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PubMed1981

[Arteriovenous malformation associated with congenital heart disease, with a remark on accompanying cardiopulmonary dysfunction (author's transl)].

A 10 years old school boy, who had been known to have a small ventricular septal defect from 2 months after birth, was admitted to us with a diagnosis of subarachnoid hemorrhage on October 24, 1978. On admission, neurological examination revealed mild right hemiparesis and miotic pupils in semicomatose state, and thrilling, that was equivalent to Levine III, pansystolic murmur and cyanosis of nails were noticed. By CT scan, intraventricular hemorrhage originated from hemorrhage in the basal ganglia was shown and the left CAG revealed an arteriovenous malformation in the left basal ganglia. Electrocardiogram showed mild cardiac failure and chest x-ray showed pulmonary congestion. Neurological symptoms and abnormal cardiopulmonary changes made well manageable after bilateral ventricular drainage. Twenty four cases of arteriovenous malformation associated with congenital heart disease were reviewed in the reported literature, and 20 cases were found to combine with Galenic aneurysm with congenital heart disease. All of those cases had been in a little pause after the birth and the most of congenital heart diseases were of patient ductus arteriosus. The rest of cases including our present case were 4 cases, who had rather small arteriovenous malformation, and the severity of the congenital heart disease was attributable to the patients mortality. In all of the report at present only our case was the single survival. Embryological investigation on arteriovenous malformation of the brain and congenital heart disease indicated that association of both conditions could be attributable to the vascular malformation in a very limited gestational stage, and that the incidence should be much less than reported. Our case showed the intracranial hypertension was found to create serious cardio-pulmonary dysfunction when congenital heart disease being accompanied, and it was presumably relating to the relatively higher incidence of Galenic aneurysm associated with ductus arteriosus.

Child↗

PubMed1999

[1000 cardiac catheterizations in congenital heart disease].

Over the past 15 years, percutaneous therapeutic cardiac catheterization has become increasingly important in the treatment of congenital heart disease. We describe our experience in 1000 such catheterizations between 1993-1997. 55% were in 1-12-year-olds; only 20% were in patients younger than 1 year old and 11.3% were in adults with congenital heart defects. In about 50% it was at least a second cardiac catheterization. Overall, there were 425 therapeutic cardiac catheterizations, increasing from 33% in the first 200 procedures, to 63% in the last 200. We performed 30 different types of therapeutic catheterizations: 23.3% were valvular dilations, 21.4% vessel angioplasties, 36.9% closure procedures, 9.2% electrophysiological procedures, and 9.2% miscellaneous. In 31.3% of therapeutic catheterizations we used 12 new procedures. Minor complications occurred in 8.5% and major in 0.6%; most complications were successfully treated or were self-limited and there was no residual damage. In this report the current role of each type of major catheterization is discussed on the basis of our experience. Further development of technology for lesions not amenable to currently available transcatheter methods, and longer follow-up for current techniques will consolidate the role of therapeutic cardiac catheterization in congenital heart disease.

Adolescent↗

PubMed2003

Secretion of A-type and B-type natriuretic peptides into the bloodstream and pericardial space in children with congenital heart disease.

OBJECTIVE: To determine the secretion of A-type and B-type natriuretic peptides into the bloodstream and pericardial space in children with congenital heart disease. METHODS: Plasma and pericardial fluid samples were obtained from 77 patients undergoing total correction for congenital heart disease. All patients underwent detailed right-sided and left-sided cardiac catheterization preoperatively. RESULTS: A-type natriuretic peptide levels in pericardial fluid were lower than those in plasma (33.0 +/- 23.1 versus 39.8 +/- 33.6 pg/mL, P <.05), and B-type natriuretic peptide levels in pericardial fluid showed marked elevations compared with those in plasma (231.9 +/- 305.6 versus 19.8 +/- 29.3 pg/mL, P <.0001). The A-type and B-type natriuretic peptide levels in plasma correlated with those in pericardial fluid (R =.522, P <.0001; R =.595, P <.0001). For A-type and B-type natriuretic peptide levels in plasma, the relation with biventricular volume had the highest correlation (R =.669, P <.0001; R =.652, P <.0001). The patients with a pulmonary-to-systemic flow ratio greater than 2 (n = 19) had high levels of natriuretic peptides not only in plasma (58.3 +/- 43.2, 40.5 +/- 49.4 pg/mL, P <.05) but also in pericardial fluid (44.4 +/- 31.5, 287.2 +/- 198.5 pg/mL, P <.05), and higher correlation between A-type and B-type natriuretic peptide plasma levels and left ventricular volume (R =.913, P <.0001; R =.787, P <.0001). CONCLUSIONS: B-type natriuretic peptide is secreted not only into the bloodstream but also into the pericardial space in children with congenital heart disease. Natriuretic peptide levels in plasma correlated well with biventricular volume. The left ventricle was considered to be the main source of secreted natriuretic peptides in the patients with a pulmonary-to-systemic flow ratio greater than 2.

