Table 1.

Patient Characteristics.

	Mild RV Hypertrophy	Moderate RV Hypertrophy	Severe RV Hypertrophy	RV Failure
Number of patients	3	10	3	5
Age (years)	11.3 ± 16.1	2.8 ± 4.3	25.2 ± 13.8	10.9 ± 6.9
Sex (Male)	33%	50%	33.3%	60%
Diagnoses	PS, VSD/DCRV	TOF, PS, VSD/DCRV	TOF, VSD/DCRV	AVSD, Shone’s complex with PHTN
Surgical Procedure	RVOT muscle bundle resection	RVOT muscle bundle resection	RVOT muscle bundle resection	Heart transplant
Peak RVOT gradient (mmHg)	57.7 ± 2.5	82.1 ± 18.8	119.3 ± 19.1	51.8 ± 32.2
RV fractional area change (%)	46 ± 6.24	49.7 ± 7.67	43 ± 6.08	26.6 ± 3.13
Left Ventricular Ejection Fraction (%)	60 ± 6.76	62 ± 8.22	69 ± 7.45	58 ± 13.86
Medications	-	-	-	Epinephrine, Dopamine, Milrinone

Right ventricular tissue was collected at the time of surgical repair from patients with mild, moderate and severe RV hypertrophy and at the time of heart transplantation from patients with RV failure secondary to congenital heart disease. All patients with mild, moderate and severe hypertrophy had RV pressure overload with preserved systolic function. Patients with RV pressure overload with RV failure had decreased systolic function and were on systemic inotropic therapy. RV – right ventricle; RVOT – right ventricular outflow tract; PS – pulmonary stenosis; VSD – ventricular septal defect; DCRV – double chambered right ventricle; TOF – tetralogy of Fallot; AVSD – atrioventricular septal defect; PHTN – pulmonary hypertension. Data are presented as mean±SD.