Noncompaction of the myocardium associated with Roifman syndrome

2001 ◽  
Vol 11 (2) ◽  
pp. 240-243 ◽  
Author(s):  
Karen Mandel ◽  
Eyal Grunebaum ◽  
Lee Benson
Keyword(s):  
Interventricular Septum ◽  
Retinal Dystrophy ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Antibody Deficiency ◽  
Left Ventricular Myocardium ◽  
Facial Dysmorphism ◽  
Ventricular Wall ◽  
Immune Deficiencies ◽  
The Right

Noncompaction of the ventricular myocardium, sometimes referred to as “spongy myocardium”, appears as excessive and prominent trabeculations and deep intratrabecular recesses within the ventricular wall, usually involving the left ventricle, although the right ventricle and interventricular septum can also be affected. It may occur with or without additional heart malformations. Roifman syndrome is a constellation of antibody deficiency, spondyloepiphyseal dysplasia, facial dysmorphism, growth retardation, and retinal dystrophy. We report a patient with Roifman syndrome who presented with noncompaction of the left ventricular myocardium. Our findings expand the spectrum of diseases associated with noncompaction. The recognition of noncompaction among patients with Roifman syndrome is important, as the immune deficiencies may be subtle and undiagnosed until adulthood. Thus, some patients may first present with cardiac failure.

scholarly journals Remodeling of the heart and main vessels in patients with marfanoid habitus

2020 ◽  
Vol 10 (5) ◽  
pp. 27-34
Author(s):  
Eugene V. Timofeev ◽  
Eduard G. Malev ◽  
Nina N. Parfenova ◽  
Eduard V. Zemtsovsky
Keyword(s):  
Marfan Syndrome ◽  
Aortic Root ◽  
Structural Changes ◽  
Systolic Function ◽  
Interventricular Septum ◽  
Age Groups ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Control Group ◽  
Left Ventricular Myocardium

For many hereditary connective tissue disorders (HCTD), especially Marfan syndrome, remodeling of the heart and main vessels is described, which is manifested by a decrease in the systolic function of the left ventricle and expansion of the thoracic aorta. Evaluation of morphometric characteristics of the heart and main vessels in patients with other HCTD, in particular marfanoid habitus (MH) has not been previously carried out. Materials and methods. Weexamined 90 young men and 74 young women between the ages of 18 to 25 years, 111 patients older age groups with stable over coronary heart disease (mean age 64.66.2 years) and 9 patients with verified Marfan syndrome (mean age 27.99.3years). All survey phenotypic and performed anthropometric survey identifying bone signs of dysembryogenesis as well as Echocardiography study on standard protocol. The results.Patients with MH as compared with control group revealed a relatively larger diameter of aortic root (30.44.7 vs 28.03.6 mm,p= 0.03) and the ascending aorta (26.64.9 vs 24.63.2 mm,p= 0.05). Also young with MH turned out to be significantly thicker myocardium of left ventricular posterior wall (8.30.8 vs 7.71.1 mm,p= 0.02) and interventricular septum (8.81.2vs 8.21.1mm,p= 0.04). When performing correlation analysis identified reliable positive correlation between such highly specialized bone signs as high palate (r= 0.31), infundibular deformation of the chest (r= 0.43), arachnodactyly (r= 0.45) andZ-test (p 0.05 for all). Expansion of the aorta (Z-criterion 2.0) have found 24% of older patients with MH. Conclusion.Inpatients with MH revealed significant structural changes of heart and main vessels which are progredient character thickening of the left ventricular myocardium and expansion of the aortic root.

scholarly journals Possibly Late-Onset Arrhythmogenic Right Ventricular Cardiomyopathy: Unique Triglyceride Deposition by Analysis of Lipid Contents

2019 ◽  
Vol 12 ◽  
pp. 117954761982871
Author(s):  
Kentaro Yamamoto ◽  
Xin Guo ◽  
Ken-ichi Mizutani ◽  
Nozomu Kurose ◽  
Motona Kumagai ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Late Onset ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
Cholesterol Content ◽  
Left Ventricular ◽  
Left Ventricular Myocardium ◽  
Ventricular Cardiomyopathy ◽  
Lipid Contents ◽  
The Right