Adolescent↗

PubMed2001

Plasma levels of asymmetrical dimethyl-L-arginine in patients with congenital heart disease and pulmonary hypertension.

Asymmetrical dimethyl-L-arginine (ADMA) is an endogenous inhibitor of nitric oxide synthase. We hypothesized that plasma levels of ADMA could be increased in patients with congenital heart disease and pulmonary hypertension. Cardiac catheterization was performed in 20 children and young adults with congenital heart disease with a median age of 10 years (range, 4 months to 33 years). The patients were assigned to group I (high flow, low pressure; n = 14) when Qp/Qs was 1.5 or greater and the mean PAP was less than 25 mm Hg or to group II (high pressure, high resistance; n = 6) when the mean PAP was greater than 25 mm Hg and Rp/Rs was greater than 0.3. Blood samples were taken from pulmonary vein or left ventricle. ADMA was measured by high-performance liquid chromatography. In addition, levels of ADMA were measured in peripheral venous blood obtained from eight control patients. Levels of ADMA in control patients (median, 0.21 microM/l; range, 0.08-0.27 microM/l) did not differ from levels obtained in group I (median, 0.30 microM/l; range, 0.06-0.49) microM/l). Patients in group II showed increased plasma levels of ADMA (median, 0.55; range, 0.25-0.79) (p < 0.01). Inhibition of nitric oxide synthase by increased levels of ADMA might contribute to pulmonary hypertension in patients with congenital heart disease.

Adolescent↗

PubMed1998

Dynamic three-dimensional echocardiography using parallel slicing: a promising diagnostic procedure in adults with congenital heart disease.

In 18 of 77 adult patients presenting with congenital heart disease, three-dimensional conceptualization was found to be insufficient using two-dimensional echocardiographic imaging. In these individuals, three-dimensional image reconstruction from digitized parallel images was carried out, providing additional information in 14 of them. Three-dimensional echocardiography and volumetry may, especially in patients with atrial septal defect, cor triatriatum and corrected transposition of the great arteries, be an asset amplifying the diagnostic information yielded by conventional two-dimensional echocardiographic approaches.

Adolescent↗

PubMed1991

The value of the autopsy in congenital heart disease.

We reviewed autopsies in 50 consecutive cases of congenital heart disease between 1980 and 1988. Autopsy results were compared to premortem clinical diagnosis. The patients ranged in age from two days to 66.5 years with a mean age of 6.42 years; 68% were less than one year of age. The most common diagnoses were hypoplastic left heart syndrome in 16 (32%) and ventricular septal defect in five (10%). Other common diagnoses included coarctation of the aorta, hypoplastic right heart syndrome, tetralogy of Fallot, transposition of the great vessels and tricuspid atresia. No patient had a missed diagnosis that would have changed survival (Class 1). Four (8%) of the patients had one missed major diagnosis that did not change their survival because they were already being symptomatically treated or because no clinical treatment was available (Class 2). The majority of autopsy derived data were missed minor diagnoses that were related [14 (28%)-Class 3] or unrelated [12 (24%) - Class 4] to the terminal disease process. However, despite the small percentage of autopsy findings that would have directly affected survival, the autopsy provided important additional clinical information in 30 (60%) patients. This information could be used to correct management errors and to develop protocols for prophylaxis against known complications. The pediatric autopsy can provide useful clinical data that may be a guide to altering clinical therapy and is essential in providing genetic counseling.

Adolescent↗

PubMed1978

A predictive study of congenital heart disease and need for care.

For long-term planning in the delivery of health care, prevalence data are essential for budget estimates in terms both of distribution and training of manpower and fiscal responsibility. From incidence figures, from the knowledge of the natural history of congenital heart disease and from predicted population estimates it is possible to construct a model that reflects the prevalence of congenital heart disease. This has been done for the state of California; the methods used and the data gathered should prove useful nationally. It is estimated that there were in 1975 in California 17,531 children under 21 years of age with congenital heart disease; 24 percent of these had ventricular septal defects and 23 percent had pulmonary stenosis, 11 percent had atrial septal defects and 9 percent had aortic stenosis; the other forms of congenital heart disease constituted the remaining 33 percent. Based on these estimates it is then possible to plan the medical resources necessary for optimal care.

Adolescent↗

PubMed2001

Cardiac output measurements in congenital heart disease: validation of a simple, portable Doppler method.

