scholarly journals Cohort study of intervened functionally univentricular heart in England and Wales (2000–2018)

Heart ◽  
2021 ◽  
pp. heartjnl-2021-319677
Author(s):  
Elena Hadjicosta ◽  
Rodney Franklin ◽  
Anna Seale ◽  
Oliver Stumper ◽  
Victor Tsang ◽  
...  

ObjectiveGiven the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome.MethodsWe performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000–2018. The primary outcome was mortality, ascertained from the Office for National Statistics in 2020.ResultsOf 53 615 patients, 1557 had FUH: 55.9% were boys and 67.4% were of White ethnic groups. The largest diagnostic categories were tricuspid atresia (28.9%), double inlet left ventricle (21.0%) and unbalanced atrioventricular septal defect (AVSD) (15.2%). The ages at staged surgery were: initial palliation 11.5 (IQR 5.5–43.5) days, cavopulmonary shunt 9.2 (IQR 6.0–17.1) months and Fontan 56.2 (IQR 45.5–70.3) months. The median follow-up time was 10.8 (IQR 7.0–14.9) years and the 1, 5 and 10-year survival rates after initial palliation were 83.6% (95% CI 81.7% to 85.4%), 79.4% (95% CI 77.3% to 81.4%) and 77.2% (95% CI 75.0% to 79.2%), respectively. Higher hazards were present for unbalanced AVSD HR 2.75 (95% CI 1.82 to 4.17), atrial isomerism HR 1.75 (95% CI 1.14 to 2.70) and low weight HR 1.65 (95% CI 1.13 to 2.41), critical illness HR 2.30 (95% CI 1.67 to 3.18) or acquired comorbidities HR 2.71 (95% CI 1.82 to 4.04) at initial palliation.ConclusionAlthough treatment pathways for FUH are complex and variable, nearly 8 out of 10 children survived to 10 years. Longer-term analyses of outcome based on diagnosis (rather than procedure) can inform parents, patients and clinicians, driving practice improvements for complex CHD.

2020 ◽  
Vol 6 (6) ◽  
pp. e300-e304
Author(s):  
Danielle LoGiurato ◽  
Zoltan Antal ◽  
Ping Zhou

Objective: Treatment of metastatic adrenocortical carcinoma (ACC) is challenging and long-term survival rates are exceedingly low. Long-term outcome data for pediatric patients who received mitotane is very limited. Methods: We describe the case of a 2-year-old boy with ACC with a lung metastasis. He was treated with surgery, chemotherapy, and mitotane, and remains disease-free 13 years after diagnosis. Results: The key endocrine issues learned from this case include: adrenal-derived sex-steroid and insulin-like growth factor-2 levels are correlated with disease status; very high doses of glucocorticoid and mineralocorticoid are required while on treatment of mitotane; and central precocious puberty needs to be detected and treated in a timely manner to preserve final adult height. Conclusion: We report a case of pediatric ACC with metastasis that was successfully treated with surgery, chemotherapy, and adjuvant therapy with mitotane. Appropriate endocrine testing and management are important for long-term survival and quality of life.


2021 ◽  
Vol 3 (2) ◽  
Author(s):  
Dr. Mridula C Jobson

Development in intensive care and medical treatment has significantly lowered mortality rates for children with complex congenital heart disease (CHD) but among pediatric survivors with complex CHD, there is a distinctive pattern of neuro-developmental and neuropsychology impairment characterized impaired social interaction, impulsive Behavior, and impaired executive functions. Children with congenital heart diseases have more Behavioral problems compared to children without chronic illnesses. Sample and sampling: The sample of 200 children with congenital heart defect was selected between age group 4-8 years using multistage stratified sampling. Method: The childhood psychopathology measurement schedule (CPMS) by Dr. Savitha Malhotra was used for assessing Behavioral problems present in children with CHD. The present investigation adopted an experimental design, described as “Pre- Post experimental design and the results were statistically analyzed using paired T test. Result: The result revealed that the effectiveness of intervention program to retrain Behavior showed high significance. Conclusion: With increased survival rates, the focus of clinical research has paralleled the population shift from short term surgical survival to the assessment of long-term morbidityKeyword: Congenital Heart Defect, Neuro-psychological retraining, Neurodevelopment, Behavioral and emotional retraining.


2021 ◽  
Vol 28 (5) ◽  
pp. 1659-1664
Author(s):  
Romain Deschamps ◽  
Julie Pique ◽  
Xavier Ayrignac ◽  
Nicolas Collongues ◽  
Bertrand Audoin ◽  
...  

