aarhus N (Denmark); Schaeffter, Tobias [King' s College London School of Medicine, Division of Imaging Sciences, London (United Kingdom); Austin, Conal [Guy' s and St. Thomas' Hospital, Department of Cardiothoracic Surgery, London (United Kingdom)

    2008-12-15

    Patient-specific preoperative planning in complex congenital heart disease may be greatly facilitated by virtual cardiotomy. Surgeons can perform an unlimited number of surgical incisions on a virtual 3-D reconstruction to evaluate the feasibility of different surgical strategies. To quantitatively evaluate the quality of the underlying imaging data and the accuracy of the corresponding segmentation, and to qualitatively evaluate the feasibility of virtual cardiotomy. A whole-heart MRI sequence was applied in 42 children with congenital heart disease (age 3{+-}3 years, weight 13{+-}9 kg, heart rate 96{+-} 21 bpm). Image quality was graded 1-4 (diagnostic image quality {>=}2) by two independent blinded observers. In patients with diagnostic image quality the segmentation quality was also graded 1-4 (4 no discrepancies, 1 misleading error). The average image quality score was 2.7 - sufficient for virtual reconstruction in 35 of 38 patients (92%) older than 1 month. Segmentation time was 59{+-}10 min (average quality score 3.5). Virtual cardiotomy was performed in 19 patients. Accurate virtual reconstructions of patient-specific cardiac anatomy can be produced in less than 1 h from 3-D MRI. The presented work thus introduces a new, clinically feasible noninvasive technique for improved preoperative planning in complex cases of congenital heart disease. (orig.)

  17. Congenital heart disease protein 5 associates with CASZ1 to maintain myocardial tissue integrity.

    Science.gov (United States)

    Sojka, Stephen; Amin, Nirav M; Gibbs, Devin; Christine, Kathleen S; Charpentier, Marta S; Conlon, Frank L

    2014-08-01

    The identification and characterization of the cellular and molecular pathways involved in the differentiation and morphogenesis of specific cell types of the developing heart are crucial to understanding the process of cardiac development and the pathology associated with human congenital heart disease. Here, we show that the cardiac transcription factor CASTOR (CASZ1) directly interacts with congenital heart disease 5 protein (CHD5), which is also known as tryptophan-rich basic protein (WRB), a gene located on chromosome 21 in the proposed region responsible for congenital heart disease in individuals with Down's syndrome. We demonstrate that loss of CHD5 in Xenopus leads to compromised myocardial integrity, improper deposition of basement membrane, and a resultant failure of hearts to undergo cell movements associated with cardiac formation. We further report that CHD5 is essential for CASZ1 function and that the CHD5-CASZ1 interaction is necessary for cardiac morphogenesis. Collectively, these results establish a role for CHD5 and CASZ1 in the early stages of vertebrate cardiac development.

  18. The Role of Cardiovascular Magnetic Resonance in Pediatric Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Taylor Andrew M

    2011-09-01

    Full Text Available Abstract Cardiovascular magnetic resonance (CMR has expanded its role in the diagnosis and management of congenital heart disease (CHD and acquired heart disease in pediatric patients. Ongoing technological advancements in both data acquisition and data presentation have enabled CMR to be integrated into clinical practice with increasing understanding of the advantages and limitations of the technique by pediatric cardiologists and congenital heart surgeons. Importantly, the combination of exquisite 3D anatomy with physiological data enables CMR to provide a unique perspective for the management of many patients with CHD. Imaging small children with CHD is challenging, and in this article we will review the technical adjustments, imaging protocols and application of CMR in the pediatric population.

  19. Correlations Between Echocardiographic Systolic and Diastolic Function with Cardiac Catheterization in Biventricular Congenital Heart Patients.

    Science.gov (United States)

    Nadorlik, H; Stiver, C; Khan, S; Miao, Y; Holzer, R; Cheatham, J P; Cua, C L

    2016-04-01

    Newer echocardiographic techniques may allow for more accurate assessment of left ventricular (LV) function. Adult studies have correlated these echocardiographic measurements with invasive data, but minimal data exist in the pediatric congenital heart population. Purpose of this study was to evaluate which echocardiographic measurements correlated best with LV systolic and diastolic catheterization parameters. Patients with two-ventricle physiology who underwent simultaneous echocardiogram and cardiac catheterization were included. Images were obtained in the four-chamber view. LV systolic echocardiographic data included ejection fraction, displacement, tissue Doppler imaging (TDI) s' wave, global longitudinal strain, and strain rate (SR) s' wave. Diastolic echocardiographic data included mitral E and A waves, TDI e' and a' waves, and SRe' and SRa' waves. E/TDI e', TDI e'/TDI a', E/SRe', and SRe'/SRa' ratios were also calculated. Catheterization dP/dt was used as a marker for systolic function, and LV end-diastolic pressure (EDP) was used as a marker for diastolic function. Correlations of the echocardiographic and catheterization values were performed using Pearson correlation. Twenty-nine patients were included (14 females, 15 males). Median age at catheterization was 3.4 years (0.04-17.4 years). dP/dt was 1258 ± 353 mmHg/s, and LVEDP was 10.8 ± 2.4 mmHg. There were no significant correlations between catheterization dP/dt and systolic echocardiographic parameters. LVEDP correlated significantly with SRe' (r = -0.4, p = 0.03), SRa' (r = -0.4, p = 0.03), and E/SRe' (r = 0.5, p = 0.004). In pediatric congenital heart patients, catheterization dP/dt did not correlate with echocardiographic measurements of LV systolic function. Further studies are needed to determine which echocardiographic parameter best describes LV systolic function in this population. Strain rate analysis significantly correlated with LVEDP. Strain rate analysis should be considered as an

  20. Congenital choledochal cysts in adults: twenty-five-year experience

    Institute of Scientific and Technical Information of China (English)

    LIU Ying-bin; CAO Li-ping; PENG Shu-you; WANG Jian-wei; Khagendra Raj Devkota; JI Zhen-ling; LI Jiang-tao; WANG Xu-an; MA Xiao-ming; CAI Wei-long; KONG Ying

    2007-01-01

    Background Choledochal cyst is rare in western countries. The relatively high incidence of coexistent hepatobiliary disease increases the difficulty of the surgical management of choledochal cyst. Here we analyze the diagnosis and treatment of congenital bile duct cyst in 122 Chinese adults.Methods The clinical data of 122 patients with congenital choledochal cysts admitted from 1981 to 2006 were analyzed.Results Clinical symptoms in most cases were nonspecific, resulting in delayed diagnosis. Sixty-one patients (50%) had coexistent pancreatobiliary disease. Among the 122 patients, 119 patients underwent ultrasonic examination;ERCP/MRCP was performed in 63 cases and CT in 102 cases. Abnormal pancreatobiliary duct junction was found in 48 patients. Sixteen patients had malignant lesions in the bile duct, arising in 11 of them from incomplete choledochal cyst that underwent various operations including cystenterostomy or cystojejunostomy. There was significant difference between the patients who underwent incomplete cyst resection and complete cyst resection in malignancy rate of bile duct (Chi square test, P= 0.000; odds ratio, 7.800; 95 % confidence interval, 2.450 to 24.836).Conclusions ERCP, CT and MRCP had proved their great values in the classification of the disease. Cyst excision with Roux-en-Y hepaticojejunostomy is recommended as the treatment of choice for patients with type Ⅰ or type Ⅳ cysts. For type Ⅴ cyst (Caroli's disease) with recurrent cholangitis, liver transplantation should be considered.

  1. Whole heart cine MR imaging of pulmonary veins in patients with congenital heart disease. Comparison with Spin Echo MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Mitsui, Hideaki [Yamagata City Hospital Saiseikan (Japan); Saito, Haruo; Ishibashi, Tadashi; Takahashi, Shoki; Zuguchi, Masayuki; Yamada, Shogo

    2002-01-01

    We evaluated the accuracy of Whole Heart Cine (WHC) magnetic resonance (MR) imaging in the depiction of pulmonary veins (PVs) in patients with congenital heart disease (CHD) compared to that of spin echo (SE) MR imaging. Among our 35 patients, 4 patients had anomalous PV return. Detectability of four PVs on each MR examination images were evaluated. MR imaging is an effective modality for the clarification of PVs, and WHC MR imaging is more useful in delineating PV anomalies than SE MR imaging. (author)

  2. Mountain climbing of the grown-up patient with non-corrected congenital heart defect.

    Science.gov (United States)

    Haponiuk, Ireneusz; Gierat-Haponiuk, Katarzyna; Szalewska, Dominika; Niedoszytko, Piotr; Bakuła, Stanisław; Chojnicki, Maciej

    2016-03-01

    Congenital heart defects (CHD) are the cause of reduced physical performance. The presence of congenital abnormalities in the heart of grown-up patients contributes to excessive hypo-kinesia. We present endurance parameters and a personalized comprehensive cardiac rehabilitation program before an extreme mountain climbing of a 27-year-old patient with an uncorrected ventricular septal defect (VSD). A 26-year-old female patient with an uncorrected congenital VSD was admitted to the department of cardiac rehabilitation before the planned high-mountain expedition. Professional preparation and assessment of actual exercise capacity was performed before scheduled extreme climbing. We conclude that physical activity associated with a heavy load in people with uncorrected CHD who have not developed pulmonary hypertension and reverse right-to-left flow seems to be safe, while participation of grown-up patients with congenital heart disease (GUCH) in extreme mountain climbing requires special preparation, individually designed endurance training and education program, conducted by the team of professionals in specialist centers. PMID:27212986

  3. The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2016 Update on Research.

    Science.gov (United States)

    Jacobs, Marshall L; Jacobs, Jeffrey P; Pasquali, Sara K; Hill, Kevin D; Hornik, Christoph; O'Brien, Sean M; Shahian, David M; Habib, Robert H; Edwards, Fred H

    2016-09-01

    The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) is the largest congenital and pediatric cardiac surgical clinical data registry in the world. With more than 400,000 total operations from nearly all centers performing pediatric and congenital heart operations in North America, the STS CHSD is an unparalleled platform for clinical investigation, outcomes research, and quality improvement activities in this subspecialty. In 2015, several major original publications reported analyses of data in the CHSD pertaining to specific diagnostic and procedural groups, age-defined cohorts, or the entire population of patients in the database. Additional publications reported the most recent development, evaluation, and application of metrics for quality measurement and reporting of pediatric and congenital heart operation outcomes. This use of the STS CHSD for outcomes research and for quality measurement continues to expand as database participation and the available wealth of data in it continue to grow. This article reviews outcomes research and quality improvement articles published in 2015 based on STS CHSD data. PMID:27492669

  4. The Society of Thoracic Surgeons Congenital Heart Surgery Database: 2016 Update on Research.

    Science.gov (United States)

    Jacobs, Marshall L; Jacobs, Jeffrey P; Pasquali, Sara K; Hill, Kevin D; Hornik, Christoph; O'Brien, Sean M; Shahian, David M; Habib, Robert H; Edwards, Fred H

    2016-09-01

    The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) is the largest congenital and pediatric cardiac surgical clinical data registry in the world. With more than 400,000 total operations from nearly all centers performing pediatric and congenital heart operations in North America, the STS CHSD is an unparalleled platform for clinical investigation, outcomes research, and quality improvement activities in this subspecialty. In 2015, several major original publications reported analyses of data in the CHSD pertaining to specific diagnostic and procedural groups, age-defined cohorts, or the entire population of patients in the database. Additional publications reported the most recent development, evaluation, and application of metrics for quality measurement and reporting of pediatric and congenital heart operation outcomes. This use of the STS CHSD for outcomes research and for quality measurement continues to expand as database participation and the available wealth of data in it continue to grow. This article reviews outcomes research and quality improvement articles published in 2015 based on STS CHSD data.

  5. Rare copy number variants contribute to congenital left-sided heart disease.

    Directory of Open Access Journals (Sweden)

    Marc-Phillip Hitz

    2012-09-01

    Full Text Available Left-sided congenital heart disease (CHD encompasses a spectrum of malformations that range from bicuspid aortic valve to hypoplastic left heart syndrome. It contributes significantly to infant mortality and has serious implications in adult cardiology. Although left-sided CHD is known to be highly heritable, the underlying genetic determinants are largely unidentified. In this study, we sought to determine the impact of structural genomic variation on left-sided CHD and compared multiplex families (464 individuals with 174 affecteds (37.5% in 59 multiplex families and 8 trios to 1,582 well-phenotyped controls. 73 unique inherited or de novo CNVs in 54 individuals were identified in the left-sided CHD cohort. After stringent filtering, our gene inventory reveals 25 new candidates for LS-CHD pathogenesis, such as SMC1A, MFAP4, and CTHRC1, and overlaps with several known syndromic loci. Conservative estimation examining the overlap of the prioritized gene content with CNVs present only in affected individuals in our cohort implies a strong effect for unique CNVs in at least 10% of left-sided CHD cases. Enrichment testing of gene content in all identified CNVs showed a significant association with angiogenesis. In this first family-based CNV study of left-sided CHD, we found that both co-segregating and de novo events associate with disease in a complex fashion at structural genomic level. Often viewed as an anatomically circumscript disease, a subset of left-sided CHD may in fact reflect more general genetic perturbations of angiogenesis and/or vascular biology.

  6. Anesthetic management of a 2-day-old with complete congenital heart block

    Directory of Open Access Journals (Sweden)

    Puneet Khanna

    2014-01-01

    Full Text Available Maternal connective tissue disorders such as Systemic Lupus Erythematosus (most common, Sjogren′s syndrome, mixed connective tissue disorders may lead to the rare condition of complete congenital heart block in the neonate. Rare fetal syndromes such as myocarditis, 18p syndrome, mucopolysaccharidoses and mitochondrial diseases are other causes. The mortality rate of this condition is inversely propotional to the age of presentation being 6 % in the neonatal age group. As the cardiac output in the neonate is heart rate dependent, it is crucial to maintain the heart rate in these patients. Pharamacological interventions with dopamine, isoprenaline, epinephrine and atropine are known for their variable response. Although permanent pacing is the most reliable mode of management, the access to it is often not readily available, especially in the developing countries. In such cases temporary pacing methods become lifesaving. Of all the modalities of temporary pacing (transcutaneous, transesophageal and transvenous transcutaneous pacing is the most readily available and immediate mode. In this case report we present a two day old neonate with isolated complete congenital heart block and a resting heart rate of 50-55/min in immediate need of palliative surgery for trachea-esophageal fistula (TEF. With pharmacological intervention the heart rate could only be raised to 75-80/min. The surgery was successfully carried out using transcutaneous pacing to maintain a heart rate of 100/min.

  7. Adults living with heart failure and fatigue

    DEFF Research Database (Denmark)

    Schjødt, Inge; Sommer, Irene; Bjerrum, Merete

    Background Fatigue is one of the most common symptoms reported by patients with heart failure (HF). Fatigue negatively impacts on patients’ everyday life, prognosis and quality of life. No specific cure or effective interventions to alleviate fatigue are available. Over the past decade, qualitative...... studies have been performed to develop more coherent and effective interventions to support self-care among heart failure patients experiencing fatigue. The findings of qualitative research should be synthesised to optimise nurses' understanding of fatigue and develop recommendations for practice. Aim...... To synthesise the best available evidence related to the lived experiences and management of fatigue in everyday life in adult patients with stable heart failure to develop effective interventions to support self-care. Specific questions on the patients’ lived experiences included: • How do patients with HF...

  8. Ultrasound findings in fetal congenital heart block associated with maternal anti-Ro/SSA and Anti-La/SSB antibodies.

    Science.gov (United States)

    Lai, Jasmine; Clark, Toshi J; Tan, Justin H; Delaney, Shani; Jolley, Jennifer A

    2015-03-01

    We present the sonographic features of a second-trimester fetus diagnosed with a bradyarrhythmia at 19 weeks' gestation. The mother carried a diagnosis of Sjögren syndrome, including the presence of SSA and SSB antibodies. Ultrasound M-mode and fetal echocardiogram revealed the etiology of the bradycardia to be a complete fetal congenital heart block, likely due to transplacental passage of autoimmune anti-Ro/SSA and anti-La/SSB antibodies. Consequential to the congenital heart block, the fetus developed hydrops fetalis at 21 weeks' gestational age. We discuss the 2 major etiologies of congenital heart block and the implications in subsequent pregnancies.

  9. Comparative study on cerebral injury after open heart surgery in patients with congenital and rheumatic heart disease

    Institute of Scientific and Technical Information of China (English)

    WANG Yong; XIAO Ying-bin; CHEN Lin; ZHONG Qian-jin; WANG Xue-feng

    2005-01-01

    Objective: To comparatively study the different effects of open heart surgery on brain tissues of patients with congenital and rheumatic heart disease. Methods: Forty patients with congenital heart disease (CHD, CHD group, n=20) or rheumatic heart disease (RHD, RHD group, n=20) underwent on-pump (cardiopulmonary bypass, CPB) heart-beating open heart surgery. Blood samples before CPB, and 20 minutes, 1 hour, 24 hours and 7 days after CPB were collected, and the levels of neuron-specific enolase (NSE) and protein S-100b in the plasma were determined with enzyme-linked immunoadsorbent assay (ELISA), respectively. All the patients were examined with electroencephalogram (EEG) before and 1 week after operation. The changes of NSE, S-100b and EEG compared to verify the difference of postoperative cerebral injury between CHD cases and RHD cases. Results: The plasma level of S-100b increased significantly 20 minutes after CPB and was still higher than the preoperative level at 24 hours after operation in both groups (P<0.01). The plasma level of NSE increased more significantly in the CHD group than in the RHD group 20 minutes after CPB and it returned to the normal level 24 hours after CPB in the CHD group but remained at a high level in the RHD group (P<0.01). The levels of NSE and S-100b returned to the normal levels on the 7th day after CPB. Abnormal EEG was found in 75% of the patients in the CHD group and 60% in the RHD group. Conclusions: On-pump heart-beating open heart surgery can cause certain cerebral injury in the patients with CHD or RHD. The injury was more severe and recovered more quickly in the CHD group than in the RHD group.

  10. A new plastic surgical technique for adult congenital webbed penis

    Institute of Scientific and Technical Information of China (English)

    Yue-bing CHEN; Xian-fan DING; Chong LUO; Shi-cheng YU; Yan-lan YU; Bi-de CHEN; Zhi-gen ZHANG; Gong-hui LI

    2012-01-01

    Objective:To introduce a novel surgical technique for correction of adult congenital webbed penis.Methods:From March 2010 to December 2011,12patients (age range:14-23 years old) were diagnosed as having a webbed penis and underwent a new surgical procedure designed by us.Results:All cases were treated successfully without severe complication.The operation time ranged from 20 min to 1 h.The average bleeding volume was less than 50 ml.All patients achieved satisfactory cosmetic results after surgery.The penile curvature disappeared in all cases and all patients remained well after 1 to 3 months of follow-up.Conclusions:Adult webbed penis with complaints of discomfort or psychological pressure due to a poor profile should be indicators for surgery.Good corrective surgery should expose the glans and coronal sulcus,match the penile skin length to the penile shaft length dorsally and ventrally,and provide a normal penoscrotal junction.Our new technique is a safe and effective method for the correction of adult webbed penis,which produces satisfactory results.

  11. Anatomical delineation of congenital heart disease using 3D magnetic resonance imaging

    Science.gov (United States)

    Adams Bornemeier, Renee; Fellows, Kenneth E.; Fogel, Mark A.; Weinberg, Paul M.

    1994-05-01

    Anatomic delineation of the heart and great vessels is a necessity when managing children with congenital heart disease. Spatial orientation of the vessels and chambers in the heart and the heart itself may be quite abnormal. Though magnetic resonance imaging provides a noninvasive means for determining the anatomy, the intricate interrelationships between many structures are difficult to conceptualize from a 2-D format. Taking the 2-D images and using a volumetric analysis package allows for a 3-D replica of the heart to be created. This model can then be used to view the anatomy and spatial arrangement of the cardiac structures. This information may be utilized by the physicians to assist in the clinical management of these children.

  12. Volume helical shuttle of high-definition CT in congenital heart disease: preliminary study

    International Nuclear Information System (INIS)

    Objective: To assess the clinical value of volume helical shuttle (VHS) of high definition CT (HDCT) in the diagnosis of congenital heart disease (CHD). Methods: Forty-three patients suffering from CHD were examined with VHS of HDCT and echocardiography (ECHO). Twenty-four patients (21 children, 3 adults) received surgery. Based on surgical data,the diagnostic accuracy of CHD by VHS of HDCT was compared with that by ECHO with respect to the intra-cardiac lesion, extra-cardiac lesion and hemodynamics. The visibility of the origin and proximal course of coronary arteries was also evaluated on VHS. CT effective doses were calculated,using mSv =DLP × K. Results: Thirty-six intra-cardiac deformities were found by operation, and the diagnostic accuracy of both VSH and ECHO were 100.0% (36/36). VHS successfully detected 42 of 44 (95.4%) cardiac-vascular connection and large vascular deformities, whereas ECHO only detected 26 (59.1% ). The right-to-left shunt and left-to-right shunt of septal defects revealed by VHS were 5 and 6 patients respectively, which all coincided with the results of ECHO.Fourteen bi-directional shunt of septal defects were detected by ECHO, and 13 of them were diagnosed as right-to-left shunt by HDCT except an illegibility in 1 patient. The origin and proximal course of coronary arteries were all visible on VHS. Effective dose for children was (3.38 ± 1.86) mSv, and for adults was (10.27 ± 2.59) mSv. Conclusions: VHS of HDCT scan is advantageous in the diagnosis of CHD. It can depict the intra- and extra- cardiac structures as well as coronary arteries and provide hemodynamic information. (authors)

  13. Altering hemodynamics leads to congenital heart defects (Conference Presentation)

    Science.gov (United States)

    Ford, Stephanie M.; McPheeters, Matthew T.; Wang, Yves T.; Gu, Shi; Doughman, Yong Qiu; Strainic, James P.; Rollins, Andrew M.; Watanabe, Michiko; Jenkins, Michael W.

    2016-03-01

    The role of hemodynamics in early heart development is poorly understood. In order to successfully assess the impact of hemodynamics on development, we need to monitor and perturb blood flow, and quantify the resultant effects on morphology. Here, we have utilized cardiac optical pacing to create regurgitant flow in embryonic hearts and OCT to quantify regurgitation percentage and resultant morphology. Embryonic quail in a shell-less culture were optically paced at 3 Hz (well above the intrinsic rate or 1.33-1.67 Hz) on day 2 of development (3-4 weeks human) for 5 minutes. The pacing fatigued the heart and led to a prolonged period (> 1 hour) of increased regurgitant flow. Embryos were kept alive until day 3 (cardiac looping - 4-5 weeks human) or day 8 (4 chambered heart - 8 weeks human) to quantify resultant morphologic changes with OCT. All paced embryos imaged at day 3 displayed cardiac defects. The extent of regurgitant flow immediately after pacing was correlated with cardiac cushion size 24-hours post pacing (p-value develop into abnormal valves and septa. Our model produces similar phenotypes as found in our fetal alcohol syndrome and velo-cardio-facial/DiGeorge syndrome models suggesting that hemodynamics plays a role in these syndromes as well. Utilizing OCT and optical pacing to understand hemodynamics in development is an important step towards determining CHD mechanisms and ultimately developing earlier treatments.

  14. Neurological outcome in isolated congenital heart block and hydrops fetalis

    NARCIS (Netherlands)

    Breur, Johannes M. P. J.; Gooskens, Rob H. J. M.; Kapusta, Livia; Stoutenbeek, Philip; Visser, Gerard H. A.; van den Berg, Paul; Meijboom, Erik J.

    2007-01-01

    Objective: Isolated fetal heart block ( HB), a condition associated with fetal hydrops, carries a high mortality rate and may result in neurodevelopmental sequelae. To the best of our knowledge, no data exist regarding the long- term outcome of such hydropic fetuses. We reviewed our experience with

  15. Rare combination of congenital heart disease and pulmonary alveolar proteinosis.

    Science.gov (United States)

    Tanaka, Yuki; Miyamoto, Takashi; Yoshitake, Shuichi; Naito, Yuji; Kobayashi, Tomio

    2015-10-01

    Here, we describe a case of total anomalous pulmonary venous return with coarctation of the aorta that was diagnosed as pulmonary alveolar proteinosis at autopsy in a male infant. Surgical repair was performed at 1 day of age, but the infant died on postoperative day 51 due to respiratory insufficiency without any evidence of pulmonary venous obstruction. He had been unexpectedly diagnosed with pulmonary alveolar proteinosis and pulmonary hypoplasia on autopsy. Congenital pulmonary alveolar proteinosis is a serious condition with a high mortality rate, which should be considered in the differential diagnosis in patients with a clinical picture of pulmonary venous obstruction, because most patients are unable to survive without proper treatment. In this report, we address specific issues that should be discussed in such cases based on our recent experience. PMID:26310609

  16. Zdravstvena nega novorojenca s prirojeno srčno napako: Nursing care of a new-born infant with congenital heart disease:

    OpenAIRE

    Urh, Irma

    1999-01-01

    The article presents nursing care of a new-borninfant with congenital heart disease based on the model of Virginia Henderson and the theory of needs of Abraham G. Maslow. Only those life activities which belong to the very top of the hierarchy of needs of a new-born infant with congenital heart disease, arepresented. Congenital heart disease often causes breathing difficulties manifested with dispnea. An early symptom of heart failure is liquid retentionwhich represents a decline in health st...

  17. Eponymous cardiovascular surgeries for congenital heart diseases--imaging review and historical perspectives.

    Science.gov (United States)

    Buethe, Ji; Ashwath, Ravi C; Rajiah, Prabhakar

    2015-01-01

    Advances in pediatric cardiology and cardiac surgical techniques over the past few decades have revolutionized the management of the patients with congenital heart disease, and many now survive into adulthood. Several eponymous surgical procedures performed for congenital heart disease have been named after eminent surgeons. In this article, we provide a short biography of the surgeons associated with these eponymous surgical procedures along with their other important scientific contributions. This is followed by a review of these surgical procedures and their most common complications. Imaging appearances of these surgical procedures along with common complications are described and illustrated, with particular emphasis on magnetic resonance imaging. The surgical procedures described in this review include Blalock-Taussig, Potts, Waterston, Glenn, Fontan, Kawashima, Norwood, Sano, Damus-Kaye-Stansel, Mustard, Senning, Jatene, LeCompte, Rastelli, Rashkind, Ross, and Waldenhausen.

  18. 1H-MRS study of brain metabolic disorder in patients with cyanosed congenital heart disease

    International Nuclear Information System (INIS)

    Objective: To study the metabolic alteration in the brain of patients with cyanosed congenital heart disease (CCHD) by using 1H-magnetic resonance spectroscopy (1H-MRS) and discover the pathophysiology of chronic hypoxic brain, which will help to diagnose and treat this disease completely. Methods: Twenty-five patients with CCHD and 25 controls were performed PRESS 1H-MRS and MRI. The areas under the resonance of metabolites were measured, the ratios of the other metabolites to Cr were calculated and compared. Results: In patients with CCHD, the mean value of NAA/Cr was significantly lower than that in controls (P 0.05). Conclusion: 1H-MRS can detect brain metabolic changes in patients with cyanosed congenital heart disease in vivo noninvasively and can detect the metabolism disorder of the energy and amino acid, so the pathophysiology of this disease can be understood

  19. 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......%) fulfilled the compliance criteria of using the eHealth application for at least 2 consecutive weeks. In the control group, 61 patients (79%) completed both exercise tests. Adjusted for baseline values, the difference between the intervention group and the control group in mean VO2 peak at 1year was -0.65ml...

  20. A Primer on Computational Simulation in Congenital Heart Disease for the Clinician

    CERN Document Server

    Clementel, Irene Vignon; Feinstein, Jeffrey A; 10.1016/j.ppedcard.2010.09.002

    2011-01-01

    Interest in the application of engineering methods to problems in congenital heart disease has gained increased popularity over the past decade. The use of computational simulation to examine common clinical problems including single ventricle physiology and the associated surgical approaches, the effects of pacemaker implantation on vascular occlusion, or delineation of the biomechanical effects of implanted medical devices is now routinely appearing in clinical journals within all pediatric cardiovascular subspecialties. In practice, such collaboration can only work if both communities understand each other's methods and their limitations. This paper is intended to facilitate this communication by presenting in the context of congenital heart disease (CHD) the main steps involved in performing computational simulation-from the selection of an appropriate clinical question/problem to understanding the computational results, and all of the "black boxes" in between. We examine the current state of the art and ...

  1. The development of a congenital heart programme quality dashboard to promote transparent reporting of outcomes.

    Science.gov (United States)

    Anand, Vijay; Cave, Dominic; McCrady, Heather; Al-Aklabi, Mohammed; Ross, David B; Rebeyka, Ivan M; Adatia, Ian

    2015-12-01

    In 2001, the Institute of Medicine identified healthcare transparency as a necessity for re-designing a quality healthcare system; however, despite widespread calls for publicly available transparent data, the goal remains elusive. The transparent reporting of outcome data and the results of congenital heart surgery is critical to inform patients and families who have both the wish and the ability to choose where care is provided. Indeed, in an era where data and means of communication of data have never been easier, the paucity of transparent data reporting is paradoxical. We describe the development of a quality dashboard used to inform staff, patients, and families about the outcomes of congenital heart surgery at the Stollery Children's Hospital. PMID:26675607

  2. Cor Triatriatum Sinistrum Presenting as Cyanotic Congenital Heart Disease A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Rajendra Gokhroo

    2015-03-01

    Full Text Available   Cor triatriatum is an acyanotic congenital heart disease. We present a rare case of cor triatriatum sinistrum in a 6-month-old female infant who was presented with cyanosis and failure to thrive. The 2D transthoracic echocardiography and the Doppler color flow imaging showed a proximal venous chamber communicating to the distal left atrium  through restrictive opening to the low-pressure, distal left atrial chamber. The Saline Contrast Echocardiography confirmed a right-to-left atrial shunt due to a minor atrial septal defect. The defect was caused by a persistent pulmonary hypertension which had raised the right atrial pressure in the infant. To the best of our knowledge, barely any such cases have been reported in the literature so far. Our report highlights the clinical utility of the Saline Contrast Echo in other cases of congenital heart diseases.

  3. Risk Factors for post-Cardiac Surgery Diaphragmatic Paralysis in Children with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Parvin Akbariasbagh

    2015-12-01

    Full Text Available Background: Injured phrenic nerve secondary to cardiac surgeries is the most common cause of diaphragmatic paralysis (DP in infants. The aim of this study was to determine the risk factors for DP caused by congenital heart defect corrective surgeries in pediatrics.Methods: This cross-sectional study, conducted in a 2-year period (2006–2008, included 451 children with congenital heart diseases admitted to the Pediatric Cardiac Surgery Ward of Imam Khomeini Hospital. The diaphragmatic function was examined via fluoroscopy, and the frequency of DP and its relevant parameters were evaluated.Results: Of the 451 patients, comprising 268 males and 183 females at an age range of 3 days to 204 months (28.2 ± 33.4 months, 25 (5.5% infants (60% male and 40% female, age range = 15 days to 132 months, 41.2 ± 28.1 months had DP as follows: 48% unilateral right-sided and 36% unilateral left-sided. Additionally, 68% had cyanotic congenital heart disease and 84% had DP following total correction surgery.  The highest prevalence rates of DP resulting in phrenic hemiparesis were observed after arterial switch operation, Fontan procedure, and Blalock–Taussig shunt surgery, respectively. Thirteen (52% of the 25 DP patients underwent surgical diaphragmatic plication because of severe respiratory distress and dependency on mechanical ventilation, and most of the cases of plication underwent arterial switch operation. The rate of mortality was 24% (6 patients.Conclusion: DP with a prevalence of 5.5% was one of the most common complications secondary to cardiac surgeries in the infants included in the present study. Effective factors were age, weight, cyanotic congenital heart defects, and previous cardiac surgery. Diaphragmatic plication improved prognosis in severe cases.

  4. Pulse oximetry screening: a review of diagnosing critical congenital heart disease in newborns

    OpenAIRE

    Engel MS; Kochilas LK

    2016-01-01

    Melissa S Engel,1 Lazaros K Kochilas2 1Division of Neonatology, University of Minnesota, Minneapolis, MN, 2Children’s Healthcare of Atlanta, Emory University, Atlanta, GA, USA Abstract: Congenital heart disease (CHD) is one of the most common birth defects, with an incidence of nine out of every 1,000 live births. The mortality of infants with CHD has decreased over the past 3 decades, but significant morbidity and mortality continue to occur if not diagnosed shortly after birth. Pulse...

  5. Pulse oximetry screening: a review of diagnosing critical congenital heart disease in newborns

    OpenAIRE

    Engel, Melissa

    2016-01-01

    Melissa S Engel,1 Lazaros K Kochilas2 1Division of Neonatology, University of Minnesota, Minneapolis, MN, 2Children’s Healthcare of Atlanta, Emory University, Atlanta, GA, USA Abstract: Congenital heart disease (CHD) is one of the most common birth defects, with an incidence of nine out of every 1,000 live births. The mortality of infants with CHD has decreased over the past 3 decades, but significant morbidity and mortality continue to occur if not diagnosed shortly after birth. P...

  6. The Prevalence of Celiac Disease in Down syndrome Children with and without Congenital Heart Defects

    OpenAIRE

    Noor Mohammad Noori; Alireza Teimouri; Maryam Nakhaey Moghaddam; Touran Shahraki

    2016-01-01

    Background The prevalence of celiac disease (CD) is remarkably varied in Down syndrome(DS)patientscompared with other diseases.  This study aimed to assess celiac disease prevalence in Down syndrome children with and without congenital heart defects (CHD) and its comparison with controls. Materials and Methods This case-control study was performed at a single center on 132 participants in three groups. Clinical and genetic tests were performed on all patients suspected with Down syndrome to c...

  7. Stem Cells and Progenitor Cells for Tissue-Engineered Solutions to Congenital Heart Defects

    OpenAIRE

    Yang Gao; Jacot, Jeffrey G.

    2015-01-01

    Synthetic patches and fixed grafts currently used in the repair of congenital heart defects are nonliving, noncontractile, and not electrically responsive, leading to increased risk of complication, reoperation, and sudden cardiac death. Studies suggest that tissue-engineered patches made from living, functional cells could grow with the patient, facilitate healing, and help recover cardiac function. In this paper, we review the research into possible sources of cardiomyocytes and other cardi...

  8. Methionine synthase reductase deficiency results in adverse reproductive outcomes and congenital heart defects in mice

    OpenAIRE

    Deng, Liyuan; Elmore, C. Lee; Lawrance, Andrea K.; Matthews, Rowena G.; Rozen, Rima

    2008-01-01

    Low dietary folate and polymorphisms in genes of folate metabolism can influence risk for pregnancy complications and birth defects. Methionine synthase reductase (MTRR) is required for activation of methionine synthase, a folate- and vitamin B12-dependent enzyme. A polymorphism in MTRR (p.I22M), present in the homozygous state in 25% of many populations, may increase risk for neural tube defects. To examine the impact of MTRR deficiency on early development and congenital heart defects, we u...

  9. The role of stents in the treatment of congenital heart disease: Current status and future perspectives

    OpenAIRE

    Peters Bjoern; Ewert Peter; Berger Felix

    2009-01-01

    Intravascular or intracardiac stenoses occur in many forms of congenital heart disease (CHD). Therefore, the implantation of stents has become an accepted interventional procedure for stenotic lesions in pediatric cardiology. Furthermore, stents are know to be used to exclude vessel aneurysm or to ensure patency of existing or newly created intracardiac communications. With the further refinement of the first generation of devices, a variety of “modern” stents with different design characteri...

