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.

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.

Echocardiographic assessment of left and right heart hemodynamics in a patient with Lutembacher's syndrome.

We present a case of a 53-year-old woman with intractable shortness of breath that was originally ascribed to bronchiolitis obliterans organizing pneumonia. Subsequently evaluation by echocardiography and cardiac catheterization revealed that she had Lutembacher's syndrome, an uncommon combination of congenital atrial septal defect (ASD) and acquired mitral stenosis that is difficult to diagnose clinically. Our case illustrates the pitfalls and advantages of echocardiographic assessment of the mitral valve area (MVA) and the left atrial pressure (LAP). The pressure half-time method used most commonly for estimating MVA echocardiographically is inaccurate and may lead to underestimation of the severity of mitral stenosis in patients with Lutembacher's syndrome. On the other hand, the presence of ASD provides an additional method of calculating LAP, the most important determinant of symptoms in patients with mitral stenosis.

Use of Amplatzer septal occluder in a case of residual atrial septal defect causing bidirectional shunting after percutaneous Inoue mitral balloon valvuloplasty.

We describe a 70-year-old woman who underwent successful percutaneous Inoue antegrade-technique mitral valvuloplasty. Three months later, the patient developed right-sided heart failure. Color Doppler echocardiography and cardiac catheterization demonstrated an atrial septal defect with bidirectional shunting and no restenosis of the mitral valve (iatrogenic Lutembacher's syndrome). Percutaneous treatment was successfully accomplished using a 13 mm Amplatzer Septal Occluder (AGA Medical Corporation, Golden Valley, Minnesota). The causes of right-to-left shunting and the effects of body position on gas exchange in such patients are also discussed.

Partial type of common atrioventricular canal defect associated with mitral stenosis.

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.

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.

[Lutembacher's syndrome]. / Síndrome de Lutembacher.

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.

Síndrome de Lutembacher / Lutembacher's 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

Value and limitations of Doppler echocardiographic determination of mitral valve area in Lutembacher syndrome.

OBJECTIVES: Our objective was to compare the Doppler pressure half-time, Doppler continuity equation and two-dimensional echocardiographic planimetric methods of estimating mitral valve area in Lutembacher syndrome. BACKGROUND: Fluid dynamics theory predicts that mitral pressure half-time varies inversely with mitral valve area and directly with net chamber compliance and the peak early diastolic transmitral gradient in pure mitral stenosis. The effects of an atrial shunt on these interrelations have not been investigated. METHODS: Correlation and agreement between mitral valve area estimates obtained by the three methods and that obtained by cardiac catheterization was ascertained in 11 patients with Lutembacher syndrome. RESULTS: Valve areas determined by planimetry and the continuity equation method correlated and agreed well with catheterization measurements (r = 0.83 and 0.81, respectively). The pressure half-time method consistently overestimated mitral valve area; the extent of overestimation was greater in patients with larger atrial shunts. The hemodynamic pressure half-time was independent of the mitral valve area, chamber compliance and the peak transmitral gradient. It was dependent on the magnitude of the atrial shunt, although the correlation obtained was only fair (r = 0.61). CONCLUSIONS: These findings suggest that the Doppler pressure half-time method is an inaccurate measure of mitral valve area whenever an atrial shunt coexists with mitral stenosis. Planimetry and the Doppler continuity equation methods yield accurate estimates of mitral valve area in 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.

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.