The precordial R′ wave: A novel discriminator between cardiac sarcoidosis and arrhythmogenic right ventricular cardiomyopathy in patients presenting with ventricular tachycardia

Hoogendoorn, Jarieke C; Venlet, Jeroen; Out, Yannick N.J.; Man, Sumche; Kumar, Saurabh; Šramko, Marek; Dechering, Dirk G.; Nakajima, Ikutaro; Siontis, Konstantinos C.; Watanabe, Masaya; Nakamura, Yoshinori; Tedrow, Usha B.; Bogun, Frank; Eckardt, Lars; Peichl, Petr; Stevenson, William G.; Zeppenfeld, Katja

doi:10.1016/j.hrthm.2021.04.032

Cited by 16 publications

(15 citation statements)

References 25 publications

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“…However, very recently, an ECG algorithm including a PR interval of ≥220 ms, the presence of R′ wave and the surface area of the maximum R′ wave in leads V 1-3 ≥ 1.65 mm 2 was developed to distinguish CS with RV and LV involvement from ACM (Figure 5A). 14 It was either nega- tive or positive for CS in our patient in its first step, as the PR interval of the ECG shown in Figure 1B was 210 ms; however, the PR interval of the ECG shown in Figure 1C was 230 ms. R′ wave was present in V 1-3 and the surface area of maximum R′ wave in V 1-3 was ≥1.65 mm 2 (Figure 5B) in lead V 2 in our patient, which established the diagnosis of CS. Thus, if we had known this ECG algorithm in 2015, we could have suggested the diagnosis of CS at the initial presentation, which we could not verify in vivo by the application of the most sophisticated imaging modalities.…”

Section: Distinguishing Acm From Cs In Our Patientmentioning

confidence: 66%

“…But, because the patient died right after the gallium scintigraphy, only histological examination ( Figure ) of the heart during autopsy verified the presence of CS in our patient, illustrating the great difficulty to diagnose CS in some patients. However, very recently, an ECG algorithm including a PR interval of ≥220 ms, the presence of R′ wave and the surface area of the maximum R′ wave in leads V 1–3 ≥ 1.65 mm 2 was developed to distinguish CS with RV and LV involvement from ACM ( Figure ) 14 . It was either negative or positive for CS in our patient in its first step, as the PR interval of the ECG shown in Figure was 210 ms; however, the PR interval of the ECG shown in Figure was 230 ms. R′ wave was present in V 1–3 and the surface area of maximum R′ wave in V 1–3 was ≥1.65 mm 2 ( Figure ) in lead V 2 in our patient, which established the diagnosis of CS.…”

Section: Discussionmentioning

confidence: 99%

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Cardiac sarcoidosis completely mimicking biventricular arrhythmogenic cardiomyopathy

et al. 2022

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Cardiac sarcoidosis (CS) is a chameleon of cardiology, and it can mimic different cardiac diseases; among them is arrhythmogenic cardiomyopathy (ACM). We admitted a 70-year-old female patient with heart failure symptoms in 2015, who fulfilled all major ECG and non-invasive imaging criteria of biventricular ACM. She was well with the recommended medications for 3 years, showing only isolated cardiac involvement, but in 2018, cervical and mediastinal lymphadenopathy appeared and cervical lymph node core biopsy histology, bronchoalveolar lavage flow cytometry strongly suggested extracardiac sarcoidosis. Therefore, our suspicion was that sarcoidosis is responsible for the cardiac involvement, which was not confirmed by PET-CT and gallium scintigraphy examinations. At the end of 2018, she died in septicaemia with multiorgan failure, and only autopsy verified her CS. A new ECG algorithm published in 2021 for the differential diagnosis of CS and biventricular ACM, when applied on her ECGs recorded in 2015, suggested the diagnosis of CS.

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Section: Distinguishing Acm From Cs In Our Patientmentioning

confidence: 66%

Section: Discussionmentioning

confidence: 99%

Cardiac sarcoidosis completely mimicking biventricular arrhythmogenic cardiomyopathy

et al. 2022

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“… 15 Of interest, prior mapping studies performed in patients with arrhythmogenic right ventricular cardiomyopathy or right ventricular cardiac sarcoidosis have shown that RV conduction delay can significantly alter the terminal QRS, specifically in lead V1. 21 , 26 , 27 …”

Section: Discussionmentioning

confidence: 99%

“…For comprehensive analysis of the ECG, Leiden ECG Analysis and Decomposition Software (LEADS) was employed. 20 , 21 Raw electrocardiographic data, consisting of 8-channel recordings of 10 s ECGs in comma-separated value files, were inserted into this MATLAB program (The Mathworks Inc., Natick, MA, version: 2007b). With LEADS, the QRS complexes and T waves are automatically detected in the spatial velocity signal and the program proposes selected beats for averaging based on minimization of baseline drifts and artefacts, which can afterwards be manually adjusted based on visual inspection.…”

