Left Ventricular Non-Compaction Cardiomyopathy-Still More Questions than Answers

Paluszkiewicz, Jerzy; Milting, Hendrik; Kałużna‐Oleksy, Marta; Pyda, Małgorzata; Janus, Magdalena; Körperich, Hermann; Piran, Misagh

doi:10.3390/jcm11144135

Cited by 14 publications

(23 citation statements)

References 77 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…offers an enhanced sensitivity, as CMR can detect myocardial trabeculations that can be enhanced with steady-state free-precession (SSFP) cine imaging [53,54]. In CMR, the myocardial trabeculations, apex region and end-diastole ratio can be well monitored over time.…”

Section: Schematic Overview Of Influencing Factors In Myocardial Nonc...mentioning

confidence: 99%

Emerging Hallmarks of Mitochondrial Biochemistry in Cardiac Trabecular Morphogenesis and Left Ventricular Noncompaction (LVNC)

Mahendran¹,

Schwarz²

2024

New Insights on Cardiomyopathy

View full text Add to dashboard Cite

Functioning as a pivotal platform for energy production and transduction, mitochondria generate ATP to meet the dynamic demands of embryonic development. Consequently, disruption or alteration in mitochondrial activity influences not only cellular status, but also can impact organ formation. Disrupted mitochondrial performance not only impairs cardiovascular function but can also disrupt cardiac maturation through prevention of the myocardium’s transition between the trabeculation to the compaction phase. During embryonic development, proliferating cardiomyocytes create a trabecular mesh network. Gradual compaction of this network transforms the intra-trabecular spaces into the capillaries of the coronary circulation. Achievement of functional compaction and ultimately normal cardiac function is dependent in part on mitochondrial well-being with failure to complete remodeling of the inner trabecular layer contributing to disrupted endocardial vasculature and fibrosis, left ventricular noncompaction (LVNC). LVNC, commonly associated with mitochondrial genetic alterations, is speculated to occur due to an interruption during the process of compaction at the early developmental stages of the left ventricle (LV). Mitochondrial mutations, remain the common etiology of LVNC with a wide spectrum of these genes associated with other cardiomyopathies related to LVNC. Understanding the impact that mitochondrial genetic alterations have on the evolution of cardiac noncompaction could provide new treatment opportunities.

show abstract

Section: Schematic Overview Of Influencing Factors In Myocardial Nonc...mentioning

confidence: 99%

Emerging Hallmarks of Mitochondrial Biochemistry in Cardiac Trabecular Morphogenesis and Left Ventricular Noncompaction (LVNC)

Mahendran¹,

Schwarz²

2024

New Insights on Cardiomyopathy

View full text Add to dashboard Cite

show abstract

“…LV noncompaction (LVNC) is another rare, hereditary cardiomyopathy characterized by an embryogenic defect resulting in myocardial trabeculation [45,46]. Most data regarding LVNC in pregnancy are limited to case reports and small single-center cohorts.…”

Section: Noncompaction Cardiomyopathymentioning

confidence: 99%

Considerations for specific cardiomyopathies during pregnancy

Abrams

Magun

DeFilippis

2023

Current Opinion in Cardiology

View full text Add to dashboard Cite

Purpose of reviewMaternal mortality in the United States continues to increase. Cardiovascular disease, and in particular, cardiomyopathy and heart failure, serves as one of the principal causes of pregnancy-related mortality. The goal of this review is to summarize current knowledge on various cardiomyopathies in pregnancy, with an emphasis on cardiomyopathies beyond the most common in pregnancy, peripartum cardiomyopathy.Recent findingsAlthough existing literature is somewhat limited, outcome studies of cardiomyopathy in pregnancy have demonstrated increased risk for adverse cardiovascular events and maternal morbidity and mortality. Herein, we review and synthesize recent studies focusing on preconception risk and counseling, pharmacologic management, and maternal outcomes in various cardiomyopathies during pregnancy including: dilated cardiomyopathy, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, noncompaction cardiomyopathy, and chemotherapy-induced cardiomyopathy.SummaryIn an effort to optimize maternal outcomes, it is critical to recognize the risk associated with various cardiomyopathies in pregnancy and understand the tools for risk stratification and antepartum management. Further research on less common cardiomyopathies in pregnancy is warranted to provide more standardized recommendations.

