Bak Minjung, Kim Minyeong, Lee Boram, Kim Eun Kyoung, Park Taek Kyu, Yang Jeong Hoon, Kim Duk-Kyung, Chang Sung-A
Division of Cardiology, Department of Internal Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Korean Circ J. 2023 Mar;53(3):170-184. doi: 10.4070/kcj.2022.0252.
Several cases involving severe right ventricular (RV) failure in advanced cancer patients have been found to be pulmonary tumor thrombotic microangiopathies (PTTMs). This study aimed to discover the nature of rapid RV failure syndrome with a suspected diagnosis of PTTM for better diagnosis, treatment, and prognosis prediction in clinical practice.
From 2011 to 2021, all patients with clinically suspected PTTM were derived from the one tertiary cancer hospital with more than 2000 in-hospital bed.
A total of 28 cases of clinically suspected PTTM with one biopsy confirmed case were included. The most common cancer types were breast (9/28, 32%) and the most common tissue type was adenocarcinoma (22/26, 85%). The time interval from dyspnea New York Heart Association (NYHA) Grade 2, 3, 4 to death, thrombocytopenia to death, desaturation to death, admission to death, RV failure to death, cardiogenic shock to death were 33.5 days, 14.5 days, 7.4 days, 6.4 days, 6.1 days, 6.0 days, 3.8 days and 1.2 days, respectively. The NYHA Grade 4 to death time was 7 days longer in those who received chemotherapy (7.1 days vs. 13.8 days, p value=0.030). However, anticoagulation, vasopressors or intensive care could not change clinical course.
Rapid RV failure syndrome with a suspected diagnosis of PTTM showed a rapid progressive course from symptom onset to death. Although chemotherapy was effective, increased life survival was negligible, and treatments other than chemotherapy did not help to improve the patient's prognosis.
在晚期癌症患者中，数例严重右心室（RV）衰竭病例被发现为肺肿瘤血栓性微血管病（PTTM）。本研究旨在探究疑似PTTM的快速右心室衰竭综合征的本质，以便在临床实践中进行更好的诊断、治疗及预后预测。
2011年至2021年，所有临床疑似PTTM的患者均来自一家拥有2000多张住院床位的三级癌症医院。
共纳入28例临床疑似PTTM病例，其中1例经活检确诊。最常见的癌症类型为乳腺癌（9/28，32%），最常见的组织类型为腺癌（22/26，85%）。从纽约心脏协会（NYHA）2级、3级、4级呼吸困难到死亡、血小板减少到死亡、血氧饱和度下降到死亡、入院到死亡、右心室衰竭到死亡、心源性休克到死亡的时间间隔分别为33.5天、14.5天、7.4天、6.4天、6.1天、6.0天、3.8天和1.2天。接受化疗的患者从NYHA 4级到死亡的时间比未接受化疗的患者长7天（7.1天对13.8天，p值=0.030）。然而，抗凝、血管活性药物或重症监护并不能改变临床病程。
疑似PTTM的快速右心室衰竭综合征从症状出现到死亡呈现快速进展的病程。尽管化疗有效，但生存时间的增加微不足道，且化疗以外的治疗无助于改善患者预后。
