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PubMed1981

Congenital cardiac anomalies in calves.

Seven cases of congenital cardiac anomalies in calves were reviewed from the files of the Ohio Veterinary Diagnostic Laboratory. The collection of material occurred during a six-month period from June 1977 to January 1978. The major clinical signs were dyspnoea, failure to gain weight and sudden death in young animals. The cardiac defects included two patent ductus arteriosus, two anomalies of the coronary vessels, one persistent truncus arteriosus, one transposition of the aorta and pulmonary artery and one ventricular septal defect.

Animals↗

PubMed2026

Clinical presentation and genotype-phenotype correlation of a novel pure duplication of 6p25.2-p22.3: a case report and literature review.

Duplication of 6p is a rare genetic syndrome of which about 25% have one or more congenital cardiac defects including cardiac septal defects, pulmonary artery hypoplasia and patent ductus arteriosus. We present the first case of a fetus with functional single ventricle and persistent truncus arteriosus during prenatal diagnosis whose genomic analysis revealed a novel pure duplication of 6p25.2-p22.3. The duplication fragment was confirmed to be associated with intrachromosomal insertion from mother via chromosome karyotype. The presentation of this case aims to expand the existing knowledge regarding this rare condition and facilitate its diagnosis in the future. Based on the comparison of cases with 6p duplication syndrome, we notice that the 6p terminal region, especially at 6p25.1 to 6p25.2, could be the critical region associated with heart complications or anomalies of the pulmonary arteries. The duplication of the potential modifier genes, RIPK1 and FARS2, were proposed to be attributed to heart defects in our study and should be further researched.

6p duplication↗

PubMed1970

Congenital heart disease in Ceylon.

An analysis of 555 consecutive cases of congenital heart disease in Ceylon has shown that for the whole series interatrial septal defect is the commonest cardiac lesion; next is interventricular septal defect, followed by persistent ductus arteriosus. Interventricular and interatrial septal defects are equally common among all the patients below 16 years of age, but interventricular septal defects are commonest among those below 11 years. Persistent truncus arteriosus is unusually common, while coarctation of the aorta and congenital aortic stenosis are rare, as compared with series reported from other places.

Adolescent↗

PubMed1989

Correction of interrupted aortic arch.

Twelve consecutive infants with interrupted aortic arch, ventricular septal defect, and persistent ductus arteriosus have had the anomaly repaired without the use of synthetic grafts. In two infants (5 and 9 months) the ductus arteriosus was used for the arch repair. In three patients (mean age 14 days) the left carotid artery was turned down to form the new arch. In the remaining seven (mean age 12 days) a direct anastomosis was achieved, but one of these patients died at operation. Two others in this group also had persistent truncus arteriosus. Five patients have required another operation (two for stenosis of the anastomosis with one death). The 10 survivors (mean follow-up 5 years) are well and support our belief that complete repair without the use of synthetic grafts is the treatment of choice in this rare and difficult group.

Anastomosis, Surgical↗

PubMed2006

Cardiac outflow tract: a review of some embryogenetic aspects of the conotruncal region of the heart.

A review concerning some embryogenetic aspects of the cardiac outflow tract is presented. Two main topics are discussed: the truncal septation and the secondary heart field. In the context of the septation of the truncus arteriosus, the development of the arterial valves is largely discussed, particularly in reference to the sinuses of Valsalva. Emphasis is also given to the fate of the external myocardial wall of the truncus arteriosus, as this primordial myocardial surface disappears later in the development. Molecular genetics data concerning Sox4 and NF-Atc transcription factors are correlated in the present review with rare forms of truncus malformations encountered in human pathology. The roles exerted by the secondary heart field and the neural crest on the development and growth of the conotruncal musculature are largely discussed. Reported experimental ablations of both secondary heart field and neural crest, showed conotruncal defects such as persistent truncus arteriosus, tetralogy of Fallot, and double-outlet right ventricle, which were considered as the result of a short outflow tract causing, ultimately, a lack of conotruncal rotation. In this regard, some morphologic correlations are carried out, in the present review, between these experimental animal models and human malformations, and it is thought that this sort of conotruncal defects cannot be explained always in terms of conotruncal hypoplasia. Finally, influence of Pitx2c, a left-right laterality signaling gene, on the modulation of the conotruncal rotation, as most recently reported, is emphasized in terms of very likely multifactorial contributions in the embryogenesis of the conotruncal region of the heart.

