Ms. V. Tea is a 55-year-old woman with a history of cardiac sarcoidosis, heart failure with mildly reduced ejection fraction (HFmrEF – EF 40%), and ventricular tachycardia with CRT-D who presents with recurrent VT. She has undergone several attempts at catheter ablation of VT in the past and previously had been trialed on amiodarone which was discontinued due to hepatotoxicity. She now continues to have episodic VT requiring anti-tachycardia pacing and ICD shocks despite medical therapy with mexiletine, metoprolol, and sotalol. Her most recent PET scan showed no active areas of inflammation. Currently, her vital signs are stable, and labs are unremarkable. What is the best next step for this patient?

A

Evaluation for heart transplant

B

Evaluation for LVAD

C

Dobutamine

D

Prednisone

E

None of the above

Explanation

The correct answer is A – evaluation for heart transplant.

For selected patients with advanced heart failure despite GDMT, cardiac transplantation is indicated to improve survival and quality of life (Class 1, LOE C-LD). Heart transplantation, in this context, provides intermediate economic value.

Clinical indicators include refractory or recurrent ventricular arrhythmias with frequent ICD shocks. Patient selection for heart transplant includes assessment of comorbidities, goals of care, and various other factors. The United Network of Organ Sharing Heart Transplant Allocation Policy was revised in 2018 with a 6-tiered system to better prioritize unstable patients and minimize waitlist mortality. VT puts the patient as a Status 2 on the transplant list. There was a contemporary analysis of patients with end-stage cardiomyopathy due to cardiac sarcoidosis, published in Journal of Cardiac Failure, in 2018 that demonstrated similar 1-year and 5-year survival after heart transplant between patients with and without cardiac sarcoidosis.

Choice B (evaluation for LVAD) is incorrect. While bridge to transplant with LVAD is definitely a potential next step in patients with cardiac sarcoidosis, it is not recommended in patients presenting primarily with refractory ventricular arrhythmias due to granuloma-induced scarring. In this situation, patients benefit from direct heart transplant rather than bridge to transplant LVAD approach. The same study, described before in the Journal of Cardiac Failure, also showed similar 1-year and 5-year survival after bridge-to-transplant mechanical circulatory support between patients with and without cardiac sarcoidosis. Since cardiac sarcoidosis is not just limited to the left ventricle, patients being considered for LVAD need hemodynamic assessment to determine the risk of post-LVAD RV failure.

Choice C (dobutamine) is incorrect. The patient is currently not decompensated in terms of contractility nor is showing signs of cardiogenic shock. Further, dobutamine may worsen arrhythmia burden.

Choice D (prednisone) is incorrect as there is no sign of active inflammation on the PET scan. The recurrent ventricular arrhythmias are being driven by granuloma-induced scar.

Main Takeaway

Cardiac transplantation has a Class 1 (LOE C-LD) recommendation for eligible patients with advanced HF despite GDMT to improve survival and quality of life. Specifically, direct heart transplantation is the best next step in patients with cardiac sarcoidosis and refractory ventricular arrhythmias rather than a bridge-to-transplant approach.

Guideline Loc.

Section 8.5