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PubMed2006

Long-term neuromotor outcome at school entry of infants with congenital heart defects requiring open-heart surgery.

OBJECTIVES: Increased survival in infants with complex congenital heart defects (CHDs) has resulted in greater focus on morbidity. This prospective study was conducted to investigate whether or not neuromotor impairments evident acutely persist long-term. STUDY DESIGN: A total of 94 infants with CHD were reassessed at school entry (age 64.2 +/- 11.3 months) by blinded examiners using the Peabody Developmental Motor Scale and neurologic examination. RESULTS: Neurologic abnormalities were noted in 28.4% of the subjects, with hypotonia and developmental delay most commonly observed. Microcephaly was documented in 15.4% of subjects, with head circumference >50th percentile in only 14.1%. Mean gross and fine motor quotients were shifted downward (82.7 +/- 12.3 and 86.2 +/- 16.3, respectively), and gross and fine motor delays were prevalent (49.4% and 39.0%, respectively). Severe disability was rare (<5%). Predictors of neurologic impairment included type of surgery (palliative/corrective), abnormal postoperative neurologic examination, and presence of microcephaly at initial surgery. Gross motor delay was more likely if palliated. Deep hypothermic circulatory arrest time, palliative surgery, microcephaly, and number of hospitalizations were associated with fine motor delay. CONCLUSIONS: Neurologic impairments and motor delay are common in survivors of open-heart surgery, persisting to age of school entry. Early indicators of medical complications and acute neurologic compromise are associated with increased risk. Early identification and intervention may optimize performance in those at risk.

Cardiac Surgical Procedures↗
PubMed2000

Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery.

BACKGROUND: Neurodevelopmental disabilities in children with congenital heart defects (CHDs) have been primarily attributed to intraoperative events without consideration of preoperative and postoperative factors. OBJECTIVE: To describe the preoperative and postoperative neurodevelopmental status of newborns and infants with CHDs. STUDY DESIGN: One hundred thirty-one children (56 newborns and 75 infants) were evaluated before and after surgery by using standardized neurobehavioral (newborn) and motor assessments (infant) and neurologic examinations. RESULTS: In newborns, neurobehavioral abnormalities were documented in >50% before surgery, with abnormalities persisting in most after surgery. In infants, neurodevelopmental abnormalities were observed in 38% before surgery. There was a significant association between preoperative and postoperative neurodevelopmental status, with status remaining unchanged in most. Newborns with acyanotic heart lesions were more likely to demonstrate neurologic compromise than those with cyanotic defects. For infants, arterial oxygen saturations <85% were significantly associated with an abnormality. There was a trend for a longer circulatory arrest time to be associated with greater risk for neurologic sequelae in newborns, whereas prolonged cardiopulmonary bypass was an important risk factor for infants. CONCLUSIONS: Neurodevelopmental abnormalities are common in young infants with CHDs and are often present before open heart surgery. These developmental concerns are clinically underappreciated. Early systematic developmental screening may be warranted in this population of interest.

Cardiac Surgical Procedures↗
PubMed1996

Radiofrequency catheter ablation of right ventricular tachycardia late after repair of congenital heart defects.

BACKGROUND: Ventricular arrhythmias after repair of congenital heart defects are a common finding and possibly contribute to sudden death in these patients. Optimal antiarrhythmic management has not yet been defined. METHODS AND RESULTS: The study population consisted of 16 patients in whom ventricular arrhythmias occurred 11 to 42 years after complete surgical repair of congenital heart defects. Fifteen patients had a history of symptomatic sustained or nonsustained ventricular tachycardia, and 1 had frequent nonsustained ventricular tachycardia. The diagnostic mapping procedure to identify the origin of the arrhythmia included pace mapping during sinus rhythm, activation mapping, and pacing interventions during ventricular tachycardia. Catheter ablation was carried out by means of radiofrequency energy in the temperature-controlled mode. The follow-up period was 6 to 33 months (mean, 16 months). A right ventricular origin of the tachycardia in the surgically corrected area could be determined in all patients. Catheter ablation was carried out without complications. Immediate noninducibility was achieved in 15 of the 16 patients. One patient in whom the tachycardia was again inducible at repeat stimulation 1 week later was successfully treated with amiodarone. Eleven patients were taken off antiarrhythmic drugs. During follow-up, none of them had a recurrence of the tachycardia that had been ablated. CONCLUSIONS: In patients with symptomatic or frequent ventricular tachycardia late after complete surgical repair of congenital heart defects, catheter ablation by means of radiofrequency energy is feasible and safe and thus might be taken into consideration for these patients. Short-term follow-up results are promising.

Adolescent↗
PubMed1992

[Congenital heart defects also in adulthood?].

