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Case Reports

Arrhythmogenic Right Ventricular Cardiomyopathy in Pregnancy


A Case Report and Review of the Literature
Aysen Agir,1 MD, Serdar Bozyel,1 MD, Umut Celikyurt,1 MD, Onur Argan,1 MD,
Irem Yilmaz,1 MD, Kurtulus Karauzum,1 MD, and Ahmet Vural,1 MD

Summary
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is predominantly a genetically determined heart muscle
disorder that is characterized by fibro-fatty replacement of the right ventricular (RV) myocardium.1) The clinical spec-
trum of ARVC may represent from asymptomatic premature ventricular complexes to ventricular tachycardia (VT) and
sudden cardiac death (SCD). It is a well-known leading cause of SCD in young adults.2,3)
There is no general consensus on the management of ARVC in pregnancy, and the preferred mode of delivery is
uncertain. Herein, we report a case of ARVC diagnosed at 20 weeks of gestation following a sustained VT and treated
with an implantable cardiac defibrillator (ICD). We also reviewed the current knowledge and approach to ARVC in preg-
nancy since the literature on this condition is based on case reports. (Int Heart J 2014; 55: 372-376)

Key words: Implantable cardioverter defibrillator

A
 RVC is a primary disease of the myocardium charac- complex regular tachycardia, she was transferred to our emer-
terized by fibro-adipocytic replacement of myocytes, gency department without any treatment by an ambulance in
mostly in the right ventricle. It should be suspected 10 minutes. She had no history of cardiovascular or any other
in a young or middle aged adult who has ventricular arrhyth- disease. The patient was not on any medication but was taking
mias with left bundle-branch block (LBBB) morphology and supportive multivitamins. There was a history of smoking, but
does not have any other cardiac disease. Following the diagno- no alcohol consumption or illicit drug use. She had not experi-
sis of ARVC, the most important management decision is enced any problems during her previous pregnancies or deliv-
whether or not to implant an ICD for the treatment of sustained eries. Her father had coronary artery disease but there was no
ventricular arrhythmias for prevention of SCD. It is now a family history of SCD among young adults.
standard practice for ARVC patients presenting with sustained In the admission period, she was hemodynamically sta-
VT and/or ventricular fibrillation (VF) to undergo placement ble. On physical examination, her blood pressure was 110/60
of an ICD because of a high risk of recurrent VT and/or mmHg and heart rate was 200-210 bpm. Electrocardiography
SCD.4-6) (ECG) showed sustained VT with inferior axis and negative
The number of woman with ARVC reportedly continues concordance (Figure 1). In the emergency department, she was
to increase and due to insufficient published data, it is rather given adenosine 6 mg and then 12 mg intravenously (IV) in
difficult to evaluate the risk and management of ARVC during order to distinguish the tachycardia origin. However, sinus
pregnancy and delivery. The diagnosis of ARVC based on de- rhythm could not be restored so she was referred to us. The
velopment of VT during pregnancy and subsequent ICD im- spontaneous sinus rhythm was established when we evaluated
plantation is a rarely seen situation. the patient. She was then admitted to the coronary intensive
Here we describe a patient diagnosed as ARVC at 20 care unit.
weeks of gestation and managed with ICD placement during Ventricular tachycardia (VT), at a rate of 200-210/min,
pregnancy. that neither disturbed the hemodynamics nor caused any symp-
toms other than palpitations, occurred during admission to the
coronary intensive care unit. Initially, 5 mg (IV) metoprolol
Case Report was given followed by a second dose of 5 mg as sinus rhythm
was not restored. Approximately 4 minutes after achieving a
A-30-year-old woman (gravida 3, para 2), was admitted short period of sinus rhythm, VT at a rate of 190-200/min oc-
to Gynaecology and Obstetrics Hospital due to heart palpita- curred again, causing no change in the hemodynamics. As VT
tions at 20 weeks of gestation. She had been asymptomatic un- was terminated and reoccurred again and again with the re-
til 20 weeks. When cardiac monitoring showed wide QRS peated dose of metoprolol 5 mg, amiodarone 150 mg (IV) was

