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Frank L Hanley - One of the best experts on this subject based on the ideXlab platform.
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surgical algorithm and results for repair of pulmonary atresia with Ventricular Septal Defect and major aortopulmonary collaterals
The Journal of Thoracic and Cardiovascular Surgery, 2018Co-Authors: Richard D Mainwaring, William L Patrick, Stephen J Roth, Komal Kamra, Lisa Wisefaberowski, Michal Palmon, Frank L HanleyAbstract:Abstract Objective Pulmonary atresia with Ventricular Septal Defect and major aortopulmonary collateral arteries is a complex and heterogeneous form of congenital heart disease. There is a controversy regarding the optimal treatment of pulmonary atresia with Ventricular Septal Defect and major aortopulmonary collateral arteries. The purpose of this study was to summarize our algorithm and surgical results for pulmonary atresia with Ventricular Septal Defect and major aortopulmonary collateral arteries. Methods This was a retrospective review of 307 patients undergoing primary surgical treatment of pulmonary atresia with Ventricular Septal Defect and major aortopulmonary collateral arteries. Excluded from this analysis were patients who had undergone prior surgical treatment at another institution and patients with single ventricle and major aortopulmonary collateral arteries. There were 3 surgical pathways, including midline unifocalization (n = 241), creation of an aortopulmonary window (n = 46), and other (n = 20). Results For the 241 patients who underwent midline unifocalization, 204 (85.4%) had a single-stage complete repair. There were 37 patients who underwent a midline unifocalization and central shunt, and 24 have subsequently undergone complete repair. Forty-six patients underwent an aortopulmonary window, of whom 36 have subsequently had a complete repair. There were 20 patients who had complex anatomy and underwent procedures other than described, and14 have subsequently undergone complete repair. Thus, for the patients currently eligible, 280 (93.0%) have achieved complete repair. For the 204 patients who had a single-stage complete repair, the mean right ventricle to aortic pressure ratio was 0.36 ± 0.09. Seventy-six patients underwent a staged repair, and the mean right ventricle to aortic pressure ratio was 0.40 ± 0.09 (P Conclusions The data demonstrate that more than 90% of patients with pulmonary atresia with Ventricular Septal Defect and major aortopulmonary collateral arteries achieved complete repair. The overall mortality was significantly lower in the subgroup of patients who underwent single-stage complete repair.
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one stage complete unifocalization in infants when should the Ventricular Septal Defect be closed
The Journal of Thoracic and Cardiovascular Surgery, 1997Co-Authors: Mohan V Reddy, Edwin Petrossian, Doff B Mcelhinney, Phillip Moore, David F Teitel, Frank L HanleyAbstract:Abstract Background: The decision whether to close the Ventricular Septal Defect at the time of unifocalization in patients with pulmonary atresia, Ventricular Septal Defect, and major aortopulmonary collaterals may be difficult. The purpose of this study was to develop morphologic and physiologic methods to aid in deciding whether to close the Ventricular Septal Defect in patients undergoing one-stage unifocalization. Methods: Between July 1992 and April 1996, 27 infants with pulmonary atresia, Ventricular Septal Defect, and aortopulmonary collaterals were treated at our institution. Midline complete unifocalization was performed in 25 patients—the Ventricular Septal Defect was closed in 17 and left open in eight. Two patients with severe distal collateral stenoses underwent staged unifocalization. Pulmonary artery and collateral sizes were measured from preoperative angiograms and used to calculate the indexed cross-sectional area of the total neopulmonary artery bed. An intraoperative pulmonary flow study previously validated with experiments in neonatal lambs was performed in six patients: the unifocalized