Outcomes for patients with an aortopulmonary window, and the impact of associated cardiovascular lesions

2004 ◽  
Vol 14 (5) ◽  
pp. 473-480 ◽  
Author(s):  
Rajesh Bagtharia ◽  
Kalyani R. Trivedi ◽  
Harold M. Burkhart ◽  
William G. Williams ◽  
Robert M. Freedom ◽  
...  
Keyword(s):  
Aortic Arch ◽  
Correct Diagnosis ◽  
Interrupted Aortic Arch ◽  
Clinical Findings ◽  
Aortopulmonary Window ◽  
Kaplan Meier ◽  
Associated Lesions ◽  
Lost To Follow Up ◽  
The Impact

An aortopulmonary window is a communication between the ascending aorta and pulmonary trunk in the presence of two separate arterial valves, and is often complicated by other associated defects. We sought to determine management and related outcomes in patients with this malformation.We identified those patients presenting between 1969 and 1999 from the databases held in our Departments of Cardiology, Pathology and Cardiovascular Surgery. We obtained data relating to issues concerning demography, clinical findings, imaging, management and outcome.The median age at presentation for the 42 patients identified, of whom 23 were female, was 62 days, with a range from birth to 6 years. Associated cardiac defects were present in 34 patients, including interruption of the aortic arch in 6 patients. The correct diagnosis was initially missed in 13 patients. Of the patients, six died without surgical repair, and 1 patient was lost-to-follow-up. Repair was performed in 35 patients, subsequent to repair of other defects in 4, in association with repair of other defects in 17, of whom 3 died, and as an isolated procedure in 14 patients, one of the latter being treated by transcatheter closure. Overall, there were 9 deaths, all in patients with complex associated defects, except 1 patient with a missed aortopulmonary window after repair of aortic coarctation. Kaplan-Meier estimates of survival were 81% at 3 months until 11.5 years, and 69% up to 21 years. Only the presence of interrupted aortic arch was independently associated with increased time-related mortality, the hazard ratio being 5.87 (p = 0.009).The outcome for an isolated lesion is excellent. Mortality occurs mainly before repair, mostly with complex associated lesions, particularly interruption of the aortic arch.

The Efficacy of Etoposide-Based Therapy in Adult Secondary Hemophagocytic Lymphohistiocytosis

2021 ◽  
pp. 1-9
Author(s):  
Leonard Naymagon ◽  
Douglas Tremblay ◽  
John Mascarenhas
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Proportional Hazards ◽  
Multivariable Analysis ◽  
Cox Proportional Hazards ◽  
Rank Test ◽  
Kaplan Meier ◽  
Hazard Ratios ◽  
Significant Difference ◽  
The Impact

Data supporting the use of etoposide-based therapy in hemophagocytic lymphohistiocytosis (HLH) arise largely from pediatric studies. There is a lack of comparable data among adult patients with secondary HLH. We conducted a retrospective study to assess the impact of etoposide-based therapy on outcomes in adult secondary HLH. The primary outcome was overall survival. The log-rank test was used to compare Kaplan-Meier distributions of time-to-event outcomes. Multivariable Cox proportional hazards modeling was used to estimate adjusted hazard ratios (HRs) with 95% confidence intervals (CIs). Ninety adults with secondary HLH seen between January 1, 2009, and January 6, 2020, were included. Forty-two patients (47%) received etoposide-based therapy, while 48 (53%) received treatment only for their inciting proinflammatory condition. Thirty-three patients in the etoposide group (72%) and 32 in the no-etoposide group (67%) died during follow-up. Median survival in the etoposide and no-etoposide groups was 1.04 and 1.39 months, respectively. There was no significant difference in survival between the etoposide and no-etoposide groups (log-rank <i>p</i> = 0.4146). On multivariable analysis, there was no association between treatment with etoposide and survival (HR for death with etoposide = 1.067, 95% CI: 0.633–1.799, <i>p</i> = 0.8084). Use of etoposide-based therapy was not associated with improvement in outcomes in this large cohort of adult secondary HLH patients.

