Cardiology Research, ISSN 1923-2829 print, 1923-2837 online, Open Access
Article copyright, the authors; Journal compilation copyright, Cardiol Res and Elmer Press Inc
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Original Article

Volume 15, Number 5, October 2024, pages 377-395


Prevalence, Clinical Manifestations, and Adverse Outcomes of Left Ventricular Noncompaction in Adults: A Systematic Review and Meta-Analysis

Figures

Figure 1.
Figure 1. PRISMA diagram for the study selection process. PRISMA: Preferred Reporting Items for Systematic Reviews and Meta-Analyses.
Figure 2.
Figure 2. Funnel plot analysis of adverse outcomes in LVNC. LVNC: left ventricular noncompaction; CVD: cardiovascular disease; LVEF: left ventricular ejection fraction; CI: confidence interval; NYHA: New York Heart Association.
Figure 3.
Figure 3. Forest plot for the prevalence of LVNC in adult population. LVNC: left ventricular noncompaction; CI: confidence interval.
Figure 4.
Figure 4. Forest plot for the prevalence of LVNC in adult population on echocardiography. LVNC: left ventricular noncompaction; CI: confidence interval.
Figure 5.
Figure 5. Forest plot for the prevalence of LVNC in adult population on CMR. LVNC: left ventricular noncompaction; CI: confidence interval; CMR: cardiovascular magnetic resonance.
Figure 6.
Figure 6. Forest plot for the prevalence of mortality in adults with LVNC. LVNC: left ventricular noncompaction; CI: confidence interval.
Figure 7.
Figure 7. Forest plot for the incidence of heart transplantation in adults with LVNC. LVNC: left ventricular noncompaction; CI: confidence interval.
Figure 8.
Figure 8. Forest plot for the prevalence of heart failure in adults with LVNC. LVNC: left ventricular noncompaction; CI: confidence interval.
Figure 9.
Figure 9. Forest plot for the prevalence of arrhythmic events in adults with LVNC. LVNC: left ventricular noncompaction; CI: confidence interval.
Figure 10.
Figure 10. Forest plot for the prevalence of thromboembolic events in adults with LVNC. LVNC: left ventricular noncompaction; CI: confidence interval.
Figure 11.
Figure 11. Forest plot for the predictors of death or heart transplantation in adults with LVNC. LVNC: left ventricular noncompaction; CVD: cardiovascular disease; LVEF: left ventricular ejection fraction; NYHA: New York Heart Association; HR: hazard ratio; CI: confidence interval.

