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PubMed1981

Dermatoglyphs in congenital heart disease.

The palmar dermatoglyphs of 800 patients with anatomically proven congenital heart disease were compared with prints from 1000 controls. A review of the previous studies revealed major technical deficiencies, and the present study failed to confirm most of the previously reported positive findings. An overall increase in the incidence of hypothenar patterns was found, probably explaining the previous suggestion of increased atd angle in congenital heart disease. A large number of statistical comparisons inevitably produced a few 'significant' results, most of which were inconsistent in various ways. Two percent of cases were found to have rare epidermal ridge malformation, ridge dissociation. The nature of the relationship between this and congenital heart disease is obscure. Claims that there are diagnostically useful dermatoglyphic changes in congenital heart disease can be disregarded.

Dermatoglyphics↗
PubMed2004

Counselling following a diagnosis of congenital heart disease.

Counselling the parents following a diagnosis of fetal congenital heart disease (CHD) is as important a task for the fetal cardiologist, as the skill involved in achieving an accurate diagnosis. The counsellor will base prognosis not only on the diagnosis itself but also on the security of diagnosis, the stage in gestation and potential for change, the association with extracardiac malformations and the known results of treatment. Depending on the gestational age and legal situation the counsellor is operating in, termination of pregnancy may be one of the options to consider and one that should always be raised in discussion. Thus, the parents may be in the position of making a crucial decision concerning the management of the pregnancy on the basis of the information received, so it is vital that the counsellor is truly able to communicate with them, whatever be their level of understanding.

Counseling↗
PubMed2005

Indications and limitations for a neonatal pulse oximetry screening of critical congenital heart disease.

AIMS: Critical congenital cardiovascular malformations (CCVMs) require surgical correction during the first month of life, physical examination is unable to detect >50% of affected infants. An oximetry screening has been previously proposed. Our aim was to verify the usefulness and consistency of a pulse oximetry screening for early detection of CCVMs in a small size nursery. METHODS: A single determination of SpO2 was performed on 5292 consecutive apparently healthy newborns, discharged from nursery at a median age of 72 h during the period May 1, 2000 and November 30, 2004. Infants showing signs of congenital heart disease before the screening and those with a prenatal diagnosis were excluded. Cardiac ultrasound was performed on all infants with SpO2< or =95% at >24 h. The accuracy of the screening in identifying CCVMs was assessed by receiver-operating characteristic (ROC) curves analysis. RESULTS: We found 2 (0.038%) true positives, 1 (0.019%) false negative, 1 (0.019%) false positive, and 5288 (99.92%) true negatives. Prevalence of critical CCVMs was 1 in 1764. Clinical follow-up showed no evidence of CCVMs in the negative cases. A pulse-oximetry cut-off value of < or =95% showed 66.7% sensitivity (95% CI: 11.6-94.5), 100% specificity (95% CI: 99.9-100.0), 50% positive predictive value, 100% negative predictive value and AUC of 0.833 (standard error: 0.145) (95% CI: 0.823 to 0.843) in identifying CCVMs. CONCLUSIONS: Our findings indicate that pulse oximetry is a non-invasive and specific screening tool for an early detection of CCVMs, and is easily applicable to a small size nursery.

Female↗
PubMed2000

Clinical and basic laboratory assessment of children for possible congenital heart disease.

Accurate and cost-effective detection of congenital heart disease is a priority for the primary care physician, but there are many diagnostic modalities and strategies available. Within the past year, investigators have reported factors that contribute to the failure to diagnose congenital heart disease before autopsy. Recent research also highlights the strengths and weaknesses of the clinical evaluation by the primary care physician. An examination by a pediatric cardiologist is a more specific discriminator between heart disease and innocent murmur and can, in many instances, eliminate the expense of relying on echocardiography for diagnosis. Current reports document substantial limitations of diagnostic utility of electrocardiography and chest roentgenography. Suspicions that echocardiography can be misleading when performed in laboratories without expertise in congenital heart disease are confirmed in recent organized comparisons of accuracy. High-quality, well-designed tele-echocardiography programs are shown to be effective solutions when maldistribution of pediatric cardiology services hampers diagnostic efficiency.

