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PubMed2003

Congenital heart disease: the original heart failure syndrome.

Heart failure is characterised by a triad comprising cardiac abnormality, exercise limitation and neurohormonal activation. The 2% of the adult population who suffer with heart failure are known to derive both symptomatic and prognostic benefit from exercise and pharmacologic neurohormonal antagonism. The existence of heart failure has traditionally been considered in the context of ischaemic, hypertensive, valvular and myopathic disease but in this article we develop the argument that patients with congenital heart disease also manifest all the pathophysiological criteria that constitute the chronic heart failure syndrome. We discuss the inherent limitations to a purely anatomical approach to congenital heart disease, bring attention to the large and rapidly growing population of adults with this condition and conclude by calling for a therapeutic approach to congenital heart disease that is based on the heart failure paradigm.

Adult↗
PubMed1999

Outcome of pregnancy in women with congenital heart disease.

Improvements in diagnosis and surgical technique for correction have led to an increasing number of women with congenital heart disease reaching the child-bearing age. Pregnancy places considerable strain on the heart and circulation and necessitates marked cardiorespiratory adaptation. Today, with the exception of the Eisenmenger syndrome, there is no increased mortality associated with pregnancy in congenital heart disease. In contrast, there is still considerable morbidity, due to congestive heart failure, thromboembolic complications and disturbances of rhythm. Fetal outcome is complicated by a high rate of spontaneous abortions (20-25%), retardation of fetal growth, and premature delivery (almost 100% in cyanotic mothers). Based on an extensive review of the literature, we discuss the specific risks in pregnancy depending on the hemodynamic situations produced by different heart defects. We also discuss the risks and advantages of different regimens for anticoagulation. Counselling concerning contraception is frequently inadequate. The most important problems are thromboembolic complications with the use of hormonal contraception, and hyper- and dysmenorrhea in those using intrauterine devices. Finally, the genetic risks must be considered, differentiating between single gene defects and the sex of the parents suffering from congenital heart diseases.

Female↗
PubMed2000

[Adjustable interatrial communication in management of severe congenital heart disease].

OBJECTIVE: To assess the use of adjustable interatrial communication in patient with severe congenital heart disease undergoing open heart surgery. METHODS: From February 1998 to June 1999, 13 patients with severe and complex congenital heart disease underwent open heart surgery. Seven patients underwent total cavopulmonary connection, five patients repair of Ebstein's anomaly, and one patient right atrial-pulmonary connection. Adjustable interatrial communication was made in all patients. Four patients had the communication closed at the time of operation, 4 patients had the communication opened at the time of operation, and 5 patients had the communication adjusted and closed postoperatively. RESULTS: All patients had a smooth postoperative process. There were no postoperative death and no severe postoperative complications. CONCLUSION: Adjustable interatrial communication for the patients undergoing modified Fontan procedure or correction of Ebstein's anomaly may be advantageous.

Adolescent↗
PubMed2006

Three-dimensional CT scanning: a new diagnostic modality in congenital heart disease.

New generation multislice CT technology has changed the approach to non-invasive assessment of congenital heart disease, in both paediatric and adult patients. This is mainly because of rapid advances in spatial and temporal resolution and in post-processing capability. At Hôpital Necker-Enfants Malades, CT with multiplanar and three-dimensional reconstruction has become a routine examination in the evaluation of congenital heart disease planning surgery, complex interventional catheterisations and for follow-up. It has proved to be an invaluable diagnostic and decision-aiding methodology in these situations, as a complement to echocardiography and, increasingly, as a substitute for diagnostic angiography (which is usually associated with higher-dose radiation and longer sedation times, as well as occasional morbidity). This review illustrates the current status of 64-slice CT in congenital heart diseases, including assessment of the aorta, the coronary arteries, the pulmonary arteries, the systemic and pulmonary veins, and other intra- and extracardiac malformations.

Aorta, Thoracic↗
PubMed1980

[New angles of view in cineangiography of congenital heart disease (author's transl)].

Axial oblique views were performed during cineangiography of congenital heart disease on patients of a paediatric cardiological unit for a period of two years. The resulting angle-patterns are shown in a synoptical review, and indications for angiographic demonstration of morphological conditions in congenital heart disease are discussed. In comparison with the literature to date, this is the first report of experience with a biplane synchronised "C" arm cineangiographic system.

Child↗
PubMed1979

Prevalence and characteristics of disporportionate ventricular septal thickening in infants with congenital heart disease.

