Transcatheter Treatment of Lutembacher Syndrome: Combined Balloon Mitral Valvuloplasty and Percutaneous Atrial Septal Defect Cl

Lutembacher syndrome is a rare combination of congenital ASD and acquired rheumatic mitral stenosis. It is usually treated by surgical correction. We describe a case of Lutembacher syndrome that was treated successfully with percutaneous Inoue balloon mitral valvuloplasty and closure of the atrial septal defect with an Amplatzer atrial septal occluder. Case Report. A 39-year-old female was sent for evaluation for open heart surgery for mitral stenosis and ASD. She complained of dyspnea, palpitations and decreased exercise tolerance. Transthoracic echocardiogram (TTE) showed moderate mitral stenosis with a mitral valve area calculated from planimetry and Doppler of 1.4 cm2. The valve was pliable and without calcium. There was poor mobility of the posterior leaflet. The Wilkins echo score was less than 8. The diameter of the ASD was estimated to be 1.6 cm x 1.4 cm on transesophageal echocardiogram (TEE). The patient underwent cardiac catheterization. This showed the presence of a left to right shunt at the atrial level with a ratio of pulmonary flow to systemic flow of 2.3:1. The systolic pulmonary artery pressure was 36 mmHg. Mitral regurgitation was mild, and no trans-aortic valve gradient was detected. The mitral valve was crossed and dilated with an Inoue balloon catheter. Two inflations of 25 mm and 26 mm, respectively, were done with TTE guidance. The mitral valve area increased to 2.1 cm2. After balloon dilatation, the mitral regurgitation was grade I. The stretched diameter of the ostium secundum atrial septal defect was determined to be 22 mm using a sizing balloon catheter, and using TEE it appeared to be 22 mm. Under TEE guidance a 24 mm Amplatzer ASD occluder was deployed first without success and then upsized to a 28 mm occluder with total occlusion of the defect and no residual left-to-right shunt. At 1–month follow-up after the combined procedure, the patient was well with good symptomatic improvement. The chest X-ray showed a significant decrease in heart size. The cardiothoracic index was 52% pre-procedure vs. 45% after 1 week. The patient returned for 2-month follow-up. She had transient palpitations with a normal ECG one month post-procedure, but remained asymptomatic afterwards. The mitral valve area by planimetry was 2.1cm2 and Grade II/IV mitral regurgitation was noted. No left-to-right shunt was seen. Discussion. The letter that the anatomist Johan Friedrich Meckel sent from Berlin on May 5, 1750 to Albrecht von Haller in Gottingen contains the earliest reference to an unusual observation made by the former.¹ In fact, it is probably the first description of what is known today as Lutembacher syndrome, based on his 1916 report.² The hemodynamic effects of these coexisting conditions are interesting. Mitral stenosis augments the left-to-right shunt through the atrial septal defect which decompress the left atrium, reducing the mitral gradient. Thus, patients with this syndrome may have a relatively benign clinical course. In this condition, the mitral valve area should be determined by planimetry because the Doppler pressure half-time method is an inaccurate measure of mitral valve area.³ Traditionally, this condition has been corrected by surgical treatment.⁴ The experience with percutaneous approaches is small.^5-12 Ruiz et al. described a case in which catheter therapy was used as a bridge to surgery in a patient with Lutembacher’s syndrome and severe pulmonary hypertension.⁵ She was treated with single balloon aortic valvuloplasty, double balloon mitral valvuloplasty, and a Lock clamshell occluder for the 20 mm ASD. She refused surgery and died suddenly a few weeks after the percutaneous repair. Subsequent reports demonstrated the feasibility of percutaneous balloon mitral valvuloplasty and ASD closure using a variety of balloon catheter techniques and the Amplatzer closure device. Currently, the Inoue balloon is most widely used for percutaneous balloon mitral valvuloplasty, and the Amplatzer atrial septal occluder for correction of atrial septal defect. The advantages of percutaneous correction over traditional surgical correction include the avoidance of complications associated with open heart surgery, general anesthesia, and blood transfusions, and quicker recovery with shorter hospital stay. One obvious consequence of this approach is that repeat transseptal procedures cannot be performed. In the event that a patient develops mitral valve restenosis, either surgery or retrograde percutaneous therapy would be necessary. The potential for transseptal approaches for catheter ablation for arrhythmias would also be impaired. The creation of iatrogenic Lutembacher syndrome is a well known entity that occurs in the setting of percutaneous mitral balloon valvuloplasty for mitral stenosis.^13-17 Because the procedure is performed by transseptal approach, it requires an atrial septal puncture to access the mitral apparatus. Echocardiographic demonstration of an atrial septal defect immediately after balloon mitral valvuloplasty depends on the method of detection, with oximetry identifying only 7% to 33% of ASDs. TTE identifies ASD in approximately 50%, whereas TEE detects ASDs in almost 90% of patients within 24 hours after balloon mitral valvuloplasty. n the event that a persistent significant ASD is created by a transseptal procedure, the means to treat it using percutaneous methods is now available.¹⁸ Oximetric shunting is detected in less than 5% of patients after six months following balloon mitral valvuloplasty, usually with shunt ratios less than 1.5.^19,20 Nonetheless, there are occasional residual large iatrogenic ASDs for which percutaneous therapy is suitable. A special case of acute atrial septal defect is the situation in which the inferior right atrial septum is punctured, the transseptal needle transverses the transverse pericardial sinus, and then enters the left atrium. Thus, perforation of both atria results. This is ordinarily not recognized until the end of a mitral valvuloplasty procedure when the catheters are withdrawn and the large puncture is opened. It is common practice for some valvuloplasty operators to leave a wire across the transseptal puncture at the conclusion of mitral valvuloplasty procedures to observe for this potential complication. In the event that it is detected, devise closure of the iatrogenic defect might prevent an otherwise emergency surgical procedure.

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