High Resolution Three-dimensional Maps of the Right Chambers in Patient Diagnosed With Cardiac Amyloidosis

University Hospital Center of Martinique24 enrolled

Overview

Amyloid heart disease is an accumulation of fibrillar proteins in the extracellular sector of the heart. Identified on echocardiography as Ventricular hypertrophy. The investigation of a Left Ventricular hypertrophy (LVH) is the most frequent discovery circumstance of amyloid heart disease. Pathophysiological mechanisms poorly understood, resulting in late diagnosis. Transthyretin amyloid heart disease (CATTR) is the most common form of cardiac amyloidosis in the West Indies due to an abnormally high frequency of the Val122Ile and Val107Ile mutations of the transthyretin gene in this population. Val122Ile and Val107Ile mutated-transthyretin are the substitution of valine for isoleucine at codon 122 of the TTR gene ( V122I) and at codon 107 of the TTR gene (V107I). Complications of CATTR are functional changes in heart cells or even death due to mechanical abnormalities (loss of contractility and increased wall stiffness cardiac arousal and conduction disturbances). These disorders result from an electrical abnormality of the heart the reason why the cardiologist performs preventive performance of electrophysiological explorations with EnSite Precision™. It's a registration system used to detect foci of necrosis within the myocardium. Amyloid deposits are areas devoid of electrical activity. Do they detectable by the EnSite Precision™ recording system ?

Transthyretin's amyloid heart disease (CATTR) is a rare disease whose frequency is high in the Caribbean's due to a high frequency of Val122Ile, an amyloidosis prone mutation in the Transthyretin gene. The Val122Ile variant might be present in 15 to 20 000 subjects in Martinique, placing them at high risk to develop the CATTR. CATTR results from the accumulation of amyloid deposits between the intercellular spans, resulting in mechanical cardiac abnormalities, but also in latent excitation or conduction defects: atrial and ventricular hyperexcitability, bundle branch blocks, atrio-ventricular blocks. These abnormalities require systematic electrophysiological studies and if necessary, antiarrythmic medications or pacemaker placement. Electro-mapping of the cardiac chambers offers high-resolution three-dimensional maps of cardiac electrical activity which has been used recently to detect focal myocardial infarction. This anatomo-functional imaging, used only once in cardiac amyloidosis, showed a correlation between areas of low voltage of the left atrial myocardium and areas of late gadolinium enhancement, a marker of amyloidosis deposit, found in cardiac MRI.

Study Type

INTERVENTIONAL

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

Inclusion Criteria: Controls: * Aged 18 and more * Have had an echocardiogram within 6 months prior to inclusion * Have had a cardiac Holter in the 6 months prior to inclusion. * Informed Consent given Cases : * Aged 18 and over * Presence of cardiac amyloidosis with Transthyretin * Have had an echocardiogram within 6 months prior to inclusion * Have had a cardiac Holter monitoring in the 6 months prior to inclusion. * Informed Consent given Exclusion Criteria: Controls * Known case of amyloidosis in the immediate family * Patient known to have amyloidosis * Left ventricular wall thickness greater than or equal to 14 mm * Hyperechogenicity of the left ventricular walls * Cardiac disease which may affect electro-anatomic mapping: Right ventricular dysplasia, myocardial infarction, congenital heart disease. * Contraindication such as pregnancy to radiological exams * Presence of an anomaly of the vena cava * Presence of intracavitary thrombus at cardiac echocardiography * Patients with a pacemaker Cases * Cardiac disease which may affect electro-anatomic mapping: Right ventricular dysplasia, myocardial infarction, congenital heart disease. * Contraindication such as pregnancy to radiological exams * Presence of an anomaly of the vena cava * Presence of intracavitary thrombus at cardiac echocardiography

Outcomes

Primary Outcomes

Percentage of zones with abnormal electrical activity

Percentage of subject with at least one area of electrical inactivity (\<0.1 mV) or at least one area of continuous low-voltage activity

