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.

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.

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.

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's syndrome].

OBJECTIVE: To compare classical and acquired Lutembacher's syndrome (mitral restenosis after percutaneous balloon mitral valvuloplasty) in attempt to know their different in pathophysiology, diagnosis, and surgical treatment. METHODS: The data from 22 cases of classical and acquired Lutembacher's syndrome who underwent surgical treatment in the same period were analyzed. RESULTS: No death occurred in the classical group. In the acquired group the mortality however was 42.8% for those highly complicated with pulmonary hypertension and right heart failure. CONCLUSIONS: Classical Lutembacher's syndrome can be corrected satisfactorily. The acquired one deteriorates rapidly with severe pulmonary hypertension and right heart failure, and needs early surgical intervention reduce the operation mortality.

[A case report of incomplete endocardial cushion defect with mitral stenosis in an elderly patient].

The following paper describes a mitral valve replacement (SJM 27 mm), the patch closure (EPTFE) of an ostium primum atrial septal defect and tricuspid annuloplasty (De Vega's method) in a 64-year-old female patient with an incomplete endocardial cushion defect and mitral stenosis. Surgery revealed thickened, mitral valve leaflets and the presence of a cleft, findings similar to those observed in case of rheumatic degeneration. Investigation of patient hemodynamics confirmed a diagnosis of Lutembacher syndrome and a lower with left ventricle volume. After surgery, the volume of left ventricle increased and the patients clinical course was uneventful.

Aortic valve stenosis causing a left-to-right shunt in persistent left superior vena cava communicating with the left atrium.

This study demonstrated a rare anomaly of a persistent left superior vena cava draining into the left atrium in a patient with developing left-to-right shunt caused by bicuspid aortic stenosis. The venous system, including the coronary sinus, was otherwise normal. We believe that, in this anatomic situation, a marked increase in left ventricular impedance caused a moderate left-to-right shunt from the left atrium into the left innominate vein. At operation, the aortic valve was replaced with a mechanical prosthesis and the anomalous vein was ligated. The convalescence was uneventful.

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.

Surgical indications and treatment of mitral valve disease associated with secundum atrial septal defect with special reference to left ventricular geometry and function.

There are few reports that discussed surgical indications and selection of surgical procedures for secundum atrial septal defect (ASD) associated with mitral valve disease from the viewpoint of left ventricular geometry and function. Our study on 20 patients (6 patients with ASD and MS, 14 patients with ASD and MR) indicated that adult patients with ASD and MS can be treated surgically even when left ventricular end-diastolic volume index (LVEDVI) is as low as 35 ml/m2 (one third of the normal level). Left ventricular wall movement returned to normal one year following surgery. Therefore, rather than myocardial impairment, right ventricular volume load disturbing left ventricular dilatation would be the more likely cause of diminished left ventricular function before surgery in the ASD and MS group. In both groups, two patients who initially had undergone mitral valve replacement died from low output syndrome because of the mismatch between the left ventricular volume and the prosthesis used, whereas there were no deaths in the patients who had mitral valve repair. For this reason, mitral valve repair is the treatment of choice for patients with both secundum ASD and mitral valve disease. When valve replacement is the only alternative, selection of prosthetic valves and maintenance of cardiac output by temporary cardiac pacing are important considerations. A low profile valve with a larger orifice area should be selected to avoid the mismatch between a left ventricular volume and a prosthesis.

[Changes in left ventricular pressure volume loop of Lutembacher syndrome].

A 49-year-old woman with Lutembacher syndrome, atrial septal defect (ASD), mitral stenosis (MS), tricuspid regurgitation and cardiac cachexia underwent a mitral valve replacement with a 27 mm CarboMedics valve, patch closure of ASD and tricuspid annuloplasty to 29 mm by DeVega's method. The pressure volume loop (PV loop) was measured, both preoperatively and postoperatively (immediately and two months after the operation) by a Miller catheter and echocardiogram. The left ventricular stroke work (LVSW), left ventricular end diastolic volume (LVEDV) and left ventricular end diastolic pressure (LVEDP) markedly increased immediately after the operation. LVSW and LVEDV further increased, whereas LVEDP decreased with improvements in diastolic compliance two months after the operation. It was likely that those change of LV function was a sum of simple ASD and MS both of which have similar but less significant characters than Lutembacher syndrome.

Long-term follow-up of cardiac rhythms after biatrial transseptal approach (Dubost's incision).

Biatrial transseptal approach (Dubost's incision) was performed 54 times on 47 patients from November 1973 to December 1982 at National Fukuoka Higashi Hospital in Japan. The patients consisted of 19 males and 28 females, with ages ranging from 14 to 66 (mean 45.9 years). Forty-four out of 47 cases had rheumatic heart disease while one had endocardial cushion defect, one had Lutembacher's syndrome, and one had left atrial myxoma. Preoperative electrocardiograph showed atrial fibrillation in 37 cases (78.7%) and normal sinus rhythm in 10 cases (21.3%). The follow-ups of the patients were a minimum of 8 years and a maximum of 16 years (mean 9.6 years) with 97.8% completion. Cumulative follow-up period was 448.9 patient years. Postoperatively, atrial fibrillation persisted in all except two. In those patients, normal sinus rhythm was observed until postoperative six months and seven years, respectively. Normal sinus rhythm persisted in six cases, and changed into junctional rhythm in four. One of them changed into atrial fibrillation at 10.6 years postoperatively. We conclude that Dubost's incision provides an excellent operative field for mitral and tricuspid valve surgery without serious internodal conduction disturbances.

Síndrome de Lutembacher / Lutembacher's syndrome

Mulher de 48 anos, com Síndrome de Lutembacher, onde predominavam as características clínicas e laboratoriais da CIA, do tipo "ostium secundum", sem hipertensäo pulmonar de monta. Os autores chamam atençäo para a dificuldade diagnóstica da síndrome, no pré-operatório, onde as pistas diagnósticas, da EM, foram dadas pela curva eletrocardiográfica de sobrecarga atrial esquerda, pelo padräo ecocardiográfica de estenose mitral e de aumento do átrio esquerdo. No pós-operatório enfatizam a grande atenuaçäo dos sinais da CIA e a intensificaçäo dos da EM. Embora näo se tenha tocado na valva mitral, durante o ato cirúrgico, a paciente teve boa evoluçäo e atualmente acha-se em classe funcional I