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Pediatric and Developmental Pathology

, Volume 3, Issue 4, pp 353–366 | Cite as

Absent Pulmonary Valve with Tricuspid Atresia or Severe Tricuspid Stenosis: Report of Three Cases and Review of the Literature

  • Silvio Litovsky
  • Michael Choy
  • Jeanny Park
  • Mark Parrish
  • Brenda Waters
  • Mitsugi Nagashima
  • Richard Van Praagh
  • Stella Van Praagh
Article

Abstract

Absence of the pulmonary valve occurs usually in association with tetralogy of Fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic.

Key words: absent pulmonary valve with tricuspid stenosis or atresia, asymmetric hypertrophy of the ventricular septum, right ventricular dysplasia with fibrosis and sinusoids 

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Copyright information

© Society for Pediatric Pathology 2000

Authors and Affiliations

  • Silvio Litovsky
    • 1
  • Michael Choy
    • 2
  • Jeanny Park
    • 2
  • Mark Parrish
    • 2
  • Brenda Waters
    • 3
  • Mitsugi Nagashima
    • 4
  • Richard Van Praagh
    • 1
  • Stella Van Praagh
    • 1
  1. 1.Department of Pathology, Children's Hospital and Harvard Medical SchoolBostonUSA
  2. 2.Division of Pediatric Cardiology, UC Davis Medical CenterSacramentoUSA
  3. 3.Department of Pathology, Fletcher Allen Health Care and University of VermontBurlingtonUSA
  4. 4.Department of Cardiovascular Surgery, Children's Hospital and Harvard Medical SchoolBostonUSA

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