The clinical presentation of patients with Lutembacher syndrome depends on several factors - the size of the defect, severity of mitral valve stenosis and the ability of the right ventricle to overcome increased pressure [1]. Patients may be asymptomatic for decades or they can suffer from severe hemodynamic instability and heart failure early on [2] [3]. An atrial septal defect (ASD) invariably causes left-to-right shunting, while the occurrence of mitral stenosis further increases pressure in the left atria, directly contributing to the transfer of blood into the right heart [2] [3]. Consequently, symptoms arise due to reduced left ventricular output - exercise intolerance, palpitations, and fatigue [3] [4]. Prolonged shunting and severe mitral stenosis (or even insufficiency) can lead to atrial dilation and rhythm abnormalities, such as atrial fibrillation [3]. On the other hand, right ventricular overload and reduced compliance may progress to pulmonary hypertension and congestive heart failure, in which case a poor prognosis is observed [3] [4]. The majority of reports suggest that a predilection toward female gender exists [2] [3], whereas rheumatic fever, particularly in developing countries, seems to be the most important precipitating factor for mitral valve disease [3]. Although approximately 4% of ASDs are accompanied by a mitral valve defect, Lutembacher syndrome is very rarely reported in general practice [2] [4]. Nevertheless, its early recognition may be pivotal, especially in the setting of severe left-to-right shunting and pulmonary hypertension.

• Atrial Septal Defect

  SEPTAL DEFECT and an acquired MITRAL VALVE STENOSIS. [doctor.am]

  Lutembacher syndrome refers to the rare combination of congenital atrial septal defect and acquired mitral stenosis. This condition is usually treated by mitral valve operation with concomitant closure of the atrial septal defect. [ncbi.nlm.nih.gov]

  The haemodynamic effects of this syndrome are a result of the interplay between the relative effects of the atrial septal defect and mitral stenosis. Mitral stenosis augments the left to right shunt through the atrial septal defect. [ajol.info]

  […] with an extremely large atrial septal defect. [unboundmedicine.com]

• Surgical Procedure

  Includes pearls, tips, and memory aids, making it perfect as a handy surgical review for board exams or clinical reference. Covers all of today’s most common surgical procedures and techniques. [books.google.com]

  and Surgical Procedures Myocardial Disease and Cardiomyopathies Pericardial Disease Peripheral Vascular Disease Special Patient Populations Valvular Heart Disease KASUS GANGGUAN KARDIOLOGI Arrhythmias Accelerated Idioventricular Rhythm Arrhythmogenic [dokterairlangga.com]

  Appropriate surgical procedures, be it transcatheter procedures or open heart surgeries should be opted for, after an overall assessment of disease progression and cardiac anatomy. [jpma.org.pk]

  Reported incidence is decreasing because of many fewer cases of rheumatic mitral valve disease, fewer cases of iatrogenic atrial septal defect (ASD) in surgical procedures and more accurate diagnosis since the widespread use of echocardiography. [patient.info]

  In the event that it is detected, devise closure of the iatrogenic defect might prevent an otherwise emergency surgical procedure. References 1. Wiedemann HR. [invasivecardiology.com]

• Inflammation

  […] transcript Síndrome de Dravet tema publicado hace 6 meses mostrar transcripción Letra Peter Pan Syndrome de A Toys Orchestra original Letra Peter Pan Syndrome de A Toys Orchestra en español De Quervain syndrome De Quervain's syndrome consists of an inflammation [context.reverso.net]

  PMID: 27390782 [PubMed] PMCID: PMC4933318 EC Microbiology Reactive Oxygen Species in HIV Infection PMID: 28580453 [PubMed] PMCID: PMC5450819 EC Microbiology A Review of the CD4 T Cell Contribution to Lung Infection, Inflammation and Repair with a Focus [ecronicon.com]

  Dermis showing minimal perivascular inflammation. No eccrine glands or ducts seen. [e-ijd.org]

  Annotated MeSH : disease : coord IM with COMMON BILE DUCT DISEASES (NIM) ; inflammation ; coord IM with CHOLANGITIS (IM) ; neopl : coord IM with COMMON BILEDUCT NEOPLASMS (IM) 91(75) ; was see under VATER'S AMPULLA 1975-90, Reggero Oddiは、１９世紀イタリア（Perugia [sisoken.la.coocan.jp]

