Pulmonary vascular complications of chronic liver disease: Pathophysiology, imaging, and treatment

Khan, Ali; Al-Jahdali, Hamdan; Abdullah, Khalid; Irion, Klaus; Sabih, Quratulain; Gouda, Alaa

doi:10.4103/1817-1737.78412

Cited by 16 publications

(4 citation statements)

References 59 publications

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“…Finally, selection of eligible patients by ICD-10 codes and the need to have radionuclide scanning may have biased selection and could limit generalizability. In spite of these limitations, our study showed that echocardiography, a test that does not involve radiation and provides valuable cardiovascular information, could also quantify the degree of right-to-left shunt in patients with advanced liver cirrhosis, useful information since a more pronounced right-to-left shunt has been associated with greater hypoxemia and mortality in cirrhotic patients 12 . Because an abnormal degree of shunt was never observed in patients with microbubbles beyond seven cardiac cycles, the absence of bubbles beyond this 7-cycle criterion may obviate the need for Tc-99m MAA in such patients.…”

Section: Discussionmentioning

confidence: 85%

“…A more pronounced right-to-left shunt has been associated with greater mortality in cirrhotic patients 12 but there are limited data on whether saline contrast-enhanced echocardiography can help quantify this shunt. In addition, a fundamental unanswered question regards the number of cardiac cycles that need to be reviewed by echocardiography before a significant intrapulmonary shunt can be excluded or the total number of cycles over which bubble appearance should be quantitated.…”

Section: Introductionmentioning

confidence: 99%

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Assessing the kinetics of microbubble appearance in cirrhotic patients using transthoracic saline contrast‐enhanced echocardiography

et al. 2017

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Background The number of cardiac cycles that need to be reviewed by echocardiography before a significant intrapulmonary shunt can be excluded remains unclear. Methods We retrospectively identified patients with cirrhosis who underwent technetium-99m-labeled macroaggregated albumin scanning. The kinetics of bubble appearance after the injection of agitated saline during transthoracic echocardiograms were assessed in these patients. Results For the 64 eligible patients, the mean ± SD age was 56 ± 9 years. The median (IQR) shunt fraction by radionuclide scanning was 7.7 % (2.8 – 19.9%). Microbubbles were seen in the left atrium (LA) and left ventricle (LV) at a median (IQR) of 4 (2–5) and 4 (2–5) beats, respectively. The number of heart cycles before microbubbles appeared in the LA or LV was inversely associated with the nuclear scanning shunt fraction (R=−0.42, p=0.001, R=−0.46, p <0.001, respectively). If no microbubbles were detected by heart cycle 7, the shunt fraction was uniformly less than 3%. Patients with arterial oxygen < 60 mmHg, compared to ≥ 60 mmHg, had earlier appearance of microbubbles in the left heart chambers (2.6 ± 1.9 versus 4.0 ± 2.3 beats, p=0.046). Conclusions In patients with advanced cirrhosis suspected of having hepatopulmonary syndrome, a greater shunt fraction during nuclear scanning was associated with more pronounced hypoxemia and a prompt and more intense appearance of microbubbles in the left-sided heart chambers. Patients with a shunt fraction above 3% have microbubbles in the LA or LV at some point during the first seven heart cycles.

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Section: Discussionmentioning

confidence: 85%

Section: Introductionmentioning

confidence: 99%

Assessing the kinetics of microbubble appearance in cirrhotic patients using transthoracic saline contrast‐enhanced echocardiography

et al. 2017

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“…However, patients with HPS are well adapted to hypoxemia [ 1 ]. These patients often present after a prolonged period of undiagnosed hypoxemia and are encouraged to exercise to preserve muscle mass despite significant exercise desaturation (which occurs even with oxygen supplementation) [ 44 , 45 ]. Furthermore, many patients who require oxygen do not use it at all times [ 46 ].…”

Section: Discussionmentioning

confidence: 99%

Supine vs upright exercise in patients with hepatopulmonary syndrome and orthodeoxia: study protocol for a randomized controlled crossover trial