show abstract

“…Отличается ли прогноз НКМП от других форм КМП? Является ли НКМП независимым фактором риска инсульта и системных тромбоэмболий [1,2]?…”

unclassified

“…Клиническая картина НКМП отличается значительным разнообразием и варьирует от минимальных симптомов до терминальной стадии СН, летальных аритмий, внезапной сердечной смерти (ВСС) и тромбоэмболических осложнений (ТЭО) (инсульт, транзиторная ишемическая атака, периферические эмболии, мезентериальные и почечные тромбозы, инфаркт миокарда) [1,2,[10][11][12][13][14]. Частота тромбоэмболий у больных с НКМП составляет примерно 15%.…”

unclassified

“…Таким образом, пациенты с НКМП имеют широкий спектр клинических проявлений -от бессимптомной формы до симптомов тяжелой СН, от клинически незначимой экстрасистолии до жизнеугрожающих аритмий/ВСС, ФП и ТЭО [1][2][3][4][5][6][7][8]. В большинстве опубликованных исследований авторы анализировали когорты НКМП в целом, без разделения их на подтипы, что приводило к погрешностям при сравнении фенотипов и генотипов, к неоднозначной оценке клинических исходов и прогноза НКМП в разных работах и, в итоге, -к противоречивым результатам [13,17,18].…”

unclassified

See 1 more Smart Citation

Non-compaction and dilated cardiomyopathy: genotypic, phenotypic and prognostic differences

et al. 2022

View full text Add to dashboard Cite

Aim. To study and compare genotypic and phenotypic signs in patients with non-compaction cardiomyopathy (NCM) and dilated cardiomyopathy (DCM), to conduct a comparative analysis of clinical outcomes and 5-year cumulative survival of patients with NCM and DCM.Material and methods. The study included 144 unrelated patients with cardiomyopathy: NCM (n=74) and DCM (n=70). Mean age was 39 [30; 49] years (men, 96 (66,7%); left ventricular ejection fraction (LVEF) was 30,5 [24; 46]%. A comprehensive clinical examination included electrocardiography, Holter monitoring, echocardiography, cardiac magnetic resonance imaging, coronary angiography, DNA diagnostics (NGS+Sanger), cascade screening, and cosegregation analysis. To assess clinical outcomes, the NCM group was divided into 2 subtypes according to baseline LV systolic function (NCM/DCM phenotype — 50 individuals with LVEF ≤49%; and isolated NCM — 24 patients with LVEF ≥50%). The following adverse events were assessed as the composite endpoint: cardiovascular death, heart transplantation (HT), sustained ventricular tachycardia, ventricular fibrillation, successful cardiopulmonary resuscitation, cerebral thromboembolism. The follow-up period was 62 months.Results. Among patients with LVEF ≤49% at a 5-year follow-up, 37 (74,0%) of 50 patients with the NCM/DCM phenotype and 41 (58,6%) of 70 patients with DCM achieved composite endpoint. Out of 24 patients with NCM with LVEF ≥50% (median LVEF, 56 [51; 61]%), 2 (8,3%) patients achieved composite endpoint (χ2=28,8; p=0,001). In the NCM/DCM group with LVEF ≤49%, a higher level of pathogenic genetic variants (64% vs 41,4%/DCM vs 29,2%/NCM; χ2=11,4; p=0,003), cerebral thromboembolism (χ2=11,8; p=0,003) and HT (χ2=10,6; p=0,005). The results of the 5-year survival analysis (Kaplan-Meier) showed a worse prognosis for NCM with LVEF ≤49% compared with DCM (log rang: χ2=11,5; p=0,001) and isolated NCM (log rang: χ2=17,02; p=0,0001). In the overall cohort (n=144), gene-positivity was also associated with worse prognosis (log rang: χ2=5,38; p=0,02).Conclusion. In the present study, patients with dilated subtype of NCM showed a worse prognosis compared with DCM and isolated NCM. Heart failure progression and cerebral thromboembolism were the most common complications in patients with NCM/DCM.

show abstract

Left Ventricular Non-Compaction Cardiomyopathy-Still More Questions than Answers

Cited by 14 publications

References 77 publications

Emerging Hallmarks of Mitochondrial Biochemistry in Cardiac Trabecular Morphogenesis and Left Ventricular Noncompaction (LVNC)

Emerging Hallmarks of Mitochondrial Biochemistry in Cardiac Trabecular Morphogenesis and Left Ventricular Noncompaction (LVNC)

Considerations for specific cardiomyopathies during pregnancy

Non-compaction and dilated cardiomyopathy: genotypic, phenotypic and prognostic differences

Contact Info

Product

Resources

About