Prevalence of Short Stature and Growth Hormone Deficiency and Factors Associated With Short Stature After Fontan Surgery

Matsumura, Shun; Yana, Akiko; Kuwata, Seiko; Kurishima, Clara; Saiki, Hiroshi; Iwamoto, Yoichi; Ishido, Hirotaka; Masutani, Satoshi; Senzaki, Hideaki

doi:10.1253/circrep.cr-20-0009

Cited by 3 publications

(4 citation statements)

References 27 publications

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“…This is especially relevant in children with a Fontan circulation who may have numerous risk factors for poor growth including hypoxemia, relatively low cardiac output, poor nutrition, and nitrogen-depleting conditions such as the complication of protein-losing enteropathy (PLE). Studies have shown a higher prevalence of short stature in children and adolescents after Fontan compared to the general population [ 18 – 20 ]. Even though, on average, the height deficits post-Fontan are mild, there are subgroups at higher risk for more severe growth problems and it is in these cases where a timely intervention is mandatory to investigate comorbidities and allow time for catch-up growth.…”

Section: The Multidisciplinary Approach: Areas Of Focusmentioning

confidence: 99%

A Path FORWARD: Development of a Comprehensive Multidisciplinary Clinic to Create Health and Wellness for the Child and Adolescent with a Fontan Circulation

et al. 2022

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Today, it is anticipated most individuals diagnosed with single-ventricle malformation will survive surgical reconstruction through a successful Fontan operation. As greater numbers of patients survive, so has the recognition that individuals with Fontan circulation face a variety of challenges. The goal of a normal quality and duration of life will not be reached by all. The hurdles fall into a variety of domains. From a cardiovascular perspective, the Fontan circulation is fundamentally flawed by its inherent nature of creating a state of chronically elevated venous pressure and congestion, accompanied by a relatively low cardiac output. Ventricular dysfunction, atrioventricular valve regurgitation, and arrhythmia may directly impact cardiac performance and can progress with time. Problems are not limited to the cardiovascular system. Fontan circulatory physiology impacts a multitude of biological processes and health parameters outside the heart. The lymphatic circulation is under strain manifesting as variable degrees of protein-rich lymph loss and immune system dysregulation. Organ system dysfunction develops through altered perfusion profiles. Liver fibrosis is ubiquitous, and a process of systemic fibrogenesis in response to circulatory stressors may affect other organs as well. Somatic growth and development can be delayed. Behavioral and mental health problems are common, presenting as clinically important levels of anxiety and depression. Most striking is the high variability in prevalence and magnitude of these complications within the population, indicating the likelihood of additional factors enhancing or mitigating their emergence. We propose that optimal care for the individual with single ventricle and a Fontan circulation is ideally offered in a comprehensive multidisciplinary manner, with attention to elements that are beyond cardiac management alone. In this report, we share the concepts, our experiences, and perspectives on development of a clinic model—the “Fontan rehabilitation, wellness and resilience development” or FORWARD program. We provide insights into the mechanics of our multidisciplinary model of care and the benefits offered serving our growing population of individuals with a Fontan circulation and their families.

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Section: The Multidisciplinary Approach: Areas Of Focusmentioning

confidence: 99%

A Path FORWARD: Development of a Comprehensive Multidisciplinary Clinic to Create Health and Wellness for the Child and Adolescent with a Fontan Circulation

et al. 2022

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“…Alteration in this mechanism may be implicated in the high incidence of short stature seen in Fontan populations. 3,6 TRH triggers thyroid-stimulating hormone (TSH) production by the anterior pituitary gland; this system may be involved in the high incidence of hypothyroidism as well as short stature in Fontan populations. 3,4,6 Gonadotropin-releasing hormone (GnRH) neurons are found in the medial preoptic nucleus (in the anterior superior subunit) and in the arcuate nucleus.…”

Section: Discussionmentioning

confidence: 99%

“…3,6 TRH triggers thyroid-stimulating hormone (TSH) production by the anterior pituitary gland; this system may be involved in the high incidence of hypothyroidism as well as short stature in Fontan populations. 3,4,6 Gonadotropin-releasing hormone (GnRH) neurons are found in the medial preoptic nucleus (in the anterior superior subunit) and in the arcuate nucleus. These trigger follicular stimulating hormone (FSH) and luteinizing hormone (LH) production by the anterior pituitary gland via the portal circulation.…”