Adult↗
PubMed1995

Pregnancy in patients with congenital heart disease.

Pregnancy and delivery in women with congenital heart disease remain inadvisable for patients at high risk due to the following congenital diseases: (1.) Severe left ventricular outflow tract obstruction (increase of pressure gradient); (2.) Eisenmenger syndrome (increase of right to left shunt with worsening of cyanosis and fetal growth retardation); (3.) Marfan syndrome with enlarged aortic root (risk of aortic dissection). In women suffering from cyanotic congenital heart disease the main risk is fetal complications. These are correlated to oxygen saturation and to the type of maternal disease, particularly if a palliative shunt operation has never been performed. Women with left to right shunt, right outflow tract obstruction or previous correction of the disease can go through pregnancy with low risk of mortality. The complications (mainly heart failure and arrhythmias) can be well managed with medical treatment, and the fetal outcome is similar to that in the general population.

Eisenmenger Complex↗
PubMed1993

Adult congenital heart disease: principles and management guidelines: Part II.

The treatment of congenital heart disease may be palliative because many residua and sequelae persist into adulthood. Except for trivial lesions and anomalies such as PDA or secundum ASD where surgical cure is possible, continued supervision is mandatory. These patients deserve expert medical assessment from adult cardiologists and from other specialists when appropriate. The prevalence of postoperative adult congenital heart disease is increasing: by the year 2000 it is estimated that over 2000 in each million of the adult population will have congenital heart disease, one third of these having undergone cardiac surgery. It is important that some adult cardiologists in each major centre develop skills in adult congenital heart disease for this new patient population.

Adult↗
PubMed2004

[National registry and DNA-bank of patients with congenital heart disease: the CONCOR-project].

Although survival of patients with congenital heart disease has dramatically improved since surgical repair has become available, cure is seldom achieved. Exact data on long-term outcome are not available, however, because a national registry is lacking. Furthermore, little is known about the role of genetic defects in the development of congenital heart disease. The CONCOR-project (CONgenital CORvitia) has been set up to facilitate the investigation of the long-term outcome and molecular basis of specific congenital heart defects and their treatment. It will also facilitate the development of an efficient organisational structure for the improvement of healthcare for patients with congenital heart disease.

Cardiac Surgical Procedures↗
PubMed1997

Sotalol for atrial tachycardias after surgery for congenital heart disease.

Atrial tachycardias, in particular atrial flutter after surgery for congenital heart disease, is associated with a high mortality. Treatment with various antiarrhythmic drugs and/or antitachycardia pacemakers is not very successful. Sotalol, a Class III drug, has shown to be a promising drug in adults with atrial tachycardias. However, the experience with sotalol in children after surgery for congenital heart disease is limited. Therefore, we describe our results here. Between December 1990 and February 1997, 26 children with atrial tachycardias, most of them with atrial flutter or fibrillation (n = 20), after surgery for congenital heart disease were treated with sotalol orally. The age of the children at the start of treatment was 7.5 +/- 5.8 years (mean +/- SD). The time interval between surgery and the start of atrial tachycardia ranged from 1 day to 14.3 years (3.8 +/- 3.8 years). Conversion to sinus rhythm was achieved in 16 out of 22 hemodynamically stable children with a dosage of 4.0 +/- 1.6 mg/kg per day. The six children without sinus rhythm on sotalol and four hemodynamically unstable patients were treated prophylactically with sotalol after DC cardioversion for their tachycardias. Two children complained of mild transient fatigue. Heart rate decreased during therapy (95 +/- 33 vs 81 +/- 21 beats/min; P = 0.01). QTc-intervals did not change. Proarrhythmias such as torsades de pointes were not encountered. Two children with a preexistent sick sinus syndrome showed aggravation of bradycardia and needed pacemaker implantation. The percentage of children with a recurrence-free interval of 1 and 2 years was 96% and 81%, respectively, for all atrial tachycardias, and 92% and 66% for atrial flutter. The recurrences of atrial tachycardias during the follow-up period, which ranged from 0.1-6.1 years (2.5 +/- 1.8 years) could be treated with only an increase of the dosage of sotalol in all but one patient. We conclude that sotalol is an effective drug for the treatment and prevention of atrial tachycardia in children after surgery for congenital heart disease.

