Morphologic study of the ascending aorta and aortic arch in hypoplastic left hearts: Surgical implications

ObjectivesThe ascending aorta and aortic arch in patients with hypoplasia of the left heart are hypoplastic as a result of diminished blood flow. In this study, the presence and degree of obstruction owing to areas of narrowing or infolding within the diminutive aorta are quantified, and their surgical significance is discussed.MethodsNinety-six specimens with hypoplasia of the left heart were studied and measurements were taken at specified sites to evaluate areas of narrowing. Quantitative assessments of infoldings and their contribution to obstruction of flow are made.ResultsNarrowing of the distal ascending aorta was found in 60 (62.5%) specimens, with a decrease in circumference of the distal ascending aorta (0.72 ± 1.06 mm) present when compared with its midpoint (P < .05). Tissue infolding at the orifice of the brachiocephalic artery and its junction with the distal ascending aorta was observed in 56 (58.3%) hearts, with major infolding in 29 (30.2%) and minor infolding in 27 (28.5%). Tissue infolding at this site correlated with a smaller ascending aorta (P < .001) but not with narrowing in the distal ascending aorta (P = .53). Ductal coarctations were detected in 77 (81.1%) specimens. Their presence correlated with a smaller diameter of the ascending aorta (P < .05), and their severity correlated with the presence of aortic and mitral valvular atresia (P < .05).ConclusionsImportant areas of obstruction in the ascending aorta in patients with hypoplasia of the left heart were found, and their pathogenesis is discussed. The findings highlight the importance of incorporating the ascending aorta into the aortic reconstruction at the time of initial palliation for patients with hypoplasia of the left heart

Prioritising systemic cancer therapies applying ESMO's tools and other resources to assist in improving cancer care globally:the Kazakh experience

BACKGROUND: In Kazakhstan, cancer is the second leading cause of death with a major public health and economic burden. In the last decade, cancer care and cancer medicine costs have significantly increased. To improve the efficiency and efficacy of cancer care expenditure and planning, the Kazakhstan Ministry of Health requested assistance from the World Health Organization (WHO) and the European Society for Medical Oncology (ESMO) to review its systemic cancer treatment protocols and essential medicines list and identify high-impact, effective regimens. MATERIALS AND METHODS: ESMO developed a four-phase approach to review Kazakhstan cancer treatment protocols: (i) perform a systematic analysis of the country’s cancer medicines and treatment protocols; (ii) cross-reference the country’s cancer protocols with the WHO Model List of Essential Medicines, the ESMO-Magnitude of Clinical Benefit Scale and the European Medicines Agency’s medicine availability and indications database; (iii) extract treatment recommendations from the ESMO Clinical Practice Guidelines; (iv) expert review for all cancer medicines not on the WHO Model List of Essential Medicines and the country treatment protocols. RESULTS: This ESMO four-phase approach led to the update of the Kazakhstan national essential cancer medicines list and the list of cancer treatment protocols. This review has led to the withdrawal of several low-value or non-evidence-based medicines and a budget increase for cancer care to include all essential and highly effective medicines and treatment options. CONCLUSION: When applied effectively, this four-phase approach can improve access to medicines, efficiency of expenditure and sustainability of cancer systems. The WHO–ESMO collaboration illustrated how, by sharing best practices, tools and resources, we can address access to cancer medicines and positively impact patient care

Quality indicators for systemic anticancer therapy services: a systematic review of metrics used to compare quality across healthcare facilities.

PURPOSE: The number of systemic anticancer therapy (SACT) regimens has expanded rapidly over the last decade. There is a need to ensure quality of SACT delivery across cancer services and systems in different resource settings to reduce morbidity, mortality, and detrimental economic impact at individual and systems level. Existing literature on SACT focuses on treatment efficacy with few studies on quality or how SACT is delivered within routine care in comparison to radiation and surgical oncology. METHODS: Systematic review was conducted following PRISMA guidelines. EMBASE and MEDLINE were searched and handsearching was undertaken to identify literature on existing quality indicators (QIs) that detect meaningful variations in the quality of SACT delivery across different healthcare facilities, regions, or countries. Data extraction was undertaken by two independent reviewers. RESULTS: This review identified 63 distinct QIs from 15 papers. The majority were process QIs (n = 55, 87.3%) relating to appropriateness of treatment and guideline adherence (n = 28, 44.4%). There were few outcome QIs (n = 7, 11.1%) and only one structural QI (n = 1, 1.6%). Included studies solely focused on breast, colorectal, lung, and skin cancer. All but one studies were conducted in high-income countries. CONCLUSIONS: The results of this review highlight a significant lack of research on SACT QIs particularly those appropriate for resource-constrained settings in low- and middle-income countries. This review should form the basis for future work in transforming performance measurement of SACT provision, through context-specific QI SACT development, validation, and implementation

Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation

Aim of the study: To investigate the long-term outcome of surgical treatment for congenitally corrected transposition of the great arteries (CCTGA), in patients with biventricular repair with the right ventricle as systemic ventricle.Methods: A total of 32 patients with CCTGA were operated between January 1972 and October 2008. These operations comprised 18 patients with a repair with a normal left ventricular outflow tract, 11 patients with a Rastelli repair of the left ventricle to the pulmonary artery and 3 patients with a cardiac transplantation.Results: Excluding the cardiac transplantation patients, mean age at operation was 16 years (sd 15 years, range 1 week - 49 years). Median follow-up was 12 years (sd 10 years, range 7 days - 32 years). Survival obtained from Kaplan-Meier analysis at 20 years after surgery was 63% (CI 53-73%). For the non-Rastelli group these data at 20 years were

