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PubMed1985

The thymus in congenital heart disease.

Some cases of thymus developmental defects (DiGeorge's syndrome) are associated with the development of defects of the heart and great vessels. To see if anomalies of the heart are also associated with anomalies of the thymus, a material of thymus tissue removed during operation for congenital heart disease was compared with thymus tissue from a forensic autopsy material representing the same age group, but without cardiac lesions. Among 27 cases of congenital heart disease, occurrence in the thymus of solid epithelial cords were seen in 6 and cyst- or duct-like structures were found in 7. Among 47 forensic cases, 1 showed cords and another showed duct-like structures. Of these, one had congenital anomalies in the form of hepatic hamartomas and nesidioblastosis, whereas the other had died from morbilli, raising the possibility of congenital immune deficiency. Anyhow, a striking occurrence of minor morphological deviations in the structure of the thymus was found in children with congenital heart disease. Such material therefore may be less suitable for studying the morphology and biology of the normal thymus gland.

Abnormalities, Multiple↗
PubMed1983

Familial congenital heart disease: how are the various types related?

The distribution of congenital heart lesions was studied in 238 families with at least 2 affected members. A statistical analysis was performed. Concordant lesions were found in 48% of the affected first degree relatives and in 28% of the affected second and third degree relatives. The concordance rate is highly significant for all lesions studied in the first degree relatives, with the exception of ventricular septal defect (VSD). Among the discordant pairs of lesions, some occur significantly more often than expected (tetralogy of Fallot associated with VSD, pulmonary stenosis, and transposition of the great arteries); others, such as the association between VSD and pulmonary stenosis, are significantly less common than would be expected on a random hypothesis. An explanation is proposed suggesting that malformations anatomically dissimilar but resulting from the same heart segment disorder may have some common genes, and that interaction between genes may be responsible for "antagonism" between 2 defects. The embryologic segmental approach to congenital heart disease is reinforced by this genetic study.

Female↗
PubMed1999

Trends in presentation of congenital heart disease in a population-based study in Malta.

Differing pathological haemodynamics in cardiac malformations lead to varying modes and timings of presentation. This study identifies historical trends in presentation of congenital heart disease in a population-based study. All patients diagnosed as having congenital heart disease in Malta between 1960-1994 were included (n = 868). Analysis was carried out on trends in referral sources, modes of presentation and birth prevalence. The number of patients diagnosed with congenital heart disease increased over the period under study. For both patients not requiring intervention (n = 283) and those requiring intervention (n = 585), the proportion diagnosed prior to hospital discharge increased (p < or = 0.005). There was a decreasing trend for general practitioners to refer cases (p < 0.0001), and an increasing trend for paediatricians to refer such patients (p < or = 0.0003). The commonest presentation to the general practitioner was an incidental finding (92%), while paediatricians referred more patients for cyanosis or heart failure (p < or =, 0.005). For lesions not requiring intervention, the commonest lesion referred was ventricular septal defect from all sources. For lesions requiring intervention, the commonest lesion detected prior to hospital discharge was tetralogy of Fallot. Atrial septal defects were the commonest lesions detected after discharge by both paediatricians and general practitioners. An increase in the proportion of hospital diagnoses is attributed to increasing rate of hospital delivery, and greater training and experience in doctors performing neonatal examinations prior to discharge. Patients diagnosed after discharge are increasingly diagnosed by paediatricians due to an increasing pool of paediatricians and better parent awareness and education.

Family Practice↗
PubMed1978

Studies of the HLA complex in families of children with congenital heart disease.

In order to test the hypothesis that HLA-linked developmental determinants are involved in the pathogenesis of congenital heart disease, we performed tissue typing on 10 families in which two or more siblings had cardiac malformations. No association between the occurrence of congenital heart disease and the HLA complex was found.

Epitopes↗
PubMed1978

Causes of death, right ventricular hypertrophy, and congenital heart disease in scoliosis.

Among the causes of death of 43 scoliotics were 5 directly due to complications of congenital heart disease. Over half (57.9%) of the remaining 38 died of cardiac or respiratory causes. The paralytic scolitoics tended to die of pneumonia or respiratory failure, while the nonparalytic scoliotics died of cardiac failure. Right ventricular hypertrophy was present in 65% of the 17 subjects examined postmortem. Electrocardiographic evidence of right ventricular hypertrophy correlated well with the postmortem findings. The vital capacity was less than 1.75 liters in 84% of the dead subjects. The case records of a further 719 living scoliotics were examined for evidence of congenital heart disease. This was found in: 34 (4.5%) of the whole group of 762, 6.9% of the congenital ; 3.4% of the idiopathic scoliotics; 22.7% of those with Marfan's syndrome.

