CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 6, July 2013
AFRICA
201
Letter to the Editor
Left ventricular hypertrabeculation/non-compaction in a
pregnant woman
Dear Sir
We read with interest the article by Kilic
et al
. about a 19-year-
old female who developed exertional dyspnoea, neck vein
distension and pulmonary congestion shortly after delivery.
Transthoracic echocardiography revealed a dilated left ventricle,
an ejection fraction of 20%, pulmonary artery hypertension, and
surprisingly, left ventricular hypertrabeculation/non-compaction
(LVHT).
1
We have the following comments and concerns.
LVHT is not only due to an arrest in the cardiac compaction
process during embryogenesis, it may have another pathogenetic
background as well. In acquired LVHT, which has occasionally
been reported, LVHT is not present at birth but develops during
the patient’s lifetime.
2
Some studies show an increased frequency of stroke or
embolism among patients with LVHT, and other studies do not
confirm these findings. Did the presented patient have a history
of stroke or embolism, or were thrombi detected within the
intertrabecular spaces? Some studies report an increased risk of
stroke or embolism only in association with systolic dysfunction
or atrial fibrillation.
3
Was a cerebral MRI carried out?
Despite a negative family history for heart failure, it could
be useful to look for LVHT in other family members. In a study
of 15 families, with at least a single index patient with LVHT,
familial LVHT was detected in 27% of the cases.
4
LVHT is frequently associated with neuromuscular disorders
or chromosomal abnormalities, in particular muscular dystrophies,
myotonic dystrophies, metabolic myopathies, inclusion body
myositis, Barth syndrome, or 1q36 syndrome.
5
Was the presented
patient seen by a neurologist? Were there clinical indications of a
neuromuscular disorder or a chromosomal defect? Was there facial
or limb dysmorphism?What were the CK values and was an electro-
myogram recorded? Since some of the neuromuscular disorders
present with only mild manifestations, it is essential to refer each
patient with LVHT to a neurologist specialised in neuromuscular
disorders so as not to overlook associated muscle disease.
Altenberger
et al.
reported single patients with LVHT in
whom LVHT had disappeared during their lifetime.
6
Was
echocardiography carried out after delivery? Was there ever
a change observed in the morphology or extension of LVHT
between initial and follow-up echocardiographic investigations?
The patient received treatment with beta-blockers, angiotensin-
converting inhibitors, diuretics, and acetyl-salicylic acid.
1
Did
the morphology or extension of LVHT change on treatment?
LVHT is visible on ultrasound in foetuses during intra-
uterine development.
7
Was echocardiography carried out on the
patient’s child during pregnancy or after delivery? Since LVHT
can be detected during intra-uterine development, foetuses of
mothers with LVHT should be screened for the abnormality,
since isolated and non-isolated LVHT are associated with an
increased risk of developing heart failure, severe arrhythmias, or
stroke or embolism.
Overall, there are a number of points that need to be
addressed before drawing final conclusions about LVHT. LVHT
patients require close cardiological follow up so as not to
overlook deterioration of systolic function or the development
of ventricular arrhythmias. Patients with LVHT require
neurological investigations to confirm or exclude the presence
of a neuromuscular disorder or chromosomal abnormality.
JOSEF FINSTERER, MD, PhD,
Krankenanstalt Rudolfstiftung, Vienna, Austria
SINDA ZARROUK MAHJOUB, PhD
Biochemistry Laboratory, UR Human Nutrition and Metabolic
Disorders, Faculty of Medicine, Monastir, Tunisie
References
1.
Kilic ID, Tanriverdi H, Evrengul H, Uslu S, Sungur MA. Left ventricu-
lar non-compaction in pregnancy.
Cardiovasc J Afr
2013;
24
: e1–2.
2.
Finsterer J, Stöllberger C, Bonner E. Acquired noncompaction associ-
ated with coronary heart disease and myopathy.
Heart Lung
2010;
39
:
240–241.
3.
Stöllberger C, Blazek G, Dobias C, Hanafin A, Wegner C, Finsterer J.
Frequency of stroke and embolism in left ventricular hypertrabecula-
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Am J Cardiol
2011;
108
: 1021–1023.
4.
Finsterer J, Stöllberger C, Blazek G, Sehnal E. Familal left ventricular
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Int J Cardiol
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5.
Finsterer J, Stöllberger C, Fazio G. Neuromuscular disorders in left
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Curr Pharm Des
2010;
16
: 2895–2904.
6.
Altenberger J, Hasenauer G, Granitz M, Stöllberger C, Finsterer J.
Disappearance of left ventricular hypertrabeculation/noncompaction
and sudden death in a patient with Turner mosaic syndrome.
Am J
Cardiol
2012;
110
: 314–315.
7.
Gardiner H, Holder S, Karatza A. Prenatal diagnosis of fetal left
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