• Malnutrition

  A 45-year-old female patient, Jehovah's Witness, was diagnosed with Lutembacher syndrome, New York Heart Association (NYHA) class IV congestive heart failure (CHF) and severe malnutrition. [scielo.br]

  […] with Lutembacher Syndrome as a Bridge to Definitive Surgical Intervention A 45-year-old female patient, Jehovah’s Witness, was diagnosed with Lutembacher syndrome, New York Heart Association (NYHA) class IV congestive heart failure (CHF) and severe malnutrition [elsevier.es]

  Falcão, Nilzo Ribeiro, Lucia Duarte, Fabio Soares, José Carlos Brito ABSTRACT A 45-year-old female Jehovah’s Witness patient was diagnosed with Lutembacher syndrome, New York Heart Association (NYHA) class IV congestive heart failure (CHF), and severe malnutrition [kundoc.com]

  Malnutrition (low intake of proteins and micronutrients), overcrowding, low socioeconomic status, inadequate penicillin prophylaxis and increased genetic susceptibility linked to the human leukocyte antigen (HLA) system may have been contributory [6,13 [escardio.org]

• Short Stature

  STATURE, AND IMPAIRED GLUCOSE METABOLISM + MICROCEPHALY, SHORT STATURE, AND IMPAIRED GLUCOSE METABOLISM 1 MICROCEPHALY, SHORT STATURE, AND IMPAIRED GLUCOSE METABOLISM 2 MICROCEPHALY, SHORT STATURE, AND POLYMICROGYRIA WITH OR WITHOUT SEIZURES Microcephaly-Capillary [rgd.mcw.edu]

• Regurgitation

  There was trace mitral regurgitation and Wilkin’s score was 7/ 16. Patient also had moderate tricuspid regurgitation and severe pulmonary hypertension with calculated right ventricular systolic pressure of 93 mmHg (Figure 2). [peertechz.com]

  Figure 7: Transthoracic echocardiogram color Doppler of patient of Lutembacher's syndrome showing pulmonary regurgitation. [medical-dictionary.thefreedictionary.com]

  Note anteriorly directed jet of moderate-to-severe mitral regurgitation during systole. [emedicine.medscape.com]

  Surgical approach to functional tricuspid regurgitation: Should we be more aggressive? Curr Opin Cardiol 2014;29:133-9. 5. Rogers JH, Bolling SF. Approaches to the management of functional tricuspid regurgitation. J Comp Eff Res 2015;4:665-76. 6. [annals.in]

  We discuss the case of a 24-year-old woman with Lutembacher syndrome and severe tricuspid regurgitation (TR) who underwent surgical closure of atrial septal defect and mitral valve replacement without tricuspid annuloplasty despite a severe TR and a large [ncbi.nlm.nih.gov]

• Heart Disease

  Keywords :Lutembacher's syndrome, congenital heart disease, valvular heart disease, atrial septal defect, mitral stenosis [ajol.info]

  Amprehensive Approach to Congenital Heart Diseases. 1st ed. Jaypee Brothers Medical Publisher, India2013: 908-916 Perloff J.K. The clinical recognition of congenital heart disease. 4th ed. WB Saunders, Philadelphia1994: 323-328 Ananthasubramaniam K. [ajconline.org]

  Guidelines on the management of valvular heart disease: The Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology. Eur Heart J 2007; 28 :230-68. [ PubMed ] [ Google Scholar ] 38. [ncbi.nlm.nih.gov]

  Heart J. 3: 37, 1941. CrossrefMedlineGoogle Scholar 23. DexterHaynesBurwellEppingerSosmanEvans LFWCSECMCJM: Studies of congenital heart disease. III. [acpjournals.org]

  Diagnosis and Management of Adult Congenital Heart Disease, by Drs. Gatzoulis, Webb, and Daubeney, is a practical, one-stop resource designed to help you manage the unique challenges of treating long-term adult survivors of congenital heart disease. [books.google.com]

• Hypertension

  Our patient is in elderly age and already developed pulmonary hypertension, operative procedure is not suitable. So the patient should be kept in conservative treatment. [banglajol.info]

  A 17 year old unmarried female presented with severe Mitral stenosis and a 19 mm almost circular Ostium secundum ASD with moderate pulmonary artery hypertension and dilated right sided chambers. She was managed in a staged manner. [ncbi.nlm.nih.gov]

