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- W3080408068 abstract "SINUS OF Valsalva aneurysms (SVAs) may remain asymptomatic or present with complications such as aneurysm rupture, aortic insufficiency, disruption of the cardiac conduction system, compression of a coronary artery, or thrombus formation with subsequent embolization.1Bass D. Tivakaran V.S. Sinus of Valsalva aneurysm. StatPearls Publishing, Treasure Island, FL2020Google Scholar Their most feared complication, SVA rupture, carries a high mortality unless managed with early surgical intervention. Given its rarity, varied presentations, and proximity to multiple critical structures, a ruptured SVA poses a diagnostic challenge even among experts. Imaging modalities, such as transthoracic echocardiography (TTE), multidetector cardiac computed tomography (MDCT), and cardiac magnetic resonance (CMR) imaging, play an important role in surgical planning; however, transesophageal echocardiography (TEE) remains the modality of choice during definitive intervention to assist real time in identifying subtle, yet important, findings that can alter the surgical plan. In the current issue of the Journal of Cardiothoracic and Vascular Anesthesia, Togashi et al. reported 2 cases of SVA rupture associated with a concomitant ventricular septal defect (VSD) for which the intraoperative TEE assessment differed from the preoperative diagnosis and revealed additional heart defects, resulting in a modification of the initial surgical plan.2Togashi K. Paez F.G. Sheu R. Sinus of Valsalva aneurysm rupture associated with a ventricular septal defect: The importance of multi-angle assessment by intraoperative transesophageal echocardiography.J Cardiothorac Vasc Anesth. 2020; 34: 3372-3377Abstract Full Text Full Text PDF Scopus (1) Google Scholar Although the cardiac lesions associated with SVA and their echocardiographic assessment have been well-described in the literature, the authors provided a focused intraoperative TEE approach to help differentiate a VSD from a ruptured SVA. In addition, the authors stressed the importance of multi-angle TEE assessment using a combination of color-flow Doppler, spectral Doppler, and 3-dimensional (3D) imaging modalities to correctly diagnose the presence of a VSD in patients with a ruptured SVA. The most common accompanying cardiac defect in a patient with an SVA is a VSD. Approximately 60% of patients with congenital SVAs have associated VSDs, typically of the perimembranous type in the Western population and of the subpulmonic type in the Asian population.3Feldman D.N. Gade C.L. Roman M.J. Ruptured aneurysm of the right sinus of Valsalva associated with a ventricular septal defect and an anomalous coronary artery.Tex Heart Inst J. 2005; 32: 555-559PubMed Google Scholar,4Hartlage G.R. Consolini M.A. Pernetz M.A. et al.Bad company: Supracristal VSD presenting with ruptured sinus of Valsalva aneurysm. A case presentation with echocardiographic depiction and an analysis of contemporary literature.Echocardiography. 2015; 32: 575-583Crossref PubMed Scopus (5) Google Scholar Discriminating an isolated SVA rupture from an SVA rupture with a concomitant VSD can be quite challenging. A VSD may go undetected, especially if the aneurysm itself or a prolapsing cusp of the aortic valve occludes it.5Cheng T.O. Yang Y.L. Xie M.X. et al.Echocardiographic diagnosis of sinus of Valsalva aneurysm: A 17-year (1995-2012) experience of 212 surgically treated patients from one single medical center in China.Int J Cardiol. 2014; 173: 33-39Abstract Full Text Full Text PDF PubMed Scopus (34) Google Scholar In addition, when an SVA is in close proximity to a VSD, an unruptured SVA may be misdiagnosed as an SVA with rupture, as opposed to an intact SVA with a coexisting VSD. The presence of concomitant aortic regurgitation with an SVA can further complicate the diagnosis and masquerade the presence of a VSD.6Jain P.K. Narula J. Hasija S. et al.Is it really ruptured sinus of Valsalva? The crucial role of comprehensive transesophageal echocardiography in clinical decision-making.Ann Card Anaesth. 