  10. Procalcitonin as a biomarker of bacterial infection in pediatric patients after congenital heart surgery

    OpenAIRE

    Chakravarti, Sujata B; Diane A Reformina; Lee, Timothy M; Malhotra, Sunil P; Mosca, Ralph S; Puneet Bhatla

    2016-01-01

    Background: Bacterial infection (BI) after congenital heart surgery (CHS) is associated with increased morbidity and is difficult to differentiate from systemic inflammatory response syndrome caused by cardiopulmonary bypass (CPB). Procalcitonin (PCT) has emerged as a reliable biomarker of BI in various populations. Aim: To determine the optimal PCT threshold to identify BI among children suspected of having infection following CPB. Setting and Design: Single-center retrospective observationa...

  11. Prepregnancy diabetes and offspring risk of congenital heart disease - A nationwide cohort study

    OpenAIRE

    Øyen, Nina; 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 melli...

  12. Identifying improvements to complex pathways: evidence synthesis and stakeholder engagement in infant congenital heart disease

    OpenAIRE

    Crowe, Sonya; Knowles, Rachel; Wray, Jo; Tregay, Jenifer; Ridout, Deborah A; Utley, Martin; Franklin, Rodney; Bull, Catherine L; Brown, Katherine L

    2016-01-01

    Objectives Many infants die in the year following discharge from hospital after surgical or catheter intervention for congenital heart disease (3–5% of discharged infants). There is considerable variability in the provision of care and support in this period, and some families experience barriers to care. We aimed to identify ways to improve discharge and postdischarge care for this patient group. Design A systematic evidence synthesis aligned with a process of eliciting the perspectives of f...

  13. Intra-operative trans-esophageal echocardiography in congenital heart disease

    OpenAIRE

    Garg Rajnish; Murthy Keshava; Rao Shekhar; Muralidhar Kanchi

    2009-01-01

    Intra-operative trans-esophageal echocardiography (TEE) is an important monitoring and diagnostic tool used during surgery for repair of congenital heart disease. In several studies,TEE has been shown to provide additional intra-cardiac anatomic information. Its ability to be used intra- operatively before and after cardiac repair makes it a unique tool. Before TEE was available for intra-operative use, significant residual abnormalities were frequently not detected. The result was ofte...

  14. Universal Pulse Oximetry Screening for Early Detection of Critical Congenital Heart Disease

    OpenAIRE

    Praveen Kumar

    2016-01-01

    Critical congenital heart disease (CCHD) is a major cause of infant death and morbidity worldwide. An early diagnosis and timely intervention can significantly reduce the likelihood of an adverse outcome. However, studies from the United States and other developed countries have shown that as many as 30%–50% of infants with CCHD are discharged after birth without being identified. This diagnostic gap is likely to be even higher in low-resource countries. Several large randomized trials have s...

  15. Cor Triatriatum Sinistrum Presenting as Cyanotic Congenital Heart Disease A Rare Case Report

    OpenAIRE

    Rajendra Gokhroo; Bhanwar Ranwa; Kamal Kapoor; Ananthraj Avinash; Kumari Priti; Devendra Bisht; Sajal Gupta

    2015-01-01

      Cor triatriatum is an acyanotic congenital heart disease. We present a rare case of cor triatriatum sinistrum in a 6-month-old female infant who was presented with cyanosis and failure to thrive. The 2D transthoracic echocardiography and the Doppler color flow imaging showed a proximal venous chamber communicating to the distal left atrium  through restrictive opening to the low-pressure, distal left atrial chamber. The Saline Contrast Echocardiography confirmed a right-to-left atrial shunt...

  16. Treatment of Congenital Complete Atrioventricular Heart Block With Permanent Epicardial Pacemaker in Neonatal Lupus Syndrome

    OpenAIRE

    Tanriverdi, Sema; Ulger, Zulal; Siyah Bilgin, Betul; Kultursay, Nilgun; Yalaz, Mehmet; Atay, Yuksel; Koroglu, Ozge Altun

    2015-01-01

    Introduction: Neonatal lupus syndrome (NLS) is a passively acquired autoimmune condition due to the transplacental passage of maternal anti-Ro/SSA and anti-La/SSB antibodies in mothers with systemic lupus erythematosus (SLE), and congenital complete heart block (CHB) is its most serious manifestation. Skin and hepatic involvement may occur in later infancy. Case Presentation: A term infant with fetal bradycardia, detected at the 23rd gestational age, was diagnosed with CHB due to NLS and was ...

  17. Early therapeutic experience with the endothelin antagonist BQ-123 in pulmonary hypertension after congenital heart surgery

    OpenAIRE

    Prendergast, B; Newby, D; Wilson, L.; Webb, D; Mankad, P

    1999-01-01

    OBJECTIVE—To assess the effect of endothelin type A (ETA) receptor antagonism in infants with pulmonary hypertension following corrective surgery for congenital heart disease.
DESIGN—Open label, preliminary study.
SETTING—Tertiary paediatric cardiothoracic surgical centre.
PATIENTS—Three infants (aged 3 weeks, 7 weeks, and 8 months) with postoperative pulmonary hypertension unresponsive to conventional treatment, including inhaled nitric oxide.
INTERVENTIONS—Patients received incremental intr...

  18. Intestinal microbiota and blue baby syndrome: Probiotic therapy for term neonates with cyanotic congenital heart disease

    OpenAIRE

    Ellis, Collin L.; Rutledge, John C; Underwood, Mark A.

    2010-01-01

    Necrotizing enterocolitis (NEC) is the most common intestinal emergency among premature infants. Risk factors in premature infants include immature intestinal immunity and an intestinal microbiota dominated by hospital-acquired bacteria. Some probiotics have been shown to decrease the incidence of NEC in premature infants. Among term infants, NEC is rare. However, among term infants with cyanotic congenital heart disease (CCHD), the incidence of NEC is similar to that of premature infants but...

  19. De novo mutations in histone modifying genes in congenital heart disease

    OpenAIRE

    Zaidi, Samir; Choi, Murim; Wakimoto, Hiroko; Ma, Lijiang; Jiang, Jianming; Overton, John D; Romano-Adesman, Angela; Bjornson, Robert D.; Breitbart, Roger E.; Brown, Kerry K.; Carriero, Nicholas J.; Cheung, Yee Him; Deanfield, John; Depalma, Steve; Fakhro, Khalid A.

    2013-01-01

    Congenital heart disease (CHD) is the most frequent birth defect, affecting 0.8% of live births1. Many cases occur sporadically and impair reproductive fitness, suggesting a role for de novo mutations. By analysis of exome sequencing of parent-offspring trios, we compared the incidence of de novo mutations in 362 severe CHD cases and 264 controls. CHD cases showed a significant excess of protein-altering de novo mutations in genes expressed in the developing heart, with an odds ratio of 7.5 f...

  20. De novo mutations in histone modifying genes in congenital heart disease

    OpenAIRE

    Zaidi, Samir; Choi, Murim; Wakimoto, Hiroko; Ma, Lijiang; Jiang, Jianming; Overton, John D; Romano-Adesman, Angela; Bjornson, Robert D.; Breitbart, Roger E.; Brown, Kerry K.; Carriero, Nicholas J.; Cheung, Yee Him; Deanfield, John; Depalma, Steve; Fakhro, Khalid A.

    2013-01-01

    Congenital heart disease (CHD) is the most frequent birth defect, affecting 0.8% of live births 1 . Many cases occur sporadically and impair reproductive fitness, suggesting a role for de novo mutations. By analysis of exome sequencing of parent-offspring trios, we compared the incidence of de novo mutations in 362 severe CHD cases and 264 controls. CHD cases showed a significant excess of protein-altering de novo mutations in genes expressed in the developing heart, with an odds ratio of 7.5...

  1. Acute heart failure associated with congenital complete atrioventricular block due to neonatal lupus: case series report

    International Nuclear Information System (INIS)

    Neonatal lupus (NL) defines a set of clinical syndromes characterized by maternal autoantibodies against the RNA protein complex (Ro/SSA or La/SSB) that cross the placenta and potentially lead to fetal tissue damage. Little is known about other cardiac manifestations of NL different from congenital heart block (CHB), as heart failure (HF). Four cases of LN with BCC and acute HF at the first three days of life on average were reported. No complications or deaths were reported at 4,9 months of follow-up. All infants had anti-Ro high titles at the time of diagnosis.

  2. Congenital heart block with hydrops fetalis treated with high-dose dexamethasone; a case report.

    Science.gov (United States)

    Chua, S; Ostman-Smith, I; Sellers, S; Redman, C W

    1991-11-26

    A 32-year-old woman with systemic lupus erythematosus was found to have a fetus with heart block and fetal ascites at 23 weeks gestation. Treatment with high-dose corticosteroids ameliorated the early signs of heart failure, although the fetal heart rate gradually fell from 48 beats/min to 42 beats/min by 34 weeks. Sudden deterioration of the fetal state occurred at 35 weeks, and this only partially responded to digitalisation. Neonatal death occurred on Day 18 from the consequences of severe birth asphyxia. The relationship and pathogenesis of anti-Ro antibodies, congenital heart block and hydrops fetals are discussed, together with the in utero management of this condition. PMID:1765211

  3. Ophthalmic Evaluation of Children from the Tibet Plateau with Congenital Heart Disease

    Institute of Scientific and Technical Information of China (English)

    Guiqin Wang; Qian Shi; Lin Sun; Jing Wang; Lei Li; Tianchang Li; Wei Wang

    2014-01-01

    Purpose:.To perform ophthalmic examinations to evaluate the ocular characteristics of children living in the Tibet plateau and diagnosed with congenital heart disease.Methods:.Children with congenital heart disease underwent a conventional ocular examination including distant acuity test, slit-lamp examination,.fundus examination,.non-contact in-traocular pressure measurement,.measurement of corneal thickness, and fundus photography.Results: Forty-two Tibetan children, aged between 4 and 18 years and diagnosed with congenital heart disease,.were en-rolled in this study..The percentage of low visual acuity was 4.76%, mean intraocular pressure was (13.67±2.15) mmHg, average corneal thickness was (492.55±33.79) μm,.96.43%had an anterior chamber depth of 1 / 2 corneal thickness (CT), and 35.7% had an obvious fundus vascular tortuosity.Conclusion:.This study adds to the understanding of the ocu-lar status of the population living in the Tibet plateau, thereby offering clinical evidence for the prevention and treatment of eye diseases in this area. (Eye Science 2014; 29:134-137)

  4. Congenital tracheoesophagel fistula with no esophageal atresia (H-type) in adult (a case report)

    Energy Technology Data Exchange (ETDEWEB)

    Byun, W. M.; Jung, K. H.; Cho, K. H.; Kim, S. Y.; Hwang, M. S.; Chang, J. C.; Chung, M. K.; Lee, C. J. [Yeungnam University College of Medicine, Gyengsan (Korea, Republic of)

    1986-08-15

    Congenital tracheoesophageal fistula with no esophageal atresia is considered a rate type anomaly, especially in adult age. We report a case of isolated tracheoesophageal fistula with no esophageal atresia with review of literature.

  5. Adult congenital diaphragmatic hernia of the liver: a rare case report

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Congenital diaphragmatic hernia (CDH), which mainly occurs in the newborn or in childhood with severe respiratory distress and high mortality, is rarely found in adult, especially for those uncommon right CDH [1–4]. Whereas, liver as the main hernial

  6. Management of patients with pulmonary arterial hypertension due to congenital heart disease: recent advances and future directions.

    Science.gov (United States)

    Blok, Ilja M; van Riel, Annelieke C M J; Mulder, Barbara J M; Bouma, Berto J

    2015-12-01

    Pulmonary arterial hypertension is a serious complication of adult congenital heart disease associated with systemic-to-pulmonary shunts. Although early shunt closure restricts development of pulmonary arterial hypertension, patients remain at risk even after repair. The development of pulmonary arterial hypertension is associated with a markedly increased morbidity and mortality. It is important to identify patients with a poor prognosis using disease specific markers. Echocardiography and biomarkers arise as practical tools to determine the risk of mortality. Although pulmonary arterial hypertension cannot be cured, four classes of disease-targeting therapies are currently available and several promising therapies are being studied. There is a shift in drug studies towards more clinically relevant endpoints such as time to clinical worsening and morbidity and mortality events.

  7. [Optimization of postoperative medical therapy of infective endocarditis in patients with congenital valvular heart disease].

    Science.gov (United States)

    Chistyakov, I S; Medvedev, A P; Pichugin, V V

    2016-01-01

    The purpose of this study was to evaluate the effectiveness of combined surgical and medical treatment of infective endocarditis in patients with congenital valvular heart disease when included in a regimen of the drug Reamberin. In this regard, the analysis of the effectiveness of a combination regimen of 74 patients with valvular congenital heart diseases complicated with infective endocarditis. Given the indications for surgical correction operative technique features and possible technical difficulties in carrying out such operations, due to the inflammatory changes and tissue destruction, and ways to overcome them. For the correction of metabolic disorders in the postoperative period, 47 patients (main group) was appointed Reamberin: once, intravenous drip 400 ml/day during the first 5 days after surgery. 27 patients (control group) was conducted infusion therapy depending on the severity of the condition according to the classical scheme. In addition to standard clinical and laboratory examination, to assess the effectiveness of Reamberin was investigated catalase activity of CPK in blood serum in the dynamics of observation (1, 3 and 5 days after surgery). It is revealed that surgical approach, used in complex treatment of patients with valvular congenital heart diseases, including reorganization of the cavities of the heart, increasing the frequency of joints and the use of reinforcing strips of synthetic material that prevents the cutting of sutures through the inflamed tissue has achieved good short-and long-term results. Infective endocarditis and destruction of the valvular annulus fibrosus the use of a frame of strips of polytetrafluoroethylene allows you to restore its integrity and to implant a mechanical prosthesis. The inclusion in the regimen of patients with infective endocarditis complicated by cardiac insufficiency in the early postoperative period the drug Reamberin improves the efficiency of treatment by a more rapid restoration of the normal

  8. Exercise capacity in adult patients with congenitally corrected transposition of the great arteries

    OpenAIRE

    Fredriksen, P; Chen, A.; Veldtman, G; S Hechter; Therrien, J.; Webb, G.

    2001-01-01

    OBJECTIVE—To examine cardiopulmonary values, static lung function, and ejection fraction in adult patients with congenitally corrected transposition of the great arteries (CCTGA).
PATIENTS AND METHODS—41 patients who had undergone static lung function testing and cardiopulmonary exercise tests with measurements of ejection fraction were identified at the Toronto Congenital Cardiac Centre for Adults.
RESULTS—Aerobic capacity in patients with CCTGA was severely diminished, varying from 30-50% o...

  9. Congenitally corrected transposition in the adult: detection by radionuclide angiocardiography

    Energy Technology Data Exchange (ETDEWEB)

    Guit, G.L.; Kroon, H.M.; Chin, J.G.; Pauwels, E.K.; van Voorthuisen, A.E.

    1985-11-01

    Congenitally corrected transposition (CCT) of the great vessels is an uncommon anomaly usually detected in children because of associated severe cardiac malformations. When these are absent, patients may be hemodynamically normal, but associated cardiac abnormalities are usually present in CCT, leading to symptoms in adulthood. When CCT is suggested in an adult, diagnosis by means of ultrasound may be difficult. Radionuclide angiocardiography (RA) is a simple, noninvasive method by which to diagnose CCT. The authors found consistent morphologic scintigraphic results in 13 patients with proved CCT, leading to establishment of reliable diagnostic criteria. In all instances of situs solitus the aorta ascends and descends on the left with vertical orientation. In the case of situs inversus, the aorta ascends and descends on the right with vertical orientation. The authors criteria are independent of the situs and cardiac position, unlike earlier reports by others. They believe images obtained in the anterior projection are sufficient for the study. The practical application of RA study in patient diagnosis is demonstrated, giving special attention to patients referred because of situs solitus and dextrocardia, in which CCT is known to be present in 50% of cases.

  10. The alteration of interelemental ratios in myocardium under the congenital heart disease (SRXRF)

    Science.gov (United States)

    Trunova, V. A.; Zvereva, V. V.; Okuneva, G. N.; Levicheva, E. N.

    2007-05-01

    It is the myocardium that bears the basic functional loading during heart working, including muscle contractility and enzyme activity. The elemental concentrations in myocardium tissue of heart were determined by SRXRF technique. Our investigation is systematical: the elemental content in each compartment (left and right ventricles, left and right auricles) of hearts of healthy and diseased children (congenital heart diseases, transposition of main vessels (TMV)) was analyzed. The elemental distribution in myocardium of four heart chambers of human fetuses was also analyzed. Following elements were determined: S, Cl, K, Ca, Cr, Mn, Fe, Ni, Cu, Zn, As, Se, Br, Rb, Sr. It was revealed that the elemental concentrations in myocardium of both ventricles are almost constant in heart of fetuses and healthy children. The transition from pre-natal study (fetus) to post-natal study is accompanied by the redistribution of chemical elements in myocardium. The higher concentrations of S, Fe, Ca, Sr and Cu in myocardium of children are observed, the content of K, Br, Rb and especially Se is lower than in heart of fetuses. The elemental distribution in myocardium of children TMV is considerably different in comparison with the healthy children: the higher levels of Cu are observed. The content of Se is lower.

  11. Pancreatic hypoplasia presenting with neonatal diabetes mellitus in association with congenital heart defect and developmental delay.

    Science.gov (United States)

    Balasubramanian, M; Shield, J P H; Acerini, C L; Walker, J; Ellard, S; Marchand, M; Polak, M; Vaxillaire, M; Crolla, J A; Bunyan, D J; Mackay, D J G; Temple, I K

    2010-02-01

    Congenital pancreatic hypoplasia is a rare cause of neonatal diabetes. We report on a series of three patients with pancreatic agenesis and congenital heart defects. All had abdominal scan evidence of pancreatic agenesis. In addition, Patient 1 had a ventricular septal defect, patent ductus arteriosus and pulmonary artery stenosis; Patient 2 had a truncus arteriosus and Patient 3 had tetralogy of Fallot. Two of the three patients have developmental delay. All three patients were isolated cases within the family. Investigations included sequencing of GCK, ABCC8, IPF1, NEUROD1, PTF1A, HNF1B, INS, ISL1, NGN3, HHEX, G6PC2, TCF7L2, SOX4, FOXP3 (Patients 1 and 2), GATA4 and KCNJ11 genes (all three patients), but no mutations were found. Genetic investigation to exclude paternal UPD 6, methylation aberrations and duplications of 6q24 was also negative in all three. 22q11 deletion was excluded in all three patients. Array CGH in Patient (1) showed a approximately 250 kb, paternally inherited duplication of chromosome 12q [arr cgh 12q24.33 (B35:CHR12:131808577-132057649++) pat], not found in the other two patients. Permanent neonatal diabetes mellitus due to pancreatic hypoplasia with congenital heart defects has been reported before and may represent a distinct condition. We discuss this rare association and review previously reported literature. PMID:20082465

  12. PETER’S TYPE II ANOMALY ASSOCIATED WITH CONGENITAL HEART DEFECT: RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Darshan Kumar

    2015-03-01

    Full Text Available PURPOSE: Reporting a case of Peters’ type II with congenital heart defect a rare form of anterior segment dysgenesis in which abnormal cleavage of the anterior chamber occurs. It is characterized by central corneal opacification secondary to defects in a posterior corneal stroma, Descemet's membrane, and endothelium. Majority of cases are sporadic, bilateral , and associated with other systemic malformations. MATERIAL AND METHOD: A18 year old female presented with a history of bilateral cloudy corneas and diminution of vision since early child hood. Visual acuity was RE 6/60, LE 6/60 with PH improvement in B E 6/36 . Slit lamp examination showed BE micro cornea with central and paracentral macular grade opacity with lenticulo - irido - corneal adhesions. Iris was found to be fused with the posterior s urface of the peripheral cornea with poor formation of the anterior chamber with coloboma of iris. Lens was cataractous. Fundus examination in the left eye showed no abnormality. She was diagnosed as Peters’ type II anomaly. Systemic examination revealed congenital heart defects. Echocardiography showed congenital bicuspid aortic valve, grade 3 aortic regurgitation. RESULT: Patient require penetrating keratoplasty with combined procedure ( C ataract extraction with trabeculectomy. CONCLUSION: Early detectio n of the disease will help in early treatment, with good vision development to combat amblyopia.

  13. Heart Health Tests for Diabetes Patients

    Science.gov (United States)

    ... Heart Defects Symptoms & Diagnosis of Congenital Heart Defects Care & Treatment for Congenital Heart Defects Congenital Heart Defects Tools & Resources Diabetes About Diabetes Why Diabetes Matters Understand Your Risk ...

  14. The Comparison of Pulse Oximetry and Cardiac Catheterization in Managing the Treatment of Children with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    R Abbasi

    2015-03-01

    Full Text Available Bachground & aim: Pulse oximetry and cardiac catheterization are concerned in the treatment of children with congenital heart disease. Diagnosis of arterial oxygen saturation in patients with congenital heart disease (CHD can be used to assess and manage their effecacy. The purpose of this study was to compare pulse oximetry and cardiac catheterizations in treatment manage of children with congenital heart disease. Methods: In the present cross sectional study, 110 patients with cyanic and non syani heart disease were studied undergoing right and left heart catheterization by pulse oximetry of index finger and simultaneously, oxygen saturation was measured by cardiac catheterization. Data were analyzed with SPSS software by using Pearson correlation and linear regression. Results: A significant correlation was seen between arterial oxygen saturation measured by pulse oximetry and arterial oxygen saturation (p<0.0001 as well as heart rate, electrocardiogram and pulse oximetry (p<0.0001 respectively. Furthermore, the presence of cyanosis (p=0.001, digital clubbing of the fingers ((p=0.001, low oxygen saturation in the superior vena cava and right atrium (p=0.002 can reduce the accuracy of pulse oximetry for detection of arterial oxygen saturation. The mean right atrial pressure can effect on accuracy of pulse oximetry to detect heartbeat (p=0.034. Maximum sensitivity and specificity for detection of pulse oximetry oxygen saturation was 88 % and 88 heart rate per minute. Conclusion: Pulse oximetric is a useful tool for estimating the arterial oxygen saturation and heart rate in children with congenital heart disease (CHD and is a non-invasive method in comparison with cardiac catheterization. Key words: Pulse oximeter, Congenital Heart Disease, Cardiac Catheterization

  15. Anomalous origin of the left circumflex coronary artery from the pulmonary artery. A very rare congenital anomaly in an adult patient diagnosed by cardiovascular magnetic resonance

    OpenAIRE

    Giannitsis Evangelos; Ringwald Gerd; Korosoglou Grigorios; Katus Hugo A

    2008-01-01

    Abstract Here we report for the first time on the diagnostic potential of cardiovascular magnetic resonance (CMR) to delineate the proximal course of an anomalous left circumflex coronary artery (LCX) originating from the right pulmonary artery in an adult patient with no other form of congenital heart disease. The patient was referred to our institution due to exertional chest discomfort. X-Ray coronary angiography showed a normal left anterior descending coronary artery (LAD) and right coro...

  16. 67Ga imaging in the patients with infective endocarditis after surgery for congenital heart disease

    International Nuclear Information System (INIS)

    67Ga imaging was performed in sixteen patients (age: 8 m.-18 y.) who had persistent fever and positive acute phase reactants after surgery for congenital heart disease. Abnormal uptake of 67Ga over the heart and the lungs was evaluated with a computer. Abnormal uptake of 67Ga was observed in seven patients. Of them, three showed it in the area of peripheral pulmonary artery and the other four showed it in the area of artificial vessels for pulmonary artery reconstruction. In six patients with positive blood cultures, five showed abnormal uptake of 67Ga and in ten patients with negative blood cultures, two showed it. Vegetation was detected with 2D-echocardiography in four patients and all of them showed abnormal uptake of 67Ga, while in 12 patients without vegetation three showed it. In conclusion, 67Ga imaging was useful to detect the foci of infective endocarditis or pulmonary embolism caused by the vegetation in infective endocarditis in the patients after surgery for congenital heart disease, especially in the peripheral pulmonary arteries and artificial vessels which could not be detected with 2D-echo. (author)

  17. Cine magnetic resonance imaging for evaluation of cardiac structure and flow dynamics in congenital heart disease

    International Nuclear Information System (INIS)

    Cine magnetic resonance imaging (Cine MRI) was performed in 20 patients aged 19 days to 13 years (mean 4.0 years), who had congenital heart disease confirmed at echocardiography or angiography. Prior to cine MRI, gated MRI was performed to evaluate for cardiac structure. Cine MRI was demonstrated by fast low fip angle shot imaging technique with a 30deg flip angle, 15 msec echo time, 30-40 msec pulse repetition time, and 128 x 128 acquisition matrix. Abnormalities of cardiac structure were extremely well defined in all patients by gated MRI. Intracardiac or intravascular blood flow were visualized in 17 (85%) of 20 patients by cine MRI. Left to right shunt flow through ventricular septal defect, atrial septal defect, and endocardial cushion defect were visualized with low signal intensity area. Low intensity jets flow through the site of re-coarctation of the aorta were also visualized. However, the good recording of cine MRI was not obtained because of artifacts in 3 of 20 patients (15%) who had severe congestive heart failure or respiratory arrhythmia. Gated MRI provides excellent visualization of fine structure, and cine MRI can provide high spatial resolution imaging of flow dynamic in a variety of congenital heart disease, noninvasively. (author)

  18. sup 67 Ga imaging in the patients with infective endocarditis after surgery for congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Kohata, Tohru; Ono, Yasuo; Kamiya, Tetsuro; Nishimura, Tsunehiko; Takamiya, Makoto; Yagihara, Toshikatsu (National Cardiovascular Center, Suita, Osaka (Japan))

    1991-11-01

    {sup 67}Ga imaging was performed in sixteen patients (age: 8 m.-18 y.) who had persistent fever and positive acute phase reactants after surgery for congenital heart disease. Abnormal uptake of {sup 67}Ga over the heart and the lungs was evaluated with a computer. Abnormal uptake of {sup 67}Ga was observed in seven patients. Of them, three showed it in the area of peripheral pulmonary artery and the other four showed it in the area of artificial vessels for pulmonary artery reconstruction. In six patients with positive blood cultures, five showed abnormal uptake of {sup 67}Ga and in ten patients with negative blood cultures, two showed it. Vegetation was detected with 2D-echocardiography in four patients and all of them showed abnormal uptake of {sup 67}Ga, while in 12 patients without vegetation three showed it. In conclusion, {sup 67}Ga imaging was useful to detect the foci of infective endocarditis or pulmonary embolism caused by the vegetation in infective endocarditis in the patients after surgery for congenital heart disease, especially in the peripheral pulmonary arteries and artificial vessels which could not be detected with 2D-echo. (author).

  19. Cardiovascular and Neonatal Outcomes in Pregnant Women With High-Risk Congenital Heart Disease.

    Science.gov (United States)

    Pillutla, Priya; Nguyen, Tina; Markovic, Daniela; Canobbio, Mary; Koos, Brian J; Aboulhosn, Jamil A

    2016-05-15

    Congenital heart disease (CHD) increases the risk of adverse maternal and neonatal outcomes. However, previous studies have included mainly women with low-risk features. A single-center, retrospective analysis of pregnant women with CHD was performed. Inclusion criteria were the following high-risk congenital lesions and co-morbidities: maternal cyanosis; New York Heart Association (NHYA) functional class >II; severe ventricular dysfunction; maternal arrhythmia, single ventricle (SV) physiology, severe left-sided heart obstruction and severe pulmonary arterial hypertension. Multivariate analyses for predictors of adverse maternal cardiovascular and neonatal outcomes were performed. Forty-three women reported 61 pregnancies. There were no maternal or neonatal deaths. Maternal cardiac (31%) and neonatal (54%) complications were frequent. The most frequent cardiac events were pulmonary edema, arrhythmia, and reduced NYHA class. Previous arrhythmia conferred a 12-fold increase in the odds of experiencing at least one major cardiac complication. Maternal SV physiology was an independent risk factor for low birth weight, risk of neonatal intensive care unit admission and lower gestational age. Maternal cyanosis and severe pulmonary arterial hypertension also predicted adverse neonatal outcomes. In conclusion, mothers without antepartum arrhythmia or functional incapacity are unlikely to experience arrhythmias or a decrease in NYHA class during pregnancy. In addition, SV physiology is a robust predictor of neonatal complications. Antepartum counseling and assessment of maternal fitness are crucial for the woman with CHD. PMID:27055756

  20. 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

  1. The value of flat-detector computed tomography during catheterisation of congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Gloeckler, Martin [University Hospital Erlangen, Department of Pediatric Cardiology, Erlangen (Germany); Friedrich-Alexander University Erlangen-Nuernberg, Department of Pediatric Cardiology, Erlangen (Germany); Koch, Andreas; Greim, Verena; Shabaiek, Amira; Dittrich, Sven [University Hospital Erlangen, Department of Pediatric Cardiology, Erlangen (Germany); Rueffer, Andre; Cesnjevar, Robert [University Hospital Erlangen, Department of Congenital Heart Surgery, Erlangen (Germany); Achenbach, Stephan [University Hospital Erlangen, Department of Cardiology, Erlangen (Germany)

    2011-12-15

    To analyse the diagnostic utility of flat-detector computed tomography imaging (FD-CT) in patients with congenital heart disease, including the value of image fusion to overlay three-dimensional (3D) reconstructions on fluoroscopic images during catheter-based interventions. We retrospectively analysed 62 consecutive paediatric patients in whom FD-CT was used during catheterisation of congenital heart disease. Expert operators rated the clinical value of FD-CT over conventional fluoroscopic imaging. Added radiation exposure and contrast medium volume were evaluated. During a 12-month period, FD-CT was performed in 62 out of 303 cardiac catheterisations. Median patient age was 3.5 years. In 32/62 cases, FD-CT was used for diagnostic purposes, in 30/62 cases it was used in the context of interventions. Diagnostic utility was never rated as ''misleading''. It was classified as ''not useful'' in six cases (9.7%), ''useful'' in 18 cases (29.0%), ''very useful'' in 37 cases (59.7%) and ''essential'' in one case (1.6%). The median added dose-area product was 111.0 {mu}Gym{sup 2}, the required additional quantity of contrast medium was 1.6 ml/kg. FD-CT provides useful diagnostic information in most of the patients investigated for congenital heart disease. The added radiation exposure and contrast medium volume are reasonable. (orig.)

  2. EARLY DETECTION OF CRITICAL CONGENITAL HEART DISEASE IN NEW BORNS USING PULSE OXIMETRY SCREENING

    Directory of Open Access Journals (Sweden)

    ShahForum,Chatterjee Rajib, PatelPrashant C, Kunkulol Rahul R

    2015-01-01

    Full Text Available Background: Congenital heart diseases which are dependent on the ductusarteriosus to maintain adequate oxygenation or systemic blood flow are termed as a critical congenital heart disease (CCHD. Delay in the diagnosis of CCHD is the major cause leading to morbidity and mortality in newborn infants. Clinical evaluation is likely to miss the diagnosis in first few hours of hospital stay after birth due to absence of signs and symptoms of CCHD. In the absence of clinical findings during early neonatal period, the best parameter that can be assessed is the detection of hypoxemia by pulse oximetry screening. Objectives: To record the value of Pulse Oximetry within 24 hours of birth and evaluate Pulse Oximetry as screening tool for early diagnosis of CCHD. &Methods: Longitudinal descriptive study was conducted on total 700 intramural neonates, satisfying the inclusion and exclusion criteria, who were evaluated within 24 hours of birth with currently available pulse oximeter, after the Institutional Ethical Committee approval. The study was conducted over a period of 4 months. Part-A: Neonatal Case Record, Part-B: Pulse Oximetry Screening Record, Part-C: Clinical Examination Record, Part-D: Echocardiography Record. Results and Conclusion: Total 700 neonates were screened by pulse oximeter with consecutive sampling method. 4 (0.57% subjects were detected to have positive screen and the diagnosis was confirmed by echocardiography. Study revealed that Pulse Oximetry Screening can be an important primary screening tool in routine neonatal care for early detection of Critical Congenital Heart Diseases particularly in rural setup

  3. Case series: Dexmedetomidine and ketamine for anesthesia in patients with uncorrected congenital cyanotic heart disease presenting for non-cardiac surgery

    Directory of Open Access Journals (Sweden)

    Rakhee Goyal

    2013-01-01

    Full Text Available The number of patients with uncorrected congenital cyanotic heart disease is less but at times some may present for non-cardiac surgery with a high anesthetic risk. Some of these may even be adults with compromised cardiopulmonary physiology posing greater challenges to the anesthesiologist. The authors have used a combination of dexmedetomidine and ketamine for anesthesia for non cardiac surgery in five patients with cyanotic heart disease and right to left shunt (3-Eisenmenger′s syndrome, 2-Tetralogy of Fallot. The sympathoinhibitory effects of dexmedetomidine were balanced with the cardiostimulatory effects of ketamine, thereby maintaining good cardiovascular stability. The analgesia was good and there was no postoperative agitation.This drug combination was effective and safe for patients with cyanotic heart disease for non cardiac surgeries.

  4. Congenital tracheoesophageal fistula: A rare and late presentation in adult patient

    Directory of Open Access Journals (Sweden)

    Waseem M Hajjar

    2012-01-01

    Full Text Available Congenital H-type tracheoesophageal fistula (TEF in adults is a rare presentation and can test the diagnostic acumen of a surgeon, endoscopist, and the radiologist. These undetected fistulas may present as chronic lung disease of unknown origin because repeated aspirations can lead to recurrent lung infections and bronchiectasis. Congenital TEFs should be considered in the diagnosis of infants and young adults with recurrent respiratory distress and/or infections. Here, we present the successful management of this rare case in an adult patient.

  5. Developing and Evaluating Virtual Cardiotomy for Preoperative Planning in Congenital Heart Disease

    DEFF Research Database (Denmark)

    Sørensen, Thomas Sangild; Beerbaum, Philipp; Mosegaard, Jesper;

    2009-01-01

    Careful preoperative planning is of outmost importance -- in particular when considering complex corrective surgery on congenitally malformed hearts. As an aid to such decisionsmaking we describe a system for virtual reconstruction of patient-specific morphology from 3D-capable imaging modalities...... such as MRI and CT. We introduce and illustrate the concept of virtual cardiotomy as a new tool to preoperatively evaluate the feasibility of different surgical strategies by investigating the anatomical spatial relations through any number of virtual incisions. Ve review the technical and clinical...

  6. Oral-Dental Hygiene and Oral Microorganisms in Children With and Without Congenital Heart Disease

    OpenAIRE

    Panggabean, Emiwaty P.; Irsa, Lily; Anwar, Rosihan; Lubis, Munar; Rusdidjas; Pasaribu, Syahril

    2005-01-01

    Objective This study was done to compare the dental and oral hygiene status of children with and without congenital heart disease (CHD) by investigating oral microorganisms and dental and oral hygiene indices. Methods A cross-sectional study was done on children aged 2 to 15 years from April 2002 to December 2003 at the Department of Child Health, Haji Adam Malik Hospital, Medan. There were two subject groups, the first consisting of children with CHD and the second of those without. The d...

