Table 1. Spectrum of the morphological right ventricle in congenital heart disease.

1. Subpulmonary RV (pulmonary artery arises from the morphological RV)

a. Pressure loaded RV

(a) Mechanical obstructions in the subpulmonary circulation:

a) Supravalvular pulmonary stenosis

b) Pulmonary valve stenosis

c) Infundibular pulmonary stenosis

d) Double chambered right ventricle

(b) Pulmonary hypertension (including IPAH/HPAH with incidental pre-/post-tricuspid shunt, significant shunt lesions, Eisenmenger syndrome)

b. Volume loaded RV

(a) Pulmonic valve regurgitation (Tetralogy of Fallot)

(b) Tricuspid valve regurgitation (Ebstein anomaly)

(c) ASD

2. Systemic RV (aorta arises from the morphological RV)

a. Biventricular circulation

(a) ccTGA (most common: S.L.L., TGA; less common: I.D.D., TGA)

(b) d-TGA (S.D.D., TGA) after atrial switch repair

b. Univentricular circulation

(a) HLHS

(b) DORV-DIRV with subpulmonary stenosis

(c) (DIRV)

ASD, atrial septal defect; ccTGA, congenitally corrected transposition of the great arteries; DIRV, double inlet right ventricle; DORV, double-outlet right ventricle; d-TGA, dextro-transposition of the great arteries; HLHS, hypoplastisch left heart syndrome; HPAH, hereditary pulmonary arterial hypertension; IPAH, idiopathic pulmonary arterial hypertension; RV, right ventricle.