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Intra- and inter-reader reproducibility of blood flow measurements on the ascending aorta and pulmonary artery using cardiac magnetic resonance.

The aim of our study was to estimate the intra- and inter-reader reproducibility of blood flow measurements in the ascending aorta and main pulmonary artery using cardiac magnetic resonance (CMR) and a semi-automated segmentation method. The ethics committee approved this retrospective study. A total of 50 consecutive patients (35 males and 15 females; mean age±standard deviation 27±13 years) affected by congenital heart disease were reviewed. They underwent CMR for flow analysis of the ascending aorta and main pulmonary artery (1.5 T, through-plane phase-contrast sequences). Two independent r

Effects of surgical en bloc rotation of the arterial trunk on the conduction system in children with transposition of the great arteries, ventricular septal defect and pulmonary stenosis.

OBJECTIVES: The standard surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is the Rastelli operation. Recently, en bloc rotation of the arterial trunk, by cutting out the aortic and the pulmonary root in one block and by rotating it 180°, has been introduced as a new option for anatomical repair. METHODS: To evaluate the effects of this surgical method on the conduction system, pre-operative, post-operative, and follow-up electrocardiograms as well as patient charts were reviewed retrospectively. A total of 16 consecuti

Clinical results of cryopreserved valved conduits in the pulmonary ventricle-to-pulmonary artery position.

Aortic valved homograft conduits (AVHC) have become valuable in the pulmonary ventricle (PV)-to-main pulmonary artery (MPA) reconstruction in congenital heart defects. Since 1985, 45 patients, ranging in age from 12 days to 32 years, underwent PV-to-MPA reconstruction utilizing cryopreserved AVHC. Operative deaths included seven patients (16%), six of whom died as a result of the complexity of their underlying heart defects. One late death (2%) occurred as a result of infective endocarditis 48 months after conduit placement. The 38 patients who survived the operation remained in the intensive

Late results after extracardiac conduit repair for congenital cardiac defects.

Analysis of the late results in 352 patients surviving insertion of an extracardiac conduit before mid 1977 has provided a mean follow-up interval of 65 months. Three fourths of the patients remain in improved condition after operation. Serial measurements of transconduit gradient are available in 90. The median change was +7 mm Hg and the mean +21 mm Hg. Reoperation was required in 16 percent of patients (mortality rate 9 percent), most commonly (77 percent) because of progressive conduit stenosis, more commonly for transposition of the great arteries than for other types of anomalies, and mo

Baz1b Dosage Influences Cardiovascular Function, Predisposing to Dilated Cardiomyopathy.

BAZ1B is one of several genes deleted in Williams-Beuren Syndrome (WBS), a complex, multisystem genetic condition that occurs in ~1 in 8000 … did not reveal congenital heart disease typical of neural crest defects (e.g. tetralogy of Fallot, truncus arteriosus, double outlet right ventricle, or interrupted aortic arch). Echocardiograms, performed at 1-month to align with the growth analysis timeline, revealed mildly decreased ejection fraction (EF, median: WT 64%, Baz1b+/- 56%, p<0.01) and fractional shor …

Ovarian Metastases of Colorectal Origin: Treatment Patterns and Factors Affecting Outcomes.

AIM: The study was done to determine the maternal and fetal outcome of pregnancies complicated by maternal diabetes either Gestational Diabe … with Menigomyelocoele, there was one case of isolated Hydrocephalus, and there was also one case of Truncus arteriosus. CONCLUSIONS: The study analyses maternal and fetal complication in the GDM group and also preexisting diabetes group. In our centre, the 60 % women were GDM while 40 % were having pre-existing diabetes. Total rate of fetal/neonatal complication ra …

[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 interpretat … 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 echocardiogra …

Effect of Stiffened and Dilated Ascending Aorta on Aerobic Exercise Capacity in Repaired Patients With Complex Congenital Heart Disease.

Several studies have reported aortic dilation and increased stiffness of the ascending aorta in patients after repair of congenital heart disease (CHD), which may be a predominant cardiovascular risk. However, the clinical significance has not been described in detail. In this retrospective study, 175 repaired patients with complex CHD achieving biventricular circulation and age-matched 39 control subjects were reviewed (median age: 14.9 and 15.7 years, respectively). We measured the diameters of the ascending aorta and descending aorta from catheterization angiograms to yield Z-scores and sti

Specific birth defects in pregnancies of women with diabetes: National Birth Defects Prevention Study, 1997-2011.

BACKGROUND: Diabetes is associated with an increased risk for many birth defects and is likely to have an increasing impact on birth defect … nce interval, 6.2-16.5), heterotaxy (adjusted odds ratio, 12.3; 95% confidence interval, 7.3-20.5), truncus arteriosus (adjusted odds ratio, 14.9; 95% confidence interval, 7.6-29.3), atrioventricular septal defect (adjusted odds ratio, 10.5; 95% confidence interval, 6.2-17.9), and single ventricle complex (adjusted odds ratio, 14.7; 95% confidence interval, 8.9-24.3 …

Impaired Fetal Environment and Gestational Age: What Is Driving Mortality in Neonates With Critical Congenital Heart Disease?

