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Christopher A Caldarone - One of the best experts on this subject based on the ideXlab platform.
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repair type influences mode of Pulmonary Vein stenosis in total anomalous Pulmonary venous drainage
The Annals of Thoracic Surgery, 2015Co-Authors: Mauro Lo Rito, Glen S Van Arsdell, Christopher A Caldarone, Tamadhir Gazzaz, Travis J Wilder, Arezou Saedi, Devin Chetan, Shi Joon Yoo, Osami HonjoAbstract:Background We hypothesized that primary sutureless (SL) repair of total anomalous Pulmonary venous drainage (TAPVD) may have a lower incidence of postrepair Pulmonary Vein Obstruction (PVO) and different modes of PVO compared with standard repair (SR). Methods One hundred ninety-five patients who underwent TAPVD repair (1990 to 2012) with the exception of congenital Pulmonary Vein stenosis, isomerism, and single-ventricle anomalies were included. Survival, reintervention, incidence, degree of PVO were compared between groups. The mode of PVO was expressed as central or peripheral. The Mann-Whitney test, Kaplan-Meier analysis, and Cox regression were used. Results The SL group had more infracardiac or mixed TAPVD ( p = 0.02) and preoperative PVO ( p = 0.07). There were no differences between SR and SL groups in survival (5-year survival, 83.1% versus 82.5%, respectively; p = 0.73) and composite outcome (death, intervention, PVO, 5-year survival, 76.4% versus 80.7%, respectively; p = 0.225). The SL group had a lower incidence of PVO of moderate or greater degree (SR, 11.3% versus SL, 2.9%; p = 0.05) than the SR group, especially in the infracardiac and mixed TAPVD cohort ( p = 0.011), with a lower Pulmonary Vein score (SR, 8 versus SL, 4; p = 0.01). The SL group had peripheral PVO exclusively (100%), whereas the SR group predominantly had central PVO (76.4%; p = 0.005). There was a trend toward less reoperation in the SL group (SR, 10.4% versus SL, 2.9%; p = 0.08). Survival after reoperation was comparable to primary TAPVD repair types as well as reoperation repair types. Conclusions Primary SL appeared to be associated with a lower incidence and severity of PVO. The primary SL repair eliminated the risk of developing central PVO, although a relatively benign type of peripheral PVO could occur.
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anatomical risk factors surgical treatment and clinical outcomes of left sided Pulmonary Vein Obstruction in single ventricle patients
The Journal of Thoracic and Cardiovascular Surgery, 2015Co-Authors: Yasuhiro Kotani, Jiaquan Zhu, Lars Grossewortmann, Osami Honjo, John G Coles, Glen S Van Arsdell, Christopher A CaldaroneAbstract:Abstract Background Patients with single-ventricle physiology frequently develop left-sided Pulmonary Vein Obstruction (PVO), in which the Pulmonary Veins traverse the descending thoracic aorta. We hypothesized that a combination of cardiomegaly and an anteriorly positioned descending aorta is associated with PVO. Methods Among 494 consecutive single-ventricle patients, 15 were diagnosed with PVO by cardiac magnetic resonance, defined as anatomically localized narrowing of the Pulmonary Vein diameter. Using axial slices at the level of the left lower Pulmonary Vein, normalized dimensions were obtained to characterize the anatomic relationships of intrathoracic structures. Measurements were compared between patients with PVO and “control” patients (single-ventricle patients with normal Pulmonary Veins, n = 12). Results Patients with cardiac magnetic resonance–diagnosed PVO had larger cardiac size and more antero-laterally located descending aorta when compared with controls (normalized dimensions: cardiac/thoracic area ratio: 0.43 vs 0.38, P = .035, distance from vertebra to descending aorta normalized by the horizontal dimension of thoracic cavity: 0.09 vs 0.08, P = .049). Seven (47%) patients underwent PV sutureless repair, and 3 (of 7) failed to achieve Fontan. Patients who failed to achieve Fontan had a larger normalized cardiac size than those who achieved Fontan (cardiac/thoracic area ratio: 0.49 vs 0.39, P = .001). Conclusions The combination of relative cardiomegaly within the context of the thoracic cavity at the level of the Pulmonary Veins and antero-lateral displacement of the aorta is associated with left-sided PVO and subsequent failure to achieve Fontan completion. Further characterization of these unique geometric relationships may help inform both surveillance strategies and decision making in the timing of interventions, and guide the intraoperative objectives at the time of PVO repair.
