Path ahead for ‘low risk’ adolescents living with a Fontan circulation

Heart ◽  
2020 ◽  
pp. heartjnl-2020-317619
Author(s):  
David W Baker ◽  
Mark R Dennis ◽  
Diana Zannino ◽  
Chris Schilling ◽  
Patricia D Moreno ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Ventricular Dysfunction ◽  
Fontan Circulation ◽  
Low Risk ◽  
Transition To Adult Care ◽  
Adult Care ◽  
Protein Losing Enteropathy ◽  
Atrioventricular Valve Regurgitation ◽  
Increased Risk ◽  
Fontan Failure

ObjectiveA high risk of morbidity and mortality is well documented in adults with a Fontan circulation. The difference in outcomes between those with and without significant morbidity at the time of transition to adult care has not been well characterised.MethodsWe analysed clinical outcomes in patients enrolled in the Australian and New Zealand Fontan Registry ≥16 years of age. Low risk (LR) Fontan patients were defined as those without history of sustained arrhythmia, thromboembolic event, transplantation, Fontan conversion, protein-losing enteropathy, plastic bronchitis, New York Heart Association class III/IV and/or moderate/severe atrioventricular valve regurgitation or ventricular dysfunction. Increased risk (IR) patients had one or more risk factor.ResultsInclusion criteria were met in 822 patients; mean age 26±8 years, median follow-up from age 16 was 9 years, 203 had atriopulmonary connection (APC) and 619 had total cavopulmonary connection (TCPC). Survival at 30 years was higher in the LR versus IR; 94% versus 82% (p=0.005), 89% versus 77% (p=0.07) for APC and 96% versus 89% (p=0.05) for TCPC. LR patients experienced less Fontan failure (HR 0.34, 95% CI 0.23 to 0.49, p<0.001) and ventricular dysfunction (HR 0.46, 95% CI 0.29 to 0.71, p=0.001) compared with IR patients. For LR TCPC patients, modelled survival projections at 60 years were 49%–67%.ConclusionsClinical outcomes for adolescents LR at transition to adult care are markedly superior to those who have established risk factors for Fontan failure, which is an important consideration when formulating individualised long-term risk estimates and counselling patients.

Abstract 17017: Risk Prediction of Death and Failure in Patients With Atrio-pulmonary Connections: 40 Years Experience in Australia and New Zealand

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Chin L Poh ◽  
Diana Zannino ◽  
Robert G Weintraub ◽  
David S Winlaw ◽  
Dorothy J Radford ◽  
...  
Keyword(s):  
New Zealand ◽  
Left Ventricular ◽  
Protein Losing Enteropathy ◽  
Severe Left Ventricular Dysfunction ◽  
Atrioventricular Valve Regurgitation ◽  
Increased Risk ◽  
Fontan Failure ◽  
Antiarrhythmic Medications ◽  
Fontan Conversion

Objective: To identify parameters predictive of death and failure in patients with an atrio-pulmonary connection Fontan. Methods: Retrospective analysis of long-term follow-up data using the Australia and New Zealand Fontan Registry Results: There were 245 patients surviving hospital discharge after an atriopulmonary Fontan from 1975 to 1994. At latest follow-up, 54 pts died, 29 underwent heart transplantation and 93 had Fontan failure (death, transplantation, Fontan conversion, protein losing enteropathy, severe left ventricular dysfunction on echocardiography or NYHA3). Twenty-eight years survival, freedom from death and transplantation and freedom from Fontan failure were respectively 62% (95%CI 55-71%), 58% (95% CI 50-67%) and 40% (95% CI 33-50%). Predictors of death and transplantation were dextrocardia (HR 1.8;p= 0.05), longer Fontan bypass time (HR= 1.01, p=0.05) and moderate or more late atrioventricular valve regurgitation (HR 3.97; p=0.02). One hundred and seven patients developed atrial arrhythmias, with first documented event at 15.7 years (IQR 10.5-19.3) post Fontan completion. Development of arrhythmia increased the likelihood of death or transplantation (HR 3.05, p<0.001) and Fontan failure (HR 4.7, p<0.001). Thromboembolic events occurred in 42 patients, 30 (71%) of whom were on warfarin but less than half (12/30) within therapeutic range. Thirty-nine patients underwent Fontan conversion. Threshold for conversion was lower in one center in which patients were operated earlier with better functional status, shorter history of arrhythmias and less antiarrhythmic medications. Conversion resulted in a reduced risk of Fontan failure (HR 0.48) in patients cared for by the center with lower conversion threshold but an increased risk in the remaining centres (HR 5.65) (p=0.023). Conclusions: Arrhythmias is an early sign predicting failure of the Fontan circulation of those with an atrio-pulmonary Fontan. Fontan conversion is only associated with improved outcomes when timely performed.