From the 1 Department of Cardiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey.
Address for correspondence:Aysen A. Agir, MD, Department of Cardiology, Faculty of Medicine, Kocaeli University, Umuttepe, 41380, Kocaeli, Turkey. E-mail:
aagacdiken@yahoo.com
Received for publication August 28, 2013. Revised and accepted January 2, 2014.
Released in advance online on J-STAGE June 5, 2014.
All rights reserved by the International Heart Journal Association.
372
Vol 55
No 4 RIGHT VENTRICULAR CARDIOMYOPATHY IN PREGNANCY 373

Figure 1. ECG showed sustained ventricular tachycardia with inferior Figure 3. Cardiac MRI demonstrated the typical manifestation of ARVC;
axis and negative concordance. (arrows) enlargement of right ventricle and atrium without enlargement of
left ventricle and atrium; note severely and globally hypokinetic right ven-
tricle with lateral wall akinesia.

ed normal sinus rhythm with frequent premature ventricular


complexes (PVC) (3995/day), ventricular couplets (400/day),
and triplets (30/day), and runs of non-sustained VT, up to 11
beats in duration (2 times/day, 11 salvos at maximum). Cardiac
MRI presented the typical manifestation of ARVC indicating
an enlargement of RV and right atrium without enlargement of
the left ventricle and atrium; the RV was severely globally hy-
pokinetic with lateral wall akinesia and the RV ejection frac-
tion was 19% (Figure 3). The diagnosis of ARVC was based
upon a set of major and minor criteria proposed by the Interna-
tional Task Force.7) She had two major criteria; 1) By MRI, her
right ventricle was severely hypokinetic with lateral wall aki-
nesia and the RV ejection fraction was 19%. 2) Her ECG dem-
onstrated inverted T-waves in leads V1–V4 in the absence of
Figure 2. 12-lead ECG demonstrated normal sinus rhythm with incom- complete RBBB. The patient also had one minor criterion; On
plete right bundle-branch block. Inverted T-waves in leads V1–V4 were Holter analysis there were 3995 ventricular extrasystoles per
observed. day. Thus, our patient fulfilled the latest criteria for definite
ARVC diagnosis.
A fetal echocardiogram was performed at the 21st week
given. Instead of amiodarone, electrical cardioversion was con- of pregnancy and no abnormality was detected. Her other two
sidered, but a single dose of amiodarone was preferred for sta- children were also evaluated by echocardiogram and no abnor-
bilizing the rhythm as the patient refused electrical cardiover- mality was found. The relative benefits of ICD placement vs.
sion. Along with ongoing VT the patient reported feeling antiarrhythmics for the prevention of SCD were discussed with
dizzy. Shortly afterwards she had a severe vomiting episode the patient. After obtaining informed consent, a single chamber
and then her blood pressure decreased notably. Subsequent to ICD was placed under local anaesthesia without any complica-
the vomiting attack, the VT was noticed to be terminated with- tions of note at 21 weeks of gestation. The abdomen of the pa-
in seconds and the patient became hemodynamically stable. tient was protected by a lead blanket. The patient tolerated the
Once the sinus rhythm was re-established, the follow-up was procedure well. The patient and fetus were monitored for 3
uneventful. days after ICD implantation. In this period, numerous nonsus-
A 12-lead ECG demonstrated normal sinus rhythm with tained VT episodes were observed and metoprolol was given
incomplete right bundle-branch block (RBBB). Inverted T- to the patient to decrease her palpitations. A consultation with
waves in leads V1–V4 were apparent (Figure 2). CKMB levels the Department of Obstetrics about the usage and dosage of
were modestly elevated and troponin I was weakly positive. metoprolol therapy revealed no contraindication to beta-block-
Serial cardiac enzymes were: CK-MB 2.7 – 5.1 – 8.7 (normal ers for her pregnancy. Finally, she was discharged on metopro-
2.0-7.2 ng/mL), troponin I 0.085–0.19–0.10 (normal 0.010- lol (50 mg/day). She was monitored very closely for the re-
0.023 ng/mL); other laboratory tests and chest X-rays were maining trimesters of her pregnancy. At 32 weeks of gestation,
normal. Transthoracic echocardiography showed enlargement an echocardiogram demonstrated no changes in her cardiac
of RV (31 mm) and moderate tricuspid valve insufficiency. The functions and the device did not record any arrhythmias. She
RV ejection fraction (EF) was 30% and the left ventricular EF had no further problems during the rest of her pregnancy.
was normal. Twenty-four hour Holter monitoring demonstrat- She delivered her baby in the 37th week of pregnancy by
Int Heart J
374 AGIR, ET AL July 2014

