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PubMedDate not supplied

A survey of congenital heart disease in patients with oral clefts.

The purpose of this study is to determine the prevalence of congenital heart disease in patients with cleft lip and/or palate. We undertook a retrospective study of 1148 cases, age < 15 years old, with cleft lip and/or palate from January 1991 to December 1998, of which congenital heart disease was associated in 62 patients. The overall prevalence of congenital heart disease in patients with clefts was 5.4%. Of the 62 patients, there were 38 boys and 24 girls. We classified clefts into one of three categories; group 1: cleft lip alone; group 2: cleft lip and palate; group 3: cleft palate alone. The cleft lip and palate (group 2) was present in the majority (27 of 62; 44%) of patients with congenital heart disease. Twenty-five patients (40%) had cleft palate, and ten patients (16%) had cleft lip. Isolated atrial septal defect and ventricular septal defect are the two common congenital defects, which presented 23% and 21% of patients, respectively. Apart from congenital heart disease and cleft lip or palate, 56% (35 of 62 patients) and additional abnormalities. Central nervous system and skeletal malformations were the most common associated abnormalities. In our study, congenital heart disease was more common in patients of group 2 and group 3 than of group 1. In addition, there was a significantly greater proportion of patients associated with other systemic anomalies in groups 2 and 3 than in group 1 (chi-square chi 2 = 7.535, p = 0.023), but no significant difference was noted between group 2 and group 3. We recommend that it would be appropriate for all cleft patients to receive a routine examination for associated anomalies by a pediatrician. With the widespread use of echocardiography and/or brain sonography, the early diagnosis and treatment of these anomalies are possible.

Adolescent↗
PubMedDate not supplied

[Congenital rubella syndrome and the occurrence of congenital heart disease].

Aiming to document the incidence and type of associated congenital heart disease, 20 children affected with the congenital rubella syndrome have been evaluated during a 5 year period, starting 1988. Congenital heart disease was detected in 45% of the cases. Persistent ductus arteriosus was the most frequent finding, followed by ventricular and atrial septal defects. Hearing disturbances occurred in 83% of the 12 cases studied. Ophthalmic lesions appeared in 69% of the 13 cases analysed, congenital cataracts being the most frequent one. An association of 66% was found between ophthalmic and heart lesions. Regarding the nutritional index, 70% of the cases were situated between the 25th and 50th percentile. The relevant incidence of congenital defects and the documentation of an important number of cases in a short period of time point out to a significant prevalence of the syndrome with imperative need improve the vaccination programs.

English Abstract↗
PubMed2006

[Fetal echocardiography in diagnosing congenital heart disease prenatally: a multicenter clinical study].

OBJECTIVE: To evaluate the detection and accuracy of fetal echocardiography for congenital heart defects among high-risk populations. METHODS: A prospective observational study of prenatal diagnosis of congenital heart disease was conducted in two tertiary obstetrics and gynecology hospitals between January 2003 and December 2004. Consecutive fetuses at risk of congenital heart disease underwent detailed fetal echocardiography during the study period. B-mode and colour/pulsed Doppler flow imaging were used in all cases. Follow-up was sought for all pregnancies. Indications for referral, maternal and gestational age at diagnosis, as well as prenatal and postnatal diagnosis were recorded prospectively. By comparing prenatal and postnatal diagnoses, sensitivity, specificity, and predictive values were estimated. RESULTS: A series of 2063 high-risk fetuses underwent detailed fetal echocardiography during the study period. The mean gestational age at examination was 26.5 weeks, ranging from 16 to 42 weeks. The most common indications for fetal echocardiography were advanced maternal age (31.7%), fetal arrhythmias (13.5%) and maternal infections (10.4%). Forty-three cases of fetal congenital heart disease were detected. The mean gestational age at prenatal diagnosis was 27.3 weeks ranging from 16 to 40 weeks. There were 3 false-negatives and 1 false-positive. The sensitivity, specificity, positive and negative predictive values were 92.1%, 99.9%, 97.2%, and 99.8%, respectively. Diagnostic accuracy was 86.1%. A cardiac defect suspected on routine prenatal sonography accounted for the highest proportion of abnormal cases (67.4%). As for pregnancy outcome, there were 24 (52.1%) terminations; 2.2% died in utero, 13% postnatally, and 28.3% survived. CONCLUSIONS: (1) Fetal congenital heart disease can be identified reliably by prenatal echocardiography. (2) Possible congenital heart disease or suspected heart defect noted on a screening obstetric sonogram is an important indication for fetal echocardiography. (3) A sequential segmental approach is critical for correct evaluation of the cardiac malformation. (4) The outcome of the patients with congenital heart disease is poor and a multidisciplinary approach is needed to the parental counseling and perinatal management planning.

