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. 2023 Jan 23;19(2):E220322202505. doi: 10.2174/1573403X18666220322092706

Quadricuspid Pulmonary Valve: Case Report and the Comparison with Quadricuspid Aortic Valve

Toshiharu Miyake 1,*, Tomohiro Inoue 1, Sotaro Mushiake 1
PMCID: PMC10201900  PMID: 35319379

Abstract

Background

Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly. Simple QPV had been mainly diagnosed at the time of autopsy before 2000, and the frequency rates of QPV are approximately 0.02%–0.41%. QPV was initially diagnosed using transthoracic echocardiography (TTE) after 2000 and with contrast computed tomography (CT) or cardiac magnetic resonance imaging (CMR) after 2009. Obtaining the cross-sectional view of the pulmonary valve using TTE is difficult. We aimed to review the papers regarding the incidence, embryology, diagnosis, associated congenital heart anomalies, and prognosis in patients with QPV, and furthermore to compare with those in patients with quadricuspid aortic valve (QAV).

Case Presentation

We diagnosed QPV with mild stenosis in a 12-month-old infant. With a slight angulation of the transducer superiorly from the left high parasternal short-axis view, a short-axis view of QPV was obtained.

Results

In QPV cases diagnosed at autopsy, Hurwitz’s type-b with three equal cusps and one smaller cusp is dominant, whereas Hurwitz’s type-a with four equal cusps is dominant in clinically diagnosed cases. Congenital heart anomaly and valvular stenosis are more frequent in patients with QPV than in patients with QAV. Coronary artery anomalies and infectious endocarditis are more frequent in patients with QAV than in patients with QPV. The incidence of PR is more common in type-a QPV than in type-b QPV. There is no difference between type-a QAV and type-b QAV with respect to the incidence of aortic regurgitation (AR). It is assumed that QPV is a risk factor for a Ross operation. However, QPVs have been used as autografts in certain patients.

Conclusion

Between QPV and QAV, various differences were found in frequency rates, diagnostic methods, valve morphology, valve function, associated congenital heart diseases, and frequencies of infectious endocarditis.

Keywords: Aortic regurgitation, echocardiography, infant, neural crest cell, pulmonary regurgitation, pulmonary stenosis

1. INTRODUCTION

Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly with a frequency of approximately 0.02%–0.41% [1-11]. Because it is clinically silent in the majority of cases, the main method to diagnose this cardiac malformation until the beginning of the 21th century was at the time of autopsy. In 2000, a case of QPV diagnosis using transthoracic echocardiography (TTE) was reported [12]. A limited number of patients with pulmonary artery dilatation caused by valvular stenosis were diagnosed because obtaining the short-axis view of the pulmonic valve using TTE is difficult. After 2009, contrast computed tomography (CT) [13, 14] and cardiac magnetic resonance imaging (CMR) [15, 16] were used to diagnose QPV in adults. A few QPV patients with congenital heart disease were reported at the time of surgical operation. The purpose of our study was to collect published cases of clinically diagnosed patients with quadricuspid semilunar valve and to analyse the diagnostic, morphological, and prognostic characteristics of such patients.

2. CASE PRESENTATION

A 1-month-old girl was identified with a heart murmur during a medical checkup. She was born at 40 weeks gestation and weighed 2994g. There was no known family history of congenital heart disease. TTE revealed pulmonary valve stenosis. In the left parasternal right ventricular outflow tract view, thickened pulmonary valves showed dome formation, and the main pulmonary artery was dilated. Continuous Doppler echocardiography demonstrated a systolic pressure gradient of 15 mmHg between the right ventricle and the main pulmonary artery. At 12 months of age, with a slight angulation of the transducer superiorly from the left high parasternal short-axis view, a short-axis view of QPV was obtained (Fig. 1). The pulmonary valve diameter was 12.5 mm (106% of the normal). Color Doppler echocardiography showed trivial pulmonary regurgitation (PR) from the center of four equal-sized cusps (Hurwitz’s type-a). Mild pulmonary stenosis and trivial PR remained unchanged at 4 years of age.

Fig. (1).

Fig. (1)

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High parasternal short-axis view; equal-sized four pulmonary valves.

