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PubMedDate not supplied

Morphologic and arteriographic changes in the pulmonary vasculature in children with congenital heart disease.

This study was undertaken to determine the morphologic changes occurring in the pulmonary vasculature in congenital heart disease. Autopsy was performed in twenty cases of congenital heart disease ranging in age from newborn (full term) to 14 years. Postmortem arteriography was performed in most of them followed by histomorphometry. It was found that medial hypertrophy of the muscular pulmonary arteries was the most consistent change and the smallest muscular arteries were the most severely affected. The lesions were focal and randomly distributed. Hence, morphologic observations on open lung biopsies may not be accurate.

Adolescent↗
PubMed1989

Congenital heart disease in Down's syndrome patients: a decade of surgical experience.

Patients with Down's syndrome represent a significant subset of patients with congenital heart disease. Fifty-five patients with Down's syndrome have undergone surgical treatment for congenital heart disease at our institution in the past decade. Twenty-six had atrioventricular canal, 11 had ventricular septal defect, 7 had secundum atrial septal defect, 7 had tetralogy of Fallot, 3 had primum atrial septal defect and 1 patient had double outlet right ventricle. The thirty day mortality following operative intervention was 16.4%. Mortality was highest for tetralogy of Fallot followed by atrioventricular canal and ventricular septal defect. Long term mortality for all lesions was 27.3% over our follow-up period which averaged 33 months. Thirty day mortality compared similarly to previous reports of surgically treated Down's syndrome patients. When compared to our patients without Down's syndrome, the Down's population did not exhibit an increased risk for surgical treatment of congenital heart disease.

Adolescent↗
PubMed2003

Physical activity levels of adolescents with congenital heart disease.

Regular physical activity prevents chronic disease and moderate to vigorous participation provides additional health benefits. Therefore, adolescents with congenital heart disease risk developing latent diseases due to real or perceived physical activity restrictions. Habitual physical activity levels, psychological determinants and advice received were examined by postal survey of 434 West Australian adolescents aged 12-18 years with congenital heart disease. Survey results (n = 153) were compared with published normative adolescent data. Total activity was classified as vigorous, adequate or inadequate according to metabolic equivalents, reported frequency and duration. Comparable numbers of respondents and healthy peers were active (winter 62% vs 74%; and summer 73% vs 82% respectively, p = 0.27). However, significantly fewer male respondents were classified as vigorously active compared with healthy peers, in both winter (48% vs 67%, p < 0.02), and summer (48% vs 69%, p = 0.04). Similar, but non-significant, trends were found when comparing female respondents with healthy peers and for mild versus severe disease groups. Self-efficacy ratings did not explain differences in physical activity intensity. Congenital heart disease may impact on the intensity of physical activity undertaken by affected adolescents thus denying additional health benefits. Physiotherapists could facilitate these adolescents to achieve more moderate to vigorous physical activity, to offset adult sedentary behaviour.

Adolescent↗
PubMed1991

Fetal echocardiographic screening for congenital heart disease.

During a 30-month period, 7,531 obstetrical ultrasound examinations were performed on 3,624 fetuses at a community hospital. All fetuses were evaluated for congenital heart disease and 16 complex defects were identified. Only two of the cardiac defects had predisposing clinical factors or an elevated serum alpha-fetal protein. Discovery of cardiac defects in-utero permitted referral of the patients to tertiary care facilities when delivery was desired. This allowed prompt therapy by a pediatric cardiologist or cardiothoracic surgeon, if necessary.

Echocardiography↗
PubMed1995

Effect of low dosage inhaled nitric oxide on pulmonary hypertension in congenital heart disease.

An evaluation of the pulmonary vascular resistance and the reversibility of pulmonary vascular reaction in children with congenital heart disease is essential for determining the surgical indication and for assessing the long-term prognosis. We report the clinical efficacy of low dosage inhaled nitric oxide and investigate the relationship between its effect and hemodynamic parameters in 18 patients with congenital heart disease. The patients were divided into 3 Groups; Group 1 consisting of 3 patients with Eisenmenger syndrome, Group 2 of 10 patients whose mean pulmonary artery pressure was more than 30 mmHg, and Group 3 consisting of 5 patients whose mean pulmonary artery pressure was less than 30 mmHg. High concentration (90%) oxygen, and also normal oxygen (21%) containing 10 parts per million nitric oxide were administered by cardiac catheterization. In Group 1, both the 90% oxygen and the normal oxygen with nitric oxide showed no affect on the hemodynamical variables. In Group 2, the pulmonary artery pressure and the pulmonary vascular resistance both significantly decreased with the 90% oxygen, and with the nitric oxide inhalation, but these decreases were not found in Group 3. In the 15 patients (of Groups 2 and 3 combined) who were considered to have reversible pulmonary vascular changes, significant correlations were found between the baseline pulmonary artery pressure and the magnitude of pulmonary vasodilation. No clinical evidence of toxicity was seen with the administration of the inhaled nitric oxide. These data suggested that inhaled nitric oxide, even in a low dosage, was a potent and selective pulmonary vasodilator in patients with congenital heart disease complicated with pulmonary hypertension. Since a positive correlation was found between the baseline pulmonary artery pressure and the magnitude of pulmonary vasodilation, this examination demonstrated potential efficacy for objective analysis in patients with pulmonary hypertension.

