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\begin{document}

\title{An unusual pattern of late gadolinium enhancement (LGE) in a
bi-ventricular non-compaction patient}



\author[1]{Fatma Elkafrawy}%
\author[2]{Mahmoud Abdelnabi}%
\author[1]{Abdelraman Assal}%
\author[1]{Hoda Shehata}%
\affil[1]{Alexandria University Faculty of Medicine}%
\affil[2]{Alexandria University Medical Research Institute}%


\vspace{-1em}



  
  \date{May 30, 2024}


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\begin{abstract}
Myocardial non-compaction (NC) is a rare genetically heterogeneous
cardiomyopathy which is characterized by excessively prominent
ventricular trabeculations with deep intertrabecular recesses. It is
commonly believed to be due to an intrauterine arrest of the
endomyocardial morphogenesis. Left ventricle (LV) is the usual site of
affection, but very rarely right ventricular (RV) or both ventricles may
be involved. It can be isolated or associated with other cardiac
diseases or neuromuscular disorders. It has a wide spectrum of clinical
manifestations that range from asymptomatic status, progressive
ventricular dysfunction, arrhythmias and congestive heart failure that
have been found to be associated with myocardial fibrosis. Cardiac
Magnetic resonance (CMR) is the gold standard non-invasive modality for
the diagnosis of cardiomyopathies. In NC cases, late gadolinium
enhancement (LGE) of the trabeculated layer or mild fibrosis of the
compacted layer was previously described in the literature, however
extensive fibrosis in the compacted layer only with no fibrosis at the
trabeculated layer was not described before. We report a male patient
presented with heart failure symptoms, echocardiogram showed severe LV
and RV systolic dysfunction with prominent bi-ventricular
trabeculations. CMR confirmed the diagnosis of bi-ventricular
non-compaction with peculiar pattern extensive fibrosis of the compacted
layer of LV in LGE. To the best of our knowledge, our case is the first
case to report extensive fibrosis in the compacted layer only with no
fibrosis at the trabeculated layer.%
\end{abstract}%



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\section*{Case presentation}

{\label{case-presentation}}

A 30-year-old male patient, non-diabetic, non-hypertensive, smoker
presented to our facility complaining of a recent onset dyspnea grade
III, orthopnea and paroxysmal nocturnal dyspnea since 3 weeks. Three
days before admission he was complaining of atypical left sided chest
pain and worsening of dyspnea from grade III to IV. On examination, he
appeared tachypneic with bilateral congested pulsating neck veins. Vital
signs, 110/70mmHg, heart rate of 110 beats per minute. His laboratory
tests were unremarkable. His ECG was unremarkable except for a wide QRS
complex and interventricular conduction delay. Echocardiography (Figure
1, Panel A, Videos 1,2,3,4) showed dilated dimensions with global
hypokinesia mostly at the septum with marked trabeculations of the
apical inferior and lateral walls. Coronary angiography revealed ectatic
vessels with slow flow but no obstructive lesions. CMR (Figure 1, Panel
B,C,D,E,F, Videos 5,6) confirmed the presence of biventricular NC, in
the LV, involved 11/17 segments notably the lateral wall and to a lesser
extent the anterior and inferior walls. The ratio of non-compacted to
compacted layer at end diastole was 4.2. Hypertrophied RV with extensive
trabeculations was also noted. Marked global biventricular hypokinesia
with nearly akinetic septum and very poor biventricular function
(LVEF=15\%, RVEF=27\%). On late gadolinium enhancement (LGE) images,
extensive fibrosis was found representing 30\% of the total myocardial
mass. Different patterns of fibrosis were found in the form of
basal/midventricular mid-wall fibrosis in the septum, subepicardial to
mid wall fibrosis in the anterior and inferior walls (mounting to near
transmural) and mid-ventricular subendocardial to mid wall fibrosis in
the lateral wall as well as transmural fibrosis of all apical segments.
No thrombi were found. So, the diagnosis of biventricular NC with
extensive fibrosis was reported. Accordingly, the patient was maintained
on anti-failure measures and anticoagulation. In our case, CMR confirmed
the diagnosis of biventricular NC with accurate measurement of the
biventricular volumes and function. It also added the presence of
extensive myocardial fibrosis (which is correlated to clinical
manifestations) with the ability of its quantification. The previously
reported most common LGE patterns of fibrosis are subendocardial and
transmural distribution in non-compacted segments and mid-wall
distribution in compacted segments. \textsuperscript{(1,2)} However, in
our case, there was extensive subendocardial, subepicardial, transmural
and mid wall fibrosis within the compacted layer with no fibrosis at the
non-compacted layer. The pathophysiological mechanisms of fibrosis in
isolated NC are still uncertain. Subendocardial and transmural
distribution of the non-compacted layer might be explained by the
defects in the coronary microcirculation which affects the
vascularization of the abnormal myocardium. \textsuperscript{(2,3)} The
mid-wall enhancement of the compacted layer (mainly at the septum) is
thought to be the result of many factors such as genetic predisposition,
microvascular ischemia and the maladaptive processes related to
increased wall stress caused by progressive LV remodeling that may
result in focal myocyte necrosis, preferentially affecting the middle,
circumferential layer. \textsuperscript{(4)}

\textbf{Disclosure: The authors declare no conflict of interest}

\textbf{References:}

\begin{enumerate}
\tightlist
\item
  Szemraj-Rogucka Z, Majos A Left Ventricular Non-Compaction:
  Mid-myocardial Distribution of Late Gadolinium Enhancement in
  Compacted Segments. OMICS J Radiol 2017;6: 246.
\item
  Lachhab A, Doghmi N, Elfakir Y, Taoussi O, Benyass A, et al. (2012)
  Insights from magnetic resonance imaging of left ventricular
  non-compaction in adults of North African descent. Int Arch Med 5: 10
\item
  Towbin JA, Lorts A, Jefferies JL Left ventricular non-compaction
  cardiomyopathy. The Lancet 2015;386: 813-825.
\item
  Nucifora G, Aquaro GD, Pingitore A, Masci PG, Lombardi M Myocardial
  fibrosis in isolated left ventricular non-compaction and its relation
  to disease severity. Eur J Heart Fail 2011;13: 170-176.
\end{enumerate}\selectlanguage{english}
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