Commissural fusion present at birth

After birth

No commissural fusion

Cusps are mobile or fixed, dysplastic

Mostly mobile

Almost fixed and frozen

Valvular regurgitation is uncommon


Sometimes more than stenosis

Calcific deposits little. Occasionally dense.

More pronounced

No calcification, bright fibrous plaques on endocardial surface of cardiac chambers and valves (composed of smooth muscle cells embedded in acid mucopolysaccharide rich matrix with sparse of collagen fibers and lack of elastic fibers)

Symptoms at neonatal period with cyanosis or remain asymptomatic

Mostly symptomatic with history of febrile episodes and joint pains during childhood

Symptomatic with episodic cutaneous flushing, prolonged diarrhea lepisodes, bronchospasm and labile hypertension

Right ventricular free wall, septal and infundibular hypertrophy severe

Less severe

Hypertrophy is Uncommon, whitish right ventricular endocardial fibrous plaques present

High right ventricular systolic pressures with suprasystemic levels frequent

Suprasystemic level is uncommon

Right ventricular pressures are not much increased

Isolated or associated with other congenital heart defects

May affect all four valves

Usually right sided valves and occasionally all four valves (primary or metastatic lung carcinoid) affected and may require quadruple valve replacement (mechanical valves preferable for both sides)

Pin-point valvular stenosis is usually progressive. Ventricular hypertrophy get regress after valvuloplasty or valve replacement with bioprostheses if calcific

May regress with treatment. Need valvuloplasty or valve replacement. (Bioprostheses for right-sided lesions and mechanical valves for left -sided lesions)

Symptoms may respond to somatostatinanaloges (octreotide, lanreotide). Oral ketanserin for hypertensive crisis and intravenous octreotide to control hypotension.