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. 2017 Apr-Jun;7(2):78–79. doi: 10.4103/ajm.AJM_184_16

Hyperthyroidism and noncompaction

Josef Finsterer 1,, Claudia Stöllberger 1
PMCID: PMC5398009  PMID: 28469992

Editor,

With interest, we read the article by Habib et al. about a 26-year-old Afro-American female with left ventricular hypertrabeculation/noncompaction (LVHT) in whom hyperthyroidism (Grave's disease) was assumed to have triggered heart failure.[1] We have the following comments and concerns.

We do not agree with the notion that exclusively hyperthyroidism triggered the development of heart failure.[1] Although hyperthyroidism may induce heart failure,[2] it has to be excluded that heart failure was due to LVHT alone, in which heart failure is a common complication.[3]

Autoimmune disease in a single organ can be associated with autoimmune disease in other organs, such as the liver, heart, intestines, or muscle.[4] Were there indications for extrathyroid autoimmune disease or manifestations of hyperthyroidism in the skin? Was the family history positive for autoimmune disease?

LVHT has familial occurrence.[5] Were first-degree relatives screened for LVHT? In how many of these family members, was LVHT detected in addition to the presented patient? Did first-degree relatives manifest with cardiomyopathies other than LVHT?

The authors regard LVHT as a genetic cardiomyopathy.[1] Although LVHT has been reported in association with mutations in >40 different genes, with polymorphisms, and numerous chromosomal defects,[6] a causal relation between these defects and the occurrence of LVHT awaits to be established. Arguments which make a causal relation unlikely are that LVHT can be acquired, that in case of autosomal dominant transmission, LVHT may not occur in each generation, that cardiac disease associated with a particular genetic defect can be highly variable within a family, that in families with an inherited genetic defect and LVHT, LVHT can be found only in some of the mutation carriers (LVHT does not segregate with a certain mutation), and that the number of mutated genes claimed to cause LVHT is large.[7]

LVHT is associated with neuromuscular disorders in up to 80% of the cases, particularly if patients with a neuromuscular disorder are systematically screened for cardiac involvement.[8] In addition, hyperthyroidism may go along with secondary myopathy. Was the patient seen by a neurologist? Were there indications for a neuromuscular disorder or weakness, wasting, easy fatigability, myalgias, muscle cramps, hyperCKemia, or a myopathic electromyography?

Overall, the report about this interesting case could profit from provision of a family screening for LVHT or other cardiomyopathies, exclusion of autoimmune disease in organs other than the thyroid gland, and from neurological investigation of the index case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

REFERENCES

  • 1.Habib H, Hawatmeh A, Rampal U, Shamoon F. Left ventricular noncompaction diagnosed following Graves' disease. Avicenna J Med. 2016;6:113–6. doi: 10.4103/2231-0770.191448. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 2.Martinez F. Thyroid hormones and heart failure. Heart Fail Rev. 2016;21:361–4. doi: 10.1007/s10741-016-9556-5. [DOI] [PubMed] [Google Scholar]
  • 3.Finsterer J, Stollberger C, Towbin JA. Cardiac, neuromuscular, and genetic implications of noncompaction. Nat Rev Cardiol. 2017 doi: 10.1038/nrcardio.2016.207. In press. [DOI] [PubMed] [Google Scholar]
  • 4.Ochs HD, Torgerson TR. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked inheritance: Model for autoaggression. Adv Exp Med Biol. 2007;601:27–36. doi: 10.1007/978-0-387-72005-0_3. [DOI] [PubMed] [Google Scholar]
  • 5.Finsterer J, Stöllberger C, Blazek G, Sehnal E. Familal left ventricular hypertrabeculation (noncompaction) is myopathic. Int J Cardiol. 2013;164:312–7. doi: 10.1016/j.ijcard.2011.07.012. [DOI] [PubMed] [Google Scholar]
  • 6.Finsterer J. Cardiogenetics, neurogenetics, and pathogenetics of left ventricular hypertrabeculation/noncompaction. Pediatr Cardiol. 2009;30:659–81. doi: 10.1007/s00246-008-9359-0. [DOI] [PubMed] [Google Scholar]
  • 7.Finsterer J, Zarrouk-Mahjoub S. Considerations about the genetics of left ventricular hypertrabeculation/non-compaction. Cardiol Young. 2015;25:1435–7. doi: 10.1017/S1047951115000694. [DOI] [PubMed] [Google Scholar]
  • 8.Stöllberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol. 2002;90:899–902. doi: 10.1016/s0002-9149(02)02723-6. [DOI] [PubMed] [Google Scholar]

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