Valve-Sparing Root and Total Arch Replacement for Cutis Laxa Aortopathy

2017 ◽  
Vol 10 (3) ◽  
pp. 376-379
Author(s):  
Anji T. Yetman ◽  
James Hammel ◽  
Jennifer N. Sanmann ◽  
Lois J. Starr
Keyword(s):  
Genetic Disorder ◽  
Snp Array ◽  
Aortic Aneurysms ◽  
Cutis Laxa ◽  
Cannulation Technique ◽  
Aorta Replacement ◽  
Valve Sparing ◽  
Regions Of Homozygosity ◽  
Ascending Aorta Replacement

Aortic aneurysms requiring surgery in early childhood are rare. Herein we describe the case of a three-year-old with massive aneurysmal aortic dilation secondary to the rare and often lethal genetic disorder, cutis laxa. Initial thoracic aortic aneurysm gene panel was negative. Parents of the child were not known to be consanguineous, but high-density SNP array revealed several regions of homozygosity. This prompted targeted sequence analysis that identified a novel homozygous missense mutation in the gene for cutis laxa, EFEMP2. The patient underwent aortic valve–sparing aortic root and ascending aorta replacement and total aortic arch replacement, with continuous, moderately hypothermic cardiopulmonary bypass, using a dual cannulation technique. He was discharged well on the third postoperative day and remains free of aneurysmal disease at two-year follow-up.

Early safety performance of a modified technique of aorta replacement with sinotubular stabilization

2021 ◽  
pp. 021849232110150
Author(s):  
Marco Moscarelli ◽  
Nicola Di Bari ◽  
Giuseppe Nasso ◽  
Khalil Fattouch ◽  
Thanos Athanasiou ◽  
...  
Keyword(s):  
Artery Bypass ◽  
Aortic Insufficiency ◽  
Ascending Aorta ◽  
Safety Performance ◽  
Modified Technique ◽  
Sinotubular Junction ◽  
Aorta Replacement ◽  
Valve Sparing ◽  
Ascending Aorta Replacement

Background We sought to determine if a modified technique for ascending aorta replacement with sinotubular junction reduction and stabilization was safe. Methods This technique was performed by suspension of the three commissures, invagination of the aortic Dacron graft and advancing the graft into the ventricles. We included patients with dilatation of the ascending aorta, normal sinuses of Valsalva dimension (<45 mm), with or without aortic annulus enlargement (>25 mm) and with various degree of aortic insufficiency (from grade 1 to 3). Results From April to October 2019, 20 patients were recruited from two centers; mean age was 66.9 ± 12.8 years, 13 were male; grade 1, 2 and 3 was present in 12, 2 and 6 patients, respectively. All patients underwent ascending aorta replacement with modified technique; an additional open subvalvular ring was used in 8 patients with aortic insufficiency ≥ 2; cusps repair was performed in 6 patients (5 plicating central stitches/1 shaving); concomitant coronary artery bypass grafting was performed in 10 patients. There was no 30-day mortality. One patient was re-explored for bleeding. All patients completed six-month follow-up; at the transthoracic echocardiography, there was no aortic insufficiency ≥ 1 except one patient with aortic insufficiency grade 1 who underwent ascending aorta replacement and subvalvular ring; no patients underwent reintervention. Conclusions This modified technique for ascending aorta replacement and sinotubular junction stabilization was safe. It could be associated with other aortic valve sparing techniques. However, such remodeling approach has to be validated in a larger cohort of patients with longer follow-up.

scholarly journals Fate of mild-to-moderate bicuspid aortic valve disease untreated during ascending aorta replacement

2020 ◽  
Author(s):  
Alessandro Verzini ◽  
Marta Bargagna ◽  
Guido Ascione ◽  
Alessandra Sala ◽  
Davide Carino ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Bicuspid Aortic Valve ◽  
Ascending Aorta ◽  
Term Survival ◽  
Kaplan Meier ◽  
Aortic Root Surgery ◽  
Increased Risk ◽  
Aorta Replacement ◽  
Ascending Aorta Replacement

