Outcomes in patients with hepatopulmonary syndrome undergoing liver transplantation

Kim, H.Y.; Choi, Moon Seok; Lee, S.C.; Park, S.W.; Lee, J.H.; Koh, Kwang Chul; Paik, Seung Woon; Yoo, Byung Chul; Rhee, Jong Chul

doi:10.1016/j.transproceed.2004.10.002

Cited by 30 publications

(15 citation statements)

References 7 publications

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“…For comparative purposes, our 30‐day mortality post‐LT (11%) is comparable to other series (N > 5 cases) reported by other investigators4‐17 (Table 4). Importantly, no intraoperative deaths were noted in this or any other study.…”

Section: Discussionsupporting

confidence: 88%

Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era

et al. 2013

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Hepatopulmonary syndrome (HPS) is a pulmonary vascular disorder occurring as a consequence of advanced liver disease, characterized by hypoxemia due to intrapulmonary vascular dilatations. HPS independently increases mortality, regardless of the cause or severity of liver disease. Liver transplantation (LT) improves survival in HPS. We present the largest consecutive series of HPS patients specifically addressing long-term survival relative to the degree of hypoxemia and the era in which LT was conducted. We evaluated 106 HPS patients at the Mayo Clinic from 1986 through 2010. Survival was assessed using Kaplan-Meier methodology. LT was accomplished in 49 HPS patients. Post-LT survival (1, 3, 5, and 10 years) did not differ between groups based on baseline partial pressure of arterial oxygen (PaO 2 ) obtained at the time of HPS diagnosis. Improvements in overall survival at 1, 3, and 5 years post-LT in those HPS patients transplanted after January 1 2002 (n 5 28) (92%, 88%, and 88%, respectively) as compared with those transplanted prior to that time (n 5 21) (71%, 67%, and 67%, respectively) did not reach statistical significance (5-year P 5 0.09). Model for Endstage Liver Disease (MELD) exception to facilitate LT was granted to 21 patients since January 1 2002 with post-LT survival of 19/21 patients and one wait-list death. Conclusion: Long-term outcome after LT in HPS is favorable, with a trend towards improved survival in the MELD exception era since 2002 as compared to earlier HPS transplants. Survival after LT was not associated with PaO 2 levels at the time of HPS diagnosis. (HEPATOLOGY 2013;57:2427-2435 See Editorial on Page 2097 H epatopulmonary syndrome (HPS) is a pulmonary vascular disorder characterized by a clinical triad of hepatic dysfunction (usually with portal hypertension), arterial hypoxemia, and intrapulmonary vascular dilatations. HPS is not uncommon and affects between 5%-32% of individuals being assessed for liver transplantation (LT), depending on the criteria used to define arterial hypoxemia. 1 LT is the only therapy that has been shown to consistently and significantly improve or resolve HPS.Mortality associated with HPS, however, can be significant and not necessarily related to the severity of liver disease as measured by MELD (Model for Endstage Liver Disease) or CTP (Child-Turcotte-Pugh) scores. For those HPS patients with partial pressure of arterial oxygen (PaO 2 ) <60 mmHg, a MELD exception has been granted in an attempt to improve survival for those with no other contraindications to LT. 2 In the pre-MELD exception era (up to 2002), we had previously shown that HPS has a poor prognosis, with a 5-year survival of 23% without LT as compared to a 76% survival with LT. 3 In this report we present additional and extended 10-year survival data for our cohort of 106 patients with HPS, including 49 patients who underwent LT since the inception of our LT program. We aimed to further define the role of arterial hypoxemia as measured by baseline PaO 2 (at the time of HPS diagnosis) in ...

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Section: Discussionsupporting

confidence: 88%

Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era

et al. 2013

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“…Furthermore, we were able to extubate most of them in the operating room. Even if previous reports indicate that patients with HPS require ventilatory support after OLT (35, 36), two patients remained on mechanical ventilation and they were extubated two and five days post‐transplant, respectively. Experiences related with outcome of HPS after OLT are limited; portal venous thrombosis, intracranial events, multiorgan failure are seen more commonly in patients with HPS, and these complications are associated with a higher mortality rate (37).…”

Section: Discussionmentioning

confidence: 96%

Childhood cirrhosis, hepatopulmonary syndrome and liver transplantation

Tümgör

Arıkan²,

Yüksekkaya³

et al. 2008

Pediatric Transplantation

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“…The 5-year survival of HPS patients who received LT was 76%,2 which was similar to those without HPS who received LT. Although previous studies showed that the overall mortality rate was relatively high of 16-30%,1,10,11 recent studies showed less overall mortality rate of 5% 3,12. It is known that high postoperative mortality was reported in case of preoperative PaO 2 ≤50 mmHg alone or with a shunt fraction of more than 20% 1.…”

Section: Discussionmentioning

confidence: 96%

Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

et al. 2014

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Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.

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Outcomes in patients with hepatopulmonary syndrome undergoing liver transplantation

Cited by 30 publications

References 7 publications

Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era

Hepatopulmonary syndrome: Favorable outcomes in the MELD exception era

Childhood cirrhosis, hepatopulmonary syndrome and liver transplantation

Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

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