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• Natural History of Bicuspid Aortic Valve without Stenosis
  Jerome Liebman
  Published on: 9 January 2022
• Aortic changes with bicuspid aortic valve
  John P Veinot
  Published on: 9 January 2022

• Published on: 9 January 2022

  Natural History of Bicuspid Aortic Valve without Stenosis

  • Jerome Liebman, Professor of Pediatrics (Cardiology)

  Dear Editor:

  The excellent review by Ward on the clinical significance of the bicuspid aortic valve (Heart 2000;83:81-85) is of great interest and value. The extensive reference list as well put a good bit of the pertinent bicuspid valve literature in one place. The paper adds greatly to our knowledge.

  However, without critiquing every issue in the paper, there are two areas in particular I would...

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  Dear Editor:

  The excellent review by Ward on the clinical significance of the bicuspid aortic valve (Heart 2000;83:81-85) is of great interest and value. The extensive reference list as well put a good bit of the pertinent bicuspid valve literature in one place. The paper adds greatly to our knowledge.

  However, without critiquing every issue in the paper, there are two areas in particular I would like to discuss. As a pediatric cardiologist who has been in one division for 40 years, there is considerable natural history which has been learned, although, perhaps in order to make my statements I would really have to work in one place for 70 years.

  I have seen a huge number of patients with a true bicuspid aortic valve and I have never seen a patient with a dissecting aneurysm. As Dr. Ward states, the reason for the reported high incidence of aortic dissection in the presence of an aortic bicuspid aortic valve is unclear, particularly since the author states that the bicuspid valve is usually normally functioning. Although the post bicuspid valve dilatation of the ascending aorta in the presence of systemic hypertension certainly makes the environment possible, Dr. Ward’s paper makes no mention as to whether he has personally seen a patient with dissection. The estimated incidence of 5% of patients with bicuspid valve having dissection of the aorta seems very much too high.

  The second issue refers to whether or not the patient born with bicuspid aortic valve and no stenosis is expected to progress to reach real aortic stenosis as the patient ages in the absence of infective endocarditis. The accepted knowledge over the years has been that the elderly with calcific aortic stenosis began with a bicuspid aortic valve, including the valve with no stenosis to begin with. Evidence for such has never been documented and perhaps cannot, unless a 100 year prospective study is planned and funded. The implication of Dr. Ward’s article is not quite that, for he doesn’t really discuss the patient with bicuspid aortic valve and no stenosis. He only states, appropriately, that patients with mild aortic stenosis are expected to progress in adulthood, even patients with echocardiographic mean gradient of less than 25 mmHg. However, this does not answer the question as to whether the patients with true bicuspid aortic valve and no stenosis are expected to progress. In order to discuss this issue, one needs the physical examination. Is there a difference in natural history between the patient with true bicuspid aortic valve with no stenosis and a true bicuspid aortic valve with mild stenosis? In the early two decades of my 40 years, the diagnosis of bicuspid aortic valve without stenosis was made by recognizing an aortic ejection click with no heart murmur. I accept that occasionally an error can be made in that the presumed aortic ejection click is in actual fact an unusually loud tricuspid valve component of a normally split first sound. But in the recent two decades, with echocardiography, confirmation has been readily available. The patient with a bicuspid aortic valve and mild aortic stenosis is diagnosed by having the aortic ejection click plus a normally split second sound and an aortic stenosis murmur at the second right interspace that is significant but usually stops clearly before aortic valve closure. The latter will usually have a mean gradient of less than 25 mmHg in the echo, just as will obviously the patient with no stenosis. Dr. Ward because of the nature of his review does not separate these groups. In my 40 years, I have seen many patients with mild stenosis progress, but I have seen none with no stenosis progress. In addition, the subgroup of patients with coarctation of the aorta has a very high incidence of bicuspid valve without stenosis. I have never seen one of these develop aortic stenosis either.