2015; 18: 221-224Crossref PubMed Scopus (1) Google Scholar Both aortic regurgitation and a ruptured SVA demonstrate diastolic flow reversal in the descending aorta, and are associated with a predominantly diastolic jet. Careful imaging of the origin of the jets in relation to the aortic valve annulus and the timing of the jets in relation to the cardiac cycle are paramount in distinguishing these 2 distinct entities.2Togashi K. Paez F.G. Sheu R. Sinus of Valsalva aneurysm rupture associated with a ventricular septal defect: The importance of multi-angle assessment by intraoperative transesophageal echocardiography.J Cardiothorac Vasc Anesth. 2020; 34: 3372-3377Abstract Full Text Full Text PDF Scopus (1) Google Scholar VSDs originate below the annulus, with a predominantly systolic flow, whereas ruptured SVAs originate at a supra-annular level, with a continuous flow throughout the cardiac cycle, predominantly in diastole.7McGregor P. Temtanakitpaisan Y. Hiltbolt A. et al.A spectrum of sinus of Valsalva aneurysm-from the young to the old.Echocardiography. 2017; 34: 1524-1530Crossref PubMed Scopus (3) Google Scholar As such, there are no established guidelines for testing and diagnosis of SVA or its associated complications. TTE is widely used as the first-line imaging modality in patients suspected of having an SVA for its convenience, portability, and accuracy. In a single-center study of 212 patients with SVA in which TTE findings were corroborated with the surgical findings, the sensitivity, specificity, and accuracy of TTE were 93.9%, 99.9%, and 99.8%, respectively.5Cheng T.O. Yang Y.L. Xie M.X. et al.Echocardiographic diagnosis of sinus of Valsalva aneurysm: A 17-year (1995-2012) experience of 212 surgically treated patients from one single medical center in China.Int J Cardiol. 2014; 173: 33-39Abstract Full Text Full Text PDF PubMed Scopus (34) Google Scholar Approximately 72% of the SVAs were ruptured, most commonly into the right ventricle (67.9%), followed by the right atrium (27.4%). The most frequently misdiagnosed associated lesions with SVAs were right ventricular outflow tract obstruction and VSDs. Interestingly, of the 13 SVAs that were missed on TTE, 77% were small aneurysms of the right coronary sinus extending into the right ventricle across a VSD.5Cheng T.O. Yang Y.L. Xie M.X. et al.Echocardiographic diagnosis of sinus of Valsalva aneurysm: A 17-year (1995-2012) experience of 212 surgically treated patients from one single medical center in China.Int J Cardiol. 2014; 173: 33-39Abstract Full Text Full Text PDF PubMed Scopus (34) Google Scholar The authors suggested that these lesions were missed by TTE either as a result of the complex anatomy around the aortic root or because of the Bernoulli effect of the VSD shunt, resulting in a negative-pressure suction of surrounding tissues into the septal defect. Although TTE serves well as a screening tool, approximately one quarter of patients may have poor acoustic windows, thereby precluding accurate diagnosis of an SVA or its associated conditions. There are no large comparative studies to assess the diagnostic accuracy of TEE versus TTE for SVA; however, there are several case reports or case series that point to the incremental value of TEE over TTE.6Jain P.K. Narula J. Hasija S. et al.Is it really ruptured sinus of Valsalva? The crucial role of comprehensive transesophageal echocardiography in clinical decision-making.Ann Card Anaesth. 2015; 18: 221-224Crossref PubMed Scopus (1) Google Scholar,8Martín A.G. Ruiz J.M.O. González A.E. et al.Multiplane transesophageal echocardiography in the preoperative evaluation of the sinus of Valsalva fistula to right chambers.Rev Esp Cardiol. 2002; 55: 29-36PubMed Google Scholar,9Dhawan I. Malik V. Sharma K.P. et al.Transthoracic echocardiography versus transesophageal echocardiography for rupture sinus of Valsalva aneurysm.Ann Card Anaesth. 