Developing an adolescent and adult Fontan Management Programme

2021 ◽  
pp. 1-6
Author(s):  
Adam M. Lubert ◽  
Tarek Alsaied ◽  
Andrew T. Trout ◽  
Jonathan R. Dillman ◽  
Joseph J. Palermo ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Multiple Organ ◽  
Fontan Circulation ◽  
Management Programme ◽  
Adverse Outcomes ◽  
Cardiac Complications ◽  
Organ Systems ◽  
Adolescents And Adults ◽  
Care Models

Abstract Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.

scholarly journals Fontan circulation and implications for future reproduction

2016 ◽  
Vol 10 (3) ◽  
pp. 104-106
Author(s):  
Niharika Mehta ◽  
Srilakshmi Mitta
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Cardiac Complications ◽  
Preconception Counseling ◽  
Passive Flow ◽  
History Of ◽  
Future Reproduction ◽  
Long Term Prognosis

This article summarizes and critiques four recent publications looking at preconception counseling, pregnancy outcomes, and cardiac complications in women with history of Fontan circulation. The Fontan procedure is a palliative strategy for single-ventricle type congenital heart disease and involves passive flow of venous return into the pulmonary circulation, bypassing the ventricles. Pregnancy in these patients is not without risk and preconception counseling and contraception practices vary widely. High rates of miscarriage, prematurity, and small-for-gestational-age babies are reported. Cardiac complications include mainly arrhythmias. Whether long-term prognosis in these patients is affected by pregnancy is not yet known.

scholarly journals The Biologic Syndrome of Frailty in Heart Failure

2014 ◽  
Vol 8s1 ◽  
pp. CMC.S15720 ◽  
Author(s):  
Rita Jermyn ◽  
Snehal Patel
Keyword(s):  
Heart Failure ◽  
Cardiovascular Disease ◽  
Multiple Organ ◽  
Adverse Outcomes ◽  
Risk Scores ◽  
Subjective Symptoms ◽  
Organ Systems ◽  
End Stage Heart Failure ◽  
End Stage ◽  
Current Risk

As we continue to care for an older and sicker end-stage heart failure population, it has become challenging to evaluate patients based on current risk scores that mainly focus on subjective symptoms and patient disability. For generations, geriatricians have sought to identify the body's underlying vulnerabilities that characterize frailty. More recently, cardiologists have begun to recognize this entity in their own practice. Several studies have suggested rates of frailty as high as 50% in patients with cardiovascular disease. However, despite recognizing frailty, it remains difficult to define. Like heart failure, frailty is a biologic syndrome that affects multiple organ systems. Measures of frailty are shown to strongly correlate with adverse outcomes in the health care system.

scholarly journals A review on tramadol toxicity: mechanism of action, clinical presentation, and treatment

2021 ◽  
Author(s):  
Samaneh Nakhaee ◽  
Christopher Hoyte ◽  
Richard C. Dart ◽  
Masoumeh Askari ◽  
Roland J. Lamarine ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Mechanism Of Action ◽  
Cardiopulmonary Arrest ◽  
Endocrine System ◽  
Multiple Organ ◽  
Adverse Outcomes ◽  
Low Grade ◽  
Organ Systems ◽  
Management Alternative

Abstract Aims As an analgesic that acts upon the central nervous system (CNS), tramadol has gained popularity in treating moderate to severe pain. Recently, it has been increasingly reported as a drug of misuse with intentional overdoses or intoxications. This review focuses on tramadol intoxication in humans and its effects on different systems. Subject and method This narrative review provides a comprehensive view of the pharmacokinetics, mechanism of action, and incidence of tramadol toxicity with an in-depth look at its side effects. In addition, the main approaches to the management of tramadol poisoning are described. Results Tramadol poisoning can affect multiple organ systems: gastrointestinal, central nervous system (seizure, CNS depression, low-grade coma, anxiety, and over time anoxic brain damage), cardiovascular system (palpitation, mild hypertension to life-threatening complications such as cardiopulmonary arrest), respiratory system, renal system (renal failure with higher doses of tramadol intoxication), musculoskeletal system (rhabdomyolysis), endocrine system (hypoglycemia), as well as, cause serotonin syndrome. Seizure, a serious nervous disturbance, is more common in tramadol intoxication than with other opioids. Fatal tramadol intoxications are uncommon, except in ingestion cases concurrent with other medications, particularly CNS depressants, most commonly benzodiazepines, and ethanol. Conclusion With the increasing popularity of tramadol, physicians must be aware of its adverse effects, substantial abuse potential, and drug interactions, to weigh its risk–benefit ratio for pain management. Alternative therapies might be considered in patients with a previous overdose history to reduce risks for adverse outcomes.