caesarean section due to fetal distress (oligohydramnios) under can induce fatal arrhythmia in ARVC patients. The initiation of
spinal anaesthesia. The device was left off during delivery and VT was associated with a typical catecholamine surge seen on
no complications, such as arrhythmias, were observed during exercise in the setting of the presence of arrhythmogenic sub-
labour. The newborn was a healthy boy, with a birth weight of strate characteristic of these patients.21,22) Therefore, as exercise
3800 grams and APGAR score of 9/10 at the 1st and 5th min- may increase heart rate in a pregnant woman with a diagnosis
utes. Echocardiography of the baby was normal. After 3 days of ARVC, it is better to recommend to avoid exercise.
of hospitalization, she was discharged with metoprolol (50 mg/ Considering the paucity of reported data in regard to the
day) and continued breastfeeding for 6 months. The analysis of management of ARVC patients during pregnancy, it is still un-
stored electrograms revealed no significant ventricular arrhyth- known whether an arrhythmic event could be preventable by
mia throughout the 6 months of ICD implantation. any treatments. In a case reported by Iriyama, et al,18) the pro-
pranolol treatment that the patient had been taking was not
given during the pregnancy on her own request and VT oc-
Discussion curred at the 34th week. Similarly in the case of Güdücü, et
al,17) the patient taking regular metoprolol and propafenone
ARVC is characterized pathologically by myocardial at- treatment for 3 years with the diagnosis of ARVC refused to
rophy, fibrofatty replacement, fibrosis and ultimate thinning of use the drugs on her own request and she became symptomatic
the wall with chamber dilatation and aneurysm formation.8) at the 32nd week. In her Holter ECG at the 32nd week, frequent
These changes consequently produce electrical instability pre- PVC attacks were detected. In our case, the patient was dis-
cipitating VT and SCD.9) ARVC should be suspected in a charged with metoprolol treatment after implantation of an
young patient with palpitations, syncope, or aborted SCD. VT ICD at the 21st week and she was asymptomatic until delivery.
with LBBB morphology is the classic presentation. Other elec- The analysis of stored electrograms revealed no significant
trocardiographic abnormalities such as inverted T waves in ventricular arrhythmia at 6 months post ICD implantation un-
right precordial leads (V1–V3) and frequent premature ven- der metoprolol treatment.
tricular complexes (PVCs), even in asymptomatic patients, Treatment of ARVC is based on the prevention of ar-
should arouse the suspicion for this cardiomyopathy.10) The rhythmia with beta-blockers, especially with sotalol, which
main goal of therapy is to prevent serious events, which re- prevents arrhythmia caused by ARVC in 60% to 70% of pa-
quires identifying high-risk patients for malignant arrhythmias tients.23) Propafenone was reported to be safe in the treatment
and SCD.11-13) of ventricular arrhythmia during pregnancy.24) Combination
The diagnosis of ARVC is based upon a set of major and therapy (Class IC drugs plus beta-blocking drugs, or amiodar-
minor criteria proposed by the International Task Force.7) Pa- one plus beta-blocking drugs) frequently is effective in pre-
tients must either meet two major criteria, one major and two venting recurrent VT. Unfortunately, amiodarone therapy dur-
minor criteria, or 4 minor criteria to be diagnosed as ARVC. ing pregnancy (category D) may cause fetal/neonatal
