Document Detail

Amiodarone and catheter ablation as cardiac resynchronization therapy for children with dilated cardiomyopathy and wolff-Parkinson-white syndrome.
Jump to Full Text
MedLine Citation:
PMID:  23407697     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Preexcitation by accessory pathways (APs) is known to cause dyssynchrony of the ventricle, related to ventricular dysfunction. Correction of ventricular dyssynchrony can improve heart failure in cases of dilated cardiomyopathy (DCMP) with preexcitation. Here, we report the first case of a child with DCMP and Wolff-Parkinson-White (WPW) syndrome treated with amiodarone and radiofrequency catheter ablation (RFCA) in Korea. A 7-year-old boy, who suffered from DCMP and WPW syndrome, showed improved left ventricular function and clinical functional class after treatment with amiodarone to eliminate preexcitation. QRS duration and left ventricular ejection fraction (LVEF) were inversely correlated with amiodarone dosage. After confirming the reduction of preexcitation effects in DCMP, successful RFCA of the right anterior AP resulted in LVEF improvement, along with the disappearance of preexcitation. Our findings suggest that ventricular dyssynchrony, caused by preexcitation in DCMP with WPW syndrome, can worsen ventricular function and amiodarone, as well as RFCA, which should be considered as a treatment option, even in young children.
Sung Hoon Kim; Soo In Jeong; June Huh; I-Seok Kang; Heung Jae Lee
Related Documents :
618627 - Effect of dopamine on hemodynamics and myocardial metabolism in shock following acute m...
3232957 - Individual free fatty acids and lactate uptake in the human heart during severe sepsis.
23323117 - Left ventricular dysfunction and dilated cardiomyopathy in infants and children with wo...
9389857 - Effects of magnesium sulphate on the cardiovascular system, coronary circulation and my...
12224727 - Nontransplant cardiac surgery as a bridge to heart transplantation in pediatric dilated...
3986057 - Relation between mitral valve closure and early systolic function of the left ventricle.
Publication Detail:
Type:  Journal Article     Date:  2013-01-31
Journal Detail:
Title:  Korean circulation journal     Volume:  43     ISSN:  1738-5520     ISO Abbreviation:  Korean Circ J     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-02-14     Completed Date:  2013-02-15     Revised Date:  2013-04-12    
Medline Journal Info:
Nlm Unique ID:  101247141     Medline TA:  Korean Circ J     Country:  Korea (South)    
Other Details:
Languages:  eng     Pagination:  57-61     Citation Subset:  -    
Department of Pediatrics, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Korea.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Full Text
Journal Information
Journal ID (nlm-ta): Korean Circ J
Journal ID (iso-abbrev): Korean Circ J
Journal ID (publisher-id): KCJ
ISSN: 1738-5520
ISSN: 1738-5555
Publisher: The Korean Society of Cardiology
Article Information
Download PDF
Copyright © 2013 The Korean Society of Cardiology
Received Day: 26 Month: 4 Year: 2012
Revision Received Day: 04 Month: 6 Year: 2012
Accepted Day: 13 Month: 6 Year: 2012
Print publication date: Month: 1 Year: 2013
Electronic publication date: Day: 31 Month: 1 Year: 2013
Volume: 43 Issue: 1
First Page: 57 Last Page: 61
PubMed Id: 23407697
ID: 3569569
DOI: 10.4070/kcj.2013.43.1.57

Amiodarone and Catheter Ablation as Cardiac Resynchronization Therapy for Children with Dilated Cardiomyopathy and Wolff-Parkinson-White Syndrome
Sung Hoon Kim, MD1
Soo In Jeong, MD2
June Huh, MD3
I-Seok Kang, MD3
Heung Jae Lee, MD3
1Department of Pediatrics, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Korea.
2Department of Pediatrics, Kangdong Sacred Heart Hospital, Hallym University School of Medicine, Seoul, Korea.
3Department of Pediatrics, Cardiac and Vascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Correspondence: Correspondence: June Huh, MD, Department of Pediatrics, Cardiac and Vascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul 135-710, Korea. Tel: 82-2-3410-3539, Fax: 82-2-3410-0043,


Wolff-Parkinson-White (WPW) syndrome is a preexcitation of the heart, caused by an accessory pathway (AP) that bypasses the atrioventricular node, and is associated with supraventricular tachycardia.1) Incessant or recurrent AP-mediated supraventricular tachycardia can cause dilated cardiomyopathy (DCMP).2), 3) However, overt ventricular preexcitation in children can cause ventricular dysfunction, even in the absence of recurrent and incessant tachyarrhythmia. 4), 5) Preexcitation by an AP is associated with decreased left ventricular function, depending on the location of the AP.5) Patients with a right-sided AP, with preexcitation, have more severe cardiac dysfunction.5-8) It is well known that in most patients, cardiac function improves and reverse remodeling initiates after preexcitation stops.6-8) The etiology of cardiac dysfunction should be evaluated for the appropriate treatment of DCMP with WPW syndrome. It is important to differentiate tachycardia-induced cardiomyopathy from idiopathic cardiomyopathy with coexisting WPW syndrome.

