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 |
Present |
| Fibrosis | Absent | Present |
IVS: inter entricular septum; RVEF: right ventricular ejection fraction; CHD: congenital heart disease; RV-arterial coupling: right ventricular arterial coupling; mROS: mitochondrial reactive oxygen species; p53: p53 protein; HIF-1α: hypoxia inducible factor 1 alpha; VEGF: vascular endothelial growth factor; PDK4: pyruvate dehydrogenase kinase 4; Glut1: glucose transporter 1.