Congenital heart diseases that escape detection are a rarity in adulthood (less than 1%). The number of patients with such abnormalities operated on during childhood or adolescence is, in contrast, on the increase. The most common heart abnormalities diagnosed for the first time in adulthood are atrial septal and ventricular septal defects, but anomalies of the left ventricular outflow tract or coarctation of the aorta is sometimes also detected only in adulthood on the occasion of an investigation of hypertension. In addition to the findings at auscultation, clinical pictures, ECG and chest X-ray, echocardiography is becoming ever decisive for the diagnostic work-up. Further, the extent of the haemodynamic changes can now be determined non-invasively, and may thus tip the balance when deciding for and against an operation.

Adult↗
PubMed1987

Using a national register for the epidemiological study of congenital heart defects.

Data validity is a fundamental problem in epidemiology. An objective of birth defect registers is the collection of high quality data, often from a number of sources, for use in epidemiological research. This paper examines the use of data from a national register in New Zealand in the study of congenital heart defects. The prevalence rates of congenital heart defects are shown to depend on the definition of the terms employed, the methods and completeness of the case ascertainment, the correctness of the diagnoses, the nature and size of the population under study, and the duration of followup. Recommendations are made for increasing the utility of this register in the study of this major group of malformations.

Autopsy↗
PubMed1986

[Doppler echocardiographic evaluation of pressure in the pulmonary artery in children with congenital heart defects].

In children with congenital heart disease serial noninvasive assessment of the pulmonary vascular bed is desirable in order to determine the appropriate timing of cardiac catheterization and corrective surgery. To assess the value of pulmonary Doppler echocardiography for the estimation of pulmonary arterial pressure we correlated the rightsided systolic time intervals (preejection period, acceleration period, ejection period) derived from pulmonary artery Doppler traces with catheterization data (systolic, mean and diastolic pulmonary arterial pressure, total and vascular pulmonary resistances). 62 children aged from 1 month to 15 years suffering from congenital heart disease (n = 52), from rheumatic heart disease (n = 4), from cardiomyopathy (n = 5) or from primary pulmonary hypertension (n = 1) were investigated. The major finding was a highly significant correlation between the acceleration period and the systolic pulmonary arterial pressure (r = 0.79; p less than or equal to 0.0001). However the acceleration period could not be used for prediction of pulmonary arterial pressure in children with poor myocardial contractility.

Adolescent↗
PubMed1998

Congenital heart defect in sibs with discordant karyotypes.

Congenital heart defects (CHDs) are genetically heterogeneous, associated with a variety of genetic conditions. Familial aggregation of CHD in patients with and without Down syndrome is rare. We report on the occurrence of concordant CHD in three sets of sibs with discordant karyotypes. In the first family, atrioventricular canal (AVC) was diagnosed in a chromosomally normal child and in his brother with Down syndrome. In the second family, AVC was associated with trisomy 21 in one sib and with trisomy 18 in the other. In the third family, tetralogy of Fallot was present in one patient with Down syndrome and in his nonsyndromic sister. Although the genetic heterogeneity of Down and non-Down CHD is not disputed, a susceptibility to both euploid and aneuploid CHDs could exist, and common predisposing factors could play a role in both conditions.

Down Syndrome↗
PubMed1992

Lung perfusion scans in patients with congenital heart defects.

In 63 patients with various congenital heart defects, lung perfusion was evaluated with technetium-99mm macroaggregated albumin. Right lung perfusion abnormalities were documented in 34 patients (54%). A particularly high incidence occurred in patients who had undergone a systemic to pulmonary artery shunt operation as an initial palliative procedure or who had had right ventricular outflow reconstruction and in those with bilateral pulmonary artery stenosis. Serial studies were helpful in evaluating the functional results of different transcatheter interventions for optimizing pulmonary blood flow. The quantitative relative perfusion radionuclide method was a more sensitive means of detecting cases of abnormal lung perfusion than was chest radiology.

Cardiac Catheterization↗
PubMed1975

The association of scoliosis and congenital heart defects.

The effects of cardiac surgery on patients with congenital heart defects and the subsequent development of scoliosis were studied. A group of 998 patients with congenital heart defects who were less than sixteen years old were operated on at the Mayo Clinic during the ten-year period 1950 through 1959. Standing roentgenograms of the spine were made of 377 of the patients ten years or more after surgery. The ages of the patients at follow-up ranged from ten years and seven months to thirty-five years and three months, with a mean age of twenty-one years and four months. The average length of follow-up was fourteen years and eleven months. Of the 377 patients, thirty-two (8.5 per cent) had curves greater than 20 degrees. The female:male ratio of patients with congenital heart defects was 1:1, whereas of those who developed scoliosis it was 5:3. There was no correlation between scoliosis and the following: patient's sex, cardiac abnormality, size or side of the heart, side of the aortic arch, presence of cyanosis, age at surgery, number and type of surgical incisions, number and side of ribs removed, or number and type of surgical procedures.

