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- Titel:
Differentiating primary sarcomeric hypertrophic cardiomyopathy from Noonan syndrome: can the electrocardiogram be of use?
- Dokumenttyp:
- Journal Article
- Autor(en):
- Hauptmeijer, Robert W L; Lippert, Lea; Ten Cate, Floris E A Udink; Fejzic, Zina; Leenders, Erika; Wolf, Cordula M; Draaisma, Jos M T
- Abstract:
- Noonan syndrome is a multi-system genetic disorder and patients may suffer from hypertrophic cardiomyopathy. Previous studies have identified electrocardiographic features that may support a diagnosis of Noonan syndrome. In this two-centre retrospective study, we analysed typical Noonan syndrome-related electrocardiographic features in 30 patients with Noonan syndrome with hypertrophic cardiomyopathy and compared these with the electrocardiographic features in 15 children with sarcomeric hypertrophic cardiomyopathy. Typical Noonan syndrome-related electrocardiographic features are a negative aVF, small left precordial R-waves, large right precordial S-waves, and abnormal Q-wave. We also analysed electrocardiographic features of hypertrophic cardiomyopathy: ST-segment abnormalities and T-wave abnormalities. A negative aVF was seen in 83% of patients with Noonan syndrome-related hypertrophic cardiomyopathy in contrast to 27% of patients with primary sarcomeric hypertrophic cardiomyopathy (p < 0.001). An extreme QRS axis in the north-west was seen only in patients with Noonan syndrome-related hypertrophic cardiomyopathy. This QRS axis deviation is likely to be determined by the Noonan syndrome-related hypertrophic cardiomyopathy and not by the type of hypertrophic cardiomyopathy. There were no differences between the two groups in the frequency of large right precordial S-waves and small R-waves in the left precordial leads V5 and V6. However, an abnormal R/S ratio was more often seen in patients with Noonan syndrome-related hypertrophic cardiomyopathy (p < 0.001). Pathologic Q-waves were seen statistically more frequently in patients with sarcomeric hypertrophic cardiomyopathy (p = 0.009). The occurrence of ST-segment and T-wave pathology did not statistically differ between the two groups. Electrography can be of use in differentiating sarcomeric hypertrophic cardiomyopathy from Noonan syndrome-related hypertrophic cardiomyopathy.
- Zeitschriftentitel:
- Cardiol Young
- Jahr:
- 2024
- Band / Volume:
- 34
- Heft / Issue:
- 3
- Seitenangaben Beitrag:
- 597-603
- Volltext / DOI:
- doi:10.1017/S1047951123003177
- PubMed:
- http://view.ncbi.nlm.nih.gov/pubmed/37649442
- Print-ISSN:
- 1047-9511
- TUM Einrichtung:
- Klinik für Kinderkardiologie und angeborene Herzfehler (DHM) (Prof. Ewert)
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