PDF(Pubmed)
Abstract：
UNASSIGNED: Left ventricular noncompaction (LVNC) is characterized by excessive trabeculations of the left ventricular (LV) wall.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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).
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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).
UNASSIGNED: 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.
摘要：
■左心室致密化不全（LVNC）的特征是左心室（LV）壁的过度小梁。
■作者旨在研究出生时2至4岁有和无LVNC儿童的LV功能和形态的变化，并描述一级亲属中LVNC的患病率。
■有和没有LVNC（匹配1：4）的儿童在2至4岁以及一级亲属中进行了超声心动图检查。对LVNC进行了盲目评估，并将其定义为在≥1个LV节段中≥2的非致密心肌与致密心肌的比率。小梁表示为具有LVNC的节段的数量占节段总数的百分比。
■总共，14(中位年龄3岁，71％男性）出生时患有LVNC的16名儿童和无LVNC的56名儿童（中位年龄4岁，71%男性），37名LVNC儿童的一级亲属(平均年龄31岁，46％的男性）和146名无儿童的一级亲属（中位年龄33岁，包括50％的男性）。在患有LVNC的儿童中，小梁(8%对13%，P=0.81）和左心室射血分数（50%vs49%，P=0.91）从出生到随访没有变化，但LV射血分数低于无LVNC的儿童（49%vs60%，P<0.001)。在LVNC儿童的亲属中，与无亲属的无LVNC儿童相比，37例中的11例（30％）符合LVNC标准（P<0.001）。
■在2到4岁时，出生时诊断为LVNC的儿童与无LV功能障碍或小梁形成程度的儿童相比,收缩功能降低.在LVNC儿童的一级亲属中，30%符合标准。
■作者旨在研究出生时2至4岁有和无LVNC儿童的LV功能和形态的变化，并描述一级亲属中LVNC的患病率。
■有和没有LVNC（匹配1：4）的儿童在2至4岁以及一级亲属中进行了超声心动图检查。对LVNC进行了盲目评估，并将其定义为在≥1个LV节段中≥2的非致密心肌与致密心肌的比率。小梁表示为具有LVNC的节段的数量占节段总数的百分比。
■总共，14(中位年龄3岁，71％男性）出生时患有LVNC的16名儿童和无LVNC的56名儿童（中位年龄4岁，71%男性），37名LVNC儿童的一级亲属(平均年龄31岁，46％的男性）和146名无儿童的一级亲属（中位年龄33岁，包括50％的男性）。在患有LVNC的儿童中，小梁(8%对13%，P=0.81）和左心室射血分数（50%vs49%，P=0.91）从出生到随访没有变化，但LV射血分数低于无LVNC的儿童（49%vs60%，P<0.001)。在LVNC儿童的亲属中，与无亲属的无LVNC儿童相比，37例中的11例（30％）符合LVNC标准（P<0.001）。
■在2到4岁时，出生时诊断为LVNC的儿童与无LV功能障碍或小梁形成程度的儿童相比,收缩功能降低.在LVNC儿童的一级亲属中，30%符合标准。