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect and it is responsible for an increased risk of developing aortic valve and ascending aorta complications. In case of mild to moderate BAV disease in patients undergoing supracoronary ascending aorta replacement, it is unclear whether a concomitant aortic valve replacement should be performed. Methods: From June 2002 to January 2020, 75 patients with mild-to-moderate BAV regurgitation (± mild-to-moderate stenosis) who underwent isolated supracoronary ascending aorta replacement were retrospectively analyze. Clinical and echocardiographic follow-up was 100% complete (mean: 7.4±3.9 years, max 16.4). Kaplan Meier estimates were employed to analyze long-term survival. Cumulative incidence function for time to re-operation, recurrence of aortic regurgitation (AR)≥3+ and aortic stenosis (AS) greater than moderate, with death as competing risk, were computed. Results: There was no hospital mortality and no cardiac death occurred. Overall survival at 12 years was 97.4±2.5%, 95% CI [83.16-99.63]. At follow-up there were no cases of aortic root surgery whereas 3 patients underwent AV replacement. At 12 years the CIF of reoperation was 2.6±2.5%, 95% CI [0.20-11.53]. At follow up, AR 3+/4+ was present in 1 pt and AS greater than moderate in 3. At 12 years the CIF of AR>2+/4+ was 5.1±4.98% and of AS>moderate 6.9±3.8%. Conclusions: In our study mild to moderate regurgitation of a BAV did not significantly worse at least up to 10 years after isolated supracoronary ascending aorta replacement.

scholarly journals Case Report: Occurrence of Severe Thoracic Aortic Aneurysms (Involving the Ascending, Arch, and Descending Segments) as a Result of Fibulin-4 Deficiency: A Rare Pathology With Successful Management

2021 ◽  
Vol 8 ◽  
Author(s):  
Paul Thomas ◽  
Aparna Venugopalan ◽  
Siddharth Narayanan ◽  
Thomas Mathew ◽  
Lakshmi Parvathi Deepti Cherukuwada ◽  
...  
Keyword(s):  
Matrix Protein ◽  
Genetic Disorder ◽  
Female Child ◽  
Aortic Aneurysms ◽  
Cutis Laxa ◽  
Extracellular Matrix Protein ◽  
Successful Outcome ◽  
High Morbidity ◽  
Aortic Diseases ◽  
Thoracic Aortic Aneurysms

Aortic diseases requiring surgery in childhood are distinctive and rare. Very few reports in the literature account for the occurrence of multiple thoracic aortic aneurysms in the same pediatric patient because of a genetic cause. We report a rare occurrence of severe thoracic aortic aneurysms (involving the ascending, arch and descending aortic segments) with severe aortic insufficiency in a 7-year-old female child secondary to the extremely rare and often lethal genetic disorder, cutis laxa. She was eventually identified as a carrier of a homozygous EFEMP2 (alias FBLN4) mutation. This gene encodes the extracellular matrix protein fibulin-4, and its mutation is associated with autosomal recessive cutis laxa type 1B that leads to severe aortopathy with aneurysm formation and vascular tortuosity. Parents of the child were not known to be consanguineous. Significant symptomatic improvement in the patient could be discerned after timely intervention with the valve-sparing aortic root replacement (David V procedure) and a concomitant aortic arch replacement. This is a unique report with a successful outcome that highlights the occurrence of a rare hereditary aortopathy associated with a high morbidity and mortality, and the importance of an early diagnosis and timely management. It also offers insight to physicians in having a very broad differential and multimodal approach in handling rare pediatric cardio-pathologies with a genetic predisposition.

