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CC BY 4.0 · Aorta (Stamford) 2016; 04(05): 178-180
DOI: 10.12945/j.aorta.2016.16.033
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

David-V Procedure in a Patient with Aortic Dilation and Competent Quadricuspid Aortic Valve: Are Genetics to Blame?

Katherine R. Hebeler
1   Department of Cardiothoracic Surgery, The Heart Hospital Baylor Plano, Plano, Texas, USA
,
John J. Squiers
1   Department of Cardiothoracic Surgery, The Heart Hospital Baylor Plano, Plano, Texas, USA
,
Heike Baumgarten
1   Department of Cardiothoracic Surgery, The Heart Hospital Baylor Plano, Plano, Texas, USA
,
J. Michael DiMaio
1   Department of Cardiothoracic Surgery, The Heart Hospital Baylor Plano, Plano, Texas, USA
,
William T. Brinkman
1   Department of Cardiothoracic Surgery, The Heart Hospital Baylor Plano, Plano, Texas, USA
› Author Affiliations
Further Information

Publication History

03 June 2016

24 October 2016

Publication Date:
24 September 2018 (online)

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Abstract

Quadricuspid aortic valves (QAVs) are extremely rare. In this case study, we report a David-V valve-sparing aortic root replacement with reimplantation of a native QAV in a patient with aortic dilation, normal valve function, and a family history of aortic dissection. Microscopic pathological examination of the excised section of the aorta revealed scattered small foci of cystic medical degeneration throughout. A genetic predisposition for aortic dilation may be present in patients with QAV, even in the setting of a competent valve. Regular screening for aortic dilation in patients with known QAV should therefore be considered.

Key Words

Quadricuspid aortic valve - Aortic dilation - Genetics
 

  • References

  • 1 Babington BG. Case of cyanosis dependent patent ductus arteriosus. London Medical Gazette 1847; 4: 822-823
    PubMedGoogle Scholar
  • 2 Hurwitz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973; 31: 623-626 . DOI: 10.1016/0002-9149(73)90332-9
    CrossrefPubMedGoogle Scholar
  • 3 Uspenskiy VE, Osadchii AM, Gordeev M. Quadricuspid aortic valve combined with moderate ascending aortic dilatation: a report of four cases. AORTA (Stamford) 2015; 3: 187-190 . DOI: 10.12945/j.aorta.2015.15.004
    Article in Thieme ConnectPubMedGoogle Scholar
  • 4 Jagannath AD, Johri AM, Liberthson R, Larobina M, Passeri J, Tighe D. , et al. Quadricuspid aortic valve: a report of 12 cases and a review of the literature. Echocardiography 2011; 28: 1035-1040 . DOI: 10.1111/j.1540-8175.2011.01477.x
    CrossrefPubMedGoogle Scholar
  • 5 Idrees IJ, Roselli EE, Arafat A, Johnston DR, Svensson LG, Sabik 3rd JF. , et al. Outcomes after repair or replacement of dysfunctional quadricuspid aortic valve. J Thorac Cardiovasc Surg 2015; 150: 79-82 . DOI: 10.1016/j.jtcvs.2015.03.019
    CrossrefPubMedGoogle Scholar
  • 6 Tsang MYC, Abudiab MM, Ammash NM, Nagvi TZ, Edwards WD, Nkomo VT. , et al. Quadricuspid aortic valve: Characteristics, associated structural cardiovascular abnormalities, and clinical outcomes. Circulation 2016; 133: 312-319 . DOI: 10.1161/CIRCULATIONAHA.115.017743
    CrossrefPubMedGoogle Scholar
  • 7 Tsukioka K, Nobara H, Takano T, Wada Y, Amano J. Quadricuspid aortic valve with ascending aortic aneurysm: a case report and histopathological investigation. Ann Thorac Cardiovasc Surg 2011; 17: 418-421 . DOI: 10.5761/atcs.cr.10.01567
    CrossrefPubMedGoogle Scholar
  • 8 Marsalese DL, Moodie DS, Lytle BW, Cosgrove DM, Ratliff NB, Goormastic M. , et al. Cystic medial necrosis of the aorta in patients without Marfan’s syndrome: Surgical outcome and long-term follow-up. J Am Coll Cardiol 1990; 16: 68-73 . DOI: 10.1016/0735-1097(90)90458-2
    CrossrefPubMedGoogle Scholar