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PubMed1992

Current method of repair of truncus arteriosus.

Surgical treatment of truncus arteriosus has been demonstrated to be optimized when approached in early infancy. In the past, the materials available, namely the 12-mm porcine valve Dacron conduit, restricted the size of the patient in whom early repair could be performed with increasing risk associated with decreasing age and size. The availability of homografts for reconstruction of right ventricular pulmonary artery continuity has allowed repair even in the neonatal period. Two hundred forty-four patients with truncus arteriosus have been operated on at the University of California San Francisco since 1975. In the past 5 years, 49 have been repaired using homograft reconstruction. The method of repair differs markedly from that of Dacron conduits, and specific points concerning homograft insertion including orientation, method of proximal anastomosis, and sizing are discussed in detail and the difference in insertion from the Dacron bovine heterograft conduit are discussed.

Age Factors↗
PubMed1993

Semilunar valve replacement with a cylindrical valve.

A cylindrical valve was designed to prevent regurgitation of the semilunar valve. The valve is made of a sheet of polytetrafluoroethylene (PTFE) or porcine pericardium, and has three cusps and three commissures. The diameter of the valve is equal to the height of the cusps. We have used these valves in pulmonary stenosis after Jatene's operation and total correction of tetralogy of Fallot, and for truncal valve regurgitation. Regurgitation was trivial on color Doppler echocardiography in all cases. Advantages in comparison with the implantation of commercially available artificial valves include the ability to insert a larger size and no compression of the valve ring when closing the sternum. Outflow tract obstruction does not occur even when the valve is implanted in a small infant. In the present report, we describe this simple technique.

Animals↗
PubMedDate not supplied

Severe truncal valve stenosis: diagnosis and management.

Severe truncal valve stenosis, due to dysplastic valve, may be found in a small number of patients with truncus arteriosus. Its presence complicates the surgical management of this anomaly due to lack of optimal therapeutic options and associated ventricular dysfunction. We report a case of truncal root replacement using a homograft for the management of such condition, and review the literature related to its management and outcome.

Aortic Valve Stenosis↗
PubMed1986

Pulsed Doppler echocardiography in patients with aorto-pulmonary connection.

The pulsed Doppler principle enables us to study the bloodflow velocity at specific points in the heart and great vessels. In such aorto-pulmonary connections as patent ductus arteriosus, aorto-pulmonary window and truncus arteriosus the bloodflow velocity patterns are typical of the malformation. Based on knowledge of these abnormal flow patterns the diagnoses of these malformations can be made.

Aortopulmonary Septal Defect↗
PubMed1984

Dependence of thymus development on derivatives of the neural crest.

Elimination of limited areas of the cephalic neural crest in stage 9 or 10 chick embryos markedly reduced the size of the thymus gland or resulted in its absence. Small thymic lobes contained both thymocytes and epithelial cells but showed delayed development. Parathyroid and thyroid glands sometimes were reduced in size or missing from the normal location on one or both sides. Heart defects were consistently present. Thymus development may depend on direct interaction of mesenchymal derivatives of the neural crest with pharyngeal epithelium. Multiple defects, such as the Di George syndrome, may result from failure of neural crest derivatives to migrate and interact properly.

Animals↗
PubMed1987

Plasma renin activity in infants with congenital heart disease.

Plasma renin activity was estimated in 11 infants with severe congestive heart failure. The infants had congenital heart disease with left to right shunts and were receiving diuretic treatment. Plasma renin activity was measured by radioimmunoassay of generated concentrations of angiotensin I. The mean (SD) plasma renin activity was 84 (21) ng angiotensin I/ml/hour, which is considerably above normal infant values. A hyperactive renin-angiotensin system may be detrimental in these patients. Angiotensin converting enzyme inhibitors may be of value in treating infants with severe congestive heart failure.

Double Outlet Right Ventricle↗
PubMed2003

Common arterial trunk in the fetus: characteristics, associations, and outcome in a multicentre series of 23 cases.

