Author + information
- Josef Finsterer, MD, PhD∗ ( and )
- Claudia Stöllberger, MD
- ↵∗Krankenanstalt Rudolfstiftung, Postfach 20, Vienna 1180, Austria
We read with interest the review about isolated left ventricular hypertrabeculation/noncompaction (LVHT) by Hussein et al. (1). However, the paper raises a number of concerns.
We disagree that LVHT exclusively results from incomplete intrauterine compaction of the developing myocardium. LVHT can be acquired in neuromuscular disorders (NMDs) (2), in pregnant women, and in athletes and may disappear in single cases, which are strong arguments against the noncompaction hypothesis.
We also disagree with the statement that LVHT was first described by Grant in 1926 (3). His case does not meet current diagnostic criteria because trabeculations were predominantly seen in the right portion of the single ventricle because the basal segments were predominantly affected and because intertrabecular spaces communicated with myocardial and epicardial vessels.
Earlier than Engberding in 1984, Feldt reported in 1969 a biventricular bizarre spongy myocardial pattern in an autopsy case and Westwood (4) presented a figure of a case (case 3) unambiguously showing biventricular hypertrabeculation.
We also disagree with classifying LVHT as a genetic disorder for the following reasons: LVHT has been associated with >30 different mutated genes and even more chromosomal defects, thus rendering it unlikely to be responsible for the same morphological abnormality; only a limited number of mutation carriers presents with LVHT (LVHT often does not segregate with a mutation); a specific mutation associated with LVHT in 1 family member may be associated with variable cardiac abnormalities in another family member; LVHT can be acquired.
Application of the LVHT mass as a cardiac magnetic resonance imaging criterion is misleading because this parameter does not differentiate between mass of intertrabecular tissue (blood) and mass of trabeculations (myocardium). Because the relationship between these 2 figures varies considerably between patients, LVHT mass does not appear to be a reliable diagnostic marker.
When considering transplantation as a treatment of intractable heart failure, myotoxic immunosuppression should be avoided in patients with an NMD to prevent further neurological deterioration.
Overall, uncertainties about diagnosis, treatment, and prognosis of LVHT may remain unsolved as long as the pathogenetic background of LVHT is not fully elucidated.
Please note: The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- 2016 American College of Cardiology Foundation