Administration, Oral↗
PubMed1996

Lung transplantation in children with pulmonary hypertension and congenital heart disease.

Pulmonary vascular disease with or without associated congenital heart disease is the indication for lung transplantation in 30% of all children requiring this procedure. Although many of these children will have primary pulmonary hypertension, a substantial number are children whose pulmonary vascular disease is either associated with a congenital heart lesion or secondary to it. Heart-lung transplantation has been performed in these children in the past, but because of the scarcity of availability of donor heart-lung blocks, lung transplantation with repair of associated congenital cardiac lesions has of necessity been performed more frequently. As one might expect, the operative and postoperative management of these patients is a good deal more complex than pulmonary transplantation for primary lung diseases. The early mortality is correspondingly higher as well, although survival beyond that time is quite similar to that of lung transplantation for other diseases. Factors that increase the early mortality include previous thoracic procedures and the complexity of the underlying cardiac diagnosis. Although the overall experience with this complex group of patients is small, the results seem similar to the prior experience with heart-lung transplantation. Thus, we remain optimistic that this will continue to be a viable form of therapy for children with pulmonary vascular disease associated with congenital heart lesions. The quality of life of the surviving patients is excellent and limited only by those factors impacting upon lung transplantation in general, such as rejection, infection, and the development of bronchiolitis obliterans.

Adolescent↗
PubMed1991

Congenital heart disease, parental stress, and infant-mother relationships.

The effect of congenital heart disease on early social relationships was assessed by observing 42 infants with the disease and 46 healthy infants in a standardized laboratory setting with their mothers. Significantly fewer infants with congenital heart disease, in comparison with healthy peers, were considered to have secure relationships with their mothers. The quality of the infant-mother relationship in the group with congenital heart disease was not related to parents' reports of their own stress or psychologic well-being. Severity of illness did not have a direct effect on the quality of the infant-mother relationship, but securely attached infants showed more subsequent improvement in health than insecurely attached peers showed. Attention to the infant-mother relationship in clinical care may improve the social development of babies with congenital heart disease and may have positive effects on physical health as well.

Adult↗
PubMed1995

Nutritional status of children with congenital heart disease.

OBJECTIVE: To assess the nutritional status of children with congenital heart disease. DESIGN: Six anthropometric, 24 biochemical, and five haematological markers of nutritional wellbeing were measured in children with congenital heart disease. SETTING: The west of Scotland. PATIENTS: 48 children admitted consecutively for surgical correction of congenital heart disease. MAIN OUTCOME MEASURES: Height, weight, and triceps and subscapular skin fold thicknesses were considered abnormal if they were below the third centile compared with standard reference data for age matched British children. Mid-arm circumference and arm muscle circumference were considered abnormal if they fell below the fifth centile compared with standard data. Biochemical and haematological data were compared with age matched and locally validated laboratory normals. RESULTS: A marked degree of undernutrition was evident in all children; 52% had weight less than the third centile, 37% were below the third centile for height, and 12.5% were below the third centile for triceps skin fold thickness and 18.8% for subscapular skin fold thickness. Mid-arm circumference and arm muscle circumference were below the fifth centile in 20.1% and 16.7% of children respectively. Five or more of the 29 biochemical and haematological measurements were abnormal in 83.3% of patients; 10 or more were abnormal in 12.5% of patients. CONCLUSIONS: Children with congenital heart disease are frequently undernourished, irrespective of the nature of cardiac defect and the presence or absence of cyanosis.

Arm↗
PubMed1999

Seasonality in live births with congenital heart disease in Malta.

