Predictors of Adverse Outcome in Adolescents and Adults With Isolated Left Ventricular Noncompaction

Greutmann, Matthias; Mah, May Ling; Silversides, Candice K.; Klaassen, Sabine; Jost, Christine H. Attenhofer; Jenni, Rolf; Oechslin, Erwin

doi:10.1016/j.amjcard.2011.08.043

Cited by 106 publications

(72 citation statements)

References 20 publications

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“…16 As in all series of patients with LVNC the absence of generally accepted diagnostic criteria for this disease entity remains a dilemma. While the criteria proposed by our group may be too sensitive, particularly in black individuals, they may identify symptomatic patients of a Caucasian population at high risk for cardiovascular complications.…”

Section: Discussionmentioning

confidence: 99%

“…4,5 These criteria have been shown to identify a subset of symptomatic patients at risk for adverse outcome. 15 In brief, the criteria include a combination of 1) segmental thickening of the left ventricular myocardial wall 2) consisting of an inner non-compacted and an outer compacted layer with 3) a thickness ratio ≥ 2:1 at end-systole, measured in the were assessed according to established guidelines for two-dimensional echocardiography.…”

Section: Patientsmentioning

confidence: 99%

“…[5][6][7][8][9][10][11][12][13][14][15] Typical complications include congestive heart failure, ventricular arrhythmias and thromboembolic events. [12][13][14]16,17 Recently, the association of LVNC with metabolic diseases, genetic syndromes and other cardiac abnormalities has been reported. [18][19][20] Genetic studies have shown sarcomere gene mutations in some patients with isolated LVNC similar to those with dilated or hypertrophic cardiomyopathy, and a recent study has found similar mutations in patients with Ebstein anomaly.…”

Section: Since Its First Description Left Ventricular Non-compactionmentioning

confidence: 99%

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Left ventricular non‐compaction: Prevalence in congenital heart disease

Stähli

Gebhard

Biaggi

et al. 2013

International Journal of Cardiology

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129

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INTRODUCTION: Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy, originally described as an isolated disease without other structural cardiac abnormalities. The aim of this study was to explore the prevalence of LVNC among adults with different types of congenital heart disease. METHODS: From our databases we identified adults with congenital heart disease who fulfilled diagnostic criteria for LVNC. We report frequencies of associated congenital cardiac defects and the prevalence of LVNC among patients with different congenital heart defects. RESULTS: From a total of 202 patients with LVNC, 24 patients (12%; mean age 32±11years, 19 males) had additional congenital cardiac defects. Associated defects were left ventricular outflow tract abnormalities in 11 patients (46%), including 7 uni-or bicuspid aortic valves; two aortic coarctations; one diffuse aortic hypoplasia and one subaortic stenosis, Ebstein anomaly in 6 patients (25%), tetralogy of Fallot in two (8%), and double outlet right ventricle in one patient (4%). In our cohort, the prevalence of LVNC was highest among patients with Ebstein anomaly (6/40, 15%), followed by aortic coarctation (2/60, 3%), tetralogy of Fallot (3/129, 2%) and uni-or bicuspid aortic valves (7/963, 1%). CONCLUSION: In adults, various forms of congenital heart disease are associated with LVNC, particularly stenotic lesions of the left ventricular outflow tract, Ebstein anomaly, and tetralogy of Fallot. In the future, studying these patients in more depth may provide a better understanding of the interplay between genetic and hemodynamic factors that lead to the phenotype of LVNC.

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Section: Discussionmentioning

confidence: 99%

Section: Patientsmentioning

confidence: 99%

Section: Since Its First Description Left Ventricular Non-compactionmentioning

confidence: 99%

See 1 more Smart Citation

Left ventricular non‐compaction: Prevalence in congenital heart disease

Stähli

Gebhard

Biaggi

et al. 2013

International Journal of Cardiology

Self Cite

129

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“…However, the presence of heart failure, history of sustained ventricular tachycardia or systemic thromboembolism seem to be associated with an unfavourable prognosis among other phenotypes with distinct outcomes [21,22]. In a recent study, mortality did not differ significantly between patients with isolated LVNC and control patients with dilated cardiomyopathy, [24] suggesting that LV dysfunction rather than the phenotype itself is the risk-increasing mechanism.…”

