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Results for “HEART DISEASE, CONGENITAL”

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PubMed1996

Referral of young adult patients with congenital heart disease to adult centres. The Canadian Adult Congenital Heart Network.

OBJECTIVE: To establish a process of referral for young adult patients with congenital heart disease from pediatric to adult centres. DESIGN: Directors of pediatric cardiology units across Canada were asked to complete a questionnaire detailing their process of referral of young adult patients with congenital heart disease to adult centres. They were also asked to respond to specific case scenarios. Adult cardiologist members of the Canadian Adult Congenital Heart Network were asked to respond to the same case scenarios. MAIN RESULTS: Most pediatric cardiology centres refer patients to adult cardiologists at 18 years of age. The process of referral generally involves a referral letter and relevant parts of the chart. Few centres arrange a booked appointment with the adult cardiologist, although this would be preferred by the majority of the responding adult cardiologists. Generally good agreement existed between pediatric and adult cardiologists with regard to the kind of patients who required specialized care at an adult congenital heart centre. CONCLUSION: Within Canada, a process is rapidly evolving to facilitate the transfer of care of young adults with congenital heart disease.

Adolescent↗
PubMed2006

The right ventricle in congenital heart disease.

In patients with congenital heart disease the right ventricle (RV) may support the pulmonary (subpulmonary RV) or the systemic circulation (systemic RV). During the last 50 years evidence is accumulating that RV dysfunction develops in many of these patients and leads to considerable morbidity and mortality. Therefore RV function in certain groups of congenital heart disease patients needs close surveillance and timely and appropriate intervention to optimise outcomes. Despite major progress being made, assessing the RV either in the subpulmonary or the systemic circulation remains challenging, often requiring a multi-imaging approach and expertise (echocardiography, magnetic resonance imaging, nuclear and occasionally invasive assessment with angiography). This review discusses the implications of volume and pressure loading of the RV in the context of congenital heart disease and describes the most relevant imaging modalities for monitoring RV function.

Echocardiography↗
PubMed2003

[Anesthesia for non-cardiac surgery in children with congenital heart diseases].

The incidence of congenital heart diseases accounts for 8-10 over 1000 liveborn. In Italy about 4000-4500 babies each year are born with congenital heart diseases; 50% of those babies (2000-2200) need cardiac surgery shortly after birth or within the first few months of life. Of the remaining 50%, half undergoes cardiac surgery later on in life and half does not necessitate any surgery; 30% of all cardiac operations consist of palliative procedures and the remaining 70% consist of one-stage corrective procedures. Improvements achieved both in surgical and anesthesiologic techniques, and in cardiopulmonary bypass and myocardial protection, have led to better results in pediatric cardiac surgery, with excellent long term survival rate, even for the more complex variants of congenital heart malformations. Therefore anesthesiologists are now more often required to deal with patients affected by congenital heart defects, for other than cardiac problems. Accurate investigation of patient's clinical history is strongly suggested. Moreover knowledge and familiarity with the modifications of the physiology, occurring in congenital heart disease patients, are mandatory for the choice of the more appropriate anesthesiologic strategy for each patient, in order to optimise the risk-benefits ratio and achieve a less traumatic impact on the cardio-circulatory and respiratory equilibrium. With the aim of achieving better results, interaction between anesthesiologist, cardiologist, pediatrician, surgeon and sometime neonatologist and cardiac surgeon, is strongly recommended in the evaluation of risks, and in decision making of strategies and timing of treatment.

Anesthesia↗
PubMedDate not supplied

Spinal deformity after combined thoracotomy and sternotomy for congenital heart disease.

UNLABELLED: Patients with congenital heart disease are at an increased risk to develop scoliosis. The purpose of this study was to determine the incidence of spinal deformity in patients after thoracotomy and sternotomy for congenital heart disease. METHODS: Sixty-eight patients underwent thoracotomy followed by a sternotomy and met inclusion criteria. The medical records were reviewed to gather demographic data and medical and surgical history. Serial radiographs were reviewed. RESULTS: Scoliosis developed in 26% of the patients (10 boys, 8 girls). The mean Cobb angle was 40 degrees (range, 15-78 degrees). The mean age at diagnosis of scoliosis was 10.7 years (range, 2.9-17 years). The mean follow-up was 14.9 years (range, 5-20 years). Twelve percent (8 patients) required posterior spinal fusion. A kyphotic deformity developed in 21% (14 patients). In patients with scoliosis, the mean kyphosis was 38 degrees (range, 2-88 degrees). Patients with a cyanotic cardiac condition had a 4-fold incidence of scoliosis. There was no correlation between the development of scoliosis or kyphosis and the age at time of procedures, number of surgeries, sex, heart size, or side of the aortic arch. CONCLUSIONS: The risk of developing scoliosis in children with congenital heart disease is more than 10 times that of idiopathic scoliosis. Spinal deformities, including scoliosis and/or hyperkyphosis, were found in 38% of the patients. Curves develop at a younger age, which increases the risk of progression. The sagittal alignment in scoliosis patients tends toward hyperkyphosis. The thoracic spine receives a "double hit" when both procedures are combined.

