Anatomical variations of the aortic valve include unicuspid, bicuspid, and quadricuspid.   The bicuspid aortic valve is most common and affects 1%-2% of the general population.   QAV is generally an isolated malformation, and adults most commonly present with AR.   Coronary anomalies have been reported in up to 10% of patients with QAV.   Prompt and accurate imaging will lead to corrective surgery

Kimball, Thomas Richard

Lewis, LaTasha

Pigula, Frank

Tedga, Anne Aurelie

English

PubMed

DigitalCommons@The Texas Medical Center

From the Division of Ca(F.P.), and Division of COrleans, New Orleans,Correspondence: Annepital of New Orleans,Anne.Tedga@lcmcheaCopyright 2022 by theElsevier Inc. This is an ocreativecommons.org/2468-6441https://doi.org/10.10166Quadricuspid Aortic Valve With GuardedLeft Coronary Artery Ostium in an Infant WithMyocardial InfarctionAnne Aurelie Tedga, MD, Frank Pigula, MD, LaTasha Lewis, MD, andThomas Richard Kimball, MD, New Orleans, LouisianaVIDEO HIGHLIGHTSVideo 1: Parasternal short-axis view demonstrates quad-ricuspid leaflets with smaller left posterior leaflet (arrow) en-trapping the left coronary ostium. A, Anterior; AoV, aortic valve;L, left; LA, left atrium; PV, pulmonary valve.INTRODUCTIONA quadricupid aortic valve (QAV) is a rare congenital anomaly.Patients with QAV typically present with aortic valve regurgitation(AR) in the fifth and sixth decade of life, often leading to aortic valverepair or replacement.1 We present the case of an infant diagnosedwith QAVand a guarded left coronary ostium in our cardiac intensivecare unit (CICU) after a resuscitated cardiac arrest.Viewthevideocontentonlineatwww.cvcasejournal.com.CASE PRESENTATIONA previously healthy 2-month-old boy was transferred to our CICUafter presenting to an outside emergency department with a cardiacarrest. The patient had an uneventful perinatal history and was exclu-sively breastfed. On the day of presentation, the patient becameincreasingly irritable and diaphoretic during feeding and then becameunresponsive. After successful resuscitation, the outside transthoracicechocardiogram (TTE) showed severely diminished left ventricularglobal function with a left ventricular ejection fraction (LVEF) of 28%.The infant was admitted to the CICU onmilrinone and mechanicalventilation. There was profound STsegment depression in the antero-lateral leads of the electrocardiogram suggestive of non-ST elevationmyocardial infarction (Figure 1). Troponin I levels peaked at 42 ng/mL. A complete TTE demonstrated a QAV with an unusual appear-ance of the left coronary artery ostium guarded by a leaflet of the valve(Figure 2; Video 1). The left posterior leaflet entrapped the left sinus ofValsalva (Figure 3) with resultant mild AR but no stenosis. The LVEFwas 25% (biplane Simpson’s). After surgical conference discussion,a cardiac computed tomography scan was performed that demon-strated normal coronary origins, caliber, and course. Given the clearlydelineated pathology seenwith TTE (Figure 4) and the findings on car-diac computed tomography scan, invasive angiography was deemedunnecessary.With inotropic support (calcium chloride, dopamine, and epineph-rine infusions) and afterload reduction with milrinone, the left ventric-ular function improved to an LVEF of 43%. However, the patient had2 consecutive runs of nonsustained pulseless ventricular tachycardiawith Torsades de Pointe morphology. These were felt to be secondaryrdiology (A.A.T., T.R.K.), Division of Cardiothoracic Surgeryardiac Intensive Care Unit (L.L.), Children’s Hospital of NewLouisiana.Aurelie Tedga, MD, Division of Cardiology, Children’s Hos-200 Henry Clay Avenue, New Orleans, LA 70118. (E-mail:lth.org).American Society of Echocardiography. Published bypen access article under the CC BY-NC-ND license (http://licenses/by-nc-nd/4.0/)./j.case.2022.10.005to ischemic changes from the myocardial infarction rather than a pri-mary channelopathy. Due to focal seizures (felt to be secondary to thecardiac arrest), a brain magnetic resonance imaging was performedwith normal findings, and the patient was cleared for cardiovascularsurgery. After the troponin levels stabilized, the patient went to theoperating room for aortic valve exploration on day 7 of admission.Intraoperatively, a QAV was confirmed with the left sinus ofValsalva having both a major and a minor component, the minor be-ing adjacent to the noncoronary sinus. The left