neopulmonary arteries were perfused with a known flow and pulmonary artery pressures were recorded. Results: The neopulmonary artery index was greater in patients who underwent Ventricular Septal Defect closure than in those who did not (p = 0.001), although the values did overlap. This index correlated with the postoperative right Ventricular/left Ventricular pressure ratio (p = 0.037). Mean pulmonary artery pressures obtained during the intraoperative flow study and after bypass were comparable. Conclusion: The total neopulmonary artery index correlates with postrepair right Ventricular/left Ventricular pressure ratio and is useful in deciding when to close the Ventricular Septal Defect if it is larger than 200 mm2/m2. The pulmonary flow study is helpful in deciding whether to close the Ventricular Septal Defect in all patients. (J Thorac Cardiovasc Surg 1997;113:858-68)
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midline one stage complete unifocalization and repair of pulmonary atresia with Ventricular Septal Defect and major aortopulmonary collaterals
The Journal of Thoracic and Cardiovascular Surgery, 1995Co-Authors: Mohan V Reddy, John R Liddicoat, Frank L HanleyAbstract:Abstract Traditionally patients with pulmonary atresia, Ventricular Septal Defect, diminutive or absent central pulmonary arteries, and multiple aortopulmonary collaterals have been managed by staged procedures necessitating multiple operations. We have taken a different approach to this lesion. Between August 1992 and March 1994, ten patients aged 1.43 months to 37.34 years (median 2.08 years) at the severe end of the morphologic spectrum of this lesion underwent a one-stage complete unifocalization and repair from a midline sternotomy approach. The median Nakata index of true pulmonary arteries was 50.0 (range 0 to 103.13) and they provided vascular supply to up to nine lung segments (median 5 segments). The number of collaterals per patient ranged from two to five with a median of four. The collaterals provided vascular supply to a median of 15 lung segments per patient (range 11 to 20). Complete unifocalization was achieved in all patients with emphasis on native tissue-to-tissue connections via anastomosis of collaterals to other collaterals and to the native pulmonary arteries. In only one patient (37.34 years old) was it necessary to use a non-native conduit for peripheral pulmonary artery reconstruction. The Ventricular Septal Defect was left open in one patient (5 years old) because of diffuse distal hypoplasia and stenosis of the pulmonary arteries and the collaterals. The postrepair peak systolic right Ventricular/left Ventricular pressure ratio ranged from 0.31 to 0.58 (median 0.47). There were no early deaths. Complications were bleeding necessitating reexploration in one patient, phrenic nerve palsy in three patients, and severe bronchospasm in three patients. Follow-up (median 8 months, range 2 to 19 months) was complete in all patients. One patient was reoperated on for pseudoaneurysm of the central homograft conduit and then again for stenosis of the left lower lobe collateral. After this last operation at 13 months after the initial repair she died of a preventable cardiac arrest caused by pneumothorax. The patient with open Ventricular Septal Defect underwent balloon dilation of the unifocalized pulmonary arteries, with a current pulmonary/systemic flow ratio of 1.4 to 1.8:1, and is awaiting Ventricular Septal Defect closure. One other patient underwent balloon dilation of the reconstructed right pulmonary artery, with a good result. All survivors (9/10) are clinically doing well. This approach establishes normal cardiovascular physiology early in life, eliminates the need for multiple systemic-pulmonary artery shunts and use of prosthetic material, and minimizes the number of operations required. Long-term follow-up is essential to determine whether this approach will limit the need for further operations to central homograft conduit changes only. (J THORAC CARDIOVASC SURG 1995;109:832-45)
Julian Ayer - One of the best experts on this subject based on the ideXlab platform.