Aortopulmonary Window Associated with Interrupted Aortic Arch: Report of Surgical Repair of Eight Cases and Review of Literature

2012 ◽  
Vol 60 (03) ◽  
pp. 215-220 ◽  
Author(s):  
Peter Murin ◽  
Nicodème Sinzobahamvya ◽  
Hedwig Blaschczok ◽  
Joachim Photiadis ◽  
Christoph Haun ◽  
...  
Keyword(s):  
Aortic Arch ◽  
Surgical Repair ◽  
Interrupted Aortic Arch ◽  
Aortopulmonary Window ◽  
Review Of Literature

The impact of multimodality therapies in marginally inoperable soft tissue sarcomas (STS): The Toronto Sarcoma Program (TSP) experience.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 11548-11548
Author(s):  
Olga Vornicova ◽  
Jay Wunder ◽  
Peter W. M. Chung ◽  
Abha A. Gupta ◽  
Rebecca Anne Gladdy ◽  
...  
Keyword(s):  
Cox Regression ◽  
Soft Tissue Sarcomas ◽  
Tumor Board ◽  
Disease Characteristics ◽  
Kaplan Meier ◽  
Progression Of Disease ◽  
Modality Approach ◽  
Poor Outcomes ◽  
The Impact

11548 Background: The mainstay therapy of operable STS remains surgery, which may include (neo)adjuvant therapies. Within the TSP, marginally inoperable STS are often treated with sequential chemo (CTX) and radiation (RT) therapy, followed by surgery (SX). Herein we present our experience of multi-modality therapies for marginally inoperable STS patients (pts). Methods: This was a dual-center, single program, retrospective review. Pts were included if deemed to have marginally inoperable primary or recurrent STS, as determined at the TSP tumor board. Pts included must have had CTX with the intent of having RT and SX after. Pts demographics, treatment details and clinical outcomes data were collected. Relapse free survival (RFS) and overall survival (OS) were estimated using the Kaplan-Meier method. Multivariate analysis of the influence of disease characteristics and treatment on outcomes was assessed using Cox regression. Results: From June 2005 to May 2019, 75 pts were identified. Median age was 52 years (range 16-72). Pts were predominantly male (55%). Histological subtypes included dedifferentiated liposarcoma (29%), leiomyosarcoma (27%), synovial sarcoma (19%) and others (25%). Primary tumor was located in the retroperitoneum (48%), extremity (23%), pelvis (12%), thorax (9%), and other sites (8%). All pts had doxorubicin and ifosfamide CTX (median 4 cycles; range 1-6), while RT dose delivered was 50.4Gy/28 fractions in 58 (77%) of cases. Twenty three pts (31%) achieved partial response, 40 pts (53%) had stable disease and 12 pts (16%) had progression of disease (PD) on CTX, of which half (8%) did not undergo further treatment. Nine pts (12%) underwent CTX followed by SX due to significant response, 9 pts (12%) underwent CTX and RT without SX due to persistent tumor unresectability or PD. The final 50 pts (67%) completed multi-modality treatment (CTX, RT & SX). Overall, 59 pts (79%) had SX; negative margins were achieved in 53 (71%). 19 pts (25%) had postoperative complications, causing death in 2 pts (2.7%). With a median follow-up of 72 months, median RFS and OS were 26.9 months (95% CI: 0-86.0), and 65 months (95% CI: 13.5-116.4). Extremity location was associated with superior RFS (median not reached [NR], HR 0.28 95% CI 0.09-0.83, p = 0.022), and OS (median NR, HR 0.29 95% CI 0.09-0.90, p = 0.032). Receipt of RT was associated with superior RFS (median NR, HR 0.23 95% CI 0.10-0.52, p < 0.001); and OS (median NR, HR 0.21 95% CI 0.09-0.50, p < 0.001). Pts who had PD after CTX were associated with poor outcomes - RFS (median 4.7 months, HR 2.03 95% CI 0.61-6.76, p = 0.24); and OS (median 21.9 months, HR 2.48 95% CI 0.73-8.47, P = 0.144). Conclusions: Multi-modality approach resulted in successful resection for most pts with marginally inoperable STS. Extremity location and RT administration were associated with better RFS and OS, while progression on CTX confers worse survival outcomes.

Interrupted aortic arch and aortopulmonary window: One-stage repair in the first week of life

1993 ◽  
Vol 56 (3) ◽  
pp. 554-556 ◽  
Author(s):  
Gaetano Gargiulo ◽  
Lucio Zannini ◽  
Sonia-Bernadette Albanese ◽  
Guido Frascaroli ◽  
Maria-Cristina Santordli ◽  
...  
Keyword(s):  
Aortic Arch ◽  
Interrupted Aortic Arch ◽  
Aortopulmonary Window ◽  
One Stage

scholarly journals Impact of Moderate to Severe Renal Impairment on Mortality and Appropriate Shocks in Patients with Implantable Cardioverter Defibrillators

2010 ◽  
Vol 2010 ◽  
pp. 1-6 ◽  
Author(s):  
Venkata M. Alla ◽  
Kishlay Anand ◽  
Mandeep Hundal ◽  
Aimin Chen ◽  
Showri Karnam ◽  
...  
Keyword(s):  
Linear Models ◽  
Severe Renal Impairment ◽  
Medical Center ◽  
Implantable Cardioverter Defibrillators ◽  
Free Survival ◽  
Kaplan Meier ◽  
Appropriate Shocks ◽  
The Impact ◽  
Cardioverter Defibrillators