Administration, Inhalation↗
PubMed1995

Adult congenital heart disease.

Much of the literature published in 1993 on the topic of adult congenital heart disease described long-term follow-up of patients following repair in childhood. This report reviews the most important papers published during the past year related to long-term follow-up, and also describes new information on the noninvasive evaluation of congenital heart disease in adults as well as the interventional cardiologic techniques that are currently being applied to treat some defects in the catheterization laboratory.

Adult↗
PubMed2004

Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study.

BACKGROUND: Operator-independent isotropic 3D MRI may greatly simplify the assessment of complex morphology in congenital heart disease. We sought to evaluate the reliability of this new approach. METHODS AND RESULTS: In 31 adolescent and adult patients (age, 6 to 42 years; median, 16 years) with congenital heart disease, cardiac morphology was determined with free-breathing (navigator-gated), isotropic, 3D steady-state free-precession (3D SSFP) MRI and independently evaluated by 2 observers. Cardiac diagnoses and multiple distance measurements were compared with conventional MR reference sequences (ie, spin-echo, cine gradient-echo, contrast-enhanced MR angiography) and with echocardiography/cine cardioangiography or surgery. Of the 31 patients, 24 had native congenital heart defects or residual defects after repair that warranted immediate treatment. None of these defects was missed by 3D SSFP. Novel diagnostic issues were discovered in 4 of 31 patients (coronary anomalies, n=3; left juxtaposition of the right atrial appendage in double-outlet right ventricle and transposition of the great arteries, 1). For sizes of valves and vessels, we found minor mean differences of -1.1 to 1.6 mm, with SD ranging from 1.2 to 2.9 mm, demonstrating overall good agreement with standard MRI (Bland-Altman analysis). Interobserver variability of 3D SSFP distance measures was low; mean differences ranged from -1.5 to 1.0 mm, and SD ranged from 0.8 to 2.5 mm. Scatter was lower for extracardiac than intracardiac measures. CONCLUSIONS: In adolescents and adults, isotropic 3D SSFP MRI allows reliable assessment of complex cardiac morphology. Distance measurements are accurate and reproducible. Thus, a single operator-independent acquisition may substitute for conventional 2D MRI sequences to accelerate and simplify MR scanning in congenital heart disease.

Adolescent↗
PubMed2004

Caliber of quality-of-life assessments in congenital heart disease: a plea for more conceptual and methodological rigor.

BACKGROUND: Interest in quality-of-life issues in children, adolescents, and adults with congenital heart disease has increased significantly during the past decades. In general, quality-of-life research is stimulated by an overall interest in outcomes. More specifically, quality-of-life studies add to mortality and morbidity data by taking reported physical, psychological, and social problems into consideration. However, quality of life can be an ambiguous concept that is hindered by methodological weaknesses, a situation that can prevent the drawing of firm conclusions. OBJECTIVE: To appraise the conceptual and methodological rigor of published quality-of-life assessments in patients with congenital heart disease. DESIGN: A review of 70 articles published in the medical literature, using the 10 criteria previously developed by Gill and Feinstein. For each criterion, the number of articles complying with that criterion was counted, and the percentage was calculated. To indicate how well individual articles performed on the respective criteria, a summary score was calculated by summing the number of criteria an article fulfilled and dividing this sum by the number of criteria for which the article was eligible to be evaluated; the resulting value was then multiplied by 100. MAIN OUTCOME MEASURE: Summary scores ranging from 0, for articles complying with none of the criteria, to 100, for articles complying with all of the criteria. RESULTS: We found that quality-of-life assessments in patients with congenital heart disease showed major conceptual and methodological drawbacks (median summary score, 0). Moreover, we found that the term quality of life was often used inappropriately, an observation supported by several articles in which the authors drew conclusions about patients' quality of life, even though quality of life was not specifically measured. CONCLUSIONS: A poor conceptual and methodological basis for quality of life implies that many results from quality-of-life studies performed in patients with congenital heart disease contribute little to the scientific knowledge base of quality of life in this patient population. Future quality-of-life studies need to invest in a rigorous conceptualization, an adequate operational definition, and a sound measurement of quality of life.