This study was carried out to analyse seasonal variations in live births with congenital heart disease in the overall population of Malta. Included were all patients diagnosed as having congenital heart disease by echocardiography, cardiac catheterization, surgery or post mortem, by 1 year of age, and who were born between in Malta between 1990 and 1994. Results were analysed by X2 and by Edwards' cyclic method. There were 231 cases of live born congenital heart disease, which were divided into 114 cases not requiring intervention and 117 cases requiring intervention. Although the overall prevalence of congenital heart disease did not demonstrate any seasonal variation, the lesions requiring intervention showed a significant peak in September by Edwards' method (p = 0.03), which was enhanced by seasonal analysis (p = 0.003). These results were confirmed by conventional X2 and X2 for trend. The lesions not requiring intervention, which were comprised almost entirely (96%) of mild pulmonary stenosis and small ventricular septal defects, failed to show any seasonal trend. The literature regarding such seasonal trends in these malformations and statistical analysis of seasonality of congenital heart disease are reviewed. An environmental factor such as a maternal viral infection or treatment of such infections during the first trimester of pregnancy from November to January may precipitate congenital heart disease in predisposed Maltese foetuses.

Female↗
PubMed1986

HLA antigens in multiplex families with isolated congenital heart disease.

Twelve multiplex families with isolated congenital heart diseases (CHD) were included in the study. In 9 families the types of CHD in the living sibs were concordant and in the other 3 were discordant. All families were subjected to the following: (1) Pedigree construction, (2) clinical examination of the parents, affected and unaffected sibs, (3) investigations of patients, to establish the diagnosis, (4) chromosomal analysis for the patients, (5) HLA antigen typing for the parents, affected and unaffected sibs for 9 antigens at the A locus, 15 at B and 6 at the DR locus. The results can be summarized as: (a) sibs with two different types of CHD showed identical haplotypes; (b) the segregation of haplotypes among disease sibpairs is inconsistent with Mendelian segregation; (c) increased frequency of concordant HLA haplotypes among diseased siblings; (d) Morton's exact test revealed that the data best fit a hypothesis of a recessive susceptibility gene, linked to HLA.

Disease Susceptibility↗
PubMed2001

What do adult patients with congenital heart disease know about their disease, treatment, and prevention of complications? A call for structured patient education.

OBJECTIVE: To assess how much adults with congenital heart disease understand about their heart defect, its treatment, and the preventive measures necessary to avoid complications. DESIGN: Descriptive, cross sectional study. SETTING: Adult congenital heart disease programme in one tertiary care centre in Belgium. PATIENTS: 62 adults with congenital heart disease (47 men; 15 women), median age 23 years. MAIN OUTCOME MEASURES: Patients' knowledge was assessed during an outpatient visit using the Leuven knowledge questionnaire for congenital heart diseases, a 33 item instrument developed for this study. RESULTS: Patients had adequate knowledge (> 80% correct answers) about their treatment, frequency of follow up, dental practices, occupational choices, appropriateness of oral contraceptives, and the risks of pregnancy. Knowledge about the name and anatomy of the heart defect, the possibility of recurrent episodes of endocarditis during their lifetime, and the appropriateness of different physical activities was moderate (50-80% correct answers). There was poor understanding (< 50% correct answers) about the reasons for follow up, the symptoms of deterioration of the heart disease, the definition, characteristics, and risk factors of endocarditis, the impact of smoking and alcohol on the heart disease, the hereditary nature of the condition, and the suitability of intrauterine devices as contraceptives. CONCLUSIONS: Adults with congenital heart disease have important gaps in their knowledge about their condition. The results of this study can be used as a basis for developing or optimising structured educational interventions to enhance patients' health behaviour.

Adult↗
PubMed1995

Heart transplantation in children with congenital heart disease.

