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Gerhard Blazek

Publications and source records attributed to Gerhard Blazek.

10 recordsLinked to original sources
PubMed2006

Prognosis of left ventricular hypertrabeculation/noncompaction is dependent on cardiac and neuromuscular comorbidity.

BACKGROUND AND OBJECTIVES: Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality frequently associated with neuromuscular disorders (NMD). The data about long-term prognosis of patients with LVHT are controversial. Aim of the study in a cohort of LVHT patients was to assess the long-term prognosis regarding mortality, cardiac and neuromuscular comorbidity. METHODS AND RESULTS: In 86 patients LVHT was diagnosed echocardiographically between June 1995 and December 2004 (21 female, mean age 52 +/- 14, range 14-94 years). All patients underwent a baseline cardiologic investigation and were invited for a neurologic investigation. A specific NMD was diagnosed in 21, a NMD of unknown etiology in 33, the neurologic investigation was normal in 13 and 19 patients refused. During a mean follow-up of 51 months (range 3-106 months) the mortality rate was 5.3%/year. Predictors for an increased mortality were increased age (p=0.0134), presence of NMD (p=0.0324), exertional dyspnoea (p=0.0329), edema (p=0.0049), heart failure (p=0.0048), left anterior hemiblock (p=0.0078) and a left ventricular fractional shortening <25% (p=0.0648). CONCLUSION: The mortality of LVHT in adult patients depends on cardiac and neurologic comorbidity. Predictors for mortality are increased age, neuromuscular disorder, heart failure, left ventricular dilatation and systolic dysfunction.

Adolescent↗
PubMed2006

Cardiologic and neurologic findings in left ventricular hypertrabeculation/noncompaction relating to echocardiographic indication.

BACKGROUND: Left-ventricular hypertrabeculation/noncompaction (LVHT) is characterized by prominent trabeculations and intertrabecular recesses. LVHT is usually diagnosed if a patient is referred for echocardiography. The study assessed if cardiologic and neurologic findings differ relating to indication for echocardiography. METHODS: Included were patients in whom LVHT was diagnosed in one echocardiographic laboratory between June 1995 and December 2005. All patients underwent a cardiologic examination and were invited for a neurological investigation. RESULTS: LVHT was diagnosed in 93/35,181 patients (26 female, 53+/-15 years). Heart failure was the most frequent indication (n=49), followed by chest pain (n=21), syncope (n=8), search for cardiac involvement in myopathy (n=7), stroke or embolism (n=3), arterial hypertension (n=3) and screening of LVHT patients' relatives (n=2). Patients with the indication "heart failure" were older than patients with other indications (59.4+/-13.1 versus 44.9+/-12.9 years, p<0.001), had more often a neuromuscular disorder of unknown etiology (53% versus 32%, p<0.05), exertional dyspnoea (96% versus 32%, p<0.001), edema (25% versus 7%, p<0.05) and advanced heart failure (NYHA III: 41% versus 11%, p<0.01; NYHA IV: 29% versus 9%, p<0.05), suffered less often from arterial hypertension (22% versus 41%, p<0.05), angina pectoris (14% versus 34%, p<0.05) and palpitations or syncope (10% versus 30%, p<0.05). Patients with the indication "heart failure" had less frequent normal ECG than patients with other indications (2% versus 18%, p<0.01), had more frequent > or = 2 ECG abnormalities (57% versus 36%, p<0.05), left bundle branch block (29% versus 9%, p<0.05), a larger left-ventricular enddiastolic diameter (69.9+/-9.7 versus 57.4+/-12.2 mm, p<0.001), a lower left-ventricular fractional shortening (16.9+/-6.1% versus 31.1+/-11.5%, p<0.001) and more often valvular abnormalities (76% versus 30%, p<0.001). Location and extension of LVHT did not differ between indication groups. CONCLUSION: Echocardiographers should be aware of LVHT in any indication for echocardiography.

Adolescent↗
PubMed2005

Neuromuscular implications in left ventricular hypertrabeculation/noncompaction.

