Table 3 Differences between etiologies of cardiac hypertrophy associated versus not associated with hyperinsulinism.
Etiologies of cardiac hypertrophy associated with hyperinsulinism (n = 22, 31%)* | Etiologies of cardiac hypertrophy not associated with hyperinsulinism (n = 25, 35%) | p value | |
---|---|---|---|
Echocardiography | |||
Age of first echocardiography with hypertrophy (days/months) | 2d (1d-1.8m) | 4.4m (12d-7.7m) | < 0.01 |
Interventricular septum thickness | |||
mm | 7.3 (6.2–8.1) | 8.7 (6.1–10.9) | 0.24 |
Z-score | + 2.9 (2.4–3.6) | + 3.4 (2.1–4.6) | 0.45 |
Left ventricular posterior wall thickness | |||
mm | 4.9 (3.6–5.5) | 6.4 (5.4–8.1) | < 0.01 |
Z-score | + 2.4 (1.0–3.0) | + 3.3 (2.9–4.5) | < 0.01 |
Distribution left ventricular hypertrophy | |||
Z-score ≥ 2.0 [n (%)] | |||
Interventricular septum | 8 (36) | 1 (4) | 0.01 |
Left ventricular posterior wall | 3 (14) | 3 (12) | 1.00 |
Interventricular septum and left ventricular posterior wall | 11 (50) | 21 (84) | 0.03 |
Ratio interventricular septum:left ventricular posterior wall | 1.5 (1.3–2.0) | 1.2 (1.1–1.5) | 0.02 |
Asymmetrical septal hypertrophy [n (%)] | 13 (59) | 8 (32) | 0.08 |
Left ventricular outflow tract obstruction [n (%)] | 3 (14) | 7 (28) | 0.30 |
Left ventricular function | |||
Decreased left ventricular systolic function [n (%)] | 5 (23) | 10 (40) | 0.23 |
Symptomatic [n (%)] | 10 (46) | 11 (44) | 1.00 |
Etiology [n (%)] | |||
Maternal diabetes mellitus | 13 (59) | 0 (0) | |
Malformation syndromes | 6 (27)* | 17 (68) | |
Sarcomeric disease | 0 (0) | 2 (8) | |
Metabolic disease | 0 (0) | 5 (20) | |
Congenital hyperinsulinism | 3 (14) | 0 (0) | |
Neuromuscular disease | 0 (0) | 1 (4) | |
Prognosis | |||
Cardiac hypertrophy normalized [n (%)] | 13 (59) | 7 (28) | 0.01 |
Age cardiac hypertrophy normalized (days/months/years) | 102 (52–603) | 904 (602–1830) | < 0.01 |
Cardiac hypertrophy normalized in first year [n (%)] | 9 (41) | 0 (0) | 0.01 |
All cause mortality [n (%)] | 3 (14) | 11 (44) | 0.03 |
Age of death (days/months) | 65 (35–73) | 138 (66–249) | 0.09 |
Cardiac hypertrophy cause of death [n (%)] | 1 (33) | 7 (64) | 0.54 |