International Journal of Cardiology 203 (2016) 1133–1134
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Correspondence
Non-sustained ventricular tachycardia in a pregnant woman with repaired Tetralogy of Fallot: A challenging case Seiji Ito ⁎, Karen K. Stout, Melissa R. Robinson University of Washington, 1959 NE Pacific Street Seattle, WA 98195, USA
a r t i c l e
i n f o
Article history: Received 26 August 2015 Accepted 19 September 2015 Available online 21 November 2015 Keywords: Tetralogy of Fallot Ventricular arrhythmia Sudden cardiac death Pregnancy Implantable cardioverter–defibrillator
Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease, and most patients survive to reproductive age in the current era. Pulmonary regurgitation, right ventricular (RV) dilation and dysfunction are commonly seen in adult patients with repaired TOF, thus pulmonary valve replacement may be indicated to restore pulmonary valve function in symptomatic patients. Also, ventricular arrhythmia such as ventricular tachycardia (VT) and sudden cardiac death (SCD) are known morbidity and mortality. The incidence of SCD in this population is reported to be 1.2 to 3% per decade. Antiarrhythmic agents, VT ablation, or implantable cardioverter–defibrillator (ICD) placement, are among treatment options. The purpose of this report is to describe a case of pregnant woman with repaired Tetralogy of Fallot, who had unique challenge in the management of non-sustained ventricular tachycardia (NSVT) during pregnancy. A 25 year-old pregnant woman at 11 weeks of gestation was referred to our adult congenital heart disease (ACHD) clinic. The patient had history of TOF, and undergone complete repair at age of 3 years, including patch closure of a ventricular septal defect (VSD) and transannular patch repair. At the age of 10, she required an aortic root replacement with bioprosthetic aortic valve for a dilated aortic root and progressive aortic regurgitation in the presence of bicuspid aortic valve. At the ACHD clinic visit, the patient had only mild fatigue and occasional palpitations. Initial echocardiography in our institution showed normal left ventricular function, moderate aortic stenosis, mild aortic regurgitation, moderate to severe pulmonary regurgitation (PR), RV ⁎ Corresponding author. E-mail address: [email protected] (S. Ito).
http://dx.doi.org/10.1016/j.ijcard.2015.09.046 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
dilation, and no residual VSD. Her cardiac magnetic resonance imaging, which was performed during second trimester, showed severe PR with regurgitant fraction of 54%, RV end-diastolic volume index of 215 mL/body surface area, RV ejection fraction (EF) of 48%. Shortly after the initial clinic visit, a 30-day event monitor was placed for increased episodes of palpitation. At 15 weeks of gestation, she had 2–3 days history of increasing fatigue and chest tightness, therefore, she was hospitalized for further management. Brain natriuretic peptide level, troponin, blood cultures, and electrocardiogram were obtained and they were unremarkable. A follow-up echocardiogram showed normal biventricular function, and other findings were unchanged. However, an event monitor tracing activated for palpitation showed NSVT (Fig. 1). After discussion among ACHD, high-risk obstetrics, and electrophysiology teams, low dose Atenolol was started. Unfortunately, patient developed symptomatic sinus bradycardia and hypotension on Atenolol, while patient continued to have episodes of NSVT. At this point, after thorough discussion with patient and her husband, decision was made to proceed with ICD placement. Implantation of dual chamber ICD in pre-pectoral space was performed under moderate sedation with three-dimension electroanatomic mapping without use of fluoroscopy at 16 weeks of gestation. Lower rate limit was set at 60 beat per minute for back up pacing, and the patient was started on Sotalol. With titration of Sotalol dose, her palpitation and fatigue improved. Within 3 days after ICD placement, no more NSVT was seen on telemetry monitor. The patient was discharged home on Sotalol. In follow up visits, she was doing well without episodes of NSVT or ICD shocks. This case illustrated unique challenge in management of NSVT during pregnancy in a patient with repaired TOF. An electrophysiology study with mapping and ablation, and surgical pulmonary valve replacement could have been offered if the case had not been complicated with pregnancy. Management of this case was also complicated with inability to tolerate an antiarrhythmic agent due to sinus bradycardia and hypotension. In the recent expert consensus statement, ICD was recommended as class I recommendation for these three groups: 1. Survivors of cardiac arrest due to ventricular arrhythmia, 2. Spontaneous sustained VT, and 3. Left ventricular EF equal or less than 35%. Although supporting data are less robust, this consensus statement also recommended ICD therapy as class IIa recommendation, specifically for adults with TOF with risk factors for SCD, including left ventricular systolic or diastolic dysfunction, NSVT, QRS duration equal or grater than 180 ms, extensive RV scarring, or inducible sustained VT. Catheter ablation of VT was listed as class I recommendation, as an adjunctive therapy for recurrent
1134
Correspondence
Fig. 1. An event monitor tracing of NSVT in the case. VT seen starting the third beat (a fusion beat) in the pre-trigger tracing, which continued through the half of post-trigger tracing before the rhythm spontaneously converted back to sinus rhythm.