3. DISCUSSION

3.1. Incidence of Quadricuspid Semilunar Valve

The frequency of QPV diagnosis in autopsy cases is greater than that of QAV (0.02%–0.41% vs. 0%–0.11%) [1-11]. Japan has the greatest frequency of QPVs in autopsies of individuals aged ≥60 years (0.41%) [7], and the majority of these cases are not diagnosed with valvular dysfunction. Sharif et al. [17] performed a screening of 19,000 consecutive echocardiographic examinations and found the prevalence of QAV to be 0.06%. The sex distribution of QPV is male predominant (male 52: female 27) [5], whereas the sex distribution of QAV was found to be either male predominant (male 100: female 62) [18] or almost equal (male 24: female 26) (Table 1) [19].

Table 1.

The incidence of quadricuspid semilunar valve at autopsy and donor heart (Number of >3000).

Author Year Country QPV QAV Number
Simpson [1] 1890 England 4 (0.09) 0 4252
Thilo* 1909 Germany 2 (0.05) 1 (0.02) 5814
de Varies [2] 1918 Netherland 9 (0.25) 1 (0.03) 3600
Houck** 1929 USA 2 (0.04) ND 5000
Dagnini** 1930 Italy 5 (0.10) ND 5000
McGinn*** 1936 USA 3 (0.04) 0 7500
Szypulski*** 1937 USA 2 (0.03) 3 (0.04) 7500
Rannels*** 1937 USA 1 (0.02) 0 4255
Koletsky [3] 1941 USA 6 (0.17) 0 3600
Becker [4] 1972 Netherland 20 (0.40) ND 5000
Hurwitz [5] 1973 USA 8 (0.13) 2 (0.03) 6000
Lewis [6] 1984 USA 11 (0.11) ND 10384
Ohkawa [7] 2009 Japan 35 (0.41) 9 (0.11) 8500
Jashari [8]***** 2009 Belgium 8 (0.21) 0 3861
Demircin [9] 2013 Turkey 2 (0.06) 0 3493
Total Number (%) 118 (0.14) 16 (0.03)
Range (0.02-0.41) (0-0.11)
Autopsy Number 83759 58375
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ND, not described; QAV, quadricuspid aortic valve; QPV, quadricuspid pulmonary valve; *, cited from Simonds [10]; **, cited from Kissin [11]; ***, cited from Koletsky [3]; ****, isolated lesion; *****, donor heart.

3.2. Variations in the Morphology of Quadricuspid Semilunar Valve at Autopsy

The classification of seven types by Hurwitz and Roberts [5] is generally used in both quadricuspid semilunar valves: (a) four equal cusps; (b) three equal cusps, one smaller cusp; (c) two equal larger cusps, two equal smaller cusps; (d) one large, two intermediate, one small cusp; (e) three equal cusps, one larger cusp; (f) two equal larger cusps, two unequal smaller cusps; (g) four unequal cusps. In cases of QPV diagnosed at autopsy, type-b is the most frequent (60%), followed by type-c (15%) and type-a (12%) [5]. Another anatomical classification of QPV was proposed by Becker [4], which is based on the degree of the supernumerary cusp development: (1) four cusps, mutually well delineated; (2) a supernumerary cusp, imperfectly separated from its neighboring cusps; and (3) a rudimentary supernumerary cusp, indicated only by a raphe in the conjoined cusp. In cases of QAV diagnosed using TTE, type-a (32%) and type-b (32%) are the most frequent followed by type-c (16%), type-d (8%), type-f (8%), and type-e (2%) [19].

3.3. Embryology of Quadricuspid Semilunar Valve

Abnormal cusp formation is due either to the aberrant fusion of the aorticopulmonary septum or to the abnormal proliferations in the common trunk [5]. QPVs resulted from the partition of one of three valve cushions at a very early stage of the valvulogenesis in a Syrian hamster [20]. QAVs result from the partition of one of the normal mesenchymal cushions, which normally gives rise to tricuspid valves [21]. Phillips et al. [22] reported that neural crest cells condensation within the cardiac jelly is required for correct positioning of the outflow cushions and that this process is essential for normal patterning of the arterial valve leaflets with disruption leading to a spectrum of valve leaflet patterning anomalies, abnormal positioning of the orifices of the coronary arteries. In a 26-year-old man with type-b QPV, the right ventricular myocardium exceeded the level of the hinge in all three normal leaflets, which was not observed in the additional leaflet. This abnormality is likely to be a result of disturbed embryonic development [23].