  7. Health-related fitness profiles in adolescents with complex congenital heart disease

    DEFF Research Database (Denmark)

    Klausen, Susanne Hwiid; Wetterslev, Jørn; Søndergaard, Lars;

    2015-01-01

    PURPOSE: This study investigates whether subgroups of different health-related fitness (HrF) profiles exist among girls and boys with complex congenital heart disease (ConHD) and how these are associated with lifestyle behaviors. METHODS: We measured the cardiorespiratory fitness, muscle strength...... in the Robust clusters reported leading a physically active lifestyle and participants in the Less robust cluster reported leading a sedentary lifestyle. Diagnoses were evenly distributed between clusters. CONCLUSIONS: The cluster analysis attributed some of the variability in cardiorespiratory fitness among...

  8. Identity dynamics and peer relationship quality in adolescents with a chronic disease: the sample case of congenital heart disease

    OpenAIRE

    Rassart, Jessica; Luyckx, Koen; Apers, Silke; Goossens, Eva; Moons, Philip

    2012-01-01

    Objective: Identity formation has been found to relate to psychosocial and disease-specific functioning in chronically ill adolescents. Therefore, examining antecedent factors of identity formation in this population is needed. The main goal of the present longitudinal study was to examine how peer relationship quality influenced identity formation in adolescents with congenital heart disease (CHD). Method: Adolescents with CHD were selected from the database of pediatric and congenital ...

  9. Heart Transplantation for Congenital Heart Disease in the First Year of Life

    OpenAIRE

    Chinnock, Richard E; Bailey, Leonard L.

    2011-01-01

    Successful infant heart transplantation has now been performed for over 25 years. Assessment of long term outcomes is now possible. We report clinical outcomes for322 patients who received their heart transplant during infancy. Actuarial graft survival for newborn recipients is 59% at 25 years. Survival has improved in the most recent era. Cardiac allograft vasculopathy is the most important late cause of death with an actuarial incidence at 25 years of 35%. Post-transplant lymphoma is estima...

  10. Profile and risk factors for congenital heart defects: A study in a tertiary care hospital

    Science.gov (United States)

    Abqari, Shaad; Gupta, Akash; Shahab, Tabassum; Rabbani, MU; Ali, S Manazir; Firdaus, Uzma

    2016-01-01

    Introduction: Congenital heart defects (CHDs) are an important cause of mortality and morbidity in children representing a major global health burden. It is thus important to determine their prevalence and spectrum and identify risk factors associated with the development of heart defects. Materials and Methods: A case-control study was carried out in the Department of Pediatrics and Center of Cardiology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India, from February 2014 to August 2015. All patients referred with complaints or clinical examination suggestive of CHDs were further evaluated with echocardiography. On Echocardiography, patients having CHDs were included as cases and those having a normal echocardiographic study were included as controls. Healthy controls were also included. 400 cases and 400 controls were thus identified; preterms having patent ductus arteriosus and patent foramen ovale and those with acquired heart defects were excluded. Risk factors among cases and controls were further studied. Results: Acyanotic heart defects were 290 (72.50%) of the total heart defects, whereas the contribution of cyanotic heart defects was 110 (27.50%). Out of all CHDs, ventricular septal defect was the most common lesion with contribution of 152 (38%) cases, whereas among the cyanotic heart defects, Tetralogy of Fallot was the most common lesion (18% of total cases). Out of the total 400 cases, 261 were males (65.25%). On univariate analysis, paternal age (odds ratio, OR, 2.01), bad obstetric history (OR, 2.65), antenatal febrile illness (OR, 4.12), and advanced maternal age (OR, 3.28) were found to increase the risk of CHD whereas intake of multivitamin (OR, 3.02) was found to be protective. The risk factors were further analyzed with multivariate logistic regression analysis and all the above factors were found to be significantly associated. Conclusion: We noted that the profile of CHD in our population was similar

  11. Heart MRI

    Science.gov (United States)

    Magnetic resonance imaging - cardiac; Magnetic resonance imaging - heart; Nuclear magnetic resonance - cardiac; NMR - cardiac; MRI of the heart; Cardiomyopathy - MRI; Heart failure - MRI; Congenital heart disease - MRI

  12. Nutrition Support for Children Undergoing Congenital Heart Surgeries: A Narrative Review.

    Science.gov (United States)

    Wong, Judith J M; Cheifetz, Ira M; Ong, Chengsi; Nakao, Masakazu; Lee, Jan Hau

    2015-07-01

    Energy imbalance in infants and children with congenital heart disease (CHD) is common and influenced by age, underlying cardiac diagnoses, and presence or absence of congestive heart failure. During the surgical hospitalization period, these children are prone to nutritional deterioration due to stress of surgery, anesthetic/perfusion techniques, and postoperative care. Poor nutrition is associated with increased perioperative morbidity and mortality. This review aims to examine various aspects of nutrition in critically ill children with CHD, including (1) energy expenditure, (2) perioperative factors that contribute to energy metabolism, (3) bedside practices that are potentially able to optimize nutrient delivery, and (4) medium- to long-term impact of energy balance on clinical outcomes. We propose a nutrition algorithm to optimize nutrition of these children in the perioperative period where improvements in nutrition status will likely impact surgical outcomes.

  13. Diaphragmatic palsy after cardiac surgical procedures in patients with congenital heart

    Directory of Open Access Journals (Sweden)

    Talwar Sachin

    2010-01-01

    Full Text Available Paralysis of diaphragm on one or, exceptionally, both sides is a common cause of delayed recovery and excessive morbidity following pediatric cardiac surgery. The consequences of this complication after all forms of congenital heart surgery in newborns and young infants can be potentially serious. The impact of diaphragmatic palsy on the physiology after single ventricle palliations is particularly significant. It is necessary for all professionals taking care of children with heart disease to be familiar with the etiology, diagnosis, and management of this condition. Early recognition and prompt management of diaphragmatic palsy can potentially reduce the duration of mechanical ventilation and intensive care in those who develop this complication. This review summarizes the anatomy of the phrenic nerves, reasons behind the occurrence of diaphragmatic palsy, and suggests practical guidelines for management.

  14. [Management of heart diseases in pregnancy: rheumatic and congenital heart disease, myocardial infarction and post partum cardiomyopathy].

    Science.gov (United States)

    Westhoff-Bleck, M; Hilfiker-Kleiner, D; Günter, H H; Schieffer, E; Drexler, H

    2008-07-01

    Heart disease is present in 0.5-1% of all pregnancies. It is the leading non-obstetric cause of maternal mortality accounting for about 10-15% of all maternal death. Over the last decades the underlying cardiac disease has changed. Also new therapeutic options have been developed. In western industrial countries the incidence of acquired rheumatic heart disease has declined. In contrast, as a result of neonatal corrective or palliative surgery, congenital heart disease has become an increasing and challenging problem. Maternal older age and the increase in women's smoking habits amplify the likelihood of coronary artery disease. Multiple therapeutic options including percutaneous interventions are available and novel therapeutic concepts are emerging i.e. for peripartum cardiomyopathy. Management of pregnancy, labor and delivery requires accurate diagnosis of the underlying cardiac disorder. Hemodynamic changes physiologically occurring during pregnancy have a different impact depending on the type and severity of cardiac anomalies. Management of these patients requires teamwork of obstetricians, neonatologists, cardiologists, anesthetists and sometimes cardiac surgeons.

  15. Genetic Modifiers Predisposing to Congenital Heart Disease in the Sensitized Down Syndrome Population

    Science.gov (United States)

    Li, Huiqing; Cherry, Sheila; Klinedinst, Donna; DeLeon, Valerie; Redig, Jennifer; Reshey, Benjamin; Chin, Michael T.; Sherman, Stephanie L.; Maslen, Cheryl L.; Reeves, Roger H.

    2012-01-01

    Background About half of people with Down syndrome (DS) exhibit some form of congenital heart disease (CHD). However, trisomy for human chromosome 21 (Hsa21) alone is insufficient to cause CHD as half of all people with DS have a normal heart, suggesting that genetic modifiers interact with dosage sensitive gene(s) on Hsa21 to result in CHD. We hypothesize that a threshold exists in both Down syndrome and euploid populations for the number of genetic perturbations that can be tolerated before CHD results. Methods and Results We ascertained a group of individuals with DS and complete atrioventricular septal defect (AVSD) and sequenced two candidate genes for CHD, CRELD1, which is associated with AVSD in people with or without DS, and HEY2, whose mouse ortholog produces septal defects when mutated. Several deleterious variants were identified but the frequency of these potential modifiers was low. We crossed mice with mutant forms of these potential modifiers to the Ts65Dn mouse model of Down syndrome. Crossing loss-of-function alleles of either Creld1 or Hey2 onto the trisomic background caused a significant increase in the frequency of CHD, demonstrating an interaction between the modifiers and trisomic genes. We showed further that although either of these mutant modifiers is benign by itself, they interact to affect heart development when inherited together. Conclusions Using mouse models of Down syndrome and of genes associated with congenital heart disease we demonstrate a biological basis for an interaction that supports a threshold hypothesis for additive effects of genetic modifiers in the sensitized trisomic population. PMID:22523272

  16. Chromosome 8p23.1 deletions as a cause of complex congenital heart defects and diaphragmatic hernia.

    Science.gov (United States)

    Wat, Margaret J; Shchelochkov, Oleg A; Holder, Ashley M; Breman, Amy M; Dagli, Aditi; Bacino, Carlos; Scaglia, Fernando; Zori, Roberto T; Cheung, Sau Wai; Scott, Daryl A; Kang, Sung-Hae Lee

    2009-08-01

    Recurrent interstitial deletion of a region of 8p23.1 flanked by the low copy repeats 8p-OR-REPD and 8p-OR-REPP is associated with a spectrum of anomalies that can include congenital heart malformations and congenital diaphragmatic hernia (CDH). Haploinsufficiency of GATA4 is thought to play a critical role in the development of these birth defects. We describe two individuals and a monozygotic twin pair discordant for anterior CDH all of whom have complex congenital heart defects caused by this recurrent interstitial deletion as demonstrated by array comparative genomic hybridization. To better define the genotype/phenotype relationships associated with alterations of genes on 8p23.1, we review the spectrum of congenital heart and diaphragmatic defects that have been reported in individuals with isolated GATA4 mutations and interstitial, terminal, and complex chromosomal rearrangements involving the 8p23.1 region. Our findings allow us to clearly define the CDH minimal deleted region on chromosome 8p23.1 and suggest that haploinsufficiency of other genes, in addition to GATA4, may play a role in the severe cardiac and diaphragmatic defects associated with 8p23.1 deletions. These findings also underscore the importance of conducting a careful cytogenetic/molecular analysis of the 8p23.1 region in all prenatal and postnatal cases involving congenital defects of the heart and/or diaphragm.

  17. Chromosome 8p23.1 Deletions as a Cause of Complex Congenital Heart Defects and Diaphragmatic Hernia

    Science.gov (United States)

    Wat, Margaret J.; Shchelochkov, Oleg A.; Holder, Ashley M.; Breman, Amy M.; Dagli, Aditi; Bacino, Carlos; Scaglia, Fernando; Zori, Roberto T.; Cheung, Sau Wai; Scott, Daryl A.; Kang, Sung-Hae Lee

    2009-01-01

    Recurrent interstitial deletion of a region of 8p23.1 flanked by the low copy repeats 8p-OR-REPD and 8p-OR-REPP is associated with a spectrum of anomalies that can include congenital heart malformations and congenital diaphragmatic hernia (CDH). Haploinsufficiency of GATA4 is thought to play a critical role in the development of these birth defects. We describe two individuals and a monozygotic twin pair discordant for anterior CDH all of whom have complex congenital heart defects caused by this recurrent interstitial deletion as demonstrated by array comparative genome hybridization. To better define the genotype/phenotype relationships associated with alterations of genes on 8p23.1, we review the spectrum of congenital heart and diaphragmatic defects that have been reported in individuals with isolated GATA4 mutations and interstitial, terminal, and complex chromosomal rearrangements involving the 8p23.1 region. Our findings allow us to clearly define the CDH minimal deleted region on chromosome 8p23.1 and suggest that haploinsufficiency of other genes, in addition to GATA4, may play a role in the severe cardiac and diaphragmatic defects associated with 8p23.1 deletions. These findings also underscore the importance of conducting a careful cytogenetic/molecular analysis of the 8p23.1 region in all prenatal and postnatal cases involving congenital defects of the heart and/or diaphragm. PMID:19606479

  18. Three-dimensional cine MRI in free-breathing infants and children with congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Seeger, Achim; Fenchel, Michael C.; Kramer, Ulrich; Bretschneider, Christiane; Doering, Joerg; Claussen, Claus D.; Miller, Stephan [University of Tuebingen (Germany). Department of Diagnostic and Interventional Radiology; Greil, Gerald F. [St. Thomas Hospital, Division of Imaging Sciences, King' s College London (United Kingdom); Martirosian, Petros [University of Tuebingen, Section of Experimental Radiology, Tuebingen (Germany); Sieverding, Ludger [University of Tuebingen, Department of Pediatric Cardiology, Tuebingen (Germany)

    2009-12-15

    Patients with congenital heart disease frequently have complex cardiac and vascular malformations requiring detailed non-invasive diagnostic evaluation including functional parameters. To evaluate the morphological and functional information provided by a novel 3-D cine steady-state free-precession (SSFP) sequence. Twenty consecutive children (mean age 2.2 years, nine boys) were examined using a 1.5-T MR system including 2-D cine gradient-recalled-echo sequences, static 3-D SSFP and 3-D cine SSFP sequences. Measurement of ventricular structures and volumes showed close agreement between the 3-D cine SSFP sequence and the 2-D cine gradient-recalled-echo and static 3-D SSFP sequences (left ventricular volumes mean difference 1.0-1.9 ml and 8.8-11.4%, respectively; right ventricular volumes 1.7-2.1 ml and 9.9-16.9%, respectively). No systematic bias was observed. 3-D cine MRI provides anatomic as well as functional information with sufficient spatial and temporal resolution in free-breathing infants with congenital heart disease. (orig.)

  19. Repeatability of cardiac-MRI-measured right ventricular size and function in congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Walsh, Rowan; Salem, Yishay [Mount Sinai School of Medicine, Division of Pediatric Cardiology, New York, NY (United States); Shah, Amee; Lai, Wyman W. [Morgan Stanley Children' s Hospital of New York Presbyterian, New York, NY (United States); Nielsen, James C. [Mount Sinai School of Medicine, Division of Pediatric Cardiology, New York, NY (United States); Mount Sinai Children' s Heart Center, Box 1201, New York, NY (United States)

    2011-08-15

    The measurement error for right ventricular (RV) size and function assessed by cardiac MRI (CMRI) in congenital heart disease has not been fully characterized. As CMRI parameters are being increasingly utilized to make clinical decisions, defining error in the clinical setting is critical. This investigation examines the repeatability of CMRI for RV size and function. Forty consecutive people with congenital heart disease involving the RV were retrospectively identified. Contouring of RV volumes was performed by two expert CMRI clinicians. The coefficient of variability and repeatability coefficients were calculated. Repeatability coefficients were multiplied by the mean value for each group studied to define a threshold beyond which measurement error was unlikely to be responsible. The variability for indexed RV end-diastolic volume = 3.2% and 3.3% for intra- and interobserver comparisons, respectively. The repeatability coefficients were 13.2% and 14.9% for intra- and interobserver comparisons, which yielded threshold values of 15.1 ml/m{sup 2} and 20.2 ml/m{sup 2}, respectively. For RV ejection fraction (EF), the repeatability coefficients for intra- and interobserver comparisons were 5.0% and 6.0%, which resulted in threshold values of 2.6 EF% and 3.0 EF%. The threshold values generated can be used during serial assessment of RV size and function. (orig.)

  20. Complete Congenital Heart Block in a Newborn Associated with Maternal Systemic Lupus Erythematosus: A Case Report

    Directory of Open Access Journals (Sweden)

    Mahmoud A. Kiblawi

    2013-12-01

    Full Text Available Background: Complete Congenital Heart Block (CCHB is a rare disease of the newborn that carries significant morbidity and mortality. CCHB can be diagnosed early or late in life. In newborns, it is usually associated with maternal autoantibodies or a congenital cardiac structural abnormality. The most common presentation of CCHB is bradycardia that can be diagnosed by an electrocardiogram. Results: This is a case report of a male infant born to a mother with an autoimmune disease, Systemic Lupus Erythematosus (SLE, who was found to have third degree heart block at birth. Conclusion: Early diagnosis and prompt management of the case is important for a better prognosis and prevention of associated complications. Neonates with CCHB should be managed at a tertiary care center and the only definite treatment is insertion of a pacemaker. Moreover, prenatal diagnosis and specific obstetric counseling of pregnant women with SLE along with careful monitoring with serial ultrasonography and echocardiography are of paramount importance in prevention of the disease in subsequent offspring.

  1. Intra-operative trans-esophageal echocardiography in congenital heart disease

    Directory of Open Access Journals (Sweden)

    Garg Rajnish

    2009-01-01

    Full Text Available Intra-operative trans-esophageal echocardiography (TEE is an important monitoring and diagnostic tool used during surgery for repair of congenital heart disease. In several studies,TEE has been shown to provide additional intra-cardiac anatomic information. Its ability to be used intra- operatively before and after cardiac repair makes it a unique tool. Before TEE was available for intra-operative use, significant residual abnormalities were frequently not detected. The result was often substantial post-operative morbidity and mortality and sometimes the need for re-operation. According to practice guidelines established by the Society of Cardiovascular Anesthesiologists and the American Society of Anesthesiologists, there is strong evidence for the usefulness of TEE in surgery for congenital heart disease because it significantly improves the clinical outcome of these patients. Before surgical correction, TEE helps confirm diagnosis and spot any additional lesion, while after the surgical correction, it provides baseline parameters for comparison after the surgical correction.

  2. Challenges in the management of congenital heart disease in Vietnam: A single center experience

    Directory of Open Access Journals (Sweden)

    Vu Minh Phuc

    2015-01-01

    Full Text Available Vietnam, in Asia, is a low middle-income country with a relatively large population to cater to. Not many know about Vietnam, or its healthcare sector especially the field of pediatric cardiology and congenital heart disease. In contrast to the developed world, congenital heart disease (CHD is not diagnosed early. Since most of the patients visit the hospital only in later stages of the disease there are many complications during the operation and post-operatively. But during the past 5 years (from 2009, there has been major improvement in the treatment of CHD, both by intervention and surgery. At present, all kinds of CHD, both simple and complex are being successfully treated in our country. Today in Vietnam, all children under 6 years of age have health insurance coverage, under which almost all operations and catheter interventions are done free in government hospitals. It is helping many patients, especially those from the poor socioeconomic background. However, the present infrastructure is inadequate and a long waiting list has accumulated for treatment of CHD.

  3. Challenges in the management of congenital heart disease in Vietnam: A single center experience

    International Nuclear Information System (INIS)

    Vietnam, in Asia, is a low middle-income country with a relatively large population to cater to. Not many know about Vietnam, or its healthcare sector especially the field of pediatric cardiology and congenital heart disease. In contrast to the developed world, congenital heart disease (CHD) is not diagnosed early. Since most of the patients visit the hospital only in later stages of the disease there are many complications during the operation and post-operatively. But during the past 5 years (from 2009), there has been major improvement in the treatment of CHD, both by intervention and surgery. At present, all kinds of CHD, both simple and complex are being successfully treated in our country. Today in Vietnam, all children under 6 years of age have health insurance coverage, under which almost all operations and catheter interventions are done free in government hospitals. It is helping many patients, especially those from the poor socioeconomic background. However, the present infrastructure is inadequate and a long waiting list has accumulated for treatment of CHD

  4. Growth Status of Iranian Children with Hemodynamically Important Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Mohammad Dalili

    2011-02-01

    Full Text Available The relationship between congenital heart disease (CHD and growth retardation is well documented. We investigated the growth condition of Iranian children with several types of congenital heart disease (CHD and compared it with worldwide researches. Growth condition was investigated in 469 patients with important CHD aged from 1 month to 18 years. The patients were divided into two groups; infants (aged 12 months or less, and children (1-18 yrs of age. Children with hemodynamically unimportant small VSDs or small ASDs were not studied. Other exclusion criteria were prematurity, known genetic disorders and neurologic disease affecting growthd. All patients' cardiac diagnoses were made on the basis of clinical and laboratory examinations; including electrocardiography, echocardiography, cardiac catheterization, and angiography. Body weight and height of all patients were measured using conventional methods and compared with standard growth charts. In all patients body weights and heights were significantly lower than normal population. This difference was greater in the weight of female children. Other risk factors for growth failure were large left-to-right intracardiac shunts, pulmonary hypertension and cyanosis. Iranian children with CHD have growth failure somewhat different from other countries. Lower body weights of cyanotic patients and female children indicated that these patients need more nutritional and psychosocial attention.

  5. The Application of an Anatomical Database for Fetal Congenital Heart Disease

    Institute of Scientific and Technical Information of China (English)

    Li Yang; Qiu-Yan Pei; Yun-Tao Li; Zhen-Juan Yang

    2015-01-01

    Background:Fetal congenital heart anomalies are the most common congenital anomalies in live births.Fetal echocardiography (FECG) is the only prenatal diagnostic approach used to detect fetal congenital heart disease (CHD).FECG is not widely used,and the antenatal diagnosis rate of CHD varies considerably.Thus,mastering the anatomical characteristics of different kinds of CHD is critical for ultrasound physicians to improve FECG technology.The aim of this study is to investigate the applications of a fetal CHD anatomic database in FECG teaching and training program.Methods:We evaluated 60 transverse section databases including 27 types of fetal CHD built in the Prenatal Diagnosis Center in Peking University People's Hospital.Each original database contained 400-700 cross-sectional digital images with a resolution of 3744 pixels × 5616 pixels.We imported the database into Amira 5.3.1 (Australia Visage Imaging Company,Australia) three-dimensional (3D) software.The database functions use a series of 3D software visual operations.The features of the fetal CHD anatomical database were analyzed to determine its applications in FECG continuing education and training.Results:The database was rebuilt using the 3D software.The original and rebuilt databases can be displayed dynamically,continuously,and synchronically and can be rotated at arbitrary angles.The sections from the dynamic displays and rotating angles are consistent with the sections in FECG.The database successfully reproduced the anatomic structures and spatial relationship features of different fetal CHDs.We established a fetal CHD anatomy training database and a standardized training database for FECG.Ultrasound physicians and students can learn the anatomical features of fetal CHD and FECG through either centralized training or distance education.Conclusions:The database of fetal CHD successfully reproduced the anatomic structures and spatial relationship of different kinds of fetal CHD.This database can be

  6. Mortality from Congenital Heart Disease in Mexico: A Problem on the Rise.

    Directory of Open Access Journals (Sweden)

    José Luis Torres-Cosme

    Full Text Available Temporal trends in mortality from congenital heart disease (CHD vary among regions. It is therefore necessary to study this problem in each country. In Mexico, congenital anomalies were responsible for 24% of infant mortality in 2013 and CHD represented 55% of total deaths from congenital anomalies among children under 1 year of age. The objectives of this study were to analyze the trends in infant mortality from CHD in Mexico (1998 to 2013, its specific causes, age at death and associated socio-demographic factors.Population-based study which calculated the compounded annual growth rate of death rom CHD between 1998 and 2013. Specific causes, age at which death from CHD occurred and risk factors associated with mortality were analyzed for the year 2013.Infant mortality from CHD increased 24.8% from 1998 to 2013 (114.4 to 146.4/ 100,000 live births. A total of 3,593 CHD deaths occurred in 2013; the main causes were CHD with left-to-right shunt (n = 487; 19.8/100,000 live births and cyanotic heart disease (n = 410; 16.7/100,000. A total of 1,049 (29.2% deaths from CHD occurred during the first week of life. Risk factors associated with mortality from CHD were, in order of magnitude: non-institutional birth, rural area, birth in a public hospital and male sex.Mortality from CHD has increased in Mexico. The main causes were CHD with left-to-right shunt, which are not necessarily fatal if treated promptly. Populations vulnerable to death from CHD were identified. Approximately one-third of the CHD occurred during the first week of life. It is important to promote early diagnosis, especially for non-institutional births.

  7. Bochdalek Congenital Diaphragmatic Hernia in an Adult Sheep

    Science.gov (United States)

    Williams, R. D.; Katz, M. G.; Fargnoli, A. S.; Kendle, A. P.; Mihalko, K. L.; Bridges, C. R.

    2016-01-01

    Summary Congenital diaphragmatic hernia (CDH) is a rare condition. The aetiology of CDH is often unclear. In our case, a hollow mass was noted on MRI. Cardiac ejection fraction was diminished (47.0%) compared to 60.5% (average of 10 other normal animals, P < 0.05). The final diagnosis of congenital diaphragmatic hernia (Bochdalek type) was made when the sheep underwent surgery. The hernia was right-sided and contained the abomasum. Lung biopsy demonstrated incomplete development with a low number of bronchopulmonary segments and vessels. The likely cause of this hernia was genetic malformation. PMID:26293994

  8. Heart Transplant

    Science.gov (United States)

    ... 2015 A heart transplant gives a patient with congenital heart disease the opportunity to have a normal heart with ... pulmonary artery and left atrium. In patients with congenital heart disease, the surgeon may simultaneous transplant the lungs and ...

  9. Assessment of congenital heart disease (CHD): Is there a role for fetal magnetic resonance imaging (MRI)?

    Energy Technology Data Exchange (ETDEWEB)

    Manganaro, L. [Department of Radiological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy); Savelli, S. [Department of Radiological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy)], E-mail: sarasavelli@hotmail.it; Di Maurizio, M.; Perrone, A.; Francioso, A.; La Barbera, L.; Totaro, P.; Fierro, F.; Tomei, A.; Coratella, F. [Department of Radiological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy); Giancotti, A. [Department of Gynaecological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy); Ballesio, L. [Department of Radiological Sciences, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy); Ventriglia, F. [Department of Pediatric Cardiology, UMBERTO I Hospital, SAPIENZA University of Rome, Viale del Policlinico 155, 00161 Rome (Italy)

    2009-10-15

    Purpose: To review our experience with fetal magnetic resonance imaging (MRI) to evaluate congenital heart disease (CHD). Methods: We performed fetal MRI in 32 fetuses with an echocardiographically assessed CHD. Both direct and indirect signs of CHD were investigated. Direct signs considered were: morpho-volumetric abnormalities of the heart; malrotations; ventricular and atrial septal defects; anomalies of the origin, size and course of the great arteries. Indirect signs considered were: difficulty to recognize a 'normal' anatomical structures in the reference projections; increase of the vascular size before a stenosis; hypertrophy of the papillary muscles; cardiomegaly and pericardial effusion. All MRI findings were compared with postnatal or autoptic findings. Results: MRI allowed the CHD to be visualised by direct signs in 17 fetuses, indirect signs in 5 and both direct and indirect signs in 9 fetuses, excluding the prenatal echocardiographic suspect of hypoplastic left heart syndrome in 1 fetus. Postnatal echocardiograms or autoptic findings confirmed a normal heart in 1 fetus and CHD in 31 fetuses including a single cardiac anomaly or syndrome in 19 fetuses, 2 associated cardiac abnormalities in 11 and 3 cardiac anomalies in 1 fetus. However, in 2 fetuses MRI detected a ventricular septal defect successively disclosed by gold standard. Conclusions: MRI is a promising method for further assessment of the cardiovascular pathologies diagnosed by echocardiography, and may be a valuable tool in assessing associated extracardiac anomalies.

  10. MRI of congenital heart disease in childhood; MR-Tomographie kongenitaler Herzvitien im Kindesalter

    Energy Technology Data Exchange (ETDEWEB)

    Weiss, F.; Habermann, C.R.; Adam, G. [Radiologisches Zentrum, Klinik und Poliklinik fuer Diagnostische und Interventionelle Radiologie, Universitaetsklinikum Hamburg-Eppendorf (Germany); Lilje, C.; Weil, J. [Klinik und Poliklinik fuer Kinder- und Jugendmedizin, Abt. Paediatrische Kardiologie, Universitaetsklinikum Hamburg-Eppendorf (Germany)

    2004-02-01

    MRI provides a non-invasive diagnostic tool complementing echocardiography on one hand, and showing advantages over echocardiography, on the other hand, especially after corrective procedures. The multiple different MRI sequences need to be adapted to examinations of children and patients with congenital heart disease (CHD), and can be used to detect morphologic changes, blood-flow in the heart and thoracic vessels and diastolic or systolic function of myocardium. Several factors determine the success of the examination of a complex congenital heart disease or a postoperative situs. Pediatric radiologists and radiologists experienced in congenital heart diseases have to work in close cooperation. Echocardiography should be performed before MRI. The results of prior examinations and the clinical history of the patients, including possible palliative or reconstructive operations, must be available before MRI to guide the planning of the examination. With a systematic segmental approach to the situs, to the atrial and ventricular morphology, and to the visceroatrial, atrioventricular and ventriculoarterial connections, most CHDs can be diagnosed correctly. With appropriate knowledge, MRI can also be performed quite accurately after complex operations and may avoid or delay invasive diagnostic procedures. The aim of this article is to impart knowledge, to mention problems and to provide guidance in the performance of cardiac MRI in patients with CHD. (orig.) [German] Die MRT bietet sich als nicht-invasive Methode zur Diagnostik kongenitaler Herzvitien im Kindesalter einerseits als Ergaenzung zur Echokardiographie an, andererseits ergeben sich auch Vorteile insbesondere nach Korrektureingriffen. Die verschiedenen MRT-Sequenzen muessen an die Untersuchung von Kindern adaptiert werden und koennen Aufschluss ueber die Morphologie, Blutfluesse im Herz und in den thorakalen Gefaessen und ueber die myokardiale Wandbewegung geben. Die sichere Durchfuehrung und Befundung der

  11. Heart Failure: Unique to Older Adults

    Science.gov (United States)

    ... provider may suggest other ways to help your body reduce the extra fluid. Depression and Heart Failure If you have chronic heart ... more often, tell your healthcare professional right away. Depression in ... and other chemicals in your body that can be damaging to your heart and ...

  12. The Burden of Care: Mothers’ Experiences of Children with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Sakinne Sabzevari

    2016-10-01

    Full Text Available Background: Mothers play a key role in caring for their sick children. Their experiences of care were influenced by culture, rules, and the system of health and care services. There are few studies on maternal care of children with congenital heart disease. Also, each of them has studied a particular aspect of care. The present research aimed to understand care experiences of mothers of children with congenital heart disease. Methods: A conventional content analysis was used to obtain rich data. The goal of content analysis is “to provide knowledge and deeper understanding of the phenomenon under the study”. The study was conducted in Kerman, Iran in 2014, on mothers of children with CHD. The purposive sampling technique was used to select the participants. Participants were 14 mothers of children with CHD and one father and one nurse of open heart surgery unit, from two hospitals affiliated with Kerman University of Medical Sciences. Eighteen semi-structured interviews were constructed. Data were analyzed using conventional content analysis. MAXQDA 2007 software (VERBI GmbH, Berlin, Germany was used to classify and manage the coding. Constant comparative method was done for data analysis. The reliability and validity of the findings, including the credibility, confirm ability, dependability, and transferability, were assessed. Results: According to the content analysis, the main theme was the catastrophic burden of child care on mothers that included three categories: 1 the tension resulting from the disease, 2 involvement with internal thoughts, and 3 difficulties of care process Conclusion: The results of this study may help health care professionals to provide supportive and educational packages to the patients, mothers and Family members until improving the management of patient’s care.

  13. The Burden of Care: Mothers’ Experiences of Children with Congenital Heart Disease

    Science.gov (United States)

    Sabzevari, Sakinne; Nematollahi, Monirsadat; Mirzaei, Tayebeh; Ravari, Ali

    2016-01-01

    ABSTRACT Background: Mothers play a key role in caring for their sick children. Their experiences of care were influenced by culture, rules, and the system of health and care services. There are few studies on maternal care of children with congenital heart disease. Also, each of them has studied a particular aspect of care. The present research aimed to understand care experiences of mothers of children with congenital heart disease. Methods: A conventional content analysis was used to obtain rich data. The goal of content analysis is “to provide knowledge and deeper understanding of the phenomenon under the study”. The study was conducted in Kerman, Iran in 2014, on mothers of children with CHD. The purposive sampling technique was used to select the participants. Participants were 14 mothers of children with CHD and one father and one nurse of open heart surgery unit, from two hospitals affiliated with Kerman University of Medical Sciences. Eighteen semi-structured interviews were constructed. Data were analyzed using conventional content analysis. MAXQDA 2007 software (VERBI GmbH, Berlin, Germany) was used to classify and manage the coding. Constant comparative method was done for data analysis. The reliability and validity of the findings, including the credibility, confirm ability, dependability, and transferability, were assessed. Results: According to the content analysis, the main theme was the catastrophic burden of child care on mothers that included three categories: 1) the tension resulting from the disease, 2) involvement with internal thoughts, and 3) difficulties of care process Conclusion: The results of this study may help health care professionals to provide supportive and educational packages to the patients, mothers and Family members until improving the management of patient’s care. PMID:27713900

  14. Pediatric cardiology. Clinical and practical experiences with heart diseases of children, juveniles and young adults

    International Nuclear Information System (INIS)

    The book on pediatric cardiology covers the following chapters: (I) Fundamentals and diagnostics: pediatric cardiologic anamnesis, electrocardiograms, thorax X-radiography, MRT and CT of the heart, nuclear medical diagnostics, exercise tests, heart catheter examination, electrophysiological tests. (II) Leading symptoms: Cyanosis, cardiac murmur, thorax pain, palpitation, syncopes. (III) Disease pictures: congenital heart defects, acquired heart defects, cardiomyopathies, heart rhythm disturbances, heart insufficiency, arterial hypertension, pulmonary hypertension, other heart involving syndromes. (IV) Therapy: Catheter interventional therapy, post-surgical pediatric cardiac therapy, surgery involving the life-support machine, mechanical cardiovascular support systems, initial treatment of newborns with critical heart defects, heart transplantation, vaccination of children with heart diseases, medicinal therapy.

