Grand Rounds Report: A Great Two Strike Hitter

by Academy of Clinical Excellence on December 3, 2010

Roy C. Ziegelstein, MD, along with Reda E. Girgis, MB, BCh and Kathleen T. Grieve, RN, BSN, MHA, CC presented at Grand Rounds this week “A Great Two Strike Hitter”.Mrs. MB, an 83 year old patient who was kind enough to share her experiences in person, and the presenters discussed Mrs. B’s experience living a long and productive life despite complications of rheumatic heart disease requiring surgery twice for mechanical mitral valves in adulthood (strike 1) and GI bleeding in the setting of life-long anticoagulation for that mechanical valve (strike 2).

Mrs. B related how she was “extremely sick” when she was 6 years old.The doctor heard a heart murmur and told the mother that he did not expect Mrs. B to reach her 18th birthday.Mrs. B did, obviously, make it to her 18th birthday, and lead a successful and productive adult life.She had a daughter, and did well until she reached her forties, when atrial fibrillation developed.She was found to have severe mitral valve disease and severe pulmonary hypertension in 1977, and underwent surgery for her first mechanical mitral valve.


The next 20 years were “good ones” for Mrs. B, but by 2003 her she was experiencing shortness of breath with minimal exertion, and episodes of syncope.Her first mechanical mitral valve showed severe stenosis by catheter, and her pulmonary artery pressures were 100 mmHg.Despite the high risk, she underwent a re-do of her mechanical mitral valve, with the hope that her high PA pressures would resolve, at least in part with improvement in her mitral valve function.She did well surgically.Unfortunately, her episodes of syncope continued, as did her very high PA pressures.This leads us to the first clinical pearl: left heart disease accounts for 80% of pulmonary hypertension.Left atrial pressures drive pulmonary hypertension.Fortunately, experimental treatment with an edothelin receptor antagonist (bosentan), improves her symptoms.


Six months later however, she developed GI bleeding.Mrs. B required multiple, multiple transfusions, but EGD and colonoscopy could not find the source.Enteroscopy to the jejunum and capsule endoscopy are negative.She was initially assumed (and later confirmed by enteroscopy) to have bleeding AVMs.This leads us to the second clinical pearl: 5% of patients with GI bleeding have no source identified, and this is called GI bleeding of obscure origin.Most of these patients are bleeding from the small bowel, and commonly from AVMs.Mrs. B also had moderate to severe aortic stenosis, leading to the third clinical pearl: Heyde’s syndrome, or AVM bleeding associated with aortic stenosis.Heyde’s syndrome is thought to represent an increased tendency to bleed, rather than an increase in AVMs.


The fourth clinical pearl is AVM growth may be related to increases of vascular endothelial growth factor, or VEGF and Thalidomide and its analogue lenalidomide (Revlimid) may be an effective treatment.Mrs. B did well for two years on Thalidomide treatment, but eventually it had to be withdrawn due to peripheral neuropathy.

Lastly, the presenters showcased how patient centered care (in this case using the Guided Care model), with tight coordination of outpatient resources, including physicians, nurses and even infusion centers can avoid hospitalizations and improve patient outcomes. Mrs. B was able to avoid hospitalizations, by getting close oversight and even blood transfusions as an outpatient. This leads us to the fifth and final clinical pearl: Patient-centered, coordinated care may have significant benefits to patients with chronic illness.

Please share your comments.

Eric E. Howell, M.D.
Division Director, Collaborative Inpatient Medicine Service
Division of Hospital Medicine
Johns Hopkins Bayview Medical Center

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