Animals↗

PubMed2000

Timing of surgery/catheter intervention in common congenital cardiac defects.

A large load of children with congenital heart disease (CHD) exists in our country. Fortunately facilities for treatment of almost all types of defects are available, although in few centres only. Many children are however, referred late for correction making them either high risk for surgery or inoperable due to development of irreversible pulmonary vascular obstructive disease. Therefore, it is important to recognise the ideal time of intervention in different types of CHD. A child with large atrial septal defect should have its closure by 3 to 4 years of age; on the other hand, a large ventricular septal defect (VSD) producing congestive heart failure needs very early surgery. Infants with small VSD may be medically managed as chances of spontaneous closure are high. Ligation of patent ductus arteriosus should be done at about one year of age if there is no pulmonary arterial hypertension or congestive heart failure. Similarly, coarctation should be dealt with either balloon dilatation or surgery at about one year of age provided the left ventricular function is normal and blood pressure in upper limb is well under control. For tetralogy of Fallot, correction is best done at about 9 months to one year of age. In case the child is symptomatic early, either a palliative shunt is done or total correction is performed if anatomy is suitable. In admixture lesions like transposition of great arteries (TGA), persistent truncus arteriosus or total anomalous pulmonary venous drainage, a very early intervention is needed so as to avoid development of severe hypoxia and early pulmonary vascular obstructive changes. Arterial switch operation is best performed in first two to three weeks of life for TGA with intact ventricular septum cases. Knowledge of natural history of the CHD is essential to plan for optimal timing of surgery or catheter intervention.

Cardiac Catheterization↗

PubMed2000

Timing of surgery/catheter intervention in common congenital cardiac defects.

Cardiac Catheterization↗

PubMed1995

Seven-year experience of noninvasive preoperative diagnostics in children with congenital heart defects: comprehensive analysis of 2,788 consecutive patients.

The spectrum of patients operated on without preoperative catheterization and angiography, the accuracy of echocardiographic diagnosis and its impact on the results of surgical treatment were prospectively assessed in 2,788 children consecutively operated for congenital heart defects (CHD) between 1986 and 1992. The overall percentage of surgery based solely on noninvasive preoperative examination increased from 63% in 1986 to 81% in 1990 and decreased to 72% in 1992. There were no differences in the preoperative diagnostic approach between groups of newborn, infants and children. A high percentage of patients with patent ductus arteriosus (96.5%), atrial septal defect (94%), incomplete atrioventricular septal defect (88.6%), ventricular septal defect (86.3%), coarctation of the aorta (80.2%) and total anomalous pulmonary venous connection (79.3%) was referred for surgery without prior invasive examination, while a lower percentage was found in univentricular heart (48.4%), pulmonary atresia (34.6%) and double outlet right ventricle (27.7%). More patients with pulmonary and tricuspid atresia were catheterized before complete repair compared to those who underwent palliative surgery (p < 0.01 and p < 0.0001, respectively). The echocardiographic diagnosis was correct in 96% of patients. Two patients of those with incomplete preoperative diagnosis died early postoperatively, both with missed apical ventricular septal defect. One with tetralogy of Fallot died after reoperation, the other with persistent truncus arteriosus due to sepsis. When the echocardiographic findings are in full agreement with the clinical status, physical examination, ECG and chest X-ray, we recommend cardiac surgery without prior catheterization in many patients with CHD.

Adolescent↗

PubMed1991

Abnormal development of cardiovascular systems in rat embryos treated with bisdiamine.

