PDF(Pubmed)
Abstract：
UNASSIGNED: Massive tricuspid regurgitation (TR) is the most common feature of pulmonary atresia with intact ventricular septum (PA/IVS), and mild or absent TR is observed in severe right ventricular (RV) dysplasia or RV-to-coronary fistulous connections, resulting in non-biventricular (BV) outcomes postnatally.
UNASSIGNED: We report a case of fetal severe pulmonary stenosis with IVS diagnosed at 26 weeks of gestation. The severity of RV hypoplasia did not worsen or reach indications for intrauterine intervention, while the jet velocity of TR decreased significantly during pregnancy. The fetus was definitely diagnosed with PA/IVS with mild RV dysplasia after birth. Unusually, the fetus did not experience severe TR and myocardial sinusoids, the TR jet velocity was maintained at 2.0 m/s, and the coronary artery was almost normal. The incapable RV cannot pump blood into pulmonary circulation after RV decompression from valvular perforation and balloon dilation. It may be an extraordinary finding of subsystemic RV.
UNASSIGNED: PA/IVS is a heterogeneous disease with various degrees of RV dysplasia. Mild or no baseline TR is a reliable indicator with non-BV outcomes for fetal PA/IVS, even with acceptable dysplasia RV structures.
UNASSIGNED: We report a case of fetal severe pulmonary stenosis with IVS diagnosed at 26 weeks of gestation. The severity of RV hypoplasia did not worsen or reach indications for intrauterine intervention, while the jet velocity of TR decreased significantly during pregnancy. The fetus was definitely diagnosed with PA/IVS with mild RV dysplasia after birth. Unusually, the fetus did not experience severe TR and myocardial sinusoids, the TR jet velocity was maintained at 2.0 m/s, and the coronary artery was almost normal. The incapable RV cannot pump blood into pulmonary circulation after RV decompression from valvular perforation and balloon dilation. It may be an extraordinary finding of subsystemic RV.
UNASSIGNED: PA/IVS is a heterogeneous disease with various degrees of RV dysplasia. Mild or no baseline TR is a reliable indicator with non-BV outcomes for fetal PA/IVS, even with acceptable dysplasia RV structures.
摘要：
■大量三尖瓣反流（TR）是室间隔完整的肺动脉闭锁（PA/IVS）的最常见特征，在严重的右心室（RV）发育不良或RV-冠状动脉瘘连接中观察到轻度或无TR，导致出生后非双心室（BV）结局。
■我们报告一例在妊娠26周时诊断为IVS的胎儿重度肺动脉狭窄。右心室发育不全的严重程度并未恶化或达到宫内介入的指征，而TR的喷射速度在怀孕期间显着降低。胎儿出生后明确诊断为PA/IVS伴轻度RV发育不良。通常情况下，胎儿没有经历严重的TR和心肌窦，TR射流速度保持在2.0m/s，冠状动脉几乎正常.在RV从瓣膜穿孔和球囊扩张减压后，无能力的RV无法将血液泵入肺循环。这可能是亚系统RV的非凡发现。
■PA/IVS是一种异质性疾病，具有不同程度的RV发育不良。轻度或无基线TR是胎儿PA/IVS非BV结局的可靠指标，即使有可接受的发育不良RV结构。
■我们报告一例在妊娠26周时诊断为IVS的胎儿重度肺动脉狭窄。右心室发育不全的严重程度并未恶化或达到宫内介入的指征，而TR的喷射速度在怀孕期间显着降低。胎儿出生后明确诊断为PA/IVS伴轻度RV发育不良。通常情况下，胎儿没有经历严重的TR和心肌窦，TR射流速度保持在2.0m/s，冠状动脉几乎正常.在RV从瓣膜穿孔和球囊扩张减压后，无能力的RV无法将血液泵入肺循环。这可能是亚系统RV的非凡发现。
■PA/IVS是一种异质性疾病，具有不同程度的RV发育不良。轻度或无基线TR是胎儿PA/IVS非BV结局的可靠指标，即使有可接受的发育不良RV结构。
