Venkateshwaran Subramanian, Krishnamoorthy Kavassery Mahadevan, Sivasankaran Sivasubramonian
Department of Cardiology, Sreechitra Tirunal Institute For Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Catheter Cardiovasc Interv. 2014 May 1;83(6):968-70. doi: 10.1002/ccd.25275. Epub 2013 Nov 19.
An eight-year-old girl was evaluated for unexplained cyanosis. A contrast echo was suggestive of pulmonary arteriovenous fistula. Further evaluation revealed Abernethy malformation (type 2) leading to hepatopulmonary syndrome. Percutaneous device closure of Abernethy malformation was done after anatomical and physiological evaluation of the portal circulation. Prior to closure, it is important to ensure adequate portal radicals into the liver and normal portal pressure after test balloon occlusion. Subcostal echocardiography can diagnose and guide device closure of Abernethy malformation, a treatable cause of hepatopulmonary syndrome.
一名8岁女孩因不明原因的发绀接受评估。造影超声心动图提示存在肺动静脉瘘。进一步评估发现艾伯内西畸形（2型）导致肝肺综合征。在对门静脉循环进行解剖和生理评估后，对艾伯内西畸形进行了经皮装置封堵。在封堵之前，重要的是要确保有足够的门静脉分支进入肝脏，并且在试验性球囊闭塞后门静脉压力正常。肋下超声心动图可以诊断并指导对艾伯内西畸形进行装置封堵，艾伯内西畸形是肝肺综合征的一个可治疗病因。