Tables

Table 1. Summary of the Characteristics of Included Studies
 
StudyDesignLocation/countryFollow-up periodNumber of patientsMean ageM/FImaging modalityDiagnostic criteriaTreatment strategy (number/% of patients administered)Main outcomes assessed
M/F: male/female; ACE: angiotensin-converting enzyme; ARBs: angiotensin receptor blockers; OAC: oral anticoagulants; VT/VF: ventricular tachycardia/ventricular fibrillation; ILVNC: isolated left ventricular noncompaction; CMR: cardiac magnetic resonance imaging; ICD: implantable cardioverter defibrillator; CRT-D: cardiac resynchronization therapy with defibrillator; ESNCCR: end-systolic noncompacted-to-compacted ratio; EDNCCR: end-diastolic noncompacted-to-compacted ratio; TMTMT: trabecular mass-to-total mass ratio; TTE: transthoracic echocardiography; NS: not specified; AF: atrial fibrillation.
Greutmann et al, 2012 [11]ProspectiveSwitzerland7.9 years (mean)1324146/86EchocardiographyJenniACE inhibitors/ARBs (36%), beta-blockers (38%), and diuretics (26%); OAC (36%) and low-dose aspirin (7%)Survival free from cardiovascular death or transplantation
Habib et al, 2011 [12]ProspectiveFrance2.33 years (mean)1054569/36EchocardiographyJenniBeta-blockers (n = 69), digoxin (n = 23), diuretics (n = 59), ACE inhibitors (n = 73), aldosterone antagonists (n = 27), and vitamin K antagonists (n = 45)Predictors of adverse outcome: death or transplantation
Lofiego et al, 2007 [13]Retrospective cohortItaly6 - 193 months6542NSElectrocardiographyJenniBeta-blockers (n = 29), ACE inhibitors (n = 41), diuretics (n = 38), spironolactone (n = 19), and warfarin (n = 30). In the non-symptom subgroup, beta-blockers (n = 2) patients, ACE inhibitor (n = 5), and warfarin (n = 4). 11 patients in the symptom-based subgroup received implantable devices.Mortality, cardiovascular events, predictors of adverse outcomes
Stanton et al, 2009 [14]ProspectiveUSA2001 to 2006303918/12EchocardiographyJenniBeta-blockers (n = 22), ACE inhibitor/ARBs (n = 18), aspirin (n = 5), warfarin (n = 9), digoxin (n = 8), class III antiarrhythmic (n = 2)Clinical characteristics and outcomes in ILVNC (primary outcome: all-cause mortality; secondary outcome: stroke, onset of AF)
Murphy et al, 2005 [15]Cohort studyUKJanuary 1992 to June 2002453728/17EchocardiographyJenniLong-term anticoagulation (n = 18); ACE inhibitors/ARBs (n = 27); beta-blockers (n = 14), amiodarone (n = 2). 3 patients received biventricular pacemakers.Prognosis and familial incidence of ILVNC
Gao et al, 2023 [16]RetrospectiveChina2006 to 2022ILVNC = 3739.416/21TTE ± CMRJenni or PetersenACE inhibitor/ARBs/angiotensin receptor neprilysin inhibitor (n = 13), aldosterone receptor antagonist (n = 6), beta-blockers (n = 13), diuretics (n = 4), antiarrhythmic drugs (n = 0), antiplatelet drugs (n = 2), anticoagulant drugs (n = 2)Adverse cardiovascular events
Gerard et al, 2022 [17]ProspectiveFrance5 years9846.658.1%/41.9%TTE ± CMRJenniNSCardiovascular death, cardiovascular events, heart transplantation
Fazlinezhad et al, 2016 [18]Case seriesIran2010 to 20134232.926/16ElectrocardiogramJenniNSEchocardiographic and clinical features of ILVNC patients
Ishige te al, 2006 [19]Case seriesJapanJune 2000 to February 20059968/31EchocardiographyOechslin and JenniNSPrevalence, clinical characteristic of ILVNC echo cardiographically
Stacey et al, 2013 [20]RetrospectiveUSAJanuary 2007 to April 20111225772/50CMROechslin, Petersen, and JenniNSComparison of ESNCCR, EDNCCR, and TMTMR
Peters et al, 2012 [21]Prospective case-control studySub-Saharan AfricaJuly 2009 to December 2010ILVNC patients = 54, control = 54ILVNC = 45.4, control = 38.9ILVNC = 30/24, control = 28/26EchocardiographyJenniNSClinical and echocardiographic features of ILVNC patients
Tian et al, 2014 [22]RetrospectiveChinaMarch 2003 to April 20121064683/23EchocardiographyJenniCarvedilol (n = 44), beta-blockers (n = 46), ACE inhibitors/ARBs (n = 76), warfarin (n = 10), spironolactone (n = 82), device implantation (n = 8); ICD (n = 7), CRT-D (n = 1)Prognosis of ILVNC and predictors of adverse outcomes
Femia et al, 2021 [23]Prospective studyAustralia78 months (median)9847 (median)56/42CMRPetersenNSComposite of cardiac death, cardiac transplantation, VT/VF, and ischemic stroke
Oechslin et al, 2000 [24]Prospective studySwitzerlandJanuary 1984 to December 1998344225/9EchocardiographyJenniNSCharacteristics and outcome in ILVNC
Steffel et al, 2011 [25]Prospective cohortSwitzerlandJanuary 1995 to November 2008744253/21EchocardiographyMirvis, Romhilt, SurawiczBeta-blockers (n = 20), ACE inhibitor/ARBs (n = 24), amiodarone (n = 6), aspirin/oral anticoagulation (n = 28)Mortality due to cardiovascular cause or heart transplantation
Nucifora et al, 2011 [26]ProspectiveItalyNS424626/16CMRPetersenMedical therapy for heart failure (n = 19); beta-blockers (n = 17), ACE inhibitors/ARBs (n = 12), and diuretics (n = 9)Prevalence, location, and extent of myocardial fibrosis in patients with ILVNC, relation to clinical status and LV systolic function
Dellegrottaglie et al, 2012 [27]Case seriesItalyApril 2006 to December 2008164011/5CMRPetersenNSThe impact severity of ventricular noncompaction on LV systolic function in ILVNC
Cheng et al, 2011 [28]Case seriesChinaJanuary 2007 to December 2008284417/11CMRJenniNSCMR characteristics in patients with ILVNC and its significance in the diagnosis of ILVNC
Aras et al, 2006 [29]Retrospective cohort studyTurkeyFebruary 2001 to September 2005674144/23EchocardiographyRitteret, Oechslin, and JenniAntithrombotic therapy (all patients), spironolactone (18 patients), cardiac glycosides (22 patients) loop diuretics (20 patients), ACE-inhibitors (38 patients), beta-blockers (43 patients), Amiodarone (9 patients) Anticoagulant therapy with warfarin (19 patients)Prevalence, electrocardiographic abnormalities, complications, mortality