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outcomes after surgical treatment of children with partial anomalous Pulmonary venous connection
The Annals of Thoracic Surgery, 2007Co-Authors: Bahaaldin Alsoufi, William G Williams, Glen S Van Arsdell, Christopher A Caldarone, Sally Cai, John G ColesAbstract:Background. We explore early results and time-related morbidity after surgical repair of partial anomalous Pulmonary venous connection (PAPVC) at our institution. Methods. Between 1982 and 2006, 306 consecutive patients underwent surgery for PAPVC; of these, 236 (77%) were children with a median age of 5.3 years (range, 0.47 to 18 years). Clinical and echocardiographic follow-up was obtained. Results. PAPVC was right-sided in 214 patients (90%), left-sided in 17 (7%), and bilateral in 5 (2%). Anomalous Veins were partial in 186 patients (79%) and involved the entire lung in 50 (21%). The most common type was right PAPVC into the superior vena cava in 175 (74%), with 87% associated with sinus-venosus atrial septal defect; followed by right PAPVC into the right atrium in 29 (12%), left PAPVC into the innominate Vein in 22 (9%), and scimitar syndrome in 15 (6%). Repair strategy included intracardiac baffle in 203, Pulmonary Vein implantation in 22, and SVC division with reimplantation on the right atrial appendage in 14. There was no early or late mortality. Freedom from reoperation, vena cava Obstruction, Pulmonary Vein Obstruction, and pacemaker implantation at 15 years was 97%, 97.8%, 86%, and 99.1%, respectively. Pulmonary Vein Obstruction was significantly more common in patients with scimitar compared with others (22.4% versus 98.3%, p < 0.0001). Postoperative quantitative lung perfusion scans in 13 of 15 scimitar patients showed decreased right lung perfusion (mean, 22.5%). Conclusions. Surgical treatment of PAPVC is associated with excellent outcomes and low time-related morbidity. However, management of children with scimitar syndrome is complicated by an exceptionally high incidence of postoperative Pulmonary venous Obstruction and abnormally diminished perfusion of the right lung.
John G Coles - One of the best experts on this subject based on the ideXlab platform.
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anatomical risk factors surgical treatment and clinical outcomes of left sided Pulmonary Vein Obstruction in single ventricle patients
The Journal of Thoracic and Cardiovascular Surgery, 2015Co-Authors: Yasuhiro Kotani, Jiaquan Zhu, Lars Grossewortmann, Osami Honjo, John G Coles, Glen S Van Arsdell, Christopher A CaldaroneAbstract:Abstract Background Patients with single-ventricle physiology frequently develop left-sided Pulmonary Vein Obstruction (PVO), in which the Pulmonary Veins traverse the descending thoracic aorta. We hypothesized that a combination of cardiomegaly and an anteriorly positioned descending aorta is associated with PVO. Methods Among 494 consecutive single-ventricle patients, 15 were diagnosed with PVO by cardiac magnetic resonance, defined as anatomically localized narrowing of the Pulmonary Vein diameter. Using axial slices at the level of the left lower Pulmonary Vein, normalized dimensions were obtained to characterize the anatomic relationships of intrathoracic structures. Measurements were compared between patients with PVO and “control” patients (single-ventricle patients with normal Pulmonary Veins, n = 12). Results Patients with cardiac magnetic resonance–diagnosed PVO had larger cardiac size and more antero-laterally located descending aorta when compared with controls (normalized dimensions: cardiac/thoracic area ratio: 0.43 vs 0.38, P = .035, distance from vertebra to descending aorta normalized by the horizontal dimension of thoracic cavity: 0.09 vs 0.08, P = .049). Seven (47%) patients underwent PV sutureless repair, and 3 (of 7) failed to achieve Fontan. Patients who failed to achieve Fontan had a larger normalized cardiac size than those who achieved Fontan (cardiac/thoracic area ratio: 0.49 vs 0.39, P = .001). Conclusions The combination of relative cardiomegaly within the context of the thoracic cavity at the level of the Pulmonary Veins and antero-lateral displacement of the aorta is associated with left-sided PVO and subsequent failure to achieve Fontan completion. Further characterization of these unique geometric relationships may help inform both surveillance strategies and decision making in the timing of interventions, and guide the intraoperative objectives at the time of PVO repair.
