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Journal of the College of Physicians and Surgeons Pakistan 2009, Vol. 19 (2): 117-119 117 INTRODUCTION Dextrocardia makes fluoroscopy-guided transcatheter procedures challenging; specially Percutaneous Transvenous Mitral Commissurotomy (PTMC), where the unusual cardiac anatomy considerably increases the risk of complications during transseptal puncture and entry into left ventricular cavity. 1 Although, PTMC has become the standard of care for selected patients with rheumatic mitral stenosis (MS), there are only a few reports on successful PTMC in atypical cardiac anatomy. This particular case was peculiar in that Closed Mitral Valvotomy (CMV) had already been done and now PTMC was being performed in the same patient for relief of restenosis. Literature search did not show any reported case with this peculiar combination of therapeutic challenges. CASE REPORT A 30-year-old woman having situs inversus and suffering from ‘mitral restenosis’ was referred to AFIC/NIHD for PTMC by her cardiac surgeon. A CMV for severe mitral stenosis had been done 8 years earlier. The patient presented with shortness of breath NYHA class-II. A transthoracic and transesophageal echo- cardiographic evaluation revealed a mitral valve area of 0.6 cm 2 with pliable leaflets and no mitral regurgitation. She had an MGH score of 6 and her pulmonary artery pressure was 100 mmHg. Presence of dextrocardia, AV and VA concordance was demonstrated. PTMC was performed using a 24 mm Inoue balloon under local anaesthesia. Both groins were prepared for vascular access, however, the left femoral artery and vein were cannulated with a 5F arterial and 6F venous sheaths. A 5F pigtail catheter was passed retrograde into the aorta and taken to the left ventricle where pressures were recorded and then an LV cine angiogram was performed in an LAO 40 projection. No mitral regurgitation was seen. The pigtail catheter was then withdrawn and parked in the aortic root on top of the aortic valve. A 0.032” guide wire was then passed up the femoral vein into the IVC and up into the left sided SVC via the left sided ‘right atrium’. An 8 F Mullins sheath was passed up on the guide wire, into the left SVC. A curved Brockenbrough needle was passed up into the sheath stopping just short of the tip. For septal puncture, the patient was imaged in LAO 40 o projection. The Brockenbrough needle was oriented to 9 O’ clock position in the SVC. Septal descent was done by withdrawing the needle and the sheath in tandem into the heart with the needle pointer in 7-8 O’ clock position. The needle was withdrawn into the heart upto the level of the pigtail, and then further one disc space. The puncture point was chosen to be the point one disc space below the horizontal line stretching across the lower level of the pigtail; and the point was roughly midway between the posterior wall of the LA and the ABSTRACT A woman, aged 30 years with situs inversus, dextrocardia and severe rheumatic mitral restenosis was referred to AFIC/NIHD for commissurotomy. A Closed Mitral Valvotomy (CMV) for severe mitral stenosis had already been done 8 years earlier, and her symptoms had reappeared. She was evaluated with transthoracic and transesophageal echocardiography and found to have a mitral valve area of 0.6 cm 2 , dextrocardia along with atrioventricular (AV) and ventriculo-atricular (VA) concordance was demonstrated. Successful percutaneous transvenous mitral commissurotomy (PTMC) was done. Transseptal catheterization was done via the left femoral vein. Pigtail catheter was placed in the non- coronary aortic sinus; interatrial septal puncture was done with the transseptal needle rotated to a 7 O'clock position. There were no procedural complications. Reduction in trans-mitral pressure gradient on cardiac catheterization data, and standard echocardiographic parameters confirmed a successful procedure. PTMC can be accomplished safely in patients with this unusual cardiac anatomy with a few modifications in the standard technique, even if surgical treatment has already been carried out. Key words: Percutaneous mitral commissurotomy. Mitral restenosis. Dextrocardia. Situs inversus. Closed mitral valvotomy. Department of Cardiology, Armed Forces Institute of Cardiology, National Institute of Heart Diseases, Rawalpindi. Correspondence: Dr. Waqar Ahmed, 1018, Gulshan Abad, Adyala Road, Rawalpindi. Email : [email protected] Received July 29, 2008; accepted November 11, 2008. Percutaneous Transvenous Mitral Commissurotomy in Mitral Restenosis with Situs Inversus Sajjad Hussain and Waqar Ahmed CASE REPORT

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Journal of the College of Physicians and Surgeons Pakistan 2009, Vol. 19 (2): 117-119 117

INTRODUCTION

Dextrocardia makes fluoroscopy-guided transcatheterprocedures challenging; specially PercutaneousTransvenous Mitral Commissurotomy (PTMC), wherethe unusual cardiac anatomy considerably increases therisk of complications during transseptal puncture andentry into left ventricular cavity.1 Although, PTMC hasbecome the standard of care for selected patients withrheumatic mitral stenosis (MS), there are only a fewreports on successful PTMC in atypical cardiacanatomy. This particular case was peculiar in thatClosed Mitral Valvotomy (CMV) had already been doneand now PTMC was being performed in the samepatient for relief of restenosis. Literature search did notshow any reported case with this peculiar combinationof therapeutic challenges.