scholarly journals High Healthcare Utilization in Adolescents with Sickle Cell Disease Prior to Transition to Adult Care: A Retrospective Study

2019 ◽  
Vol 6 (3) ◽  
pp. 174-184 ◽  
Author(s):  
Julie Kanter ◽  
Menaka Bhor ◽  
Xin Li ◽  
Yunfeng Li ◽  
Jincy Paulose
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Index Date ◽  
Transition To Adult Care ◽  
Cell Disease ◽  
Adult Care ◽  
Old Patients ◽  
Management Interventions ◽  
Increased Risk ◽  
One Year

Background: The transition from pediatric to adult care in the US is often difficult for individuals with sickle cell disease (SCD). Young adults (18 to 25 years of age) have higher acute care utilization and an increased risk of poor outcomes. The current study was designed to provide greater insights into patients (16 to 18 years of age) with SCD prior to their transition to adult care. Objectives: To describe current treatment patterns, pain crises prevalence, SCD-related complications, and healthcare resource utilization (HCRU) in 16-to-18-year-old patients with SCD. Methods: From 1/1/2015-6/30/2017 using MarketScan Databases, patients were included if they were 16 to 18 years old at the index date, had ≥1 inpatient or 2 outpatient SCD diagnosis claims during the identification period, and were continuously enrolled in the database for at least one year prior (baseline) and post index date. Outcomes included medications, disease management interventions, Charlson Comorbidity Index (CCI), vaso-occlusive (VOC) crises requiring healthcare visits, HCRU, and SCD complications. Results: 1,186 patients were included; most (64.3%) were female. The mean CCI was 1.3 (SD: 0.7). In the overall cohort, patients experienced an average of 3.9 (SD: 4.2) VOCs and most patients (61.1%, n=725) had chronic complications during the one-year follow-up. Pulmonary disease (31.1%, n=369) was the most frequent complication; blood transfusions (mean: 8.4 [SD:7.0]) and iron-chelating therapies (mean: 8.6 [SD:10]) were the most common interventions and medications, respectively. In the 16 to 18 year old group, patients with SCD had 2.0 (SD = 2.3) hospital admissions, 3.4 (SD = 4.0) ER visits, and 5.0 (SD = 4.2) office visits. Conclusions: Prior to transition to adult care, adolescents with SCD already have significant acute and chronic disease-related complications, possibly contributing to frequent healthcare visits. Increased attention to this age group, including improvements in disease modifying therapy, are needed prior to transition to adult care systems to improve outcomes.

Adolescent and parent perspectives prior to involvement in a Fontan transition program

2017 ◽  
Vol 31 (4) ◽  
Author(s):  
Karin Du Plessis ◽  
Evelyn Culnane ◽  
Rebecca Peters ◽  
Yves d’Udekem
Keyword(s):  
Help Seeking ◽  
Health Knowledge ◽  
Transition Program ◽  
Palliative Surgery ◽  
Fontan Circulation ◽  
Transition To Adult Care ◽  
Emotional Wellbeing ◽  
Adult Care ◽  
Poor Knowledge ◽  
Transition Clinic

Abstract Background Successful transition to adult healthcare is particularly important for congenital heart disease (CHD) patients who have undergone palliative surgery, as they risk adverse events if not followed closely. This study examines young people at the worst end of the CHD spectrum who are born with a single ventricle (pumping heart chamber), and who undergo a series of operations that culminate in the Fontan surgical procedure. Purpose To explore adolescents with a Fontan circulation, and their parents’, readiness for transition to adult care and pre-implementation of a transition program. Methods Seventeen adolescents (15–18 years) and 15 of their parents completed questionnaires at the start of their first transition clinic. Results Adolescents reported poor knowledge about their Fontan circulation, and 41% had a poor understanding of the purpose of their medications/treatments. Over half of the adolescents had poor knowledge around medical help-seeking (when, who, how). Most reported feeling comfortable with discussing their medical issues with their cardiologist, but considerably less so about sensitive adolescent issues, in particular, emotional wellbeing. Parents reported high levels of anxiety around transition to adult care services. Conclusion Findings pre-program indicate poor adolescent health knowledge, a lack of focus in health services on emotional wellbeing and high parental anxiety. These findings highlight the need for dedicated programs that focus on early preparation, parental involvement and acknowledgment, transition as a process, strong integration and prioritisation in the health system with a youth-friendly and holistic focus, in particular, around emotional wellbeing.

