Clinical Guidance

LMNA-related dilated cardiomyopathy (DCM) is characterized by left ventricular enlargement and/or reduced systolic function frequently preceded or accompanied by significant conduction system disease. Age of onset. While LMNA-related DCM usually presents in adulthood either with conduction system disease commonly accompanied by arrhythmias or with symptomatic DCM (including heart failure or embolus from a left ventricular mural thrombus), it may also be present in asymptomatic individuals: conduction system disease, arrhythmia, or DCM may be discovered during a medical evaluation conducted for another reason (e.g., a routine preoperative EKG) or clinical screening of at-risk relatives . Presenting signs, timing, and progression.

Diagnostic Criteria

Suggestive Findings

LMNA-related dilated cardiomyopathy (DCM) should be considered in individuals with the following clinical findings and family history.

Clinical findings

Source: GeneReviews — "LMNA-Related Dilated Cardiomyopathy"

Management Guidelines

Guidelines for the clinical evaluation and surveillance of individuals with LMNA-related DCM and other genetic cardiomyopathies have been published . Additional guidance regarding the management of arrhythmic disease in LMNA-related DCM is also available . Evaluations Following Initial Diagnosis To establish the extent of disease and needs in an individual diagnosed with LMNA-related DCM, the evaluations summarized (if not performed as part of the evaluation that led to the diagnosis) are recommended. Table 3. Recommended Evaluations Following Initial Diagnosis in Individuals with LMNA-Related DCM

Surveillance Guidelines

Table 5.

Recommended Surveillance for Individuals with LMNA-related DCM

System/Concern | Evaluation | Frequency

LMNA variant w/any EKG abnormality

| Cardiovascular eval for disease progression w/EKG, 24-48 hr rhythm monitoring, LV function measurement | Annually (at a minimum)

LMNA variant in an asymptomatic person