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Mystery Jets: A World of Possibilities

Published:January 05, 2023DOI:https://doi.org/10.1053/j.jvca.2022.12.033
      SOME 60,000 YEARS AGO, homo sapiens started migrating around the world. Their migration historically depended on climate, pressures of the population, and the invention of technologies. Today, similar factors determine the migration and movement of people across the globe, and with these movements, global physicians have a duty to learn about the diseases that are prevalent in different people from other areas of the world.
      Frequently, perioperative physicians will encounter clinical findings that surprise us and force us to act quickly and decisively. Aortic regurgitation is not uncommon in a patient undergoing cardiac surgery. However, its origin and source can provide vital information for cardiac anesthesiologists and surgeons intraoperatively. Understanding these global clues will help us know the etiology of the disease process and its management as we manage patients in the global era.
      In this issue, Ren et al. described a fascinating case of a 67-year-old Chinese male patient admitted to the hospital with shortness of breath, edema in the bilateral lower extremities, and signs and symptoms suggestive of congestive heart failure, who was diagnosed with severe aortic regurgitation with bicuspid aortic valve and was scheduled for a Bentall procedure.
      • Ren J
      • Chen M
      • Tang L
      An unusual cause of aortic regurgitation in a patient with bicuspid aortic valve [e-pub ahead of print].
      The authors described the aortic regurgitation originating from disrupted fibrous bands of tissue originating in the aortic valve as an embryologic remnant. Bicuspid aortic valves have aortic regurgitation in 13% of the patients.
      • Sabet HY
      • Edwards WD
      • Tazelaar HD
      • et al.
      Congenitally bicuspid aortic valves: A surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases.
      The etiology of aortic regurgitation in the bicuspid aortic valve can range from mechanical wear and tear of the valves to intrinsic embryologic defects.
      • Maurer G.
      Aortic regurgitation.
      Fibrous connective tissue bands represent embryologic remnants of the cusps of the aortic valve and may be a causative factor, as described by the authors. Geographically, most cases of aortic valve fibrous bands have been prevalent in Japan and Southeast Asia.
      • Yuan S
      • Mou R
      • Sun X
      • et al.
      Aortic chordae tendineae strands with significant aortic regurgitation.
      Some postulated mechanisms for the regurgitation are the rupture of the fibrous strands between the aortic valve and cusps and the aortic wall or perforation of the cusps.
      • Bouchachi A-A
      • Folliguet T
      • Hébert J-L
      • et al.
      A severe restrictive aortic regurgitation resulting from valve tenting by unusual aortic chordae tendineae strands.
      It is important to note that knowing the geographic and embryologic origin of these fibrous strands will allow us to make informed decisions about the care of these patients. Identifying predisposed populations can help obtain early imaging like transesophageal echocardiography (TEE) or sophisticated imaging modalities like computed tomography or magnetic resonance imaging. The use of TEE was shown to be superior in recognition of these fibrous bands in the aortic valves in comparison to transthoracic echocardiography, and it is important to bring TEE early into your imaging algorithm.
      • Nishida H
      • Suenaga E
      • Ishii K.
      Acute aortic regurgitation following fibrous strand rupture of aortic valve successfully diagnosed by transesophageal echocardiography.
      As perioperative physicians and echocardiographers, it is important to differentiate the common causes of aortic regurgitation, such as infective endocarditis, aortic root dilation, rheumatic valve disease, neoplasms, and aortic dissection, from these rare causes like fibrous bands causing regurgitation secondary to band perforation found commonly in a subset of patients from another part of the world. Recognition of these exact pathologies by echocardiographers will help the decision-making process and the surgical approach for these patients. A clear differentiation from an infective vegetative process will allow for careful and timely surgical planning and a better postoperative outcome for these patients. A minority of the valves with ruptured fibrous strands will be amenable to valve repair with a suspension of the cusp, plication, or reinforcement with geometric annuloplasty.
      • Ren J
      • Chen M
      • Tang L
      An unusual cause of aortic regurgitation in a patient with bicuspid aortic valve [e-pub ahead of print].
      Modern humans, much like our early ancestors, need to learn from the experiences of different people from different regions of the world, their local environmental factors, and their habitats that predispose them to certain pathologies. Our early ancestors carved and etched their experiences on the walls of ancient architecture, Vedas, texts, manuscripts, and tablets. Modern humans like us need to continue the tradition of documenting the origins of many such pathologies in different parts of the world.

      Conflict of Interest

      None.

      References

        • Ren J
        • Chen M
        • Tang L
        An unusual cause of aortic regurgitation in a patient with bicuspid aortic valve [e-pub ahead of print].
        J Cardiothorac Vasc Anesth. 2023; https://doi.org/10.1053/j.jvca.2022.12.024
        • Sabet HY
        • Edwards WD
        • Tazelaar HD
        • et al.
        Congenitally bicuspid aortic valves: A surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases.
        Mayo Clin Proc. 1999; 74: 14-26
        • Maurer G.
        Aortic regurgitation.
        Heart. 2006; 92: 994-1000
        • Yuan S
        • Mou R
        • Sun X
        • et al.
        Aortic chordae tendineae strands with significant aortic regurgitation.
        Int Heart J. 2021; 62: 1160-1163
        • Bouchachi A-A
        • Folliguet T
        • Hébert J-L
        • et al.
        A severe restrictive aortic regurgitation resulting from valve tenting by unusual aortic chordae tendineae strands.
        Circulation. 2012; 126: e139-e141
        • Nishida H
        • Suenaga E
        • Ishii K.
        Acute aortic regurgitation following fibrous strand rupture of aortic valve successfully diagnosed by transesophageal echocardiography.
        Echocardiography. 2018; 35: 753-754

      Linked Article

      • An Unusual Cause of Aortic Regurgitation in a Patient With Bicuspid Aortic Valve
        Journal of Cardiothoracic and Vascular Anesthesia
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          Aortic fibrous strands are considered residual tissue from aortic valve development. Rupture of these strands is an important albeit uncommon cause of aortic regurgitation (AR). The authors describe a 67-year-old man who was admitted to the authors’ hospital with sudden onset shortness of breath and diagnosed with severe AR. The patient was scheduled for Bentall surgery. The transesophageal echocardiogram (TEE) found multiple fibrous strands that were present in multiple locations of the aortic valve, some of which were ruptured.
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