Traversing the liminal: what can Fontan adults’ transition experiences and perspectives teach us about optimizing healthcare?

2018 ◽  
Vol 0 (0) ◽  
Author(s):  
Karin du Plessis ◽  
Rebecca Peters ◽  
Evelyn Culnane ◽  
Yves d’Udekem
Keyword(s):  
Health Condition ◽  
Fontan Circulation ◽  
Patient Perspectives ◽  
Support Network ◽  
Transition To Adult Care ◽  
Adult Care ◽  
Health Service Provision ◽  
Adult Congenital ◽  
Transition Experiences

Abstract Background Successfully transitioning to adult care is important for congenital heart disease (CHD) patients, particularly those at the worst end of the spectrum with a Fontan circulation, as their ongoing health engagement affects their health outcomes. Yet, there is a lack of literature exploring patient perspectives about their transition experiences, and what helps or hinders successful transition to adult care. Methods Young adults with a Fontan circulation (n = 18) from the Australian and New Zealand Fontan Registry participated in in-depth phone interviews. Thematic analyses were used to analyze the qualitative interview data. Results We identified six key themes which stood out from patient experiences, including differences between pediatric and adult congenital cardiac care, the need for preparation through formal transition programs, and the important role of support systems. Conclusion To become active managers of their healthcare and remain engaged with the healthcare system, young people would benefit from coordinated efforts between pediatric and adult care to formally prepare them for transition to adult care. This should include involvement from their support network and ongoing education about their health condition. The study highlights the importance of studying patient perspectives through qualitative research to better inform health service provision in the CHD space.

Pharmacological Considerations When Transitioning the Care of Epilepsy Patients from Pediatric to Adult Epilepsy Centers

2020 ◽  
Vol 09 (04) ◽  
pp. 177-185
Author(s):  
Natalie Guido-Estrada ◽  
Shifteh Sattar
Keyword(s):  
Medication Compliance ◽  
Lifestyle Changes ◽  
Transition To Adult Care ◽  
Pediatric Epilepsy ◽  
Transition Of Care ◽  
Adult Health ◽  
Adult Care ◽  
Adult Model ◽  
Antiepileptic Medications ◽  
The Many

AbstractThere is scarce evidence in review of the available literature to support a clear and superior model for the transition of care for epilepsy patients from pediatric to adult centers. Anecdotally, there is a common perception that families are reluctant to make this change and that the successful transition of care for epilepsy can be a challenge for patients, families, and physicians. As part of the effort to prepare the patient and family for the adult model of care, several treatment issues should be addressed. In this article, we discuss the specific challenges for physicians in transition of care for epilepsy patients from a pharmacological standpoint, which include differences in metabolism and pharmacodynamics that can impact tolerability or efficacy of antiepileptic medications, lifestyle changes affecting medication compliance and seizure control, acquired adult health conditions necessitating new medications that may result in adverse drug interactions, and adult neurologists' potential lack of familiarity with certain medications typically used in the pediatric epilepsy population. We offer this as a guide to avoid one of the many possible pitfalls when epilepsy patients transition to adult care.

An Unusual Association: Right Atrial Myxoma and Severe Left Ventricular Dysfunction. Case Report and Review of the Literature

2015 ◽  
Vol 17 (6) ◽  
pp. 285
Author(s):  
Lucian Florin Dorobantu ◽  
Ovidiu Chioncel ◽  
Alexandra Pasare ◽  
Dorin Lucian Usurelu ◽  
Ioan Serban Bubenek-Turconi ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Ventricular Dysfunction ◽  
Left Ventricular Systolic Dysfunction ◽  
Left Ventricular ◽  
Right Atrial ◽  
Left Ventricular Ejection ◽  
Cardiac Tumors ◽  
Severe Left Ventricular Dysfunction ◽  
Ventricular Ejection Fraction ◽  
Increased Risk

Myxomas comprise 50% of all benign cardiac tumors in adults, with the right atrium as their second most frequent site of origin. Surgical resection is the only effective therapeutic option for patients with these tumors. The association between right atrial myxomas and severe left ventricular systolic dysfunction is extremely rare and makes treatment even more challenging. This was the case for our patient, a 47-year-old male with a right atrial mass and a severely impaired left ventricular function, with a 20% ejection fraction. Global enlargement of the heart was also noted, with moderate right ventricular dysfunction. The tumor was successfully excised using the on-pump beating heart technique, with an immediate postoperative improvement of the left ventricular ejection fraction to 35%. The technique proved useful, with no increased risk to the patient.

scholarly journals Cross-sectional observational study of epidemiology of COVID-19 and clinical outcomes of hospitalised patients in North West London during March and April 2020

