ABSTRACT
BACKGROUND: Left ventricular non-compaction (LVNC) is a rare form of cardiomyopathy resulting from a disorder of endomyocardial morphogenesis associated with significantly increased risk of cardiovascular morbidity and premature mortality. Despite the widespread use of echocardiography, LVNC is commonly overlooked, often due to lack of knowledge about this disorder. METHODS AND RESULTS: A complex diagnostic process and follow-up was analysed in 24 patients diagnosed with LVNC between March 2002 and February 2016 (16 boys, 8 girls; age at presentation 9 days - 18 years; follow-up 2-7 years). 17 patients were initially overlooked and followed-up for different diagnoses. After retrospective evaluation by a senior specialist in paediatric cardiology, LVNC was identified in 3 patients initially diagnosed with dilated cardiomyopathy, 11 patients followed-up with various forms of arrhythmias, and 3 patients with congenital heart disease. The diagnosis of LVNC was confirmed using magnetic resonance imaging in all patients. The classical triad of complications - heart failure, ventricular arrhythmias and systemic embolic events - was not confirmed in this study, electrocardiographic findings were abnormal in 87.5% of patients. Isolated non-compaction of the left ventricular myocardium was a dominant form of non-compaction. CONCLUSIONS: The high variability of morphological findings and clinical manifestations of LVNC results in frequent overlooking of this disorder. Therefore, it is important to make the specialists more familiar with this condition and its pathology. Magnetic resonance imaging represents a conducive method to make correct diagnosis of LVNC under several specific conditions, particularly in case of non-conclusive echocardiographic finding.
Subject(s)
Delayed Diagnosis/statistics & numerical data , Isolated Noncompaction of the Ventricular Myocardium/diagnosis , Isolated Noncompaction of the Ventricular Myocardium/epidemiology , Magnetic Resonance Imaging , Adolescent , Child , Child, Preschool , Diagnosis, Differential , Female , Follow-Up Studies , Humans , Infant , Infant, Newborn , Male , Predictive Value of Tests , Retrospective Studies , Risk Factors , Ventricular Dysfunction, Left/diagnosis , Ventricular Dysfunction, Left/epidemiologyABSTRACT
OBJECTIVES: Prognosis of patients with anomalous origin of the left coronary artery from pulmonary artery has dramatically improved as a result of both, early diagnosis and improvements in surgical techniques. Post surgical complications are rare and most patients show quick improvement of the left ventricular performance after repair with complete functional recovery within one year after surgery. Exercise-induced electrocardiographic changes have been found in patients postoperatively and scars and perfusion deficits of the left ventricle may not be detected by standard echocardiographic evaluation. METHODS: Authors present 6 cases of anomalous origin of the left coronary artery from pulmonary artery observed at Martin Univesity Hospital and Pediatric Cardiology Clinic over the last eight-year period. In order to assess the presence of myocardial injury, cardiovascular magnetic resonance imaging with late gadolinium enhancement technique was performed in all 6 cases one year after surgical correction. RESULTS: One patient died 1.5 year after surgical treatment. One year after surgery, the heart size and myocardial functions returned to normal in all patients. Cardiovascular magnetic resonance imaging demonstrated subendocardial late gadolinium enhancement in varios segments of the left ventricle, representing myocardial fibrosis in all patients one year after surgical correction. CONCLUSION: Because of the presence of scarr tissue, the long term prognosis of these patients remains unclear. The damaged tissue may have arrhythmogenic potential, therefore close follow-up, excercise testing and avoidance of high-level sport activities may be needed.
