Tags

Type your tag names separated by a space and hit enter

Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear.
Circ Arrhythm Electrophysiol. 2014 Apr; 7(2):230-6.CA

Abstract

BACKGROUND

Cardiac sarcoidosis (CS) may show overlap in the clinical presentation with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). We sought to investigate patients with CS who were misdiagnosed with ARVD/C and identify clinical features to distinguish these 2 groups.

METHODS AND RESULTS

Among patients enrolled in the Johns Hopkins ARVD/C registry, 15 patients with definite 2010 diagnostic criteria for ARVD/C were subsequently diagnosed with CS. Forty-two pathogenic desmosomal mutation carriers with definite ARVD/C based on the 2010 diagnostic criteria served as a control group. Patients with CS were older at the age of symptom onset, more likely to have comorbidities, and develop heart failure symptoms over time (P<0.05). Electrocardiographically, PR interval prolongation and high-grade atrioventricular block were exclusively associated with CS (P<0.05). HV interval prolongation and increased number of ventricular tachycardias induced were also associated with CS (P<0.05). Radiographically, significant left ventricular dysfunction, myocardial delayed enhancement of the septum, and mediastinal lymphadenopathy were more often see in those with CS (P<0.05).

CONCLUSIONS

The 2010 diagnostic criteria for ARVD/C have limited discrimination in distinguishing between ARVD/C and CS. Despite the overlay in clinical presentation, older age of symptom onset, presence of cardiovascular comorbidities, nonfamilial pattern of disease, PR interval prolongation, high-grade atrioventricular block, significant left ventricular dysfunction, myocardial delayed enhancement of the septum, and mediastinal lymphadenopathy should raise the suspicion for CS.

Authors+Show Affiliations

Section of Cardiac Electrophysiology, Cardiovascular Institute, Warren Alpert Medical School of Brown University, Providence, RI.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Multicenter Study
Observational Study
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

24585727

Citation

Philips, Binu, et al. "Arrhythmogenic Right Ventricular Dysplasia/cardiomyopathy and Cardiac Sarcoidosis: Distinguishing Features when the Diagnosis Is Unclear." Circulation. Arrhythmia and Electrophysiology, vol. 7, no. 2, 2014, pp. 230-6.
Philips B, Madhavan S, James CA, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear. Circ Arrhythm Electrophysiol. 2014;7(2):230-6.
Philips, B., Madhavan, S., James, C. A., te Riele, A. S., Murray, B., Tichnell, C., Bhonsale, A., Nazarian, S., Judge, D. P., Calkins, H., Tandri, H., & Cheng, A. (2014). Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear. Circulation. Arrhythmia and Electrophysiology, 7(2), 230-6. https://doi.org/10.1161/CIRCEP.113.000932
Philips B, et al. Arrhythmogenic Right Ventricular Dysplasia/cardiomyopathy and Cardiac Sarcoidosis: Distinguishing Features when the Diagnosis Is Unclear. Circ Arrhythm Electrophysiol. 2014;7(2):230-6. PubMed PMID: 24585727.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Arrhythmogenic right ventricular dysplasia/cardiomyopathy and cardiac sarcoidosis: distinguishing features when the diagnosis is unclear. AU - Philips,Binu, AU - Madhavan,Srinivasa, AU - James,Cynthia A, AU - te Riele,Anneline S J M, AU - Murray,Brittney, AU - Tichnell,Crystal, AU - Bhonsale,Aditya, AU - Nazarian,Saman, AU - Judge,Daniel P, AU - Calkins,Hugh, AU - Tandri,Harikrishna, AU - Cheng,Alan, Y1 - 2014/03/01/ PY - 2014/3/4/entrez PY - 2014/3/4/pubmed PY - 2014/6/4/medline KW - arrhythmogenic right ventricular dysplasia KW - diagnosis KW - sarcoidosis SP - 230 EP - 6 JF - Circulation. Arrhythmia and electrophysiology JO - Circ Arrhythm Electrophysiol VL - 7 IS - 2 N2 - BACKGROUND: Cardiac sarcoidosis (CS) may show overlap in the clinical presentation with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). We sought to investigate patients with CS who were misdiagnosed with ARVD/C and identify clinical features to distinguish these 2 groups. METHODS AND RESULTS: Among patients enrolled in the Johns Hopkins ARVD/C registry, 15 patients with definite 2010 diagnostic criteria for ARVD/C were subsequently diagnosed with CS. Forty-two pathogenic desmosomal mutation carriers with definite ARVD/C based on the 2010 diagnostic criteria served as a control group. Patients with CS were older at the age of symptom onset, more likely to have comorbidities, and develop heart failure symptoms over time (P<0.05). Electrocardiographically, PR interval prolongation and high-grade atrioventricular block were exclusively associated with CS (P<0.05). HV interval prolongation and increased number of ventricular tachycardias induced were also associated with CS (P<0.05). Radiographically, significant left ventricular dysfunction, myocardial delayed enhancement of the septum, and mediastinal lymphadenopathy were more often see in those with CS (P<0.05). CONCLUSIONS: The 2010 diagnostic criteria for ARVD/C have limited discrimination in distinguishing between ARVD/C and CS. Despite the overlay in clinical presentation, older age of symptom onset, presence of cardiovascular comorbidities, nonfamilial pattern of disease, PR interval prolongation, high-grade atrioventricular block, significant left ventricular dysfunction, myocardial delayed enhancement of the septum, and mediastinal lymphadenopathy should raise the suspicion for CS. SN - 1941-3084 UR - https://www.unboundmedicine.com/medline/citation/24585727/Arrhythmogenic_right_ventricular_dysplasia/cardiomyopathy_and_cardiac_sarcoidosis:_distinguishing_features_when_the_diagnosis_is_unclear_ L2 - https://www.ahajournals.org/doi/10.1161/CIRCEP.113.000932?url_ver=Z39.88-2003&amp;rfr_id=ori:rid:crossref.org&amp;rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -