Congenital | Rheumatic | Carcinoid |
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 | Frequent | 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. |