coronary artery ostium,severely hypoplastic at 1.5 mm in diameter, resided in the rudimen-tary minor sinus. A left commissuroplasty was performed, and theaortic valve was reconstructed. The surgery was well tolerated, andthe postoperative course was uneventful. Light inotropic supportwas required, and the patient was weaned off of it 2 days postsurgery.The patient remained onmechanical ventilation for 1 day and was ex-tubated to high-flow nasal cannula and subsequently to room air.They were discharged from the cardiology step-down unit 8 dayspostsurgery. The left ventricular function had normalized to anLVEF of 54%. The echo showed relief of the guarding of the ostiumwith good flow into the left main coronary artery demonstrated by co-lor Doppler imaging. There was residual moderate AR (initially mild)and still no stenosis. Postop and until this day, there has been no recur-rence of ventricular tachycardia.DISCUSSIONThe normal aortic valve comprises 3 leaflets: the right, left, and non-coronary sinuses of Valsalva, named after their relationships with cor-responding coronary arteries. Anatomical variations of the aortic valvehave been described in the literature including the unicuspid, bicuspid,and quadricuspid valves. Among these, the most common anomaly isthe bicuspid aortic valve, affecting 1%-2% of the general population.2The QAV is a rare malformation and has an incidence of 0.008% onautopsy, 0.043% on echocardiography, and 1% in patients undergo-ing surgery for isolated AR.3 Hurwitz and Roberts described 7anatomic variations of QAV.4 Type a consists of 4 equal sized coronarycusps. Type b, the most common variation, consists of 3 equal sizedcusps and 1 smaller cusp and is consistent with the anatomy of our pa-tient. Type c consists of 2 equal large and 2 equal smaller cusps. TypeFigure 2 Parasternal short-axis view demonstrates quadricuspid leaflets with 3 equal leaflets and a smaller left posterior leaflet (arrow)entrapping the left coronary ostium. A, Anterior; AoV, aortic valve; L, left; LA, left atrium; PV, pulmonary valve.Figure 1 Electrocardiogram of a 2-month-old patient with non-ST elevation myocardial infarction. It demonstrates ST segmentdepression in leads I, II, and aVF, inferior leads (arrows). It also shows T-wave inversion in the precordial leads.CASE: Cardiovascular Imaging Case ReportsVolume 7 Number 1Tedga et al 7d consists of 1 large cusp, 2 intermediate equal cusps, and 1 smallercusp. Type e consists of 1 large cusp and 3 equal smaller cusps.Type f consists of 2 equal large cusps and 2 small unequal cusps.The last type, type g, consists of 4 unequal cusps.QAV has a male predilection with a male:female ratio of 1.6:1.1QAV is generally an isolatedmalformation, and adult patients typicallypresent with aortic valve dysfunction such as regurgitation, the mostcommon hemodynamically significant lesion, in their fifth and sixthdecade.2,5 Sometimes it is associated with other congenital malforma-tions such as coronary anomalies, including displacement anddislodgement of coronary ostia, which are reported in up to 10% ofpatients with a QAV.1,5 This is important preoperative informationfor surgeons as it helps them to prevent ostial obstruction duringplacement of a prosthetic valve. There have only been a handful ofpediatric case reports requiring surgery due to a coronary ostialobstruction, one of these occurring in Italy.6 This child (in Italy) hadan anatomically similar left coronary artery ostium, also obstructedby a rudimentary accessory left sinus of Valsalva. However, ratherthan ostial coronary artery repair, he had Konno surgery with a19 mm St. Jude mechanical valve placement. Our patient’s presenta-tion with a QAV and coronary ostial anomaly in infancy is rarebecause of associated myocardial infarction leading to cardiac arrest.A 2-month-old boy with the same diagnosis described in Japan byHarada et al.7 had a different presentation. That patient was admittedwith poor weight gain and progressive inability to suckle. The ability todiagnose QAV has been greatly enhanced by echocardiography; how-ever, the aortic leaflets may not be well visualized on TTE.3Sometimes transesophageal echocardiography may provide addi-tional diagnostic value. Surgical correction was delayed in the infantfrom Japan because ostial obstruction was not clearly visualized onFigure 3 Parasternal long-axis view demonstrates abnormal left posterior leaflet (arrow) entrapping