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Complete atrioventricular block persisting after regression of infectious myocarditis
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Journal of Electrocardiology 41 (2008) 665 – 667 www.jecgonline.com
Complete atrioventricular block persisting after regression of infectious myocarditis Philippe Maury, MD,⁎ Talia Chilon, MD, Nicolas Dumonteil, MD, Anthony Fontan, MD Federation of Cardiology, University Hospital Rangueil, Toulouse, France Received 1 May 2008; accepted 3 June 2008
Abstract
Atrioventricular block is usually transient during the course of infectious myocarditis. We report the case of a patient presenting with complete infra-hisian atrioventricular block occurring in the setting of infectious myocarditis and in whom a pacemaker should be finally implanted. Borderline serologies for picornavirus were present 6 weeks later. Complete atrioventricular block persisted during the two years of follow-up despite otherwise complete regression of the myocarditis. We then discuss the main features of conduction disturbances complicating infectious myocarditis, including a list as complete as possible of all the causal agents possibly involved. © 2008 Elsevier Inc. All rights reserved.
Keywords:
Myocarditis; Atrioventricular block; Picornavirus; Coxsackie
Observation A 47-year-old woman, without medical history and free of any medical therapy, presented with flu-like syndrome, with fever, sweating, vomiting, abdominal, and chest pain. The patient was urgently referred to cardiac intensive care unit for the documentation of an altered left ventricular systolic function associated with electrocardiographic (ECG) features evoking an anterior acute myocardial infarction with QRS showing right bundle-branch block and left axis, rapidly complicated by complete atrioventricular (AV) block. First echocardiography showed a left ventricular ejection fraction (LVEF) of 35% with diffuse hypokinesia but without cardiac chamber enlargement, pericardial effusion, or valvular abnormality. Coronary angiography was unremarkable. She recalled of a fleeting erythematous eruption on the right thigh 3 months before, but without any remembrance of having been bitten. An electrophysiologic investigation performed 9 days later due to the persistence of complete AV block revealed an infrahisian block with slow ventricular escape rhythm (Fig. 1). Biological investigations showed elevated C-reactive protein, creatine phosphokinase and troponin Ic, polynucleosis, and
hepatic cytolysis. Serology for Lyme disease was negative, but a probabilistic antibiotic therapy with ceftriaxone was prescribed for 3 weeks. Serologies for rheumatic diseases as well as complete bacterial and viral serologies and cultures or detection of viral antigen were all negative. No myocardial biopsy was performed. Because of the persisting complete AV block after 2 weeks, a permanent dual chamber pacemaker was implanted. Because of spontaneous improvement of the LEVF (45% at the hospital discharge) concomitant with normalization of biological parameters, it was concluded to be a diagnosis of myocarditis. Available control serologies performed 6 weeks later remained negative, except for the picornavirus, which were borderline (total antibody 40, normal ≤20). Three months after the acute phase, left ventricular systolic function had completely normalized; thus, myocarditis was considered cured despite the persistence of complete AV block as revealed during temporary inhibition of the pacemaker (Fig. 1). The chronicity of conduction disturbances was later confirmed by the persistence of complete AV block at 2 years follow-up. Discussion
⁎ Corresponding author. Fédération de Cardiologie,Unité de Rythmologie et de Stimulation Cardiaque, Hôpital Universitaire Rangueil, 31059 Toulouse Cedex 09, France. Tel.: +33 5 61 32 20 94; fax: +33 5 61 32 22 46. E-mail address: [email protected] 0022-0736/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jelectrocard.2008.07.001
Conduction disturbances may variably complicate the evolution of infectious myocarditis and may be observed with numerous causal agents (Table 1). Children seem to be particularly vulnerable to these complications,1-3 and a
666
P. Maury et al. / Journal of Electrocardiology 41 (2008) 665–667
Fig. 1. Upper tracing, 9 days after admission: II, III and V1 surface ECG leads showing persistent complete AV block with slow ventricular escape rhythm at a rate of 35 beats per minute (bpm). Intracardiac recording showed that AV block was located below the His bundle. Escape rhythm was not preceded by His potential, was unstable, and only moderately accelerated by atropine infusion to a rate of 40 bpm. Sinus rhythm was fast (110 bpm) with preserved nodal conduction (AH interval, 90 milliseconds). Lower tracing, 3 months later, during pacemaker control: underlying persistent complete AV block is revealed by the temporary inhibition of the pacemaker (II and III surface ECG leads, 25 mm/s). A indicates atrial electrogram; H, His bundle potential; V, ventricular electrogram.
