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PubMed1987

Maternal transmission of congenital heart diseases: new recurrence risk figures and the questions of cytoplasmic inheritance and vulnerability to teratogens.

A review of 8 studies involving 3,996 offspring of parents who have congenital heart disease revealed that the risk for all defects was substantially higher if the affected parent was the mother rather than the father. The risk ratio ranged from a high of 6.39 for aortic stenosis to a low of 1.48 for patent ductus arteriosus, and the ratio was statistically significant in aortic stenosis (p = 0.025) and ventricular septal defect (p less than 0.001). Despite the relatively large number of cases, there were still too few patients to reveal statistical significance for a malformation such as atrioventricular canal, in which there were 5 affected offspring among 36 children of mothers who had atrioventricular canal and no affected children among 16 offspring of affected fathers (p = 0.12). The possible reasons for the preponderance of affected offspring of mothers with a congenital heart disease was studied in the context of various modes of inheritance and maternal physiology. The preliminary conclusion is that although many familial cases of congenital heart disease are compatible with multifactorial inheritance and vulnerability to teratogens, an important subset of cases, particularly in some high-risk families, may be better explained by cytoplasmic inheritance than by multifactorial or mendelian modes. Current genetic counseling should take into account the differences in risk to offspring of affected mothers while confirmation and further investigation proceeds.

Abnormalities, Drug-Induced↗
PubMed2000

Efficacy of leukocyte-depleted terminal blood cardioplegia in congenital heart disease.

OBJECTIVE: Activated leukocytes and their products play a significant role in reperfusion injury and cause microvascular occlusion--the 'no-reflow phenomenon'--which decreases coronary blood flow after the release of the aortic cross-clamp during open-heart surgery. This study was designed to evaluate the efficacy of leukocyte-depleted terminal blood cardioplegia in patients with right ventricular pressure or volume overloaded congenital heart disease undergoing intracardiac repair. SUBJECTS AND METHODS: Fourteen infants and children undergoing intracardiac repair for congenital heart defects were the subjects in this study. Leukocyte-depleted terminal blood cardioplegia was employed in seven patients (Group I). For the control, terminal blood cardioplegia with leukocytes was employed in seven patients (Group II). In order to evaluate the myocardial anaerobic metabolism, the arteriovenous difference in the pyruvate and lactate levels was compared between both groups. Blood samples were taken simultaneously from both the arterial limb of the bypass circuit and the coronary sinus immediately after, and at 5, 10, and at 20 minutes after, the release of the aortic cross-clamp. RESULTS: The coronary arteriovenous difference in the pyruvate and lactate levels demonstrated that the myocardial anaerobic metabolism in Group II was significantly higher than in Group I. The delta excess lactate demonstrated that myocardial hypoxia in Group II persisted significantly longer than in Group I. There was a tendency for the creatine kinase-MB level at 6 hr after surgery to be lower in Group I than in Group II. CONCLUSION: Leukocyte-depleted terminal blood cardioplegia may reduce the myocardial anaerobic metabolism in patients with right ventricular pressure or volume overloaded congenital heart disease undergoing intracardiac repair.

Blood↗
PubMed2001

Inhaled nitric oxide versus aerosolized iloprost in secondary pulmonary hypertension in children with congenital heart disease: vasodilator capacity and cellular mechanisms.

BACKGROUND: Inhaled nitric oxide (iNO) has been used to assess the vasodilator capacity of the pulmonary vascular bed in children with congenital heart disease and elevated pulmonary vascular resistance. Inhaled iloprost is a pulmonary vasodilator for the long-term treatment of pulmonary hypertension (PHT). Because these 2 vasodilators act through different pathways (release of cGMP or cAMP, respectively), we compared the pulmonary vasodilator capacity of each. METHODS AND RESULTS: A total of 15 children with congenital heart disease and PHT who had elevated pulmonary vascular resistance (preoperative, n=10; immediately postoperative, n=5) were first given 20 ppm of iNO for 10 minutes; then, after baseline values were reached again, they were given aerosolized iloprost at 25 ng. kg(-1). min(-1) for another 10 minutes. Finally, iNO and iloprost were given simultaneously for 10 minutes. With iNO, the pulmonary vascular resistance and systemic vascular resistance ratio decreased from 0.48+/-0.38 to 0.27+/-0.16 (P:<0.001). Similarly, iloprost decreased the ratio from 0.49+/-0.38 to 0.26+/-0.11 (P:<0.05). The combination had no additional effect on the resistance ratio. Plasma cGMP increased from 17.6+/-11.9 to 34.7+/-21.4 nmol/L during iNO (P:<0.01), and plasma cAMP increased from 55.7+/-22.9 to 65.1+/-21.2 nmol/L during iloprost inhalation (P:<0.05). CONCLUSIONS: In children with PHT and congenital heart disease, both iNO and aerosolized iloprost are equally effective in selectively lowering pulmonary vascular resistance through an increase in cGMP or cAMP, respectively. However, the combination of both vasodilators failed to prove more potent than either substance alone. Aerosolized iloprost might be an alternative to iNO for early testing of vascular reactivity and for the postoperative treatment of acute PHT.

