Current Knowledge and Recent Advances of Right Ventricular Molecular Biology and Metabolism from Congenital Heart Disease to Chronic Pulmonary Hypertension
Table 2
Common features of functional and dysfunctional remodeled right ventricles in congenital heart disease and pulmonary hypertension.
Characteristics
Functional remodeled right ventricle
Dysfunctional remodeled right ventricle
Morphology
Chambers size
Normal
Dilated (i.e., RV/LV > 0,6)
Free wall thickness
Thick (>5 mm)
Thin
IVS motion
Normal
End-diastolic bowing in the left ventricle
Pericardial effusion
Absent or minimal
Moderate to important
CHD common features
Coarse trabeculation Hypertrophied and muscular moderator band Abnormal tricuspid septal leaflet insertion (mitral valve proximity)
Function
RVEF
Preserved
Decreased
Contractility
Hypercontractility
Decreased
Cardiac index
Preserved
Decreased Bad prognosis < 2 l/min/m2
RV-arterial coupling
Preserved
Uncoupling
Rhythm
Mostly preserved
Arrhythmias
CHD common features
Tricupid and pulmonary regurgitations prior to dilation
Metabolic features
Mitochondria
Adapted sizes and shapes
Small, abnormal shapes, clustered
Mitochondrial function
Increased
Decreased
mROS production
Continuous and Low
High accumulation
Signaling pathway
Down-regulation of p53 Up-regulation of HIF1α-VEGF pathway
Up-regulation of p53 Inhibition of HIF1α-VEGF pathway
Energetic substrates
Carbohydrates > fatty acids High use of PDK4, Glut1 = glycolytic shift
Total substrates deprivation Energy starvation = reversed glycolytic shift
Cellular and Tissular features
Myocytes
Hypertrophied
?
Capillary density
Increased
Rarefaction
Ischemia
Present With role of CHD-associated coronary malformations
