A rare case of severe juvenile rheumatic triple valve disease

摘要

Case presentation: A 13 years old girl presented to us with historyof dyspnea on exertion since 15days. On examination she had apansystolic murmur in mitral area and an ejection systolic murmurin aortic area. ECG done showed RBBB with features suggestiveof biatrial enlargement. Patient did not give any previoushistory of rheumatic fever. Patient was diagnosed to have rheumaticheart disease and was advised to get 2D echocardiography.2D echo showed dilated LA, RA and RV. The anterior mitral leafletwas thick and domed with restricted movement of PML and Dopplerinterrogation revealed a peak gradient of 31 mmHg and meangradient of 22 mm of Hg. Colour Doppler study showed severemitral regurgitation. Aortic valve was thickened and was showingrestricted movement. Doppler study showed mild AR with severeAS (peak gradient 82 mmof Hg and mean gradient of 47 mmof Hg).There was organic tricuspid valve disease with severe TR andsevere TS (peak gradient 14 mm of Hg and mean gradient of7 mm of Hg). Pulmonary valve was normal except for mild PRprobably due to associated severe PAH. Patient also had LV concentrichypertrophy with normal LVEF. Final diagnosis of rheumaticheart disease with severe triple valve involvement withsevere PAH was made and patient was sent for surgery.Discussion: Rheumatic heart disease in India is characterized byrapid progression and death at younger age. Nearly 32% ofpatients die before the age of 20 years. In an autopsy studyconducted on 144 children below the age of 18 years mitral valvewas affected in 100% of cases. Involvement of aortic, tricuspid andpulmonary valves was seen in 63.89%, 54.86% and 12.5% respectively.Multivalvular disease was noted in 75.69% cases; but doublevalve and triple valve disease which possibly would haverequired surgerywas present in only 8.33% and 3.5%cases respectively.This case showed severe rheumatic involvement of aortic,mitral, and tricuspid valve in a juvenile patient with is very rarelyseen.Conclusion: In India, rheumatic fever is endemic and remains oneof the major causes of cardiovascular disease, accounting fornearly 25–45% of the acquired heart disease. This case demonstratessmoldering rheumatic activity that can be seen in Indianchildren at a very young age leading to triple valve involvement.Not only the cost of treatment in such patients are phenomenal,the morbidity and mortality is also enormous. Hence ‘‘Preventionis better than cure’’ is very apt for rheumatic fever.