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The goal of this research is to prospectively evaluate the performance of the electromechanical window according to a phonographic method, as a mean of diagnosis of long QT syndrome, and to compare its performance with routine tests used.
Familial long QT syndrome (LQTS) is a hereditary cardiac disorder in which most affected family members have delayed ventricular repolarization manifest on the electrocardiogram (ECG) as QT prolongation. This disease is associated with an increased propensity to palpitations, syncope, polymorphous ventricular tachycardia and sudden arrhythmic death. The diagnosis relies mostly on resting ECG findings and on genetic testing. In clinical practice however, this diagnosis is complicated by 2 main reasons: 1) a significant overlap in ECG findings between healthy and diseased individuals and 2) a frequent identification of genetic variants of unknown significance. Recent studies have suggested that echocardiographic measurement of the electromechanical window (EMW - the delay between the end of mechanical contraction and electrical activation of the heart) has better performance in the diagnosis of LQTS. The echocardiographic technique is however too complicated for routine clinical use. Preliminary work conducted at the University Hospital of Bordeaux and at the University Hospital of Reunion Island has demonstrated that a phonocardiographic approach leads to similar results with an improved feasibility and a good reproducibility.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Prospective FEMQT Group | Experimental | Propositus patients with genetically proven LQTS and their relatives |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| ECG coupled to phonocardiography | Diagnostic Test | A standard ECG is recorded along with a phonocardiographic recording. The phonocardiographic recording is obtained by using a specific electronic stethoscope plugged into the auxiliary port of the ECG machine. The recording takes less than 30 seconds. The two recording methods are concomitant. QT interval is measured on the ECG recording, EMW is measured with ECG coupled to phonocardiography. |
| Measure | Description | Time Frame |
|---|---|---|
| Negative predictive value (NPV) obtained by the two methods of diagnosis | The NPV is measured in percentage. The NPV of LQTS diagnosis obtained by phonocardiographic method (measurement of the EMW) and the NPV obtained by standard ECG method (QT measurement prolongation) will be compared. | Day 1 |
| Measure | Description | Time Frame |
|---|---|---|
| Values of sensitivity obtained by the two methods of diagnosis | Value of sensitivity is measured in percentage. The values of sensitivity of EMW and standard ECG will be compared. | Day 1 |
| Values of specificity obtained by the two methods of diagnosis |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Josselin DUCHATEAU, MD | University Hospital, Bordeaux | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Nantes University Hospital | Nantes | 44093 | France | |||
| Bordeaux University Hospital |
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| ID | Term |
|---|---|
| D008133 | Long QT Syndrome |
| ID | Term |
|---|---|
| D001145 | Arrhythmias, Cardiac |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
| D000075224 | Cardiac Conduction System Disease |
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|
Value of specificity is measured in percentage. The values of specificity of EMW and standard ECG will be compared.
| Day 1 |
| Positive predictive values obtained by the two methods of diagnosis | Positive predictive value is measured in percentage. Positive predictive values of EMW and standard ECG will be compared. | Day 1 |
| Area Under the Curves (AUC) obtained by the two methods of diagnosis | The AUC obtained by Receiver Operating Characteristic (ROC) analyses is measured by a value between 0 and 1. The AUC obtained by ROC of EMW and standard ECG will be compared. | Day 1 |
| Pessac |
| 33604 |
| France |
| La Réunion University Hospital | Saint-Pierre | 97410 | France |
| Toulouse University Hospital | Toulouse | 31400 | France |
| D006330 | Heart Defects, Congenital |
| D018376 | Cardiovascular Abnormalities |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |