The Science Journal of the American Association for Respiratory Care

2011 OPEN FORUM Abstracts

LUNG BYPASS FOR HERMANSKY-PUDLAK SYNDROME (HPS) PATIENT ELIMINATING NEED FOR MECHANICAL VENTILATION WHILE AWAITING LUNG TRANSPLANT.

Tiffanee Singer, Brenda Singer, Daniel Herr; University of Md, Baltimore, MD

INTRODUCTION: HPS, an often fatal heterogeneous autosomal recessive disorder characterized by tyrosinase-postive oculocutaneous albinism, bleeding diathesis, lysosomal and kidney dysfunctions, pulmonary fibrosis, and colitis, is prevalent in northern Puerto Rico. 1 in 21 individuals'carries the gene and 1 in 1800 individuals is symptomatic. This HPS patient suffered from many of those complications the most prevalent being pulmonary fibrosis making mechanical ventilation impossible. Veno-Venous (V-V) ecmo with balloon atrial septostomy provided complete ventilatory support while awaiting lung transplantation. Case summary: 43 y/o intubated male admitted for lung transplant evaluation, after being rejected from several facilities, diagnosis HPS, severe pulmonary fibrosis, pulmonary hypertension. Day two of admission patient was cannulated via right intrajugular and right femoral placed on V-V ecmo. Oxygenation continued to be suboptimal with SaO2's < 88% and PaO2's < 60mmHg. On day three, the patient was taken to the catheterization lab where a balloon atrial septostomy was performed relieving right sided heart pressure providing a right to left shunt and increasing oxygenation to the left. On day four, due to advance stage of patient's disease, pulmonary hemorrhaging began; lung compliance decreased rapidly, minute volumes 0.19L/min to 0.8L/min, with no aeration. At this time it was decided to discontinue mechanical ventilation. Patient tolerated extubation. Twelve days following the original septostomy saturations began to decline. It was discovered the septostomy closed and needed to be reopened. After fourteen days on ECMO, two septostomy procedures, and ten days off the ventilator patient received double lung transplant. On day five following transplant, tracheostomy was performed second to failure to extubate; within two weeks patient started his first aerosol trach collar trial. Discussion: Using ECMO as a bridge to transplant, in this unique case, accompanied by an atrial septostomy and using the patients pulmonary pressures allowed for right to left shunting, thus increasing oxygenation producing adequate saturations and effective lung bypass.
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