1453Ann Thorac Surg CASE REPORT WILLIAMS ET AL2013;95:1453–5 TRICUSPIDIZATION OF QUADRICUSPID AORTIC VALVE

FEA

TU

RE

AR

TIC

LE

S

sliding plasty or foldoplasty to reduce the height of theposterior leaflet, and ring annuloplasty are usually suffi-cient to eliminate regurgitation and minimize the risk ofSAM [4], it can still occur after repair. The use of anedge-to-edge Alfieri repair has been reported for treat-ment and prevention of post-repair SAM [5], reducingthe prevalence of SAM from an estimated 5.7% to 1.1%and with 1 late recurrence of SAM [2].

Systolic anterior motion is an extremely rare problem inchildren and its management has rarely been reported.Delmo-Walters and colleagues [6] described septal myec-tomy and commissural plasties as retention of the anteriormitral leaflet to reduce SAM in 12 children with hypertro-phic obstructive cardiomyopathy. The edge-to-edge Alfierirepair was useful in our patient to address SAM after MVrepair, and could be considered as an additional tool incongenital heart surgeons’ armamentarium for valve repair.Although this repair strategy is not associated with mitralstenosis in adults [2], no medium-term or long-term fol-low-up is available in children.

References

1. Varghese R, Anyanwu AC, Itagaki S, Milla F, Castillo J,Adams DH. Management of systolic anterior motion aftermitral valve repair: An algorithm. J Thorac Cardiovasc Surg2012;143(Suppl 4):S2–7.

2. Myers PO, Khalpey Z, Maloney A, Brinster DR, D’Ambra MN,Cohn LH. Edge-to-edge repair for prevention and treatmentof mitral valve systolic anterior motion. J Thorac CardiovascSurg 2012 [Epub ahead of print].

3. Lee KS, Stewart WJ, Lever HM, Underwood PL, CosgroveDM. Mechanism of outflow tract obstruction causing failedmitral valve repair. Anterior displacement of leaflet coapta-tion. Circulation 1993;88(5 Pt 2):II24–9.

4. Cevasco M, Myers PO, Elbardissi AW, Cohn LH. Foldoplasty:a new and simplified technique for mitral valve repair thatproduces excellent medium-term outcomes. Ann Thorac Surg2011;92:1634–7.

5. Brinster DR, Unic D, D’Ambra MN, Nathan N, Cohn LH.Midterm results of the edge-to-edge technique for complexmitral valve repair. Ann Thorac Surg 2006;81:1612–7.

6. Delmo Walter EM, Siniawski H, Hetzer R. Sustained im-provement after combined anterior mitral valve leaflet reten-tion plasty and septal myectomy in preventing systolic ante-rior motion in hypertrophic obstructive cardiomyopathy inchildren. Eur J Cardiothorac Surg 2009;36:546–52.

Tricuspidization of QuadricuspidAortic ValveLivia Williams, MBBS, Paul Peters, FRACS, andPallav Shah, FRACS

Department of Cardiac Surgery, Princess Alexandra Hospital,Woolloongabba, Brisbane, Australia

A 65-year-old woman presented with New York HeartAssociation class II–III symptoms, no overt signs of heart

Accepted for publication Aug 1, 2012.

Address correspondence to Dr Williams, Department of Cardiac Surgery,

Princess Alexandra Hospital, Ipswich Road, QLD, 4102; e-mail:livia_williams@health.qld.gov.au.

© 2013 by The Society of Thoracic SurgeonsPublished by Elsevier Inc

failure, and echocardiographic findings of a quadricus-pid aortic valve, Hurwitz type C, with severe aorticregurgitation, dilated left ventricle (7 cm), and moderateleft ventricular dysfunction (45%). She subsequentlyunderwent tricuspidization of the valve at the level of theabnormal commissure with subcommissural annulo-plasty. At her 6-week follow-up visit, the patient was inNew York Heart Association class I, with reduction ofleft ventricular diastolic dimensions, trace aortic regurgi-tation, and good mobility of the leaflets.

(Ann Thorac Surg 2013;95:1453–5)© 2013 by The Society of Thoracic Surgeons

Quadricuspid aortic valve (QAV) is a rare congenitalheart defect, which presents in adulthood with

aortic regurgitation requiring surgical management. Wedescribe the presentation, surgical management, andfollow-up of a 65-year-old patient with this diagnosis.