Background. Due to underrepresentation of patients with chronic kidney disease (CKD) in large Implantable-Cardioverter Defibrillator (ICD) clinical trials, the impact of ICD remains uncertain in this population.Methods. Consecutive patients who received ICD at Creighton university medical center between years 2000–2004 were included in a retrospective cohort after excluding those on maintenance dialysis. Based on baseline Glomerular filtration rate (GFR), patients were classified as severe CKD: GFR < 30 mL/min; moderate CKD: GFR: 30–59 mL/min; and mild or no CKD: GFR ≥ 60 mL/min. The impact of GFR on appropriate shocks and survival was assessed using Kaplan-Meier method and Generalized Linear Models (GLM) with log-link function.Results. There were 509 patients with a mean follow-up of 3.0 + 1.3 years. Mortality risk was inversely proportional to the estimated GFR: 2 fold higher risk with GFR between 30–59 mL/min and 5 fold higher risk with GFR < 30 mL/min. One hundred and seventy-seven patients received appropriate shock(s); appropriate shock-free survival was lower in patients with severe CKD (GFR < 30) compared to mild or no CKD group (2.8 versus 4.2 yrs).Conclusion. Even moderate renal dysfunction increases all cause mortality in CKD patients with ICD. Severe but not moderate CKD is an independent predictor for time to first appropriate shock.

Surgical repair of interrupted aortic arch with ventricular septal defect

1998 ◽  
Vol 8 (2) ◽  
pp. 217-220 ◽  
Author(s):  
Lindsey D Allan ◽  
Howard D Apfel ◽  
Yosef Levenbrown ◽  
Jan M Quaegebeur
Keyword(s):  
Ventricular Septal Defect ◽  
Aortic Arch ◽  
Septal Defect ◽  
Ventricular Outflow Tract ◽  
Interrupted Aortic Arch ◽  
Left Ventricular ◽  
Subaortic Stenosis ◽  
Current Debate ◽  
Aortic Orifice

AbstractBackgroundInterrupted aortic arch is often associated with subaortic narrowing and hypoplasia of the aortic orifice. The best surgical strategy for the management of these additional lesions is a matter of current debate.Methods and ResultsBetween 1986 and 1996, 19 patients underwent repair of interrupted aortic arch with closure of ventricular septal defect in a single stage, with no attempt at subaortic resection, irrespective of the dimensions of the left ventricular outflow tract. There was no perioperative hospital mortality, and all patients were alive at 1 year. Follow-up ranges from 0.75 −10 years, with a mean 4.2 ± 3.0 years. Seven patients (37%) have required reintervention for relief of subaortic stenosis, 2 of whom have died.ConclusionsOur results suggest that primary one-stage biventricular repair can be accomplished with low perioperative mortality without addressing the subaortic region. Further long-term follow-up will determine whether this is accomplished at the expense of later morbidity and mortality.

Interrupted aortic arch associated with aortopulmonary window in a 20 year-old young adult

2011 ◽  
Vol 149 (3) ◽  
pp. e120-e122 ◽  
Author(s):  
Alper Ucak ◽  
Kaan Inan ◽  
Burak Onan ◽  
Selami Suleymanoglu ◽  
Veysel Temizkan ◽  
...  
Keyword(s):  
Young Adult ◽  
Aortic Arch ◽  
Interrupted Aortic Arch ◽  
Aortopulmonary Window

Surgical Management for Late Presentation of Interrupted Aortic Arch with Aortopulmonary Window

2021 ◽  
pp. 215013512110543
Author(s):  
Héctor Díliz Nava ◽  
Roberto Perezgrovas Olaria ◽  
Luis García Benítez ◽  
Orlando Tamariz Cruz ◽  
Krystell Martinez Balderas ◽  
...  
Keyword(s):  
Decision Making ◽  
Aortic Arch ◽  
Surgical Management ◽  
Late Presentation ◽  
Interrupted Aortic Arch ◽  
Surgical Correction ◽  
Aortopulmonary Window ◽  
Excellent Opportunity ◽  
Aortic Arch Repair ◽  
Repair Techniques

We report a case of advanced age at presentation of interrupted aortic arch with aortopulmonary window. Although the association between these two defects is well established, their overall prevalence is extremely low. This case presents us with an excellent opportunity to discuss decision making regarding aortic arch repair techniques according to age at which primary surgical correction is intended.

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