  15. 76. Profile and spectrum of congenital heart defect in pediatric patient with down syndrome

    Directory of Open Access Journals (Sweden)

    G. Alsuhaibani

    2016-07-01

    Full Text Available Down syndrome is one of the most common chromosomal abnormality worldwide. It occurs in 1 of every 800 live births. Almost one-half of patients with Down Syndrome have congenital heart defect. Our objective is to describe the frequency and spectrum of congenital heart defect (CHD among children with Down Syndrome in Saudi Arabia and identify the rate of primary and secondary pulmonary hypertension among pediatric patients with Down syndrome. Cross-sectional, retrospective study of the cardiac anomalies among 331 pediatric patients (0–18 years with Down Syndrome in King Khalid University Hospital (KKUH from August 2001 till October 2014. The demographic data, reason for referral, echocardiography data including systolic function parameters, the presence of CHD, type and details of CHD, presence of pulmonary hypertension (PHTN, history of cardiac surgeries or transcatheter interventions. Among the 331 pediatric patients with Down Syndrome; 230 patients (69.5% have Congenital Heart Defect (CHD. The patients with CHD were significantly younger (median age 3 months with lower weight (P-value <0.05 and height (P-value <0.05 compared to patients with no CHD. The median age at first assessment was 3 months. The most common type of CHDs among DS pediatric patients was atrial septal defect secundum (ASD II which account for 33.5% of all CHD followed by ventricular septal defect (VSD which account for 26.5%, then atrioventricular septal defect (AVSD 21.7% and moderate to large patent ductus arteriosus (PDA 21.7%. There is another (11.7% who have other CHDs. Pulmonary hypertension was present in 32% of patients with CHD vs 4% among patients with no CHD. There is significant relationship between CHD and pulmonary hypertension with odds ratio 11.3 (CI 3.99–31.83, P-value <0.05. 15% of patients underwent either cardiac surgery or transcatheter intervention. Almost two thirds of Down Syndrome patients have CHD with pulmonary hypertension affecting almost

  16. Giant Congenital Cerebriform Melanocytic Nevus of the Scalp in Adult

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    Shashikala Krishnamurthy

    2014-04-01

    Full Text Available Congenital melanocytic nevi are defined as benign nevomelanocytic proliferation present at birth. They are rare lesions and occur in approximately 1% of new-borns and are usually classified according to their size. We report a case of 32 year old female who presented with a giant cerebriform lesion of the scalp which was present since birth. Diagnosis of Giant congenital melanocytic nevi was made on the basis of definite histological criteria. Wide excision of the lesion was done with split skin graft. This case is unique due to the fact that this young female lived with this disfiguring lesion for 3 decades and never consulted any physician for cosmetic purpose and fortunately the lesion never turned malignant. [J Interdiscipl Histopathol 2014; 2(2.000: 108-111

  17. Over-expression of DSCAM and COL6A2 cooperatively generates congenital heart defects.

    Directory of Open Access Journals (Sweden)

    Tamar R Grossman

    2011-11-01

    Full Text Available A significant current challenge in human genetics is the identification of interacting genetic loci mediating complex polygenic disorders. One of the best characterized polygenic diseases is Down syndrome (DS, which results from an extra copy of part or all of chromosome 21. A short interval near the distal tip of chromosome 21 contributes to congenital heart defects (CHD, and a variety of indirect genetic evidence suggests that multiple candidate genes in this region may contribute to this phenotype. We devised a tiered genetic approach to identify interacting CHD candidate genes. We first used the well vetted Drosophila heart as an assay to identify interacting CHD candidate genes by expressing them alone and in all possible pairwise combinations and testing for effects on rhythmicity or heart failure following stress. This comprehensive analysis identified DSCAM and COL6A2 as the most strongly interacting pair of genes. We then over-expressed these two genes alone or in combination in the mouse heart. While over-expression of either gene alone did not affect viability and had little or no effect on heart physiology or morphology, co-expression of the two genes resulted in ≈50% mortality and severe physiological and morphological defects, including atrial septal defects and cardiac hypertrophy. Cooperative interactions between DSCAM and COL6A2 were also observed in the H9C2 cardiac cell line and transcriptional analysis of this interaction points to genes involved in adhesion and cardiac hypertrophy. Our success in defining a cooperative interaction between DSCAM and COL6A2 suggests that the multi-tiered genetic approach we have taken involving human mapping data, comprehensive combinatorial screening in Drosophila, and validation in vivo in mice and in mammalian cells lines should be applicable to identifying specific loci mediating a broad variety of other polygenic disorders.

  18. Providing Guidance for School Personnel Making Decisions in the Service of School Children with Congenital Heart Disease

    Science.gov (United States)

    Roberts, Jillian; MacMath, Sheryl

    2006-01-01

    Due to improved medical procedures, more and more children with congenital heart disease are entering the school system. In order to help both school and health professionals involved in the education of children, we provide a brief review of the literature, review real-life dilemmas that school personnel face on a daily basis, and interpret the…

  19. "What I Wish You Knew": Social Barriers toward Physical Activity in Youth with Congenital Heart Disease (CHD)

    Science.gov (United States)

    Moola, Fiona; Fusco, Caroline; Kirsh, Joel A.

    2011-01-01

    Despite the benefits of physical activity for youth with congenital heart disease (CHD), most patients are inactive. Although literature has addressed medical and psychological barriers to participation, little is known about the social barriers that youth encounter. This qualitative study explored sociocultural barriers to physical activity from…

  20. Maternal intake of fat, riboflavin and nicotinamide and the risk of having offspring with congenital heart defects

    NARCIS (Netherlands)

    Smedts, H.P.M.; Rakhshandehroo, M.; Verkleij-Hagoort, A.C.; Vries, de J.H.M.; Ottenkamp, J.; Steegers, E.A.P.; Steegers-Theunissen, R.P.M.

    2008-01-01

    With the exception of studies on folic acid, little evidence is available concerning other nutrients in the pathogenesis of congenital heart defects (CHDs). Fatty acids play a central role in embryonic development, and the B-vitamins riboflavin and nicotinamide are co-enzymes in lipid metabolism. Ai

  1. Prospective validation and assessment of cardiovascular and offspring risk models for pregnant women with congenital heart disease

    NARCIS (Netherlands)

    Balci, A.; Sollie-Szarynska, K.M.; Bijl, A.G. van der; Ruys, T.P.; Mulder, B.J.; Roos-Hesselink, J.W.; Dijk, A.P.J. van; Wajon, E.M.; Vliegen, H.W.; Drenthen, W.; Hillege, H.L.; Aarnoudse, J.G.; Veldhuisen, D.J. van; Pieper, P.G.

    2014-01-01

    OBJECTIVES: Adequate prepregnancy prediction of maternal cardiovascular and offspring risk is important for counselling and management of pregnancy in women with congenital heart disease (CHD). Therefore we performed a study to identify the optimal assessment strategy for estimating the risk of preg

  2. Prospective validation and assessment of cardiovascular and offspring risk models for pregnant women with congenital heart disease

    NARCIS (Netherlands)

    Balci, Ali; Sollie-Szarynska, Krystyna M.; van der Bijl, Antoinette G. L.; Ruys, Titia P. E.; Mulder, Barbara J. M.; Roos-Hesselink, Jolien W.; van Dijk, Arie P. J.; Wajon, Elly M. C. J.; Vliegen, Hubert W.; Drenthen, Willem; Hillege, Hans L.; Aarnoudse, Jan G.; van Veldhuisen, Dirk J.; Pieper, Petronella G.

    2014-01-01

    Objectives Adequate prepregnancy prediction of maternal cardiovascular and offspring risk is important for counselling and management of pregnancy in women with congenital heart disease (CHD). Therefore we performed a study to identify the optimal assessment strategy for estimating the risk of pregn

  3. Early pregnancy exposure to antihistamines and risk of congenital heart defects : results of two case-control studies

    NARCIS (Netherlands)

    Smedts, Huberdina P. M.; de Jonge, Linda; Bandola, Sarah J. G.; Baardman, Marlies E.; Bakker, Marian K.; Stricker, Bruno H. C.; Steegers-Theunissen, Regine P. M.

    2014-01-01

    UNLABELLED: We aimed to study the association between use of antihistamines in early pregnancy and congenital heart defects (CHD) in the offspring. DESIGN: Two case-control studies. SETTING: HAVEN study, Erasmus MC, University Medical Centre, Rotterdam, and Eurocat Northern Netherlands (NNL), Univer

  4. High maternal vitamin E intake by diet or supplements is associated with congenital heart defects in the offspring

    NARCIS (Netherlands)

    Smedts, H.P.M.; Vries, de J.H.M.; Rakhshandehroo, M.; Wildhagen, M.F.; Verkleij-Hagoort, A.C.; Steegers, E.A.P.; Steegers-Theunissen, R.P.M.

    2009-01-01

    Objective To study associations between maternal dietary and supplement intake of antioxidants vitamin E, retinol and congenital heart defects (CHDs). Design Case–control study. Setting Erasmus MC, University Medical Center Rotterdam, the Netherlands. Population Participants were 276 case mothers of

  5. High maternal vitamin E intake by diet or supplements is associated with congenital heart defects in the offspring

    NARCIS (Netherlands)

    Smedts, H. P. M.; de Vries, J. H.; Rakhshandehroo, M.; Wildhagen, M. F.; Verkleij-Hagoort, A. C.; Steegers, E. A.; Steegers-Theunissen, R. P. M.

    2009-01-01

    To study associations between maternal dietary and supplement intake of antioxidants vitamin E, retinol and congenital heart defects (CHDs). Case-control study. Erasmus MC, University Medical Center Rotterdam, the Netherlands. Participants were 276 case mothers of a child with CHD and 324 control mo

  6. First-trimester measurement of the ductus venosus pulsatility index and the prediction of congenital heart defects

    NARCIS (Netherlands)

    Timmerman, E.; Clur, S. A.; Pajkrt, E.; Bilardo, C. M.

    2010-01-01

    Objective This study was carried out to evaluate the additional predictive value of ductus venosus pulsatility index for veins (DV-PIV) in the identification of congenital heart defects (CHDs) in fetuses with an enlarged nuchal translucency (NT) and a normal karyotype. Methods All chromosomally norm

  7. The impact of congenital heart diseases on the quality of life of patients and their families in Saudi Arabia

    OpenAIRE

    Azhar, Ahmad S.; AlShammasi, Zahra H.; Higgi, Rawan E.

    2016-01-01

    Objectives: To assess the impact of congenital heart diseases (CHDs) on bio-psychosocial aspects of the quality of life (QOL) of patients and their families. Methods: A cross-sectional study was carried out between May 2014 and August 2015, including children aged

  8. Multidetector CT for congenital heart patients: what a paediatric radiologist should know

    Energy Technology Data Exchange (ETDEWEB)

    Paul, Jean-Francois; Rohnean, Adela; Sigal-Cinqualbre, Anne [Radiology Unit, Marie Lannelongue Hospital, Plessis-Robinson (France)

    2010-06-15

    Multidetector CT (MDCT) is increasingly used for imaging congenital heart disease (CHD) patients in addition to echocardiography, due to its ability to provide high quality three-dimensional images, giving a comprehensive evaluation of complex heart malformations. Using 4-slice or 16-slice CT, diagnostic information in CHD patients is limited to extra-cardiac anatomy, mainly the pulmonary arteries, aorta and venous connections. Due to high heart rates in babies however, coronary evaluation and intra-cardiac analysis were not reliable with the first generations of MDCT. Larger detector size with 64-slice CT and faster acquisition time, up to 75 ms for one slice, has progressively improved coronary and intra-cardiac visualization. Because radiation dose is the main concern, especially in children, every attempt to minimize dose whilst preserving image quality is important: the ALARA concept should always be applied in this population. The 80 kVp setting is now well accepted as a standard for more and more radiological teams involved in CT of children. Different acquisition strategies are now possible for childhood coronary imaging, using retrospective or even prospective gating. Using the latest technology, sub-mSv acquisitions are now attainable for scanning a whole thorax, providing a complete analysis of any 3-D cardiac malformation, including coronary artery course visualisation. This review will describe how technological developments have improved image quality with continuous reduction of radiation dose. (orig.)

  9. Sociodemographic and clinical profile of children with congenital heart disease assisted at a hospital institution

    Directory of Open Access Journals (Sweden)

    Mirna Albuquerque Frota

    2014-06-01

    Full Text Available Objective: To characterize the sociodemographic and clinical profile of children with congenital heart disease assisted at a hospital institution. Methods: A descriptive, crosssectional study conducted in the pediatric cardiology outpatient clinic of a hospital in Fortaleza, CE, Brazil, in the period from March to August 2012. It comprised a nonrandom convenience sample of 80 parents of children with heart disease aged 5 to 12 years. Information was obtained from primary data of medical records and through questionnaires covering the parent’s sociodemographic characteristics, family data and the children’s clinical aspects. Results: Regarding socioeconomic data, 77 (96.2% caregivers were female, 50 (62.5% were married or in a stable relationship, 39 (48.7% had 10-12 years of formal education, and 69 (82.6% reported a household income between 1 and 2 minimum wages. In the sample of children studied, 43 (53.8% were female. The median age of the children was 8.4 years, ranging from 5-13 years. As to the age at the heart disease diagnosis, 44 (55% occurred under the age of 6 months, regardless of the type of cardiopathy. It was detected that 19 (23.75% were related to the disease. Conclusion: Children’s caregivers were predominantly their mothers, with low income and satisfactory schooling. Early diagnosis was predominant, mainly among the children with cyanotic cardiopathy. doi:10.5020/18061230.2014.p239

  10. Partnership for the heart. German-Estonian health project for the treatment of congenital heart defects in Estonia.

    Science.gov (United States)

    Köhler, Friedrich; Schierbaum, Clausjürgen; Konertz, Wolfgang; Schneider, Martin; Kern, Hartmut; Int, Eve; Tael, Kristi; Siigur, Urmas; Kleinfeld, Klaus; Bühlmeyer, Konrad; Fotuhi, Parwis; Winter, Stefan Frank

    2005-08-01

    Congenital heart defects (CHD) occur in 8 per 1000 live births. If they remain untreated, 70-80% of these patients die in early childhood. With modern diagnostic and treatment procedures, 90% of these patients reach adulthood. Within 8 years following reunification of Germany, it was possible to improve treatment for CHD in former East Germany to West German standards. Based on the experience gained in this process, a plan for improvement of care of Estonian patients with CHD was developed and implemented in the German-Estonian project, "Partnership for the Heart". The main elements of the project were (1) the training of Estonian physicians in Germany, (2) training courses conducted by German and Estonian specialists in Estonia and (3) use of telemedicine for consultation on a continuous basis. During the project 15 Estonian patients underwent cardiac surgery and/or catheter interventions performed by a joint team of German and Estonian specialists. The infant mortality due to CHD in Estonia fell by 28% during the project period. Key techniques of cardiac surgery are now being employed in Estonia without outside support, indicating the success of the training program and the long-term improvements to cardiac health care in Estonia. The total project costs were 314,252 Euro (euro), which is 50% lower than the estimated cost of treating the 15 patients abroad in Western Europe. The structure of "Partnership for the Heart" and the modified self-sufficiency model of medical care have not only produced results for Estonia but can be taken as a template for future bilateral health projects with other transition countries and for other fields of medical specialisation, and thus might aid a European health policy.

  11. Asplenia in children with congenital heart disease as a cause of poor outcome.

    Science.gov (United States)

    Erdem, Semiha Bahceci; Genel, Ferah; Erdur, Baris; Ozbek, Erhan; Gulez, Nesrin; Mese, Timur

    2015-01-01

    The absence of a spleen is a well-known risk factor for severe bacterial infections, especially due to encapsulated bacteria. Congenital asplenia can be part of multiple congenital abnormalities as in heterotaxy including Ivemark syndrome with congenital anomalies of the heart or great vessels, or it can be isolated, which is extremely rare. In these cases, asplenia is an important factor effecting mortality. In this report, the clinical courses of five children with asplenia and concomitant minor or complex cardiac anomalies are presented. The ages of the children ranged between 1.5 and 17 months at the time of diagnosis. All of the cases had had a history of hospitalisation for infectious diseases before the diagnosis. The patient who was diagnosed at 17 months old had a history pneumonia, urinary tract infection, and bacterial meningitis beginning at five months old. Three children had complex cardiac anomalies, one child had ventricular septal defect, and one child had atrial septal defect. Howell-Jolly bodies were determined in peripheral blood smear in all of the patients. The diagnoses of asplenia were confirmed with spleen scintigraphy. One of the patients with complex cardiac anomalies died a short time after diagnosis, because of cardiac failure. The rest of the four patients were vaccinated for encapsulated bacteria and were taken under antibiotic prophylaxis. These children did not need hospitalisation for infectious diseases during the follow-up period (5-40 months). In asplenic children, early diagnosis, antibiotic prophylaxis, and immunisation for encapsulated bacteria can decrease the risk of morbidity and mortality. PMID:26557043

  12. Cardiovascular Magnetic Resonance Imaging in the Assessment of Myocardial Blood Flow, Viability, and Diffuse Fibrosis in Congenital and Acquired Heart Disease

    OpenAIRE

    Pham, Hoang Minh

    2014-01-01

    Myocardial ischemia may occur after surgical correction of congenital heart defects involving the coronary arteries, in patients with congenital coronary artery anomalies, or in acquired heart disease. To assess myocardial ischemia disease in children, we used cardiac MRI as a non-invasive and radiation-free method to evaluate myocardial perfusion, viability, diffuse myocardial fibrosis, and heart function, to guide further therapy and for a better understanding of the pathophysiology of the ...

  13. Prevalence of arrhythmias among children below 15 years of age with congenital heart diseases attending Mulago National Referral Hospital, Uganda

    OpenAIRE

    Batte, Anthony; Lwabi, Peter; Lubega, Sulaiman; Kiguli, Sarah; Nabatte, Violette; Karamagi, Charles

    2016-01-01

    Background In Uganda, few children with congenital heart diseases (CHD) benefit from early corrective cardiac surgery. These children are at high risk of developing heart failure and electrolyte imbalances; factors which increase their risk of developing arrhythmias. This study aimed to determine the prevalence and factors associated with arrhythmias among children with congenital heart diseases receiving care at Mulago Hospital. Methods This was a cross-sectional study carried out from Augus...

  14. Defining the phenotype and diagnostic considerations in adults with congenital disorders of N-linked glycosylation

    NARCIS (Netherlands)

    Wolthuis, D.F.; Janssen, M.C; Cassiman, D.; Lefeber, D.J.; Morava-Kozicz, E.

    2014-01-01

    Congenital disorders of N-glycosylation (CDG) form a rapidly growing group of more than 20 inborn errors of metabolism. Most patients are identified at the pediatric age with multisystem disease. There is no systematic review on the long-term outcome and clinical presentation in adult patients. Here

  15. Spirituality: A Coping Mechanism in the Lives of Adults with Congenital Disabilities

    Science.gov (United States)

    Specht, Jacqueline A.; King, Gillian A.; Willoughby, Colleen; Brown, Elizabeth G.; Smith, Linda

    2005-01-01

    The purpose of this study was to gain insight into the perspective of individuals with congenital disabilities about spirituality as a coping mechanism during crucial times in their lives, Qualitative analysis of interviews assessing turning points in the lives of 15 adults (6 women and 9 men; M = 37 years) with spina bifida, cerebral palsy, or…

  16. Intervening on Affective Involvement and Expression of Emotions in an Adult with Congenital Deafblindness

    Science.gov (United States)

    Martens, Marga A. W.; Janssen, Marleen J.; Ruijssenaars, Wied A. J. J. M.; Huisman, Mark; Riksen-Walraven, J. Marianne

    2014-01-01

    This study examined the effects of a 20-week intervention to foster affective involvement during interaction and communication between an adult with congenital deafblindness (CDB) and his caregivers in a group home and a daytime activities center. Using a single-subject design, we examined whether the intervention increased affective involvement…

  17. Congenital heart diseases: post-operative appearance on multi-detector CT - a pictorial essay

    Energy Technology Data Exchange (ETDEWEB)

    Tomasian, Anderanik; Malik, Sachin; Shamsa, Kamran; Krishnam, Mayil S. [UCLA Medical Center, Department of Radiological Science, Los Angeles, CA (United States)

    2009-12-15

    Echocardiography is considered as an initial imaging modality of choice in patients with congenital heart disease (CHD), and magnetic resonance (MR) imaging is preferred for detailed functional information. Multi-detector computed tomography (CT) plays an important role in clinical practice in assessing post-operative morphological and functional information of patients with complex CHD when echocardiography and MR imaging are not contributory. Radiologists should understand and become familiar with the complex morphology and physiology of CHD, as well as with various palliative and corrective surgical procedures performed in these patients, to obtain CT angiograms with diagnostic quality and promptly recognise imaging features of normal post-operative anatomy and complications of these complex surgeries. (orig.)

  18. Recent Decrease in the Prevalence of Congenital Heart Defects in Europe

    DEFF Research Database (Denmark)

    Khoshnood, Babak; Loane, Maria; Garne, Ester;

    2013-01-01

    OBJECTIVES: To examine trends in the prevalence of congenital heart defects (CHDs) in Europe and to compare these trends with the recent decrease in the prevalence of CHDs in Canada (Quebec) that was attributed to the policy of mandatory folic acid fortification. STUDY DESIGN: We used data...... increased until about 2000 and decreased thereafter. Trends for severity group III (the most prevalent group) paralleled those for all CHDs combined. CONCLUSIONS: The prevalence of CHDs decreased in recent years in Europe in the absence of a policy for mandatory folic acid fortification. One possible...... explanation for this decrease may be an as-yet-undocumented increase in folic acid intake of women in Europe following recommendations for folic acid supplementation and/or voluntary fortification. However, alternative hypotheses, including reductions in risk factors of CHDs (eg, maternal smoking...

  19. 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.

  20. The importance of early involvement of paediatric palliative care for patients with severe congenital heart disease.

    Science.gov (United States)

    Bertaud, Sophie; Lloyd, David F A; Laddie, Joanna; Razavi, Reza

    2016-10-01

    Growing numbers of patients with severe congenital heart disease (CHD) are surviving into late childhood and beyond. This increasingly complex patient group may experience multiple formidable and precarious interventions, lifelong morbidity and the very real risk of premature death on many occasions throughout their childhood. In this paper, we discuss the advantages of a fully integrated palliative care ethos in patients with CHD, offering the potential for improved symptom control, more informed decision-making and enhanced support for patients and their families throughout their disease trajectory. These core principles may be delivered alongside expert cardiac care via non-specialists within pre-existing networks or via specialists in paediatric palliative care when appropriate. By broaching these complex issues early-even from the point of diagnosis-an individualised set of values can be established around not just end-of-life but also quality-of-life decisions, with clear benefits for patients and their families regardless of outcome.

  1. Cellular and molecular basis of RV hypertrophy in congenital heart disease.

    Science.gov (United States)

    Iacobazzi, D; Suleiman, M-S; Ghorbel, M; George, S J; Caputo, M; Tulloh, R M

    2016-01-01

    RV hypertrophy (RVH) is one of the triggers of RV failure in congenital heart disease (CHD). Therefore, improving our understanding of the cellular and molecular basis of this pathology will help in developing strategic therapeutic interventions to enhance patient benefit in the future. This review describes the potential mechanisms that underlie the transition from RVH to RV failure. In particular, it addresses structural and functional remodelling that encompass contractile dysfunction, metabolic changes, shifts in gene expression and extracellular matrix remodelling. Both ischaemic stress and reactive oxygen species production are implicated in triggering these changes and will be discussed. Finally, RV remodelling in response to various CHDs as well as the potential role of biomarkers will be addressed.

  2. Managing the Morbidity Associated with Respiratory Viral Infections in Children with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Joseph M. Geskey

    2012-01-01

    Full Text Available Children with congenital heart disease (CHD are at risk for increased morbidity from viral lower respiratory tract infections because of anatomical cardiac lesions than can worsen an already compromised respiratory status. Respiratory syncytial virus (RSV remains an important pathogen in contributing toward the morbidity in this population. Although the acute treatment of RSV largely remains supportive, the development of monoclonal antibodies, such as palivuzumab, has reduced the RSV-related hospitalization rate in children with CHD. This review highlights the specific cardiac complications of RSV infection, the acute treatment of bronchiolitis in patients with CHD, and the search for new therapies against RSV, including an effective vaccine, because of the high cost associated with immunoprophylaxis and its lack of reducing RSV-related mortality.

  3. Is Pulse Oximetry Useful for Screening Neonates for Critical Congenital Heart Disease at High Altitudes?

    Science.gov (United States)

    Hoffman, Julien I E

    2016-06-01

    Now that pulse oximetry is used widely to screen for critical congenital heart disease, it is time to consider whether this screening method is applicable to those who live at high altitudes. Consideration of basic physical principles and reports from the literature indicate that not only is the 95 % cutoff point for arterial oxygen saturation incorrect at high altitudes, but the lower saturations are accompanied by greater variability and therefore there is the possibility of a greater percentage of false-positive screening tests at high altitudes. Because of ethnic differences in response to high altitudes, normative data will have to be collected separately in different countries and perhaps for different ethnic groups.

  4. ALTERATIONS IN FREQUENCY OF ULNAR LOOPS AND ‘ATD’ ANGLE IN CONGENITAL HEART DISEASE

    Directory of Open Access Journals (Sweden)

    Jaywant

    2016-01-01

    Full Text Available INTRODUCTION Dermatoglyphics is a scientific study of epidermal ridge configuration on palm, soles and fingertips valuable for medico legal and genetic investigations. Dermatoglyphics form in utero during early gestation and may be influenced by genetic and environmental factors operating at that time. Present investigation was undertaken to study alterations in dermatoglyphic patterns with special reference to various congenital heart diseases (CHD. The study involved 102 cases of CHD and 100 cases of normal individuals. It was observed that percent frequency of ulnar loops significantly increased in CHD group as compared to control group. Mean 'atd' angle was also increased in CHD group as compared to control group indicating distal displacement of palmar axial triradius (t. Thus, rise in frequency of ulnar loops and increase in 'atd' angle can be considered as one of the diagnostic criteria for CHD.

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

    International Nuclear Information System (INIS)

    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

  6. Children with hemodynamically significant congenital heart disease can be identified through population-based registers

    DEFF Research Database (Denmark)

    Bergman, Gunnar; Hærskjold, Ann; Stensballe, Lone Graff;

    2015-01-01

    as having hemodynamically significant CHD according to the recommendations for treatment with palivizumab. CONCLUSION: It was possible to identify a subgroup of children with hemodynamically significant CHD using an epidemiological approach and an algorithm with high validity. Our results will enable well-powered......BACKGROUND: Epidemiological research is facilitated in Sweden by a history of national health care registers, making large unselected national cohort studies possible. However, for complex clinical populations, such as children with congenital heart disease (CHD), register-based studies...... is indicated as a prophylactic treatment against respiratory syncytial virus infections in children with hemodynamically significant CHD. AIM: The aim of the study reported here was to develop and validate an algorithm to identify children with hemodynamically significant CHD according to recommendations...

  7. Congenital heart diseases: post-operative appearance on multi-detector CT - a pictorial essay

    International Nuclear Information System (INIS)

    Echocardiography is considered as an initial imaging modality of choice in patients with congenital heart disease (CHD), and magnetic resonance (MR) imaging is preferred for detailed functional information. Multi-detector computed tomography (CT) plays an important role in clinical practice in assessing post-operative morphological and functional information of patients with complex CHD when echocardiography and MR imaging are not contributory. Radiologists should understand and become familiar with the complex morphology and physiology of CHD, as well as with various palliative and corrective surgical procedures performed in these patients, to obtain CT angiograms with diagnostic quality and promptly recognise imaging features of normal post-operative anatomy and complications of these complex surgeries. (orig.)

  8. Perioperative pharmacological management of pulmonary hypertensive crisis during congenital heart surgery.

    Science.gov (United States)

    Brunner, Nathan; de Jesus Perez, Vinicio A; Richter, Alice; Haddad, François; Denault, André; Rojas, Vanessa; Yuan, Ke; Orcholski, Mark; Liao, Xiaobo

    2014-03-01

    Pulmonary hypertensive crisis is an important cause of morbidity and mortality in patients with pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD) who require cardiac surgery. At present, prevention and management of perioperative pulmonary hypertensive crisis is aimed at optimizing cardiopulmonary interactions by targeting prostacyclin, endothelin, and nitric oxide signaling pathways within the pulmonary circulation with various pharmacological agents. This review is aimed at familiarizing the practitioner with the current pharmacological treatment for dealing with perioperative pulmonary hypertensive crisis in PAH-CHD patients. Given the life-threatening complications associated with pulmonary hypertensive crisis, proper perioperative planning can help anticipate cardiopulmonary complications and optimize surgical outcomes in this patient population.

  9. Legal and Ethical Considerations in Allowing Parental Exemptions From Newborn Critical Congenital Heart Disease (CCHD) Screening.

    Science.gov (United States)

    Hom, Lisa A; Silber, Tomas J; Ennis-Durstine, Kathleen; Hilliard, Mary Anne; Martin, Gerard R

    2016-01-01

    Critical congenital heart disease (CCHD) screening is rapidly becoming the standard of care in the United States after being added to the Recommended Uniform Screening Panel (RUSP) in 2011. Newborn screens typically do not require affirmative parental consent. In fact, most states allow parents to exempt their baby from receiving the required screen on the basis of religious or personally held beliefs. There are many ethical considerations implicated with allowing parents to exempt their child from newborn screening for CCHD. Considerations include the treatment of religious exemptions in our current legal system, as well as medical and ethical principles in relation to the rights of infants. Although there are significant benefits to screening newborns for CCHD, when a parent refuses for religious or personal beliefs, in the case of CCHD screening, the parental decision should stand.

  10. MR and cine-MR imaging in the diagnosis of infantile congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    朱会英; 韩立新; 黄新华; 王霞

    2001-01-01

    目的评价磁共振成像(MRI)及电影磁共振成像(cine-MRI)在小儿先天性心脏病(简称先心病)中的诊断 价值。方法对34例先心患儿常规行横轴位、冠状位、矢状位成像,并根据诊断需要选择垂直于室间隔的长轴 位、短轴位及平行于室间隔的长轴位。在图像中选择感兴趣的层面做心脏cine-MRI,每个心动周期内采集 16-32帧图像,以动态观察心脏及大血管变化,并与二维超声心动图(2DE)、心血管造影(CAG)及手术结 果进行对照分析。 结果MRI测量6例左向右分流型先心病患儿缺损口或内径值与手术所见相吻合(P=0.924),与2DE呈 正相关(r=0.973),且MRI较2DE更接近手术实测值。MRI及cine-MRI诊断28例复杂型先心病,27例与 手术诊断相一致。 结论MRI能准确显示房、室间隔的缺损、动脉导管未闭部位及内径,cine-MRI能以电影的方式显示间隔 缺损的分流、心脏与大血管血流状况及畸形的病理形态。本组资料证实MRI及cine-MRI是一种安全、无 创的诊断小儿先天性心脏病的检查方法,有较大的临床应用价值。%Objective To assess the effectiveness of magnetic resonanceimaging (MRI) and cine magnetic resonance imaging (cine-MRI) in the diagnosis of infantile congenital heart disease. Methods A total of 34 cases were studied with MRI and cine-MRI. The data were analyzed and compared with those of two-dimensional echocardiography (2DE), cardioangiography (CAG) and surgery. Results The size of the defect or its caliber obtained from MRI in 6 patients with left to right shunt congenital heart disease was compatible with that observed in surgery (P=0.924). Comparison of cine-MRI and CAG in 28 patients with complicated congenital heart disease showed that the diagnosis of 27 cases by cine-MRI was the same as that by CAG. Conclusion Both MRI and cine-MRI play an important role in diagnosing infantile congenital heart disease.

  11. Computed tomography for imaging of pediatric congenital heart disease; Die Computertomographie bei der Bildgebung von Kindern mit kongenitalen Herzvitien

    Energy Technology Data Exchange (ETDEWEB)

    Glaser-Gallion, N.; Stinn, B.; Wildermuth, S.; Leschka, S. [Kantonsspital St.Gallen, Universitaet Zuerich, Institut fuer Radiologie, St. Gallen (Switzerland); Alkadhi, H. [Universitaetsspital Zuerich, Institut fuer Diagnostisch und Interventionelle Radiologie, Zuerich (Switzerland); Lell, M. [Universitaetsklinikum Erlangen, Institut fuer Radiologie, Erlangen (Germany); Goo, H.W. [University of Ulsan, College of Medicine, Asan Medical Center, Department of Radiology, Seoul (Korea, Republic of); Paul, J.F. [Marie Lannelongue Hospital, Radiology Unit, Plessis Robinson (France)

    2011-01-15

    Congenital heart diseases are the most common congenital abnormalities of development. In general, echocardiography and cardiac catheter angiography are considered the gold standard for the evaluation of congenital heart disease. Cardiac magnetic resonance imaging has become an important supplementary imaging modality because of its ability to provide an accurate morphological and functional evaluation. The role of cardiac computed tomography in the imaging of patients with congenital heart disease is becoming increasingly more important due to the development of low radiation dose protocols and improvements in the spatial and temporal resolution. In the preoperative depiction and follow-up after surgical repair of congenital heart diseases, cardiac computed tomography provides detailed information of the heart, the venous and arterial pulmonary circulation as well as systemic arteries. This article reviews the technical aspects of cardiac CT and the modification of examination protocols according to the expected pathology and patient age. The potentials and limitations of the various radiation dose reduction strategies are outlined. (orig.) [German] Kongenitale Herzfehler sind die haeufigsten kongenitalen Fehlbildungen. Echokardiographie und Katheterangiographie gelten allgemein als Goldstandard zur Abklaerung angeborener Herzerkrankungen. Die Magnetresonanztomographie ist aufgrund ihrer Faehigkeit, Herzvitien morphologisch und funktionell zu charakterisieren, als ein wichtiges ergaenzendes Verfahren anzusehen. Durch mehr und mehr dosissparende Untersuchungsprotokolle der neuesten Geraetegenerationen und eine gleichzeitig bessere zeitliche und raeumliche Aufloesung findet die Computertomographie zunehmend Eingang in die Abklaerung kongenitaler Herzfehler. In der praeoperativen Planung und der postoperativen Kontrolle erlaubt sie eine uebersichtliche Darstellung komplexer Fehlbildung nicht nur des Herzens, sondern auch der pulmonalvenoesen und -arteriellen

  12. Non Invasive Transcutaneous Carbondioxide Monitoring in Adult Open Heart Surgery

    OpenAIRE

    UÇAR, P.; GAZİOĞLU, G.; ERDEMLİ, Ö.; ÇİÇEK, Ö. F.; DEMİR, A.

    2013-01-01

    Non Invasive Transcutaneous Carbondioxide Monitoring in Adult Open Heart SurgeryObjective: Follow-up of CO level during open heart surgery is crucial in terms of monitoring and management of the metabolic status. In this prospective observational study,measurement of end tidal CO and arterial CO levels in adult open heart surgery was compared with transcutaneous CO monitoring which is a non-invasive method.Material and Method: The study included 22 ASA II-III patients with an age range of 30-...

  13. Unenhanced steady state free precession versus traditional MR imaging for congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Dandan, E-mail: chchsister@163.com [Department of Diagnostic Radiology, the First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong (China); Kong, Xiangquan, E-mail: kxq0525@126.com [Department of Radiology, the Affiliated Union Hospital, Huazhong University of Science and Technology, Wuhan, Hubei (China); Zhou, Xuhui, E-mail: xiaolintongqq@126.com [Department of Diagnostic Radiology, the First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong (China); Li, Shurong, E-mail: 80917333@qq.com [Department of Diagnostic Radiology, the First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong (China); Wang, Huanjun, E-mail: 463822507@qq.com [Department of Diagnostic Radiology, the First Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong (China)

    2013-10-01

    Purpose: To assess potential benefits of three dimensional (3D) steady state free precession (SSFP) magnetic resonance sequence for congenital heart disease (CHD). Materials and methods: Twenty consecutive patients with CHD (male:female ratio,14:6, mean age, 27.5 ± 8.5 years) underwent both 3D SSFP and traditional MR imaging (TMRI) [including two dimensional (2D) SSFP and contrast enhanced magnetic resonance angiography (CEMRA)]. Image quality and diagnosis were compared, and Bland–Altman analysis was used to evaluate consistency of 3D SSFP and CEMRA for diameter measurements. Results: A total of 35 intra and 81 extra cardiac anomalies were identified in all patients. The image quality of 3D SSFP and TMRI for either intra or extra cardiac anomalies of all patients scored ≥3, which allowed an establishment of diagnosis for all cases. The diagnostic sensitivity, specificity, and accuracy of 3D SSFP for the detection of intra cardiac anomalies were all 100%, whereas for extra cardiac anomalies they were 93.8%, 93.8%, 100%, respectively. Mean differences (3D SSFP minus CEMRA) for aorta and pulmonary arteries were 0.5 ± 1.2 mm and 0.0 ± 1.7 mm, respectively, showing good consistency of 3D SSFP and CEMRA for diameter measurements. Conclusion: 3D SSFP MRI can be an alternative image modality to TMRI for patients with congenital heart disease, especially for those who have renal insufficiency, breath-hold difficulty or who are allergic to contrast agent. It can also provide powerful complementary information for patients who undergo TMRI, especially at ventriculoarterial connection site.