Noninvasive Doppler cardiac output measurements are clinical standards in adults and children. Presently, these standard Doppler methods generally require relatively expensive and large imaging equipment with complex signal-processing techniques. Thus, universal access to these important measurements has been limited. Simple, portable Doppler methods have been validated previously and applied to infants and children without cardiac disease. Nevertheless, these specific, inexpensive techniques have not been validated in children with complex congenital heart disease. Accordingly, we compared a noninvasive, suprasternal, non-image-guided, pulsed Doppler cardiac output with standard invasive Fick cardiac outputs in 20 patients (age range, 1 month to 15 years) with congenital heart disease. Doppler cardiac output was displayed by multiplying the mean velocity with an operator-selected angiographic or echographic estimate of the aortic diameter. The values from linear regression analysis were r = 0.96; Doppler cardiac output = 0.98 x Fick cardiac output - 0.08 L/min (range, 0.55-3.10 L/min). The 95% confidence limits were less than 35% of the mean of Doppler and Fick cardiac outputs across the range of measurements. We conclude that relatively linear and accurate measurements of cardiac output can be made by this simple, inexpensive, portable method in selected infants and children with various forms of congenital heart disease.

Adolescent↗

PubMedDate not supplied

Outcomes of congenital heart disease: a review.

One million Americans are affected by congenital heart disease (CHD) (American Heart Association [AHA], 2003). As survival has improved, evidence has accumulated that CHD touches many aspects of the lives of those affected. Children and adults report difficulties with physical growth and/or stature. Gross motor abnormalities are common in both groups, and significantly reduced exercise tolerance is reported in adults with CHD, including those with minor defects. Behavioral and psychiatric abnormalities are reported in adolescents and young adults, including a significant incidence of depression and anxiety disorders. The data are conflicting regarding quality of life (QOL) in children and adults with CHD. More research is needed examining outcomes, particularly outcomes of those diagnosed, managed, and repaired using modern technology.

Heart Defects, Congenital↗

PubMed1997

[A study on the genetic epidemiology of congenital heart disease].

In order to explore the genetic model of congenital heart disease (CHD), 375 individuals of CHD were investigated. The results showed that the incidence of primary relatives was 6.9%, the heritability was 66.13 +/- 1.13%, the incidence of secondary relatives was 1.7% and the heritability was 38.25 +/- 8.3% respectively. The average heritability among primary, secondary relatives was 65.65 +/- 1.09% which suggested that the genetic factor played an important role in pathogenesis. The sequence of the incidence in the relatives showed: primary relatives (6.9%) > secondary relatives (1.7%) > distant relatives (0.86%) and was in accordance with polygenic incidence, suqqesting that gene play an important role in the liability variation of CHD.

Adolescent↗

PubMed1993

Transesophageal echocardiography in congenital heart disease in the adult.

Progress achieved during the last three decades in medical and surgical treatment of infants and children with congenital heart disease has resulted in a significant number of patients surviving to adulthood. The complexity of the basic malformations in addition to the superimposed surgical interventions makes accurate diagnostic imaging essential. The scope of information obtainable from high-quality TEE supports its use in the follow-up of operated and unoperated adult patients, as well as in surgical and interventional procedures. Application of the segmental analysis of anatomy is as important during TEE as it is during TTE. Supplemental transthoracic 2-D and spectral Doppler flow measurements may be needed when poor beam alignment during TEE precludes accurate gradient determinations. Although, to date, most reports of TEE in patients with congenital heart disease have involved children and adolescents, the informational advantage is potentially greater in older adolescents and adults in whom transthoracic windows are frequently limited. In our experience with 125 TEE studies in adult patients with congenital heart disease (the majority using a biplane probe), horizontal plane TEE imaging provided important additional information concerning pulmonary venous connections, atrial baffle function, atrioventricular anatomy and function, left ventricular outflow tract lesions, and great artery positional relationships. Vertical plane TEE enhances imaging of systemic and pulmonary venous connections, atrial situs, venosus atrial septal defects, atrioventricular anatomy and function, and the ventricular septum and outflow tracts and is essential for complete assessment of complex right ventricular outflow tract anatomy, ventriculoarterial alignment, and the sizing of all aortic segments in coarctation of the aorta.

Adult↗

PubMed2002

The role of the atria in congenital heart disease.

The atria play an important role in adult congenital heart disease. Atrial function is often altered due to longstanding pressure or volume overload. Cardiac surgery inflicts lasting damage to the atria, which leads to loss of atrial compliance. Both the history of atrial overload and the atrial scarring form substrates for atrial tachycardias. There has been a growing interest in the interatrial septum in the past few years. There is evidence for a role of the persisting foramen ovale and atrial septal aneurysm as a causative or permissive factor in cerebral stroke. Catheter closure of the PFO may be an attractive option, especially for younger patients.

Adult↗

PubMed1995

Uniparental isodisomy for chromosome 16 in a growth-retarded infant with congenital heart disease.