China↗
PubMed1991

Retinopathy of prematurity in infants with cyanotic congenital heart disease.

We undertook a study of premature infants with cyanotic congenital heart disease to determine whether these infants develop retinopathy of prematurity despite a persistent hypoxemic state. Using the computerized registry of the neonatal intensive care unit of Vanderbilt University Medical Center, Nashville, Tenn, we identified six premature infants (less than 37 weeks' gestational age, with birth weights of 1100 to 2050 g) with cyanotic congenital heart disease who survived the neonatal period and underwent ophthalmologic evaluation. Review of their charts revealed that three of six infants developed retinopathy of prematurity (two had grade 1 and one had grade 3 disease), but none required treatment. Our data support the findings of other investigators that elevated arterial oxygen tension is not the sole factor leading to the development of retinopathy of prematurity. Premature infants with cyanotic congenital heart disease can develop retinopathy of prematurity despite persistent hypoxemia. Cyanotic premature infants should be screened for retinopathy of prematurity with the same thoroughness as other premature infants.

Blood Gas Analysis↗
PubMed2003

Care of children who have had surgery for congenital heart disease.

Children who have had surgical correction for congenital heart disease can present to the ED with an acute illness that could be associated with their cardiac lesion. There is no data available to summarize complications that could be associated with surgically corrected congenital heart disease. This work was undertaken to describe the common procedures used, list known complications of these procedures, and review general management principles in caring for the acutely ill child who has had heart surgery.

Child↗
PubMed1976

Absent mesosternum in congenital heart disease.

The association of developmental abnormalities of the sternum with congenital heart disease is well known. These abnormalities include hypoplasia, multiple centers, and premature fusion. The absence of the sternal bodies in patients with congenital heart disease unrelated to a specific syndrome complex has not been previously described. Possible association with congenital pulmonary vascular disease was noted.

Child↗
PubMed2006

[Plasma levels of adrenomedullin in children with congenital heart disease].

OBJECTIVE: This study was designed to investigate the pathophysiological role of adrenomedullin (ADM) in congenital heart disease. METHODS: Forty-eight children with congenital heart disease confirmed by cardiac echocardiography and catheterization were studied. The patients were divided into three groups on the basis of hemodynamic indices measured during cardiac catheterization: high pulmonary blood flow with (group 1) or without (group 2) pulmonary hypertension (mean pulmonary arterial pressure > 20 mmHg) and a cyanosis group (without high pulmonary blood flow) (group 3). Six children who recovered from Kawasaki disease were used as a Control group. Plasma ADM levels were measured by radioimmunoassay. RESULTS: The plasma ADM levels from the femoral vein were significantly higher than those from femoral artery in patients with congenital heart disease. The patients from group 1 and group 3 had higher plasma ADM levels (1.9 +/- 1.8 pmol/L and 2.4 +/- 1.3 pmol/L, respectively) than the controls (1.0 +/- 1.4 pmol/L; P < 0.01). Plasma ADM levels were significantly negatively correlated with mean systemic arterial pressure, oxygen saturation in mixed vein and oxygen saturation in systemic artery (r=-0.401, -0.562, -0.600, respectively; P < 0.01) but positively correlated with pulmonary vascular resistance (r=0.406; P < 0.01). CONCLUSIONS: Plasma ADM levels are increased in congenital heart disease with high pulmonary blood flow and hypertension or with cyanosis. Plasma ADM levels are related to pulmonary arterial resistance and hypoxemia. Increased ADM levels may play roles in reducing the pulmonary arterial resistance and alleviating hypoxemia in these patients.

Adolescent↗
PubMed2006

[Psychological symptoms and body image in patients after surgery of congenital heart disease].