  14. THE ROLE OF PHYSICAL THERAPY IN DIMINISHING THE EFFECTS OF THE CONGENITAL HEART DEFECT - CASE STUDY

    Directory of Open Access Journals (Sweden)

    Croitoru Ramona

    2014-03-01

    Full Text Available In ROMANIA, fetal abnormalities are the cause of 20-30% of infant mortality, being second only to prematurity - 53% in 2005. The restriction of the fetal growth represents the main cause for intrauterine fetal death (60-70%, and the second cause of neonatal deaths - 41%. Perinatal mortality is caused by 6 obstetrical complications: prematurity, multiple pregnancy, pregnancy-induced hypertension, uteroplacental apoplexy, placenta praevia, pelvic presentation. The termination of pregnancy for prenatally diagnosed fetal abnormalities is excluded from the perinatal mortality statistics, according to the epidemiological information. The motivation for choosing a physical therapy intervention in the case of the pediatric congenital heart defect is represented by its number of pediatric cases, and by the problems confronted by the medical teams, the professional literature not being always very clear about the situations encountered in the everyday practice. As the number of congenital heart and blood vessels defects is the highest among pediatric cardiovascular pathologies, they represent a field of maximum interest for rehabilitation in pediatric cardiology.Several modern medicine studies show that physical therapy plays an important role in the rehabilitation of cardiovascular patients.Starting from several cases we researched in relation to our theme, but also from the consulted professional literature, we chose this theme to highlight the importance of this malformative syndrome, and the difficulties that can appear during functional rehabilitation.In conclusion, the results obtained after conducting this study allow us to say that our hypotheses, stating that through a correct and complete assessment of the motor potential one can intervene, using physical therapy means, to reestablish a lost function, and by selecting correctly the means and methods based on the extremely complex diagnosis of the case study, one can contribute to the improvement

  15. Repeatability of heart rate variability in congenital hypothyroidism as analysed by detrended fluctuation analysis

    International Nuclear Information System (INIS)

    The analysis of heart rate fluctuations, or heart rate variability (HRV), may be applied to explore children's neurodevelopment. However, previous studies have reported poor reliability (repeatability) of HRV measures in children at rest and during light exercise. Whether the reliability can be improved by controlling variables such as physical activity, breathing rate and tidal volume, or by selecting non-conventional techniques for analysing the data remains as an open question. We evaluated the short-term repeatability of RR-interval data from medicated children with congenital hypothyroidism (CH). The α1 exponents, obtained by detrended fluctuation analysis (DFA), from the data of 21 children collected at two different sessions were compared. Elapsed days between sessions were 59 ± 33, and data were obtained during 10 min, trying to restrict the children's activity while being seated. We found statistical agreement between the means of α1 exponents for each session (p = 0.94) and no bias with a low-coefficient variation (9.1%); an intraclass correlation coefficient ri = 0.48 ([0.14 0.72], 95% confidence interval) was also estimated. These findings, which were compared with results obtained by conventional time and frequency techniques, indicate the existence of agreement between the α1 exponents obtained at each session, thereby providing support concerning the repeatability of HRV data as analysed by DFA in children with congenital hypothyroidism. Of particular interest was also the agreement found by using the central frequency of the high-frequency band and the parameter pNN20, both showing better or similar ri than α1 (0.77 [0.57 0.89] and 0.51 [0.17 0.74], respectively), yet considerably better repeatability than other conventional time and frequency parameters

  16. Repeatability of heart rate variability in congenital hypothyroidism as analysed by detrended fluctuation analysis.

    Science.gov (United States)

    Echeverría, J C; Solís, L I; Pérez, J E; Gaitán, M J; Rivera, I R; Mandujano, M; Sánchez, M C; González-Camarena, R

    2009-10-01

    The analysis of heart rate fluctuations, or heart rate variability (HRV), may be applied to explore children's neurodevelopment. However, previous studies have reported poor reliability (repeatability) of HRV measures in children at rest and during light exercise. Whether the reliability can be improved by controlling variables such as physical activity, breathing rate and tidal volume, or by selecting non-conventional techniques for analysing the data remains as an open question. We evaluated the short-term repeatability of RR-interval data from medicated children with congenital hypothyroidism (CH). The alpha(1) exponents, obtained by detrended fluctuation analysis (DFA), from the data of 21 children collected at two different sessions were compared. Elapsed days between sessions were 59 +/- 33, and data were obtained during 10 min, trying to restrict the children's activity while being seated. We found statistical agreement between the means of alpha(1) exponents for each session (p = 0.94) and no bias with a low-coefficient variation (9.1%); an intraclass correlation coefficient ri = 0.48 ([0.14 0.72], 95% confidence interval) was also estimated. These findings, which were compared with results obtained by conventional time and frequency techniques, indicate the existence of agreement between the alpha(1) exponents obtained at each session, thereby providing support concerning the repeatability of HRV data as analysed by DFA in children with congenital hypothyroidism. Of particular interest was also the agreement found by using the central frequency of the high-frequency band and the parameter pNN20, both showing better or similar ri than alpha(1) (0.77 [0.57 0.89] and 0.51 [0.17 0.74], respectively), yet considerably better repeatability than other conventional time and frequency parameters.

  17. Propofol effect on cerebral oxygenation in children with congenital heart disease.

    Science.gov (United States)

    Fleck, Thilo; Schubert, Stephan; Ewert, Peter; Stiller, Brigitte; Nagdyman, Nicole; Berger, Felix

    2015-03-01

    Propofol is a short-acting, intravenously administered hypnotic agent which is used in procedural sedation in children. Propofol is known to decrease systemic vascular resistance, arterial blood pressure and can lead to desaturations and decreased systemic perfusion in children with cardiac shunting. This may result in a reduction in cerebral blood flow and oxygenation. Near-infrared spectroscopy (NIRS) can monitor cerebral tissue oxygenation in the frontal neocortex. The objective of our study was to measure the changes in cerebral oxygen and blood supply after Propofol infusion in children with congenital heart disease. Propofol infusion may reduce cerebral oxygenation in children with congenital heart disease. The study group consisted of 32 children (f:m = 18:14), with median age of 49 (5-112) months and median weight of 15 (5-34) kg. We performed NIRS derived continuous measurement of cerebral oxygenation and cardiac output using Electrical velocimetry for 5 min before and after sedation with Propofol (1-2 mg/kg i.v.) for cardiac catheterization. Simultaneously, non-invasive arterial blood pressure and transcutaneous oxygen saturation were measured. Propofol sedation led to a significant decrease in mean arterial pressure (79 ± 16 vs. 67 ± 12 mmHg) (p = 0.01) and cardiac index (3.2 ± 0.8 vs. 2.9 ± 0.6 ml/min/m(2)) (p = 0.03). In contrast, cerebral tissue oxygenation index, increased significantly from 57 ± 11 to 59 ± 10 % (p blood pressure. This may be caused by a decreased oxygen consumption of the sedated brain with intact cerebral auto-regulation. PMID:25311762

  18. Hypoplastic left heart syndrome

    Science.gov (United States)

    HLHS; Congenital heart - hypoplastic left heart; Cyanotic heart disease - hypoplastic left heart ... Hypoplastic left heart is a rare type of congenital heart disease. It is more common in males than in ...

  19. Bochdalek Congenital Diaphragmatic Hernia in an Adult Sheep.

    Science.gov (United States)

    Williams, R D; Katz, M G; Fargnoli, A S; Kendle, A P; Mihalko, K L; Bridges, C R

    2016-06-01

    Congenital diaphragmatic hernia (CDH) is a rare condition. The aetiology of CDH is often unclear. In our case, a hollow mass was noted on MRI. Cardiac ejection fraction was diminished (47.0%) compared to 60.5% (average of 10 other normal animals, P type) was made when the sheep underwent surgery. The hernia was right-sided and contained the abomasum. Lung biopsy demonstrated incomplete development with a low number of bronchopulmonary segments and vessels. The likely cause of this hernia was genetic malformation. PMID:26293994

  20. Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

    Directory of Open Access Journals (Sweden)

    Felipe Alves Mourato

    2014-06-01

    Full Text Available OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1% and 112 (81.2% were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

  1. Tracheal compression due to an elongated aortic arch in patients with congenital heart disease: evaluation using multidetector-row CT

    Energy Technology Data Exchange (ETDEWEB)

    Watanabe, Noriko; Hayabuchi, Yasunobu; Inoue, Miki; Sakata, Miho; Nabo, Manal Mohamed Helmy; Nakagawa, Ryuji; Saijo, Takahiko; Kagami, Shoji [University of Tokushima, Department of Pediatrics, Tokushima (Japan)

    2009-10-15

    The airway can become obstructed as a result of compression by an elongated aortic arch. In this study we evaluated tracheal compression using multidetector-row CT in patients with congenital heart disease and an elongated aortic arch. The trachea was measured at the level of the aortic arch in 205 children and young adults and then the severity of tracheal compression was determined by measuring the tracheal diameter ratio (short axis diameter/long axis diameter). Patients were divided as follows: group I (normal aortic arch; n=166), group II (transversely running aortic arch; n=22), and group III (elongated aortic arch; n=17). From the viewpoint of the relationship of the great arteries, group II had D-malposition, and group III had L-malposition. Age, height, weight and body surface area were significantly correlated with the short and long axis diameter in group I. There was a negative correlation between tracheal diameter ratio and the physical size parameters. The tracheal diameter ratio in group III was 0.50{+-}0.13, which was significantly lower than in groups I and II (P<0.01 and 0.05, respectively). Even apparently asymptomatic patients with an elongated aortic arch can have tracheal compression. An elongated aortic arch may be a useful predictor of tracheal compression. (orig.)

  2. Treatment of a giant congenital melanocytic nevus in the adult: review of the current management of giant congenital melanocytic nevus.

    Science.gov (United States)

    Su, Jeannie J; Chang, Daniel K; Mailey, Brian; Gosman, Amanda

    2015-05-01

    Giant congenital melanocytic nevi (GCMNs) create cosmetic disfigurements and pose risk for malignant transformation. Adult GCMN cases are uncommon because most families opt for surgical treatment during childhood. We review the current literature on GCMN and present an interesting case of an adult with a GCMN encompassing the entire back with painful nodules exhibiting gross involvement of his back musculature, without pathologic evidence of malignancy. Surgical management was deferred in childhood because of parental desires to allow the patient to make his own decision, and treatment in adulthood was pursued on the basis of the significant impairment of the patient's quality of life and self-esteem due to the massive size and deforming nature of the nevus. The treatment strategy used for this young adult male patient involved a massive en bloc excision of the GCMN with partial resection of the latissimus dorsi, followed by a 5-week staged reconstructive process using dermal regenerative matrices and split-thickness skin grafting. Because of the shift in GCMN management from early surgical management to more conservative management, we may see an increase in adult cases of GCMN. Thus, it is critical to better understand the controversy surrounding early versus delayed management of GCMN. PMID:25664413

  3. Congenital Heart Disease Genetics Uncovers Context-Dependent Organization and Function of Nucleoporins at Cilia.

    Science.gov (United States)

    Del Viso, Florencia; Huang, Fang; Myers, Jordan; Chalfant, Madeleine; Zhang, Yongdeng; Reza, Nooreen; Bewersdorf, Joerg; Lusk, C Patrick; Khokha, Mustafa K

    2016-09-12

    Human genomics is identifying candidate genes for congenital heart disease (CHD), but discovering the underlying mechanisms remains challenging. In a patient with CHD and heterotaxy (Htx), a disorder of left-right patterning, we previously identified a duplication in Nup188. However, a mechanism to explain how a component of the nuclear pore complex (NPC) could cause Htx/CHD was undefined. Here, we show that knockdown of Nup188 or its binding partner Nup93 leads to a loss of cilia during embryonic development while leaving NPC function largely intact. Many data, including the localization of endogenous Nup188/93 at cilia bases, support their direct role at cilia. Super-resolution imaging of Nup188 shows two barrel-like structures with dimensions and organization incompatible with an NPC-like ring, arguing against a proposed "ciliary pore complex." We suggest that the nanoscale organization and function of nucleoporins are context dependent in a way that is required for the structure of the heart. PMID:27593162

  4. Planning of vessel grafts for reconstructive surgery in congenital heart diseases

    Science.gov (United States)

    Rietdorf, U.; Riesenkampff, E.; Schwarz, T.; Kuehne, T.; Meinzer, H.-P.; Wolf, I.

    2010-02-01

    The Fontan operation is a surgical treatment for patients with severe congenital heart diseases, where a biventricular correction of the heart can't be achieved. In these cases, a uni-ventricular system is established. During the last step of surgery a tunnel segment is placed to connect the inferior caval vein directly with the pulmonary artery, bypassing the right atrium and ventricle. Thus, the existing ventricle works for the body circulation, while the venous blood is passively directed to the pulmonary arteries. Fontan tunnels can be placed intra- and extracardially. The location, length and shape of the tunnel must be planned accurately. Furthermore, if the tunnel is placed extracardially, it must be positioned between other anatomical structures without constraining them. We developed a software system to support planning of the tunnel location, shape, and size, making pre-operative preparation of the tunnel material possible. The system allows for interactive placement and adjustment of the tunnel, affords a three-dimensional visualization of the virtual Fontan tunnel inside the thorax, and provides a quantification of the length, circumferences and diameters of the tunnel segments. The visualization and quantification can be used to plan and prepare the tunnel material for surgery in order to reduce the intra-operative time and to improve the fit of the tunnel patch.

  5. An empowerment health education program for children undergoing surgery for congenital heart diseases.

    Science.gov (United States)

    Ni, Zhihong; Chao, Yannfen; Xue, Xiaoling

    2016-09-01

    Since the surgery for congenital heart disease (CHD) is considered highly risky, appropriate postoperative care is crucial. After the surgery, children are often discharged with unhealed wounds, incomplete recovery, and continuing pain. Health education programs based on empowerment education model can assist clients to develop skills in self-management. This study aimed to evaluate the effectiveness of an empowerment health education program for improving caregiving knowledge, caring behaviors, and self-efficacy of parents caring for children after corrective surgery for CHD. This prospective clinical trial enrolled pediatric patients undergoing surgical correction for CHD. Patients were divided into two groups: the control group (n = 42), which received the standard education program, and the intervention group (n = 44), which participated in the empowerment theory-based education program. We collected data on left ventricular ejection fraction (LVEF); peripheral oxygen saturation (SpO2); New York Heart Association classification of the patients; and the parents' caregiving knowledge, caring behaviors, and self-efficacy before surgery and one month and three months after surgery. At one month and three months after surgery, the intervention group scored higher than the control group in caregiving knowledge, caring behavior, and self-efficacy. By the third month after surgery, the intervention group had significantly higher values of LVEF and SpO2 than the control group. PMID:26105060

  6. Voxelwise atlas rating for computer assisted diagnosis: Application to congenital heart diseases of the great arteries.

    Science.gov (United States)

    Zuluaga, Maria A; Burgos, Ninon; Mendelson, Alex F; Taylor, Andrew M; Ourselin, Sébastien

    2015-12-01

    Atlas-based analysis methods rely on the morphological similarity between the atlas and target images, and on the availability of labelled images. Problems can arise when the deformations introduced by pathologies affect the similarity between the atlas and a patient's image. The aim of this work is to exploit the morphological dissimilarities between atlas databases and pathological images to diagnose the underlying clinical condition, while avoiding the dependence on labelled images. We propose a voxelwise atlas rating approach (VoxAR) relying on multiple atlas databases, each representing a particular condition. Using a local image similarity measure to assess the morphological similarity between the atlas and target images, a rating map displaying for each voxel the condition of the atlases most similar to the target is defined. The final diagnosis is established by assigning the condition of the database the most represented in the rating map. We applied the method to diagnose three different conditions associated with dextro-transposition of the great arteries, a congenital heart disease. The proposed approach outperforms other state-of-the-art methods using annotated images, with an accuracy of 97.3% when evaluated on a set of 60 whole heart MR images containing healthy and pathological subjects using cross validation.

  7. Apheresis in high risk antiphospholipid syndrome pregnancy and autoimmune congenital heart block.

    Science.gov (United States)

    Ruffatti, Amelia; Favaro, Maria; Brucato, Antonio; Ramoni, Veronique; Facchinetti, Myriam; Tonello, Marta; Del Ross, Teresa; Calligaro, Antonia; Hoxha, Ariela; Grava, Chiara; De Silvestro, Giustina

    2015-12-01

    In the first part a prospective cohort study was reported to evaluate the efficacy and safety of a treatment protocol including plasma exchange (PE) or PE plus intravenous immunoglobulins (IVIG) or immunoadsorption (IA) plus IVIG administered in addition to conventional therapy to 22 pregnant women with high-risk APS. The results indicate that PE or IA treatments administered along with IVIG and conventional antithrombotic therapy could be a valuable and safe therapeutic option in pregnant APS women with triple antiphospholipid antibody positivity along with a history of thrombosis and/or one or more severe pregnancy complications. In the second part the efficacy and safety of PE combined with IVIG and steroids were evaluated for the treatment of 10 patients with autoimmune congenital heart block (CHB) by comparing maternal features, pregnancy outcome and side effects with those of 24 CHB patients treated with steroids only. The patients treated with the combined therapy showed a statistically significant regression of 2nd degree blocks, an increase in heart rate at birth and a significantly lower prevalence of pacing in the first year of life. Moreover, no side effects were observed except for a few steroid-related events. If these results are confirmed by large-scale studies, the apheretic procedures could lead to improved outcomes in the treatment of these devastating diseases. PMID:26626966

  8. Comparison of diagnostic accuracy of dual-source CT and conventional angiography in detecting congenital heart diseases

    International Nuclear Information System (INIS)

    Cardiac dual-source computed tomography (DSCT) is primarily used for coronary arteries. There are limited studies about the application of DSCT for congenital heart diseases. The aim of this study was to determine the diagnostic value of DSCT in the cardiac anomalies. The images of DSCTs and conventional angiographies of 36 patients (21 male; mean age: 8.5 month) with congenital heart diseases were reviewed and the parameters of diagnostic value of these methods were compared. Cardiac surgery was the gold standard. A total of 105 cardiac anomalies were diagnosed at surgery. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of DSCT were 98.25%, 97.9%, 98.1%, 99.07%, and 98.2%, respectively. The corresponding values of angiography were 95.04%, 98.7%, 97.8%, 98.1%, and 98%, respectively. Only one atrial septal defect (ASD) and two patent ductus arteriosus (PDA) were missed by DSCT. Angiography missed two ASD and two PDA. DSCT also provided important additional findings (n=35) about the intrathoracic or intraabdominal organs. DSCT is a highly accurate diagnostic modality for congenital heart diseases, obviating the need for invasive modalities. Beside its noninvasive nature, the advantage of DSCT over the angiography is its ability to provide detailed anatomical information about the heart, vessels, lungs and intraabdominal organs

  9. Congenital amusia: a group study of adults afflicted with a music-specific disorder.

    Science.gov (United States)

    Ayotte, Julie; Peretz, Isabelle; Hyde, Krista

    2002-02-01

    The condition of congenital amusia, commonly known as tone-deafness, has been described for more than a century, but has received little empirical attention. In the present study, a research effort has been made to document in detail the behavioural manifestations of congenital amusia. A group of 11 adults, fitting stringent criteria of musical disabilities, were examined in a series of tests originally designed to assess the presence and specificity of musical disorders in brain-damaged patients. The results show that congenital amusia is related to severe deficiencies in processing pitch variations. The deficit extends to impairments in music memory and recognition as well as in singing and the ability to tap in time to music. Interestingly, the disorder appears specific to the musical domain. Congenital amusical individuals process and recognize speech, including speech prosody, common environmental sounds and human voices, as well as control subjects. Thus, the present study convincingly demonstrates the existence of congenital amusia as a new class of learning disabilities that affect musical abilities. PMID:11844725

  10. Value of Multidetector Computed Tomography in Evaluation of Thoracic Venous Abnormalities among Pediatrics with Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Dalia F Elbeih*, Mervat M El-Gohary *, Naglaa H Shebrya*,Mohammed A Saleh

    2013-04-01

    Full Text Available Introduction: Venous anomalies of the thorax can involve systemic or pulmonary veins and range from isolated incidental findings to components of more complex anomalies, most often congenital heart disease (CHD. Although echocardiography and catheter directed cardiac angiography are generally accepted as the primary imaging techniques for evaluation of CHD, CT and MRI are important complementary diagnostic tools. Multi-detector computed tomography (MDCT with its increasing availability and utility is now becoming a further method of imaging CHD. In light of its widespread availability, MDCT and 3D imaging are increasingly considered as a viable “one-stop shop” for preoperative imaging evaluation of cardiovascular structures in selected pediatric patients.Aim of the work: The aim of this work is to evaluate the role of MDCT in visualization of the thoracic venous system in pediatrics with congenital heart disease, show prevalence and types of venous anomalies and to compare this data with echocardiographic findings.Methods: The studied group included 30 cases referred to us by pediatric cardiologists to be examined by MSCT angiography of the heart and thoracic vessels. All the patients were known cases of congenital heart disease and underwent echocardiography. They were referred to answer specific anatomic question raised by inconclusive echocardiography, to assess suspected systemic and suspected pulmonary venous anomalies. All patients were subjected to full history taking, clinical examination and MDCT examination with CT lightspeed VCT XT 64-detectors row scanner (General Electric, Medical Systems, Milwaukee, Wisconsin, USA.Results: CTA findings had 77.8 % concordance with echocardiographic findings regarding SVC anomalies, 66.7 % concordance with echocardiography regarding IVC anomalies and 90 % concordance regarding pulmonary venous anomalies.Conclusion: Low dose protocol CTA is a promising method that complementary to Echocardioraphy

  11. Screening for Critical Congenital Heart Defects with Pulse Oximetry: Medical Aspects.

    Science.gov (United States)

    Ewer, Andrew K

    2016-09-01

    The detection of newborn babies with potentially life-threatening, critical congenital heart defects (CCHDs) before they collapse or expire remains an important clinical challenge. The absence of physical signs and the difficulty assessing mild cyanosis means that the newborn baby check misses up to a third of babies. Fetal anomaly ultrasound scanning identifies an increasing proportion, but this screen is operator-dependent and therefore highly variable; although some units report very high detection rates, overall most babies with CCHD are still missed. Pulse oximetry screening (POS) is an additional test that meets the criteria for universal screening. POS increases overall detection of CCHD to over 90% and also identifies babies with noncardiac, hypoxemic conditions (such as congenital pneumonia, early-onset sepsis, and pulmonary hypertension), which are usually included in the false positives. There is a wealth of published data on the POS, both in a research setting and more recently in routine clinical practice, and consideration of POS is becoming increasingly widespread particularly among high-income countries. But a degree of controversy still remains, and debate continues regarding the most appropriate time to screen, the most effective screening pathway, and screening outside the well-baby nursery. So, should all newborn babies be screened with POS, if so, when and where should screening take place, what saturations are acceptable, and which conditions are we trying to identify? This review will look at the available evidence and try to suggest the way forward for those considering its introduction into their clinical practice. PMID:27603536

  12. Evaluation of High Resolution Melting for MTHFR C677T Genotyping in Congenital Heart Disease.

    Directory of Open Access Journals (Sweden)

    Ying Wang

    Full Text Available High resolution melting (HRM is a simple, flexible and low-cost mutation screening technique. The methylenetetrahydrofolate reductase (MTHFR gene encoding a critical enzyme, potentially affects susceptibility to some congenital defects like congenital heart disease (CHD. We evaluate the performance of HRM for genotyping of the MTHFR gene C677T locus in CHD cases and healthy controls of Chinese Han population.A total of 315 blood samples from 147 CHD patients (male72, female 75 and 168 healthy controls (male 92, female 76 were enrolled in the study. HRM was utilized to genotype MTHFR C677T locus of all the samples. The results were compared to that of PCR-RFLP and Sanger sequencing. The association of the MTHFR C677T genotypes and the risk of CHD was analyzed using odds ratio with their 95% confidence interval (CIs from unconditional logistic regression.All the samples were successfully genotyped by HRM within 1 hour and 30 minutes while at least 6 hours were needed for PCR-RFLP and sequencing. The genotypes of MTHFR C677T CC, CT, and TT were 9.52%, 49.66%, and 40.82% in CHD group but 29.17%, 50% and 20.83% in control group, which were identical using both methods of HRM and PCR-RFLP, demonstrating the sensitivity and specificity of HRM were all 100%.MTHFR C677T is a potential risk factor for CHD in our local residents of Shandong province in China. HRM is a fast, sensitive, specific and reliable method for clinical application of genotyping.

  13. Research in congenital heart disease: a comparative bibliometric analysis between developing and developed countries.

    Science.gov (United States)

    Farhat, Theresa; Abdul-Sater, Zahi; Obeid, Mounir; Arabi, Mariam; Diab, Karim; Masri, Samer; Al Haless, Zohair; Nemer, Georges; Bitar, Fadi

    2013-02-01

    Congenital heart disease (CHD) is the most common congenital anomaly, affecting 1 % of live births. The field of pediatric cardiology has witnessed major advances over the past 25 years triggered by research initiatives focusing on CHD. However, large disparities exist in research capabilities between Arab developing nations and the developed nations. This study used bibliometric analysis to assess the contribution of the Arab countries to CHD research. To identify articles on CHD published in the Arab countries, the United States, and Europe, a systematic search was run on MEDLINE, PubMed, and Scopus. The Arab countries, with an estimated population of 362 million, published 530 research articles addressing CHD in the last 25 years (average, 1.5 articles/10(6) population). This compares with 12,936 research articles published in the United States (average, 41 articles/10(6) population) and 12,260 published in Europe (average, 24.3 articles/10(6) population). Basic research relating to genetics and animal models of CHD is emerging sparsely in the Arab world, with few articles published in high-impact-factor journals. The Arab world research output in the field of CHD per capita is substantially low, estimated to be 29 times less than in developed countries. Despite the minimal increase in published research articles in global periodicals, most of the research relating to CHD continues to be far from innovative. Regional collaborations with international linkage are starting to evolve. The research facilities in the Arab countries need to increase substantially in research and infrastructure funding to keep up with the pace of research in developing countries. PMID:22878810

  14. Self-Management of Heart Disease in Older Adults

    OpenAIRE

    Huynh-Hohnbaum, Anh-Luu T.; Marshall, Lia; Villa, Valentine M.; Lee, Gi

    2015-01-01

    The American Heart Association estimates that 81% of people who die of coronary heart disease are 65 years old or older. The leading risk health behaviors include physical inactivity, poor diet, smoking and binge drinking. Using the 2011–2012 California Health Interview Survey (CHIS), this study looked at how self-management, which includes a plan developed by a medical professional and the confidence to manage one’s disease, may decrease negative risk behaviors in older adults. The presence ...

  15. 小儿先天性心脏病封堵术后的护理体会%Nursing Experience of Children with Congenital Heart Disease after Transcatheter Closure of Congenital Heart Disease

    Institute of Scientific and Technical Information of China (English)

    曾珍; 郭文馨

    2015-01-01

    目的:探讨小儿先天性心脏病封堵术后护理要点。方法回顾性分析我科自2014年1月~2015年5月共50例小儿先天性心脏病封堵术的案例。结果手术成功,术后恢复快,无严重并发症。结论患者年龄小,体重低,护理难度大,规范有效的护理能有效预防并发症的发生,促进术后恢复。%Objective To explore the nursing points of the patients with congenital heart disease in children. Methods A retrospective analysis of 50 cases of congenital heart disease in children from January 2014 to May 2015, a total of cases of congenital heart disease. Results The operation was successful, and the recovery was fast and no serious complication. Conclusion Patients with low age, low weight, dif iculty of nursing, standardized and ef ective nursing can ef ectively prevent the occur ence of complications, promote postoperative recovery.

  16. Role of CT and MRI prior to redo sternotomy in paediatric patients with congenital heart disease

    International Nuclear Information System (INIS)

    Aim: To assess the value of computed tomography (CT) and magnetic resonance imaging (MRI) in the preoperative evaluation of congenital heart disease patients about to undergo redo sternotomy. Materials and methods: Forty-three patients (26 male, 17 female, median age 65 months) underwent contrast-enhanced CT and 24 patients (16 male, eight female, median age 89 months) underwent MRI prior to redo sternotomy for congenital heart disease. The informed consent requirement was waived by the institutional review board for this Health Insurance Portability and Accountability Act (HIPAA) compliant study. A standardized CT protocol utilizing a 64 section multidetector machine and an unenhanced and contrast-enhanced MRI multisequence protocol was used. Images were qualitatively graded and retrosternal distances were measured as part of a risk assessment score. Results: There were no statistically significant differences between the observers' grades regarding most retrosternal structures at both CT and MRI, separately (p > 0.05); furthermore, both readers found CT to be superior to MRI in qualitative grading except one reader's grades for shunts/conduits (p = 0.107). κ-values were good to excellent (κ ≥ 0.61) for most structures in qualitative grading of cases and risk assessment scores. There were no significant differences between grades of important structures using the same technique based on Friedman's test (p > 0.05). Analysis showed no difference between risk scores given to CT and MRI patients (p > 0.05). Conclusion: The present findings demonstrate the value of imaging to evaluate retrosternal anatomy. CT could be more valuable than MRI and could play a pivotal role in the surgical planning of CHD patients prior to redo operations. - Highlights: • Cardiac CT is valuable before redo sternotomy in CHD patients. • CT effectively evaluates major retrosternal structures before redo surgery. • In qualitative image grading, CT was superior

  17. Decreased plasma ADAMTS-13 activity as a predictor of postoperative bleeding in cyanotic congenital heart disease

    Directory of Open Access Journals (Sweden)

    Rosangela P.S. Soares

    2013-04-01

    Full Text Available OBJECTIVE: To analyze the preoperative plasma antigenic concentration and activity of von Willebrand factor and its main cleaving protease ADAMTS-13 in pediatric patients with cyanotic congenital heart disease undergoing surgical treatment and investigate possible correlations with postoperative bleeding. METHODS: Plasma antigenic concentrations (von Willebrand factor:Ag and ADAMTS-13:Ag were measured using enzyme-linked immunoassays. Collagen-binding assays were developed to measure biological activities (von Willebrand factor:collagen binding and ADAMTS-13 activity. The multimeric structure of von Willebrand factor was analyzed using Western immunoblotting. Demographic, diagnostic, and general and specific laboratory data and surgery-related variables were subjected to univariate, bivariate, and multivariate analysis for the prediction of postoperative bleeding. RESULTS: Forty-eight patients were enrolled, with ages ranging from 9 months to 7.6 years (median 2.5 years. The plasma concentrations of von Willebrand factor:Ag and ADAMTS-13:Ag were decreased by 65 and 82%, respectively, in the patients compared with the controls (p<0.001. An increased density of low-molecular-weight fractions of von Willebrand factor, which are suggestive of proteolytic degradation (p = 0.0081, was associated with decreased ADAMTS-13 activity, which was likely due to ADAMTS-13 consumption (71% of controls, p = 0.0029 and decreased von Willebrand factor:collagen binding (76% of controls, p = 0.0004. Significant postoperative bleeding occurred in 13 patients. The preoperative ADAMTS-13 activity of <64.6% (mean level for the group, preoperative activated partial thromboplastin time, and the need for cardiopulmonary bypass were characterized as independent risk factors for postoperative bleeding, with respective hazard ratios of 22.35 (95% CI 1.69 to 294.79, 1.096 (95% CI 1.016 to 1.183, and 37.43 (95% CI 1.79 to 782.73. CONCLUSION: Low plasma ADAMTS-13

  18. Neonatal pulse oximetry screening improves detecting of critical congenital heart disease

    Institute of Scientific and Technical Information of China (English)

    MA Xiao-jing; HUANG Guo-ying

    2013-01-01

    Objective Life-threatening critical congenital heart disease (cCHD) is often not detected in the neonatal period.Unrecognized cCHD results in high morbidity and mortality rates.As a non-invasive,convenient,quick and accurate measuring method,pulse oximetry is considered to be a promising strategy to screen for cCHD in neonates.This article is a review on the neonatal pulse oximetry screening for cCHD.Data sources Articles on neonatal pulse oximetry screening for cCHD were accessed from PubMed,using keywords including congenital heart defects,neonatal screening and oximetry.Study selection Original articles and critical reviews selected were relevant to the review's theme.Results The factors in the course of implementation,including threshold for positive pulse-oximetry screening results,the pulse oximeters used,timing of the screening,and the measuring position,influence the accuracy of the screening.It is recommended that the screening is completed on the second day of life,before hospital discharge.Motion-tolerant pulse oximeters,which can also be applied to measure the saturation in low-perfusion conditions,should be used.The probe should be put on both the right hand and on one foot.Thresholds of <95% in either limb or a difference of >3% between the limbs as a positive result may be appropriate.It should be emphasized that pulse-oximetry screening cannot be used as the only way to detect cCHD,clinical examination is also important in this situation.Cost-benefit analysis in the United Kingdom revealed it was plausible to use pulse oximetry as an adjunct to clinical examination.However,it is still controversial as to whether pulse oximetry can be used as a routine screening method for cCHD in neonates.Conclusions Neonatal pulse oximetry screening improves detection of cCHD.Further studies should be carried out before it becomes one of the routine newborn screening programs.

  19. Evaluation of Oral Prostaglandin E1 in Management of Ductus Dependent Congenital Heart Disease

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    Huseyin Altunhan

    2012-06-01

    Full Text Available Purpose: Intravenous prostaglandin E1 (PGE1 infusion is a treatment which has been proven to be effective in ductus dependent congenital heart disease. However, PGE1 is very expensive, needed continuous infusion and its supply is difficult by every center. When its long term use is necessary, these problems become more important. The aim of this study was to show whether oral PGE1could keep the ductus open or not till the supply of intravenous PGE1. Method: Ten patients, who were admitted to newborn intensive care unit with the diagnosis of ductus dependent congenital heart disease and received oral PGE1 till the supply of intravenous PGE1, were evaluated. The PO2 with the arterial blood gas analysis and SO2 levels with pulse oxymeter at skin were recorded before and after the administration of oral and intravenous PGE1. Results: The mean oral PGE1 initiation age was 5.5 hours (0.5–25, and mean administration period was 28 hours (18–46. It was observed that the PO2 and SO2 levels of patients measured 2 hours after the initiation of oral PGE1 were significantly increased compared to the levels before initiation of PGE1. The improvement in PO2 and SO2 levels continued till the initiation of intravenous PGE1. It was also observed that the PO2 and SO2 levels of patients measured 2 hours after the initiation of intravenous PGE1 were slightly increased compared to levels before initiation of intravenous PGE1. Conclusion: Although intravenous PGE1 is more effective than oral PGE1 in short term usage, oral PGE1 is also sufficiently effective in keeping the dustus open. For this reason until the intravenous PGE1 is supplied oral PGE1 may be used as an alternative treatment choice. We think that in long term use oral PGE1, which is cheaper and easy to use, could be used instead of intravenous PGE1 without need of admission to hospital and opening intravenous line. However for this further studies are needed to confirm this assumption. [Cukurova Med J

  20. Right oblique subxiphoid view for two-dimensional echocardiographic visualization of the right ventricle in congenital heart disease.

