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PubMed1988

The frequency of aneuploidy in prenatally diagnosed congenital heart disease: an indication for fetal karyotyping.

The frequency of chromosomal abnormalities in infants with congenital heart disease has been estimated from postnatal clinic data to be 5% to 10%. Over an 18-month period we performed fetal echocardiograms on 502 fetuses and found cardiac anomalies in 34. Eleven of these fetuses (32%) had abnormal chromosomes. This marked discrepancy from the data found in the pediatric literature may relate to nonviable fetuses identified in utero, who escape pediatric case identification. We conclude that in cases of fetal heart disease the workup should include either amniocentesis or fetal blood sampling for chromosome analysis. The information can be used for complete parental counseling and for managing delivery and neonatal care.

Aneuploidy↗
PubMed1976

Recurrence risks in children having one parent with a congenital heart disease.

The risk of recurrence of a congenital cardiovascular malformation in a child having one parent with congenital heart disease has been determined for each of the seven most common anomalies presently compatible with survival to reproductive age. The range of risk is 2.5% to 4.3% depending on the lesion. This is within the range of expectation for the model of multifactorial inheritance previously used to predict recurrence in other first-degree relatives of probands (siblings and parents) with congenital heart disease. The cardiovascular abnormality occurring in the child was most often the same as in the parent or was a closely related variant of it.

Adult↗
PubMed2001

[Influential factors in mortality rate from congenital heart disease. Study of 1,216 children in the Autonomous Community of Murcia (1978-1990)].

INTRODUCTION: In the last few years, important progress has taken place in management of congenital heart disease. These changes have had an influence on diagnosis, preoperative management, surgery treatment and postoperative care, giving rise to better results in the treatment of children suffering from congenital heart disease. AIM: To assess the results of congenital heart diseases in a reference hospital by comparing two periods with reference to both diagnosis and therapeutical management. We also intend to investigate the influence that factors such as the existence of extracardiac congenital malformations and heart surgery have on mortality. PATIENTS AND METHODS: Our sample group was made up of 1,216 children suffering from congenital heart disease. Their ages ranged from 1 day to 7 years old. These children were born over a period of thirteen years and studied at the paediatric cardiology unit in a reference hospital in the Autonomous Community of Murcia, a region of Spain. We retrospectively analysed their development by individual heart diseases (and their associated factors), and the global results. Our research was divided into two periods: between 1978 and 1983, and between 1984 and 1990. Differences were found regarding diagnosis and treatment. RESULTS: a) Mortality rate from congenital heart disease decreased in the period between 1984 and 1990 in comparison to the period between 1978 and 1983, from 28 to 21,7% (p < 0.05); b) individually, the mortality rate decreased with statistical significance in two diseases: interventricular communication and patent ductus arteriosus, and c) there is a higher mortality rate of patients with no surgery treatment and/or extracardiac malformations. CONCLUSION: Progress in the management of congenital heart disease has led to a more favourable outcome in the last years.

Child↗
PubMed1994

Exercise Doppler echocardiography identifies abnormal hemodynamics in adults with congenital heart disease.

The hemodynamic factors contributing to reduced exercise capacity in adults with congenital heart disease are not fully defined. We hypothesized that they would demonstrate reduced exercise capacity compared with age-matched control subjects because of abnormal exercise hemodynamics and ventricular performance demonstrable by echocardiography. We retrospectively analyzed the results of all studies in all patients with congenital heart disease (from 1988 to 1993) and found that the peak work load and double product achieved were decreased in comparison with normal values (79 +/- 47 W vs 149 +/- 52 W [p < 0.0001] and 19,210 +/- 6805 vs 25,701 +/- 6440 [p = 0.005], respectively). Seven patients achieved work loads < 40 W associated with an exaggerated exercise pulmonary artery pressure response, decreases in right and/or left ventricular function with exercise, or both. Normal or near-normal exercise tolerance was observed in the following patient groups: (1) repaired tetralogy of Fallot, (2) uncomplicated I-transposition of the great arteries, and (3) Ebstein's anomaly with atrial septal defect. We conclude that exercise echocardiography provides new hemodynamic information for evaluating functional limitations in congenital heart disease.