3.4. Clinical Diagnosis

3.4.1. Diagnosis of QPV using TTE

Zuppiroli et al. [24] described a characteristic of the long-axis view of the pulmonary valve in a patient with QPV diagnosed at autopsy. An abnormal linear echo, parallel to the major axis of the pulmonary artery, was present; in diastole, it was attached to the echoes, which are a normal feature of the pulmonary valve, and it disappeared in systole.

Hedayat et al. [12] reported the short-axis view of QPV using TTE for the first time in 2000. They described that it is not noted more frequently due to (1) being rarely associated with other cardiac anomalies and clinically quiescent, thus there is no clinical reason for obtaining a TTE; (2) also, there is a certain amount of technical difficulty in imaging this area of the cardiac anatomy due to the oblique lie of the pulmonic valve on the parasternal view (Table 2) [12, 25-37].

Table 2.

Patients diagnosed using transthoracic echocardiography.

Authors Year Age Sex Congenital
Heart
Defect 		Dilated
Pulmonary
Artery 		RV-PA
PG
(mmHg) 		Pulmonary
Regurgitation 		QPV
Type
(Hurwitz) 		Intervention
Hedayat [12] 2000 6hr F PS (-) - - - b -
Hirooka [25] 2001 42 M PS BAV ND 1.8m/sec* mild-mod. b -
Kotani [26] 2002 74 M PS + 34 Sellers III b PTPV
Ricci [27] 2005 NB F TGA - ND - g arterial switch
Fernández-Armenta [28] 2009 66 F PS (-) + ND + a -
Yoon [29] 2011 76 F PS (-) + ND mod. a -
Gouveia [30] 2011 6 M QAV (type f) + 15 - ND -
Aboitiz-Rivera [31] 2015 1m F PS, ASD + 16 - a -
Jung [32] 2015 53 M PS + 16 mild b -
Granados [33] 2016 ND ND ND ND ND unknown a ND
Hoshino [34] 2017 80's F PS + ND significant d -
Ganesan [35] 2018 13 M PS + 23 trivial a -
Wei [36] 2020 8m M PS + 4.4m/sec* ND a -
Wang [37] 2020 7 M PS + 1.7m/sec* mod.-severe a -
Miyake 2022 12m F PS + 14 trivial a -
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Abbreviations: ASD, atrial septal defect; BAV, bicuspid aortic valve; NB, newborn; ND, not described; RV-PA PG, right ventricle-pulmonary artery systolic pressure gradient; PS, pulmonary stenosis; PTPV, percutaneous transluminar pulmonary valvuloplasty; QAV, quadricuspid aortic valve; QPV, quadricuspid pulmonary valve; VSD, ventricular septal defect; *, transvalvular flow velocity.

Three out of four adult patients with PR diagnosed using TTE had moderate or severe PR [26, 29, 34]. The majority of QPV cases diagnosed using TTE exhibited marked pulmonary artery dilatation. It is pointed out that a cross-sectional view of the pulmonary valve was detected because the pulmonary valve was displaced in or near the chest wall. In most children, the cross-sectional view of the pulmonary valve can be displayed, from which pulmonary leaflet number abnormalities and morphologic malformations can be distinguished [37]. Cross-sectional views of the pulmonary valve were obtained with the angulation of the transducer superiorly from the left parasternal window in an adult with tetralogy of Fallot and with the high-parasternal long-axis view in a neonate with pulmonary stenosis [38]. In our 12-month-old infant with pulmonary valve stenosis, a high-parasternal short-axis view of the pulmonic valve was easily obtained because the pulmonary arterial trunk was dilated. The thymus gland allows visualization of a short-axis view of the pulmonary valve [33]. In the early stage of life, normal right ventricular dominance in term newborns contributes to a different anatomical position of the pulmonary annulus, making the high transsternal approach an excellent way to obtain a cross-sectional view of the pulmonary valve [33]. Granados et al. [33] stated that the high transsternal view should be included as a part of the routine protocol for echocardiographic examinations performed in this age group. Elsayed et al. [39] reported that three-dimensional TTE proved useful in conferring additional benefits over two-dimensional TTE in the assessment of the pulmonary valve in both adolescents and adults.

