scholarly journals The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales

2021 ◽  
Vol 9 ◽  
Author(s):  
Kate E. Best ◽  
Nicola Miller ◽  
Elizabeth Draper ◽  
David Tucker ◽  
Karen Luyt ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Cox Regression ◽  
Surgical Techniques ◽  
Population Based ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Term Survival ◽  
England And Wales ◽  
Hypoplastic Left Ventricle ◽  
Kaplan Meier

Background: Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect (CHD) characterised by the underdevelopment of the left side of the heart with varying levels of hypoplasia of the left atrium, mitral valve, left ventricle, aortic valve and aortic arch. In the UK, age 12 survival for cases born between 1991 and 1993 was 21%. UK survival estimates corresponding to cases born between 2000 and 2015 were improved at 56%, but survival was examined up to age five only. Contemporary long-term survival estimates play a crucial role in counselling parents following diagnosis. The aim of this study was to report survival estimates up to age 15 for children born with HLHS or hypoplastic left ventricle with additional CHD in England and Wales between 1998 and 2012.Methods: Cases of HLHS notified to four congenital anomaly registers in England and Wales during 1998–2012, matched to Office for National Statistics mortality information, were included. Kaplan-Meier survival estimates to age 15 were reported. Cox regression models were fitted to examine risk factors for mortality.Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality.Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.

scholarly journals Impact of gender on the survival of patients with glioblastoma

2018 ◽  
Vol 38 (6) ◽  
Author(s):  
Minjie Tian ◽  
Wenying Ma ◽  
Yueqiu Chen ◽  
Yue Yu ◽  
Donglin Zhu ◽  
...  
Keyword(s):  
Sex Hormones ◽  
Survival Data ◽  
Cox Regression ◽  
Population Based ◽  
Cancer Specific Survival ◽  
Term Survival ◽  
Kaplan Meier ◽  
Male Patients ◽  
Stratified Analysis ◽  
The Impact

Background: Preclinical models have suggested a role for sex hormones in the development of glioblastoma multiforme (GBM). However, the impact of gender on the survival time of patients with GBM has not been fully understood. The objective of the present study was to clarify the association between gender and survival of patients with GBM by analyzing population-based data. Methods: We searched the Surveillance, Epidemiology, and End-Results database who were diagnosed with GBM between 2000 and 2008 and were treated with surgery. Five-year cancer specific survival data were obtained. Kaplan–Meier methods and multivariable Cox regression models were used to analyze long-term survival outcomes and risk factors. Results: A total of 6586 patients were identified; 61.5% were men and 38.5% were women. The 5-year cancer-specific survival (CSS) rates in the male and female groups were 6.8% and 8.3%, respectively (P=0.002 by univariate and P<0.001 by multivariate analysis). A stratified analysis showed that male patients always had the lowest CSS rate across localized cancer stage and different age subgroups. Conclusions: Gender has prognostic value for determining GBM risk. The role of sex hormones in the development of GBM warrants further investigation.

Total anomalous pulmonary venous connection mimicking hypoplastic left heart syndrome

2021 ◽  
pp. 1-3
Author(s):  
Ryohei Matsuoka ◽  
Jun Muneuchi ◽  
Yoshie Ochiai
Keyword(s):  
Left Ventricle ◽  
Systemic Circulation ◽  
Z Score ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Transverse Arch ◽  
Anomalous Pulmonary Vein ◽  
Pulmonary Arterial ◽  
Left Heart Syndrome ◽  
Anomalous Pulmonary Venous Connection

Abstract A newborn with supracardiac total anomalous pulmonary venous connection vein presented the small left ventricle with z score of −7.5, retrograde blood supply in the transverse arch, and the dutcus-dependent systemic circulation. The patient underwent the repair of the anomalous pulmonary vein and bilateral pulmonary arterial banding soon after the birth and then transcatheter pulmonary arterial debanding at the age of 10 months because of an appropriate growth of the left ventricle.

