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Cardiol Res 2018 Dec 7;9(6):385-391. Epub 2018 Dec 7.

Intervention Center, Chung-Ang University Hospital, Seoul 06973, Korea.

Even cardiac surgery has been accepted as the standard therapy for Lutembacher syndrome, a combination of congenital ostium secundum atrial septal defect (ASD) and acquired mitral valve stenosis (MVS), it also owns many limitations and disadvantages. Therefore, seeking for a less invasive therapy with the same efficacy may be worthwhile. Thanks to the development in technology and experience gaining in cardiovascular intervention, the combination of the two proved effective procedures, including percutaneous MVS treatment using balloon valvuloplasty and percutaneous ASD closure using atrial septal occluders, can be utilized as an attractive alternative therapy for these conditions. Read More

J Assoc Physicians India 2018 01;66(1):100-1

Senior Resident, Department of Cardiology, Dr. S.N. Medical College Jodhpur, Rajasthan.

Lutembacher syndrome is a rare cardiac abnormality characterized by a combination of congenital atrial septal defect (ASD) and acquired rheumatic mitral stenosis (MS). Here we report a case of 18-year-old male with Lutembacher syndrome successfully treated percutaneously with transcatheter Accura balloon valvuloplasty and Amplatzer septal occluder device closure. Read More

J Pak Med Assoc 2018 Feb;68(2):340-342

Dow University of Health Sciences, Karachi.

Lutembacher syndrome is characterized by a congenital ostium secundum atrial septal defect and an acquired mitral valve stenosis. We present a similar case in a 31-year old male who came in with orthopnoea, central cyanosis and pedal oedema. Examination revealed cardiac murmurs in tricuspid and apical regions. Read More

Asian Cardiovasc Thorac Ann 2018 Nov 6;26(9):690-693. Epub 2017 Mar 6.

GB Pant Institute of Postgraduate Medical Education and Research, New Delhi, India.

Situs inversus with dextrocardia and Lutembacher syndrome is a rare cardiac anomaly. It is associated with other complex cardiac anomalies and anatomical defects. A 30-year-old woman with this condition underwent mitral valve replacement and closure of a secundum atrial septal defect. Read More

J Heart Valve Dis 2017 05;26(3):368-371

First Department of Cardiology, AHEPA Hospital, Aristotle University, Thessaloniki, Greece.

Atrial septal defects (ASDs) are common immediately after percutaneous mitral commissurotomy (PMC). They are usually small, hemodynamically insignificant, and tend to decrease or disappear within 6 to 12 months. Herein, a case is described of persistent ASD in a patient with mitral valve stenosis who had undergone successful PMC three years previously. Read More

Ann Card Anaesth 2017 Oct-Dec;20(4):456-458

Department of Anaesthesiology, Christian Medical College, Vellore, Tamil Nadu, India.

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 tricuspid annulus on preoperative echo. The pathophysiology of Lutembacher syndrome is discussed below. The utility of perioperative echocardiography in assessing the annular diameter, tenting area and coaptation depth and thus providing insights into the functioning of the tricuspid valve will also be emphasized. Read More

Indian Heart J 2017 Jan - Feb;69(1):20-23. Epub 2016 Jul 9.

Dr. PK Sen Department of Cardiovascular & Thoracic Surgery, Seth GS Medical College and KEM Hospital, Mumbai, India.

The mitral valve disease (MVD) in Lutembacher's syndrome has been infrequently analyzed from a pathological standpoint. In this study, we have attempted to elucidate the pathology of MVD in this interesting syndrome in 44 autopsied cases of combined non-primum atrial septal defect (ASD) and MVD collected over 16 years. The patients were divided into 3 groups: Group 1: non-primum ASD with clinically diagnosed mitral stenosis (MS)±regurgitation, Group 2: non-primum ASD with clinically diagnosed mitral regurgitation (MR) and, Group 3: non-primum ASD with no clinically evident MVD, but with mitral valve pathology diagnosed at autopsy. Read More

Sudan J Paediatr 2017 ;17(2):64-67

(2) Sudan Heart Center & Department of Paediatrics and Child Health, University of Khartoum, Sudan.

