Device Guidelines for Patients with Genetic Heart Disease: Pacemakers
Permanent Pacemaker (2008 guidelines)
Long QT Syndrome (LQTS)
- Permanent pacing is reasonable for high-risk patients with congenital long-QT syndrome.
- Permanent pacing is not indicated for frequent or complex ventricular ectopic activity without sustained VT in the absence of the long-QT syndrome.
Hypertrophic Obstructive Cardiomyopathy (HoCM)
- Permanent pacing is indicated for SND or AV block in patients with HCM as described previously.
- Permanent pacing may be considered in medically refractory symptomatic patients with HCM and significant resting or provoked LV outflow tract obstruction. As for Class I indications, when risk factors for SCD are present, consider a DDD ICD
- Permanent pacemaker implantation is not indicated for patients who are asymptomatic or whose symptoms are medically controlled.
- Permanent pacemaker implantation is not indicated for symptomatic patients without evidence of LV outflow tract obstruction.
HOCM Guidelines (2011)
- In patients with HCM who have had a dual-chamber device implanted for non-HCM indications, it is reasonable to consider a trial of dual-chamber atrial-ventricular pacing (from the right ventricular apex) for the relief of symptoms attributable to LVOT obstruction.
- Permanent pacing may be considered in medically refractory symptomatic patients with obstructive HCM who are suboptimal candidates for septal reduction therapy.
Class III: No Benefit
- Permanent pacemaker implantation for the purpose of reducing gradient should not be performed in patients with HCM who are asymptomatic or whose symptoms are medically controlled.
- Permanent pacemaker implantation should not be performed as a first-line therapy to relieve symptoms in medically refractory symptomatic patients with HCM and LVOT obstruction who are candidates for septal reduction.
Inherited Primary Arrhythmia Syndromes Guidelines (2013)
Progressive Cardiac Conduction System Disease
- Pacemaker implantation is recommended in patients with a diagnosis of PCCD and the presence of:
- a) Intermittent or permanent third-degree or high-grade AV block or
- b) Symptomatic Mobitz I or II second-degree AV block.
- Pacemaker implantation can be useful in patients with a diagnosis of PCCD and the presence of bifascicular block with or without first-degree AVblock.