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PubMed1999

Neurologic status of newborns with congenital heart defects before open heart surgery.

UNLABELLED: Controversy exists regarding the integrity of the nervous system in the newborn with a congenital heart defect who must undergo corrective or palliative open heart surgery. Neurodevelopmental sequelae have been primarily attributed to surgical procedures without standardized evaluation of the preoperative neurologic status. OBJECTIVE: To determine whether newborns with congenital heart defects demonstrate abnormalities in neurobehavioral status before surgery. STUDY DESIGN: In this prospective study, a standardized neonatal neurobehavioral assessment and a neurologic examination were conducted independently in a consecutive series of 56 neonates referred to our hospital for investigation of open heart surgery. RESULTS: Neurobehavioral and neurologic abnormalities were documented in greater than half of the cohort and included hypotonia, hypertonia, jitteriness, motor asymmetries, and absent suck. Poor state regulation (62%) and feeding difficulties (34%) also were commonly observed. Furthermore, 3 subjects had seizures, 35.7% were microcephalic, and 12.5% were macrocephalic. The overall likelihood of neurobehavioral abnormalities was not enhanced by indicators of cardiorespiratory compromise. Interestingly, newborns with acyanotic congenital heart defects were more likely to demonstrate neurologic compromise than were those with cyanotic defects. CONCLUSIONS: Findings suggest that the prevalence of neurobehavioral abnormalities before surgery in newborns with congenital heart defects has been underappreciated and would indicate that factors other than intraoperative procedures should be considered in the genesis of brain injury in this population.congenital heart defects, neurologic examination, newborn.

Heart Defects, Congenital↗
PubMedDate not supplied

Prenatal diagnosis and correction of congenital heart defects.

The improvement in heart scanning expertise during the routine obstetric scan has resulted in more congenital heart disease being recognized prenatally. Early diagnosis of severe heart malformation results in the majority of parents choosing to interrupt the pregnancy or allows the mother to give birth in a centre with obstetric and paediatric cardiac facilities.

Aorta, Thoracic↗
PubMed1987

Severity and recurrence risk of congenital heart defects exemplified by atrial septal defect secundum.

It has been reported that the frequency of congenital heart defects (CHD) in children of an affected parent is now three to five times what it was 20 years ago. One conceivable cause of this is that patients with CHD of a severity that would have precluded parenthood before the advent of treatment are now able to (and consequently do) have children once the defect has been corrected. The mean severity of CHD among parents who have it would have thereby increased and this might, in turn, have increased the frequency of CHD in their children. But the latter would occur only if severely affected parents confer a greater risk on their children than parents who are mildly affected. To determine whether this is the case, we investigated 83 probands, each of whom had had an atrial septal defect secundum whose dimensions had been recorded at the time of operation. Of their 166 children, 17 had CHD. The defects of probands with affected children were not found to be significantly different in size from the defects in probands whose children were all normal. Analysis of the hemodynamic data available yielded a similar result. Our findings decisively exclude surgical correction as a significant explanation for the increased recurrence of atrial septal defect.

Canada↗
PubMed1994

Genetic study of congenital heart defects in Northern Ireland (1974-1978).

Congenital heart defects are a major congenital abnormality and are assuming increasing importance. A study was undertaken to estimate the incidence of congenital heart defects in Northern Ireland over a five year period (1974-1978), to determine the age at diagnosis and to assess the risk of recurrence in sibs. An incidence rate of 7.3 per 1000 total births was found. This reduced to 3.1 per 1000 total births if only invasive methods of diagnosis (catheter studies, surgery, or necropsy) were considered. The overall risk of recurrence for sibs (excluding index patients with chromosomal abnormalities and syndromes) was 3.1%. In addition, excluding families with an affected parent and child gave a recurrence risk of 2.6%. By 6 weeks of age 63% of index patients had been diagnosed and this figure had risen to 88% by 1 year. This has important implications for studies which include only congenital heart defects diagnosed in the first year of life. Of 388 patients with a congenital heart defect confirmed by invasive criteria, 96 (24.7%) were found to have an extracardiac abnormality (ECA). Excluding those with epilepsy or mental handicap as the sole ECA left 91 (23.5%) with an ECA. This highlights the importance of looking for other abnormalities in a child with a congenital heart defect. The 388 index patients had 952 sibs of whom 72 (7.6%) had an ECA. Excluding those with minor abnormalities (inguinal hernias, undescended testes) as the sole ECA left 62 (6.5%) with a major ECA. In addition, excluding those with epilepsy or mental handicap as the sole ECA left 51 (5.4%) with a major ECA. Since parents are often reassured after the birth of a child with a congenital heart defect that their risk of having a child with a noncardiac abnormality is no greater than the general population this finding has important implications for genetic counselling.

Abnormalities, Multiple↗
PubMed2003

Do multivitamin or folic acid supplements reduce the risk for congenital heart defects? Evidence and gaps.

Congenital heart defects are among the most common congenital anomalies and are the leading cause of infant death due to congenital anomalies. Except for a few known measures, effective primary prevention is not yet feasible for most heart anomalies. Recent reports have associated the use of multivitamin supplements around the time of conception and during early pregnancy with a reduced risk for heart defects in the offspring. We review and discuss the evidence and suggest a framework for further investigation in this area.

