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PubMed1985

The pattern and frequency of congenital heart disease among blacks.

The types of cardiac malformations in 804 black patients of all ages in whom the diagnosis was confirmed by cardiac catheterization, surgery or autopsy are reported. The most frequent anomalies were ventricular septal defect, patent ductus arteriosus and tetralogy of Fallot. Among infants under 1 year of age, complete transposition of the great vessels accounted for the third-largest group of malformations. While pulmonary venous anomalies were extremely rare and hypoplastic left heart defects rare in the neonate, it is not considered that a racial predilection for differences in the frequency of various cardiac malformations exists. The frequency of coarctation of the aorta in the group as a whole was not lower than that found in studies among whites. Among black patients of all ages with heart disease (studied in a hospital environment) congenital cardiac malformations ranked as the second most common form of heart disease with a frequency of 26%. Among children aged 15 years or less, congenital heart disease ranked first with a frequency of 53%. It is suggested that a diagnosis of congenital heart disease is not made in the majority of blacks born with such malformations.

Adolescent↗
PubMed1985

[Fetal echocardiography: diagnosis of congenital cardiomyopathies in a population at risk].

2-Dimensional and M-mode Echocardiography were performed in 142 fetuses at specific risk for congenital heart disease between 16 and 40 weeks of gestational age. In 105 cases the diagnosis was checked at delivery; prevalence of cardiac malformations was 5.7%. In 5 cases out of 7 the malformation was correctly diagnosed at Echocardiographic examination: ectopia cordis of thoracic type with tetralogy of Fallot, ectopia cordis of abdominal type with normal intracardiac anatomy, atrio-ventricular canal, left atrial membrane, single heart in jointed twins. There were 3 diagnostic errors: two false negative (a case of pulmonary atresia and a case of univentricular heart) and one false positive (a case of suspected transposition of the great vessels in a normal heart). The sensitivity of the test was 71.4%, the specificity was 98.9%, the predictive value of the positive test was 83.3%. M-mode Echo was particularly useful in cases of arrhythmias. 3 cases of benign rhythm disorders were identified: 2 cases of conducted and blocked atrial ectopic beats and one case of transient 2:1 A-V block. In 5 cases there was a wide variability of the sinus rhythm. 2-D Echocardiogram seems a reliable method for the diagnosis of fetal heart diseases in a population at risk. Its clinical usefulness to guide therapeutic decisions in our population was limited by the coexistence of multiple malformations.

Arrhythmias, Cardiac↗
PubMed1979

Pulsatile perfusion versus conventional high-flow nonpulsatile perfusion for rapid core cooling and rewarming of infants for circulatory arrest in cardiac operation.

Thirty consecutive infants undergoing hypothermia and circulatory arrest for repair of ventricular septal defect, transposition of the great vessels, or atrioventricular canal defects were alternately selected for conventional high flow nonpulsatile perfusion or pulsatile perfusion during core cooling and rewarming. All received morphine anesthesia, 30 mg/kg of Solu-Medrol, and 10 to 15 mcg/kg of phentolamine. Those receiving nonpulsatile flow were perfused at a rate of 160 to 180 cc/kg/min with a roller pump and oxygenator with arterial pressure of 50 to 55 mm Hg. In the pulsatile flow group, a roller pump and oxygenator were used, and an especially constructed Datascope PAD (pulsatile assist device) was interposed in the arterial line to provide pulsatile perfusion with 75/40 mm Hg pressure at slightly reduced flow (150 cc/kg/min). The average rectal, esophageal, and tympanic membrane temperatures were reduced to approximately 16 degrees C prior to circulatory arrest. Following repair, perfusion was resumed until these temperatures returned to 37 degrees C. Cooling and rewarming were enhanced by pulsatile perfusion, with over 30% reduction in total pump time. Additionally, the larger patients in the pulsatile group cooled almost as rapidly as the smaller. The rates of decline and subsequent rise of rectal, esophageal, and tympanic membrane temperatures were equal in the pulsatile group, but the rectal temperature lagged far behind in the nonpulsatile group. Urine production during bypass was 100% greater in the pulsatile group. The plasma free hemoglobin was similar in both groups. The average postrewarming pH was 7.31 in the nonpulsatile group and 7.42 in the pulsatile group. Infants receiving pulsatile flow awakened more quickly, were more alert, and required less postoperative mechanical ventilation. We suggest that pulsatile perfusion for core cooling and rewarming of infants is safe and is more rapid and physiological than conventional high-flow nonpulsatile perfusion.

Acid-Base Equilibrium↗
PubMed1983

[Outcome and monitoring of children following cardiac surgery].

