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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Concomitant tuberculous meningitis and Lutembacher syndrome with multiple atrial septal defects and infective endocarditis.

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. Tuberculous meningitis is a chronic infection due to haematogenous dissemination of tubercle bacilli from lungs. It can lead to complications like cranial nerve palsies, hydrocephalus, cerebral oedema or focal neurological deficits presenting as stroke. The treatment should include antituberculous therapy for one year and corticosteroids for initial 4-6 weeks depending on the symptoms of the patient. This report describes the concomitant occurrence of all these conditions at a time in a 45 years old lady.