Cardiac MRI prior to liver biopsy in a Fontan patient: A case report.

The management of adults with complex congenital heart disease requires a multidisciplinary team (Heart Team).

Endobronchial valve deployment in severe α-1 antitrypsin deficiency emphysema: a case series.

INTRODUCTION: Patients with end-stage emphysema because of α-1 antitrypsin (AAT) deficiency represent a challenging clinical management problem, and studies of volume reduction therapy to date have largely excluded these patients. We report the outcome of bronchoscopic volume reduction with the insertion of Emphasys endobronchial valves (Emphasys Medical, Redwood City, CA, USA) in six patients with end-stage emphysema because of AAT deficiency. CASE SERIES: Of 51 patients with end stage emphysema referred for transplantation, we studied six patients with AAT deficiency and utilized the BODE index and lung allocation score for survival estimation. MEASUREMENTS AND MAIN RESULTS: The forced expiratory volume in 1s improved from a median of 0.575L to 0.905L (P=0.028). There was a median reduction in total lung capacity (TLC) of 0.61L. The residual volume /TLC fell from 74.0% to 58.4%. Before treatment, four patients had a BODE index of greater than eight units, which correlates with a 4-year survival of 18%. After treatment, two patients improved their BODE index to below seven units, which correlates with an estimated 4-year survival of over 50%. CONCLUSIONS: The data from this case series suggest that this intervention may provide bridging therapy to subsequent transplantation for younger AAT patients with end-stage emphysema.