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61. Lutembacher's Syndrome: A Comprehensive Review of Pathophysiology, Hemodynamics, and Clinical Management
Authors:
Mohammed Fatah Uddin Kamran
Patil Sanjana Satish
Pravez
Pullurwar Sanskruti Praveen
Sameer Saifi
Seksharia Manas
Thakare Unnati Keshav
Kurmanaliev Nurlanbek
( 1,2,3,4,5,6,7, Student, International Medical Faculty, Osh State University, Osh, Kyrgyzstan
8, Teacher, , International Medical Faculty, Osh State University, Osh, Kyrgyzstan)
Lutembacher's Syndrome (LS) is a rare and intriguing cardiac condition classically defined by the combination of a congenital atrial septal defect (ASD) and acquired mitral stenosis (MS), almost always of rheumatic origin. This unique pathophysiology creates a complex hemodynamic interplay that influences the presentation and progression of the disease. The initial lesion, typically the rheumatic MS, induces elevated left atrial pressure. The subsequent development or presence of an ASD then provides a low-resistance "pop-off" valve, shunting blood from the left to the right atrium. This ameliorates the symptoms of left atrial hypertension and pulmonary venous congestion but leads to significant right ventricular volume overload, pulmonary hypertension, and eventual right heart failure. While the classic definition is specific, the concept has been broadened in modern cardiology to include any combination of a left-to-right interatrial shunt and MS. Diagnosis is confirmed through echocardiography, which delineates the anatomy and hemodynamic consequences. Treatment is primarily surgical or percutaneous, aimed at correcting both defects to normalize intracardiac pressures and flow. This article systematically reviews the etiopathogenesis, clinical presentation, diagnostic criteria, and contemporary management strategies for Lutembacher's Syndrome, highlighting its unique place in cardiovascular pathology.
Keywords: Lutembacher's Syndrome, Atrial Septal Defect, Mitral Stenosis, Rheumatic Heart Disease, Congenital Heart Disease, Hemodynamics, Pulmonary Hypertension.
Lutembacher's Syndrome represents a fascinating confluence of congenital and acquired heart disease within a single patient. Its name is derived from the French cardiologist René Lutembacher, who, in 1916, described a case of mitral stenosis associated with a patent foramen ovale, though similar cases had been reported earlier. The syndrome occupies a unique niche in cardiology, illustrating profound hemodynamic principles through its specific anatomical lesions...
This narrative review was conducted through a systematic search of the available scientific literature. Electronic databases, including PubMed, Scopus, Web of Science, and the Cochrane Library, were queried for relevant articles published from the early 20th century to 2024.
Lutembacher's Syndrome is a compelling clinical entity that exemplifies the complexity of cardiac pathophysiology. The coexistence of a congenital interatrial communication and acquired mitral stenosis creates a unique hemodynamic profile where the decompressive effect of the shunt on the left atrium ameliorates the symptoms of pulmonary congestion but at the expense of imposing a severe volume load on the right heart...
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