Total Anomalous Pulmonary Venous Return with No Connection

摘要

class="kwd-title">Keywords: Total anomalous pulmonary venous connection, Pulmonary vein atresia, Echocardiography class="head no_bottom_margin" id="sec1title">IntroductionTotal anomalous pulmonary venous return with no connection, also known as common pulmonary vein atresia, presents immediately after birth in the neonate with severe, life-threatening cyanosis. Echocardiography is helpful in diagnosing specific characteristics of this disease after birth. Patients with this disease are often in respiratory failure and are frequently placed on high-frequency oscillator ventilation. Moreover, this diagnosis can mimic pulmonary hypertension of the newborn and lead to a management strategy that would be ineffective. Because of limited acoustic windows, other imaging modalities may be required to confirm the diagnosis. Management options for total anomalous pulmonary venous return with no connection are quite limited because there is significant damage to the pulmonary vascular bed, and the cyanosis is often irreversible despite ventilation strategies. Extracorporeal membrane oxygenation can support the circulation for a limited period of time, but often surgery to connect the pulmonary venous confluence to the left atrium is unsuccessful and does not reverse the lung disease. For these reasons, the majority of patients with this rare disorder succumb to this disease. This case report highlights the features of this congenital heart defect seen on echocardiography and also describes the limitations and potential need for other imaging modalities in the diagnosis and management of these patients.