A 65-year-old woman presented with New York HeartAssociation class II–III symptoms. Her medical historyincluded hypertension, atrial fibrillation, glaucoma, andhemithyroidectomy. Clinical assessment revealed anearly diastolic murmur and peripheral signs of severeaortic regurgitation (AR), with no overt signs of cardiacfailure.

Preoperative transesophageal and transthoracicechocardiography demonstrated left ventricular dila-tion of 7 cm with a left ventricular ejection fraction of45%. The quadricuspid valve leaflets (two large equaland two small equal, Hurwitz type C) were pliable,without calcification, with leaflet tips being slightlythickened and no prolapse (Fig 1A). The mechanism ofsevere central AR was leaflet coaptation failure creat-ing a regurgitant orifice (6 � 6 mm, area 0.22 cm2, venacontracta 7 mm, and AR pressure half-time 276 ms).The left ventricular outflow tract diameter was 2.03 cm;transsinus, 2.97 cm; sinotubular, 2.69 cm and ascendingaorta, 3.5 cm. No other pathological cardiac featureswere noted. A coronary angiogram was normal.

The operation was performed through a standard me-dian sternotomy with normothermic cardiopulmonarybypass. The heart was arrested by direct ostial and coldretrograde blood cardioplegia.

The quadricuspid valve was exposed through atransverse aortotomy carried out at the level of thesinotubular junction. The aortic valve comprised twolarger leaflets of equal size (right coronary and non-coronary sinus) and two smaller equal leaflets sur-rounding the left coronary sinus. The three commis-sures of the larger leaflets were normal, whereas thefourth commissure between the two smaller leafletswas thickened, fenestrated, and incomplete. The repairconsisted of detachment of the abnormal commissurefrom the aortic wall to the annulus and excision ofthickened tissue. The two leaflets were joined togetherby two layers of interlocking 6 – 0 Prolene continuoussutures followed by a few interrupted reinforcement

sutures. We checked for prolapse; all three leaflets

0003-4975/$36.00http://dx.doi.org/10.1016/j.athoracsur.2012.08.019

1454 CASE REPORT WILLIAMS ET AL Ann Thorac SurgTRICUSPIDIZATION OF QUADRICUSPID AORTIC VALVE 2013;95:1453–5

FEA

TU

RE

AR

TIC

LE

S

were on level and no further plication stitch wasrequired. The end result was a tricuspid valve withgood coaptation. Subcommissural annuloplasty wasperformed with 2– 0 Ticron pledgeted sutures along allthree commissures to further stabilize the annulus andincrease the coaptation among the three leaflets (Fig 2).The aortotomy was closed in a single layer with 4 – 0Prolene continuous sutures.

The patient was successfully weaned from cardiopul-monary bypass, followed by decannulation and routine

Fig 1. (A) Preoperative transoesophageal echocardiogram of quadri-cuspid aortic valve prior to repair. (B and C) Short axis views posttricuspidisation.

closure. Intraoperative transesophageal echocardiogra-

phy revealed trace aortic regurgitation, a tricuspid aorticvalve with good mobility, and coaptation length of 6 mm(Fig 1B). The postoperative course was uneventful, andthe patient was discharged day 4.

The 6-week follow-up visit showed that the patient wasin New York Heart Association class I. A transthoracicechocardiogram showed a tricuspid valve with goodmobility, trace AR, and a reduction in left ventriculardilation to 5.2 cm.

Comment

Disordered development of the primordia of the semilu-nar cusps within the wall of the aortic trunk may result inaortic valves with two, four, or even five leaflets [1]. Themost extensive review of quadricuspid valve has re-ported a mean age of presentation as 50.7 years and amale predominance [1]. A seven subgroup classificationsystem, based on leaflet size and the degree of cuspequality, was described by Hurwitz, with a valve withfour equally sized cusps was found to be the mostcommon morphology [2]. Nakamura and colleaguesadded to this classification system by defining four sub-categories detailing the location of the accessory cusp.Interestingly, the location of the accessory cusp has notbeen shown to correlate with the occurrence of aorticregurgitation. [3]. Our patient had a Hurwitz type C valve.

The incidence of coronary and cardiac anomalies asso-ciated with quadricuspid valve has been reported to beapproximately 18.3%, with coronary anomalies being themost common (10.2%) [1]. Displacement of the coronaryostia is a frequent finding [3]. A unique case of acute leftmain ostial occlusion by a small accessory leaflet adher-ing to the aortic wall has been described in the pediatricpopulation [4].