H.N.Parikh

Lui

Faughnan

et al. 2021

Trials

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Background The hepatopulmonary syndrome (HPS) is a pulmonary complication of liver disease found in 10 to 32% of patients with cirrhosis and is characterized by intrapulmonary vascular dilatations and abnormal oxygenation. Liver transplantation is the only effective therapy for this disease. Patients with HPS have significant exercise limitations, impacting their quality of life and associated with poor liver transplant outcomes. Many patients with HPS exhibit orthodeoxia—an improvement in oxygenation in the supine compared to the upright position. We hypothesize that exercise capacity will be superior in the supine compared to the upright position in such patients. Methods We propose a randomized controlled crossover trial in patients with moderate HPS (PaO2 < 80 mmHg) and orthodeoxia (supine to upright PaO2 decrease > 4 mmHg) comparing the effect of supine vs upright position on exercise. Patients with pulmonary hypertension, FEV1/FVC ratio < 0.65, significant coronary artery disease, disorders preventing or contraindicating use of a cycle ergometer, and/or moderate or severe ascites will be excluded. Participants will be randomized to cycle ergometry in either the supine or upright position. After a short washout period (a minimum of 1 day to a maximum of 4 weeks), participants will crossover and perform an exercise in the alternate position. Exercise will be performed at a constant work rate of 70–85% of the predicted peak work rate until the “stopping time” is reached, defined by exhaustion, profound desaturation, or safety concerns (drop in systolic blood pressure or life-threatening arrhythmia). The primary outcome will be the difference in the stopping time between exercise positions, compared with a repeated measures analysis of variance method with a mixed effects model approach. The model will be adjusted for period effects. P < 0.05 will be considered statistically significant. Discussion HPS patients have hypoxemia leading to significant exercise limitations. If our study is positive, a supine exercise regimen could become a routine prescription for patients with HPS and orthodeoxia, enabling them to exercise more effectively. Future studies could explore the corresponding effects of a supine exercise training regimen on physiologic variables such as long-term exercise capacity, quality of life, dyspnea, and liver transplantation outcomes. Trial registration ClinicalTrials.gov Protocol Registration and Results System (PRS) NCT04004104. Registered on 1 July 2019

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“…14,15 The severity of chronic liver disease contributes to the development of hypoxemia and worsens the prognosis of these patients. 16,17 The underlying pathophysiology of hypoxemia in patients with cirrhosis is mainly caused by abnormal pulmonary perfusion pathways. 18 Previous studies suggest that a rightward shift in the oxyhemoglobin dissociation curve seen in patients with cirrhosis may be the primary physiological mechanism that explains this association.…”

Section: Hypoxemia Associated With Liver Failurementioning

confidence: 99%

Pulmonary Complications in Candidates for Liver Transplantation

Razavi-Khorasani

Moazzami

Moghadam

et al. 2020

Middle East J Dig Dis

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The liver plays a pivotal role in maintaining the homeostasis of various organ systems. Also, end-stage liver disease and its complications are major causes of morbidity and mortality among adults. Individuals who develop a chronic liver disease are at increased risk of progression to multi-organ dysfunction, including the pulmonary system. The clinical complications of pulmonary problems related to the presence of liver disease range from mild (such as hypoxemia) to life-threatening diseases (such as portopulmonary hypertension and hepatopulmonary syndrome). Herein, the major pulmonary complications related to liver cirrhosis and considerations for performing liver transplantation are reviewed.

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Pulmonary vascular complications of chronic liver disease: Pathophysiology, imaging, and treatment

Cited by 16 publications

References 59 publications

Assessing the kinetics of microbubble appearance in cirrhotic patients using transthoracic saline contrast‐enhanced echocardiography

Assessing the kinetics of microbubble appearance in cirrhotic patients using transthoracic saline contrast‐enhanced echocardiography

Supine vs upright exercise in patients with hepatopulmonary syndrome and orthodeoxia: study protocol for a randomized controlled crossover trial

Pulmonary Complications in Candidates for Liver Transplantation

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