Annals of Pediatric Cardiology
About us | Current Issue | Archives | Ahead of Print | Instructions | Submission | Subscribe | Advertise | Contact | Login 


    Advanced search

    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Report
    Article Figures
    Article Tables

 Article Access Statistics
    PDF Downloaded131    
    Comments [Add]    

Recommend this journal


Table of Contents   
Year : 2019  |  Volume : 12  |  Issue : 1  |  Page : 56-59
Double whammy: A case of bilateral bicuspid arterial valves in transposition, with a review of the literature

1 Department of Pediatrics, Division of Cardiology, The University of Arkansas for Medical Sciences, Little Rock, AR, USA
2 Cardiovascular Research Center, Institute of Genetic Medicine, Newcastle University, Newcastle-Upon-Tyne, United Kingdom

Click here for correspondence address and email

Date of Web Publication14-Jan-2019


Bicuspidity of both the semilunar valves is rarely reported. We report the first ever case of bilateral bicuspid semilunar valves in a case of transposition.

Keywords: Bicuspid aortic valve, bicuspid pulmonary valve, transposition

How to cite this article:
Shivaram P, Shah K, Dossey A, Reemtsen B, Anderson RH. Double whammy: A case of bilateral bicuspid arterial valves in transposition, with a review of the literature. Ann Pediatr Card 2019;12:56-9

How to cite this URL:
Shivaram P, Shah K, Dossey A, Reemtsen B, Anderson RH. Double whammy: A case of bilateral bicuspid arterial valves in transposition, with a review of the literature. Ann Pediatr Card [serial online] 2019 [cited 2022 Nov 27];12:56-9. Available from:

   Introduction Top

The bicuspid aortic valve is a common congenital anomaly, having an incidence of from 1% to 2% in the general population.[1] Bicuspid pulmonary valves, in contrast, are very rare, having an incidence of only 0.1%.[2] The bicuspid aortic valve is commonly seen in association with left-sided heart disease but has rarely been reported in the setting of transposition,[3] defined on the basis of concordant atrioventricular and discordant ventriculoarterial connections. The presence of both arterial valves with two leaflets is exceedingly rare. To our knowledge, such a finding has been reported only in postmortem studies and in few isolated case reports in the setting of concordant ventriculoarterial connections. We report here a neonate with bicuspidity of both arterial valves in the setting of transposition.

   Case Report Top

A male neonate was transferred on prostaglandin E1 infusion on the 1st day of life to our facility after he failed the critical congenital heart screen. He had been born at term and weighed 2.68 kg. Physical examination revealed a patient small for gestational age, with mild dysmorphism. There was posterior sloping of forehead, a mildly depressed nasal bridge, and low-set ears. Cardiac examination revealed normal rate and rhythm. A systolic ejection murmur graded at 2 from 6 was heard at the cardiac base. All pulses were palpable and strong. Cross-sectional echocardiography revealed usual atrial arrangement with concordant atrioventricular connections, right-handed ventricular topology, and discordant ventriculoarterial connections, with the aorta positioned anterior and rightward relative to the pulmonary trunk. He was found to have both aortic and pulmonary valves with two leaflets. The pulmonary valve annulus, measured at the level of the virtual basal plane, had a diameter of 12 mm, with a Z-score of 2.32. The comparable value for the aortic valve was 9 mm, equating to a Z-score of 2.83 (Boston Z-score) [[Figure 1] and Video 1]. The pulmonary valve was mildly thickened and domed in systole, permitting mild regurgitation but producing no stenosis. The zone of apposition between the two leaflets of the aortic valve ran from front to back, with one coronary artery arising from each of the valvar sinuses. The left coronary artery arose from the left-sided sinus, while the right coronary artery arose from the right-sided sinus. The left coronary artery then branched into the circumflex and anterior interventricular arteries. He had a tiny perimembranous ventricular septal defect, an oval foramen of moderate size, and a large persistently patent arterial duct. He underwent emergent balloon atrial septostomy and subsequently, on the 13th day of life, underwent an arterial switch operation, with ligation of the duct and closure of atrial septal defect on day 13 of life. Postoperatively, he has developed moderate insufficiency of the new aortic valve, with dilation of the sinuses and moderate dilation of the left ventricle [Figure 2]. The bicuspid nature of both valves was confirmed intraoperatively. The zones of apposition of the valves, however, were aligned, making it possible to transfer the coronary arteries to the adjacent pulmonary valvar sinuses during the arterial switch operation.
Figure 1: Bicuspid aortic and pulmonary valve: Aortic is anterior and to the right of pulmonary trunk. True bicuspidity of both the semilunar valves noted

Click here to view
Figure 2: Postoperatively, bicuspid dilated neoaortic valve

Click here to view

   Discussion Top

In the early days of the arterial switch operation, a bicuspid pulmonary valve was considered a potential impediment to successful repair. With advancement in operative techniques, the switch procedure has increasingly performed in patients with bicuspid pulmonary valves. Although the bicuspid nature of both the aortic valve and the pulmonary valve have previously been described in the setting of transposition, we are unaware of any previous description of both valves being bicuspid in the same patient with transposition.