Background Infants with critical congenital heart disease (CCHD) are more likely to be small for gestational age (SGA) or born to mothers with maternal placental syndrome. The objective of this study was to investigate the relationship between maternal placental syndrome, SGA, and gestational age (GA) on 1-year mortality in infants with CCHD. Methods and Results In a population-based administrative database of all live-born infants in California (2007-2012) we identified all infants with CCHD without chromosomal anomalies. Our primary predictor was an impaired fetal environment (IFE), defined

Clinical utility of repeat fetal echocardiography in congenital heart disease.

OBJECTIVES: To investigate the utility of repeat fetal echocardiography (FE) following a diagnosis of structural congenital heart disease (C … . No management change occurred in fetuses diagnosed with isolated ventricular septal defect (VSD), truncus arteriosus, pulmonary vein anomaly or isolated arch sidedness/branching abnormality. Compared to those with a VSD, management was significantly more likely to be changed in fetuses with a balanced AVC defect (P = 0.025) and left heart lesions (P = 0.002). Righ …

Repair of interrupted aortic arch: a ten-year experience.

Eighty-two consecutive patients with interrupted aortic arch were referred to our institution between 1985 and 1995. Three died before any a … both subaortic stenosis and complex association). Aortopulmonary window was found in four patients, truncus arteriosus was found in eight, and transposition of the great arteries was found in five, double-outlet right ventricle was found in one, single ventricle was found in three, multiple ventricular septal defects were found in two and superior-inferior ventricle …

Establishing right ventricle-pulmonary artery continuity by autologous tissue: an alternative approach for prosthetic conduit repair.

BACKGROUND: In conventional conduit operations, longevity has been essentially limited by the inevitable need for conduit replacement. This study was undertaken to compare long-term results of the use of equine pericardial conduits, autologous pericardial conduits, and direct anastomosis repair. METHODS: Between 1982 and 2001, 366 patients underwent primary establishment of right ventricle-pulmonary artery continuity at our institution. The mean age at the time of operation was 6.2 years (range, 4 days to 28 years) and mean weight was 17.2 kg (range, 1.6 to 61 kg). Three different repair tech

Cardiovascular malformations among preterm infants.

OBJECTIVE: Preterm birth and cardiovascular malformations are the 2 most common causes of neonatal and infant death, but there are no publis … greater incidence of coarctation of the aorta, double-inlet left ventricle, hypoplastic left heart, truncus arteriosus, double-outlet right ventricle, and atrioventricular septal defect among stillbirths. This spectrum of malformations is similar to that in our study and to those in other reports. Whether the increased prevalence of cardiovascular malformations amon …

Accuracy of fetal echocardiography in detecting lesions associated with, and predicting surgical plan for, dextro-transposition of the great arteries and double outlet right ventricle with subpulmonary ventricular septal defect and predicted transposition physiology.

OBJECTIVES: In dextro-transposition of the great arteries (D-TGA) and double outlet right ventricle with subpulmonary ventricular septal def … 15 with DORV, and one neonate was diagnosed prenatally with DORV-SPV but postnatally diagnosed with truncus arteriosus. In the majority (65%) of patients, serial FE was performed. The percentage concordance between fetal and postnatal echocardiogram diagnoses was high for simple D-TGA (95% (42/44)) and D-TGA with VSD and PS (100% (3/3)). The concordance was progress …

Inborn Errors of Immunity and Cancer.

Inborn Errors of Immunity (IEI) are a heterogeneous group of disorders characterized by a defect in the function of at least one, and often more, components of the immune system. The aim of this narrative review is to discuss the epidemiology, the pathogenesis and the correct management of tumours in patients with IEI. PubMed was used to search for all of the studies published over the last 20 years using the keywords: "inborn errors of immunity" or "primary immunodeficiency" and "cancer" or "tumour" or "malignancy". Literature analysis showed that the overall risk for cancer in children with

Совпадение в полном тексте источника
… Patients with 22q11.2DS have different clinical findings: cardiac anomalies (tetralogy of Fallot, interrupted aortic arch, ventricular septal defect and truncus arteriosus), endocrine anomalies (hypocalcaemia and growth hormone deficiency), palatal anomalies (cleft palate, submucosa cleft palate, velopharyngeal …

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The Genetics and Epigenetics of 22q11.2 Deletion Syndrome.

Chromosome 22q11.2 deletion syndrome (22q11.2del) is a complex, multi-organ disorder noted for its varying severity and penetrance among those affected. The clinical problems comprise congenital malformations; cardiac problems including outflow tract defects, hypoplasia of the thymus, hypoparathyroidism, and/or dysmorphic facial features. Additional clinical issues that can appear over time are autoimmunity, renal insufficiency, developmental delay, malignancy and neurological manifestations such as schizophrenia. The majority of individuals with 22q11.2del have a 3 Mb deletion of DNA on chrom

Совпадение в полном тексте источника
… It can include congenital heart disease (CHD; especially conotruncal malformations, tetralogy of Fallot, aortic arch abnormalities, truncus arteriosus, ventricular septal defects and vascular rings) and abnormalities of the palate (clefts and velopharyngeal incompetence) ( Kobrynski and Sullivan, 2007 …

Abstracts from the World Congress of Cardiology/Brazilian Congress of Cardiology 2022

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СРП 2013

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