In the last 20 years the survival rate of patients with congenital heart disease has increased considerably, thus psychological consequences of living with a heart defect have attracted considerable scientific attention. In our study psychological symptoms and the body image of patients with congenital heart disease were compared with the respective scores of general population samples (age: 14 - 45 years). Psychological symptoms were measured by means of the Brief Symptom Inventory (BSI; subscales: somatization, obsessive-compulsive thoughts, interpersonal sensitivity, depression, anxiety, hostility, phobic anxiety, paranoid ideation, psychoticism). Body image was assessed with the FKB-20 body image questionnaire (subscales: rejection of the body, vitality). The patient group consisted of 361 women and men with congenital heart disease. For comparisons with the BSI, a sample of 1165 subjects was available. Comparisons with the FKB-20 were performed with data from a separate survey (N = 1169). After stratification for age and gender, in females only a few differences were found for both instruments. In males higher scores were obtained for "rejection of the body", and lower ones for "vitality". This does not apply to the highest age group (36 - 45 years). For all BSI-subscales except "depression", "phobic anxiety", and "psychoticism" marked differences between patients and controls were found in males and over all age groups. In women group differences emerged on some subscales (obsessive-compulsive thoughts, interpersonal sensitivity, anxiety, hostility, and phobic anxiety), but this does not hold for all age groups. These results do not apply to patients with the severest impairments due to congenital heart disease, because their number in our study was too low.

Adaptation, Psychological↗
PubMed2003

Usefulness of three-dimensional electron beam computed tomography for evaluating tracheobronchial anomalies in children with congenital heart disease.

This study was undertaken to delineate tracheobronchial anomalies associated with congenital heart disease. From June 1995 to December 2000, 1,245 children with congenital heart disease underwent cardiac electron beam computed tomography with 3-dimensional reconstruction on an independent workstation. Tracheobronchial anomalies are strongly associated with congenital heart disease and accompanying tracheal stenosis is not uncommon. With 3-dimensional reconstruction, electron beam computed tomography provided excellent anatomic definition of the central tracheobronchial abnormalities.

Abnormalities, Multiple↗
PubMed1998

[Catheter intervention for adult congenital heart diseases].

The efficacy of catheter intervention for adult congenital heart diseases was evaluated in 27 patients aged 20 to 52 years (mean age at catheterization 25 +/- 7 years) from 1986 to 1996. Four patients had pulmonary valve stenosis, four had aortic valve stenosis, three had coarctation of the aorta, 10 had pulmonary artery stenosis, four had cyanotic heart diseases and aorto-pulmonary collateral arteries, one had patent ductus arteriosus, and one had cyanotic heart disease and stenotic Blalock-Taussig shunt. Balloon dilation was successful in all patients with pulmonary valve stenosis, and follow-up evaluation (1-8 years) showed no restenosis in any patients. Balloon dilation was successful in all patients with aortic valve stenosis, and follow-up evaluation (0.5-5 years) showed transvalvular pressure gradient < 50 mmHg. Stenosis was relieved successfully in all patients with coarctation of the aorta, and follow-up evaluation showed no restenosis. Balloon dilation was successful in eight of 13 locations (62%) in patients with pulmonary artery stenosis. Coil embolization was successful in all patients with cyanotic heart diseases and aortopulmonary collateral arteries. In a patient with patent ductus arteriosus, two coils were placed in the ductus arteriosus but were retrieved because hemolysis was observed after the embolization. These data indicate that catheter intervention in young adults with congenital heart diseases is as effective as in children.

Adult↗
PubMed1980

Collateral vessels between the coronary and bronchial arteries in patients with cyanotic congenital heart disease.

Selective coronary arteriography (using the Sones technique) was performed in 67 patients with congenital heart disease aged 1 to 33 years. Five of the 23 patients with cyanotic congenital heart disease had collateral vessels between the coronary and bronchial arteries; none of the 44 patients with noncyanotic congenital heart disease had such vessels. Each of the five patients with collateral vessels had severe obstruction of the right ventricular outflow tract or the pulmonary valve plus a ventricular septal defect and a right to left shunt. It appears that such collateral vessels from the coronary arteries provide an increment to pulmonary blood flow in patients with cyanotic congenital heart disease and diminished pulmonary flow.

Adolescent↗
PubMed1978

Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease.

Fifty patients with congenital heart disease, ages 2 days-30 years (median 12 months) at cardiac surgery, underwent lung biopsy to assess pulmonary vascular disease (PVD). Twenty-six had ventricular septal defects (VSD), 17 d-transposition of the great arteries (D-TGA), and seven, defects of the atrioventricular canal (AVC). Quantitative morphologic data was correlated with hemodynamic data. Three new grades of PVD were observed. Abnormal extension of muscle into peripheral arteries (grade A) was found in all patients; all had increased pulmonary blood flow. In addition, 38 of 50 patients had an increase in percentage arterial wall thickness (grade B); this correlated with elevation in pulmonary artery (PA) pressure (r = 0.59). Another 10 of 50 patients had, in addition to A and B, a reduction in the number of small arteries (grade C); nine of 10 were patients with elevated PA resistance greater than 3.5 mu/m2 (P less than 0.005). All three patients with Heath-Edwards changes of grade III or worse also had grade C. Reduction in peripheral arterial number probably precedes obliterative PVD and may identify those patients in whom, despite corrective surgery, PVD will progress.

