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The systolic click-murmur syndrome: clinical recognition and management
Insights
Midsystolic click-late systolic murmur syndrome involves mitral valve prolapse due to myxomatous degeneration. Diagnosis is confirmed by echocardiography, with prognosis generally excellent but rare risks of sudden death.
Area of Science:
- Cardiology
- Valvular Heart Disease
Background:
- Midsystolic click-late systolic murmur syndrome stems from myxomatous degeneration of mitral valve leaflets, causing prolapse.
- Other cardiac conditions can mimic this syndrome, presenting with mitral valve prolapse and regurgitation.
Purpose of the Study:
- To elucidate the pathophysiology and clinical presentation of midsystolic click-late systolic murmur syndrome.
- To differentiate this primary syndrome from other causes of systolic clicks and mitral regurgitation.
Main Methods:
- Clinical evaluation including assessment of hemodynamic interventions.
- Echocardiography for confirmation of mitral valve prolapse.
- Cardiac catheterization for evaluating coronary artery disease and mitral regurgitation severity.
Main Results:
- The syndrome can be familial and presents with variable symptoms including chest pain, palpitations, dyspnea, or fatigue.
- Echocardiography confirms characteristic mitral valve leaflet prolapse.
- Prognosis is typically excellent, though severe complications like sudden death or progressive regurgitation can occur.
Conclusions:
- Midsystolic click-late systolic murmur syndrome requires accurate diagnosis, often aided by echocardiography.
- Management includes medical therapy for symptomatic patients and antibiotic prophylaxis.
- Further research is needed to fully understand this complex cardiac entity.
Abstract:
The midsystolic click-late systolic murmur syndrome is a complex entity with variable manifestations that involves a primary process causing myxomatous degeneration of the mitral valve leaflet(s) and subsequent systolic mitral valve leaflet prolapse. Other cardiac diseases may cause mitral valve prolapse and regurgitation associated with a midsystolic click that mimics this primary syndrome. The prolapsing mitral valve leaflet(s) syndrome occasionally may be familial. Most patients are asymptomatic but some complain of chest pain, palpitation, dyspnea or fatigue. Prolapsing mitral valve leaflet(s) can be distinguished from other causes of systolic clicks and mitral regurgitation murmurs by the characteristic movement of the clikmurmur complex in systole with various hemodynamic interventions. The clinical diagnosis usually can be confirmed by echocardiography, which demonstrates the abnormally prolapsdrome usually is minimal but can be progressive and lead to the need for prosthetic valve replacement. Most symptomatic patients can be managed medically but some require cardiac catheterization to evaluate the possibility of coexistent coronary artery disease, to assess the degree of mitral regurgitation and to evaluate other associated cardiac lesions. All patients with this syndrome should receive antibiotic prophylaxis prior to any surgical or dental procedures. Those patients suspected of having arrhythmias should be evaluated by continuous ambulatory ECG monitoring and dangerous arrhythmias probably should be treated. The prognosis usually is excellent, but sudden death and rapidly progressive mitral regurgitation due to ruptured chordae tendineae have been reported. Although more than a decade has elapsed since the midsystolic click-late systolic murmur syndrome was first recognized, much remains to be learned about this common but complex clinical entity.