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| {{CMG}} '''Associate Editor-in-Chief:''' [[User:Ayokunle Olubaniyi|Ayokunle Olubaniyi, M.B,B.S]] | | {{Lutembacher's syndrome}} |
| | {{CMG}}; {{AE}} <font color="#777777">{{AO}}</font> |
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| ==Overview== | | ==[[{{PAGENAME}} overview|Overview]]== |
| Lutembacher's syndrome is a rare form of [[congenital heart disease]]. It refers to a combination of congenital [[atrial septal defect]], or even a [[patent foramen ovale]] (PFO) complicated by an acquired [[mitral stenosis]].<ref name=Cecil_400>{{Harvnb|Goldman|2011|pp=400}}</ref> The atrial septal defect is usually a specific type called a [[ostium secundum|secundum]] atrial septal defect. This syndrome was named after René Lutembacher, a french cardiologist.
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| ==Pathophysiology== | | ==[[{{PAGENAME}} historical perspective|Historical Perspective]]== |
| The presence of both ASD and mitral stenosis occuring together, usually modify the clinical and hemodynamic manifestation of each other.
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| The presence of an ASD creates a second exit ([[left-to-right shunt]]) for the blood in the left atrium; consequently reducing the hemodynamic effects of a severe mitral stenosis. In the same fashion, the pressure in the left atrium, pulmonary veins and the pulmonary capillaries decrease if the ASD is large. Therefore, the typical presentation of mitral stenosis as a result of pulmonary venous congestion such as orthopnea, paroxsymal nocturnal dyspnea, hemoptysis and pulmonary edema are attenuated or diminished, and are often substituted by symptoms of low volume output such as weakness and fatigue.<ref name="pmid16198889">{{cite journal| author=Olivares-Reyes A, Al-Kamme A| title=Lutembacher's syndrome with small atrial septal defect diagnosed by transthoracic and transesophageal echocardiography that underwent mitral valve replacement. | journal=J Am Soc Echocardiogr | year= 2005 | volume= 18 | issue= 10 | pages= 1105 |pmid=16198889 | doi=10.1016/j.echo.2005.01.017 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16198889 }} </ref>
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| ==Epidemiology and Demographics== | | ==[[{{PAGENAME}} pathophysiology|Pathophysiology]]== |
| This is a very rare disease. The incidence is 0.001/1000000<ref name="pmid3354470">{{cite journal| author=Berry NS, Bauman JL, Gallastegui JL, Bauma W, Beckman KJ, Hariman RJ| title=Analysis of antiarrhythmic drug concentrations determined during electrophysiologic drug testing in patients with inducible tachycardias. | journal=Am J Cardiol | year= 1988 | volume= 61 | issue= 11 | pages= 922-4 | pmid=3354470 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3354470 }} </ref>. This syndrome is more frequently seen in adults because the mitral stenosis is usually an acquired valvulopathy of rheumatic origin. It is also more commonly observed in female patients because both ASD and MS are more prevalent in this gender.
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| ==Complications and Prognosis== | | ==[[{{PAGENAME}} epidemiology and demographics|Epidemiology and Demographics]]== |
| Complications are usually related to a late diagnosis. They include [[pulmonary hypertension]] and [[heart failure]]. Early diagnosis and surgical treatment has a good prognostic value.
| | [[{{PAGENAME}} epidemiology and demographics#Epidemiology|Epidemiology]] |
| | | [[{{PAGENAME}} epidemiology and demographics#Demographics|Demographics]] |
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| | ==[[{{PAGENAME}} natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| | [[{{PAGENAME}} natural history, complications and prognosis#Natural History|Natural History]] |
| | | [[{{PAGENAME}} natural history, complications and prognosis#Complications|Complications]] |
| | | [[{{PAGENAME}} natural history, complications and prognosis#Prognosis|Prognosis]] |
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| ==Diagnosis== | | ==Diagnosis== |
| ===History and Symptoms===
| | [[{{PAGENAME}} history and symptoms|History and Symptoms]] |
| The presentation depends on the size of ASD, extent of mitral stenosis, compliance of the right ventricle and degree of changes in the pulmonary circulation.<ref name="pmid16198889">{{cite journal| author=Olivares-Reyes A, Al-Kamme A| title=Lutembacher's syndrome with small atrial septal defect diagnosed by transthoracic and transesophageal echocardiography that underwent mitral valve replacement. | journal=J Am Soc Echocardiogr | year= 2005 | volume= 18 | issue= 10 | pages= 1105 | pmid=16198889 | doi=10.1016/j.echo.2005.01.017 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16198889 }} </ref>.
| | | [[{{PAGENAME}} physical examination|Physical Examination]] |
| | | | [[{{PAGENAME}} electrocardiogram|Electrocardiogram]] |
| ===Physical Examination===
| | | [[{{PAGENAME}} X-Ray|X-Ray]] |
| | | | [[{{PAGENAME}} echocardiography|Echocardiography]] |
| ===Chest X Ray===
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| ===Echocardiography===
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| ==Treatment== | | ==Treatment== |
| ===Medical Therapy===
| | [[{{PAGENAME}} percutaneous approach|Percutaneous Approach]] |
| | | | [[{{PAGENAME}} surgical approach|Surgical Approach]] |
| ===Surgery===
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| ==References==
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| {{Reflist|2}} | |
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| {{Congenital malformations and deformations of circulatory system}} | | ==[[{{PAGENAME}} case studies|Case Studies]]== |
| | [[{{PAGENAME}} case study one|Case #1]] |
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| | [[Category:Up-To-Date]] |
| [[Category:Cardiology]] | | [[Category:Cardiology]] |
| | [[Category:Disease]] |
| | [[Category:Medicine]] |
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| {{WikiDoc Help Menu}} | | {{WikiDoc Help Menu}} |
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