  @inproceedings{Datta2016CompletePM, title={Complete Percutaneous Management of Lutembacher Syndrome with Severe Pulmonary Hypertension in a Middle Aged Indian Female: Is this the Magic Wand?} [semanticscholar.org]

  The right ventricle will be forced to generate higher pressures to try to overcome the pulmonary hypertension. [marmur.com]

• Fracture

  Patients Candidate for Coronary Artery Angiography … MR Najarzadegan, AR Riazi, H Nough Journal of Shahid Sadoughi University of Medical Sciences 20 (6), 796-804, 2013 1 2013 Management of iatrogenic rupture of profunda femoris artery after femoral fracture [scholar.google.com]

  Second are “button-hole”, densely sclerotic valves with fibrous tissue that need great force to fracture. However, the most common pathologies are commissural, cuspal or chordal fusion or a combination thereof [6,7]. [escardio.org]

• Brachydactyly

  Type A2, With Microcephaly brachydactyly-syndactyly syndrome Brachymesomelia Renal Syndrome Brachymorphism-Onychodysplasia-Dysphalangism Syndrome brachyolmia-amelogenesis imperfecta syndrome Brain-Lung-Thyroid Syndrome branched-chain keto acid dehydrogenase [rgd.mcw.edu]

• Withdrawn

  This is ordinarily not recognized until the end of a mitral valvuloplasty procedure when the catheters are withdrawn and the large puncture is opened. [invasivecardiology.com]

  The device is then withdrawn provided that the connecting stalk is within the ASD and the left disc is adherent to atrial septum. [cdt.amegroups.com]

• Global Developmental Delay

  DEVELOPMENTAL DELAY, ABSENT OR HYPOPLASTIC CORPUS CALLOSUM, AND DYSMORPHIC FACIES Global Developmental Delay, Lung Cysts, Overgrowth, and Wilms Tumor GLUT1 Deficiency Syndrome GLYCOSYLPHOSPHATIDYLINOSITOL BIOSYNTHESIS DEFECT 15 GLYCOSYLPHOSPHATIDYLINOSITOL [rgd.mcw.edu]

Despite its rare nature, Lutembacher syndrome can be diagnosed only if a thorough workup is conducted. Firstly, a detailed patient history should reveal the onset and course of symptoms. Secondly, a meticulous physical examination, particularly cardiac auscultation and inspection of the thorax, must be conducted and serves as one of the key steps in making an initial diagnosis [3] [4]. A loud first heart sound (S1) accompanied by an early-to-mid diastolic murmur is a typical sign of mitral stenosis, whereas splitting of the second heart sound (S2) and presence of third and fourth heart sounds (S3 and S4, respectively), most prominently on the left sternal border during inspiration, are features that may point to ASD [3]. In addition, systolic murmurs at the left parasternal area can be heard on the grounds of increased pulmonary valve flow, holosystolic murmurs increasing during respiration may arise because of tricuspid regurgitation, while continuous murmurs might be encountered as well, the reason being persistent shunting through the ASD [3]. Physical examination can also reveal weak arterial pulses (due to reduced systemic blood flow) and elevated jugular venous pressure [3]. When valid clinical suspicion toward valvular or structural heart disease exists, electrocardiography (ECG), plain chest radiography and echocardiography are necessary to confirm the diagnosis [3]. P wave (bifid P wave in lead II) and QRS complex abnormalities (right bundle branch block, right ventricular hypertrophy, and right axis deviation) can be seen on ECG [3]. In addition, left atrial enlargement and pulmonary congestion may be noted on chest X-rays [3]. Doppler echocardiography, however, is the gold standard in the diagnosis of Lutembacher syndrome, as it may detect altered hemodynamic pathways and visualize ASD and impaired flow through the mitral valve [3].

