Outcome of Cardiac Sarcoidosis Presenting With High-Grade Atrioventricular Block

Hanna-Kaisa Nordenswan1, Jukka Lehtonen1, Kaj Ekström1

  • 1Heart and Lung Center (H.-K.N., J.L., K.E., R.K., P.S., M.K.).

Insights

Cardiac sarcoidosis with high-grade atrioventricular block (AVB) carries a significant risk of sudden cardiac death. Early implantation of an intracardiac cardioverter defibrillator is recommended for patients needing permanent pacing.

Area of Science:

  • Cardiology
  • Electrophysiology
  • Immunology

Background:

  • Symptomatic high-grade atrioventricular block (AVB) is a primary manifestation of cardiac sarcoidosis.
  • The risk of fatal arrhythmias, particularly in lone AVB, is not well-established.
  • Current recommendations favor intracardiac cardioverter defibrillator (ICD) over pacemakers for cardiac sarcoidosis.

Purpose of the Study:

  • To analyze presentations, left ventricular (LV) function, and arrhythmias in cardiac sarcoidosis patients.
  • To determine the incidence of sudden cardiac death and ventricular arrhythmias in relation to AVB severity and LV dysfunction.
  • To evaluate the risk stratification for fatal arrhythmias in cardiac sarcoidosis.

Main Methods:

  • Utilized the Myocardial Inflammatory Diseases in Finland Study Group Registry (1988-2015).
  • Analyzed 325 cardiac sarcoidosis cases, focusing on 143 patients with high-grade AVB (Mobitz II or third degree) without other cardiac causes.
  • Assessed LV function, ventricular arrhythmias, and outcomes including sudden cardiac death during a median 2.8-year follow-up.

Main Results:

  • Of 143 patients, 90 presented with lone AVB, 29 with nonsevere LV dysfunction, and 20 with severe LV dysfunction or ventricular tachycardia.
  • The 5-year composite incidence of arrhythmic events was 56% in severe LV dysfunction/VT group, 24% in nonsevere LV dysfunction group, and 24% in lone AVB group.
  • The 5-year incidence of sudden cardiac death was 34% in the severe LV dysfunction/VT group, 14% in the nonsevere LV dysfunction group, and 9% in the lone AVB group.

Conclusions:

  • Cardiac sarcoidosis with high-grade AVB presents a substantial risk of sudden cardiac death, irrespective of LV dysfunction or ventricular tachycardia.
  • The findings support the consensus recommendation for ICD implantation in patients with cardiac sarcoidosis requiring permanent pacing.
  • Risk stratification based on LV function and arrhythmias is crucial for managing these patients.
Abstract

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