Symptomatic arrhythmia in the form of multiple premature ventricular extrasystoles as the first symptom of polymyositis

Abstract

Polymyositis (PM) is an autoimmune disease characterized by the involvement of multiple internal organs, including the cardiovascular system. The involvement of heart is observed in up to 75% of patients with PM. Conduction and rhythm disorders are one of the most common cardiological abnormalities in these patients. The presented clinical case is the patient where ventricular arrhythmia (in the form of multiple premature ventricular extrasystoles) was the first symptom of polymyositis.

1. CASE REPORT

A forty‐year‐old patient reported to the cardiologist due to palpitations occurring for 2 months, general weakness, and difficulty in standing up from a sitting position.

During physical examination besides psychomotor retardation, no significant deviation was observed.

In ECG, 75/min sinus rhythm and premature ventricular extrasystoles (VES) with left bundle branch block (LBBB) morphology were detected.

In echocardiography examination, size and contraction of left heart ventricle were found normal with ejection fraction of 65%, mild aortic valve regurgitation, and prolapse of the anterior leaflet of the mitral valve with its mild regurgitation.

Predominant sinus rhythm with a mean frequency of 85/min (range 54–120/min) was detected with twenty‐four‐hour ECG Holter monitoring. 10,240 premature, monomorphic ventricular extrasystoles, 424 pairs, one episode of nonsustained, monomorphic ventricular tachycardia (10 impulses) and overall 970 episodes of ventricular bigeminy and trigeminy were detected (Figure 1).

Figure 1.

Twenty‐four‐hour ECG Holter monitoring—conducted on the initial stage of the diagnostics

Moreover, severe microcytic anemia with iron deficiency, as well as enzymatic symptoms of liver damage, an elevated level of muscle markers (Table 1), gastritis with positive urease test, the presence of antinuclear antibodies (ANA), antimitochondrial antibodies (AMA), and also myopathy in EMG were observed.

Table 1.

Results of patient laboratory tests on the start of diagnostics and after the introduction of therapy with ursodeoxycholic acid, prednisone, and oral iron supplementation

Parameter	Result of patient laboratory tests on the start of diagnostics	Control results after initiation of the therapy	Standard range—reference values (Gajewski & Szczeklik, 2014)
Leukocytes (K/μg)	6.8	9.7	4.0–10.0
Erythrocytes (M/μl)	4.45	4.24	3.7–4.7
Hemoglobin (g/dl)	7.9	11.4	12–16
Mean blood volume (fl)	65	84.4	80–90
Mean hemoglobin content in the blood cell (pg)	19	26.9	27–31
Thrombocytes (K/μg)	478	364	150–450
Blood iron (μg/dl)	27		37–145
Aspartate aminotransferase (U/L)	176	27	10–31
Alanine aminotransferase (U/L)	132	28	10–31
Alkaline phosphatase (U/L)	227	36	35–105
Lactate dehydrogenase (U/L)	765	176	135–214
Gamma‐glutamyltransferase (U/L)	566		9–35
Myoglobin (ng/ml)	662	211	0–70
Creatine kinase (U/L)	6,288	217	30–135
Creatine kinase MB isoenzyme (U/L)	208		7–25
Troponin‐T (ng/ml)	0.252		0–0.014
C‐reactive protein (mg/L)	3.6	<1	0–5
Erythrocyte sedimentation rate (mm/hr)	26	3	0–12

Based on the overall clinical picture and met diagnostic criteria, the patient was diagnosed with polymyositis (increased activity of creatine kinase in serum, electromyographic characteristics of primary muscle damage, weakness of the pelvic girdle muscles) (Bohan & Peter, 1975) and primary biliary cirrhosis (positive AMA and increased level of alkaline phosphatase).

The patient was treated with bisoprolol (1 × 5 mg/day); prednisone (1 × 20 mg/day), ursodeoxycholic acid (15 mg/kg bw/day in three divided doses), oral iron supplementation and Helicobacter pylori eradication treatment. As the result increases in the physical performance, gradual improvement in blood morphological parameters, and improvement in the activity of muscle and hepatic enzymes were observed (Table 1).

However, the patient still experienced heart palpitation occurring predominantly during the night despite the β‐blocker therapy and the improvement of red blood cell parameters.

Control 24 hr ECG Holter monitoring showed 9,536 individual ventricular extrasystoles (which constitute 7% of all beats). Furthermore, 390 pairs, 1,084 episodes of bigeminy and 639 episodes of ventricular trigeminy were detected. Interpolated ventricular beats were dominant (Figure 2).

Figure 2.

