eMedicine Specialties > Cardiology > Cancer and the Heart

Carney Complex: Treatment & Medication

Author: Craig T Basson, MD, PhD, FAHA, FACC, Director, Cardiovascular Research, The New York Presbyterian Hospital; Professor, Greenberg Division of Cardiology, Department of Medicine, Weill Medical College of Cornell University
Coauthor(s): Luke K Kim, MD, Fellow, Department of Internal Medicine, Division of Cardiology, New York Presbyterian Hospital, Weill Cornell Medical Center; Carl J Vaughan, MD, MRCPI, Adjunct Assistant Professor, Department of Internal Medicine, Division of Cardiology, Weill Medical College of Cornell University; Consulting Cardiologist, Mercy University Hospital, Ireland
Contributor Information and Disclosures

Updated: Mar 18, 2009

Treatment

Medical Care

  • Evaluation usually can be performed in an outpatient setting.
  • No specific drug is useful in the management of myxomas. Anticoagulation is not indicated in patients with myxomas and does not reduce the risk of emboli. Emboli arising from intracardiac myxomas are due to fragments of the tumor that break off and embolize.
  • Medical treatment of endocrine overactivity may be required.

Surgical Care

  • Mass lesions usually require biopsy/resection to provide a histopathologic diagnosis.
  • Surgery is necessary to resect intracardiac myxomas and to prevent embolic stroke or valvular obstruction.
    • Recognize that even in the presence of resections with adequate surgical margins, intracardiac myxomas may recur at sites distant from the initial operative site. These recurrences do not reflect a failure of the initial surgical treatment but, rather, a genetic predisposition to recurrent tumor formation.
    • Although recurrence of cardiac myxomas (and similarly extracardiac myxomas) is a feature of Carney complex, it is not a feature of typical nonsyndromic sporadic atrial myxomas.
  • Resect extracardiac myxomas and nonmyxomatous benign lesions if they produce symptoms by local extension of the space-occupying lesion.
  • Rare malignant tumors require resection and may require other adjunctive therapy and referral to an oncologist.

Consultations

  • Cardiologist
  • Geneticist
  • Cardiothoracic surgeon
  • Endocrinologist
  • Oncologist

Diet

No special diet is required.

Activity

In the absence of heart failure or persistent cardiac sequelae, no activity limitation is required.

Medication

No medications are effective in treating patients with Carney complex.

More on Carney Complex

Overview: Carney Complex
Differential Diagnoses & Workup: Carney Complex
Treatment & Medication: Carney Complex
Follow-up: Carney Complex
Multimedia: Carney Complex
References
[ CLOSE WINDOW ]

References

  1. Basson CT, MacRae CA, Korf B, Merliss A. Genetic heterogeneity of familial atrial myxoma syndromes (Carney complex). Am J Cardiol. Apr 1 1997;79(7):994-5. [Medline].

  2. Stratakis CA, Carney JA, Lin JP, et al. Carney complex, a familial multiple neoplasia and lentiginosis syndrome. Analysis of 11 kindreds and linkage to the short arm of chromosome 2. J Clin Invest. Feb 1 1996;97(3):699-705. [Medline].

  3. Casey M, Mah C, Merliss AD, et al. Identification of a novel genetic locus for familial cardiac myxomas and Carney complex. Circulation. Dec 8 1998;98(23):2560-6. [Medline].

  4. Casey M, Vaughan CJ, He J, et al. Mutations in the protein kinase A R1alpha regulatory subunit cause familial cardiac myxomas and Carney complex. J Clin Invest. Sep 2000;106(5):R31-8. [Medline].

  5. Veugelers M, Bressan M, McDermott DA, Weremowicz S, Morton CC, Mabry CC, et al. Mutation of perinatal myosin heavy chain associated with a Carney complex variant. N Engl J Med. Jul 29 2004;351(5):460-9. [Medline].

  6. Reynen K. Cardiac myxomas. N Engl J Med. Dec 14 1995;333(24):1610-7. [Medline].

  7. Goldstein MM, Casey M, Carney JA, Basson CT. Molecular genetic diagnosis of the familial myxoma syndrome (Carney complex). Am J Med Genet. Sep 3 1999;86(1):62-5. [Medline].

  8. Carney JA, Gordon H, Carpenter PC, et al. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore). Jul 1985;64(4):270-83. [Medline].

  9. Kanda T, Umeyama S, Sasaki A, et al. Interleukin-6 and cardiac myxoma. Am J Cardiol. Nov 1 1994;74(9):965-7. [Medline].

  10. Veugelers M, Wilkes D, Burton K, et al. Comparative PRKAR1A genotype-phenotype analyses in humans with Carney complex and prkar1a haploinsufficient mice. Proc Natl Acad Sci U S A. Sep 28 2004;101(39):14222-7.

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

carney complex, lentigines, atrial myxomas, mucocutaneous myxomas, blue nevi, LAMB syndrome, nevi, atrial myxoma, myxoid neurofibroma, ephelides, NAME syndrome, rhabdomyoma, myxoma, cardiac myxomas, cardiac tumor, heart tumor, cutaneous myxoma, spotty pigmentation of the skin, endocrinopathy, endocrine tumor, nonendocrine tumor

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Craig T Basson, MD, PhD, FAHA, FACC, Director, Cardiovascular Research, The New York Presbyterian Hospital; Professor, Greenberg Division of Cardiology, Department of Medicine, Weill Medical College of Cornell University
Craig T Basson, MD, PhD, FAHA, FACC is a member of the following medical societies: American College of Cardiology and American Heart Association
Disclosure: Nothing to disclose.

Coauthor(s)

Luke K Kim, MD, Fellow, Department of Internal Medicine, Division of Cardiology, New York Presbyterian Hospital, Weill Cornell Medical Center
Disclosure: Nothing to disclose.

Carl J Vaughan, MD, MRCPI, Adjunct Assistant Professor, Department of Internal Medicine, Division of Cardiology, Weill Medical College of Cornell University; Consulting Cardiologist, Mercy University Hospital, Ireland
Carl J Vaughan, MD, MRCPI is a member of the following medical societies: American College of Cardiology, American College of Physicians, and American Heart Association
Disclosure: Nothing to disclose.

Medical Editor

Justin D Pearlman, MD, PhD, ME, MA, Director of Advanced Cardiovascular Imaging, Professor of Medicine, Professor of Radiology, Adjunct Professor, Thayer Bioengineering and Computer Science, Dartmouth-Hitchcock Medical Center
Justin D Pearlman, MD, PhD, ME, MA is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, International Society for Magnetic Resonance in Medicine, and Radiological Society of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Frank M Sheridan, MD, Cardiology, Providence Everett Medical Center
Frank M Sheridan, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Richard A Lange, MD, Professor and Executive Vice Chairman of Medicine, University of Texas Health Science Center at San Antonio
Richard A Lange, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, and Association of Subspecialty Professors
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.