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Ann Thorac Surg 1999;67:1213-1214
© 1999 The Society of Thoracic Surgeons
a Departments of Cardiothoracic Surgery and Cardiology, St. Jude Medical Center, 100 E. Valencia Mesa Dr, Suite 301, Fullerton, CA 92835, USA
To the Editor
As a result of the discovery of an unusual valvular heart disease associated with the use of fenfluramine-phentermine (fen-phen) in a cluster of relatively young women without preexisting history of heart or lung pathology in Fargo, North Dakota, as reported by Newman [1] and Connolly and associates [2], the U.S. Food and Drug Administration issued a Public Health Advisory requesting other cases be reported [3]. The echocardiographic morphology and the histopathologic findings have established a strong association between fen-phen therapy and valvular disease. However, in the absence of a control group or case-control study, definitive association has yet to be determined [2].
Recently, we saw a 44-year-old female, previously treated with appetite suppressants, who presented with increasing shortness of breath, evidence of congestive heart failure, and a new murmur. An echocardiogram showed biatrial dilatation, right ventricular enlargement with abnormal septal wall motion, severe mitral regurgitation with a large central jet that entered the left atrial appendage and the left upper pulmonary vein, and severe tricuspid regurgitation. A heart catheterization was performed in preparation for operation, which revealed a pulmonary artery pressure of 66/28 mm Hg and confirmed the echocardiographic findings. The patient underwent an uneventful mitral valve replacement, preserving the subvalvular apparatus, and a tricuspid valve repair. Grossly, the mitral valve was glistening white, the leaflets were tethered, and the chordae were shortened, resembling tricuspid valves we have previously seen in patients with carcinoid heart disease. Histologically, a portion of the anterior leaflet showed intact architecture with a focal prominent fibroproliferative surface lesion with a "stuck on" appearance consistent with fen-phen–related valve disease. Except for a dilated annulus, the tricuspid valve appeared normal. The patient was discharged 3 days after the operation. A follow-up echocardiogram demonstrated good valvular function and normalization of pulmonary artery pressures. The patient remains symptom-free at 6 months of follow-up.
This would have been just another case of fen-phen–related valvular heart disease except for the fact that this patient has an identical twin who, despite having been treated with the same anorexic medications, remains symptom-free and without abnormal echocardiographic findings. Both the patient and her sister took fen-phen for 2 years and stopped taking the medication in September 1997. The patient, however, took a daily dose of fenfluramine 60 to 120 mg (frequently as much as 240 mg) and phentermine 90 mg (at times up to 180 mg), whereas her twin sister adhered to the daily amount prescribed (fenfluramine 60 mg and phentermine 24 mg). This seems to underscore the relevance of dosage in the production of this pathology.
References
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  2006 WRITING COMMITTEE MEMBERS, R. O. Bonow, B. A. Carabello, K. Chatterjee, A. C. de Leon Jr, D. P. Faxon, M. D. Freed, W. H. Gaasch, B. W. Lytle, R. A. Nishimura, et al. 2008 Focused Update Incorporated Into the ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons Circulation, October 7, 2008; 118(15): e523 - e661. [Full Text] [PDF]
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 R. O. Bonow, B. A. Carabello, K. Chatterjee, A. C. de Leon Jr, D. P. Faxon, M. D. Freed, W. H. Gaasch, B. W. Lytle, R. A. Nishimura, P. T. O'Gara, et al. 2008 Focused Update Incorporated Into the ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons J. Am. Coll. Cardiol., September 23, 2008; 52(13): e1 - e142. [Full Text] [PDF]
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R. O. Bonow, B. A. Carabello, K. Chatterjee, A. C. de Leon Jr, D. P. Faxon, M. D. Freed, W. H. Gaasch, B. W. Lytle, R. A. Nishimura, P. T. O'Gara, et al. ACC/AHA 2006 Guidelines for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease) Developed in Collaboration With the Society of Cardiovascular Anesthesiologists Endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons J. Am. Coll. Cardiol., August 1, 2006; 48(3): e1 - e148. [Full Text] [PDF]
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