Survival After Reconstructive Surgery for Hypoplastic Left Heart Syndrome

Circulation ◽  
2000 ◽  
Vol 102 (suppl_3) ◽  
Author(s):  
William T. Mahle ◽  
Thomas L. Spray ◽  
Gil Wernovsky ◽  
J. William Gaynor ◽  
Bernard J. Clark
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Reconstructive Surgery ◽  
Fontan Procedure ◽  
Stage I ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Term Survival ◽  
Late Death ◽  
Entire Cohort ◽  
Left Heart Syndrome

Background —There are limited data regarding the long-term survival of patients who have undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed the 15-year experience at our institution to examine survival in the context of continued improvements in early operative results. Methods and Results —Between 1984 and 1999, 840 patients underwent stage I surgery for HLHS. From review of medical records and direct patient contact, survival status was determined. The 1-, 2-, 5-, 10-, and 15-year survival for the entire cohort was 51%, 43%, 40%, 39%, and 39%, respectively. Late death occurred in 14 of the 291 patients discharged to home after the Fontan procedure, although only 1 patient has died beyond 5 years of age. Heart transplantation after stage I reconstruction was performed in 5 patients. Later era of stage I surgery was associated with significantly improved survival ( P <0.001). Three-year survival for patients undergoing stage I reconstruction from 1995 to 1998 was 66% versus 28% for those patients undergoing surgery from 1984 to 1988. Age >14 days at stage I and weight <2.5 kg at stage I were also associated with higher mortality ( P =0.004 and P =0.01, respectively). Other variables, including anatomic subtype, heterotaxia, and age at subsequent staging procedures, were not associated with survival. Conclusions —Over the 15-year course of this study, early- and intermediate-term survival for patients with HLHS undergoing staged palliation increased significantly. Late death and the need for cardiac transplantation were uncommon.

scholarly journals Down-Regulation of LncRNA DGCR5 Correlates with Poor Prognosis in Hepatocellular Carcinoma

2016 ◽  
Vol 40 (3-4) ◽  
pp. 707-715 ◽  
Author(s):  
Ruyi Huang ◽  
Xiaochen Wang ◽  
Wenjie Zhang ◽  
Guangyan Zhangyuan ◽  
Kangpeng Jin ◽  
...  
Keyword(s):  
Roc Curve ◽  
Cox Regression ◽  
Cancer Specific Survival ◽  
Term Survival ◽  
Diagnosis And Prognosis ◽  
Kaplan Meier ◽  
High Expression Group ◽  
Negative Prognostic Factor ◽  
Potential Biomarker ◽  
Low Expression

Background/Aims: Long non-coding RNAs (lncRNAs) have been reported to play pivotal roles in multiple tumors and can act as tumor biomarkers. In this study, we explored the association of the expression of an lncRNA, DGCR5 with clinicopathological features and prognosis in HCC. Methods: Expression levels of DGCR5 were detected by quantitative real-time PCR (qRT-PCR) and the clinical data was obtained, including basic information, data of clinicopathology and cancer specific survival rate. Receiver operating characteristic (ROC) curve, Kaplan-Meier methods and multivariable Cox regression models were used to analyze predictive efficiency, long-term survival outcomes and risk factors. Results: DGCR5 was found down-regulated in HCC tissues (P<0.001) and serum (P = 0.0035) and low expression of DGCR5 was correlated with a poor cancer specific survival (CSS) (P = 0.0019), as the overall 5-year CSS rates were 10.3% (low expression group) and 36.6% (high expression group), respectively. A stratified analysis demonstrated that low DGCR5 expression was an independent negative prognostic factor for HCC. In addition, the area under the ROC curve was 0.782 with a sensitivity of 0.633 and a specificity of 0.833. Conclusions: Our results suggest that DGCR5 may be a participator in HCC and can serve as potential biomarker for the diagnosis and prognosis in HCC.

scholarly journals Association of masseter area and radiodensity with three-month survival after proximal anterior circulation occlusion

