Case Presentation: A 68-year-old man who initially presented about 2 years prior with complaints of melanotic stools and coffee ground emesis. On his initial presentation, he was found to have elevated liver enzymes – AST/ALT/ALK were elevated, imaging revealed liver nodularity, EGD revealed grade I varices which were banded patient was diagnosed with NASH with esophageal varices. He was medically managed and discharged. Two years later, he presented with another episode of melanotic stools and coffee ground emesis, but this time patient had also developed hypoxic respiratory failure, which did not improve on use of Lasix. CTA of the chest ruled out PE and he was found to have features of ILD. Clinically desaturations were noticed when patient sits up with improvement with laying down and he was therefore diagnosed with platypnea-orthodeoxia. An echo with bubble study was performed revealing bubbles in later cycle confirming there was an extra-cardiac shunt. Nuclear perfusion lung scan revealed shunt fractions of 51% and 41% in anterior and posterior projections respectively. Patient was diagnosed with hepatopulmonary syndrome. His respiratory status continued to decline with PaO2 of less than 50 mmHg on 6 lit/min of nasal cannula, unfortunately, this disqualified him from getting a liver transplantation. Since the RHC (right heart catheterization) did not show any evidence of increased intrapulmonary pressures, pulmonary hypertension was ruled out. Since there was a lack of a large anatomical shunt on CTA, high shunt fraction was consistent of severe HPS. He underwent TIPSS as a possible bridge to liver transplantation and post TIPSS, he showed significant improvement in oxygenation. In fact, the patient was saturating at 92-93% on room air post procedure. Repeat shunt study showed reduction in the intrapulmonary shunting.
Discussion: TIPSS is generally used to reduce the pressures in the portal circulation and its utility to reduce intrapulmonary shunting is largely unexplored and there is limited literature on this. The mechanism of this phenomenon is not well understood. The literature review showed several case reports that showed its possible utility as a bridge to liver transplant to improve the patient’s candidacy for it. A review published in 2015 that compiled 12 reports of HPS showed improvement in 9 patients however more data and research is required to establish this.
Conclusions: TIPSS appears to improve hypoxemia in patients with portal hypertension. A better understanding of it’s mechanism would encourage its widespread use as a bridge to liver transplantation