A Study of SGT-501 Gene Therapy in Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)

NCT ID: NCT07148089

Last Updated: 2025-12-29

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

PHASE1

Total Enrollment

18 participants

Study Classification

INTERVENTIONAL

Study Start Date

2026-01-05

Study Completion Date

2031-05-31

Brief Summary

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This is a Phase 1b, Multicenter, Open-Label, Dose Finding Study to Investigate the Safety and Tolerability of a Single Intravenous Dose of SGT-501 in participants with catecholaminergic polymorphic ventricular tachycardia (CPVT). The first-in-human (FIH) safety study will focus on obtaining safety data in adult participants. Cohort 1 and Cohort 2 (optional for dose exploration) will include participants ≥ 18 years of age. Cohort 3 will include participants ≥ 7 to \< 18 years of age and will be initiated following data and safety monitoring board (DSMB) recommendations. Participants will be monitored for 5 years post-administration of SGT-501 including the active treatment period (1 year) and long-term follow-up (LTFU) (4 years) period.

Detailed Description

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Conditions

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Catecholaminergic Polymorphic Ventricular Tachycardia

Keywords

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Catecholaminergic polymorphic ventricular tachycardia (CPVT) SGT-501 Ryanodine Receptor 2 (RYR2) adeno-associated virus serotype 8 (AAV8) Cardiac Gene Therapy

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Cohort 1 (≥ 18 years of age)

Participants will receive a single intravenous (IV) infusion (dose 1) of SGT-501.

Group Type EXPERIMENTAL

SGT-501

Intervention Type DRUG

IV for infusion

Cohort 2 (≥ 18 years of age)

Participants will receive a single IV infusion (dose 2) of SGT-501.

Group Type EXPERIMENTAL

SGT-501

Intervention Type DRUG

IV for infusion

Cohort 3 (≥ 7 to < 18 years)

Participants will receive a single IV infusion (level at or below dose(s) assessed in adults) of SGT-501.

Group Type EXPERIMENTAL

SGT-501

Intervention Type DRUG

IV for infusion

Interventions

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SGT-501

IV for infusion

Intervention Type DRUG

Eligibility Criteria

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Inclusion Criteria

Type of Participant and Disease Characteristics:

* Clinical diagnosis of CPVT, based on documented history of polymorphic or bidirectional non-sustained ventricular tachycardia with exercise or ventricular ectopy in a pattern consistent with CPVT on EST.
* Central Screening laboratory determination of a RYR2 variant that is pathogenic or likely pathogenic for CPVT.
* Documented history of life-threatening ventricular arrhythmic event defined as: survived sudden cardiac arrest, sudden cardiac arrest with appropriate implantable cardioverter defibrillator (ICD) shock, arrhythmic syncope, or sustained ventricular tachycardia (30 seconds or more) with or without ICD shock.
* On stable dose (defined as no change in dose by more than 50% for at least 1 month prior to Screening) of standard-of-care therapy defined as a beta-blocker and/or flecainide.
* Documented prior history of EST demonstrating a ventricular arrythmia score (VAS) score of ≥ 2.
* For the first 2 participants in each cohort only: a properly functioning ICD device in place. Following review of data from Cohorts 1 and 2, the Data Safety and Monitoring Board (DSMB) will determine if this criterion is required for participants in Cohort 3.
* Must be up to date with meningococcal vaccination per national guidelines or willing to receive meningococcal vaccine to achieve this.

Exclusion Criteria

* Abnormal liver function: gamma-glutamyl transferase (GGT) \> 1.5 × upper limit of normal \[ULN\] or total bilirubin \> ULN).
* Abnormal renal function defined by estimated glomerular filtration rate \< 60 milliliter /minute (mL/min)/1.73-square meter (m\^2) using Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) Formula.
* Clinically significant abnormalities of coagulation including international normalized ratio or activated partial thromboplastin time \> 1.2 × ULN or platelets \< 150,000 cells/cubic millimeter (mm\^3).
* Potential concomitant cardiomyopathy or inherited arrhythmia as evidenced by pathogenic or likely pathogenic mutation other than RYR2 obtained on cardiac panel during Screening.
* Current or prior treatment with an approved or investigational gene transfer drug.
* Exposure to another investigational drug within 90 days prior to Screening or 5 half-lives since last administration, whichever is longer.
* Contraindication or unwillingness to receive required immunosuppression regimen.
* Body mass index ≥ 30 kilograms per square meter (kg/m\^2).
Minimum Eligible Age

7 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Solid Biosciences Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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Boston Children's Hospital

Boston, Massachusetts, United States

Site Status NOT_YET_RECRUITING

Mayo Clinic

Rochester, Minnesota, United States

Site Status RECRUITING

Cleveland Clinic

Cleveland, Ohio, United States

Site Status NOT_YET_RECRUITING

University of Pennsylvania

Philadelphia, Pennsylvania, United States

Site Status NOT_YET_RECRUITING

St. Paul's Hospital

Vancouver, British Columbia, Canada

Site Status RECRUITING

Countries

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United States Canada

Central Contacts

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Solid Bio Clinical Trials

Role: CONTACT

Phone: 617-337-4680

Email: [email protected]

Facility Contacts

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Kolawole Olarinoye

Role: primary

Brianna Davies

Role: primary

Other Identifiers

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SGT-501-101

Identifier Type: -

Identifier Source: org_study_id