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Noncompaction of the Right Ventricle

Authors:
LETTER TO THE EDITOR
Noncompaction of the Right Ventricle
Giovanni Fazio Monica Lunetta Emanuele Grassedonio Alessandro Gullotti
Giovani Ferro Daniela Bacarella Giuseppe Lo Re Giuseppina Novo
Midiri Massimo Emiliano Maresi Salvatore Novo
Published online: 13 February 2010
ÓSpringer Science+Business Media, LLC 2010
Noncompaction of the ventricular myocardium is a disease
characterized by an increase of the ventricular trabecular
meshwork caused by arrest of the normal endomyocardial
morphogenesis (Figs. 1,2,3).
In accordance with the normal human anatomy, the left
ventricular wall is well compacted with a few thin tra-
beculae; on the contrary, the normal right ventricular wall
is furrowed by many trabeculae (the trabecula of the
marginal septum as well as other ones). For this reason, the
term ‘‘noncompaction’’ usually refers to an exclusive or
prevalent disease of the left ventricle [116]. Recently
Song and Aragona et al. [13] reported two cases of iso-
lated right-ventricular noncompaction. According to the
data coming from scientific literature and from our own
experience, in some patients noncompaction is biventric-
ular [5,6], and an increase of the right ventricular mesh-
work is often evident in such cases, even more so than on
the left side.
The main diagnostic criterion of noncompaction, that is,
the only one that is accepted and recognized, is evaluation
of the ratio between the spongiosus and the compact
thickness of the ventricular wall, which must be [2[616].
This ratio is easy to calculate for the left ventricle; on the
contrary, it is more difficult to calculate for the right ven-
tricle. Only one case of right-ventricular noncompaction
has been reported in the literature [7,8].
In our personal clinical experience, we have found that
many cases of biventricular noncompaction are reported in
the register of Italian Society of Cardiovascular Echocar-
diography. Recently two patients with an inexplicable
dilatation of the right ventricle caught our attention: Both
of them showed a more prominent trabecular meshwork on
G. Fazio (&)M. Lunetta E. Grassedonio A. Gullotti
G. Ferro D. Bacarella G. L. Re G. Novo M. Massimo
E. Maresi S. Novo
Department of Cardiology, University of Palermo,
Via Albiri 3 a, 90125 Palermo, Italy
e-mail: giovanni.fazio-aaaa@poste.it
G. Fazio M. Lunetta E. Grassedonio A. Gullotti G. Ferro
D. Bacarella G. L. Re G. Novo M. Massimo
E. Maresi S. Novo
Department of Radiology, University of Palermo,
Via Albiri 3 a, 90125 Palermo, Italy
Fig. 1 Four-chamber view of isolated noncompaction in the first
patient. Note the normal compaction of the left ventricle
123
Pediatr Cardiol (2010) 31:576–578
DOI 10.1007/s00246-010-9652-6
the right ventricle, and this was more stressed in particular
areas of the same ventricle. The spongious-to-compact
ratio measured in the more trabecular areas was consis-
tently [3. The right ventricular volume was increased. One
of these patients had complex ventricular extrasystoles, and
magnetic resonance imaging confirmed the presence of
prominent trabecular meshwork on the right ventricle and
its dilatation. The second patient, a 3-year-old child sud-
denly and unexplainable died. A third patient, who
underwent repair of tetralogy of Fallot, showed an isolated
abnormal dilatation of the right ventricle.
What should be done in circumstances like these?
Should a diagnosis of isolated noncompaction of the right
ventricle be made, or should this aspect be described as an
accentuated trabecular meshwork? In contrast, in two of the
three cases previously described the increase of the tra-
becular meshwork was associated with major adverse
cardiac events, e.g., complex ventricular arrhythmias and
sudden death.
Our opinion is that a diagnosis of isolated noncompac-
tion of the right ventricle should be made only when
accentuation of the trabecular meshwork is present in
certain areas (while in others spongious-to-compact ratio
is \2) and it is associated with dilatation of the right
ventricle.
References
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magnetic resonance imaging: a case report. J Cardiol 50:149–154
Fig. 3 Magnetic resonance characterization of the heart of the child
affected by noncompaction and sudden death. Note the many
prominent trabeculae
Fig. 2 Magnetic resonance characterization of the isolated noncom-
paction areas in the second patient. The noncompaction was confined
to the right lateral wall
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... This condition is characterized by prominent ventricular trabeculae, a thin myocardium, and deep intertrabecular recesses among the trabeculae (10)(11)(12)(13). Although LVNC mainly affects the left ventricle, isolated right ventricular or biventricular noncompaction has also been reported (14)(15)(16). Clinically, LVNC is associated with left ventricular dilation, hypertrophy, or other forms of congenital cardiac Left ventricular noncompaction (LVNC) is one of the most common forms of genetic cardiomyopathy characterized by excessive trabeculation and impaired myocardial compaction during fetal development. Patients with LVNC are at higher risk of developing left/right ventricular failure or both. ...
