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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. Clinical examination revealed central cyanosis, raised jugular venous pressure bilaterally, clear breath sounds bilaterally with no dry and wet rales, hyperdynamic apex beat, and dull heart sounds. His vital signs on admission included blood pressure of 90/60 mmHg, irregular pulse of 76 beats/min, and oxygen saturation of 90.4%. Echocardiography revealed moderate to severe MS with ASD (ostium secundum). The patient's condition deteriorated after initial medical management, and he underwent open heart surgery for mitral valve replacement, ASD repair, tricuspid annuloplasty, and right atrial volume reduction., Conclusions: This case report describes the successful surgical management of this rare condition.

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., (© Journal of the Association of Physicians of India 2011.)

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., (Copyright © 2016. Published by Elsevier B.V.)

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. We describe a patient with Lutembacher's syndrome with severe pulmonary hypertension who underwent successful surgical repair., (© 2014 Wiley Periodicals, Inc.)

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. Evaluation by echocardiography followed by cine angiography confirmed the cause of cyanosis to be drainage of the LSVC into the LA together with an ASD and rheumatic mitral stenosis, a combination of Raghib and Lutembacher syndromes. The boy underwent successful surgical correction. The authors believe this is the second such case to be reported in the English literature and the first of its kind to be managed by surgical intervention.

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.

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. In cardiology unit he was diagnosed as a case of Lutembacher's syndrome on the basis of history, physical examination and it was confirmed by X-Ray, ECG and Echocardiography study. As the patient developed pulmonary hypertension with calcified mitral valve leaflet so percutaneous transseptal mitral commissurotomy and or surgery is not indicated. So the patient was managed by medical therapy alone.

We encountered a 78-year-old female with jaundice induced by Lutembacher's syndrome, which, to our knowledge, is the first such case to be reported. Laboratory data and ultrasonographic examination revealed liver congestion with dilatation of the inferior vena cava and hepatic veins. A large atrial septal defect was found using two-dimensional echocardiography; the mitral valve area measured 1.6 cm2 by planimeter. Clinicians should be aware of congenital heart disease as an underlying cause of jaundice.

We report a 63-year-old woman, with a partial type of common atrioventricular canal and mitral stenosis, who was hospitalized because of dyspnea on exertion. Two-dimensional echocardiogram showed an ostium primum atrial septal defect with two well-formed AV valves located at the same level. However, both anterior and posterior mitral leaflets were markedly thickened with a thickened subvalvular apparatus, and the commisures were fused. Echocardiographic measurements demonstrated a mitral valve area of 1.48 cm2 with mild mitral regurgitation. Cardiac catheterization demonstrated mild pulmonary artery hypertension with a large left to right shunt (72%) at the atrial level. The combination of the partial type of common atrioventricular canal and mitral stenosis is rare; only one similar case has been reported previously in the literature.

We describe the first reported patient with a persistent left superior vena cava that communicates directly with the left atrium as an isolated congenital defect. She developed mitral stenosis and physiologic conditions that favored left-to-right shunting--a modified Lutembacher's syndrome. Noninvasive cardiac imaging completely elucidated her cardiac anatomy and physiology.

A patient with rheumatic mitral and aortic stenosis is described in whom balloon dilatation of the mitral valve was complicated by an iatrogenic atrial septal defect with a small left to right shunt. Over the course of 4 years, the patient became progressively cyanosed and clubbed and was found to have reversed the shunt across the atrial septal defect due to the occurrence of severe tricuspid stenosis. The tricuspid valve was successfully dilated with re-establishment of a net left to right shunt. This syndrome, the first of its kind, should be appropriately termed the reversed Lutembacher syndrome.

A 48-years-old woman with Lutembacher syndrome with a clinical and laboratory finding of atrial septal defect, of the "ostium secundum" type and without pulmonary hypertension was reported. We observed, from diagnostic viewpoint, that the electrocardiographic finding of left atrial overload and the echocardiographic pattern of mitral stenosis where the most important diagnostic sign the mitral valve involvement. The patient underwent surgery and the atrial septal defect was closed with a patch. After the surgery the signs of atrial septal defect where very poor and the signs of mitral stenosis dominated the clinical picture of the patient. Although we did not make any intervention at the mitral valve, the patient had a good outcome and until the present date she is in functional class I.

In two patients with Lutembacher syndrome cardiac catheterization demonstrated a large atrial septal defect but no diastolic gradient across the mitral valve. Echocardiography was compatible with right ventricular volume overload and mitral stenosis. Surgical exploration of the mitral valve at the time of atrial septal defect correction is warranted when the echocardiogram is suggestive of mitral stenosis.

This report describes the usefulness of real time two-dimensional echocardiography in the diagnosis of left ventricular inflow obstruction associated with atrial septal defect. Echocardiographically, left ventricular inflow obstruction was at the level of the mitral valve in case one, mitral and subvalvular in cases 2 and 3 and supravalvular in case 4 (left atrial membrane). In all patients the defect was of the secundum variety and was seen as a constant discontinuity in the atrial septal image. The diagnosis was confirmed in all patients by cardiac catheterization, surgery or autopsy.

The findings in a patient with surgically proven Lutembacher's syndrome (coexisting mitral stenosis and atrial septal defect) are presented. In addition to a typical pattern of mitral stenosis, the preoperative echocardiogram revealed paradoxical septal motion, thereby providing, prior to cardiac catheterization, a basis for the suspicion of an associated lesion due to diastolic overload of the right ventricle.

Pre- and post-operative vectorcardiograms (Frank system) and angiocardiograms in a patient with Lutembacher's syndrome, were correlated. Preoperative vectorcariogram showed the initial conduction delay, complete RBBB, increased QRS voltage, and open QRS loop, while left ventriculography and left atriography demonstrated the markedly posteriorly dislocated left ventricular major axis surrounded by the huge right ventricle and right atrium. Two months after operation, when the left ventricular major axis shifted from abnormally posteriorly directed to left and inferiorly, with the decreased right-sided heart chambers, the initial conduction delay disappeared with decreased QRS voltage. As these vectorcardiographic and angiocardiographic changes at the postoperative stage were parallel, we concluded that the initial conduction delay was a representation of "the pseudo-W-P-W syndrome" and due to the abnormally posteriorly dislocated left ventricular apex and the delayed excitation of the right ventricular free wall caused by the huge dilatation.

The typical echocardiographic features of mitral stenosis in association with an enlarged right ventricle and abnormal septal motion are generally consistent with pulmonary hypertension and functional tricuspid regurgitation. In the absence of clinical and echocardiographic features of pulmonary hypertension, the combination of mitral stenosis and "volume overload" of the right ventricle should alert the clinician to the diagnosis of Lutembacher's syndrome. This report discusses a patient with these echocardiographic findings in whom the diagnosis of Lutembacher's syndrome was confirmed by cardiac catheterization.