The spectacular advances in the surgery for congenital heart disease in children and infants have led to the appearance of a new pathology, late complications which warrant a systematic cardiological surveillance, to allow the patient to lead as normal a life as possible, compatible with the potential sequelae of this type of surgery. These complications arise essentially after ventricular open heart surgery, especially for tetralogy of Fallot and for interventricular communications. The ventricular function may be altered, even if the functional status appears to be good, so it must be systematically monitored, in particular by isotope methods. Arrhythmias and conduction disorders require special attention as they are often delayed in their appearance and can be serious, even causing sudden death, hence the value of electrophysiological investigations and, more especially, Holter monitoring and stress tests. The problems are even more complex when the operation was only aimed at a physiological correction, especially after operations for complete transposition of the great vessels and so-called "ortho-terminal" operations for tricuspid atresia or a single ventricle. In these cases, as well as ventricular dysfunction, we also see arrhythmias which can sometimes be severe, requiring anti-arrhythmic treatment and sometimes electro-systolic pacing.

Cardiac Surgical Procedures↗
PubMed1984

Sacral meningocele with conotruncal heart defects: a possible autosomal recessive trait.

Three of four siblings had sacral meningocele with subsequent development of hydrocephaly; two died during the neonatal period due to conotruncal heart defects (transposition of the great vessels and truncus arteriosus type I, respectively). An in utero diagnosis of open neural tube defect was made on the third sibling; persistent slightly elevated alpha-fetoprotein levels in amniotic fluid and increased number of rapidly adhering cells in short term amniotic cell culture were found. The unique combination of sacral meningocele and conotruncal malformations in this sibship suggests a new autosomal recessive condition. It also emphasizes the heterogeneity of both the open neural tube defects and congenital heart defects. Awareness of this variant is necessary in regard to the 25% recurrence risk instead of the 3% to 5% recurrence risk given for both congenital heart defects and open neural tube defects as isolated anomalies. The difficult prenatal diagnosis for the small neural tube defect should be appreciated.

Female↗
PubMedDate not supplied

[Right aortic arch associated with congenital heart defects].

The author's experience in the association of right aortic arch and congenital cardiac malformations is reported. We found 56 cases out of 1200 congenital cardiac malformations which constitutes 4.6%. In the classification of Knight and Edwards, type I right aortic arch was the most frequent (96%) and it was associated principally with Tetralogy of Fallot (46%), transposition of the great vessels (14.3%), truncus arteriosus (12%), tricuspid atresia (10.7%) and complex cardiac malformations associated with the syndrome of visceral heterotaxy (10.7%). In type II and type III we found only one case of each and these were associated with pulmonary valvular stenosis, atrial septal defect and ventricular septal defect. It is noted that the right aortic arch has never been associated with total anomalous pulmonary venous return nor with mitral or aortic valvular malformations.

Aorta, Thoracic↗
PubMed1983

[Outcome of patients undergoing open-heart surgery for congenital heart disease in early childhood].

Corrective surgery for congenital heart disease is performed on progressively younger children in order to restore normal haemodynamics during infancy and to avoid the disadvantages of palliative operations. The authors consider the long-term outcome of children who have been operated for complete transposition of the great vessels--by physiological correction or by anatomical correction--for tetralogy of Fallot, interventricular communication, atrio-ventricular channel and aortic stenosis. The sequelae and complications of these various operations are reviewed and the authors stress, in particular, the risk of delayed arrhythmias and disturbances of ventricular function. The risk of delayed complications justifies long-term follow-up of all these patients, even those who are leading a normal life, i.e. the majority of them.

Cardiac Surgical Procedures↗
PubMed1983

Permanent endocardial pacing in pediatric patients.

Improved technology with smaller pulse generators and passive and active fixation endocardial lead systems has the potential for facilitating safe, reliable pacing in pediatric patients. Of 18 pediatric patients (mean age 11.1 years) undergoing permanent transvenous cardiac pacing during the period April, 1977, to January, 1981, two (11%) required reoperation during a mean follow-up of 18.8 months. Satisfactory stable pacing was maintained even in patients with dual-chamber pacing systems and in patients with transposition of the great vessels in whom a lead was placed in a morphologically left atrium. This reoperation rate of 11% contrasts with a reoperation rate of 32% in 22 pediatric patients (mean age 8.7 years) who underwent placement of ventricular-demand epicardial pacing systems during the same period. In our experience, transvenous endocardial pacing is the preferred route in pediatric patients because of the improved lead system survival and the potential for atrial or atrioventricular sequential pacing.

Adolescent↗
PubMed1976

Congenital malformations in infants of diabetic mothers.

In a series of 701 infants born to diabetic women between 1950 and 1974 ,57(8-1 percent) had congential malformations which is a rate three to four timeshigher than in the normal populations of Birmingham.. In 26 cases(3-8 per cent) the malformations were fatal and accounted for 26 per cent of the perinatal mortality in the series. Central nervous system, cardiovascular and skeletal abnormalities were equally common and there was a high incidence of anencephalus, spina bifida, transposition of the great vessels and sacral dysgenesis...

Abnormalities, Drug-Induced↗
PubMed1975

[Levocardia with ventricular inversion and a single atrium in normal position].

A 12-year child had complete heart block, an abdominal situs inversus with laevocardia. Further investigation demonstrated in addition an inferior vena cava ending into an azygos vein, a shunt at the atrial level and a pulmonary valve stenosis. At operation there was a lone atrium in normal position, a ventricular inversion without transposition of the great vessels. In spite of a corrective operation of the Mustard type, the child died the following day through haemorrhage and low output.

