Schaefer Andreas, Sill Bjoern, Schoenebeck Jeannette, Schneeberger Yvonne, Reichenspurner Hermann, Gulbins Helmut
Department of Cardiovascular Surgery, University Heart Center Hamburg, Martinistraße 52, 20246, Hamburg, Germany.
J Cardiothorac Surg. 2015 Mar 27;10:41. doi: 10.1186/s13019-015-0238-5.
Carcinoid tumor with consecutive endocardial fibroelastosis of the right heart, known as carcinoid heart valve disease (CHVD) or Hedinger's syndrome, is accompanied by combined right-sided valvular dysfunction with regurgitation and stenosis of the affected valves. Cardiac surgery with replacement of the tricuspid and/or pulmonary valve is an established therapeutic option for patients with Hedinger's syndrome. Little is known about the long term outcome and the choice of prosthesis for the pulmonal position is still a matter of debate.
The authors report three cases of pulmonary valve replacement with stentless bioprostheses (Medtronic Freestyle, Medtronic PLC, Minneapolis, MN, USA) due to severe pulmonary valve degeneration in consequence of Hedinger's syndrome.
All patients presented with re-stenosis of the pulmonal valve conduit at the height of the anastomoses in a premature fashion. Due to the increased risk for repeat surgical valve replacement, two patients were treated by transcatheter heart valves.
We do not recommend the replacement of the pulmonary valve with stentless bioprostheses in patients with CHVD. These valves presented with an extreme premature degeneration and consecutive re-stenosis and heart failure.
类癌瘤伴右心连续性心内膜纤维弹性组织增生，即类癌性心脏瓣膜病（CHVD）或黑丁格综合征，伴有受累瓣膜反流和狭窄的联合右侧瓣膜功能障碍。对于黑丁格综合征患者，行三尖瓣和/或肺动脉瓣置换的心脏手术是一种既定的治疗选择。关于长期预后知之甚少，肺动脉位置人工瓣膜的选择仍存在争议。
作者报告了3例因黑丁格综合征导致严重肺动脉瓣退变而行无支架生物瓣膜（美敦力Freestyle，美敦力公司，美国明尼阿波利斯）置换肺动脉瓣的病例。
所有患者均过早出现肺动脉瓣管道吻合处高度的再狭窄。由于再次手术瓣膜置换风险增加，2例患者接受了经导管心脏瓣膜治疗。
我们不建议在CHVD患者中使用无支架生物瓣膜置换肺动脉瓣。这些瓣膜出现了极早的退变以及连续性再狭窄和心力衰竭。
