Prevalence of echocardiographic features suggesting cardiac sarcoidosis in patients with pacemaker or implantable cardiac defibrillator

Abstract

Background and objectives: Basal septal thinning or localized aneurysmal dilatation without coronary artery disease has been described as a characteristic finding suggestive of cardiac sarcoidosis. We sought to assess the prevalence of this characteristic echocardiographic finding in patients with pacemaker (PM) or implantable cardiac defibrillator (ICD).

Subjects and methods: Echocardiography of patients who received PM or ICD were retrospectively analyzed. Patients with marked thinning and akinesia confined to the basal septum (type 1), or posterolateral wall resulting in localized aneurysmal outward bulging (type 2) without history of myocardial infarction or significant coronary stenosis were included for analysis.

Results: Among 1,357 consecutive patients, 21 exhibited suggestive echocardiographic findings (type 1/2=15/6) with a mean ejection fraction of 37±11%. The prevalence was 1.2% in the PM group and 4.0% in the ICD group. Only 3 patients showed histologically confirmable sarcoidosis in lymph nodes, lung and heart, respectively. Endomyocardial biopsy was attempted in 6 patients, but failed to demonstrate sarcoidosis. The 1-, 2-, 4- and 6-year clinical events (death, cardiac transplantation and hospital admission)-free survival rates were 100%, 85.7±7.6%, 75.0±9.7% and 48.6±12.4%, respectively. During follow-up, two patients with PM underwent ICD implantation, and another underwent heart transplantation.

Conclusion: Prevalence of echocardiographic features suggesting prevalence of cardiac sarcoidosis is low in patients who underwent device implantation. However, considering the very low yield of endomyocardial biopsy and the rare extracardiac manifestations in cardiac sarcoidosis, characteristic echocardiographic findings could be an adjunctive diagnostic criterion in these patients.

Keywords: Echocardiography; Implantable cardioverter-defibrillators; Pacemaker; Sarcoidosis.

Fig. 1

Representative echocardiographic images suggestive of cardiac involvement of sarcoidosis. Type 1 morphologic change is characterized by thinning and akinesia of the basal septum (A and B). Aneurysmal dilatation involving inferior or posterolateral wall is classified as type 2 (C and D).

Fig. 2

(A) and (C) are computed tomographic images of a 53-year old patient those demonstrate numerous enlarged lymph nodes compressing the adjacent structures. (B) is the echocardiographic image in which the arrow points at the basal septum with thinning. Panel D is a micrograph of a supraclavicular lymph node with non-caseating granuloma (hematoxylin and eosin stain, 200×; Scale bar=100 µm).

Fig. 3

A 44-year old man who had undergone pacemaker insertion 2 years before due to complete atrio-ventricular block complained of recurrent palpitation and dyspnea. He was diagnosed with atrial flutter and received radiofrequency catheter ablation. (A) Thereafter, thinning of basal septum became more prominent while symptoms of heart failure are more aggravated over the next 16 months. (C) denotes the echocardiography at the time of heart transplantation. (D) is a micrograph showing non-caseating granuloma from tricuspid valve of extracted heart (hematoxylin and eosin stain, 200×; Scale bar=100 µm).

Fig. 4

Event free survival of the overall group (A), and comparisons with those according to echocardiographic type (B), and the class of cardiac device inserted (C). Adverse clinical events included death, cardiac transplantation, and hospital admission due to cardiac causes during the follow-up period. ICD: implantable cardioverter-defibrillator, PM: pacemaker.