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.

Surgical Treatment of Lutembacher Syndrome with a Huge Right Atrium: A Case Report.

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.

Percutaneous Transcatheter Treatment of Lutembacher Syndrome.

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.

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.

Iatrogenic Lutembacher Syndrome after Percutaneous Mitral Commissurotomy.

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. The patient had signs and symptoms of right heart failure and severe tricuspid regurgitation (TR) with borderline right ventricular systolic function on echocardiography, in addition to the ASD. Cardiac magnetic resonance (CMR) imaging played a significant role in decision-making by clarifying the anatomy of the ASD and severity of the shunt, measuring right ventricular systolic function, and providing absolute quantification for TR. The right ventricular systolic function was normal on CMR, rendering the patient suitable for surgical treatment. Persistent iatrogenic ASDs have become an increasingly common finding after invasive procedures requiring trans-septal puncture and the manipulation of catheters. Multimodality imaging can provide significant aid in the management of patients with valvular heart disease complicated by iatrogenic shunts.

Surgical correction of Lutembacher's syndrome in a patient with severe pulmonary artery hypertension.

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.

Lutembacher syndrome with unroofed left superior vena cava: a diagnostic dilemma.

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.

Non-surgical treatment of Lutembacher syndrome: combined percutaneous transcatheter therapy.

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 cm2) and a large ostium secundum atrial septal defect (21 mm) with a left to right shunt and severe pulmonary artery hypertension. She was diagnosed with Lutembacher syndrome and was evaluated for suitability of a percutaneous approach. She was subjected to a combined procedure of percutaneous transluminal mitral commissurotomy followed by device closure of the atrial septal defect. The patient tolerated the procedure, remained haemodynamically stable and was discharged after 4 days. 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.

Pacemaker Lead-induced Tricuspid Valve Stenosis and Reverse Lutembacher Syndrome.

Reverse Lutembacher syndrome is a rare cause of hypoxia characterized by the triad of tricuspid valve stenosis, 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. Tricuspid valve replacement using a bioprosthetic valve and patent foramen ovale closure improved her hypoxia.

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.

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

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.

[Percutaneous treatment of Lutembacher syndrome: a case report]. / Lutembacher sendromlu bir olguda perkütan tedavi.

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. On pretreatment transthoracic echocardiography, planimetric mitral valve area was 1.5 cm(2), maximum diastolic gradient was 17 mmHg, and mean diastolic gradient was 9 mmHg. Combined percutaneous treatment was performed including balloon valvuloplasty for MS and closure of the ASD with the Amplatzer septal occluder. The patient was discharged uneventfully. Transthoracic echocardiography performed a week later showed planimetric mitral valve area as 2.1 cm(2), maximum diastolic gradient as 9 mmHg, and mean diastolic gradient as 4 mmHg. Complete closure of the ASD was achieved. Transcatheter treatment may be an effective alternative to surgery in selected patients with Lutembacher syndrome.

Transcatheter treatment of Lutembacher's syndrome.

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. Closure of ASD was successfully achieved with an Amplatzer atrial septal occluder under transthoracic echocardiogram (TTE) guidance without general anesthesia.

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.

Clinical efficiency and safety analysis of transcatheter interventional therapy for compound congenital cardiovascular abnormalities.

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 +/- 10.52, with compound congenital cardiovascular abnormalities underwent transcatheter interventional procedure. These patients included 11 with perimembranous ventricular septal defect (PVSD) and patent ductus arteriosus (PDA), 8 patients with PVSD and atrial septal defect (ASD), 8 patients with ASD and PDA, 7 patients with ASD and pulmonary stenosis (PS), 1 patient with ASD and mitral stenosis(MS), 1 patient with coarctation of aorta (COA) and PDA. According to the principle of "easy first, hard second," balloon valvuloplasties of PS or MS were performed before the closure of PVSD, and of PDA and ASD. Electrocardiogram and transthoracic echocardiogram were examined at 4 days, 1, 2, 6 and 12 months, respectively, after each procedure. RESULTS: Transcatheter interventional therapy for compound congenital cardiovascular abnormalities was successful in all patients. Among these, 2 occluders were planted in each of 27 patients, 7 patients with ASD combined with PS and 1 patient with ASD combined with MS underwent successfully performed balloon valvuloplasty and ASD closure, 1 patient with COA combined with PDA underwent successfully performed balloon valvuloplasty and subsequent covered stent implantation. No patient encountered serious adverse events during the (30.5 +/- 14.6) months of follow-up. CONCLUSIONS: Transcatheter interventional therapy for compound congenital cardiovascular abnormalities could obtain satisfactory results with technical feasibility.

Transcatheter therapy of Lutembacher syndrome.

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. Nowadays, these 2 diseases are amenable to transcatheter treatment without the need for surgery. Here, we describe a 28-year-old female with Lutembacher syndrome who benefited from combined transcatheter therapy of balloon valvuloplasty for MS and device closure for ASD with an Amplatzer septal occluder.

Lutembacher syndrome: Dilemma of doing a tricuspid annuloplasty.

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.

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.