Unicuspid Aortic Valve

HomeCirculationVol. 140, No. 22Unicuspid Aortic Valve Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessLetterPDF/EPUBUnicuspid Aortic ValveDemographics, Comorbidities, Echocardiographic Features, and Long-Term Outcomes Brody D. Slostad, MD, Chance M. Witt, MD, Patrick W. O'Leary, MD, Joseph J. Maleszewski, MD, Christopher G. Scott, MS, Joseph A. Dearani, MD and Patricia A. Pellikka, MD Brody D. SlostadBrody D. Slostad Brody D. Slostad, MD, Department of Internal Medicine, Mayo Clinic, 200 1st St SW, Rochester, MN 55905. Email E-mail Address: [email protected] Departments of Internal Medicine (B.D.S.), Mayo Clinic, Rochester, MN. , Chance M. WittChance M. Witt Cardiovascular Medicine (C.M.W., P.W.O., J.J.M., P.A.P.), Mayo Clinic, Rochester, MN. , Patrick W. O'LearyPatrick W. O'Leary Cardiovascular Medicine (C.M.W., P.W.O., J.J.M., P.A.P.), Mayo Clinic, Rochester, MN. Pediatric and Adolescent Medicine, Division of Pediatric Cardiology (P.W.O.), Mayo Clinic, Rochester, MN. , Joseph J. MaleszewskiJoseph J. Maleszewski Cardiovascular Medicine (C.M.W., P.W.O., J.J.M., P.A.P.), Mayo Clinic, Rochester, MN. Laboratory Medicine and Pathology (J.J.M.), Mayo Clinic, Rochester, MN. , Christopher G. ScottChristopher G. Scott Biomedical Statistics and Informatics (C.G.S.), Mayo Clinic, Rochester, MN. , Joseph A. DearaniJoseph A. Dearani Cardiovascular Surgery (J.A.D.), Mayo Clinic, Rochester, MN. and Patricia A. PellikkaPatricia A. Pellikka Patricia A. Pellikka, MD, Department of Cardiovascular Medicine, 200 1st St SW, Rochester, MN 55905. Email E-mail Address: [email protected] Cardiovascular Medicine (C.M.W., P.W.O., J.J.M., P.A.P.), Mayo Clinic, Rochester, MN. Originally published25 Nov 2019https://doi.org/10.1161/CIRCULATIONAHA.119.041835Circulation. 2019;140:1853–1855Unicuspid aortic valve (UAV) is a rare disease in the general population. However, it is more common in populations with surgically excised stenotic aortic valves, with an estimated incidence of 5%.1 We identified all pathologically confirmed cases of UAV between June 29, 1978, and July 6, 2016, at the Mayo Clinic. Here, we describe the echocardiographic characteristics, associated cardiac comorbidities, and long-term outcomes.This study was approved by the Mayo Clinic Institutional Review Board; all patients allowed access to their records for research. Pathology diagnosis of UAV was defined as congenital (not acquired) fusion resembling an exclamation point of at least 2 commissures based on the presence of at least 2 of the following 3 criteria: an obtuse angle between fused cusps at the commissure, absent cleavage plane between cusps at the fusion point, and raphe present at fused commissures (Figure, A).2 Only cases diagnosed by an experienced cardiac pathologist after aortic valve excision were included, given the limitations of intraoperative surgical UAV diagnosis.3 Echocardiographic data (in accordance with guidelines) were included when available. The Kaplan-Meier method was used for overall survival (log-rank test used for between-group comparisons). Values of P≤0.05 were considered significant.Download figureDownload PowerPointFigure. Unicuspid aortic valve pathology characteristics and long-term outcomes.A, Surgically resected unicuspid (unicommissural) aortic valve (UAV) that exhibits 2 discrete raphes (asterisks) and a single commissure (arrow). The 2 raphes represent the regions of congenital fusion of the nascent aortic valve cusps. B, Kaplan–Meier curve demonstrating similar overall survival of patients with UAV and the general Minnesota population of similar age and sex (P=0.24). C, Kaplan–Meier curve demonstrating similar overall survival of patients with UAV who underwent aortic valve replacement (AVR) alone and those who underwent AVR plus concomitant aortic surgery (P=0.29).Seventy-five pathologically confirmed cases of adults with UAV were identified (71 after surgery, 4 at autopsy; 100% unicommissural; incidence, 0.5%). Age at first presentation to Mayo Clinic was 37±12 years; age at first known congenital aortic valve disease was 35±15 years; and 48 (64%) were male. Index echocardiographic aortic valve status was available for 58 of the 75 patients. Aortic stenosis (AS) of any degree, aortic regurgitation (AR) of any degree, mixed AR/AS, and pure AS were present in 100% (58 of 58), 93% (54 of 58), 93% (54 of 58), and 7% (4 of 58) of patients, respectively. In 7 patients, echocardiography suggested that the predominant lesion was regurgitation because of considerable systolic valve mobility; in 6 patients, the valve was thought to be bicuspid on the basis of echocardiography. However, all 7 patients exhibited systolic doming and mean systolic gradients ranging from 21 to 38 mm Hg, consistent with mixed AR/AS. AS and AR progression before aortic valve intervention occurred in 6 and 12 patients, respectively, during a median follow-up of 1.4 months (interquartile range, 0.2–3.9 months; range, 1 day–15 years). Eight