 

Table 2. Quality Assessment of Included Studies Using Newcastle-Ottawa Scale
 
StudySelection (Max = 4)Comparability (Max = 2)Outcome (Max = 3)Total score (Max = 9)Quality
Greutmann et al, 2012 [11]3227High
Habib et al, 2011 [12]3126Moderate
Lofiego et al, 2007 [13]2125Moderate
Stanton et al, 2009 [14]3216Moderate
Murphy et al, 2005 [15]2125Moderate
Gao et al, 2023 [16]3115Moderate
Gerard et al, 2022 [17]3126Moderate
Fazlinezhad et al, 2016 [18]3227High
Ishige et al, 2006 [19]3227High
Stacey et al, 2013 [20]3126Moderate
Peters et al, 2012 [21]2125Moderate
Tian et al, 2014 [22]3227High
Femia et al, 2021 [23]3126Moderate
Oechslin et al, 2000 [24]3126Moderate
Steffel et al, 2011 [25]3115Moderate
Nucifora et al, 2011 [26]3216Moderate
Dellegrottaglie et al, 2012 [27]2125Moderate
Cheng et al, 2011 [28]3114Moderate
Aras et al, 2006 [29]3227High

 

Table 3. Clinical Manifestations, Adverse Outcomes, and Predictors of LVNC in Adults
 
StudyDeathHeart transplantationHeart failure/hospitalization for heat failureArrhythmiasSystemic embolismMain predictors of adverse outcomes (death or transplantation)
LVNC: left ventricular noncompaction; AF: atrial fibrillation; NYHA: New York Heart Association; LVEF: left ventricular ejection fraction; VT: ventricular tachycardia; LV: left ventricular; LBBB: left bundle branch block; LVEDD: left ventricular end-diastolic diameter; LAD: left atrial diameter; HR: hazard ratio; SBP/DBP: systolic/diastolic blood pressure; PH: pulmonary hypertension; LVFS: left ventricular fractional shortening; VES: ventricular ectopic beat; HR: hazard ratio; NS: not specified.
Greutmann et al, 2012 [11]2161555Cardiovascular complication HR 20.6 (4.9 - 87.5)
NYHA III or grater HR 8.8 (3.2 - 24.0)
Habib et al, 2011 [12]129334311NYHA III or IV HR 6.69 (2.21 - 20.21)
Elevated LV filling pressure HR 7.59 (2.20 - 26.14)
LVEF HR 0.93 (0.89 - 0.98)
Hospitalization for heart failure HR 13.55 (4.81 - 38.14)
Lofiego et al, 2007 [13]7822133NYHA class III-IV 5.8 HR (2.0 - 17.0)
Sustained ventricular arrhythmias HR 6.1 (1.6 - 23.1)
Left atrial size (cm) HR 2.5 (1.5 - 4.4)
LVEF HR 0.9 (0.8 - 1.0)
AF HR 3.3 (0.7 - 15.2)
LBBB HR 0.50 (0.1 - 1.6)
Age HR 1.007 (0.98 - 1.04)
Male sex HR 1.5 (0.5 - 4.9)
Number of non-compacted segments > 5 HR 0.4 (0.2 - 1.9)
Stanton et al, 2009 [14]3NSNS5NSNS
Murphy et al, 2005 [15]1NS192NS
Gao et al, 2023 [16]40203
Gerard et al, 2022 [17]4818245NS
Fazlinezhad et al, 2016 [18]NSNSNSNSNSNS
Ishige te al, 2006 [19]NSNSNSNSNSNS
Stacey et al, 2013 [20]6NS3568NS
Peters et al, 2012 [21]NSNSNSNSNSNS
Tian et al, 2014 [22]24446124Advanced heart failure, and presence of PH, enlarged left heart with dysfunction, low SBP
Femia et al, 2021 [23]32NS34NS
Oechslin et al, 2000 [24]12418148NYHA, AF, BBB, LVEDD
Steffel et al, 2011 [25]11Death + heart transplantation = 1125106Repolarization abnormalities, increase in PQ- (HR 1.032, P = 0.004) and QTc-duration (HR 1.037, P = 0.001)
Nucifora et al, 2011 [26]NSNSNSNSNSMyocardial fibrosis
Dellegrottaglie et al, 2012 [27]NSNSNSNSNSDegree of LV dysfunction
Cheng et al, 2011 [28]NSNSNSNSNSNS
Aras et al, 2006 [29]10023166NYHA, VT, LVEF, LVEDD