Adolescent↗
PubMed2002

Neurohormonal activation and the chronic heart failure syndrome in adults with congenital heart disease.

BACKGROUND: Neurohormonal activation characterizes chronic heart failure, relates to outcome, and is a therapeutic target. It is not known whether a similar pattern of neurohormonal activation exists in adults with congenital heart disease and, if so, whether it relates to common measures of disease severity or whether cardiac anatomy is a better discriminant. METHODS AND RESULTS: Concentrations of atrial natriuretic peptide (ANP), brain natriuretic peptide (BNP), endothelin-1 (ET-1), renin, aldosterone, norepinephrine, and epinephrine were determined in 53 adults with congenital heart disease, comprising 4 distinct anatomic subgroups (29 female; 33.5+/-1.5 years of age; New York Heart Association class 2.0+/-0.1, mean+/-SEM) and 15 healthy control subjects (8 female; 32.3+/-1.3 years of age). Systemic ventricular function was graded by a blinded echocardiographer as normal or mildly, moderately, or severely impaired. Adults with congenital heart disease had elevated levels of ANP (56.6 versus 3.1 pmol/L), BNP (35.8 versus 5.7 pmol/L), ET-1 (2.5 versus 0.7 pmol/L, all P<0.0001), renin (147 versus 16.3 pmol/L), norepinephrine (2.2 versus 1.6 pmol/L, both P<0.01) and aldosterone (546 versus 337 pmol/L, P<0.05). There was a highly significant stepwise increase in ANP, BNP, ET-1, and norepinephrine according to New York Heart Association class and systemic ventricular function, with even asymptomatic patients having evidence of significant neurohormonal activation. In contrast, there was no direct relationship between the 4 anatomic subgroups and any of the neurohormones studied. CONCLUSIONS: Neurohormonal activation in adult congenital heart disease bears the hallmarks of chronic heart failure, relating to symptom severity and ventricular dysfunction and not necessarily to anatomic substrate. Neurohormonal antagonism across this large and anatomically diverse population should be considered.

Adult↗
PubMed2003

Cardiac catheterisation guided by MRI in children and adults with congenital heart disease.

BACKGROUND: Fluoroscopically guided cardiac catheterisation is an essential tool for diagnosis and treatment of congenital heart disease. Drawbacks include poor soft tissue visualisation and exposure to radiation. We describe the first 16 cases of a novel method of cardiac catheterisation guided by MRI with radiographic support. METHODS: In our cardiac catheterisation laboratory, we combine magnetic resonance and radiographic imaging facilities. We used MRI to measure flow and morphology, and real-time MRI sequences to visualise balloon angiographic catheters. 12 patients underwent diagnostic cardiac catheterisation, two had interventional cardiac catheterisations, and for two patients, MRI was used to plan radiofrequency ablation for treatment of tachyarrhythmias. FINDINGS: In 14 patients, some or all of the cardiac catheterisation was guided by MRI. In two patients undergoing radiofrequency ablation, catheters were manipulated with use of fluoroscopic guidance and outcome was assessed with MRI. All patients received lower amounts of radiation than controls. There was some discrepancy between pulmonary vascular resistance calculated by flow derived from MRI and the traditional Fick method. We were able to superimpose fluoroscopic images of electro physiology electrode catheters on the three dimensional MRI of the cardiac anatomy. INTERPRETATION: We have shown that cardiac catheterisation guided by MRI is safe and practical in a clinical setting, allows better soft tissue visualisation, provides more pertinent physiological information, and results in lower radiation exposure than do fluoroscopically guided procedures. MRI guidance could become the method of choice for diagnostic cardiac catheterisation in patients with congenital heart disease, and an important tool in interventional cardiac catheterisation and radiofrequency ablation.

Adult↗
PubMedDate not supplied

Respiratory syncytial virus infection in children with congenital heart disease: a review.

