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Case Report

. 2013; 4(3): 195-197

Juvenile severe mitral stenosis predisposing Eisenmenger syndrome in a case with ventricular septal defect, patent ductus arteriosus, coarctation of aorta & hypoplastic aortic arch: Report of first case of rare association

Soumya Patra, Basant Kumar, Kanchanahalli Siddegowda Sadananda,Santhosh Krishnappa, Harsha Basappa, Manjunath Cholenahalli Nanjappa.

We are reporting the first case of rare association between multiple congenital cardiac malformations
with severe rheumatic mitral stenosis which is an acquired structural cardiac disease. A 16 years old
female patient presented with progressive dyspnoea & cyanosis for the last one month with past history
of recurrent pneumonia since infancy. Physical examination revealed presence of cyanosis, grade I
clubbing, radio-radial & radio-femoral delay, loud & single second heart sound, apical long mid diastolic
murmur and left parasternal ejection systolic murmur. Transthoracic echocardiography revealed severe
rheumatic mitral stenosis, multiple ventricular septal defects (VSD) with bidirectional shunt, hypoplastic
aortic arch, Coarctation of aorta and severe pulmonary hypertension. Transesophageal echocardiography
revealed the same findings along with the presence of moderate mitral regurgitation and 9 mm perimembranous
VSD extending into muscular septum. Cardiac catheterization study confirmed the echocardiographic
findings and demonstrated large patent ductus arteriosus (PDA). We have planned for
high-risk percutaneous transmitral commissurotomy (PTMC) for this patient to decrease the back
pressure on pulmonary vasculature. So that right to left shunt will be decreased and cyanosis will also
improve. But parents refused to give consent for PTMC. She was on treatment with regular penicillin
prophylaxis, diuretics, sildenafil and infective endocarditic prophylaxis. We should be aware of this kind
of complex association between congenital and acquired structure heart disease. Eisenmenger syndrome
could also be a presentation of juvenile severe rheumatic mitral stenosis when it is associated with
congenital shunt lesion like VSD/PDA in our case.

Key words: Juvenile mitral stenosis Eisenmenger syndrome Ventricular septal defects Hypoplastic aortic arch Patent ductus arteriosus

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