Rheumatic heart disease (RHD) is the most common cause of acquired heart disease in children and young adults in developing countries. It results from throat infection with group A beta hemolytic streptococcus that proceeds to acute rheumatic fever (ARF). We report a 13 years old girl from Darfur presenting with recurrent acute rheumatic fever for 4 years that led to affection of all her heart valves with severe mitral and tricuspid regurgitation together with moderate pulmonary and mild aortic valve regurgitation. Read More

J Clin Diagn Res 2016 Oct 1;10(10):OD08-OD09. Epub 2016 Oct 1.

Assistant Professor, Department of CVT, Kasturba Medical College, Manipal University , Manipal, Karnataka, India .

Lutembacher syndrome is defined as a combination of congenital Atrial Septal Defect (ASD) with an acquired Mitral Stenosis (MS). There are various challenges involved in the percutaneous management of Lutembacher syndrome. Here, we present a case that had a very small Left Atrium (LA) and Left Ventricle (LV) cavities with an anteriorly placed ASD. Read More

Cardiovasc Diagn Ther 2015 Apr;5(2):122-32

1 Department of Internal Medicine, Faculty of Health Sciences, University of Buea, Buea, Cameroon ; 2 Clinical Research Education, Networking and Consultancy, Cameroon ; 3 Department of Internal Medicine, Cardiology Unit, General Hospital Douala, Douala, Cameroon & Department of Medicine, Faculty of Health Sciences, University of Cape Town, South Africa ; 4 Global Health Systems Solutions, Limbe, Cameroon ; 5 Department of Medicine, Cardiology Unit, University Teaching Hospital, Yaoundé, Cameroon ; 6 Department of Medicine, Groote Schuur Hospital, Cape Town, South Africa ; 7 Hatter Institute for Cardiovascular Research in Africa, University of Cape Town, South Africa ; 8 Non-Communicable Diseases Research Unit, South African Medical Research Council and University of Cape Town, South Africa.

Lutembacher syndrome (LS) is a rare cardiac abnormality characterized by any combination of a congenital or iatrogenic atrial septal defect (ASD) and a congenital or acquired mitral stenosis (MS). Clinical features and hemodynamic effects of LS depend on the balance of effects of the MS and the ASD. Prognosis is influenced by several factors [pulmonary vascular resistance, right ventricle (RV) compliance, size of ASD and MS severity] but the occurrence of secondary pulmonary hypertension and congestive heart failure is commonly associated with poor outcome. Read More

Minerva Cardioangiol 2014 Oct;62(5):433-6

Department of Cardiology, SVIMS, Tirupati, Andhra Pradesh, India -

Kardiol Pol 2014 ;72(7):660

Gumussuyu Military Hospital, Department of Cardiology.

Cardiovasc Diagn Ther 2014 Jun;4(3):263-6

1 Department of Internal Medicine, Faculty of Health Sciences, University of Buea, Buea, Cameroon ; 2 Department of Internal Medicine, General Hospital Douala, Douala, Cameroon ; 3 St Elisabeth Catholic General Hospital, Cardiothoracic Centre, Shisong, Cameroon.

Introduction: Lutembacher syndrome (LS) is a rare cardiac clinical entity marked by the combination of an atrial septal defect (ASD) and mitral stenosis (MS). Its prognosis is influenced by several factors.

Case Report: We present the case of a young adult male who presented with a 10-month history of exertional dyspnea, orthopnoea, fatigue and cough. Read More

Indian Heart J 2014 May-Jun;66(3):355-7. Epub 2014 May 15.

Professor, Department of Cardiology, Dr RML Hospital and PGIMER, New Delhi, India. Electronic address:

Background: The surgical management of Lutembacher syndrome is straight forward but percutaneous management, though technically demanding, is always desirable.

Methods: 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. Read More

J Card Surg 2014 Jul 15;29(4):569-71. Epub 2014 May 15.

