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Results for “HEART DEFECTS, CONGENITAL”

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PubMed1989

[Morphologic changes of the coronary vessels in patients with congenital heart defects].

Results of the study of the structural changes in the coronary vessels at various levels of ramification in patients with heart defects are presented and their role in the heart compensation and decompensation is shown. 127 hearts with congenital defects and 45 control children's hearts were studied. It is established, by means of anatomical, histologic and morphometric methods, that the heart defects are followed by the hypertrophy of the heart regions involved functionally, this being combined with an adaptive and pathologic rearrangement of coronary arteries and veins. The former is expressed in the vessels hyperplasia, thickening of their wall, and in the appearance in the vascular wall of oblique and longitudinal smooth myocytes (this is of importance for the maintenance of the heart compensation state). The latter is manifested in sclerosis, sometimes also thrombosis of the coronary arteries and veins this creating the basis for the heart failure.

Cardiomegaly↗

PubMed1978

Surgery for congenital heart disease in Ibadan. I: Non-cyanotic heart defects.

Congenital heart disease represents about 5% of all cardiovascular diseases in Nigeria today. Its incidence has increased in the last decade because of improved neonatal care, increased awareness and clinical acumen of physicians, better diagnostic tools and the introduction of newer techniques in cardiac catheterization. It is now possible to make the diagnosis with certainty and to define the extent of the pathology thus making it possible to select the appropriate surgical therapy for these children. This article reviews the commonest types of noncyanotic heart disease, the indications for surgery and the types of surgical procedures available in Nigeria today. The simple extracardiac defects, such as patent ductus arteriosus and coarctation of the aorta can be corrected with little or no mortality, but the more complex intracardiac defects can only be palliated pending the introduction of open heart surgery in Nigeria.

Aortic Coarctation↗

PubMed1990

[The rate and time indices of mitral and tricuspid valve movement during the cardiac cycle in congenital heart defects].

Ultrasonic Doppler echotachocardiogram of the mitral and tricuspid valves together with polycardiogram were recorded in 44 patients with interatrial septal defect and in 18 patients with interventricular septal defect. The patients with interatrial septal defect manifested an increase of the velocity of the movement of the mitral valve during its opening, deceleration of the movement velocity in the atrial systole, a tendency towards movement deceleration during the closure, and a rise of the time of the mitral valve during its closure and opening. In interventricular septal defect, there was a tendency towards deceleration of the movement velocity of the mitral valve in the atrial systole. The velocity and time parameters of the movement of the tricuspid valve remained unchanged in patients with congenital defects under study.

Adolescent↗

PubMed2001

[Electrocardiographic signs of autonomic imbalance in infants with congenital heart defects].

Infants with congenital heart disease (CHD) and heart failure have elevated plasma norepinephrine levels (NE) as a sign for sympathetic activation. We analysed ECGs of 64 infants with CHD and found normal heart rates on average in four groups split up according to their NE. Mean heart rate in Holter ECGs was significantly reduced in infants with low NE (below 350 ng/l) but normal in the other groups (NE < 350 ng/l: 121 +/- 10/min; NE = 350-700 ng/l: 139 +/- 11/min; NE = 700-1300 ng/l: 142 +/- 13/min; NE > 1300 ng/l: 135 +/- 12/min). An analysis of heart rate variability in a subgroup of 25 infants showed significantly reduced values in patients with elevated NE in comparison to 70 healthy infants. Significantly reduced frequency domain measures in infants with elevated NE but also normal NE are evidence for a high diagnostic sensitivity of an analysis of heart rate variability for autonomic imbalance with sympathetic activation and parasympathetic withdrawal in infants with CHD.

Age Factors↗

PubMed1999

Comparison of prenatal ultrasound and postmortem findings in fetuses and infants with congenital heart defects.