The prevalence of type-a QPVs in cases diagnosed using TTE was greater than that of type-b QPVs, whereas both type-b and type-c QPVs occupied the majority of those diagnosed at autopsy. We believe that the reasons for this prevalence are as follows: 1) The diagnosis of type-a QPV having four equal-sized cusps is easier than that of type-b QPV, which has a small supernumerary cusp. 2) PR occurs more easily in type-a QPVs than in type-b QPVs. In QAV, the sum of type-a and type-b was 64%, and both types had almost similar rates [19].

3.4.2. Diagnosis of QPV using CT and MRI

Cases of QPV diagnosed using CT scan or CMR were limited to adults. Except for five cases diagnosed using TTE, adult patients associated with other congenital heart diseases except for pulmonary stenosis were diagnosed using contrast CT scan or CMR after 2009 [13-16]. As with TTE, the frequency of valve morphology in cases diagnosed using CT scan or CMR was largely different from that in cases diagnosed at autopsy (Table 3) [13-16, 40-55]. It might be reasonable to actively look for QPV in patients who have marked aneurysmal dilatation of the main pulmonary artery, with or without an extension of the dilatation into pulmonary artery branches [54]. Table 4 shows differences in the frequency of QPV using diagnostic methods.

Table 3.

Patients diagnosed using CT or CMR.

Author Year Age Sex Pulmonary Stenosis Pulmonary Regurgitation Diagnostic Method Type of QPV Therapy
Czekajske-Chehab [13] 2009 70 F - mild-mod. CT c medication
Seo [14] 2009 17 M mod. - CT ND surgery
Gentille Lorente [15] 2009 65 F - mod. CMR a ND
Ascione [16] 2009 65 F - severe CMR c medication
Khan [40] 2012 41 M mod. - CT b PTPV
F- 23 F - - CT a -
Kwon [41] 2012 41 M - + CT a medication
Teis [42] 2013 38 F - mild CMR a -
Nollen [43] 2013 58 M - mild-mod. CMR a -
Rajiah [44] 2014 65 F ND + CT a ND
Shah [45] 2014 51 M - mild CMR b -
Dunay [46] 2015 20 M - mod. CT a -
Romero-Reyes [47] 2017 22 M ND mild CMR b -
Sawai [48] 2018 70 F mild ND CMR, CT b ND
Guijarro-Contreras [49] 2018 67 M mild mod. CMR a medication
Lee [50] 2018 58 F - - CT a -
Shimizu [51] 2018 62 M mod.* - CT a PTPV
Manuel [52] 2018 50 F mild mild CT a medication
Escalon [53] 2019 67 M - ND CT b ND
Ansari-Gilani [54] 2019 53 F mild mod. CT b ND
- - 78 F mod. mild CT b ND
- - 64 F mild mod. CT a ND
- - 68 M - mod. CT a ND
Guo [55] 2020 32 F mild mild-mod. CMR b -
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CMR, cardiac magnetic resonance; CT, computed tomography; mod., moderate; ND, not described; PTPV, percutaneous transluminar pulmonary valvuloplasty; *, pulmonary artery/right ventricle peak gradient of 82 mmHg.

Table 4.

Diagnostic methods and QPV types of Hurwitz and Roberts.

Diagnostic Methods Hurwitz and Roberts’s type
a b c d e f g -
Autopsy
Hurwitz’s Review (1973) 15 72 18 7 4 3 2 121
Excluded Hurwitz’ review 11 44 7 2 1 0 3 68
Donor heart 1 2 1 0 0 1 3 8
Surgery 3 2 1 0 0 0 1 7
Total number 30 120 27 9 5 4 9 204
% 14.7 58.8 13.2 4.4 2.5 2.0 4.1
TTE 8 6 0 1 0 0 0 15
CT and CMR 9 8 2 0 0 0 0 19
Total number 17 14 2 1 0 0 0 34
% 50.0 41.2 5.9 2.9 0 0 0 -
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Abbreviations: CT, computed tomography; CMR, cardiac magnetic resonance; TTE, transthoracic echocardiography.