Time frame: 1 month

Secondary Outcomes

Characteristics of zones with electrical inactivity

Number of areas of electrical inactivity (\<0.1 mV)

Time frame: 1 month

Zones with electrical inactivity

Area of surface of electrical inactivity (\<0.1 mV)

Time frame: 1 month

Characteristics of zones with abnormal electrical activity

Number of areas of continuous low voltage activity

Time frame: 1 month

Zones with abnormal electrical activity

Surface of areas of continuous low voltage activity

Time frame: 1 month

Compare electrical activity anomaly to total longitudinal strain on cardiac ultrasound

Number of areas of electrical inactivity vs. total longitudinal strain on cardiac ultrasound.

Time frame: 1 month

Electrical activity anomaly and total longitudinal strain on cardiac ultrasound

Number of areas of continuous low voltage activity vs. Total longitudinal strain on cardiac ultrasound.

Time frame: 1 month

Compare electrical activity anomaly (surface) to total longitudinal strain on cardiac ultrasound

Surface of areas of electrical inactivity vs. Total longitudinal strain on cardiac ultrasound.

Time frame: 1 month

Electrical activity anomaly (continuous low voltage activity) and total longitudinal strain on cardiac ultrasound

Surface of areas of continuous low voltage activity vs. Total longitudinal strain on cardiac ultrasound.

Time frame: 1 month

Compare electrical activity anomaly to Brain Natriuretic Peptide (BNP) value

Number of areas of electrical inactivity vs. BNP value.

Time frame: 1 month

Electrical activity and Brain Natriuretic Peptide (BNP) value

Number of areas of continuous low voltage activity vs. BNP value.

Time frame: 1 month

Compare electrical activity anomaly (surface) to Brain Natriuretic Peptide (BNP) value

Surface of areas of electrical inactivity vs. BNP value.

Time frame: 1 month

Electrical activity anomaly (continuous low voltage activity) and Brain Natriuretic Peptide (BNP) value

Surface of areas of continuous low voltage activity vs. BNP value.

Time frame: 1 month

Compare electrical activity anomaly to the presence of severe ventricular arrhythmia

Number of areas of electrical inactivity vs. the presence of severe ventricular arrhythmia.

Time frame: 1 month

Electrical activity anomaly and presence of severe ventricular arrhythmia

Number of areas of continuous low voltage activity vs. the presence of severe ventricular arrhythmia.

Time frame: 1 month

Compare electrical activity anomaly (surface) to the presence of severe ventricular arrhythmia

Surface of areas of electrical inactivity vs. the presence of severe ventricular arrhythmia.

Time frame: 1 month

Electrical activity anomaly (continuous low voltage activity) and presence of severe ventricular arrhythmia

Surface of areas of continuous low voltage activity vs. the presence of severe ventricular arrhythmia.

Time frame: 1 month

Compare electrical activity anomaly and to the presence of an atrial arrythmia

Number of areas of electrical inactivity vs. the presence of an atrial fibrillation load.

Time frame: 1 month

Electrical activity anomaly and presence of an atrial arrythmia

Number of areas of continuous low voltage activity vs. the presence of an atrial fibrillation load.

Time frame: 1 month

Compare electrical activity anomaly (surface) to the presence of an atrial arrythmia

Surface of areas of electrical inactivity vs. the presence of an atrial fibrillation load.

Time frame: 1 month

Electrical activity anomaly (continuous low voltage activity) and presence of an atrial arrythmia

Surface of areas of continuous low voltage activity vs. the presence of an atrial fibrillation load.

Time frame: 1 month

High Resolution Three-dimensional Maps of the Right Chambers in Patient Diagnosed With Cardiac Amyloidosis

Overview

Conditions

Interventions

Eligibility

Locations (1)

Outcomes

Primary Outcomes

Secondary Outcomes

Central Contacts