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Left ventricular noncompaction (LVNC) is one of the most common forms of genetic cardiomyopathy characterized by excessive trabeculation and impaired myocardial compaction during fetal development. LVNC patients are at higher risk to develop left or right ventricular failure, or both. While key regulators for cardiac chamber development are well studied, the role of Semaphorin-Plexin signaling in this process remains poorly understood. Here, we demonstrate that genetic deletion of Plxnd1, a class 3 semaphorin receptor in endothelial cells, leads to severe cardiac chamber defects, as characterized by excessive trabeculation and non-compaction similar to LVNC patients. Loss of Plxnd1 results in decreased expression of extracellular matrix (ECM) proteolytic genes leading to excessive deposition of cardiac jelly. We demonstrate that Plxnd1 deficiency is associated with an increase in the expression of Notch and its downstream target genes. In addition, inhibition of Notch signaling pathway can partially rescue the excessive trabeculation and non-compaction phenotype present in Plxnd1 mutants. Furthermore, we demonstrate that Semaphorin 3e (Sema3e), one of Plxnd1’s ligands is expressed in the developing heart and is required for myocardial compaction. Collectively, our results demonstrate that the Sema3e-Plxnd1 pathway is essential for myocardial trabeculation and compaction.
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Isolated noncompaction of left ventricular myocardium is a rare congenital heart disease, characterized by an excessive prominence of trabecular meshwork, spaced out by deep intertrabecular recesses, consequent to the arrest of the normal myocardial embryogenesis. Although there are numerous descriptions, the pathophysiological effects of the structural alterations, like the clinical spectrum and the evolution of the disease, are not fully clarified. In this paper we evaluated the natural history of the disease, the family incidence and the alterations of the systolic and diastolic function. An interesting case report is described concerning a patient affected by noncompaction and atrial fibrillation.
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A 88 year-old man admitted with iterative severe chest distress and breathlessness associated with activity without chest complaint for 5 years. He underwent an initial 2DE that did not confirm HVM/NVM and 3DE that strengthened the diagnosis of HVM/NVM. In contrast to 2DE, 3DE provides for pyramid-shaped datasets that encompass the entire right ventricular. Specifically, right ventricular can be sectioned in user selected planes and an unlimited number and angles of such planes can be used. The present case should be regarded as isolated right ventricular HVM/NVM because there were no any factors that could explain the arrest of the development of the myocardial structure.
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Isolated noncompaction of left ventricular myocardium is a rare disorder of endomyocardial morphogenesis characterized by numerous, excessively prominent ventricular trabeculations and deep intertrabecular recesses. This study comprised eight cases, including three at necropsy. Ages ranged from 11 months to 22.5 years, with follow-up as long as 5 years. Gross morphological severity ranged from moderately abnormal ventricular trabeculations to profoundly abnormal, loosely compacted trabeculations. Echocardiographic images were diagnostic and corresponded to the morphological appearances at necropsy. The depths of the intertrabecular recesses were assessed by a quantitative echocardiographic X-to-Y ratio and were significantly greater than in normal control subjects (p less than 0.001). Clinical manifestations of the disorder included depressed left ventricular systolic function in five patients, ventricular arrhythmias in five, systemic embolization in three, distinctive facial dysmorphism in three, and familial recurrence in four patients. We conclude that isolated noncompaction of left ventricular myocardium is a rare if not unique disorder with characteristic morphological features that can be identified by two-dimensional echocardiography. The incidence of cardiovascular complications is high. The disorder may be associated with facial dysmorphism and familial recurrence.
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Isolated left ventricular noncompaction is a genetically heterogeneous congenital disorder characterized by an altered structure of the myocardial wall. This cardiomyopathy is thought to be due to an arrest of intrauterine compaction of the myocardial fibers in the absence of any other structural heart disease. Noncompaction of the left ventricular myocardium is an uncommon finding and remains frequently overlooked even by experienced echocardiographers. However, a correct diagnosis of noncompaction has important implications due to the possible association with other cardiac abnormalities and/or muscle disorders, progressive left ventricular dysfunction, risk of thromboembolism, and life-threatening arrhythmias. Furthermore, because of the familial association described with ventricular noncompaction, screening with echocardiography of first relatives is recommended. Since echocardiography is the diagnostic technique of choice, missed diagnoses may be due to nonoptimal imaging of the lateral and apical myocardium, and/or insufficient disease awareness by echocardiographers. To increase awareness of left ventricular noncompaction, the present paper reviews embryology, genetics, clinical features and pathophysiology, diagnosis, treatment and prognosis of patients affected by isolated left ventricular noncompaction.
Article
The Tissue doppler analysis is a new echocardiographic approach to evaluate the diastolic function. In this report we performed a Tissue doppler analysis in a series of 15 children affected by non-compaction. The bidimensional echo-cardiogram showed a systolic function which was reduced only in 8 patients. Alterations of the diastolic function were founded in 7 patients: in 2 cases, a reduction of the E tissue wave was present in all segments. In 3 patients the diastolic dysfunction was limited to apical and lateral segments. In the last 2 children a reduction of the E wave interested only the apical segments. There was a strong correlation between systolic and diastolic dysfunction: in fact all patients with diastolic dysfunction also presented a severe reduction of the systolic function.
Article
Isolated left ventricular non-compaction is the result of incomplete myocardial morphogenesis, leading to persistence of the embryonic myocardium. The condition is recognized by an excessively prominent trabecular meshwork and deep intertrabecular recesses of the left ventricle. Whether these intertrabecular recesses are a favorable substrate for ventricular arrhythmias is unclear. Some reports have found that the fatal ventricular arrhythmias may occur in approximately half of the patients. In this report we investigated about this association. In total we evaluated a continuous series of 238 patients affected by non-compaction. Periodic Holter monitoring was performed every 6 months for 4 years. Only 11 patients had documented ventricular tachycardia, which was sustained in two cases and non-sustained in nine. In no cases we observed ventricular fibrillation. Non-compaction alone does not seem to be a risk factor for malignant ventricular arrhythmias.