Université Paris Cité, Paris, France.
Cardiology, Bichat and Beaujon Hospitals, APHP, Paris, France.
Heart. 2023 Dec 20;110(2):132-139. doi: 10.1136/heartjnl-2023-322945.
Despite recent advances in surgical and interventional techniques, knowledge on the management of carcinoid heart disease (CHD) remains limited. In a cohort of patients with liver metastases of midgut neuroendocrine tumours (NETs), we aimed to describe the perioperative management and short-term outcomes of CHD.
From January 2003 to June 2022, consecutive patients with liver metastases of midgut NETs and severe CHD (severe valve disease with symptoms and/or right ventricular enlargement) were included at Beaujon and Bichat hospitals. All patients underwent clinical evaluation and echocardiography.
Out of 43 (16%) consecutive patients with severe CHD and liver metastases of midgut NETs, 79% presented with right-sided heart failure. Tricuspid valve replacement was performed in 26 (53%) patients including 19 (73%) cases of combined pulmonary valve replacement. The 30-day postoperative mortality rate was high (19%), and preoperative heart failure was associated with worse survival (p=0.02). Epicardial pacemakers were systematically implanted in operated patients and 25% were permanently paced. A postoperative positive right ventricular remodelling was observed (p<0.001). A greater myofibroblastic infiltration was observed in pulmonary versus tricuspid valves (p<0.001), suggesting that they may have been explanted at an earlier stage of the disease than the tricuspid valve, with therefore potential for evolution.
We observed a high postoperative mortality rate and baseline right-sided heart failure was associated with worse outcome. In surviving patients, a positive right ventricular remodelling was observed. Prospective, multicentre studies are warranted to better define the management strategy and to identify biomarkers associated with outcome in CHD.
尽管外科和介入技术取得了最近的进展，但对类癌心脏疾病（CHD）的管理仍知之甚少。在一组患有中肠神经内分泌肿瘤（NET）肝转移的患者中，我们旨在描述 CHD 的围手术期管理和短期结果。
从 2003 年 1 月至 2022 年 6 月，在博让和比沙医院连续纳入患有中肠 NET 肝转移和严重 CHD（严重瓣膜疾病伴有症状和/或右心室扩大）的患者。所有患者均接受临床评估和超声心动图检查。
在 43 例（16%）患有严重 CHD 和中肠 NET 肝转移的连续患者中，79%的患者出现右心衰竭。26 例（53%）患者行三尖瓣置换术，其中 19 例（73%）患者行肺动脉瓣置换术。术后 30 天死亡率较高（19%），术前心力衰竭与生存率降低相关（p=0.02）。手术患者系统植入心外膜起搏器，25%的患者被永久起搏。观察到术后右心室重塑呈阳性（p<0.001）。在肺瓣和三尖瓣中观察到更大的肌成纤维细胞浸润（p<0.001），这表明它们可能在疾病的早期阶段被切除，因此可能有进展的潜力。
我们观察到高术后死亡率，基线右心衰竭与不良结局相关。在存活患者中，观察到右心室重塑呈阳性。需要进行前瞻性、多中心研究，以更好地定义 CHD 的管理策略，并确定与结局相关的生物标志物。