Fortis Escorts Heart Institute, New Delhi, India.

Lutembacher's syndrome is an uncommon combination of a congenital ostium secundum atrial septal defect (ASD) with acquired mitral stenosis (MS). The incidence of this condition is very rare. The symptoms are dependent upon the size of the ASD, severity of the MS, compliance of the right ventricle and pulmonary artery hypertension. Read More

Ann Pediatr Cardiol 2014 Jan;7(1):37-40

Department of Cardiology, Advanced Cardiac Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

An 18-year-old male with Lutembacher's syndrome underwent balloon mitral valvotomy (BMV) and device closure of the atrial septal defect (ASD). BMV necessitated technical modification of taking the Inoue balloon over the wire (OTW) into the left ventricle (LV). The procedure was complicated by slippage of ASD device into the right atrium, which was managed successfully by percutaneous retrieval, and deployment of a larger device. Read More

Pediatr Cardiol 2013 Apr 25;34(4):1050-1. Epub 2012 Oct 25.

Department of Cardiology, All India Institute of Medical Sciences, Cardiothoracic Centre, New Delhi, 110029, India.

Pediatr Cardiol 2013 13;34(8):1985-8. Epub 2012 Oct 13.

Department of Pediatric and Congenital Heart Diseases, Fortis Escorts Heart Institute, 123, Anand Kunj, Vikas Puri, New Delhi, 110018, India,

Lutembacher syndrome involving the association of congenital atrial septal defect (ASD), usually of the ostium secundum variety, and mitral valve disease is a well-known entity. Its association with a coronary sinus, ASD, and a persistent left superior vena cava (LSVC) draining into the left atrium (LA) (Raghib syndrome) is rarely described in the literature. This association in a 15-year-old boy erroneously deemed to be inoperable before referral to the authors' hospital due to cyanosis in the presence of atrial septal defect (ASD) and mitral stenosis is described in this report. Read More

J Coll Physicians Surg Pak 2012 Oct;22(10):666-8

Department of Medicine, Mayo Hospital, Lahore, Pakistan.

Lutembacher syndrome is a rare combination of atrial septal defect (ASD) and mitral stenosis. Symptoms depend on the size of ASD, extent of mitral stenosis and degree of changes in the pulmonary circulation. Presentation can be due to cardiac failure, atrial arrhythmias, dyspnoea, exercise intolerance, paradoxical emboli or other disease related complications like pulmonary hypertension and infective endocarditis. Read More

J Cardiovasc Dis Res 2012 Apr;3(2):179-81

Department of Medicine, Krishna Institute of Medical Sciences University, Dhebewadi Road, Karad, Dist. Satara, Maharashtra, India.

The definition of Lutembacher's syndrome has undergone many changes. It refers to combination of congenital Atrial Septal Defect with acquired mitral stenosis. Lutembacher's syndrome is a very rare disease and in the past, it has been either overdiagnosed or misdiagnosed. Read More

Heart Views 2011 Jul;12(3):107-11

Department of Medicine, Mahatma Gandhi Institute of Medical Sciences, Sewagram, Wardha, Maharashtra, India.

Incidence of congenital cardiac anomalies in dextrocardia with situs inversus is low as compared to congenital cardiac anomalies in isolated dextrocardia. We describe the first ever case of situs inversus with dextrocardia, Lutembacher's syndrome, and pericardial effusion. The pericardial effusion in our case was acquired and was tubercular in etiology. Read More

J Invasive Cardiol 2012 Mar;24(3):E54-6

Department of Cardiology, Yashoda Hospital, Secunderabad, India.

Most cases of combination congenital cardiac anomalies are treated with open-heart surgeries because the coexisting anomalies change the cardiac anatomy in an adverse way, making catheter manipulations complex. Lutembacher syndrome is a combination of acquired mitral stenosis and congenital ostium secundum atrial septal defect. The large defect in the septum makes an Inoue balloon catheter unstable, which provides excessive space for free floatation of the catheter, making its passage into the left ventricle difficult by Inoue technique. Read More

Medicina (B Aires) 2011 ;71(4):366-8

Servicio de Cardiología, Hospital Privado Centro Médico de Córdoba, Argentina.