Adolescent↗
PubMed2002

Management of ventricular arrhythmias in adults with congenital heart disease.

The management of ventricular arrhythmias in adults with congenital heart disease is becoming increasingly more important. Ventricular arrhythmias are associated with a number of congenital heart defects (particularly tetralogy of Fallot), and sudden death. A number of invasive and noninvasive methods have been applied to identify those patients at risk, although indications and the prognostic value of these tests are unclear. Treatment of ventricular arrhythmias in this population include antiarrhythmic medications, catheter ablation, surgery, and implantable cardiac defibrillators.

Adult↗
PubMed2000

Psychosocial profile of adults with complex congenital heart disease.

OBJECTIVE: Despite the increasing number of adults with congenital heart disease (CHD), little is known of the emotional life of these long-term survivors; hence, we undertook a study to establish a psychological profile of these individuals so as to optimize their care. PATIENTS AND METHODS: We performed psychiatric evaluation by both interview and questionnaire on 29 ambulatory patients with CHD (mean age, 38 years; range, 26-56 years). RESULTS: Although many met symptomatic criteria for psychiatric diagnosis, most were functional in day-to-day life and used denial in adapting to their CHD. In childhood, extended absences from school, cyanosis, scars, and sports restrictions hindered friendships and conflicted with a sense of "normalcy"; teasing and low self-esteem were more notable in boys; girls more readily hid behind makeup, nail polish, and clothing. In adulthood most were concerned about shortened life expectancy, disability, and childbearing and child rearing. Virtually all were highly committed to both school and work performance. All but those with complete, uncomplicated early repair expressed unresolved frustration, anger, and fears of a future that might include further medical complications. CONCLUSION: Listening to this selected group of patients offers insight that may be helpful to the clinical care of patients with CHD and other patients with lifelong disease.

Adaptation, Psychological↗
PubMed1990

[Digital subtraction angiography for congenital heart disease].

From January 1988 to May 1989, ten infant patients (15 times) with congenital heart disease were studied by digital subtraction angiography (DSA) pre and/or postoperatively. The diagnoses of the patients include total anomalous pulmonary venous return (TAPVR), transposition of great arteries, patent ductus arteriosus with pulmonary hypertension, giant hemangioma of the left leg, asplenia (two cases), tetralogy of Fallot (two), and tricuspid atresia (two). Two newborn patients (TAPVR, giant hemangioma) were in critical condition preoperatively, but were diagnosed precisely and nonivasively by DSA. Ten cases were studied for estimation of operative repair and consequently five of them were diagnosed to require reoperation. It is concluded that DSA is of use for diagnosis of congenital heart diseases and for postoperative evaluation of infant patients under critical condition.

Angiography, Digital Subtraction↗
PubMed1991

Assessment of right ventricular overload by a thallium-201 SPECT study in children with congenital heart disease.

Thallium-201 planar and SPECT studies were performed in 38 children with congenital heart disease using high-resolution three-headed SPECT. The 201Tl total or average right ventricular-to-left ventricular (RV/LV) count ratios were measured by summed short-axis images. In the planar study, the RV/LV systolic pressure ratio correlated with 201Tl RV/LV average count ratio (r = 0.91) better than that in the SPECT study (r = 0.78). However, in the SPECT study, patients with atrial septal defect (n = 9), ventricular septal defect (n = 13), tetralogy of Fallot and double outlet right ventricle (n = 6) showed a characteristic distribution in the plot of RV/LV pressure ratio versus 201Tl RV/LV total count ratio. A prospective study showed that the RV/LV average count ratio was a good predictor of RV/LV pressure ratio (n = 19, r = 0.91). Three-headed SPECT is useful for pediatric cardiac studies, and the overload types found in congenital heart disease seemed to be differentiated by the SPECT study.

Adolescent↗
PubMed1996

[Bone marrow transplantation from an unrelated donor in patient with congenital heart diseases].

A 9-year-old with chronic myelogenous leukemia (CML) who received bone marrow transplantation from an unrelated donor (UBMT), is reported. He also suffered from congenital heart disease (CHD) consisting of corrected transposition of the great arteries and dextrocardia. The cardiac output was within normal limits. The conditioning regimen included busulfan, melphalan, ALG and TLI. Cyclophosphamide was not used because of it cardiotoxicity. The HLA-phenotype of the donor was identical with that of the patient. DNA typing showed was haplodentical DRB1. The patient is alive 18 months after the UBMT. This case showed that UBMT was possible in a CML patient with CHD, without congestive heart failure.

Adult↗
PubMed1997

Pregnancy and delivery prognostic risk score for women with congenital heart disease and acquired valvular heart disease.