CASE REPORTA 30-year-old woman having situs inversus andsuffering from ‘mitral restenosis’ was referred toAFIC/NIHD for PTMC by her cardiac surgeon. A CMVfor severe mitral stenosis had been done 8 years earlier.The patient presented with shortness of breath NYHAclass-II. A transthoracic and transesophageal echo-cardiographic evaluation revealed a mitral valve area of

0.6 cm2 with pliable leaflets and no mitral regurgitation.She had an MGH score of 6 and her pulmonary arterypressure was 100 mmHg. Presence of dextrocardia, AVand VA concordance was demonstrated. PTMC wasperformed using a 24 mm Inoue balloon under localanaesthesia.

Both groins were prepared for vascular access,however, the left femoral artery and vein werecannulated with a 5F arterial and 6F venous sheaths. A5F pigtail catheter was passed retrograde into the aortaand taken to the left ventricle where pressures wererecorded and then an LV cine angiogram was performedin an LAO 40 projection. No mitral regurgitation wasseen. The pigtail catheter was then withdrawn andparked in the aortic root on top of the aortic valve. A0.032” guide wire was then passed up the femoral veininto the IVC and up into the left sided SVC via the leftsided ‘right atrium’. An 8 F Mullins sheath was passedup on the guide wire, into the left SVC. A curvedBrockenbrough needle was passed up into the sheathstopping just short of the tip.

For septal puncture, the patient was imaged in LAO 40o

projection. The Brockenbrough needle was oriented to9 O’ clock position in the SVC. Septal descent was doneby withdrawing the needle and the sheath in tandem intothe heart with the needle pointer in 7-8 O’ clock position.The needle was withdrawn into the heart upto the levelof the pigtail, and then further one disc space. Thepuncture point was chosen to be the point one discspace below the horizontal line stretching across thelower level of the pigtail; and the point was roughlymidway between the posterior wall of the LA and the

ABSTRACTA woman, aged 30 years with situs inversus, dextrocardia and severe rheumatic mitral restenosis was referred toAFIC/NIHD for commissurotomy. A Closed Mitral Valvotomy (CMV) for severe mitral stenosis had already been done8 years earlier, and her symptoms had reappeared. She was evaluated with transthoracic and transesophagealechocardiography and found to have a mitral valve area of 0.6 cm2, dextrocardia along with atrioventricular (AV) andventriculo-atricular (VA) concordance was demonstrated. Successful percutaneous transvenous mitral commissurotomy(PTMC) was done. Transseptal catheterization was done via the left femoral vein. Pigtail catheter was placed in the non-coronary aortic sinus; interatrial septal puncture was done with the transseptal needle rotated to a 7 O'clock position. Therewere no procedural complications. Reduction in trans-mitral pressure gradient on cardiac catheterization data, andstandard echocardiographic parameters confirmed a successful procedure. PTMC can be accomplished safely in patientswith this unusual cardiac anatomy with a few modifications in the standard technique, even if surgical treatment hasalready been carried out.

Key words: Percutaneous mitral commissurotomy. Mitral restenosis. Dextrocardia. Situs inversus. Closed mitral valvotomy.

Department of Cardiology, Armed Forces Institute ofCardiology, National Institute of Heart Diseases, Rawalpindi.

Correspondence: Dr. Waqar Ahmed, 1018, Gulshan Abad,Adyala Road, Rawalpindi.Email : [email protected]

Received July 29, 2008; accepted November 11, 2008.

Percutaneous Transvenous Mitral Commissurotomy in MitralRestenosis with Situs Inversus

Sajjad Hussain and Waqar Ahmed

CASE REPORT

imaginary line drawn vertically from the pigtail catheter’sshaft. After engaging the interatrial septum, we gentlycrossed it with just the needle tip, and changed theprojection to RAO 45o and squirted a bit of contrast intothe LA; firstly, to document the correct entry into the LAand secondly to fathom the depth of the LA. Mullinsheath was gently pushed halfway down the availabledepth while keeping the needle tip within the sheath.Then, the needle was withdrawn and the sheath leftin situ. A ‘loopy’ wire was then passed through thesheath and the latter was withdrawn leaving the loopywire inside. The 14F dilator was then used to make theentry point wider. The balloon was then threaded overthe loopy wire to enter the LA. The balloon was flushedand simultaneous LA/LV pressures were taken. Now,the J-wire was used to guide the balloon into the LV.There was difficulty pushing the balloon probablybecause of the muscular entry point in the IAS, so an 8Fperipheral balloon was used to dilate the puncture site(Figure 1). For withdrawing this balloon while stillkeeping the wire in the LA, the balloon shaft was cut 20cm from the proximal hub and removed it without anyhassle (Figure 2). The maneuverability of the balloonwas remarkably improved in opening up the mitral valveafter a single dilatation (Figure 3).

The final area after the ballooning was 1.86 cm2 by 2Dand 3D echocardiography. No MR was documented bycheck LV angiogram.