Administration, Inhalation↗
PubMed1984

Investigation of a temporal cluster of left sided congenital heart disease.

During October and November 1977, eight newborns with critical congenital heart disease, six of these with left sided lesions, were admitted to a New Jersey newborn unit serving two predominantly rural counties with 9700 annual live births. The six left heart lesions (three cases of hypoplastic left heart, two of interrupted aortic arch, and one of aortic coarctation) represented 30% of all neonatal left sided lesions seen at the unit in the three year period 1976-8. The scan statistic for temporal clustering was significant (p less than 0.05). A case-control study was performed in which an average of four controls were matched to each case. A questionnaire was administered to the mothers of cases and controls concerning occupation, periconceptional and prenatal nutrition, radiation and chemical exposure, use of alcohol and tobacco, medications, immunisations, infections, and other exposures. No statistically significant differences between cases and controls were found on any of these items. Although no aetiology for this cluster of congenital heart anomalies could be found, it is of interest that three temporal clusters of fetuses or newborns with chromosomal trisomies have been reported in the medical literature whose conceptions were roughly contemporaneous with those of the infants in our series. These occurred in Rhode Island/Massachusetts, Maryland, and New York City. A speculative possibility is that these four point epidemics represented exposure to a common teratogenic agent, perhaps influenza B, in the winter of 1976/77 in the northeastern United States.

Aorta, Thoracic↗
PubMed1995

Congenital heart disease in adults.

Patients reaching adulthood with unoperated and operated congenital heart disease require attention to issues of exercise, antibiotic prophylaxis, contraception, and pregnancy. A careful clinical history is important to establish the degree of a person's disability, if any, and the symptoms responsible for the disability, whether due to heart failure, cyanosis, or both. The findings of a physical examination and a noninvasive evaluation, including electrocardiogram, chest x-ray film, and echocardiography, are often sufficient to establish a diagnosis and to assess the adequacy of a previous operation. Transesophageal echocardiography and magnetic resonance imaging are adjunctive procedures that are indicated when routine transthoracic echocardiography is limited. Cardiac catheterization may be necessary when the noninvasive data are ambiguous and to assess coronary artery disease (congenital and acquired) in patients considered for surgical therapy. Cardiac catheterization is increasingly therapeutic (such as percutaneous pulmonary balloon valvuloplasty) as well as diagnostic. Primary surgical repair or additional surgical palliative procedures should be considered in symptomatic adults. A patient with Eisenmenger's syndrome--severe pulmonary hypertension--is a special case that may be amenable only to transplantation.

Adult↗
PubMed1994

Adult congenital heart disease. An emerging population.

Although there are few cures for congenital heart disease (CHD), more patients are now surviving to adulthood. Possible causes of CHD include teratogenic, genetic, and random factors, or an interaction of various factors. The risk of CHD recurrence is dependent on its presumed mode of inheritance. Because there may be a greater risk of transmission as survival rates increase, at-risk parents and families need to recognize the potential benefits of prevention. For the adult survivors, life remains a constant challenge of coping with persistent health problems, uncertain long-term functional status, and the influence of societal values.

Adult↗
PubMed1991

Echocardiography as a tool for determining the incidence of congenital heart disease in newborn babies: a pilot study in Hong Kong.