left sinus of Valsalva. A, Anterior;Ao, aortic valve; LA, left atrium; LV, left ventricle; RV, right ventricle; S, superior.Figure 4 Parasternal short-axis view: two-dimensional image demonstrates quadricuspid leaflets with the smaller left posterior leafletentrapping the left coronary ostium (white arrow). Color Doppler image demonstrates aliasing of flow as it crosses this rudimentaryleaflet coursing to the left coronary artery (yellow arrow). A, Anterior; Ao, aortic valve; L, left; PV, pulmonary valve.8 Tedga et al CASE: Cardiovascular Imaging Case ReportsJanuary 2023both TTE and coronary angiography initially. However, a transesopha-geal echocardiography and repeat coronary angiography performed1 month later revealed near occlusion of the left coronary arteryostium by a pouch.7 In our case, prompt and accurate diagnosis ofthe aortic valve on initial TTE allowed a valve-sparing repair, correct-ing the ostial obstruction and stenosis while converting the quadricus-pid aortic valve to tricuspid morphology.CONCLUSIONQAV is a rare congenital heart malformation, which can cause signif-icant AR, requiring surgery in late adulthood. Infrequently, it can pre-sent early in life, especially when associated with coronary artery ostialanomalies. Coronary artery anomalies should be ruled out in thesetting of aortic valve abnormalities and evidence of myocardialischemia. The presence of myocardial ischemia is of particular impor-tance because it can predispose to life-threatening cardiac arrest lead-ing to higher mortality and morbidity.ETHICS STATEMENTThe authors declare that the work described has been carried out inaccordance with The Code of Ethics of the World MedicalAssociation (Declaration of Helsinki) for experiments involving hu-mans.CONSENT STATEMENTThe authors declare that written patient consent was not provided forthe following reason: Verbal consent was obtained from patient’sCASE: Cardiovascular Imaging Case ReportsVolume 7 Number 1Tedga et al 9mother onOctober 18, 2022; other than the patient’s age and gender,no other identifiers or personal pictures were used.FUNDING STATEMENTThe authors declare that this report did not receive any specific grantfrom funding agencies in the public, commercial, or not-for-profit sec-tors.DISCLOSURE STATEMENTThe authors report no conflict of interest.ACKNOWLEDGMENTSSpecial thanks to the following sonographers who helped acquire im-ages: Marilyn Perkins-Capers, RDCS (performed the initial TTE thatled to the diagnosis and prompt treatment for the patient); KimPhuong Nguyen, RDCS; Vanessa Mansey, RDCS; CristhelFernandez, RDCS; Ann Cuenca, RDCS; Kristy Reimonenq, RDCS;Nhavan Nguyen, RDCS; Rebecca Kearns, RDCS; Kristie Kussman,RDCS; Baillie Pinion, RDCS; Melinda Tidwell, RDCS; Ally Frye,RDCS.SUPPLEMENTARY DATASupplementary data to this article can be found online at https://doi.org/10.1016/j.case.2022.10.005.REFERENCES1. Tutarel O. The quadricuspid aortic valve: a comprehensive review. J HeartValve Dis 2004;13:534-7.2. MolinaryMC,Martin AB, Asevedo EE,McIlwain EF, Cospolich B, Kerut EK.Normally functioning quadricuspid aortic valve mistaken for bicuspid valvein a septuagenarian. Echocardiography 2021;38:365-7.3. Timperley J, Milner R, Marshall AJ, Gilbert TJ. Quadricuspid aortic valves.Clin Cardiol 2002;25:548-52.4. Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol1973;31:623-6.5. Tsang MYC, Abudiab MM, Ammash NM, Naqvi TZ, Edwards WD,Nkomoand VT, et al. Quadricuspid aortic valve characteristics, associatedstructural cardiovascular abnormalities, and clinical outcomes. Circulation2016;133:312-9.6. Benassi F, DiBenedetto G, Labia C, Stefanelli G. Left coronary ostium isola-tion and aortic valve dysplasia in a young boy: a case report. J Heart ValveDisease 2012;21:135-7.7. Harada T, Fukae K, Ando Y. Surgical repair of ostial obstruction of the cor-onary artery for quadricuspid aortic valve in an infant. Interact CardiovascThorac Surg 2017;24:634-5.

Quadricuspid Aortic Valve With Guarded Left Coronary Artery Ostium In An Infant With Myocardial Infarction

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Quadricuspid Aortic Valve With Guarded Left Coronary Artery Ostium in an Infant With Myocardial Infarction

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Quadricuspid Aortic Valve With Guarded Left Coronary Artery Ostium in an Infant With Myocardial Infarction

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