Here, we report the first case of a child with DCMP and WPW syndrome, treated with amiodarone and radiofrequency catheter ablation (RFCA) in Korea. The change in preexcitation in response to amiodarone can help evaluate its effect on the dyssynchrony of DCMP, and determine if RFCA should be used for children with DCMP and WPW syndrome.


A previously healthy 3-month-old boy was presented with an abnormal electrocardiography (ECG) at a routine check-up for a hydrocele operation. His body weight was 5.5 kg, and his cardiac examination was notable for a normal S1 and a grade II/VI regurgitant systolic murmur heard at the apex. At admission, overt ventricular preexcitation was noted on 12-lead ECG. The vector of the AP suggested a right-sided pathway. A chest X-ray showed severe cardiomegaly with increased pulmonary vascular markings. Echocardiography revealed paradoxical septal motion, severe mitral regurgitation and global hypokinesia with a 22.9% of left ventricle ejection fraction (LVEF, normal range: 55-65% at his age) and dilated left ventricle, with an end-diastolic dimension (LVEDD) of 54.6 mm (normal range: 22-28 mm at his age). Digoxin, captopril, diuretics, and carvedilol were given, but cardiac function remained poor.

At five of his age, supraventricular tachycardia developed. Based on poor LVEF and tachycardia, a trial of amiodarone was administered. After amiodarone treatment, a shortening of the QRS duration (130 ms vs. 117 ms) and delta wave disappearance were noted on ECG, with improved general condition.

There was significant improvement of LVEF (27% vs. 39%, normal range: 55-65% at his age) and reduction of LVEDD (58 mm vs. 52 mm, normal range 30-38 mm). However, reduction of amiodarone dosage brought the increase of the QRS duration (117 ms vs. 126 ms), with deterioration of the LVEF (Figs. 1A, B and 2). After confirming the effect of preexcitation on left ventricular function, RFCA of the right anterior AP was successfully performed, resulting in LVEF improvement (19% vs. 38%), reduction of LVEDD (58.3 mm vs. 54.6 mm), shortening of QRS duration (126 ms vs. 80 ms) and disappearance of preexcitation (Fig. 1C). Echocardiography also showed disappearance of left ventricle dyssynchrony (Fig. 3). The patient is doing well with gradual improvement of left ventricular function.


WPW syndrome is caused by an anomalous atrioventricular AP, and manifests as preexcitation on the surface ECG. Dyssynchronous ventricular depolarization occurs in WPW patients. Abnormalities of interventricular septal motion by preexcitation in WPW syndrome can result in global left ventricular dysfunction, mimicking idiopathic DCMP. Therefore, differentiating between tachycardia-induced cardiomyopathy and idiopathic cardiomyopathy with coexisting WPW syndrome is important. Although the pathogenic mechanism, underlying the development of DCMP in patients with WPW syndrome, is not clearly understood, suggestions are that 1) children with asymptomatic ventricular preexcitation are at risk of developing DCMP during follow-up, even in the absence of persistent and recurrent tachycardias; 2) after the disappearance of preexcitation, significant recovery of left ventricular function and reverse remodeling occurs, suggesting a causal relationship between ventricular preexcitationmediated dyssynchrony and the development of DCMP; and 3) left ventricular dyssynchrony may be more pronounced in the septal and paraseptal AP, compared with right free-wall and left-sided APs.5), 7), 9), 10)

Interestingly, a significant improvement in left ventricular function occurred after successful catheter ablation of the AP, in our reported case. Preexcitation-mediated left ventricular dyssynchrony disturbs the myocardial regional workload and wall stress, which may result in wall motion abnormalities, myocardial perfusion defects, changes in coronary blood flow, adverse remodeling with progressive left ventricular dilation and asymmetrical changes in left ventricular wall thickness.11), 12) In contrast to the right free wall pathways, a septal AP may cause preexcitation of a substantial part of the interventricular septum, inducing dyskinetic septal motion and significant left ventricular dyssynchrony.10) After a loss of preexcitation, RFCA, in particular, can result in mechanical resynchronization, reverse remodeling and improvement of left ventricular function.8), 10) After RFCA, shortening of the anteroseptal to posterior wall delay was noted on echocardiography and synchronous contraction was demonstrated with beneficial reverse remodeling of the left ventricle in our case.