Development of arterial valves and Bicuspid semilunar valves

During the 7th week of gestation in man, the arterial valves develop in the middle component of the developing outflow tract. It is the appearance of the intercalated cushions that herald the appearance of the developing arterial roots. The central parts of the major outflow cushions fuse together during this period of development, whereas the parietal parts remain unfused. It is then the interdigitation of the unfused parietal parts with the intercalated cushions that form the primordiums of the two roots. The leaflets of the valves then excavate from the distal ends of the cushions, which become populated by cells derived from the endothelium and neural crest.[4],[5] Valves with two leaflets can be the consequence either of the fusion between developing cushions[4] and hypoplasia of the intercalated cushions.[5] It is the latter mechanism that would produce leaflets having aligned zones of apposition. Various mechanisms have been proposed to account for bicuspid valves at a molecular level, such as deficiency of C-type natriuretic peptide receptor 2,[6] loss of function mutation in GATA4 gene,[7] and NOTCH1, GATA5, and SMAD6 gene.[8] Linkage analysis of the genome-wide marker has demonstrated a linkage of bicuspid aortic valve to loci on chromosome 18, 5, and 13q.[9] Bilateral bicuspid arterial valves have been noted in association with trisomy 18 and trisomy 13.[10] Our patient, however, had normal chromosomal microarray.

Review of literature

[Table 1] summarizes the current literature on the existence of bicuspidity of both arterial valves. Our extensive search of literature shows this finding to be reported with an incidence of 0.01%–0.03%. Most of the cases have been encountered at postmortem or in anatomical specimens. In the few cases reported preoperatively, all patients had concordant ventriculoarterial connections. Surprisingly, however, two such neonates with concordant ventriculoarterial connections have parallel arterial trunks, as opposed to spiraling trunks. The arrangement with parallel trunks is often described as “anatomically corrected transposition.”[19]
Table 1: Review of literature of bilateral bicuspid semilunar valve

Click here to view

Surgical outcomes with bicuspid valves

Bicuspid pulmonary valves are not uncommon in the setting of transposition, being reported at 4% and 7% in 2 large series.[11],[12] Such bicuspid valves are known to cause complications postoperatively with the need for replacement of the new aortic root.[10] Others have shown, nonetheless, that in the short to mid-term, the arterial switch procedure can be performed with low morbidity and mortality in the setting of well-performing bicuspid neoaortic valves. To the best of our knowledge, there are no long-term data currently available. In our patient, in the short term, there is some dilation of the neoaortic sinuses, with mild-to-moderate valvar insufficiency. The presence of the bicuspid aortic valve does create potential problems in describing the sinusal origin of the coronary arteries, which is the basis of the Leiden classification. In our patient, nonetheless, the sinuses were located in the right- and left-sided locations, with each sinus giving rise to the appropriate coronary artery.