2020 ◽  
pp. jrheum.191004
Author(s):  
Noortje Groot ◽  
Anne Kardolus ◽  
Marc Bijl ◽  
Radboud Dolhain ◽  
Onno Teng ◽  
...  

Objective Long-term outcome data in adults with childhood-onset SLE are limited. Here, we report the effects of cSLE on education, vocation and employment in a large cohort of adults with cSLE. Methods Patients were seen for a single study visit containing a structured history and physical examination. Medical records were retrieved to supplement information obtained during the study visit. Education and employment status were assessed by questionnaires. Health-related quality of life (HRQOL) was measured with the SF36. Results 106 cSLE patients (93% female, 73% white), with a median disease duration of 20 years, completed the visit and questionnaires. Almost all patients stated that cSLE had influenced their education, but level of completed education was similar to the general Dutch population. Half of the patients had adjusted their vocational choice due to the disease. Still, 44% of patients who had finished education did not have a paid job. Of the employed patients, 61% worked part-time. Disease damage was equally prevalent in patients with and without paid employment. A high percentage of patients (51%) were declared work disabled, which was related to damage. Patients who did not have paid employment were often work disabled. Both had a negative influence on HRQOL. Conclusion The effect of cSLE on academic achievements and employment is substantial, despite adjusting educational and vocational choices to the disease. Ongoing support, not only to help patients find suitable education and vocation, but also to offer guidance regarding potential adjustments during their career, is necessary to optimise participation in the community.


2020 ◽  
Vol 128 (10) ◽  
pp. 687-692 ◽  
Author(s):  
Joachim Feldkamp ◽  
F. Grünwald ◽  
Markus Luster ◽  
Kerstin Lorenz ◽  
Christian Vorländer ◽  
...  

AbstractThyroid nodules and cysts are frequently diagnosed in Germany with a prevalence of about 20% in young adults reaching up to 70% in older adults. Surgery is the standard treatment of symptomatic nodules, nodules with suspicion of malignancy and thyroid cancer. Radioiodine treatment is applied for autonomously functioning nodules. During the last years new non-surgical and non-radioiodine techniques have been introduced to treat thyroid nodules. These techniques include ethanol/polidocanol treatment, radiofrequency, microwave, and laser ablation, and high frequency ultrasound ablation. A significant reduction in nodule size could be documented for these techniques in several studies, but long-term outcome data are missing. Until now, there is no general consensus regarding the appropriate indications for these methods. For this reason, the Thyroid Section (German Society for Endocrinology), the Thyroid Working Committee (German Society for Nuclear Medicine), and the German Association of Endocrine Surgeons (CAEK) for the German Society of General and Visceral Surgery (DGAV) reviewed the respective literature, discussed the pro and cons and developed a consensus statement and recommendation to help physicians and patients in their decision making.


2008 ◽  
Vol 123 (3) ◽  
pp. 298-302 ◽  
Author(s):  
R J Sim ◽  
A H Jardine ◽  
E J Beckenham

AbstractA number of authors have suggested that surgery for suspected perilymph fistula is effective in preventing deterioration of hearing and in improving hearing in some cases in the short term. We present long-term hearing outcome data from 35 children who underwent exploration for presumed perilymph fistula at The Children's Hospital, Sydney, Australia, between 1985 and 1992.Methods:The pre-operative audiological data (mean of 500, 1000, 2000 and 4000 Hz results) were compared with the most recently available data (range two to 15 years) and the six-month post-operative data.Results:The short-term results showed no significant change in hearing at six months, with a subsequent, statistically significant progression of hearing loss in both operated and non-operated ears (Wilcoxon signed rank test: operated ear, p < 0.017; non-operated ear, p < 0.009).Conclusion:In this case series, exploratory surgery for correction of suspected perilymph fistula did not prevent progression of long-term hearing loss.


2017 ◽  
Vol 81 (4) ◽  
pp. 476-484 ◽  
Author(s):  
An-Hsun Chou ◽  
Tien-Hsing Chen ◽  
Chun-Yu Chen ◽  
Shao-Wei Chen ◽  
Chao-Wei Lee ◽  
...  

2006 ◽  
Vol 21 (9) ◽  
pp. 1266-1273 ◽  
Author(s):  
Jaana Ronkainen ◽  
Marja Ala-Houhala ◽  
Helena Autio-Harmainen ◽  
Timo Jahnukainen ◽  
Olli Koskimies ◽  
...  

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