Cardiology Research, ISSN 1923-2829 print, 1923-2837 online, Open Access |
Article copyright, the authors; Journal compilation copyright, Cardiol Res and Elmer Press Inc |
Journal website https://www.cardiologyres.org |
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
Tables
Study | Design | Location/country | Follow-up period | Number of patients | Mean age | M/F | Imaging modality | Diagnostic criteria | Treatment 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] | Prospective | Switzerland | 7.9 years (mean) | 132 | 41 | 46/86 | Echocardiography | Jenni | ACE 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] | Prospective | France | 2.33 years (mean) | 105 | 45 | 69/36 | Echocardiography | Jenni | Beta-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 cohort | Italy | 6 - 193 months | 65 | 42 | NS | Electrocardiography | Jenni | Beta-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] | Prospective | USA | 2001 to 2006 | 30 | 39 | 18/12 | Echocardiography | Jenni | Beta-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 study | UK | January 1992 to June 2002 | 45 | 37 | 28/17 | Echocardiography | Jenni | Long-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] | Retrospective | China | 2006 to 2022 | ILVNC = 37 | 39.4 | 16/21 | TTE ± CMR | Jenni or Petersen | ACE 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] | Prospective | France | 5 years | 98 | 46.6 | 58.1%/41.9% | TTE ± CMR | Jenni | NS | Cardiovascular death, cardiovascular events, heart transplantation |
Fazlinezhad et al, 2016 [18] | Case series | Iran | 2010 to 2013 | 42 | 32.9 | 26/16 | Electrocardiogram | Jenni | NS | Echocardiographic and clinical features of ILVNC patients |
Ishige te al, 2006 [19] | Case series | Japan | June 2000 to February 2005 | 99 | 68/31 | Echocardiography | Oechslin and Jenni | NS | Prevalence, clinical characteristic of ILVNC echo cardiographically | |
Stacey et al, 2013 [20] | Retrospective | USA | January 2007 to April 2011 | 122 | 57 | 72/50 | CMR | Oechslin, Petersen, and Jenni | NS | Comparison of ESNCCR, EDNCCR, and TMTMR |
Peters et al, 2012 [21] | Prospective case-control study | Sub-Saharan Africa | July 2009 to December 2010 | ILVNC patients = 54, control = 54 | ILVNC = 45.4, control = 38.9 | ILVNC = 30/24, control = 28/26 | Echocardiography | Jenni | NS | Clinical and echocardiographic features of ILVNC patients |
Tian et al, 2014 [22] | Retrospective | China | March 2003 to April 2012 | 106 | 46 | 83/23 | Echocardiography | Jenni | Carvedilol (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 study | Australia | 78 months (median) | 98 | 47 (median) | 56/42 | CMR | Petersen | NS | Composite of cardiac death, cardiac transplantation, VT/VF, and ischemic stroke |
Oechslin et al, 2000 [24] | Prospective study | Switzerland | January 1984 to December 1998 | 34 | 42 | 25/9 | Echocardiography | Jenni | NS | Characteristics and outcome in ILVNC |
Steffel et al, 2011 [25] | Prospective cohort | Switzerland | January 1995 to November 2008 | 74 | 42 | 53/21 | Echocardiography | Mirvis, Romhilt, Surawicz | Beta-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] | Prospective | Italy | NS | 42 | 46 | 26/16 | CMR | Petersen | Medical 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 series | Italy | April 2006 to December 2008 | 16 | 40 | 11/5 | CMR | Petersen | NS | The impact severity of ventricular noncompaction on LV systolic function in ILVNC |