Administration of N,N'-bis(dichloroacetyl)-1,8-octamethylenediamine, bisdiamine, in pregnant Donryu rats on day 10 of gestation induces a high incidence of cardiovascular anomalies in fetuses. Bisdiamine administration induced aplasia of the sixth aortic arch artery, with both the right and left primitive pulmonary arteries being directly linked to the truncus, and resulting in four types of malformation of pulmonary arteries (PAs). When two primitive PAs shared a single root, the consequence was either pulmonary trunk hypoplasia, as is seen in tetralogy of Fallot, or type I persistent truncus arteriosus (PTA) as classified by Collet and Edwards. When root portions of two PAs did not fuse, either type II or type III PTA resulted. In controls, the right dorsal aorta (DA) between the right seventh intersegmental artery (IA) and the site where both DAs fuse degenerated and the left aortic arch (AA) and the right subclavian artery (SA) were formed. Bisdiamine administration induced two additional types of vascular anomalies. In one of these, the right DA between the right 4AA and the right 7IA degenerated and a left AA accompanied by an aberrant right SA resulted. In the other type, the left DA between the left 4AA and the left 7IA degenerated and a right AA accompanied by an aberrant left SA resulted. These results indicate that administration of bisdiamine induces malformation in the great blood vessels by disturbing persistency and degeneration of aortic arch arteries and DAs.

Abnormalities, Drug-Induced↗

PubMed2000

Malformations in offspring of diabetic rats: morphometric analysis of neural crest-derived organs and effects of maternal vitamin E treatment.

BACKGROUND: We have previously reported on a malformation-prone Sprague-Dawley rat substrain (U), which presents a high frequency of micrognathia in the offspring of diabetic mothers. This malformation is related to impaired development of the cranial neural crest cells (NCC); the defect may be prevented by antioxidative treatment of the mother. METHODS: We have therefore investigated whether fetuses of diabetic rats display other malformations associated with altered cranial NCC development and whether maternal vitamin E supplementation may affect such malformations. RESULTS: Fetuses of diabetic rats showed low-set external ears, severely malformed Meckel's cartilage, small thyroid and thymus, and absence of parathyroid glands. Cardiac anomalies were frequently observed, including rightward displacement of the aorta, double outlet right ventricle (DORV), persistent truncus arteriosus (PTA) combined with ventricular septal defects due to a malaligned outlet septum. The malformations in the outflow tract included abnormalities of the great arteries; right-sided aortic arch/descending aorta, and double aortic arches. These defects tended to occur together within individual fetuses. Maternal dietary treatment with 2% vitamin E markedly reduced the severity of the malformations. CONCLUSIONS: The phenotypic appearance of these defects is strikingly similar to the DiGeorge anomaly in humans, which has been found in children of diabetic mothers together with an overrepresentation of PTA and DORV. The malformations associated with defective NCC development in the offspring of diabetic U rats show several morphological similarities to those in humans; hence the teratogenic mechanisms may be similar and accessible for study.

Animals↗

PubMed2000

Variability in the phenotypic expression of fryns syndrome: A report of two sibships.

We report on two sibships with four fetuses of 12, 15, 17, and 20 weeks of gestation, respectively, and 1 preterm baby of 31 weeks of gestation affected by a multiple congenital disorder with manifestation suggestive of Fryns syndrome. In addition to the characteristic malformation pattern in Fryns syndrome, they presented with fetal hydrops, cystic hygroma, and multiple pterygias, allowing prenatal ultrasound diagnosis as early as in the 11th week of gestation. The two affected fetuses of family 1 showed severe craniofacial anomalies with bilateral cleft lip and palate, acral hypoplasia, postaxial oligodactyly, persistent truncus arteriosus, and interrupted aortic arch, asplenia sequence, and complex central nervous system midline malformations. In family 2 with three affected sibs, ear anomalies with atresia of the auditory canals, postaxial hexadactyly, intestinal atresias, callosal defects, and eye colobomas were the most outstanding features. On the basis of the present findings and former reports, the inter- and intrafamiliar phenotypic variability in Fryns syndrome, possible pathogenetic mechanisms, and the value of prenatal diagnosis are discussed. In the pathogenetic discussion, a special emphasis is put on the neural crest cell developmental field.