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biVentricular repair versus fontan completion for patients with d or l transposition of the great arteries with Ventricular Septal Defect and left Ventricular outflow tract obstruction
The Journal of Thoracic and Cardiovascular Surgery, 2019Co-Authors: Christian P Brizard, David S Winlaw, Nelson Alphonso, Yves Dudekem, Lucas Eastaugh, Supreet P Marathe, Douglas Bell, Julian AyerAbstract:Abstract Objectives D-transposition of the great arteries and l-transposition of the great arteries with Ventricular Septal Defect and left Ventricular outflow tract obstruction are complex biVentricular congenital heart diseases for which decision-making regarding surgical strategy remains challenging. We investigated the intermediate-term outcomes of Fontan versus biVentricular procedures in these patients. Methods We analyzed 129 patients with d-transposition of the great arteries/Ventricular Septal Defect/left Ventricular outflow tract obstruction (n = 85) or l-transposition of the great arteries/Ventricular Septal Defect/left Ventricular outflow tract obstruction (n = 44) and 2 functional ventricles from Australia who had primary surgical management (29 Fontan, 100 biVentricular repair) undertaken between 1990 and 2015. Results Median operative age of patients was 2.9 years (range, 0.2-26.8 years). During a median follow-up of 6.2 years (range, 2 days to 25.8 years), 9 patients died after biVentricular repair (3 early and 6 late deaths). One patient received a transplant 1.2 years after Fontan completion. Overall transplant-free survivals at 1, 5, 10, and 15 years were 95%, 93%, 92%, and 90%, respectively. Overall reintervention-free survivals at 1, 5, 10, and 15 years were 79%, 64%, 45%, and 29% respectively. BiVentricular repair tended to be associated with a higher rate of death, transplantation, or reintervention than the Fontan pathway (hazard ratio, 1.83; 95% confidence interval, 0.90-3.71; P = .10). Some 73% of transplant-free survivors had New York Heart Association class I. Functional status was similar between the Fontan and biVentricular groups. Conclusions Intermediate-term outcomes were comparable between patients with d-transposition of the great arteries/Ventricular Septal Defect/left Ventricular outflow tract obstruction and patients with l-transposition of the great arteries/Ventricular Septal Defect/left Ventricular outflow tract obstruction. Both Fontan and biVentricular pathways are associated with excellent mortality and functional outcomes. BiVentricular patients have a greater risk of reintervention. The Fontan procedure is a viable option when anatomic risk factors preclude biVentricular repair.
Mohan V Reddy - One of the best experts on this subject based on the ideXlab platform.
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one stage complete unifocalization in infants when should the Ventricular Septal Defect be closed
The Journal of Thoracic and Cardiovascular Surgery, 1997Co-Authors: Mohan V Reddy, Edwin Petrossian, Doff B Mcelhinney, Phillip Moore, David F Teitel, Frank L HanleyAbstract:Abstract Background: The decision whether to close the Ventricular Septal Defect at the time of unifocalization in patients with pulmonary atresia, Ventricular Septal Defect, and major aortopulmonary collaterals may be difficult. The purpose of this study was to develop morphologic and physiologic methods to aid in deciding whether to close the Ventricular Septal Defect in patients undergoing one-stage unifocalization. Methods: Between July 1992 and April 1996, 27 infants with pulmonary atresia, Ventricular Septal Defect, and aortopulmonary collaterals were treated at our institution. Midline complete unifocalization was performed in 25 patients—the Ventricular Septal Defect was closed in 17 and left open in eight. Two patients with severe distal collateral stenoses underwent staged unifocalization. Pulmonary artery and collateral sizes were measured from preoperative angiograms and used to calculate the indexed cross-sectional area of the total neopulmonary artery bed. An intraoperative pulmonary flow study previously validated with experiments in neonatal lambs was performed in six patients: the unifocalized neopulmonary arteries were perfused with a known flow and pulmonary artery pressures were recorded. Results: The neopulmonary artery index was greater in patients who underwent Ventricular Septal Defect closure than in those who did not (p = 0.001), although the values did overlap. This index correlated with the postoperative right Ventricular/left Ventricular pressure ratio (p = 0.037). Mean pulmonary artery pressures obtained during the intraoperative flow study and after bypass were comparable. Conclusion: The total neopulmonary artery index correlates with postrepair right Ventricular/left Ventricular pressure ratio and is useful in deciding when to close the Ventricular Septal Defect if it is larger than 200 mm2/m2. The pulmonary flow study is helpful in deciding whether to close the Ventricular Septal Defect in all patients. (J Thorac Cardiovasc Surg 1997;113:858-68)
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midline one stage complete unifocalization and repair of pulmonary atresia with Ventricular Septal Defect and major aortopulmonary collaterals
