eMedicine Specialties > Cardiology > Cancer and the Heart

Carney Complex: Follow-up

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

Follow-up

Further Inpatient Care

Admit the patient for testing and surgical intervention.

Further Outpatient Care

  • Monitor patients annually with echocardiography to detect the occurrence of new cardiac myxomas.
  • Annual history and physical examination also is required to detect the development of extracardiac myxomas, other tumors, and signs and symptoms of endocrine dysfunction.

Inpatient & Outpatient Medications

  • No specific medications are indicated for this condition.
  • Antibiotic prophylaxis is required if an associated valvular insufficiency exists.
  • Medical management of endocrine dysfunction may be appropriate.

Transfer

Transfer of the patient may be required for further diagnostic evaluation and surgical intervention.

Deterrence/Prevention

No particular deterrent is available.

Complications

  • Recurrent myxoma growth
  • Congestive heart failure
  • Stroke
  • Peripheral embolization including coronary, retinal, renal, celiac, and femoral and pulmonary arteries
  • Arrhythmia
  • Pulmonary hypertension

Prognosis

Prognosis generally is good and depends on the proclivity of cardiac myxomas to recur. The risks associated with open-heart surgery increase with each successive surgery.

Patient Education

The family should be aware that this is an autosomal dominant disorder with a 50% chance that the offspring of an affected individual will have the disorder.

Miscellaneous

Medicolegal Pitfalls

  • Failure to perform echocardiography to rule out an intracardiac source of emboli in patients presenting with stroke or transient ischemic attack (particularly at a young age)
  • Failure to recognize the potential for myxoma formation in family members of an individual affected by Carney complex
  • Failure to recognize the potential for recurrent myxoma formation in individuals with prior myxoma resection
  • Failure to recognize rare malignant tumors
  • Failure to provide appropriate genetic counseling and properly inform the patient of the heritable nature of the disorder
  • Failure to recognize endocrine dysfunction in a patient 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
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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.

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Further Reading

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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

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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.

 
 
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