Abnormalities, Multiple↗

PubMed2000

Conotruncal anomalies in the trisomy 16 mouse: an immunohistochemical analysis with emphasis on the involvement of the neural crest.

The trisomy 16 (Ts16) mouse is generally considered a model for human Down's syndrome (trisomy 21). However, many of the cardiac defects in the Ts16 mouse do not reflect the heart malformations seen in patients suffering from this chromosomal disorder. In this study we describe the conotruncal malformations in mice with trisomy 16. The development of the outflow tract was immunohistochemically studied in serially sectioned hearts from 34 normal and 26 Ts16 mouse embryos ranging from 8.5 to 14.5 embryonic days. Conotruncal malformations observed in the Ts 16 embryos included double outlet right ventricle, persistent truncus arteriosus, Tetralogy of Fallot, and right-sided aortic arch. This spectrum of malformations is remarkably similar to that seen in humans suffering from DiGeorge syndrome (DGS). As perturbation of neural crest development has been proposed in the pathogenesis of DGS we specifically focussed on the fate of neural crest derived cells during outflow tract development of the Ts16 mouse using an antibody that enabled us to trace these cells during development. Severe perturbation of the neural crest-derived cell population was observed in each trisomic specimen. The abnormalities pertained to: 1) the size of the columns of neural crest-derived cells (or prongs); 2) the spatial orientation of these prongs within the mesenchymal tissues of the outflow tract; and 3) the location in which the neural crest cells interact with the myocardium. The latter abnormality appeared to be responsible for ectopic myocardialization found in trisomic embryos. Our observations strongly suggest that abnormal neural crest cell behavior is involved in the pathogenesis of the conotruncal malformations in the Ts16 mouse.

Animals↗

PubMed1993

Backtransplantation of chick cardiac neural crest cells cultured in LIF rescues heart development.

The cardiac neural crest is essential for normal development of the cardiovascular system. Cardiac neural crest cells are derived from the neural folds located between the mid-otic placodes and the caudal limit of somite 3. These crest cells can differentiate into a variety of mesenchymal cell types that support cardiovascular development, in addition to neurogenic cells. When cultured, many express alpha-smooth muscle actin or neurofilaments and lose their undifferentiated neural crest phenotype as shown by a decrease in HNK-1 reactivity. We wanted to determine whether cultured cardiac neural crest cells maintained the potency to support normal heart development when backtransplanted into embryos lacking their native cardiac neural crest. Under usual circumstances removal of the cardiac neural crest results in 80-100% incidence of persistent truncus arteriosus. The present study reports a system in which cardiac neural folds are cultured for 3 days and the cells backtransplanted into chick embryos after laser-induced ablation of the intrinsic cardiac neural folds. Rescue of heart development was improved 50% when cultured cells were backtransplanted and almost 200% when the backtransplanted cells had been cultured in leukemia inhibitory factor (LIF). To determine whether the cultured cells are capable of following normal migratory routes, cultured homospecific cardiac neural crest cells were tagged with DiI. Initially, fluorescent cells were found concentrated around the neural tube. By the second day following backtransplantation, the cells had migrated to the circumpharyngeal crest, populated the pharyngeal arches and aortic arch arteries, and were in the region of the cardiac outflow tract. By the third day, the labeled cells had dispersed, but could be found around the neural tube, esophagus, cardiac outflow tract, and within the dorsal root ganglia. Interestingly, a cranial migration to the periphery of the eyes was also noted. With the exception of the cranial migration to the eyes, cultured and backtransplanted cardiac neural crest cells followed normal migratory pathways to the cardiac outflow tract. LIF is used for the in vitro maintenance of the pluripotential phenotype of embryonic stem cells. In an effort to understand why LIF improves the ability of cultured neural crest cells to support normal heart development, we have examined the relationship of neural crest expression of HNK-1 antigen, alpha-smooth muscle actin, and neurofilament protein in neural crest cells cultured in LIF. LIF treatment resulted in an expanded period of expression of HNK-1 antigen, associated with a decrease in expression of alpha-smooth muscle actin.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗

PubMed1994

Myocardial enlargement in defective heart development.

