CASE REPORT

Ventricular arrhythmias originating from the epicardialventricular outflow tract complicatedwith peripartum cardiomyopathy

Takumi Yamada & H. Thomas McElderry &

Masahiro Muto & Taro Okada & Yoshimasa Murakami &Harish Doppalapudi & Andrew E. Epstein &

Vance J. Plumb & G. Neal Kay

Received: 1 April 2008 /Accepted: 18 September 2008 /Published online: 26 November 2008# Springer Science + Business Media, LLC 2008

Abstract We report two cases undergoing electrophysiolog-ical studies for ventricular arrhythmias (VAs) associated withperipartum cardiomyopathy. Those two cases demonstratedthat subsequent pregnancies might result in deterioration ofVAs even though they exhibit no symptoms of heart failure.Those findings may clinically impact the decision makingwhen women with a history of peripartum cardiomyopathydesire to become pregnant again. The VA foci in both caseswere determined or suggested to be in the epicardium of theventricular outflow tract from the results of the catheterablation and electrophysiological study. Therefore, catheterablation of those VAs may be feasible but challenging.

Keywords Ventricular arrhythmia . Epicardial .

Ventricular outflow tract . Peripartum cardiomyopathy .

Radiofrequency catheter ablation

1 Introduction

Peripartum cardiomyopathy is a rare life-threatening car-diomyopathy of unknown cause that occurs in the peri-

partum period in previously healthy women [1, 2]. Thoughthere have been isolated case reports describing ventriculararrhythmias (VAs) associated with this cardiomyopathy, [3,4] the details about those arrhythmias remain unknown. Wereport two cases with those VAs that underwent anelectrophysiologic study.

2 Case 1

A 30-year-old woman was referred because of a reducedleft ventricular systolic function (ejection fraction=45%)and non-sustained ventricular tachycardia (NSVT). She hadbeen diagnosed with peripartum cardiomyopathy andfrequent multifocal PVCs (12,000 beats/day) after thedelivery of her second child. Six months after the diagnosis,her left ventricular systolic function had normalized(ejection fraction=58%). One year after the diagnosis, shehad a new pregnancy and NSVT with pre-syncope wasdocumented. Though she had no symptoms associated withheart failure, she underwent abortion. Written, informedconsent was obtained, and an electrophysiologic study wasperformed after all antiarrhythmic drugs had been discon-tinued for more than at least five half-lives prior to thestudy.

At baseline, there were frequent PVCs, and multifocalPVCs were recognized. All the PVCs exhibited a leftbundle branch block and right inferior axis QRS morphol-ogy and the precordial transition zone varied between leadsV3 and V4. None of those PVCs exhibited any electrocar-diographic characteristics that suggested an epicardialorigin [5, 6]. The main PVCs with a precordial transitionzone between leads V3 and V4 were identical to the

J Interv Card Electrophysiol (2009) 25:53–57DOI 10.1007/s10840-008-9325-z

T. Yamada (*) :H. T. McElderry :H. Doppalapudi :A. E. Epstein :V. J. Plumb :G. N. KayDivision of Cardiovascular Disease,University of Alabama at Birmingham,VH B147, 1670 University Boulevard, 1530 3rd AVE S,Birmingham, AL 35294-0019, USAe-mail: takumi-y@fb4.so-net.ne.jp

M. Muto : T. Okada :Y. MurakamiDivision of Cardiology,Aichi Prefectural Cardiovascular and Respiratory Center,Ichinomiya, Japan

documented NSVT (Fig. 1). Electroanatomic mapping wasperformed using a 7-French, 4-mm tip non-irrigatedablation catheter (Navi-Star™, Biosense Webster, DiamondBar, CA, USA) through a long sheath (8-French, SR0™, St.Jude Medical, AF Division, Minnetonka, MN, USA) viathe right femoral vein with the main PVC as the target aspreviously reported [7]. That revealed two separate siteswith early ventricular activation preceding the QRS onset atthe antero-septal aspect of the RVOT (Fig. 2). Pacing at thelower site (site 1) with the earliest ventricular activation of−18 ms relative to the QRS onset did not exhibit anexcellent pace map (Fig. 1), and pacing at the upper site(site 2) with a ventricular activation of −16 ms relative tothe QRS onset did not capture the myocardium even withthe maximum output. The voltage map of the RVOTrevealed a low voltage area in the peri-pulmonary valvularand anterior walls and those two sites with early ventricularactivation were located within the low voltage area (Fig. 3).Mapping of the left ventricular outflow tract (LVOT) wasthen performed using the same ablation catheter via theright femoral artery, but no early ventricular activationpreceding the QRS onset was found in the LVOT.Following that, mapping within the great cardiac vein(GCV) was performed using a 2-Fr octapolar electrodecatheter (Pathfinder™, Cardima, Fremont, CA, USA)(Fig. 2). Though during the targeted PVCs, the ventricularactivation within the GCV slightly preceded the QRS onset,it was later than that in the RVOT (Fig. 1). Finally, a coupleof RF applications with a target temperature of 60°C andmaximum power output of 50 W were delivered at sites 1and 2, resulting in no interruption of the PVCs (Fig. 2). Atthis point, an epicardial approach by a pericardial puncturewas considered. However, the patient refused the proposal ofthat approach and the session was ended. No complications