OBJECTIVE: To assess the accuracy of prenatal diagnosis, the incidence of extracardiac and chromosomal anomalies, and the perinatal outcome in a population of fetuses with common arterial trunk (CAT). DESIGN: Observational study of 23 fetuses from three referral centres with a confirmed diagnosis of CAT. All underwent fetal echocardiography, detailed anatomical scanning, and karyotyping. In 19 cases, FISH analysis was done to detect 22q11 microdeletion. The following variables were evaluated: gestational age at diagnosis, anatomical variants of the CAT, presence of extracardiac and chromosomal anomalies, pregnancy, and fetal-neonatal outcome. Necropsy reports and postnatal files were available for confirmation of the prenatal diagnosis in all cases. RESULTS: The prenatal diagnosis proved correct in 23 of 24 cases, the last being pulmonary atresia with ventricular septal defect (PAVSD). A second cardiovascular anomaly was present in eight cases (34.8%); extracardiac anomalies were found in 10 (43.4%). FISH analysis showed 22q11 microdeletion in six of 19 cases (31.6%). Outcomes were as follows: eight terminations of pregnancy (34.8%), two intrauterine deaths (8.7%), five postnatal deaths (before or after surgery) (21.7%); the remaining eight neonates (34.8%) are alive and thriving after surgery (six) or awaiting surgery (two). CONCLUSIONS: CAT can be reliably diagnosed and characterised in prenatal life, although differentiation from PAVSD may be challenging. The association with chromosomal anomalies is consistent (8.7%), but there is a higher risk of 22q11 microdeletion (31.6%), in agreement with postnatal studies. The relatively poor survival rate (34.8%) reflects the high rate of terminations and the unfavourable cardiac anatomy in some cases.

Aorta, Thoracic↗
PubMed1976

Anatomical study of truncus arteriousus communis with embryological and surgical considerations.

Twelve specimens of truncus arteriosus communis have been studied anatomically, with special reference to the conal anatomy and to the associated cardiac anomalies which can create additional problems if surgical repair is planned. A wide spectrum of conal morphology has been observed, suggesting that differential conal absorption is a developmental characteristic of truncus arteriousus as well as of transposition complexes. The invariable absence of septation of the ventricular infundibula and semilunar valves, in spite of the variable anatomy of the free wall of the conus, indicates that all types of truncus arteriosus, ontogenetically, should be considered as a single undivided conotruncus. Various types of ventircular septal defect were found: (a) ventricular septal defect with absent crista, in which no remnants of conal septum are present; (b) supracristal ventricular septal defect, in which vestigial conal septum is seen in front of the membranous septum; (c) bulloventricular foramen, associated with univentricular origin of the truncus from the right ventricle. Frequent associated anomalies are underdevelopment of the aortic arch, truncal valve malformations, and obstructive ventricular septal defect. The AV conduction system studied in one case showed an arrangement similar to Fallot's tetralogy with the His bundle and the left bundle-branch in a safe position behind the posteroinferior rim of the defect. The postoperative fate of the frequently abnormal truncal valve and the theoretical indications for total repair for Type IV truncus are also discussed.

Adult↗
PubMed1979

Truncus arteriosus communis with intact ventricular septum.

This is the first documented case of truncus arteriosus communis with intact ventricular septum in which extensive clinical, haemodynamic, angiographic, and pathological data are available. Angiography suggested the presence of two discrete semilunar valves but necropsy showed a basically single semilunar valve. This case fills a gap in the spectrum of aorticopulmonary, truncal, and infundibular septal defects, and reinforces the belief that the essence of truncus arteriosus communis is a single semilunar valve, common to both ventricles, which need not be associated with the defects in the adjacent parts of the structural continuum.

Angiocardiography↗
PubMed1982

Two dimensional echocardiographic assessment of communications between ascending aorta and pulmonary trunk or individual pulmonary arteries.

