Prospective cardiovascular magnetic resonance imaging in adults with Alström syndrome: silent progression of diffuse interstitial fibrosis

Table 3 Changes over time in markers

	*No. Patients*	*No. Scans*	Correlation Analysis		GEE Analysis
	*No. Patients*	*No. Scans*	*Rho*	*p-Value*	*Gradient (95% CI)*	*p-Value*
T1 SAX Basal (ms)	30	104	0.415	< 0.001	25.8 (20.0, 31.7)	< 0.001
T1 SAX Mid (ms)	30	104	0.451	< 0.001	21.8 (17.4, 26.1)	< 0.001
T1 SAX Basal ECV (%, pp)	30	94	0.181	0.081	1.4 (0.8, 2)	< 0.001
T1 SAX Mid ECV (%, pp)	30	94	0.192	0.064	1.1 (0.6, 1.6)	< 0.001
LVEDV Index (ml/m²)	30	106	−0.113	0.247	0.04 (−0.60, 0.68)	0.909
LVESV Index (ml/m2)	30	106	−0.164	0.094	0.92 (−0.06, 1.90)	0.067
LVEF (%, pp)	30	106	0.104	0.289	−0.27 (−1.23, 0.68)	0.575
LV Mass Index (g/m²)	30	104	0.257	0.008	2.8 (1.9, 3.7)	< 0.001
LAV Biplane (ml/m²)	29	60	−0.221	0.090	−2.0 (−6.7, 2.7)	0.409
GLS	30	95	0.032	0.756	− 0.04 (− 0.33, 0.26)	0.809
GRS	28	86	0.010	0.924	−0.36 (−1.18, 0.47)	0.394
E/A	29	72	0.133	0.267	2.3 (−2.2, 6.9)	0.313
E/e’ Average	30	68	0.161	0.189	0.3 (−0.2, 0.8)	0.299
HBA1c (mmol/mol)	29	100	− 0.116	0.250	−0.9 (−2.5, 0.7)	0.284
C-Peptide (pmol/L)	30	94	−0.028	0.788	−90.7 (− 364.6, 183.2)	0.516
NTpro BNP (ng/L)	30	105	−0.101	0.306	34.4 (−33.9, 102.8)	0.324

Correlation analysis – results are from Spearman’s rho correlation coefficients between the marker and the timing of the scan, relative to the baseline scan
GEE analysis - results are from generalised estimating equation models, as described in the methods. The “gradient” represents the average yearly change in the marker over the period
Bold p-values are significant at p < 0.05. ECV and EF were measured as percentages, hence gradients represent percentage point (pp) increases
E/A mitral early filling (E)/atrial filling (A), E/e’, mitral early filling (E)/early myocardial relaxation velocity (e’), ECV extracellular volume, HbA1c glycated haemoglobin A1c, LAV left atrial volume, LV left ventricular, LVEDV index, left ventricular end-diastolic volume indexed to body surface area, LVEF left ventricular ejection fraction, LVESV index, left ventricular end-systolic volume indexed, NT-proBNP serum Nterminal pro B natriuretic peptide; SAX, short axis

ISSN: 1750-1172