Unmasking of patent ductus arteriosus on cardiopulmonary bypass: Role of intraoperative trans-esophageal echocardiography in a patient with severe pulmonary hypertension due to pulmonary vein stenosis and cor triatriatum

Abstract

Interesting Images venous obstruction. After induction of general anesthesia, transesophageal echocardiography (TEE) probe was inserted. TEE confirmed the diagnosis made earlier. Further, a restrictive fleshy membrane in the left atrium (cor triatriatum) contributing to pulmonary vein stenosis was observed. Surgery commenced via mid-sternotomy. Total body heparinisation was achieved with 6000 IU of heparin; the resultant activated clotting time was 450 s. The pulmonary artery (PA) pressure measured via a fine needle inserted in the main pulmonary artery revealed supra-systemic PA pressure. The systemic pressure was 73/56 and mean 63 mmHg, while the PA pressure was 98/60 mmHg. Cardiopulmonary bypass (CPB) was instituted without events after cannulation of ascending aorta and cannulation of superior and inferior vena cavae. It is our institution policy to confirm the empty status of the heart with adequate venous return and absence of aortic regurgitation after establishing CPB using TEE. During such routine examination in this patient, hitherto unreported ductal flow was detected and patent ductus arteriosus (PDA) was diagnosed [Video 1]. PDA was visualized in the upper esophageal view. As the tee probe is withdrawn gradually from the mid-esophageal position, just beyond A 15-year-old African girl, weighing 27 kilos, with a height of 148 cm was admitted to the hospital for repair of pulmonary venous obstruction and cor triatriatum. She was comfortable at rest, but had severe limitation of activities beyond those of daily living. Pulse oximetry on room air was 92%, which improved to 95% with oxygen.Blood investigations were unremarkable except for hemoglobin 19 g/dL and her Sickling test was negative. Preoperative transthoracic echocardiogram revealed stenosis of right upper and left lower pulmonary veins with a peak gradient of 15 mmHg at their respective junctions with the left atrium (LA). Normal flow was noted in the other pulmonary veins. A membrane was visualized in the left atrium without significant obstruction to flow. Severe pulmonary hypertension (94/65 mmHg) was detected. The pulmonary artery pressure was noted to be 95% of the systemic pressure. Flow velocities in the pulmonary artery and descending aorta were normal, there was no pericardial effusion. A computerized tomographic angiogram confirmed the anatomical findings noted by the transthoracic echocardiogram. The patient was scheduled for repair of pulmonary

Síndrome de Silver-Russell (hemihipertrofia) y cor triatriatum en un neonato

“…[1][2][3][4][5][6] Hasta donde conocemos, la asociación de CT y síndrome de hemihipertrofia (SH) congénita no ha sido descrita.…”