patients had valvotomy before surgery to treat AS, and 1 patient had previous resection of a subvalvular fibrous ridge. Fifty-seven patients (76%) had aortic root or ascending aorta dilatation at index UAV diagnosis. Aortic dimensions progressed in 14 of 47 patients with follow-up ascending aortic imaging (30%) by ≥2 mm (mean 4.4±2.1 mm) during a median follow-up of 10.6 years (interquartile range, 6.3–14.2 years). Twenty patients (27%) had additional structural cardiac comorbidities, including patent foramen ovale in 4, coarctation in 2, hypertrophic cardiomyopathy in 1, and aortic dissection in 1. Family history data, available for 65 patients, included congenital aortic valve disease in 7 (11%).During a median clinical follow-up of 13.2 years (interquartile range, 5.3–17.2 years), 71 patients had surgery involving the aortic valve (age, 38±12 years), including 66 aortic valve replacements (AVRs; 43 mechanical, 12 bioprosthetic, 6 aortic homografts, 4 Ross, 1 unknown), 4 unknown types, and 1 heart transplantation. AVR indications included AS (32), mixed AR/AS (21), AR (9), and ascending aortic dilatation (8). Four had multiple indications. An additional 39 underwent surgical intervention of the aorta (age, 42±11 years), including 22 supracoronary aortic grafts, 15 aortic root replacements, and 2 reduction aortoplasties. Ascending aortic surgery occurred concomitantly with AVR in 35 and after AVR in 4 patients. After surgery, 14 patients had redo AVR, 9 had postoperative pacemaker placement for atrioventricular block, 10 had postoperative atrial fibrillation, 2 had postoperative transient ischemic attack, and 2 had postoperative stroke.Overall survival was 97% at both 5 and 10 years and was similar to that of the general Minnesota population matched for age and sex (P=0.24; Figure, B). Outcomes were similar for patients undergoing AVR alone and AVR with concomitant aorta surgery (P=0.29; Figure, C).Our study confirms that UAV is an uncommon disease. AS (93% mixed with AR, 7% isolated) was the most common initial presentation. Aortopathy was higher than previously described.4 Although similar diseases (ie, bicuspids) have familial inheritance patterns, no familial UAV cases have been described.5 Contrary to this, our study demonstrated a family history of congenital aortic valve disease in 11% of patients with UAV, suggesting a possible genetic mechanism. Overall survival was very good for patients with UAV, and outcomes were similar for those with AVR alone and patients with concomitant aortic surgery. Differences between our study and previous studies are likely because previous studies used surgical or echocardiographic for UAV diagnosis. These have been shown to be poor diagnostic tools3 because of the difficulty distinguishing true congenital UAVs from bicuspid aortic valves compared with the gold standard of postsurgery pathological diagnosis used in our study. Consequently, our results may more accurately represent initial presentations and outcomes of UAV.Sources of FundingFunding was provided by the Department of Cardiovascular Medicine, Mayo Clinic.DisclosuresNone.FootnotesAll data, analytical methods, and study materials supporting the findings of this article are available from the corresponding authors on request.https://www.ahajournals.org/journal/circBrody D. Slostad, MD, Department of Internal Medicine, Mayo Clinic, 200 1st St SW, Rochester, MN 55905. Email [email protected]comPatricia A. Pellikka, MD, Department of Cardiovascular Medicine, 200 1st St SW, Rochester, MN 55905. Email pellikka.[email protected]eduReferences1. Roberts WC, Ko JM. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation.Circulation. 2005; 111:920–925. doi: 10.1161/01.CIR.0000155623.48408.C5LinkGoogle Scholar2. Edwards JE. Pathologic aspects of cardiac valvular insufficiencies.AMA Arch Surg. 1958; 77:634–649. doi: 10.1001/archsurg.1958.04370010166017CrossrefMedlineGoogle Scholar3. Slostad BD, Witt CM, O'Leary PW, Maleszewski JJ, Scott CG, Dearani JA, Pellikka PA. Diagnostic accuracy of echocardiography and intraoperative surgical inspection of the unicuspid aortic valve.Am J Cardiol. 2019; 123:967–971. doi: 10.1016/j.amjcard.2018.12.010CrossrefMedlineGoogle Scholar4. Mookadam F, Thota VR, Garcia-Lopez AM, Emani UR, Alharthi MS, Zamorano J, Khandheria BK. Unicuspid aortic valve in adults: a systematic review.J Heart Valve Dis. 2010; 19:79–85.MedlineGoogle Scholar5. Clementi M, Notari L, Borghi A, Tenconi R. 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November 26, 2019Vol 140, Issue 22 Advertisement Article InformationMetrics © 2019 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.119.041835PMID: 31765262 Originally publishedNovember 25, 2019 Keywordsheart valve diseasescardiac surgical proceduresaortic valve stenosisechocardiographyaortic valvePDF download Advertisement SubjectsAortic Valve Replacement/Transcatheter Aortic Valve ImplantationCardiovascular SurgeryCongenital Heart DiseaseEchocardiographyValvular Heart Disease