Abstract 4139262: Combined Dilation of Rheumatic Mitral and Tricuspid Stenosis in Youngest Patient With Quadrivalvular Disease

Background: Rheumatic heart disease with involvement of all the four valves is extremely rare with anecdotal case reports. Severe symptomatic multivalvular involvement is also rare. Case: A 12-year-old boy presented with gradually progressive dyspnea over two years to current New York Heart Association (NYHA) class IV status. He was hospitalized for a week with fever, sore throat and migratory polyarthralgia three years ago. Examination- pallor, facial and pedal edema, malnutrition (height= 127 cm, weight= 27 kg, body-mass index= 16.8 kg/m 2 ), Harrison’s sulcus, pulse= 90/minute, blood pressure= 104/64 mm of Hg, severe mitral and tricuspid stenosis, severe pulmonary arterial hypertension, and right ventricular failure. Electrocardiogram- sinus rhythm with right axis deviation and QRS morphology consistent with systemic pulmonary hypertension and severe mitral stenosis. X-Ray chest- bi-atrial enlargement with pulmonary arterial and venous hypertension. Echocardiography- severe mitral stenosis (mitral valve area= 0.3 cm 2 ), trivial mitral regurgitation, Wilkins Score= 8/16, mean gradient= 16 mm of Hg (maximum= 40 mm of Hg), doming of the thickened tricuspid valve with severe tricuspid stenosis, thickened aortic valve leaflets with mild aortic stenosis and trivial aortic regurgitation, and doming of thickened pulmonary valve with a c notch and trivial pulmonary regurgitation. There was associated severe pulmonary hypertension (suprasystemic) with right ventricular systolic pressure of 140 mm of Hg. Discussion: In view of young age, malnutrition and a fulminant presentation of severe mitral and tricuspid stenosis, the management hinged around the percutaneous versus the surgical options. Key factors included young age, financial constraints, and repeated reluctance of parents for open heart surgery. The patient underwent a percutaneous dilation of mitral and tricuspid stenosis with an Inoue balloon in a single intervention. Conclusion: We describe quadrivalvular rheumatic heart disease with severe symptomatic mitral and tricuspid stenosis in the youngest ever reported case. The combined balloon dilation of both valves at this age is also not reported.