  I realize that 40 years is not 70 years, but since it is unlikely to be able to find all these patients with no stenosis for an associate to follow for 30 or 40 more years answering the question for certain at this time appears unlikely. However, it would be in cumbent upon all of us to modify what we teach our students and what we advise our families. As far as we know, the bicuspid aortic valve without stenosis remains without stenosis.

  Sincerely,

  Jerome Liebman MD
  Division of Pediatric Cardiology
  Professor of Pediatrics
  Rainbow Babies & Children’s Hospital
  Case Western Reserve University

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  Conflict of Interest:
  None declared.

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• Published on: 9 January 2022

  Aortic changes with bicuspid aortic valve

  • John P Veinot, Pathologist

  To the Editor;

  We read with interest the review of congenitally bicuspid aortic valve by Ward.[1] The complications of congenitally bicuspid aortic valve, including aortic stenosis, aortic regurgitation and aortic dissection, are well documented in this review.

  It is interesting to note the association of congenitally bicuspid aortic valve with aortic medial disease (cystic medial necrosis), coarct...

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  To the Editor;

  We read with interest the review of congenitally bicuspid aortic valve by Ward.[1] The complications of congenitally bicuspid aortic valve, including aortic stenosis, aortic regurgitation and aortic dissection, are well documented in this review.

  It is interesting to note the association of congenitally bicuspid aortic valve with aortic medial disease (cystic medial necrosis), coarctation and congenital aortic arch abnormalities.[2][3] Some investigators have postulated a common pathogenesis of aortic valve and aorta diseases with evidence that the aortic valve and portions of the aorta may share a common embryonic origin.[4]

  We recently studied the degree of aortic medial degenerative changes associated with congenitally bicuspid and tricuspid aortic valves in patients with aortic stenosis, who died shortly after aortic valve replacement.[5] Patients with aortic dissection were excluded. The association between bicuspid aortic valve and dissection, and thus with aortic medial degenerative changes (cystic medial necrosis), is usually derived from case reports or series of patients dying from or being operated upon for aortic dissection. We thought that the data were probably biased to reflect patients with severe medial disease, and thus we decided to re-evaluate these changes in patients without dissection.

  To our surprise, computer aided morphometry demonstrated that the aortas of congenitally bicuspid aortic valve patients had less elastic tissue than those patients with tricuspid aortic valves. Routine histological evaluation by light microscopy of the same aortic sections, even with the use of elastic stains, did not demonstrate a significant difference.

  These independent findings also support the echocardiographic observations of Pachulski and colleagues, who demonstrated an association of aortic dilatation with bicuspid aortic valve in the absence of hemodynamic valvular abnormalities.[6]

  We agree with the conclusions of Ward.[1] Patients with congenitally bicuspid aortic valve may be prone to aortic degeneration with age and should be closely monitored. Alterations in the aortic media may be part of the disease spectrum of congenitally bicuspid aortic valve.

  John P. Veinot M.D., FRCPC
  Assoc. Professor Pathology
  University of Ottawa
  University of Ottawa Heart Institute
  Ottawa Hospital
  Ottawa, Ontario, Canada

  REFERENCES

  1. Ward C. Clinical significance of the bicuspid aortic valve. Heart 2000;83:81-85.
  2. Roberts WC. The congenitally bicuspid aortic valve: a study of 85 autopsy cases. Am J Cardiol 1970;26:72-83.
  3. Larson EW, Edwards WD. Risk factors for aortic dissection: A necropsy study of 161 cases. Am J Cardiol 1984;53:849-855.
  4. Schievink WI, Mokri B. Familial aorto-cervicocephalic arterial dissections and congenitally bicuspid aortic valve. Stroke 1995;26:1935- 1940.
  5. Parai JL, Masters RG, Walley VM, Stinson WA, Veinot JP. Aortic medial changes associated with bicuspid aortic valve: Myth or reality ? Can J Cardiol 1999;15:1233-1238.
  6. Pachulski RT, Weinberg AL, Chan KL. Aortic aneurysm in patients with functionally normal or minimally stenotic bicuspid aortic valve. Am J Cardiol 1991;67:781-782.

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  Conflict of Interest:
  None declared.

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