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Neelam Desai - One of the best experts on this subject based on the ideXlab platform.

  • Supravalvar Aortic Stenosis: 19 years of surgical experience
    Indian Journal of Thoracic and Cardiovascular Surgery, 2015
    Co-Authors: Trushar Gajjar, Neelam Desai
    Abstract:

    Objective Supravalvar Aortic Stenosis (SVAS) is an uncommon congenital cardiac anomaly characterized by varying degrees of left ventricular outflow tract obstruction distal to the Aortic valve. We would like to share our experience with this condition over a period of 19 years. Methods Between April 1994 and June 2013, 22 consecutive patients of Supravalvar Aortic Stenosis underwent surgical correction at our institute. There were 16 males and 6 females. Age ranged from 3 to 38 years. William’s syndrome was present in seven patients. Out of 22 cases 19 were of a localized type and 3 were diffuse in nature. The mean peak gradient was 110.4 mmHg (range 72–140 mmHg). Of the 19 patients with localized type of Stenosis, 17 were successfully treated with single patch aortoplasty while two patients were treated with extended aortoplasty. The patients with diffuse type required extended aortoplasty with extensive endarterectomy. Three patients in all had coronary ostial involvement that required additional intervention. Results Twenty patients were discharged in stable condition while two patients died in the immediate postoperative period. Four patients developed neurological events including the two early deaths. The remaining 18 patients were asymptomatic. Postoperative echocardiogram obtained for all patients before discharge showed average peak gradient of 21 mmHg (range 0–50 mmHg). Follow up was done at 3 months, 1, 3, 5, and 10 years. All patients were in NYHA class I, their average peak gradient was 23 mmHg, and there was no late mortality. Conclusion Our study demonstrates that standard patch aortoplasty provides good postoperative results. We recommend use of a large patch, extending from the valve hinge in the sinus of Valsalva to the ascending aorta well above the obstructive waist. Coronary ostial involvement requires individualized treatment. Good surgical outcome can be achieved with an appropriate method of treatment in patients with both the forms of Supravalvar Aortic Stenosis.

  • Supravalvar Aortic Stenosis: 19 years of surgical experience
    Indian Journal of Thoracic and Cardiovascular Surgery, 2015
    Co-Authors: Nageswar Rao, Trushar Gajjar, Neelam Desai
    Abstract:

    Objective Supravalvar Aortic Stenosis (SVAS) is an uncommon congenital cardiac anomaly characterized by varying degrees of left ventricular outflow tract obstruction distal to the Aortic valve. We would like to share our experience with this condition over a period of 19 years.

  • Surgical Management of Supravalvar Aortic Stenosis with Coronary Artery Involvement
    Journal of cardiac surgery, 2012
    Co-Authors: Trushar Gajjar, Jinaga Nageswar Rao, Neelam Desai
    Abstract:

    Supravalvar Aortic Stenosis is characterized by an obstruction of the left ventricular outflow tract distal to the Aortic valve. Its association with left main coronary artery Stenosis is well known but right coronary artery involvement has not been reported. We describe two cases of Supravalvar Aortic Stenosis with coronary artery Stenosis and its surgical management. (J Card Surg 2012;27:678-681)

Trushar Gajjar - One of the best experts on this subject based on the ideXlab platform.

  • Supravalvar Aortic Stenosis: 19 years of surgical experience
    Indian Journal of Thoracic and Cardiovascular Surgery, 2015
    Co-Authors: Trushar Gajjar, Neelam Desai
    Abstract:

    Objective Supravalvar Aortic Stenosis (SVAS) is an uncommon congenital cardiac anomaly characterized by varying degrees of left ventricular outflow tract obstruction distal to the Aortic valve. We would like to share our experience with this condition over a period of 19 years. Methods Between April 1994 and June 2013, 22 consecutive patients of Supravalvar Aortic Stenosis underwent surgical correction at our institute. There were 16 males and 6 females. Age ranged from 3 to 38 years. William’s syndrome was present in seven patients. Out of 22 cases 19 were of a localized type and 3 were diffuse in nature. The mean peak gradient was 110.4 mmHg (range 72–140 mmHg). Of the 19 patients with localized type of Stenosis, 17 were successfully treated with single patch aortoplasty while two patients were treated with extended aortoplasty. The patients with diffuse type required extended aortoplasty with extensive endarterectomy. Three patients in all had coronary ostial involvement that required additional intervention. Results Twenty patients were discharged in stable condition while two patients died in the immediate postoperative period. Four patients developed neurological events including the two early deaths. The remaining 18 patients were asymptomatic. Postoperative echocardiogram obtained for all patients before discharge showed average peak gradient of 21 mmHg (range 0–50 mmHg). Follow up was done at 3 months, 1, 3, 5, and 10 years. All patients were in NYHA class I, their average peak gradient was 23 mmHg, and there was no late mortality. Conclusion Our study demonstrates that standard patch aortoplasty provides good postoperative results. We recommend use of a large patch, extending from the valve hinge in the sinus of Valsalva to the ascending aorta well above the obstructive waist. Coronary ostial involvement requires individualized treatment. Good surgical outcome can be achieved with an appropriate method of treatment in patients with both the forms of Supravalvar Aortic Stenosis.