This review focuses on recent advances in the association between left ventricular hypertrabeculation/noncompaction (LVHT), a form of unclassified cardiomyopathy, and neuromuscular disorders (NMD). So far, LVHT has been found in single patients with dystrophinopathy, dystrobrevinopathy, laminopathy, zaspopathy, myotonic dystrophy, infantile glycogenosis type II (Pompe's disease), myoadenylate-deaminase deficiency, mitochondriopathy, Barth syndrome, Friedreich ataxia, and Charcot-Marie-Tooth disease. Most frequently LVHT is found in patients with Barth syndrome and mitochondrial disorders. The prevalence of LVHT in NMD patients is not known. On the contrary, NMD can be detected in up to four fifths of the patients with LVHT. Because LVHT is associated with an increased risk of rhythm abnormalities and heart failure, it is essential to detect LVHT as soon as possible. Because of adequate therapeutic options, all patients with NMD should undergo a comprehensive cardiological examination as soon as their neurological diagnosis is established. In reverse, all patients with LVHT should undergo a comprehensive neurological investigation following the detection of LVHT.

Heart Defects, Congenital↗
PubMed2005

Age-dependency of cardiac and neuromuscular findings in left ventricular noncompaction.

BACKGROUND: Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality characterized by prominent trabeculations and intertrabecular recesses, and frequently associated with neuromuscular disorders (NMD). Initially described in children and young adults, LVHT has been found also in elderly. Aim of the study was to assess the age-dependency of clinical, electrocardiographic (ECG) and echocardiographic findings, and whether they differ according to the neurologic diagnosis. METHODS AND RESULTS: In 86 patients LVHT was diagnosed echocardiographically between June 1995 and December 2004 (65 male, median age 52 years, range 14-94). All patients underwent a baseline cardiologic investigation and were invited for a neurologic investigation. A specific NMD was diagnosed in 21 (metabolic myopathy, n = 14; Leber's hereditary optic neuropathy, n = 3; myotonic dystrophy, n = 2; Becker muscular dystrophy, n = 1; Duchenne muscular dystrophy, n = 1), a NMD of unknown etiology in 32. The neurologic investigation was normal in 13 and was refused by 20 patients. Patients above median age had more often heart failure (43% versus 95%, p < 0.02) and valvular abnormalities (71% versus 36%, p < 0.02) than below median age. Patients >71 years had more often exertional dyspnoea (39% versus 81%, p < 0.02), left bundle branch block (9% versus 38%, p = 0.031), larger left-ventricular enddiastolic diameters (68 versus 60 mm, p < 0.02) and a lower left-ventricular fractional shortening than patients <34 years (18% versus 29%, p < 0.02). No age-dependency was detected regarding location and extension of LVHT and the neurologic diagnosis. CONCLUSION: There is no age-dependent typical pattern of LVHT regarding clinical, echocardiographic and neurologic findings. Echocardiographers should be aware of this cardiac abnormality when investigating patients of any age.

Adolescent↗
PubMed2004

Left ventricular hypertrabeculation/noncompaction with and without neuromuscular disorders.

BACKGROUND: Left ventricular hypertrabeculation/noncompaction (LVHT) is frequently associated with neuromuscular disorders (NMD). It is unknown, whether LVHT patients differ according to the presence or absence of NMD. Aim of the study was to assess, if clinical, ECG or echocardiographic findings differ between LVHT patients with and without NMD. METHODS: Included were all patients, in whom LVHT was diagnosed between June 1995 and February 2003 in one echocardiographic laboratory. All patients underwent a cardiologic examination and were invited for a neurologic investigation. RESULTS: Of 77 patients with LVHT (19 female, mean age 52 years), 59 were investigated neurologically. Eleven were neurologically normal, 21 had a definite NMD (metabolic myopathy, n=15; Leber's hereditary optic neuropathy, n=3; myotonic dystrophy, n=2 and Becker muscular dystrophy, n=1). The remaining 27 had a NMD of unknown etiology. Neurologically normal patients had more often anginal chest pain than patients with definite NMD (64% vs. 14%, P=0.0042) or NMD of unknown etiology (64% vs. 26%, P=0.0157). Neurologically normal patients were more often in NYHA class 0 or I than patients with NMD of unknown etiology (64% vs. 26%, P=0.0289) and had a thinner interventricular septum than patients with NMD (10.6 mm vs. 12.8 mm, P=0.0253). CONCLUSIONS: Cardiac abnormalities are hardly different between patients with and without NMD.

Electrocardiography↗