A Case Series on Successful Surgical Management of Patients Presenting With Lutembacher's Syndrome to a Tertiary Healthcare Center in Northern India.

Atrial septal defect (ASD), whether congenital or iatrogenic, and mitral stenosis (MS), whether congenital or acquired, may all come together into a condition called Lutembacher's syndrome (LS). The ASD is typically larger than 15 mm in a normal LS case. However, congenital ASD is less frequent than residual iatrogenic ASD in the current era of percutaneous balloon mitral valvuloplasty for acquired MS. This is referred to as iatrogenic LS by cardiologists. Hereby, we report a case series of three patients who presented to us, were diagnosed with LS, a very rare entity, and were managed successfully.

Neuraxial anesthesia in Lutembacher's syndrome? Yes, we can!

Lutembacher's syndrome is a rare congenital cardiac syndrome comprising of a combination of an atrial septal defect complicated by congenital or acquired mitral stenosis. The applied physiology of the patient depends upon the severity and the interactions of the lesions. They pose certain difficulties to the administration of both general or neuraxial anesthesia. A preference of one form of anesthesia over the other should be based on the understanding of the physiology of the patient. There should not be an orthodox avoidance of neuraxial anesthesia in complex cardiac pathologies as general anesthesia can be associated with certain complications of its own. Here, we report our successful experience of neuraxial anesthesia being administered in a patient with Lutembacher's syndrome.

A Rare Case of Severe Isolated Right Heart Failure with Secundum Type Atrial Septal Defect and Mitral Regurgitation Without Pulmonary Hypertension.

Typically, right heart failure (RHF) may occur following left heart failure (LHF) in chronic volume overload states such as chronic severe mitral regurgitation (MR) through chronically elevated pulmonary pressures. In Lutembacher syndrome (LS), the direct shunting through a secundum type atrial septal defect (ASD) results in congestive heart failure in the setting of severe mitral stenosis (MS) with or without elevated pulmonary arterial or venous pressures. We report a rare case of severe isolated RHF and bi-atrial enlargement resulting from the direct shunting through a secundum type ASD in the presence of a severe eccentric primary MR. There are no significant cases documented like this after a thorough search using PubMed, Medline, and Google Scholar. A review of the literature suggests that LS is also caused by a combination of mitral regurgitation and a secundum-type atrial septal defect without mitral stenosis, though rarely. Because this is a primary MR, we feel it is a case of LS with MR, ruling out a combination of secondary MR and secundum-type atrial septal defect.

Lutembacher syndrome in a middle-aged woman in Ghana with an extremely large atrial septal defect.

Lutembacher syndrome (LS) is a rare heart disorder characterized by a congenital or acquired combination of the atrial septal defect (ASD) and mitral stenosis. In LS, patients may be asymptomatic for years, but early detection and treatment result in a better prognosis. In contrast, the prognosis is usually poor, with conservative treatment if the diagnosis is late and the patient develops heart failure and pulmonary hypertension. Although rheumatic heart disease (RHD) and congenital heart disease are prevalent in Ghana, cases of LS are not reported. Here, we report the case of a 45-year-old woman with rheumatic mitral valve stenosis and an exceptionally large ASD with bidirectional flow who was diagnosed with LS and treated conservatively for heart failure at a cardiology clinic in Ghana.

Lutembacher syndrome presenting as heart failure in a young Nigerian at Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife: a case report.

Lutembacher syndrome (LS) is a rare syndrome comprising a combination of atrial septal defect (ASD) and mitral stenosis. We present the case of a 28-year-old man, who presented with progressively worsening dyspnea of 2 months associated with orthopnea, paroxysmal nocturnal dyspnea, bilateral leg swelling and productive cough. Chest X-ray revealed plethoric lung fields with prominent pulmonary conus and cardiomegaly. Transthoracic echocardiography revealed a large ostium secundum ASD with left to right shunt, mild mitral stenosis, severe mitral and tricuspid regurgitations and pulmonary hypertension. A diagnosis of Lutembacher syndrome in heart failure with pulmonary hypertension was made. The patient was managed conservatively, but declined surgery primarily because of financial reasons. This rare case of LS presenting with heart failure and complicated by pulmonary hypertension is the first reported case in our centre and our region. The patient's inability to afford the cost of definitive care posed a significant problem in his management.

Rapid progression of mitral stenosis after atrial septal defect closure in a Lutembacher's syndrome patient.

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. There is no consensus about caring this patient population for now. Here, we present a 58-year-old female with mild MS and coexistent ASD experiencing rapid progression of mitral valve lesions after percutaneous ASD closure. This case might identify the effect of ASD on delaying MS progression. From this point of view, MS and coexisting ASD should be evaluated and treated as a whole.

Total surgical correction of Lutembacher syndrome associated with partial anomalous connection of the pulmonary veins and tricuspid regurgitation.

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.

Lutembacher syndrome with mitral valve calcification in a 31-year old male.

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. Chest x-ray showed signs of pulmonary congestion and ventricular enlargement. Electrocardiogaphy (ECG) revealed right axis deviation and right bundle branch block along with atrial fibrillation and Transthoracic Echocardiography (TTE) showed abnormal valves (mitral stenosis with calcification and tricuspid regurgitation) and dilated cardiac chambers. The patient was consequently treated with beta-blockers and diuretics and scheduled for valvular and septal repair via open heart surgery. The purpose of this case report is to assist cardiologists in diagnosing this syndrome accurately on the basis of symptoms and investigations.

Combined Percutaneous Procedure in Patient with Lutembacher Syndrome: A Case Report and Real-World Experience Review.

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. Here, we present a successful percutaneous intervention in Lutembacher syndrome using the combination of mitral balloon valvuloplasty and ASD device closure and thoroughly review the experience of using this combined procedure existing in the literature.

