Abstract

Introduction: Lutembacher’s syndrome is a rare clinical entity. In 1916, French physician Rene Lutembacher described his first case of 61 years old woman with a combination of congenital ASD with acquired MS. But he attributed this mitral lesion to congenital mitral stenosis. The definition of this syndrome named after him has undergone many changes since then. The current consensus defines Lutembacher Syndrome (LS) as any combination of ASD (congenital or iatrogenic) and MS (congenital or acquired). The clinical presentation of LS is a result of the interplay between the size of ASD, the severity of MS, pulmonary vascular resistance and RV compliance. Financial constraints also add to the delayed diagnosis and adequate treatment in our part of the world. Here we have discussed a case of LS with severe TR and moderate PAH in congestive cardiac failure which was successfully treated by surgery thereby demonstrating cost-effective and successful surgical management of a rare cardiac disease where vascular resistance, compliance, size and severity of lesions interplay causing cardiac failure.

Case report: A 42 years old thin built, poorly nourished housewife presented with chest discomfort and fatigue for the last 4 years. She was diagnosed with an atrial septal defect four years back but could not receive appropriate treatment due to financial constraints. For the last 15 days, she had breathlessness even at rest and paroxysmal episodes that interrupted her sleep. Icterus and pedal edema was also present. As we evaluated the patient, her clinical examination could not be entirely explained by ASD alone. Soon, our suspicion of LS was confirmed by TTE and the diagnosis of Lutembacher Syndrome with Severe Tricuspid Regurgitation with Moderate PAH was made. As the patient presented to us with atrial fibrillation & heart failure, she was admitted for optimization. The patient was then successfully managed by MVR [#27 SJM, metallic] with pericardial patch closure of ASD with Devaga’s Tricuspid Valve annuloplasty and discharged on a postop day 11.

Conclusions: Early diagnosis and surgical treatment bear a good prognosis and prolong survival. If a patient is diagnosed late, pulmonary hypertension and heart failure set in and the outcome becomes poor. The higher cost of surgical intervention and low socio-economic distribution of the disease in developing countries like India should not challenge the patient’s right to receive the standard treatment.