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BMJ Case Rep 2022 May 18;15(5). Epub 2022 May 18.

Periodontology, AB Shetty Memorial Institute of Dental Sciences, Mangalore, Karnataka, India.

A woman in her 30s presented with progressive worsening of dyspnoea for 6 months. On evaluation, she was diagnosed with severe rheumatic mitral stenosis (mitral valve area of 0.6 cm) and a large ostium secundum atrial septal defect (21 mm) with a left to right shunt and severe pulmonary artery hypertension. Read More

Braz J Cardiovasc Surg 2022 Apr 18. Epub 2022 Apr 18.

Cardiology Service, Hospital das Clínicas/Universidade Federal de Pernambuco (HC/UFPE), Recife, Pernambuco, Brazil.

J Card Surg 2022 Apr 20;37(4):1066-1068. Epub 2022 Jan 20.

Department of Cardiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

The combination of the atrial septal defect (ASD) and mitral stenosis (MS) is an unusual clinical entity called Lutembacher's syndrome. The hemodynamic interaction between the two cardiac malformations modifies the disease progression of each other. The symptom and progression of MS were thought to be slowed because of the existence of a left-to-right shunt that relived the blood flow through the mitral orifice. Read More

Ann Thorac Surg 2021 Dec 15. Epub 2021 Dec 15.

Department of Cardiovascular Surgery, Akita University Graduate School of Medicine, Hondo 1-1-1, Akita, 010-8543, Japan.

Reverse Lutembacher syndrome is a rare cause of hypoxia characterized by the triad of tricuspid valve stenosis (TS), elevated right atrial pressure, and an interatrial right-to-left shunt. We report a case of pacemaker lead-induced reverse Lutembacher syndrome in a 45-year-old woman who presented with dyspnea. The patient also developed pacemaker lead-induced superior vena cava obstruction accompanied by a right-to-left shunt through systemic-to-pulmonary venous collaterals, which exacerbated the hypoxia. Read More

Am J Cardiovasc Dis 2021 15;11(4):499-503. Epub 2021 Aug 15.

Instituto do Coração, do Hospital das Clínicas, da Faculdade de Medicina, da Universidade de São Paulo Brazil.

Lutembacher syndrome is characterized by the association of mitral stenosis and atrial septal defect (ASD), usually of the ostium secundum type. The association between superior vena cava-type ASD and partial anomalous pulmonary venous connection is unusual and there are few descriptions in the literature. We report this condition in a 24-year-old woman who was admitted to the hospital with a 1-year history of progressive dyspnea and describe the successful surgical repair following mitral commissurotomy and tunneling of the anomalous pulmonary veins to the left atrium, which determines the closure of the ASD and tricuspid repair. Read More

CASE (Phila) 2021 Aug 21;5(4):227-229. Epub 2021 Apr 21.

Aurora Cardiovascular and Thoracic Services, Aurora Sinai/Aurora St. Luke's Medical Centers, Milwaukee, Wisconsin.

Int J Cardiovasc Imaging 2021 Oct 9;37(10):2871-2879. Epub 2021 Jun 9.

Cardiology Department, Mehmet Akif Ersoy Thoracic And Cardiovascular Surgery Training and Research Hospital, University Of Health Sciences Istanbul, Turgut Ozal Bulvarı no:11, 34303, Istanbul, Küçükçekmece, Turkey.

Mitral stenosis (MS) is tolerated for an extended period in patients with atrial septal defect (ASD) known as Lutembacher syndrome due to depressurizing effect. In a similar way, patients with patent foramen ovale (PFO) may have clinical benefits in severe MS. We aim to evaluate the clinical effects of PFO in rheumatic MS. Read More

Heart Surg Forum 2021 Apr 7;24(2):E359-E362. Epub 2021 Apr 7.

Department of Cardiovascular Surgery, Yan'an Affiliated Hospital of Kunming Medical University, Kunming, China.

Background: Lutembacher's syndrome (LS) is a rare cardiovascular anomaly that is defined as any combination of congenital or iatrogenic atrial septal defect (ASD) with congenital or acquired mitral stenosis (MS). The clinical features and hemodynamic effects of LS depend on the balance between ASD and MS.

Case Report: In this case report, we describe a rare case of LS with a huge right atrium in a 39-year-old male patient who was admitted to the hospital with worsening fatigue and breathlessness on exertion. Read More

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

Indian J Thorac Cardiovasc Surg 2018 Oct 30;34(4):516-518. Epub 2018 Apr 30.

Department of Cardiovascular Thoracic Surgery, Meenakshi Hospital, Trichy Road, Tanjore, Tamil Nadu State 613005 India.

Lutembacher's syndrome refers to the rare combination of congenital atrial septal defect, usually secundum type and acquired mitral stenosis. However, the presence of sinus venosus atrial septal defect along with severe mitral stenosis and severe pulmonary hypertension is rarely seen, and this article reports on this rarity with its management. 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