Percutaneous Treatment of Lutembacher Syndrome in a Case with Difficult Mitral Valve Crossing

ABSTRACT: 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.

J INVASIVE CARDIOL 2012;24(3):E54-E56

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Despite tremendous refinements in interventional technology over the last couple of decades, most patients with compound congenital cardiac anomalies are still treated with open-heart surgeries because the coexisting anomalies change the cardiac anatomy in an adverse way, making catheter manipulations complex. The resulting interventional procedures are, therefore, not simply a combination of 2 simple procedures.

Lutembacher syndrome (ie, appearance of acquired rheumatic mitral stenosis [MS] in the presence of congenital ostium secundum atrial septal defect [ASD]), predisposes to development of early severe pulmonary arterial hypertension. Although simultaneous catheter-based treatment of this combination cardiac anomaly has been reported a couple of times, the reports of definitive treatment are still anecdotal.^1,2

One might presume that presence of ASD simplifies percutaneous balloon mitral valvuloplasty (BMV) by precluding the need for a crucial but sometimes difficult step: the atrial septal puncture. On the contrary, the large defect in the septum makes the Inoue balloon catheter unstable, which provides excessive space for free floatation of the catheter, making its passage into the left ventricle (LV) difficult by Inoue technique.

In this case, we planned a definitive percutaneous treatment for Lutembacher syndrome, discussing the technical difficulties faced in mitral valve crossing and reviewing the possible strategies to improve chances of success.

Case Report. A 32-year-old male presented in the outpatient department with progressive exertional dyspnea. On clinical and echocardiographic evaluation, he was diagnosed with an ostium secundum ASD with acquired severe MS of rheumatic etiology. The ASD was 17 mm and the mitral valve area calculated by planimetry was 1.0 cm². The valve was pliable. It was suitable for percutaneous BMV with no commissural calcification and only trivial mitral regurgitation (MR). The subvalvular fusion was moderate and calculated RV systolic pressure was 46 mm Hg. Transesophageal echo confirmed the 19 mm ASD and revealed presence of a thin aneurysmal segment of inter atrial septum (IAS), making superior rim of ASD. It also excluded presence of left atrial appendage thrombus and coexistent additional anomalies and confirmed the degree of M to be trivial.

Patient was taken up for elective simultaneous catheter-based treatment of ASD and MS. Patient received 300 mg of clopidogrel and 325 mg of aspirin one day before procedure. Infective endocarditis prophylaxis was given. Five thousand units of heparin were administered after obtaining femoral arterial and venous access. Transmitral gradients were not recorded because these were not considered relevant due to simultaneous presence of ASD decompressing left atrium (LA) into right atrium (RA).

A Mullin’s sheath dilator was easily advanced into LA from RA through right femoral vein. A pigtail Inoue guidewire (0.025″) was parked in LA. Inoue balloon catheter was advanced over it. A couple of attempts were made to cross mitral valve by standard Inoue method. As anticipated, on each counter clockwise rotation of Inoue balloon catheter, instead of entering into LV, it jumped back towards ASD and then into the RA. This happened because the shaft of the balloon catheter floated free in the space provided by ASD. This situation is unlike normal IAS, which supports the catheter shaft, allowing it to maintain a curve and orienting balloon tip toward mitral valve. We decided to cross the mitral valve over a 0.032-inch exchange length J-tip guidewire.

Balloon catheter was removed over Inoue pigtail guidewire and exchanged for the dilator of Mullin’s sheath. The tip of Mullin’s dilator was manipulated to face mitral valve orifice. A 0.032-inch exchange length J-tip guidewire was advanced through the tip of Mullin’s dilator into LV. After placing Mullin’s dilator into LV, again pigtail tip Inoue wire was placed in LV. This step was considered necessary to avoid chances of LV perforation, which could occur while forcing balloon catheter over 0.032-inch J-tip wire. Inoue balloon catheter could easily be manipulated over pigtail guidewire and mitral valve dilatation was successfully performed with Inoue balloon inflation. The balloon was withdrawn into LA and echocardiographic planimetry was performed to confirm adequacy of the procedure. The MV area was 1.9cm². Doppler interrogation excluded presence of significant MR.

Following this, the stretched diameter of ASD was assessed using Amplatzer sizing balloon. Adjusting for the septal aneurysm, a 30 mm atrial septal occluder was deployed successfully across IAS through a 10 Fr delivery sheath under fluoroscopic guidance. Echocardiographic and color Doppler imaging revealed absence of residual shunt across IAS. The procedures were without any complication and patient’s further hospital stay was uneventful.

Echocardiography on following day revealed a well placed septal occluder and MV area of 1.9 cm² as assessed by planimetry. Patient was discharged with advice to continue clopidogrel 75 mg once daily for 1 month, aspirin 150 mg once daily for 6 months, and infective endocarditis prophylaxis.

Discussion. In view of the high success rate and safety of percutaneous treatment of secundum ASD and MS, Lutembacher syndrome appears to be a perfect heart disease for percutaneous management.

