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Left Ventricular Noncompaction

LVNC


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]
Respiratory Distress
  • At three months he was admitted with failure to thrive, lethargy and respiratory distress due to heart failure. Cardiac studies revealed dilated cardiomyopathy with a spongiform trabeculated pattern of the left ventricle.[ncbi.nlm.nih.gov]
Failure to Thrive
  • At three months he was admitted with failure to thrive, lethargy and respiratory distress due to heart failure. Cardiac studies revealed dilated cardiomyopathy with a spongiform trabeculated pattern of the left ventricle.[ncbi.nlm.nih.gov]
  • Less common presentations included cyanosis, syncope, dysmorphic features, or failure to thrive.[dx.doi.org]
Macrocephaly
  • 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]
  • . & Azak, E. 3-HMG coenzyme A lyase deficiency: macrocephaly and left ventricular noncompaction with a novel mutation . Indian J. Pediatr. 82 , 645–648 (2015). 198. Egan, K. R. , Ralphe, J. C. , Weinhaus, L. & Maginot, K. R.[nature.com]
Cesarean Section
  • Results Family A The proband (AII.2) was a white male, born after an uncomplicated term gestation by an emergent cesarean section for low heart rate; he was intubated briefly. His birth weight was 7lb 14oz.[dx.doi.org]
Anuria
  • At 11 days of life, he presented in cardiogenic shock with profound hypotension, anuria, and acidosis. His hospital course included inotropic support that was subsequently weaned, allowing him to be discharged briefly.[dx.doi.org]
Tremor
  • Neurologic examination revealed positional tremor and generally reduced tendon reflexes. During 8 weeks, his condition improved under pharmacotherapy.[ncbi.nlm.nih.gov]
Lethargy
  • At three months he was admitted with failure to thrive, lethargy and respiratory distress due to heart failure. Cardiac studies revealed dilated cardiomyopathy with a spongiform trabeculated pattern of the left ventricle.[ncbi.nlm.nih.gov]

Workup

  • Subsequent cardiology workup revealed hypertrabeculation vs LVNC.[ncbi.nlm.nih.gov]
  • Her preliminary workup including holter monitor, urine toxicology, vasculitis screen, hypercoagulable screen, and lumbar puncture were unrevealing.[neurology.org]
  • 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]
Left Axis Deviation
  • Verapamil-sensitive fascicular ventricular tachycardia is a rare arrhythmogenic condition characterized by a right bundle-branch block pattern and left-axis deviation with a relatively narrow QRS complex.[ncbi.nlm.nih.gov]
  • Other common findings included isolated or diffuse T-wave inversion (39%), ST-segment abnormalities or strain (34%), atrial enlargement (19%), and left-axis deviation (9%).[dx.doi.org]
Hypoketotic Hypoglycemia
  • HMG-CoA lyase deficiency should be thought in the patients with hypoketotic hypoglycemia, hyperammonemia, elevated liver function tests, noncompaction left ventricle and characteristic white matter changes and in the differential diagnosis of macrocephaly[ncbi.nlm.nih.gov]

Treatment

  • Medical and surgical treatment is similar to other systolic dysfunction cardiomyopathies and depends on the presenting clinical manifestations.[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

  • 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]

Etiology

  • Left ventricular noncompaction is an unusual but increasingly recognized cardiomyopathy, the etiology of which is still not definitely established.[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]
  • The remainder of the exhaustive etiological assessment was not contributory. No biological thrombophilia was found.[karger.com]

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]
  • Noncompaction cardiomyopathy is caused by a 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: marakhonov@generesearch.ru Laboratory of Genetic Epidemiology[doi.org]
  • The epidemiology of childhood cardiomyopathy in Australia. N Engl J Med. 2003 ; 348 :1639–1646. Crossref Medline Google Scholar 13. Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, Moss AJ, Seidman CE, Young JB.[dx.doi.org]
  • American Heart Association; Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; Council on Epidemiology[journals.lww.com]
Sex distribution
Age distribution

Pathophysiology

  • This finding may be related to the increased arrhythmic risk observed in this cardiomyopathy, giving a new insight into the pathophysiology of LVNC.[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.[oadoi.org]

Prevention

  • Although ICD is an effective option for preventing SCD, data on the long-term follow-up of patients with LVNC is limited.[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]

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