Segmental MRI pituitary and hypothalamus volumes post Fontan: An analysis of the Australian and New Zealand Fontan registry

IF 0.8 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS International journal of cardiology. Congenital heart disease Pub Date : 2024-10-22 DOI:10.1016/j.ijcchd.2024.100549
Waverley Gee , Joseph Yuan-Mou Yang , Tom Gentles , Sonja Bastin , Ajay J. Iyengar , Jian Chen , Dug Yeo Han , Rachael Cordina , Charlotte Verrall , Craig Jefferies , The Australian and New Zealand Fontan Registry
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Abstract

Objective

Short stature, central hypothyroidism and infertility are common in those with a Fontan circulation. Given that the Fontan circulation often results in hepatic portal venous congestion, we hypothesize that the hypothalamic-pituitary portal circulation is also affected, contributing to subsequent hypothalamic-pituitary axis dysfunction.

Methods

MRI data from the Australian and New Zealand Fontan Registry (86 cases) was compared to 86 age- and sex-matched normal published controls. Total pituitary volumes (both anterior and posterior glands) were measured using a manual tracing segmentation method, and hypothalamic (and subunit) volumes using an automated segmentation tool. Measured gland volume was normalized to total brain volumes. A generalized linear model was used for statistical analysis.

Results

Normalized total pituitary volumes (nTPV) were increased in Fontan patients compared to controls (p < 0.0001), due to an increase in anterior pituitary volumes (nAPV) (p < 0.0001), with no difference in normalized posterior pituitary volumes (p = 0.7). Furthermore, normalized anterior and tubular hypothalamic subunit groups) were increased in Fontan patients compared to the controls (p < 0.01 and p < 0.0001, respectively).
The time between Fontan and MRI was positively related to nTPV, nAPV and bilateral hypothalamic volumes. nTPV increased with age, and the increase in nAPV was greater in Fontan patients.

Conclusions

Segmental MRI Pituitary and Hypothalamus volumes post Fontan are increased and are related to the time since Fontan procedure. These findings are consistent with venous congestion of the anterior hypothalamic-pituitary portal venous system and may explain the high frequency of endocrine dysfunction in this patient group.
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丰坦术后垂体和下丘脑的分段 MRI 容量:澳大利亚和新西兰Fontan登记分析
目的:身材矮小、中枢性甲状腺功能减退症和不育症常见于Fontan循环患者。鉴于Fontan循环通常会导致肝门静脉充血,我们假设下丘脑-垂体门静脉循环也会受到影响,从而导致随后的下丘脑-垂体轴功能障碍。方法将澳大利亚和新西兰Fontan登记处的MRI数据(86例)与86例年龄和性别匹配的正常发表对照组进行比较。垂体总体积(包括前部和后部腺体)采用手动追踪分割法测量,下丘脑(和亚单位)体积采用自动分割工具测量。测量的腺体体积与大脑总体积进行了归一化处理。结果与对照组相比,Fontan 患者的正常化垂体总体积(nTPV)增加(p < 0.0001),这是由于垂体前叶体积(nAPV)增加(p < 0.0001),而正常化垂体后叶体积没有差异(p = 0.7)。此外,与对照组相比,Fontan 患者的正常化垂体前叶和管状下丘脑亚单位组增加(分别为 p < 0.01 和 p < 0.0001)。Fontan 和 MRI 之间的时间间隔与 nTPV、nAPV 和双侧下丘脑体积呈正相关。nTPV随着年龄的增长而增加,而nAPV的增加在Fontan患者中更大。结论Fontan术后分段MRI垂体和下丘脑体积增加,并与Fontan术后的时间有关。这些发现与下丘脑-垂体前叶门静脉系统的静脉充血相一致,并可解释该患者群为何频繁出现内分泌功能障碍。
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来源期刊
International journal of cardiology. Congenital heart disease
International journal of cardiology. Congenital heart disease Cardiology and Cardiovascular Medicine
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