Although the true incidence of QAV is less than 0.04%(range, 0.013% to 0.043%) amongst reviewed literature todate, it is suggested that almost half of such valves havepathologic hemodynamics requiring treatment [1]. AR ishypothesized to occur as a result of the unequal distri-bution of force across the asymmetric, abnormally coapt-ing and often fibrosed leaflets of the quadricuspid valve[3]. The cusps may be thickened, restricted, or prolapsingat the time of surgical inspection [5], and central orcommissural regurgitant jets may result from coaptationfailure or abnormal commissural morphology [6]. Cuspfenestrations have been noted in case reports [3, 5, 7].When the condition is detected, 75% of patients presentwith pure AR and 8.5% with Aortic stenosis (AS)/AR;16.5% remain normal. Of the patients with quadricuspidvalve, 45% go on to have surgical treatment [1]. The mostcommon method of surgical treatment for the compro-mised valve has classically involved valve replacement[7]. However, AR, the most frequently occurring patho-logic feature, may be amenable to surgical repair. Tricus-pidization is the typical method of repair for types A, B,or C. This method has been clearly described by the ElKhoury group [6]. The same technique was performedin our patient. The durability of such surgical interven-

tion has only been anecdotally demonstrated to be

1455Ann Thorac Surg CASE REPORT WILLIAMS ET AL2013;95:1453–5 TRICUSPIDIZATION OF QUADRICUSPID AORTIC VALVE

FEA

TU

RE

AR

TIC

LE

S

between 1 and 2.5 years [5, 6] with good results. Bicus-pidization of the QAV was recently described in the pres-ence of two smaller noncoronary leaflets (type G). In thiscase, fusion of the two small noncoronary leaflets to eachother and subsequently to the left coronary leaflet wasperformed. This repair was completed by a subcommis-sural annuloplasty [7], and such bicuspidization was shownto be stable 18 months postoperatively.

Tricuspidization has also been performed after the intra-operative videoscopic evaluation of a QAV through theascending aorta at the time of crystalloid cardioplegia. Sucha direct vision of the valve enabled the surgeon to view thephysiologic effects of a pressurized aortic root on the val-vular leaflets and to plan the surgical strategy [3].

After the incidental finding of this anatomic anomaly, thenonpathologic QAV warrants careful serial monitoring,given the frequency with which this valve can becomehemodynamically compromised over time. Whereas thequadricuspid valve has traditionally been managed byvalve replacement, preoperative assessment with trans-esophageal echocardiogram allows the consideration andplanning of aortic valve repair and the avoidance of pros-

thetic implantation. Long-term follow-up is necessary to

test the durability of repair procedures involving the quad-ricuspid valve before the repair can be considered a stan-dardized procedure.

References

1. Tutarel O. The quadricuspid valve: a comprehensive review.J Heart Valve Dis 2004;13:534–7.

2. Hurwitz LE, Roberts WC. Quadricuspid semilunar valve.Am J Cardiol 1973;31:623–626.

3. Naito K, Ohteki H, Yunoki J, Hisajima K, Sato H, Narita Y.Aortic valve repair for quadricuspid aortic valve associatedwith aortic regurgitation and ascending aortic aneurysm.J Thorac Cardiovasc Surg 2004;128:759–60.

4. Mutsuga M, Tamaki S, Yokoyama Y, et al. Acute occlusion ofleft coronary ostium associated with congenital quadricuspidaortic valve. Ann Thorac Surg 2005;79:1760–1.

5. Schmidt KI, Jeserich M, Aicher D, Schäfers H-J. Tricuspidiza-tion of the quadricuspid aortic valve. Ann Thorac Surg2008;85:1087–9.

6. Jeanmart H, de Kerchove L, El Bitar F, et al. Tricuspidisationof quadricuspid aortic valve: case reports. J Heart Valve Dis2007;16:148–50.

7. Luciani GB, Morjan M, Faggian G, Mazzucco A. Repair ofquadricuspid aortic valve by bicuspidization: a novel tech-

Fig 2. Operative photograph. (A)Quadricuspid valve highlightingraphe between the two smallercusps (arrow). (B) Quadricuspidvalve with all four cusps. (C, D)Excision of raphe from aortic wallto the annulus with tricuspidisa-tion of the valve and subcommis-sural annuloplasty.

nique. Interact Cardiovasc Thorac Surg 2010;11:348–50.