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Gepubliceerd in: Netherlands Heart Journal 2/2016

Open Access 04-01-2016 | Rhythm Puzzle Answer

A regularly irregular rhythm—what is the diagnosis?

Auteurs: A.-D. Margulescu, O.A. Enescu, D. Vinereanu

Gepubliceerd in: Netherlands Heart Journal | Uitgave 2/2016

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Answer

The ECG on admission shows ectopic atrial tachycardia with multilevel block: variable Mobitz type II exit block to the atrium and Wenckebach phenomena in the atrioventricular node (see Fig. 1 for detailed explanation).
This complex arrhythmia was highly unusual, and suggested a cardiac infiltrative process. Transthoracic echocardiogram showed normal cardiac function, but the interatrial septum and both atrial roofs were abnormally thick (not shown). Chest computed tomography scan also showed mediastinal adenopathy (not shown). This combination of findings narrowed the differential diagnosis to atrial infiltration due to sarcoidosis or lymphoma/tumours. Biopsies collected during minimal thoracotomy established the diagnosis of sarcoidosis (not shown).
In cardiac sarcoidosis, conduction disturbances are related to granulomatous infiltration of the conduction system [1]. Their evolution is unpredictable and device therapy (either permanent pacing or cardiac defibrillator implantation) is recommended [2]. The patient initially declined device implantation. Prednisolone was started but had to be discontinued 7 months later due to development of Cushing’s syndrome. Eighteen months after diagnosis the patient was readmitted with complete heart block and consented to dual-chamber pacemaker implantation. One year afterwards her ventricular function remained normal; interrogation of the device disclosed no ventricular arrhythmias at follow-up.
This case underlines that when conduction disturbances are seen in younger patients, a search for cardiac structural abnormalities is warranted and sarcoidosis should be suspected [3].

Funding sources

None.

Conflict of interest

The authors have no conflict of interest relevant to this article to disclose.
Open Access This article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited.
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Netherlands Heart Journal

Het Netherlands Heart Journal wordt uitgegeven in samenwerking met de Nederlandse Vereniging voor Cardiologie en de Nederlandse Hartstichting. Het tijdschrift is Engelstalig en wordt gratis beschikbaa ...

Literatuur
1.
go back to reference Lynch JP III, Hwang J, Bradfield J, Fishbein M, Shivkumar K, Tung R. Cardiac involvement in sarcoidosis: evolving concepts in diagnosis and treatment. Semin Respir Crit Care Med. 2014;35:372–90. PubMedCentralCrossRefPubMed Lynch JP III, Hwang J, Bradfield J, Fishbein M, Shivkumar K, Tung R. Cardiac involvement in sarcoidosis: evolving concepts in diagnosis and treatment. Semin Respir Crit Care Med. 2014;35:372–90. PubMedCentralCrossRefPubMed
2.
go back to reference Epstein AE, DiMarco JP, Ellenbogen KA, et al. 2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities. Circulation. 2013;127:e283–352. CrossRefPubMed Epstein AE, DiMarco JP, Ellenbogen KA, et al. 2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities. Circulation. 2013;127:e283–352. CrossRefPubMed
3.
go back to reference Kandolin R, Lehtonen J, Kupari M. Cardiac sarcoidosis and giant cell myocarditis as causes of atrioventricular block in young and middle-aged adults. Circ Arrhythm Electrophysiol. 2011;4:303–9. CrossRefPubMed Kandolin R, Lehtonen J, Kupari M. Cardiac sarcoidosis and giant cell myocarditis as causes of atrioventricular block in young and middle-aged adults. Circ Arrhythm Electrophysiol. 2011;4:303–9. CrossRefPubMed
Metagegevens
Titel
A regularly irregular rhythm—what is the diagnosis?
Auteurs
A.-D. Margulescu
O.A. Enescu
D. Vinereanu
Publicatiedatum
04-01-2016
Uitgeverij
Bohn Stafleu van Loghum
Gepubliceerd in
Netherlands Heart Journal / Uitgave 2/2016
Print ISSN: 1568-5888
Elektronisch ISSN: 1876-6250
DOI
https://doi.org/10.1007/s12471-015-0789-z