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Atypical clinical presentation of arrhythmogenic biventricular cardiomyopathy.

Rangel I, Vasconcelos M, Campelo M, Frutuoso C, Madureira AJ, Maciel MJ - Arq. Bras. Cardiol. (2014)

View Article: PubMed Central - PubMed

Affiliation: Centro Hospitalar de São João, Porto, Portugal.

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease affectingthe heart muscle... We present a caseof ARVC with left ventricle (LV) involvement, with no typical symptoms and no relevantmedical history... In addition, ongoing maintenance therapy with azathioprine for Crohn's disease anda nonfunctional adrenal adenoma were observed... Family history was unremarkable, andphysical examination results were normal... Electrocardiography revealed a normal sinus rhythm, left axis deviation, and T-waveinversion in precordial leads from V2 to V6... Further, an epsilon wave in leads V1-V3(Figure 1) led to the suspicion of ARVC.Monitoring using a 24-h Holter electrocardiogram documented only occasional ventricularextrasystoles with left bundle branch block morphology and no significant cardiacarrhythmia... These findings suggested a diagnosis of ARVC with LVinvolvement... In this case involving a patient with atypical symptoms, no clinical arrhythmia, and anegative family history, three major criteria led to the definite diagnosis of ARVC:inverted T waves in the precordial leads in the absence of complete right bundle branchblock; epsilon wave in the right precordial leads; and regional RV dyskinesia withindexed end-diastolic volume ≥ 100 ml/m... LV involvement in ARVC has been recognized in several studies and can occur in > 75%patients with disease progression.CMR is an ideal technique to aid in the diagnosis of this condition... Particularly, in asymptomatic patients,the differential diagnosis of ARVC should be considered.

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Resting 12-lead ECG showing T-wave inversion from V2 to V6 (major diagnosticcriteria) and epsilon waves in V1–V3 (major diagnostic criteria).
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f01: Resting 12-lead ECG showing T-wave inversion from V2 to V6 (major diagnosticcriteria) and epsilon waves in V1–V3 (major diagnostic criteria).

Mentions: Electrocardiography revealed a normal sinus rhythm, left axis deviation, and T-waveinversion in precordial leads from V2 to V6. Further, an epsilon wave in leads V1-V3(Figure 1) led to the suspicion of ARVC.Monitoring using a 24-h Holter electrocardiogram documented only occasional ventricularextrasystoles with left bundle branch block morphology and no significant cardiacarrhythmia. Further evaluation included cardiac magnetic resonance (CMR) imaging, whichrevealed right ventricular (RV) enlargement with an indexed end-diastolic volume of 110ml/m2 and mild dysfunction (ejection fraction = 44%). No signs of fattytissue infiltration were observed, but regional RV dyskinesia in the free wall andoutflow tract was identified (Figure 2A). LV wasmildly enlarged (90 ml/m2), and systolic function was at the lower limit ofnormal. Flow analysis revealed no shunts in the aorta and pulmonary artery.Subepicardial delayed enhancement was observed the lateral basal and middle segments ofLV as well as a small focal area of the RV free wall (Figure 2B). These findings suggested a diagnosis of ARVC with LVinvolvement.


Atypical clinical presentation of arrhythmogenic biventricular cardiomyopathy.

Rangel I, Vasconcelos M, Campelo M, Frutuoso C, Madureira AJ, Maciel MJ - Arq. Bras. Cardiol. (2014)

Resting 12-lead ECG showing T-wave inversion from V2 to V6 (major diagnosticcriteria) and epsilon waves in V1–V3 (major diagnostic criteria).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3987383&req=5

f01: Resting 12-lead ECG showing T-wave inversion from V2 to V6 (major diagnosticcriteria) and epsilon waves in V1–V3 (major diagnostic criteria).
Mentions: Electrocardiography revealed a normal sinus rhythm, left axis deviation, and T-waveinversion in precordial leads from V2 to V6. Further, an epsilon wave in leads V1-V3(Figure 1) led to the suspicion of ARVC.Monitoring using a 24-h Holter electrocardiogram documented only occasional ventricularextrasystoles with left bundle branch block morphology and no significant cardiacarrhythmia. Further evaluation included cardiac magnetic resonance (CMR) imaging, whichrevealed right ventricular (RV) enlargement with an indexed end-diastolic volume of 110ml/m2 and mild dysfunction (ejection fraction = 44%). No signs of fattytissue infiltration were observed, but regional RV dyskinesia in the free wall andoutflow tract was identified (Figure 2A). LV wasmildly enlarged (90 ml/m2), and systolic function was at the lower limit ofnormal. Flow analysis revealed no shunts in the aorta and pulmonary artery.Subepicardial delayed enhancement was observed the lateral basal and middle segments ofLV as well as a small focal area of the RV free wall (Figure 2B). These findings suggested a diagnosis of ARVC with LVinvolvement.

View Article: PubMed Central - PubMed

Affiliation: Centro Hospitalar de São João, Porto, Portugal.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease affectingthe heart muscle... We present a caseof ARVC with left ventricle (LV) involvement, with no typical symptoms and no relevantmedical history... In addition, ongoing maintenance therapy with azathioprine for Crohn's disease anda nonfunctional adrenal adenoma were observed... Family history was unremarkable, andphysical examination results were normal... Electrocardiography revealed a normal sinus rhythm, left axis deviation, and T-waveinversion in precordial leads from V2 to V6... Further, an epsilon wave in leads V1-V3(Figure 1) led to the suspicion of ARVC.Monitoring using a 24-h Holter electrocardiogram documented only occasional ventricularextrasystoles with left bundle branch block morphology and no significant cardiacarrhythmia... These findings suggested a diagnosis of ARVC with LVinvolvement... In this case involving a patient with atypical symptoms, no clinical arrhythmia, and anegative family history, three major criteria led to the definite diagnosis of ARVC:inverted T waves in the precordial leads in the absence of complete right bundle branchblock; epsilon wave in the right precordial leads; and regional RV dyskinesia withindexed end-diastolic volume ≥ 100 ml/m... LV involvement in ARVC has been recognized in several studies and can occur in > 75%patients with disease progression.CMR is an ideal technique to aid in the diagnosis of this condition... Particularly, in asymptomatic patients,the differential diagnosis of ARVC should be considered.

Show MeSH
Related in: MedlinePlus