monomorphic VT, a VT storm, or multiple appropriate shocks that are not manageable by ICD programming or antiarrhythmic therapy in ACHD patients [1]. Antiarrhythmic agents such as Amiodarone or Sotalol can be used to reduce VT burden and ICD shocks in this population. Amiodarone use for pregnant women is not favored due to its known adverse fetal risk. Also, long-term use of Amiodarone in young patients is generally discouraged due to adverse effects. Methods to minimize radiation dose during ICD placement were reported including the use of transesophageal echocardiogram or threedimension electroanatomic mapping [2]. Recent retrospective study of 20 pregnancies in 12 women (including 2 women with congenital heart disease) carrying ICD did not show clear association between ICD and adverse obstetric or neonatal outcomes [3]. In conclusion, an ICD implantation in conjunction with the use of antiarrhythmic agent is safe, and can be an option for management of
NSVT during pregnancy in selected ACHD patients. The number of similar cases is expected to increase in the near future with the growth of ACHD population.
References [1] P. Khairy, G.F. Van Hare, S. Balaji, C.I. Berul, F. Cecchin, M.I. Cohen, et al., PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease, Heart Rhythm 11 (2014) e102–e165. [2] S. Sideris, A. Kasiakogias, C. Aggeli, K. Manakos, G. Trantalis, K. Gatzoulis, et al., Implantation of a defibrillator in a pregnant woman with hypertrophic cardiomyopathy under echocardiographic guidance: a case report, Int. J. Cardiol. 179 (2015 Jan 20) 323–324. [3] S. Boulé, L. Ovart, C. Marquié, E. Botcherby, D. Klug, C. Kouakam, et al., Pregnancy in women with an implantable cardioverter–defibrillator: is it safe? Europace 16 (11) (2014 Nov) 1587–1594.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Correspondence
Non-sustained ventricular tachycardia in a pregnant woman with repaired Tetralogy of Fallot: A challenging case Seiji Ito ⁎, Karen K. Stout, Melissa R. Robinson University of Washington, 1959 NE Pacific Street Seattle, WA 98195, USA
a r t i c l e
i n f o
Article history: Received 26 August 2015 Accepted 19 September 2015 Available online 21 November 2015 Keywords: Tetralogy of Fallot Ventricular arrhythmia Sudden cardiac death Pregnancy Implantable cardioverter–defibrillator
Tetralogy of Fallot (TOF) is a common cyanotic congenital heart disease, and most patients survive to reproductive age in the current era. Pulmonary regurgitation, right ventricular (RV) dilation and dysfunction are commonly seen in adult patients with repaired TOF, thus pulmonary valve replacement may be indicated to restore pulmonary valve function in symptomatic patients. Also, ventricular arrhythmia such as ventricular tachycardia (VT) and sudden cardiac death (SCD) are known morbidity and mortality. The incidence of SCD in this population is reported to be 1.2 to 3% per decade. Antiarrhythmic agents, VT ablation, or implantable cardioverter–defibrillator (ICD) placement, are among treatment options. The purpose of this report is to describe a case of pregnant woman with repaired Tetralogy of Fallot, who had unique challenge in the management of non-sustained ventricular tachycardia (NSVT) during pregnancy. A 25 year-old pregnant woman at 11 weeks of gestation was referred to our adult congenital heart disease (ACHD) clinic. The patient had history of TOF, and undergone complete repair at age of 3 years, including patch closure of a ventricular septal defect (VSD) and transannular patch repair. At the age of 10, she required an aortic root replacement with bioprosthetic aortic valve for a dilated aortic root and progressive aortic regurgitation in the presence of bicuspid aortic valve. At the ACHD clinic visit, the patient had only mild fatigue and occasional palpitations. Initial echocardiography in our institution showed normal left ventricular function, moderate aortic stenosis, mild aortic regurgitation, moderate to severe pulmonary regurgitation (PR), RV ⁎ Corresponding author. E-mail address: [email protected] (S. Ito).
http://dx.doi.org/10.1016/j.ijcard.2015.09.046 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.