Presence of combination structural heart disease modifies the presentation and unfavorably influences the natural history and procedural success of catheter-based treatment. MS augments the left to right inter atrial shunt, while ASD serves to decompress the LA. This masks MS symptoms but causes early rise of pulmonary artery pressure. Apart from this, it also makes cardiac catheterization-based as well as Doppler-based assessments of MS severity unreliable, leaving 2-dimensional ECG-based planimetry as the most reliable method.

Percutaneous treatment avoids morbidity and mortality associated with cardiac surgery and simultaneously is preferred by most individuals due to cosmetic reasons. Another important consideration is that if surgery is considered the preferred option the first time, the patients with pliable MV will be treated with closed or open mitral commissurotomy. These both have restenosis rates comparable to BMV. In such cases, if restenosis occurs, the BMV would be very difficult due to atrial septal patch and redo surgery would be complicated. While surgery will continue to be a low-risk option if required in a case previously treated percutaneously, in most of cases of Lutembacher syndrome, ASD poses technical difficulties in pushing the Inoue balloon across stenosed mitral valve. This is unlikely to be accomplished with routine Inoue method, which warrants use of the alternative methods for mitral valve crossing. Although, not described in the context of Lutembacher syndrome, any of these can be tried according to operator’s choice or multiple maneuvers can be tried if one is not successful. Some of the described methods are: use of floatation balloon catheters, reverse loop method, catheter sliding method, over-the-wire method, modified over-the-wire technique, and reshaping the stylet.

Modified Inoue technique³ involves placement of Judkins right coronary catheter in LA with its tip pointing towards mitral orifice. This is followed by crossing of mitral valve with a back-up valvuloplasty wire (Schneider), which is later substituted by pigtail Inoue wire through a Judkins right catheter, already advanced in LV. Inoue balloon is finally advanced over the Inoue wire. The same technique was used in this case, with the only difference being that instead of back-up valvuloplasty wire, we used a 0.032-inch exchange length guidewire, already available on the table as part of BMV set.

Another described method⁴ consists of advancing a balloon floatation catheter (Swan-Ganz) into LV through mitral valve, maneuvering it into descending aorta through aortic valve, placing a 0.021-inch back-up valvuloplasty wire through this catheter into descending aorta, and advancing Inoue balloon catheter across MV over the back-up wire.

Modified over-the-wire technique involves placing the 0.025 Inoue wire into LA through atrial septum; advancing Mullin’s sheath over this wire; placing Mullin’s sheath end hole near mitral orifice; and placement of Inoue pigtail wire directly into LV. Manipulation of pigtail wire through mitral valve might be difficult compared to that of a J-tip 0.032-inch guidewire as done in modified Inoue technique.

Another method that can be useful in this situation⁵ involves making a large radius curve in the stylet; the apex of the stylet curve is placed at the atrial septum, keeping the ascending curve of stylet in RA, and descending curve in the LA. This stylet curve coaxially aligns the balloon with mitral orifice.  

Apart from these, retrograde crossing of mitral valve⁶ and transjugular approach⁷ are other options.

Conclusion

Percutaneous interventional treatment of Lutembacher syndrome is feasible, but can be complex, requiring alternative or modified methods for mitral valve crossing. A pre-planned strategy with prior anticipation of difficulties in catheter manipulations and a skillful approach make the procedure feasible and improve the chances of success.

References

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2. Ruiz CE, Gamra H, Mahrer P, AllenJW, O’Laughlin MP, Lau FY. Percutaneous closure of a secundum atrial septal defect and double balloon valvotomies of a severe mitral and aortic valve stenosis in a patient with Lutembacher’s syndrome and severe pulmonary hypertension. Cathet Cardiovas Diagn. 1992 Apr;25(4):309-312.
3. Meier B. Modified lnoue technique for difficult mitral balloon commissurotomy. Cathet Cardiovasc Diagn.1992 Aug;26(4):316-318.
4. Mehan VK, Meier B. Impossibility to cross a stenotic mitral valve with the Inoue balloon: success with a modified technique. Indian Heart J. 1994 Jan-Feb;46(1):51-52.
5. Inoue K, Feldman T. Percutaneous transvenous mitral commissurotomy using the lnoue balloon catheter. Cathet Cardiovasc Diagn.1993 Feb;28(2):119-125.
6. Zureikat HY, Karsheh IE, Naber NM, Najjar SM, el-Dibs NR, Salch SS. Mitral balloon valvuloplasty using a retrograde transventricular approach via the brachial artery. Cathet Cardiovasc Diagn.1989 Jul;17(3):183-185.
7. Joseph G, Rajendiran G, Rajpal KA. Transjugular approach to concurrent mitral-aortic and mitral-tricuspid balloon valvuloplasty. Catheter Cardiovasc Interv. 2000 Mar;49(3):335-341.

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From the Department of Cardiology, Yashoda Hospital, Secunderabad, India.
Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.
Manuscript submitted August 30, 2011 and accepted September 14, 2011.
Address for correspondence: Anupam Bhambhani. Consultant Cardiologist, Yashoda Hospital, Behind Harihara Kala bhavan, S.P. Road, Secunderabad 500003. AP. India. Email: anupam.bhambhani@yahoo.in