Lymphopenia in the Adult Population With Fontan Physiology: A Potential New Marker for Disease Assessment

IF 2.5 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS CJC Open Pub Date : 2024-06-01 DOI:10.1016/j.cjco.2024.01.012
Anna L. Scandinaro MD , Michael D. McCann MD, MBA , Anisa Chaudhry MD , Allen Kunselman MA , Elisa A. Bradley MD , William R. Davidson Jr. MD
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Abstract

Background

Patients with complex congenital heart disease and Fontan palliation frequently develop extracardiac disease, including hematologic abnormalities, such as lymphopenia. However, the clinical implications of this finding are poorly understood and are therefore the topic of this investigation.

Methods

Patients with Fontan physiology in our centre (1999-2018) were evaluated for the presence and impact of lymphopenia. The cohort was divided into a group with lymphopenia (L) (2 consecutive absolute lymphocyte counts ≤ 1∗103 K/ μL) and a group who had never had lymphopenia (NL). Clinical characteristics and hospital admissions (762 patient-years) were evaluated.

Results

In 62 adult patients with Fontan physiology (aged 34 ± 9 years; 32 women [52%]), the patients who developed lymphopenia earliest did so 8 years after Fontan completion, with up to 60% of patients developing lymphopenia by 30 years. Lymphopenia was found to be associated with portal hypertension (varices, ascites, splenomegaly, and thrombocytopenia [VAST] score)—NL: 0 (0-2) vs L: 2 (0-4), P < 0.0001). A total of 76 heart failure and 81 arrhythmia-associated admissions occurred per 1000 patient-years. At 40 years post-Fontan, the probability of a heart failure admission was higher in the L group (L: 51 [86%] vs NL: 8 [14%], P < 0.01).

Conclusions

Adult patients with Fontan physiology and lymphopenia demonstrated portal hypertension and lymphatic dysfunction more commonly, perhaps suggesting that this may be a marker of Fontan congestion and early Fontan failure. Further investigation into the relationship between lymphopenia, clinical outcomes, and Fontan function is needed.

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成人 Fontan 群体中的淋巴细胞减少症:疾病评估的潜在新标记物
背景复杂先天性心脏病和Fontan姑息治疗患者经常会出现心外疾病,包括血液学异常,如淋巴细胞减少症。方法对本中心的丰坦生理学患者(1999-2018 年)进行了淋巴细胞减少症存在及其影响的评估。研究对象分为淋巴细胞减少症(L)组(连续两次淋巴细胞绝对计数≤1∗103 K/μL)和从未出现淋巴细胞减少症(NL)组。结果 在 62 名患有丰坦生理学的成年患者(年龄为 34 ± 9 岁;32 名女性 [52%])中,最早出现淋巴细胞减少症的患者是在丰坦手术完成后 8 年出现的,多达 60% 的患者在 30 年前出现淋巴细胞减少症。淋巴细胞减少症与门静脉高压有关(静脉曲张、腹水、脾肿大和血小板减少[VAST]评分)--NL:0 (0-2) vs L:2 (0-4),P < 0.0001)。每 1000 患者年中,共有 76 例心力衰竭和 81 例心律失常相关入院。结论成人丰坦生理学和淋巴细胞减少症患者更常表现为门脉高压和淋巴功能障碍,这可能是丰坦充血和早期丰坦衰竭的标志。需要进一步研究淋巴细胞减少症、临床结果和Fontan功能之间的关系。
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来源期刊
CJC Open
CJC Open Medicine-Cardiology and Cardiovascular Medicine
CiteScore
3.30
自引率
0.00%
发文量
143
审稿时长
60 days
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