Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation

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)

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Rare problems associated with the Fontan circulation

Cardiology in the Young ◽

10.1017/s1047951110001162 ◽

2010 ◽

Vol 20 (S3) ◽

pp. 113-119 ◽

Cited By ~ 35

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.

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

Cardiology in the Young ◽

10.1017/s1047951111001636 ◽

2011 ◽

Vol 21 (S2) ◽

pp. 77-79 ◽

Cited By ~ 6

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.

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Tissue Plasminogen Activator Release in Chronic Venous Hypertension due to Heart Failure

Thrombosis and Haemostasis ◽

10.1055/s-0038-1656373 ◽

1992 ◽

Vol 68 (03) ◽

pp. 321-324 ◽

Cited By ~ 3

Author(s):

Irena Keber ◽

Dušan Keber ◽

Mojca Stegnar ◽

Nina Vene

Keyword(s):

Heart Failure ◽

Plasminogen Activator ◽

Tissue Plasminogen Activator ◽

Fibrinolytic Activity ◽

Venous Pressure ◽

Venous Hypertension ◽

Plasminogen Activator Inhibitor 1 ◽

Control Subjects ◽

Tissue Plasminogen ◽

Pai 1

SummaryIn order to study the effects of chronic venous hypertension due to heart failure on blood fibrinolytic activity, tissue plasminogen activator (t-PA) antigen, plasminogen activator inhibitor 1 (PAI-1) antigen, t-PA activity and PAI activity were measured before and after venous occlusion of the arm for 20 min in 15 patients with right-sided heart failure, 15 patients with left-sided heart failure, and 30 control healthy subjects. Central venous pressure, measured by observing the jugular veins, was above 15 cm of the blood column in all patients with right-sided heart failure, and normal (below 8 cm) in all patients with left-sided heart failure and control subjects. There was no difference in the basal concentrations of t-PA (11.0, 10.2 and 10.8 ng/ml; all values medians) and PAI-1 antigens and their activities between right and left-sided heart failure and the control subjects. After the occlusion, t-PA antigen increased significantly less in right-sided heart failure (28.6 ng/ml) than in left-sided heart failure and the control subjects (54.5 and 45.9 ng/ml, respectively). It was concluded that the poor increase in fibrinolytic activity that had already been reported in patients with heart failure, was due to low t-PA release during occlusion and not to a high basal PAI level. It was limited to the patients with right-sided heart failure and was probably the consequence of chronic systemic venous hypertension.

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Fluid dynamics in chronic congestive heart failure: An interpretation of the mechanisms producing the edema, increased plasma volume and elevated venous pressure in certain patients with prolonged congestive failure

American Heart Journal ◽

10.1016/s0002-8703(44)90340-6 ◽

1944 ◽

Vol 27 (6) ◽

pp. 877-878

Keyword(s):

Fluid Dynamics ◽

Heart Failure ◽

Congestive Heart Failure ◽

Plasma Volume ◽

Venous Pressure ◽

Chronic Congestive Heart Failure ◽

Congestive Failure

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Early Surgical Intervention for Infective Endocarditis

Asian Cardiovascular and Thoracic Annals ◽

10.1177/021849230201000403 ◽

2002 ◽

Vol 10 (4) ◽

pp. 298-301 ◽

Cited By ~ 1

Author(s):

Hong Sheng Zhu ◽

Pei Yan Yao ◽

Jia Hao Zheng ◽

A Thomas Pezzella

Keyword(s):

Heart Failure ◽

Congestive Heart Failure ◽

Heart Disease ◽

Infective Endocarditis ◽

Complex Disease ◽

Surgical Intervention ◽

Elective Surgery ◽

Postoperative Bleeding ◽

Arterial Embolization ◽

Early Surgical Intervention

Infective endocarditis remains a serious and complex disease with significant morbidity and mortality. Sixty cases of infective endocarditis were retrospectively reviewed, consisting of 41 males and 19 females aged 7 to 50 years (mean, 30 years). Congenital heart disease was diagnosed in 19 of the patients and rheumatic heart disease in 41. Congestive heart failure occurred in 36 and systemic embolism in 8 cases. Blood cultures were positive in only 21.7% of the cases, while vegetations were detected by 2-dimensional echocardiography in 70%. Elective surgery was performed in 57 patients and emergent operation for systemic arterial embolization and/or intractable congestive heart failure in 3 patients. Two patients required reoperation for postoperative bleeding. All but 2 patients had been followed up for 6 to 160 months with no evidence of reinfection. Three patients with mechanical valve implantation later died of intracranial bleeding due to over-anticoagulation. The remaining 55 resumed normal activity. The encouraging outcomes were the result of an aggressive diagnostic approach and early surgical intervention.

