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 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)

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.

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.

Effective use of corticosteroid in a child with life-threatening plastic bronchitis after Fontan operation

2003 ◽  
Vol 45 (1) ◽  
pp. 107-109 ◽  
Author(s):  
YOICHI ONOUE ◽  
YUICHI ADACHI ◽  
FUKIKO ICHIDA ◽  
TOSHIO MIYAWAKI
Keyword(s):  
Fontan Operation ◽  
Plastic Bronchitis ◽  
Life Threatening ◽  
Effective Use

scholarly journals Trauma-Induced Coagulopathy: Overview of an Emerging Medical Problem from Pathophysiology to Outcomes

Medicines ◽  
2021 ◽  
Vol 8 (4) ◽  
pp. 16
Author(s):  
Gabriele Savioli ◽  
Iride Francesca Ceresa ◽  
Luca Caneva ◽  
Sebastiano Gerosa ◽  
Giovanni Ricevuti
Keyword(s):  
Major Trauma ◽  
Management Strategies ◽  
Medical Problem ◽  
Current Evidence ◽  
Potential Drug ◽  
The Past ◽  
Trauma Induced Coagulopathy ◽  
Threatening Condition ◽  
Life Threatening ◽  
Trauma Mortality

Coagulopathy induced by major trauma is common, affecting approximately one-third of patients after trauma. It develops independently of iatrogenic, hypothermic, and dilutive causes (such as iatrogenic cause in case of fluid administration), which instead have a pejorative aspect on coagulopathy. Notwithstanding the continuous research conducted over the past decade on Trauma-Induced Coagulopathy (TIC), it remains a life-threatening condition with a significant impact on trauma mortality. We reviewed the current evidence regarding TIC diagnosis and pathophysiological mechanisms and summarized the different iterations of optimal TIC management strategies among which product resuscitation, potential drug administrations, and hemostatis-focused approaches. We have identified areas of ongoing investigation and controversy in TIC management.

scholarly journals Prognostic Value of Liver and Spleen Stiffness in Patients with Fontan Associated Liver Disease (FALD): A Case Series with Histopathologic Comparison

2021 ◽  
Vol 8 (3) ◽  
pp. 30
Author(s):  
Massimo Padalino ◽  
Liliana Chemello ◽  
Luisa Cavalletto ◽  
Annalisa Angelini ◽  
Marny Fedrigo
Keyword(s):  
Portal Hypertension ◽  
Liver Disease ◽  
Liver Fibrosis ◽  
Prognostic Value ◽  
Fontan Operation ◽  
Vena Cava ◽  
Case Series ◽  
Morbidity And Mortality ◽  
Post Mortem ◽  
Spleen Stiffness

The Fontan operation is the current surgical procedure to treat single-ventricle congenital heart disease, by splitting the systemic and pulmonary circulations and thus permitting lifespan to adulthood for the majority of newborns. However, emerging data are showing that Fontan-associated liver disease (FALD) is an increasing related cause of morbidity and mortality in patients with the Fontan circuit. We described the clinical, laboratory, and transient elastography (TE) findings in a case series of adults with the Fontan circuit, and also correlated data with post-mortem histological features, aimed to define the prognostic value of TE in the staging of FALD. All patients presented signs of a long-standing Fontan failure, characterized by reoperation need, systemic ventricle dysfunction, and FALD stigmata (liver and spleen enlargement, portal vein and inferior vena cava dilation, and abnormal liver function tests). Liver and spleen stiffness (LS and SS) values were indicative of significant liver fibrosis/cirrhosis and the presence of suggestive portal hypertension (LS mean 35.9; range 27.3–44.7 kPa; SS mean 42.1, range 32.2–54.5 kPa). Post-mortem evaluations confirmed a gross hepatic architecture distortion in all cases. All patients died from severe complications related to liver dysfunction and bleeding. TE correlated well with pathological findings and FALD severity. We propose this validated and harmless technique to monitor liver fibrosis extension and portal hypertension over time in Fontan patients, and to identify the optimal timing for surgical reoperations or orthotopic-heart transplantation (OHT), avoiding a higher risk of morbidity and mortality in cases with severe FALD.

scholarly journals Pathological and Immunological Approaches for a Patient Complicated with Protein-losing Enteropathy Following the Fontan Operation

2012 ◽  
Vol 28 (4) ◽  
pp. 224-229
Author(s):  
Kentaro Ueno ◽  
Hideaki Nakamura ◽  
Ryohei Gatayama ◽  
Sadamitsu Yanagi ◽  
Hideaki Ueda ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Protein Losing Enteropathy

scholarly journals Myelodysplastic Syndrome Clinically Presenting with the “Classic TTP Pentad”