Sinus venosus atrial septal defect with severe mitral stenosis: a rare variant of Lutembacher's syndrome.

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.

Surgical challenge in situs inversus with dextrocardia and Lutembacher syndrome.

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. We describe the surgical approach, position of the surgeon, and bicaval cannulation technique for this anatomical aberration.

Lutembacher's syndrome: Is the mitral pathology always rheumatic?

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. All 44 patients were symptomatic. There were 26 males (59%). The ages ranged from 13 to 73 years. A history of rheumatic fever was available in 2 patients while 16 patients had undergone surgery or intervention for the disease. Of the 18 patients in Group 1, six patients did not show histological features of rheumatic heart disease, although they shared similar gross morphological features. Furthermore, the mitral regurgitation in 12 of 19 patients in Group 2 was non-rheumatic. Also, only one patient had histological evidence of rheumatic activity among seven cases in Group 3. In spite of a high rheumatic load at our center, more than half (54.5%) of patients had "non-rheumatic" mitral valve pathology. Thus, the mitral valvular lesions commonly labeled 'rheumatic' in Lutembacher's syndrome are not always so. The distinction into rheumatic and non-rheumatic MVD in non-primum ASD has to be made on the basis of microscopic criteria.

Lutembacher syndrome variant: Rheumatic heart disease involving all four valves and associated with an atrial septal defect in a child.

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. There was an associated atrial septal defect (Lutembacher syndrome variant). The disease was severe and led to cardiogenic shock and death while awaiting surgery. The case highlights the impact of RHD on young people and the need to implement control programs for RHD in Sudan.

Current diagnostic and treatment strategies for Lutembacher syndrome: the pivotal role of echocardiography.

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. Echocardiography remains the gold standard for diagnosis and evaluation of LS. Timely diagnosis is critical for modifying the natural course, by allowing patients to benefit from currently available percutaneous trans-catheter therapies with favorable effects on the outcomes. This article is a review of published literature on the current diagnostic and therapeutic modalities for LS, focusing on the pivotal role of echocardiography as the key diagnostic tool. Clinical suspicion of LS should prompt extensive investigation with non-invasive and where possible, invasive technics. Multicenter registers have a potential to assist the evaluation of long term outcomes of percutaneous trans-catheter therapies in patients with LS.

Occurrence of Lutembacher syndrome in a rural regional hospital: case report from Buea, 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. Clinical examination revealed features suggestive of advanced congestive heart failure. Echocardiography revealed severe MS and a secundum type ASD with pulmonary hypertension. Patient died on second day of admission. CONCLUSIONS: LS is a very rare condition. The outcome is better if treated before the onset of heart failure and pulmonary hypertension. However, surgical and percutaneous trans-catheter therapy is costly and not readily available in low-income settings in developing countries.

Successful percutaneous management of Lutembacher syndrome.

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. Percutaneous trans mitral commissurotomy (PTMC) was done first, using a 26 mm Inoue balloon catheter set, and after 48 h, ASD was closed with a 20 mm Cocoon Septal Occluder. RESULTS: The mitral valve area increased after PTMC from 0.8 cm2 to 2.1 cm2 and QP/QS decreased from 4.9 to 2. ASD was successfully closed under echocardiographic and fluoroscopic guidance. CONCLUSION: Percutaneous management of the Lutembacher syndrome (PTMC and ASD device closure) is an effective and low risk procedure and avoids considerable morbidity and mental trauma for the patients.

Transcatheter therapy for Lutembacher's syndrome: The road less travelled.

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. The case highlights the challenges associated with the seemingly easy transcatheter therapy for this disease entity.

Lutembacher's syndrome.

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. Here, we will discuss a case of a pregnant lady who developed breathlessness during her third trimester of pregnancy and on detailed examination and investigation, she was found to be having Lutembacher's syndrome.

Oclusión transitoria de comunicación interauricular en el síndrome de Lutembacher / Temporary occlusion of atrial septal defect in the Lutembacher syndrome

Se describe una paciente de 82 años de edad, con síntomas de insuficiencia cardíaca avanzada e hipertensión arterial pulmonar. Un ecocardiograma transtorácico mostró una comunicación interauricular tipo ostium secundum y estenosis valvular mitral concomitante (síndrome de Lutembacher). La valoración ecocardiográfica de la enfermedad mitral se vio dificultada por la presencia del defecto interauricular. Se realizó test de oclusión percutánea transitoria de la comunicación interauricular, observándose la aparición de estenosis valvular mitral grave. El tamaño del defecto interauricular modificó las manifestaciones clínicas y el test de oclusión transitoria ayudó a decidir la conducta terapéutica.

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. Transient percutaneous occlusion test of the atrial septal defect was performed and severe mitral valve stenosis was detected. Atrial septal defect size modified the clinical manifestations and the transient occlusion test helped to decide the therapeutic strategy.

Turbulent left-to-right shunt flow through the interatrial septum suggesting high left atrial pressure in patients with heart failure with preserved ejection fraction.

Lutembacher's syndrome is a combination of interatrial septal (IAS) defect or patent foramen ovale (PFO), associated with mitral stenosis. High left atrial (LA) pressure in mitral stenosis exaggerates left-to-right shunt in patients with interatrial communication. We present a case of heart failure with preserved ejection fraction. Echocardiography revealed normal left ventricular systolic function without mitral stenosis and turbulent left-to-right shunt through the IAS. The peak velocity of the shunt flow was 2.3 m/s and the estimated pressure gradient was 22 mmHg, indicating high LA pressure. The presence of turbulent left-to-right shunt through the IAS is helpful for detecting high LA pressure.