Child↗
PubMed2003

Psychological interventions for depression in adolescent and adult congenital heart disease.

BACKGROUND: Adult and adolescent congenital heart disease is increasing in prevalence as better medical care means more children are surviving to adulthood. People with chromic disease often also experience depression. There are several non-pharmacological treatments that might be effective in treating depression and improving quality of life for adults and young adults with congenital heart disease. The aim of this review was to assess the effects of treatments such as psychotherapy, cognitive behavioural therapies and talking therapies for treating depression in this population. OBJECTIVES: To assess the effects (both harms and benefits) of psychological interventions for treating depression in young adults and adults with congenital heart disease. SEARCH STRATEGY: We searched the Cochrane Controlled Trials Register (CCTR) (on The Cochrane Library issue 4, 2002), MEDLINE (1966 to August 2002), EMBASE (1980 to August 2002), PsycLIT (1887 to August 2002), the Database of Abstracts of Reviews of Effectiveness (DARE) (Issue 4, 2002 of the Cochrane Library), Biological Abstracts (January 1980 to August 2002), and CINAHL (January 1980 to August 2002). Abstracts from national and international cardiology and psychology conferences and dissertation abstracts were also searched. SELECTION CRITERIA: Randomised controlled trials comparing psychological interventions with no intervention for people over 15 years with depression who have congenital heart disease. DATA COLLECTION AND ANALYSIS: Two reviewers independently screened titles and abstracts of studies that were potentially relevant to the review. Studies that were clearly ineligible were rejected. Two reviewers independently assessed the abstracts or full papers for inclusion criteria. Further information was sought from the authors where papers contained insufficient information to make a decision about eligibility. MAIN RESULTS: No randomised controlled trials were identified. REVIEWER'S CONCLUSIONS: Depression is common in patients with congenital heart disease and can exacerbate the physical consequences of the illness. There are effective pharmacological and non-pharmacological treatments for depression, but we have not been able to identify any trials showing the effectiveness of non-pharmacological treatments. A well designed randomised controlled trial is needed to assess the effects of psychological interventions for depression in congenital heart disease.

Adolescent↗
PubMed2003

Risky business: insuring adults with congenital heart disease.

Accurate prognostication in congenital heart disease is vital for purposes of obtaining insurance, yet can be problematic for patients, physicians and insurers. This article discusses the scope of the problem, and describes the process of evaluating life insurance. Mortality data as well as predictors of adverse outcomes for individual congenital heart lesions are reviewed. Practical tips for patients and their physicians are given to aid in successful application for insurance. To expand the possibility of future patients obtaining insurance coverage, the ongoing reporting and constant updating of very long-term survival data in congenital heart disease is emphasized.

Adult↗
PubMed1987

Food, growth and congenital heart disease.

The effect of congenital heart disease on growth is reviewed. Whether being small matters is questioned, and reasons why infants with congenital heart disease are small are discussed. Methods of improving growth, and catch-up growth are described. Finally management of the child with CHD and failure to thrive is considered.

Body Weight↗
PubMed1996

Right ventricular regional wall motion abnormality in congenital heart disease.

Four cases of congenital heart disease with right ventricular overload and echocardiographic evidence of persistent right ventricular regional wall motion abnormalities are presented. Right ventricular infarction could be a possibility. Such regional wall motion abnormalities could add to overall right ventricular dysfunction in these cases. Echocardiography is useful in diagnosis. Right ventricular wall motion abnormalities should be studied in detail in all cases of congenital heart disease with right ventricular overload.

Adult↗
PubMed1983

Coronary artery compression with fatal myocardial ischemia. A rare complication of valved extracardiac conduits in children with congenital heart disease.