OBJECTIVES: The aim of this study was to describe heart transplantation in children with congenital heart disease and to compare the results with those in children undergoing transplantation for other cardiac diseases. BACKGROUND: Reports describe decreased survival after heart transplantation in children with congenital heart disease compared with those with cardiomyopathy. However, transplantation is increasingly being considered in the surgical management of children with complex congenital heart disease. Present-day results from this group require reassessment. METHODS: The diagnoses, previous operations and indications for transplantation were characterized in children with congenital heart disease. Pretransplant course, graft ischemia time, post-transplant survival and outcome (rejection frequency, infection rate, length of hospital stay) were compared with those in children undergoing transplantation for other reasons (n = 47). RESULTS: Thirty-seven children (mean [+/- SD] age 9 +/- 6 years) with congenital heart disease underwent transplantation; 86% had undergone one or more previous operations. Repair of extracardiac defects at transplantation was necessary in 23 patients. Causes of death after transplantation were donor failure in two patients, surgical bleeding in two, pulmonary hemorrhage in one, infection in four, rejection in three and graft atherosclerosis in one. No difference in 1- and 5-year survival rates (70% vs. 77% and 64% vs. 65%, respectively), rejection frequency or length of hospital stay was seen between children with and without congenital heart disease. Cardiopulmonary bypass and donor ischemia time were significantly longer in patients with congenital heart disease. Serious infections were more common in children with than without congenital heart disease (13 of 37 vs. 6 of 47, respectively, p = 0.01). CONCLUSIONS: Despite the more complex cardiac surgery required at implantation and longer donor ischemic time, heart transplantation can be performed in children with complex congenital heart disease with success similar to that in patients with other cardiac diseases.

Adolescent↗
PubMed2005

Clinical course and complications of infective endocarditis in patients growing up with congenital heart disease.

BACKGROUND: Although a high number of patients with congenital heart disease (CHD) undergo surgical palliation or definite correction up to adolescence, adult congenital heart disease (ACHD) may remain a potential lifelong risk factor for infective endocarditis (IE) in patients growing up with congenital heart disease (GUCH). METHODS: In a retrospective case study of a tertiary care center long-term clinical course and complications of patients with IE and GUCH were analysed. RESULTS: Data of 52 patients with CHD, who fulfilled the Saiman criteria for infective endocarditis and were treated between April 1986 and March 2001, were identified: Risk factors for infective endocarditis were previous cardiovascular operation (51.9%), use of foreign material (38.5%), dental or other surgical procedures without recommended antibiotic prophylaxis (25.0%), or cardiac catheterization (5.8%). Staphylococcal (38.9%) or streptococcal species (35.2%) were cultivated in most cases as causative microorganisms. Complications were: recurrence of IE (7.7%), septic embolisms (30.8%) leading to central nervous complications (7.7%), embolism of pulmonary arteries (7.7%), renal arteries (1.9%), arteries of the extremities (9.6%), or infarction of spleen (1.9%). Other cardiac (23.1%) or extracardiac (13.5%) complications were frequent. The need of re-operations during or after IE was high (67.3%). The hospital mortality was 1.9%, late mortality was 7.7%. CONCLUSIONS: Patients with IE and CHD show a broad clinical spectrum of cardiac and extracardiac complications. They may lead to a complicative short- and long-term course with the potential risk of death and a high number of re-operation. Efforts have to be made to improve long-term outcome of patients with ACHD by an interdisciplinary cooperation.

Adolescent↗
PubMed1986

Pulse oximetry during cardiac catheterization in children with congenital heart disease.

One hundred and five children with congenital heart disease were monitored by pulse oximetry during cardiac catheterization. Excellent correlation (r = 0.95) was found between oxygen saturation values obtained with pulse oximetry and those obtained from arterial blood in 133 data pairs. This correlation was described by the regression equation y = 0.91x + 8.1. The correlation was also excellent in 47 data pairs with saturation values of less than 90% (r = 0.94, y = 0.93x + 6.0) from 36 cyanotic children. The clinical usefulness of pulse oximetry in the early recognition of decreased pulmonary blood flow or partial airway obstruction was demonstrated. Early diagnosis of changes in oxygenation was especially helpful in children with cyanotic congenital heart disease, in whom small changes in arterial oxygen tension may cause large changes in oxygen saturation.

Adolescent↗