The Journal of Thoracic and Cardiovascular Surgery, 1995Co-Authors: Mohan V Reddy, John R Liddicoat, Frank L HanleyAbstract:Abstract Traditionally patients with pulmonary atresia, Ventricular Septal Defect, diminutive or absent central pulmonary arteries, and multiple aortopulmonary collaterals have been managed by staged procedures necessitating multiple operations. We have taken a different approach to this lesion. Between August 1992 and March 1994, ten patients aged 1.43 months to 37.34 years (median 2.08 years) at the severe end of the morphologic spectrum of this lesion underwent a one-stage complete unifocalization and repair from a midline sternotomy approach. The median Nakata index of true pulmonary arteries was 50.0 (range 0 to 103.13) and they provided vascular supply to up to nine lung segments (median 5 segments). The number of collaterals per patient ranged from two to five with a median of four. The collaterals provided vascular supply to a median of 15 lung segments per patient (range 11 to 20). Complete unifocalization was achieved in all patients with emphasis on native tissue-to-tissue connections via anastomosis of collaterals to other collaterals and to the native pulmonary arteries. In only one patient (37.34 years old) was it necessary to use a non-native conduit for peripheral pulmonary artery reconstruction. The Ventricular Septal Defect was left open in one patient (5 years old) because of diffuse distal hypoplasia and stenosis of the pulmonary arteries and the collaterals. The postrepair peak systolic right Ventricular/left Ventricular pressure ratio ranged from 0.31 to 0.58 (median 0.47). There were no early deaths. Complications were bleeding necessitating reexploration in one patient, phrenic nerve palsy in three patients, and severe bronchospasm in three patients. Follow-up (median 8 months, range 2 to 19 months) was complete in all patients. One patient was reoperated on for pseudoaneurysm of the central homograft conduit and then again for stenosis of the left lower lobe collateral. After this last operation at 13 months after the initial repair she died of a preventable cardiac arrest caused by pneumothorax. The patient with open Ventricular Septal Defect underwent balloon dilation of the unifocalized pulmonary arteries, with a current pulmonary/systemic flow ratio of 1.4 to 1.8:1, and is awaiting Ventricular Septal Defect closure. One other patient underwent balloon dilation of the reconstructed right pulmonary artery, with a good result. All survivors (9/10) are clinically doing well. This approach establishes normal cardiovascular physiology early in life, eliminates the need for multiple systemic-pulmonary artery shunts and use of prosthetic material, and minimizes the number of operations required. Long-term follow-up is essential to determine whether this approach will limit the need for further operations to central homograft conduit changes only. (J THORAC CARDIOVASC SURG 1995;109:832-45)
David S Winlaw - One of the best experts on this subject based on the ideXlab platform.
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biVentricular repair versus fontan completion for patients with d or l transposition of the great arteries with Ventricular Septal Defect and left Ventricular outflow tract obstruction
The Journal of Thoracic and Cardiovascular Surgery, 2019Co-Authors: Christian P Brizard, David S Winlaw, Nelson Alphonso, Yves Dudekem, Lucas Eastaugh, Supreet P Marathe, Douglas Bell, Julian AyerAbstract:Abstract Objectives D-transposition of the great arteries and l-transposition of the great arteries with Ventricular Septal Defect and left Ventricular outflow tract obstruction are complex biVentricular congenital heart diseases for which decision-making regarding surgical strategy remains challenging. We investigated the intermediate-term outcomes of Fontan versus biVentricular procedures in these patients. Methods We analyzed 129 patients with d-transposition of the great arteries/Ventricular Septal Defect/left Ventricular outflow tract obstruction (n = 85) or l-transposition of the great arteries/Ventricular Septal Defect/left Ventricular outflow tract obstruction (n = 44) and 2 functional ventricles from Australia who had primary surgical management (29 Fontan, 100 biVentricular repair) undertaken between 1990 and 2015. Results Median operative age of patients was 2.9 years (range, 0.2-26.8 years). During a median follow-up of 6.2 years (range, 2 days to 25.8 years), 9 patients died after biVentricular repair (3 early and 6 late deaths). One patient received a transplant 1.2 years after Fontan completion. Overall transplant-free survivals at 1, 5, 10, and 15 years were 95%, 93%, 92%, and 90%, respectively. Overall reintervention-free survivals at 1, 5, 10, and 15 years were 79%, 64%, 45%, and 29% respectively. BiVentricular repair tended to be associated with a higher rate of death, transplantation, or reintervention than the Fontan pathway (hazard ratio, 1.83; 95% confidence interval, 0.90-3.71; P = .10). Some 73% of transplant-free survivors had New York Heart Association class I. Functional status was similar between the Fontan and biVentricular groups. Conclusions Intermediate-term outcomes were comparable between patients with d-transposition of the great arteries/Ventricular Septal Defect/left Ventricular outflow tract obstruction and patients with l-transposition of the great arteries/Ventricular Septal Defect/left Ventricular outflow tract obstruction. Both Fontan and biVentricular pathways are associated with excellent mortality and functional outcomes. BiVentricular patients have a greater risk of reintervention. The Fontan procedure is a viable option when anatomic risk factors preclude biVentricular repair.