We report the case of an 82 year-old woman with symptoms of advanced heart failure and pulmonary arterial hypertension. An echocardiogram showed an ostium secundum type atrial septal defect and concomitant mitral valve stenosis (Lutembacher syndrome). Echocardiographic assessment of mitral pathology was hampered by the interatrial septal defect. Read More

Acta Med Iran 2011 ;49(5):327-30

Department of Pediatrics, Division of Pediatric Cardiology, Yazd University of Medical Science, Yazd, Iran.

Lutembacher syndrome refers to the rare combination of a congenital atrial septal defect and acquired mitral stenosis. Traditionally, Lutembacher syndrome has been corrected by surgical treatment. We describe two patients treated percutaneouly with a combined Inoue balloon valvuloplasty and septal defect closure using the Amplatzer septal occlusion device. Read More

Circulation 2011 Feb;123(5):e222-4

Northwestern Memorial Hospital, 201 E Huron, Chicago, IL 60611, USA.

Chest 2011 Feb;139(2):443-445

Division of Cardiovascular Diseases, Department of Medicine, University of Miami Hospital, Miami, FL.

We describe an unusual case of orthodeoxia platypnea syndrome exacerbated by right ventricular inflow obstruction due to iatrogenic steroid-induced adipose deposition in cardiac tissues. A 68-year-old man on long-term prednisone therapy for eosinophilic pneumonia presented with progressive dyspnea worsened by bending forward. By using pulse oximetry, he was noted to have positional hypoxemia. Read More

Turk Kardiyol Dern Ars 2010 Jan;38(1):47-9

Department of Cardiology, Medicine Faculty of Ankara University, Ankara, Turkey.

Lutembacher syndrome is a rare combination of congenital atrial septal defect (ASD) and acquired mitral stenosis (MS). 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. Read More

Cardiovasc Intervent Radiol 2009 May 13;32(3):563-7. Epub 2008 Sep 13.

Dipartimento di Malattie Cardiovascolari, Ospedale Civile, Via Riva Ospedale, Cittadella, Padova, 35013, Italy.

We report the case of a very large anomalous connection of the veins draining the upper lobe of the left lung to both the left-sided vertical vein and the left atrium, associated with mild rheumatic mitral valve stenosis, in which the atrial septum was intact and the remaining venous system, including the coronary sinus, was otherwise normal (a variant of Lutembacher's syndrome). In order to abolish the left-to-right shunting, a transcatheter approach to close this venous structure was successfully attempted using an Amplatzer ASD Occluder device. The technical aspects and the alternative options of performing a procedure with a device for a purpose outside the scope of its approved label are discussed. Read More

J Coll Physicians Surg Pak 2008 Feb;18(2):105-6

Department of Cardiology, Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi.

Lutembacher's syndrome is a rare combination of atrial septal defect (ASD) and rheumatic mitral stenosis. Traditionally, this condition is treated surgically. We present a case of Lutembacher's syndrome that was successfully treated with percutaneous transcatheter mitral commissurotomy (PTMC) using the Inoue balloon. Read More

Echocardiography 2008 Apr 3;25(4):436-9. Epub 2008 Jan 3.

Department of Cardiology, King Fahd Armed Force Hospital, Jeddah, Saudi-Arabia.

Lutembacher's syndrome is a rare combination of congenital atrial septal defect and mitral stenosis (almost always rheumatic). The hemodynamic effect of this combination makes the clinical diagnosis difficult due to a wide variation of clinical presentation. Echocardiography and cardiac catheterization are useful tools for proper diagnosis and planning of the therapeutic strategy. Read More

Clin Cardiol 2007 Oct;30(10):518-21

Department of Cardiology, Southwest Hospital, The Third Military Medical University, Chongqing, China.

