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.

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 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.

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.

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.

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.

Percutaneous treatment of Lutembacher syndrome in a case with difficult mitral valve crossing.

Most cases of combination congenital cardiac anomalies are treated with open-heart surgeries because the coexisting anomalies change the cardiac anatomy in an adverse way, making catheter manipulations complex. Lutembacher syndrome is a combination of acquired mitral stenosis and congenital ostium secundum atrial septal defect. The large defect in the septum makes an Inoue balloon catheter unstable, which provides excessive space for free floatation of the catheter, making its passage into the left ventricle difficult by Inoue technique. We present a case of elective definitive percutaneous treatment of Lutembacher syndrome, discussing the technical difficulties faced in mitral valve crossing and reviewing the possible strategies to improve chances of success.

Valvotomia mitral percutânea em paciente com síndrome de Lutembacher como ponte para intervenção definitiva / Percutaneous mitral valvotomy in a patient with Lutembacher Syndrome as a bridge to definitive surgical intervention

Paciente com 45 anos de idade, do sexo feminino, testemunha de Jeová, portadora de síndrome de Lutembacher, com insuficiência cardíaca congestiva (ICC) grau funcional IV da New York Heart Association (NYHA) e desnutrição grave. O ecocardiograma revelou comunicação interatrial tipo ostiumsecundum, com 38 mm de diâmetro; área valva mitral de 0,5 cm², com escore de Wilkins de 10; hipertensão pulmonar grave, com pressão sistólica estimada em 96 mmHg; ventrículodireito com significativa dilatação e disfunção grave; e insuficiência valvar tricúspide grave. Apesar do tratamentoclínico otimizado, não houve melhora do quadro de ICC nem do estado geral, motivando a mudança da conduta detratamento cirúrgico inicial para tratamento em dois tempos,primeiramente por meio de valvotomia mitral por balão, como ponte para a cirurgia. A área valvar mitral pós-procedimento aumentou para 1,34 cm². A paciente evoluiu com significativamelhora clínica, sendo realizada cirurgia 120 dias após, com substituição da válvula mitral por uma prótese mecânica e atriosseptoplastia com patch de pericárdio bovino, além de cerclagem da válvula tricúspide. A alta hospitalar ocorreu 11 dias após a cirurgia. Atualmente, encontra-se no sexto mêspós-procedimento cirúrgico, evoluindo com estabilidade clínica e melhora da qualidade de vida.

A 45-year-old female patient, Jehovah's Witness, was diagnosed with Lutembacher syndrome, New York Heart Association (NYHA) class IV congestive heart failure (CHF) and severe malnutrition. Echocardiogram showed an ostium secundum atrial septal defect, 38 mm in diameter, mitral valve area of 0.5 cm2, Wilkins score of 10, severe pulmonary hypertension, estimated systolic pressure of 96 mmHg, right ventricle with significant dilation and severe dysfunction and severe tricuspid valve insufficiency. Despite optimal clinical treatment, there was no improvement of CHF or the patient's overall condition, which led to a change in the initial conduct of surgical treatment to a two-stage therapy, starting with balloon mitral valvotomy, as a bridge to surgery. Postoperative mitral valve area increased to 1.34 cm2. The patient evolved with significant clinical improvement, and surgery was performed 120 days later with mitral valve replacement by a mechanical valve and atrioseptoplasty using a bovine pericardial patch in addition to tricuspid valve cerclage. Patient was discharged 11 days after the surgery and is currently on the sixth postoperative month, evolving with clinical stability and improvement in quality of life.

Partial anomalous pulmonary venous connection associated with Lutembacher's syndrome.

Lutembacher's syndrome is a rare combination of congenital atrial septal defect and mitral stenosis (almost always rheumatic). The hemodynamic effect of this combination makes the clinical diagnosis difficult due to a wide variation of clinical presentation. Echocardiography and cardiac catheterization are useful tools for proper diagnosis and planning of the therapeutic strategy. We present a 28-year-old female with Lutembacher's syndrome associated with partial anomalous pulmonary venous connection (PAPVC), which was surgically corrected. To our knowledge, this combination has never been reported in the literature.

Síndrome de Lutembacher em paciente portadora de Doença de Chagas / Lutembacher syndrome in patient with Chagas' Disease

Relata-se neste artigo o caso de uma paciente, mulher de 58 anos, sabidamente portadora de Doença de Chagas à 35 anos, com síndrome de Lutembacher, sem apresentar características clínicas e laboratoriais da comunicação interatrial do tipo ostium secundum, ou hipertensão pulmonar de monta. Observa-se que, apesar de tantos motivos para descompensação cardíaca, sobretudo das câmaras direitas, a paciente apresenta-se clinicamente bem, tendo permanecido hemodinamicamente estável já por vários anos com a medicação empregada, sem apresentar sinais de deterioração cardíaca importante devida à concomitância das doenças. Não foi encontrado na literatura qualquer relato da concomitância dessas duas entidades clínicas num mesmo paciente.

Suggested new operative approach in the treatment of Lutembacher's syndrome, with moderately hypoplastic left ventricle.

Lutembacher's syndrome or ASD secondum and rheumatic mitral stenosis is treated with surgery but in some instances of presence of small left ventricle surgery will lead to death. Nazem's approach which is our Staged Suggested Method has saved several patients with this kind of pathology.

Lutembacher's syndrome complicated by jaundice.

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.

A unique left-to-right shunt characterized by multimodality cardiac imaging.

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.

Cyanosis and clubbing in a patient with iatrogenic Lutembacher syndrome.

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.

Continuous murmur in Lutembacher syndrome analyzed by Doppler echocardiography.

A continuous murmur was heard in a 47-year-old woman with Lutembacher syndrome. Transesophageal and intraoperative Doppler echocardiography revealed the murmur originating from the accelerated blood flow passing through the small atrial septal defect. To our knowledge, this is the first reported case of a continuous murmur in Lutembacher syndrome analyzed by Doppler echocardiography.

[Aneurysm of the interatrial septum associated with Lutembacher's syndrome. Echocardiographic and Doppler study]. / Aneurisma de septo interauricular asociado a síndrome de Lutembacher. Estudio ecocardiográfico y con técnica Doppler.

The case of a patient with suspected atrial septal defect is presented. Echocardiographic-Doppler study confirms the defect associated with mitral stenosis (Lutembacher syndrome) and shows an atrial septal aneurysm through which the shunt is revealed.