Left Ventricular Noncompaction Cardiomyopathy: Left Ventricular Dilation and Dysfunction at Baseline Portend the Risk of Death or Heart Transplantation

Cardoso, Bárbara; Jeewa, Aamir; Minn, Sunghoon; Ashkanase, Jenna; Lynch, Áine; Jean‐St‐Michel, Emilie

doi:10.1016/j.cjca.2022.01.030

Cited by 6 publications

(4 citation statements)

References 21 publications

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“…Isolated LVNC is generally considered a benign myocardial alteration, with a 5-year incidence of cardiovascular events of <10% [ 17 ]. Although a higher risk of mortality and heart transplantation in children with dilated than isolated LVNC has been reported [ 25 ], studies comparing the long-term prognosis between LVNC subtypes in non-pediatric patients are limited.…”

Section: Introductionmentioning

confidence: 99%

Long-Term Prognosis of Different Subtypes of Left Ventricular Noncompaction Cardiomyopathy Patients: A Retrospective Study in China

Gao,

Zhang,

Wang

et al. 2023

JCDD

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Left ventricular noncompaction (LVNC) is a heterogeneous cardiomyopathy that can be classified into different subtypes based on morphologic and functional features. However, the prognosis of the dilated and isolated subtypes of non-pediatric LVNC remains unknown. We retrospectively studied 101 patients with LVNC diagnosed at Peking Union Medical College Hospital from 2006 to 2022 using the Jenni criteria of transthoracic echocardiography. The patients were grouped into those with dilated LVNC (n = 64) or isolated LVNC (n = 37), and 88 patients (54 with dilated LVNC and 34 with isolated LVNC) were followed up successfully. The primary outcome was major adverse cardiovascular events (a composite of cardiovascular mortality, heart failure, severe ventricular arrhythmia, and systolic embolism). The median follow-up time was 5.24 years. The incidence of major adverse cardiovascular events was 43.2%; patients with dilated LVNC had a higher risk (adjusted hazard ratio, 4.43; 95% confidence interval, 1.24–15.81; p = 0.02) than those with isolated LVNC. None of the isolated LVNC patients had cardiovascular deaths or severe ventricular arrhythmias. The risk of systemic embolism was similar between patients with dilated and isolated LVNC. Our findings indicate that transthoracic echocardiography is a useful tool for classifying LVNC into subtypes with distinct clinical outcomes. Dilated LVNC is associated with a poor prognosis, while the isolated subtype is probably a physiological condition.

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Section: Introductionmentioning

confidence: 99%

Long-Term Prognosis of Different Subtypes of Left Ventricular Noncompaction Cardiomyopathy Patients: A Retrospective Study in China

Gao,

Zhang,

Wang

et al. 2023

JCDD

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“…Our methodology has been described previously. 17 Briefly, patients aged between 0 and 18 years with a diagnosis of HLHS and ventricular dysfunction ≥30 days were considered eligible for inclusion. Patients never seen at SickKids or those who have never had an echocardiogram performed at SickKids were excluded.…”

Section: Methodsmentioning

confidence: 99%

Outcomes of Children With Hypoplastic Left Heart Syndrome and Heart Failure on Medical Therapy

Lynch,

Jeewa,

Minn

et al. 2024

JACC: Advances

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“…The SickKids Heart Failure (HF) database is a retrospective cohort study of pediatric patients with cardiomyopathy or HF seen at a single, quaternary cardiac transplant center, the Hospital for Sick Children, Toronto, ON and diagnosed between January 2001 and July 2018 ( 18 ). Subjects were identified by screening the electronic medical record and the electronic echocardiogram (echo) database for the diagnosis of “restrictive cardiomyopathy”.…”

Section: Methodsmentioning

confidence: 99%

Symptomatic presentation influences outcomes in pediatric restrictive cardiomyopathy

Lorenzo,

Lynch,

Ashkanase

et al. 2023

Front. Pediatr.

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IntroductionChildren with restrictive cardiomyopathy (RCM) traditionally have a poor prognosis, with most patients either dying or requiring heart transplantation within 2 years of diagnosis. The development of symptoms in RCM suggests advanced disease. However, as screening practices evolve and lead to diagnosis of early disease, identifying appropriate timing of transplant listing becomes increasingly important. In this context we compared outcomes of children with RCM presenting with clinical symptoms to those asymptomatic at initial presentation.MethodsThis retrospective cohort study included 25 patients with RCM presenting to a quaternary care center between 2001 and 2018. Times to transplantation, death, and a composite outcome of adverse cardiac events (CPR, cardioversion, inotropic support, mechanical ventilation, mechanical support, or heart transplant) were compared between those symptomatic and asymptomatic at presentation.ResultsAt 2 years following diagnosis, patients asymptomatic at presentation had a significantly better transplant-free survival at 57% compared to 17% for symptomatic patients (p = 0.03). Those asymptomatic at diagnosis also had significantly improved cardiac event-free survival at 71% compared to symptomatic patients at 25% (p = 0.01). In multivariable analysis, cardiac symptoms at presentation remained an independent risk factor for heart-transplant or death [hazard ratio 5.17 (1.28–20.85), p = 0.02].ConclusionPatients with RCM who are symptomatic at time of diagnosis have significantly worse transplant-free survival and cardiac event-free survival. Given current practice variability in timing of transplant listing, the presence of any cardiac symptoms is an important negative prognostic marker and should prompt urgent transplant listing.

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Left Ventricular Noncompaction Cardiomyopathy: Left Ventricular Dilation and Dysfunction at Baseline Portend the Risk of Death or Heart Transplantation

Cited by 6 publications

References 21 publications

Long-Term Prognosis of Different Subtypes of Left Ventricular Noncompaction Cardiomyopathy Patients: A Retrospective Study in China

Long-Term Prognosis of Different Subtypes of Left Ventricular Noncompaction Cardiomyopathy Patients: A Retrospective Study in China

Outcomes of Children With Hypoplastic Left Heart Syndrome and Heart Failure on Medical Therapy

Symptomatic presentation influences outcomes in pediatric restrictive cardiomyopathy

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