Section: Methodsmentioning

confidence: 99%

The R″ wave in V1 and the negative terminal QRS vector in aVF combine to a novel 12-lead ECG algorithm to identify slow conducting anatomical isthmus 3 in patients with tetralogy of Fallot

et al. 2023

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Aims Patients with repaired tetralogy of Fallot (rTOF) have an increased risk of ventricular tachycardia (VT), with slow conducting anatomical isthmus (SCAI) 3 as dominant VT substrate. In patients with right bundle branch block (RBBB), SCAI 3 leads to local activation delay with a shift of terminal RV activation towards the lateral RV outflow tract which may be detected by terminal QRS vector changes on sinus rhythm electrocardiogram (ECG). Methods and results Consecutive rTOF patients aged ≥16 years with RBBB who underwent electroanatomical mapping at our institution between 2017–2022 and 2010–2016 comprised the derivation and validation cohort, respectively. Forty-six patients were included in the derivation cohort (aged 40±15 years, QRS duration 165±23 ms). Among patients with SCAI 3 (n = 31, 67%), 17 (55%) had an R″ in V1, 18 (58%) had a negative terminal QRS portion (NTP) ≥80 ms in aVF, and 12 (39%) had both ECG characteristics, compared to only 1 (7%), 1 (7%), and 0 patient without SCAI, respectively. Combining R″ in V1 and/or NTP ≥80 ms in aVF into a diagnostic algorithm resulted in a sensitivity of 74% and specificity of 87% in detecting SCAI 3. The inter-observer agreement for the diagnostic algorithm was 0.875. In the validation cohort [n = 33, 18 (55%) with SCAI 3], the diagnostic algorithm had a sensitivity of 83% and specificity of 80% for identifying SCAI 3. Conclusion A sinus rhythm ECG-based algorithm including R″ in V1 and/or NTP ≥80 ms in aVF can identify rTOF patients with a SCAI 3 and may contribute to non-invasive risk stratification for VT.

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“…In another cohort of patients with LBBB-pattern VT with either ARVC or CS who were referred for radiofrequency catheter ablation, 5/8 (63%) of patients with CS fulfilled the diagnostic ARVC criteria ( 23 ). Recently a novel electrocardiogram (ECG)-based algorithm to differentiate CS from ARVC in patients presenting with right ventricular (RV) VT was developed, by assessing differences in terminal activation in leads V1–V3 ( 24 ). It is hypothesised that the RBBB pattern seen in ARVC results from fibro-fatty infiltration predominantly in the RV causing diffuse conduction delay and reduced voltages, compared to the focal and patchy granulomas in CS causing local block and preserved voltages reflected by an R’ wave with a higher voltage.…”

Section: Electrophysiological Manifestations Of Cardiac Sarcoidosismentioning

confidence: 99%

Management of the arrhythmic manifestations of cardiac sarcoidosis

Cherrett

Lee

Bart

et al. 2023

Front. Cardiovasc. Med.

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Cardiac sarcoidosis (CS) is characterised by a high burden of arrhythmic manifestations and cardiac electrophysiologists play an important role in both the diagnosis and management of this challenging condition. CS is characterised by the formation of noncaseating granulomas within the myocardium, which can subsequently lead to fibrosis. Clinical presentations of CS are varied and depend on the location and extent of granulomas. Patients may present with atrioventricular block, ventricular arrhythmias, sudden cardiac death or heart failure. CS is being increasing diagnosed through use of advanced cardiac imaging, however endomyocardial biopsy is often still required to confirm the diagnosis. Due to the low sensitivity of fluoroscopy-guided right ventricular biopsies, three-dimensional electro-anatomical mapping and electrogram-guided biopsies are being investigated as a means to improve diagnostic yield. Cardiac implantable electronic devices are often required in the management of CS, either for pacing or for primary or secondary prevention of ventricular arrhythmias. Catheter ablation for ventricular arrythmias may also be required, although this is often associated with high recurrence rates due to the challenging nature of the arrhythmogenic substrate. This review will explore the underlying mechanisms of the arrhythmic manifestations of CS, provide an overview of current clinical practice guidelines, and examine the important role that cardiac electrophysiologists play in managing patients with CS.

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The precordial R′ wave: A novel discriminator between cardiac sarcoidosis and arrhythmogenic right ventricular cardiomyopathy in patients presenting with ventricular tachycardia

Cited by 16 publications

References 25 publications

Cardiac sarcoidosis completely mimicking biventricular arrhythmogenic cardiomyopathy

Cardiac sarcoidosis completely mimicking biventricular arrhythmogenic cardiomyopathy

The R″ wave in V1 and the negative terminal QRS vector in aVF combine to a novel 12-lead ECG algorithm to identify slow conducting anatomical isthmus 3 in patients with tetralogy of Fallot

Management of the arrhythmic manifestations of cardiac sarcoidosis

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