This paper reviews recent changes in morbidity and mortality of respiratory syncytial virus (RSV) infection in infants with congenital heart disease. Mortality since the late 1970s has declined substantially, from approximately 37% to 3%. Although the frequency of admission to intensive care units has declined from approximately 60% to 30%, the frequency for mechanical ventilatory support has not changed significantly. Because mortality dropped prior to the widespread use of ribavirin, it is difficult to ascribe the improvement to this therapy. In infants with congenital heart disease (CHD), nosocomial infection remains a significant problem, accounting for approximately 33% of the RSV cases. Some authors report significant reductions in hospital-acquired RSV by use of gloves and gowns for contacts with infectious cases. Efforts at primary prevention have encountered problems with development of an RSV vaccine. Preliminary data from studies of passive immunization using immune globulins with high RSV antibody titers suggest that this therapy may reduce the severity of RSV infection in infants with serious heart disease.

Antiviral Agents↗
PubMed2006

Delivery of care for adult patients with congenital heart disease in Europe: results from the Euro Heart Survey.

AIMS: The increasing number of adults with congenital heart disease (CHD) has prompted the development of recommendations for the management of these patients and for the organization of their healthcare. The aim of this report is to describe the delivery of care in Europe for adults with congenital cardiac anomalies. METHODS AND RESULTS: As part of the Euro Heart Survey on Adult Congenital Heart Disease, we obtained data from 71 voluntarily participating centres that detailed their care practices for these patients. Forty-eight of these centres were specialist centres and 23 were non-specialist centres. We found that only 19% of the specialist centres complied with defined standards for optimal care structure. The criteria that appeared to be most difficult for all centres to achieve were performing 50 congenital heart operations or more per year and involving nurse specialists in the care of these patients. CONCLUSION: This survey indicated that the provision of care in Europe for adults with congenital heart defects is suboptimal. To fully realize the benefits of cardiac surgery performed in infants and children, continuous effort must be applied by healthcare professionals in order to implement the recommendations on the organization of care for these patients.

Adult↗
PubMed2006

MR evaluation of cardiovascular physiology in congenital heart disease: flow and function.

Cardiovascular magnetic resonance (CMR) has become the method of choice in the evaluation of a number of questions in congenital heart disease. In addition to morphology, modern CMR techniques allow the visualization of function and flow in a temporally resolved manner. Among the pathologies where these methods play a major role are shunts, septal defects, aortic coarctation, anomalies of the pulmonary arteries, and valvular regurgitation. This paper explains the basics of functional and flow encoded CMR and discusses their application in the assessment of several types of congenital heart disease.

Heart↗
PubMed1992

Pleuroperitoneal shunts for refractory chylothorax after operation for congenital heart disease.

Between 1980 and 1990, 10 of 12 children with a symptomatic chylothorax after operation for congenital heart disease failed to respond to traditional medical therapy (thoracentesis, tube thoracostomy, low-fat diet). All 10 patients underwent placement of a pleuroperitoneal shunt, with complete resolution of the chylothorax in 9 patients (90%). Cardiac catheterization, performed before placement of the pleuroperitoneal shunt in 5 patients, demonstrated elevated right atrial pressure in all patients (range, 10 to 25 mm Hg). The pleuroperitoneal shunt functioned effectively in 4 patients with moderately elevated right atrial pressures (range, 10 to 16 mm Hg; median, 13.5 mm Hg) but not in 1 patient with a right atrial pressure of 25 mm Hg. Pleuroperitoneal shunting as treatment for chylothorax after operation for congenital heart disease is safe and effective, even in the face of moderate elevations in right atrial pressure.

Chylothorax↗
PubMed1999

Telemedicine consultations in congenital heart disease: assessment of advanced technical capabilities.

OBJECTIVE: To assess the value of adding remote pointer and dynamic display capabilities to a telemedicine system designed to provide consultative services for patients with congenital heart disease. MATERIAL AND METHODS: Independent observations by the referring physician and the consulting physician provided the data for the assessment. Fifty-four teleconsultations involving 38 patients with 21 different congenital heart diseases were analyzed. The teleconsultations were based on previously obtained cineangiograms that were digitized and then transmitted by combined satellite and terrestrial-based technology. The observations, recorded by each physician at his workstation at the time of each teleconsultation, were summarized and analyzed statistically. RESULTS: In 108 observations, the pointer was believed to be helpful in 72 (67%), and dynamic display was helpful in 96 (89%). CONCLUSION: This study suggests that use of a pointer and dynamic display enhances teleconsultations for patients with congenital heart disease.

Arizona↗
PubMed1987

Serum erythropoietin levels in patients with congenital heart disease.

