Presentation
Time zero for survival analysis was at time of diagnosis/presentation (only factors present at the time of diagnosis were included). [dx.doi.org]
Discordance for congenital heart disease was observed in the twins: Twin A presented with pulmonary atresia with an intact ventricular septum, critical tricuspid stenosis, and severe right-ventricular hypoplasia, whereas twin B presented with mild mitral [ncbi.nlm.nih.gov]
Last year, she presented a renal arterial embolism requiring a thrombectomy. [karger.com]
Entire Body System
- Coronary Atherosclerosis
Eight patients underwent catheterization to assess the presence of coronary atherosclerosis: 2 had abnormal examination, and 1 patient had complications of coronary artery disease, with previous history of acute myocardial infarction. [onlineijcs.com]
Neurologic
- Stroke
Abstract Strokes in young patients may be the clinical expression of many complex and extremely rare diseases. Uncommon causes constitute less than 5% of all strokes, but are present in 30% of strokes in young patients. [karger.com]
The mitral regurgitant fraction (MRF) was calculated by dividing the difference between the RV and LV stroke volumes by the LV stroke volume. The total papillary muscle (TPM) area was measured from the mid short-axis view. [ncbi.nlm.nih.gov]
It is often associated with other congenital cardiac abnormalities, arrhythmias or heart failure, and has been described as an etiology for stroke. [neurology.org]
- Seizure
The authors report case of an 8-mo-old infant with HMG-CoA lyase deficiency, who presented with macrocephaly, left ventricular noncompaction, recurrent pulmonary infections, nonketotic hypoglycemia, seizure and metabolic acidosis. [ncbi.nlm.nih.gov]
The patient developed low cardiac output, atrial fibrillation with high ventricular response (heart rate 160 bpm), worsening level of consciousness, hypotension and seizures, requiring orotracheal intubation and synchronized electrical cardioversion. [elsevier.pt]
[[Anoikis]] [[Anomia]] [[Anophthalmia Esophageal Genital Syndrome]] [[Anorektale Malformation]] [[Anorektische Wirkstoffe]] [[Anorexia nervosa]] [[Anorexie]] [[Anorganische Biochemie]] [[Anorthoscopic perception]] [[Anosognosie]] [[Anoxic epileptic seizures [kidney.de]
Stöllberger C, Huber JO, Enzelsberger B, Finsterer J, et al.Fatal outcome of epileptic seizure-induced takotsubo syndrome with left ventricular rupture. Eur J Neurol. 2009;16:e116–7. PubMed CrossRef 83. [springermedizin.at]
Seizures, ataxia, and ocular abnormalities can occur. LGMD2T - GDP-mannose pyrophosphorylase B (GMPPB ) mutation. [emedicine.medscape.com]
Workup
Subsequent cardiology workup revealed hypertrabeculation vs LVNC. [ncbi.nlm.nih.gov]
However, comprehensive workup with medical information from the past revealed a different cause of cardiomyopathy. In 2010, the patient had already been referred to our hospital because of dyspnea. [circ.ahajournals.org]
Her preliminary workup including holter monitor, urine toxicology, vasculitis screen, hypercoagulable screen, and lumbar puncture were unrevealing. [neurology.org]
Blocks
- Complete Left Bundle Branch Block
There have been reported cases presenting with atrioventricular block, left bundle branch block and sudden cardiac death11. [romanianjournalcardiology.ro]
Treatment
Effects of β-blocker treatment in LVNC require validation in prospective controlled studies. [ncbi.nlm.nih.gov]
Treatment for other types of cardiomyopathy - if someone has LVNC alongside another type of cardiomyopathy, they may need additional treatment. If someone does not have any symptoms, they will not usually require any treatment. [cardiomyopathy.org]
Prognosis
Whether prognosis can be improved by early diagnosis-treatment. Comprehension of the poor genotype-phenotype correlation. [escardio.org]
HF at diagnosis and LVPWC hypoplasia are major determinants of poor prognosis. KEYWORDS: Heart failure; Infants; Juveniles; Noncompaction; Prognosis [Indexed for MEDLINE] Free full text [ncbi.nlm.nih.gov]
Prognosis of left ventricular hypertrabeculation/noncompaction is dependent on cardiac and neuromuscular comorbidity. Int J Cardiol 2007;121:189-93. 10. Kohli SK, Pantazis AA, Shah JS, et al. [bcs.com]
This case stresses the importance of an accurate diagnosis of stroke causes, as it can be associated with poor prognosis. LVNC, rare as it may be, should be considered as a possible cause of stroke in young adults. [karger.com]
Etiology
Left ventricular noncompaction is an unusual but increasingly recognized cardiomyopathy, the etiology of which is still not definitely established. [ncbi.nlm.nih.gov]
The remainder of the exhaustive etiological assessment was not contributory. No biological thrombophilia was found. [karger.com]
It is often associated with other congenital cardiac abnormalities, arrhythmias or heart failure, and has been described as an etiology for stroke. [neurology.org]
Epidemiology
This state-of-the-art review discusses new insights into the basic mechanisms leading to LVNC, its clinical manifestations, treatment modalities, anatomy and pathology, embryology, genetics, epidemiology, and imaging. [ncbi.nlm.nih.gov]
[…] novel in‐frame desmin ( DES ) deletion mutation within the 1A coiled‐coil rod segment leading to a severe filament assembly defect Corresponding Author E-mail address: [email protected] http://orcid.org/0000-0002-0972-5118 Laboratory of Genetic Epidemiology [doi.org]
Association scientific statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology [genome.jp]
The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med. 2003; 348(17):1639-46. doi: 10.1056/ NEJMoa021737 [ Links ] 9. Kovacevic-Preradovic T, Jenni R, Oechslin EN, Noll G, Seifert B, Attenhofer Jost CH. [scielo.edu.uy]
Pathophysiology
The pathophysiology of left ventricular dysfunction, which becomes apparent beyond the 4th decade of life, is unclear. [ncbi.nlm.nih.gov]
Ryan, Pathophysiology of Cardiomyopathies, Fetal and Neonatal Physiology, 10.1016/B978-0-323-35214-7.00154-2, (1563-1575.e4), (2017). Z. Khuchua and J.A. [dx.doi.org]
Prevention
Evidence-based recommendations for preventing thromboembolic events in isolated left ventricular noncompaction have not been established. [ncbi.nlm.nih.gov]
The primary goal is to improve cardiac function and to prevent symptoms. Medications are used to treat symptoms, reverse heart damage and to prevent ongoing damage to the heart muscle. [cincinnatichildrens.org]