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Comparison of Iodixanol and Iopromide in Patients With Renal Insufficiency and Congestive Heart Failure Undergoing Coronary Angiography by Hemodynamic Monitoring

Angiology ◽

10.1177/0003319717701868 ◽

2017 ◽

Vol 68 (10) ◽

pp. 907-913 ◽

Cited By ~ 2

Author(s):

Geng Qian ◽

Yong-qiang Yang ◽

Wei Dong ◽

Feng Cao ◽

Yun-dai Chen

Keyword(s):

Heart Failure ◽

Congestive Heart Failure ◽

Contrast Media ◽

Hemodynamic Monitoring ◽

Venous Pressure ◽

Cardiac Preload ◽

Cardiac Events ◽

Lung Water ◽

Water Index ◽

The Impact

We investigated the impact of contrast media (CM) with different osmolality on cardiac preload in patients with chronic kidney disease (CKD) and congestive heart failure (CHF). Patients with CKD and CHF were equally randomized to receive either iso-osmolar contrast media (IOCM) iodixanol or low-osmolar contrast media iopromide. We measured cardiac preload indexes by invasive hemodynamic monitoring before and after CM injection. Major adverse cardiac events postprocedures were recorded. Increase in extravascular lung water index was only seen in the iopromide group ( P < .001), while global end diastolic index and central venous pressure were all significantly increased from baseline in the both groups ( P < .001, respectively), and the increase in cardiac preload indexes was significantly greater in the iopromide group than in the iodixanol group ( P < 0.001). The overall incidence of acute heart failure was more frequently observed in the iopromide group ( P = 0.027). Low-osmolar contrast media iopromide significantly increased cardiac preload in patients with CKD and CHF undergoing cardiac catheterization procedures compared with IOCM iodixanol.

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Current treatment options for the Failing Fontan Circulation

Current Cardiology Reviews ◽

10.2174/1573403x18666220106114518 ◽

2022 ◽

Vol 18 ◽

Author(s):

Bart. W. Driesen ◽

Michiel Voskuil ◽

Heynric B. Grotenhuis

Keyword(s):

Myocardial Dysfunction ◽

Fontan Procedure ◽

Treatment Options ◽

Fontan Operation ◽

Current Treatment ◽

Fontan Circulation ◽

Protein Losing Enteropathy ◽

Resistance Protein ◽

Failing Fontan

Abstract: The Fontan operation was introduced in 1968. For congenital malformations where biventricular repair is not suitable, the Fontan procedure has provided a long-term palliation strategy with improved outcome compared to the initially developed procedures. Despite these improvements, several complications merely as a result of a failing Fontan circulation (including myocardial dysfunction, arrhythmias, increased pulmonary vascular resistance, protein losing enteropathy, hepatic dysfunction, plastic bronchitis and thrombo-embolism) will limit life-expectancy in this patient cohort. This review provides an overview of the most common complications of the Fontan circulation and the currently available treatment options.

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Fontan-associated liver disease and hepatocellular carcinoma in adults

Scientific Reports ◽

10.1038/s41598-020-78840-y ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Tomomi Kogiso ◽

Katsutoshi Tokushige

Keyword(s):

Hepatocellular Carcinoma ◽

Liver Disease ◽

Fontan Procedure ◽

Fontan Operation ◽

Venous Pressure ◽

Palliative Therapy ◽

Nationwide Survey ◽

Venous Drainage ◽

Cardiac Complications ◽

Sinusoidal Dilatation

AbstractThe Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This causes congestion and hypoxia in the liver, leading to Fontan-associated liver disease (FALD). Non-invasive and invasive markers enable diagnosis and evaluation of the fibrosis status in chronic liver disease; however, these markers have not been validated in FALD. Additionally, regenerative nodules such as focal nodular hyperplasia (FNH) are frequently found. The severity of fibrosis correlates with the duration of the Fontan procedure and the central venous pressure. Cirrhosis is a risk factor for hepatocellular carcinoma (HCC), the annual risk of which is 1.5–5.0%. HCC is frequently difficult to diagnose and treat because of cardiac complications, coagulopathy, and congenital abnormalities. The mortality rate of FALD with liver cirrhosis and/or FALD-HCC was increased to ~ 29.4% (5/17 cases) in a nationwide survey. Although there is no consensus on the surveillance of patients with FALD, serial monitoring of the alpha fetoprotein level and imaging at 6-month intervals is required in patients with cirrhosis.

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Transplant-Amenable Hepatocellular Carcinoma in a Fontan Patient

Case Reports in Gastroenterology ◽

10.1159/000501198 ◽

2019 ◽

Vol 13 (2) ◽

pp. 275-279 ◽

Cited By ~ 2

Author(s):

Brian Patrick Rutledge ◽

Anupama Devara ◽

Sindhuri Benjaram ◽

Stephanie Judd ◽

Murray Ehrinpreis

Keyword(s):

Hepatocellular Carcinoma ◽

At Risk ◽

Liver Transplant ◽

Fontan Procedure ◽

Venous Pressure ◽

Fontan Patient ◽

Number Of Patients ◽

Fontan Physiology ◽

The Ideal

The Fontan circulation alters a patient’s physiology and imparts long-term risks related to chronically elevated systemic venous pressure. An increasing number of patients with Fontan physiology are surviving into adulthood and are at risk of hepatic sequalae. The ideal timeline and method of hepatic surveillance in the Fontan population remains to be defined. In this case, the patient was diagnosed with hepatocellular carcinoma more than 20 years after undergoing the Fontan procedure and was a candidate for combined heart-liver transplant. That her disease progressed prior to organ availability supports the argument for hepatic surveillance in this population.

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