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PubMed1997

Pax3 is required for cardiac neural crest migration in the mouse: evidence from the splotch (Sp2H) mutant.

Neural crest cells originating in the occipital region of the avian embryo are known to play a vital role in formation of the septum of the cardiac outflow tract and to contribute cells to the aortic arches, thymus, thyroid and parathyroids. This 'cardiac' neural crest sub-population is assumed to exist in mammals, but without direct evidence. In this paper we demonstrate, using RT-PCR and in situ hybridisation, that Pax3 expression can serve as a marker of cardiac neural crest cells in the mouse embryo. Cells of this lineage were traced from the occipital neural tube, via branchial arches 3, 4 and 6, into the aortic sac and aorto-pulmonary outflow tract. Confirmation that these Pax3-positive cells are indeed cardiac neural crest is provided by experiments in which hearts were deprived of a source of colonising neural crest, by organ culture in vitro, with consequent lack of up-regulation of Pax3. Occipital neural crest cell outgrowths in vitro were also shown to express Pax3. Mutation of Pax3, as occurs in the splotch (Sp2H) mouse, results in development of conotruncal heart defects including persistent truncus arteriosus. Homozygotes also exhibit defects of the aortic arches, thymus, thyroid and parathyroids. Pax3-positive neural crest cells were found to emigrate from the occipital neural tube of Sp2H/Sp2H embryos in a relatively normal fashion, but there was a marked deficiency or absence of neural crest cells traversing branchial arches 3, 4 and 6, and entering the cardiac outflow tract. This decreased expression of Pax3 in Sp2H/Sp2H embryos was not due to down-regulation of Pax3 in neural crest cells, as use of independent neural crest markers, Hoxa-3, CrabpI, Prx1, Prx2 and c-met also revealed a deficiency of migrating cardiac neural crest cells in homozygous embryos. This work demonstrates the essential role of the cardiac neural crest in formation of the heart and great vessels in the mouse and, furthermore, shows that Pax3 function is required for the cardiac neural crest to complete its migration to the developing heart.

Animals↗
PubMed1999

A requirement for neuropilin-1 in embryonic vessel formation.

Neuropilin-1 is a membrane protein that is expressed in developing neurons and functions as a receptor or a component of the receptor complex for the class 3 semaphorins, which are inhibitory axon guidance signals. Targeted inactivation of the neuropilin-1 gene in mice induced disorganization of the pathway and projection of nerve fibers, suggesting that neuropilin-1 mediates semaphorin-elicited signals and regulates nerve fiber guidance in embryogenesis. Neuropilin-1 is also expressed in endothelial cells and shown to bind vascular endothelial growth factor (VEGF), a potent regulator for vasculogenesis and angiogenesis. However, the roles of neuropilin-1 in vascular formation have been unclear. This paper reported that the neuropilin-1 mutant mouse embryos exhibited various types of vascular defects, including impairment in neural vascularization, agenesis and transposition of great vessels, insufficient aorticopulmonary truncus (persistent truncus arteriosus), and disorganized and insufficient development of vascular networks in the yolk sac. The vascular defects induced by neuropilin-1 deficiency in mouse embryos suggest that neuropilin-1 plays roles in embryonic vessel formation, as well as nerve fiber guidance.

Animals↗
PubMed1994

Transvaginal echocardiographic examination of the fetal heart between 13 and 15 weeks' gestation in a low-risk population.

The object of our investigation was to evaluate the potential of transvaginal echocardiography for a complete anatomic study of the fetal heart, and for detection of congenital heart diseases in low-risk pregnant patients between 13 and 15 weeks' gestation. High-frequency (6.5 MHz and 7.5 MHz) transvaginal probes were employed for fetal echocardiographic assessment. Six hundred and sixty patients considered at low risk for congenital heart disease underwent in utero ultrasonographic studies, neonatal records, postnatal imaging studies, and pathologic examination. The four-chamber view was obtained in 100% of the fetuses, while the extended fetal heart examination was completed in 98%. Six fetuses with cardiac defects were diagnosed during the study: three had major heart defects (aortic atresia, tetralogy of Fallot, and persistent truncus arteriosus), all detected at the early transvaginal scan; three cardiac anomalies escaped our early diagnosis; two fetuses had a minor ventricular septal defect diagnosed only postnatally; and one had multiple cardiac rhabdomyoma diagnosed in the third trimester. Our results demonstrate that between 13 and 15 weeks' gestation transvaginal echocardiographic assessment of the fetal heart in the low-risk population is feasible. Moreover, some severe anomalies may be detected at such an early gestational age.

