Andressa Mussi Soares

Aortopulmonary window. Clinical and surgical assessment of 18 cases

OBJECTIVE Aortopulmonary window (APW) is an uncommon congenital malformation. Its clinical presentation is dependent on the size of the defect and on the associated lesions. We evaluated our experience with this anomaly and compared it with 296 cases reported in the literature. METHODS Retrospective study of 18 patients diagnosed as having APW (age range from 13 days to 31 years, 13 (72.2%) females), divided into two groups: Group A (GA): 10 patients with isolated APW, and Group B (GB): 8 patients with associated lesions. RESULTS Heart failure occurred in 14 patients, and cyanosis in 3:2 from GB (tetralogy of Fallot--TF, and double outlet right ventricle--DORV), and one from GA with pulmonary hypertension. In 5 patients from GA the diagnosis of mitral regurgitation was made based on a systolic murmur and LV hypertrophy on the EKG. In GB, clinical findings were determined by the associated defect. Diagnosis was established by echocardiography in 11 (61.2%) of the patients. In 3 patients, a wrong diagnosis of mitral regurgitation was made, in 1 a patent ductus arteriosus was diagnosed and in 3 others, the diagnosis of APW was masked by other important associated defects (2 cases of DORV and 1 case of TF). The diagnosis was made by catheterization in 3 (16.6%) patients, by surgery in 3 (16.6%) and by necropsy in 1 (5.5%). Corrective surgery was performed in 14 (77.7%) patients, with one immediate death and good long-term follow-up in the remaining patients. CONCLUSION APW can be confused with other defects. Clinical findings, associated with an adequate echocardiogram can provide the information for the correct diagnosis.

Anomalous subaortic course of the left brachiocephalic (innominate) vein: echocardiographic diagnosis and report of an unusual association

Subaortic left brachiocephalic, or innominate, vein is an uncommon finding in congenital heart disease, usually associated with obstruction of the right ventricular outflow tract. We describe our experience with 14 patients in whom the lesion was identified echocardiographically, 12 of them with right ventricular obstruction, one with totally anomalous pulmonary venous connection in the absence of obstruction to the right ventricular outflow tract, and the final one with a normal heart. A precise diagnosis of this venous anomaly is of great importance, since it needs to be differentiated from a central pulmonary artery, a pulmonary venous confluence, or an ascending vertical vein in totally anomalous pulmonary venous connection. In patients referred for surgery without catheterization, an incorrect echocardiographic diagnosis could lead to disastrous surgical results.

Common arterial trunk associated with atrioventricular septal defect

Described are the clinical, cross-sectional echocardiographic, electrocardiographic and angiographic findings, together with the results at autopsy, in a 5-month old infant with a common arterial trunk associated with an atrioventricular septal defect. As far as is known, this is only the 13th such case to be reported in the literature. A particularly unusual feature of this case was an intact atrial component of the atrioventricular septal defect.

Non-invasive assessment of right ventricular function in the late follow-up of the Senning procedure

Deteriorating ventricular function is a major concern after the Senning operation. A Doppler-derived non-geometric measurement, the so-called myocardial performance index, has been described for use in adults and children. We aimed to assess the utility of this index as a method for quantification of right ventricular function in patients in the late follow-up of the Senning procedure, and to correlate the right ventricular ejection fraction and the first derivative of right ventricular pressure as derived using echocardiography with the ejection fraction determined using magnetic resonance imaging. We studied 44 patients within a mean postoperative period of 15.3 years. We calculated the right ventricular myocardial performance index by pulsed wave Doppler interrogation of tricuspid inflow and aortic outflow, the ejection fraction by Simpsons rule, and the first derivative of right ventricular pressure by continuous wave Doppler from tricuspid regurgitation. Mean values of right ventricular myocardial performance index, ejection fraction, and the first derivative of right ventricular pressure were 0.50, 39 percent and 1,398 millimetres of mercury per second, respectively. A cut-off value of 0.47 for the right ventricle myocardial performance index was determined, with a sensitivity of 75 percent and a specificity of 62.5 percent. We found no correlation between ejection fraction and the first derivative of right ventricular pressure as estimated by echocardiography and the ejection fraction as shown by magnetic resonance imaging (r2 equal to 0.29 and 0.04 respectively). We concluded, first, that patients with preserved right ventricular function had values for the right ventricular myocardial performance index lower than 0.47, and second, that ejection fraction and the first derivative of right ventricular pressure as determined echocardiographically did not correlate with values derived using magnetic resonance imaging.

Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study

Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study Andressa Mussi Soares1,2,3 Hospital Evangélico de Cachoeiro de Itapemirim,1 Cachoeiro de Itapemirim, ES Brasil Faculdade de Medicina da Universidade de São Paulo,2 São Paulo, SP Brasil Departamento de Cardiopatia Congênita e Cardiologia Pediátrica Gestão 2018-19,3 RJ Brasil Short Editorial related to the article: Mortality for Critical Congenital Heart Diseases and Associated Risk Factors in Newborns. A Cohort Study