BACKGROUND: The cardiac neural crest (neural crest extending from the mid-otic placode to the caudal region of somite 3) provides ectomesenchymal cells that contribute to aortic arch development and are essential for aortico-pulmonary septation of the outflow tract. Bilateral ablation of the cardiac neural crest in the chick embryo, prior to migration, leads to aortic arch anomalies and failure of septation of the cardiac outflow tract, which produces a severe defect known as persistent truncus arteriosus (PTA). Altered hemodynamics resulting from abnormal aortic arch artery development and PTA and other unknown factors related to the absence of neural crest, are likely to alter the developmental history of the myocardium. METHODS: In this study the wet and dry weights of ventricles and whole embryos, the total number of myocytes per ventricle and the myocyte density (number of myocytes per unit volume of ventricular myocardium) were compared in control (unwindowed eggs), sham-operated and cardiac neural crest ablated chick embryos at day 11 of incubation. RESULTS: We found that the wet and dry weights of ventricles from hearts with PTA were not different from normal hearts in control and sham-operated embryos. However, the embryos with PTA weighed less than embryos with normal hearts. Thus, the ventricle to embryo weight ratios were greater in embryos with PTA compared to control and sham-operated embryos for both wet (14 and 20%, respectively) and dry (30 and 59%) weights. The data further implied that more water was present with respect to body weight in comparison with sham-operated and control embryos which indicated that the embryos with PTA were edematous. The total number of myocytes and the number of myocytes per unit volume were not different when comparing sham-operated with PTA. Further, there was no indication that the myocardium from hearts with PTA was abnormal despite the small size and edema of the embryos. CONCLUSIONS: It appears that hemodynamic stresses, resulting from the structural defects produced by neural crest ablation, are insufficient to increase heart growth, although cardiac function is depressed as evidenced by edema and failure of the embryo to thrive.

Animals↗

PubMed2003

Developmental toxicity evaluation of 3-chloro-4-(dichloromethyl)-5-hydroxy-2(5H)-furanone (MX) in Wistar rats.

3-Chloro-4-(dichloromethyl)-5-hydroxy-2(5H)-furanone (MX) is a genotoxic chlorination by-product in drinking water. There is some evidence that it has developmental toxic effects in vitro but its potential to cause developmental effects in vivo is not known. The developmental effects were evaluated in Wistar rats. Rats (22-26 dams per dose group) were administered MX by gavage at the dose levels of 3, 30, or 60 mg/kg in water on gestation days 6-19. Control animals received plain water. Clinical signs, body weight, and food and water consumption were recorded for the dams. On gestation day 20, a cesarean section was performed and the ovaries anduterine contents of the dams were examined and the liver, kidneys, spleen, and thyroid glands weighed. The fetuses of all dose groups were weighed, sexed, and observed for external and skeletal malformations and the fetuses of the two highest dose groups were evaluated for visceral malformations. The highest dose, 60 mg/kg of MX, was slightly toxic to the dams. It decreased the corrected body weight gain of dams by 32% and the water consumption by 16-17%. Kidney and liver weights were slightly increased. MX did not affect the number of implantations nor did it cause any resorptions. The body weights of fetuses were not significantly affected. MX did not cause external malformations or skeletal anomalies. Two fetuses at 60 mg/kg and one fetus at 30 mg/kg had major visceral malformations (persistent truncus arteriosus, diaphragmatic hernia, dilated aorta with a stenosis of pulmonary arteries) and two minor artery abnormalities were observed in those animals. The frequency of unilateral displaced testis was slightly higher (9.2%) in the 60-mg/kg dose group than in controls (1.6%). Since the abnormalities did not form a consistent pattern and occurred most at maternally toxic dose, we conclude that MX can be regarded as non-teratogenic.