dilation, and no residual VSD. Her cardiac magnetic resonance imaging, which was performed during second trimester, showed severe PR with regurgitant fraction of 54%, RV end-diastolic volume index of 215 mL/body surface area, RV ejection fraction (EF) of 48%. Shortly after the initial clinic visit, a 30-day event monitor was placed for increased episodes of palpitation. At 15 weeks of gestation, she had 2–3 days history of increasing fatigue and chest tightness, therefore, she was hospitalized for further management. Brain natriuretic peptide level, troponin, blood cultures, and electrocardiogram were obtained and they were unremarkable. A follow-up echocardiogram showed normal biventricular function, and other findings were unchanged. However, an event monitor tracing activated for palpitation showed NSVT (Fig. 1). After discussion among ACHD, high-risk obstetrics, and electrophysiology teams, low dose Atenolol was started. Unfortunately, patient developed symptomatic sinus bradycardia and hypotension on Atenolol, while patient continued to have episodes of NSVT. At this point, after thorough discussion with patient and her husband, decision was made to proceed with ICD placement. Implantation of dual chamber ICD in pre-pectoral space was performed under moderate sedation with three-dimension electroanatomic mapping without use of fluoroscopy at 16 weeks of gestation. Lower rate limit was set at 60 beat per minute for back up pacing, and the patient was started on Sotalol. With titration of Sotalol dose, her palpitation and fatigue improved. Within 3 days after ICD placement, no more NSVT was seen on telemetry monitor. The patient was discharged home on Sotalol. In follow up visits, she was doing well without episodes of NSVT or ICD shocks. This case illustrated unique challenge in management of NSVT during pregnancy in a patient with repaired TOF. An electrophysiology study with mapping and ablation, and surgical pulmonary valve replacement could have been offered if the case had not been complicated with pregnancy. Management of this case was also complicated with inability to tolerate an antiarrhythmic agent due to sinus bradycardia and hypotension. In the recent expert consensus statement, ICD was recommended as class I recommendation for these three groups: 1. Survivors of cardiac arrest due to ventricular arrhythmia, 2. Spontaneous sustained VT, and 3. Left ventricular EF equal or less than 35%. Although supporting data are less robust, this consensus statement also recommended ICD therapy as class IIa recommendation, specifically for adults with TOF with risk factors for SCD, including left ventricular systolic or diastolic dysfunction, NSVT, QRS duration equal or grater than 180 ms, extensive RV scarring, or inducible sustained VT. Catheter ablation of VT was listed as class I recommendation, as an adjunctive therapy for recurrent
1134
Correspondence
Fig. 1. An event monitor tracing of NSVT in the case. VT seen starting the third beat (a fusion beat) in the pre-trigger tracing, which continued through the half of post-trigger tracing before the rhythm spontaneously converted back to sinus rhythm.
monomorphic VT, a VT storm, or multiple appropriate shocks that are not manageable by ICD programming or antiarrhythmic therapy in ACHD patients [1]. Antiarrhythmic agents such as Amiodarone or Sotalol can be used to reduce VT burden and ICD shocks in this population. Amiodarone use for pregnant women is not favored due to its known adverse fetal risk. Also, long-term use of Amiodarone in young patients is generally discouraged due to adverse effects. Methods to minimize radiation dose during ICD placement were reported including the use of transesophageal echocardiogram or threedimension electroanatomic mapping [2]. Recent retrospective study of 20 pregnancies in 12 women (including 2 women with congenital heart disease) carrying ICD did not show clear association between ICD and adverse obstetric or neonatal outcomes [3]. In conclusion, an ICD implantation in conjunction with the use of antiarrhythmic agent is safe, and can be an option for management of
NSVT during pregnancy in selected ACHD patients. The number of similar cases is expected to increase in the near future with the growth of ACHD population.
References [1] P. Khairy, G.F. Van Hare, S. Balaji, C.I. Berul, F. Cecchin, M.I. Cohen, et al., PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease, Heart Rhythm 11 (2014) e102–e165. [2] S. Sideris, A. Kasiakogias, C. Aggeli, K. Manakos, G. Trantalis, K. Gatzoulis, et al., Implantation of a defibrillator in a pregnant woman with hypertrophic cardiomyopathy under echocardiographic guidance: a case report, Int. J. Cardiol. 179 (2015 Jan 20) 323–324. [3] S. Boulé, L. Ovart, C. Marquié, E. Botcherby, D. Klug, C. Kouakam, et al., Pregnancy in women with an implantable cardioverter–defibrillator: is it safe? Europace 16 (11) (2014 Nov) 1587–1594.
