Doppler echocardiography and MR angiography for diagnosis of systolic murmurs in pulmonary sequestration

A 1-month-old boy was referred from another institution because of a systolic murmur. A grade 2 systolic murmur was heard at the left lower sternal border with radiation to the left chest and back. A chest radiograph showed slight cardiomegaly and the electrocardiogram showed mild left ventricular volume overload. Echocardiography showed no cardiac abnormalities, except for mild left ventricular volume overload. Meticulous color Doppler examination of the aorta showed anteriorly directed arterial blood flow arising from the descending aorta in the retrocardiac region above the left hemidiaphragm (Figure, A). Magnetic resonance angiography (MRA) with contrast enhancement revealed a single large artery that ran leftward and downward from the descending aorta in the retrocardiac region above the left hemidiaphragm (Figure, B and D). Abnormal vessels were also observed to the left of the descending aorta in the retrocardiac region (Figure, B and C). The left lower pulmonary vein was dilated, so venous drainage was considered to occur through this vein. From these findings, a diagnosis of sequestration of the left lower lobe was strongly suspected.

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• Figure. 

  A, Meticulous Doppler examination of the aorta showed anteriorly directed arterial blood flow (arrow) arising from the descending aorta (triangle) in the retrocardiac region above the left hemidiaphragm. “Upper” and “Lower” indicate orientation. B, Enhanced MRA, short-axis view. A single large artery (arrow) runs leftward from the descending aorta (triangle). Abnormal vessels (asterisk) can also be observed to the left of the descending aorta in the retrocardiac region. H, Heart; Rt, right; Lt, left. C, Enhanced MRA, left anterior oblique view. Abnormal vessels (asterisk) can be observed to the left of the descending aorta (triangle). H, Heart. D, Enhanced MRA, right anterior oblique view. A single large artery (arrow) runs leftward and downward from the descending aorta (triangle). H, Heart.