Echocardiography↗
PubMed2001

[Diagnosing complicated congenital heart disease with two-dimensional echocardiography and three-segment concept].

OBJECTIVE: To apply three-segment heart anatomy concept to two-dimensional echocardiography (2DE) in the diagnosis of complicated congenital heart disease (CHD). METHODS: 920 cases of CHD were diagnosed with 2DE and three-segment heart anatomy concept. 822 out of the 920 cases underwent angiocardiography, and 584 cases were confirmed by operation. RESULTS: According to three-segment heart anatomy concept of atrial position, 855 cases were situs solitus (including 89 cases of left superior vena cava and 31 cases of complete anomalous pulmonary venous drainage), 53 cases were situs inversus, and 12 cases were situs ambigus. As to atrial-ventricular valve, 747 cases were both opening, 112 cases had one-side atresia, and 61 cases had one together valve; As to ventricle position, 606 cases were dextro-loop, 111 cases had levo-loop, and 203 cases had single ventricle (including 24 cases), As to cor biloculare of great artery relation, 304 cases had normal relation of great arteries, including 35 cases of pulmonary atresia, 219 cases had right-transposition of great arteries, 240 cases had left-transposition of great arteries, 32 cases were mirror to normal, and 25 cases had persistent truncus arteriosus. 823 cases were diagnosed with 2DE, with a total accurate rate of 89.5%. CONCLUSION: Applying three-segment heart anatomy concept to 2DE to diagnose complicated CHD and analyzing the heart segment by segment will make the diagnosis simple and systematic, providing a concise basis for heart surgeries.

Adolescent↗
PubMedDate not supplied

[Tricuspid atresia: A therapeutic problem].

1. The authors present 80 cases of tricuspid atresia of the Children's Cardiology Service of the Instituto Nacional de Cardiología de México. They propose their own classification based on the position of the visceral suits and of the great arteries and the possible existance of a persistent truncus arteriosus. 2. The requirements that should be fulfiled to establish an anatomic and hemodynamic diagnosis are determined; and the need to know in detail the anatomy of the right ventricle; of its exit chamber, of the pulmonary valve and of the pulmonary truncus is emphasized; as well as the measures in the auricles and the left ventricle. 3. They insist upon the need, when the pulmonar arterial pressure cannot be measured directly, to introduce a catheter in a pulmonar vein, because the pressures obtained are quite similar to the pressure in the pulmonar arterial territory. 4. The fundamental data of the anatomic and hemodynamic study are discussed and illustrated. They confirm the close relation that exists between the diameter of the exit chamber of the right ventricle and of the valvular ring, the pulmonary truncus and its branches. 5. In all cases of tricuspid atresia with AQRS deviated to the right and downwards in the frontal plane; transposition of the great arteries should be thought of when it deviates to the left and upwards, in patients with crossed great arteries. 6. In the cases operated with sistemic-pulmonary fistula, there was an intrahospital mortality range of 25%, similar to that obtained by most authors. 7. The paliative surgical procedures are analyzed, and those that at present could be considered as "corrective" such as Fontan and Kreutzer's operations. The need, to gather several diagnostic parameters. To establish the surgical indication in the sistemicpulmonary and venus shunts, is emphasized.

Angiocardiography↗
PubMed2004

[Diagnostic value of color echocardiography in neonatal cyanotic congenital heart disease].

OBJECTIVE: To study the value of color echocardiography in diagnosis of neonatal cyanotic congenital heart disease (NCHD). METHOD: A total of 149 cyanotic neonates were examined by color echocardiography, of whom 25 were diagnosed having NCHD and analyzed for classification. RESULTS: Such complex deformities as transposition of the major arteries, tetralogy of Fallot, single ventricle, persistent truncus arteriosus were found in these 25 cases of NCHD, of which major artery transposition was the most common defects, followed by tetralogy of Fallot and then by single ventricle. CONCLUSION: Color echocardiography can be used routinely in diagnosis of congenital heart disease of cyanotic neonates.

Echocardiography, Doppler, Color↗
PubMed2005

[Pathological diagnosis of conotruncal defects by transthoracic three-dimensional echocardiography].