Case-Control Studies↗
PubMed1975

[Use of the platinum-hydrogen test for diagnosis of left-right ejections in congenital heart defects].

In 120 patients with "functional" murmurs and congental cardiac defects with sinistodextral shunts at the aorto-pulmonary level and at that of the cardiac cavities the platinum-hydrogen test was employed in diagnostic probing. In all cases of the shunts this test revealed a distinct ejection of the body from the left to the right, whereas the oxygenonetry of the blood samples failed to indicate any increased oxygenation at a corresponding level, or else it proved questionable in 26 per cent of the interatrial septum defects, in 32 per cent of the interventrical septum defects and in 19 per cent of aorto-pulmonary communications. An analysis of the material showed the method to display a high degree of sensitivity. Its simplicity, reliability and safety for the patient justify recommending the platinum-hydrogen test for a wider application in the clinical practice.

Action Potentials↗
PubMed1981

Lung biopsy with frozen section as a diagnostic aid in patients with congenital heart defects.

In eight patients with congenital heart defects and equivocal preoperative hemodynamic data, lung biopsy with frozen section was performed and quantitative morphometric analysis carried out to help select a palliative or corrective surgical procedure. During catheterization it had been possible in only six of the eight patients to enter the pulmonary artery and obtain pressure measurements; four patients had moderate to severe elevation of pulmonary vascular resistance whereas the other two had only mild elevation of pulmonary arterial pressure, but they were being considered for a Fontan procedure. The lung biopsy tissue was taken inflated and fixed inflated in hot glutaraldehyde. From cryostat sections arterial concentration was assessed relative to alveolar concentration, and the degree of arterial muscularity was assessed by wall thickness and extension of muscle; the more advanced Health-Edwards arterial changes were also evaluated. On the basis of the morphologic assessment, six of the eight patients underwent corrective surgery and two underwent a palliative procedure. Postoperative hemodynamic data available in four of the patients who underwent corrective surgery revealed either a significant reduction in pulmonary vascular resistance or return to a normal level of pulmonary arterial pressure, whereas no change has occurred in the one patient studied who underwent a palliative procedure. Assessment of pulmonary arterial development and structure on lung biopsy with frozen section proved helpful in deciding between a palliative and corrective surgical procedure.

Adolescent↗
PubMed1990

Management of major tracheal hemorrhage after repair of complex congenital heart defects.

Two patients with complex congenital heart defects (a 4-year-old with transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and a 3 1/2-year-old with double-outlet right ventricle, subpulmonary stenosis, and complete atrio-ventricular septal defect) suffered multiple major hemorrhages from the tracheobronchial tree (28 and 7 bleeding events, respectively). Successful management included tracheostomy, sedation and paralysis, systemic hypotension, and systemic hypothermia for a period of seven days. Both patients survived.

Anesthesia↗
PubMed1982

[The prognosis of congenital heart defects in changed].

Congenital heart disease occurs in approximately 8 per mille of all live births. The prognosis for these patients has been radically changed throughout the last decades, both for length and for quality of life. Many patients now have a normal or near to normal life expectancy. This is due not only to better surgical techniques, but improvement of anesthesia, myocardial preservation and postoperative intensive care also have contributed to the astonishing results. The early complete and even in details correct diagnosis and the close cooperation between the local practitioner, pediatrician and the central pediatric cardiac unit is basic for optimal treatment benefit.

Echocardiography↗
PubMed2001

Developmental screening of children diagnosed with congenital heart defects.

Children with congenital heart disease (CHD) are more likely than normal children to have developmental delays. The development of 64 children with CHD less than 6 years old was screened with the Denver II. Thirty-five of the 64 children had CHD that required surgical or catheter intervention. These 35 children were significantly less likely than other children with CHD to be normal on developmental screening (46% vs 86%, respectively). Thirty-four percent of children with more severe CHD were referred for early intervention. As research shows the efficacy of early intervention, results indicate the need for early developmental evaluation of children with CHD of hemodynamic significance.

Analysis of Variance↗
PubMed2002

[Hemorheology and morphofunctional characteristics of blood cells in children with congenital heart defects].

Patients with congenital heart diseases (CHD) aged 4 months to 12 years, hospitalized for surgical treatment, were examined. Complex viscosity of the blood and the constituents of this parameter (dynamic viscosity and elastic component) were evaluated in the range of shift strain corresponding to the range of shift velocities 0.37-500 s-1 at a frequency of 2 Hz. The threshold blood fluidity was estimated for evaluating the conditions under which erythrocyte disaggregation begins. The relationship between blood rheology and morphofunctional characteristics of blood cells was evaluated. In accordance with the findings, the patients were divided into 3 groups with different suspension stability of the blood. In group 1 the threshold fluidity approximated the norm. In group 2 with normal blood rheology the shifts were compensated and regulation of rheology was in general intact. In group 3 the hematocrit values, mean erythrocyte volume, mean concentration of hemoglobin in erythrocyte, leukocyte counts, and complex viscosity of the blood were the highest, while the suspension stability was the lowest, which indicates depletion of the adaptive potential of the organism. Hence, blood rheology in patients with CHD differs by the type of regulation, mechanisms and compensation of changes, and depend largely on the mean erythrocyte volume, mean hemoglobin concentration in erythrocytes, and leukocyte counts.

Blood Cell Count↗