scholarly journals Aetiology and 30-Year Long-Term Outcome of Children with Cardiomyopathy Necessitating Heart Transplantation

2020 ◽  
Vol 10 (4) ◽  
pp. 251
Author(s):  
Martin Zschirnt ◽  
Josef Thul ◽  
Hakan Akintürk ◽  
Klaus Valeske ◽  
Dietmar Schranz ◽  
...  

Studies assessing the long-term outcome after heart transplantation HTX in patients with cardiomyopathy (CM) in the paediatric age range are rare. The aim of this study was to determine the survival rate of children with CM undergoing HTX and to analyse how aetiology of cardiomyopathy influenced morbidity and mortality. We retrospectively analysed the medical records of children; who were transplanted in our centre between June 1988 and October 2019. 236 heart transplantations were performed since 1988 (9 re-transplants). 98 of 227 patients (43.2%) were transplanted because of CM. Survival rates were 93% after 1; 84% after 10 and 75% after 30 years. Overall; the aetiology of CM could be clearly identified in 37 subjects (37.7%). This rate increased up to 66.6% (12/19) by applying a comprehensive diagnostic workup since 2016. The survival rate was lower (p < 0.05) and neurocognitive deficits were more frequent (p = 0.001) in subjects with systemic diseases than in individuals with cardiac-specific conditions. These data indicate that the long-term survival rate of children with CM after HTX in experienced centers is high. A comprehensive diagnostic workup allows unraveling the basic defect in the majority of patients with CM undergoing HTX. Aetiology of CM affects morbidity and mortality in subjects necessitating HTX.

2017 ◽  
Vol 25 (6) ◽  
pp. 440-445 ◽  
Author(s):  
Marine Peretti ◽  
Dana M Radu ◽  
Karel Pfeuty ◽  
Antoine Dujon ◽  
Marc Riquet ◽  
...  

Background Pulmonary inflammatory pseudotumors are rare lesions that remain problematic in several aspects, especially regarding the therapeutic strategy. The goal of this study was to evaluate long-term survival in a multicenter series of patients who required surgery for pulmonary inflammatory pseudotumors. Methods Thirty-six cases of pulmonary inflammatory pseudotumors, operated on in 3 French thoracic surgery departments between 1989 and 2015, were studied retrospectively. We recorded pre-, peri- and postoperative data for each patient, and long-term survival was analyzed. Results There were 22 men and 14 women. Mean age was 53.5 years (range 14–81 years). Three pneumonectomies, 1 bilobectomy, 19 lobectomies, 2 segmentectomies, 10 wedge resections, and 1 biopsy were performed. Complete resection was carried out in 32 (88.8%) patients. Median follow-up was 76 months. Five-year and 10-year survival rates were respectively 86.8% and 81.7% (96% and 90% for patients with R0 resection). Conclusions Long-term survival was excellent for patients with pulmonary inflammatory pseudotumors who benefited from surgery, especially when surgical resection was complete. These results confirm that surgical resection must be proposed as the first-line treatment for patients with pulmonary inflammatory pseudotumors.


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.


2006 ◽  
Vol 24 (30) ◽  
pp. 4862-4866 ◽  
Author(s):  
Nirupa Murugaesu ◽  
Peter Schmid ◽  
Gairin Dancey ◽  
Roshan Agarwal ◽  
Lydia Holden ◽  
...  

Purpose Malignant ovarian germ cell tumors are rare and knowledge about prognostic parameters currently is limited. This study was undertaken to evaluate long-term outcome of patients with malignant ovarian germ cell tumors (MOGCTs) after chemotherapy and to assess prognostic parameters. Patients and Methods A total of 113 patients with stage IC to IV MOGCTs were included into this retrospective study. Patients were treated at two large regional cancer centers between 1977 and 2003. Results Ten-year recurrence-free and overall survival rates were 82% and 81%, respectively. A total of 20 patients experienced relapse, all within the first 8 years. Outcome after relapse was poor, with only 10% of patients achieving long-term survival. Univariate and multivariate analyses demonstrated that initial stage of disease (relative risk [RR], 5.96; 95% CI, 3.47 to 10.22; P = .03) and elevation of serum markers β-human chorionic gonadotropin and alpha-fetoprotein (RR, 3.90; 95% CI, 1.40 to 10.9; P = .009) were significant predictors of overall survival, whereas age at diagnosis was of no prognostic value. Conclusion This is the first study to identify stage and tumor markers as prognostic parameters for patients with MOGCTs. This might help to select patients for risk-adapted treatment. There is need for improvement of therapeutic strategies after relapse.


2017 ◽  
Vol 2 (2) ◽  
Author(s):  
Barnaby Luke Dean ◽  
Sophie Adamantos

<p><strong>Clinical bottom line:</strong></p><p>Currently available literature on canine pyothorax management is low quality, so it is difficult to draw meaningful conclusions from it. With this in mind, however, the literature does suggest that invasive medical management (thoracic drainage and lavage via in-dwelling thoracostomy tubes, and antimicrobial therapy) and surgical management (in conjunction with antimicrobial therapy) of canine pyothorax provide better long-term survival rates compared to non-invasive medical management (antimicrobial therapy, with or without thoracocentesis) alone. More definitive conclusions cannot be made until higher quality evidence (prospective, randomised, blinded) is available on the topic.</p><br /> <img src="https://www.veterinaryevidence.org/rcvskmod/icons/oa-icon.jpg" alt="Open Access" /> <img src="https://www.veterinaryevidence.org/rcvskmod/icons/pr-icon.jpg" alt="Peer Reviewed" />


2011 ◽  
Vol 59 (S 01) ◽  
Author(s):  
J Sindermann ◽  
I Alejnik ◽  
J Hoffmann ◽  
S Klotz ◽  
A Löher ◽  
...  

