Cardiac rhythm abnormalities - An underestimated cardiovascular risk in adult patients with Mucopolysaccharidoses.
Mol Genet Metab
; 130(2): 133-139, 2020 06.
Article
in En
| MEDLINE
| ID: mdl-32241717
ABSTRACT
Patients with Mucopolysaccharidosis (MPS) have an increased risk of cardiovascular complications, conduction tissue abnormalities and arrhythmia; all rare but underestimated. It has been reported that conduction system defects are progressive in this group of patients and may result in sudden cardiac death. The aim of this study is to review our current practice and suggest best practice guidelines regarding the frequency of cardiac rhythm monitoring in this patient group. Seventy-seven adult MPS patients who attended metabolic clinics between 2013 and 2019 were included in this retrospective observational study. Patients were affected with different MPS types MPS I (n = 33), MPS II (n = 16), MPS IV (n = 19), VI (n = 8) and VII (n = 1). The assessments included 12lead electrocardiogram (ECG), 24-h ECG (Holter monitor), loop recorder/pacemaker interrogation assessment. Data from 12lead ECG (available from 69 patients) showed a variety of abnormalities T wave inversion in a single lead III (n = 19), left ventricular hypertrophy (n = 14), early repolarization (n = 14), right axis deviation (RAD, n = 11), partial RBBB (n = 9), right bundle branch block (RBBB) (n = 1) and first degree AV block (n = 1). ECG changes of bundle branch block, RAD (left posterior fascicular block) could represent conduction tissue abnormality and equally could be related to the underlying lung tissue abnormality which is present in most of the patients with MPS. T wave abnormality in a single lead is usually insignificant in healthy individuals; however in MPS patients it could be as a result of chest shape. Among the 34 patients for who 24-hour ECG was available, sinus tachycardia was the most common rhythm noted (n = 9), followed by sinus bradycardia (n = 4), atrial fibrillation (AF) (n = 1) and atrio-ventricular nodal re-entry tachycardia (AVNRT) (n = 1). Permanent pacemaker was inserted in two patients. AF was observed in one patient with MPS II. In conclusion, we postulate that regular cardiac monitoring is required to warrant early detection of underlying conduction tissue abnormalities. In addition, 12lead ECG is the first line investigation that, if abnormal, should be followed up by 24-hour Holter monitoring. These findings warrant further research studies.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Cardiovascular Diseases
/
Mucopolysaccharidoses
Type of study:
Etiology_studies
/
Guideline
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
/
Screening_studies
Limits:
Adolescent
/
Adult
/
Aged
/
Female
/
Humans
/
Male
/
Middle aged
Language:
En
Journal:
Mol Genet Metab
Journal subject:
BIOLOGIA MOLECULAR
/
BIOQUIMICA
/
METABOLISMO
Year:
2020
Document type:
Article
Affiliation country:
Reino Unido