A 91 y.o. with a hx of partial anomalous pulmonary venous return presents with shortness of breath.

Why does she have this?

The picture below is a patient with the same problem.

what do the arrows represent?

This represents Scimitar syndrome where venous return from the R lung enters the systemic venous drainage rather than the L atrium.  This was first described by Catherine Neill in 1960.

The Scimitar is shown here

Our patient had partial anomalous pulmonary venous return which was uncorrected after her ASD repair at age 51. As a consequence, with high flow returning to the R atrium she developed pulmonary hypertension and heart failure. Partial anomalous pulmonary venous drainage is often asymptomatic or as in this patient, presents later in life. In the general population the reported incidence is 0.7% but is much higher in autopsy series since so many patients are asymptomatic. This depends on how many of the four pulmonary veins are actually draining into the venous circulation. It is important to recognize because it is often associated with other congenital cardiac anomalies; as it was associated with an ASD in our patient.

Pulmonary veins draining into the SVC

Surgery is recommended if there is a hemodynamically significant left-to-right shunt( a ratio of pulmonary to systemic flow greater than 2:1) Our patient did not require surgery and was successfully diuresed for her heart failure.

TRIVIA QUESTION

What is a scimitar and why was it important?

A scimitar is a short sword with a curved blade used in horse warfare in central Asia. Because of their light weight and curved design they were good for slashing opponents while riding a horse. Scimitars changed medieval warfare since knights who fought in the Middle Ages fought with straight swords and heavy armor. They were at a disadvantage when fighting in the heat and often their blades stuck into opponents but were necessary to pierce heavy armor. Of course armor could be pierced more easily after the invention of gunpowder by the Chinese.

Holt P, Merdon W, Marans X et al. Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome.  Pediatric Radiology34(5):409-13.

Najm H, Williams W, coles J, et al. scimitar syndrome :twenty years’ experience and results of repair.  J Thorac Cardiovasc Surg 1996;112:1161-8; discussion 1168-9.10.1016/S0022-5223(96)70129-0.

Oransky I, “Catherine Neill” the Lancet 367(9519):1312

Farkas Z, Haidry A, Yandrapalli S, et al. Abnormal chest X-ray leading to diagnosis of partial anomalous pulmonary venous connection. AnnTransl Med 2018 Apr:6(8):156.