Left Ventricular Noncompaction in Childhood: Echocardiographic Follow-Up and Prevalence in First-Degree Relatives
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Left Ventricular Noncompaction in Childhood : Echocardiographic Follow-Up and Prevalence in First-Degree Relatives. / Kock, Thilde O.; Børresen, Marie F.; Sillesen, Anne Sophie; Vøgg, Ruth O.B.; Norsk, Jakob B.; Pærregaard, Maria M.; Vejlstrup, Niels G.; Christensen, Alex H.; Iversen, Kasper K.; Bundgaard, Henning; Axelsson Raja, Anna.
In: JACC: Advances, Vol. 3, No. 3, 100829, 2024.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - Left Ventricular Noncompaction in Childhood
T2 - Echocardiographic Follow-Up and Prevalence in First-Degree Relatives
AU - Kock, Thilde O.
AU - Børresen, Marie F.
AU - Sillesen, Anne Sophie
AU - Vøgg, Ruth O.B.
AU - Norsk, Jakob B.
AU - Pærregaard, Maria M.
AU - Vejlstrup, Niels G.
AU - Christensen, Alex H.
AU - Iversen, Kasper K.
AU - Bundgaard, Henning
AU - Axelsson Raja, Anna
N1 - Publisher Copyright: © 2024 The Authors
PY - 2024
Y1 - 2024
N2 - Background: Left ventricular noncompaction (LVNC) is characterized by excessive trabeculations of the left ventricular (LV) wall. Objectives: The authors aimed to examine changes in LV function and morphology in 2 to 4-year-old children with and without LVNC at birth and to describe the prevalence of LVNC in first-degree relatives. Methods: Echocardiograms in children with and without LVNC (matched 1:4) were performed at 2 to 4 years and in first-degree relatives. LVNC was blindly assessed and defined as a ratio of non-compact to compact myocardium of ≥2 in ≥1 LV segment. Trabeculations were expressed as a percentage of the number of segments with LVNC out of the total number of segments. Results: In total, 14 (median age 3 years, 71% male) of 16 children with LVNC at birth and 56 children without (median age 4 years, 71% male), 37 first-degree relatives of children with LVNC (median age 31 years, 46% male) and 146 first-degree relatives of children without (median age 33 years, 50% male) were included. In children with LVNC, trabeculation (8% vs 13%, P = 0.81) and LV ejection fraction (50% vs 49%, P = 0.91) were unchanged from birth to follow-up but LV ejection fraction was lower compared to children without LVNC (49% vs 60%, P < 0.001). In relatives of children with LVNC, 11 of 37 (30%) fulfilled LVNC criteria compared to no relatives to children without LVNC (P < 0.001). Conclusions: At 2 to 4 years, children with LVNC diagnosed at birth had reduced systolic function compared to children without but did not have progression of LV dysfunction or extent of trabeculations. In first-degree relatives to children with LVNC, 30% fulfilled criteria.
AB - Background: Left ventricular noncompaction (LVNC) is characterized by excessive trabeculations of the left ventricular (LV) wall. Objectives: The authors aimed to examine changes in LV function and morphology in 2 to 4-year-old children with and without LVNC at birth and to describe the prevalence of LVNC in first-degree relatives. Methods: Echocardiograms in children with and without LVNC (matched 1:4) were performed at 2 to 4 years and in first-degree relatives. LVNC was blindly assessed and defined as a ratio of non-compact to compact myocardium of ≥2 in ≥1 LV segment. Trabeculations were expressed as a percentage of the number of segments with LVNC out of the total number of segments. Results: In total, 14 (median age 3 years, 71% male) of 16 children with LVNC at birth and 56 children without (median age 4 years, 71% male), 37 first-degree relatives of children with LVNC (median age 31 years, 46% male) and 146 first-degree relatives of children without (median age 33 years, 50% male) were included. In children with LVNC, trabeculation (8% vs 13%, P = 0.81) and LV ejection fraction (50% vs 49%, P = 0.91) were unchanged from birth to follow-up but LV ejection fraction was lower compared to children without LVNC (49% vs 60%, P < 0.001). In relatives of children with LVNC, 11 of 37 (30%) fulfilled LVNC criteria compared to no relatives to children without LVNC (P < 0.001). Conclusions: At 2 to 4 years, children with LVNC diagnosed at birth had reduced systolic function compared to children without but did not have progression of LV dysfunction or extent of trabeculations. In first-degree relatives to children with LVNC, 30% fulfilled criteria.
KW - cardiomyopathy
KW - child
KW - echocardiography
KW - myocardium
KW - trabeculation
U2 - 10.1016/j.jacadv.2024.100829
DO - 10.1016/j.jacadv.2024.100829
M3 - Journal article
AN - SCOPUS:85184783781
VL - 3
JO - JACC: Advances
JF - JACC: Advances
SN - 2772-963X
IS - 3
M1 - 100829
ER -
ID: 384570777