DISCUSSION

There are only a few reported cases of PTMC in patientswith dextrocardia and situs inversus. Whether, thisreflects the tendency to avoid transseptal puncture andPTMC in these technically difficult cases, thus referringthem to cardiac surgeons for mitral valve replacement isopen to speculation.

In general, transseptal catheterization is considered atechnical challenge in anatomically malpositionedhearts, as it is fraught with a higher risk of cardiacperforation. Over time various modifications of thestandard Inoue technique have evolved and are being

used by different operators to suit the needs of thesepatients with uncharacteristic cardiac anatomy. Theproblem has been addressed in pregnancy withsuccessful PTMC in anatomically challenging hearts.1Transseptal catheterization is performed from the leftgroin to reduce the puncture needle angulations at theconfluence of the iliac veins to the left-sided inferiorvena cava.2 The catheter placed in the non-coronaryaortic sinus marks the antero-superior limit of the IAS.Septal descent is done by rotating the external indicatorof the needle at the 7 O’clock position.2-4 Entry into theLA, and it’s depth can be confirmed by squintingcontrast into the left atrium. This also aids in confirmingthe limits of the interatrial septum. Levophasepulmonary angiography has been used for IASdelineation in a patient with isolated dextrocardia andnormal atrial situs.5 Transesophageal and intracardiacecho are important adjunctive pathfinders for theinterventionist cases as complex as this.3,6 Thetransjugular approach is thought to overcome many ofthe technical problems encountered with thetransfemoral route in cases with anatomical alterations.7Despite the challenging anatomy, PTMC has beendemonstrated to be a safe and feasible option for MS inpatients with unusual cardiac anatomy.8,9

Pertinent to this particular case, the increase in mitralvalve area with PTMC is inversely related to thepresence of previous surgical mitral commissurotomy,PTMC can produce a good outcome in this group ofpatients. The mean mitral valve area in 102 patients withprevious surgical commissurotomy was 1.7±0.1 cm2

compared with a valve area of 2.0±0.1 cm2 in patientswithout previous surgical commissurotomy. In this groupof patients, an echocardiographic score of 8 was againthe most important predictor of a successful hemo-dynamic immediate outcome.10

REFERENCES1. Said SA, Veerbeek A, van der Wieken LR. Dextrocardia, situs

inversus and severe mitral stenosis in a pregnant woman:successful closed commissurotomy. Eur Heart J 1991; 12:825-8.

118 Journal of the College of Physicians and Surgeons Pakistan 2009, Vol. 19 (2): 117-119

Sajjad Hussain and Waqar Ahmed

Figure 1: 8F peripheral balloon being used todilate puncture site in the interatrial septum (arrow).

Figure 3: PTMC balloon seen during full dilatationacross mitral valve. Pigtail catheter (arrow) sited inaortic root.

Figure 2: The balloon shaft cut into two parts forremoval.

2. Namboodiri N, Harikrishnan SP, Ajitkumar V, Tharakan JA.Percutaneous mitral commissurotomy in a case of mirror-imagedextrocardia and rheumatic mitral stenosis. J Invasive Cardiol 2008;20:E33-5.

3. Nallet O, Lung B, Cormier B, Porte JM, Garbarz E, Michel PL,et al. Specifics of technique in percutaneous mitralcommissurotomy in a case of dextrocardia and situs inversuswith mitral stenosis. Cathet Cardiovasc Diagn 1996; 39:85-8.

4. Chow WH, Fan K, Chow TC. Balloon mitral commissurotomy ina patient with situs inversus and dextrocardia. J Heart Valve Dis1996; 5:307-8.

5. Verma PK, Bali HK, Suresh PV, Varma JS. Balloon mitralvalvotomy using Inoue technique in a patient of isolateddextrocardia with rheumatic mitral stenosis. Indian Heart J 1999;51: 315-7.

6. Shalganov TN, Paprika D, Borbás S, Temesvári A, Szili-Török T.

Preventing complicated transseptal puncture with intracardiacechocardiography: case report. Cardiovasc Ultrasound 2005; 1:3-5.

7. Joseph G, George OK, Mandalay A, Sathe S. Transjugularapproach to balloon mitral valvuloplasty helps overcomeimpediments caused by anatomical alterations. Catheter CardiovascInterv 2002; 57: 353-62.

8. Patel TM, Dani SI, Thakore SB, Chaq MC, Shah SC, Shah UG,et al. Balloon mitral and aortic valvuloplasty in mirror-imagedextrocardia. J Invasive Cardiol 1996; 8:164-8.

9. Raju R, Singh S, Kumar P, Rao S, Kapoor S, Raju BS.Percutaneous balloon valvuloplasty in mirror-imagedextrocardia and rheumatic mitral stenosis. Cathet Cardiovasc Diagn1993; 30:138-40.

10. Herrmann HC. Interventional cardiology: percutaneousnoncoronary intervention. Totowa: Humana Press, Totowa, NJ;2005:p.12.

Journal of the College of Physicians and Surgeons Pakistan 2009, Vol. 19 (2): 117-119 119

Percutaneous transvenous mitral commissurotomy in mitral restenosis with situs inversus

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