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Adams-Stokes attack as the first symptom of acute rheumatic fever: report of an adolescent case and review of the literature.
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MedLine Citation:
PMID:  23110777     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Acquired complete heart block, in pediatric age is mainly the results of direct injury to conduction tissue during cardiac surgery or cardiac catheterisation. It can also be observed in different clinical settings as infectious diseases, neoplasia, and inflammatory diseases. It has a wide range of presentation and in some settings it can appear a dramatic event. Although a rare finding during acute rheumatic fever, with a transient course, it may need a specific and intensive treatment.
CASE PRESENTATION: We report the case of an Adams-Stokes attack in an adolescent with acute rheumatic carditis and complete atrio-ventricular block. The attack was the first symptom of carditis.We reviewed the literature and could find 25 cases of complete atrio-ventricular block due to rheumatic fever. Ten of the 25 patients experienced an Adams-Stokes attack. Nineteen of the 25 patients were certainly in the pediatric age group. Seven of the 19 pediatric cases experienced an Adams-Stokes attack. In 16/25 cases, the duration of the atrio-ventricular block was reported: it lasted from a few minutes to ten days. Pacemaker implantation was necessary in 7 cases.
CONCLUSION: Rheumatic fever must be kept in mind in the diagnostic work-up of patients with acquired complete atrio-ventricular block, particularly when it occurs in pediatric patients. The insertion of a temporary pacemaker should be considered when complete atrio-ventricular block determines Adams-Stokes attacks. Complete heart block during acute rheumatic fever is rare and is usually transient. Along with endocarditis, myocarditis and pericarditis, complete atrio-ventricular block has been recognized, rarely, during the course of acute rheumatic carditis.
Authors:
Nicola Carano; Ilaria Bo; Bertrand Tchana; Erica Vecchione; Silvia Fantoni; Aldo Agnetti
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2012-10-30
Journal Detail:
Title:  Italian journal of pediatrics     Volume:  38     ISSN:  1824-7288     ISO Abbreviation:  Ital J Pediatr     Publication Date:  2012  
Date Detail:
Created Date:  2012-12-13     Completed Date:  2013-03-12     Revised Date:  2013-07-11    
Medline Journal Info:
Nlm Unique ID:  101510759     Medline TA:  Ital J Pediatr     Country:  England    
Other Details:
Languages:  eng     Pagination:  61     Citation Subset:  IM    
Affiliation:
Department of Paediatrics-Paediatric Cardiology Unit, University of Parma, via Gramsci, 14 43126, Parma, Italy. nicola.carano@unipr.it
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MeSH Terms
Descriptor/Qualifier:
Acute Disease
Adams-Stokes Syndrome / diagnosis*,  therapy
Adolescent
Anti-Inflammatory Agents / therapeutic use
Diagnosis, Differential
Echocardiography
Electrocardiography
Humans
Male
Pacemaker, Artificial
Rheumatic Fever / diagnosis*,  therapy
Chemical
Reg. No./Substance:
0/Anti-Inflammatory Agents
Comments/Corrections

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

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Journal ID (nlm-ta): Ital J Pediatr
Journal ID (iso-abbrev): Ital J Pediatr
ISSN: 1824-7288
Publisher: BioMed Central
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Copyright ©2012 Carano et al.; licensee BioMed Central Ltd.
open-access:
Received Day: 8 Month: 8 Year: 2012
Accepted Day: 11 Month: 10 Year: 2012
collection publication date: Year: 2012
Electronic publication date: Day: 30 Month: 10 Year: 2012
Volume: 38First Page: 61 Last Page: 61
PubMed Id: 23110777
ID: 3520864
Publisher Id: 1824-7288-38-61
DOI: 10.1186/1824-7288-38-61

Adams-Stokes attack as the first symptom of acute rheumatic fever: report of an adolescent case and review of the literature
Nicola Carano1 Email: nicola.carano@unipr.it
Ilaria Bo2 Email: ilaria.bo@studenti.unipr.it
Bertrand Tchana1 Email: btchana@ao.pr.it
Erica Vecchione2 Email: erica.vecchione@studenti.unipr.it
Silvia Fantoni2 Email: silvia.fantoni@studenti.unipr.it
Aldo Agnetti1 Email: aldo.agnetti@unipr.it
1Department of Paediatrics – Paediatric Cardiology Unit, University of Parma, via Gramsci, 14 43126, Parma, Italy
2Post – Graduate School of Paediatrics - Department of Paediatrics, University of Parma, via Gramsci, 14 43126, Parma, Italy

