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Isolated left ventricular noncompaction: unusual cause of decompensated heart failure and indication of heart transplantation in the early infancy--case report and literature review.

Barbosa ND, Azeka E, Aiello VD, Viana F, Jatene M, Tanamati C, Marcial MB, Macial MB - Clinics (Sao Paulo) (2008)

View Article: PubMed Central - PubMed

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The isolated noncompaction of the left ventricle (IVNC) is a rare disease, of which only about 100 cases have been reported so far... It is supposedly the result of an arrest in the normal endomyocardial embryogenesis, which leads to the persistence of intertrabecular recesses and the development of a spongy myocardium... During normal intrauterine development, the myocardium condenses and the intertrabecular recesses are reduced to capillaries... She weighed 6.8 Kg and was 75.5 cm tall... Cardiovascular examination revealed a decreased-volume peripheral pulse and a third heart sound... The patient evolved with refractory heart failure as a result of severely deteriorated left ventricular function and became a candidate for heart transplantation... The compacted epicardial layer appears as a compact band of uniform tissue while the much thicker endocardial non-compacted layer consists of trabecular meshwork with deep endomyocardial spaces surrounded by exaggerated hypertrophy of the trabeculae... Although it must be emphasized that, strictly speaking, a two layered structure is found only in IVNC, and not in left ventricular hypertrophy (LVH), dilated cardiomyopathy (DCM), or any other condition, it may sometimes appear difficult to define where prominent trabeculation (such as DCM) ends and non-compactation begins... Patients with depressed ventricular function may present signs of heart failure similar to those observed in patients with dilated cardiomyopathy, and which were seen in this infant... Familial cases have been reported, where the mode of inheritance is heterogeneous... In the X-linked form of the disease, a locus has been found on Xq28, and mutations have been reported in G4.5 gene., This genetic linkage is located close to other areas that have been associated with various other myopathies... Jenni et al have described diagnostic echocardiographic criteria for IVNC that include 1) the absence of co-existing cardiac abnormalities; 2) a 2-layered structure of the ventricular wall with an end-systolic ratio of the noncompacted to the compacted layer >2; 3) the finding of this morphologic presentation in apical and in mid-ventricular areas, and 4) direct blood flow from the ventricular cavity into the deep intertrabecular recesses, as assessed by Doppler echo-cardiography... Magnetic resonance imaging and myocardial perfusion scanning can be of additional help in detecting sub-endocardial perfusion defects., A recent resonance magnetic study concluded that a ratio of non-compacted to compacted myocardium greater than 2.3 would reliably the disease entity... A review of the literature identified only 9 patients with IVNC who underwent cardiac transplantation., This infant is the youngest child reported with IVNC and clinical signs of decompensated heart failure to have been successfully treated with heart transplantation... Although a rare entity, isolated ventricular noncompactation can lead to decompensated heart failure in the early infancy and may be successfully treated with heart transplantation.

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Gross aspect of the explanted heart, showing the characteristic non-compaction involving the inlet and apical portion of the left ventricle but sparing the ventricular septum.
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f1-cln63_1p0136: Gross aspect of the explanted heart, showing the characteristic non-compaction involving the inlet and apical portion of the left ventricle but sparing the ventricular septum.

Mentions: The pathology analysis of the explanted heart revealed ventricles with proeminent ventricular trabeculation and deep recesses, involving the inlet and the apical portion. The compact myocardium was thin (3mm) and endocardium showed diffuse thickening. The ratio of thickness of noncompacted-to-compacted myocardium was 3.3. The septum was 8 mm thick and there was myocardial hypertrophy, consistent with the diagnosis of dilated cardiomy-opathy with isolated noncompaction of the left ventricular myocardium or “spongy myocardium” (Figure 1). Microscopically, the deep recesses were lined by a thickened en-docardial layer and the myocardial fibers showed mild hypertrophy (Figure 2). No inflammatory infiltrate was detected.


