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Table 4 Baseline Clinical Assessments in the NHS Cohort. Results of vitals, physical exam, pulmonary function test, chest wall deformity and scoliosis assessments (present/absent) based on ECG classification. Chest wall deformity and scoliosis percentages represent fraction of subgroup with chest wall deformity or scoliosis present. Subjects with conduction abnormalities were significantly less likely to have chest wall deformities (*p < 0.05)

From: Patients with ACVR1R206H mutations have an increased prevalence of cardiac conduction abnormalities on electrocardiogram in a natural history study of Fibrodysplasia Ossificans Progressiva

  Normal Abnormal Conduction Abnormality
Vitals
 Diastolic Blood Pressure (mmHg) 68 ± 10 (N = 43) 67 ± 10 (N = 57) 64 ± 9 (N = 45)
 Systolic Blood Pressure (mmHg) 110 ± 14 (N = 43) 110 ± 13 (N = 57) 107 ± 12 (N = 45)
 Height (cm) 153.9 ± 17.9 (N = 43) 154.0 ± 20.9 (N = 58) 150.4 ± 22.0 (N = 45)
 Heart Rate (beats/min) 88 ± 13 (N = 44) 87 ± 15 (N = 62) 88 ± 14 (N = 48)
 Knee to Heel (cm) 46.6 ± 7.6 (N = 21) 46.0 ± 7.6 (N = 39) 45.7 ± 7.8 (N = 36)
 Respiratory Rate (breaths/min) 20 ± 4 (N = 44) 21 ± 6 (N = 61) 22 ± 6 (N = 48)
 Temperature (C) 36.7 ± 0.4 (N = 41) 36.5 ± 0.5 (N = 60) 36.5 ± 0.6 (N = 46)
 Weight (kg) 50.0 ± 17.7 (N = 44) 50.7 ± 25.2 (N = 60) 49.1 ± 26.0 (N = 47)
Pulmonary Function Tests (PFTs)
 FEV1 (L) 1.66 ± 0.73 (N = 40) 1.69 ± 0.74 (N = 57) 1.78 ± 0.79 (N = 43)
 FVC (L) 1.85 ± 0.88 (N = 40) 1.86 ± 0.83 (N = 57) 1.96 ± 0.89 (N = 43)
 FEV1/FVC (%) 90.7 ± 7.4 (N = 40) 91.7 ± 8.9 (N = 57) 92.0 ± 9.4 (N = 45)
 O2 Saturation (%) 98 ± 1 (N = 43) 98 ± 2 (N = 59) 98 ± 2 (N = 43)
 FEV1 (% predicted) 56 ± 17 (N = 40) 56 ± 18 (N = 57) 61 ± 17 (N = 43)
 FVC (% predicted) 53 ± 17 (N = 40) 54 ± 18 (N = 57) 59 ± 17 (N = 43)
 FEV1/FVC (% predicted) 105 ± 9 (N = 40) 105 ± 11 (N = 57) 104 ± 10 (N = 45)
 Presence of Chest Wall Deformity (%) 67.4% (29/43) 56.1% (32/57) 48.9% (22/45)
 Presence of Scoliosis (%) 62.8% (27/43) 59.6% (34/57) 55.6% (25/45)
  1. FEV1 Forced Expiratory Volume in 1 s, FVC Forced Vital Capacity