Adolescent↗
PubMed2002

Hospital experiences of young adults with congenital heart disease: divergence in expectations and dissonance in care.

BACKGROUND: Despite increasing survival for adults with congenital heart disease, little is known about hospitalization for young adult patients with this disease and for their families. Because of the complexity of the disease and its management during the life span, young adults are often hospitalized on both pediatric and adult units during a stay in the hospital. OBJECTIVES: To explore the experience of hospitalization of young adults with congenital heart disease, the experience of their families, and the views of the nurses who cared for these patients and to generate substantive theory on interactions between patients, patients' families, and nurses. METHODS: Semistructured interviews and naturalistic observations were conducted with young adults with congenital heart disease (mean age, 28.6 years), their family members, and nurses who cared for the patients during hospitalization (N=34). Dimensional analysis was used to analyze interviews and field notes from observations. RESULTS: A grounded theory was derived, explaining how the hospital context and relationships between patients, patients' families, and nurses affect patients' hospital experiences. Expectations differed among the groups, leading to dissonance in care, as exemplified by role confusion and power struggles over control of care. This dissonance resulted in interpersonal conflict, distrust, anxiety, and dissatisfaction with the care and caring experiences. CONCLUSIONS: Changes in hospital units, a better understanding of the healthcare needs of young adults with congenital heart disease, and acknowledgment of the expertise of patients and patients' families are needed to improve nursing care for these patients and their families.

Adult↗
PubMed2005

[Potential of endovascular surgery in children with congenital heart diseases].

Bakulev Scientific Center for Cardiovascular Surgery has very broad experience (3,774 cases) in endovascular operations on patients with different congenital heart diseases. 648 patients have undergone atrioseptostomy, 1,500 patients--balloon valvuloplastic, 546 patients--balloon angioplasty, 565 patients--coil embolization; stents have been used in 86 patients; in 114 cases the surgeons of the Center have used different occluders to close pathological communications, in 315 patients others types of treatment have been applied. In many cases of congenital heart diseases (valvular stenosis of pulmonary artery and aorta, patent ductus arteriosus, atrium and ventricular septal defect, coronary-cardial fistula, coarctation and recoarctation of aorta) endovascular techniques present a good alternative to open surgical procedures. In some cases endovascular techniques are palliative (Rashcind's and Park's procedure, balloon valvuloplasty in stenosis of pulmonary artery and angioplasty in stenosis of peripheral pulmonary artery and aorto-pulmonary anastomosis in patient with cyanotic congenital heart disease, stenting of PDA, coil embolization of collateral artery from aorta to pulmonary artery etc.) Multi-stage endovascular and surgical treatment allows radical or hemodynamic correction in patient with complex congenital heart disease. Nowadays the spectrum of endovascular treatment is wide; it is going to develop in the future, which will allow endovascular techniques to be of wide use in treatment of patient with complex heart diseases.

Angioscopy↗
PubMed1989

Growth and nutritional intake of infants with congenital heart disease.

Poor weight and length gain of infants with congenital heart disease is generally considered to be related to inadequate nutritional intake, but no longitudinal data on growth and nutritional intake of such infants are available. We compared weight, length, subscapular and triceps skinfold thickness, energy and protein intake (24-h dietary intake records) as well as serum prealbumin and albumin of infants with cyanotic heart disease (n = 8) or isolated left-to-right shunt (n = 8) with those of healthy infants aged (n = 8) 45-365 days. Weight, length, and combined (subscapular and triceps) skinfold thickness of the two groups with congenital heart disease (CHD) were significantly less from 183 through 365 days of age. However, energy and protein intake was similar to that of the control group from 45 through 365 days of age. Normal serum prealbumin and albumin in the infants with CHD ruled out protein-calorie malnutrition. It is concluded that a low level of food intake was not the main cause of inadequate growth and of small subcutaneous fat stores in these two small, but homogeneous, groups of infants with CHD.

Body Height↗
PubMedDate not supplied

Clinical features and outcome of cerebral abscess in congenital heart disease.