Adolescent↗
PubMed1990

Comparative roles of intraoperative epicardial and early postoperative transthoracic echocardiography in the assessment of surgical repair of congenital heart defects.

In 94 consecutive patients undergoing surgical repair of congenital heart defects the results of intraoperative (after cardiopulmonary bypass) epicardial two-dimensional and Doppler color flow imaging were compared with those of sequential transthoracic echocardiography performed within 24 h of surgery and again before hospital discharge to define the precise role of intraoperative imaging. In 6 of 7 patients with a residual defect requiring immediate surgical revision, intraoperative imaging correctly identified the defect; spectral Doppler imaging underestimated or did not identify a residual outflow tract gradient in 17 patients. Left atrioventricular (AV) valve regurgitation after repair of complete AV septal defect was underestimated in three patients. Although intraoperative documentation of good ventricular function was usually associated with a good outcome, in three patients poor systemic ventricular function after cardiopulmonary bypass was not associated with early mortality. A minor degree of shunting around the patch was a common finding on epicardial and early postoperative imaging and persisted at the time of hospital discharge in 17 of 46 patients who had undergone patch closure of a ventricular septal defect as part of the surgical procedure. Additional trabecular septal defects were missed on color flow imaging after cardiopulmonary bypass in three patients, one of whom required subsequent reoperation. Although intraoperative two-dimensional and color flow imaging permitted the recognition of the majority of residual defects requiring immediate revision, residual outflow obstruction or AV valve regurgitation was usually underestimated.

Child↗
PubMed1994

Paternal age and the risk of congenital heart defects.

The effect of paternal age on the risk of birth defects among offspring is less well studied than the effect of maternal age, with few comprehensive epidemiologic studies having been conducted. Advanced paternal age has been shown to be associated with an increase in new dominant mutations that result in particular congenital anomalies. The relationship between paternal age and more common birth defects, for example, cardiac defects, has not been as extensively evaluated. Therefore, a total of 4,110 cases of congenital heart defects was identified from the British Columbia Health Surveillance Registry. Matched controls were obtained from the birth files of British Columbia for the years 1952-1973. Prevalence odds ratios for paternal age, adjusted for maternal age and other factors, were estimated for 8 cardiac defect groups. A suggestive general pattern of increasing risk with increasing age among cases (excluding chromosomal anomalies) relative to controls was found for ventricular septal defects (VSD), atrial septal defects (ASD), and patent ductus arteriosus (PDA). In addition, an increased risk among men younger than 20 yr was found for VSD and ASD. These findings are consistent with the results of some previous epidemiologic studies. Based on the results of this study it is estimated that for cardiac defects such as VSD, approximately 5% of cases may be due to advanced paternal age (> 35 yr), possibly through dominant mutations.

Adult↗
PubMed1990

[Points of departure in sports counseling of children with congenital heart defects].

When counseling children with congenital heart disease who want to participate in sports, it is, generally speaking, preferable to choose a dynamic sport rather than a static one. Also, the degree of intensity is of major importance. In this connection it is useful to differentiate between recreation-oriented and achievement-oriented participation in sports. The extra risks for a child with congenital heart disease are progression of the severely of the anomaly, rhythm-disturbances, sudden death, and complications due to medication. For the majority of these children the recommendation will be a strictly individual one, based on the (exercise)electrocardiogram, the 24-hours-ECG monitoring, and the echo-Doppler-cardiogram. Invasive techniques are usually unnecessary.

Arrhythmias, Cardiac↗
PubMed1994

Prevention of lung injury during open heart operations for congenital heart defects.

To elucidate free radical-induced lung injury associated with open heart operations for congenital heart defects, we studied 23 such patients. Maximum plasma chemiluminescence level (a marker of peroxylipids) in patients with pulmonary hypertension (n = 8) was higher than in patients with cyanotic disease (n = 8) (1,115.4 +/- 189.9 versus 728.8 +/- 48.3 counts; p < 0.05). There was a significant correlation between the maximum chemiluminescence level and preoperative pulmonary to systemic arterial pressure ratio (r = 0.929; p < 0.05). To investigate the effect of allogeneic leukocytes, we compared pulmonary hypertensive patients without allogeneic leukocyte transfusion during operation (n = 7) with the group with pulmonary hypertension. Both maximum chemiluminescence level during bypass (712.4 +/- 24.9 versus 1,115.4 +/- 189.9 counts; p < 0.05) and percent decrease in pulmonary arterial pressure after bypass (44.7% +/- 6.2% versus 28.2% +/- 4.5%; p < 0.05) were significantly improved, suggesting that depletion of leukocytes decreased the lung injury induced by free radical reaction.

Adolescent↗