2017; 20: 245-246Crossref PubMed Scopus (4) Google Scholar Martín et al., in their case series of ruptured SVA, found TEE to be more accurate compared with TTE and angiography for detection of the sinus involved, presence of aneurysm prolapse through a VSD, and detection of other congenital or acquired cardiac anomalies.8Martín A.G. Ruiz J.M.O. González A.E. et al.Multiplane transesophageal echocardiography in the preoperative evaluation of the sinus of Valsalva fistula to right chambers.Rev Esp Cardiol. 2002; 55: 29-36PubMed Google Scholar Similar to the cases described by Togashi et al. in this Journal, Gürgün et al. reported on a patient for whom TTE detected the SVA, but a coexistent VSD was missed, which was diagnosed subsequently with the assistance of TEE.10Gürgün C. Özerkan F. Akin M. Ruptured aneurysm of sinus of Valsalva with ventricular septal defect: The role of transesophageal echocardiography in diagnosis.Int J Cardiol. 2000; 74: 95-96Abstract Full Text Full Text PDF PubMed Scopus (11) Google Scholar Kumar et al. also previously reported in this Journal on a case that was misdiagnosed as an SVA with preoperative imaging that included TTE and MDCT.11Kumar P.A. Brandon M.W. Kyle R.W. et al.Unusual finding in a patient for sinus of Valsalva aneurysm repair.J Cardiothorac Vasc Anesth. 2012; 26: 352-353Abstract Full Text Full Text PDF PubMed Scopus (1) Google Scholar An intraoperative TEE after induction of anesthesia revealed that the right coronary artery had a funnel-shaped ostium, a normal variant, which made the sinus of Valsalva appear prominent and aneurysmal and led to the misdiagnosis. Proximity of the aortic valve, aortic root, and the ascending aorta to the esophagus results in high-quality and high-resolution echocardiographic images with TEE and, thereby, a higher diagnostic accuracy of SVA and its associated complications. Advances in spatial and temporal resolution in 3D echocardiography, both for TTE and TEE, may further assist in improving the assessment of SVA and adjacent cardiac structures. The ability to obtain a pyramidal volume scan rather than a sector scan provides elevational resolution to allow for the simultaneous visualization of closely located structures such as SVA and VSD. Real-time 3D TEE imaging also plays an important role in guiding transcatheter repair of SVAs and assessment of adequacy of the surgical repair.12Chigurupati K. Kumaresan B. Gadhinglajkar S. et al.Multiple unruptured aortic sinus of Valsalva aneurysms: A rare presentation.Echocardiography. 2017; 34: 317-319Crossref PubMed Scopus (4) Google Scholar Data on tomographic assessment using imaging modalities such as MDCT and CMR for the diagnosis of SVAs are limited. Undoubtedly, MDCT and CMR provide accurate delineation of SVA and measurement of the sinus and vascular dimensions. Sinus-to-sinus, as opposed to sinus-to-commissure, in end-diastole is recommended for measurement of aortic sinus dimensions as per the 2015 Guidelines for Multimodality Imaging of Diseases of the Thoracic Aorta.13Goldstein S.A. Evangelista A. Abbara S. et al.Multimodality imaging of diseases of the thoracic aorta in adults: From the American Society of Echocardiography and the European Association of Cardiovascular Imaging: Endorsed by the Society of Cardiovascular Computed Tomography and Society for Cardiovascular Magnetic Resonance.J Am Soc Echocardiogr. 2015; 28: 119-182Abstract Full Text Full Text PDF PubMed Scopus (286) Google Scholar Unfortunately, MDCT does not provide accurate functional assessment regarding flow in cases of rupture, aortic regurgitation, or coexisting VSDs.14Bricker A.O. Avutu B. Mohammed T.L. et al.Valsalva sinus aneurysms: Findings at CT and MR imaging.Radiographics. 2010; 30: 99-110Crossref PubMed Scopus (69) Google Scholar The advantages of CMR imaging, aside from its lack of exposure to ionizing radiation or iodinated contrast material, are its ability to provide a functional assessment of cardiac function and flow.15Xu B. Kocyigit D. Betancor J. et al.Sinus of Valsalva aneurysms: A state-of-the-art imaging review.J Am Soc Echocardiogr. 