Adult↗
PubMed2002

Wide complex tachycardia and congenital heart disease.

We present a patient with congenital heart disease and haemodynamically poorly tolerated wide QRS tachycardia. Differential diagnosis and therapy are discussed. After the patient underwent heart transplantation, and the substrates for ECG abnormalities and arrhythmias were demonstrated in the explanted heart.

Adult↗
PubMed1996

[The effect of adaptive behavior training on rehabilitation of children with congenital heart disease].

The adaptation behaviors of 102 children with congenital heart disease were assessed by Children's Adaptation Behaviors Scale. The result indicated that behavior training should be given according to their adaptive behaviors before operation. 106 children with congenital heart disease were divided into two groups. 67 were in behavior training group and 39 were in control group. The result showed that self-care abilities and compliance of therapeutic regimen of the training group were much better than that of the control group. The length of stay in the hospital of the training group was 3.01 days shorter than that of the control group.

Adaptation, Psychological↗
PubMed2001

Comparative analysis of pattern, management and outcome of pre- versus postnatally diagnosed major congenital heart disease: a population-based study.

OBJECTIVES: Most pregnant women in New South Wales undergo obstetric ultrasound examination, including some assessment of fetal cardiac anatomy. We aimed to review the spectrum of cardiac defects, management and outcome data of all fetuses with diagnosis of major congenital heart disease between 1994 and 1996 and compare them to major congenital heart disease in infants born during the same 3-year study period. METHODS: Descriptive comprehensive study of the New South Wales population. Study centers included the single fetal echocardiographic referral service and the two pediatric cardiac centers of New South Wales. RESULTS: Ninety-seven fetuses and 562 infants with major congenital heart disease were identified (240,000 livebirths), resulting in a prenatal detection rate of 15%. Anomalies detectable by cardiac four-chamber views were diagnosed at an average rate of 30% (68/229) in utero. By contrast, lesions associated with abnormal ventricular outflow and great artery views were detected in only 6.7% (29/430; P < 0.0001) of cases prior to birth. Of the 97 fetuses, 29 were aborted, 16 died in utero, and 9 died early postnatally without treatment. Within 2 weeks of age, 23% with fetal and 40% (P < 0.05) with infant major congenital heart disease diagnosis required an intervention, mainly for patent ductus arteriosus dependent lesions. Postnatal survival was similar for the fetal and infant series up to 2 years of age: 77% (95% confidence interval 64-90%) vs. 85% (95% confidence interval 82-88%). CONCLUSIONS: Prenatal diagnosis has important implications for pregnancy outcome, in particular for univentricular lesions. However, the present mode of obstetric routine ultrasound scanning fails to identify most ductus arteriosus dependent cardiac lesions with a predictable need for early postnatal intervention.

Female↗
PubMed2005

[Catheter ablation for supraventricular tachycardia in children and congenital heart diseases].

INTRODUCTION: Recently catheter ablation has been accepted as standard therapy for symptomatic supraventricular tachycardia in children. Nature of childhood and the variability of congenital heart diseases and congenital heart surgery distinguishes pediatric catheter ablation from the adult practice. OBJECTIVES: The aim of the present study was to summarize a single-center experience of the first 30 consecutive patients regarding the electrophysiological studies and catheter ablations, moreover to report on the national adoption of these interventions for pediatric patients in Hungary. METHODS: Between April 1996 and September 2004 catheter ablation was offered for 30 children as treatment of their supraventricular tachycardia because of failure of pharmacological therapy or parents preference. RESULTS: The mean age of the patients was 13.7 years (2.3-18.0 years) and the mean weight was 52.0 kg (12.0-81 kg). Electrophysiology study revealed 33 arrhythmogenic substrates in 30 patients, 30 of those 33 were congenital while 3 were acquired. Catheter ablation was attempted in 27 patients with acute success in 24 cases (89%). Recurrence was observed in 2 patients and the redo ablation was effective in both, although a second recurrence occurred later in one of them. There were no major complications, but two minor ones (pseudoaneurysm of arteria femoralis, transient ventricular ectopy) occurred. CONCLUSIONS: Catheter ablation is safe and effective in children with congenital heart disease. Our results are comparable with the international data.

Adolescent↗
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↗