Partial Aortic Root Remodeling in Case of Ascending Aortic Aneurysms

2013 ◽  
Vol 8 (4) ◽  
pp. 1-5 ◽  
Author(s):  
Fabrizio Sansone ◽  
Edoardo Zingarelli ◽  
Fabrizio Ceresa ◽  
Francesco Patanè
Keyword(s):  
Aortic Root ◽  
Pathological Process ◽  
Ascending Aorta ◽  
Aortic Aneurysms ◽  
Bleeding Complications ◽  
Sinotubular Junction ◽  
Computed Tomographic ◽  
Aortic Root Remodeling ◽  
Aorta Replacement ◽  
Ascending Aorta Replacement

Objective In degenerative ascending aortic aneurysms (AAAs), the pathological process may extend into the aortic root, causing aortic regurgitation (AR). As often one or two sinuses are involved, ascending aorta replacement should be associated with selected sinus replacement. Methods Thirty patients (21 men and 9 women; mean ± SD age, 70.0 ± 10.4) were operated on for ascending aorta and selected sinus replacement. All patients had degenerative AAA with sinotubular junction and partial root dilatation: one or two sinuses of Valsalva were involved. Mild to moderate-severe AR was present in all patients. The mean ± SD logistic EUROscore 1 was 15.4 ± 12.5. Twenty patients had ascending aorta replacement associated with noncoronary sinus replacement; 8 patients, associated with both right and noncoronary sinuses; 1 patient, associated with both left and noncoronary sinuses; and 1 patient, associated with left coronary sinus alone. Results There were no hospital or late deaths. No thromboembolic event or bleeding complications were reported. Postoperative echocardiography did not show significant AR, and computed tomographic scanning revealed a normal positioning of the vascular graft in the ascending aorta. Conclusions Remodeling of the sinotubular junction with selected sinus replacement in degenerative AAA is a valuable approach for aortic root remodeling, leading to a significant reduction of AR when the aortic leaflets are normal.

scholarly journals Valve-sparing aneurysmal root/ascending aorta replacement in a 9-year-old patient with Marfan syndrome

2018 ◽  
Vol 22 (1) ◽  
pp. 61
Author(s):  
V. N. Bogdanov ◽  
I, V. Gladyshev ◽  
E. F. Kharisova ◽  
I. V. Harenko
Keyword(s):  
Marfan Syndrome ◽  
Tricuspid Valve ◽  
Conflict Of Interest ◽  
Clinical Case ◽  
Surgical Repair ◽  
Severe Form ◽  
Ascending Aorta ◽  
Aorta Replacement ◽  
Valve Sparing ◽  
Ascending Aorta Replacement

<p>A clinical case report of a 9-year-old patient with a severe form of Marfan syndrome and significant enlargement of the aortic root and mitral/tricuspid valve failure is presented. The patient underwent successful one-stage surgical repair including reimplantation of the aortic valve using David procedure, mitral valve annuloplication and De Vega valvuloplasty of the tricuspid valve.</p><p>Received 21 November 2017. Revised 19 February 2018. Accepted 27 February 2018.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p><p><strong>Author contributions</strong><br />All authors contributed equally to the work.</p>

scholarly journals Early and mid-term outcomes of modified aortic root repair for acute stanford type A aortic dissection

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
L C Wang ◽  
Y X Liu ◽  
Y J Dun ◽  
X G Sun
Keyword(s):  
Aortic Valve ◽  
Aortic Root ◽  
False Lumen ◽  
Ascending Aorta ◽  
Aortic Sinus ◽  
Root Repair ◽  
Aorta Replacement ◽  
Aortic Root Repair ◽  
Ascending Aorta Replacement