Necropsy studies were performed in 125 infants, 2 years old or less, with a variety of congenital heart malformations. Disproportionate ventricular septal thickening (septal-to-left ventricular free wall thickness ratio greater than or equal to 1.3) was present in 31 (25%) of the 125 patients. In the majority of patients, an abnormal septal-to-free wall ratio did not appear to be a manifestation of genetically transmitted hypertrophic cardiomyopathy, since: 1) marked disorganization of septal myocardium (involving greater than or equal to 5% of the tissue section) was present in only five of the 31 patients with disproportionate septal thickening; and 2) echocardiographic studies in first degree relatives of four other patients with disproportionate septal thickening and normal septal architecture did not show asymmetric septal hypertrophy. The relatively high prevalence of septal-to-free wall ratios of 1.3 or greater in this population of infants with congenital heart disease appeared to be due in part to minor differerences in ventricular wall thicknesses (in patients with relatively thin absolute wall thicknesses) which may produce particularly large deviations from unity in septal-to-free wall ratio. Hence, 1) nongenetically transmitted disproportionate septal thickening is relatively common in infants with congenital heart diseases studied at necropsy: and 2) a septal-to-free wall ratio of greater than or equal to 1.3 cannot be used as the sole criterion for identifying associated hypertrophic cardiomyopathy in infants with other congenital heart diseases, particularly if marked absolute septal thickening is absent.

Cardiomegaly↗
PubMed2005

Heart failure in the adult patient with congenital heart disease.

BACKGROUND: Adult survival is now the rule for most congenital heart defects. However, despite surgical "repair," heart failure remains a common long-term complication of congenital heart disease. Heart failure specialists are seeing an increasing number of patients with congenital defects as the underlying etiology of their heart failure. METHODS AND RESULTS: Familiarity with the anatomy, physiology, and long-term sequelae of surgical correction are essential for providing care to this complex group of patients. CONCLUSION: This review discusses the diagnosis, pathophysiology, and management of the adult congenital heart patient with concomitant heart failure.

Adult↗
PubMedDate not supplied

Pericardial extramedullary hematopoiesis in a neonate with congenital heart disease. A case report.

A case of pericardial extramedullary hematopoiesis in a neonate with congenital heart disease was diagnosed by fine needle aspiration cytology. Undifferentiated blasts, promyelocytes, metamyelocytes and nucleated red blood cells were evident in the pericardial fluid specimen. The differential diagnosis of bone marrow contamination of pericardial fluid secondary to inadvertent sampling of neonatal hypodense bone tissue is also discussed. To our knowledge, this is the first reported case of neonatal pericardial extramedullary hematopoiesis associated with congenital heart disease.

Biopsy, Needle↗
PubMed1997

Chromosome abnormalities in congenital heart disease.

Refinements in cytogenetic techniques have promoted progress in understanding the role that chromosome abnormalities play in the cause of congenital heart disease. To determine if mutations at specific loci cause congenital heart disease, irrespective of the presence of other defects, and to estimate the prevalence of chromosome abnormalities in selected conotruncal cardiac defects, we reviewed retrospectively cytogenetic and clinical databases at St. Louis Children's Hospital. Patients with known 7q11.23 deletion (Williams syndrome), Ullrich-Turner syndrome (UTS), and most autosomal trisomies were excluded from this analysis. Two groups of patients were studied. Over a 6.5-year period, 57 patients with chromosomal abnormalities and congenital heart disease were identified. Of these, 37 had 22q11 deletions; 5 had abnormalities of 8p; and 15 had several other chromosome abnormalities. The prevalence of chromosome abnormalities in selected conotruncal or aortic arch defects was estimated by analysis of a subgroup of patients from a recent 22-month period. Chromosome abnormalities were present in 12% of patients with tetralogy of Fallot, 26% in tetralogy of Fallot/pulmonary atresia, 44% in interrupted aortic arch, 12% in truncus arteriosus, 5% in double outlet right ventricle, and 60% in absent pulmonary valve. We conclude that chromosome analysis should be considered in patients with certain cardiac defects. Specifically, fluorescent in situ hybridization (FISH) analysis of 22q11 is indicated in patients with conotruncal defects or interrupted aortic arch. High resolution analysis should include careful evaluation of the 8p region in patients with either conotruncal or endocardial cushion defects.

Chromosome Deletion↗
PubMed2004

[Piercing and tattoos in patients with congenital heart disease -- is it a problem?].

