Increased Pituitary Volumes in Children after Fontan Operation: Congestion in the Other Portal Circulation

Muneuchi, Jun; Nagatomo, Yusaku; Okada, Seigo; Iida, Chiaki; Shirozu, Hiromitsu; Sugitani, Yuichiro; Watanabe, Mamie

doi:10.1016/j.jpeds.2017.09.065

Cited by 7 publications

(10 citation statements)

References 11 publications

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“…Muneuchi et al reported that pituitary gland volume measured on magnetic resonance imaging significantly increased after Fontan surgery compared with that in age-matched control children, and that increased pituitary volume is related to increased CVP. 25 We also previously reported that cerebral circulation in patients with Fontan circulation was impaired and that it might affect neurological development. 6,26 This suggests that volume retention and congestion, the prominent features of Fontan circulation, affect physical growth partly due to pituitary gland dysfunction, highlighting the need for the screening and treatment of this condition after Fontan surgery.…”

Section: Discussionmentioning

confidence: 84%

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

et al. 2020

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Background: Fontan circulation is characterized by many features commonly observed in heart failure that may affect physical growth regardless of pituitary gland dysfunction status. The aims of the present study were to investigate the prevalence of short stature and growth hormone deficiency (GHD) and determine the factors associated with short stature after Fontan surgery. Methods and Results: On retrospective evaluation of 47 patients after Fontan surgery, a very high prevalence of short stature was observed (38.3%). In the short stature group, 5 patients were diagnosed with GHD (10.6% of patients after Fontan Surgery), which is much higher than the frequency of 1/10,000 in the general population. Central venous pressure (CVP) was significantly higher (14.6±4.5 vs. 12.2±1.9 mmHg, P<0.05) and the blood pressure and arterial oxygen saturation were significantly lower in the short stature group. Laboratory data also indicated volume retention and congestion in the short stature group. Mean change in stature from catheterization 1 year after Fontan surgery to the most recent visit was significantly lower in the short stature group (−1.1±1.1 SD vs. 0.0±0.8 SD, P<0.05) and significantly negatively correlated with CVP (r=−0.42, P<0.05). Conclusions: Volume retention and congestion, the prominent features of Fontan circulation, affect physical growth partly due to pituitary gland dysfunction, highlighting the need for the screening for and treatment of this condition after Fontan surgery.

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Section: Discussionmentioning

confidence: 84%

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

et al. 2020

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“…Whether these findings relate to neurocognitive outcome is not clearly established. Contrary to other brain structures, the pituitary has an increased volume in Fontan patients ( 18 ). This is further explored in the Endocrinology section of this review.…”

Section: Resultsmentioning

confidence: 99%

“…The pituitary is an important organ in many endocrine axes. The volume of the pituitary, in contrast to most brain structures, is larger in Fontan patients than control subjects ( 18 ). Pituitary volumes in Fontan patients increased with age, but at a rate similar to healthy controls ( 18 ).…”

Section: Resultsmentioning

confidence: 99%

“…The volume of the pituitary, in contrast to most brain structures, is larger in Fontan patients than control subjects ( 18 ). Pituitary volumes in Fontan patients increased with age, but at a rate similar to healthy controls ( 18 ). Increased pituitary volumes may relate to the circulation of the pituitary, which has a portal system, similar to the liver.…”

Section: Resultsmentioning

confidence: 99%

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Fontan Circulation Associated Organ Abnormalities Beyond the Heart, Lungs, Liver, and Gut: A Systematic Review

Ritmeester

Veger

Ven

et al. 2022

Front. Cardiovasc. Med.

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IntroductionPatients with a Fontan circulation are at risk for sequelae of Fontan physiology during follow-up. Fontan physiology affects all organ systems and an overview of end-organ damage is needed.MethodsWe performed a systematic review of abnormalities in multiple organ systems for patients with a longstanding Fontan circulation. We searched online databases for articles describing abnormalities in multiple organ systems. Cardio-pulmonary abnormalities, protein losing enteropathy, and Fontan associated liver disease have already extensively been described and were excluded from this systematic review.ResultsOur search returned 5,704 unique articles. After screening, we found 111 articles relating to multiple organ systems. We found abnormalities in, among others, the nervous system, pituitary, kidneys, and musculoskeletal system. Pituitary edema—relating to the unique pituitary vasculature- may affect the thyroid axis. Renal dysfunction is common. Creatinine based renal function estimates may be inappropriate due to myopenia. Both lean muscle mass and bone mineral density are decreased. These abnormalities in multiple organ systems may be related to Fontan physiology, cyanosis, iatrogenic factors, or lifestyle.ConclusionsHealth care providers should be vigilant for hypothyroidism, visual or hearing deficits, and sleep disordered breathing in Fontan patients. We recommend including cystatin C for assessment of renal function. This review may aid health care providers and guide future research.Systematic Review Registration:https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42021232461, PROSPERO, identifier: CRD42021232461.

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“…Cerebral MRI has shown morphological differences in some cerebral structures of Fontan patients compared with those of healthy controls 53 , 54 . Interestingly, the pituitary gland, supplied by a portal venous system similar to the liver, appears to be enlarged following Fontan surgery 55 . The relevance of this possible congestion on the endocrine system remains unclear.…”

Section: State Of the Artmentioning

confidence: 99%

State of the art of the Fontan strategy for treatment of univentricular heart disease

et al. 2018

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In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. In this review, we discuss the state of the art of the Fontan strategy by assessing survival and risk factors for mortality. Complications of the Fontan circulation, such as cardiac arrhythmia, thromboembolism, and protein-losing enteropathy, are discussed. Common surgical and catheter-based interventions following Fontan completion are outlined. We describe functional status measurements such as quality of life and developmental outcomes in the contemporary Fontan patient. The current role of drug therapy in the Fontan patient is explored. Furthermore, we assess the current use and outcomes of mechanical circulatory support in the Fontan circulation and novel surgical innovations. Despite large improvements in outcomes for contemporary Fontan patients, a large burden of disease exists in this patient population. Continued efforts to improve outcomes are warranted. Several remaining challenges in the Fontan field are outlined.

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Increased Pituitary Volumes in Children after Fontan Operation: Congestion in the Other Portal Circulation

Cited by 7 publications

References 11 publications

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

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

Fontan Circulation Associated Organ Abnormalities Beyond the Heart, Lungs, Liver, and Gut: A Systematic Review

State of the art of the Fontan strategy for treatment of univentricular heart disease

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