Increased vascular endothelial growth factor in patients with cyanotic congenital heart disease may not be normalized after fontan type operation

AbstractTetralogy of Fallot is the most common cyanotic congenital heart disease with decreased pulmonary blood flow. Right-to-left shunt and infundibular pulmonary stenosis in this disease lead to a decrease in arterial O2 saturation. Hypoxia is a strong stimulus for angiogenesis; however, the reason for insufficiency in the pulmonary vascular growth in patients despite chronic arterial hypoxia is still not known. This study was planned considering that the impairment in vascular endothelial growth factor-receptor relationship or the vascular endothelial growth factor-receptor deficiency in the pulmonary vascular bed during development may cause insufficiency of pulmonary vascular growth. A total of 24 patients were grouped as cyanotic – including 13 patients with tetralogy of Fallot – and acyanotic – including 11 patients with left-to-right shunt lesions. During cardiac catheterisation, vascular endothelial growth factor measurements were performed; and oxygen saturations, pressures, and haemoglobin levels were measured. Perioperative lung biopsy for vascular endothelial growth factor receptors was performed in the cyanotic group. Vascular endothelial growth factor of the aorta was higher in the acyanotic group. There was a significant negative correlation between vascular endothelial growth factor levels and aortic O2 saturation in the cyanotic group (p < 0.05). Vascular endothelial growth factor tissue staining was negative in 11 out of 13 (84.6%) patients. KDR/Flk-1 receptor was positive in four out of 13 (30.7%) patients; Flt-1 receptor was positive in six out of 13 (46.1%) patients. Vascular endothelial growth factor values were found to be lower than those of the acyanotic patients in this study. Low serum vascular endothelial growth factor levels of the cyanotic group, in spite of the hypoxia, demonstrated the importance of studying vascular endothelial growth factor tissue levels and vascular endothelial growth factor receptors in these patients.

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Endothelial function state following repair of cyanotic congenital heart diseases

Cardiology in the Young ◽

10.1017/s104795111300187x ◽

2013 ◽

Vol 25 (2) ◽

pp. 222-227 ◽

Cited By ~ 4

Author(s):

Mohammad Reza Sabri ◽

Hooman Daryoushi ◽

Mojgan Gharipour

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Endothelial Function ◽

Tetralogy Of Fallot ◽

Congenital Heart ◽

Cyanotic Congenital Heart Disease ◽

Vascular Endothelial ◽

Vascular Endothelial Function ◽

Flow Mediated Dilatation ◽

The Mean

AbstractBackgroundRepairing cyanotic congenital heart disease may be associated with preserving endothelial function. The present study aimed to evaluate vascular endothelial function in patients with repaired cyanotic congenital heart disease.MethodsIn a case–control study conducted in 2012 in Isfahan, Iran, 42 consecutive patients aged <35 years who had suffered from different types of cyanotic congenital heart disease and had undergone complete repair of their congenital heart defect were assessed in regard to their endothelial function state by measuring flow-mediated dilatation and other cardiac function indices. They were paired with 42 sex- and age-matched healthy controls.ResultsThe mean flow-mediated dilatation was lower in patients with repaired cyanotic congenital heart disease than in the controls [6.14±2.78 versus 8.16±1.49 respectively (p<0.001)]. Significant adverse correlations were found between flow-mediated dilatation, age, and body mass indexes, in those who underwent repair surgery. In addition, flow-mediated dilatation had a positive association with the shortening fraction, ejection fraction, and tricuspid annular plane systolic excursion value, and it was also inversely associated with carotid intima-media thickness and the myocardial performance index. The mean of the flow-mediated dilatation was significantly higher in the group with tetralogy of Fallot along with complete repair before the age of 2.5 years and also in those patients with total anomalous pulmonary venous connection or transposition of the great arteries repaired with an arterial switch operation before 6 months of age, compared with the other two subgroups. This includes patients with a tetralogy of Fallot defect repaired after 4 years of age and those with complex cyanotic congenital heart disease that was repaired after 2.5 years of age (mean age at repair 9±6.1 years).ConclusionEarly repair of a cyanotic defect can result in the protection of vascular endothelial function and prevent the occurrence of vascular accidents at an older age.

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