2020 ◽  
Vol 13 (1) ◽  
pp. 25-29 ◽  
Author(s):  
Iisa Lindström ◽  
Sara Protto ◽  
Niina Khan ◽  
Jussi Hernesniemi ◽  
Niko Sillanpää ◽  
...  
Keyword(s):  
Regression Analysis ◽  
Ischemic Stroke ◽  
Acute Ischemic Stroke ◽  
Cox Regression ◽  
Multivariable Logistic Regression Analysis ◽  
Anterior Circulation ◽  
Term Survival ◽  
Cox Regression Analysis ◽  
Kaplan Meier

BackgroundMasseter area (MA), a surrogate for sarcopenia, appears to be useful when estimating postoperative survival, but there is lack of consensus regarding the potential predictive value of sarcopenia in acute ischemic stroke (AIS) patients. We hypothesized that MA and density (MD) evaluated from pre-interventional CT angiography scans predict postinterventional survival in patients undergoing mechanical thrombectomy (MT).Materials and methods312 patients treated with MT for acute occlusions of the internal carotid artery (ICA) or the M1 segment of the middle cerebral artery (M1-MCA) between 2013 and 2018. Median follow-up was 27.4 months (range 0–70.4). Binary logistic (alive at 3 months, OR <1) and Cox regression analyses were used to study the effect of MA and MD averages (MAavg and MDavg) on survival.ResultsIn Kaplan–Meier analysis, there was a significant inverse relationship with both MDavg and MAavg and mortality (MDavg P<0.001, MAavg P=0.002). Long-term mortality was 19.6% (n=61) and 3-month mortality 12.2% (n=38). In multivariable logistic regression analysis at 3 months, per 1-SD increase MDavg (OR 0.61, 95% CI 0.41 to 0.92, P=0.018:) and MAavg (OR 0.57, 95% CI 0.35 to 0.91, P=0.019) were the independent predictors associated with lower mortality. In Cox regression analysis, MDavg and MAavg were not associated with long-term survival.ConclusionsIn acute ischemic stroke patients, MDavg and MAavg are independent predictors of 3-month survival after MT of the ICA or M1-MCA. A 1-SD increase in MDavg and MAavg was associated with a 39%–43% decrease in the probability of death during the first 3 months after MT.

scholarly journals Twenty-Year Time Trends in Long-Term Case-Fatality and Recurrence Rates After Ischemic Stroke Stratified by Etiology

Stroke ◽  
2020 ◽  
Vol 51 (9) ◽  
pp. 2778-2785 ◽  
Author(s):  
Viktoria Rücker ◽  
Peter U. Heuschmann ◽  
Martin O’Flaherty ◽  
Michael Weingärtner ◽  
Manuela Hess ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
Time Trends ◽  
Cox Regression ◽  
Case Fatality ◽  
Population Based ◽  
Artery Occlusion ◽  
Recurrence Rates ◽  
Term Survival ◽  
Small Artery Occlusion

Background and Purpose: Data on long-term survival and recurrence after stroke are lacking. We investigated time trends in ischemic stroke case-fatality and recurrence rates over 20-years stratified by etiological subtype according to the Trial of ORG 10172 in Acute Stroke Treatment classification within a population-based stroke register in Germany. Methods: Data was collected within the Erlangen Stroke Project, a prospective, population-based stroke register covering a source population of 105 164 inhabitants (2010). Case fatality and recurrence rates for 3 months, 1 year, and 5 years were estimated with Kaplan-Meier estimates. Sex-specific time trends for case-fatality and recurrence rates were estimated with Cox regression. We adjusted for age, sex, and year of event and stratified for etiological subtypes. A sensitivity analysis with competing risk analysis for time trends in recurrence were performed. Results: Between 1996 and 2015, 3346 patients with first ischemic stroke were included; age-standardized incidence per 100 000 was 75.8 in women and 131.6 in men (2015). Overall, 5-year survival probabilities were 50.4% (95% CI, 47.9–53.1) in women and 59.2% (95% CI, 56.4–62.0) in men; 5-year survival was highest in patients with first stroke due to small-artery occlusion (women, 71.8% [95% CI, 67.1–76.9]; men, 75.9% [95% CI, 71.3–80.9]) and lowest in cardioembolic stroke (women, 35.7% [95% CI, 31.0–41.1]; men, 47.8% [95% CI, 42.2–54.3]). Five-year recurrence rates were 20.1% (95% CI, 17.5–22.6) in women and 20.1% (95% CI, 17.5–22.7) in men; 5-year recurrence rate was lowest in women in stroke due to small artery occlusion 16.0% (95% CI, 11.7–20.1) and in men in large-artery atherosclerosis 16.6% (95% CI, 8.7–23.9); highest risk of recurrence was observed in undefined strokes (women, 22.3% [95% CI, 17.8–26.6]; men, 21.4% [95% CI, 16.7–25.9]). Cox regression revealed improvements in case-fatality rates over time with differences in stroke causes. No time trends in recurrence rates were observed. Conclusions: Long-term survival and recurrence varied substantially by first stroke cause. Survival probabilities improved over the past 2 decades; no major trends in stroke recurrence rates were observed.

