Tetralogy of Fallot

Objectives: Factors influencing results of balloon valvuloplasty (BVP) of pulmonary valve stenosis (PS) in children are investigated. Background: BVP has become the standard of care for PS, medium-term results are not uniform and depend on various preconditions. Methods: We analysed the medium-term results of BVP of PS in children in an observational, single centre study. Need for additional procedure was defined as outcome after initial BVP. Results: We included 143 children (83 female) at a median (IQR) age of 2.6 (0.26-9.24) months and body weight of 5 (3.4-8) kg at BVP with a follow-up of 5.04 (1.6-10.2) years. We used balloon size of 10 (9-14) mm and maximal balloon pressure of 4 (3.5-10) atm, resulting in balloon-to-pulmonary annulus ratio of 1.28 (1.2-1.4). Systolic pressure gradient of PS was reduced with BVP (43.5 mmHg vs. 14.0 mmHg, p < 0.001) and confirmed by echocardiography (68.0 mmHg vs. 25.0 mmHg, p < 0.001) day 1 post procedure. Pulmonary BVP with associated supravalvular PS resulted in a relevant reduction of systolic pressure gradient in 23 of 31 patients (74.2%). Early additional procedure was necessary in 14 patients (9.8%) after 0.2 (0.1-0.7) years due to residual PS (n = 13) and infective endocarditis (n = 1). Factors for additional procedures were associated supravalvular PS with a higher residual pressure gradient, but not genetic syndrome. During further follow-up of 5.04 (1.6-10.2) years no further additional procedures were needed. Conclusions: Pulmonary BVP of native pulmonary valve stenosis leads to excellent medium-term results, even in 3 of 4 infants with associated supravalvular obstruction sufficient pressure relief can be obtained.

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Congenital Heart Disease in Down Syndrome

Morrison¹,

McMahon²

2018

Advances in Research on Down Syndrome

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Down syndrome remains the most common chromosomal abnormality in live-born infants in the world today. The association between Down syndrome and congenital heart disease (CHD) is well known, and it is widely recognized that CHD contributes significantly to the morbidity of children with Down syndrome. The reported incidence of CHD in Down syndrome patients is between 40 and 60%. The most commonly described defect is complete atrioventricular septal defect (AVSD), which comprises 30-40% of all cardiac defects. Complex genetic factors are involved. Routine cardiac screening of all newborn babies with Down syndrome is recommended. Expert groups suggest that the cardiac status of all children with Down syndrome should be established by 6 weeks of age to permit appropriate and timely treatment avoiding the establishment of irreversible pulmonary vascular disease that would make corrective surgery impossible.

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Tetralogy of Fallot

Cited by 3 publications

References 181 publications

Cardiovascular Imaging

Cardiovascular Imaging

Medium-Term Results of Balloon Valvuloplasty of Native Pulmonary Valve Stenosis with and without Supravalvular Obstruction in Childhood

Congenital Heart Disease in Down Syndrome

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