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PubMed1996

Training in cardiac catheterization at high-volume and low-volume centers: is there a difference in case mix?

Current guidelines recommend that cardiology trainees participate+ in a minimum of 100 cardiac catheterization procedures during their clinical training (volume minimum level 1 training). To examine the differences in case mix seen by cardiology trainees during their training in cardiac catheterization, we examined the first 100 cases done by two trainees at different hospitals. One hospital was a high-volume center performing > 5500 procedures/year, and the other was a low-volume center performing < 1500 procedures/year. Demographic and clinical characteristics of the patients undergoing cardiac catheterization were similar at the two hospitals. Indications were also similar, with the only exception being a higher rate of urgent/emergent cases among patients seen at the low-volume center (8% vs 1%). Minor differences in procedural techniques were present at the two hospitals, with the trainee at the high-volume center having more experience with arm cases (4% vs 0%) and left ventriculograms (77% vs 48%) and the trainee at the low-volume center having more experience with right-heart catheterizations (36% vs 11%) and temporary pacing wires (5% vs 2%). Neither trainee had significant experience with valvular or adult congenital heart disease (2%, low-volume center; 1%, high-volume center). These results suggest that current volume minimums may ensure relatively uniform case mix among physicians who are training in cardiac catheterization at different centers. However, training may be deficient in several areas such as valvular heart disease, congenital heart disease, and arm cases.

Adult↗

PubMed2003

Infective Endocarditis in Adults with Congenital Heart Disease.

Although modern medicine has improved survival in congenital heart disease dramatically, the long-term course carries a risk of late complications. The incidence of infective endocarditis in adults with congenital heart disease is more than 10 times higher than that of the normal population. Identification of the high-risk groups, a high diagnostic alert, and no random prescription of antibiotics to these patients are important issues to health care providers. There is also a need for a structured education of patients on preventive measures and symptoms of infective endocarditis.

Journal Article↗

PubMed2004

Occupational performance challenges for children with congenital heart disease: a literature review.

BACKGROUND: Paediatric specialists have advocated for exercise training programs for children with congenital heart disease without addressing other common deficits, such as behavioural and psychological problems. Despite evidence of the role of occupational therapy in rehabilitation for adults with cardiac disease, there has been little published about occupational therapy for children with congenital heart disease. PURPOSE: This literature review on the outcomes of congenital heart disease, guided by the Canadian Model of Occupational Performance, highlights the comprehensive needs of these children. RESULTS: The findings of the review are that congenital heart disease can have wide-ranging neurological and psychosocial implications. This suggests that occupational therapists should be concerned about limitations in the participation of these children in age-appropriate childhood occupations related to self-care, productivity or leisure. PRACTICE IMPLICATIONS: As cardiac rehabilitation program planning becomes more commonplace for children, comprehensive programs that promote optimal occupational performance need to be developed, implemented and evaluated.

Activities of Daily Living↗

PubMed1994

Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus.

In a series of 416 women with congenital heart disease seen in the Royal Brompton National Heart and Lung Hospital, London, and the Hospital Giovanni Bosco, Torino, Italy, there were 822 pregnancies. The outcomes of 96 pregnancies in 44 patients with cyanotic congenital heart disease were studied. Patients with the Eisenmenger reaction were excluded. Patients were divided arbitrarily into groups according to the type of maternal congenital cardiac anomaly, and factors influencing maternal and fetal outcome were evaluated. The incidence of maternal cardiovascular complications was high (32%), with one death from endocarditis 2 months after delivery. Forty-one (43%) of 96 pregnancies resulted in a live birth; 15 (37%) were premature. Mean weight of full-term infants was 2575 g. Univariate analysis suggested that maternal disease, Ability Index, hemoglobin, and arterial oxygen saturation before the pregnancy were factors that discriminated between successful and unsuccessful fetal outcome, with hemoglobin and arterial oxygen saturation being the most important predictors. Women with cyanotic congenital heart disease can go through pregnancy with a low risk to themselves, with frequent treatable complications, but there is a high incidence of miscarriage, premature births, and low birth weights. An incidence of congenital heart disease in the fetus of 4.9% (2 of 41 live births) is higher than that found in the normal population.

