A surgically challenging case of an unruptured Sinus of Valsalva aneurysm (SoVA) with severe aortic regurgitation (AR) due to cusp prolapse is presented. Sinus reconstruction with a patch cut out from the sinus portion of a Gelweave Valsalva graft (Terumo Vascutek) was performed. Intraoperative measurements showed insufficient effective height of the right coronary cusp; therefore, cusp plication and pericardial patch augmentation of the right coronary cusp were performed with satisfying result

Regeer, Madelien V

Schoof, Paul H

van Aarnhem, Egidius E H L

van der Zwaan, Heleen B

PubMed

Utrecht University Repository

considered benign. FLNA shows significant variable expressivity inphenotype, which could be elucidated by obtaining a thoroughfamily history. As genetic testing becomes more accessible and knowl-edge of variants becomes more refined, we advocate that genetic eval-uation, counseling, and screening become routine in the care ofcongenital cardiac patients with aortopathy and potentially for allpatients where treatment decisions may be affected.Author’s StatementThe patient consented to publication of this case.Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect tothe research, authorship, and/or publication of this article.FundingThe author(s) received no financial support for the research, author-ship, and/or publication of this article.ORCID iDAdam J. Lobbestael https://orcid.org/0000-0001-7595-1319References1. Reinstein E, Frentz S, Morgan T, et al. Vascular and connectivetissue anomalies associated with X-linked periventricular heteroto-pia due to mutations in Filamin A. Eur J Hum Genet. 2013;21:494‐502.2. Valentin V, Bervar JF, Vincent-Delorme C, et al. Filamin A muta-tions: a new cause of unexplained emphysema in adults? Chest.2021;159(3):e131‐e135.3. Chen M, Choudhury S, Hirata M, et al. Thoracic aortic aneurysm inpatients with loss of function Filamin A mutations: clinical charac-terization, genetics, and recommendations. Am J Med Genet. 2018;176A:337‐350.4. Fukunaga N, Seidman M, David T. Valve-sparing root replacementin a patient with a Filamin A variant. J Thorac Cardiovasc Surg.2021;161:e353‐e355.Surgical Repair of a Sinus ofValsalva AneurysmMadelien V. Regeer, MD, PhD1 ,Heleen B. van der Zwaan, MD, PhD1,Paul H. Schoof, MD, PhD2, and Egidius E. H. L. vanAarnhem, MD31Department of Cardiology, University Medical Center Utrecht,Utrecht, the Netherlands2Department of Cardiothoracic Surgery, Wilhelmina Children'sHospital (WKZ), University Medical Center Utrecht, Utrecht, TheNetherlands3Department of Cardiothoracic Surgery, University Medical CenterUtrecht, Utrecht, the NetherlandsDOI: 10.1177/21501351221109193AbstractA surgically challenging case of an unruptured Sinus of Valsalva aneu-rysm (SoVA) with severe aortic regurgitation (AR) due to cusp pro-lapse is presented. Sinus reconstruction with a patch cut out fromthe sinus portion of a Gelweave Valsalva graft (Terumo Vascutek)was performed. Intraoperative measurements showed insufficienteffective height of the right coronary cusp; therefore, cusp plicationand pericardial patch augmentation of the right coronary cusp wereperformed with satisfying result.IntroductionSinus of Valsalva aneurysm (SoVA) is an uncommon clinical entity. Inthe majority of cases, repair of the SoVA is performed using a polyte-trafluoroethylene (PTFE) or autologous pericardial patch.1 Additionalaortic valve repair or replacement is often necessary for patients withaortic regurgitation (AR). We present a patient with a large SoVAwith severe AR who was treated with selective reconstruction of theright coronary sinus with a Valsalva graft patch and additional cusp pli-cation and extension.CaseA 62-year-old man presented at the emergency department after syncopeduring exertion. On physical examination, he had a diastolic murmur andblood pressure of 171/66 mm Hg. Transthoracic echocardiography(TTE) showed severe AR. Three-dimensional transesophageal echocar-diography (3D-TEE) revealed a large SoVA of the right coronary sinuswith prolapse of the right coronary cusp resulting in leaflet malcoaptationand consequent severe eccentric AR (Figure 1A) with a preservedannular dimension (25 mm). Additional computed tomography (CT)imaging was performed to assess the relation between the aneurysmand the origin of the right coronary artery (RCA), which was justdistal to the SoVA at the sinutubular junction (Figure 1B).Under general anesthesia, a median sternotomy was performed andcardiopulmonary bypass was instituted. After transection of the aorta,the sinus of Valsalva aneurysm was inspected. Neither rupture of theaneurysm into the right ventricle nor a ventricular septal defect wasfound (Figure 2A). Selective sinus reconstruction using a patch cutfrom the sinus portion of a 26 mm Valsalva aortic prosthesisGelweave ValsalvaTM graft (Terumo Vascutek) was performed. TheGelweave ValsalvaTM graft sinus part has longitudinal corrugationsand is concave shaped, which is appropriate for use as a neo-sinus.The SoVA was entirely excluded by implantation of the Valsalvagraft patch. In addition, the prolapse of the right cusp was addressed.The effective height of the right coronary cusp was <9 mm; therefore,central plication and extension of the cusp with a strip of untreatedautologous pericardium were performed. Perioperative 3D-TEESubmitted February 20, 2022; Accepted June 5, 2022Corresponding Author:Heleen B. van der Zwaan, Department of Cardiology, University MedicalCenter Utrecht, Heidelberglaan 100, 3584 CX Utrecht, the Netherlands.Email: h.b.vanderzwaan-3@umcutrecht.nlRegeer et al 807Figure 1. (A) Transesophageal echocardiography: sinus of Valsalva aneurysm with prolapse of the right coronary cusp with severe eccentricaortic regurgitation. (B) Computed tomography: relation between sinus of Valsalva aneurysm (*) and right coronary artery (arrow).Figure 2. Intraoperative photographs and schematic line drawings of the various surgical stages. (A) Inspection of the sinus of Valsalvaaneurysm. (B) 26 mm GelweaveTM Valsalva prosthesis of which the left- and noncoronary sinus of the graft are removed, is implanted in theright coronary sinus. (C) Extension of the right coronary cusp with a strip of autologous pericardium to increase effective height to >9 mm.808 World Journal for Pediatric and Congenital Heart Surgery 13(6)showed a satisfying result with good coaptation length (10-11 mm) andeffective height (13-14 mm) with only a trace residual central AR(grade <1). The postoperative course was uneventful, and the patientwas fully recovered 3 months postoperatively.CommentThe diagnosis of SoVA is made using multimodality imaging. In par-ticular, when there is an indication for surgery due to the size of theaneurysm and/or severe AR, TEE, and CT are useful to clarify theexact anatomy and determine the surgical strategy.2 In cases with sig-nificant AR, accurate preoperative echocardiographic analysis is ofparamount importance when a valve-sparing approach is desired.3The preoperative TEE protocol consists of measurement of aorta diam-eters, determination of aortic valve phenotype (bicuspid or tricuspid),assessment of the mechanism of AR, and evaluation of valve tissue(by measuring effective height and coaptation length).3Even when the coexisting AR is mild in SoVA, there is a substantialrisk of postoperative worsening of the AR. In a series of 198 SoVAcases, 75 had accompanying AR of which 74 were mild and nonewere severe.4 Of these cases, 44 underwent aortic valve repair orreplacement. However, in almost 20%, postoperative AR progressionwas present, which was newly developed in about half of the cases.4This observation highlights the importance of considering restoringvalvular competence even in cases with limited AR. Lansac and col-leagues have proposed a standardized approach aiming for an effectiveheight of ≥9 mm, which is protective from postoperative significant ARand reoperation.5 In addition, even mild residual AR (grade ≥1) on intra-operative TEE may be associated with recurrent significant AR duringfollow-up.6 Controversy persists regarding the preferred material foraortic valve cusp extension. In some reports, the use of glutaraldehyde-treated pericardium for aortic valve cusp extension has been associatedwith recurrent AR and the need for reoperation.7,8 When the effectiveheight is <9 mm, our preference is to utilize other cusp repair techniques,including central plication, and to use untreated autologous pericardiumwhen cusp extension is required. In the case presented, both the aneurysmand cusp prolapse could be effectively repaired by limited surgery creatinga new sinus wall and repairing the pertinent coronary cusp.ConclusionIn patients with SoVA and associated AR, repair of the sinus and cuspshould be performed, aiming for an effective height of ≥9 mm to obtain adurable result. The use of the concave-shaped Gelweave ValsalvaTM graftallows shaping of the neo-sinus and might improve valvular competence.Authors’ StatementThe permission to publish this case report was obtained from the parents.Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect tothe research, authorship, and/or publication of this article.FundingThe author(s) received no financial support for the research, author-ship, and/or publication of this article.ORCID iDMadelien V. Regeer https://orcid.org/0000-0002-1562-8365References1. Jaswal V, Kumar V, Thingnam SKS, Goverdhan DP. Surgicalrepair of ruptured sinus of Valsalva aneurysm: 13-year singlecenter experience. J Card Surg. 2021;36(4): 1264‐1269.2. Nguyen Q, Vervoort D, Phan K, Luc JGY. Surgical managementfor unruptured sinus of Valsalva aneurysms: a narrative review ofthe literature. J Thorac Dis. 2021;13(3): 1833‐1850.3. Berrebi A, Monin JL, Lansac E. Systematic echocardiographicassessment of aortic regurgitation—what should the surgeonknow for aortic valve repair? Ann Cardiothorac Surg. 2019;8(3):331‐341.4. Luo X, Li B, Ju F, et al. Risk factors for aortic regurgitation progres-sion after repair of Sinus of Valsalva aneurysm. Heart Lung Circ.2021;S1443-9506(21)01197-5 (online).5. Lansac E, Di Centa I, Sleilaty G, et al. Remodeling root repair withan external aortic ring annuloplasty. J Thorac Cardiovasc Surg.2017;153(5): 1033‐1042.6. Karciauskas D, Mizariene V, Jakuska P, et al. Long-term outcomesand predictors of recurrent aortic regurgitation after aortic valve-sparing and reconstructive cusp surgery: a single centre experience.J Cardiothorac Surg. 2019;14(1): 194.7. Ram E, Moshkovitz Y, Shinfeld A, et al. Pericardial patchaugmentation is associated with a higher risk of recurrent aorticinsufficiency. Ann Thorac Surg. 2018;106(4): 1171‐1177.8. Myers PO, Tissot C, Christenson JT, Cikrikcioglu M, Aggoun Y,Kalangos A. Aortic valve repair by cusp extension for rheumaticaortic insufficiency in children: long-term results and impact ofextension material. J Thorac Cardiovasc Surg. 2010;140(4):836‐844.Regeer et al 809

Surgical Repair of a Sinus of Valsalva Aneurysm

Regeer, Madelien V.

van der Zwaan, Heleen B.

Schoof, Paul H.

van Aarnhem, Egidius E. H. L.

PubMed Central

https://dspace.library.uu.nl/bitstream/1874/448352/1/21501351221109193.pdf

Surgical Repair of a Sinus of Valsalva Aneurysm

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