Rare problems associated with the Fontan circulation

2010 ◽  
Vol 20 (S3) ◽  
pp. 113-119 ◽  
Author(s):  
David J. Goldberg ◽  
Kathryn Dodds ◽  
Jack Rychik
Keyword(s):  
Fontan Operation ◽  
Venous Pressure ◽  
Multiple Organ ◽  
Univentricular Heart ◽  
Protein Losing Enteropathy ◽  
Congenital Cardiac Disease ◽  
Organ Systems ◽  
Coagulation Protein ◽  
Life Threatening ◽  
Fontan Physiology

AbstractThe Fontan operation, originally described for the surgical management of tricuspid atresia, is now the final surgery in the strategy of staged palliation for a number of different forms of congenital cardiac disease with a functionally univentricular heart. Despite the improved technical outcomes of the Fontan operation, staged palliation does not recreate a normal physiology. Without a pumping chamber delivering blood to the lungs, the cardiovascular system is less efficient; cardiac output is generally diminished, and the systemic venous pressure is increased. As a result, patients with “Fontan physiology” may face a number of rare but potentially life-threatening complications including hepatic dysfunction, abnormalities of coagulation, protein-losing enteropathy, and plastic bronchitis. Despite the staged palliation resulting in remarkable survival, the possible complications for this group of patients are complex, involve multiple organ systems, and can be life threatening. Identifying the mechanisms associated with each of the rare complications, and developing strategies to treat them, requires the work of many people at many institutions. Continued collaboration between sub-specialists and between institutions will be required to optimise the care for this group of survivors with functionally univentricular hearts.

scholarly journals Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation

2018 ◽  
Vol 38 (6) ◽  
pp. e5-e12 ◽  
Author(s):  
Christine Peyton
Keyword(s):  
Heart Failure ◽  
Congestive Heart Failure ◽  
Complex Disease ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Venous Hypertension ◽  
Plastic Bronchitis ◽  
Protein Losing Enteropathy ◽  
Fontan Physiology

Protein-losing enteropathy and plastic bronchitis remain challenging to treat despite recent treatment advances. Protein-losing enteropathy and plastic bronchitis have been diagnosed in patients with cardiomyopathy, constrictive pericarditis, and congestive heart failure. This article focuses on patients with protein-losing enteropathy or plastic bronchitis following the Fontan procedure. Patients with single-ventricle physiology who have undergone the Fontan procedure are at risk for these conditions. Fontan physiology predisposes patients to chronically low cardiac output, increased central venous pressure, and congestive heart failure. These altered hemodynamics lead to increased mesenteric vascular resistance, resulting in venous hypertension and congestion in protein-losing enteropathy. Plastic bronchitis is a complex disease in which chronic high lymphatic pressures from Fontan physiology cause acellular bronchial casts to develop. These entities may also occur in patients with normal Fontan hemodynamics. This article also covers medical and surgical interventions for protein-losing enteropathy and plastic bronchitis. (Critical Care Nurse. 2018;38[6]:e5–e12)

New concepts: development of a survivorship programme for patients with a functionally univentricular heart

2011 ◽  
Vol 21 (S2) ◽  
pp. 77-79 ◽  
Author(s):  
David J. Goldberg ◽  
Kathryn Dodds ◽  
Jack Rychik
Keyword(s):  
Fontan Operation ◽  
Univentricular Heart ◽  
Plastic Bronchitis ◽  
Complete Understanding ◽  
Protein Losing Enteropathy ◽  
New Concepts ◽  
Fontan Physiology ◽  
The Impact ◽  
Valuable Service

AbstractChildren with functionally univentricular hearts are now surviving into their third and fourth decades of life. Although survival alone is a remarkable achievement, a lot must still be done to improve the quality and duration of life after the Fontan operation. Challenges that may be faced by these patients include the impact of the Fontan operation on the liver and the density of bone, protein-losing enteropathy, and plastic bronchitis. Paediatric cardiologists are familiar with the haemodynamic issues inherent in Fontan physiology; however, training in cardiology is often not sufficient to give us a complete understanding of the pathophysiology of the complications or of the options for treatment. Collaboration with other subspecialists including gastroenterologists, endocrinologists, and pulmonologists is essential in order to provide the rigorous and nuanced care that our patients need and deserve. A clinic in which a patient can see multiple subspecialists, and in which the subspecialists, as a group, can discuss each patient, can provide a unique and valuable service for patients with a functionally univentricular heart.

