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Results for “HEART DEFECTS, CONGENITAL”

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PubMed1998

[Status and after-care of young adults with congenital heart defects].

INTRODUCTION: We studied patients with congenital heart defects born in 1975 and followed by our institution. We were interested in the outcome of these patients at the age of 20 years with regard to professional status, physical activity and psychosocial aspects. Furthermore, we investigated the handover from the paediatric to the adult cardiologist as well as the information level concerning antibiotic prophylaxis against bacterial endocarditis. METHODS: From a previous follow-up study to the age of 16 years, we knew 224 patients with congenital heart disease born in 1975, 119 were considered to need further cardiological follow-up. In 112 (94%) we have a complete follow-up to the age of 20 years with a structured interview by phone. RESULTS: Mortality and medical care during the period between 16 and 20 years of age were at a lower level than in the previous periods. Except in patients suffering from complex or surgically treated cyanotic heart disease, the results, compared to the standard population at the age of 20 years, are better with regard to professional status, and equal for physical activity and psychosocial integration. Only 60% of the patients were followed by an adult cardiologist and only 45% of the patients needing antibiotic prophylaxis against bacterial endocarditis are aware of this. CONCLUSION: Except in patients with complex congenital or surgically treated cyanotic heart diseases, the outcome at the age of 20 years with regard to professional status, physical activity and psychosocial integration is very good compared with the standard population at the age of 20 years. The handover to the adult cardiologist is inadequate and knowledge and awareness of antibiotic prophylaxis against bacterial endocarditis is also insufficient-both aspects need improvement.

Adult↗
PubMed2004

Clinical signs of heart failure are associated with increased levels of natriuretic peptide types B and A in children with congenital heart defects or cardiomyopathy.

AIM: To study whether natriuretic peptide types B (BNP) and A (ANP) reflect clinical signs of heart failure (CSHF) in children with congenital heart defects or cardiomyopathy resulting in different types of haemodynamic situations, such as pressure overload in coarctation of the aorta (CoA), volume overload in ventricular septal defect (VSD) or systolic dysfunction in dilated cardiomyopathy (DCM). METHODS: Blood samples for plasma P-BNP and P-ANP were taken before procedures during regular investigation from 26 children (9 CoA, 11 VSD and 6 DCM). The ordinary paediatric cardiologist performed the cardiac evaluation and the data were retrieved from medical charts. CSHF was considered positive if two of the following criteria were fulfilled: reduced physical capacity, feeding disorders, dyspnoea, tachypnoea, hepatomegaly and oedema. The statistical methods were non-parametric. RESULTS: 0/9 children with CoA, 5/11 with VSD and 6/6 with DCM had CSHF. In children with CSHF, P-BNP and P-ANP were higher, 263 ng l(-1) (range 47.5-1300) and 303 ng l(-1) (range 168-466), than in those without CSHF, 12.3 ng l(-1) (range 4.8-30.8) and 42.9 ng l(-1) (range 13.7-189), respectively (p < 0.001, Mann-Whitney U-test), irrespective of the diagnosis. The same relationship was also found in the group of children with VSD. CONCLUSION: Plasma levels of ANP and BNP increase in children with CSHF. This increase is seen irrespective of whether it is due to systolic dysfunction, as in children with DCM, or to a volume overload with a normal systolic function, as in children with VSD.

Adolescent↗
PubMedDate not supplied

Mechanical valve replacement in congenital heart defects in the era of international normalized ratio self-management.

As the number of patients with congenital heart defects requiring heart valve replacement increases, the need for durable valve substitutes with good hemodynamic performance and a low incidence of complications becomes more apparent. The use of porcine xenografts is burdened with early fibrocalcific degeneration, whereas the use of mechanical heart valves led to an increased number of thromboembolic events, especially when implanted in the right side of the heart. We report on our experiences implanting bileaflet heart valves in congenital heart defects since the introduction of international normalized ratio (INR) self-management. The data of 68 long-term survivors (33 males, 35 females) who underwent mechanical heart valve replacement in congenital heart defect were reviewed. Patient age at the time of valve replacement ranged from 5 months to 61 years (mean 21 years). Underlying diagnoses were tetralogy of Fallot (n=33), morbus Ebstein (n=4), atrioventricular canal (n=13), truncus arteriosus communis (n=5), transposition of the great arteries (n=10), and congenitally corrected transposition of the great arteries (n=3). In all patients, bileaflet valves were implanted (St. Jude Medical n=40, Carbomedics n=28). Anticoagulation was performed using dicumarol (Marcumar) and INR self-management in all cases. The mean follow-up was 72 months (range 6-132 months; 409 patient-years). Valve thrombosis developed in 3 of 68 patients (4.4%, all three had tetralogy of Fallot, mean age 9.8 years) after a mean follow-up of 3.5 years. In two of these three patients, re-pulmonary valve replacement was necessary, whereas the third patient was treated by thrombolysis. From our experience, we conclude that mechanical heart valve replacement is a good therapy option with a low complication rate for patients with congenital heart defects requiring valve replacement, especially when INR self-management is performed.

Adolescent↗
PubMed2002

Fetal magnetocardiography in the investigation of congenital heart defects.

OBJECTIVES: To investigate the changes of the fetal magnetocardiography (FMCG), a new noninvasive diagnostic tool in the analysis of electrophysiologic changes of the heart, in cases of congenital heart defect (CHD). METHODS: The FMCG was analysed and compared to the postnatal ECG in eight cases of CHD: atrial septal defect ASDII (three cases), a combination of atrioventricular-septal-defect (AVSD) and Tetralogy of Fallot (TOF) (one case ), complete transposition of great arteries (d-TGA) (two cases), coarctation of aorta (COA) (one case), stenosis of the pulmonary artery (PS) and right ventricular hypoplasia (one case). RESULTS: (1) The following FMCG changes were observed: a split R-wave (AVSD/TOF, ASDII), prolongation of QRS complex (COA, PS). (2) The notch of the R-wave could not be observed in the newborn with AVSD/TOF. (3) Neither the fetal FMCG nor the neonatal ECG revealed any changes in the cases of d-TGA. (4) All other neonatal ECGs were corresponding to the FMCG. CONCLUSIONS: The FMCG can unearth changes of the cardiac electrophysiologic activity in the case of CHD. The method provides additional information concerning the effect of a CHD on the cardiac conductory system. As in the neonate, the FMCG changes do not reflect the severity of the CHD. FMCG cannot serve as a primary diagnostic tool in the case of CHD as compared to echocardiography.

Adult↗
PubMed2002

Craniosynostosis, ectopia lentis, and congenital heart defects: further delineation of an autosomal dominant syndrome with incomplete penetrance.

The association of craniosynostosis with ectopia lentis is extremely rare. This was recently reported in monozygotic twin sisters, supporting a genetic etiology for this syndromic association. We report on female first cousins once removed who were born with unilateral coronal synostosis. One cousin also had peripheral pulmonic branch stenosis at birth and was later found to have ectopia lentis and severe myopia. The other cousin had an atrial septal defect, mitral valve prolapse, and only mild myopia. Their intelligence is normal. The inheritance is likely autosomal dominant with variable expression and incomplete penetrance and further defines this syndrome to include congenital heart defects. These findings will have important implications for genetic counseling.

Adolescent↗