Complete Percutaneous Management of Lutembacher Syndrome with Severe Pulmonary Hypertension in a Middle Aged Indian Female: Is this the Magic Wand?

Abstract

Lutembacher syndrome is a combination of ostium secundum atrial septal defect with acquired rheumatic mitral stenosis. This is a case of middle aged woman who had lutembacher syndrome with severe pulmonary hypertension with class IV symptoms. She had very severe mitral stenosis with valve area of 0.7 cm 2 and ostium secundum atrial septal defect of 14 mm size. The management of lutembacher syndrome was only surgical till 1990s. Total percutaneous management has been described in late 1990s. Percutaneous management is relatively cheaper option and saves the patient from artificial valve and related complications. However, only a small number of cases of total percutaneous management of this condition are available in world literature.This patient was managed with percutaneous transmitral commissurotomy with Inoue balloon. Immediately after commissurotomy, her pulmonary artery pressure decreased by one-third. Device closure was done for her atrial septal defect in the same sitting. Her pulmonary artery pressure decreased further. The procedure was successful without any complications. She was symptomatically better on first post-procedure visit. IntroductionLutembacher syndrome is a combination of ostium secundum atrial septal defect with acquired rheumatic mitral stenosis. The management of lutembacher syndrome was only surgical till 1990s. Total percutaneous management has been described in late 1990s. Percutaneous management is relatively cheaper option and saves the patient from artificial valve and related complications. However, only a small number of cases of total percutaneous management of this condition are available in world literature. Case PresentationA fifty-five years old female was referred to our institute from a remote place for double valve replacement (aortic and mitral) with atrial septal defect closure. She had a history of breathlessness on exertion for one year that had progressively increased from class II to class IV. The clinical examination revealed a low grade mid diastolic rumble and loud pulmonary component of second heart sound. A mid systolic murmur was present at left upper parasternal region and a pansystolic murmur was present at left lower parasternal region. The ECG showed normal sinus rhythm with left atrial abnormality. Echocardiogram revealed rheumatic heart disease with very severe mitral stenosis with mitral valve area of 0.