We studied, with echocardiography as the main tool, the incidence of congenital heart disease in newborn babies in Hong Kong. The population examined was the 20,928 babies who were born alive in the Prince of Wales Hospital from January 1987 to December 1989. All had a thorough physical examination by a paediatrician after birth, and an estimated 95% of the babies who were discharged from hospital received another routine physical examination at one of the five local Maternal and Child Health Centres within two months of birth. All babies with either suspected congenital heart disease or multiple congenital abnormalities were referred to the paediatric cardiologists in the Prince of Wales Hospital for further cardiovascular assessment that included echocardiographic examination. In all, 492 babies had Doppler and cross-sectional echocardiographic studies. Various abnormalities of the cardiovascular system were diagnosed in 216 babies. Almost all the babies who died within two months of life underwent autopsy. This proved the presence of congenital cardiac malformations in 15 babies, of whom 10 had correct echocardiographic diagnoses during life. One had a patent arterial duct which was missed by the echocardiography. The other four babies did not have echocardiographic examination while alive, either because of early death or absence of clinical suspicion. In total, congenital cardiac malformations were confirmed in 221 babies by echocardiographic examination and autopsy. Excluding 82 premature babies with patency of the arterial duct, and 6 babies with transient tricuspid regurgitation, there were 133 cases of structural cardiac malformation in the studied population, giving an incidence of 6.35 per thousand live births.(ABSTRACT TRUNCATED AT 250 WORDS)

Echocardiography↗
PubMed1976

Brain abscess and congenital heart disease.

The clinical features and management of 18 patients with congenital heart disease and a brain abscess are reviewed. Except for one patient all had cyanosis. All abscesses were large, containing at least 50-60 ml of pus, and mostly unilocular. Bilocular and multilocular abscesses were also encountered. The organisms cultured from the pus had little relation to the mortality. Tapping may be the final investigation, and is also the treatment of choice. The high mortality (50%) is attributed to the delay in referral of these patients to a neurosurgical unit.

Adolescent↗
PubMedDate not supplied

Captopril-induced pulmonary infiltrates with eosinophilia in an infant with congenital heart disease.

We report a case of an infant with complex congenital heart disease who was placed on captopril for afterload reduction following cardiac surgery and subsequently developed pulmonary infiltrates with eosinophilia. The patient was readmitted with symptoms of rhinorrhea, poor feeding, and decreased activity level. She was found to have diffuse pulmonary infiltrates on chest radiograph and a marked peripheral eosinophilia without leukocytosis. After discontinuing captopril and starting systemic steroids, her symptomatology rapidly improved, and her eosinophilia and radiographic abnormalities both resolved.

Angiotensin-Converting Enzyme Inhibitors↗
PubMed1994

[Relationship between deep body temperature and hemodynamics in pediatric patients with congenital heart disease].

Relationship of the abdomen-sole deep body temperature difference (DBT) to the hemodynamics was examined in patients with congenital heart disease. Hemodynamic data were obtained by routine cardiac catheter examination, and DBT was measured during the catheterization. Significant positive correlation was found between DBT and systemic-pulmonary flow ratio (Qp/Qs) (r = 0.85, p = 0.001). DBT was maintained below 2 C degrees in almost every patient when the Qp/Qs was less than 2, whereas DBT rose rapidly when the Qp/Qs was more than 2. Furthermore, systemic blood flow and arterial-venous oxygen difference ranged widely within the normal values when DBT was less than 2 C degrees. On the other hand, when DBT was more than 2 C degrees, systemic blood flow and arterial-venous oxygen difference tended to show fixed values which were around the normal upper or lower limits. This means that systemic circulation is marginally adapted when the Qp/Qs is more than 2. The measurement of deep body temperature difference is noninvasive, easy and useful as a circulatory monitor in pediatric patients with congenital heart disease.

Adolescent↗
PubMed1993

Circulating endothelin in children with congenital heart disease.

