Interstitial Lung Disease in Patients with Hepatopulmonary Syndrome: A Case Series and New Observations

Shahangian, Sharareh; Shino, Michael Y.; Susanto, Irawan; Belperio, John A.; Fishbein, Michael C.; Wang, T.

doi:10.1007/s00408-014-9571-z

Cited by 7 publications

(3 citation statements)

References 18 publications

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“…This may be partly due to the contribution of the ILD to the hypoxemia itself and related challenges in distinguishing HPS versus intrinsic lung disease "components" of hypoxemia. Smaller studies have also suggested that ILD is more common in HPS, [18] and this may be mediated by short telomere syndrome. [19] It should be noted, however, that all comparisons between HPS and non-HPS subjects in our study are limited by a referral bias to the HPS clinic.…”

Section: Discussionmentioning

confidence: 99%

Physiologic changes in the hepatopulmonary syndrome before and after liver transplant: A longitudinal and predictor analysis

Zhao,

Kotha,

Nayyar

et al. 2023

Hepatology

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Background and Aims: Hepatopulmonary syndrome (HPS) is a common complication of liver disease defined by abnormal oxygenation and intrapulmonary vascular dilatation, treated with liver transplantation. Little is known about changes in HPS physiological parameters over time. We sought to describe baseline clinical and physiological characteristics in HPS and their relationships, temporal changes in physiological parameters before and after transplant, and predictors of changes in oxygenation. Approach and Results: This was a retrospective cohort study in the Canadian HPS Program (n=132). Rates of change after diagnosis were: -3.7 (-6.4, -0.96) mmHg/year for PaO2; -26 (-96, 44) m/year for six-minute walk distance, and 3.3% (-6.6, -0.011) predicted/year for diffusion capacity. Non-invasive shunt of ≥ 20% predicted a slower PaO2 decline by 0.88 (0.36, 1.4) mmHg/month. We identified two PaO2 deterioration classes – “very severe disease, slow decliners” (PaO2 45.0 mmHg; -1.0 mmHg/year); and “moderate disease, steady decliners” (PaO2 65.5 mmHg; -2.5 mmHg/year). PaO2 increased by 6.5 (5.3, 7.7) mmHg/month in the first year after transplant. Median time to normalization was 149 (116–184) days. Post-transplant improvement in PaO2 was 2.5 (0.1, 4.9) mmHg/month faster for every 10 mmHg greater pre-transplant orthodeoxia. Conclusions: We present the largest and longest longitudinal data analysis in HPS. In addition to rates of physiological decline and improvement before and after liver transplantation, we present novel predictors of PaO2 decline and improvement rates. Our findings enhance understanding of the natural history of HPS and provide pathophysiologic clues. Importantly, they may assist providers in prognostication and prioritization before and after transplant.

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Section: Discussionmentioning

confidence: 99%

Physiologic changes in the hepatopulmonary syndrome before and after liver transplant: A longitudinal and predictor analysis

Zhao,

Kotha,

Nayyar

et al. 2023

Hepatology

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“…Up to 20% of LT candidates may develop HPS, a pulmonary vascular disorder that leads to hypoxemia due to intrapulmonary shunting caused by dilation of pulmonary capillaries in the setting of liver failure. HPS may be associated with other comorbidities, 46,47 and adversely impacts outcomes when progressive hypoxemia occurs. 48,49 HPS is typically diagnosed on contrast echocardiography, and severe HPS patients are eligible for MELD exception points due to the high risk of deterioration without LT.…”

Section: Preoperative Pulmonary Assessmentmentioning

confidence: 99%

Perioperative Considerations in Liver Transplantation

Lopez

Steadman

Wray

et al. 2018

Semin Respir Crit Care Med

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Liver transplantation (LT) has the potential to cure patients with acute and chronic liver failure as well as a number of hepatic and biliary malignancies. Over time, due to the increasing demand for organs as well as improvements in the survival of LT recipients, patients awaiting LT have become sicker, and often undergo the procedure while critically ill. This trend has made the process of preoperative assessment and planning, intraoperative management, and postoperative management even more crucial to the success of LT programs. Multidisciplinary and specialized teams are essential and include anesthesiologists, surgeons, and intensivists. This article focuses on the preoperative evaluation, intraoperative care, and postoperative management of the liver transplant patient. Management relevant to the critically ill patient is discussed, with a focus on the management of postoperative cardiopulmonary conditions including the care of special populations such as those with hepatopulmonary syndrome and portopulmonary hypertension.

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“…9,10 Until now, less attention has been given to the presence of chronic intrinsic respiratory conditions which may coexist with HPS and may affect both preoperative and postoperative hypoxemia and outcome. 2,11,12 This study aimed to determine the outcome after LT in a cohort of patients with HPS with versus without concomitant respiratory disease.…”

Section: Introductionmentioning

confidence: 99%

Outcomes of liver transplantation for hepatopulmonary syndrome in patients with concomitant respiratory disease

Koc,

Aslan,

Kramer

et al. 2023

Clinical Transplantation

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Background & aimsConcomitant respiratory disease is a common finding in patients with hepatopulmonary syndrome (HPS). Among patients who underwent liver transplantation (LT) for HPS, we compared characteristics and outcome of patients with versus without concomitant respiratory disease.MethodsThis single center retrospective observational study included patients with HPS who underwent LT between 1999 and 2020.ResultsDuring the study period, 32 patients with HPS received a LT; nine (28%) with concomitant respiratory disease of whom one required a combined lung‐liver transplantation. Patients with concomitant respiratory disease had higher PaCO2 (38 vs. 33 mm Hg, p = .031). The 30‐day postoperative mortality was comparable, but the estimated cumulative probability of resolution of oxygen therapy after LT in HPS patients with versus those without concomitant respiratory disease was lower: 63% versus 91% at 12 months and 63% versus 100% at 18 months (HR 95% CI .140–.995, p = .040). In addition to the presence of concomitant respiratory disease (p = .040), history of smoking (p = .012), and high baseline 99mTcMAA shunt fraction (≥20%) (p = .050) were significantly associated with persistent need of oxygen therapy. The 5‐year estimated cumulative probability of mortality in patients with concomitant respiratory disease was worse: 50% versus 23% (HR 95% CI .416–6.867, p = .463).ConclusionsThe presence of a concomitant respiratory disease did not increase the short‐term postoperative mortality after LT in patients with HPS. However, it resulted in a longer need for oxygen therapy.

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Interstitial Lung Disease in Patients with Hepatopulmonary Syndrome: A Case Series and New Observations

Cited by 7 publications

References 18 publications

Physiologic changes in the hepatopulmonary syndrome before and after liver transplant: A longitudinal and predictor analysis

Physiologic changes in the hepatopulmonary syndrome before and after liver transplant: A longitudinal and predictor analysis

Perioperative Considerations in Liver Transplantation

Outcomes of liver transplantation for hepatopulmonary syndrome in patients with concomitant respiratory disease

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