OBJECTIVE: Recently, it has been demonstrated in several studies that three-dimensional echocardiography (3DE) with any cut view interpretation of the cardiac morphological structures and its dynamic three-dimensional display can conform to the need of accurate anatomical diagnosis of complex congenital heart diseases including conotruncal defects (CTD). 3DE can not only provide functional and morphological information of CTD to decide on surgical repair but also simulate the intra operative visualization of complex anatomical cardiac structures to improve the understanding of the anatomy of CTD, but there have been scarce studies to assess systematically the potential noninvasive diagnostic value of transthoracic 3DE for CTD in children. Thus, this study attempted to evaluate the diagnostic value of transthoracic 3DE for the pathological diagnosis of CTD in children. METHODS: HP Sonos 5500 echocardiographic system interfaced with Tomtec 3DE imaging system (echo-view 4.2 software) was used to diagnose 210 patients (132 male, 78 female, aged 0-15 years, mean 3.2 +/- 3.2 years) with CTD in Shanghai Children's Medical Center from May 2000 to November 2003, including 62 patients with double outlet right ventricle, 48 patients with transposition of great arteries, 48 patients with tetralogy of Fallot, 38 patients with pulmonary atresia with interventricular septal defect and 14 patients with persistent truncus arteriosus. Diagnostic method consisting of ten designed 3DE sectional views and Van Praagh's sequential segmental approach in congenital heart diseases was used in this study. And the results were compared with those diagnosed by two-dimensional echocardiography (2DE) and angiocardiography. Surgical findings were considered as the "gold standard". RESULTS: There were 526 image acquisitions of transthoracic 3DE in the 210 patients with CTD including 200 through subcostal window, 254 through parasternal window and 72 through apical window, among which 477 image acquisitions (93.2%) could demonstrate good spatial cardiovascular structures. Meanwhile, six 3DE sectional views of the diagnostic method of the ten designed 3DE sectional views were identified for the diagnosis of CTD as follows: en-face view of atrial and ventricular septa from left ventricle (L1a); en-face view of atrial and ventricular septa from right ventricle (L2a); four-chamber view of whole-heart oblique frontal plane (Ha and Hb); en-face view of atrial-ventricular valve from atria (S1a) and en-face view of atrial-ventricular valve from ventricles (S2a). The 3DE sectional diagnosis showed that 3DE could provide more information of inner cardiac structure than 2DE, such as ventricular configuration identification, atrial-ventricular and ventricular-arterial continuity, location and shape of ventricular septal defect (VSD), and appreciation of the defects related with aorta and pulmonary arteries, etc. In 147 cases of surgical repair, both 3DE and 2DE were in accordance with the surgical findings, but 3DE had new findings in 18 (12.2%) cases and corrected the diagnoses in 10 (6.8%) cases as compared with 2DE. CONCLUSION: The 3DE sectional diagnostic method could make a successfully sectional diagnosis of pathological morphology in CTD by demonstrating more spatial information of inner cardiac structure than 2DE and angiocardiography.

Adolescent↗
PubMed2006

Repeat right ventricular outflow tract reconstruction using the Medtronic Freestyle porcine aortic root.

BACKGROUND AND AIM OF THE STUDY: A variety of valve substitutes are used for right ventricular outflow tract (RVOT) reconstruction in children and young adults after previous RVOT surgery that has led to significant pulmonary insufficiency and/or stenosis. Herein, the authors' experience with pulmonary valve replacement (PVR) using a porcine valved conduit late after previous RVOT surgery was reviewed. METHODS: Between August 2002 and April 2005, 31 patients (mean age 14.5 +/- 9.5 years; range: 1.2-33.1 years) underwent PVR using the Medtronic Freestyle porcine aortic root. These patients averaged two prior operations (range: 1-5) for the following diagnoses: tetralogy of Fallot +/- pulmonary atresia (n = 21); persistent truncus arteriosus (n = 5); aortic stenosis (Ross-Konno procedure) (n = 2); pulmonary atresia with intact ventricular septum; (n = 1); congenital pulmonary stenosis (n = 1); and transposition of the great arteries (n = 1). Of these patients, 29 (93.5%) underwent additional procedures at the time of pulmonary valve insertion including: branch pulmonary artery reconstruction (n = 21), atrial septal defect closure (n = 5), ascending aorta replacement (n = 4), pacemaker or defibrillator placement (n = 3), tricuspid valve repair (n = 2), ventricular septal defect closure (n = 2), and other procedures (n = 2). RESULTS: There were no early or late deaths. The mean hospital stay was 8.3 +/- 5.9 days (range: 4-25 days). One patient had a subarachnoid hemorrhage with transient left hemiparesis, and two patients had acute tubular necrosis with temporary dialysis treatment. All patients were well at a mean follow up of 13 +/- 9.3 months (range: 0.5-31 months). Echocardiography showed trivial or no pulmonary insufficiency in 30 patients (96.7%). The calculated mean peak systolic RVOT gradient by echocardiography was 23.4 +/- 7.6 mmHg. CONCLUSION: The Medtronic Freestyle bioprostheses demonstrated excellent short-term results for repeat RVOT reconstruction. This valve's hemodynamic characteristics are comparable to those of homografts, and it is an attractive alternative given the limited availability of homograft valves. A lack of availability in sizes smaller than 19 mm limits use of this valve in pediatric patients, and long-term results remain to be determined.