OBJECTIVE: To evaluate whether circulating endothelin, a peptide that is thought to play a part in mediating vascular tone, might be high in pulmonary hypertensive congenital heart disease. DESIGN: A prospective study with a radioimmunoassay technique to estimate urinary and plasma endothelin concentrations. SETTING: A supraregional referral centre for patients with congenital heart disease. PATIENTS: The 12 hour urinary endothelin concentration in young children with an increased pulmonary blood flow (n = 24, median age eight months) were compared with those in children with right ventricular outflow tract obstruction (n = 14, median age 1.5 years) and with those in healthy controls (n = 16, median age 1.8 years). The concentrations were also measured in adolescents with irreversible pulmonary vascular disease (n = 17, median age 18 years) and compared with those in controls of similar age (n = 19, median age 18.5 years). Also the plasma concentrations in the left atrium and pulmonary artery were measured in young children with either high (n = 11, median age 10.8 months) or low (n = 5, median age 1.0 year) pulmonary blood flow, in the peripheral arterial and venous blood of young children with either high (n = 13, median age 10.8 months) or low (n = 6 median age 1.9 years) pulmonary blood flow, and in the peripheral venous blood of seven healthy young children (median age 1.7 years). RESULTS: The urinary excretion of endothelin was similar in young children of a similar age, whether they had high, low, or normal pulmonary blood flow. Also, urinary endothelin excretion in older patients with irreversible pulmonary vascular disease was similar to that in normal subjects of similar age. Urinary endothelin excretion in normal young children, however, was significantly greater than that in normal older subjects (p = 0.0001). There was no transpulmonary or arteriovenous difference detected in either children with high or low pulmonary blood flow, and plasma concentrations sampled from the left atrium, pulmonary artery, and systemic artery and vein were similar in both groups. CONCLUSION: There was no evidence to implicate circulating endothelin in the pathogenesis of pulmonary vascular disease.

Adolescent↗
PubMed1980

Left ventricular function in cyanotic congenital heart disease.

Left ventricular function was studied with quantitative biplane cineangiocardiography in 39 preoperative and 23 postoperative patients with cyanotic congenital heart disease. Diagnoses included pulmonary atresia or critical pulmonary stenosis with intact ventricular septum (group 1), tricuspid atresia (group 2) and pulmonary atresia with ventricular septal defect (group 3). Preoperative patients ranged in age from 1 day to 7 years and postoperative patients from 7 weeks to 23 years. Left ventricular end-diastolic volume was increased in preoperative patients in groups 1 and 2 (132 and 136 percent of normal, respectively) but was normal in patients in group 3. Left ventricular ejection fraction was decreased to a similar extent in preoperative groups 1 to 3: 0.54, 0.55 and 0.56, respectively. After a shunt procedure left ventricular end-diastolic volume increased to 228 and 266 percent of normal in groups 1 and 2, respectively, but remained within normal limits in group 3. Left ventricular ejection fraction was normal in postoperative group 1 patients, whose ages averaged 1.8 years, but remained decreased in group 2 and 3 patients, whose ages averaged 8.1 and 5.6 years, respectively. Duration of cyanosis and degree of left ventricular dilatation appear to be important variables in regard to pump function in patients with cyanotic congenital heart disease.

Adolescent↗
PubMed1989

Evaluation of pulmonary arterial morphology in cyanotic congenital heart disease by magnetic resonance imaging.

Before and after surgical therapy, the anatomy of the pulmonary arteries in cyanotic congenital heart disease is often distorted. Pulmonary arterial anatomy was evaluated by magnetic resonance imaging (MRI) and angiography in 20 patients, ages 3 months to 20 years, with cyanotic heart disease associated with decreased pulmonary blood flow. Excellent correlation between MRI and angiographic estimates of pulmonary artery diameter was obtained (main pulmonary artery, r = 0.96; right pulmonary artery, r = 0.93; left pulmonary artery, r = 0.96). A similar excellent correlation (kappa = 0.83) was found in the assessment of the presence and severity of proximal pulmonary arterial stenoses. However, stenoses in the peripheral pulmonary arteries visualized with angiography were missed with MRI. MRI and angiography showed complete agreement in determining the patency of 11 surgical shunts. MRI did not demonstrate all of the systemic collateral vessels present with angiography, and the distal connections of collaterals were not detected with MRI. MRI is comparable to angiography in the evaluation of central pulmonary arterial anatomy over a wide range of ages. These findings suggest an important role for noninvasive MRI in the serial evaluation of pulmonary artery morphology in patients with cyanotic congenital heart disease before and after surgical repair.

Adolescent↗
PubMed1970

Congenital heart disease in Ceylon.

An analysis of 555 consecutive cases of congenital heart disease in Ceylon has shown that for the whole series interatrial septal defect is the commonest cardiac lesion; next is interventricular septal defect, followed by persistent ductus arteriosus. Interventricular and interatrial septal defects are equally common among all the patients below 16 years of age, but interventricular septal defects are commonest among those below 11 years. Persistent truncus arteriosus is unusually common, while coarctation of the aorta and congenital aortic stenosis are rare, as compared with series reported from other places.