Serum erythropoietin levels were measured by radioimmunoassay in 146 children and young adults with congenital heart disease to assess the relationship between erythropoietin and clinical factors (heart failure, anemia, cyanosis) and hemodynamic variables affecting oxygen delivery and utilization. Erythropoietin values were in the normal range (10 to 30 microU/mL) in 73% (58 of 80) of the patients with and 82% (54 of 66) of those without cyanosis. Elevated erythropoietin values in cyanotic patients were associated with lower mixed venous oxygen saturation and tension than in cyanotic patients with normal erythropoietin levels, even though the degree of polycythemia was similar. In contrast, most of the acyanotic patients who had elevated erythropoietin levels were anemic. Of the blood oxygen measurements, mixed venous oxygen saturation and tension had the closest inverse correlation with erythropoietin values. The normal erythropoietin values in most patients are in accord with other observations that show that an elevation in erythropoietin level in response to hypoxia will be transient if it results in a rise in hemoglobin concentration "appropriate" to the degree of hypoxia. Persistent elevation of erythropoietin in patients with congenital heart disease may indicate harmful impairment of hemoglobin production that is potentially correctable.

Adolescent↗
PubMedDate not supplied

Nitric oxide in the evaluation of congenital heart disease with pulmonary hypertension: factors related to nitric oxide response.

Inhaled nitric oxide (NO) has been used in the preoperative evaluation of patients with congenital heart disease and pulmonary hypertension. The purpose of this study was to characterize responses in pulmonary vascular resistance (PVR) to oxygen and increasing doses of NO during cardiac catheterization and to determine if any related factors affect the response of the pulmonary vascular bed to NO. A prospective analysis of 42 patients (median age, 3.0 years) with congenital heart disease and pulmonary hypertension who underwent NO testing was performed. Systemic vascular resistance (SVR) and PVR were assessed in room air, 100% oxygen, and oxygen plus 20, 40, and 80 parts per million (ppm) NO. Changes in pulmonary artery pressure, PVR, and SVR were assessed. The response to NO was then correlated to individual patient's age, gender, type of heart defect, the presence of trisomy 21, and baseline PVR/SVR. There was a greater decrease in PVR and PVR/SVR with 20 ppm NO than with oxygen alone. There was no additional decrease at 40 or 80 ppm NO. There was no correlation between age, gender, type of congenital heart disease, and baseline PVR/SVR ratio with the degree of response to NO. Patients with trisomy 21 had less of a response to NO (p = 0.017) than patients without trisomy 21. There is no difference in determining PVR response with doses of NO beyond 20 ppm during cardiac catheterization. Age, gender, and baseline PVR/SVR ratio are not associated with responsiveness to NO. Patients with trisomy 21 may be less responsive to NO.

Administration, Inhalation↗
PubMed1984

Flying and congenital heart disease.

Only those congenital defects carrying a very low risk of complication (either before or after surgical correction) were considered. Atrial Septal Defects--(a) Ostium primum defects should be treated with caution either before or after surgical correction because of the risk of progressive conduction disorders and mitral regurgitation. (b) Ostium secundum defects could be considered for licensing (if the defect is small) or with surgical repair if the right ventricular systolic pressure is normal. (c) Sinus venosus defects--if too small to require surgical repair, licensing may be considered provided ambulatory electrocardiographic monitoring shows no evidence of arrhythmias. Surgery increases the risk of sino-atrial disease, thus licensing should be permitted only where there is no evidence of arrhythmia and adequate cardiological follow-up is possible. Ventricular Septal Defects--Subjects with very small defects not requiring surgical closure may be considered for licensing. Subjects who have had surgical closure have a risk of arrhythmias and should be carefully evaluated. Pulmonary Stenosis--If mild (either before or after surgery) may be licensed, but regular assessment perhaps including right heart catheterization is needed to demonstrate stability of the lesion. Persistent Ductus Arteriosus--Surgical closure should be recommended on diagnosis and need not affect licensing. Isolated Bicuspid Aortic Valve--Need not debar from licensing, but careful annual examination (with electrocardiogram 2-D echocardiography and fluroscopy ) is required to detect calcification, stenosis or regurgitation. Coarctation of aorta--Subjects who have had a repair before the age of 12 years may be considered for licensing after examination of other risk factors (blood pressure at rest and on exercise in particular). Those repaired over the age of 12 may be considered for restricted licensing if normotensive. These recommendations will need review in the light of further long-term studies currently under way.