OBJECTIVE: Detection of congenital heart defects by prenatal ultrasound examination has been one of the great challenges since the investigation for fetal anomalies became part of the routine fetal examination. This prospective study was designed to evaluate the concordance of prenatal ultrasound findings with autopsy examination in a population consisting of both referred women and non-selected pregnant women. DESIGN: Criteria for inclusion were an ultrasound examination at the National Center for Fetal Medicine and an autopsy performed during the years 1985-94. Results from the ultrasound and autopsy examinations were systematized into categories depending on the degree of concordance. RESULTS: Of 408 infants and fetuses with developmental anomalies, 106 (26%) had congenital heart defects. In 63 (59%) of these 106 cases, the heart defect was the principal reason for the termination of pregnancy or the cause of death. Excluding five cases with a secundum atrial septal defect, there was complete agreement between the ultrasound examination and the autopsy findings in 74 (73%) of 101 cases. In 18 cases, there were minor discrepancies between ultrasound and autopsy findings. The main diagnosis was thus correct in 92 cases (91%). From the first time period (1985-89) to the second (1990-94), the detection rate of all heart defects increased from 48% to 82%. CONCLUSION: This study confirms a good correlation between ultrasound and autopsy diagnoses in fetuses and infants with congenital heart defects. A significant improvement in the detection of heart defects occurred from the first time period to the second and was probably due to increased experience and technical advances.

Abnormalities, Multiple↗

PubMedDate not supplied

[Rate of prenatal detection of congenital right heart defects].

Congenital right heart lesions (including tetralogy of Fallot, pulmonary valve stenosis, pulmonary atresia with intact ventricular septum, Ebstein's anomaly and dysplastic tricuspid valve) account for about 19% of congenital cardiac anomalies. We performed a retrospective study in order to assess the percentage of patients with significant right heart lesions (requiring therapy in the first year of life), which is detected prenatally and referred to a centre for perinatal treatment. From 1/1990 until 12/1997 congenital right heart lesions were diagnosed in 21 fetuses and 190 infants (211 patients. The majority of patients had tetralogy of Fallot (64%), less frequently we found critical pulmonary valve stenosis (9%), pulmonary atresia with intact ventricular septum (9%), tricuspid atresia (14%) and Ebstein's anomaly or dysplastic tricuspid valve (4%). Prenatally the cardiac anomaly was diagnosed in all 21 cases who were referred to our center (10%). The highest referral and detection rate was found among fetuses with Ebstein's anomaly or dysplastic tricuspid valve (5/8 patients = 63%) followed by fetuses with pulmonary atresia and intact ventricular septum (5/20 = 25%), critical pulmonary stenosis (4/18 = 22%) or tricuspid atresia (4/29 = 14%). The prenatal referral rate was disappointing in children with tetralogy of Fallot (3/136 = 2.2%). A higher prenatal detection rate of congenital right heart lesions can be achieved only by an improvement of prenatal screening including the 4-chamber view and the origin of the great arteries. A first step would be the inclusion of the fetal 4-chamber view into the routine examination during the 18th-20th week of pregnancy (stage 1 of a multistage concept of prenatal screening) and by assessment of the outflow tracts and the great arteries in pregnancies associated with risk factors or anomalies of the fetus (stage 2 and 3 of a multistage concept).

Echocardiography↗

PubMed2006

Maternal MTHFR 677C>T is a risk factor for congenital heart defects: effect modification by periconceptional folate supplementation.

AIMS: Periconceptional folate supplementation prevents neural tube defects and possibly congenital heart defects (CHD) as well. The search for candidate genes involved in the folate metabolism includes the methylenetetrahydrofolate reductase (MTHFR) 677C > T polymorphism. We studied the association between MTHFR 677C > T variants and CHD risk. The interaction with periconceptional folate supplementation was also investigated. METHODS AND RESULTS: A case-control study and a family-based transmission disequilibrium test (TDT) were conducted to explore this association. In 133 triads, the TDT revealed no association of the fetal 677T allele with the development of a heart defect. In 158 mothers with a CHD-affected child, the maternal MTHFR 677CT and TT genotypes in combination with no use of periconceptional folate supplements were associated with, respectively, a three-fold (OR 3.3 95% CI 1.46-7.32) and six-fold (OR 6.3 95% CI 2.32-17.27) increased risk for conotruncal heart defects in offspring. In a case-only study, the interaction between periconceptional folate supplementation and maternal MTHFR genotype was significant (P = 0.012). CONCLUSION: The maternal MTHFR 677C > T variants are a risk factor for CHD in offspring, confined to conotruncal heart defects. A gene-environment interaction between maternal MTFHR 677CT and TT genotypes with periconceptional folate supplementation was observed. These findings provide a mechanism of the protective role of folate and support the thesis that periconceptional folate supplementation might prevent CHD.

