Echocardiographic diagnosis of totally anomalous pulmonary venous connection to the azygos vein

Totally anomalous pulmonary venous connection to the azygos vein is a rare congenital heart malformation in which all the pulmonary venous blood retur ns anomalously to the azygos vein. Among 111 consecutive patients with tota lly anomalous pulmonary venous connection undergoing surgical correction at our institution between June 1982 and September 1997, this malformation wa s present in seven cases. By echocardiography, using a subxyphoid short-axi s view at the atrial level and a modified suprasternal sagittal view, the m alformation was diagnosed when the pulmonary venous confluence was traced p osteriorly and superiorly relative to the right pulmonary artery and right bronchus, finally reaching reach the superior caval vein. Totally anomalous pulmonary venous connection to the azygos vein was misdiagnosed in the fir st two patients, both by echocardiography and angiocardiography. In the sub sequent five patients, a precise diagnosis was obtained by echocardiography . Echocardiography, therefore, can be considered an accurate diagnostic too l permitting recognition of totally anomalous pulmonary venous connection t o the azygos vein, and permitting corrective surgery without recourse to ca theterization and angiography.