Constantine Mavroudis - One of the best experts on this subject based on the ideXlab platform.
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congenital heart surgery nomenclature and database project Ventricular Septal Defect
The Annals of Thoracic Surgery, 2000Co-Authors: Jeffrey P Jacobs, James A Quintessenza, Redmond P Burke, Constantine MavroudisAbstract:The extant nomenclature for Ventricular Septal Defect (VSD) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Four basic VSD types are described: Subarterial, Perimembranous, Inlet, and Muscular. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analysis. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.
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Surgical management of the conal (supracristal) Ventricular Septal Defect
The Journal of Thoracic and Cardiovascular Surgery, 1991Co-Authors: Carl L. Backer, Farouk S. Idriss, Vincent R. Zales, Michel N. Ilbawi, Serafin Y. Deleon, Alexander J. Muster, Constantine MavroudisAbstract:Surgical management of the conal (supracristal) Ventricular Septal Defect differs significantly from the management of the perimembranous (infracristal) Ventricular Septal Defect. The absence of a portion of the conal septum can lead to prolapse of the right cusp of the aortic valve, which predisposes these patients to aortic insufficiency. Between January 1980 and December 1989, 36 children with conal Ventricular Septal Defect underwent intracardiac repair. Diagnosis was by echocardiography, cardiac catheterization, and intraoperative exploration. Preoperative evaluation showed that 26 patients (72%) had aortic valve prolapse and 16 (44%) had aortic insufficiency. Pulmonary-to-systemic flow ratios ranged from 1:1 to 3.5:1 (mean 2.0:1.0). Ten patients (27%) were believed to have clinical congestive heart failure. Age at the time of operation ranged from 2 weeks to 18 years (mean 5.5 years). Operative exposure was through the pulmonary artery (26), aorta (4), right ventricle (3), or right atrium (3). Simultaneous aortic valve suspension for aortic insufficiency was performed in four patients. Operative survival was 100%. Follow-up is complete in all patients and ranges from 0.5 to 9 years (mean 4.3 years). All patients are in normal sinus rhythm. No residual Ventricular Septal Defects have been identified. Twenty-three of 36 patients (64%) have no evidence of aortic insufficiency; 12 of 36 (33%) have trivial or mild aortic insufficiency. One patient with initial severe aortic insufficiency underwent repeat aortic valvuloplasty 3 years after Ventricular Septal Defect closure and aortic valve suspension. No patients have required aortic valve replacement Surgical management of the conal Ventricular Septal Defect differs from that of the perimembranous Ventricular Septal Defect in two critical aspects. The operative approach should be through the pulmonary artery. This allows the best exposure of the remaining conal septum and the pulmonary and aortic valve leaflets, facilitating closure of the Defect without injury to the valves or conduction system. Conal Ventricular Septal Defects should undergo early closure, regardless of shunt volume, to prevent progressive aortic valve prolapse and aortic insufficiency.
