Late Presentation of Lutembacher Syndrome with Severe Pulmonary Hypertension and Tricuspid Regurgitation Complicated by Lower Respiratory Tract Infection in an Elderly Female: A Fatal Case Report

Abstract

Lutembacher syndrome is a rare clinical entity, characterized by the coexistence of atrial septal defect and mitral stenosis. The hemodynamic interplay between these lesions often delays symptom onset, making late presentations uncommon. Advanced disease may result in severe pulmonary hypertension, right heart failure, and poor clinical outcomes, especially when compounded by superimposed infections. A 68-year-old female presented with progressive breathlessness for 2 years, recently worsening, along with cough with expectoration and bilateral pedal edema. Clinical examination revealed raised jugular venous pressure, loud pulmonary component of second heart sound, pansystolic murmur, and bilateral crepitations. Investigations demonstrated leukocytosis, elevated inflammatory markers, and type II respiratory failure on arterial blood gas analysis. Chest imaging showed bilateral patchy consolidation suggestive of lower respiratory tract infection. Two-dimensional echocardiography confirmed Lutembacher syndrome with severe pulmonary arterial hypertension, severe tricuspid regurgitation, and right-sided chamber dilation. The patient was managed with oxygen therapy, noninvasive ventilation, intravenous antibiotics, diuretics, and supportive care. Despite intensive management, her condition progressively deteriorated, culminating in cardiopulmonary arrest. This case highlights the rare occurrence of late-presenting Lutembacher syndrome in the elderly and underscores the adverse impact of advanced pulmonary hypertension and superimposed respiratory infection. Early diagnosis and timely intervention are critical to prevent irreversible hemodynamic changes and improve the outcomes in such patients.