Background

Acquired complete heart block, in paediatric age is mainly the results of direct injury to conduction tissue during cardaic surgery or cardiac catheterisation. It can also be observed in different clinical settings as infectious diseases, neoplasia, and inflammatory diseases. It has a wide range of presentation and in some settings it can appear a dramatic event. Although a rare finding during acute rheumatic fever, with a transient course, it may need a specific and intensive treatment. We report a case of complete atrio-ventricular (AV) block in whom an Adams-Stokes attack was the first symptom of acute rheumatic carditis. We also reviewed the literature on complete atrio-ventricular block in acute rheumatic fever.


Case report

A 14-year-old Italian boy, weight 50 kg, was admitted to the emergency room of our Paediatric Department for syncope which occurred at home after he got out of bed. He had complained of transient thoracic pain the day before. On admission, the patient appeared extremely pale. Severe bradycardia (30 beats/minute) was found, blood pressure was 115/65 mmHg, respiratory rate 24/minute and transcutaneous oxygen saturation was 98%. A grade 2/6 systolic murmur was audible at the apex. The remaining physical examination was unremarkable.

The ECG showed a complete AV block with narrow QRS and a ventricular rate of 30 beats/minute (Figure 1). A 5.52 second period of asystole was recorded as well (Figure 2). Transthoracic echocardiography revealed mild mitral regurgitation, no cardiac chamber enlargement (left ventricle end-diastolic diameter was 47 mm) and normal contractility (ejection fraction 67%, shortening fraction 37%); a temporary pacemaker was implanted via the right femoral vein. In the suspicion of an inflammatory etiology, intravenous methyl-prednisolone (20 mg b.i.d) was started.

History pointed out a febrile pharyngitis occurred about one month before. At that moment, a rapid antigen detection test was positive for β-haemolytic group A Streptococcus. Amoxicillin plus clavulanate, 1 gram b.i.d, had been prescribed for ten days.

Laboratory investigations revealed neutrophilic leukocytosis (WBC 17.750/mm3, N 82%), elevation of ESR and CRP (72 mm/hr and 136 mg/L, respectively), elevated streptococcal antibodies (ASO titre 3.220 U/mL, streptozyme test positive 1/5000, anti-streptokinase antibodies positive 1/2560). The throat culture for β-haemolytic group A Streptococcus was negative. Myocardial necrosis indices and Borrelia Burgdorferi antibodies also were negative.

After 24 hours, the patient recovered sinus rhythm (HR = 80 beats/minute) with first degree AV block (PR duration 250 milliseconds). A second echocardiography confirmed the mild mitral regurgitation, but also showed a slight thickening of the aortic leaflets with trivial aortic regurgitation. The temporary pacemaker was removed and the anti-inflammatory treatment was continued with oral prednisone 25 mg b.i.d. for two weeks. When the normalisation of the inflammatory indices was achieved, steroid treatment was progressively tapered and acetylsalicylic acid 750 mg q.i.d. was started and continued for four weeks. ECG performed on fourth day after admission showed a normal sinus rhythm with a normal PR interval duration. Forty days after the first examination, echocardiography showed complete resolution of both mitral and aortic regurgitation; the Holter ECG showed a sinus rhythm with normal AV conduction.

The final diagnosis was Adams-Stokes attack due to complete AV block in the course of acute rheumatic carditis.


Discussion

The most common cause of acquired complete AV block in the paediatric age group is direct injury to conduction tissue during cardiac surgery or cardiac catheterisation. In addition, complete atrio-ventricular block can be observed in infectious diseases as viral myocarditis, diphtheria, Lyme disease, in inflammatory illnesses such as acute rheumatic fever, metabolic diseases as Kearns-Sayre syndrome, drug toxicity (digoxin, beta-blockers, calcium-channel blockers), Chagas disease, tuberous sclerosis, intra-cardiac tumours, ischemia during coronary events or after mediastinal radiation.