Adolescent↗

PubMed1986

Hearing loss in children with congenital heart disease: a preliminary report.

Life expectancy for children with serious congenital heart defects has dramatically increased in recent years due to advances in medical and surgical care. Anatomic studies of temporal bones in patients with non-syndromic congenital heart disease have demonstrated a high incidence of middle and inner ear anomalies. Systematic study of auditory function in these patients has not been reported. Fifty children with serious congenital heart disease have been evaluated with otologic examination and auditory brainstem-evoked response (ABR) testing. The patients ranged in age from 3 days to 17 years-8 months. Eight patients had hearing loss and normal otologic examinations, 14 patients had hearing loss and associated otitis media with effusion, and 28 patients had normal hearing by ABR testing. These results show a high incidence (16%) of hearing loss not associated with otitis media with effusion in the study population. We suggest that children with serious congenital heart disease are at high risk for hearing loss. This patient group will benefit from early identification, and appropriate medical and audiologic management.

Acoustic Stimulation↗

PubMed2004

State of the art catheter interventions in adults with congenital heart disease.

Catheter interventions in adults with congenital heart disease have rapidly advanced. Transcatheter valve repair and replacement techniques have been added to the existing spectrum of well-established procedures. This review summarizes current transcatheter management strategies for congenital cardiac anomalies seen in the adult population.

Adult↗

PubMed1989

Brain abscess associated with congenital heart disease.

Between 1952 and 1985, 25 cases of brain abscess with congenital heart disease were treated at the Department of Neurosurgery, Tianjin Medical College Hospital. Patients' ages ranged from 5 to 38 years. The most common form of congenital heart disease was tetralogy of Fallot, occurring in 13 cases. The abscesses were located in the parietal, frontal, and temporal lobes. The pus from the abscesses was sterile in 13 of 19 cases. In the rest, Streptococcus was the predominant organism. Twenty-three cases were treated by aspiration of the abscess through a burr hole. In two cases, initial aspiration was followed by excision of the abscess. The mortality rate of the whole group was 32%. The etiology of brain abscess in patients with congenital heart disease is discussed.

Adolescent↗

PubMed1995

Adults with congenital heart disease: patient knowledge of endocarditis prophylaxis.

OBJECTIVE: To determine whether adults with congenital heart disease have adequate knowledge of infective endocarditis and endocarditis prophylaxis and to ascertain whether an educational program effectively improves patient knowledge and compliance. MATERIAL AND METHODS: We asked 102 consecutive patients to complete a 12-question survey to assess their knowledge of heart disease, infective endocarditis, and endocarditis prophylaxis. RESULTS: Of 102 patients, 100 (98%) completed the questionnaire. Sixty-eight patients knew the name of their heart disease. Fifty patients correctly defined endocarditis, but only 43 knew hygiene measures that could prevent endocarditis. Ninety-six patients knew that they needed to take "a medicine" before dental procedures, and 76 of those patients (79%) knew that an antibiotic was necessary. Patient use of cardiac medications and a history of endocarditis correlated significantly with knowledge of endocarditis. Patients who had been to the Adult Congenital Heart Disease Clinic at least once knew endocarditis prevention measures and the importance of regular dental and cardiology follow-up significantly more frequently than did first-time attendees. Despite educational counseling, however, patient recall of endocarditis and its prevention is disappointing. CONCLUSION: Many adults with congenital heart disease have inadequate knowledge of their cardiac lesion, endocarditis, and endocarditis prophylaxis. Educational efforts for adults with congenital heart disease need to be updated and reinforced regularly.

Adolescent↗

PubMed1994

Adult congenital heart disease.

