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Cardiac and extra-cardiac abnormalities associated with noncompaction

In their eminent article, Saraiva et al. reported about a 34-yo-male with heart-failure, AV-block-I, and left-bundle-branch-block (LBBB), reduced systolic function and left-ventricular hypertrabeculation / noncompaction (LVHT) [1]. We have the following comments and concerns.

The authors claim that the patient had LBBB. Assuming that the registration speed was 25mm/s, QRS-duration is borderline in figures 1 and 2. There is no AV-block I. Deviation of the electrical axis to +30° and -145° is not reconstructible.

Though ST-elevation in V1-V3 could be attributed to the assumed LBBB it would be interesting if the patient had coronary-heart-disease. Did he complain about angina or were CK and troponin-values ever elevated? Did he have a history of myocardial infarction? Did he undergo stress-testing, myocardial scintigraphy, or coronary angiography?

Which is the cause of dilative cardiomyopathy and heart-failure? Was there any indication for chronic alcoholism, coronary-heart-disease? borreliosis, viral infection, storage-disease, or neuromuscular disorder (NMD)?

The patient had a viral respiratory infection. Did he develop concomitant myocarditis or pulmonary embolism? Was there acute right-heart-strain on clinical examination, ECG, or echocardiography?

According to which definition was LVHT diagnosed? Öchslin’s, Stöllberger’s or Chin’s criteria [2-4]? Does figure 2 show an apical or cross-sectional view? Which value had the ratio noncompaction / compacted layer?

LVHT is located in the apex and the interventricular septum. In our series of >100 LVHT-patients none showed affection of the medial or basal septum. How to explain the occurrence of septal involvement?

Was the patient ever investigated for NMD or other rare hereditary disease, like dystrophinopathy, dystrobrevinopathy, myotonic dystrophy, zaspopathy, myoadenylate-deaminase-deficiency, Charcot-Marie-Tooth-disease, mitochondrial disorder, Barth- syndrome, Friedreich-ataxia, Pompe’s disease, Turner-syndrome, Ohtahara-syndrome, Roifman-syndrome, Noonan-syndrome, nail-patella-syndrome, Melnick-needles-syndrome, Melnick-Fraser-syndrome, MIDAS-syndrome, DiGeorge-syndrome, congenital adrenal hyperplasia, distal 4q-trisomy and distal 1q-monosomy, chromosome 1q43-deletion, distal chromosome 5q-deletion, trisomy 11, or trisomy 13 [5]?

Was the history positive for stroke/embolism, occasionally associated with LVHT? Which therapy was given, which were the results on the long-term follow-up, and which the outcome?
Rarely, LVHT develops during life. Were previous echocardiograms reviewed for LVHT? Did LVHT change concerning morphology and extent over time?

Were first degree family members investigated for LVHT? Since LVHT is suggestive of hereditary disease a family screening is strongly recommended to eventually clarify the nature of LVHT and associated underlying other disorders.

Overall, LVHT is usually not exclusively confined to the heart why more comprehensive investigations are mandatory in LVHT. If additional cardiac and extra-cardiac co-morbidity is not fully elucidated, the pathogenesis of LVHT will remain elusive.

Article Info

Article Type
Letter to the Editor
Publication history
Received: Thu 24, Jan 2019
Accepted: Tue 19, Feb 2019
Published: Thu 28, Feb 2019
Copyright
© 2019 Finsterer J. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved.
DOI: 10.31487/j.RGM.2019.01.002

Author Info

Corresponding Author
Finsterer J
Krankenanstalt Rudolfstiftung, Messerli Institute, Veterinary University of Vienna, Vienna, Austria, ORCID: 0000-0003-2839-7305

Figures & Tables

References

  1. Saraiva LR, Parente GB, Loureiro R, Leao TB (2006) Progression of ventricular depolarisation in left bundle branch block in non-compaction of the myocardium. Arq Bras Cardiol 87: 217-218.
  2. Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R (2000) Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 36: 493-500. [Crossref]
  3. Stöllberger C, Finsterer J (2004) Left ventricular hypertrabeculation/noncompaction. J Am Soc Echocardiogr 17: 91-100. [Crossref]
  4. Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R (1990) Isolated noncompaction of left ventricular myocardium. A study of eight cases. Circulation 82: 507-513. [Crossref]
  5. Finsterer J, Stollberger C, Blazek G (2006) Neuromuscular implications in left ventricular hypertrabeculation/noncompaction. Int J Cardiol 110: 288-300. [Crossref]