Piercing and tattooing enjoy widespread popularity in modern society. Patients with congenital heart disease are at elevated risk for infective endocarditis. However, it is not yet known whether piercing and tattooing are dangerous for these patients.A search of the literature provided 10 published cases of infective endocarditis after piercing or tattooing. Affected patients were adolescents or young adults ranging in age from 13 to 30 years (5 male, 5 female). Four of the patients had a known cardiac risk factor for endocarditis (bicuspid aortic valve, postoperative trans-position of the great arteries, postoperative coarctation, postoperative aortic valve stenosis). Piercing preceded endocarditis in 9 cases (4 times mouth, 2 ear, 1 nose, 1 breast, 1 navel), one tattoo. The following agents were isolated: S. aureus in 4 cases, 2 S. epidermidis, 1 Str. viridans, 1 Neisseria mucosa, 1 Haemophilus aphrophilus, 1 Haemophilus parainfluenzae. All patients were treated with antibiotics. Six patients underwent cardiac surgery (5 of them valve replacement). Patients with congenital heart disease constitute less than 1% of the population. Thus, they are clearly overrepresented in the published literature. Epidemiologic conclusions are not possible from these data. However, patients with congenital heart disease and their parents should be strongly advised against piercing and tattooing with regard to the risk of infective endocarditis.

Adolescent↗
PubMed2006

Using multidetector-row CT in neonates with complex congenital heart disease to replace diagnostic cardiac catheterization for anatomical investigation: initial experiences in technical and clinical feasibility.

BACKGROUND: Echocardiography is the first-line modality for the investigation of neonatal congenital heart disease. Diagnostic cardiac catheterization, which has a small but recognized risk, is usually performed if echocardiography fails to provide a confident evaluation of the lesions. OBJECTIVE: To verify the technical and clinical feasibilities of replacing diagnostic cardiac catheterization with multidetector-row CT (MDCT) in neonatal complex congenital heart disease. MATERIALS AND METHODS: Over a 1-year period we prospectively enrolled all neonates with complex congenital heart disease referred for diagnostic cardiac catheterization after initial assessment by echocardiography. MDCT was performed using a 40-detector-row CT scanner with dual syringe injection. A multidisciplinary congenital heart disease team evaluated the MDCT images and decided if further diagnostic cardiac catheterization was necessary. The accuracy of MDCT in detecting separate cardiovascular anomalies and bolus geometry of contrast enhancement were calculated. RESULTS: A total of 14 neonates were included in the study. No further diagnostic cardiac catheterization was needed in any neonate. The accuracy of MDCT in diagnosing separate cardiovascular anomalies was 98% (53/54) with only one atrial septal defect missed in a patient with coarctation syndrome. The average cardiovascular enhancement in evaluated chambers was 471 HU. No obvious beam-hardening artefact was observed. CONCLUSION: The technical and clinical feasibility of MDCT in complex congenital heart disease in neonates is confirmed. After initial assessment with echocardiography, MDCT could probably replace diagnostic cardiac catheterization for further anatomical clarification in neonates.

Cardiac Catheterization↗
PubMed1994

Insurability issues facing the adolescent and adult with congenital heart disease.

Access to comprehensive health care coverage for congenital heart disease patients is often limited because of their "preexisting condition." Life insurance may be difficult to obtain if the congenital lesion carries a higher than standard mortality rating. Various insurance program options are available to many of these patients. Knowledgeable health care professionals can assist these individuals to obtain the needed coverage, enabling better medical and financial security for this growing patient population.

Adolescent↗
PubMed1995

Congenital heart disease and sudden death in the young.

Sudden death is a frequent mode of fatal outcome in cardiac disease and does not exclude young people. The aim of this investigation was to establish whether and to what extent sudden death in the young may be ascribable to the substrate of underlying congenital heart disease. Among 182 young people (< or = 35 years) who died of cardiac sudden death and underwent postmortem examination, 58 (32%) had congenital heart disease. Seven showed an intrapericardial rupture of aortic dissection, in the setting of Marfan syndrome in two, isolated bicuspid aortic valve in two, and bicuspid aortic valve and isthmic coarctation in three; all exhibited equally severe degeneration of the aortic wall. Sixteen cases had conduction system anomalies, mostly bypass tracts; 15 coronary artery anomalies (three ostial valve-like stenosis, five origin from the wrong aortic sinus, and seven deep intramyocardial course); 12 hypertrophic cardiomyopathy; five postoperative congenital heart disease including scar following ventriculotomy, conduction system injury, and defects left unrepaired; and three congenital aortic valve stenosis. One third of sudden deaths in the young was ascribable to structural defects present since birth. A large spectrum of congenital heart disease involves the risk of sudden death, but most structural defects are usually not considered to be life threatening. Some of these concealed defects are potentially detectable in life by clinical imaging techniques.

