Isolated Noncompaction of the Left Ventricular Myocardium in an Elderly Patient

Sakuma, Masashi; Hayashi, Terumi; Kamishirado, Hirotoshi; Akiya, Kaori; Takayanagi, Kunio; Morooka, Shigenori

doi:10.1253/circj.68.964

Cited by 15 publications

(8 citation statements)

References 18 publications

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“…Histological work-up of LVHT in an 80-year-old female with metabolic myopathy revealed atheromatous arteries within the trabeculations, some necrotic cardiomyocytes, fibrocytes, hit cells and scars [9]. Fibrosis within the trabeculations is attributed to ischemia by some authors [10]. In the present patient, trabeculations were built up of a regular myocardial texture without an increase in fibrosis or fat tissue.…”

Section: Discussionmentioning

confidence: 58%

Noncompaction in Mitochondrial Myopathy: Visible on Microscopy but Absent on Macroscopic Inspection

Finsterer

Stöllberger²,

Grassberger³

et al. 2013

Cardiology

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Objectives: Disappearance of left ventricular hypertrabeculation (LVHT) over time has been occasionally recognized, but absence on echocardiography and autopsy and presence on histological examination after autopsy has not been reported. Methods: Routine investigations such as chocardiography, cardiac MRI and coronary angiography were applied. Autopsy studies included macroscopic inspection and dissection but also histological work-up. Results: In a 64-year-old male, LVHT was diagnosed at age 51 years during diagnostic work-up for hypertrophic cardiomyopathy. He had a history of mitochondrial myopathy which was diagnosed long before the cardiac problem became evident. Thickening of the left ventricular myocardium increased over years, resulting also in thickening of the trabeculations and the disappearance of the intertrabecular recesses. This is why LVHT was no longer visible on echocardiography shortly before death at age 64 years. The autopsy revealed that macroscopically no LVHT was visible but upon histological work-up the preformed recesses were still visible but had become unfolded. Conclusions: This case shows that LVHT may disappear due to thickening of the trabeculations but may remain visible on postmortem histological examination in patients with hypertrophic cardiomyopathy from a mitochondrial myopathy.

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Section: Discussionmentioning

confidence: 58%

Noncompaction in Mitochondrial Myopathy: Visible on Microscopy but Absent on Macroscopic Inspection

Finsterer

Stöllberger²,

Grassberger³

et al. 2013

Cardiology

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“…1 However, we want to mention the following concerns. The authors say that LVHT is exclusively a congenital disorder and do not seem aware that indivdual patients may acquire LVHT during their lifetime.…”

Section: Uncertainties In Left Ventricular Hypertrabeculation/noncompmentioning

confidence: 99%

Uncertainties in Left Ventricular Hypertrabeculation/Noncompaction

Stöllberger

Finsterer

2005

Circ J

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We read with interest the article by Sakuma et al of a 65-year-old woman in whom echocardiography revealed left ventricular hypertrabeculation/noncompaction (LVHT), and who died 5 years later from heart failure and ventricular fibrillation. 1 However, we want to mention the following concerns. The authors say that LVHT is exclusively a congenital disorder and do not seem aware that indivdual patients may acquire LVHT during their lifetime. Three members of a family who subsequently developed LVHT did not show characteristic changes on the fetal echocardiographic studies and therefore in those cases LVHT developed either postnatally or was not visible on fetal echocardiography. 2 In a further case, LVHT was not echocardiographically present in the first days of life and manifested at the age of 5 weeks. 3 In another case, LVHT was documented to develop during the subject's lifetime. 4,5 Overall, the etiology of LVHT remains unknown.The authors describe LVHT as a rare disorder, but it has to be mentioned that LVHT is frequently overlooked and that its recognition depends on the awareness of the echocardiographers and the available technology. Thus, the prevalence of LVHT is at present also unknown. According to our experience in an echocardiographic laboratory, the prevalence of LVHT in adults is 0.25%/year. 6 The echocardiographic diagnostic criteria for LVHT are only one of several different definitions of LVHT. 7 Why did the authors chose that particular definition, and would all 9 patients also fulfill the other 2 criteria for LVHT? 8,9 It would be interesting to know how the authors differentiated between trabeculations, papillary muscles and tendinal chords when quantifying the extent of the trabecular meshwork at the level of the mitral valve and at the level of the papillary muscles. Were the intertrabecular recesses measurable and detectable at each location of LVHT?Did the morphology of LVHT change during follow-up, as has been reported in a patient with LVHT in whom after successful pharmacological therapy, an increase in left ventricular ejection fraction and a decrease in both left ventricular volume and end-diastolic pressure were associated with a decrease of the non-compacted myocardial areas? 10 The association of LVHT with embolism is in other's and our experience 11,12 not as high as previously reported. 7 Did the patient suffer from any embolic events during her lifetime? Did she receive oral anticoagulants?The oldest patient with LVHT, so far reported, is 80 years old. 13 It would be of interest to know the previous history of the presented patient, her laboratory and electrocardiographic findings and results of neurologic examinations because neuromuscular disorders are a frequent finding in patients with LVHT. 9 Regarding the pathoanatomic and pathohistological findings, it would be of interest to know the size and age of the thrombi and if conductive structures could be detected in the trabeculations. Did the authors recognize the intertrabecular thrombi already intra vitam by echocardiogra...