Adolescent↗
PubMedDate not supplied

Short rib-polydactyly syndrome: lethal chondrodysplasia associated with brain malformations in a 35-week-gestation infant.

This case report describes the neuropathological findings in an autopsy case of short rib-polydactyly syndrome (SRPS). The patient was a Japanese female neonate who was born at 35 weeks of gestation and died soon after birth due to severe cardiopulmonary insufficiency. Clinical and radiological findings were most consistent with SRPS type I (Saldino-Noonan type). General autopsy findings included situs inversus, persistent truncus arteriosus and endocardial cushion defect, hypoplastic lungs and adrenal glands, and vaginal atresia. Fixed brain weight was 330 g. Three different categories of pathological changes were detected in the brain. These were as follows: (1) multiple cyst formation in the parenchyma, (2) primary malformations of the nervous and mesenchymal tissues, and (3) deposition of an unusual substance in the cerebral white matter. The multiple cysts or cavities in the parenchyma may be due to severe hypoxic-ischemic insults related to the congenital heart anomaly. The primary malformations were summarized as follows: (1) capillary telangiectasia of the pia mater and choroid plexus, (2) olfactory dysplasia with asymmetry, (3) focal cortical dysplasia in the frontal lobe and cerebellum, (4) olivary dysplasia, and (5) enlargement of the posterior part of the lateral ventricle. Dysplastic changes of the nervous tissue can be classified into the group of neuronal migration disorders. Although biochemical properties of the unknown substance were not determined, it is considered to be some product derived from an inborn error of metabolism. Morphological data of SRPS is still scarce, and pathognomonic changes have not yet been elucidated. The present data suggests that coexistence of the nervous and mesenchymal malformations may be highly characteristic of SRPS.

Brain↗
PubMed1985

Pulmonary artery banding.

Between January 1981 and December 1983 22 children underwent pulmonary artery banding at the Red Cross War Memorial Children's Hospital, Cape Town. The indications for banding in this group were control of pulmonary overperfusion and severe congestive cardiac failure. The underlying cardiac defects were tricuspid atresia with ventricular septal defect (VSD), transposition of the great arteries and univentricular heart, large or multiple VSDs, double-outlet right ventricle, atrioventricular canal defect, persistent truncus arteriosus and the VSD-patent ductus arteriosus-coarctation syndrome. Three children died (13,6%) in the first 30 days postoperatively and another 3 died 5-7 weeks after surgery.

Blood Pressure↗
PubMedDate not supplied

[Echocardiographic study of different forms of pulmonary atresia].

Echocardiographic recognition of pulmonary atresia with either ventricular septal defect or intact ventricular septum is of great value in the differential diagnosis with other forms of right ventricular outflow tract obstruction or hypoplasia of the right ventricle. We studied nine patients with pulmonary atresia seven with ventricular septal defect and two with intact ventricular septum. It was not possible in either group of these patients to register systolic opening and diastolic closure movements of the pulmonary valve. In the group with ventricular septal defect, aortic straddling was present in all cases. In one patient it was possible to register the echoes of an imperforated pulmonary valve. In the remaining patients, the differential diagnosis with persistent truncus arteriosus was not possible. M-mode and Two-dimensional echocardiographic features of pulmonary atresia with intact ventricular septum are specific of this malformation and can differentiate it from other forms of hypoplasia of the right ventricle.

Child↗
PubMed1994

First-trimester diagnosis of fetal congenital heart disease by transvaginal ultrasonography.