BACKGROUND: Cerebral abscess is a serious life threatening complication of several diseases. The objective of this study was to look at the clinical profile, microbiology and outcome of children with cerebral abscess having an underlying congenital heart disease as compared to other predisposing conditions. METHODS: Thirty children aged less than 15 years were reviewed. There were 15 males and 15 females. The mean age of presentation was 5.6 +/- 4.4 years. RESULTS: The duration of illness at the time of admission was 17.6 +/- 24.6 days. Typically patients presented with fever, vomiting, headache and seizures. The predisposing conditions found were cyanotic congenital heart disease in 11 (37%) of children, meningitis in 6 (20%), septicemia in 7 (23%) and no underlying cause was found in 5 (17%) children. The most common microbe in children with cyanotic congenital heart disease was of the Streptococcus milleri group (52%). Computerized tomography confirmed the diagnosis and the most common location of the abscess was the parietal lobe of the cerebral hemisphere. All abscesses were large, more than 2 cm in diameter and were aspirated surgically. Excision was performed in 6 children. Five children expired, one due to a intracranial bleeding and the others due to severe cerebral edema and tentorial herniation. Complications were seen in 20 children and 16 had sequelae, hemiparesis in 11 and seizure disorder in 5. CONCLUSION: Brain abscess is a serious infection with poor outcome if diagnosed late. Delayed surgical drainage has high morbidity and mortality. The threshold for diagnosis should be low particularly in children with a predisposing condition like cyanotic congenital heart disease.

Adolescent↗
PubMed2006

Prevalence of cardiovascular risk factors in adults with congenital heart disease.

BACKGROUND: Empirical evidence indicates that patients with congenital cardiac anomalies may be prone to developing coronary heart disease. Although primary prevention of ischaemic heart disease in patients with congenital heart defects is important, data on the prevalence of cardiovascular risk factors in these patients are not available. The aims of this study are therefore to describe the prevalence of risk factors for coronary heart disease in a large sample of adults with congenital cardiac anomalies, and compare this with the prevalence in the general population. DESIGN: A retrospective analysis of computerized patient records. METHODS: At our outpatient clinic, all patients are examined by an advanced practice nurse and a congenital heart disease cardiologist. Data on smoking behaviour, sports participation, blood pressure, body mass index, and the diagnosis of diabetes are recorded systematically. Data on the general population were derived from national health surveys. RESULTS: In a 4-year period, we collected data on 1976 individual patients. Male patients had a significantly higher prevalence of smoking and elevated blood pressure, whereas women were less engaged in sports activities and were more often obese. In comparison with the general population, our patients reported less smoking and more participation in sports, but presented more often with hypertension or diabetes. Only 20.4% of men and 21.0% of women have a fully heart-healthy lifestyle, as they presented without any risk factor. CONCLUSION: A substantial number of patients had one or more cardiovascular risk factors. Therefore, primary prevention by strengthening educational efforts becomes critically relevant in patients with congenital heart disease, to avoid the additional burden of coronary events in this growing population of patients.

Adult↗
PubMed2001

Exercise and sports participation after surgery for congenital heart disease: the European perspective.

The success of surgery for congenital heart disease which has been performed since many years has created a population of patients who require careful follow-up in order to determine their clinical progress and to establish the type and intensity of physical activity which they can safely perform. The authors illustrate the opinion of the European Community regarding children, sport and organizational aspects and also problems concerning the management of pediatric cardiac patients in Europe and Italy. Cardiological and surgical aspects are considered together with the practice of physical activity, with emphasis upon the differences between the various countries. Particular attention is paid to the Italian legislation regarding the certification to participate in competitive or non-competitive sport in such a population of patients. Great importance is given to pediatric cardiac rehabilitation programs which aim at improving the aerobic fitness of patients operated upon for complex congenital heart disease and at illustrating their own cardiovascular limitations so that they can perform physical exercise with the utmost safety.

Cardiac Surgical Procedures↗
PubMed2000

Usefulness of the myocardial performance index for assessing right ventricular function in congenital heart disease.

