Lymphatic Disorders and Management in Patients With Congenital Heart Disease

Tomasulo, Catherine E.; Chen, Jonathan M.; Smith, Christopher L.; Maeda, Katsuhide; Rome, Jonathan J.; Dori, Yoav

doi:10.1016/j.athoracsur.2020.10.058

Cited by 29 publications

(29 citation statements)

References 49 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Immunologic complications of Fontan physiology can include hypogammaglobulinemia and lymphopenia, particularly of naïve T-cells. In patients with PLE, secondary intestinal lymphangectasia leads to loss of serum proteins and lymphocytes [ 25 ]. While production of lymphocytes and immunoglobulin is normal, in those with PLE, loss into the bowel surpasses the rate of production.…”

Section: The Multidisciplinary Approach: Areas Of Focusmentioning

confidence: 99%

A Path FORWARD: Development of a Comprehensive Multidisciplinary Clinic to Create Health and Wellness for the Child and Adolescent with a Fontan Circulation

et al. 2022

View full text Add to dashboard Cite

Today, it is anticipated most individuals diagnosed with single-ventricle malformation will survive surgical reconstruction through a successful Fontan operation. As greater numbers of patients survive, so has the recognition that individuals with Fontan circulation face a variety of challenges. The goal of a normal quality and duration of life will not be reached by all. The hurdles fall into a variety of domains. From a cardiovascular perspective, the Fontan circulation is fundamentally flawed by its inherent nature of creating a state of chronically elevated venous pressure and congestion, accompanied by a relatively low cardiac output. Ventricular dysfunction, atrioventricular valve regurgitation, and arrhythmia may directly impact cardiac performance and can progress with time. Problems are not limited to the cardiovascular system. Fontan circulatory physiology impacts a multitude of biological processes and health parameters outside the heart. The lymphatic circulation is under strain manifesting as variable degrees of protein-rich lymph loss and immune system dysregulation. Organ system dysfunction develops through altered perfusion profiles. Liver fibrosis is ubiquitous, and a process of systemic fibrogenesis in response to circulatory stressors may affect other organs as well. Somatic growth and development can be delayed. Behavioral and mental health problems are common, presenting as clinically important levels of anxiety and depression. Most striking is the high variability in prevalence and magnitude of these complications within the population, indicating the likelihood of additional factors enhancing or mitigating their emergence. We propose that optimal care for the individual with single ventricle and a Fontan circulation is ideally offered in a comprehensive multidisciplinary manner, with attention to elements that are beyond cardiac management alone. In this report, we share the concepts, our experiences, and perspectives on development of a clinic model—the “Fontan rehabilitation, wellness and resilience development” or FORWARD program. We provide insights into the mechanics of our multidisciplinary model of care and the benefits offered serving our growing population of individuals with a Fontan circulation and their families.

show abstract

Section: The Multidisciplinary Approach: Areas Of Focusmentioning

confidence: 99%

A Path FORWARD: Development of a Comprehensive Multidisciplinary Clinic to Create Health and Wellness for the Child and Adolescent with a Fontan Circulation

et al. 2022

View full text Add to dashboard Cite

show abstract

“…Medications, such as inotropes, have been shown to cause rhythmic lymphatic contractions in a dose-dependent manner (7). In patients with CHD, central venous pressure is particularly important as it increases lymphatic production, especially by the liver, and impedes lymphatic drainage at the lymphovenous junctions (8).…”

Section: Lymphatic Anatomy and Physiologymentioning

confidence: 99%

“…Lymphatic interventions in general can be divided into two groups, namely, those that are meant to occlude unwanted lymphatic ducts, such as thoracic duct embolization (TDE), and those that are meant to decompress the entire lymphatic system, such as lymphovenous anastomosis (LVA) (8). Minimally invasive procedures that occlude flow include ethiodized oil embolization, TDE, selective lymphatic duct embolization (SLDE), as well as several other procedures that are meant to selectively target lymphatic networks that are causing symptoms.…”

Section: Lymphatic Interventionsmentioning

confidence: 99%

Lymphatic Disorders in Patients With Single Ventricle Heart Disease

Dori

Smith

2022

Front. Pediatr.

Self Cite

View full text Add to dashboard Cite

Lymphatic abnormalities in patients with single ventricle physiology can lead to early Fontan failure and severe Fontan complications, such as protein-losing enteropathy (PLE), plastic bronchitis (PB), chylothorax, and edema. Recent developments in lymphatic imaging and interventions have shed new light on the lymphatic dysfunction in this patient population and the role of the lymphatic circulation in PLE, PB, and chylothorax. In this study, we reviewed some of the latest developments in this field and discuss new treatment options for these patients.

show abstract

“…or lymphangiomatosis [14,17,18]. While some patients develop chyloptysis without developing plastic bronchitis, lymphatic leakage into the airways has been strongly associated with plastic bronchitis [41,42].…”

Section: Casts In Structural Heart Diseasesmentioning

confidence: 99%

Recycling plastic: diagnosis and management of plastic bronchitis among adults

et al. 2021

View full text Add to dashboard Cite

Plastic bronchitis is a rare, underdiagnosed and potentially fatal condition. It is characterised by the formation and expectoration of branching gelatinous plugs that assume the shape of the airways. These airway plugs differ from the allergic mucin that characterises allergic bronchopulmonary aspergillosis and mucoid impaction of the bronchi. Plastic bronchitis is most often encountered in the paediatric population following corrective cardiac surgery, such as the Fontan procedure. It also occurs in adults. Plastic bronchitis in adults is rare, heterogeneous in its aetiology, and can lead to respiratory distress or even life-threatening airway obstruction. Plastic bronchitis in adulthood should not be overlooked, particularly in patients with chronic inflammatory lung diseases. This review presents current understanding of the presentation, aetiology, pathogenesis, pathology and management of plastic bronchitis in adults.

show abstract

Lymphatic Disorders and Management in Patients With Congenital Heart Disease

Cited by 29 publications

References 49 publications

A Path FORWARD: Development of a Comprehensive Multidisciplinary Clinic to Create Health and Wellness for the Child and Adolescent with a Fontan Circulation

A Path FORWARD: Development of a Comprehensive Multidisciplinary Clinic to Create Health and Wellness for the Child and Adolescent with a Fontan Circulation

Lymphatic Disorders in Patients With Single Ventricle Heart Disease

Recycling plastic: diagnosis and management of plastic bronchitis among adults

Contact Info

Product

Resources

About