Department of Cardiology, Children's Hospital and Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA.
Ultrasound Obstet Gynecol. 2012 Jul;40(1):55-61. doi: 10.1002/uog.10115. Epub 2012 Jun 15.
Neonatal congenital tricuspid valve (TV) dysplasia and/or displacement (Ebstein's malformation) with severe tricuspid regurgitation (TR) is a challenging condition in which outcomes are frequently poor. Little is known about left ventricular (LV) function during the perinatal period in patients with congenital TV disease. The objective of this study was to evaluate LV function in fetuses with congenital TV anomalies associated with significant TR.
Serial fetal echocardiograms in 16 fetuses with congenital TV dysplasia and/or displacement (five neonatal survivors and 11 fetal or neonatal deaths) were reviewed. LV stroke volume, LV end-diastolic volume (LVEDV), LV end-diastolic dimension (LVIDd), the LV eccentricity index, thoracic and cardiac areas and the cardiothoracic area ratio (CTAR), the right atrium area index, and LV longitudinal strains were compared according to gestational age and clinical outcome.
The gestational age-adjusted LVEDV (Z-score) was lower in late gestation (-1.2 ± 1.2 at last examination ≥ 28 weeks) than earlier in gestation (0.3 ± 1.5 at last examination < 28 weeks) and LV output was lower than reported late-gestation normal values. LV short-axis dimension correlated with LV volume and CTAR. LV mid-septal strain was lower than the normal average of fetal mid-septal strain and correlated with the LV eccentricity index. Among these parameters, only the LV eccentricity index differed between survivors and non-survivors.
LV function and anatomy are abnormal in fetuses with severe congenital TV anomalies and may be important contributors to outcome.
新生儿三尖瓣先天性畸形（Ebstein 畸形）伴重度三尖瓣反流（TR）是一种预后较差的挑战性疾病。关于先天性三尖瓣疾病患者围生期左心室（LV）功能的了解甚少。本研究的目的是评估与重度 TR 相关的先天性三尖瓣畸形胎儿的 LV 功能。
对 16 例先天性三尖瓣发育不良和/或移位（5 例新生儿存活和 11 例胎儿或新生儿死亡）的胎儿进行了连续胎儿超声心动图检查。根据胎龄和临床结果比较了 LV 每搏量、LV 舒张末期容积（LVEDV）、LV 舒张末期内径（LVIDd）、LV 偏心指数、胸腔和心脏面积以及心胸面积比（CTAR）、右心房面积指数和 LV 纵向应变。
与早期妊娠（末次检查 < 28 周时为 0.3 ± 1.5）相比，晚期妊娠（末次检查≥28 周时为-1.2 ± 1.2）时 LVEDV（Z 评分）的胎龄调整值较低，LV 输出低于晚期妊娠的正常参考值。LV 短轴直径与 LV 容量和 CTAR 相关。LV 中隔中段应变低于胎儿中隔中段应变的正常平均值，与 LV 偏心指数相关。在这些参数中，只有 LV 偏心指数在幸存者和非幸存者之间存在差异。
严重先天性三尖瓣畸形胎儿的 LV 功能和解剖异常，可能是影响预后的重要因素。