Risk of COVID-19 death for people with a pre-existing cancer diagnosis prior to COVID-19-vaccination:A systematic review and meta-analysis

While previous reviews found a positive association between pre-existing cancer diagnosis and COVID-19-related death, most early studies did not distinguish long-term cancer survivors from those recently diagnosed/treated, nor adjust for important confounders including age. We aimed to consolidate higher-quality evidence on risk of COVID-19-related death for people with recent/active cancer (compared to people without) in the pre-COVID-19-vaccination period. We searched the WHO COVID-19 Global Research Database (20 December 2021), and Medline and Embase (10 May 2023). We included studies adjusting for age and sex, and providing details of cancer status. Risk-of-bias assessment was based on the Newcastle-Ottawa Scale. Pooled adjusted odds or risk ratios (aORs, aRRs) or hazard ratios (aHRs) and 95% confidence intervals (95% CIs) were calculated using generic inverse-variance random-effects models. Random-effects meta-regressions were used to assess associations between effect estimates and time since cancer diagnosis/treatment. Of 23 773 unique title/abstract records, 39 studies were eligible for inclusion (2 low, 17 moderate, 20 high risk of bias). Risk of COVID-19-related death was higher for people with active or recently diagnosed/treated cancer (general population: aOR = 1.48, 95% CI: 1.36-1.61, I2 = 0; people with COVID-19: aOR = 1.58, 95% CI: 1.41-1.77, I2 = 0.58; inpatients with COVID-19: aOR = 1.66, 95% CI: 1.34-2.06, I2 = 0.98). Risks were more elevated for lung (general population: aOR = 3.4, 95% CI: 2.4-4.7) and hematological cancers (general population: aOR = 2.13, 95% CI: 1.68-2.68, I2 = 0.43), and for metastatic cancers. Meta-regression suggested risk of COVID-19-related death decreased with time since diagnosis/treatment, for example, for any/solid cancers, fitted aOR = 1.55 (95% CI: 1.37-1.75) at 1 year and aOR = 0.98 (95% CI: 0.80-1.20) at 5 years post-cancer diagnosis/treatment. In conclusion, before COVID-19-vaccination, risk of COVID-19-related death was higher for people with recent cancer, with risk depending on cancer type and time since diagnosis/treatment.</p

Risk of COVID-19 death for people with a pre-existing cancer diagnosis prior to COVID-19-vaccination : A systematic review and meta-analysis

Research Funding National Health and Medical Research Council. Grant Number: APP1194679 World Health Organization Article Funding Open access publishing facilitated by The University of Sydney, as part of the Wiley - The University of Sydney agreement via the Council of Australian University Librarians.Peer reviewedPublisher PD

Cardiac tumours in children

Cardiac tumours are benign or malignant neoplasms arising primarily in the inner lining, muscle layer, or the surrounding pericardium of the heart. They can be primary or metastatic. Primary cardiac tumours are rare in paediatric practice with a prevalence of 0.0017 to 0.28 in autopsy series. In contrast, the incidence of cardiac tumours during foetal life has been reported to be approximately 0.14%. The vast majority of primary cardiac tumours in children are benign, whilst approximately 10% are malignant. Secondary malignant tumours are 10–20 times more prevalent than primary malignant tumours. Rhabdomyoma is the most common cardiac tumour during foetal life and childhood. It accounts for more than 60% of all primary cardiac tumours. The frequency and type of cardiac tumours in adults differ from those in children with 75% being benign and 25% being malignant. Myxomas are the most common primary tumours in adults constituting 40% of benign tumours. Sarcomas make up 75% of malignant cardiac masses. Echocardiography, Computing Tomography (CT) and Magnetic Resonance Imaging (MRI) of the heart are the main non-invasive diagnostic tools. Cardiac catheterisation is seldom necessary. Tumour biopsy with histological assessment remains the gold standard for confirmation of the diagnosis. Surgical resection of primary cardiac tumours should be considered to relieve symptoms and mechanical obstruction to blood flow. The outcome of surgical resection in symptomatic, non-myxomatous benign cardiac tumours is favourable. Patients with primary cardiac malignancies may benefit from palliative surgery but this approach should not be recommended for patients with metastatic cardiac tumours. Surgery, chemotherapy and radiotherapy may prolong survival. The prognosis for malignant primary cardiac tumours is generally extremely poor

Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support

Surgical management of tetralogy of Fallot (TOF) results in anatomic and functional abnormalities in the majority of patients. Although right ventricular volume load due to severe pulmonary regurgitation can be tolerated for many years, there is now evidence that the compensatory mechanisms of the right ventricular myocardium ultimately fail and that if the volume load is not eliminated or reduced by pulmonary valve replacement the dysfunction might be irreversible. Cardiovascular magnetic resonance (CMR) has evolved during the last 2 decades as the reference standard imaging modality to assess the anatomic and functional sequelae in patients with repaired TOF. This article reviews the pathophysiology of chronic right ventricular volume load after TOF repair and the risks and benefits of pulmonary valve replacement. The CMR techniques used to comprehensively evaluate the patient with repaired TOF are reviewed and the role of CMR in supporting clinical decisions regarding pulmonary valve replacement is discussed