occurred. The patient also refused the proposal to implantan implantable cardioverter defibrillator and has beenfollowed up with the administration of amiodarone and abeta-blocker.

3 Case 2

A 43-year-old woman had three pregnancies in the past,with complications of preeclampsia and peripartum cardio-myopathy with the last pregnancy about 10 years ago. Shealso sometimes had short episodes of palpitations for over7 years. After she had a new pregnancy, the palpitationattacks became worse, but she had no symptoms associatedwith heart failure. Because she was diagnosed withsupraventricular tachycardia, frequent PVCs (right bundlebranch block and a right inferior axis QRS morphology,more than 20,000 beats/day) and a reduced left ventricularsystolic function (ejection fraction=25%), she was referredfor catheter ablation. Written, informed consent wasobtained, and an electrophysiologic study was performedafter all antiarrhythmic drugs had been discontinued formore than at least five half-lives prior to the study.

At baseline, the 12-lead electrocardiogram exhibitedbigeminal PVCs with a left bundle branch block and rightinferior axis QRS morphology and no pre-excitationsduring sinus beats (Fig. 4). In our laboratory, the transitionzone during the electrophysiological study sometimesdiffered from that before the catheter ablation probablybecause the right precordial leads were positioned superiorto the typical position, where a cutaneous pad forextrathoracic cardioversion was placed (Fig. 4). Formapping and pacing, multipolar electrode catheters werepositioned in the coronary sinus (CS), His bundle (HB)

200 ms 1 mV

I

II

III

aVR

aVL

aVF

SRPM

PVC

V1

V2

V3

V4

V5

V6

SRPM

PVC

IIIIII

aVR

aVLaVF

V1

V2

V3

V4

V5V6

GCVd

GCVm

GCVp

ABLdABLp

300 ms

V-QRS

V-QRS = 5 ms

ABL site 2(Site 1)(Site 1)

–

= 16 ms–

Fig. 1 Twelve-lead electrocar-diograms and cardiac tracingsshowing the ablation (ABL) sitein case 1. ABL The ablationcatheter, GCV the mappingcatheter within the great cardiacvein, PM pace map, PVCpremature ventricular contrac-tion, SR sinus rhythm, V-QRSthe local ventricular activationrelative to the QRS onset; X d(m,p) the distal (mid, proximal)electrode pair of the relevantcatheter

54 J Interv Card Electrophysiol (2009) 25:53–57

region and right ventricular apex. Supraventricular tachy-cardia was induced by programmed ventricular stimulationand diagnosed as atrioventricular reciprocating tachycar-dia. Successful ablation of the accessory pathway could beachieved at the right posterior septum without anycomplications. Next, mapping of the PVCs was performed.Because ventricular activation preceding the QRS onsetwas never obtained in the right ventricular outflow tract,mapping of the LVOT was then performed using 7-French,4-mm tip non-irrigated ablation catheter (Celsius™, Bio-sense Webster, Diamond Bar, CA, USA) via the rightfemoral artery. Although the earliest ventricular activationwhich preceded the QRS onset by 17 ms was obtained inthe left coronary cusp, pacing at that site did not exhibit anexcellent pace map (Figs. 4 and 5). The CS catheter wasthen advanced to the GCV and the earlier ventricularactivation which preceded the QRS onset by 33 ms was

recorded from the distal electrode pair of the CS catheter(Fig. 5). Pacing at that site exhibited an excellent pace map(Fig. 4). The CS catheter was pulled back and instead theablation catheter was introduced into the GCV via the rightjugular vein. An RF application with a target temperatureof less than 55°C and power output titrated up to 30 W wasthen delivered at the earliest ventricular activation site inthe GCV, resulting in the elimination of the PVCs. Onlyfluoroscopic imaging was used and no cineography imageswere recorded in order to reduce the X ray exposure to thepregnant patient. No complications occurred. Soon afterthe procedure, the patient underwent an abortion becausethe early interruption of the pregnancy might prevent anyprogression of her cardiomyopathy. During more than3 months of follow-up, the patient has been free of anysupraventricular or ventricular arrhythmic episodes. How-ever, echocardiographic examination performed 3 months