We report a growth-retarded infant with congenital heart disease and maternal isodisomy for chromosome 16. Non-mosaic trisomy 16 was detected at mid-trimester chorionic villus sampling, performed because biochemical screening indicated an increased Down's syndrome risk. Further karyotyping analysis of the placenta, after delivery, showed a 50 per cent mosaic trisomy 16. The infant had an atrioventricular (A-V) canal defect, scoliosis, and several minor dysmorphic features. Although uniparental disomy for chromosome 16 has been reported previously, to our knowledge this is the first case of uniparental isodisomy for chromosome 16 which has been investigated with multiple DNA probes.

Abnormalities, Multiple↗

PubMed1999

Repair of pediatric pectus deformity and congenital heart disease as a combined procedure.

Coexisting pectus deformity and congenital heart disease is not uncommon. Traditionally, the approach to this problem has been to repair each one with a separate surgical procedure because of fear of increased complications from bleeding, infections, and anesthesia. More recently, many reports of successful combined repair have been published, particularly in adults with coronary artery or aortic pathology. The authors wished to determine the feasibility of this combined procedure in younger patients, particularly those with a severe pectus deformity. Three patients underwent repair, including a 17 year old with Marfan's syndrome and a severe pectus excavatum deformity. The postoperative course was smooth for these patients, and all had good short- and long-term (over 18 months) results.

Adolescent↗

PubMed1989

Development of personality and intelligence in children with congenital heart disease.

One hundred and twenty-eight children with congenital heart disease (CHD) and 89 healthy controls were compared according to their development of personality and intelligence. Presence of physical disability in daily life was the independent variable for dividing children with CHD into two groups: children with physical handicap due to CHD (n = 77) have lower IQ scores than healthy children. Unexpectedly, in the parents' opinion they show no difference in behaviour patterns. On introspective examination, these cardiac patients showed an increased feeling of inferiority and of basic anxiety and a more impetuous behaviour as their way of self-protection, but a reduced need for independence due to parental overprotection was not confirmed. None of these observations could be detected in children with CHD who are physically fit (n = 51).

Adolescent↗

PubMedDate not supplied

Breastfeeding success among infants with congenital heart disease.

It is a widespread misconception that infants with congenital heart disease (CHD) are not able to breastfeed. The purpose of this study was to describe breastfeeding duration and outcomes among a high-risk group of infants with CHD. Mothers of 68 infants at least 6 months of age, who had experienced cardiac surgery during the neonatal period, were surveyed regarding breastfeeding and milk expression habits. Results for this sample of infants were compared to a benchmark study conducted in 1993 at the same institution that described breastfeeding outcomes for 45 infants with CHD. Improved outcomes for the 1998-2000 sample are attributed to an active lactation consultation program instituted in 1998. These findings suggest that given support and education necessary to initiate and maintain lactation, mothers can successfully breastfeed their infants with CHD for durations recommended by the Healthy People 2010 initiative.

Adolescent↗

PubMed1984

Longitudinal smooth muscle in pulmonary arteries. Occurrence in congenital heart disease.

In lung biopsy specimens of 19 patients with congenital heart disease and pulmonary hypertension, in addition to the common features of plexogenic arteriopathy, longitudinal smooth muscle cells were found in small pulmonary arteries. These cells were arranged in bundles or layers, particularly in the intima but sometimes within the media or adventitia of the arteries. They often caused severe narrowing of the lumen. Corrective surgery of the cardiac defect was performed in 14 patients. The results suggested that even when these changes are wide-spread and severe, they do not stand in the way of a favourable post-operative course. In one patient who underwent a banding procedure of the pulmonary artery, virtually complete regression of the smooth muscle layers could be demonstrated in a second biopsy, taken 5 years later during a corrective operation.

Adolescent↗

PubMed1989

Prenatal diagnosis and management of congenital heart disease.

Thirty-two fetuses were diagnosed as having congenital heart disease (CHD). The major indications for level II echocardiography other than suspected cardiac abnormalities were fetal malformations, nonimmune hydrops and cardiac arrhythmia. Only three patients had a previous history of fetal CHD. No false-abnormal diagnosis of severe CHD was made. Aortic arch anomalies represented the major diagnostic problem among the six correct but incomplete diagnoses. Sixty-one percent of the fetuses were growth retarded, thus confirming the severity of their CHD. Chromosomal anomalies and extracardiac malformations were associated in 19% and 44% of the fetuses, respectively. Obstetric management and fetal prognosis in cases of extracardiac malformations were greatly influenced by the diagnosis of CHD. The poorest perinatal outcome was associated with heart failure. The only intrauterine deaths occurred in that group, and only one neonate survived. The outcome was more favorable in neonates without other malformations or heart failure. Four of ten (40%) of those neonates survived, while the overall perinatal survival rate was 24%.

Chromosome Aberrations↗