SESSION TITLE: PAH and the Heart
SESSION TYPE: Original Investigation Slide
PRESENTED ON: Wednesday, October 28, 2015 at 08:45 AM - 10:00 AM
PURPOSE: The prevalence, pathophysiology, and clinical outcomes of tunneled atrial level shunts (T-ASD) among patients with Pulmonary Arterial Hypertension (PAH) are unknown. Tunnel-like Patent Foramen Ovale defects (PFO) have been identified among patents with cryptogenic stroke by three dimensional echocardiography. An autopsy case series has anatomically described a ‘slit-like defect’ with length and depth that represents a T-ASD.
METHODS: We performed a prospective study of 632 consecutive patients who were referred to our single center Pulmonary Hypertension Clinic, and underwent an entry echocardiographic study with agitated saline injection between January 2012 and December 2014. Eighty-eight patients had T-ASD’s.
RESULTS: Of 88 patients with T-ASD’s (mean age 64.7 years [range 20-89]; 56 (64%) female), 73 (83%) were diagnosed with PAH, 70 (80%) received targeted PAH therapies, and 3 (4%) were observed. Clinical follow-up for the patients with PAH was 16.3 +/-6.4 months. Of the 73 PAH patients, 11 (15.7%) demonstrated spontaneous shunt closure and 29 (33.3%) underwent therapeutic shunt closure (27 catheter based, 2 surgical). The remaining 30 patients continued active PAH therapy. Of the 29 patients who had either spontaneous or interventional shunt closure, 23 were able to completely wean off infusion therapies and 3 were able to down titrate drug dosages.
CONCLUSIONS: The described T-ASD’s were commonly observed in this population, with the majority of patients presenting with PAH or Eisenmenger Syndrome. We have demonstrated that treatment of this patient population is identical to that of other patients with congenital heart disease and/or PAH. Their PAH was successfully treated, permitting closure of the shunts, and eventual down titration/cessation of the PAH medications, with reversal of the PAH pathophysiology and resolution or marked improvement of the symptoms.
CLINICAL IMPLICATIONS: Additional studies are warranted to substantiate that this patient population represents treatable/reversible pathophysiology of PAH and/or Eisenmenger Syndrome permitting medical and interventional therapies in lieu of heart/ lung transplantation.
DISCLOSURE: The following authors have nothing to disclose: Dianne Zwicke, Sara Paulus, Mamatha Pinninti, Bijoy Khandheria, Tanvir Bajwa, Christopher Kramer, Vinay Thohan
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