RESUMEN
A 17-year-old male was referred for evaluation by Pediatric Cardiology following a pre-sports medical screening, which revealed abnormal ventricular repolarization (negative T wave from V3-V6) on a 12-lead ECG. For 8 years, he has been a competitive football player. There was no relevant personal or family medical history, and he denied any prior cardiovascular symptoms. His cardiovascular physical examination was normal. In the initial assessment, a transthoracic echocardiogram was conducted, revealing an apical aneurysm of the left ventricle (LV) with regional dyskinesia, accompanied by mild mitral regurgitation. The examination also demonstrated globally preserved left ventricular systolic function, with no images suggestive of vegetations or intracardiac thrombi found. For further anatomical and myocardial characterization, a cardiac magnetic resonance (CMR) was performed. The results revealed a wide apical aneurysm of the LV, predominantly lateral, with thin walls, ridges within the outpouching, and local dyskinesia. No areas of late gadolinium enhancement (LGE) were found, suggestive of fibrosis, anomalous infiltration, or scar from a previous infarction. After excluding other etiologies, the diagnosis of a congenital ventricular aneurysm was determined. This is a very rare condition (0.12%) with a wide and variable clinical presentation, ranging from benign arrhythmias to sudden death. Various imaging techniques aid in its diagnosis, with CMR being particularly useful for excluding inflammation and cardiomyopathies, identifying LGE, and evaluating left ventricular volumes and function. There is no standard treatment, and controversy surrounds indications for surgical intervention. If a conservative approach is adopted, measures aimed at preventing endocarditis and embolism must be considered.
RESUMEN
ALCAPA syndrome is a rare congenital heart disease and a cause of myocardial ischemia in pediatric population. The authors present the case of a 10-year-old girl admitted to the emergency room after experiencing cardiac arrest at school. In the echocardiogram, the inability to identify the origin of the left coronary artery raised the hypothesis of abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA). A CT-scan and a cardiac catheterization were performed confirming the diagnosis. Infants with the syndrome may have myocardial infarction and congestive heart failure. She successfully underwent cardiac surgery. Most undiagnosed patients die within the first year of life, and it is necessary to develop an extensive network of collaterals to ensure survival. ALCAPA syndrome rarely manifests in late childhood, teenagers, and adults and may be an important cause of sudden cardiac arrest.
RESUMEN
BACKGROUND: Vena cava anomalies are rare congenital defects due to incorrect development during fetal life, ranging from minor asymptomatic anatomic variations to complex life-threatening abnormalities. Echocardiography plays a fundamental role in the diagnosis, with advanced imaging techniques allowing detailed anatomic delineation. Invasive cardiology techniques are a promising therapeutic approach, but surgery is probably the best option when diffuse compromise of the systemic veins is present. CASE REPORT: An 8-month-old infant presenting episodes of labial cyanosis and a failure to thrive was diagnosed with severe superior vena cava (SVC) stenosis at the right atrium ostium, with decompression via azygos vein and mild inferior vena cava (IVC) stenosis. The patient was referred for surgery, with IVC enlargement and removal of a fibromuscular tissue band on the anterior aspect of the SVC ostium. At a 6-month follow-up, the patient is asymptomatic, with excellent surgical outcome. To the authors' knowledge, only two cases of congenital SVC stenosis have been previously described.