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outcomes after surgical treatment of children with partial anomalous Pulmonary venous connection
The Annals of Thoracic Surgery, 2007Co-Authors: Bahaaldin Alsoufi, William G Williams, Glen S Van Arsdell, Christopher A Caldarone, Sally Cai, John G ColesAbstract:Background. We explore early results and time-related morbidity after surgical repair of partial anomalous Pulmonary venous connection (PAPVC) at our institution. Methods. Between 1982 and 2006, 306 consecutive patients underwent surgery for PAPVC; of these, 236 (77%) were children with a median age of 5.3 years (range, 0.47 to 18 years). Clinical and echocardiographic follow-up was obtained. Results. PAPVC was right-sided in 214 patients (90%), left-sided in 17 (7%), and bilateral in 5 (2%). Anomalous Veins were partial in 186 patients (79%) and involved the entire lung in 50 (21%). The most common type was right PAPVC into the superior vena cava in 175 (74%), with 87% associated with sinus-venosus atrial septal defect; followed by right PAPVC into the right atrium in 29 (12%), left PAPVC into the innominate Vein in 22 (9%), and scimitar syndrome in 15 (6%). Repair strategy included intracardiac baffle in 203, Pulmonary Vein implantation in 22, and SVC division with reimplantation on the right atrial appendage in 14. There was no early or late mortality. Freedom from reoperation, vena cava Obstruction, Pulmonary Vein Obstruction, and pacemaker implantation at 15 years was 97%, 97.8%, 86%, and 99.1%, respectively. Pulmonary Vein Obstruction was significantly more common in patients with scimitar compared with others (22.4% versus 98.3%, p < 0.0001). Postoperative quantitative lung perfusion scans in 13 of 15 scimitar patients showed decreased right lung perfusion (mean, 22.5%). Conclusions. Surgical treatment of PAPVC is associated with excellent outcomes and low time-related morbidity. However, management of children with scimitar syndrome is complicated by an exceptionally high incidence of postoperative Pulmonary venous Obstruction and abnormally diminished perfusion of the right lung.
Glen S Van Arsdell - One of the best experts on this subject based on the ideXlab platform.
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repair type influences mode of Pulmonary Vein stenosis in total anomalous Pulmonary venous drainage
The Annals of Thoracic Surgery, 2015Co-Authors: Mauro Lo Rito, Glen S Van Arsdell, Christopher A Caldarone, Tamadhir Gazzaz, Travis J Wilder, Arezou Saedi, Devin Chetan, Shi Joon Yoo, Osami HonjoAbstract:Background We hypothesized that primary sutureless (SL) repair of total anomalous Pulmonary venous drainage (TAPVD) may have a lower incidence of postrepair Pulmonary Vein Obstruction (PVO) and different modes of PVO compared with standard repair (SR). Methods One hundred ninety-five patients who underwent TAPVD repair (1990 to 2012) with the exception of congenital Pulmonary Vein stenosis, isomerism, and single-ventricle anomalies were included. Survival, reintervention, incidence, degree of PVO were compared between groups. The mode of PVO was expressed as central or peripheral. The Mann-Whitney test, Kaplan-Meier analysis, and Cox regression were used. Results The SL group had more infracardiac or mixed TAPVD ( p = 0.02) and preoperative PVO ( p = 0.07). There were no differences between SR and SL groups in survival (5-year survival, 83.1% versus 82.5%, respectively; p = 0.73) and composite outcome (death, intervention, PVO, 5-year survival, 76.4% versus 80.7%, respectively; p = 0.225). The SL group had a lower incidence of PVO of moderate or greater degree (SR, 11.3% versus SL, 2.9%; p = 0.05) than the SR group, especially in the infracardiac and mixed TAPVD cohort ( p = 0.011), with a lower Pulmonary Vein score (SR, 8 versus SL, 4; p = 0.01). The SL group had peripheral PVO exclusively (100%), whereas the SR group predominantly had central PVO (76.4%; p = 0.005). There was a trend toward less reoperation in the SL group (SR, 10.4% versus SL, 2.9%; p = 0.08). Survival after reoperation was comparable to primary TAPVD repair types as well as reoperation repair types. Conclusions Primary SL appeared to be associated with a lower incidence and severity of PVO. The primary SL repair eliminated the risk of developing central PVO, although a relatively benign type of peripheral PVO could occur.