Three children with congenital heart disease died after surgical procedures involving the placement of valved extracardiac conduits; their deaths were caused by myocardial ischemia following coronary artery compression by the metallic stent of the conduit valve. The first and second patients died of acute myocardial ischemia or infarction during the immediate postoperative period, whereas the third patient died of chronic myocardial ischemia and progressive heart failure several months after the operation. In a fourth patient the problem of possible coronary artery compression was suspected on completion of the surgical procedure, and the valve stent was then repositioned away from the coronary artery; this resulted in marked hemodynamic improvement. Fatal myocardial ischemia from coronary artery compression is a rare but potential complication of valved extracardiac conduit placement in children with congenital heart disease. Preoperative assessment of coronary artery distribution is indicated in those patients with prior intrapericardial operations and subsequent pericardial adhesions. Such assessment in previously unoperated patients may be undertaken at the time of conduit operation. Proper conduit placement and intraoperative recognition of possible coronary artery compression by the conduit are important in preventing significant ischemic complications.

Child↗
PubMed2006

Radiation exposure during follow-up of adults with congenital heart disease.

BACKGROUND: Adult patients with congenital heart disease under follow-up often need to undergo diagnostic procedures which expose them to radiation. AIM: To evaluate radiation doses in adult patients with congenital heart disease during follow-up. METHODS: Data on diagnostic procedures were used from the European Heart Survey on adult congenital heart disease, a multicenter retrospective cohort study. Lesions included in the survey were Atrial Septal Defect, Ventricular Septal Defect, Fallot, Fontan, Coarctation, Transposition of the Great Arteries, Marfan, and Cyanotic lesions. A total of 4110 patients (52% female) with a mean age of 32 years (range 17-85) were included. The follow-up time ranged from 0 to 72 months, with a median of 61 months. There were a total of 18,403 patient-years of follow-up. During this time, a mean of 4.5 visits per patient took place. Radiation doses were calculated using the number of examinations in each patient's data file. Effective radiation doses are given in millisievert (mSv). RESULTS: The average cumulative annual effective dose per patient was 0.46 mSv. The relative contributions to these doses were 3% by chest X-rays, 39% by computed tomography scans, 42% by angiography, and 16% by nuclear scans. Effective doses were higher in patients with Fontan, Coarctation, Marfan and Cyanotic lesions, as well as in patients with Atrial Septal Defects. CONCLUSIONS: Exposure to radiation during follow-up of patients with adult congenital heart disease mainly stems from computed tomography scans and angiography. Patients with Fontan, Coarctation, Marfan and Cyanotic lesions are more likely to get high doses from computed tomography. In these lesions, therefore, particular care should be taken to use non-ionizing imaging procedures whenever possible.

Adolescent↗
PubMed2004

Surgical management of infants with congenital lobar emphysema and concomitant congenital heart disease.

OBJECTIVE: Congenital lobar emphysema (CLE) is an uncommon cause of infantile respiratory distress. It is diagnosed on the basis of evidence of lobar overaeration, mediastinal shift, and compression of the adjacent lobe. Concomitant congenital heart disease (CHD) and CLE is not uncommon. In the literature a 12% to 20% concomitance rate is given. The optimal treatment of respiratory symptoms associated with CLE and CHD is not clear; however, there has been a great deal of progress in the treatment of CLE and CHD. The aim of this study was to evaluate a clinical experience with and long-term follow-up of the surgical treatment of 13 patients with concomitant CLE and CHD. MATERIAL AND METHODS: We reviewed the cases of 13 patients with concomitant CLE and CHD. The medical records were evaluated with reference to age, type of CHD, pulmonary artery pressure, clinical symptoms, and results of surgical management. RESULTS: One patient died. This patient had ventricular septal defect (VSD) and left upper lobe emphysema in the postoperative period. The remaining patients undergoing follow-up were clinically well at the final evaluation. Postoperative thoracic computed tomography revealed complete spontaneous regression of emphysema 3 months after division of ductus arteriosus in 1 patient. Pulmonary hypertensive episode was seen in 3 patients after the early postoperative period. Five of the patients were discharged with bronchodilator treatment after surgery. Six patients needed positive inotropic support. Among the patients with pulmonary hypertension and those with VSD who had undergone cardiopulmonary bypass, we found a greater need for inotropic support, a higher risk of postoperative infection, and a longer intubation period. Echocardiography in the late postoperative revealed decreased pulmonary artery diameter and pressure; myocardial performance was normal. Results of blood gas analyses revealed increased oxygen saturation and decreased partial pressure of carbon dioxide. Normal exercise activity was found in all patients. DISCUSSION: The presence of CHD, especially in infants with unusual respiratory distress symptoms, should be kept in mind, and echocardiography and/or cardiac catheterization should be considered in the diagnosis. In patients with high pulmonary artery pressure, palliative or corrective surgery for CHD in addition to lobectomy can be considered. We believe that for lesions without high pulmonary artery pressure, such as small atrial septal defect and patent foramen ovale, clinical follow-up is sufficient treatment after lobectomy. If the cause of CLE is compression of large ductus arteriosus, only division of the patent ductus arteriosus may be considered before lobectomy and clinical and radiologic follow-up. The cardiac lesion should be assessed as to severity and ease of management. A corrective procedure can be carried out at lobectomy. Because of the technical ease with which the cardiac operation can be performed at the time of lobectomy, we suggest that in addition to lobectomy, operative treatment of cardiac lesions be performed.

