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Recognition and Clinical Outcomes of Tunneled Interatrial Shunts Among Patients With Pulmonary Hypertension

Dianne Zwicke


M. Pinninti

BK Khandheria

T. Bajwa

S. Paulus

C. Kramer

V. Thohan

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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.21.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Diane Zwicke I

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The prevalence, pathophysiology and clinical outcomes of tunneled, atrial-level shunts among patients with pulmonary arterial hypertension (PAH) are unknown. Tunnel-like patent foramen ovale (PFO) has been identified among patients with cryptogenic stroke by three-dimensional echocardiography. An autopsy case series anatomically described a slit-like defect with length and depth that may represent tunneled interatrial shunt (T-IAS).

Background: The prevalence, pathophysiology and clinical outcomes of tunneled, atrial-level shunts among patients with pulmonary arterial hypertension (PAH) are unknown. Tunnel-like patent foramen ovale (PFO) has been identified among patients with cryptogenic stroke by three-dimensional echocardiography. An autopsy case series anatomically described a slit-like defect with length and depth that may represent tunneled interatrial shunt (T-IAS).

Methods: We performed a retrospective review of 302 consecutive patients who underwent a clinic-entry echocardiographic study with agitated saline from January 2012 through December 2013 upon referral to our single-center pulmonary hypertension program. Of those 302 patients, 64 patients were identified with a T-IAS.

Results: Of 64 patients with T-IAS (mean age 64.7 years, range 20-89; 41 women), 53 were diagnosed with PAH and 51 (96.2%) were treated with targeted PAH medical therapy and 2 (3.8%) were placed under observation. Clinical follow-up for patients with PAH was 14.3±6.4 months. Of the 51 patients treated for PAH, 8 (15.7%) demonstrated spontaneous shunt closure and 17 (33.3%) underwent therapeutic shunt closure (16 catheter-based and 1 surgical). The remaining 26 patients continue active PAH therapy. Of the 25 patients who had either spontaneous or interventional closure, 10 were able to completely wean off infusion therapy and 1 was able to down titrate drug dosages.

Conclusions: Tunneled interatrial shunt is a commonly observed anatomic defect with physiologic implications in patients with pulmonary arterial hypertension. We have demonstrated that closure of T-IAS improves symptoms and facilitates down titration of IV and inhaled prostacyclin therapy for PAH. Prospective studies are warranted to further substantiate these observations.

Type: Clinical Science