The genetics of hypoplastic left heart syndrome

1999 ◽  
Vol 9 (6) ◽  
pp. 627-632 ◽  
Author(s):  
Paul D. Grossfeld
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Heart Defects ◽  
Left Ventricular ◽  
Left Ventricular Cavity ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Term Survival ◽  
Surgical Options ◽  
Systemic Ventricle ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome is one of the most therapeutically challenging congenital cardiac defects. It accounts for as many as 1.5% of all congenital heart defects, but is responsible for up to one quarter of deaths in neonates with heart disease.1The management of hypoplastic left heart syndrome is controversial. Two surgical options exist:2,3the Norwood procedure, is a three stage repair in which the morphologically right ventricle is converted to function as the systemic ventricle. Alternatively, orthotopic transplantation can be performed. Although both surgical options have had improved outcomes, the prognosis for long-term survival is guarded, with a five year survival for either approach reported to be in the region of 50–60%. In this review, I explore the evidence for a genetic etiology for the “classic” hypoplastic left heart syndrome, defined as mitral and/or aortic atresia with hypoplasia of the left ventricular cavity and the other left-sided structures.

scholarly journals Distention of the Immature Left Ventricle Triggers Development of Endocardial Fibroelastosis: An Animal Model of Endocardial Fibroelastosis Introducing Morphopathological Features of Evolving Fetal Hypoplastic Left Heart Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Shogo Shimada ◽  
Christian Robles ◽  
Ben M. W. Illigens ◽  
Alejandra M. Casar Berazaluce ◽  
Pedro J. del Nido ◽  
...  
Keyword(s):  
Animal Model ◽  
Left Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Left Heart ◽  
Endocardial Fibroelastosis ◽  
Hypoplastic Left Heart ◽  
Fetal Imaging ◽  
Adult Rat ◽  
Human Fetal Heart ◽  
Left Heart Syndrome

Background.Endocardial fibroelastosis (EFE), characterized by a diffuse endocardial thickening through collagen and elastin fibers, develops in the human fetal heart restricting growth of the left ventricle (LV). Recent advances in fetal imaging indicate that EFE development is directly associated with a distended, poorly contractile LV in evolving hypoplastic left heart syndrome (HLHS). In this study, we developed an animal model of EFE by introducing this human fetal LV morphopathology to an immature rat heart.Methods and Results.A neonatal donor heart, in which aortic regurgitation (AR) was created, was heterotopically transplanted into a recipient adult rat. AR successfully induced the LV morphology of evolving HLHS in the transplanted donor hearts, which resulted in the development of significant EFE covering the entire LV cavity within two weeks postoperatively. In contrast, posttransplants with a competent aortic valve displayed unloaded LVs with a trace of EFE.Conclusions.We could show that distention of the immature LV in combination with stagnant flow triggers EFE development in this animal model. This model would serve as a robust tool to develop therapeutic strategies to treat EFE while providing insight into its pathogenesis.

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