Cheng et al, 2011 [28] | Case series | China | January 2007 to December 2008 | 28 | 44 | 17/11 | CMR | Jenni | NS | CMR characteristics in patients with ILVNC and its significance in the diagnosis of ILVNC |
Aras et al, 2006 [29] | Retrospective cohort study | Turkey | February 2001 to September 2005 | 67 | 41 | 44/23 | Echocardiography | Ritteret, Oechslin, and Jenni | Antithrombotic 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 |
Study | Selection (Max = 4) | Comparability (Max = 2) | Outcome (Max = 3) | Total score (Max = 9) | Quality |
---|---|---|---|---|---|
Greutmann et al, 2012 [11] | 3 | 2 | 2 | 7 | High |
Habib et al, 2011 [12] | 3 | 1 | 2 | 6 | Moderate |
Lofiego et al, 2007 [13] | 2 | 1 | 2 | 5 | Moderate |
Stanton et al, 2009 [14] | 3 | 2 | 1 | 6 | Moderate |
Murphy et al, 2005 [15] | 2 | 1 | 2 | 5 | Moderate |
Gao et al, 2023 [16] | 3 | 1 | 1 | 5 | Moderate |
Gerard et al, 2022 [17] | 3 | 1 | 2 | 6 | Moderate |
Fazlinezhad et al, 2016 [18] | 3 | 2 | 2 | 7 | High |
Ishige et al, 2006 [19] | 3 | 2 | 2 | 7 | High |
Stacey et al, 2013 [20] | 3 | 1 | 2 | 6 | Moderate |
Peters et al, 2012 [21] | 2 | 1 | 2 | 5 | Moderate |
Tian et al, 2014 [22] | 3 | 2 | 2 | 7 | High |
Femia et al, 2021 [23] | 3 | 1 | 2 | 6 | Moderate |
Oechslin et al, 2000 [24] | 3 | 1 | 2 | 6 | Moderate |
Steffel et al, 2011 [25] | 3 | 1 | 1 | 5 | Moderate |
Nucifora et al, 2011 [26] | 3 | 2 | 1 | 6 | Moderate |
Dellegrottaglie et al, 2012 [27] | 2 | 1 | 2 | 5 | Moderate |
Cheng et al, 2011 [28] | 3 | 1 | 1 | 4 | Moderate |
Aras et al, 2006 [29] | 3 | 2 | 2 | 7 | High |
Study | Death | Heart transplantation | Heart failure/hospitalization for heat failure | Arrhythmias | Systemic embolism | Main 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] | 21 | 6 | 15 | 5 | 5 | Cardiovascular complication HR 20.6 (4.9 - 87.5) |
NYHA III or grater HR 8.8 (3.2 - 24.0) | ||||||
Habib et al, 2011 [12] | 12 | 9 | 33 | 43 | 11 | NYHA 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] | 7 | 8 | 22 | 13 | 3 | NYHA 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] | 3 | NS | NS | 5 | NS | NS |
Murphy et al, 2005 [15] | 1 | NS | 1 | 9 | 2 | NS |
Gao et al, 2023 [16] | 4 | 0 | 2 | 0 | 3 | |
Gerard et al, 2022 [17] | 4 | 8 | 18 | 24 | 5 | NS |
Fazlinezhad et al, 2016 [18] | NS | NS | NS | NS | NS | NS |
Ishige te al, 2006 [19] | NS | NS | NS | NS | NS | NS |
Stacey et al, 2013 [20] | 6 | NS | 35 | 6 | 8 | NS |
Peters et al, 2012 [21] | NS | NS | NS | NS | NS | NS |
Tian et al, 2014 [22] | 24 | 4 | 46 | 12 | 4 | Advanced heart failure, and presence of PH, enlarged left heart with dysfunction, low SBP |
Femia et al, 2021 [23] | 3 | 2 | NS | 3 | 4 | NS |
Oechslin et al, 2000 [24] | 12 | 4 | 18 | 14 | 8 | NYHA, AF, BBB, LVEDD |
Steffel et al, 2011 [25] | 11 | Death + heart transplantation = 11 | 25 | 10 | 6 | Repolarization abnormalities, increase in PQ- (HR 1.032, P = 0.004) and QTc-duration (HR 1.037, P = 0.001) |
Nucifora et al, 2011 [26] | NS | NS | NS | NS | NS | Myocardial fibrosis |
Dellegrottaglie et al, 2012 [27] | NS | NS | NS | NS | NS | Degree of LV dysfunction |
Cheng et al, 2011 [28] | NS | NS | NS | NS | NS | NS |
Aras et al, 2006 [29] | 10 | 0 | 23 | 16 | 6 | NYHA, VT, LVEF, LVEDD |