   Conclusion Top

We believe that we have reported the first case of bilateral bicuspidity of the arterial valves in a patient with transposition successfully undergoing the arterial switch operation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Yener N, Oktar GL, Erer D, Yardimci MM, Yener A. Bicuspid aortic valve. Ann Thorac Cardiovasc Surg 2002;8:264-7.  Back to cited text no. 1
Manivarmane R, Taylor R, Khattar R. Case of isolated bicuspid pulmonary valve. Echo Res Pract 2018;5:K13-8. Available from: [Last accessed on 2018 Feb 05].  Back to cited text no. 2
Niaz T, Poterucha JT, Johnson JN, Craviari C, Nienaber T, Palfreeman J, et al. Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects. Congenit Heart Dis 2017;12:261-9.  Back to cited text no. 3
Sans-Coma V, Cardo M, Thiene G, Fernández B, Arqué JM, Durán AC, et al. Bicuspid aortic and pulmonary valves in the syrian hamster. Int J Cardiol 1992;34:249-54.  Back to cited text no. 4
Tretter JT, Steffensen T, Westover T, Anderson RH, Spicer DE. Developmental considerations with regard to so-called absence of the leaflets of the arterial valves. Cardiol Young 2017;27:302-11.  Back to cited text no. 5
Blaser MC, Wei K, Adams RLE, Zhou YQ, Caruso LL, Mirzaei Z, et al. Deficiency of natriuretic peptide receptor 2 promotes bicuspid aortic valves, aortic valve disease, left ventricular dysfunction, and ascending aortic dilatations in mice. Circ Res 2018;122:405-16.  Back to cited text no. 6
Li RG, Xu YJ, Wang J, Liu XY, Yuan F, Huang RT, et al. GATA4 loss-of-function mutation and the congenitally bicuspid aortic valve. Am J Cardiol 2018;121:469-74.  Back to cited text no. 7
Lin X, Liu X, Wang L, Jiang J, Sun Y, Zhu Q, et al. Targeted next-generation sequencing identified ADAMTS5 as novel genetic substrate in patients with bicuspid aortic valve. Int J Cardiol 2018;252:150-5.  Back to cited text no. 8
Giusti B, Sticchi E, De Cario R, Magi A, Nistri S, Pepe G, et al. Genetic bases of bicuspid aortic valve: The contribution of traditional and high-throughput sequencing approaches on research and diagnosis. Front Physiol 2017;8:612.  Back to cited text no. 9
Bobylev D, Breymann T, Boethig D, Ono M. Aortic root replacement in a patient with bicuspid pulmonary valve late after arterial switch operation. Thorac Cardiovasc Surg 2013;61:316-9.  Back to cited text no. 10
Angeli E, Gerelli S, Beyler C, Lamerain M, Rochas B, Bonnet D, et al. Bicuspid pulmonary valve in transposition of the great arteries: Impact on outcome. Eur J Cardiothorac Surg 2012;41:248-55.  Back to cited text no. 11
Khan SM, Sallehuddin AB, Al-Bulbul ZR, Al-Halees ZY. Neoaortic bicuspid valve in arterial switch operation: Mid-term follow-up. Ann Thorac Surg 2008;85:179-84.  Back to cited text no. 12
Bobylev D, Breymann T, Boethig D, Ono M. Aortic root replacement in a patient with bicuspid pulmonary valve late after arterial switch operation. Thorac Cardiovasc Surg 2013;61:316-9.  Back to cited text no. 13
Jashari R, Van Hoeck B, Goffin Y, Vanderkelen A. The incidence of congenital bicuspid or bileaflet and quadricuspid or quadrileaflet arterial valves in 3,861 donor hearts in the European Homograft Bank. J Heart Valve Dis 2009;18:337-44.  Back to cited text no. 14
Duran AC, Frescura C, Sans-Coma V, Angelini A, Basso C, Thiene G. Bicuspid aortic valves in hearts with other congenital heart disease. J Heart Valve Dis 1995;4:581-90.  Back to cited text no. 15
Kemaloğlu Öz T, Karadeniz FÖ, Gundlapalli H, Erer B, Sharma RK, Ahmed M, et al. Coexisting Bicuspid Aortic and Pulmonary Valves with Normally Related Great Vessels Diagnosed by Live/Real Time Three-Dimensional Transesophageal Echocardiography. Echocardiography 2014;31:218-21.  Back to cited text no. 16
Sughimoto K, Nakano K, Gomi A, Nakatani H, Nakamura Y, Sato A. Pulmonary artery aneurysm with ascending aortic aneurysm concomitant with bilateral bicuspid semilunar valves. Ann Thorac Surg 2006;82:2270-2.  Back to cited text no. 17
Lee ML, Chiu IS, Wu MH, Wang JK, Lue HC, Chaou WT, Tsao LY. Transarterial approach of the pulmonary artery in anatomically corrected malposition of the great arteries by manipulating a catheter inverted with balloon floating maneuver. Int J Cardiol 1998;67:1–7.  Back to cited text no. 18
Posada-Martínez EL, Arias-Godinez JA, Romero-Cárdenas Á, Ruiz Esparza-Dueñas ME, Fritche-Salazar JF, Rodríguez-Zanella H, et al. Coexisting bicuspid aortic and pulmonary valves diagnosed by 3D transthoracic echocardiography. Echocardiography 2018;35:423-4.  Back to cited text no. 19

Correspondence Address:
Dr. Pushpa Shivaram
Department of Pediatrics, Division of Cardiology, The University of Arkansas for Medical Sciences, 1 Children's Way, Mail Slot 512-3, Little Rock, AR 72202
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/apc.APC_88_18

Rights and Permissions


  [Figure 1], [Figure 2]

  [Table 1]