ACTIONABILITY KNOWLEDGE REPOSITORY ACTIONABILITY CURATION INTERFACE
Update History
Condition: Romano-Ward Long QT Syndrome
Gene/Gene Panel: KCNQ1, KCNH2, SCN5A
Context: Pediatric
Date
Status
Release
Outcomes-Interventions
Notes
2026/01/14
Released
1.0.4
P
Sudden cardiac death (KCNQ1 KCNH2) (GroupA)
Avoidance of QT-prolonging medications (GroupA) 10CN
Beta blockers (GroupA) 11CA
Sudden cardiac death (SCN5A) (GroupB)
Avoidance of QT-prolonging medications (GroupB) 11CN
Beta blockers (GroupB) 10CA
Slight change to the outcome name to include the gene name. This allows for clarity when reading the scoring table.
Assertions
Gene Condition Pairs
Suggested Assertion
Final Assertion
KCNQ1 ⇔ 0100316 LONG QT SYNDROME 1; LQT1
Strong Actionability
Strong Actionability
KCNH2 ⇔ 0013367 LONG QT SYNDROME 2; LQT2
Strong Actionability
Strong Actionability
SCN5A ⇔ 0011377 LONG QT SYNDROME 3; LQT3
Strong Actionability
Strong Actionability
2026/01/14
Released (Under revision)
1.0.3
P
Sudden cardiac death (GroupB)
Avoidance of QT-prolonging medications (GroupB) 11CN
Beta blockers (GroupB) 10CA
Sudden cardiac death (GroupA)
Avoidance of QT-prolonging medications (GroupA) 10CN
Beta blockers (GroupA) 11CA
Internal system migration associated with MONDO name addition.
Assertions
Gene Condition Pairs
Suggested Assertion
Final Assertion
KCNQ1 ⇔ 0100316 LONG QT SYNDROME 1; LQT1
Strong Actionability
Strong Actionability
KCNH2 ⇔ 0013367 LONG QT SYNDROME 2; LQT2
Strong Actionability
Strong Actionability
SCN5A ⇔ 0011377 LONG QT SYNDROME 3; LQT3
Strong Actionability
Strong Actionability
2022/02/09
Released
1.0.3
P
Sudden cardiac death (GroupB)
Avoidance of QT-prolonging medications (GroupB) 11CN
Beta blockers (GroupB) 10CA
Sudden cardiac death (GroupA)
Avoidance of QT-prolonging medications (GroupA) 10CN
Beta blockers (GroupA) 11CA
Internal system migration associated with MONDO name addition.
Assertions
Gene Condition Pairs
Suggested Assertion
Final Assertion
KCNQ1 ⇔ 0100316 LONG QT SYNDROME 1; LQT1
Strong Actionability
Strong Actionability
KCNH2 ⇔ 0013367 LONG QT SYNDROME 2; LQT2
Strong Actionability
Strong Actionability
SCN5A ⇔ 0011377 LONG QT SYNDROME 3; LQT3
Strong Actionability
Strong Actionability
2022/02/09
Released (Under revision)
1.0.2
P
Sudden cardiac death (GroupB)
Avoidance of QT-prolonging medications (GroupB) 11CN
Beta blockers (GroupB) 10CA
Sudden cardiac death (GroupA)
Avoidance of QT-prolonging medications (GroupA) 10CN
Beta blockers (GroupA) 11CA
Obsolete MONDO ID was updated
Assertions
Gene Condition Pairs
Suggested Assertion
Final Assertion
KCNQ1 ⇔ 0100316 LONG QT SYNDROME 1; LQT1
Strong Actionability
Strong Actionability
KCNH2 ⇔ 0013367 LONG QT SYNDROME 2; LQT2
Strong Actionability
Strong Actionability
SCN5A ⇔ 0011377 LONG QT SYNDROME 3; LQT3
Strong Actionability
Strong Actionability
2021/09/16
Released
1.0.2
P
Sudden cardiac death (GroupB)
Avoidance of QT-prolonging medications (GroupB) 11CN
Beta blockers (GroupB) 10CA
Sudden cardiac death (GroupA)
Avoidance of QT-prolonging medications (GroupA) 10CN
Beta blockers (GroupA) 11CA
Obsolete MONDO ID was updated
Assertions
Gene Condition Pairs
Suggested Assertion
Final Assertion
KCNQ1 ⇔ 0100316 LONG QT SYNDROME 1; LQT1
Strong Actionability
Strong Actionability
KCNH2 ⇔ 0013367 LONG QT SYNDROME 2; LQT2
Strong Actionability
Strong Actionability
SCN5A ⇔ 0011377 LONG QT SYNDROME 3; LQT3
Strong Actionability
Strong Actionability
2021/09/16
Released (Under revision)
1.0.1
P
Sudden cardiac death (GroupB)
Avoidance of QT-prolonging medications (GroupB) 11CN
Beta blockers (GroupB) 10CA
Sudden cardiac death (GroupA)
Avoidance of QT-prolonging medications (GroupA) 10CN
Beta blockers (GroupA) 11CA
Assertions
Gene Condition Pairs
Suggested Assertion
Final Assertion
KCNQ1 ⇔ 0008646 LONG QT SYNDROME 1; LQT1
Strong Actionability
Strong Actionability
KCNH2 ⇔ 0013367 LONG QT SYNDROME 2; LQT2
Strong Actionability
Strong Actionability
SCN5A ⇔ 0011377 LONG QT SYNDROME 3; LQT3
Strong Actionability
Strong Actionability
2021/03/29
Released
1.0.1
P
Sudden cardiac death (GroupB)
Avoidance of QT-prolonging medications (GroupB) 11CN
Beta blockers (GroupB) 10CA
Sudden cardiac death (GroupA)
Avoidance of QT-prolonging medications (GroupA) 10CN
Beta blockers (GroupA) 11CA
Assertions
Gene Condition Pairs
Suggested Assertion
Final Assertion
KCNQ1 ⇔ 0008646 LONG QT SYNDROME 1; LQT1
Strong Actionability
Strong Actionability
KCNH2 ⇔ 0013367 LONG QT SYNDROME 2; LQT2
Strong Actionability
Strong Actionability
SCN5A ⇔ 0011377 LONG QT SYNDROME 3; LQT3
Strong Actionability
Strong Actionability
2020/12/16
Released (Under revision)
1.0.0
P
Sudden cardiac death (GroupB)
Avoidance of QT-prolonging medications (GroupB) 11CN
Beta blockers (GroupB) 10CA
Sudden cardiac death (GroupA)
Avoidance of QT-prolonging medications (GroupA) 10CN
Beta blockers (GroupA) 11CA
2020/05/01
Released
1.0.0
P
Sudden cardiac death (GroupB)
Avoidance of QT-prolonging medications (GroupB) 11CN
Beta blockers (GroupB) 10CA
Sudden cardiac death (GroupA)
Avoidance of QT-prolonging medications (GroupA) 10CN
Beta blockers (GroupA) 11CA
2019/12/09
In Preparation
N/A
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