We presented an unusual arrhythmogenic right ventricular cardiomyopathy (ARVC) case of a late-60s elderly man’s death, due to severe pericardial/pleural effusion and ascites, and arrhythmic events, with unique pathological features. The hypertrophic heart grossly displayed yellowish to yellow-whitish predominantly in the variably thinned wall of the dilated right ventricle. Microscopic findings showed diffuse fatty/fibrofatty replacement in not only the right but left ventricular myocardium, together with an outer lymphoplasmacytic infiltrate. According to the lipid contents analysis, the triglyceride content, but not the cholesterol content, in our patient’s right and left ventricular cardiac muscle was much higher than that in the control subject. We propose that this unique triglyceride deposition in our possibly late-onset ARVC case might be one of new clues to understand its enigmatic cause. Further prospective studies are needed to validate the presence and significance of a greater volume of triglyceride deposit, after collecting and investigating a larger number of early and late-onset ARVC cases examined.

Absence of the left ventricular myocardium in a newborn

1996 ◽  
Vol 6 (4) ◽  
pp. 344-347 ◽  
Author(s):  
Angelika Lindinger ◽  
Yvonne Masur ◽  
Hans-Gerhard Limbach
Keyword(s):  
Aortic Valve ◽  
Left Ventricle ◽  
Ventricular Myocardium ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Left Ventricular ◽  
Adolescent And Young Adult ◽  
Left Ventricular Myocardium ◽  
Fatty Replacement ◽  
Myocardial Fibers ◽  
The Right

SummaryAbsence of myocardial fibers in the right ventricle is the essence of so-called Uhl's anomaly, which should be distinguished from the fatty replacement producing arrhythmogenic right ventricular dysplasia of the adolescent and young adult. In this report, we describe a newborn with nearly complete absence of the myocardium of the left ventricle. The infant died on the seventh day because of myocardial incompetence of the left ventricle, which was unable to open the aortic valve.

scholarly journals Ventricular Myocardial Sheet

2018 ◽  
Vol 14 (1) ◽  
pp. 50-57
Author(s):  
Mostafa R. Ali
Keyword(s):  
Left Ventricle ◽  
Right Ventricle ◽  
Muscle Fibers ◽  
Single Layer ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Ventricular Wall ◽  
Myocardial Layers ◽  
Running Pattern ◽  
The Right

Background: Despite the fact that the exact architecture and orientation of ventricular myocardium are critical to cardiac functions either in health or disease, it is still debated. Objectives: Anatomical demonstration of the ventricle myocardium (VM)as a single, long and continuous muscular sheet and this muscular sheet can be dived into 3-segments. As a new anatomical concept the left ventricle is a triple layers wall; whether the right ventricle is a single layer wall.Histological demonstration of different directions of muscle-fibers at each layer of ventricular myocardium. Type of the study: Cross- sectional study. Methods: In this study 100-heart (fish, chicken, goat, sheep and cow) were dissected and analyzed. Dental lacrona and wax knife used majorly in the dissection, boiling of the hearts with distilled water and finally opening them by the “opening-technique”. Results: Ventricular myocardium is a single, long and continuous muscular sheet in 100-samples of different species which had been included in the study (passing from the fish toward the cow). VMS can be divided into 3-segments in (100% of cow, 95% of goat and 85% of sheep). The left ventricle is a triple layers wall; whether the right ventricle is single layer wall, this result observed in (100% of cow, 95% of goat and 85% of sheep).Finally different directions of muscle fibers observed at each layer of ventricular myocardium where the subendocardial layer shows transverse running pattern of muscle fibers, mesocardial layer shows longitudinal running pattern of muscle fibers and subepicardial layer shows mixed running patterns of muscle fibers. Conclusion: Ventricular myocardium is single, long and continuous muscular sheet. This sheet consists of 3-segments. These segments coils in spiral track and form the triple layers left ventricular wall and the single layer right ventricular wall. By histological examination of ventricular myocardial layers different directions of muscle fibers observed at each layer.