The most common AV conduction abnormality found during acute rheumatic fever is first degree AV block, which was recognised in 72.5% of the Clarke’s series and in 72.3% of Zalzstein’s series (1, 2). Second degree AV block of Mobitz type I is much less frequent (2.6% in Clarke’s and 1.5% in Zalzstein’s series). Complete AV block was diagnosed in 0.6% of the Clarke’s and in 4.6% of Zalzstein’s series. Other types of rhythm abnormalities recognised during acute rheumatic fever include sinus node dysfunction, junctional rhythm and junctional tachycardia, ventricular tachycardia, torsade de pointes due to QT interval prolongation and complete left bundle branch block.

In Clarke’s series, only one of the three patients with complete AV block presented with an Adams-Stokes attack (1). All three patients with complete AV block of Zalzstein’s series were asymptomatic (2).

In our case, the Adams-Stokes attack was the first symptom of acute rheumatic fever. This occurred because of the high degree of complete AV block, with periods of asystole longer than five seconds.

We examined the literature in order to collect other cases of complete AV block due to rheumatic fever. We looked through PubMed’s MeSH vocabulary by inserting “rheumatic fever”, “atrio-ventricular block”, and “Adams-Stokes attack”.

We were able to find 19 full-text papers in which 25 cases of complete AV block due to rheumatic fever were reported [1-19]. Ten of the 25 patients experienced an Adams-Stokes attack [1,3,5,6,9,11-15] (Table 1).

Nineteen of the 25 patients with complete AV block were certainly in the paediatric age group [1,2,5,9,10,12,15,18,19]. Seven of the 19 experienced an Adams-Stokes attack [1,5,6,9,12,15].

In 16 out of 25 cases, the duration of the AV block was reported: it lasted from a few minutes to ten days [1,2,4-8,10,15]; in one case, an ECG three months later showed persistence of the complete block [7]. Pacemaker implantation was necessary in seven cases.


Conclusions

Complete heart block during acute rheumatic fever is rare. Despite it can appear as a dramatic event, it is usually transient, resolving in few days after initiating anti-inflammatory treatment. Specific treatment, such as insertion of a temporary pacemaker, should be considered only when complete AV block leads to an Adams-Stokes attack. In our patient, the Adams-Stokes attack was the first symptom of rheumatic fever. Rheumatic fever must be kept in mind in the diagnostic work-up of patients with acquired complete AV block, particularly when it occurs in paediatric patients.

Written informed consent has been obtained from the parents of the patient for publication of this case report and any accompanying images.


Abbreviations

ASO: Antibodies to streptolysin O; AV: Atrio – ventricular; CRP: C-reactive proteine; ECG: Electrocardigraphy; ESR: Erytrocyte sedimentation rate; WBC: White blood cells.


Competing interests

The authors declare that they have no competing interest.


Authors’ contributions

NC: Data analysis, data interpretation and writing. IB: Literature search and writing. BT: Literature search, figures. EV: Data collection. SF: Data collection. AA: Writing. All authors read and approved the final manuscript.


