49 A case report of cardiac contractility modulation therapy for heart failure treatment in patient with amyloidosis: is to be considered a valid therapeutic option?
Authors: Mattera Agostino , Coscia Vincenzo , Brignoli Marcello , Fusco Angela , Concilio Claudia , Chianese Raffaele , Michele Alfonso De , Pariggiano Ivana , Diana Vincenzo , Viscusi Miguel , Calabrò Paolo 
Published: December 2021

Abstract

Aims
Cardiac amyloidosis (CA) is primarily associated with fibril deposits in many cardiac structures, causing biventricular wall thickness and stiffness. CA may result in arrhythmias and particularly in an aggressive form of heart failure (HF). Cardiac contractility modulation (CCM) showed to be a concrete therapeutic option in patients with symptomatic HF despite optimal medical therapy (OMT), with Left Ventricular Ejection Fraction (LVEF) between 25% and 45%, with narrow QRS complex (&lt;130 ms). This case aims to further explore the effectiveness of CCM therapy in a patient affected by concomitant ischaemic cardiomyopathy and CA.

Methods and results
A 42-year-old man with Chronic HF secondary to both post-ischaemic due to spontaneous coronary artery dissection (SCAD) and post alcoholic dilated cardiomyopathy was hospitalized at our department in February 2020 due to worsening HF (3rd HF hospitalization in the same year). The patient was a NYHA class III, with chronic kidney failure, a narrow QRS complex (100 ms) and a LVEF of 27% with familiar history of sudden death, already implanted with ICD. The patient resulted untreatable with sacubitril/valsartan, as it elicited strong hypotension. During current hospitalization the BNP value was 942.60 pg/ml, and the Quality of Life (QoL) evaluated from Minnesota Living with Heart Failure Questionnaire (MLHFQ) score was 72 points. Moreover, the patient underwent umbilical biopsy that confirmed the presence of amyloidosis. Thus, the CCM therapy device (Optimizer® Smart, Impulse Dynamics) was implanted to try to reduce HF symptoms and hospitalizations. The therapy was programmed for 10 h per day, with delivery of CCM from both septal leads with amplitude of 6.5 V at 20.56 ms pulses duration. Figure 1A and B shows the septal position of leads and a surface ECG with the CCM therapy spike after QRS.
The patient significantly improved as early as the first period after implantation. The 10-month in-office FU performed on December 2020 revealed in addition to the absence of new HF hospitalizations, a significant improvement in QoL and HF-symptoms, with a MLWHFQ score of 42, an enhancement to NHYA class II, and even a slight decrease of BNP of 767 pg/ml. The echo exam revealed no significant changes in the EF, with an improvement of global longitudinal strain and no worsening of other haemodynamic parameters. A further FU performed in June 2021 showed continuous improvement of QoL with a MLWHFQ score of 25 e no HF hospitalizations.

Conclusions
In this patient affected by multiple cardiomyopathies, including CA, CCM therapy proved to improve its QoL with no HF hospital admissions since the implantation. The absence of significant echocardiographic worsening is a positive aspect, considering the patient’s status, the concomitant aetiologies, and the presence of amyloidosis, given its progressive and infiltrative nature.