There are approximately 500,000 adults in the United States with congenital heart disease, and this group is growing at 5% per year. Adult cardiologists are, for the most part, poorly trained in the treatment of congenital heart disease; pediatric cardiologists, on the other hand, work in children's hospitals where it is difficult to care for adults. It is important, therefore, to review the current literature as it relates to adult congenital heart disease. This report details the long-term follow-up of patients who were operated on in childhood for congenital heart disease and are now adults. In addition, it reviews information related to the social adaptation of adult congenital heart patients. There are a number of interesting new reports on specific adult congenital heart anomalies. Atrial septal defects as well as the relationship between a patent foramen ovale and stroke are also reviewed.

Adult↗

PubMed1984

Anesthetic complications in surgery for congenital heart disease.

Anesthetic complications in 500 consecutive operations for congenital heart disease were studied and their contribution to mortality and morbidity were assessed. The great majority of the cases were young and sick children in ASA Class III, IV, or V with complex congenital heart disease. Anesthetic mortality was zero and hospital mortality was 6.3%. Anesthetic complications occurred in 2% of the cases. Four major complications were seen, including two tension pneumothoraces, one episode of severe hypotension on induction, and a breathing circuit malfunction on induction leading to bradycardia and hypotension. Six minor complications included two cases of premature extubation that required reintubation postoperatively, one case of subglottic edema, airway obstruction from inadvertent tracheal insertion of an esophageal temperature probe, one corneal abrasion, and one case of erosion of the nasal ala secondary to a nasotracheal tube. Half of the complications were probably preventable, but the other half of the complications occurred despite specific attempts at their prevention.

Adjuvants, Anesthesia↗

PubMed1986

Exercise recommendations for adolescents after surgery for congenital heart disease.

Due to recent improvements in medical and surgical therapy, many children with congenital heart disease are now surviving to adolescence and beyond. The pediatrician is thus presented with a new challenge: caring for the adolescent and young adult with surgically corrected congenital heart disease. The purpose of this paper is to provide recommendations regarding exercise and sports participation for adolescents after surgical repair of congenital heart disease. Four major defects are discussed in detail: aortic stenosis, coarctation of the aorta, tetralogy of Fallot and transposition of the great arteries. Exercise restrictions must be based on a thorough knowledge of each patient's postoperative hemodynamic and electrophysiologic abnormalities, and his or her response to exercise. It is emphasized that, with reasonable limits, adolescents with surgically repaired congenital heart disease should be allowed to pursue a normal, active life-style.

Activities of Daily Living↗

PubMed1999

Efficacy of routine fetal ultrasound screening for congenital heart disease in Isère County, France.

Recent literature has revealed different percentages of prenatal detection of congenital heart disease. Therefore we chose to analyse the sensitivity of this screening in Isère county and to scrutinize to which extent factors like severity of the anomaly, extracardiac anomalies and maternal age influence the sensitivity. This retrospective study covers the period from 1989 to 1995. All the pregnancies with congenital heart disease in Isère county in France were reviewed and analysed, which was possible thanks to our registry of congenital anomalies. In this period, 316 cases were registered in the central database. We obtained an overall sensitivity of 34.8 per cent. By splitting the different malformations into two groups we got a detection rate of 53.7 per cent for major malformations and 26.7 per cent for other abnormalities. This difference is significant. Nevertheless, the sensitivity remains quite low. This result stresses the need for better education of investigators in primary care units, particularly because the prenatal diagnosis of congenital heart disease has a major impact on the outcome of pregnancy, which can be seen in the increased number of abortions in this group.

Adult↗

PubMed2003

Surgical treatment of arrhythmias related to congenital heart diseases.