    Science.gov (United States)

    Marino, B; Ballerini, L; Marcelletti, C; Piva, R; Pasquini, L; Zacché, C; Giannico, S; De Simone, G

    1984-11-01

    Two-dimensional echocardiography in the right oblique subxiphoid view (ROSV) was used to visualize all right ventricular components in 351 patients younger than 2 years of age (100 normal and 251 with various types of congenital heart disease). The ROSV, which is anterior and parallel to the ventricular septum, displays all right-sided cardiac components, the left atrium and the aorta. This view provided the correct diagnosis in patients with pulmonary stenosis or atresia with intact ventricular septum, Ebstein's anomaly, tricuspid atresia, infundibular ventricular septal defect, tetralogy of Fallot and truncus arteriosus. In all patients studied with the ROSV, an excellent definition of morphologic characteristics, equivalent to that in the right oblique axial angiocardiographic view, was achieved. The ROSV should be used routinely to delineate the right ventricular anatomy in all infants with congenital heart disease. PMID:6496327

  1. Folate Deficiency and Folic Acid Supplementation: The Prevention of Neural-Tube Defects and Congenital Heart Defects

    Directory of Open Access Journals (Sweden)

    Andrew E. Czeizel

    2013-11-01

    Full Text Available Diet, particularly vitamin deficiency, is associated with the risk of birth defects. The aim of this review paper is to show the characteristics of common and severe neural-tube defects together with congenital heart defects (CHD as vitamin deficiencies play a role in their origin. The findings of the Hungarian intervention (randomized double-blind and cohort controlled trials indicated that periconceptional folic acid (FA-containing multivitamin supplementation prevented the major proportion (about 90% of neural-tube defects (NTD as well as a certain proportion (about 40% of congenital heart defects. Finally the benefits and drawbacks of three main practical applications of folic acid/multivitamin treatment such as (i dietary intake; (ii periconceptional supplementation; and (iii flour fortification are discussed. The conclusion arrived at is indeed confirmation of Benjamin Franklin’s statement: “An ounce of prevention is better than a pound of care”.

  2. Association between down syndrome and in-hospital death among children undergoing surgery for congenital heart disease a us population-based study

    OpenAIRE

    Evans, JM; Dharmar, M; Meierhenry, E; Marcin, JP; Raff, GW

    2014-01-01

    Background-The prevalence of Down syndrome (DS)-affected births has increased during the past 30 years; moreover, children with DS have a higher incidence of congenital heart disease compared with their peers. Whether children with DS have better or worse outcomes after repair of congenital heart disease is unclear. We sought to identify differences in in-hospital mortality after cardiac surgery in pediatric patients with and without DS using a large national database. Methods and Results-Chi...

  3. Validity of Sildenafil Test in Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease According to Clinical and Echocardiographic Parameters

    OpenAIRE

    Akbar Shahmohammadi; Paridokht Nakhostin Davari; Mohammad Yusof Aarabi Mogaddam; Akbar Molaei; Mahmood Meraji

    2009-01-01

    Background: Pulmonary arterial hypertension is a complication of most congenital heart diseases. We sought to assess the effect of sildenafil on patients suffering from pulmonary arterial hypertension in association with congenital heart disease on the basis of clinical and echocardiographic parameters and compare the catheterization and treatment results so as to evaluate the predictive value of sildenafil on the operability of patients. Methods: After primary echocardiography, 21 patients w...

  4. Congenital Aortic Stenosis and Aneurysms

    NARCIS (Netherlands)

    D. van der Linde (Denise)

    2013-01-01

    textabstractDue to improvements in pediatric cardio-thoracic surgery, anesthesia and diagnostics over the past decades, the number of adult patients with congenital heart disease (CHD) is growing. This causes an increasing demand in clinical practice for insight in long term outcome in both non-oper

  5. Micro-economic impact of congenital heart surgery: results of a prospective study from a limited-resource setting.

    Directory of Open Access Journals (Sweden)

    Manu Raj

    Full Text Available The microeconomic impact of surgery for congenital heart disease is unexplored, particularly in resource limited environments. We sought to understand the direct and indirect costs related to congenital heart surgery and its impact on Indian households from a family perspective.Baseline and first follow-up data of 644 consecutive children admitted for surgery for congenital heart disease (March 2013 - July 2014 in a tertiary referral hospital in Central Kerala, South India was collected prospectivelyfrom parents through questionnaires using a semi-structured interview schedule.The median age was 8.2 months (IQR: 3.0- 36.0 months. Most families belonged to upper middle (43.0% and lower middle (35.7% socioeconomic class. Only 3.9% of families had some form of health insurance. The median expense for the admission and surgery was INR 201898 (IQR: 163287-266139 [I$ 11989 (IQR: 9696-15804], which was 0.93 (IQR: 0.52-1.49 times the annual family income of affected patients. Median loss of man-days was 35 (IQR: 24-50 and job-days was 15 (IQR: 11-24. Surgical risk category and hospital stay duration significantly predicted higher costs. One in two families reported overwhelming to high financial stress during admission period for surgery. Approximately half of the families borrowed money during the follow up period after surgery.Surgery for congenital heart disease results in significant financial burden for majority of families studied. Efforts should be directed at further reductions in treatment costs without compromising the quality of care together with generating financial support for affected families.

  6. Transvenous cardiac resynchronization therapy in complex congenital heart diseases: dextrocardia with transposition of the great arteries after Mustard operation.

    Science.gov (United States)

    Zartner, Peter A; Wiebe, Walter; Volkmer, Marius; Thomas, Daniel; Schneider, Martin

    2009-04-01

    Cardiac resynchronization therapy revealed first promising results in patients with a congenital heart disease and a systemic right ventricle. Contrast-enhanced magnetic resonance imaging showed accessibility of the coronary sinus in an 18-year-old male patient with mirror dextrocardia, d-transposition of the great arteries and ventricular septal defect (VSD) after Mustard operation and VSD patch closure. In literatures, transvenous lead placement is discussed in this anatomical setting, with opposed position of the ventricular leads and reliable lead characteristics.

  7. Fetal circulation in left-sided congenital heart disease measured by cardiovascular magnetic resonance: a case–control study

    OpenAIRE

    Al Nafisi, Bahiyah; van Amerom, Joshua FP; Forsey, Jonathan; Jaeggi, Edgar; Grosse-Wortmann, Lars; Yoo, Shi-Joon; Macgowan, Christopher K; Seed, Mike

    2013-01-01

    Background The distribution of blood flow in fetuses with congenital heart disease (CHD) is likely to influence fetal growth, organ development, and postnatal outcome, but has previously been difficult to study. We present the first measurements of the distribution of the fetal circulation in left-sided CHD made using phase contrast cardiac magnetic resonance (CMR). Methods Twenty-two fetuses with suspected left-sided CHD and twelve normal controls underwent fetal CMR and echocardiography at ...

  8. Demonstration of value of optimizing ECG triggering for cardiovascular magnetic resonance in patients with congenital heart disease

    OpenAIRE

    Knesewitsch Thomas; Meierhofer Christian; Rieger Henrike; Rößler Jürgen; Frank Michael; Martinoff Stefan; Hess John; Stern Heiko; Fratz Sohrab

    2013-01-01

    Abstract Background Optimal ECG triggering is of paramount importance for correct blood flow quantification during cardiovascular magnetic resonance (CMR). However, optimal ECG triggering and therefore blood flow quantification is impaired in many patients with congenital heart disease (CHD) due to complex QRS patterns. Therefore, a new ECG-trigger algorithm was developed to address triggering problems due to complex QRS patterns. The aim of this study was to test this new ECG-trigger algorit...

  9. Gastric Duplication Cyst: A Rare Congenital Disease Often Misdiagnosed in Adults

    Directory of Open Access Journals (Sweden)

    Jessica Falleti

    2013-01-01

    Full Text Available Gastrointestinal duplication is a rare congenital disease which affected more commonly the ileum, while the stomach is rarely involved. Generally diagnosed in paediatric or young age, it could be difficult to suspect a gastrointestinal duplication in adults. Herein, we report a 55-year-old male with a gastric duplication cyst found on routinely checkup for chronic hepatitis and first misdiagnosed as a gastrointestinal stromal tumor (GIST; we also discuss its embryology.

  10. Heart rhythm and conduction disturbances in early postoperative period after surgical correction for congenital heart defects in infants

    Directory of Open Access Journals (Sweden)

    Bockeria L. A.

    2012-03-01

    Full Text Available Objective. The study aimed to determine the range of rhythm and conduction disturbances in early postoperative period in infants and prove the prognostic value of some pre- and intraoperative factors for development of such complications. Material and methods. The study included 235 children aged from 1 to 3 years who underwent surgeries for congenital heart diseases (CHD under cardiopulmonary bypass (CPB. Exclusion criteria were: age less than 1 year or more than 3 years, presence of confirmed heart rhythm disturbances before surgery except incomplete or complete right bundle branch block in presence of right ventricular myocardial hypertrophy, left anterior bundle branch block in presence of partial or complete atrioventricular canal defect and sinus tachycardia equal to circulatory failure (CF. Results. Ventricular arrhythmias such as premature ventricular contraction (PVC of different character were frequently seen in early postoperative period after surgical correction of CHD. They were noted in 36% of overall number postoperative arrhythmias. Supraventricular extrasystole occurred in 29% cases, different types of supraventricular tachycardias – in 17%, AV-blocks of different grades – in 10%. We noted the following predictors for postoperative rhythm disturbance development: age more than 25 months at the moment of surgery, prolonged CPB and aortic cross-clamping during ventricular septal defect (VSD (70 and 39 minutes, respectively and Fallot’s tetralogy correction (100 and 60 minutes, respectively and exceeding the critical end-point of aortic cross-clamping during atrial septal defect correction (ASD (24 minutes, presence of IIA and IIb grade for CF before surgery, surgical correction of ASD under hypothermia of less than 32 °C. Exceeding the critical end-points of CPB and aortic clamping in patients with Fallot’s tetralogy and exceeding the critical end-points of aortic clamping in children with VSD and ASD were shown to be the

  11. The Risk of Congenital Heart Anomalies Following Prenatal Exposure to Serotonin Reuptake Inhibitors—Is Pharmacogenetics the Key?

    Science.gov (United States)

    Daud, Aizati N. A.; Bergman, Jorieke E. H.; Kerstjens-Frederikse, Wilhelmina S.; Groen, Henk; Wilffert, Bob

    2016-01-01

    Serotonin reuptake inhibitors (SRIs) are often prescribed during pregnancy. Previous studies that found an increased risk of congenital anomalies, particularly congenital heart anomalies (CHA), with SRI use during pregnancy have created concern among pregnant women and healthcare professionals about the safety of these drugs. However, subsequent studies have reported conflicting results on the association between CHA and SRI use during pregnancy. These discrepancies in the risk estimates can potentially be explained by genetic differences among exposed individuals. In this review, we explore the potential pharmacogenetic predictors involved in the pharmacokinetics and mechanism of action of SRIs, and their relation to the risk of CHA. In general, the risk is dependent on the maternal concentration of SRIs and the foetal serotonin level/effect, which can be modulated by the alteration in the expression and/or function of the metabolic enzymes, transporter proteins and serotonin receptors involved in the serotonin signalling of the foetal heart development. Pharmacogenetics might be the key to understanding why some children exposed to SRIs develop a congenital heart anomaly and others do not. PMID:27529241

  12. The Risk of Congenital Heart Anomalies Following Prenatal Exposure to Serotonin Reuptake Inhibitors-Is Pharmacogenetics the Key?

    Science.gov (United States)

    Daud, Aizati N A; Bergman, Jorieke E H; Kerstjens-Frederikse, Wilhelmina S; Groen, Henk; Wilffert, Bob

    2016-01-01

    Serotonin reuptake inhibitors (SRIs) are often prescribed during pregnancy. Previous studies that found an increased risk of congenital anomalies, particularly congenital heart anomalies (CHA), with SRI use during pregnancy have created concern among pregnant women and healthcare professionals about the safety of these drugs. However, subsequent studies have reported conflicting results on the association between CHA and SRI use during pregnancy. These discrepancies in the risk estimates can potentially be explained by genetic differences among exposed individuals. In this review, we explore the potential pharmacogenetic predictors involved in the pharmacokinetics and mechanism of action of SRIs, and their relation to the risk of CHA. In general, the risk is dependent on the maternal concentration of SRIs and the foetal serotonin level/effect, which can be modulated by the alteration in the expression and/or function of the metabolic enzymes, transporter proteins and serotonin receptors involved in the serotonin signalling of the foetal heart development. Pharmacogenetics might be the key to understanding why some children exposed to SRIs develop a congenital heart anomaly and others do not. PMID:27529241

  13. The epidemiological aspects of congenital heart disease in central and southern district of Iran

    Directory of Open Access Journals (Sweden)

    Sara Amel-Shahbaz

    2014-01-01

    Full Text Available Background: Congenital heart disease (CHD is a major health problem and its prevalence is different around the world. The aim of study was determination of the epidemiological aspects of CHD in central and southern district of Iran. Materials and Methods: In this descriptive and analytical study, 3714 medical records were evaluated from March 21, 2001 to December 18, 2011. Medical records of inpatients from angiography and outpatients in the Heart Clinic of Afshar hospital (a referral hospital in center and south of Iran were the source of information. Types of CHD and demographic data including age, sex and residential location are collected. The data were analyzed by SPSS (version 17 software. Chi-square and Fisher′s exact tests were used to compare variables between groups. Results: At the study, the mean age of the patients at diagnosis time was 8.8 ± 11.6 year (at the range of one day to 76 years with median of 4 years. The percentage of females and males was 54.2 (n: 2014 and 43.8 (n: 1627, respectively. The chi-square test showed that there was significant difference in frequency of CHDs between females and males (P value < 0.0001. Ventricular septal defect (VSD was found to be the most frequent of CHDs (27%. Patent ductus arteriosus (PDA (16.8%, atrial septal defect (ASD (15.8%, pulmonary stenosis (PS (11% and Tetralogy of Fallot (TOF (8.9% were more prevalent in CHDs after VSD. Conclusions: The frequency of CHDs in female was more than male and VSD, PDA, ASD, PS, and TOF were most common in CHDs, respectively.

  14. Identification of functional mutations in GATA4 in patients with congenital heart disease.

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    Erli Wang

    Full Text Available Congenital heart disease (CHD is one of the most prevalent developmental anomalies and the leading cause of noninfectious morbidity and mortality in newborns. Despite its prevalence and clinical significance, the etiology of CHD remains largely unknown. GATA4 is a highly conserved transcription factor that regulates a variety of physiological processes and has been extensively studied, particularly on its role in heart development. With the combination of TBX5 and MEF2C, GATA4 can reprogram postnatal fibroblasts into functional cardiomyocytes directly. In the past decade, a variety of GATA4 mutations were identified and these findings originally came from familial CHD pedigree studies. Given that familial and sporadic CHD cases allegedly share a basic genetic basis, we explore the GATA4 mutations in different types of CHD. In this study, via direct sequencing of the GATA4 coding region and exon-intron boundaries in 384 sporadic Chinese CHD patients, we identified 12 heterozygous non-synonymous mutations, among which 8 mutations were only found in CHD patients when compared with 957 controls. Six of these non-synonymous mutations have not been previously reported. Subsequent functional analyses revealed that the transcriptional activity, subcellular localization and DNA binding affinity of some mutant GATA4 proteins were significantly altered. Our results expand the spectrum of GATA4 mutations linked to cardiac defects. Together with the newly reported mutations, approximately 110 non-synonymous mutations have currently been identified in GATA4. Our future analysis will explore why the evolutionarily conserved GATA4 appears to be hypermutable.

  15. Does malnutrition influence outcome in children undergoing congenital heart surgery in a developing country?

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    Eva M. Marwali

    2016-06-01

    Full Text Available Background Most children undergoing cardiac surgery forcongenital heart disease (CHD in developing countries aremalnourished. Malnutrition is known as a co-morbidity factorthat might predict and influence outcomes after surgery.Objectives To evaluate the effect of malnutrition and otherassociated risk factors on post-operative outcomes in childrenwith CHDs underwent cardiac surgery.Methods We conducted a retrospective cohort study in a singlecenter tertiary pediatric cardiac intensive care unit (PCICUin Indonesia. Our cohort included all children between 5 and36 months of age undergoing congenital heart surgery withcardiopulmonary bypass from November 2011 until February2014. Outcomes measured were the length of intubation and thelength of ICU stay. Variables for potential influence investigatedwere the nutritional status, age, gender, type of cardiac anomaly(acyanotic vs. cyanotic, Aristotle score, cardiopulmonary bypasstime, aortic cross-clamp time, and Pediatric Risk of Mortality(PRISM III score.Results Out of 249 patients included, 147 (59% showedmalnourishment on admission. Malnourished patients weresignificantly younger in age, presented with an acyanotic heartdefects, and had higher PRISM III score. Additionally, they alsohad a longer mechanical ventilation time and ICU stay thanthose with a normal nutritional status. After adjusting for variousvariables using a multiple logistic regression model it could bedemonstrated that a higher Z-score for weight to age was asignificant protective factor for the intubation time of more than29 hours with an odds ratio of 0.66 (95% CI 0.48 to 0.92, P =0.012. Non-malnourished patients had a 49% significantly higherchance for extubation with a hazard ratio of 1.49 (95% CI 1.12to 1.99, P= 0.007.Conclusion Malnourishment is clearly associated in a linearfashion with longer mechanical ventilation and ICU stay. As one ofsignificant and potentially treatable co-morbidity factors, preventionof malnourishment

  16. Population-based evaluation of a suggested anatomic and clinical classification of congenital heart defects based on the International Paediatric and Congenital Cardiac Code

    Directory of Open Access Journals (Sweden)

    Goffinet François

    2011-10-01

    Full Text Available Abstract Background Classification of the overall spectrum of congenital heart defects (CHD has always been challenging, in part because of the diversity of the cardiac phenotypes, but also because of the oft-complex associations. The purpose of our study was to establish a comprehensive and easy-to-use classification of CHD for clinical and epidemiological studies based on the long list of the International Paediatric and Congenital Cardiac Code (IPCCC. Methods We coded each individual malformation using six-digit codes from the long list of IPCCC. We then regrouped all lesions into 10 categories and 23 subcategories according to a multi-dimensional approach encompassing anatomic, diagnostic and therapeutic criteria. This anatomic and clinical classification of congenital heart disease (ACC-CHD was then applied to data acquired from a population-based cohort of patients with CHD in France, made up of 2867 cases (82% live births, 1.8% stillbirths and 16.2% pregnancy terminations. Results The majority of cases (79.5% could be identified with a single IPCCC code. The category "Heterotaxy, including isomerism and mirror-imagery" was the only one that typically required more than one code for identification of cases. The two largest categories were "ventricular septal defects" (52% and "anomalies of the outflow tracts and arterial valves" (20% of cases. Conclusion Our proposed classification is not new, but rather a regrouping of the known spectrum of CHD into a manageable number of categories based on anatomic and clinical criteria. The classification is designed to use the code numbers of the long list of IPCCC but can accommodate ICD-10 codes. Its exhaustiveness, simplicity, and anatomic basis make it useful for clinical and epidemiologic studies, including those aimed at assessment of risk factors and outcomes.

  17. Effect of esmolol on myocardial protection in pediatrics congenital heart defects

    OpenAIRE

    Fazelifar, Saeed; Bigdelian, Hamid

    2015-01-01

    Background: Although it is accepted that inducing cardioplegia is the gold standard in myocardial protection, there is still no consensus on the exact type of the cardioplegia. There are fewer studies on the type of the cardioplegia in hearts of the children than adults and they are contradictory. The effects of esmolol have been reviewed (a type of ultrashort-acting beta-adrenergic antagonist, i.e., ß-blockers) in conjunction with the cardioplegia due to the effect of the β-blockers in reduc...

  18. Distinct genetic architectures for syndromic and nonsyndromic congenital heart defects identified by exome sequencing.

    Science.gov (United States)

    Sifrim, Alejandro; Hitz, Marc-Phillip; Wilsdon, Anna; Breckpot, Jeroen; Turki, Saeed H Al; Thienpont, Bernard; McRae, Jeremy; Fitzgerald, Tomas W; Singh, Tarjinder; Swaminathan, Ganesh Jawahar; Prigmore, Elena; Rajan, Diana; Abdul-Khaliq, Hashim; Banka, Siddharth; Bauer, Ulrike M M; Bentham, Jamie; Berger, Felix; Bhattacharya, Shoumo; Bu'Lock, Frances; Canham, Natalie; Colgiu, Irina-Gabriela; Cosgrove, Catherine; Cox, Helen; Daehnert, Ingo; Daly, Allan; Danesh, John; Fryer, Alan; Gewillig, Marc; Hobson, Emma; Hoff, Kirstin; Homfray, Tessa; Kahlert, Anne-Karin; Ketley, Ami; Kramer, Hans-Heiner; Lachlan, Katherine; Lampe, Anne Katrin; Louw, Jacoba J; Manickara, Ashok Kumar; Manase, Dorin; McCarthy, Karen P; Metcalfe, Kay; Moore, Carmel; Newbury-Ecob, Ruth; Omer, Seham Osman; Ouwehand, Willem H; Park, Soo-Mi; Parker, Michael J; Pickardt, Thomas; Pollard, Martin O; Robert, Leema; Roberts, David J; Sambrook, Jennifer; Setchfield, Kerry; Stiller, Brigitte; Thornborough, Chris; Toka, Okan; Watkins, Hugh; Williams, Denise; Wright, Michael; Mital, Seema; Daubeney, Piers E F; Keavney, Bernard; Goodship, Judith; Abu-Sulaiman, Riyadh Mahdi; Klaassen, Sabine; Wright, Caroline F; Firth, Helen V; Barrett, Jeffrey C; Devriendt, Koenraad; FitzPatrick, David R; Brook, J David; Hurles, Matthew E

    2016-09-01

    Congenital heart defects (CHDs) have a neonatal incidence of 0.8-1% (refs. 1,2). Despite abundant examples of monogenic CHD in humans and mice, CHD has a low absolute sibling recurrence risk (∼2.7%), suggesting a considerable role for de novo mutations (DNMs) and/or incomplete penetrance. De novo protein-truncating variants (PTVs) have been shown to be enriched among the 10% of 'syndromic' patients with extra-cardiac manifestations. We exome sequenced 1,891 probands, including both syndromic CHD (S-CHD, n = 610) and nonsyndromic CHD (NS-CHD, n = 1,281). In S-CHD, we confirmed a significant enrichment of de novo PTVs but not inherited PTVs in known CHD-associated genes, consistent with recent findings. Conversely, in NS-CHD we observed significant enrichment of PTVs inherited from unaffected parents in CHD-associated genes. We identified three genome-wide significant S-CHD disorders caused by DNMs in CHD4, CDK13 and PRKD1. Our study finds evidence for distinct genetic architectures underlying the low sibling recurrence risk in S-CHD and NS-CHD.

  19. Rare variants in NR2F2 cause congenital heart defects in humans.

    Science.gov (United States)

    Al Turki, Saeed; Manickaraj, Ashok K; Mercer, Catherine L; Gerety, Sebastian S; Hitz, Marc-Phillip; Lindsay, Sarah; D'Alessandro, Lisa C A; Swaminathan, G Jawahar; Bentham, Jamie; Arndt, Anne-Karin; Louw, Jacoba; Low, Jacoba; Breckpot, Jeroen; Gewillig, Marc; Thienpont, Bernard; Abdul-Khaliq, Hashim; Harnack, Christine; Hoff, Kirstin; Kramer, Hans-Heiner; Schubert, Stephan; Siebert, Reiner; Toka, Okan; Cosgrove, Catherine; Watkins, Hugh; Lucassen, Anneke M; O'Kelly, Ita M; Salmon, Anthony P; Bu'lock, Frances A; Granados-Riveron, Javier; Setchfield, Kerry; Thornborough, Chris; Brook, J David; Mulder, Barbara; Klaassen, Sabine; Bhattacharya, Shoumo; Devriendt, Koen; Fitzpatrick, David F; Wilson, David I; Mital, Seema; Hurles, Matthew E

    2014-04-01

    Congenital heart defects (CHDs) are the most common birth defect worldwide and are a leading cause of neonatal mortality. Nonsyndromic atrioventricular septal defects (AVSDs) are an important subtype of CHDs for which the genetic architecture is poorly understood. We performed exome sequencing in 13 parent-offspring trios and 112 unrelated individuals with nonsyndromic AVSDs and identified five rare missense variants (two of which arose de novo) in the highly conserved gene NR2F2, a very significant enrichment (p = 7.7 × 10(-7)) compared to 5,194 control subjects. We identified three additional CHD-affected families with other variants in NR2F2 including a de novo balanced chromosomal translocation, a de novo substitution disrupting a splice donor site, and a 3 bp duplication that cosegregated in a multiplex family. NR2F2 encodes a pleiotropic developmental transcription factor, and decreased dosage of NR2F2 in mice has been shown to result in abnormal development of atrioventricular septa. Via luciferase assays, we showed that all six coding sequence variants observed in individuals significantly alter the activity of NR2F2 on target promoters.

  20. Identification of respiratory virus in infants with congenital heart disease by comparison of different methods

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    Tatiana Mitiko Kanashiro

    2011-10-01

    Full Text Available Respiratory virus infections are the main cause of infant hospitalization and are potentially severe in children with congenital heart disease (CHD. Rapid and sensitive diagnosis is very important to early introduction of antiviral treatment and implementation of precautions to control transmission, reducing the risk of nosocomial infections. In the present study we compare different techniques in the diagnosis of respiratory viruses in CHD infants. Thirty-nine samples of nasopharyngeal aspirate were obtained from CHD infants with symptoms of respiratory infection. The Multiplex PCR (Seeplex® RV 12 ACE Detection driven to the detection of 12 respiratory viruses was compared with the direct immunofluorescence assay (DFA and PCR, both targeting seven respiratory viruses. The positivity found by DFA, Multiplex and PCR was 33.3%, 51.3% and 48.7%, respectively. Kappa index comparing DFA and Multiplex, DFA and PCR and PCR and Multiplex PCR was 0.542, 0.483 and 0.539, respectively. The concordance between techniques was considered moderate. Both Multiplex PCR (p = 0.001 and PCR (p = 0.002 detected significantly more respiratory virus than DFA. As the performance of the tests may vary, the combination of two or more techniques may increase diagnostic sensitivity favoring the diagnosis of co-infections, early introduction of antiviral therapy and implementation of appropriate measures.

  1. Disorder of Sexual Development and Congenital Heart Defect in 47XYY: Clinical Disorder or Coincidence?

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    Hanane Latrech

    2015-01-01

    Full Text Available Background. 47XYY syndrome is a rare sex chromosome variation characterized by an additional Y chromosome. Most patients with 47XYY karyotype have normal phenotype. This disorder seems associated with a higher risk of developing behavioral and cognitive problems, tall stature, and infertility in adulthood. Sexual development disorder is a rare finding. We report a first case with an abnormal left coronary artery originating from the pulmonary artery in a 47XYY patient. Case. A one-month-old child was referred for ectopic testis and micropenis. Physical examination revealed facial dysmorphia, micropenis, and curvature of the penis with nonpalpable testis. Laboratory tests showed decreased total testosterone and anti-Mullerian hormone (AMH levels. Blood karyotyping revealed a 47XYY chromosomal formula. At the age of 3 months, the patient developed dyspnea and tachycardia. Echocardiography revealed an anomalous left coronary artery from pulmonary artery with left ventricular dysfunction requiring surgical revascularization by direct reimplantation of the left coronary artery system. Our second case was a 3-year-old child referred for hypospadias with nonpalpable left testicle. Physical examination showed hypertelorism. Blood karyotyping revealed a 47XYY chromosomal formula. Conclusion. To our knowledge, this is the first case of 47XYY syndrome associated with this congenital heart malformation and a sexual development disorder.

  2. A Novel Approach to Critical Congenital Heart Disease (CCHD Screening at Moderate Altitude

    Directory of Open Access Journals (Sweden)

    Erin Lueth

    2016-07-01

    Full Text Available The American Academy of Pediatrics (AAP has endorsed Critical Congenital Heart Disease (CCHD screening using pulse oximetry nationwide, but, however, acknowledges that altitude may impact failure rates and alternative algorithms may be required at high altitudes. We therefore evaluated a modified screening protocol at an altitude of 6200 feet with the hypothesis that modifications could decrease failure rates. We evaluated 2001 well, newborn infants ≥35 weeks gestation using a modified protocol, which included a lower saturation cutoff for the first screen (85% instead of the AAP recommended 90% and an oxygen hood intervention between the first two screens. Using our modified screening algorithm, we found a 0.3% failure rate, which was similar to the 0.2% sea-level rate and statistically different from the 1.1% rate identified in a recent study at similar altitude. Had the AAP protocol been used, the failure rate would have increased to 0.8%, which is similar to prior reports near this altitude. Echocardiograms were performed on failing newborns with no CCHD identified. A Birth Defects Registry Database review demonstrated one newborn with CCHD was missed after meeting AAP passing criteria. Overall, this study demonstrates that an alternative algorithm can be implemented at moderate altitude with decreased failure rate and comparable false negative rate.

  3. The role of stents in the treatment of congenital heart disease: Current status and future perspectives

    Directory of Open Access Journals (Sweden)

    Peters Bjoern

    2009-01-01

    Full Text Available Intravascular or intracardiac stenoses occur in many forms of congenital heart disease (CHD. Therefore, the implantation of stents has become an accepted interventional procedure for stenotic lesions in pediatric cardiology. Furthermore, stents are know to be used to exclude vessel aneurysm or to ensure patency of existing or newly created intracardiac communications. With the further refinement of the first generation of devices, a variety of "modern" stents with different design characteristics have evolved. Despite the tremendous technical improvement over the last 20 years, the "ideal stent" has not yet been developed. Therefore, the pediatric interventionalist has to decide which stent is suitable for each lesion. On this basis, currently available stents are discussed in regard to their advantages and disadvantages for common application in CHD. New concepts and designs developed to overcome some of the existing problems, like the failure of adaptation to somatic growth, are presented. Thus, in the future, biodegradable or growth stents might replace the currently used generation of stents. This might truly lead to widening indications for the use of stents in the treatment of CHD.

  4. Clinical classification in pediatric pulmonary arterial hypertension associated with congenital heart disease.

    Science.gov (United States)

    Zijlstra, Willemijn M H; Douwes, Johannes M; Ploegstra, Mark-Jan; Krishnan, Usha; Roofthooft, Marcus T R; Hillege, Hans L; Ivy, D Dunbar; Rosenzweig, Erika B; Berger, Rolf M F

    2016-09-01

    Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore whether this classification accurately reflects patient/disease characteristics and survival. All children with CHD from a contemporary cohort of consecutive pediatric PAH patients followed in three major referral centers (Denver, New York, the Netherlands) were characterized and classified on the basis of the latest proposed clinical classification for PAH-CHD (World Symposium on Pulmonary Hypertension, Nice, 2013). According to this classification, 24% of 134 children were classified into group 1, 14% into group 2, 19% into group 3, and 30% into group 4; 11% could not be classified. Types of CHD and hemodynamic profile differed between groups, with the highest right atrial pressure in group 4 (P classification. Pediatric PAH-CHD is a heterogeneous condition frequently associated with extracardiac, developmental factors that are believed to affect disease development. The Nice CHD classification identifies groups with specific patient/disease characteristics. However, a substantial proportion of children could not be classified. Group 3 forms a distinct disease entity. Its prognostic value could not be determined because of the low number of events. The Nice CHD classification supports clinical characterization of PAH-CHD; however, further refinement is needed to classify all children with PAH-CHD. PMID:27683607

  5. Multidisciplinary management of pregnancy in complex congenital heart disease: a model for coordination of care.

    Science.gov (United States)

    Harris, Rachel C; Fries, Melissa H; Boyle, Annelee; Adeniji-Adele, Hassan; Cherian, Zacharia; Klein, Nancy; John, Anitha S

    2014-01-01

    With advancements in medical care, many women with complex congenital heart disease (CHD) are now living into adulthood and childbearing years. The strains of pregnancy and parturition can be dangerous in such patients, and careful interdisciplinary plans must be made to optimize maternal and fetal health through this process. Several large studies have been published regarding risk prediction and medical management of pregnancy in complex CHD, though few case studies detailing clinical care plans have been published. The objective of this report is to describe the process of developing a detailed pregnancy and delivery care plan for three women with complex CHD, including perspectives from the multidisciplinary specialists involved in the process. This article demonstrates that collaboration between specialists in the fields of cardiology, anesthesiology, high-risk obstetrics, maternal fetal medicine, and neonatology results in clinically successful individualized treatment plans for the management of pregnancy in complex CHD. Multidisciplinary collaboration is a crucial element in the management of pregnancy in complex CHD. We provide a template used in three cases which can serve as a model for the design of future care plans.

  6. The role of stents in the treatment of congenital heart disease: Current status and future perspectives

    International Nuclear Information System (INIS)

    Intravascular or intracardiac stenoses occur in many forms of congenital heart disease (CHD). Therefore, the implantation of stents has become an accepted interventional procedure for stenotic lesions in pediatric cardiology. Furthermore, stents are know to be used to exclude vessel aneurysm or to ensure patency of existing or newly created intracardiac communications. With the further refinement of the first generation of devices, a variety of “modern” stents with different design characteristics have evolved. Despite the tremendous technical improvement over the last 20 years, the “ideal stent” has not yet been developed. Therefore, the pediatric interventionalist has to decide which stent is suitable for each lesion. On this basis, currently available stents are discussed in regard to their advantages and disadvantages for common application in CHD. New concepts and designs developed to overcome some of the existing problems, like the failure of adaptation to somatic growth, are presented. Thus, in the future, biodegradable or growth stents might replace the currently used generation of stents. This might truly lead to widening indications for the use of stents in the treatment of CHD

  7. Respiratory syncytial virus prevention in children with congenital heart disease: who and how?

    Directory of Open Access Journals (Sweden)

    Nam Kyun Kim

    2011-05-01

    Full Text Available Respiratory syncytial virus (RSV is a major cause of respiratory infection in children. Most of the pediatric population have RSV infection before the age of 2, and recurrent infections are common even within one season. Chronic lung disease, prematurity, along with congenital heart disease (CHD are major risk factors in severe lower respiratory infection. In hemo-dynamically significant CHD patients with RSV infection, hospitalization is usually needed and the possibility of treatment in intensive care unit and the use of mechanical ventilator support are known to increase. Therefore the prevention of RSV infection in CHD patients is mandatory. The current standard for RSV prevention is immunoprophylaxis by palivizumab. Immunoprophylaxis is recommended monthly in hemodynamically significant CHD patients, up to 5 months. Motabizumab, a second generation drug and newly developing RSV vaccines are also expected to play a key role in RSV prevention in the future. The prophylaxis of RSV infection in CHD patients is cost-effective in both the medical aspect of the patients as well as the socio-economic aspect. Therefore an effort to promote prevention should be made by not only the family of the patients but also by the government.

  8. [MORPHO-FUNCTIONAL CHANGES OF THYMUS TISSUES IN CHILDREN WITH CONGENITAL HEART DISEASE].

