Jivanji Salim G M, Daubeney Piers, Franklin Rodney, Sheppard Mary
Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
Department of Histopathology, Royal Brompton & Harefield NHS Foundation Trust, London, UK.
BMJ Case Rep. 2014 Oct 21;2014:bcr2014205041. doi: 10.1136/bcr-2014-205041.
A newborn presenting with cyanosis at day 9 of life was admitted to the local hospital. Initial local echocardiography confirmed a cardiac issue and the patient was transferred to a tertiary cardiac hospital. Further imaging confirmed a rare presentation of cardiomyopathy with severe right ventricular outflow tract obstruction. Surgery was performed but with postoperative haemodynamic instability complicated by incessant ventricular tachycardia. Following discussion with the family, care was withdrawn. Postmortem demonstrated a rare form of hypertrophic cardiomyopathy with right ventricular outflow tract obstruction not previously described in a neonate.
一名出生9天出现青紫的新生儿被收治于当地医院。初步的当地超声心动图检查证实存在心脏问题，该患者被转至一家三级心脏专科医院。进一步的影像学检查证实为一种罕见的心肌病表现，伴有严重的右心室流出道梗阻。进行了手术，但术后出现血流动力学不稳定，并伴有持续性室性心动过速。在与家属讨论后，停止了治疗。尸检显示为一种罕见的肥厚型心肌病，伴有右心室流出道梗阻，此前在新生儿中未曾有过描述。