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“…1 According to previous reports, isolated noncompaction of the left ventricular myocardium (INVM) results from a congenital disorder in which abnormal endomyocardial morphogenesis is caused by abnormal cessation of embryonic development of the left ventricular myocardium. The nomenclature of noncompaction is thus derived from this disorder of embryonic development.…”

Section: Author's Reply Isolated Noncompaction Of the Left Ventriculamentioning

confidence: 99%

Isolated Noncompaction of the Left Ventricular Myocardium in an Elderly Patient: Additional Description

Sakuma

Hayashi

Kamishirado

et al. 2005

Circ J

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We appreciate the interest of Drs Stöllberger and Finsterer in our report. 1 According to previous reports, isolated noncompaction of the left ventricular myocardium (INVM) results from a congenital disorder in which abnormal endomyocardial morphogenesis is caused by abnormal cessation of embryonic development of the left ventricular myocardium. The nomenclature of noncompaction is thus derived from this disorder of embryonic development. The recent development of excellent tools for diagnostic imaging, including echocardiography, CT and MRI, enables easy and clear detection of prominent trabeculations and deep recesses of the ventricles.Cases of hypertrabeculation of the left ventricle (LVHT) have accumulated and some were found to have acquired the condition during their life, not congenitally. We agree with our correspondents that the etiology of LVHT remains unclear. Unfortunately, we were unable to obtain data regarding the patient as a fetus or as a child or young adult. We thus cannot exclude the possibility that she acquired the disease, but could be considered as a mild case of congenital INVM, as she survived to 70 years of age.The prevalence of LVHT in Japan remains unknown, but we consider INVM or LVHT as still rare in Japanese. Ichida et al conducted a nationwide survey in 1996 of INVM among Japanese children, including 150 hospitals in Japan and collected only 27 cases. 2 In our echocardiography laboratory we have encountered only 3 patients who have strictly matched the criteria for INVM as defined by Chin et al 3 from among 55,000 examinations since 1984. The criteria described by Chin et al are the most reliable and generally accepted, 4-7 because they include pathological evidence and offer practical descriptions of how to measure the height of the abnormal trabeculations and the depth of the recesses. 3 Many articles have thus quoted these criteria. We suspect that some differences may exist between Western and Asian peoples.We have taken steps to avoid overlooking LVHT or INVM, and very well trained sonographers and cardiologists have undergone echocardiographic examinations with a special focus on trabeculations. We reported the first cases in an elderly Japanese in 1997. 8 Differentiation between the trabeculations, papillary muscles and tendinal cords was easily achieved with anatomical knowledge. Papillary muscles and tendinal cords connect to the mitral leaflets and move regularly with the cardiac cycle. Two papillary muscles were located under the mitral valves and were detected as round-shaped muscles on the short-axis view at the papillary muscle level in the left ventricle. Tendinal cords are seen as string-like echoes connecting papillary muscles to the mitral valves. Conversely, trabeculations protrude from the ventricular wall into the ventricular cavity and do not connect with tendinal cords or mitral valves. These features are also independent from both papillary muscles and mitral valves, and motion is independent of the mitral valves. Careful observation of the motion of eac...

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Isolated Noncompaction of the Left Ventricular Myocardium in an Elderly Patient

Cited by 15 publications

References 18 publications

Noncompaction in Mitochondrial Myopathy: Visible on Microscopy but Absent on Macroscopic Inspection

Noncompaction in Mitochondrial Myopathy: Visible on Microscopy but Absent on Macroscopic Inspection

Uncertainties in Left Ventricular Hypertrabeculation/Noncompaction

Isolated Noncompaction of the Left Ventricular Myocardium in an Elderly Patient: Additional Description

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