Table 1 Reported infectious agents responsible for myocarditis with conduction disease, Trypanosoma cruzi (Chagas disease) and Borrelia burgdorferi (Lyme disease) excluded Bacteria
Virus
Parasites
Diphtheria Cholera Leptospirosis Mycoplasma pneumoniae Rickettsia Streptococcus Meningococcus Listeriosis Staphylococcus Tuberculosis Syphilis Shigella Salmonella
Epstein-Barr virus Mumps Cytomegalovirus Rubella
Candidosis Aspergillosis Trichinosis Hydatidosis
Poliomyelitis Parainfluenza Influenza Varicella Herpes measles Arbovirosis Hepatitis HIV Coxsackie B Adenovirus Respiratory syncytial virus Hantaan virus Enterovirus
Toxoplasmosis Paludism Leishmaniosis Bilharziosis Trypanosoma gambiense Cysticercosis Opisthorchiasis Paragonimiasis
The underlined data are agents for which the implantation of a permanent pacemaker has been reported. (complete references available on request).
myocarditis should always be suspected in them in case of the occurrence of acquired AV block, even if biological proof for the causal agent is lacking.3 Conduction disturbances can also sometimes be observed without myocardial systolic dysfunction,4 and a particular affinity of the causal agent for the specialized conduction tissues has been proposed.5 The association with various supraventricular or ventricular arrhythmias is furthermore possible. The few autopsies and biopsies performed in the acute phase have found injury of the conduction system, with mitochondrial inclusions, lymphocytes and mononuclear cells infiltrates, areas of inflammation, and necrosis. The injury related to inflammation and edema could be only transient and may explain some spontaneous recoveries observed in man.4 Scar fibrosis is present on biopsies performed in the delayed phase. The few electrophysiologic investigations performed found supra-hisian, intra-hisian, or infra-hisian block. Distal lesions are however very common because of the frequency of intraventricular conduction blocks.2,4 Delayed investigations are normal when the conduction disturbances have recovered. Atrioventricular block may need transient pacing,3,4 but regresses generally in a few days1,3,4 (two thirds have recovered at 1 week in children6); therefore, myocarditis is a
P. Maury et al. / Journal of Electrocardiology 41 (2008) 665–667
rather infrequent cause of implantation of permanent pacemaker despite the frequency of conduction disturbances during the acute phase.1,6 However, cases of persistent AV block needing the implantation of permanent pacemaker have been published2,3) (20%-30% of the cases in children,3 up to 75 % in other series5). Various causal agents can be involved (Table 1). Relationship with distal lesions is possible, but cases with supra-hisian block have also been reported.7 In case of late recovery of AV conduction, one can question about the utility of an explantation, especially because modern pacemaker with sophisticated extended memories and hysteresis functions allow detection of paroxysmal bradycardia. Picornavirus include enterovirus (poliovirus, coxsackie A and B, enterovirus, and echovirus) and rhinovirus. Atrioventricular block is a classic complication of B-coxsackie myocarditis2-4 (B1, B2, B3, B4, and B5), sometimes needing permanent pacing.2,4 The relationship between picornavirus infection and myocarditis could not be formally