References
Clarke M,Keith JD,Atrioventricular conduction in acute rheumatic feverBr Heart JournalYear: 19723447247910.1136/hrt.34.5.472
Zalzstein E,Maor R,Zucker N,Katz A,Advanced atrioventricular conduction block in acute rheumatic feverCardiol YoungYear: 200313649149414982287
Arcuri F,Rossi S,Intermittent complete atrioventricular block with Morgagni-Adam-Stokes type syncopal attacks, in a rheumatic carditis patient. Cortisone therapyMinerva CardioangiolYear: 1959769069413794097
Barold SS,Sischy D,Punzi J,Kaplan EL,Chessin L,Advanced atrioventricular block in a 39-year-old man with acute rheumatic feverPacing Clin ElectrophysiolYear: 19982111 Pt 1202520289826853
Baracchi G,On 2 cases of complete atrioventricular block (acute and reversibile) caused by rheumatic myocarditisMinerva MedYear: 1963542952296014099153
Duran NE,Sonmez K,Biteker M,Ozkan M,A case of acute rheumatic fever presenting with syncope due to complete atrioventricular blockAndalou Kardiyol DergYear: 200996673
Guven H,Ozhan B,Bakiler AR,Salar K,Kozan M,Bilgin S,A case of Henoch-Schonlein purpura and rheumatic carditis with complete atrioventricular blockEur J PediatrYear: 200616539539710.1007/s00431-006-0094-316534586
Yoo GH,Complete atrioventricular block in an adolescent with rheumatic feverKorean Circ JYear: 200939312112310.4070/kcj.2009.39.3.12119949599
Lenox CC,Zuberbuhler JR,Park SC,Neches WH,Mathews RA,Zoltun R,Arrhythmias and Stokes-Adams attacks in acute rheumatic feverPediatricsYear: 1978614599603662486
Malik JA,Hassan C,Khan GQ,Transient complete heart block complicating acute rheumatic feverIndian Heart JYear: 2002541919211999098
Mohindra R,Pannu HS,Mohan B,Kumar N,Dhooria HS,Sehgal A,Avasthi G,Syncope in a middle aged male due to acute rheumatic feverIndian Heart JYear: 200456666866915751526
Poberezovskii KA,Lu LI,Partial atrioventricular block of the 3rd degree accompanied by Morgagni-Adam-Stokes syndrome in a child with a 1st rheumatic attackPediatricaYear: 19715078082
Rojas M,Papouin G,Hadrami J,Kamblock J,Lionet P,Victor J,Adam-Stokes syncope disclosing a crisis of rheumatic fever. Apropos of a caseAnn Cardiol Angiol (Paris)Year: 1997469592594
Tampieri E,Berdondini RM,Leonardi G,Mantovani R,Complete atrioventricular block caused by rheumatic myocarditis and complicated by a Morgagni-Adam-Stokes attack in an adult hyperthyroid patient. Discussion of a clinical case and review of the literatureMinerva CardioangiolYear: 19843264214266472641
Thomas DB,Complete heart block complicating rheumatic carditisAustr PaediatrYear: 19717108110
Filberbaum MB,Griffith GC,Solley RF,Electrocardiographic abnormalities in 6000 cases of rheumatic feverCalifornia and Western MedicineYear: 1946646340346
Montano A,Esposito M,Complete atrioventricular block and acute abdominal pain: initial symptoms in a case of rheumatic feverMinerva CardioangiolYear: 19903831011032348904
Shah CK,Gupta R,Persistent complete heart block following acute rheumatic fever in a 12 year old girlJ Assoc Physic IndiaYear: 199341389390
Stocker FP,Czoniczer G,Massell BF,Nadas AS,Transient complete AV block in two siblings during acute rheumatic carditis in childhoodPediatricsYear: 1970458508565444406

Figures

[Figure ID: F1]
Figure 1 

Electrocardiogram showing complete A-V block with a ventricular rate of 30 bpm .



[Figure ID: F2]
Figure 2 

Electrocardiogram showing paroxysmal AV block and a 5.52 second period of asystolia .



Tables
[TableWrap ID: T1] Table 1 

Cases of complete atrio-ventricular block in acute rheumatic fever collected from the literature


Author
Age (years), gender
Adams-Stokes attack
Degree of AV block
Pacing
Duration of complete AV block
(Ref number)          
Arcuri [3]
47, m
Yes
Intermittent complete AV block
no
7 days
Barold [4]
39, m
No
From I to III
no
5 days
Baracchi [5]
33, m
No
III
No
4 days
 
13, m
Yes
From II for 10 days to III
no
3 days
Clarke [1]
paediatric
Yes
From I to III
yes
8 days
 
paediatric
No
From I to III
no
unknown
 
paediatric
No
From I to III
no
unknown
Duran [6]
17, f
Yes
From III to II
yes
5 days
Filberbraum [16]
unknown
unknown
III
unknown
unknown
Guven [7]
9, m
No
From II to III
no
no improvement in rhythm at the 3rd month
Hee Yoo [8]
13, m
No
From III to II
no
3 days
Lenox [9]
13, m
Yes
III
yes
unknown
Malik [10]
16, m
No
From I to III
no
a few minutes
Mohindra [11]
38, m
Yes
III
yes
unknown
Montano [17]
9, f
No
III
no
10 days
Poberezovskii [12]
paediatric
Yes
III
unknown
unknown
Rojas [13]
15, unknown
Yes
III
yes
4 days
Shah [18]
12, f
unknown
III
unknown
unknown
Stocker [19]
paediatric
unknown
III
unknown
unknown
 
paediatric
unknown
III
unknown
unknown
Tampieri [14]
37, m
Yes
III
yes
2 days
Thomas [15]
12, m
Yes
III
yes
36 hours
Zalzestein [2]
3 patients range 9 to 11 (1 m, 2 f)
No
III
No
from 30 to 48 hours
 
 
No
III
No
 
    No III No  


Article Categories:
  • Case Report

Keywords: Adams Stokes, Complete heart block, Rheumatic fever.

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