Disappearance of Left Ventricular Hypertrabeculation/Noncompaction and Sudden Death in a Patient With Turner Mosaic Syndrome

Altenberger, Johann; Hasenauer, Georg; Granitz, Marcel; Stöllberger, Claudia; Finsterer, Josef

doi:10.1016/j.amjcard.2012.02.070

Cited by 8 publications

(7 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Disappearance of LVHT, like in the presented patient, has also been observed in association with a decrease of systolic function in an adult with Turner mosaic syndrome and coronary heart disease [7]. In another LVHT-patient with mitochondrial myopathy, LVHT was neither visible at follow-up echocardiography nor by pathoanatomic macroscopic inspection, but only at histologic investigation [8].…”

Section: Contents Lists Available At Sciencedirectmentioning

confidence: 78%

Disappearance of left ventricular hypertrabeculation/noncompaction in vacuolar non-neuromuscular cardiomyopathy

Stöllberger¹,

Kolussi²,

Hackl³

et al. 2015

International Journal of Cardiology

Self Cite

View full text Add to dashboard Cite

Section: Contents Lists Available At Sciencedirectmentioning

confidence: 78%

Disappearance of left ventricular hypertrabeculation/noncompaction in vacuolar non-neuromuscular cardiomyopathy

Stöllberger¹,

Kolussi²,

Hackl³

et al. 2015

International Journal of Cardiology

Self Cite

View full text Add to dashboard Cite

“…Risks and clinical outcomes of LVNC are heterogeneous, ranging from no symptoms to arrhythmias and major events such as heart failure, thromboembolism, and sudden cardiac death. LVNC has been previously reported in only three adult patients with Turner syndrome [5–7]. We report the first pediatric case of LVNC diagnosed in a female with Turner syndrome.…”

Section: Introductionmentioning

confidence: 82%

Left Ventricular Noncompaction in a Child with Turner Syndrome

Bhatia

Qasim

Frank

et al. 2019

Case Reports in Pediatrics

View full text Add to dashboard Cite

Congenital heart disease (CHD) may cause a significant comorbidity in patients with Turner syndrome. The commonly reported CHD in these patients includes bicuspid aortic valve and coarctation of the aorta. Left ventricular noncompaction (LVNC) is a rare form of cardiomyopathy that has been reported in literature only three times in adult patients with Turner syndrome. We report the first case of a 6-year-old asymptomatic female with Turner syndrome who was referred for cardiac evaluation after her Turner syndrome diagnosis. Echocardiogram was suspicious for LVNC, which was confirmed on cardiac magnetic resonance imaging.

show abstract

“…A second explanation assumed that LVHT ‘disappeared' because of enlargement of the endocardial surface, and thus, increased left ventricular outflow and compensation of systolic dysfunction were no longer required [2]. In a second patient with Turner syndrome, ‘disappearance' of LVHT was again attributed to recovery from systolic dysfunction after successful coronary bypass grafting [3]. ‘Disappearance' of LVHT 10 years after its first recognition on echocardiography in the presented patient was explained by another mechanism, i.e.…”

Section: Discussionmentioning

confidence: 99%

“…Disappearance of LVHT over time has been occasionally recognized [2,3], but absence on echocardiography and autopsy and presence on histological examination after autopsy has not been reported.…”

Section: Introductionmentioning

confidence: 99%

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

Finsterer

Stöllberger²,

Grassberger³

et al. 2013

Cardiology

Self Cite

View full text Add to dashboard Cite

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.

show abstract

Disappearance of Left Ventricular Hypertrabeculation/Noncompaction and Sudden Death in a Patient With Turner Mosaic Syndrome

Cited by 8 publications

References 4 publications

Disappearance of left ventricular hypertrabeculation/noncompaction in vacuolar non-neuromuscular cardiomyopathy

Disappearance of left ventricular hypertrabeculation/noncompaction in vacuolar non-neuromuscular cardiomyopathy

Left Ventricular Noncompaction in a Child with Turner Syndrome

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

Contact Info

Product

Resources

About