Control twenty‐four‐hour ECG Holter monitoring conducted during patient β‐blocker therapy

Due to the symptoms and lack of effectiveness of β‐blocker therapy, the ablation of the arrhythmia substrate was conducted. RF ablation of arrhythmogenic focus was performed within the right ventricular outflow track obtaining improvement in the patient general sensation and reduction of the number of VES. Twenty‐four‐hour ECG Holter monitoring performed 3 months after the intervention gave 1,300 VES/day, and 6 months after ablation only 27/day.

The patient is currently under the supervision of the Cardiology, Rheumatology, and Hepatology Outpatient Clinic. Chronic treatment includes prednisone, dose 15 mg/day, and ursodeoxycholic acid, dose 1,000 mg/day. Currently, the disease is asymptomatic.

2. DISCUSSION

The connective tissue diseases constitute a diagnostic challenge due to the complex pathophysiology and diverse clinical picture associated with the involvement of multiple systems, including cardiovascular system (Peregud‐Pogorzelska et al., 2014).

Polymyositis, accepted as the connective tissue disease, is an acquired, chronic, idiopathic myositis. The disease can occur at every age, however two morbidity peaks can be distinguished: 10–15 year of life (children form) and 35–65 year of life. Women suffer from this disease 2 times more frequently than men. In the clinical picture, symptoms for the involvement of muscles are dominant: in general symmetrical weakness of pelvic girdle muscles, pectoral girdle muscles, and neck and back muscles, (Bohan, Peter, Bowman, & Pearson, 1977; Gajewski, & Szczeklik, 2014).

Inflammatory lesions can also involve interstitial lung tissue, heart, blood vessels. The precise incidence of the lesions in the cardiovascular system of PM patients remains unknown. Conducted studies suggest that lesions in the cardiovascular system relate to the 6%–75% of patients (wide range is the result of dissimilarities between studied groups and utilization of different diagnostic methods; Lundberg, 2005). However, in 10%–25% of PM patients, the involvement is symptomatic (Lundberg, 2005).

In resting electrocardiography, the deviations from the norm are present in 32%–72% of patients. These deviations primarily include the repolarization abnormalities (unspecific changes in ST‐T fragment) and conduction abnormalities, including most common left anterior fascicular block (13%) and right bundle branch block (9.1%) (Eisen, Arnson, Dovrish, Hadary, & Amital, 2009; Lundberg, 2005; Stern, Godbold, Chess, & Kagen, 1984) . Atrioventricular conduction abnormalities and sinoatrial node disease occur less frequent than intraventricular blocks (Alyan, Ozdemir, Geyik, & Demirkan, 2003; Haupt, & Hutchins, 1982; Eisen et al., 2009).

In the literature, little information is found regarding rhythm disturbances in PM (Lazzerini et al., 2006). Data concerning the frequency of symptoms reported by patients and individual types of arrhythmia are heterogeneous and are based mainly on the case studies and the studies on small groups of patients.

Devezy et al. found that in a group of 34 PM patients, VES was diagnosed in two patients based on the resting electrocardiogram (5.9%) (Deveza et al., 2016). In the study of Taylor et al. premature ventricular extrasystoles were observed in 69% of patients and 31% of patients reported palpitations as a symptom (Taylor et al., 1993).

Management of rhythm disorders in PM patients is not significantly different from the treatment of other patients. In most of the cases, the basis of the therapy is the antiarrhythmic drugs. Selection of the drug depends on the type of arrhythmia and concomitant diseases (e.g., heart failure, coronary heart disease). β‐blockers are most commonly used drugs. In the individual cases, the RF ablation should be performed. Current guidelines of European Society of Cardiology suggest the ablation of ventricular extrasystoles within the right ventricular outflow tract in symptomatic patients and/or when antiarrhythmic therapy, e.g., with a β‐adrenolytic drug, is ineffective (Priori et al., 2015). In such a case, the ablation offers over 95% effectiveness and low risk of complications (Priori et al., 2015).

In each patient with diagnosed polymyositis, cardiology diagnostic is advisable, because the involvement of cardiovascular system has a negative impact on the course of primary disease. Diseases of the cardiovascular system are responsible for almost 20% deaths in these patients and are the third cause of death in PM patients after systemic infections and neoplasms (Lazzerini et al., 2006; Peregud‐Pogorzelska et al., 2014). Standard, resting electrocardiography examination, as well as twenty‐four‐hour ECG Holter monitoring, should be the integral element of the PM patient diagnostics. Additionally, one should bear in mind that rhythm or conduction disorders may be the first symptom of systemic disorders and in case of justified suspicion the diagnostics should be extended, also concerning rheumatic diseases.

Peregud‐Pogorzelska M, Zielska M, Kaźmierczak J. Symptomatic arrhythmia in the form of multiple premature ventricular extrasystoles as the first symptom of polymyositis. Ann Noninvasive Electrocardiol. 2018;23:e12532 10.1111/anec.12532