Neuromuscular and cardiac comorbidity determines survival in 140 patients with left ventricular hypertrabeculation/noncompaction.
BACKGROUND
The prognosis of patients with left ventricular hypertrabeculation/noncompaction (LVHT) is controversial. We assessed cardiologic and neurologic predictors for mortality in LVHT patients and how many received implantable cardioverters/defibrillators(ICD) or cardiac resynchronization devices (CRT).


RESULTS
Included were patients with LVHT diagnosed echocardiographically between June 1995 and May 2009. All patients underwent a baseline cardiologic examination, and were invited for a neurological investigation. During June 2009, the patients were contacted by telephone and their records were screened if they had received ICD or CRT. In 140 patients (29% females, mean age 53 ± 16, range 14-94 years) LVHT was diagnosed. The neurologic investigation, carried out in 76%, disclosed a neuromuscular disorder of definite (n = 22) or unknown (n = 68) etiology or was normal (n = 16). During a follow-up of 4.5 years the mortality was 5.7%/year. Causes of death were heart failure(n = 11), pneumonia (n = 6), sudden cardiac death (n = 3), malignancy (n = 3), pulmonary embolism(n = 2), sepsis (n = 2), stroke (n = 2), hepatic failure (n = 1) or unknown (n = 6). Sixteen patients received devices (ICD n = 4, CRT n = 3, ICD plus CRT n = 9). Predictors for mortality were increased age (p = 0.0307), neuromuscular disorder of definite or unknown etiology (p = 0.0063), exertional dyspnea (p =0.0018), edema (p = 0.0000), heart failure (p = 0.0002), ventricular ectopic beats (p = 0.0119), atrial fibrillation (p = 0.0000), low voltage (p = 0.0139), presence of one or more ECG abnormalities (p = 0.0420), left ventricular fractional shortening <25% (p = 0.0046), extension of LVHT (p = 0.0063) and LVHT affecting the lateral wall (p = 0.0110).


CONCLUSIONS
Mortality in LVHT is high and due to cardiac and neuromuscular comorbidity, why monitoring and therapy, including device therapy, should be improved.
2nd Medical Department, Krankenanstalt Rudolfstiftung, Juchgasse 25, A-1030 Wien, Austria. claudia.stoellberger@chello.at
Claudia
C
Stöllberger
Gerhard
G
Blazek
Christian
C
Wegner
Maria
M
Winkler-Dworak
Josef
J
Finsterer
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002318:Cardiovascular Diseases / Q000453:epidemiology / Q000401:mortality; D015897:Comorbidity; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000453:epidemiology / Q000401:mortality* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009468:Neuromuscular Diseases / Q000453:epidemiology / Q000401:mortality* / Q000503:physiopathology; D015996:Survival Rate / Q000639:trends; D055815:Young Adult