Mrs JB

A 32-year-old postpartum female presented with dyspnea and limb swelling for 5 days. She had an eventful postpartum state with massive postpartum hemorrhage requiring multiple blood transfusions. Clinical examination showed BP 100/60 mmHg, Pulse 108 /min, bipedal edema, and Spo2 95 % onroom air. She had equal air entry on both lung fields with normal heart sounds and no murmurs audible.  She had no significant medical history of any chronic illness in the past.

Her lab parameters showed - Hb-10g/dl, ESR -64mm/hr,  ANA - >500IU/ml, AntiU1 RNP antibody -positive, viral serologies including HIV negative.She had her NT proBNP level of  998pg/ml with a negative venous doppler of the lower limb . Her echocardiogram revealed dilated RA and RV with moderate TR with moderate pulmonary hypertension (63 mmHg). Contrast-enhanced CT showed evidence of intercostal-pulmonary arterial fistula ( 6th and 7th intercostal branch) with dilated right atrium and ventricle. 

She underwent invasive angiography from the right femoral artery which demonstrated tortuous and tangling vessels draining into branches of the right inferior pulmonary artery through the right 6th  and 7th intercostal branches of the descending aorta . Her right heart catheterization study revealed tracings as - RA pressure  ( 25/10 (15 mmHg ), PCWP - 16/6( 9 mmHg), RV / ( pulmonary artery ) PA-  Pre-procedure - 52/31(38mmHg)  and PA - Post procedure 50/15 ( 27mmHg ),  Aorta - 130/90( 72 mmHg). 

Procedure -  After the angiogram of the right 6th and 7th intercostal arteries and its abnormal fistulous communication to the inferior pulmonary artery branches, the super selective cannulation into the 6th and 7th intercostal branches distally was done with a microcatheter, and successful embolization was done with a combination of lipiodol and glue injected into the distal feeding branches. Post-embolization angiography demonstrated the absence of flow into the vascular nidus and branches of pulmonary arteries. A repeat right heart catheterization was done to check for an immediate drop in pulmonary pressure which revealed that mean PA pressure dropped from 38 mmHg to 27 mmHg. 

      Traditionally, resection of the fistula and the involved lung parenchyma is considered the definitive treatment of SA-PAFs. The embolization   of  the involved feeder, and arteries is an alternative treatment since major surgery and general anesthesia are unnecessary and the loss of lung parenchyma is minimal.  In addition, in the case of SA-PAFs with multiple involved vessels, like in our case, embolization is more useful to control these multiple lesions. Our patient is in a one-year follow-up with the last repeat CT angiogram showing no evidence of fistula and an Echocardiogram showing normal cardiac chambers with normal pulmonary pressure and normal LV function.