Department of Cardiac Surgery, University Hospital Bern, University of Bern, Bern, Switzerland.
Department of Advanced Cardiopulmonary Therapies and Transplantation, The University of Texas Health Science Center at Houston, Houston, TX, USA.
Eur J Cardiothorac Surg. 2024 Sep 2;66(3). doi: 10.1093/ejcts/ezae313.
Mitral valve regurgitation and left ventricular dysfunction are cardiovascular symptoms of Marfan syndrome. There is a paucity of information on tricuspid valve regurgitation and right ventricular function. In patients with Marfan syndrome, we looked at long-term changes in right ventricular function, tricuspid valve regurgitation and freedom from tricuspid valve repair.
Retrospective-observational single-centre analysis on right ventricular function and tricuspid regurgitation in Marfan patients who underwent surgery with cardioplegic arrest between 1995 and 2020. Patients were followed-up from 1st operation until death, with echocardiographic changes analysed longitudinally. Composite end point was tricuspid annular plane systolic excursion (TAPSE) ≤16 mm, severe tricuspid regurgitation or tricuspid repair.
The study included 135 patients who underwent 193 operations, 58 of those were reoperations in 40 patients. Median age at 1st operation was 35 years [interquartile range (IQR) 26-46], median follow-up was 8.0 years (IQR 3.0-16.0) and median time to 1st reoperation was 7.5 years (IQR 3.4-12.5). The composite end point occurred in 81 observations in 40 patients, mostly as a recurrent event, after median 7.0 years (IQR 1.0-13.0). Ten-year cumulative incidence for composite end point was 22.0% (95% CI 15-31) and 9.0% (95% CI 4.4-16) for new-onset TAPSE ≤16 mm, but no significant change in TAPSE was observed at 10 years. Tricuspid regurgitation was associated with increased risk of annual progression (P < 0.001), but not clinically relevant at 10 years. Actuarial 10-year survival was 91.1%.
In Marfan patients with a history of cardiac surgery and subsequent reoperations, the right ventricular function remains stable. The incidence of severe tricuspid regurgitation and tricuspid repair remain low.
二尖瓣反流和左心室功能障碍是马凡综合征的心血管症状。关于三尖瓣反流和右心室功能的信息很少。在马凡综合征患者中，我们观察了右心室功能、三尖瓣反流以及避免三尖瓣修复的长期变化。
对 1995 年至 2020 年间接受心脏停搏下心内直视手术的马凡综合征患者的右心室功能和三尖瓣反流进行回顾性观察性单中心分析。患者从第 1 次手术开始随访，直至死亡，纵向分析超声心动图变化。复合终点为三尖瓣环平面收缩位移（TAPSE）≤16mm、严重三尖瓣反流或三尖瓣修复。
该研究纳入了 135 例患者的 193 次手术，其中 40 例患者中有 58 次为再次手术。第 1 次手术时的中位年龄为 35 岁[四分位距（IQR）26-46]，中位随访时间为 8.0 年（IQR 3.0-16.0），第 1 次再次手术的中位时间为 7.5 年（IQR 3.4-12.5）。40 例患者中有 81 例观察到复合终点，主要为复发性事件，中位时间为 7.0 年（IQR 1.0-13.0）。复合终点的 10 年累积发生率为 22.0%（95%CI 15-31），新发 TAPSE≤16mm 的 10 年累积发生率为 9.0%（95%CI 4.4-16），但 10 年时 TAPSE 无明显变化。三尖瓣反流与每年进展的风险增加相关（P<0.001），但 10 年后无临床意义。10 年生存率为 91.1%。
在有心脏手术和随后再次手术史的马凡综合征患者中，右心室功能保持稳定。严重三尖瓣反流和三尖瓣修复的发生率仍然较低。