Two of the major and one of the minor diagnostic criteria for hypothyroidism and, less frequently, goiter.25) Thus, the use of
ARVC according to the new Task Force Criteria were present amiodarone in pregnancy should be limited to VTs which are
in our patient, ie, inverted T waves in leads V1-V4 in the ab- sustained, monomorphic, hemodynamically unstable, refracto-
sence of RBBB on ECG, severe hypokinesis of the right ven- ry to electrical cardioversion, or not responding to other
tricle with lateral wall akinesia and reduced ejection fraction drugs.26) In our case, although the hemodynamics were stable,
(19%) of the RV observed by MRI, and 3995 ventricular extra- by considering the fetal side effects, electrical cardioversion
systoles per 24 hours on ECG Holter examination. which is reliable in every phase of pregnancy might have been
Pregnancies with dilated and hypertrophic cardiomyopa- preferred instead of amiodarone. However, single dose-amio-
thies are common, but only a few cases of pregnancies with darone was preferred because the patient refused the electrical
ARVC have been reported.14-18) Therefore, it is difficult to as- cardioversion. Only a 150 mg-loading dose was given to the
sess the risks of pregnancy and delivery in patients with patient and a maintenance infusion dose could not be adminis-
ARVC. Furthermore, sustained VT developed during the preg- tered.
nancy in only one of these cases. In the patient mentioned in Prophylactic therapy with a cardioselective beta-blocking
this case, VT had developed following physical exercise at the agent, such as metoprolol, may be effective.26) There is consid-
34th week of both her pregnancies. As sinus rhythm was erable experience with beta blockers and no teratogenicity has
achieved spontaneously approximately 20 seconds later, there been reported to date, even if in some cases intrauterine growth
was no need for medical or electrical cardioversion.18) In our delay and bradycardia have been reported.27) In our case, al-
case VT developed in the 20th week, during rest and continued though oligohydramnios was detected at the 37th week of preg-
for minutes. Sinus rhythm could be restored immediately by nancy, intrauterine growth retardation was not evident in the
medical cardioversion. fetus and the baby was born healthy.
During pregnancy, plasma volume, cardiac output and ICD use is recommended for patients who have a docu-
heart rate increase, hematocrit decreases, and physiologic ane- mented episode of sustained VT or cardiac arrest or who have
mia is established. VT may be triggered during pregnancy as a high-risk features for SCD. Recently, ICD therapy for the pre-
result of these hemodynamic changes.19,20) It is noteworthy that vention of SCD in patients with ARVC is becoming standard
the published reports have found that most of these patients treatment.3) However, little is known regarding the outcome of
tolerate these conditional hemodynamic changes induced by pregnancy in women with ICD. We have found only one pub-
pregnancy. Although pregnancy alone is a physiological stress lished case of a patient diagnosed with ARVC during her preg-
factor, in our patient there was no other reason inducing the ar- nancy and treated with ICD placement.15) In the last years, only
rhythmia. Nevertheless, it has been reported that exercise itself a few studies of ICD therapy during pregnancy have found that
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No 4 RIGHT VENTRICULAR CARDIOMYOPATHY IN PREGNANCY 375