    Science.gov (United States)

    Loginova, N P; Chetvertnykh, V A; Chemurziyeva, N V

    2016-01-01

    Biopsy specimens of the thymus were studied in children aged under 11 months (n = 77) with congenital heart defects and circulatory hypoxia of varying severity. Histological sections were stained with hematoxylin-eosin and Shubich's method (to demonstrate mast cells). The expression of Ki-67, CD3 and CD34 was assessed by immunohistochemistry. The ultrastructure of thymic tissues was also examined. It was found that the severity of hypoxia determined the morphological changes in the organ associated with a development of large complex of tissue reactions. A disruption of internal structure and a loss of integrity of epithelio-reticular cells and thymocytes were demonstrated in ultrathin sections. Thymocyte proliferation index (Ki-67) and thymocytopoiesis intensity (CD3+) were reduced in all the zones of the thymus. The degree of hypoxia affected the redistribution of CD3+ lymphocytes leading to their accumulation in the medulla. The processes of endogenous regeneration took place which involved the cells of fibroblastic line and progenitor cells (CD34+) together with active formation of new blood vessels. These findings suggest that the morphological changes identified in the tissues of the thymus are a manifestation of tissue adaptation to hypoxia of varying severity under conditions of endogenous regeneration, simultaneously reflecting the processes of substitution cytogenesis. PMID:27487665

  9. Endothelial dysfunction and atherosclerosis in children with irreversible pulmonary hypertension due to congenital heart disease

    International Nuclear Information System (INIS)

    To assess endothelial dysfunction and the risk for coronary atherosclerosis in children with irreversible pulmonary hypertension due to congenital heart disease (CHD). The study included 18 cyanotic patients (the mean age was 12.28 ± 3.26 years) who developed irreversible pulmonary hypertension due to cyanotic and acyanotic CHDs, and 18 control patients (the mean age was 11.78 ± 3.00 years). Study groups were compared for flow-mediated dilatation (FMD), carotid intima media thickness (CIMT) and atherosclerotic risk factors. Compared to the control group, the mean FMD was significantly reduced in the cyanotic group (5.26 ± 2.42% and 9.48 ± 2.60%, respectively; P-value < 0.001). No significant difference was observed between the groups in CIMT (0.41 ± 0.08 mm and 0.39 ± 0.06 mm, respectively; P-value = 0.299). The levels of total cholesterol, low-density lipoprotein–cholesterol and very low-density lipoprotein–cholesterol were statistically significantly lower compared tothe control group (P-value = 0.001, 0.006 and 0.014, respectively), whereas no statistically significant difference was found in the levels of high-density lipoprotein–cholesterol and triglycerides (P-value = 0.113 and 0.975, respectively). Systemic endothelial dysfunction in children with irreversible pulmonary hypertension due to CHD was noted but there was no increased risk for atherosclerosis

  10. Newborn Critical Congenital Heart Disease Screening Using Pulse Oximetry: Nursing Aspects.

    Science.gov (United States)

    Hom, Lisa A; Martin, Gerard R

    2016-09-01

    Congenital heart disease (CCHD) is the most common birth defect. Screening for the most critical forms (CCHD) using pulse oximetry was added to the Recommended Uniform Screening Panel in the United States in 2011. Since then, CCHD screening has become nearly universal in the United States. Nurses are ideally situated to contribute to the development of best practices for implementation and provide education to families on CCHD screening. Much of the standardization, advocacy, and development of national recommendations occurred with key input from nurses. Nurses often have responsibility for educating parents, performing the screening, interpreting the screening algorithm, and the documentation of results. The nurse role often includes implementing follow-up quality improvement initiatives to ensure that systematic and accurate screening occurs. Smooth implementation can be achieved by identifying champions early, obtaining input from a multidisciplinary team including both physician and nursing leaders, and identifying ways to integrate screening into already existing workflow. By knowing the basics of why screening is important, how to screen, current recommendations on the follow-up for positive screens and the limitations of CCHD screening, nurses can advocate for their patients and positively impact outcomes for infants born with CCHD through early identification before discharge. PMID:27603538

  11. Relationship Between Polymorphism of Cystathionine beta Synthase Gene and Congenital Heart Disease in Chinese Nuclear Families

    Institute of Scientific and Technical Information of China (English)

    XIAO-MING SONG; XIAO-YING ZHENG; WEN-LI ZHU; LEI HUANG; YONG LI

    2006-01-01

    Objective To study the relationship between polymorphism of cystathionine beta synthase (CBS) gene and development of congenital heart disease (CHD). Methods One hundred and twenty-seven CHD case-parent triads were recruited from Liaoning Province as patient group, and 129 healthy subjects without family history of birth defect were simultaneously recruited as control group together with their biological parents. For all subjects the polymorphism of CBS gene G919A locus was examined by PCR-ARMS method. Results The frequencies of three genotypes (w/w, w/m, and m/m) in control group were 27.2%, 58.4%, and 14.4%, respectively, with no significant difference in gender. A significant difference in the allele frequency was found between CHD patients and controls, the wild allele frequency was 67.9% in patients and 55.7% in controls.CHD parents' genotype distribution was significantly different from that in controls. Further comparison of each type of CHD showed that genotype frequencies in several CHD subtypes were significantly different from those in their corresponding controls. The results of TDT analysis showed that no allele transmission disequilibrium existed in CHD nuclear families.Conclusions CBS gene G919A mutation is associated with the development of CHD, and the mutated allele may decrease the risk of CHD.

  12. Significance of adrenomedullin under cardiopulmonary bypass in children during surgery for congenital heart disease.

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    Takeuchi M

    2001-08-01

    Full Text Available To elucidate the effect of adrenomedullin (AM on fluid homeostasis under cardiopulmonary bypass (CPB, we investigated the serial changes in plasma AM and other parameters related to fluid homeostasis in 13 children (average age, 28.2 months with congenital heart disease during cardiac surgery under CPB. Arterial blood and urine samples were collected just after initiation of anesthesia, just before commencement of CPB, 10 min before the end of CPB, 60 min after CPB, and 24 h after operation. Plasma AM levels increased significantly 10 min before the end of CPB and decreased 24 h after operation. Urine volume increased transiently during CPB, which paralleled changes in AM. Simple regression analysis showed that plasma AM level correlated significantly with urinary vasopressin, urine volume, urinary sodium excretion, and plasma osmolarity. Stepwise regression analysis indicated that urine volume was the most significant determinant of plasma AM levels. Percent rise in AM during CPB relative to control period correlated with that of plasma brain natriuretic peptide (r = 0.57, P < 0.01. Our results suggest that AM plays an important role in fluid homeostasis under CPB in cooperation with other hormones involved in fluid homeostasis.

  13. Care of the Family of an Infant With a Congenital Heart Defect During the NICU Hospitalization.

    Science.gov (United States)

    Milford, Cheryl A

    2016-01-01

    The family of an infant born with a congenital heart defect is challenged by both the short- and long-term implications of the diagnosis and the neonatal intensive care unit (NICU) hospitalization. Nurses are in a key position to support these families as they deal with the psychological, emotional, and financial impact of the NICU experience. Understanding how families perceive the NICU environment and their grief in losing the desired healthy baby provides the NICU nurse with the knowledge to engage in self-reflection on her or his interpersonal style and caregiving attitudes. Utilizing the concepts and principles of family-centered care and relationship-based practice, nurses can work together with the families to determine how to best meet the families' needs and to find the resources to support them. Families and colleagues appreciate nurses who demonstrate expertise in this approach to family-centered care. This appreciation leads to greater job satisfaction and decreased job-related stress. PMID:26813393

  14. Obstructive airway lesion in infants and small children with congenital heart disease

    International Nuclear Information System (INIS)

    New 133Xe lung function test which is applicable in infants and small children was designed and successfully applied to the patient from 6 months to 10 years of age. 133Xe washout curve was analysed by back projection method into fast and slow compartment and 3 washout indices was calculated from the data of slow compartment. These washout indices which express the grade of washout delay of poorly ventilated area of the lung made it clear that the obstructive airway lesion is present in the patient who has a sign of respiratory failure. Washout indices well correlated with the grade of alveolar septal hypertrophy (correlation coefficient = index 1:0.842, index 2:0.632, index 3:0.864) and also well correlated with the numbers of postoperative day during which intensive respiratory care was necessary (correlation coefficient = index 1:0.686, index 2:0.648, index 3:0.736). So that it can be concluded that washout indices will diagnose the alveolar histopathological change which is seen in patient with congenital heart disease without having a biopsy and give a usefull information when the planning of postoperative respiratory care is made up. (J.P.N.)

  15. Interventional treatment of common congenital heart diseases: the common view of Chinese medical experts. Part Three; interventional treatment of patent ductus arteriosus

    International Nuclear Information System (INIS)

    Patent ductus arteriosus (PDA) represents 10%-20% of all congenital heart diseases. Percutaneous transcatheter device closure of PDA has been an effective alternative to surgical treatment and has been increasingly becoming the treatment of choice for most PDAs in children and adults. The indication for percutaneous closure of PDA has been further expanded with the development of domestic devices in China and almost all the patients with PDA can be cured by this therapy. This chapter will systematically describe the indications, contraindications for percutaneous PDA occlusion, how to close PDAs by using coils and umbrella devices, procedural complications and preventions. How to treat infant PDA, PDA with severe pulmonary arterial hypertension as well as the post-surgery residual shunt will be also discussed in detail in this chapter. (authors)

  16. Is surgery recommended in adults with neglected congenital muscular torticollis? A prospective study

    Directory of Open Access Journals (Sweden)

    Sharifi Reza

    2008-11-01

    Full Text Available Abstract Background Congenital muscular torticollis is the third most common congenital musculoskeletal anomaly after dislocation of the hip and clubfoot. When diagnosed early, it is obvious that it can be managed with good or excellent results. The aim of this prospective study was to determine the efficacy of surgery in neglected adult cases. Methods From January 2003 to June 2007, 18 adult skeletally matured patients were surgically treated for neglected congenital muscular torticollis and prospectively followed (at least one year. Bipolar release was performed in all patients. Radiography and the modified Lee's scoring system which included function and cosmesis, were used to measure the surgical results. Complications were also recorded. Results Four cases were lost during follow-up. Of the remaining 14 patients, 10 cases were males and 4 females. The age at operation ranged from 18 to 32 (average 21.9 years. The mean follow-up period was 2.5 years (range 1–5 years. Excellent results were noted in 7 patients, good in 5, and poor in 2 patients. Significant improvement (>10° of the cervico-thoracic scoliosis was noted only in 3 of 10 patients. Conclusion Patients with congenital muscular torticollis can benefit from surgical treatment even in adulthood. Surgical bipolar sectioning of the sternocleidomastoid muscle should be considered even in adults with irreversible facial and skeletal deformities. The surgery restores the range of neck motion and resolves the head tilt; therefore it can improve the quality of life. This procedure is an effective and relatively complication-free method.

  17. Effect of Folic Acid Food Fortification in Canada on Congenital Heart Disease Subtypes

    Science.gov (United States)

    Joseph, K.S.; Luo, Wei; León, Juan Andrés; Lisonkova, Sarka; Van den Hof, Michiel; Evans, Jane; Lim, Ken; Little, Julian; Sauve, Reg; Kramer, Michael S.

    2016-01-01

    Background: Previous studies have yielded inconsistent results for the effects of periconceptional multivitamins containing folic acid and of folic acid food fortification on congenital heart defects (CHDs). Methods: We carried out a population-based cohort study (N=5 901 701) of all live births and stillbirths (including late-pregnancy terminations) delivered at ≥20 weeks’ gestation in Canada (except Québec and Manitoba) from 1990 to 2011. CHD cases were diagnosed at birth and in infancy (n=72 591). We compared prevalence rates and temporal trends in CHD subtypes before and after 1998 (the year that fortification was mandated). An ecological study based on 22 calendar years, 14 geographic areas, and Poisson regression analysis was used to quantify the effect of folic acid food fortification on nonchromosomal CHD subtypes (n=66 980) after controlling for changes in maternal age, prepregnancy diabetes mellitus, preterm preeclampsia, multiple birth, and termination of pregnancy. Results: The overall birth prevalence rate of CHDs was 12.3 per 1000 total births. Rates of most CHD subtypes decreased between 1990 and 2011 except for atrial septal defects, which increased significantly. Folic acid food fortification was associated with lower rates of conotruncal defects (adjusted rate ratio [aRR], 0.73, 95% confidence interval [CI], 0.62–0.85), coarctation of the aorta (aRR, 0.77; 95% CI, 0.61–0.96), ventricular septal defects (aRR, 0.85; 95% CI, 0.75–0.96), and atrial septal defects (aRR, 0.82; 95% CI, 0.69–0.95) but not severe nonconotruncal heart defects (aRR, 0.81; 95% CI, 0.65–1.03) and other heart or circulatory system abnormalities (aRR, 0.98; 95% CI, 0.89–1.11). ConclusionS: The association between food fortification with folic acid and a reduction in the birth prevalence of specific CHDs provides modest evidence for additional benefit from this intervention. PMID:27572879

  18. Anti-La (SS-B) but not anti-Ro52 (SS-A) antibodies cross-react with laminin--a role in the pathogenesis of congenital heart block?

    Science.gov (United States)

    Li, J M; Horsfall, A C; Maini, R N

    1995-03-01

    Cross-reactions between maternally derived autoantibodies and fetal cardiac antigens have been postulated to play a role in the pathogenesis of congenital heart block (CHB). We have explored the cross-reactivity of autoantibodies to the small ribonuclear autoantigens, La/SS-B and Ro/SS-A, with laminin, the major component of cardiac sarcolemmal membrane using affinity-purified antibodies from patients with Sjögren's syndrome (SS). Anti-La antibodies purified from eight of 10 patients cross-reacted significantly with mouse laminin by ELISA. In contrast, purified antibodies to Ro52 from the same 10 patients showed little or no binding to laminin. Laminin inhibited up to 70% binding of anti-La antibodies to La antigen, and La inhibited up to 65% binding of anti-La antibodies to laminin. The cross-reaction was further examined on cryosections of 10 human fetal hearts aged from 8.7 to 14.9 weeks of gestation, two normal adult hearts, and one pathological adult heart with a diagnosis of dilated cardiomyopathy. Anti-Ro52 antibodies did not bind to the surface of cardiac cells. However, anti-La antibodies from seven of 10 patients tested bound to the surface of fetal myocytes from hearts aged 9.4 to 14.9 weeks of gestation, and also to the myocytes from the pathological adult heart but not to normal adult hearts. Preincubation with La antigen abolished the binding of anti-La antibodies to the surface of adult heart myocytes with dilated cardiomyopathy, and pre-incubation with mouse laminin could partially block this binding. These results suggest that molecular mimicry between laminin and La, but not Ro52, may act as a target for specific maternal autoantibodies, and contribute to the pathogenesis of CHB at a critical stage during fetal cardiac development. PMID:7882552

  19. Data analysis of magnetocardiograms stored in the international database and their importance in diagnosing congenital heart disease

    Science.gov (United States)

    Grot, Przemyslaw; Dunajski, Zbigniew

    2003-10-01

    Fetal magnetocardiography - a modern method of measuring electroactivity of fetal's heart is gaining more popularity interest among scientists and doctor's. For the method to become a useful tool in diagnosing congenital heart disease there has been created an international database composed of data taken from various scientific centers in the world (e.g. Institute of Precision and Biomedical Engineering of Warsaw University of Technology). Cardiograms which are recorded during fMCG (fetal magnetocardiography) and fECG (fetal electrocardiography) examination can be used to classify if the fetal heart is developing in healthy or pathological way. Thus, it has been extremely vital to create the universal and univocal pattern of discrimination between healthy or pathological fetuses. In order to do this an international database with normal values of the parameters describing the cardiograms was needed. The database is a compilation of data measured at various centers using different but similar devices and different signal processing techniques. This work analyses helpfulness of the international database in establishing the universal pattern which in future could serve as the basis for the proper congenital heart disease diagnosis.

  20. Evaluation of Congenital Valvular Heart Diseases by the Pediatrician: When to Follow, When to Refer for Intervention?

    Science.gov (United States)

    Singhi, Anil Kumar; Kumar, Raman Krishna

    2015-11-01

    Isolated congenital valvular heart disease in children constitutes a small fraction of congenital heart diseases. Valve involvement is seen more along with other congenital diseases. The most commonly involved valve is the pulmonary valve followed by the aortic valve. Stenotic lesions of the pulmonary and aortic valves are more frequently encountered than mitral and tricuspid valvular lesions. The presentation depends on the severity of the lesion and the age of the patient. Symptoms range from asymptomatic status to florid symptoms of valve obstruction and/or leak. Detailed clinical assessment and various imaging techniques confirm the diagnosis and help in management planning. Transcatheter balloon dilatation for obstructive pulmonary valve has very good long-term outcomes. The results of balloon dilation of aortic valve are also good enough for it to be the treatment of choice. Significant lesions of the mitral and tricuspid valve, regurgitant lesions, sub and supra valvular obstructions require surgical correction. Most valvar lesions mandate regular follow up. Communication and coordination between the pediatric cardiologist and the pediatrician helps in the optimal management. PMID:26362423

  1. Ellis-van Creveld syndrome and congenital heart defects: presentation of an additional 32 cases.

    Science.gov (United States)

    Hills, Christine B; Kochilas, Lazaros; Schimmenti, Lisa A; Moller, James H

    2011-10-01

    Ellis-van Creveld (EVC) syndrome is a rare genetic abnormality that has been linked to a mutation in the EVC or EVC2 genes. Common atrium (CA) is an uncommon cardiac malformation, and yet it is commonly found in patients with EVC. We performed a retrospective review of the cases submitted to the Pediatric Cardiac Care Consortium (PCCC) between 1982 and 2007. A review of the English-language literature for previously published cases, as well as current genetic research findings, was also performed. Thirty-two pediatric patients with congenital heart disease (CHD) and EVC syndrome were identified in the PCCC database. Twenty-eight (88%) had an endocardial cushion defect, with 15 of these having primary failure of atrial septation resulting in CA. Persistent left superior vena cava (LSVC) and pulmonary venous connection abnormalities were common. The incidence of persistent LSVC and pulmonary venous abnormalities were greater than previously reported for patients with EVC. Our study reviews the reported literature and adds 32 additional cases from the PCCC database. Review of the cardiac phenotype in patients with EVC syndrome reveals a characteristic pattern of atrioventricular canal defects with systemic and pulmonary venous abnormalities. The frequent association of these abnormalities is strongly reminiscent of the cardiac phenotype found in patients with heterotaxy syndromes. Emerging molecular and developmental studies suggest that EVC and EVC2 proteins may be important for cilia function, which is implicated in the pathogenesis of heterotaxy syndromes. It is speculated that coordinate function between the EVC proteins is required for a cilia-dependent cardiac morphogenesis.

  2. Self-representation of children suffering from congenital heart disease and maternal competence

    Directory of Open Access Journals (Sweden)

    Giovanna Perricone

    2013-02-01

    Full Text Available Child development may be subject to forms of motor, physical, cognitive and self-representation impairments when complex congenital heart disease (CHD occurs. In some cases, inadequacy of both self-representation as well as the family system are displayed. It seems to be important to search the likely internal and external resources of the CHD child, and the possible connections among such resources, which may help him/her to manage his/her own risk condition. The research project inquires the possible resources related to the self-representation and self-esteem levels of the CHD child, and those related to maternal self-perception as competent mothers. A group of 25 children (mean age = 10.2; SD=1.8 suffering from specific forms of CHD, and a group made up of their relative mothers (mean age = 38.2; SD=5 were studied. The tools used were the Human Figure Drawing, to investigate child body-related self-representation; the TMA scale (Self-esteem Multidimensional Test, to investigate the child’s self-esteem; and the Q-sort questionnaire, to assess how mothers perceived their maternal competence. Data concerning the likely correlations between the child’s self-representation and the maternal role competence show [that] positive correlations between some indicators of maternal competence, specific aspects of CHD children’s self-representation (mothers’ emotional coping and children’s self-image adequacy and self-esteem (mothers’ emotional scaffolding and children’s self-esteem at an emotional level. By detecting the occurrence of specific correlations among resources of both child and mother, the study provides cardiologists with information that is useful for building a relationship with the families concerned, which would seem to enhance the quality of the process of the cure itself.

  3. Self-representation of children suffering from congenital heart disease and maternal competence.

    Science.gov (United States)

    Perricone, Giovanna; Polizzi, Concetta; De Luca, Francesco

    2013-02-01

    Child development may be subject to forms of motor, physical, cognitive and self-representation impairments when complex congenital heart disease (CHD) occurs. In some cases, inadequacy of both self-representation as well as the family system are displayed. It seems to be important to search the likely internal and external resources of the CHD child, and the possible connections among such resources, which may help him/her to manage his/her own risk condition. The research project inquires the possible resources related to the self-representation and self-esteem levels of the CHD child, and those related to maternal self-perception as competent mothers. A group of 25 children (mean age = 10.2; SD=1.8) suffering from specific forms of CHD, and a group made up of their relative mothers (mean age = 38.2; SD=5) were studied. The tools used were the Human Figure Drawing, to investigate child body-related self-representation; the TMA scale (Self-esteem Multidimensional Test), to investigate the child's self-esteem; and the Q-sort questionnaire, to assess how mothers perceived their maternal competence. Data concerning the likely correlations between the child's self-representation and the maternal role competence show [that] positive correlations between some indicators of maternal competence, specific aspects of CHD children's self-representation (mothers' emotional coping and children's self-image adequacy) and self-esteem (mothers' emotional scaffolding and children's self-esteem at an emotional level). By detecting the occurrence of specific correlations among resources of both child and mother, the study provides cardiologists with information that is useful for building a relationship with the families concerned, which would seem to enhance the quality of the process of the cure itself. PMID:23667730

  4. Cardiopulmonary Bypass Down-Regulates NOD Signaling and Inflammatory Response in Children with Congenital Heart Disease

    Science.gov (United States)

    Li, Yi Ping; Huang, Shungen; Zhou, Huiting; Xie, Yi; Pan, Jian; Li, Yanhong; Wang, Jiang Huai; Wang, Jian

    2016-01-01

    In the present study, we aimed to examine the impact of cardiopulmonary bypass (CPB) on expression and function of NOD1 and NOD2 in children with congenital heart disease (CHD), in an attempt to clarify whether NOD1 and NOD2 signaling is involved in the modulation of host innate immunity against postoperative infection in pediatric CHD patients. Peripheral blood samples were collected from pediatric CHD patients at five different time points: before CPB, immediately after CPB, and 1, 3, and 7 days after CPB. Real-time PCR, Western blot, and ELISA were performed to measure the expression of NOD1 and NOD2, their downstream signaling pathways, and inflammatory cytokines at various time points. Proinflammatory cytokine IL-6 and TNF-α levels in response to stimulation with either the NOD1 agonist Tri-DAP or the NOD2 agonist MDP were significantly reduced after CPB compared with those before CPB, which is consistent with a suppressed inflammatory response postoperatively. The expression of phosphorylated RIP2 and activation of the downstream signaling pathways NF-κB p65 and MAPK p38 upon Tri-DAP or MDP stimulation in PBMCs were substantially inhibited after CPB. The mRNA level of NOD1 and protein levels of NOD1 and NOD2 were also markedly decreased after CPB. Our results demonstrated that NOD-mediated signaling pathways were substantially inhibited after CPB, which correlates with the suppressed inflammatory response and may account, at least in part, for the increased risk of postoperative infection in pediatric CHD patients. PMID:27622570

  5. The Prevalence of Celiac Disease in Down syndrome Children with and without Congenital Heart Defects

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    Noor Mohammad Noori

    2016-07-01

    Full Text Available Background The prevalence of celiac disease (CD is remarkably varied in Down syndrome(DSpatientscompared with other diseases.  This study aimed to assess celiac disease prevalence in Down syndrome children with and without congenital heart defects (CHD and its comparison with controls. Materials and Methods This case-control study was performed at a single center on 132 participants in three groups. Clinical and genetic tests were performed on all patients suspected with Down syndrome to confirm their diseases.  After that in patients with confirmed Down syndrome echocardiography was carried out to diagnosis of CHD. Healthy children selected randomly among those who referred to the center for annual check-up. Statistical evaluation was done using SPSS-16. Results For the factors of age, weight, height and Body Mass Index (BMI not observed significant differences between three groups of participants, but it would be observed statistically differences for the variable of tTG- IgA.  For variables of weight, tTG- IgA and BMI was observed statistically different in the case and controls. The status of tTG- IgA (normal or 20 had significant correlation with three groups of controls, Down syndrome with and without CHD. The status of tTG- IgA also had significant correlation with groups of case and controls. In comparison of tTG- IgA in DS patients with and without CHD, no significant differences were observed. Conclusion The prevalence of CD in DS patients was higher compared the controls population; and in DS patients with CHD was higher compared the DS patients without CHD.

  6. Assessment of Pulmonary Artery Stiffness of Repaired Congenital Heart Disease Patients

    Science.gov (United States)

    Lee, Namheon; Banerjee, Rajit; Taylor, Michael; Hor, Kan

    2012-10-01

    Surgical correction or palliation of congenital heart disease (CHD) often requires augmenting the main pulmonary artery (MPA) with non-native material or placing a cylindrical graft. The degree to which this intervention affects PA compliance is largely unknown. In this study, the MPA stiffness characteristics were assessed by its compliance, distensibility, and pressure-strain modulus. Coregistered velocity encoded phase-contrast MRI and cardiac catheterization data were available for a cohort of repaired CHD patients (n=8) and controls (n=3). All patients were repaired with either an RV-PA conduit or a RV outflow tract patch. We measured the MPA area change by MRI and MPA pressure during the cath. The measurements were taken through or just distal to the conduit. The MPA compliance and distensibility for the patients were significantly lower than the controls: compliance (9.8±10.8 vs 28.3±7.7mm^2/mmHg, p<0.05), distensibility (2.2±1.5 vs 6.6±2.1%Area change/mmHg, p=0.05). The patients had a significantly higher pressure-strain modulus (152.3±116.4mmHg, p<0.05) than the controls (35.8±10.6mmHg). The abnormally elevated PA stiffness due to the rigidity of the conduit or patch material may cause a compliance mismatch resulting in high stress levels contributing to the observed progressive PA dilatation. This may be a factor in the progressive RV dilatation seen in this cohort of repaired CHD patients.

  7. Congenital Heart Disease (CHD) and Environmental Physical Activity, Kaunas, 1995-2005.

    Science.gov (United States)

    Stoupel, E.; Dulskiene, V.; Kuciene, R.; Abramson, E.; Israelevich, P.; Sulkes, J.

    2009-12-01

    Recent studies described a number of fetal development sides related to the environmental physical activity. The aim of this study was to check the possible links between congenital heart disease (CHD) born in a non-selected medical network and indices of environmental physical activity. Children born with CHD in Kaunas, Lithuania, in years 1995-2005 were analyzed at the end of the first year of life (including also those died after birth from this condition). Monthly distribution of CHD (total - 371, both gender (178 boys and 193 girls), 41435 births) were compared with parameters of solar (SA), geomagnetic (GMA) and cosmic ray (CRA) activity, as well as the year, at the month of birth, 9 months before and at year of birth and one year before. CRA was represented by neutron activity on the Earth's surface. Heliogeophysical data were obtained from space research centers in the USA, Russia and Finland. There was found a significant correlation between yearly number of births (r = - 0.9, p = 0.00012). Monthly number of CHD was correlated with SA and CRA often highly at the beginning of pregnancy both in monthly and yearly (r = - 0.7, p = 0.025 for SA, r = 0.8, p = 0.005 for CRA) comparison. For boys the correlation was stronger, but also it was significant for girls. GMA has not shown significant effects. It is concluded that the number of yearly and monthly CHD is connected with SA and CRA in pregnancy. Boys show high levels in these correlations. The mechanism of the cosmophysical effects on human development and temporal distribution of CHD deserve special studies.

  8. Polymorphism of Methionine Synthase Gene in Nuclear Families of Congenital Heart Disease

    Institute of Scientific and Technical Information of China (English)

    WEN-LI ZHU; JUN CHENG; JING-JING DAO; RU-BING ZHAO; LI-YING YAN; SHU-QING LI; AND YONG LI

    2004-01-01

    Objective To investgate the relation of methionine synthase (MS) gene variation with congenital heart disease (CHD) phenotype. Methods One hundred and ninety three CHD patients (94 males and 99 females) and their biological parents (nuclear families) in Liaoning Province were selected as the case group, and another 104 normal persons (60 males and 44 females) and their parents without family history of birth defects as the control group. For all subjects the polymorphism of MS gene A2756G locus was examined by PCR-RFLP method. Results In offspring of the control group the frequencies of MS genotype (+/-) and allele (+) were 10.7% and 5.3%, without existence of homozygote. The MS genotype distribution and allele frequencies of CHD patients and their mothers were not significantly different from the control (P > 0.05). The frequency of allele (+)in case fathers (5.0 %) was apparently lower than that in the control (9.1%, P=0.060), and the odds ratio (OR) was 0.53 (95% CI: 0.25-1.09). There was no difference in parents' genotype combination between the two groups, and in genotype distribution among different types of CHD. Analysis of genetic transmission indicated that mutation allele (+) existed transmission disequilibrium in CHD nuclear families. The percentage of allele (+) transmitted from parents was lower than that allele (-)with OR 0.26 (95% CI: 0.11-0.60). Conclusion MS gene variation in parents is associated with occurrence of CHD in offspring, and mutation allele (+) in parents may be related with the decrease of CHD risk in offspring.

  9. Endothelial dysfunction and atherosclerosis in children with irreversible pulmonary hypertension due to congenital heart disease

    Directory of Open Access Journals (Sweden)

    Murat Çiftel

    2012-01-01

    Full Text Available Objective: To assess endothelial dysfunction and the risk for coronary atherosclerosis in children with irreversible pulmonary hypertension due to congenital heart disease (CHD. Methods: The study included 18 cyanotic patients (the mean age was 12.28 ± 3.26 years who developed irreversible pulmonary hypertension due to cyanotic and acyanotic CHDs, and 18 control patients (the mean age was 11.78 ± 3.00 years. Study groups were compared for flow-mediated dilatation (FMD, carotid intima media thickness (CIMT and atherosclerotic risk factors. Results: Compared to the control group, the mean FMD was significantly reduced in the cyanotic group (5.26 ± 2.42% and 9.48 ± 2.60%, respectively; P-value < 0.001. No significant difference was observed between the groups in CIMT (0.41 ± 0.08 mm and 0.39 ± 0.06 mm, respectively; P-value = 0.299. The levels of total cholesterol, low-density lipoprotein-cholesterol and very low-density lipoprotein-cholesterol were statistically significantly lower compared tothe control group (P-value = 0.001, 0.006 and 0.014, respectively, whereas no statistically significant difference was found in the levels of high-density lipoprotein-cholesterol and triglycerides (P-value = 0.113 and 0.975, respectively. Conclusions: Systemic endothelial dysfunction in children with irreversible pulmonary hypertension due to CHD was noted but there was no increased risk for atherosclerosis.

  10. Application of new balloon catheters in the treatment of congenital heart defects

    Science.gov (United States)

    Fiszer, Roland; Szkutnik, Małgorzata; Smerdziński, Sebastian; Chodór, Beata; Białkowski, Jacek

    2016-01-01

    Introduction Balloon angioplasty (BAP) and aortic or pulmonary balloon valvuloplasty (BAV, BPV) are well-established treatment options in congenital heart defects. Recently, significant technological progress has been made and new catheters have been implemented in clinical practice. Aim To analyze the results of BAP, BAV and BPV with the new balloon catheter Valver and its second generation Valver II, which the company Balton (Poland) launched and developed. These catheters have not been clinically evaluated yet. Material and methods We performed 64 interventions with Valver I and Valver II. With Valver I the following procedures were performed: 17 BPV (including 9 in tetralogy of Fallot – TOF), 10 BAV and 27 BAP in coarctations of the aorta (CoA) – including 9 native and 18 after surgery. With Valver II ten interventions were done – 3 BPV, 2 pulmonary supravalvular BAP (after switch operations), 2 BAP of recoarctations and 3 other BAP. Age of the patients ranged from a few days to 40 years. Results All procedures were completed successfully, without rupture of any balloon catheters. The pressure gradient drop was statistically significant in all groups: BPV in isolated pulmonary valvular stenosis 28.1 mm Hg (mean), BPV in TOF 18.7 mm Hg, BAV 32.8 mm Hg, BAP in native CoA 15.4 mm Hg and in recoarctations 18.6 mm Hg. In 3 cases during rapid deflation of Valver I, wrinkles of the balloons made it impossible to insert the whole balloon into the vascular sheath (all were removed surgically from the groin). No such complication occured with Valver II. Conclusions Valver balloon catheters are an effective treatment modality in different valvular and vascular stenoses. PMID:27625686

  11. Impact on parents of bronchiolitis hospitalization of full-term, preterm and congenital heart disease infants

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    Lapillonne Alexandre

    2012-10-01

    Full Text Available Abstract Background The objective of this work was to explore the impact on parents of the bronchiolitis hospitalization of their infant using the Impact of Bronchiolitis Hospitalization Questionnaire (IBHQ©. Methods Four hundred sixty-three infants aged less than 1 year and hospitalized for bronchiolitis were included in a French observational study during the 2008–2009 season. Parents were asked to complete the IBHQ at hospital discharge and 3 months later. IBHQ scores, ranging from 0 (no impact to 100 (highest impact, were compared according to gestational age (full-term, 33–36 wGA, ≤ 32 wGA and the presence of congenital heart disease (CHD. The potential drivers of impact were explored using multivariate linear regressions. Results The study included 332 full-terms, 71 infants born at 33–36 wGA, and 60 at ≤ 32 wGA; 28 infants had a CHD. At hospital discharge, 9 of the 12 IBHQ mean scores were above 40, indicating a marked impact on parents. Three months later, all mean scores were lower but 5 were still greater than 40. At discharge, the length of hospitalization had a significant effect on IBHQ worries and distress, fear for future, guilt and impact on daily organization scores (p Conclusions Bronchiolitis hospitalization has conspicuous emotional, physical and organizational consequences on parents and siblings, which persist 3 months after hospital discharge. The main drivers of the impact were length of hospital stay and parents’ educational level, while infants’ gestational age or the presence of a CHD had little influence.

  12. Self-representation of children suffering from congenital heart disease and maternal competence.

    Science.gov (United States)

    Perricone, Giovanna; Polizzi, Concetta; De Luca, Francesco

    2013-02-01

    Child development may be subject to forms of motor, physical, cognitive and self-representation impairments when complex congenital heart disease (CHD) occurs. In some cases, inadequacy of both self-representation as well as the family system are displayed. It seems to be important to search the likely internal and external resources of the CHD child, and the possible connections among such resources, which may help him/her to manage his/her own risk condition. The research project inquires the possible resources related to the self-representation and self-esteem levels of the CHD child, and those related to maternal self-perception as competent mothers. A group of 25 children (mean age = 10.2; SD=1.8) suffering from specific forms of CHD, and a group made up of their relative mothers (mean age = 38.2; SD=5) were studied. The tools used were the Human Figure Drawing, to investigate child body-related self-representation; the TMA scale (Self-esteem Multidimensional Test), to investigate the child's self-esteem; and the Q-sort questionnaire, to assess how mothers perceived their maternal competence. Data concerning the likely correlations between the child's self-representation and the maternal role competence show [that] positive correlations between some indicators of maternal competence, specific aspects of CHD children's self-representation (mothers' emotional coping and children's self-image adequacy) and self-esteem (mothers' emotional scaffolding and children's self-esteem at an emotional level). By detecting the occurrence of specific correlations among resources of both child and mother, the study provides cardiologists with information that is useful for building a relationship with the families concerned, which would seem to enhance the quality of the process of the cure itself.