Fig. 3 Avoltage map of the rightventricular outflow tract in case 1.The purple and red indicate theareas with a voltage of the localbipolar electrogram >=1.5 mVand <=0.5 mV, respectively. Theabbreviations are as in Fig. 2

Fig. 2 An electroanatomic mapobtained during the targeted PVCs(left) and fluoroscopic imagesexhibiting the ablation sites(right) in case 1. LAO The leftanterior oblique view, PA-CAUposteroanterior-caudal, PV pul-monary valve, RAO the rightanterior oblique view. The otherabbreviations are as in Fig. 1

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after the procedure, revealed no improvement in her leftventricular systolic function.

4 Discussion

The long-term outcome of peripartum cardiomyopathymainly depends on the recovery of the left ventricularfunction. Approximately half of the patients recovercompletely [1, 2]. However, patients whose left ventriculardysfunction does not resolve within 6 months following

delivery are known to have an extremely high mortality rate[1, 3, 8]. Additionally, it has been reported that in womenwho have had peripartum cardiomyopathy, subsequentpregnancies may be associated with deleterious fetal andmaternal outcomes such as premature delivery and maternalcardiac dysfunction, including symptomatic heart failureand even death [8]. However, to the best of our knowledge,there have been no reports describing the effect ofsubsequent pregnancies on VAs in women with a historyof peripartum cardiomyopathy. In the first case in thisreport, a subsequent pregnancy led to the PVCs developing

Fig. 4 Twelve-lead electrocar-diograms in case 2. Note that theQRS morphologies of the PVCsdiffered during baseline and theelectrophysiological studybecause the right precordialleads were positioned superiorto the typical position, where acutaneous pad for extrathoraciccardioversion was placed duringthe study. LCC Left coronarycusp. The other abbreviationsare as in Fig. 1

II

V1

V3

HBd

LCCd

LCCp

I

aVR

V5

GCVd

HBp

GCVm

GCVp

V2

V4

V6

III

aVL

aVF

GCV

GCV

RV

RV

V-QRS

200 ms

V-QRS

LAO

RAO= 33 ms–

= 17 ms–

Fig. 5 The successful ablationsite in case 2. The white lineindicates the angiographic cath-eter deployed in the ostium ofthe left coronary artery. HB Hisbundle, RV right ventricle. Theother abbreviations are as in theprevious figures

56 J Interv Card Electrophysiol (2009) 25:53–57

into NSVT. In the second case, even in the absence of heartfailure, the atrioventricular reciprocating tachycardia mighthave been accentuated by the triggering PVCs whichbecame worse during the progression of the peripartumcardiomyopathy due to the subsequent pregnancy. There-fore, the present cases demonstrated that subsequentpregnancies might result in deterioration of VAs in thosewomen. This report may clinically impact the decisionmaking when those women desire to become pregnantagain. Because the present cases exhibited no symptomsassociated with heart failure, the deterioration of the VAsmight not have been caused by peripartum cardiomyopathy.However, in the first case, the arrhythmogenic substrate ofthe VAs was suggested to be located within the damagedmyocardium with a low voltage. Additionally, it has beendemonstrated that in women who have had peripartumcardiomyopathy, subsequent pregnancies may cause asymp-tomatic reworsening of the left ventricular dysfunction [8].Therefore, we believe that in the present cases, thedeterioration of the VAs might have preceded the decom-pensation of the heart failure or may have occurred in thecases with asymptomatic reworsening of the left ventriculardysfunction.