Isolated left ventricular noncompaction: unusual cause of decompensated heart failure and indication of heart transplantation in the early infancy--case report and literature review.

Barbosa ND, Azeka E, Aiello VD, Viana F, Jatene M, Tanamati C, Marcial MB, Macial MB - Clinics (Sao Paulo) (2008)

Gross aspect of the explanted heart, showing the characteristic non-compaction involving the inlet and apical portion of the left ventricle but sparing the ventricular septum.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2664197&req=5

f1-cln63_1p0136: Gross aspect of the explanted heart, showing the characteristic non-compaction involving the inlet and apical portion of the left ventricle but sparing the ventricular septum.
Mentions: The pathology analysis of the explanted heart revealed ventricles with proeminent ventricular trabeculation and deep recesses, involving the inlet and the apical portion. The compact myocardium was thin (3mm) and endocardium showed diffuse thickening. The ratio of thickness of noncompacted-to-compacted myocardium was 3.3. The septum was 8 mm thick and there was myocardial hypertrophy, consistent with the diagnosis of dilated cardiomy-opathy with isolated noncompaction of the left ventricular myocardium or “spongy myocardium” (Figure 1). Microscopically, the deep recesses were lined by a thickened en-docardial layer and the myocardial fibers showed mild hypertrophy (Figure 2). No inflammatory infiltrate was detected.

View Article: PubMed Central - PubMed

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

The isolated noncompaction of the left ventricle (IVNC) is a rare disease, of which only about 100 cases have been reported so far... It is supposedly the result of an arrest in the normal endomyocardial embryogenesis, which leads to the persistence of intertrabecular recesses and the development of a spongy myocardium... During normal intrauterine development, the myocardium condenses and the intertrabecular recesses are reduced to capillaries... She weighed 6.8 Kg and was 75.5 cm tall... Cardiovascular examination revealed a decreased-volume peripheral pulse and a third heart sound... The patient evolved with refractory heart failure as a result of severely deteriorated left ventricular function and became a candidate for heart transplantation... The compacted epicardial layer appears as a compact band of uniform tissue while the much thicker endocardial non-compacted layer consists of trabecular meshwork with deep endomyocardial spaces surrounded by exaggerated hypertrophy of the trabeculae... Although it must be emphasized that, strictly speaking, a two layered structure is found only in IVNC, and not in left ventricular hypertrophy (LVH), dilated cardiomyopathy (DCM), or any other condition, it may sometimes appear difficult to define where prominent trabeculation (such as DCM) ends and non-compactation begins... Patients with depressed ventricular function may present signs of heart failure similar to those observed in patients with dilated cardiomyopathy, and which were seen in this infant... Familial cases have been reported, where the mode of inheritance is heterogeneous... In the X-linked form of the disease, a locus has been found on Xq28, and mutations have been reported in G4.5 gene., This genetic linkage is located close to other areas that have been associated with various other myopathies... Jenni et al have described diagnostic echocardiographic criteria for IVNC that include 1) the absence of co-existing cardiac abnormalities; 2) a 2-layered structure of the ventricular wall with an end-systolic ratio of the noncompacted to the compacted layer >2; 3) the finding of this morphologic presentation in apical and in mid-ventricular areas, and 4) direct blood flow from the ventricular cavity into the deep intertrabecular recesses, as assessed by Doppler echo-cardiography... Magnetic resonance imaging and myocardial perfusion scanning can be of additional help in detecting sub-endocardial perfusion defects., A recent resonance magnetic study concluded that a ratio of non-compacted to compacted myocardium greater than 2.3 would reliably the disease entity... A review of the literature identified only 9 patients with IVNC who underwent cardiac transplantation., This infant is the youngest child reported with IVNC and clinical signs of decompensated heart failure to have been successfully treated with heart transplantation... Although a rare entity, isolated ventricular noncompactation can lead to decompensated heart failure in the early infancy and may be successfully treated with heart transplantation.

Show MeSH