Comparison of echocardiographic features of noncompaction of the left ventricle in adults versus idiopathic dilated cardiomyopathy in adults

Sengupta, Partho P.; Mohan, Jagdish C.; Mehta, Vimal; Jain, Vineet; Arora, Ramesh; Pandian, Natesa G.; Khandheria, Bijoy K.

doi:10.1016/j.amjcard.2004.04.046

Cited by 71 publications

(49 citation statements)

References 14 publications

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“…This may not truly reflect the actual age at which the disease manifested because many patients were treated for dilated cardiomyopathy with systolic heart failure prior to being referred to our clinic, where diagnosis of ILVNC was made for the first time. Similar to other studies, 4,15 severe LV dilatation and dysfunction accompanied by heart failure were the major clinical manifestations in our study population. The prevalence of heart failure was much greater in this cohort compared with other series, 4,15-17 because patients were most likely diagnosed later in their disease course and gained access to tertiary cardiac care much later than in other centers because of the resource-deprived environment in which we practice.…”

Section: Discussionsupporting

confidence: 71%

“…The time to clinical presentation of the ILVNC phenotype is highly variable 4,[15][16][17] ; the mean age at diagnosis of this cohort was 45.8 years. This may not truly reflect the actual age at which the disease manifested because many patients were treated for dilated cardiomyopathy with systolic heart failure prior to being referred to our clinic, where diagnosis of ILVNC was made for the first time.…”

Section: Discussionmentioning

confidence: 99%

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Isolated Left Ventricular Noncompaction in Sub-Saharan Africa: A Clinical and Echocardiographic Perspective

Peters

Khandheria²,

Santos³

et al. 2012

Circ: Cardiovascular Imaging

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Background-Isolated left ventricular noncompaction (ILVNC) is a cardiomyopathy caused by intrauterine failure of the myocardium to compact. Common clinical complications are heart failure, arrhythmias, and cardioembolism. A paucity of data exists relating to clinical and echocardiographic features of ILVNC in Africans. Methods and Results-This study is a single-center, prospective case-control study, whereby subjects attending a dedicated cardiomyopathy clinic were screened for and diagnosed with ILVNC, provided they had no other associated structural heart disease and fulfilled all the accompanying echocardiographic criteria: (1) end-systolic ratio of noncompacted layer to compacted layer Ͼ2, (2) presence of Ͼ3 prominent apical trabeculations, and (3) deep intertrabecular recesses that fill with blood from the ventricular cavity visualized using color Doppler ultrasound. Fifty-four subjects were identified, age 45.4Ϯ13.1 years (meanϮSD), 95% confidence interval 3.6 to 10.2, 55.6% male, and 63.0% New York Health Association Class II, and prevalence of LVNC in our clinic was 6.9%, 95% confidence interval 3.6 to 10.2. Heart failure because of systolic dysfunction was the most common clinical presentation (53 subjects, 98.1%). Left ventricular end-diastolic diameter was 61.4Ϯ7.2 mm (meanϮSD) and ejection fraction 26.7Ϯ11.9% (meanϮSD).

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Section: Discussionsupporting

confidence: 71%

Section: Discussionmentioning

confidence: 99%

Isolated Left Ventricular Noncompaction in Sub-Saharan Africa: A Clinical and Echocardiographic Perspective

Peters

Khandheria²,

Santos³

et al. 2012

Circ: Cardiovascular Imaging

View full text Add to dashboard Cite

show abstract

“…Other portions of the ventricle, including midventricular lateral wall midventricular inferior wall, and midposterior wall, may also be involved. [2][3][4] LVNC may occur in the setting of other developmental defects, such as cognitive impairment and developmental delay, where it is referred to as "syndromic LVNC." Nonsyndromic LVNC refers to the absence of any extracardiac defects, and nonsyndromic LVNC may be familial.…”

Section: Clinical Perspective On P 449mentioning

confidence: 99%

Sarcomere Mutations in Cardiomyopathy With Left Ventricular Hypertrabeculation

Dellefave

Pytel

Mewborn

et al. 2009

Circ Cardiovasc Genet

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Background-Mutations in the genes encoding sarcomere proteins have been associated with both hypertrophic and dilated cardiomyopathy. Recently, mutations in myosin heavy chain (MYH7), cardiac actin (ACTC), and troponin T (TNNT2) were associated with left ventricular noncompaction, a form of cardiomyopathy characterized with hypertrabeculation that may also include reduced function of the left ventricle. Methods and Results-We used clinically available genetic testing on 3 cases referred for evaluation of left ventricular dysfunction and noncompaction of the left ventricle and found that all 3 individuals carried sarcomere mutations. The first patient presented with neonatal heart failure and was referred for left ventricular noncompaction cardiomyopathy. Genetic testing found 2 different mutations in MYBPC3 in trans. The first mutation, 3776delA, Q1259fs, rendered a frame shift at 1259 of cardiac myosin-binding protein C and the second mutation was L1200P. The frameshift mutation was also found in this mother who displayed mild echocardiographic features of cardiomyopathy, with only subtle increase in trabeculation and an absence of hypertrophy. A second pediatric patient presented with heart failure and was found to carry a de novo MYH7 R369Q mutation. The third case was an adult patient with dilated cardiomyopathy referred for ventricular hypertrabeculation. This patient had a family history of congestive heart failure, including pediatric onset cardiomyopathy where 3 individuals in the family were found to have the MYH7 mutation R1250W. Conclusion-Genetic testing should be considered for cardiomyopathy with hypertrabeculation. (Circ Cardiovasc Genet.2009;2:442-449.)

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“…The inherited congenital malformation has characteristic findings in the endocardium with the sinusoids, which open to the ventricular chamber [1,2]. Echocardiography is a simple and useful diagnostic procedure, which reveals characteristic trabeculae and deep intratrabecular recesses [3][4][5]. Contrast left ventricular opacification helps to observe the trabeculae when the echocardiogram is not clear [6].…”

Section: Discussionmentioning

confidence: 99%