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Santiago Fabián Moscoso Martínez ◽  
Evelyn Carolina Polanco Jácome ◽  
Elizabeth Guevara ◽  
Vijay Mattoo
Keyword(s):  
Myelodysplastic Syndrome ◽  
Clinical Presentation ◽  
Renal Involvement ◽  
Medical Emergency ◽  
Complete Blood Cell Count ◽  
Blood Cell Count ◽  
Thrombocytopenic Purpura ◽  
Life Saving ◽  
Threatening Condition ◽  
Life Threatening

The clinical presentation of myelodysplastic syndrome (MDS) is not specific. Many patients can be asymptomatic and can be detected only due to an abnormal complete blood cell count (CBC) on routine exam or for other reasons while others can be symptomatic as a consequence of underlying cytopenias. Thrombotic thrombocytopenic purpura (TTP) usually is suspected under the evidence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia and because it is a life-threatening condition (medical emergency) immediate initiation of plasmapheresis could be life-saving. The following case illustrates an unusual presentation of MDS in a patient who came in to the emergency room with the classic TTP “pentad” of fever, renal involvement, MAHA, mental status changes, and thrombocytopenia. We will focus our discussion in the clinical presentation of this case.

scholarly journals Peripheral Arteriovenous Malformations: Imaging and Endovascular Management Strategies

2021 ◽  
Author(s):  
Virender Malik ◽  
Harshith Kramadhari ◽  
Jawahar Rathod ◽  
Yadav W. Munde ◽  
Uday Bhanu Kovilapu
Keyword(s):  
Arteriovenous Malformation ◽  
Vascular Malformation ◽  
Management Strategies ◽  
Treatment Strategies ◽  
Vascular Anomaly ◽  
Classification Systems ◽  
Vascular Anomalies ◽  
Final Decision ◽  
Life Threatening

AbstractThe peripheral high-flow vascular malformation (HFVM) comprises arteriovenous malformation (AVM) and fistula (AVF), shows varied clinical presentation (ranging from subtle skin lesion to life-threatening congestive heart failure), and frequently poses diagnostic and therapeutic challenges. Importance of assigning a specific diagnosis to the vascular malformation cannot be overstated, as the treatment strategy is based on the type of vascular anomaly. Although the International Society for the Study of Vascular Anomalies (ISSVA) classification system is the most commonly accepted system for classifying congenital vascular anomalies in clinical practice, the Cho–Do et al classification is of utmost help in guiding optimal mode of treatment in peripheral AVM. Although transarterial approach remains the most commonly employed route for peripheral AVM embolization, the role of transvenous and direct percutaneous approach is ever increasing and the final decision on the approach depends on angioarchitecture of the AVM. In this article, we review various commonly employed classification systems for congenital vascular anomalies, and describe clinical features, imaging and treatment strategies for peripheral arteriovenous malformation (PAVM).

Cardiac Arrhythmia Detection using Dual Tree Wavelet Transform and Convolutional Neural Network

2021 ◽  
Author(s):  
K Reddy Madhavi ◽  
Padmavathi kora ◽  
L Venkateswara Reddy ◽  
J Avanija ◽  
KLS Soujanya ◽  
...  
Keyword(s):  
Neural Network ◽  
Wavelet Transform ◽  
Cardiac Cells ◽  
P Wave ◽  
Computer Assisted ◽  
Ecg Signals ◽  
Coronary Diseases ◽  
Life Saving ◽  
Life Threatening ◽  
Assisted Diagnosis

Abstract The non-stationary ECG signals are used as a key tools in screening coronary diseases. ECG recording is collected from millions of cardiac cells’ and depolarization and re-polarization conducted in a synchronized manner as: The P-wave occurs first, followed by the QRScomplex and the T-wave, which will repeat in each beat. The signal is altered in a cardiac beat period for different heart conditions. This change can be observed in order to diagnose the patient’s heart status. There are life-threatening (critical) and non-life - threatening (noncritical) arrhythmia (abnormal Heart). Critical arrhythmia gives little time for surgery, whereas non-critical needs additional life-saving care. Simple naked eye diagnosis can mislead the detection. At that point, Computer Assisted Diagnosis (CAD) is therefore required. In this paper Dual Tree Wavelet Transform (DTWT) used as a feature extraction technique along with Convolution Neural Network (CNN) to detect abnormal Heart. The findings of this research and associated studies are without any cumbersome artificial environments. The CAD method proposed has high generalizability; it can help doctors efficiently identify diseases and decrease misdiagnosis.

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