Adult↗
PubMedDate not supplied

Computed tomography for the diagnosis of congenital heart disease in pediatric and adult patients.

The development of multi-slice spiral computed tomography (CT) has increased the clinical use of cardiac CT imaging in patients with congenital heart disease. Multi-slice CT has the advantages of fast scan speed; high spatial resolution, enabling the acquisition of isotropic volume data; and simultaneous evaluation of airways and lung parenchyma, thus increasing the ability to answer most clinical questions about structural abnormalities in patients with congenital heart disease. When coupled with electrocardiography-gating, multi-slice spiral CT can be used in functional evaluations, including ventricular wall motion, ventricular ejection fraction, and motion of cardiac valves, as well as enabling the performance of high-quality coronary CT angiography. In this article, we review imaging techniques of multi-slice spiral CT and imaging findings in pediatric and adult patients with various congenital heart diseases.

Adult↗
PubMed1999

Regional differences in birth prevalence of congenital heart disease in Malta.

AIM: Recent studies have shown a correlation between environmental pollutants and increased risk of selected congenital malformations. The South-East area of the Island of Malta is much more industrialised than the more rural North-West area. The aim of this study was to test the null hypothesis that there are no regional differences in Malta in the prevalence at birth of congenital cardiac malformations. METHODS: Live born infants with congenital cardiac malformations born between 1990 and 1994 were allocated to 10 areas, and thence to 2 regions of 5 areas each, so as to constitute North-West and South-East Malta. Official publications gave population totals and growth rates. Those infants with congenital cardiac malformations were then compared between the different regions using chi2 and the Mann-Whitney U test. RESULTS: The overall prevalence of congenital heart disease at birth was 8.8/1000 live births. The birth prevalence for the South-East region (10.1/1000 LB--95% CI 8.4-12.3/1000 live births) was significantly higher than for the North-West (7.4/1000 live births--95% CI 6.0-9.0/1000 live births)--p=0.03, Odds ratio 1.38 (95% CI 1.05-1.80). The Mann-Whitney U test showed a significant difference in the distribution amongst the 10 defined areas (p=0.016). The Central-East area had the highest prevalence of cardiac malformations in the entire Island--p=0.02, Odds ratio 1.70 (95% CI 1.10-2.61). Demography showed an efflux of individuals from the South-East of Malta. CONCLUSION: The higher prevalence of congenital heart disease noted at birth in South-East Malta is unlikely to be due to genetic factors, as these would have migrated North-West along with the population movement. An environmental factor, therefore, seems more likely to be responsible for the increased predisposition to congenital heart disease in the South-East of Malta.

Birth Rate↗
PubMed2006

Predictive factors for long-term prognosis in adults with cyanotic congenital heart disease--Japanese multi-center study.

OBJECTIVE: Adults with cyanotic congenital heart disease (CCHD) are associated with a significant incidence of morbid events and premature deaths that may be predicted during childhood. We aimed to identify predictive factors related to long-term prognosis through a Japanese multi-center cross-sectional study. METHODS: Data were collected from 253 adults with CCHD (126 men; age 28 (18 to 56) years) from 15 participating centers between 1998 and 2003. Laboratory data such as cardiothoracic ratio (CTR), percutaneous oxygen saturation (SpO2), hematocrit levels (Ht) and platelet counts (Pl-c) at the age of 15 years were collected for predictive factor analysis for death and cardiovascular and systematic events. Predictive factors were determined by multivariate Cox regression analysis. RESULTS: After a mean follow-up of 21 (0-42) years, 23 patients died with a median age of 29 (18-54) years (heart failure in 8, sudden in 6 and systematic complications in 9). Survival since 18 years of age was 91% and 84% at 10 and 20 years, respectively. Significant predictive factor for death was Pl-c<130x10(9)/l and for renal failure (n=7) was Ht>65%. 162 patients were hospitalized and predictors for hospitalization due to heart failure (n=45) were common atrioventricular canal CTR>60% and Pl-c<100x10(9)/l and that due to arrhythmias (n=44) were systematic right ventricle and CTR>60%. CONCLUSIONS: This multi-center study provides an objective basis of assessing the long-term prognosis in patients with CCHD. These data are useful in making decisions regarding medical management and in favorably altering the non-operative course of the disease.

Adolescent↗