In this report, all VA foci in both cases were determinedor suggested to be in the ventricular outflow tract by theresults of the catheter ablation and electrophysiologicalstudy. In Palma et al.’s report, a foci of NSVT associatedwith peripartum cardiomyopathy was found in the anteriorwall of the right ventricle [3]. However, the 12-leadelectrocardiogram of the VAs associated with peripartumcardiomyopathy in Gemici et al.’s report was very similar tothat in the first case in this report [4]. Those findingssuggested that the foci of the VAs associated withperipartum cardiomyopathy may be likely to be in theventricular outflow tract even though peripartum cardio-myopathy is normally a diffuse myocardial disease and notregion-specific like the other cardiomyopathies.

The VAs during the acute phase of peripartum cardio-myopathy may be expected to resolve as the left ventricularfunction recovers [4]. However, in VAs with peripartumcardiomyopathy without recovery of the left ventricularfunction, catheter ablation may be considered as an optionaltherapy to improve the prognosis as is the case with theother non-ischemic cardiomyopathies [9, 10]. In the firstcase of this report, an epicardial focus of the VAs withmultiple endocardial breakout sites was suggested by theepicardial and endocardial mapping, and in the second case,the focus of the PVCs was determined to be epicardial by

that being the successful ablation site. To the best of ourknowledge, this is the first report that has demonstratedepicardial VA foci associated with peripartum cardiomyop-athy from the electrophysiologic findings. In Palma et al.’sreport, the results of the catheter ablation and histopatho-logical analyses suggested an epicardial foci of the NSVT[3]. Those findings suggest that foci of the VAs associatedwith peripartum cardiomyopathy may likely be epicardial.Therefore, catheter ablation of VAs associated with peri-partum cardiomyopathy may be feasible but challenging.

References

1. Homans, D. C. (1985). Peripartum cardiomyopathy. The NewEngland Journal of Medicine, 312, 1432–1437.

2. Demakis, J. G., Rahimtoola, S. H., Sutton, G. C., Meadows, W.R., Szanto, P. B., Tobin, J. R., et al. (1971). Natural course ofperipartum cardiomyopathy. Circulation, 44, 1053–1061.

3. Palma, E. C., Saxenberg, V., Vijayaraman, P., Ferrick, K. J.,Gross, J. N., Kim, S. G., et al. (2001). Histopathologicalcorrelation of ablation lesions guided by noncontact mapping ina patient with peripartum cardiomyopathy and ventricular tachy-cardia. Pacing and Clinical Electrophysiology, 24, 1812–1815.

4. Gemici, G., Tezcan, H., Fak, A. S., & Oktay, A. (2004). Peripartumcardiomyopathy presenting with repetitive monomorphic ventriculartachycardia. Pacing and Clinical Electrophysiology, 27, 557–558.

5. Berruezo, A., Mont, L., Nava, S., Chueca, E., Bartholomay, E., &Brugada, J. (2004). Electrocardiographic recognition of theepicardial origin of ventricular tachycardias. Circulation, 109,1842–1847.

6. Daniels, D. V., Lu, Y. Y., Morton, J. B., Santucci, P. A., Akar, J.G., Green, A., et al. (2006). Idiopathic epicardial left ventriculartachycardia originating remote from the sinus of Valsalva:electrophysiological characteristics, catheter ablation, and identi-fication from the 12-lead electrocardiogram. Circulation, 113,1659–1666.

7. Yamada, T., Murakami, Y., Yoshida, N., Okada, T., Toyama, J.,Yoshida, Y., et al. (2007). Efficacy of electroanatomic mapping inthe catheter ablation of premature ventricular contractions origi-nating from the right ventricular outflow tract. Journal ofInterventional Cardiac Electrophysiology, 19, 187–194.

8. Elkayam, U., Tummala, P. P., Rao, K., Akhter, M. W., Karaalp, I.S., Wani, O. R., et al. (2001). Maternal and fetal outcomes ofsubsequent pregnancies in women with peripartum cardiomyop-athy. The New England Journal of Medicine, 344, 1567–1571.

9. Soejima, K., Stevenson, W. G., Sapp, J. L., Selwyn, A. P., Couper,G., & Epstein, L. M. (2004). Endocardial and epicardial radio-frequency ablation of ventricular tachycardia associated withdilated cardiomyopathy: the importance of low-voltage scars.Journal of the American College of Cardiology, 43, 1834–1842.

10. Dalal, D., Jain, R., Tandri, H., Dong, J., Eid, S. M., Prakasa, K., etal. (2007). Long-term efficacy of catheter ablation of ventriculartachycardia in patients with arrhythmogenic right ventriculardysplasia/cardiomyopathy. Journal of the American College ofCardiology, 50, 432–440.

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