Abnormalities, Drug-Induced↗

PubMed2003

Neural crest and cardiovascular development: a 20-year perspective.

BACKGROUND: Twenty years ago this year was the first publication describing a region of neural crest cells necessary for normal cardiovascular development. Ablation of this region in chick resulted in persistent truncus arteriosus, mispatterning of the great vessels, outflow malalignments, and hypoplasia or aplasia of the pharyngeal glands. METHODS: We begin with a historical perspective and then review the progress that has been made in the ensuing 20 years in determining the direct and indirect contributions of the neural crest cells, now termed cardiac neural crest cells, in cardiovascular and pharyngeal arch development. Many of the molecular pathways that are now known to influence the specification, migration, patterning and final targeting of the cardiac neural crest cells are also reviewed. RESULTS: Although much knowledge has been gained by using many genetic manipulations to understand the cardiac neural crest cells' role in cardiovascular development, most models fail to explain the phenotypes seen in syndromic and non-syndromic human congenital heart defects, such as the DiGeorge syndrome. CONCLUSIONS: We propose that the cardiac neural crest exists as part of a larger cardiocraniofacial morphogenetic field and describe several human syndromes that result from abnormal development of this field.

Abnormalities, Multiple↗

PubMed1978

Morphological and topographical variations of the outlet chamber in complex congenital heart disease: an angiocardiographic study.

The outlet chamber of the human heart can show considerable variations in its morphology and topography (relationship to main ventricular chamber), and such variations in anatomy and spacial relationship may be predictive of the associated intracardiac anatomy. Although there is considerable debate about whether the position of the outlet chamber is indicative of the type of bulboventricular loop (whether D- or L-), a right-sided and anterior outlet chamber has different implications than a left-sided, superior, and more posterior positioned one. The inflows into the outlet chamber can vary anatomically, and progressive changes in the calibers of these communications can adversely alter the natural history. One or both of the greak vessels, or neither, or a persistent truncus arteriosus can originate from the outlet chamber, and any significant morphological change in the outlet chamber may or may not adversely affect either the pulmonary or systemic blood supply, or both. Finally, any consideration of the outlet chamber raises important conceptual difference in terminologies. At present, there is not unanimity as to what constitutes either an outlet chamber (as compared to a "small right ventricle" in tricuspid atresia) or a single (primitive) ventricle. Any discussion of the morphological and topographical variations of the outlet chamber must be viewed with respect to the terminology employed.

Angiocardiography↗

PubMed1987

Cardiovascular malformations induced by bromodeoxyuridine in the chick embryo.

For the study of morphogenesis and early embryonic development, 5-bromodeoxyuridine (BUdR), a halogenated analogue of thymidine, is incorporated into replicating DNA and serves as a valuable tool. To study the teratogenicity of BUdR on the developing chick cardiovascular system, we topically administered graded doses of BUdR (32.6-325.6 nmol) in ovo during Hamburger-Hamilton stages 15 to 16. We also administered to a parallel group of embryos corresponding nanomole doses of thymidine during identical stages of development. In the thymidine-treated group, survival rates and cardiovascular anomaly rates did not differ statistically from those in the chick Ringer's control group. Both survival rates and cardiovascular anomaly rates in the BudR-treated group were dose-responsive. Among 78 embryos with cardiovascular anomalies induced by BUdR, vascular malformations were found in 96%. These anomalies included interruption of the right fourth aortic arch, absence or hypoplasia of the right and/or left sixth aortic arch, and persistence of the left fourth aortic arch. Interruption of the right fourth aortic arch was always associated with intracardiac anomalies. Intracardiac anomalies were found in 54% of the embryos; these included ventricular septal defect, double outlet right ventricle, and persistent truncus arteriosus. Subclavian artery malformations were noted in 95% of the embryos. Possible mechanisms for BUdR-induced malformations in the cardiovascular system of the chick are discussed.

Animals↗