Hemodynamic changes related to pregnancy in women with Congenital Heart Disease (ConHD) or Acquired Valvular Heart Disease (AVHD) require special medical care during pregnancy and especially immediately prior to, and after delivery. Following a five year retrospective study (1978-1982), the authors monitored prospectively over a period of 12 years (1983-1994), 378 pregnancies in 252 women with ConHD and AVHD. Based on the results of the initial clinical examination and monthly follow-up visits, a numerical risk score was assigned for each patient. The study showed good conformity between the predictive risk score values and the patient's condition in the course of pregnancy and delivery. Thus, this method represents a novel and potentially very useful clinical tool for management of pregnancy in patients with ConHD and AVHD.

Abortion, Spontaneous↗
PubMed2006

Natural history of postoperative heart block in congenital heart disease: implications for pacing intervention.

After half a century of major progress in congenital heart disease management, atrioventricular conduction block continues to complicate 1-3% of surgical procedures. Unless treated with an implanted pacemaker, permanent postoperative heart block is associated with 28-100% mortality. Postoperative heart block often proves to be transient, typically resolving within 10 days of onset. The duration of postoperative heart block is widely used as a key determinant for permanent pacemaker implantation. Current professional pacemaker implantation guidelines are largely based on this criterion. However, available natural history data suggest that other factors, such as residual conduction system injury, likely play a role in increasingly recognized cases of very late postoperative mortality and morbidity in patients who have experienced transient postoperative heart block. As growing numbers of congenital heart disease patients survive into adulthood, and artificial pacemaking capabilities continue to improve, it might be necessary to reconsider and refine currently accepted pacing indications for postoperative heart block.

Cardiac Pacing, Artificial↗
PubMed1995

Biplane intraoperative transesophageal echocardiography in congenital heart disease.

OBJECTIVE: To evaluate the accuracy, value, and safety of biplane intraoperative transesophageal echocardiography (TEE) in patients with congenital cardiac malformations. DESIGN: We reviewed the results of the first 104 consecutive biplane intraoperative TEE examinations performed during the repair of congenital heart defects at the Mayo Clinic. MATERIAL AND METHODS: TEE results were analyzed for accuracy of diagnosis, effect on the surgical procedure, and associated complications. In a subjective analysis, the relative contributions and advantages of each imaging plane (transverse and longitudinal) were also assessed. RESULTS: Biplane TEE had "significant impact" on intraoperative management in 17 of 104 examinations (16.3%). Preoperative TEE altered the planned procedure in 11 patients (10.6%). Postbypass biplane TEE led to immediate revision of the initial repair in nine patients (8.7%). Patients who underwent modified Fontan operations or subaortic resections had the greatest frequency of significant impact (40% [P = 0.006] and 33% [P = 0.03], respectively). No major complications were associated with TEE. For a complete examination, use of both imaging planes was necessary in all the patients studied. CONCLUSION: Biplane TEE is an accurate, valuable, and safe addition to the perioperative care of patients with congenital heart disease. Although intraoperative TEE is not needed in all operations for congenital heart disease, we recommend that biplane intraoperative TEE be performed routinely during modified Fontan procedures, subaortic resections, and other intracardiac operations for complex congenital cardiac malformations.

Adolescent↗
PubMed2005

Causative organism influences clinical profile and outcome of infective endocarditis in pediatric patients and adults with congenital heart disease.

BACKGROUND: Studies on microorganisms in a large cohort of patients with congenital heart disease (CHD) and infective endocarditis (IE) are rare. Using a nationwide survey, the relationship between causative microorganisms and clinical profiles in patients with CHD and IE was investigated. METHODS AND RESULTS: Data from 188 patients with CHD (pediatric patients (n=113), mean age, 6.2+/-4.9 years; adult patients (n=75), mean age 28.4+/-13.4 years) and IE from 60 institutions were analyzed. Causative microorganisms were Streptococcus species (94:50.0%), Staphylococcus species (68:36.2%), Haemophilus species (9:4.8%), Candida (5:2.7%), Pseudomonas species (4:2.1%) and other unclassified microorganisms (8:4.3%). Staphylococcal IE was observed significantly higher in perioperative IE (11/16), in cyanotic patients (32/73) and patients younger than 1 year old (11/16). Streptococcal IE was observed significantly higher in acyanotic patients (64/109) and patients aged 16 years or older (48/75). Total mortality was 20/188 (10.6%) and was high for candidial (2/5; 40%) and pseudomonal IE (2/4; 50%). Mortality was highly associated with younger age, especially infants (5/16), and methicillin-resistant staphylococcal IE (6/15). CONCLUSIONS: The causative microorganisms are significantly related to the clinical profile and outcome in patients with IE and CHD. These results form the basis for selecting appropriate antibiotics for prevention and management.

Adolescent↗