Child, Preschool↗
PubMedDate not supplied

Antiphospholipid syndrome in patients with cyanotic congenital heart disease.

Patients with cyanotic congenital heart disease exhibit an increased incidence of thrombotic episodes and are frequently thrombocytopenic. We studied the sera of 15 patients with this type of heart malformation, searching for anticardiolipin antibodies. 3/15 had positive results. The three of them were adult females; two had thrombotic episodes and a false positive VDRL. Thus, cyanotic congenital heart disease may be another disease entity associated with the antiphospholipid syndrome.

Adult↗
PubMed2003

A case of congenital midline cervical cleft with congenital heart disease.

A congenital midline cervical cleft (CMCC) is a rare developmental anomaly. It may represent failure of the branchial arches to fuse in the midline and presents at birth with a ventral midline defect of the skin of the neck. Congenital heart disease along with CMCC is rarer, and most of the cases reported are associated with chest wall defects or thoracic ectopia cordis. The authors report a case of a 5-month-old girl with CMCC and an atrial septal defect (ASD) and discuss the clinical presentation, embryologic development, and treatment.

Abnormalities, Multiple↗
PubMed2003

Electrophysiologic surgery in patients with congenital heart disease.

As patients with congenital heart disease increase in number and age, arrhythmia is becoming a more prevalent and important clinical problem. Although catheter-based therapy has revolutionized the management of arrhythmia, there remains an increasing patient population with congenital heart disease presenting for repair or reoperation with associated atrial or ventricular arrhythmias. Arrhythmia ablation may be safely and effectively included as an adjunct to repair of underlying structural cardiac lesions. Successful electrophysiologic surgery requires accurate preoperative characterization of the arrhythmia.

Arrhythmias, Cardiac↗
PubMed2006

Low arterial saturation is associated with increased sensitivity to activated protein C in children with congenital heart disease.

OBJECTIVES: Children with congenital heart disease experience both hemorrhagic and thrombotic complications. In this report the authors test the hypothesis that hypoxemia is associated with altered sensitivity to activated protein C (aPC) in pediatric patients with congenital heart lesions. DESIGN: A retrospective genetic registry review, with statistical evaluation of factors contributing to the aPC ratio. SETTING: Large university hospital. PARTICIPANTS: Cohort of 92 children with congenital heart disease undergoing cardiac catheterization procedures. INTERVENTIONS: The authors measured the aPC ratio at cardiac catheterization and evaluated the contribution of independent variables using linear regression and classification tree approaches. Independent variables included age, gender, use of aspirin, history of thrombosis, room air arterial saturation, factor VIII:C levels, presence of congestive heart failure, and heterozygosity for factor V Leiden. MEASUREMENTS AND MAIN RESULTS: At univariate analysis, factor V Leiden, female gender, room air arterial saturation, age greater than 6 months, and plasma factor VIII:C levels were associated with a lower aPC ratio (resistance to aPC). At stepwise linear regression, arterial saturation, factor VIII:C level, female gender, and factor V Leiden were independently associated with a lower aPC ratio, and these variables explained about 49% of the variability in aPC ratio. The classification tree approach confirmed the dependence of aPC ratio on factor V genotype and arterial saturation. CONCLUSIONS: The aPC ratio in this population is associated with hypoxemia, independent of factors previously observed in adults. Further studies are under way to determine how aPC resistance or sensitivity may independently affect perioperative hemostasis in this population.

Adolescent↗