Department of Ultrasound, Shenzhen Children's Hospital, Shenzhen, Guangdong, China.
Shantou University Medical College, Shantou, Guangdong, China.
J Int Med Res. 2022 Sep;50(9):3000605221126876. doi: 10.1177/03000605221126876.
Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) is a rare congenital pulmonary disease that affects newborns. Most patients with ACDMPV are born at full term and are healthy. The main clinical manifestations are refractory pulmonary hypertension and pulmonary failure with gastrointestinal, urinary, or cardiac malformations. ACDMPV often progresses rapidly, but no conventional biological or imaging tests other than genetic testing are available for its diagnosis. Lung biopsy is currently the gold standard for diagnosis. We herein report two cases of ACDMPV confirmed by pathological examination and discuss their ultrasonographic findings.
肺静脉异位引流合并肺动静脉发育不良(ACDMPV)是一种罕见的先天性肺部疾病,主要影响新生儿。大多数 ACDMPV 患者足月出生且健康,主要临床表现为难治性肺动脉高压和肺功能衰竭,常合并胃肠道、泌尿或心脏畸形。ACDMPV 通常进展迅速,但除基因检测外,目前尚无其他常规的生物学或影像学检查方法可用于诊断,肺活检是目前诊断的金标准。本文报道了两例经病理检查证实的 ACDMPV 病例,并讨论了其超声表现。