Section: Clinical Manifestationsmentioning

confidence: 98%

“…Studies of older children and adults have reported a high incidence of severe manifestations [7,17,[19][20][21][22] such as LV dysfunction, thromboembolic events, which probably originate in the deep intertrabecular recesses, arrhythmias, and sudden death. Other studies, however, have found a much lower incidence of complications, suggesting subclinical or milder cases [23].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Left Ventricular Noncompaction

Almeida¹

2017

Cardiomyopathies - Types and Treatments

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Left ventricular noncompaction (LVNC) is accepted as an unclassified (the American Heart Association) or a genetic cardiomyopathy (the European Society of Cardiology), but some argue that this phenotype may be a morphologic trait shared by different cardiomyopathies. This chapter covers the state of the art on the pathology, underlying mechanisms, its clinical manifestations, and diagnosis and treatment modalities of LVNC. LVNC may be defined as follows: an inner non-compacted layer with prominent left ventricular trabeculae and deep intertrabecular recesses and a thin outer compacted layer. Mechanisms are still debatable, with the hypothesis of compaction arrest during embryogenesis as the most accepted theory. Genetic data support LVNC as a distinct cardiomyopathy, although evidence for LVNC as a shared morphological trait is not ruled out, since LVNC may be associated with other cardiomyopathies, congenital heart diseases and in some cases may be acquired. Diagnosis is based on imaging and may be confirmed by the use of genetics. Clinical picture and prognosis and the management options are discussed.

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Biventricular imaging markers to predict outcomes in non‐compaction cardiomyopathy: a machine learning study

et al. 2020

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Aims Left ventricular non-compaction cardiomyopathy (LVNC) is a genetic heart disease, with heart failure, arrhythmias, and embolic events as main clinical manifestations. The goal of this study was to analyse a large set of echocardiographic (echo) and cardiac magnetic resonance imaging (CMRI) parameters using machine learning (ML) techniques to find imaging predictors of clinical outcomes in a long-term follow-up of LVNC patients. Methods and results Patients with echo and/or CMRI criteria of LVNC, followed from January 2011 to December 2017 in the heart failure section of a tertiary referral cardiologic hospital, were enrolled in a retrospective study. Two-dimensional colour Doppler echocardiography and subsequent CMRI were carried out. Twenty-four hour Holter monitoring was also performed in all patients. Death, cardiac transplantation, heart failure hospitalization, aborted sudden cardiac death, complex ventricular arrhythmias (sustained and non-sustained ventricular tachycardia), and embolisms (i.e. stroke, pulmonary thromboembolism and/or peripheral arterial embolism) were registered and were referred to as major adverse cardiovascular events (MACEs) in this study. Recruited for the study were 108 LVNC patients, aged 38.3 ± 15.5 years, 48.1% men, diagnosed by echo and CMRI criteria. They were followed for 5.8 ± 3.9 years, and MACEs were registered. CMRI and echo parameters were analysed via a supervised ML methodology. Forty-seven (43.5%) patients had at least one MACE. The best performance of imaging variables was achieved by combining four parameters: left ventricular (LV) ejection fraction (by CMRI), right ventricular (RV) end-systolic volume (by CMRI), RV systolic dysfunction (by echo), and RV lower diameter (by CMRI) with accuracy, sensitivity, and specificity rates of 75.5%, 77%, 75%, respectively. Conclusions Our findings show the importance of biventricular assessment to detect the severity of this cardiomyopathy and to plan for early clinical intervention. In addition, this study shows that even patients with normal LV function and negative late gadolinium enhancement had MACE. ML is a promising tool for analysing a large set of parameters to stratify and predict prognosis in LVNC patients.

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Predictors of Adverse Outcome in Adolescents and Adults With Isolated Left Ventricular Noncompaction

Cited by 106 publications

References 20 publications

Left ventricular non‐compaction: Prevalence in congenital heart disease

Left ventricular non‐compaction: Prevalence in congenital heart disease

Left Ventricular Noncompaction

Biventricular imaging markers to predict outcomes in non‐compaction cardiomyopathy: a machine learning study

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