2020; 33: 295-312Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar CMR enables dynamic assessment of SVA morphology, including its effect on the surrounding structures. It has the capability to assess left ventricular hemodynamic pattern and identify aortic regurgitation, aortocardiac shunt, or fistulous blood flow. Moreover, through acquisition of velocity-encoded cine phase-contrast sequences in the proximal ascending aorta and main pulmonary artery, CMR allows for calculation of the ratio of pulmonary blood flow-to- systemic blood flow, which is helpful in diagnosing a ruptured SVA. A caveat to consider with CMR is that the sequences are not flow-sensitive and jets sometimes can appear inconspicuous even when there is a large volume of flow across a fistulous tract.16Ho V.B. Kinney J.B. Sahn D.J. Ruptured sinus of Valsalva aneurysm: Cine phase-contrast MR characterization.J Comput Assist Tomogr. 1995; 19: 652-656Crossref PubMed Scopus (20) Google Scholar Undoubtedly, ruptured SVAs pose a significant diagnostic conundrum. They can present with confusing symptoms of dyspnea and chest pain, similar to an acute coronary syndrome. Large ruptures can cause rapid volume loading with hemodynamic collapse, whereas small ruptures may be insidious or asymptomatic. Prompt recognition and surgical repair, especially of subpulmonic VSDs in patients with SVA, are important in order to avoid further damage to the pulmonary and/or aortic valve.17Lin C.Y. Hong G.J. Lee K.C. et al.Ruptured congenital sinus of Valsalva aneurysms.J Card Surg. 2004; 19: 99-102Crossref PubMed Scopus (34) Google Scholar Also, preoperative recognition of associated left-to-right communications is important for surgical planning. If the surgical procedure involves the left side of the heart, a dual-stage venous cannulation approach is usually the preferred method for decompression of the right side of the heart. However, dual-stage venous cannulation in the presence of a left-to-right communication can entrain air upon opening of the aorta and potentially can result in inadequate venous drainage or an air lock in the venous line, both of which potentially can be catastrophic. Therefore, when a right atriotomy approach is planned for the repair of a VSD, a bicaval cannulation is required. Regardless of the imaging modality, the most important consideration with echocardiography is that it is highly operator-dependent, both for image acquisition and for its interpretation. It, therefore, requires an astute echocardiographer to painstakingly adjust and align the images while using non-standard views and different modalities (eg, 2D, color-flow Doppler, spectral Doppler, 3D) to detect and characterize defects that are in such close proximity. In addition, a thorough understanding of the anatomic spatial relations and a rigorous knowledge of congenital defects are as important as the echocardiography skills. Having a high index of suspicion is critical to prompt the echocardiographer to “seek” and therefore “find” specific defects in rare entities such as SVAs. Sinus of Valsalva Aneurysm Rupture Associated With a Ventricular Septal Defect: The Importance of Multi-Angle Assessment by Intraoperative Transesophageal EchocardiographyJournal of Cardiothoracic and Vascular AnesthesiaVol. 34Issue 12PreviewALTHOUGH RARE, sinus of Valsalva aneurysms (SVAs) have been reported in the literature as far back as early 1900s.1 When the SVA is of congenital etiology, it most commonly originates in the right coronary sinus, followed by noncoronary sinus and, on rare occasions, left coronary sinus.2,3 It is well- reported in the literature that ruptured SVA frequently is associated with ventricular septal defects (VSDs), especially when the rupture is occurring into the right ventricle (RV).4,5 Most commonly, subpulmonic VSD is associated with ruptured SVA. Full-Text PDF Erratum to ‘Ruptured Sinus of Valsalva Aneurysms: Does Transesophageal Echocardiography Have a Role in the Era of Sophisticated Cardiac Imaging?’ [Journal of Cardiothoracic and Vascular Anesthesia 34 (2020) 3382-3384]Journal of Cardiothoracic and Vascular AnesthesiaVol. 35Issue 4PreviewThe Publisher would like to inform you that the name of the second author, Priya A Kumar, was incorrectly stated in the published Editorial. Full-Text PDF" @default.
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