3.5. Quadricuspid Semilunar Valve Having Other Cardiovascular Anomaly

3.5.1. QPV Having Other Congenital Heart Diseases

Simonds [10] reported semilunar valve anomalies in individuals with other congenital heart diseases as follows: QPV in 2 of 46, and QAV in 1 of 5. Davia [56] reported that 10 of 35 patients with QPV had other congenital heart diseases. Table 5 shows cases of QPV having other congenital heart diseases, excluding isolated QPV.

Table 5.

Patients excluding isolated quadricuspid pulmonary valve after Hurwitz's manuscript.

Author Year Age Sex CHD
Excluding Isolated QPV 		PS Type of QPV Diagnostic Method
Hurwitz [5] 1973 3d F ASD + ND Autopsy
14 M partial AVC - ND Autopsy
Davia [56] 1977 1d F VSD, PDA, BAV - ND Autopsy
4d M ASD, PDA + ND Autopsy
3m F complete AVC, PDA - ND Autopsy
2d F AS, PDA - ND Autopsy
4m M VSD, PDA - ND Autopsy
3m F ASD - ND Autopsy
6w M CoA, PDA - ND Autopsy
6d F MA, AA, ASD - ND Autopsy
45 M BAV (incomplete) - ND Autopsy
55 M BAV (AS), CoA - ND Autopsy
Rivett [57] 1979 84 F aberrant coronary artery - c Autopsy
Danger [70] 1982 46 M VSD - ND Surgery
Kashikie [71] 1991 38 F ASD - a Surgery
Attie [60] 1995 18 M QAV - a Autopsy
Hirooka [25] 2001 42 M BAV (anterior-posterior) - b TTE
Ricci [27] 2005 NB F TGA, ASD, PDA - g TTE
Yew [64] 2005 13m M AA, IAA (B) - c Autopsy
15hr M AA, IAA (B), APSD, PDA + ND Autopsy
Sommer [72] 2005 48 F AS - a Surgery
Hagspiel [73] 2005 17 M HCM, LEOPARD syndrome + ND Surgery
Gouveia [30] 2011 6d M QAV - ND TTE
Iosifescu [74] 2012 16 M VSD - b* Surgery
Aboitiz-Rivera [31] 2015 1m F ASD + a TTE
Hu [75] 2017 ND ND TOF + ND Surgery
Karliova [76] 2018 13 M AS - a Surgery
Wei [36] 2019 8m M PFO, coronary fistula + a TTE
Bayle [77] 2020 4m M IAA (B), HLHC, VSD, hypoplastic BAV, AAORCA - b Surgery
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Abbreviations: AA, aortic atresia; AAORCA, anomalous aortic origin of the right coronary artery; APSD, aortopulmonary septal defect; AS, aortic stenosis; ASD, atrial septal defect; AVC, atrioventricular canal; BAV, bicuspid aortic valve; CHD, congenital heart defect; CoA, coarctation of the aorta; HCM, hypertrophic cardiomyopathy: HLHC, hypoplastic left heart complex; IAA, interruption of the aortic arch; MA, mitral atresia; ND, not described; PFO, patent foramen ovale; PDA, patent ductus arteriosus; PS, pulmonary stenosis; QAV, quadricuspid aortic valve; TGA, transposition of the great arteries; TTE, transthoracic echocardiography; VSD, ventricular septal defect; *, sparrow nest type.

3.5.2. Coexistence of the Semilunar Valve with Valve Number Abnormality

Literature has reported on three cases with both QPV and QAV [14, 30, 60], six with both QPV and bicuspid aortic valve [2, 25, 56, 77], and two with both QAV and bicuspid pulmonary valve [78, 79]. These three coexistences are extremely rare combinations.