In this case, we first attempted pharmacologic suppression of preexcitation by amiodarone because of reported effect of amiodarone on ventricular dysfunction with preexcitation, as well as relatively high risk of RFCA complications in infants.13), 14) Amiodarone showed the improvement of LV function with suppression of preexcitation. After confirming the detrimental effects of preexcitation on DCMP, RFCA of the right anterior AP was successfully performed, resulting in significant improvement in left ventricular dysfunction. Amiodarone can be considered as alternative short term treatment option for infants with DCMP and WPW syndrome, if too small infants for high risk of complications of RFCA. A change in preexcitation (QRS duration on ECG) in response to amiodarone helps to evaluate its effect on dyssynchrony of DCMP and to decide RFCA for children with DCMP and WPW syndrome. We prescribed amiodarone relatively late, at 5 years of his age, because of a relatively stable clinical state. However, we now consider early trial of amiodarone, in order to evaluate the effect of preexcitation on left ventricular function in DCMP with WPW syndrome. Regardless of the amount of amiodarone, which is effective for preexcitation suppression, RFCA should be considered in DCMP children with WPW syndrome at any age because those with overt ventricular preexcitation are at risk for developing DCMP in the absence of recurrent and incessant tachyarrhythmia. Regardless of the effectiveness of amiodarone or RFCA, preexcitation suppression can initiate reverse remodeling of a dyssynchronous left ventricle in DCMP with WPW syndrome.


The authors have no financial conflicts of interest.

1. Campbell RM,Strieper MJ,Frias PA,Collins KK,Van Hare GF,Dubin AM. Survey of current practice of pediatric electrophysiologists for asymptomatic Wolff-Parkinson-White syndromePediatricsYear: 2003111e245e24712612279
2. Schulze OC,Kammeraad J,Ramanna H,Sreeram N. Catheter ablation for tachyarrhythmia-induced cardiomyopathy in infantsInt J CardiolYear: 2000749910010912444
3. Umana E,Solares CA,Alpert MA. Tachycardia-induced cardiomyopathyAm J MedYear: 2003114515512543289
4. Niksch AL,Dubin AM. Risk stratification in the asymptomatic child with Wolff-Parkinson-White syndromeCurr Opin CardiolYear: 20062120520716601458
5. Udink ten Cate FE,Kruessell MA,Wagner K,et al. Dilated cardiomyopathy in children with ventricular preexcitation: the location of the accessory pathway is predictive of this associationJ ElectrocardiolYear: 20104314615419879594
6. Cadrin-Tourigny J,Fournier A,Andelfinger G,Khairy P. Severe left ventricular dysfunction in infants with ventricular preexcitationHeart RhythmYear: 200851320132218774110
7. Emmel M,Balaji S,Sreeram N. Ventricular preexcitation associated with dilated cardiomyopathy: a causal relationship?Cardiol YoungYear: 20041459459915679994
8. Iwasaku T,Hirooka K,Taniguchi T,et al. Successful catheter ablation to accessory atrioventricular pathway as cardiac resynchronization therapy in a patient with dilated cardiomyopathyEuropaceYear: 20091112112319028709
9. Fazio G,Mongiovi' M,Sutera L,Novo G,Novo S,Pipitone S. Segmental dyskinesia in Wolff-Parkinson-White syndrome: a possible cause of dilatative cardiomyopathyInt J CardiolYear: 2008123e31e3417292982
10. Yamanaka S,Shirayama T,Inoue K,et al. Improved cardiac function after catheter ablation in a patient with type B Wolff-Parkinson-White syndrome with an old myocardial infarctionJpn Circ JYear: 1998628608629856605
11. Prinzen FW,Cheriex EC,Delhaas T,et al. Asymmetric thickness of the left ventricular wall resulting from asynchronous electric activation: a study in dogs with ventricular pacing and in patients with left bundle branch blockAm Heart JYear: 1995130104510537484735
12. Tantengco MV,Thomas RL,Karpawich PP. Left ventricular dysfunction after long-term right ventricular apical pacing in the youngJ Am Coll CardiolYear: 2001372093210011419893
13. Hirasawa Y,Nakagomi A,Kobayashi Y,Katoh T,Mizuno K. Short-term amiodarone treatment attenuates the production of monocyte cytokines and chemokines by C-reactive protein and improves cardiac function in patients with idiopathic dilated cardiomyopathy and ventricular tachycardiaCirc JYear: 20097363964619225199
14. Deal BJ,Keane JF,Gillette PC,Garson A Jr. Wolff-Parkinson-White syndrome and supraventricular tachycardia during infancy: management and follow-upJ Am Coll CardiolYear: 198551301353964800

Article Categories:
  • Case Report

Keywords: Dilated cardiomyopathy, Wolff-Parkinson-White syndrome, Amiodarone, Radiofrequency catheter ablation, Cardiac resynchronization therapy.

Previous Document:  Normothermic cardiopulmonary bypass increases cerebral tissue oxygenation during combined valve surg...
Next Document:  Exploring a direct injection method for microfluidic generation of polymer microgels.