Objective: To investigate the efficiency and safety of transcatheter interventional therapy for compound congenital cardiovascular abnormalities.

Methods: From Nov 2001 to Jun 2006, a total of 36 patients (17 male, 19 female), aged 17.20 +/- 10. Read More

J Chin Med Assoc 2007 Jun;70(6):253-6

Section of Pediatric Cardiology, Department of Pediatrics, Taichung Veterans General Hospital, Taiwan, R.O.C.

Lutembacher syndrome is a combination of congenital atrial septal defect (ASD) and acquired mitral stenosis (MS). The combination of these 2 diseases has hemodynamic influences on each other and the degree of MS may be underestimated. Traditionally, Lutembacher syndrome is corrected by surgical treatment. Read More

Ann Cardiol Angeiol (Paris) 2006 Jun;55(3):153-6

Service de cardiologie A, hôpital Ibn-Sina, CHU de Rabat-Salé, Rabat, Maroc.

Introduction: Lutembacher syndrome refers to the rare combination of congenital atrial septal defect and acquired mitral stenosis. This condition is usually treated surgically by mitral valve operation with concomitant closure of the atrial septal defect.

Materials And Methods: Between 1993 and 2003, 4 patients with congenital Lutembacher syndrome had percutaneous mitral commissurotomy without closure of the atrial septal defect at our institution. Read More

Ann Thorac Cardiovasc Surg 2006 Feb;12(1):66-70

The Department of Cardiovascular Surgery, Nihon University School of Medicine, Tokyo, Japan.

The patient was a 29-year-old woman who, when she was an 8-year-old, had undergone atrial septal defect (ASD) closure and mitral valve replacement (MVR) using a Björk-Shiley valve (25 mm) for Lutembacher syndrome. Because of a planned pregnancy, warfarin had been replaced by heparin. During the 7th week of pregnancy, she was admitted to our hospital because of dyspnea. Read More

Chin Med J (Engl) 2006 Feb;119(4):351-2

Chin Med J (Engl) 2005 Nov;118(21):1843-5

Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha 410011, China.

J Am Soc Echocardiogr 2005 Oct;18(10):1105

Cardiology Department, The Edward W. McCready Memorial Hospital, Crisfield, Maryland, USA.

Lutembacher's syndrome is a rare clinical combination of congenital ostium secundum atrial septal defect associated with acquired mitral valve stenosis (usually rheumatic). This unusual cardiac entity is difficult to diagnose clinically because each lesion alters the hemodynamics and clinical characteristics of the other. The resulting clinical manifestations depend chiefly on the size of the defect, the severity of the mitral stenosis, and the compliance of the right ventricle. Read More

Mymensingh Med J 2005 Jul;14(2):206-8

Department of Cardiology, Mymensingh Medical College.

A 56 years old farmer from Churkhai, Mymensingh was admitted in Cardiology unit of Mymensingh Medical College Hospital 24 October, 2004 with the complaints of progressive breathlessness on exertion with the repeated respiratory tract infection. He had 3 episodes of multiple large joints swelling involving knee, ankle, wrist, during his childhood with spontaneous recovery without any residual deformity. At the age of 45 years, he was incidentally diagnosed as enlarged heart by a medical board, when applied for Foreign Service. Read More

J Invasive Cardiol 2004 Nov;16(11):678-9

Hospital Star Médica Morelia, Morelia Michoacán.

Heart Lung 2004 Jan-Feb;33(1):50-4

Department of Medicine, New Jersey Medical School, Newark, New Jersey, USA.

We present a case of a 53-year-old woman with intractable shortness of breath that was originally ascribed to bronchiolitis obliterans organizing pneumonia. Subsequently evaluation by echocardiography and cardiac catheterization revealed that she had Lutembacher's syndrome, an uncommon combination of congenital atrial septal defect (ASD) and acquired mitral stenosis that is difficult to diagnose clinically. Our case illustrates the pitfalls and advantages of echocardiographic assessment of the mitral valve area (MVA) and the left atrial pressure (LAP). Read More

Intern Med J 2003 May-Jun;33(5-6):259-60

Saudi Med J 2003 Mar;24(3):307-8

Section of Invasive Cardiology, Department of Cardiology, King Fahad Armed Forces Hospital, PO Box 8649, Jeddah 21492, Kingdom of Saudi Arabia.