3.5.3. Coronary Artery Abnormality of Quadricuspid Semilunar Valve

Only 1 of 57 cases with QPV diagnosed after 1973 presented with a coronary arterial abnormality [77]. Coronary ostium malformation of QAV was 8.8% [80]; 10.2% of QAV had a coronary malformation [18]. By contrast, the frequency of a clinically significant abnormality of the coronary ostia was only 2% [19]. Wei et al. [36] reported a case with QPV with severe pulmonary stenosis having coronary–pulmonary microfistulae.

3.5.4. Conotruncal Anomaly and Quadricuspid Semilunar Valve

Five cases with QPV had a conotruncal anomaly. Two neonates of aortic atresia with interrupted aortic arch were diagnosed as QPV at autopsy [64], and an infant with interrupted aortic arch complex was diagnosed as QPV at the time of Ross operation [77]. 1 (0.63%) of 150 cases with tetralogy of Fallot had QPV, which could not be diagnosed by both CT and TTE [75]. 1 case each for QPV and QAV had transposition of the great arteries [27, 81]. Another 2 cases with QAV had a conotruncal abnormality. 1 of 7 cases with pulmonary atresia, VSD, and major aortopulmonary collateral artery had QAV [82]. 1 of 15 cases with tetralogy of Fallot and absent pulmonary valve was associated with QAV [83].

3.6. Quadricuspid Semilunar Valve Function

3.6.1. Semilunar Valve Regurgitation

The rates of valve regurgitation at autopsy were 4% in QPV and 44% in QAV [5]. The frequency of QPV regurgitation in cases diagnosed using TTE, CT, and CMR was higher than in cases diagnosed at autopsy. Cases with moderate or severe QPV regurgitation were as follows: type-a 7/21 (33%) and type-b 2/11 (18%). Type-a QPV can produce regurgitation more easily than type-b. Enoch [84] reported endocardial fibrosis in two older patients, which may have been a sign of significant regurgitation, directly under the additional leaflet of the type-b QPV. However, a microscopic examination of type-b QPV by Solewski et al., in an autopsy of the 26-year-old man, did not reveal any build-up characteristics, thus suggesting a risk of premature valve failure [23].

In 50 cases with QAV, Tsang et al. [19] reported no QAV regurgitation in 10%, trivial–mild in 52%, mild–moderate in 12%, and more than moderate in 26%. The frequency of severe regurgitation increases rapidly after 40 years [85]. In a review conducted by Tutarel, the frequency rates of QAV regurgitation were 72.5% for type-a and 62.8% for type-b [18]. Furthermore, an association between QAV morphology and the severity of QAV regurgitation was not observed in 50 patients [19]. It is unclear whether these specific subtypes of QAV predispose patients to more severe QAV regurgitation or whether this is simply a reflection of the predominance of type-a and type-b in patients with QAV [19].

3.6.2. Semilunar Valve Stenosis

Hurwitz et al. [5] reported QPV stenosis in 2 of 158 cases diagnosed at autopsy. Out of 34 cases with QPV diagnosed clinically, 17 had pulmonary stenosis: 9 (60%) of 15 cases with QPV were diagnosed using TTE, and 8 (42%) of 19 cases were diagnosed using contrast CT or CMR. 8 (50%) of 16 cases with QPV diagnosed using contrast CT or CMR exhibited isolated pulmonary stenosis, and 17 (61%) of 28 cases with QPV having other congenital heart diseases were men. Hurwitz et al. [5] reported QAV stenosis in one of 11 cases diagnosed at autopsy. Tsang et al. [19] reported stenotic QAV of 8% at a mean age of 43.5 years.