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anatomical risk factors surgical treatment and clinical outcomes of left sided Pulmonary Vein Obstruction in single ventricle patients
The Journal of Thoracic and Cardiovascular Surgery, 2015Co-Authors: Yasuhiro Kotani, Jiaquan Zhu, Lars Grossewortmann, Osami Honjo, John G Coles, Glen S Van Arsdell, Christopher A CaldaroneAbstract:Abstract Background Patients with single-ventricle physiology frequently develop left-sided Pulmonary Vein Obstruction (PVO), in which the Pulmonary Veins traverse the descending thoracic aorta. We hypothesized that a combination of cardiomegaly and an anteriorly positioned descending aorta is associated with PVO. Methods Among 494 consecutive single-ventricle patients, 15 were diagnosed with PVO by cardiac magnetic resonance, defined as anatomically localized narrowing of the Pulmonary Vein diameter. Using axial slices at the level of the left lower Pulmonary Vein, normalized dimensions were obtained to characterize the anatomic relationships of intrathoracic structures. Measurements were compared between patients with PVO and “control” patients (single-ventricle patients with normal Pulmonary Veins, n = 12). Results Patients with cardiac magnetic resonance–diagnosed PVO had larger cardiac size and more antero-laterally located descending aorta when compared with controls (normalized dimensions: cardiac/thoracic area ratio: 0.43 vs 0.38, P = .035, distance from vertebra to descending aorta normalized by the horizontal dimension of thoracic cavity: 0.09 vs 0.08, P = .049). Seven (47%) patients underwent PV sutureless repair, and 3 (of 7) failed to achieve Fontan. Patients who failed to achieve Fontan had a larger normalized cardiac size than those who achieved Fontan (cardiac/thoracic area ratio: 0.49 vs 0.39, P = .001). Conclusions The combination of relative cardiomegaly within the context of the thoracic cavity at the level of the Pulmonary Veins and antero-lateral displacement of the aorta is associated with left-sided PVO and subsequent failure to achieve Fontan completion. Further characterization of these unique geometric relationships may help inform both surveillance strategies and decision making in the timing of interventions, and guide the intraoperative objectives at the time of PVO repair.
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outcomes after surgical treatment of children with partial anomalous Pulmonary venous connection
The Annals of Thoracic Surgery, 2007Co-Authors: Bahaaldin Alsoufi, William G Williams, Glen S Van Arsdell, Christopher A Caldarone, Sally Cai, John G ColesAbstract:Background. We explore early results and time-related morbidity after surgical repair of partial anomalous Pulmonary venous connection (PAPVC) at our institution. Methods. Between 1982 and 2006, 306 consecutive patients underwent surgery for PAPVC; of these, 236 (77%) were children with a median age of 5.3 years (range, 0.47 to 18 years). Clinical and echocardiographic follow-up was obtained. Results. PAPVC was right-sided in 214 patients (90%), left-sided in 17 (7%), and bilateral in 5 (2%). Anomalous Veins were partial in 186 patients (79%) and involved the entire lung in 50 (21%). The most common type was right PAPVC into the superior vena cava in 175 (74%), with 87% associated with sinus-venosus atrial septal defect; followed by right PAPVC into the right atrium in 29 (12%), left PAPVC into the innominate Vein in 22 (9%), and scimitar syndrome in 15 (6%). Repair strategy included intracardiac baffle in 203, Pulmonary Vein implantation in 22, and SVC division with reimplantation on the right atrial appendage in 14. There was no early or late mortality. Freedom from reoperation, vena cava Obstruction, Pulmonary Vein Obstruction, and pacemaker implantation at 15 years was 97%, 97.8%, 86%, and 99.1%, respectively. Pulmonary Vein Obstruction was significantly more common in patients with scimitar compared with others (22.4% versus 98.3%, p < 0.0001). Postoperative quantitative lung perfusion scans in 13 of 15 scimitar patients showed decreased right lung perfusion (mean, 22.5%). Conclusions. Surgical treatment of PAPVC is associated with excellent outcomes and low time-related morbidity. However, management of children with scimitar syndrome is complicated by an exceptionally high incidence of postoperative Pulmonary venous Obstruction and abnormally diminished perfusion of the right lung.
Jing Zhou - One of the best experts on this subject based on the ideXlab platform.