  13. Scoliosis after thoracotomy/sternotomy in children with congenital heart disease

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    Hojjat Hosseinpour Feiz

    2012-01-01

    Full Text Available Context: Congenital heart disease (CHD patients bear a higher risk of scoliosis during their lifetime compared to their normal counterparts. On the other hand, operation on chest wall has been shown to increase the risk of scoliosis. However, the data are inconclusive. The present retrospective analysis is undertaken to determine the frequency of post-thoracotomy/sternotomy scoliosis in children with CHD. Materials and Methods: One hundred and eighty children with CHD who underwent thoracotomy/sternotomy and had a minimum followup of 3 years in a teaching center from 1997 to 2010 were recruited. After operation, all the patients were regularly examined for the development of scoliosis. 102 patients underwent thoracotomy and 78 sternotomy. Student′s t test, Chi-square test, Fisher′s exact test were used for statistical analyses. Results: Eighty-eight males and 92 females with a mean age of 9.95 ± 2.31 (range: 5-15 years were enrolled. The mean age at operation was 2.59 ± 1.66 (range: 0-9 years and the mean follow-up period was 7.36 ± 2.12 (range: 5-13 years. Scoliosis was confirmed in two patients (1.1%: 1 (1% in the thoracotomy group (a 12-year-old female operated 2 years earlier with a spinal 22 o convexity to the right and 78 o kyphosis and another (1.1% in the sternotomy group (an 8-year-old female operated during her neonatal period with a spinal 23 o convexity to the left. Conclusion: Scoliosis is not a common finding among the operated children with CHD in our center.

  14. Pulmonary arterial hypertension in congenital heart disease: Correlation of radiologic index with hemodynamic data

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    Choi, Young Hi [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1984-09-15

    It is well known that pulmonary arterial hypertension in congenital heart disease is an important prognostic factor, as is pulmonary vascular resistance. So it is tempting to get certain radiologic index that could predict the presence and the degree of pulmonary arterial hypertension. A total of 152 cases of left to right shunt with pulmonary arterial hypertension and 50 cases of left to right shunt without pulmonary arterial hypertension is presented, in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between March 1981 and February 1983. Statistical analysis of plain radiography findings with the emphasis on the correction of radiologic index with the hemodynamic data. The results are as follows: 1. The incidence of pulmonary arterial hypertension is much less in arterial septal defect than other two disease groups of left to right shunt. 2. PA/T ratio correlates well with pulmonary arterial pressure (r=0.674), especially in mild pulmonary hypertension group. No correlation in moderate pulmonary hypertension group in significant level. 3. PA/T ratio is below 38 in total cases of normal control group and in 32 cases (21.0%) among 152 cases of pulmonary arterial hypertension group. 4. The average PA/T ratio in normal pressure group of left to right shunt is 35.3, which has no significant difference from that of normal control group. 5. The average CT ratio of pulmonary arterial hypertension group is 59.0, which is larger than 49.1 of normal control group. The CT ratio shows no correlation with the pulmonary arterial pressure in statistically significant level. 6. The higher the pulmonary arterial pressure, the larger the Rp/Rs value. The Rp/Rs in atrial septal defect is 0.193 in average, the lowest value in comparison with other two disease groups.

  15. Altered lymphatics in an ovine model of congenital heart disease with increased pulmonary blood flow.

    Science.gov (United States)

    Datar, Sanjeev A; Johnson, Eric G; Oishi, Peter E; Johengen, Michael; Tang, Eric; Aramburo, Angela; Barton, Jubilee; Kuo, Hsuan-Chang; Bennett, Stephen; Xoinis, Konstantine; Reel, Bhupinder; Kalkan, Gokhan; Sajti, Eniko; Osorio, Oscar; Raff, Gary W; Matthay, Michael A; Fineman, Jeffrey R

    2012-03-15

    Abnormalities of the lymphatic circulation are well recognized in patients with congenital heart defects. However, it is not known how the associated abnormal blood flow patterns, such as increased pulmonary blood flow (PBF), might affect pulmonary lymphatic function and structure. Using well-established ovine models of acute and chronic increases in PBF, we cannulated the efferent lymphatic duct of the caudal mediastinal node and collected and analyzed lymph effluent from the lungs of lambs with acutely increased PBF (n = 6), chronically increased PBF (n = 6), and age-matched normal lambs (n = 8). When normalized to PBF, we found that lymph flow was unchanged following acute increases in PBF but decreased following chronic increases in PBF. The lymph:plasma protein ratio decreased with both acute and chronic increases in PBF. Lymph bioavailable nitric oxide increased following acute increases in PBF but decreased following chronic increases in PBF. In addition, we found perturbations in the transit kinetics of contrast material through the pleural lymphatics of lambs with chronic increases in PBF. Finally, there were structural changes in the pulmonary lymphatic system in lambs with chronic increases in PBF: lymphatics from these lambs were larger and more dilated, and there were alterations in the expression of vascular endothelial growth factor-C, lymphatic vessel endothelial hyaluronan receptor-1, and Angiopoietin-2, proteins known to be important for lymphatic growth, development, and remodeling. Taken together these data suggest that chronic increases in PBF lead to both functional and structural aberrations of lung lymphatics. These findings have important therapeutic implications that warrant further study.

  16. Plasmatic ADAMTS-13 metalloprotease and von Willebrand factor in children with cyanotic congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Soares, R.P.S. [Fundação Pró-Sangue Hemocentro de São Paulo, São Paulo, SP (Brazil); Bydlowski, S.P.; Nascimento, N.M. [Laboratório de Investigação Médica-31, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP (Brazil); Thomaz, A.M.; Bastos, E.N.M.; Lopes, A.A. [Faculdade de Medicina, Instituto do Coração, Universidade de São Paulo, São Paulo, SP (Brazil)

    2013-04-05

    Changes in plasma von Willebrand factor concentration (VWF:Ag) and ADAMTS-13 activity (the metalloprotease that cleaves VWF physiologically) have been reported in several cardiovascular disorders with prognostic implications. We therefore determined the level of these proteins in the plasma of children with cyanotic congenital heart disease (CCHD) undergoing surgical treatment. Forty-eight children were enrolled (age 0.83 to 7.58 years). Measurements were performed at baseline and 48 h after surgery. ELISA, collagen-binding assays and Western blotting were used to estimate antigenic and biological activities, and proteolysis of VWF multimers. Preoperatively, VWF:Ag and ADAMTS-13 activity were decreased (65 and 71% of normal levels considered as 113 (105-129) U/dL and 91 ± 24% respectively, P < 0.003) and correlated (r = 0.39, P = 0.0064). High molecular weight VWF multimers were not related, suggesting an interaction of VWF with cell membranes, followed by proteolytic cleavage. A low preoperative ADAMTS-13 activity, a longer activated partial thromboplastin time and the need for cardiopulmonary bypass correlated with postoperative bleeding (P < 0.05). Postoperatively, ADAMTS-13 activity increased but less extensively than VWF:Ag (respectively, 2.23 and 2.83 times baseline, P < 0.0001), resulting in an increased VWF:Ag/ADAMTS-13 activity ratio (1.20 to 1.54, respectively, pre- and postoperative median values, P = 0.0029). ADAMTS-13 consumption was further confirmed by decreased ADAMTS-13 antigenic concentration (0.91 ± 0.30 to 0.70 ± 0.25 µg/mL, P < 0.0001) and persistent proteolysis of VWF multimers. We conclude that, in pediatric CCHD, changes in circulating ADAMTS-13 suggest enzyme consumption, associated with abnormal structure and function of VWF.

  17. Neuropsychological Status in Children After Repair of Acyanotic Congenital Heart Disease

    Science.gov (United States)

    Quartermain, Michael D.; Ittenbach, Richard F.; Flynn, Thomas B.; Gaynor, J. William; Zhang, Xuemei; Licht, Daniel J.; Ichord, Rebecca N.; Nance, Michael L.; Wernovsky, Gil

    2016-01-01

    OBJECTIVES The majority of previous studies that described the neuropsychological effects of cardiopulmonary bypass (CPB) in children were performed after surgery in infancy for complex congenital heart disease (CHD). We sought to limit confounding variables and isolate potential independent effects of CPB by describing neuropsychological function in school-aged children after repair of acyanotic CHD. METHODS This was a prospective study of patients who were aged 5 to 18 years and undergoing repair of acyanotic CHD. Neuropsychological testing battery included assessment of intelligence, memory, motor, attention, executive function, and behavior before and 6 months after CPB. The independent effects of anesthesia, surgery, and hospitalization on neuropsychological function were assessed by testing a surgical control group of patients who were undergoing repair of pectus deformities. In addition, an outpatient group of children with mild CHD were enrolled to assess the practice effects of serial testing. RESULTS Patients included CPB (n = 35), surgical control (n = 19), and nonsurgical (n = 12). Groups were comparable in age, gender, and race and demonstrated similar unadjusted group mean scores on baseline and 6-month follow-up neuropsychological testing. When adjusted for practice effects, the CPB group performed similar to the non-CPB groups in all assessed neuropsychological domains, with the exception of 1 of 4 tests of executive function. CONCLUSIONS When controlling for the non-CPB effects of surgery (eg, hospitalization, anesthesia, thoracotomy) and the practice effects of serial testing, there were no consistent independent effects of CPB on neuropsychological status in a cohort of children and adolescents 6 months after repair of acyanotic CHD. PMID:20660542

  18. Incidental finding of congenital pericardial and mediastinal pleural defect by pneumothorax in an adult

    International Nuclear Information System (INIS)

    Introduction: Congenital pericardial defect (CPD) is an uncommon anomaly. If once cardiac herniation occurs, it threatens life. We report a case of left-sided pneumothorax with consequent protrusion of the heart into left thoracic cavity through not only a large CPD but also congenital pleuropericardium window. Case presentation: A 67-year-old man presenting with sudden-onset left-sided chest pain and slight dyspnea was referred to our hospital. Chest X-ray showed a left lung collapse, and also revealed a pneumopericardium along the right border of the ascending aorta. Subsequent computed tomography (CT) scan revealed that the heart was displaced into the left hemithorax. Thus, we diagnosed the patient with pneumothorax and a defect of the pericardial and mediastinal pleurae. Subsequently, a chest tube was inserted into the left thoracic cavity, and the collapsed lung was promptly inflated. The cardiac position was reinstated within mediastinum as evidenced by follow-up CT scan. The QRS axis on his electrocardiogram (ECG) was altered from 52° to 73°. Together with the cardiac relocation evidenced by the QRS axis shift on ECG and findings of CT, we determined that there was a low potential for complications and opted against surgical repair. Discussion: When the CPD is sufficiently large, surgical intervention is not necessary. The size of the CPD can be assessed not only by CT findings, but the alteration of the QRS axis on ECG also provides useful information whether cardiac herniation can be resolved by the inflated lung. - Highlights: • We reported a case of congenital pericardial defect (CPD) with pneumothorax. • We described how to manage to alleviate life-threatening complications. • The size of CPD was assessed by CT findings and the alteration of QRS axis on ECG

  19. A Comparison of Radiation Dose Between Standard and 3D Angiography in Congenital Heart Disease

    Energy Technology Data Exchange (ETDEWEB)

    Manica, João Luiz Langer, E-mail: joca.pesquisa@gmail.com; Borges, Mônica Scott; Medeiros, Rogério Fachel de; Fischer, Leandro dos Santos; Broetto, Gabriel; Rossi, Raul Ivo Filho [Instituto de Cardiologia / Fundação Universitária de Cardiologia, Porto Alegre, RS (Brazil)

    2014-08-15

    The use of three-dimensional rotational angiography (3D-RA) to assess patients with congenital heart diseases appears to be a promising technique despite the scarce literature available. The objective of this study was to describe our initial experience with 3D-RA and to compare its radiation dose to that of standard two-dimensional angiography (2D-SA). Between September 2011 and April 2012, 18 patients underwent simultaneous 3D-RA and 2D-SA during diagnostic cardiac catheterization. Radiation dose was assessed using the dose-area-product (DAP). The median patient age and weight were 12.5 years and 47.5 Kg, respectively. The median DAP of each 3D-RA acquisition was 1093µGy.m{sup 2} and 190µGy.m{sup 2} for each 2D-SA acquisition (p<0.01). In patients weighing more than 45Kg (n=7), this difference was attenuated but still significant (1525 µGy.m{sup 2} vs.413µGy.m{sup 2}, p=0.01). No difference was found between one 3D-RA and three 2D-SA (1525µGy.m{sup 2} vs.1238 µGy.m{sup 2}, p = 0.575) in this population. This difference was significantly higher in patients weighing less than 45Kg (n=9) (713µGy.m{sup 2} vs.81µGy.m{sup 2}, P = 0.008), even when comparing one 3D-RA with three 2D-SA (242µGy.m{sup 2}, respectively, p<0.008). 3D-RA was extremely useful for the assessment of conduits of univentricular hearts, tortuous branches of the pulmonary artery, and aorta relative to 2D-SA acquisitions. The radiation dose of 3D-RA used in our institution was higher than those previously reported in the literature and this difference was more evident in children. This type of assessment is of paramount importance when starting to perform 3D-RA.

  20. Prognosis and Risk Factors for Congenital Airway Anomalies in Children with Congenital Heart Disease: A Nationwide Population-Based Study in Taiwan.

    Directory of Open Access Journals (Sweden)

    Yu-Sheng Lee

    Full Text Available The mortality risk associated with congenital airway anomalies (CAA in children with congenital heart disease (CHD is unclear. This study aimed to investigate the factors associated with CAA, and the associated mortality risk, among children with CHD.This nationwide, population-based study evaluated 39,652 children with CHD aged 0-5 years between 2000 and 2011, using the Taiwan National Health Insurance Research Database (NHIRD. We performed descriptive, logistic regression, Kaplan-Meier, and Cox regression analyses of the data.Among the children with CHD, 1,591 (4.0% had concomitant CAA. Children with CHD had an increased likelihood of CAA if they were boys (odds ratio [OR], 1.48; 95% confidence interval [CI], 1.33-1.64, infants (OR, 5.42; 95%CI, 4.06-7.24, or had a congenital musculoskeletal anomaly (OR, 3.19; 95%CI, 2.67-3.81, and were typically identified 0-3 years after CHD diagnosis (OR, 1.33; 95%CI 1.17-1.51. The mortality risk was increased in children with CHD and CAA (crude hazard ratio [HR], 2.05; 95%CI, 1.77-2.37, even after adjusting for confounders (adjusted HR, 1.76; 95%CI, 1.51-2.04. Mortality risk also changed by age and sex (adjusted HR and 95%CI are quoted: neonates, infants, and toddlers and preschool children, 1.67 (1.40-2.00, 1.93 (1.47-2.55, and 4.77 (1.39-16.44, respectively; and boys and girls, 1.62 (1.32-1.98 and 2.01 (1.61-2.50, respectively.The mortality risk is significantly increased among children with CHD and comorbid CAA. Clinicians should actively seek CAA during the follow-up of children with CHD.

  1. Increased risk of severe congenital heart defects in offspring exposed to selective serotonin-reuptake inhibitors in early pregnancy

    DEFF Research Database (Denmark)

    Knudsen, Tanja Majbrit; Hansen, Anne Vinkel; Garne, Ester;

    2014-01-01

    BACKGROUND: Previous studies suggest a possible association between maternal use of selective serotonin-reuptake inhibitors (SSRIs) during early pregnancy and congenital heart defects (CHD). The purpose of this study was to verify this association by using validated data from the Danish EUROCAT...... Register, and secondary, to investigate whether the risk differs between various socioeconomic groups. METHODS: We conducted a cohort study based on Danish administrative register data linked with the Danish EUROCAT Register, which includes all CHD diagnosed in live births, fetal deaths and in pregnancies...... terminated due to congenital anomalies. The study population consisted of all registered pregnancies (n = 72,280) in Funen, Denmark in the period 1995-2008. SSRI-use was assessed using The Danish National Prescription Registry, information on marital status, maternal educational level, income, and country...

  2. A closer look at the developmental interplay between parenting and perceived health in adolescents with congenital heart disease.

    Science.gov (United States)

    Rassart, Jessica; Luyckx, Koen; Goossens, Eva; Apers, Silke; Moons, Philip

    2014-12-01

    The present study examined associations between parenting and perceived health in adolescents with congenital heart disease (CHD) using a longitudinal trajectory approach. Adolescents with CHD were selected from the database of pediatric and congenital cardiology of the University Hospitals Leuven. A total of 429 adolescents (M age = 16 at T1) participated in the present study, comprising four measurement waves spanning approximately 3 years. Latent class growth analysis was used to identify trajectory classes of parenting and perceived health. Whereas adolescents from democratic households reported the most favorable health outcomes, adolescents from authoritarian, overprotective, and psychologically controlling families (all characterized by relatively high levels of psychological control) showed an increased risk for poor perceived health over time. Hence, the present study found substantial developmental associations between parenting and perceived health in adolescents with CHD. Future research should investigate whether working on the parent-adolescent relationship can foster patients' health. PMID:24819301

  3. Predicting mortality after congenital heart surgeries: Evaluation of the Aristotle and Risk Adjustement in Congenital Heart surgery-1 risk prediction scoring systems: A retrospective single center analysis of 1150 patients

    Directory of Open Access Journals (Sweden)

    Shreedhar S Joshi

    2014-01-01

    Full Text Available Aims and Objectives: To validate Aristotle basic complexity and Aristotle comprehensive complexity (ABC and ACC and risk adjustment in congenital heart surgery-1 (RACHS-1 prediction models for in hospital mortality after surgery for congenital heart disease in a single surgical unit. Materials and Methods: Patients younger than 18 years, who had undergone surgery for congenital heart diseases from July 2007 to July 2013 were enrolled. Scoring for ABC and ACC scoring and assigning to RACHS-1 categories were done retrospectively from retrieved case files. Discriminative power of scoring systems was assessed with area under curve (AUC of receiver operating curves (ROC. Calibration (test for goodness of fit of the model was measured with Hosmer-Lemeshow modification of χ2 test. Net reclassification improvement (NRI and integrated discrimination improvement (IDI were applied to assess reclassification. Results: A total of 1150 cases were assessed with an all-cause in-hospital mortality rate of 7.91%. When modeled for multivariate regression analysis, the ABC (χ2 = 8.24, P = 0.08, ACC (χ2 = 4.17 , P = 0.57 and RACHS-1 (χ2 = 2.13 , P = 0.14 scores showed good overall performance. The AUC was 0.677 with 95% confidence interval (CI of 0.61-0.73 for ABC score, 0.704 (95% CI: 0.64-0.76 for ACC score and for RACHS-1 it was 0.607 (95%CI: 0.55-0.66. ACC had an improved predictability in comparison to RACHS-1 and ABC on analysis with NRI and IDI. Conclusions: ACC predicted mortality better than ABC and RCAHS-1 models. A national database will help in developing predictive models unique to our populations, till then, ACC scoring model can be used to analyze individual performances and compare with other institutes.

  4. Congenital valvular aortic stenosis in young adults : Predictors for rate of progression of stenosis and aortic dilatation

    NARCIS (Netherlands)

    van der Linde, Denise; Andrinopoulou, Elini-Rosalina; Oechslin, Erwin N.; Budts, Werner; van Dijk, Arie P. J.; Pieper, Petronella G.; Wajon, Elly M. C. J.; Post, Marco C.; Witsenburg, Maarten; Silversides, Candice K.; Oxenius, Angela; Bogers, Ad J. J. C.; Takkenberg, Johanna J. M.; Roos-Hesselink, Jolien W.

    2013-01-01

    Background: Congenital aortic stenosis (AS) is the most common obstructive left-sided cardiac lesion in young adults, however little is known about the progression in adults. Therefore, we aimed to evaluate the progression rate of AS and aortic dilatation in a large multicenter retrospective cohort

  5. Swan-Ganz - right heart catheterization

    Science.gov (United States)

    ... have: Abnormal pressures in the heart arteries Burns Congenital heart disease Heart failure Kidney disease Leaky heart valves (valvular ... 2011:chap 55. Read More Burns Cardiac tamponade Congenital heart disease Heart attack Heart failure - overview Pulmonary hypertension Restrictive ...

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

    Institute of Scientific and Technical Information of China (English)

    Thomas Pickardt; Eva Niggemeyer; Ulrike MM Bauer; Hashim Abdul-Khaliq

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Thomas Pickardt

    2016-08-01

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

  8. EARLY DIAGNOSIS OF CONGENITAL HEART DISEASE%新生儿先天性心脏病早期诊断

    Institute of Scientific and Technical Information of China (English)

    冉建瑜

    2011-01-01

    [Objective] To improve the diagnosis rate, reduce mortality and morbidity of congenital heart disease, summarize the early diagnosis of congenital neart disease characteristics. [Methods] From January 2008 -December 2010, 61 neonatal children with congenital heart disease admitted to our hospital were diagnosed by color Doppler ultrasound, retrospectively analyzed to the clinical features of children with congenital heart disease performance and auxiliary examination. [ Re-Sults] The qolor Doppler ultrasound investigation showed that newborns with congenital heart disease mainly were found with ventricular septal defect, followed by atri^l septal defect. CR film showed expansion of the heart shadow in 20 cases (32.7%) , lung field congestion in 16 patients (26.2%), lung field ischemia in 9 patients (14.8%) . ECG showed right ventricular hypertrophy in 20 cases (32.7%), ECG left side in 9 cases (14.8%), arrhythmia in 8 patients (13.1%) , supraventricular brady-cardia in 3 patients (4.9%), sT-segment depression in 1 case (1.6%). [Conclusion] Neonatal congenital heart disease is deficiency in clinical manifestations, shortness of breath is common, and the majority pathological type is ventricular septal defect. Color Doppler ultrasound examination is with a unique advantage.%[目的]为提高临床诊断率,降低先心病病死率及致残率,总结新生儿先天性心脏病患者早期诊断特点.[方法]选择2008年1月-2010年12月住院新生儿科的先心病患儿共61例,所有患儿均经过彩色多普勒超声明确诊断,回顾分析心儿先天性心脏病临床特征性表现及各辅助检查结果.[结果]彩超查显示,新生儿先天性心脏病中以室间隔缺损居多,其次为房间隔缺损.CR拍片显示心影扩大者20例(32.7%),肺野淤血者16例(26.2%),肺野缺血者9例(14.8%).心电图检查显示右室肥厚者20例(32.7%),心电左偏者9例(14.8%),心率不整者8例(13.1%),室上性心动过缓者3例(4.9%),s-T段压低者1

  9. Cervical approach for repair of congenital tracheo-esophageal fistula presenting in an adult.

    Directory of Open Access Journals (Sweden)

    Albuquerque K

    1993-10-01

    Full Text Available Congenital tracheo-esophageal fistula without esophageal atresia was diagnosed on barium swallow radiography and esophagoscopy findings in an adult patient presenting with difficult in swallowing. The fistulous opening seen on the anterior wall of the esophagus led to the trachea. On bronchoscopy, the tracheal opening was located posteriorly 5 cm below the vocal cords. Successful repair was undertaken via a left cervical approach. The anatomical and developmental basis for the preferred approach is discussed with a brief literature review on the subject.

  10. Lipid profiles and inflammatory markers after periodontal treatment in children with congenital heart disease and at risk for atherosclerosis

    Directory of Open Access Journals (Sweden)

    Bresolin AC

    2013-11-01

    Full Text Available Adriana Chassot Bresolin,1 Mariana Menegassi Pronsatti,2 Larissa Nicole Pasqualotto,2 Patricia Oehlmeyer Nassar,3 Alex Sandro Jorge,4 Edson Antonio Alves da Silva,5 Carlos Augusto Nassar3 1Department of Biosciences and Health, 2School of Dentistry, 3Department of Periodontology, 4School of Biochemistry, 5Graduate Program, Western Paraná State University (UNIOESTE, Cascavel, Paraná, Brazil Abstract: Due to the biological associations between periodontal and cardiovascular diseases, as well as the fact that atherosclerosis begins in childhood, behavior based on oral health care and metabolic control from an early age is essential for patients with cardiovascular disease. The aim of this research was to examine the effect of full-mouth scaling and root planing on the reduction of periodontal disease in children with congenital heart disease. In this study, treatments were related to clinical periodontal parameters and also to blood ones, such as lipid profile and inflammatory markers. The patients were divided into two groups: group 1 (n=17, scaling and root planing; and group 2 (n=16, full-mouth scaling and root planing. The results showed a significant improvement in clinical periodontal parameters (P<0.05 in both groups. Considering lipid parameters, total cholesterol, triglycerides, and very-low-density lipoprotein parameters showed significant improvement (P<0.05. There was also an improvement in C-reactive protein (ultrasensitive in the group treated with scaling and root planing (P<0.05. Fibrinogen and interleukin-6 parameters improved (P<0.05 in both groups. We suggest that both periodontal treatments were effective in children with congenital heart disease, though neither demonstrated superiority. Keywords: periodontal disease, heart defects, congenital, lipid metabolism, inflammation mediators, children

  11. Pattern of congenital heart disease in a developing country tertiary care center: Factors associated with delayed diagnosis

    Science.gov (United States)

    Rashid, Usman; Qureshi, Ahmad U; Hyder, Syed N; Sadiq, Masood

    2016-01-01

    Objective: To determine the delay in diagnosis of various types of congenital heart defects in children and factors associated with such delay. Patients and Methods: For this observational study, 354 patients having congenital heart disease (CHD) presenting for the first time to the Department of Cardiology, Children’s Hospital, Lahore, Pakistan, between January 1, 2015 and June 30, 2015, were enrolled after obtaining informed verbal consent from the guardian of each child. Demographical profile and various factors under observation were recorded. Results: Among the 354 enrolled children (M: F 1.7:1) with age ranging from 1 to 176 months (median 24 months), 301 (85.1%) had delayed diagnosis of CHD (mainly acyanotic 65.3%), with median delay (8 months). Main factors for delay were delayed first consultation to a doctor (37.2%) and delayed diagnosis by a health professional (22.5%). Other factors included delayed referral to a tertiary care hospital (13.3%), social taboos (13.0%), and financial constraints (12.3%). Most children were delivered outside hospital settings (88.7%). Children with siblings less than two (40%) were less delayed than those having two or more siblings (60%, P < 0.001). Conclusion: Diagnosis of congenital heart defect was delayed in majority of patients. Multiple factors such as lack of adequately trained health system and socioeconomic constraints were responsible for the delay. There is a need to develop an efficient referral system and improve public awareness in developing countries for early diagnosis and management of such children. PMID:27625517

  12. Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea

    Directory of Open Access Journals (Sweden)

    Bo Kyung Jin

    2013-03-01

    Full Text Available Purpose: The use of implantable cardioverter defibrillators (ICDs to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. Methods: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. Results: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was 14.5±5.4 years (range, 2 to 22 years. The follow-up duration was 28.9±20.4 months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1, cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years. Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia.During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. Conclusion: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.

  13. Anomalous origin of the left circumflex coronary artery from the pulmonary artery. A very rare congenital anomaly in an adult patient diagnosed by cardiovascular magnetic resonance

    Directory of Open Access Journals (Sweden)

    Giannitsis Evangelos

    2008-01-01

    Full Text Available Abstract Here we report for the first time on the diagnostic potential of cardiovascular magnetic resonance (CMR to delineate the proximal course of an anomalous left circumflex coronary artery (LCX originating from the right pulmonary artery in an adult patient with no other form of congenital heart disease. The patient was referred to our institution due to exertional chest discomfort. X-Ray coronary angiography showed a normal left anterior descending coronary artery (LAD and right coronary artery (RCA, while the LCX was filled retrograde by collateral flow through the LAD and the RCA. The origin of the LCX was postulated to be the pulmonary artery, but the exact origin of the anomalous artery could not be depicted on conventional angiograms. CMR provided the unambiguous depiction of the origin of the anomalous LCX from the right pulmonary artery and the delineation of its proximal course in this case of a very rare coronary anomaly in adults.

  14. Family lifestyle factors related to children s congenital heart defects in China:a case-control study

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Objective To explore the multiple risk factors for family lifestyle of children with congenital heart defects(CHDs)in Shaanxi Province,China.Methods A 1∶1 case-control study was carried out to investigate 60 pairs of children and their parents.The univariate and multivariable logistic regression models were used to analyze the influence of risk factors related to parents' lifestyle on CHDs.Results Several possible risk factors were found for CHDs,including fever(OR=4.465,P=0.017),pesticides contact(OR=2.234...

  15. Association between SNP rs10569304 on the Second Expressed Region of Hole Gene and the Congenital Heart Disease

    Institute of Scientific and Technical Information of China (English)

    张亚莉; 徐琳; 邱健; 李志梁; 李林海; 任广立; 董爱荣; 李炳玲; 葛明晓; 蒙仕仁; 王剑青

    2010-01-01

    The correlation of single nucleotide polymorphism (SNP) rs10569304 on the second expressed region of hole gene and congenital heart disease (CHD) of human being, and the effect of hole gene on CHD were investigated. 179 patients with CHD as CHD group and 183 healthy people as control group were selected in the case-control study. DNA was abstracted from the peripheral blood by phenol-chloroform method. Primer was designed for the flanking sequence of SNP rs10569304 on the second expressed region of hole gen...

  16. 52-kDa Ro/SSA epitopes preferentially recognized by antibodies from mothers of children with neonatal lupus and congenital heart block

    OpenAIRE

    Fritsch, Christine; Hoebeke, Johan; Dali, Hayet; Isenberg, David A; Meyer, Olivier; Muller, Sylviane; Ricchiuti, Vincent

    2005-01-01

    Neonatal lupus erythematosus is a rare disorder caused by the transplacental passage of maternal autoantibodies. The 52-kDa Ro/SSA antigen (Ro52) ribonucleoprotein represents an antigenic target strongly associated with the autoimmune response in mothers whose children have neonatal lupus and cardiac conduction disturbances, mainly congenital heart block. The objective of this study was to identify putative Ro52/60-kDa Ro/SSA antigen (Ro60) epitopes associated with neonatal lupus and congenit...

  17. Image Quality of Coronary Computed Tomography Angiography with 320-Row Area Detector Computed Tomography in Children with Congenital Heart Disease.

    Science.gov (United States)

    Tada, Akihiro; Sato, Shuhei; Kanie, Yuichiro; Tanaka, Takashi; Inai, Ryota; Akagi, Noriaki; Morimitsu, Yusuke; Kanazawa, Susumu

    2016-03-01

    The objective of this study was to assess factors affecting image quality of 320-row computed tomography angiography (CTA) of coronary arteries in children with congenital heart disease (CHD). We retrospectively reviewed 28 children up to 3 years of age with CHD who underwent prospective electrocardiography (ECG)-gated 320-row CTA with iterative reconstruction. We assessed image quality of proximal coronary artery segments using a five-point scale. Age, body weight, average heart rate, and heart rate variability were recorded and compared between two groups: patients with good diagnostic image quality in all four coronary artery segments and patients with at least one coronary artery segment with nondiagnostic image quality. Altogether, 96 of 112 segments (85.7 %) had diagnostic-quality images. Patients with nondiagnostic segments were significantly younger (10.0 ± 11.6 months) and had lower body weight (5.9 ± 2.9 kg) (each p heart rate and heart rate variability between the two imaging groups were not significant. Receiver operating characteristic analyses for predicting patients with nondiagnostic image quality revealed an optimal body weight cutoff of ≤5.6 kg and an optimal age cutoff of ≤12.5 months. Prospective ECG-gated 320-row CTA with iterative reconstruction provided feasible image quality of coronary arteries in children with CHD. Younger age and lower body weight were factors that led to poorer image quality of coronary arteries.

  18. ALDH1A2 (RALDH2 genetic variation in human congenital heart disease

    Directory of Open Access Journals (Sweden)

    Mesquita Sonia MF

    2009-11-01

    Full Text Available Abstract Background Signaling by the vitamin A-derived morphogen retinoic acid (RA is required at multiple steps of cardiac development. Since conversion of retinaldehyde to RA by retinaldehyde dehydrogenase type II (ALDH1A2, a.k.a RALDH2 is critical for cardiac development, we screened patients with congenital heart disease (CHDs for genetic variation at the ALDH1A2 locus. Methods One-hundred and thirty-three CHD patients were screened for genetic variation at the ALDH1A2 locus through bi-directional sequencing. In addition, six SNPs (rs2704188, rs1441815, rs3784259, rs1530293, rs1899430 at the same locus were studied using a TDT-based association approach in 101 CHD trios. Observed mutations were modeled through molecular mechanics (MM simulations using the AMBER 9 package, Sander and Pmemd programs. Sequence conservation of observed mutations was evaluated through phylogenetic tree construction from ungapped alignments containing ALDH8 s, ALDH1Ls, ALDH1 s and ALDH2 s. Trees were generated by the Neighbor Joining method. Variations potentially affecting splicing mechanisms were cloned and functional assays were designed to test splicing alterations using the pSPL3 splicing assay. Results We describe in Tetralogy of Fallot (TOF the mutations Ala151Ser and Ile157Thr that change non-polar to polar residues at exon 4. Exon 4 encodes part of the highly-conserved tetramerization domain, a structural motif required for ALDH oligomerization. Molecular mechanics simulation studies of the two mutations indicate that they hinder tetramerization. We determined that the SNP rs16939660, previously associated with spina bifida and observed in patients with TOF, does not affect splicing. Moreover, association studies performed with classical models and with the transmission disequilibrium test (TDT design using single marker genotype, or haplotype information do not show differences between cases and controls. Conclusion In summary, our screen indicates that

  19. Consanguinity mapping of congenital heart disease in a South Indian population.

    Directory of Open Access Journals (Sweden)

    Tracy L McGregor

    Full Text Available BACKGROUND: Parental consanguinity is a risk factor for congenital heart disease (CHD worldwide, suggesting that a recessive inheritance model may contribute substantially to CHD. In Bangalore, India, uncle-niece and first cousin marriages are common, presenting the opportunity for an international study involving consanguinity mapping of structural CHD. We sought to explore the recessive model of CHD by conducting a genome-wide linkage analysis utilizing high-density oligonucleotide microarrays and enrolling 83 CHD probands born to unaffected consanguineous parents. METHODOLOGY/PRINCIPAL FINDINGS: In this linkage scan involving single nucleotide polymorphism (SNP markers, the threshold for genome-wide statistical significance was set at the standard log-of-odds (LOD score threshold of 3.3, corresponding to 1995ratio1 odds in favor of linkage. We identified a maximal single-point LOD score of 3.76 (5754ratio1 odds implicating linkage of CHD with the major allele (G of rs1055061 on chromosome 14 in the HOMEZ gene, a ubiquitously expressed transcription factor containing leucine zipper as well as zinc finger motifs. Re-sequencing of HOMEZ exons did not reveal causative mutations in Indian probands. In addition, genotyping of the linked allele (G in 325 U.S. CHD cases revealed neither genotypic nor allele frequency differences in varied CHD cases compared to 605 non-CHD controls. CONCLUSIONS/SIGNIFICANCE: Despite the statistical power of the consanguinity mapping approach, no single gene of major effect could be convincingly identified in a clinically heterogeneous sample of Indian CHD cases born to consanguineous parents. However, we are unable to exclude the possibility that noncoding regions of HOMEZ may harbor recessive mutations leading to CHD in the Indian population. Further research involving large multinational cohorts of patients with specific subtypes of CHD is needed to attempt replication of the observed linkage peak on chromosome 14

  20. Advances in noninvasive evaluation of congenital heart and great vessel disease: The roles of echo Doppler and MR imaging

    International Nuclear Information System (INIS)

    This course addresses the current use of echocardiography and MR imaging in the evaluation and management of patients with congenital abnormalities of the heart and great vessels. The imaging modalities are placed in perspective with respect to each other and to more invasive techniques. The discussion of echocardiography focuses on Doppler techniques, including the advantages and disadvantages of pulsed and continuous-wave examination, Doppler-determined velocity, flow disturbances, and the computation and measurement of pressure gradients. The integrated use of two-dimensional and Doppler examination in patients with shunts, obstructive lesions, and cyanotic heart disease is described. The MR imaging discussion covers the advantages and limitations of this imaging modality, examination techniques (including special considerations in the pediatric age group), and clinical applications, both preoperative and postoperative. Emphasis is placed on the complementary roles of MR imaging and echocardiogrphy and their combined impact on the need for catheterization and angiography