2019 ◽  
Vol 38 (4) ◽  
pp. S287-S288
Author(s):  
A. Wolfson ◽  
M. Kittleson ◽  
J. Patel ◽  
E. Kransdorf ◽  
R. Cole ◽  
...  

PEDIATRICS ◽  
1995 ◽  
Vol 96 (5) ◽  
pp. 974-976
Author(s):  
◽  
◽  
◽  

The survival rate for infants at the threshold of viability has been improving. However, there are insufficient data regarding the cost(s) of initial and ongoing care of these infants and the long-term outcome of survivors. Furthermore, there has been little study of the impact of obstetric management on the survival rates of extremely low birth weight infants and on long-term morbidities. Continued research on these issues is imperative, and physicians need to remain informed of changing statistics.


2009 ◽  
Vol 98 (3) ◽  
pp. 164-168 ◽  
Author(s):  
J. Virkkunen ◽  
M. Venermo ◽  
J. Saarinen ◽  
J. Salenius

Background and Aims: The ability to predict post-operative mortality reliably will be of assistance in making decisions concerning the treatment of an individual patient. The aim of this study was to test the GAS score as a predictor of post-operative mortality in vascular surgical patients. Material and Methods: A total of 157 consecutive patients who underwent an elective vascular surgical procedure were included in the study. The Cox proportional hazards model was used in analyzing the importance of various preoperative risk factors for the postoperative outcome. ASA and GAS were tested in predicting the short and long-term outcome. On the basis of the GAS cut-off value 77, patients were selected into low-risk (GAS low: GAS < 77) and high-risk (GAS high: GAS > = 77) groups, and the examined risk factors were analyzed to determine which of them had predictive value for the prognosis. Results: None of the patients in the GAS low group died, and mortality in the GAS high group was 4.8% (p = 0.03) at 30 days' follow-up. The 12-month survival rates were 98.6% and 78.6% (p = 0.0001), respectively, with the respective 5-year survival rates of 76.7% and 44.0% (p = 0.0001). The only independent risk factor for 30-day mortality was the renal risk factor (OR 20.2). The combination of all three GAS variables(chronic renal failure, cardiac disease and cerebrovascular disease), excluding age, was associated with a 100% two-year mortality. Conclusions: Mortality is low for patients with GAS<77. For the high-risk patients (GAS> = 77), due to its low predictive value for death, GAS yields limited value in clinical practice. In cases of patients with all three risk factors (renal, cardiac and cerebrovascular), vascular surgery should be considered very carefully.


Perfusion ◽  
2018 ◽  
Vol 33 (8) ◽  
pp. 687-695 ◽  
Author(s):  
Julia Merkle ◽  
Anton Sabashnikov ◽  
Carolyn Weber ◽  
Georg Schlachtenberger ◽  
Johanna Maier ◽  
...  

Objectives: Stanford A acute aortic dissection (AAD) is a life-threatening emergency, typically occurring in older patients and requiring immediate surgical repair. The aim of this study was to evaluate early outcome and short- and long-term survival of patients under and above 65 years of age. Methods: Two hundred and forty patients with Stanford A AAD underwent aortic surgical repair from January 2006 to April 2015 in our center. After statistical analysis and logistic regression analysis, Kaplan-Meier survival estimation was performed, with up to 9-year follow-up, comprising patients under and above 65 years of age. Results: The proportion of patients above 65 years of age suffering from Stanford A AAD was 50% (n=120). The group of patients above 65 years of age compared to the group under 65 years of age showed statistically significant differences in terms of higher odds ratios (OR) for hypertension (p=0.012), peripheral vascular disease (p=0.026) and tachyarrhythmia absoluta (p=0.004). Patients over 65 years of age also showed significantly poorer short- and long-term survival. Our subgroup analysis revealed that male patients (Breslow p=0.001, Log-Rank p=0.001) and patients suffering with hypertension (Breslow p=0.003, Log-Rank p=0.001) were reasonable for these results whereas younger and older female patients showed similar short- and long-term outcome (Breslow p=0.926, Log-Rank p=0.724). After stratifying all patients into 4 age groups (<45; 55-65; 65-75; >75years), short-term survival of the patients appeared to be significantly poorer with increasing age (Breslow p=0.026, Log-Rank p=0.008) whereas long-term survival of patients free from cerebrovascular events (Breslow p=0.0494, Log-Rank p=0.489) remained similar. Conclusions: All patients referred to our hospital for repair of Stanford A AAD with higher age had poorer short- and long-term survival, caused by male patients and patients suffering from hypertension, whereas survival of women and survival free from cerebrovascular events of the entire patient cohort was similar, irrespective of age.


Sign in / Sign up

Export Citation Format

Share Document