Key Features of Barlow’s Disease

Barlow’s Disease is a specific form of degenerative mitral valve disease characterized by myxomatous degeneration of the mitral valve, leading to mitral valve prolapse (MVP) and severe mitral regurgitation.
 

Key Features of Barlow’s Disease:

1. Pathology
– Thickened, redundant, and elongated mitral valve leaflets
– Excessive leaflet tissue with billowing or prolapsing segments
– Common involvement of both anterior and posterior leaflets
– Chordae tendineae may be elongated, thickened, or ruptured
2. Clinical Presentation
– Can be asymptomatic for years
– Symptoms (when present): Dyspnea (shortness of breath), Fatigue, Palpitations, Atypical chest pain
– Severe cases may exhibit heart failure symptoms
3. Diagnosis
– Best identified with echocardiography, especially 3D TEE
– Echo findings include prolapse of thick, bulky leaflets, mitral annular dilatation, and systolic “bowing” of leaflets into the left atrium
– Severe, often eccentric, mitral regurgitation may be observed
– Additional findings may include annular disjunction or posterior leaflet flail
4. Surgical Considerations
– Complex anatomy makes repair more challenging than in fibroelastic deficiency (FED)
– Repair by an experienced mitral valve surgeon is preferred over replacement
– Techniques may involve resection or remodeling of excess leaflet tissue, chordal transfer or artificial chordae (Gore-Tex), and the use of an annuloplasty ring to restore shape and function
5. Prognosis
– Excellent long-term outcomes with repair by experienced surgical teams
– Without repair, progressive regurgitation can lead to left ventricular dysfunction, atrial fibrillation, and heart failure.

60-year-old patient with a heart murmur for many years was diagnosed with mitral valve prolapse six years ago. She was on medication for the heart murmur and was diagnosed with an echocardiogram, at which time they discovered the mitral valve prolapse in both leaflets. The cardiologist suggested surgery, but the patient wanted a second opinion due to her hesitance. It was recommended that she have an echo every six months.

Then, the patient began to feel unwell with shortness of breath. Her doctor moved the surgery window from six months to four months to check on it. The patient decided to have the surgery. It was noted that her heart was already getting a little bit enlarged, which caused concern, as that is one of the indications of severity.

 

She was diagnosed with Barlow’s syndrome, or Barlow’s disease, which is excessive tissue of both leaflets, know as bi-leaflet prolapse. We had to repair the anterior leaflet and the posterior leaflet, and put a ring in, and we did a follow-up echo, not only in the operating room, but also right before discharge yesterday. The echo was perfect. She has no further mitral leakage nor stenosis. She does have SAM, systolic anterior motion, because she has an asymmetric septal hypertrophy (a little extra muscle knuckle).

She was discharged four days after surgery and says the pain after her conventional sternotomy is minimal.

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About The Author

Dr. Raymond Singer

Dr. Singer has been in practice since 1992 and has, to date, performed over 8,456 surgeries. His practice interests include complex valve, coronary and aneurysm surgery, as well as prevention and treatment of lung cancer.

 

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2023 Top Doc

 

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