[…] surgery has been the traditionally accepted modality of treatment. [heartasia.bmj.com]

Although it is traditionally corrected by surgical treatment, both conditions are amenable to transcatheter treatment without the need for surgery. We present a 49-year-old woman with Lutembacher syndrome. [ncbi.nlm.nih.gov]

[…] of Lutembacher’s syndrome for which surgery has been the traditionally accepted modality of treatment. [docksci.com]

61 Cheng TO 10506781 1999 50 Definitive percutaneous treatment of Lutembacher's syndrome. 61 Joseph G...Kumar KP 10506780 1999 [malacards.org]

Lutembacher's syndrome Microchapters Home Overview Historical Perspective Pathophysiology Epidemiology and Demographics Natural History, Complications and Prognosis Diagnosis History and Symptoms Physical Examination Electrocardiogram X-Ray Echocardiography [wikidoc.org]

Prognosis Mortality and morbidity rates are related to the relative severity of the individual lesions. Prognosis is generally good and patients have lived into their ninth decade without developing any cardiac symptoms. [patient.info]

It IS somewhat paradoxic that often, even in persons of advanced years, the question of possible congenital cardiac disease enters prominently into the problems of differential diagnosis and prognosis. [jamanetwork.com]

Qualifiers English: BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology [decs.bvs.br]

Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Pascal 002 Biological and medical sciences / 002B Medical sciences / 002B12 Cardiology. [pascal-francis.inist.fr]

Following atrial septal defect closure, the patient developed pulmonary oedema and on careful search she was found to have tight mitral stenosis of rheumatic etiology. [jpgmonline.com]

In this review, we present two cases, discuss the different definitions, etiologies, clinical presentation and management modalities. [heartviews.org]

Similarly, understanding of the etiology of ASD as associated with Lutembacher syndrome has evolved over time. [emedicine.com]

[…] on Lutembacher's syndrome epidemiology and demographics CDC on Lutembacher's syndrome epidemiology and demographics Lutembacher's syndrome epidemiology and demographics in the news Blogs on Lutembacher's syndrome epidemiology and demographics Directions [wikidoc.org]

English: 2008 (1963) Allowable Qualifiers English: BL blood CF cerebrospinal fluid CI chemically induced CL classification CO complications DI diagnosis DG diagnostic imaging DH diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology [decs.bvs.br]

Epidemiology It is very rare. [patient.info]

His interests are in incontinence in the elderly, the effects of medication and co-morbidity on continence status and the epidemiology and aetiology of incontinence in the elderly. [books.google.com]

The pathophysiology of Lutembacher syndrome is discussed below. [ncbi.nlm.nih.gov]

Lutembacher's syndrome Microchapters Home Overview Historical Perspective Pathophysiology Epidemiology and Demographics Natural History, Complications and Prognosis Diagnosis History and Symptoms Physical Examination Electrocardiogram X-Ray Echocardiography [wikidoc.org]

- This case highlights the importance of understanding the pathophysiology of LS for efficient diagnosis and appropriate management. Early evaluation at a Comprehensive Valve Center is recommended. [ahajournals.org]

A health care provider may recommend the atrial septal defect be closed for a child with a large atrial septal defect, even if there are few symptoms, to prevent problems later in life. [cdc.gov]

[…] diet therapy DT drug therapy EC economics EM embryology EN enzymology EP epidemiology EH ethnology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PS parasitology PA pathology PP physiopathology PC prevention [decs.bvs.br]

This procedure can prevent the morbidity and mortality associated with anaesthesia and cardiac surgery and the psychological trauma of a thoracotomy scar particularly in a female patient, as well as obviate the need for prolonged hospital stay. [casereports.bmj.com]

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]

Those that perform the procedure believe that they are preventing long-term deterioration in cardiac function and preventing progression of pulmonary hypertension. [marmur.com]

1. Olivares-Reyes A, Al-Kamme A. Lutembacher's syndrome with small atrial septal defect diagnosed by transthoracic and transesophageal echocardiography that underwent mitral valve replacement. J Am Soc Echocardiogr. 2005;18(10):1105.
2. Behjatiardakani M, Rafiei M, Nough H, Rafiei R. Trans-catheter therapy of Lutembacher syndrome: a case report. Acta Med Iran. 2011;49(5):327-330.
3. Aminde LN, Dzudie A, Takah NF, Ngu KB, Sliwa K, Kengne AP. Current diagnostic and treatment strategies for Lutembacher syndrome: the pivotal role of echocardiography. Cardiovasc Diagn Ther. 2015;5(2):122-132.
4. Kulkarni SS, Sakaria AK, Mahajan SK, Shah KB. Lutembacher's syndrome. J Cardiovasc Dis Res. 2012;3(2):179-181.