Natural History of Patients Diagnosed with Cardiac Sarcoidosis at Left Ventricular Assist Device Implantation or Cardiac Transplantation
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Published on: July 18, 2014
Tyler J Schmidt1, Andrew N Rosenbaum2, Nikhil Kolluri1
1From the Department of Internal Medicine.
Insights
Cardiac sarcoidosis (CS) diagnosed during heart transplantation or LVAD implantation often presents as severe heart failure. Outcomes for these patients are comparable to those without CS.
Area of Science:
- Cardiology
- Pathology
- Immunology
Background:
- Cardiac sarcoidosis (CS) diagnosis often occurs late, particularly when identified incidentally during advanced heart failure therapy evaluations.
- The natural history of CS diagnosed pathologically at left ventricular assist device (LVAD) implantation or cardiac transplantation is not well-documented.
Purpose of the Study:
- To describe the clinical characteristics and outcomes of patients with CS incidentally diagnosed via pathological examination of the apical core or explanted heart.
- To compare these outcomes with patients diagnosed with CS prior to advanced heart failure therapies.
Main Methods:
- Retrospective review of 232 patients meeting CS criteria.
- Pathological confirmation of CS in 54 patients, with 10 diagnosed incidentally during LVAD implantation or heart transplantation.
- Comparison of clinical data and outcomes between the 10 incidentally diagnosed patients and 10 patients with pre-existing CS.
Main Results:
- The 10 incidentally diagnosed patients often presented with severe dilated cardiomyopathy (mean ejection fraction 23%).
- Half of the incidentally diagnosed patients had no prior diagnosis of sarcoidosis.
- No significant survival difference was observed between incidentally diagnosed CS patients and those with known CS or general advanced heart failure cohorts.
Conclusions:
- Incidental pathological diagnosis of CS during LVAD or transplant evaluation can reveal severe cardiomyopathy as the initial presentation.
- Patients with CS diagnosed during advanced heart failure therapies may have outcomes similar to non-CS patients, suggesting effective management strategies.
Abstract:
To our knowledge, natural history has not been reported for cardiac sarcoidosis (CS) diagnosed by pathologic evaluation of the apical core at left ventricular assist device (LVAD) implantation or cardiac transplantation. We retrospectively identified 232 consecutive patients meeting CS criteria. Of these patients, 54 were diagnosed by pathologic confirmation of CS, 10 after evaluation of the apical core (LVAD implant) or explanted heart (transplant). We compared clinical characteristics at initial evaluation and outcomes for these 10 patients with those of 10 patients with known CS before LVAD implant/transplant. In the study group, five patients (50%) had confirmed extracardiac sarcoidosis before LVAD implant/transplant; five had not been diagnosed with sarcoidosis. Mean (standard deviation) left ventricular ejection fraction at initial evaluation was 23% (16%), and left ventricular end-diastolic dimension was 61 (10) mm. Four patients died during follow-up; however, no survival difference was found for the 10 patients diagnosed incidentally and the group with a previous diagnosis or institutional LVAD/transplant cohorts. Patients diagnosed with CS on pathological examination of the apical core/explanted heart may have severe dilated cardiomyopathy as the initial presentation. Outcomes for patients with CS after advanced heart failure therapies may be comparable with those of non-CS patients.
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