The value of two dimensional echocardiography in identifying communications between the ascending aorta and pulmonary trunk or individual pulmonary arteries was assessed in 24 children, all of whom had either angiocardiographic and surgical or angiocardiographic confirmation alone. Fourteen cases had truncus arteriosus, four aortopulmonary window, four anomalous origin of the left pulmonary artery from the ascending aorta, and two anomalous origin of the right pulmonary artery from the ascending aorta. It was possible to identify reliably each individual abnormality with a combination of suprasternal, precordial, and subcostal cuts. Problems only arose in differentiating truncus arteriosus from pulmonary atresia and ventricular septal defect when the main pulmonary artery and infundibular region of the right ventricle were extremely hypoplastic.

Aorta↗
PubMed1983

Truncus arteriosus. An anatomical-angiographic study.

A series of 25 cases of truncus arteriosus communis examined post mortem was studied retrospectively. Fifteen of them had had angiographic studies, enabling anatomo-radiographic correlative analysis to be made. All cases had situs solitus of the atria and concordant connections between atria and ventricles. The three main components of the malformation, present in all 25 cases, were: (a) ventricular septal defect, (b) single semilunar valve, (c) anomalies of the aortopulmonary septum. The ventricular septal defect was always subarterial but its size and its relation to mitral and tricuspid valves were extremely variable. The truncal valve varied greatly also in the number of its cusps and its relation to right and left ventricles. Above valvular level the presence or absence of residual aortopulmonary septum was reflected by the presence or absence of a main pulmonary artery. Additional supratruncal malformations produced variations of the anatomy of the aorta and pulmonary arteries. The angiographic demonstration of all these components was obtained best by selective angiography using special projections. From this study it appears that there is a wide degree of variability in all of the three main components of truncus arteriosus. Such anatomical variations should be identified angiographically in each patient in order to provide enough information before corrective surgery, but they do not alter the basically homogeneous anatomy of the category of truncus arteriosus and therefore do not justify complex classifications.

Angiocardiography↗
PubMed1983

Exercise induced pulmonary vasoconstriction.

Pulmonary vascular resistance normally falls or remains unchanged during exercise. Seven children with pulmonary hypertension were exercised during cardiac catheterisation after operative correction of ventricular septal defect (6) and truncus arteriosus (1). Except for the presence of moderate pulmonary hypertension, resting haemodynamics in these seven children were similar to those of normal children of equal age, but during exercise the postoperative patients showed a rise rather than a fall (+2% vs -18%) in total pulmonary vascular resistance. Two of the seven children had a substantial increase in pulmonary arteriolar resistance during exercise (from 509 to 715 dyne s cm-5 in one patient and from 606 to 828 dyne s cm-5 in the other). These two patients did not differ from normal children in respect of arterial or mixed venous oxygen saturations or of pH with exercise, nor was left atrial pressure related to the rise in pulmonary resistance. These two patients, however, had only a small rise in cardiac output during exercise (6.8% and 43.1%) in spite of a substantial increase in oxygen consumption (121% and 373%). One of the patients with exercise-induced pulmonary vasoconstriction had an 82% increase in resting pulmonary vascular resistance over a five year period subsequent to her first exercise study. Analysis of these data, and those previously reported, suggests that exercise induced pulmonary vasoconstriction may occur in 10 to 25% of patients who survive correction of certain congenital cardiac defects. The vasoconstriction cannot be attributed to abnormal changes in blood gases or left atrial pressure, and may be an early sign of progressive pulmonary hypertension.

Adolescent↗
PubMed1984

Valvar stenosis in truncus arteriosus.

Twenty three morphological specimens of truncus arteriosus were examined for evidence of stenosis of the semilunar valve. One third showed good evidence of stenosis as judged by careful measurement of the valve orifice, the valve ring, and the maximum diameter of the truncus. Correlation with measured pressure gradients was poor, but angiography and cross sectional echocardiography were better predictors of stenosis. Stenosis was invariably associated with cusp dysplasia and was more common in valves with two or four cusps.

Angiocardiography↗