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Response to: ‘Congenital tricuspid valve disease can masquerade as primary idiopathic TR’ by Rajpal
  1. Erin Amanda Fender,
  2. Chad J Zack,
  3. Rick A Nishimura
  1. Mayo Clinic, Rochester, Minnesota, USA
  1. Correspondence to Dr Rick A Nishimura, Mayo Clinic, Rochester MN 55905, USA; nishimura.rick{at}

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We received the letter by Dr Rajpal  addressing the spectrum of congenital tricuspid valve (TV) abnormalities and highlighting congenital tricuspid disease as an under-recognised cause of isolated tricuspid regurgitation (TR) in adults. We agree wholeheartedly that there is a wide spectrum of congenital tricuspid valve disease, ranging from Ebstein’s anomaly to tricuspid valve dysplasia which encompasses both primary leaflet malformations and chordal abnormalities. While Ebstein’s anomaly is a well-recognised cause of primary tricuspid regurgitation, isolated tricuspid valve dysplasia is less frequently encountered in adults without other associated congenital abnormalities.1 The rarity of isolated TV dysplasia, coupled with the technical difficulties of imaging the tricuspid valve, makes this a challenging diagnosis. In our own practice we have encountered several patients with severe isolated TR who were referred to surgery and were found intraoperatively to have previously unrecognised leaflet dysplasia. A low index of suspicion must be maintained in relatively young adults presenting with isolated tricuspid regurgitation in the absence of other potential causes, particularly as surgery has proven efficacy in treating these patients.2 3



  • Competing interests None declared.

  • Provenance and peer review Not commissioned; internally peer reviewed.