Adolescent↗
PubMed2002

Evidence-based prevention requires evidence-based performance. The case of screening for congenital heart disease in child health care.

STUDY OBJECTIVE: To illustrate to what extent the cost-effectiveness of an evidence-based prevention programme may depend on evidence-based performance, by the example of screening for congenital heart disease in Dutch child health care. METHODS: A patient follow-up study on 290 children with congenital heart disease, of which 83 with significant disorders, diagnosed over two years, and born in the south west of the Netherlands. RESULTS: Adequate screening for congenital heart disease at Dutch child health centres, compared to inadequate screening, proves to be effective (OR: 0.18; 95% CI: 0.04-0.87). However only 15% of all patients with significant disorders in this study was adequately screened. Total health care costs involved over two years amount to over $3 million. Of these costs, 13% are to be attributed to screening tests; 8% to referrals resulting from screening. The costs for screening and referrals, as they were actually performed, are estimated at about $72.000 per patient benefiting from it. Were all children to be screened adequately this sum would be reduced to about $15.000. CONCLUSION: Not only should prevention programmes be evidence-based, but also outcome and quality, monitored by periodically establishing whether they are optimally performed.

Child↗
PubMed1996

New developments in intracardiac and intravascular devices for congenital heart disease.

Techniques of transcatheter device placement for treatment of pediatric congenital heart disease have developed substantially since their introduction 20 years ago. Patent ductus arteriosus occlusion can be accomplished by umbrella deployment or coil placement. Intracardiac defects can be closed with umbrella or buttoned devices. Stenoses of vessels or conduits that are only temporarily relieved with balloon dilation can be effectively expanded with intravascular stents. Recent procedural modifications have been introduced in an attempt to minimize the size of the delivery sheath and reduce complications that can arise from device embolization. Transcatheter device placement can be an important adjunct to surgery for correction or palliation of congenital heart lesions.

Cardiac Catheterization↗
PubMed1995

Cost-effectiveness analysis of interventions for congenital heart disease.

Expansion of the options available for the treatment of congenital heart disease has been accompanied by an increasing realization of the limits of our available health care resources. Cost-effectiveness analysis is one of several analytic approaches that can improve decisions about the appropriate use of technology in interventional pediatric cardiology and other fields. In this article, cost-effectiveness analysis is distinguished from related approaches, such as cost-benefit analysis. Then, basic principles of cost-effectiveness analysis are described. Next, the application of these principles is illustrated, using a recently published comparison of transcatheter versus surgical closure of patent ductus arteriosus. Finally, potential research implications of the surprising findings of this study are discussed.

Cardiac Catheterization↗
PubMed1997

Comparison of left ventricular ejection fraction in congenital heart disease by visual versus algorithmic determination.

Two-dimensional (2D) echocardiographic visual estimation of left ventricular (LV) ejection fraction (EF) in patients with acquired heart disease yields results that are comparable to those obtained by recommended algorithms. However, there is no information concerning its accuracy in congenital heart disease. This study compares applicability, accuracy, and reproducibility of LVEF by visual estimation with that by currently used algorithms (cylinder hemiellipsoid, ellipsoid biplane, and biplane method of disks) in 92 consecutive patients with congenital heart disease but unrepaired complete atrioventricular septal defect, univentricular heart, and hypoplastic left ventricle, using 3D echocardiography as the reference method. Visual estimation of LVEF could be applied in all cases. Because of technically inadequate 2D echocardiographic images for volume measurement, analysis for comparison could be performed in 71 patients (77%), aged 1 day to 47 years. The correlation between 3D echocardiographic and visual estimation was 0.91 (SEE 3.3%), cylinder hemiellipsoid, 0.86 (SEE 3.9%), ellipsoid biplane 0.87 (SEE 3.9%), and biplane method of disks 0.93 (SEE 3.2%). Intraobserver variability was similar for all 2D echo methods. Interobserver variability was greater for visual estimation. In conclusion, visual estimation of LVEF is applicable to most patients with congenital heart disease, yielding results that are comparable to those obtained by currently used 2D echocardiographic algorithms.

Adolescent↗