Quantitative assessment of ventricular function in patients with congenital heart disease is often challenging due to distorted ventricular geometry. A myocardial performance index (MPI) has been reported in adults and children that is a Doppler-derived nongeometric measure of ventricular function. The MPI measures the ratio of isovolumic time intervals (isovolumic contraction time and isovolumic relaxation time) to ventricular ejection time. The effects of altered ventricular preload or afterload on the MPI have yet to be determined. This study assesses the impact of altered preload or afterload on right ventricular (RV) function and the RV MPI in the clinical setting of congenital heart disease. Patient groups were compared with normal pediatric and adult populations before and after repair of their congenital heart lesion. Patients with large atrial septal defects (ASDs) represented the clinical setting of increased ventricular preload, whereas patients with isolated pulmonary valve stenosis represented increased RV afterload. Patients with congenitally corrected transposition of the great arteries (CC-TGA) with severe left atrioventricular valve regurgitation represented a combined increase in RV preload and afterload. The RV MPI in 152 normal children (ages 3 to 18 years) and 37 adults (ages 18 to 51 years) was 0.32 +/- 0.03 and 0.28 +/- 0.04, respectively. In pediatric patients (n = 45) and adult patients (n = 40) with ASD, the RV MPI was 0.35 +/- 0.09 (p = NS) and 0.38 +/- 0.04 (p < 0.01 compared with normal adults), respectively. Patients with pulmonary stenosis (n = 21, ages 1 day to 19 years) had a RV MPI of 0.32 +/- 0.06 (p = NS). CC-TGA patients had a RV MPI of 0.72 +/- 0.17 (p < 0.001). No significant change in the RV MPI was seen in any postoperative patient group despite relief of RV volume or pressure overload. Thus, the MPI is a quantitative measure of RV performance that is appears to be relatively independent of changes in preload or afterload in the clinical setting.

Adolescent↗
PubMed1984

[Reconstructive valve surgery in congenital heart disease].

As part of various defects in congenital heart disease, valvular lesions are frequently encountered and remain as one of the difficult subjects. Recent advances in the management of valvular lesions and late results of repair and replacement were reported. In congenital aortic stenosis, most of patients have obtained satisfactory results with valvotomy, but some required reoperation with valve replacement even during childhood. In supracristed VSD associated with aortic regurgitation, late recurrence of regurgitation was found in some, but 70% of the patients showed free of diastolic murmur after 10 years. In tetralogy of Fallot, definitive repair of pulmonary valve was performed in last 5 years with composite patch with patient's own pericardium to reduce postoperative pulmonary regurgitation. Recently, pericardium is treated with glutaraldehyde on the operating table to prevent late shrinkage. As external conduit, homograft aorta, which had been utilized previously, showed good late results comparable to xenograft in terms of free from reoperation. Xenograft has been frequently utilized without significant late obstruction so far. In atrioventricular canal, endocardial cushion prosthesis technique has been utilized satisfactory with recent modification of the wings made by glutaraldehyde treated patient's own pericardium as well as in tetralogy.

Follow-Up Studies↗
PubMed1994

Effect of inhaled nitric oxide on raised pulmonary vascular resistance in children with congenital heart disease.

OBJECTIVE: To study the short-term effects of inhaled nitric oxide in infants and young children with congenital heart disease. SETTING: A supraregional referral centre for children with congenital heart disease. PATIENTS AND METHODS: 22 infants and children aged 3-32 months (median age 5 months) with congenital heart disease undergoing preoperative cardiac catheterisation. All but one infant had intracardiac shunt lesions and 13 had increased pulmonary vascular resistance. During catheterisation the patients inhaled nitric oxide in a concentration of 40 parts per million in room air. Pulmonary and systemic haemodynamic variables were evaluated by means of measured oxygen consumption and the Fick principle before and after 10 minutes' exposure to nitric oxide. RESULTS: Inhaled nitric oxide did not affect the systemic circulation. There was a significant reduction in the pulmonary vascular resistance, but only in the 13 infants with pulmonary hypertension, in whom pulmonary vascular resistance was reduced by 34% from 8.6 (4.6) mm Hg.min.m2.l-1 (mean (SD)) to 5.7 (3.5) mm Hg.min.m2.l-1. The pulmonary circulation in infants with normal pulmonary vascular resistance was not affected. No statistically significant increase in methaemoglobin was seen, though there were large individual differences. No other side effects were seen. CONCLUSION: The present study shows that in infants with congenital heart disease inhaled nitric oxide reduced pathologically increased pulmonary vascular resistance without affecting systemic circulation and without important side effects with brief exposure.