Lutembacher syndrome is the combination of congenital atrial septal defect and acquired mitral stenosis. The condition is usually treated surgically. We describe a patient treated percutaneously with a combined Inoue balloon valvuloplasty and septal defect closure using the Amplatzer septal occlusion device. Read More

Zhonghua Wai Ke Za Zhi 1999 Dec;37(12):747-8

Department of Cardiac Surgery, Guangdong Provincial Cardiovascular Institute, Guangzhou 510100.

Objective: To compare classical and acquired Lutembacher's syndrome (mitral restenosis after percutaneous balloon mitral valvuloplasty) in attempt to know their different in pathophysiology, diagnosis, and surgical treatment.

Methods: The data from 22 cases of classical and acquired Lutembacher's syndrome who underwent surgical treatment in the same period were analyzed.

Results: No death occurred in the classical group. Read More

J Am Soc Echocardiogr 2001 Oct;14(10):1033-5

Henry Ford Heart and Vascular Institute and the Department of Internal Medicine, Detroit, Michigan 48202, USA.

Lutembacher syndrome is an unusual clinical entity of congenital secundum atrial septal defect in combination with rheumatic mitral stenosis. Although this classic form is seldom seen by the adult cardiologist, spontaneous Lutembacher syndrome as discussed later or the iatrogenic variant is not infrequently encountered. The pathophysiologic, clinical, and hemodynamic differences of mitral valve disease in the presence of atrial septal defect compared with isolated mitral stenosis are highlighted in this case review. Read More

J Invasive Cardiol 2001 Mar;13(3):223-6

Department of Cardiovascular Disease, Cittadella Civic Hospital, Padua, Italy.

We describe a 70-year-old woman who underwent successful percutaneous Inoue antegrade-technique mitral valvuloplasty. Three months later, the patient developed right-sided heart failure. Color Doppler echocardiography and cardiac catheterization demonstrated an atrial septal defect with bidirectional shunting and no restenosis of the mitral valve (iatrogenic Lutembacher's syndrome). Read More

Catheter Cardiovasc Interv 2000 May;50(1):68-70

Department of Cardiology, Grantham Hospital, Hong Kong, China.

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. We describe a case of Lutembacher syndrome that was treated successfully with percutaneous transcatheter mitral commissurotomy using the Inoue balloon and closure of the atrial septal defect with the Amplatzer atrial septal defect occluder. Read More

Catheter Cardiovasc Interv 1999 Oct;48(2):205-6

Catheter Cardiovasc Interv 1999 Oct;48(2):199-204

Department of Cardiology, Christian Medical College Hospital, Vellore, India.

Definitive percutaneous treatment of a patient with Lutembacher's syndrome was successfully accomplished using the Amplatzer septal occluder to close a secundum atrial septal defect and the Joseph mitral balloon catheter to dilate rheumatic mitral valve stenosis. Transcatheter therapy is an effective alternative to surgery in selected patients with Lutembacher's syndrome. Cathet. Read More

Am J Med Genet 1999 Mar;83(3):193-200

Unité de Génétique Médicale, Faculté de Médecine, Université Saint-Joseph, Beirut, Lebanon.

We report on a Lebanese family in which 12 persons had an atrial septal defect and various cardiac and noncardiac anomalies. Cardiac anomalies are left axis deviation of QRS, right bundle branch block, atrial fibrillation, Wolff-Parkinson-White syndrome, nodal atrioventricular rhythm, aortic stenosis, pulmonic valve stenosis, mitral stenosis (Lutembacher syndrome), and low implantation of the tricuspid valve (Ebstein disease). Noncardiac abnormalities consisted specially of the presence of hypertelorism, cleft lip, and pectus excavatum. Read More