Adolescent↗

PubMed2005

Congenital heart defects and abnormal maternal biomarkers of methionine and homocysteine metabolism.

BACKGROUND: It is well established that folic acid prevents neural tube defects. Although the mechanisms remain unclear, multivitamins containing folic acid may also protect against other birth defects, including congenital heart defects. OBJECTIVE: Our goal was to establish a maternal metabolic risk profile for nonsyndromic congenital heart defects that would enhance current preventive strategies. DESIGN: Using a case-control design, we measured biomarkers of the folate-dependent methionine and homocysteine pathway among a population-based sample of women whose pregnancies were affected by congenital heart defects (224 case subjects) or unaffected by any birth defect (90 control subjects). Plasma concentrations of folic acid, homocysteine, methionine, S-adenosylmethionine (SAM), S-adenosylhomocysteine (SAH), vitamin B-12, and adenosine were compared, with control for lifestyle and sociodemographic variables. RESULTS: After covariate adjustment, case subjects had higher mean concentrations of homocysteine (P < 0.001) and SAH (P < 0.001) and lower mean concentrations of methionine (P = 0.019) and SAM (P = 0.014) than did control subjects. Vitamin B-12, folic acid, and adenosine concentrations did not differ significantly between case and control subjects. Homocysteine, SAH, and methionine were identified as the most important biomarkers predictive of case or control status. CONCLUSIONS: The basis for the observed abnormal metabolic profile among women whose pregnancies were affected by congenital heart defects cannot be defined without further analysis of relevant genetic and environmental factors. Nevertheless, a metabolic profile that is predictive of congenital heart defect risk would help to refine current nutritional intervention strategies to reduce risk and may provide mechanistic clues for further experimental studies.

Adult↗

PubMed1991

Seasonal differences in the incidence of congenital heart defects.

The incidence of congenital heart defects was studied prospectively in all 664 218 children born in 1977 to 1984. Those suspected of having a heart disease were examined at the centre specialized in pediatric cardiology. All children who died were autopsied and those where a heart defect was proved were included in our series. In total, 4409 infants (6,64/1000 live births) were born with a heart defect in Bohemia. Differences from one year to another were not statistically significant. The incidence of infants with a heart defect was highest in October, lowest in December, June and July (p less than 0.05). Seasonal incidence of individual forms of heart defects differed also. The seasonal variation was not influenced by the total number of live births. The high incidence of congenital heart defects in infants born in October and November coincides with the epidemics of influenza in early pregnancy.

Czechoslovakia↗

PubMed2003

Left-to-right shunting in common congenital heart defects: which patients are eligible for percutaneous interventions?

Atrial septal defects, atrio-ventricular septal defects, ventricular septal defects and the persistent arterial duct are the most common congenital heart defects which may cause, in the presence of a significant left-to-right shunt, chronic volume overload of the heart and lead to the development of pulmonary arterial hypertension. Repair is indicated to avoid these complications and evolution to right-to-left shunting (Eisenmenger syndrome). Although the long-term results of surgical interventions in uncomplicated congenital heart defects were excellent, percutaneous techniques to repair the defects became a focus of attention. The persistent arterial duct, the secundum type atrial septal defect, and, more recently, the muscular and perimembranous ventricular septal defect are currently eligible for percutaneous closure. By avoiding a sternotomy or a thoracotomy, complaints of pain become exceptional and the duration of hospitalisation is shortened. Percutaneous closure is not only better tolerated than surgery, it may also imply favourable economical aspects.

Cardiac Catheterization↗

PubMed1962