scholarly journals Galectin‐3 Is Elevated and Associated With Adverse Outcomes in Patients With Single‐Ventricle Fontan Circulation

2016 ◽  
Vol 5 (1) ◽  
Author(s):  
Alexander R. Opotowsky ◽  
Fernando Baraona ◽  
Justin Owumi ◽  
Brittani Loukas ◽  
Michael N. Singh ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Fontan Circulation ◽  
Adverse Outcomes ◽  
Galectin 3

scholarly journals Patient and Family Experience in a Multidisciplinary Clinic for Children With Single-Ventricle Heart Disease

2020 ◽  
Vol 7 (6) ◽  
pp. 1384-1390
Author(s):  
Kelly R Wolfe ◽  
Sarah L Kelly ◽  
Deborah R Liptzin ◽  
Dania Brigham ◽  
Carey Rafferty ◽  
...  
Keyword(s):  
Heart Disease ◽  
Single Ventricle ◽  
Multidisciplinary Care ◽  
Multiple Organ ◽  
Care Needs ◽  
Family Experience ◽  
Overall Acceptability ◽  
Organ Systems ◽  
Perceived Benefit ◽  
Multidisciplinary Clinic

Children with single-ventricle heart disease (SVHD) are at risk for morbidity across multiple organ systems. A single-ventricle multidisciplinary clinic (SVMDC) may address complex health-care needs by providing access to, and coordination among, pediatric subspecialties. However, the patient and family experience of multidisciplinary care for SVHD remains unexplored. We e-mailed a 26-question survey to families after an SVMDC visit, which included evaluation with subspecialists from cardiology, pulmonology, gastroenterology, neuropsychology, and pediatric psychology, as well as social activities during clinic. Responses were anonymized to protect privacy, and data were analyzed quantitatively and qualitatively. Over 3 years, 22% (27/122) of families completed the survey. Overall, families’ experiences were positive, with 100% reporting that they would recommend the SVMDC to others. Qualitative themes emerged regarding logistics, multidisciplinary care, key takeaways from clinic, and connection-making with other families. A multidisciplinary clinic demonstrated overall acceptability and perceived benefit to families of children with SVHD. Considerations for mixed experiences regarding financial commitment and connection-making among parents are discussed, as are the benefits of the synergy achieved through multidisciplinary care.

Microprobe analysis of inclusion bodies related to two benzofuran derivatives

1987 ◽  
Vol 45 ◽  
pp. 672-673
Author(s):  
T. L. Benning ◽  
P. Ingram ◽  
J. D. Shelburne
Keyword(s):  
Inclusion Bodies ◽  
Uranyl Acetate ◽  
Multiple Organ ◽  
High Dose ◽  
En Bloc ◽  
Tissue Samples ◽  
Transmission Electron ◽  
Organ Systems ◽  
Dense Inclusion ◽  
Melanin Complex

Two benzofuran derivatives, chlorpromazine and amiodarone, are known to produce inclusion bodies in human tissues. Prolonged high dose chlorpromazine therapy causes hyperpigmentation of the skin with electron-dense inclusion bodies present in dermal histiocytes and endothelial cells ultrastructurally. The nature of the deposits is not known although a drug-melanin complex has been hypothesized. Amiodarone may also cause cutaneous hyperpigmentation and lamellar lysosomal inclusion bodies have been demonstrated within the cells of multiple organ systems. These lamellar bodies are believed to be the product of an amiodarone-induced phospholipid storage disorder. We performed transmission electron microscopy (TEM) and energy dispersive x-ray microanalysis (EDXA) on tissue samples from patients treated with these drugs, attempting to detect the sulfur atom of chlorpromazine and the iodine atom of amiodarone within their respective inclusion bodies.A skin biopsy from a patient with hyperpigmentation due to prolonged chlorpromazine therapy was fixed in 4% glutaraldehyde and processed without osmium tetroxide or en bloc uranyl acetate for Epon embedding.

