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

Article Outline

• References
• Copyright

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.

• 
  • View Large Image
  • Download to PowerPoint

• 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.

Aortography displayed a single large artery (diameter, 8 mm; length, 20 mm) arising from the descending aorta.

Surgery revealed an intralobar sequestration in the left lower lobe that showed normal communication with the tracheobroncheal tree and received its arterial blood supply from the descending aorta via a single large artery (diameter; 8 mm, length; 20 mm) (Pryce type 1).¹ Venous drainage was through the left lower pulmonary vein. Lobectomy of the left lower lobe was performed.

The definitive diagnosis of pulmonary sequestration is usually made by aortography, but noninvasive methods are preferable, especially in young patients such as neonates or infants.

Doppler echocardiography and MRA were useful for the diagnosis of pulmonary sequestration presenting as a vascular murmur in an infant.

Back to Article Outline

References 

1. Pryce DM. Lower accessory pulmonary with intralobar sequestration of lung: report of seven cases. J Pathol. 1946;58:457–467
   • View In Article