Section: Discussionmentioning

confidence: 99%

“…Hypothalamic-pituitary-axis (HPA) dysfunction may be more common than realized in post Fontan circulation given the high frequency of short stature, central hypothyroidism and hypogonadic hypogonadism reported in this unique patient population. [3][4][5][6] One previous study has demonstrated significantly larger pituitary volumes in children post Fontan compared to controls, however the sample size was small, and methodology did not allow segmental volumetrics. To date these findings have not yet been replicated.…”

Section: Introductionmentioning

confidence: 99%

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Segmental MRI Pituitary and Hypothalamus Volumes post Fontan: An analysis of the Australian and New Zealand Fontan Registry

Gee,

Yang,

Gentles

et al. 2024

Preprint

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ObjectiveShort 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.MethodsMRI 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.ResultsNormalized 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 andp<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.ConclusionsSegmental 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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Efficacy, safety, and patient satisfaction of norditropin and sogroya in patients with growth hormone deficiency: a systematic review and meta-analysis of randomized controlled trials

Altobaishat,

Abouzid,

Moawad

et al. 2024

Endocrine

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Introduction Growth hormone deficiency occurs when the pituitary gland does not produce enough growth hormone. Norditropin®, a recombinant human growth hormone, and Sogroya®, an albumin-binding growth hormone derivative, are prescribed for patients with growth hormone deficiency. This systematic review assesses the efficacy, safety, and patient satisfaction associated with Norditropin and Sogroya. Methods We systematically searched PubMed, Web of Science, and Scopus databases to identify eligible comparative studies. All studies published until June 2023 were included in our analysis. Our outcomes for children included height velocity and height velocity standard deviation score. In contrast, adult outcomes included adverse events, insulin-like growth factor 1-standard deviation score (IGF-1 SDS), and the Treatment Satisfaction Questionnaire for Medication-9 (TSQM-9). Results are reported as odds ratio (OR) and mean difference (MD) with a 95% confidence interval (95% CI). Results Ten studies involving 1058 participants (665 children and 393 adults) were included in the meta-analysis. In children, Norditropin at doses of 0.034 and 0.067 mg/kg/day was compared to Sogroya at doses of 0.04, 0.08, 0.16, and 0.24 mg/kg/week. The results showed that 0.034 mg/kg/day Norditropin had a favorable impact on height velocity (MD −2.01, 95% CI −3.7 to −2.12, p < 0.00001) and height velocity standard deviation score (Mean Difference −3.61, 95% CI −5.06 to −2.16, p < 0.00001) when compared to Sogroya 0.04 mg/kg/day. Other doses showed comparable results. In adults, the only significant side effect noted was rash, which favored Sogroya (OR 0.1, 95% CI 0.04–0.27, p < 0.00001). Additionally, IGF-1 SDS was significantly higher in the Sogroya group than in the Norditropin group (MD 0.25, 95% CI 0.02–0.48, p = 0.03). Furthermore, the overall score of the TSQM-9 questionnaire, which includes three domains: convenience, effectiveness, and satisfaction, was significantly higher in the Sogroya group compared to the Norditropin group (OR 6.36, 95% CI 3.92–8.8, p < 0.00001). Conclusion Norditropin and Sogroya showed comparable efficacy and safety profiles, except for the prevalence of rash in the Norditropin group, and Sogroya has higher satisfaction among adults. More high-quality studies with more patients are required to confirm these results.

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Prevalence of Short Stature and Growth Hormone Deficiency and Factors Associated With Short Stature After Fontan Surgery

Cited by 3 publications

References 27 publications

A Path FORWARD: Development of a Comprehensive Multidisciplinary Clinic to Create Health and Wellness for the Child and Adolescent with a Fontan Circulation

A Path FORWARD: Development of a Comprehensive Multidisciplinary Clinic to Create Health and Wellness for the Child and Adolescent with a Fontan Circulation

Segmental MRI Pituitary and Hypothalamus Volumes post Fontan: An analysis of the Australian and New Zealand Fontan Registry

Efficacy, safety, and patient satisfaction of norditropin and sogroya in patients with growth hormone deficiency: a systematic review and meta-analysis of randomized controlled trials

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