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PubMed1988

Cardiovascular anomalies produced by nimustine hydrochloride in the rat fetus.

The cardiovascular teratogenicity of nimustine hydrochloride (ACNU) was studied in rat fetuses. This drug is a nitrosourea derivative anticancer agent and produces alkylation of DNA. Pregnant Donryu rats were treated with single doses of 10, 11 or 13 mg/kg of the teratogen at various stages during gestation. Examination of the hearts was performed by microdissection after sacrificing the animals on the 20th day of gestation. The highest frequency of cardiovascular anomalies was found in the groups treated on the 8th day of gestation, but there was no difference in the rates induced by the three dosages of ACNU administered. The most common cardiovascular anomalies observed were ventricular septal defect (76.8%) and double outlet right ventricle (10.3%). A considerable number of affected fetuses (37/263) showed complex cardiac anomalies with atrioventricular (AV) malalignment and other AV valve anomalies. These anomalies include: double inlet left ventricle, straddling AV valve, atresia or stenosis of the AV valve, and dysplastic AV valve. ACNU appears to be a useful teratogenic agent for inducing complexes of cardiac anomalies which include AV malalignment.

Animals↗
PubMed1990

Maternal diabetes and cardiovascular malformations: predominance of double outlet right ventricle and truncus arteriosus.

Most studies on the relationship of maternal diabetes to cardiovascular malformations (CVM) have been prospective investigations of pregnancy outcome and therefore could not identify associations with rare cardiac lesions. The results of a retrospective study shed new light on the risks of specific cardiac defects in diabetic pregnancies. The Baltimore-Washington Infant Study, a population-based case-control investigation of CVM, provides information on maternal diabetes reported in personal interviews. Among 2259 mothers of cases, 35 (1.5%) reported diabetes present before pregnancy (called "overt") and 95 (4.2%) reported diabetes only during pregnancy (called "gestational"). Among 2,801 mothers of controls, 14 (0.5%) had overt diabetes and 83 (3.0%) had gestational diabetes. Malformation-specific risks were expressed as odds ratios (OR) with 99.5% confidence intervals (CI). The strongest associations with overt maternal diabetes were found with double outlet right ventricle (OR 21.33; 99.5% CI 3.34, 136.26), and truncus arteriosus (OR 12.81; 99.5% CI 1.43, 114.64). No significant diagnosis-specific associations were found with gestational diabetes. Non-cardiac malformations were present in 23% of infants with CVM whose mothers had overt diabetes and in 26% of infants with CVM whose mother had gestational diabetes, in 32% of infants with CVM whose mothers did not have diabetes, and in 4% of controls. Double outlet right ventricle and truncus arteriosus are malformations dependent upon neural-crest-cell-derived ectomesenchymal tissues; these are precisely the conotruncal abnormalities that result from experimental ablation of the neural crest in chick embryos. The association with diabetes suggests a further etiologic link between these two lesions.

Case-Control Studies↗
PubMed1983

Myocardial visualization on a routine perfusion lung scintigram: relationship to the amount of right-to-left shunt.

Three cases of myocardial visualization on a routine perfusion lung scintigram with 99mTc-macroaggregated albumin were reported in patients with congenital heart diseases; two cases of tetralogy of Fallot and one case of truncus arteriosus type IV. Large right-to-left shunts greater than 39% and marked hypertrophy of the ventricle suggesting the presence of increased coronary blood flow were noted in all cases. In the two patients with tetralogy of Fallot myocardial activity appeared to be located in the hypertrophic right ventricles.

Adult↗
PubMedDate not supplied

Truncus arteriosus associated with trisomy 18.

The spectrum of cardiac anomalies in trisomy 18 typically includes septal defects and polyvalvular disease and only rarely complex malformations. We report the first case of trisomy 18 with truncus arteriosus type II.

Chromosome Aberrations↗
PubMed1990

The hyperdense choroid plexus: a CT finding associated with aortic arch obstruction in the newborn.

We report the first observation of choroid plexus hyperdensity depicted by CT in two newborns with aortic arch obstruction. Neither infant had intracranial hemorrhage or infarction demonstrated by autopsy or cranial ultrasonography. Although not proven, we believe that such hyperdensity in these two cases represents abnormal vascularity within the choroid plexus related to upper extremity hypertension.

Aorta, Thoracic↗
PubMed1985

An aborted human fetus with truncus arteriosus communis--possible teratogenic effect of Tedral.

We recently performed a detailed anatomicopathologic examination on an aborted human embryo whose mother had taken four tablets of Tedral (one tablet of Tedral contains 130 mg theophylline, 25 mg ephedrine, 8 mg phenobarbital) for an upper respiratory tract infection when the embryo was at approximately 30 days of development. On the same day, the mother developed acute chest pain and a fast, irregular heart beat. The abortion occurred at approximately 80 days of gestation. The heart of the fetus showed truncus arteriosus (Van Praagh type A1). Although no direct cause and effect relationship was proven in this case, the possibility of a teratogenic effect of Tedral during early pregnancy is considered.

Abnormalities, Drug-Induced↗
PubMed1988

Spectrum of Di George syndrome in patients with truncus arteriosus: expanded Di George syndrome.

A study of 26 patients with truncus arteriosus showed a high prevalence of facial dysmorphism, aortic arch abnormalities, extracardiac malformations, and significant prenatal risk factors. There was little evidence of parathyroid or thymic abnormalities. However, there was laboratory evidence of immune deficiency, especially T-helper lymphocytes, and clinical evidence of predilection to infection. These findings suggest that patients with truncus arteriosus belong to the spectrum of the Di George syndrome.