  • Supravalvar Aortic Stenosis: 19 years of surgical experience
    Indian Journal of Thoracic and Cardiovascular Surgery, 2015
    Co-Authors: Nageswar Rao, Trushar Gajjar, Neelam Desai
    Abstract:

    Objective Supravalvar Aortic Stenosis (SVAS) is an uncommon congenital cardiac anomaly characterized by varying degrees of left ventricular outflow tract obstruction distal to the Aortic valve. We would like to share our experience with this condition over a period of 19 years.

  • Surgical Management of Supravalvar Aortic Stenosis with Coronary Artery Involvement
    Journal of cardiac surgery, 2012
    Co-Authors: Trushar Gajjar, Jinaga Nageswar Rao, Neelam Desai
    Abstract:

    Supravalvar Aortic Stenosis is characterized by an obstruction of the left ventricular outflow tract distal to the Aortic valve. Its association with left main coronary artery Stenosis is well known but right coronary artery involvement has not been reported. We describe two cases of Supravalvar Aortic Stenosis with coronary artery Stenosis and its surgical management. (J Card Surg 2012;27:678-681)

Max B. Mitchell - One of the best experts on this subject based on the ideXlab platform.

  • Supravalvar Aortic Stenosis in Infancy
    Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual, 2011
    Co-Authors: Max B. Mitchell, Steven P. Goldberg
    Abstract:

    Supravalvar Aortic Stenosis (SVAS) is a rare anomaly of the Aortic root caused by a genetically based deficiency in elastin production. Concomitant primary and secondary cardiovascular lesions complicate surgical management and impact early and late outcomes. Because SVAS is a rare lesion, surgical series are relatively small and span lengthy time periods. Consequently, risk factors that influence early and late outcomes are not well defined. Patients who come to surgery during infancy are particularly challenging, but little attention has been directed as to whether or not young age influences outcomes. This review suggests that complicating associated features of elastin arteriopathy are more prevalent in patients who require relief of SVAS during infancy, and that concomitant lesions significantly increase the difficulty and risk of treating younger patients with SVAS.

  • Twenty-year surgical experience with congenital Supravalvar Aortic Stenosis.
    The Annals of thoracic surgery, 2009
    Co-Authors: Daniel J. Scott, Steven P. Goldberg, David N. Campbell, David R. Clarke, Daniel R. Karlin, Max B. Mitchell
    Abstract:

    Background Congenital Supravalvar Aortic Stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome and other elastin gene deletions. Our objectives were to review outcomes of congenital SVAS repair and to compare prosthetic patch repair techniques to all-autologous slide aortoplasty. Methods Congenital SVAS repairs from 1988 to 2008 were retrospectively reviewed. Peak instantaneous gradients were estimated by Doppler interrogation. Variables were compared by either Student's t test or Fisher's exact test. Risk factors were analyzed by χ 2 test. Survival was estimated by the Kaplan-Meier method. Results Of 25 primary SVAS repairs, there were 10 all-autologous slide aortoplasties and 15 prosthetic patch aortoplasties. The prosthetic patch group included the Doty technique (n = 9), patch-augmented slide aortoplasty (n = 3), modified Brom technique (n = 1), interposition graft (n = 1), and two-sinus patch with transverse arch augmentation (n = 1). There was 1 early and 1 late death. Cumulative survival for all patients was 96% at 5 and 10 years. Event-free survival did not differ between groups ( p = 0.481). There were 2 late reoperations (both were prosthetic patch patients with bicuspid Aortic valve: 1 with recurrent Aortic valve Stenosis and 1 with Aortic insufficiency). Bicuspid Aortic valve was the only risk factor for reoperation ( p = 0.003). Three patients weighing less than 10 kg with diffuse disease underwent attempted slide aortoplasty: 2 required patch augmentation and 1 had a recurrent gradient in less than 1 year postoperatively. Conclusions Outcomes after SVAS repair were good by any technique. No advantage to all-autologous slide aortoplasty was apparent at current follow-up. Based on our experience, slide aortoplasty is not recommended for small patients with diffuse disease.

