Development of nephropathy in an adult patient after Fontan palliation for cyanotic congenital heart disease

Hayashi, Kaori; Hashiguchi, Akinori; Ikemiyagi, Masako; Tokuyama, Hirobumi; Wakino, Shu; Itoh, Hiroshi

doi:10.1007/s13730-021-00573-2

Cited by 3 publications

(4 citation statements)

References 14 publications

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“…In this case renal dysfunction may be reversible ( 55 ). Focal segmental glomerulosclerosis may occur in older patients (ages of case reports ranged from 24 to 41 years old) ( 56 , 57 ) compared to renal dysfunction with preserved renal structure (one case report in a 14 year old patient) ( 55 ).…”

Section: Resultsmentioning

confidence: 99%

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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Section: Resultsmentioning

confidence: 99%

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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“…This case review outlined the case of an adult female with cyanotic nephropathy. Notably, cyanotic nephropathy is characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia and is regarded as a potential long-term complication of cyanotic CHD [1, 3, 4]. The risk factor for the development and progression of nephropathy in this patient is secondary polycythemia arising from cyanotic CHD.…”

Section: Discussionmentioning

confidence: 99%

“…Of note, glomerular enlargement has been documented to be associated with an elevated risk of glomerulosclerosis, progressive decline in renal function, and poorer prognosis [13]. Hayashi et al [3] recently reported a 41-year-old cyanotic nephropathy patient with kidney biopsy findings suggestive of adaptive focal segmental glomerular sclerosis. As can be deduced, renal changes may not be uniform and depend on the age of the patient, the type and severity of the congenital heart anomaly, and the age at which palliative or definitive surgery was performed [14].…”

Section: Discussionmentioning

confidence: 99%

“…As is well documented, cyanotic nephropathy is a complication of cyanotic congenital heart disease (CCHD). It is a rare disease that occurs in roughly 30–50% of patients with CCHD [1, 2] and is characterized by hyperuricemia, polycythemia, thrombocytopenia, proteinuria, and decreased estimated GFR [3, 4]. At present, the majority of reports and studies on cyanotic nephropathy predominantly focus on children or dead cases, while cases of adult cyanotic nephropathy are scarce, attributable to historical limitations in medical technology.…”

Section: Introductionmentioning

confidence: 99%

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Cyanotic Nephropathy in an Adult Patient with Eisenmenger Syndrome: A Case Report and Literature Review

Zhu,

Wen,

Tan

et al. 2024

Kidney Blood Press Res

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Introduction: Cyanotic nephropathy, a rare disease characterized by proteinuria, decreased estimated glomerular filtration rate, thrombocytopenia, polycythemia, and hyperuricemia, may occasionally be secondary to cyanotic congenital heart disease (CHD). There are currently no detailed diagnostic criteria or treatments for cyanotic nephropathy, owing to its extremely low incidence. Eisenmenger syndrome (ES) was initially defined by Paul Wood in pathophysiologic terms as “pulmonary hypertension (PH) at the systemic level, caused by a high pulmonary vascular resistance, with a reversed or bidirectional shunt at the aorto-pulmonary, ventricular, or atrial level.” It typically develops in the presence of large, unrepaired atrial or ventricular septal defects, arterial shunts, or complex forms of CHD and is the most severe hemodynamic phenotype of pulmonary arterial hypertension associated with CHD. This study aimed to outline the case of an ES patient who developed cyanotic nephropathy and successfully achieved clinical remission through primary disease treatment and symptomatic management. Overall, this case expands our understanding of cyanotic nephropathy and lays a theoretical reference for the treatment of ES. Case Presentation: A 33-year-old Chinese female attended the outpatient department with abnormal urine test results over the past two and a half years. Following a comprehensive medical history collection, she underwent the necessary tests. Cardiac color ultrasound displayed a significant widening of the pulmonary artery and PH (severe), as well as mild tricuspid regurgitation and patent ductus arteriosus. The results of the kidney biopsy, combined with clinical findings, suggested a high risk of polycythemia-related kidney disease. She was eventually diagnosed with cyanotic nephropathy and ES. Her symptoms were relieved following symptomatic treatment, such as the administration of ambrisentan, febuxostat, and home oxygen therapy. Her follow-up visit at 6 months demonstrated improvements in hyperuricemia and a significant increase in physical strength. Conclusion: Cyanotic nephropathy is a rare condition in adults. Kidney biopsy remains the gold standard of diagnosis for various nephropathies. Active treatment of CHD and alleviating hypoxia may be pivotal for the treatment of cyanotic nephropathy.

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Determinants and prognostic value of albuminuria in adult patients with congenital heart disease

Ohuchi¹,

Mori²,

Fujita³

et al. 2023

American Heart Journal

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Development of nephropathy in an adult patient after Fontan palliation for cyanotic congenital heart disease

Cited by 3 publications

References 14 publications

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

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

Cyanotic Nephropathy in an Adult Patient with Eisenmenger Syndrome: A Case Report and Literature Review

Determinants and prognostic value of albuminuria in adult patients with congenital heart disease

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