P1 NEW TREATMENT STRATEGIES IN PATIENTS WITH SENILE CARDIAC AMYLOIDOSIS

Abstract

Abstract We present the case of a 75 year–old man, diabetic, dyslipidemic, hypertensive, previous ischemic heart disease, AF, previous hospitalizations for heart failure, who underwent cardiology evaluation for worsening dyspnea (NYHA class II–III), swallen ankles and elevated BNP levels (900 pg/ml). The patient suffered from sensory–motor polyneuropathy diagnosed with EMG and ENG. Echocardiographic evaluation: severe concentric hypertrophy of the left ventricle (SIV 18 mm, PP 17 mm), granular sparkling and EF 50%, left atrium dilatation, light–moderate mitral regurgitation, moderate tricuspid regurgitation with PAPS 40–45 mmHg. Circumferential pericardial effusion (12 mm). Moreover, we noticed a decreased longitudinal contractive function of the left ventricle (TDI e‘, a’, e s’ <5 cm/s e absolute GLS < 15%), speckle–tracking ‘cherry on the top‘ and restrictive diastolic pattern. •ECG: low QRS voltage and increased wall thickness (>12 mm). •Laboratory: (SPIE and UPIE) no monoclonal protein present. •Bone scintigraphy: grade 3 cardiac uptake. •Cardiac MRI: asymmetric hypertrophy of the left ventricle with moderate biventricular systolic dysfunction. Subendocardial late gadolinium enhancement. •Genetic testing: no sarcomeric or TTS gene mutations. All findings were suggestive for senile cardiac amyloidosis (ATTR wt), the patient was discharged from the hospital with optimized medical therapy for heart failure and follow up was prescribed. After 3 months from the first diagnosis we added Tafamidis to the therapy (ESC indications class IB). ATTR–ACT, published on ‘New England Journal of Medicine’ showed that Tafamis is superior to placebo in reducing the combination of all–cause mortality and cardiovascular–related hospitalizations. Tafamidis was also associated with a significant reduction in the decline in functional capacity (as measured by the 6–minute walk test) and the decline in quality of life (as measured by the KCCQ–OS) at month 30, with differences first observed at 6 months. Tafamidis was well–tolerated, with a safety profile comparable to placebo. Follow–up: 6 months later the introduction of Tafamidis, the patient has not needed other hospitalizations for heart failure, well tolerates the drug with a subjective symptom reduction and remarkable improvement in quality of life.