BACKGROUND: Supraventricular arrhythmias complicate operated and unoperated congenital heart disease, especially when atrial dilatation coexists. METHODS: We evaluated the results of intraoperative ablation in a group of 23 patients with chronic supraventricular tachyarrhythmias (mostly intraatrial reentry) that were unresponsive to conventional medical therapy. All procedures were performed consecutively between September 1999 and November 2001. Ablation was done during redo operations (Fontan conversion to total cavopulmonary connection in 16 patients) in 18 patients and during primary surgical correction in 5 patients. The mean age at operation was 25 +/- 12 years (2 to 50 years). Cryoablation was done in 10 patients and radiofrequency ablation in 13 patients. Nineteen patients had ablation in one atrium and 4 had ablation in both atria. A generous atrial reduction was always performed at the end of the operation. RESULTS: The operative mortality rate was 13% (3 patients) from causes unrelated to ablation. In 20 survivors, the ablation was effective immediately. Eight patients required a permanent pacemaker. During a mean follow-up of 22 +/- 4 months, atrial arrhythmias recurred in 25% (5 patients) and were controlled with medical therapy, whereas 1 patient required pacemaker implantation. CONCLUSIONS: Intraoperative treatment of unresponsive atrial tachyarrhythmias associated with operated or unoperated congenital heart disease is feasible and the midterm results are encouraging.

Adolescent↗

PubMed1976

Impairment of platelet adhesiveness and platelet factor 3 activity in cyanotic congenital heart disease.

In 33 children with cyanotic congenital heart disease the platelet function has been studied. The most significant changes were reduced platelet adhesiveness to glass and impaired availability of platelet factor 3 in nearly 50% of the patients. Although clot retraction was poor in 84% of them, thrombocytopenia and prolonged bleeding time were not significant features.

Blood Coagulation Factors↗

PubMed1988

Reversibility of cortical hyperostosis following long-term prostaglandin E1 therapy in infants with ductus-dependent congenital heart disease.

Two neonates with complex cyanotic congenital heart disease, receiving long-term prostaglandin E1 infusion, for 59 and 78 days respectively, demonstrated significant radiographic changes of symmetric cortical hyperostosis of the long bones. Bone biopsies from one of the patients elucidated the histological changes and serial X-rays revealed regression of the hyperostosis. Long-term follow-up after 13 months did not reveal any late bony changes or growth disturbance.

Alprostadil↗

PubMed2003

[Genetics of congenital heart diseases].

New insights into the genetics of congenital heart diseases in human beings have been drawn these past years. The identification of genes for heart defects have led to a new clinical approach of these malformations in children and their families. These progresses have been made with the help of positional cloning as well as with the analysis of mouse models. These findings also yielded a new complexity in understanding the development of cardiac defects and led to revise the different classifications for congenital heart defects. Pediatric cardiologists have also improved their efficiency in defining cardiac phenotypes in affected individuals and in pedigrees with recurrent malformations. Genetic heterogeneity has made the molecular approach of a given defect difficult. In addition, intrafamilial variability still has scarce explanations. Finally, the contribution of epigenetic factors has to be kept in mind in specific conditions such as twin gestations. Clinical consequences of these findings remain at the present time limited for the patients themselves but in particular cases, genetic counseling has been dramatically improved.

Animals↗

PubMed1988

The association between immunodeficiency and congenital heart disease.

The predilection of children with congenital heart disease (CHD) to infection may be explained in part by an underlying immunodeficiency disorder. Some 13 syndromes in which immunodeficiency and CHD may coexist have been reported in the medical literature. In addition, immunoglobulin and T-cell deficiencies have been found in nonsyndromal patients with CHD. The diagnosis of immunodeficiency should be entertained in such children, as early recognition of an immunodeficiency disorder can result in improved antimicrobial and immunological management.

Child↗

PubMed1995

Establishing an adult congenital heart disease clinic.

The majority of babies born with congenital heart disease are now surviving to adulthood. Some have not required cardiac surgery but many have had previous palliation or repair. Because few lesions are "cured" by surgery, these patients need continued expert care to ensure both quality and length of life. Patients need to be treated by physicians with expertise in adult congenital heart disease, with adequate facilities available for both noninvasive and invasive imaging. Access to inpatient facilities also should be available, and a collaborative relationship with pediatric cardiologists, high-risk obstetricians, and anesthesiologists established. Patients need advice regarding endocarditis, employability, exercise, noncardiac surgery, life and health insurance. It is hoped that, with increasing awareness of these patients' problems, adequate lifetime care will be provided for them.

Adult↗