URIC ACID LEVEL AS A PREDICTOR OF SEVERITY IN PREGNANT WOMEN WITH SEVERE PREECLAMPSIA

2021 ◽  
pp. 327-331
Author(s):  
Natasya Natasya ◽  
Fidel Ganis Siregar ◽  
Ratna Akbari Ganie
Keyword(s):  
Developing Countries ◽  
Uric Acid ◽  
Pregnant Women ◽  
Acid Level ◽  
Developed Countries ◽  
Multiple Organ ◽  
Live Births ◽  
Obstetric Emergency ◽  
Organ Systems ◽  
Life Threatening

Preeclampsia is a pregnancy syndrome affecting multiple organ systems, characterized by hypertension and proteinuria after 20 weeks of gestation. The incidence of preeclampsia is estimated to be 3-10% of pregnancies worldwide and is the leading cause of death for pregnant women. Preeclampsia is a life-threatening obstetric emergency, so it needs prompt and precise treatment to prevent morbidity and mortality. WHO estimates that the incidence of preeclampsia is seven times higher in developing countries (2.8% of live births) than in developed countries 1,2 (0.4%).

scholarly journals Thyroid Storm and Complete Heart Block after Treatment with Radioactive Iodine

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Kaitlyn Vennard ◽  
Matthew P. Gilbert
Keyword(s):  
Heart Block ◽  
Radioactive Iodine ◽  
Radioiodine Therapy ◽  
Complete Heart Block ◽  
Multiple Organ ◽  
Thyroid Storm ◽  
Metabolic Abnormalities ◽  
Organ Systems ◽  
Life Threatening ◽  
Life Threatening Complication

Thyroid storm is a rare endocrine emergency characterized by dysfunction of multiple organ systems. Thyroid storm is more common in Graves’ disease and can be precipitated by surgery, trauma, infection, metabolic abnormalities, iodine load, and parturition. We present a diagnostically challenging case of thyroid storm precipitated by radioiodine therapy and accompanied by bradycardia, a rare but life-threatening complication related to treatment for hyperthyroidism.

scholarly journals The Indirect Implication of SARS-CoV-2 Resulting in Kayexalate Toxicity in a Patient with Acute Kidney Injury

2021 ◽  
Vol 5 (1) ◽  
pp. 6-13
Author(s):  
Charles E. Middleton IV ◽  
William Daley ◽  
Neha Varshney
Keyword(s):  
Acute Kidney Injury ◽  
Virus Disease ◽  
Severe Disease ◽  
Kidney Injury ◽  
Multiple Organ ◽  
Subsequent Treatment ◽  
Gastrointestinal Complications ◽  
Treatment Regimens ◽  
Organ Systems ◽  
Life Threatening

The clinical features of corona virus disease 2019 (COVID-19) are variable, but the majority of patients experience mild flu-like symptoms. The cases of severe disease include complications such as progressive pneumonia, acute kidney injury, multi-organ failure, and even death. This paper explores the association between COVID-19 and its effect on multiple organ systems and how the subsequent treatment of this disease can itself lead to morbidity and mortality. We present a case which emphasizes the life threatening gastrointestinal complications associated with treatment of acute kidney injury (AKI) in a patient with COVID-19. We conclude that the patients whose treatment regimens utilize medical resins should be closely monitored for gastrointestinal complications so as to mitigate the known adverse effects associated with these drugs, such as colonic mucosal ulceration, perforation, or even death.

scholarly journals Hemophagocytic Lymphohistiocytosis: A Life Threatening Cytopenic Condition

2021 ◽  
Vol 15 (2) ◽  
pp. 98-102
Author(s):  
Suranjit Kumar Saha ◽  
MM Shahin Ul Islam ◽  
Nasir Uddin Ahmed ◽  
Prativa Saha
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Gene Mutations ◽  
Multiple Organ ◽  
Treatment Modalities ◽  
Hematopoietic Stem ◽  
Organ Systems ◽  
Life Threatening ◽  
Poor Outcomes

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder that occurs in many underlying conditions in all age. This is characterized by unbridled activation of cytotoxic T lymphocytes, natural killer (NK) cells and macrophages resulting in raised cytokine level. Those cytokines and immune mediated injury occur in multiple organ systems. It may be primary and secondary. Primary HLH is familial, childhood presentation and associated with gene mutations. Secondary HLH is acquired, adulthood presentation that occurs in infections, malignancies inflammatory and autoimmune diseases etc. Clinical manifestations include fever, splenomegaly, lymphadenopathy, neurologic dysfunction, coagulopathy, features of sepsis etc. Laboratory investigation includes cytopenias, hypertriglyceridemia, hyperferritinemia, abnormal liver function, hemophagocytosis, and diminished NKcell activity. Treatment modalities include immunosuppressive, immunomodulatory agents, cytostatic drugs, T-cell antibodies, anticytokine agents and hematopoietic stem cell transplantation (HSCT). Besides those, aggressive supportive care combined with specific treatment of the precipitating factor can produce better outcome. With treatment more than 50% of children who undergo transplant survive, but adults have quite poor outcomes even with aggressive management. Faridpur Med. Coll. J. 2020;15(2): 98-102

scholarly journals Klippel-Trenaunay Syndrome Causing Life-Threatening GI Bleeding: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Salih Samo ◽  
Muhammed Sherid ◽  
Husein Husein ◽  
Samian Sulaiman ◽  
Margaret Yungbluth ◽  
...  
Keyword(s):  
Vascular Malformations ◽  
Multiple Organ ◽  
Gi Bleeding ◽  
Blood Products ◽  
Review Of The Literature ◽  
Gi Tract ◽  
Organ Systems ◽  
Life Threatening ◽  
Klippel Trenaunay Syndrome ◽  
Congenital Syndrome

Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome of vascular malformations and soft tissue and bone hypertrophy. Vascular malformations can affect multiple organ systems. Involvement of the gastrointestinal (GI) tract is uncommon in KTS, but it can be a source of life-threatening bleeding. We report a case of a 32-year-old male with a known diagnosis of KTS who presented with a life-threatening rectal bleeding and was treated with proctosigmoidectomy and massive blood products transfusion. He expired after a long hospitalization. We then review the literature on KTS and management of some of its complications.

