PMID- 24085471 OWN - NLM STAT- MEDLINE DCOM- 20150522 LR - 20211021 IS - 1615-2573 (Electronic) IS - 0910-8327 (Linking) VI - 29 IP - 5 DP - 2014 Sep TI - Isolated left ventricular noncompaction: clinical profile and prognosis in 106 adult patients. PG - 645-52 LID - 10.1007/s00380-013-0409-z [doi] AB - This study was undertaken to evaluate the clinical course of isolated left ventricular noncompaction (ILVNC) and to identify the predictors for adverse outcomes in an adult cohort with ILVNC. Between March 2003 and April 2012, 106 adult patients diagnosed with ILVNC at Fuwai Hospital were included in this study. The medical history, electrocardiograms, and echocardiograms of these patients were retrospectively analyzed by chart review. Of these patients, 64 (60 %) were in New York Heart Association (NYHA) functional class III/IV and 84 (79 %) had systolic dysfunction (left ventricular ejection fraction (LVEF) <50 %). During a follow-up of 2.9 +/- 2.1 years, 28 (26 %) patients died or underwent heart transplantation. The annual incidence of death or transplantation was 9.1 %. The determinants of death or heart transplantation included NYHA functional class III/IV (hazard ratio (HR) 4.52; 95 % confidence interval (CI) 1.57-13.04; P = 0.005), decreased left ventricular ejection fraction (HR 0.94; 95 % CI 0.90-0.97; P = 0.001), dilated left ventricular end-diastolic diameter (HR, 1.06; 95 % CI, 1.02-1.09; P = 0.001), increased left atrial diameter (HR 1.08; 95 % CI 1.03-1.14; P = 0.001), reduced systolic blood pressure (HR 0.96; 95 % CI 0.94-0.99; P = 0.003), the presence of pulmonary hypertension (HR 3.50; 95 % CI 1.63-7.51; P = 0.001), and right bundle branch block (HR 7.79; 95 % CI 2.56-23.76; P < 0.001). In conclusion, this study demonstrates that ILVNC is related to a high incidence of death or heart transplantation. Advanced heart failure, a dilated left heart with systolic dysfunction, reduced systolic blood pressure, pulmonary hypertension, and right bundle branch block predict adverse outcomes of ILVNC. FAU - Tian, Tao AU - Tian T AD - Department of Cardiology, State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, No. 167 Beilishi Road, Beijing, 100037, People's Republic of China, tiantaophd@163.com. FAU - Liu, Yaxin AU - Liu Y FAU - Gao, Linggen AU - Gao L FAU - Wang, Jizheng AU - Wang J FAU - Sun, Kai AU - Sun K FAU - Zou, Yubao AU - Zou Y FAU - Wang, Linping AU - Wang L FAU - Zhang, Lin AU - Zhang L FAU - Li, Yuehua AU - Li Y FAU - Xiao, Yan AU - Xiao Y FAU - Song, Lei AU - Song L FAU - Zhou, Xianliang AU - Zhou X LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20131002 PL - Japan TA - Heart Vessels JT - Heart and vessels JID - 8511258 SB - IM MH - Adolescent MH - Adult MH - Aged MH - Arterial Pressure MH - Bundle-Branch Block/diagnosis/physiopathology MH - China MH - Disease Progression MH - Echocardiography MH - Electrocardiography MH - Female MH - Heart Failure/diagnosis/physiopathology MH - Heart Transplantation MH - Humans MH - Hypertension, Pulmonary/diagnosis/physiopathology MH - Hypertrophy, Left Ventricular/diagnosis/physiopathology MH - Isolated Noncompaction of the Ventricular Myocardium/*diagnosis/mortality/physiopathology/therapy MH - Kaplan-Meier Estimate MH - Male MH - Middle Aged MH - Predictive Value of Tests MH - Prognosis MH - Proportional Hazards Models MH - Retrospective Studies MH - Risk Factors MH - Stroke Volume MH - Time Factors MH - Ventricular Dysfunction, Left/diagnosis/physiopathology MH - Ventricular Function, Left MH - Young Adult EDAT- 2013/10/03 06:00 MHDA- 2015/05/23 06:00 CRDT- 2013/10/03 06:00 PHST- 2013/06/09 00:00 [received] PHST- 2013/08/30 00:00 [accepted] PHST- 2013/10/03 06:00 [entrez] PHST- 2013/10/03 06:00 [pubmed] PHST- 2015/05/23 06:00 [medline] AID - 10.1007/s00380-013-0409-z [doi] PST - ppublish SO - Heart Vessels. 2014 Sep;29(5):645-52. doi: 10.1007/s00380-013-0409-z. Epub 2013 Oct 2.