proved in our case because of the only borderline serology at 6 weeks—but peak of antibodies could have been missed—and because of the absence of histologic proof. Furthermore, borderline values of markers for picorna viral infection could also be found in periods of convalescence of broad spectrum of viral infections. Further characterization of the causal agent could not be realized, but this is also the fact for many other observations. Furthermore, initial clinical presentation was compatible with coxsackie B infection, whereas Lyme disease was unlikely because of the negative
667
serology and the persistence of AV block despite antibiotic therapy. Finally, although other usual causes such as myopathy, systemic disease, or inflammatory processes could not be excluded at the time of the acute phase, the absence of such evolutive pathologic processes at 2 years follow-up make them unlikely. Likewise, although degenerative conduction disturbances could have also happen despite the relatively young age of the patient, their sudden onset at the time of an acute febrile process makes the last hypothesis very improbable. References 1. Wang JN, Tsai YC, Lee WL, Lin CS, Wu JM. Complete atrioventricular block following myocarditis in children. Pediatr Cardiol 2002;23:518. 2. Mahoney LT, Marvin Jr WJ, Atkins DL, Clark EB, Lauer RM. Pacemaker management for acute onset of heart block in childhood. J Pediatr 1985;107:207. 3. Batmaz G, Villain E, Bonnet D, Iserin L, Fraisse A, Kachaner J. Therapy and prognosis of infectious complete atrioventricular block in children. Arch Mal Coeur Vaiss 2000;93:553. 4. Gould L, Diaz R, Gomprecht R. Complete heart block in myocarditis. Recovery with a pacemaker. Chest 1972;62:230. 5. Khongphatthanayothin A, Chotivitayatarakorn P, Benjacholamas V, Muangmingsuk S, Lertsupcharoen P, Thisyakorn C. Complete heart block in children at King Chulalongkorn Memorial Hospital. J Med Assoc Thai 2001;84(Suppl 1):S111. 6. Batra AS, Epstein D, Silka MJ. The clinical course of acquired complete heart block in children with acute myocarditis. Pediatr Cardiol 2003;24: 495. 7. Friedli B, Renevey F, Rouge JC. Complete heart block in a young child presumably due to Mycoplasma pneumoniae myocarditis. Acta Paediatr Scand 1977;66:385.
Journal of Electrocardiology 41 (2008) 665 – 667 www.jecgonline.com
Complete atrioventricular block persisting after regression of infectious myocarditis Philippe Maury, MD,⁎ Talia Chilon, MD, Nicolas Dumonteil, MD, Anthony Fontan, MD Federation of Cardiology, University Hospital Rangueil, Toulouse, France Received 1 May 2008; accepted 3 June 2008
Abstract
Atrioventricular block is usually transient during the course of infectious myocarditis. We report the case of a patient presenting with complete infra-hisian atrioventricular block occurring in the setting of infectious myocarditis and in whom a pacemaker should be finally implanted. Borderline serologies for picornavirus were present 6 weeks later. Complete atrioventricular block persisted during the two years of follow-up despite otherwise complete regression of the myocarditis. We then discuss the main features of conduction disturbances complicating infectious myocarditis, including a list as complete as possible of all the causal agents possibly involved. © 2008 Elsevier Inc. All rights reserved.