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outcome predictors and implications for management of scimitar syndrome
American Heart Journal, 2013Co-Authors: Susan M Dusenbery, Tal Geva, Anna Seale, Anne Marie Valente, Jing ZhouAbstract:Background Scimitar syndrome is a rare congenital anomaly. We evaluated risk factors for postoperative Pulmonary Vein stenosis or death and predictive factors for survival without scimitar Vein surgery in patients with scimitar syndrome. Methods The records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed. Results Scimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortoPulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and Pulmonary hypertension. Of 36 patients having scimitar Vein surgery, 18 had postoperative Pulmonary Vein Obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants ( P = .10). Overall, 19 (24%) of 80 died. Multivariate risk factors for death included systolic Pulmonary pressure >0.5 systemic level ( P = .007) and left Pulmonary Vein stenosis ( P = .009). Pulmonary artery systolic pressure P = .01) and absence of CHD excluding atrial septal defect ( P = .01) were predictive factors in 28 patients who survived and did not have scimitar Vein surgery; these patients had no or mild right ventricular dilation and a ratio of Pulmonary-to-systemic flow Conclusions Postoperative Pulmonary Vein Obstruction is common after scimitar Vein surgery regardless of redirection technique. Pulmonary hypertension and left Pulmonary Vein stenosis are risk factors for death, whereas patients without significant Pulmonary hypertension or associated CHD did well without scimitar Vein surgery. These observations may guide management decisions in patients with scimitar syndrome.
William G Williams - One of the best experts on this subject based on the ideXlab platform.
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outcomes after surgical treatment of children with partial anomalous Pulmonary venous connection
The Annals of Thoracic Surgery, 2007Co-Authors: Bahaaldin Alsoufi, William G Williams, Glen S Van Arsdell, Christopher A Caldarone, Sally Cai, John G ColesAbstract:Background. We explore early results and time-related morbidity after surgical repair of partial anomalous Pulmonary venous connection (PAPVC) at our institution. Methods. Between 1982 and 2006, 306 consecutive patients underwent surgery for PAPVC; of these, 236 (77%) were children with a median age of 5.3 years (range, 0.47 to 18 years). Clinical and echocardiographic follow-up was obtained. Results. PAPVC was right-sided in 214 patients (90%), left-sided in 17 (7%), and bilateral in 5 (2%). Anomalous Veins were partial in 186 patients (79%) and involved the entire lung in 50 (21%). The most common type was right PAPVC into the superior vena cava in 175 (74%), with 87% associated with sinus-venosus atrial septal defect; followed by right PAPVC into the right atrium in 29 (12%), left PAPVC into the innominate Vein in 22 (9%), and scimitar syndrome in 15 (6%). Repair strategy included intracardiac baffle in 203, Pulmonary Vein implantation in 22, and SVC division with reimplantation on the right atrial appendage in 14. There was no early or late mortality. Freedom from reoperation, vena cava Obstruction, Pulmonary Vein Obstruction, and pacemaker implantation at 15 years was 97%, 97.8%, 86%, and 99.1%, respectively. Pulmonary Vein Obstruction was significantly more common in patients with scimitar compared with others (22.4% versus 98.3%, p < 0.0001). Postoperative quantitative lung perfusion scans in 13 of 15 scimitar patients showed decreased right lung perfusion (mean, 22.5%). Conclusions. Surgical treatment of PAPVC is associated with excellent outcomes and low time-related morbidity. However, management of children with scimitar syndrome is complicated by an exceptionally high incidence of postoperative Pulmonary venous Obstruction and abnormally diminished perfusion of the right lung.
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management and outcomes of right atrial isomerism a 26 year experience
Journal of the American College of Cardiology, 1998Co-Authors: Aijaz Hashmi, Riyadh M Abusulaiman, Brian W Mccrindle, Jeffrey F Smallhorn, William G Williams, Robert M FreedomAbstract:Abstract Objectives. We sought to determine, in a large series of patients with right atrial isomerism, factors associated with mortality. Background. Right atrial isomerism is associated with complex congenital heart disease and high morbidity and mortality. Method. All data from patients diagnosed with right atrial isomerism between January 1970 and March 1996 were reviewed. Results. A total of 91 consecutive patients (54 male) were identified. Most patients (89%) presented within the first month of life, 62% at birth. Cardiac abnormalities included common atrioventricular (AV) valve (81%), ventricular hypoplasia or single ventricle (73%), abnormal ventriculoarterial connections (96%), Pulmonary outflow tract Obstruction (84%), anomalous Pulmonary venous drainage (87%) and Pulmonary Vein Obstruction (30%). The overall mortality rate was 69%. No interventions were planned or performed in 24%, 95% of whom died. The mortality rate for patients requiring their first cardiovascular operation in the neonatal period was 75% versus 51% for those with later first operations (p Conclusions. Right atrial isomerism continues to have an associated high mortality despite surgical innovations. Management of Pulmonary Vein Obstruction remains a serious problem and is associated with high mortality.