Administration, Inhalation↗
PubMed2003

[Studies on the mutation and expression of TBX5 gene in human simple congenital heart disease].

This work is to investigate the mutation and expression of TBX5 gene in human simple congenital heart disease. The mutations of eight exons of TBX5 gene in 61 CHD family members (a total of 216 individuals including 65 patients and 151 normal relatives) were examined by PCR-DGGE. Using beta-actin as internal control, the differential expression between 34 myocardium samples from simple congenital heart disease patients and three normal controls was conducted by RT-PCR. There is no mutation detected in all samples; The mRNA expression levels of TBX5 gene show descent tendency in samples of simple congenital heart disease compared with normal controls. The mutations in coding region of TBX5 gene do not cause human simple congenital heart disease, but the abnormality in transcription level of TBX5 gene maybe a kind of mechanism causing human simple congenital heart disease.

English Abstract↗
PubMed2002

The risk of postthrombotic syndrome in children with congenital heart disease.

The lower extremities of 28 unselected children with congenital heart disease were investigated and classified according to the criteria for postthrombotic syndrome five to ten years after their first cardiac catheterization. For the clinical criteria, all patients completed a questionnaire and underwent a standardized physical examination of both legs. For the pathophysiologic criteria, the presence of venous outflow obstruction and reflux was evaluated by color duplex sonography in 24 of the 28 patients. Mild postthrombotic syndrome was present in half the patients. Partial or complete occlusion of the investigated vein was found in four patients (17%). In all patients studied, the venous valves of the deep system were competent. Postthrombotic syndrome frequently occurs in children with congenital heart disease. Prospective studies seem to be justified to investigate the precise incidence and potential risk factors.

Anticoagulants↗
PubMed1999

Value of programmed ventricular stimulation in patients with congenital heart disease.

INTRODUCTION: The role of programmed ventricular stimulation (VSTIM) for risk stratification in congenital heart disease is unclear. We analyzed the results of VSTIM in selected congenital heart disease survivors at a single center to determine whether it improved the ability to predict a serious outcome. METHODS AND RESULTS: Between July 1985 and September 1996, 140 primary VSTIM studies were performed on 130 patients (median age 18.1 years, range 0 to 51). Tetralogy of Fallot (33 %), d-transposition of the great arteries (25 %), and left ventricular outflow tract obstruction (12%) accounted for the majority of patients. Indications included spontaneous ventricular tachycardia (VT) of > or = 3 beats (72%) and/or symptoms (68%). Sustained VT was induced in 25% of the studies, and nonsustained VT in 12%. Atrial flutter or other supraventricular tachycardia was documented in 32% and bradyarrhythmias in 26%. By univariate analysis, mortality was increased in patients with positive VSTIM versus negative VSTIM (18% vs 7%, P = 0.04). Using multivariate analysis, positive VSTIM was associated with a sixfold increased risk of decreased survival and a threefold increased risk of serious arrhythmic events, allowing up to 87% sensitivity in predicting mortality. However, 7 (33%) of 21 patients with documented clinical VT had false-negative studies. CONCLUSION: VSTIM in a large, selected group of congenital heart disease patients identified a subgroup with significantly increased mortality and sudden arrhythmic events. Failure to induce VT was a favorable prognostic sign, but the frequency of false-negative studies was high. Frequent supraventricular tachycardia further complicated risk stratification. Although VSTIM appears to be a reasonable tool for evaluation of this population, a larger, multicenter trial is recommended to clarify its utility.

Adolescent↗