Korean Circ J.  2023 Mar;53(3):170-184. 10.4070/kcj.2022.0252.

Clinical Course of Suspected Diagnosis of Pulmonary Tumor Thrombotic Microangiopathy: A 10-Year Experience of Rapid Progressive Right Ventricular Failure Syndrome in Advanced Cancer Patients

• Bak M
• Kim M
• Lee B
• Kim EK
• Park TK
• Yang JH
• Kim DK
• Chang SA

• KMID: 2540395
• DOI: http://doi.org/10.4070/kcj.2022.0252

Abstract

Background and Objectives
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.
Methods
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.
Results
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.
Conclusions
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.

Keyword

Neoplasm; Chemotherapy; Hypertension; pulmonary; Heart failure

Figure

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  Figure 1 Representative figure of patient with pulmonary tumor thrombotic microangiopathy.

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  Figure 2 Representative figure of chest tomography finding in pulmonary tumor thrombotic microangiopathy. (A) Centrilobular ground glass opacities. (B) Peripheral ground glass opacities.

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  Figure 3 Time interval from symptoms/signs/clinical event to death. Data are presented as median (25 percentile–75 percentile).Gr. = grade; NYHA = New York Heart Association; RV = right ventricular.*Patients numbers used in analysis.

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  Figure 4 Clinical time interval comparison according to treatment strategy. Data are presented as median (25 percentile–75 percentile). (A) Time interval comparison between patients with (n=8) and without chemotherapy (n=20). (B) Time interval comparison between patients with (n=16) and without anticoagulation (n=12). (C) Time interval comparison between patients with (n=14) and without vasopressor or inotrope (n=14). (D) Time interval comparison between patients with (n=9) and without intensive care (n=19).CT = chemotherapy; Gr. = grade; NYHA = New York Heart Association; Tx = treatment.

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