Adolescent↗
PubMed1985

The skeletal manifestations of clubbing: a study in patients with cyanotic congenital heart disease and hypertrophic osteoarthropathy.

The skeletal manifestations of clubbing of the digits have been occasionally noted and only briefly discussed in the literature. We investigated the radiographic features of digital clubbing in 37 patients with diverse diseases including cyanotic congenital heart disease, lung malignancy-associated hypertrophic osteoarthropathy, and idiopathic cases. We identified two types of bone changes--osteolysis or bone dissolution, and bone formation or hypertrophy. The changes were more evident in the feet than in the hands, and the degree of soft tissue change did not always reflect the underlying osseous abnormalities. The relationship of these changes (ie, osteolysis, hypertrophy) to each other appear to depend in part on the underlying disease as well as the time course or disease duration. Thus, clubbing and hypertrophic osteoarthropathy may not represent distinct entities; our data suggest that they may be stages in an evolving, more generalized process of new bone formation or hypertrophy followed by osteolysis or atrophy affecting many parts of the skeleton.

Adolescent↗
PubMed1995

Dynamic three-dimensional transesophageal echocardiography using a computed tomographic imaging probe--clinical potential and limitation.

Dynamic three-dimensional echocardiography is a new diagnostic tool for spatial visualisation of cardiac anatomy and volumetric assessment. A computer-controlled probe acquires parallel tomographic slices, from which dynamic three-dimensional images of the heart can be reconstructed. Thirty adult patients with valvular heart diseases, congenital heart diseases, intracardiac masses, heart failure and other cardiac lesions, underwent conventional two-dimensional (n = 30), three-dimensional echocardiography (n = 30) and thermodilution (n = 17). The feasibility, usefulness and possibility of simulating a surgical view of intracardiac anatomy and exact volumetry were determined. The two different morphologic images were compared qualitatively. For quantitative analysis volumetry was performed using standard thermodilution technique and dynamic three-dimensional echocardiography. In more than 80% of the patients additional morphologic information was gained and a strong correlation (r = 0.75-0.95) between two volumetry assessments was found. Based on this findings, dynamic three-dimensional echocardiography is an additional and valuable approach in the perioperative and intensive care management in this group of patients.

Adult↗
PubMed1988

School adjustment of children with congenital heart disease.

A correlation study was conducted to investigate school adjustment of school-age children and adolescents with varying degrees of severity of congenital heart disease. School adjustment was inferred from scholastic achievement, scholastic grade average, social competence and behavior as assessed by mothers, and adaptive functioning and behavior as assessed by teachers. Forty-eight children with mild, moderate, and severe congenital heart disease (16 children in each group) and their mothers and teachers participated in the study. Data were obtained from: (a) the Coopersmith Self-Esteem Inventory, and the Children's Depression Inventory completed by the children; (b) the Child Behavior Checklist and demographic data sheet completed by the mothers; (c) the Child Behavior Checklist--Teacher's Report Form, and the information sheet on Absence Rate and Scholastic Grade Average completed by the teachers; and (d) the children's medical records. Two-way and three-way analysis of variance were performed between the mean scores for each of the variables. Statistical significance was found (a) between the means for absence rate, behavior as assessed by teachers, and depression with respect to severity of congenital heart disease; (b) between the means for scholastic achievement, scholastic grade average, and adaptive functions and behavior as assessed by teachers according to IQ level; (c) between the means for scholastic achievement, scholastic grade average, and social competence as assessed by mothers regarding level of self-esteem; (d) between the mean scores for social competence with respect to level of depression. There were also three significant interactions: between age and sex for the means of behavior as assessed by mothers; between severity of heart disease and I level for the means of behavior as assessed by teachers; and between severity of heart disease and depression level for the means of social competence. It was concluded that children with congenital heart disease with the added burdens of below average IQ, low self-esteem, and high depression were at particular risk for poor school adjustment.

Absenteeism↗
PubMed1992

Unsuspected congenital heart disease in neonates receiving extracorporeal life support: a review of ninety-five cases from the Extracorporeal Life Support Organization Registry.

The purpose of this study was to determine the frequency of patients with congenital heart disease who were given extracorporeal life support (ECLS) for respiratory failure. Underlying congenital heart disease "masked" by respiratory failure occurred in 2%. The most frequent pre-ECLS diagnosis that "masked" congenital heart disease was persistent fetal circulation. Of neonates with a pre-ECLS diagnosis of persistent fetal circulation, congenital heart disease was found in 56 (9%) of 623 patients.

Age Factors↗