Effect of erythrocyte deformability on myocardial hematocrit gradient

1995 ◽  
Vol 268 (1) ◽  
pp. H260-H264 ◽  
Author(s):  
O. K. Baskurt ◽  
M. Edremitlioglu ◽  
A. Temiz
Keyword(s):  
Blood Cell ◽  
Red Blood Cell ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Erythrocyte Deformability ◽  
Control Group ◽  
Left Ventricular Myocardium ◽  
Ventricular Wall ◽  
Linear Gradient ◽  
Endocardial Layer

Myocardial hematocrit gradient was determined between epicardium and endocardium of the left ventricular wall in rat heart under the influence of erythrocyte deformability alterations. Hematocrit determinations were performed by measuring two different radionuclides labeling plasma (125I-labeled albumin) and erythrocytes (99mTc) in 100-microns-thick left ventricular myocardium slices. Myocardial hematocrit gradient calculated after exchange transfusions with partially hardened red blood cell suspensions was compared with the results of the control group, in which the exchange transfusions were done using normal, hematocrit-matched blood. In the control group, the hematocrit value in the myocardium adjacent to epicardium was 0.331 +/- 0.076 l/l and decreased to 0.232 +/- 0.054 l/l near the endocardium. Myocardial hematocrit between these two was represented by a linear gradient. In the group with impaired erythrocyte deformability, the hematocrit value was 0.359 +/- 0.074 l/l in the epicardial myocardium and remained at 0.341 +/- 0.082 l/l in the endocardial layer. These results indicate that tissue hematocrit gradient in the left ventricular myocardium may be disturbed if erythrocyte deformability is altered.

scholarly journals Quantified growth of the human embryonic heart

Biology Open ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. bio057059 ◽  
Author(s):  
Jaeike W. Faber ◽  
Jaco Hagoort ◽  
Antoon F. M. Moorman ◽  
Vincent M. Christoffels ◽  
Bjarke Jensen
Keyword(s):  
Heart Development ◽  
Three Dimensional ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Embryonic Heart ◽  
Growth Patterns ◽  
Right Atrial ◽  
Left Ventricular Myocardium ◽  
Atrioventricular Canal ◽  
The Right

ABSTRACTThe size and growth patterns of the components of the human embryonic heart have remained largely undefined. To provide these data, three-dimensional heart models were generated from immunohistochemically stained sections of ten human embryonic hearts ranging from Carnegie stage 10 to 23. Fifty-eight key structures were annotated and volumetrically assessed. Sizes of the septal foramina and atrioventricular canal opening were also measured. The heart grows exponentially throughout embryonic development. There was consistently less left than right atrial myocardium, and less right than left ventricular myocardium. We observed a later onset of trabeculation in the left atrium compared to the right. Morphometry showed that the rightward expansion of the atrioventricular canal starts in week 5. The septal foramina are less than 0.1 mm2 and are, therefore, much smaller than postnatal septal defects. This chronological, graphical atlas of the growth patterns of cardiac components in the human embryo provides quantified references for normal heart development. Thereby, this atlas may support early detection of cardiac malformations in the foetus.This article has an associated First Person interview with the first author of the paper.

scholarly journals Left sided arrhythmogenic ventricular dysplasia in siblings

Heart ◽  
2001 ◽  
Vol 86 (2) ◽  
pp. 128-130
Author(s):  
C G De Pasquale ◽  
W F Heddle
Keyword(s):  
Sudden Cardiac Death ◽  
Left Ventricle ◽  
Cardiac Death ◽  
Fatty Infiltration ◽  
Ventricular Myocardium ◽  
Arrhythmogenic Right Ventricular Dysplasia ◽  
Left Ventricular ◽  
Left Ventricular Myocardium ◽  
The Family ◽  
The Right

A 32 year old man with no previous medical history suffered a sudden cardiac death. Post mortem examination revealed circumferential fibro-fatty infiltration of the left ventricular myocardium. Histological appearance was characteristic of arrhythmogenic right ventricular dysplasia but unusual for its localisation only to the left ventricle. As a result of this sudden cardiac death the family of the deceased was screened for cardiac disease. A brother of the index case was 36 years old and free of cardiac history and symptoms. Cardiac investigations revealed a functionally and electrically abnormal left ventricle with apparent sparing of the right ventricle. The brothers may have a left sided form of arrhythmogenic ventricular dysplasia and illustrate the importance of screening family members of young victims of sudden cardiac death.

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