Abnormalities, Multiple↗
PubMed1991

Truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle.

The association of truncus arteriosus with congenital left heart abnormalities is rare. We describe a case of truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle. In 11 other patients diagnosed with truncus arteriosus, none had left heart hypoplasia. Despite the truncal root being primarily or entirely committed to the right ventricle, more right than left heart abnormalities have been found. This is in contrast to double-outlet right ventricle in which left heart abnormalities are more often associated.

Female↗
PubMed1991

Balloon dilatation of a stenosed aortic homograft conduit following repair of the common arterial trunk.

An 8-year-old boy, who underwent surgical correction of a common arterial trunk at the age of 10 months, developed stenosis of the 15-mm aortic homograft. Chest x-ray demonstrated calcification of the homograft, and at cardiac catheterization there was a 57 mmHg gradient across the conduit. He underwent balloon dilatation using a 15-mm balloon catheter. After four inflations at a pressure of 5.5 atm, the gradient decreased to 34 mmHg. Six months later he had a Doppler-estimated gradient of 38 mmHg.

Angiography↗
PubMed1990

Truncus arteriosus revisited: an angiographic demonstration.

The 1965 reclassification of truncus arteriosus by Van Praagh and Van Praagh greatly enhanced our understanding of this interesting anomaly. This brief review article attempts to illustrate the various types of truncus arteriosus identified in this classification by demonstrating their angiographic features. Reemphasis of the usefulness of this classification should help students of congenital heart disease recognize the advantages of a uniform diagnostic approach to this entity.

Aorta, Thoracic↗
PubMed1990

Left ventricular thrombus following repair of truncus arteriosus.

A 6-month-old infant developed a left ventricular thrombus in association with low antithrombin-III activity following the repair of a truncus arteriosus. Conservative management was associated with spontaneous resolution of the clot. The significance of this association is discussed.

Antithrombin III↗
PubMed1986

Double mitral valve orifice.

A two-day-old baby with a type-I truncus arteriosus and double mitral valve orifice is reported. The double mitral valve orifice and additional lesions of each subdivision of the mitral valve were clearly shown by two-dimensional echocardiography. The medial orifice had a cleft leaflet and the lateral one a parachute-like disposition of the tension apparatus. This is the first report of double mitral valve orifice associated with truncus arteriosus, and the first in which anomalies of the subdivisions of the mitral valve were detected by two-dimensional echocardiography.

Echocardiography↗
PubMed1986

Echo planar imaging of normal and abnormal connections of the heart and great arteries.

Echo planar imaging (EPI) is that form of magnetic resonance imaging which provides very short image acquisition times. EPI also provides very rapid sequential imaging. The EPI method is ideal for imaging the heart and thoracic content because images are devoid of cardio-respiratory motion artefact. Previously an analysis of transectional images has been presented. This paper is concerned with the study of the heart by the use of EPI constructions in the sagittal and coronal planes. Defining connections between ventricle and great artery is of cardinal importance in paediatric cardiology. EPI constructions in the normal heart, transposition, truncus arteriosus and right heart hypoplasia are presented and discussed.

Heart Defects, Congenital↗
PubMed1986

Transectional echo planar imaging of the heart in cyanotic congenital heart disease.

Echo planar imaging is that form of magnetic resonance imaging which gives very short image acquisition times. The method has been used to produce images of the infant heart which are free of cardiorespiratory motion artefact, despite tachypnoea and tachycardia. EPI transections of the normal heart are compared with transections in truncus arteriosus, tetralogy of Fallot, right heart hypoplasia and transposition of the great arteries. The diagnosis of the cause of cyanosis in these infants was established by the noninvasive EPI method and validation of the findings may be found in transectional postmortem analyses reported in the literature.

Heart Defects, Congenital↗
PubMedDate not supplied

Surgical repair of truncus arteriosus.

This paper reviews the initial experience of radical correction of truncus arteriosus in the Soviet Union. The anatomy, hemodynamics, diagnosis and surgical technique are described in detail. Five patients were operated on; three survived. The two deaths in this series were due to mistakes in evaluating the indications for the operation.

Aortography↗
PubMed1989

Successful correction of a Collett Edwards type II truncus arteriosus after bilateral pulmonary artery banding--a case report.

A case of a 4-year-old girl who underwent successful correction of a Collett Edwards type II truncus arteriosus following bilateral pulmonary artery banding is reported herein. The diagnosis of Collett Edwards type II truncus arteriosus was made when she was 5 months of age, after which the bilateral pulmonary artery banding was performed. This proved very effective and enabled elective radial surgery to be done. The radical operation was performed safely by a modified version of Rastelli's procedure. The patient had an uneventful recovery and is now well with no symptoms one year following her operation.

Child, Preschool↗
PubMed2002

Single origin of right and left pulmonary artery branches from ascending aorta with nonbranching main pulmonary artery: relevance to a new understanding of truncus arteriosus.

We report the third known case of origin of the right and left pulmonary artery branches from the ascending aorta via a short common pulmonary artery. A large unbranching main pulmonary artery opened through a patent ductus arteriosus into the descending thoracic aorta. Preductal coarctation of the aorta and multiple congenital anomalies were also present. This rare cardiovascular malformation facilitates a new anatomic and developmental understanding of truncus arteriosus.

Aorta↗