Disease · MONDO_0019171

familial long QT syndrome

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. familial long QT syndrome. Diseases, record MONDO_0019171. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0019171

On this page

Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: familial long QT syndrome; Open Targets
Open Targets ID: MONDO_0019171; Open Targets
Other names: LQTS, Prolonged QT interval on EKG, Romano-Ward long QT syndrome, Romano-Ward syndrome, Ward-Romano syndrome, congenital long QT syndrome, hereditary long QT syndrome, long QT syndrome; Open Targets
Orphanet: ORPHA:101016; Orphanet
Orphanet: ORPHA:768; Orphanet
Highest stage of its drugs: Phase 3, for 1 of its 10 drugs, from TTD; Open Targets

Drugs10

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
ELECLAZINE	CHEMBL3707392	Small molecule	Phase 3	TTD	1	0	0	Drugs and targets
LUMACAFTOR	CHEMBL2103870	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
RANOLAZINE	CHEMBL1404	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
BEPOTASTINE BESYLATE	CHEMBL1201759	Small molecule	Unknown	ClinicalTrials.gov	1	1	0	Drugs and targets
BISOPROLOL	CHEMBL645	Small molecule	Unknown	ClinicalTrials.gov	1	1	0	Drugs and targets
CARVEDILOL	CHEMBL723	Small molecule	Unknown	ClinicalTrials.gov	1	1	0	Drugs and targets
METOPROLOL	CHEMBL13	Small molecule	Unknown	ClinicalTrials.gov	1	1	0	Drugs and targets
PROPOFOL	CHEMBL526	Small molecule	Unknown	ClinicalTrials.gov	1	1	1	Drugs and targets
PROPRANOLOL	CHEMBL27	Small molecule	Unknown	ClinicalTrials.gov	1	1	0	Drugs and targets
SPIRONOLACTONE	CHEMBL1393	Small molecule	Unknown	ClinicalTrials.gov	1	1	1	Drugs and targets

Targets27

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
ADRB1	Adrenergic receptor beta	4	Adrenergic receptor beta antagonist; Beta-1 adrenergic receptor antagonist	Unknown	https://conductscience.org/library/genes/153	Drugs and targets
ADRB2	Adrenergic receptor beta	2	Adrenergic receptor beta antagonist; Beta-2 adrenergic receptor antagonist	Unknown	https://conductscience.org/library/genes/154	Drugs and targets
ADRA1A	Adrenergic receptor alpha-1	1	Adrenergic receptor alpha-1 antagonist	Unknown	https://conductscience.org/library/genes/148	Drugs and targets
ADRA1B	Adrenergic receptor alpha-1	1	Adrenergic receptor alpha-1 antagonist	Unknown	https://conductscience.org/library/genes/147	Drugs and targets
ADRA1D	Adrenergic receptor alpha-1	1	Adrenergic receptor alpha-1 antagonist	Unknown	https://conductscience.org/library/genes/146	Drugs and targets
ADRB3	Adrenergic receptor beta	1	Adrenergic receptor beta antagonist	Unknown	https://conductscience.org/library/genes/155	Drugs and targets
CFTR	Cystic fibrosis transmembrane conductance regulator	1	Cystic fibrosis transmembrane conductance regulator stabiliser	Phase 2	https://conductscience.org/library/genes/1080	Drugs and targets
GABRA1	GABA-A receptor; anion channel	1	GABA-A receptor; anion channel positive allosteric modulator	Unknown	https://conductscience.org/library/genes/2554	Drugs and targets
GABRA2	GABA-A receptor; anion channel	1	GABA-A receptor; anion channel positive allosteric modulator	Unknown	https://conductscience.org/library/genes/2555	Drugs and targets
GABRA3	GABA-A receptor; anion channel	1	GABA-A receptor; anion channel positive allosteric modulator	Unknown	https://conductscience.org/library/genes/2556	Drugs and targets

1 to 10 of 27

See all 27 targets

Trials4

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT01648205	Long-term Efficacy Study of Sodium Channel Blocker in LQT3 Patients	Phase 2	25	2022-04-07	Long QT Syndrome	RANOLAZINE	Its record names the disease	ClinicalTrials.gov
NCT00358618	The Effects of the Anesthetic Drug Propofol on the Way the Heart Recovers Between Beats	Not applicable	60		Myocardial Repolarization	PROPOFOL	Its record names the disease	ClinicalTrials.gov
NCT03291145	Exploring Mechanisms and Morphology of QT Interval Prolongation	Not applicable	28		Long Qt Syndrome 1-2, Sudden Cardiac Death	SPIRONOLACTONE	Its record names the disease	ClinicalTrials.gov
NCT04581408	Mutation-specific Therapy for the Long QT Syndrome	Phase 2	16		Long QT Syndrome	LUMACAFTOR	Its record names the disease	ClinicalTrials.gov

Comparisons in its trials2

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

Comparison	Group 1, with the outcome	Group 2, with the outcome	Participants	Trial	Source
Ranolazine vs Placebo: deaths from any cause	1 of 25	0 of 25	50	NCT01648205	Trial comparisons
Ranolazine vs Placebo: serious adverse events	2 of 25	0 of 25	50	NCT01648205	Trial comparisons

Papers from its trials2

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
34531279	DERIVED	1	PubMed
32882399	DERIVED	1	PubMed

Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0019171

GET https://conductscience.org/api/diseases/v1/MONDO_0019171/targets?page=2

API reference For AI assistants