Electrocardiographic Changes with Age in Japanese Patients with Noonan Syndrome

Author:

Ichikawa Yasuhiro12,Kuroda Hiroyuki1ORCID,Ikegawa Takeshi1,Kawai Shun12,Ono Shin1,Kim Ki-Sung1,Yanagi Sadamitsu1,Kurosawa Kenji3,Aoki Yoko4,Iwamoto Mari5,Ueda Hideaki1

Affiliation:

1. Department of Cardiology, Kanagawa Children’s Medical Center, 2-138-4 Mutsukawa, Minami-ku, Yokohama 232-8555, Kanagawa, Japan

2. Department of Pediatric Cardiology, Yokohama City University Hospital, 3-9 Fukuura, Kanazawa-ku, Yokohama 236-0004, Kanagawa, Japan

3. Division of Medical Genetics, Kanagawa Children’s Medical Center, 2-138-4 Mutsukawa, Minami-ku, Yokohama 232-8555, Kanagawa, Japan

4. Department of Medical Genetics, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980-8574, Miyagi, Japan

5. Department of Pediatrics, Saiseikai Yokohama-shi Tobu Hospital, 3-6-1 Shimosueyoshi, Tsurumi-ku, Yokohama 230-8765, Kanagawa, Japan

Abstract

Little information is available on age-related electrocardiographic changes in patients with Noonan syndrome. This single-center study evaluated the electrocardiograms of patients with Noonan syndrome. We divided the patients (n = 112; electrocardiograms, 256) into four groups according to age: G1 (1 month–1 year), G2 (1–6 years), G3 (6–12 years), and G4 (>12 years). Typical Noonan syndrome-related electrocardiographic features such as left-axis deviation, abnormal Q wave, wide QRS complex, and small R wave in precordial leads were detected. A high percentage of QRS axis abnormalities was found in all groups. Significant differences in right-axis deviation (RAD) were noted among the groups: 56.5% of G1 patients showed RAD compared with 33.3% of G2, 21.1% of G3, and 19.2% of G4 patients. The small R was also significantly different among the groups: 32.6% of G1 patients showed a small R wave compared with 14.9% of G2, 8.5% of G3, and 15.4% of G4 patients. Of the 53 patients with Noonan syndrome aged 1 month to 2 years, 18 had T-positive V1 with a higher prevalence of pulmonary stenosis and cardiac interventions. QRS axis abnormalities, small R in V6, and T-positive V1 could help diagnose Noonan syndrome in infants or young children.

Funder

AMED

Publisher

MDPI AG

Subject

Pharmacology (medical),General Pharmacology, Toxicology and Pharmaceutics

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