the majority of women completed and tolerated pregnancy and culation 2003; 108: 3084-91.
delivered without serious complications.15,28-30) Thus, ICD 6. Roguin A, Bomma CS, Nasir K, et al. Implantable cardioverter-
defibrillators in patients with arrhythmogenic right ventricular
placement should be considered in pregnant woman with
dysplasia/cardiomyopathy. J Am Coll Cardiol 2004; 43: 1843-52.
ARVC to prevent episodes of SCD. 7. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhyth-
The preferred mode of delivery in women with ARVC is mogenic right ventricular cardiomyopathy/dysplasia: proposed
uncertain. Bauce, et al 14) reported that a caesarean section was modification of the task force criteria. Circulation 2010; 121:
performed in 4 of the 6 patients in whom the disease was con- 1533-41.
sidered to be more dangerous on the basis of both morphologi- 8. Thiene G, Basso C, Danieli G, Rampazzo A, Corrado D, Nava A.
cal changes and the history of ventricular arrhythmias. The re- Arrhythmogenic right ventricular cardiomyopathy a still underrec-
ognized clinic entity. Trends Cardiovasc Med 1997; 7: 84-90.
maining two cases with only isolated PVC or no arrhythmias 9. Mehta D, Odawara H, Ward DE, McKenna WJ, Davies MJ,
underwent spontaneous vaginal delivery. Iriyama, et al 17) pre- Camm AJ. Echocardiographic and histologic evaluation of the
ferred to perform elective caesarean section due to a history of right ventricle in ventricular tachycardias of left bundle branch
major arrhythmias. Our patient delivered by caesarean section block morphology without overt cardiac abnormality. Am J Cardi-
due to fetal indication (oligohydramnios). ol 1989; 63: 939-44.
It remains unclear whether an ICD should be on or off 10. Niroomand F, Carbucicchio C, Tondo C, et al. Electrophysiologi-
cal characteristics and outcome in patients with idiopathic right
during delivery. Recurrence of VT may result in decreasing
ventricular arrhythmia compared with arrhythmogenic right ven-
placental perfusion due to maternal hypotension and could be tricular dysplasia. Heart 2002; 87: 41-7.
dangerous for the fetus. In contrast, ICD shocks are a concern 11. Epstein AE, DiMarco JP, Ellenbogen KA, et al. ACC/AHA/HRS
for the safety of the fetus, although the amount of energy trans- 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm
ferred to the uterus is very small and the fetal heart has a high Abnormalities: a report of the American College of Cardiology/
fibrillatory threshold.28,31) In a few studies, no arrhythmias or American Heart Association Task Force on Practice Guidelines
ICD discharges were precipitated during delivery.28,29) As the (Writing Committee to Revise the ACC/AHA/NASPE 2002
Guideline Update for Implantation of Cardiac Antiarrhythmia De-
status of the ICD during delivery appears to have no effect on vices): developed in collaboration with the American Association
the overall outcome, in fact, leaving the device “on” during for Thoracic Surgery and Society of Thoracic Surgeons. Circula-
vaginal delivery is recommended, whereas it should be “off” in tion 2008; 117: e350-408.
the case of caesarean section, because electrocautery is in- 12. Wichter T, Breithardt G. Implantable cardioverter-defibrillator
volved.32) We left the device turned off during caesarean sec- therapy in arrhythmogenic right ventricular cardiomyopathy: a
tion, since electrocautery was to be used. role for genotyping in decision-making? J Am Coll Cardiol 2005;
45: 409-11.
Our case is the first report of an ARVC patient who had a
13. Zipes DP, Camm AJ, Borggrefe M, et al. ACC/AHA/ESC 2006
sustained VT that required medical cardioversion to be termi- guidelines for management of patients with ventricular arrhythmi-
nated during pregnancy. This is also the second case of a preg- as and the prevention of sudden cardiac death: a report of the
nant ARVC patient who underwent ICD implantation during American College of Cardiology/American Heart Association
her pregnancy. On the basis of other published studies and our Task Force and the European Society of Cardiology Committee
case report, it can be said that the pregnancy is well tolerated for Practice Guidelines (Writing Committee to Develop Guide-
in women affected by ARVC and can be managed successfully lines for Management of Patients With Ventricular Arrhythmias
and the Prevention of Sudden Cardiac Death). J Am Coll Cardiol
with close monitoring and antiarrhythmic drugs when neces- 2006; 48: e247-346.
sary. ICD implantation also should be considered to prevent 14. Bauce B, Daliento L, Frigo G, Russo G, Nava A. Pregnancy in
SCD during pregnancy to protect the life of the mother. Preg- women with arrhythmogenic right ventricular cardiomyopathy/
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