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 Table of Contents  
Year : 2018  |  Volume : 2  |  Issue : 2  |  Page : 112-114

Quadricuspid pulmonary valve

1 Institute of Cardiology, Madras Medical College, Chennai, Tamil Nadu, India
2 Department of Cardiology, Stanley Medical College, Chennai, Tamil Nadu, India

Date of Web Publication6-Sep-2018

Correspondence Address:
Dr. Gnanavelu Ganesan
Rajiv Gandhi Government General Hospital, Chennai - 600 003, Tamil Nadu,
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_70_17

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Quadricuspid pulmonary valve (QPV) is a rare congenital cardiac malformation which is notable for its asymptomatic behavior. With only a few hundred cases reported in literature, its clinical significance is not exactly known, although many authors have placed its implications on the Ross procedure. We report a case of QPV which was detected on routine cardiac screening.

Keywords: Congenital cardiac valvular abnormalities, quadricuspid pulmonary valve, Ross procedure

How to cite this article:
Ganesan G, Ramamoorthy H, Sangareddi V. Quadricuspid pulmonary valve . J Indian Acad Echocardiogr Cardiovasc Imaging 2018;2:112-4

How to cite this URL:
Ganesan G, Ramamoorthy H, Sangareddi V. Quadricuspid pulmonary valve . J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2018 [cited 2019 May 23];2:112-4. Available from: http://www.jiaecho.org/text.asp?2018/2/2/112/240642

  Introduction Top

Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly, rarer than quadricuspid aortic valve. Usually this is detected on casual examination or during school health screening. We report a case of quadricuspid pulmonary valve with mild pulmonary stenosis.

  Clinical Presentation Top

An asymptomatic 13-year-old boy was referred from school health screening for further evaluation of an incidentally detected cardiac murmur. There was no history of preexisting cardiac disease. On examination, the vital signs were found to be normal. The results of clinical and cardiac evaluation are summarized in [Table 1].
Table 1: Clinical and cardiac evaluation

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  Discussion Top

Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly accounting for approximately 0.005%8211;0.05% of all congenital heart diseases in major autopsy series. It is usually detected incidentally during a routine cardiac testing and is not associated with any additional anomalies, although there are some reports of coexisting aortic valvular abnormalities. At present, the number of published cases of QPV is <400. The overall incidence is quoted to be at 1 in 4008211;1 in 20,000 autopsies.[1] The absence of significant symptoms and its strategic position in the thoracic cage makes it difficult to identify the abnormality during life and most case series report only an autopsy incidence, thus underestimating the actual incidence of QPV in general population. There is a frequent requirement of specialized techniques such as cardiac catheterization and computerized tomography for the diagnosis of QPV.

There are several case reports of QPV with additional cardiac abnormalities. The earliest published case series of QPV was by Enoch, who reported four cases of isolated QPV and one case of QPV with rheumatic mitral stenosis.[1] Two of these patients had pulmonary regurgitation. Kissin reported a case of QPV with pulmonary regurgitation as early as 1936 and later noted that the most frequent pattern of the abnormal valve was that of the interposition of a rudimentary accessory cusp in between three normal cusps. The other less common pattern was a combination of two larger and two smaller cusps.[2] The large case series by Davia et al. reported 47 cases of quadricuspid semilunar valves, of which 35 were QPV and the rest were quadricuspid aortic valves (QAVs), indicating a clearly higher incidence of QPV compared to QAV. In this series, only ten out of 35 patients were symptomatic due to the presence of additional congenital abnormalities.[3]

Olivares-Reyes et al. reported a case of congenital QPV with double valvular lesions and poststenotic dilatation of left pulmonary artery.[4] Czekajska-Chehab et al. reported a case of QPV complicated by aneurysm of the pulmonary trunk.[5] Hwang et al. reported two cases of quadricuspid semilunar valves, one of which was a symptomatic QAV and the other was an asymptomatic QPV, which was incidentally detected on autopsy.[6] Ricci et al. reported a case of QPV with transposition of great arteries.[7]
Figure 1: Chest X-ray - posteroanterior view

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Figure 2: Zoomed view of quadricuspid pulmonary valve

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Figure 3: Quadricuspid pulmonary valve in diastole and systole

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Figure 4: Modified long axis view of right ventricular outflow tract

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Figure 5: Doppler interrogation reveals peak gradient of 23 mmHg

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Figure 6: Modified long axis view of right ventricular outflow tract

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The present case of QPV was an isolated anomaly of the pulmonary valve where the patient was asymptomatic, and the abnormality was picked up on a routine echocardiogram. Usually, QPV does not cause stenosis, but in our case, there was mild valvular stenosis and only trivial regurgitation associated with poststenotic dilatation of pulmonary artery. This is a unique feature in our case.

Although several authors have reported that the usual long-term consequence of QPV is regurgitation, several others have argued that the condition has no clinical implication. The most important clinical implication of QPV is during the Ross procedure, and the importance of the diagnosis of a QPV has been stressed by several authors. Present evidence suggests that the QPV has unfavorable hemodynamics which makes it unacceptable for a Ross procedure. The ability of QPV to maintain normal hemodynamics is restricted to the pulmonary circulation, and this can lead to serious consequences in the systemic circulation. It has thus been stressed that the presence of a QPV should be diagnosed before planning a Ross procedure to avoid untoward hemodynamic consequences.[8]

  Conclusion Top

QPV is a rare, most often isolated congenital cardiac abnormality which has minimal, if any, clinical consequences. Its importance arises when the Ross operation is being contemplated, making it mandatory for early detection.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Enoch BA. Quadricuspid pulmonary valve. Br Heart J 1968;30:67-9.  Back to cited text no. 1
Kissin M. Pulmonary insufficiency with a supernumerary cusp in the pulmonary valve: Report of a case with review of the literature. Am Heart J 1936;12:206.  Back to cited text no. 2
Davia JE, Fenoglio JJ, DeCastro CM, McAllister HA Jr., Cheitlin MD. Quadricuspid semilunar valves. Chest 1977;72:186-9.  Back to cited text no. 3
Olivares-Reyes A, Molina-Bello E, Espinola-Zavaleta N. Congenital quadricuspid pulmonary valve in an adult patient with double valvular lesions and poststenotic dilatation of the trunk and the left branch of the pulmonary artery: A case presentation and review of the literature. Congenit Heart Dis 2012;7:E103-8.  Back to cited text no. 4
Czekajska-Chehab E, Uhlig S, Staśkiewicz G, Widomska-Czekajska T, Drop A. Quadricuspid pulmonary valve complicated with aneurysm of pulmonary trunk diagnosed with ECG-gated computed tomography. Folia Morphol (Warsz) 2009;68:290-3.  Back to cited text no. 5
Hwang DM, Feindel CM, Butany JW. Quadricuspid semilunar valves: Report of two cases. Can J Cardiol 2003;19:938-42.  Back to cited text no. 6
Ricci M, Cohen GA, Kocyildirim E, Khambadkone S, Elliott MJ. Transposition of the great arteries and quadricuspid pulmonary valve: An unusual combination. Ann Thorac Surg 2005;79:1428-30.  Back to cited text no. 7
Berdajs D, Lajos P, Zünd G, Turina M. The quadricuspid pulmonary valve: Its importance in the Ross procedure. J Thorac Cardiovasc Surg 2003;125:198-9.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

  [Table 1]


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