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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 56-58

Complete Heart Block in Rheumatoid Arthritis


Department of Medicine, Krishna Institute of Medical Sciences University, Karad, Maharashtra, India

Date of Web Publication17-Jun-2014

Correspondence Address:
Amit K Sakaria
B/B/93, 8th Floor, Mahavir Park, Behind Big Bazzar, Pune-Satara Road, Pune - 411 037, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-449x.134588

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  Abstract 

The extra-articular manifestations of Rheumatoid Arthritis (RA) have drawn as much interest as the disease itself and thus leading to it being labeled as a chronic inflammatory joint disease with a multisystem involvement. Of the various known extra-articular manifestations, one of the rare complications is the conduction abnormalities of the heart that is seen in the active disease. Here, we are reporting a case of a female patient with RA, who to begin with had conduction defects secondary to RA which later progressed to a complete heart block, as its sequelae.

Keywords: Complete heart block in rheumatoid arthritis, conduction disturbances in rheumatoid arthritis, extra-articular manifestation of rheumatoid arthritis


How to cite this article:
Desai RR, Sakaria AK, Goel UK, Lakhotia AN, Shah KB. Complete Heart Block in Rheumatoid Arthritis. Heart India 2014;2:56-8

How to cite this URL:
Desai RR, Sakaria AK, Goel UK, Lakhotia AN, Shah KB. Complete Heart Block in Rheumatoid Arthritis. Heart India [serial online] 2014 [cited 2019 Sep 23];2:56-8. Available from: http://www.heartindia.net/text.asp?2014/2/2/56/134588


  Introduction Top


Rheumatoid arthritis (RA) is in itself, not fatal, but the associated complications may shorten the survival. The cardiac manifestations vary from asymptomatic phase to sudden cardiac death.

The cardiac involvement in RA was first reported by Charcoat in 1881, [1] and the first report of complete heart block due to a rheumatoid granuloma involving the conduction pathways was shared in 1959. [2] Until 1983, about 20 cases were reported and Ahern et al. reported a series of 8 cases. [3]

Implanting a permanent pacemaker offers a reasonably good prognosis in the absence of other cardiac lesions.


  Case report Top


The case we present here is about a 42-year-old female patient who was referred to Krishna Hospital with the complaints of low grade fever with exertional dyspnea of NYHA Class II, wheezing, nocturnal dyspnea along with postcough noncardiac left chest pain since 3 months with anorexia. The patient was a known case of RA since 9 years and was on methotrexate 10 mg once-a-week and 600 mg dose of hydroxychloroquine daily. She received steroids intermittently for flaring up of RA. There was a past history of tubercular left pleural effusion. On general examination, she was febrile 101°F, and had a pulse 120/min, blood pressure (BP)-140/90 mm of Hg, pallor present, jugular venous pressure was raised, bipedal, and sacral edema present. Cardiovascular (CVS) examination-patient had tachycardia, apical short systolic murmur Grade III/VI. Respiratory system (RS)-tachypnea with respiratory rate −40/min, bilateral fine crackles with bronchovesicular breath sounds. Abdominal examination revealed right hypochondriac tenderness. Central nervous system examination was grossly normal. The electrocardiogram (EKG) showed low voltage pattern, tachycardia and atrioventricular (A-V) dissociation. The patient was treated with diuretics; weight based anti-tubercular therapy, along with disease-modifying anti-rheumatic drug's (DMARDs) which the patient was already on. The blood picture was as follows: Hemoglobin - 8.3 g%, total count - 24,700, erythrocyte sedimentation rate (ESR) - 50 mm/h. The chest X-ray showed left pleural effusion and bilateral lower lobe consolidation. A pleural fluid analysis was found to be exudative in nature. The high-resolution computed tomography showed left lower lobe collapse, bilateral pleural effusion and pleural thickening. Two-dimensional echocardiography (2D-ECHO) suggested a large pericardial effusion. The patient gradually improved and was discharged with a provisional diagnosis of:

  • Acute left ventricular failure with myocarditis of connective tissue disorder;
  • Polyserositis with connective tissue disorder;
  • Left lower zone necrotizing pneumonia;
  • Left tubercular pleural effusion.


In following visits, patient complained of persistent arthralgia's involving the intertarsal joints, ankle joints, and knee joints without swelling. The movements were grossly restricted. Her C-reactive protein (CRP) was −2.4 IU, ESR - 80 mm/h and RA factor was strongly positive by qualitative method. Thus, she was diagnosed to have exacerbation of RA. Her DMARD's were continued and prednisolone was initiated at 40 mg twice weekly. The pericardial effusion had subsided with no signs of cardiac failure. Tuberculosis had resolved leaving scanty fibrotic scars with no pleural effusion. EKG findings were same as before.

