Echocardiographic diagnosis of transposition of the great arteries associated with anomalous pulmonary venous connection

Lilian Maria Lopes, Gláucia Maria Penha Tavares, Fred Luiz Mailho, Vicente de Paulo Cavalcante de Almeida, José Armando Mangione
2001 Arquivos Brasileiros de Cardiologia  
We report 2 cases of transposition of the great arteries associated with anomalous pulmonary venous connection emphasizing the clinical findings, the diagnosis, and the evolution of the association. One of the patients had the anomalous pulmonary venous connection in its total infradiaphragmatic form, in the portal system, and the other patient had a partial form, in which an anomalous connection of the left superior lobar vein with the innominate vein existed. At the time of hospital
more » ... the patients had cyanosis and respiratory distress with clinical findings suggesting transposition of the great arteries. The diagnosis in 1 of the cases, in which the anomalous connection was partial, was established only with echocardiography, without invasive procedures that would represent risk for the patient; in the other case, in which the anomalous connection was total, the malformation was only evidenced with catheterization. The patients underwent surgery for anatomical correction of the heart disease. Only 1 patient had a good outcome. The association of transposition of the great arteries and anomalous pulmonary venous connection is very rare 1,2 . With few exceptions, the cases reported in the international literature result from postmortem studies 3 and, many times, are part of very complex syndromes, usually with death occurring a few hours or days after birth. Therefore, we considered the report of these cases of clinical interest, due to the rarity of the association and the contribution of the sensitivity of the echocardiogram as a preoperative method of diagnosis. Case Reports Case 1 -The patient was a 22-day-old male infant with a history of mild cyanosis since his birth. On physical examination at the time of admission, the infant was moderately tachydyspneic, and on heart auscultation, an increased intensity of the 2 nd heart sound predominated. The echocardiogram showed transposition of the great arteries, ventricular septal defect of 3mm in diameter, atrial septal defect of the oval foramen type with a diameter of 4 mm, and pulmonary flow with a pattern of pulmonary hypertension. The great dilation of the right chambers was noteworthy ( fig. 1A) , but an association with anomalous connection was not suspected. The echocardiography also showed a single coronary ostium, which was confirmed on cardiac catheterization. The patient underwent cardiac catheterization for assessment of the coronary arteries and performance of atrioseptostomy. During the procedure, the echocardiographicfindings were confirmed, and an anomalous infradiaphragmatic connection of the pulmonary veins of the nonobstructive type was shown ( fig. 2) . The surgical procedure chosen was the anatomical correction of the transposition of the great arteries, implantation of the anomalous pulmonary vein in the left atrium, ventriculoseptoplasty, and resection of a small muscular band from the right ventricular outflow tract that caused a mild subaortic stenosis (intraoperative finding). The patient evolved satisfactorily, and was discharged from the hospital after 2 weeks. Case 2 -The patient was a 5-month-old male child weighing 4.029kg, who, on physical examination, was moderately cyanotic, malnourished, and tachydyspneic. On cardiac auscultation, a systolic murmur (3+/4) predominated on the medium-to-low portion of the left sternal margin, and increased intensity of the 2 nd cardiac sound in the pulmonary area was observed. Echocardiography showed transposition of the great
doi:10.1590/s0066-782x2001000700007 pmid:11500749 fatcat:dygfgfol4ndelkbpj3qoaao6pu