OBJECTIVE: To describe the feasibility of diagnosing fetal congenital heart defects by transvaginal ultrasonography during the first trimester of pregnancy. METHODS: Pregnant women presenting to the Ultrasonographic Unit at the Chaim Sheba Medical Center who had a diagnosis of fetal heart defects were reviewed retrospectively. Attention was paid to prenatal ultrasound studies, karyotype, and pathologic examinations. RESULTS: Using high-resolution transvaginal ultrasonography, we were able to detect fetal tachycardia (one case), ectopia cordis with ventricular septal defect (one case), atrioventricular septal defect (two cases), ventricular septal defect with persistent truncus arteriosus (one case), tetralogy of Fallot (two cases), and large right atrium with unguarded tricuspid valve (Uhl disease) (one case). Seven of these fetuses had normal karyotypes and all showed additional sonographic abnormalities, including septated cystic hygroma (three cases), hydrops (ascites and pericardial effusions) (two cases), omphalocele (one case), and bilateral agenesis of kidneys (one case). Only one fetus with an abnormal karyotype (45,XO) showed a combination of septated cystic hygroma with hydrops. CONCLUSIONS: High-resolution duplex Doppler transvaginal ultrasonography during the first trimester of pregnancy seems to be a useful diagnostic method for detecting some congenital heart diseases.

Abnormalities, Multiple↗
PubMedDate not supplied

[Di Giorge's syndrome with congenital heart disease (author's transl)].

Authors describe three cases of Di Giorge's syndrome (thymic and parathyroid aplasia) proved through anatomical study postmorten, associated with cardiac malformations (Tetralogy of Fallot with pulmonary atresia, persistent truncus arteriosus and large patent ductus arteriosus). Clinical characteristics of the stated syndrome are analized with special reference to congenital heart diseases that are included in it.

Abnormalities, Multiple↗
PubMed1997

Primary repair of interrupted aortic arch and associated heart lesions in newborns.

Primary repair of interrupted aortic arch and associated heart lesions was performed in 13 patients aged from 1 to 85 days. The surgery was performed through the midline sternotomy approach in extracorporeal circulation and deep hypothermia. Hypothermic circulatory arrest at 14 to 19 degrees C was used for reconstruction of the aortic arch. In all patients it was possible to perform a direct anastomosis between the ascendent and descendent aorta. At the same time closure of the ventricular septal defect was performed in 11 patients, closure of the atrial septal defect in 4, correction of persistent truncus arteriosus in 3, resection of subaortic stenosis in 2, arterial switch repair of transposition of the great arteries in 1, correction of double outlet right ventricle in 1 and patch closure of aortico-pulmonary window in 1 patient. Three (23.1%) newborns died in the early postoperative period: two from sepsis and one from multiple organ failure. Ten patients (76.9%) were followed up for 1 to 29 months postoperatively. All of them are in very good condition with a nonrestrictive aortic anastomosis. Primary one-stage repair of interrupted aortic arch and associated heart lesions is preferred to the two-stage repair in all newborns with this critical congenital heart disease.

Aorta, Thoracic↗
PubMed1998

[Primary repair of interrupted aortic arch in neonates].

In the Kardiocentrum, University Hospital Motol, Prague, protocol of the primary repair of interrupted aortic arch was introduced, and between 1993-1997, 15 neonates aged 1-26 days (median 5 days) were operated on. Treatment with prostaglandins E for maintenance of the ductal patency, correction of metabolic acidosis, and treatment of all complications were necessary before surgery. The correction was performed from the midline sternotomy approach, in extracorporeal circulation and deep hypothermia with circulatory arrest. Direct anastomosis between the ascending and the descending aorta was possible in all the patients. At the same time, associated heart lesions were corrected (ventricular septal defect in 13, persistent truncus arteriosus in 3, subaortic stenosis in 2, transposition of the great arteries, double-outlet right ventricle and aortico-pulmonary window in 1 patient, each). Four (26.7%) patients died after surgery. Out of the first 6 neonates 3 (50.0%) died, but out of the subsequent 9 patients only 1 (11.1%) died. Reoperation was necessary in 2 patients. All 11 early survivors are alive and doing well 8-54 months after the repair. In one of them restenosis at the site of aortic anastomosis and hemodynamically significant subaortic stenosis occurred. All the remaining patients have a nonrestrictive aortic anastomosis. Primary repair of interrupted aortic arch and associated heart lesions can be performed in a neonate with reasonable mortality. Treatment of complications is necessary before surgery. Results depend especially on the patient's clinical condition and experience of the center.

Aorta, Thoracic↗
PubMed1980

Persistent truncus arteriosis associated with interruption of the aortic arch.

Five patients with a combination of truncus arteriosus and interruption of the aortic arch are reported. The combination of those defects significantly increases the surgical risk. This rare cardiac malformation can only be diagnosed radiographically. An aberrant right subclavian artery is present in 25% of patients. It is helpful in suspecting the diagnosis from plain films of the chest. The diagnosis can be made from a ventriculogram, but usually a truncogram is necessary to define the anatomy of the aortic arch.

Adolescent↗