  • Composite aortoplasty for recurrent coarctation after neonatal repair in Williams syndrome
    The Annals of thoracic surgery, 2004
    Co-Authors: Jeni L Marks, Max B. Mitchell, David N. Campbell, Warren H. Toews
    Abstract:

    Supravalvar Aortic Stenosis is the most frequent operation required for Williams syndrome; however, coarctation repair is more common in patients requiring surgery in the first few months of life. We report on a child with Williams syndrome in whom extensive reoperation was required 5 months after neonatal Aortic coarctation repair. A composite left subclavian artery flap and allograft patch aortoplasty of the Aortic arch and descending aorta was performed through a left thoracotomy using cardiopulmonary bypass and circulatory arrest. Detailed anatomic evaluation of the Aortic arch and descending aorta is recommended before initial coarctation repair in neonates with Williams syndrome.

Hong-ju Shin - One of the best experts on this subject based on the ideXlab platform.

  • Aortic Valve-Sparing Surgical Treatment of Supravalvar Aortic Stenosis in a 65-Year-Old Adult.
    The Korean journal of thoracic and cardiovascular surgery, 2020
    Co-Authors: Hong-ju Shin, Jae Seung Shin
    Abstract:

    Supravalvar Aortic Stenosis (SVAS) is a rare congenital cardiac disease that usually co-occurs with Williams syndrome. In the adult population, a few SVAS cases have been reported in patients affected by homozygous familial hypercholesterolemia. However, because of the rarity of this disease entity, there is no standard surgical treatment for SVAS. Here, we present a case of successful surgical treatment using an autologous excised Aortic patch in a 65-year-old patient with SVAS.

  • modified simple sliding aortoplasty for preserving the sinotubular junction without using foreign material for congenital Supravalvar Aortic Stenosis
    European Journal of Cardio-Thoracic Surgery, 2011
    Co-Authors: Hong-ju Shin, Won Kyoung Jhang, Jeongjun Park, Hyun Woo Goo, Dong-man Seo
    Abstract:

    Objective: The surgical approach for treating Supravalvar Aortic Stenosis (SVAS) has evolved from a plain patch technique to a threedimensional patch repair, which has some drawbacks. Here, we report on the midterm outcomes after using our modified simple sliding aortoplasty preserving sinotubular junction without foreign material for surgical correction of SVAS. Methods: Between June 2001 and February 2010,18children(medianage,6.2years;range0.5—2.2years)with discreteSVASunderwentsurgicalrepair. Afterastandardmediansternotomy, cardiopulmonary bypass and cardioplegic arrest, the aorta was transected obliquely just distal to the point of Stenosis. An incision was then made into the non-coronary sinus of the proximal aorta, and a counterincision was made into the lesser curvature of the ascending aorta, after which, the proximal and distal aorta were anastomosed directly with a running suture. Peak pressure gradients were estimated using echocardiography and the data were reviewed retrospectively.Results:The median follow-up periodwas 39.6 months(range, 1—104.5 months). There was no early or late death. No patients required re-operation. Ten patients had Williams—Beuren syndrome.There were eight cases of concomitant pulmonary artery angioplasty. The mean pressure gradient decreased from 65.9 18.4 mmHg preoperatively to 15.2 8.9 mmHg at the final follow-up (P = 0.01). There was no significant, more than mild Aortic regurgitation. Conclusions: Our modified simple sliding aortoplasty showed excellent surgical results, and may be a good option for discrete SVAS. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

  • Modified Simple Sliding Aortoplasty for Supravalvar Aortic Stenosis
    The Annals of thoracic surgery, 2007
    Co-Authors: Dong-man Seo, Hong-ju Shin, Jungjun Park, Tae-jin Yun, In Sook Park, Young-hwue Kim
    Abstract:

    We describe a modified simple sliding aortoplasty for Supravalvar Aortic Stenosis. This technique is easier, has a more physiologic approach, and has the advantage of not using foreign material to provide Aortic growth potential in children. For the patients presented in this study, the mid-term results of modified simple sliding aortoplasty have showed good results.

Jae Seung Shin - One of the best experts on this subject based on the ideXlab platform.