Keywords:
Myocarditis; Atrioventricular block; Picornavirus; Coxsackie
Observation A 47-year-old woman, without medical history and free of any medical therapy, presented with flu-like syndrome, with fever, sweating, vomiting, abdominal, and chest pain. The patient was urgently referred to cardiac intensive care unit for the documentation of an altered left ventricular systolic function associated with electrocardiographic (ECG) features evoking an anterior acute myocardial infarction with QRS showing right bundle-branch block and left axis, rapidly complicated by complete atrioventricular (AV) block. First echocardiography showed a left ventricular ejection fraction (LVEF) of 35% with diffuse hypokinesia but without cardiac chamber enlargement, pericardial effusion, or valvular abnormality. Coronary angiography was unremarkable. She recalled of a fleeting erythematous eruption on the right thigh 3 months before, but without any remembrance of having been bitten. An electrophysiologic investigation performed 9 days later due to the persistence of complete AV block revealed an infrahisian block with slow ventricular escape rhythm (Fig. 1). Biological investigations showed elevated C-reactive protein, creatine phosphokinase and troponin Ic, polynucleosis, and
hepatic cytolysis. Serology for Lyme disease was negative, but a probabilistic antibiotic therapy with ceftriaxone was prescribed for 3 weeks. Serologies for rheumatic diseases as well as complete bacterial and viral serologies and cultures or detection of viral antigen were all negative. No myocardial biopsy was performed. Because of the persisting complete AV block after 2 weeks, a permanent dual chamber pacemaker was implanted. Because of spontaneous improvement of the LEVF (45% at the hospital discharge) concomitant with normalization of biological parameters, it was concluded to be a diagnosis of myocarditis. Available control serologies performed 6 weeks later remained negative, except for the picornavirus, which were borderline (total antibody 40, normal ≤20). Three months after the acute phase, left ventricular systolic function had completely normalized; thus, myocarditis was considered cured despite the persistence of complete AV block as revealed during temporary inhibition of the pacemaker (Fig. 1). The chronicity of conduction disturbances was later confirmed by the persistence of complete AV block at 2 years follow-up. Discussion
⁎ Corresponding author. Fédération de Cardiologie,Unité de Rythmologie et de Stimulation Cardiaque, Hôpital Universitaire Rangueil, 31059 Toulouse Cedex 09, France. Tel.: +33 5 61 32 20 94; fax: +33 5 61 32 22 46. E-mail address: [email protected] 0022-0736/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jelectrocard.2008.07.001
Conduction disturbances may variably complicate the evolution of infectious myocarditis and may be observed with numerous causal agents (Table 1). Children seem to be particularly vulnerable to these complications,1-3 and a
666
P. Maury et al. / Journal of Electrocardiology 41 (2008) 665–667
Fig. 1. Upper tracing, 9 days after admission: II, III and V1 surface ECG leads showing persistent complete AV block with slow ventricular escape rhythm at a rate of 35 beats per minute (bpm). Intracardiac recording showed that AV block was located below the His bundle. Escape rhythm was not preceded by His potential, was unstable, and only moderately accelerated by atropine infusion to a rate of 40 bpm. Sinus rhythm was fast (110 bpm) with preserved nodal conduction (AH interval, 90 milliseconds). Lower tracing, 3 months later, during pacemaker control: underlying persistent complete AV block is revealed by the temporary inhibition of the pacemaker (II and III surface ECG leads, 25 mm/s). A indicates atrial electrogram; H, His bundle potential; V, ventricular electrogram.
Table 1 Reported infectious agents responsible for myocarditis with conduction disease, Trypanosoma cruzi (Chagas disease) and Borrelia burgdorferi (Lyme disease) excluded Bacteria
Virus
Parasites
Diphtheria Cholera Leptospirosis Mycoplasma pneumoniae Rickettsia Streptococcus Meningococcus Listeriosis Staphylococcus Tuberculosis Syphilis Shigella Salmonella
Epstein-Barr virus Mumps Cytomegalovirus Rubella
Candidosis Aspergillosis Trichinosis Hydatidosis
Poliomyelitis Parainfluenza Influenza Varicella Herpes measles Arbovirosis Hepatitis HIV Coxsackie B Adenovirus Respiratory syncytial virus Hantaan virus Enterovirus
Toxoplasmosis Paludism Leishmaniosis Bilharziosis Trypanosoma gambiense Cysticercosis Opisthorchiasis Paragonimiasis
The underlined data are agents for which the implantation of a permanent pacemaker has been reported. (complete references available on request).