Abstract Background Acute Stanford type A aortic dissection (ATAAD) is the most common catastrophic aortic event. Most ATAAD involves the aortic root which has many important anatomical structures such as aortic valve, so the proper treatment of dissected root can ensure a good prognosis for patients. However, there is still no consensus on root management strategies for ATAAD patients with aortic root involvement. Purpose This clinical study aimed to evaluate the therapeutic effect of modified aortic root repair in ATAAD. Methods From September 2017 to September 2020, Participants with root involvement of ATAAD were recruited who underwent modified aortic root repair as well as some additional procedure such as aortic valve junction suspension plasty based on the aortic sinus tear extent. During this novel procedure, the proximal anastomosis plane was at the level of the sinu-tubular junction and the false lumen below it was retained. We collected and analyzed the perioperative clinical data and follow-up imaging data of patients, and further evaluated the early and mid-term efficacy of this surgical approach. Results A total of 79 patients were enrolled, including 59 males and 20 females, the age was (52.4±11.3) years old (28–73 years), the diameter of aortic sinus was (38.6±4.1) mm, and the diameter of sinu-tubular junction was (41.8±4.8) mm. In this group, 75 patients (94.9%) received ascending aorta replacement, total arch replacement and frozen elephant trunk, 2 patients (2.5%) received ascending aorta replacement and hybrid total arch replacement, 2 patients (2.5%) received ascending aorta replacement and partial arch replacement. Cardiopulmonary bypass time was (197.2±58.6) min (118–455 min), blocking time was (132.6±38.9) min (73–323 min), circulatory arrest time was (10.3±7.0) min (0–27 min). There was no perioperative death, no paraplegia, one secondary thoracotomy, five renal failures needing hemodialysis treatment and two cerebral infarctions. Before patients discharged, aortic CTA showed that the residual false lumen in the sinus disappeared. And the diameter of the aortic sinus was (35.5±3.1) mm, the diameter of the junction of the aortic sinus was (30.0±3.0) mm. The patients were followed up for (18±12) months (3–35 months). There was one patient died during follow-up and no further surgical intervention at the root of the aorta. Follow-up aortic CTA showed no residual or new dissection in the aortic sinus and no significant difference in the diameters of aortic sinu-tubular junction (P=0.122) or aortic sinus (P=0.37) between postoperative period and follow-up period. Echocardiography showed that the structure and function of the aortic valve were normal. Conclusions The modified aortic root repair for ATAAD is relatively simple, easy to learn and safe in perioperative period. Early and mid-term follow-up image examination showed that the structure of aortic sinus returned to normal. The long-term clinical effect requires close attention. FUNDunding Acknowledgement Type of funding sources: None. Modified aortic root repair procedure Aortic root diameter change under CTA

Valve-Sparing Root and Ascending Aorta Replacement After Heart Transplantation

2012 ◽  
Vol 94 (6) ◽  
pp. 2114-2115 ◽  
Author(s):  
Abdelsalam M. Elhenawy ◽  
Christopher M. Feindel ◽  
Heather Ross ◽  
Jagdish Butany ◽  
Terrence M. Yau
Keyword(s):  
Heart Transplantation ◽  
Ascending Aorta ◽  
Aorta Replacement ◽  
Valve Sparing ◽  
Ascending Aorta Replacement

Combined Rastelli procedure with supra-coronary ascending aorta replacement

2020 ◽  
pp. 1-3
Author(s):  
Yatin Arora ◽  
Velayoudam Devagourou ◽  
Tanushree Kar
Keyword(s):  
Pulmonary Atresia ◽  
Ascending Aorta ◽  
Complete Repair ◽  
Aorta Replacement ◽  
Ascending Aorta Replacement

Abstract We present a 5-year-old child with pulmonary atresia palliated at infancy with midline shunt now for complete repair with aneurysmally dilated aorta. Patient underwent a combined Rastelli procedure with supra-coronary ascending aorta replacement with hemi-arch repair. Patient was discharged on post-operative day 10 and was asymptomatic on follow-up.

scholarly journals In vitroinvestigation of a novel elastic vascular prosthesis for valve-sparing aortic root and ascending aorta replacement

2015 ◽  
Vol 49 (5) ◽  
pp. 1370-1373 ◽  
Author(s):  
Michael Scharfschwerdt ◽  
Moritz Leonhard ◽  
Judith Lehmann ◽  
Doreen Richardt ◽  
Helmut Goldmann ◽  
...  
Keyword(s):  
Aortic Root ◽  
Vascular Prosthesis ◽  
Ascending Aorta ◽  
Aorta Replacement ◽  
Valve Sparing ◽  
Ascending Aorta Replacement
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