BMJ Open ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. e044384
Author(s):  
Guduru Gopal Rao ◽  
Alexander Allen ◽  
Padmasayee Papineni ◽  
Liyang Wang ◽  
Charlotte Anderson ◽  
...  
Keyword(s):  
Mechanical Ventilation ◽  
Clinical Outcomes ◽  
Severe Infection ◽  
Older Age ◽  
Cross Sectional ◽  
North West ◽  
Icu Admission ◽  
Risk Of Death ◽  
Hospitalised Patients ◽  
Increased Risk

ObjectiveThe aim of this paper is to describe evolution, epidemiology and clinical outcomes of COVID-19 in subjects tested at or admitted to hospitals in North West London.DesignObservational cohort study.SettingLondon North West Healthcare NHS Trust (LNWH).ParticipantsPatients tested and/or admitted for COVID-19 at LNWH during March and April 2020Main outcome measuresDescriptive and analytical epidemiology of demographic and clinical outcomes (intensive care unit (ICU) admission, mechanical ventilation and mortality) of those who tested positive for COVID-19.ResultsThe outbreak began in the first week of March 2020 and reached a peak by the end of March and first week of April. In the study period, 6183 tests were performed in on 4981 people. Of the 2086 laboratory confirmed COVID-19 cases, 1901 were admitted to hospital. Older age group, men and those of black or Asian minority ethnic (BAME) group were predominantly affected (p<0.05). These groups also had more severe infection resulting in ICU admission and need for mechanical ventilation (p<0.05). However, in a multivariate analysis, only increasing age was independently associated with increased risk of death (p<0.05). Mortality rate was 26.9% in hospitalised patients.ConclusionThe findings confirm that men, BAME and older population were most commonly and severely affected groups. Only older age was independently associated with mortality.

scholarly journals 270. Clinical Outcomes of Ceftriaxone versus Penicillin G for Complicated Viridans Group Streptococci Bacteremia

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S137-S137
Author(s):  
Stephanie Wo ◽  
Yanina Dubrovskaya ◽  
Justin Siegfried ◽  
John Papadopoulos ◽  
Shin-Pung Jen
Keyword(s):  
Clinical Outcomes ◽  
Hospital Readmission ◽  
Protective Factor ◽  
Primary Outcome ◽  
Bloodstream Infections ◽  
Penicillin G ◽  
Eligibility Criteria ◽  
Extended Spectrum Beta Lactamase ◽  
Increased Risk ◽  
Viridans Group Streptococci

Abstract Background Viridans group streptococci (VGS) is an infrequent yet significant cause of bloodstream infections, and complicated cases may require prolonged antibiotic therapy. Ceftriaxone (CTX) and penicillin G (PCN G) are both considered first line options for VGS infections, but comparisons between these agents are limited. We evaluated the clinical outcomes amongst patients treated with CTX and PCN G for complicated VGS bacteremia. Methods This was a single-center, retrospective study of adult patients with ≥1 positive VGS blood culture who were treated with either CTX or PCN G/ampicillin (both included in PCN G arm) between January 2013 and June 2019. The primary outcome was a composite of safety endpoints, including hospital readmission due to VGS or an adverse event (AE) from therapy, Clostridioides difficile infections, treatment modification or discontinuation due to an antibiotic-related AE, and development of extended-spectrum beta lactamase resistance. Secondary outcomes included the individual safety endpoints, VGS bacteremia recurrence, hospital readmission, and all-cause mortality. Results Of 328 patients screened for inclusion, 94 patients met eligibility criteria (CTX n= 64, PCN G n=34). Median age was 68 years (IQR 56–81) and 68% were male. Study patients did not present with critical illness, as reflected by a median Pitt bacteremia score of 0 in the CTX and 1 in the PCN G arms, P=0.764. Streptococcus mitis was the most common VGS isolate and infective endocarditis (IE) was the predominant source of infection. CTX was not significantly associated with increased risk of the primary outcome (14% vs. 27%; P= 0.139). The driver of the composite outcome was hospital readmission due to VGS bacteremia or therapy complications. Results were similar in the subgroup of patients with IE (12.5% vs. 23.5%). No secondary endpoints differed significantly between groups. On multivariate analysis, source removal was a protective factor of the primary outcome (OR 0.1; 95% CI 0.020–0.6771; P= 0.017). Conclusion Despite potential safety concerns with the prolonged use of CTX in complicated VGS bacteremia, this study did not demonstrate a higher rate of treatment failure, adverse events, or resistance. These findings warrant further exploration. Disclosures All Authors: No reported disclosures

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