myocarditis should always be suspected in them in case of the occurrence of acquired AV block, even if biological proof for the causal agent is lacking.3 Conduction disturbances can also sometimes be observed without myocardial systolic dysfunction,4 and a particular affinity of the causal agent for the specialized conduction tissues has been proposed.5 The association with various supraventricular or ventricular arrhythmias is furthermore possible. The few autopsies and biopsies performed in the acute phase have found injury of the conduction system, with mitochondrial inclusions, lymphocytes and mononuclear cells infiltrates, areas of inflammation, and necrosis. The injury related to inflammation and edema could be only transient and may explain some spontaneous recoveries observed in man.4 Scar fibrosis is present on biopsies performed in the delayed phase. The few electrophysiologic investigations performed found supra-hisian, intra-hisian, or infra-hisian block. Distal lesions are however very common because of the frequency of intraventricular conduction blocks.2,4 Delayed investigations are normal when the conduction disturbances have recovered. Atrioventricular block may need transient pacing,3,4 but regresses generally in a few days1,3,4 (two thirds have recovered at 1 week in children6); therefore, myocarditis is a
P. Maury et al. / Journal of Electrocardiology 41 (2008) 665–667
rather infrequent cause of implantation of permanent pacemaker despite the frequency of conduction disturbances during the acute phase.1,6 However, cases of persistent AV block needing the implantation of permanent pacemaker have been published2,3) (20%-30% of the cases in children,3 up to 75 % in other series5). Various causal agents can be involved (Table 1). Relationship with distal lesions is possible, but cases with supra-hisian block have also been reported.7 In case of late recovery of AV conduction, one can question about the utility of an explantation, especially because modern pacemaker with sophisticated extended memories and hysteresis functions allow detection of paroxysmal bradycardia. Picornavirus include enterovirus (poliovirus, coxsackie A and B, enterovirus, and echovirus) and rhinovirus. Atrioventricular block is a classic complication of B-coxsackie myocarditis2-4 (B1, B2, B3, B4, and B5), sometimes needing permanent pacing.2,4 The relationship between picornavirus infection and myocarditis could not be formally proved in our case because of the only borderline serology at 6 weeks—but peak of antibodies could have been missed—and because of the absence of histologic proof. Furthermore, borderline values of markers for picorna viral infection could also be found in periods of convalescence of broad spectrum of viral infections. Further characterization of the causal agent could not be realized, but this is also the fact for many other observations. Furthermore, initial clinical presentation was compatible with coxsackie B infection, whereas Lyme disease was unlikely because of the negative
667
serology and the persistence of AV block despite antibiotic therapy. Finally, although other usual causes such as myopathy, systemic disease, or inflammatory processes could not be excluded at the time of the acute phase, the absence of such evolutive pathologic processes at 2 years follow-up make them unlikely. Likewise, although degenerative conduction disturbances could have also happen despite the relatively young age of the patient, their sudden onset at the time of an acute febrile process makes the last hypothesis very improbable. References 1. Wang JN, Tsai YC, Lee WL, Lin CS, Wu JM. Complete atrioventricular block following myocarditis in children. Pediatr Cardiol 2002;23:518. 2. Mahoney LT, Marvin Jr WJ, Atkins DL, Clark EB, Lauer RM. Pacemaker management for acute onset of heart block in childhood. J Pediatr 1985;107:207. 3. Batmaz G, Villain E, Bonnet D, Iserin L, Fraisse A, Kachaner J. Therapy and prognosis of infectious complete atrioventricular block in children. Arch Mal Coeur Vaiss 2000;93:553. 4. Gould L, Diaz R, Gomprecht R. Complete heart block in myocarditis. Recovery with a pacemaker. Chest 1972;62:230. 5. Khongphatthanayothin A, Chotivitayatarakorn P, Benjacholamas V, Muangmingsuk S, Lertsupcharoen P, Thisyakorn C. Complete heart block in children at King Chulalongkorn Memorial Hospital. J Med Assoc Thai 2001;84(Suppl 1):S111. 6. Batra AS, Epstein D, Silka MJ. The clinical course of acquired complete heart block in children with acute myocarditis. Pediatr Cardiol 2003;24: 495. 7. Friedli B, Renevey F, Rouge JC. Complete heart block in a young child presumably due to Mycoplasma pneumoniae myocarditis. Acta Paediatr Scand 1977;66:385.