Impairment Rating and Spinal Cord Injuries: Revisiting the Guides

2010 ◽  
Vol 15 (3) ◽  
pp. 1-7
Author(s):  
Richard T. Katz
Keyword(s):  
Spinal Cord ◽  
Spinal Cord Injuries ◽  
Functional Independence ◽  
Outcome Data ◽  
Multiple Organ ◽  
Loss Of Function ◽  
Sixth Edition ◽  
Organ Systems ◽  
Cord Injury ◽  
Impairment Ratings

Abstract This article addresses some criticisms of the AMA Guides to the Evaluation of Permanent Impairment (AMA Guides) by comparing previously published outcome data from a group of complete spinal cord injury (SCI) persons with impairment ratings for a corresponding level of injury calculated using the AMA Guides, Sixth Edition. Results of the comparison show that impairment ratings using the sixth edition scale poorly with the level of impairments of activities of daily living (ADL) in SCI patients as assessed by the Functional Independence Measure (FIM) motor scale and the extended FIM motor scale. Because of the combinations of multiple impairments, the AMA Guides potentially overrates the impairment of paraplegics compared with that of quadriplegics. The use and applicability of the Combined Values formula should be further investigated, and complete loss of function of two upper extremities seems consistent with levels of quadriplegia using the SCI model. Some aspects of the AMA Guides contain inconsistencies. The concept of diminishing impairment values is not easily translated between specific losses of function per organ system and “overall” loss of ADLs involving multiple organ systems, and the notion of “catastrophic thresholds” involving multiple organ systems may support the understanding that variations in rating may exist in higher rating cases such as those that involve an SCI.

Renal Manifestations of Tuberous Sclerosis Complex

2020 ◽  
Vol 7 (3) ◽  
pp. 5-19
Author(s):  
Nikhil Nair ◽  
Ronith Chakraborty ◽  
Zubin Mahajan ◽  
Aditya Sharma ◽  
Sidarth Sethi ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Gene Disruption ◽  
Treatment Guidelines ◽  
Renal Cysts ◽  
Skin Lesions ◽  
Multiple Organ ◽  
Tsc2 Gene ◽  
Genetic Condition ◽  
Organ Systems

Tuberous sclerosis complex (TSC) is a genetic condition caused by a mutation in either the TSC1 or TSC2 gene. Disruption of either of these genes leads to impaired production of hamartin or tuberin proteins, leading to the manifestation of skin lesions, tumors and seizures. TSC can manifests in multiple organ systems with the cutaneous and renal systems being the most commonly affected. These manifestations can secondarily lead to the development of hypertension, chronic kidney disease, and neurocognitive declines. The renal pathologies most commonly seen in TSC are angiomyolipoma, renal cysts and less commonly, oncocytomas. In this review, we highlight the current understanding on the renal manifestations of TSC along with current diagnosis and treatment guidelines.

scholarly journals Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry

2021 ◽  
pp. 1-9
Author(s):  
Lars Søndergaard ◽  
Jamil Aboulhosn ◽  
Yves d’Udekem ◽  
Céline Faure ◽  
Wayne J Franklin ◽  
...  
Keyword(s):  
Creatinine Clearance ◽  
Receptor Antagonist ◽  
Functional Class ◽  
Fontan Circulation ◽  
Endothelin Receptor Antagonist ◽  
Endothelin Receptor ◽  
Converting Enzyme Inhibitors ◽  
Nyha Functional Class ◽  
Lateral Tunnel ◽  
Adolescents And Adults

Abstract The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with “stable” Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening. Patient characteristics at enrolment are described; available data were collected during a single registration visit. Of the 266 screened patients, 254 were included in this analysis. At enrolment, median (interquartile range) age was 24 (20;30) years, 37%/63% of patients were from the USA/Europe, 54% were male, 54%/47% had undergone extra-cardiac conduit/lateral tunnel procedures, and 95% were in NYHA functional class I or II. History of arrhythmia was more common in older patients and patients with lateral tunnel; overall prevalence was 19%. Most laboratory values were within the normal range but mean creatinine clearance was abnormally low (87.7 ml/min). Angiotensin-converting enzyme inhibitors were used by 48% of patients and their use was associated with creatinine clearance <90 ml/min (p = 0.007), as was Fontan completion at an older age (p = 0.007). 53.4% of patients had clinical characteristics that could potentially meet an endothelin receptor antagonist trial’s eligibility criteria. The PREpArE-Fontan registry describes a cohort of patients who could potentially participate in an endothelin receptor antagonist trial and identified early subtle signs of Fontan failure, even in “stable” patients.

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