3.7. Therapeutic Interventions for Quadricuspid Pulmonary Valve

Hurwitz et al. [5] demonstrated that QPV typically persists in normal function throughout the lifetime. However, pulmonary valve replacement [70, 73, 86, 87] or pulmonary valvuloplasty [71, 74] for QPV regurgitation have been reported. The Ross surgery for the congenital anomaly of the pulmonic valve is unfavorable [62]. Sommer et al. [72] performed the first study reporting on the Ross surgery for QPV. Immediately after surgery, TTE did not detect regurgitation of the new aortic valve. However, second-degree valve regurgitation was detected using echocardiography 4 years later. Two patients with QPV, who had severe valvular regurgitation due to aging, were reported. A 13-year-old case (type-a) had aortic valve plasty 4 years later [76], and a 32-year-old case (type-c) underwent a David surgery 16 years later [88]. Karliova et al. [76] concluded that congenital anomaly in the pulmonary valve should lead to careful decision making before using it as an autograft in the aortic position, but should not preclude a Ross operation per se. Liebrich et al. [88] concluded that up-to-date knowledge of aortic valve reconstruction may be applied to increasing challenging anatomy, such as a non-trileaflet pulmonary autograft. In a 4-month infant with QPV (type-b) for which the Ross–Konno procedure was performed, mild AR occurred 2 years later [77]. QPV, found unexpectedly at the time of operation, can be used as an autograft in the Ross operation, particularly in neonates and infants in whom no good alternatives for aortic valve replacement exist [77]. A complete evaluation of the morphology of both semilunar valves in patients with coronary ostial malformations is advised [78]. In a letter to the editor, Gennari et al. [89] reported a donor case with QPV.

Since 1999, four patients with stenotic QPV have undergone a successful percutaneous transluminal pulmonary valvuloplasty [36, 40, 51, 90]. By contrast, transcatheter aortic valve implantation for stenotic QAV was performed in 2011 for the first time [91].

3.8. Infective Endocarditis in Quadricuspid Semilunar Valve

Simonds [10] reported the frequency of infectious endocarditis in numeral semilunar valvular abnormalities at autopsy: QAV 1/5 (20%) and QPV 0/44. To the best of our knowledge, only 2 cases of QPV with infective endocarditis were reported. 1 patient with a ventricular septal defect and no stenotic QPV was detected at autopsy [70]. Additionally, 1 of 13 patients with congenital heart disease and with right-sided infectious endocarditis revealed an isolated QPV [92]. Sharif et al. [17] reported that the prevalence of infectious endocarditis in QAV was 9% in patients followed up via TTE. QPV was less likely to be associated with infectious endocarditis than was QAV.

CONCLUSION

The prevalence of a QPV diagnosis ranges from 0.02% to 0.41% at autopsy. QPV was initially diagnosed using TTE after 2000 and with contrast CT or CMR after 2009. Diagnosis of QPV using TTE is very difficult because of the anatomical disposition of the valve with respect to the thoracic wall. Using TTE, we could obtain the cross-sectional view of the pulmonary valve in an infant with a stenotic QPV. Patients with QPV have a higher frequency of congenital heart disease and have a lower frequency of coronary abnormality and infective endocarditis when compared to patients with QAV. We consider that more cases of type-b QPV are diagnosed at autopsy because type-a QPV is more likely to cause PR than type-b QPV. Ross surgery for the congenital anomaly of the pulmonic valve as QPV is unfavorable. However, QPVs have been used as autografts in certain patients.

ACKNOWLEDGEMENTS

The authors would like to thank Enago (www.enago.jp) for the English language review.

LIST OF ABBREVIATIONS

AR

Aortic Regurgitation

CMR

Cardiac Magnetic Resonance Imaging

CT

Computed Tomography

PR

Pulmonary Regurgitation

QAV

Quadricuspid Aortic Valve

QPV

Quadricuspid Pulmonary Valve

TTE

Transthoracic Echocardiography

ETHICS APPROVAL AND CONSENT TO PARTICIPATE

The ethics review committee of Kindai University Nara Hospital granted permission to conduct the study. Regarding case presentation, we explained to the mother of the girl, obtained informed consent, and recorded it in the electronic medical record of Kindai University Nara Hospital. Our case presentation does not include any therapy, including an intervention. No reference number has been allotted to this manuscript.

HUMAN AND ANIMAL RIGHTS

No animals were used that are the basis of this study. The procedures in this study were conducted in accordance with the ethical standards of the committee responsible for human experimentation and following the Helsinki Declaration

CONSENT FOR PUBLICATION

Consent was obtained from the mother of the child.

STANDARDS OF REPORTING

CARE guidelines were followed in this study.

AVAILABILITY OF DATA AND MATERIALS

Not applicable.

FUNDING

None.

CONFLICT OF INTEREST

The authors declare no conflict of interest, financial or otherwise.

SUPPLEMENTARY MATERIAL

CARE checklist is available on the publisher’s website along with the published article.

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