Pyrazinamide was omitted, anticipating pyrazinamide arthropathy. Diuretics and angiotensin receptor blocker's were omitted on the assumption of reverted myocarditis and the patient was advised to continue other anti-tubercular drugs for 9 months. The subsequent two follow-up's were uneventful, other than symptoms related to wrist ankylosis. Prednisolone and hematinics were discontinued. In the next follow-up, patient complained of mild, continuous type of chest pain in the mid-chest and the high epigastric region associated with exertional dyspnea NYHA Class I, which would increase by activity including talking. On examination-patients weight remained constant, afebrile, BP - 130/80 mm of Hg, pulse - 60/min, jugular venous pressure normal, no edema, cyanosis, and icterus. CVS - same apical systolic murmur was present with no gallop. RS was clear, per abdomen was suggestive of epigastric tenderness, with mild hepatomegaly. Chest X-ray revealed-distinct cardiomegaly as compared to previous films [Figure 1], dilated pulmonary arteries, bilateral pleural effusion, and diffuse reticular and alveolar shadows of pulmonary edema. The EKG findings were A-V dissociation with junctional rhythm [Figure 2]. The provisional diagnosis then was left ventricular failure with pulmonary edema. She was started on diuretics. Prednisolone was restarted in a dose of 40 mg twice weekly and DMARD's were continued. The patient refused hospitalization hence was managed as an outpatient. She gradually improved with re-inclusion of steroids, which probably suggests that its omission relapsed the cardiac failure and was advised to continue the same treatment. One month later, she was asymptomatic and hence diuretics were omitted. Her complete blood counts had returned to normal, ESR - 24 mm/h, CRP-negative, EKG-junctional rhythm, with A-V dissociation. The patient remained asymptomatic for next 6 months except for a few episodes of arthralgias not warranting a medical consult. Thus, she was given a trial of omission of steroids. Patient remained asymptomatic for the next 3 months after which she presented with breathlessness NYHA Class III, with bradycardia associated with syncope and healed ulcers at finger tips, CVS examination revealed bradycardia apical systolic murmur RS-occasional fine crackles. Left ankle and metatarsophalangeal joints were tender without swelling. Right wrist and metacarpophalangeal joint had mild swelling with tenderness. EKG revealed ventricular complexes predominantly left bundle branch block with rate of 30/min, BP - systolic 70 mm of Hg. She was restarted on steroids and cardiology consult was sought for percutaneous coronary angiography (PCA) to rule out coronary artery disease with permanent pacemaker implantation.
Figure 1: Chest radiograph showing cardiomegaly, interstitial edema, left pleural effusion

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Figure 2: Electrocardiogram of the patient with rheumatoid arthritis showing complete heart block

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Her 2D-ECHO was normal, her PCA showed normal epicardial coronaries. A VVIR permanent pacemaker was implanted following which she has remained asymptomatic so far.


  Discussion Top


Complete A-V dissociation is a rare complication of RA does not respond to anti-inflammatory and immunosuppressive therapy. [4] The approximate incidence of complete heart block in RA mentioned by Ahern et al. is 1 in 1000 patients of RA, whereas Raskar and Cosh found the incidence of 1 in 1600 patients. [3],[5] Females are more prone to develop complete heart block and are more prevalent in patients with subcutaneous nodules there are case reports of trans-placental passage of autoantibodies from mother's positive for anti-Ro/SSA (and/or anti-La/SSB) to their newborns. [3],[6] On an average 0.1-3% of infants whose mothers are antibody-positive develop congenital heart block. [7] The recurrence risk is 10 times higher in the subsequent pregnancies. [8]

Conduction defects are generally seen in patients with RA with established erosive nodular rheumatoid disease (mainly seen during the active phase of the disease, especially with a high titer of circulating rheumatoid factors). The postulated mechanisms for pathophysiology are: [2]

  1. Primary infiltration of the A-V node or other conducting tissue by mononuclear cells or rheumatoid granulomas.
  2. Vasculitis of the arterial supply to conductive tissue.
  3. Extension of the inflammatory process from the base of the aorta or mitral valves to the conduction pathways.
  4. Amyloidosis.
  5. Hemorrhage into a rheumatoid nodule.


The conduction defect is progressive and often asymptomatic. However, it may also present as a sudden onset of complete block complicated with episodes of syncope. Often the blocks are irreversible and demand an implantation of permanent pacemaker. However, the prognosis is guarded once patients develop congestive cardiac failure before implantation of a pacemaker. In patients with dilated cardiomyopathy and severely impaired contractility resynchronization therapy provides beneficial effect on hemodynamics and long-term survival. Sudden cardiac death in patients with RA generally occurs due to atherosclerotic coronary artery disease, leading to acute coronary syndrome, and ventricular arrhythmias.

In our patient, we found that patient's symptoms aggravated on three separate occasions while attempting to withdraw glucocorticoids, possibly showing that the disease is active during the high circulating antibody titers.

 
  References Top

1.Charcot JL. Clinical Lectures on Senile and Chronic Diseases. Vol. 95. London: Sydenham Society; 1881. p. 172-5.  Back to cited text no. 1
    
2.Handforth CP, Woodbury JF. Cardiovascular manifestations of rheumatoid arthritis. Can Med Assoc J 1959;80:86-90.  Back to cited text no. 2
[PUBMED]    
3.Ahern M, Lever JV, Cosh J. Complete heart block in rheumatoid arthritis. Ann Rheum Dis 1983;42:389-97.  Back to cited text no. 3
[PUBMED]    
4.Seferoviæ PM, Ristiæ AD, Maksimoviæ R, Simeunoviæ DS, Ristiæ GG, Radovanoviæ G, et al . Cardiac arrhythmias and conduction disturbances in autoimmune rheumatic diseases. Rheumatology (Oxford) 2006;45 Suppl 4:iv39-42.  Back to cited text no. 4
    
5.Rasker JJ, Cosh JA. Cause and age at death in a prospective study of 100 patients with rheumatoid arthritis. Ann Rheum Dis 1981;40:115-20.  Back to cited text no. 5
[PUBMED]    
6.Owlia MB, Mostafavi Pour Manshadi SM, Naderi N. Cardiac manifestations of rheumatological conditions: A narrative review. ISRN Rheumatol 2012;2012:463620.  Back to cited text no. 6
    
7.Maisch B, Ristiæ AD. Immunological basis of the cardiac conduction and rhythm disorders. Eur Heart J 2001;22:813-24.  Back to cited text no. 7
    
8.Cimaz R, Duquesne A. Neonatal lupus syndromes. Arch Pediatr 2006;13:473-8.  Back to cited text no. 8
    


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