End-organ consequences of the Fontan operation: liver fibrosis, protein-losing enteropathy and plastic bronchitis

2013 ◽  
Vol 23 (6) ◽  
pp. 831-840 ◽  
Author(s):  
Jack Rychik ◽  
David Goldberg ◽  
Elizabeth Rand ◽  
Edisio Semeao ◽  
Pierre Russo ◽  
...  
Keyword(s):  
Liver Fibrosis ◽  
Fontan Operation ◽  
Management Strategies ◽  
Plastic Bronchitis ◽  
Protein Losing Enteropathy ◽  
State Management ◽  
Life Saving ◽  
Circulatory Insufficiency ◽  
Life Threatening ◽  
Organ Complications

AbstractThe Fontan operation, although part of a life-saving surgical strategy, manifests a variety of end-organ complications and unique morbidities that are being recognised with increasing frequency as patients survive into their second and third decades of life and beyond. Liver fibrosis, protein-losing enteropathy and plastic bronchitis are consequences of a complex physiology involving circulatory insufficiency, inflammation and lymphatic derangement. These conditions are manifest in a chronic, indolent state. Management strategies are emerging, which shed some light on the origins of these complications. A better characterisation of the end-organ consequences of the Fontan circulation is necessary, which can then allow for development of specific methods for treatment. Ideally, the goal is to establish systematic strategies that might reduce or eliminate the development of these potentially life-threatening challenges.

scholarly journals Cardiac Effects of Lightning Strikes

2017 ◽  
Vol 6 (3) ◽  
pp. 114 ◽  
Author(s):  
Theodoros Christophides ◽  
Sarosh Khan ◽  
Mahmood Ahmad ◽  
Hossam Fayed ◽  
Richard Bogle ◽  
...  
Keyword(s):  
Electrical Current ◽  
Aortic Injury ◽  
Multiple Organ ◽  
Myocardial Contusion ◽  
Pericardial Disease ◽  
Lightning Strikes ◽  
Cardiac Effects ◽  
Cardiac Implantable Electronic Devices ◽  
Organ Systems ◽  
Life Threatening

Lightning strikes are a common and leading cause of morbidity and mortality. Multiple organ systems can be involved, though the effects of the electrical current on the cardiovascular system are one of the main modes leading to cardiorespiratory arrest in these patients. Cardiac effects of lightning strikes can be transient or persistent, and include benign or life-threatening arrhythmias, inappropriate therapies from cardiac implantable electronic devices, cardiac ischaemia, myocardial contusion, pericardial disease, aortic injury, as well as cardiomyopathy with associated ventricular failure. Prolonged resuscitation can lead to favourable outcomes especially in young and previously healthy victims.

scholarly journals Precision genetic cellular models identify therapies protective against endoplasmic reticulum stress

2020 ◽  
Author(s):  
Irina V. Lebedeva ◽  
Michelle V. Wagner ◽  
Sunil Sahdeo ◽  
Yi-Fan Lu ◽  
Anuli Anyanwu-Ofili ◽  
...  
Keyword(s):  
Endoplasmic Reticulum ◽  
Er Stress ◽  
Drug Screening ◽  
Multiple Organ ◽  
Cellular Models ◽  
Intractable Seizures ◽  
Organ Systems ◽  
Life Threatening ◽  
Molecular Phenotypes ◽  
Novel Drug

AbstractCongenital disorders of glycosylation (CDG) and deglycosylation (CDDG) are a collection of rare pediatric disorders with symptoms that range from mild to life threatening. They typically affect multiple organ systems and usually present with neurological abnormalities including hypotonia, cognitive impairment, and intractable seizures. Several genes have been implicated in the thirty-six types of CDG, but currently NGLY1 is the only known CDDG gene. A common biological mechanism among CDG types and in CDDG is endoplasmic reticulum (ER) stress. Here, we develop two isogenic human cellular models of CDG (PMM2, the most prevalent type of CDG, and DPAGT1) and of the only CDDG (NGLY1) in an effort to identify drugs that can alleviate ER stress. Systematic phenotyping identified elevated ER stress and autophagy levels among other cellular and morphological phenotypes in each of the cellular models. We screened a complex drug library for compounds able to correct aberrant morphological phenotypes in each of the models using an agnostic phenotypic cell painting assay based on >300 cellular features. The image-based screen identified multiple candidate compounds able to correct aberrant morphology, and we show a subset of these are able to correct cellular and molecular defects in each of the models. These results provide new directions for the treatment of rare diseases of glycosylation and deglycosylation and a framework for new drug screening paradigms for more common neurodegenerative diseases characterized by ER stress.Summary sentenceNovel drug screening modality identifies compounds that correct aberrant molecular phenotypes in precision cellular models of glycosylation defects.

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