Autopsy↗
PubMedDate not supplied

Aneurysm of the membranous ventricular septum producing subpulmonic outflow tract obstruction.

The authors report the case of a 31-year-old asymptomatic male who, following investigations for cardiac murmur, was found to have congenitally corrected (status solitus of the atria, left looping of the ventricles, leftward aorta in relation to the pulmonary artery [S,L,L]) transposition of the great vessels with significant right-sided ventricular outflow tract obstruction due to a large aneurysm of the membranous ventricular septum. Diagnosis was made with transesophageal echocardiography and confirmed during corrective surgery. The authors review the literature with regard to aneurysms of the membranous ventricular septum and their association with congenital heart disease, and they discuss the use of noninvasive tests aiding the diagnosis.

Adult↗
PubMed1993

[Study of cor triatriatum. Apropos of 6 cases].

The authors report 6 cases of triatrial heart with various and non-specific manifestations and attribute the difficulty of its clinical identification above all to the frequency of associated lesions, which were present in 5 of the 6 cases reported here. An atrial septal defect and left superior vena cava were each seen twice while a ventricular septal defect, sub-valvular aortic stenosis, mitral insufficiency and transposition of the great vessels were each seen once. It was routine echocardiography which led to the discovery of triatrial heart in each of these 6 cases. The value of this investigation in the positive diagnosis of triatrial heart proved, in these patients, to be greater than that of cardiac catheterisation.

Adolescent↗
PubMed1996

[Congenital heart diseases surgically treated in childhood. Long-term results].

Surgery for hereditary cardiopathies has made much progress over the last 25 years. For left/right ventricular shunts, mortality is 5% and quality of life is satisfactory in 85% at five years. All interventricular communications with major pulmonary hypertension should be operated during the first year of life. When surgery is indicated for atrial left/right shunt the operation should be done before the age of 4 or 5 years. Results for Fallot's tetralogy are now excellent with an actuarial survival of more than 30 years in 86% of simple forms after curative surgery. Complete repair should be done early (12 to 18 months) to reduce dysrhythmia and right ventricular hypertrophy. Corrective atrial surgery for transposition of the great vessels, a totally physiological operation, has been replaced by anatomic correction, improving prognosis to more than 90% survival and only 10% reoperation rate. Surgery for coarctation of the aorta gives very good results modulated by the risk of recurrence, especially when performed early (before 6 months). Medical-surgical management of hereditary cardiopathies begins at birth. Surgery for all operable malformations should be performed before the end of the second year of life, allowing for nearly normal life, both at home and at school.

Child↗
PubMed1997

[Magnetic resonance of congenital cardiopathies].

OBJECTIVE: The objective of this study was to determine the usefulness of magnetic resonance in the diagnosis and follow-up in congenital heart disease. PATIENTS AND METHODS: Between 1992 and 1994, we have evaluated 250 patients (between 1 day-22 years of age) with congenital heart disease. We used a Philips-Gyroscan 0.5 Tesla set-up and the studies were made with ECG-gated multislice sequences with the time of repetition adapted to the cardiac frequency. The slice thicknesses were 3-10 mm and the field of view was 100-400 mm. Three axes were used: axial, oblique coronal and 30-60 degrees oblique sagittal. Three groups were made: A) Patients in whom MR imaging was the elected technique for establishment of the diagnosis; B) Patients were MR imaging was an alternative technique; and C) Patients were MR imaging was complementary to other techniques. All patients underwent a Doppler echocardiographic study or an angiocardiogram. RESULTS: Group A) One hundred and fifty-one patients were included with the following conditions: Eighty-two with coarctation of the aorta or aortic arch interruption, 12 with vascular ring, 5 with truncus arteriosus in order to evaluate the pulmonary branches, 28 cases operated for Fallot or transposition of the great vessels for the evaluation of the pulmonary branches, 16 with single ventricle for the evaluation of the bulboventricular foramen, 5 with complex congenital heart disease and 3 with pericardial agenesia-herniation. Group B) Sixty-five patients were included in this group with the following diagnoses: Ten with aorto-pulmonary colateral arteries, 13 with anomalous pulmonary drainage of the systemic veins, 38 with tronco-conal anomalies and 4 with supravalvular aortic stenosis. Group C) Thirty-four patients with the following conditions were included in this group: Ten with common atrioventricular septal defects, 6 with tricuspid atresia, 6 with atrioventricular and ventriculoarterial discordance and 12 with other anomalies. CONCLUSIONS: MR imaging can replace other techniques in the diagnosis and follow-up of some congenital heart diseases and in a non-invasive way.

Child↗
PubMed1976

Total correction of double-outlet ventricle with concordant atrioventricular relation, dextrocardia, pulmonary stenosis, and situs inversus.

A case of successful surgical correction of double-outlet right ventricle with concordant atrioventricular relation, pulmonic stenosis, and situs inversus is described. Transposition of the great vessels was not present. Intracardiac repair with a Teflon tunnel conduit gave an excellent result. Proper resection of the subpulmonic obstructing fibromuscular band required no patch reconstruction of the outflow tract.

Cardiopulmonary Bypass↗