Liver Transplantation for Patients with Portopulmonary Hypertension: a Case Report and Review of the Literature

2015 ◽  
Vol 25 (2) ◽  
pp. 19-23
Author(s):  
Andrius Macas ◽  
Limas Kupčinskas ◽  
Giedrius Barauskas ◽  
Darius Trepenaitis ◽  
Juozas Kupčinskas ◽  
...  

Portopulmonary hypertension is one of the complications for people with end stage chronic liver disease. This condition occurs to 5-8% of people, who need liver transplantation, and it causes a higher risk of perioperative complications or these patients are even banned from this procedure. Therefore, it is very important to diagnose this pathology in time using right heart catheterization and provocative volume and dobutamine samples for evaluation of the right ventricle function. Fortunately, adequate medical treatment with its most important part – vasodilator drugs - cures portopulmonary hypertension or lowers the perioperative risk caused by this condition. This case report presents a 50-year-old male hospitalized for liver transplantation. The patient has cirrhosis of the liver caused by hepatitis C, liver failure and portopulmonary hypertension. He has been taking vasodilator drugs for almost 8 months in order to prepare for a surgical treatment and when the operation’s day came, all tests needed including echocardiography shown that the transplantation could be performed. During the operation patient’s heart function was monitored with transesophageal echocardiography. Even though the mean pulmonary arterial pressure was marginal, the operation was successful and the patient could be extubated right after the procedure. Systolic pulmonary arterial pressure of the recipient dropped from 70 mmHg to 60.

2021 ◽  
Vol 16 (4) ◽  
pp. 344-352
Author(s):  
Jungchan Park ◽  
Myung Soo Park ◽  
Ji-Hye Kwon ◽  
Ah Ran Oh ◽  
Seung-Hwa Lee ◽  
...  

Background: The clinical efficacy of preoperative 2D-echocardiographic assessment of pulmonary arterial pressure (PAP) has not been evaluated fully in liver transplantation (LT) recipients.Methods: From October 2010 to February 2017, a total of 344 LT recipients who underwent preoperative 2D-echocardiography and intraoperative right heart catheterization (RHC) was enrolled and stratified according to etiology, disease progression, and clinical setting. The correlation of right ventricular systolic pressure (RVSP) on preoperative 2D-echocardiography with mean and systolic PAP on intraoperative RHC was evaluated, and the predictive value of RVSP > 50 mmHg to identify mean PAP > 35 mmHg was estimated.Results: In the overall population, significant but weak correlations were observed (R = 0.27; P < 0.001 for systolic PAP, R = 0.24; P < 0.001 for mean PAP). The positive and negative predictive values of RVSP > 50 mmHg identifying mean PAP > 35 mmHg were 37.5% and 49.9%, respectively. In the subgroup analyses, correlations were not significant in recipients of deceased donor type LT (R = 0.129; P = 0.224 for systolic PAP, R = 0.163; P = 0.126 for mean PAP) or in recipients with poorly controlled ascites (R = 0.215; P = 0.072 for systolic PAP, R = 0.21; P = 0.079 for mean PAP). Conclusion: In LT recipients, the correlation between RVSP on preoperative 2D-echocardiography and PAP on intraoperative RHC was weak; thus, preoperative 2D-echocardiography might not be the optimal tool for predicting intraoperative PAP. In LT candidates at risk of pulmonary hypertension, RHC should be considered.


PEDIATRICS ◽  
1961 ◽  
Vol 27 (4) ◽  
pp. 621-626
Author(s):  
Robert E. Stanton ◽  
Donald C. Fyler

The natural history of the pulmonary arterial pressure in 23 children with isolated ventricular septal defects has been evaluated by serial night heart catheterization. In none of the 23 patients was there a significant increase in pulmonary arterial pressure. Eight patients showed a significant decrease. The direction of shunts did not change between procedures, and the clinical status was stable. Our experience correlated well with the majority of institutions. It would appear that the natural course of pulmonary arterial pressure in ventricular septal defect is not commonly one of progressive rise.


2018 ◽  
Vol 243 (9) ◽  
pp. 754-761
Author(s):  
Jie Liu ◽  
Lei Fei ◽  
Guang-Qing Huang ◽  
Xiao-Ke Shang ◽  
Mei Liu ◽  
...  

Right heart catheterization is commonly used to measure right ventricle hemodynamic parameters and is the gold standard for pulmonary arterial hypertension diagnosis; however, it is not suitable for patients’ long-term follow-up. Non-invasive echocardiography and nuclear medicine have been applied to measure right ventricle anatomy and function, but the guidelines for the usefulness of clinical parameters remain to be established. The goal of this study is to identify reliable clinical parameters of right ventricle function in pulmonary arterial hypertension patients and analyze the relationship of these clinical parameters with the disease severity of pulmonary arterial hypertension. In this study, 23 normal subjects and 23 pulmonary arterial hypertension patients were recruited from January 2015 to March 2016. Pulmonary arterial hypertension patients were classified into moderate and severe pulmonary arterial hypertension groups according to their mean pulmonary arterial pressure levels. All the subjects were subjected to physical examination, chest X-ray, 12-lead electrocardiogram, right heart catheterization, two-dimensional echocardiography, and technetium 99m (99mTc) myocardial perfusion imaging. Compared to normal subjects, the right heart catheterization indexes including right ventricle systolic pressure, right ventricle end diastolic pressure, pulmonary artery systolic pressure, pulmonary artery diastolic pressure, pulmonary vascular resistance, and right ventricle end systolic pressure increased in pulmonary arterial hypertension patients and were correlated with mean pulmonary arterial pressure levels. Echocardiography parameters, including tricuspid regurgitation peak velocity, tricuspid regurgitation pressure gradient, tricuspid annular plane systolic excursion and fractional area, right ventricle-myocardial performance index, were significantly associated with the mean pulmonary arterial pressure levels in pulmonary arterial hypertension patients. Furthermore, myocardial perfusion imaging was not observed in the normal subjects but in pulmonary arterial hypertension patients, especially severe pulmonary arterial hypertension subgroup, and showed potential diagnostic properties for pulmonary arterial hypertension. In conclusion, mean pulmonary arterial pressure levels are correlated with several right heart catheterization and echocardiography markers in pulmonary arterial hypertension patients; echocardiography and 99mTc myocardial perfusion can be used to evaluate right ventricle performance in pulmonary arterial hypertension patients. Impact statement In this study, we analyzed the clinical parameters for evaluating RV function, including right ventricle catheterization (RHC), echocardiography, and technetium 99m (99mTc) myocardial perfusion imaging (MPI) in normal Asian subjects and PAH patients ( n = 23 for each group). Our results demonstrated that six RHC indexes, four echocardiography indexes and MPI index were significantly altered in PAH patients and correlated with the levels of mean pulmonary arterial pressure. Importantly, we evaluated the diagnostic performance of MPI and found that MPI has a strong diagnostic accuracy in PAH patients. The findings from this study will be of interest to clinical investigators who make diagnosis and therapeutic strategies for PAH patients.


2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093129
Author(s):  
Seda Tanyeri ◽  
Ozgur Y. Akbal ◽  
Berhan Keskin ◽  
Aykun Hakgor ◽  
Ali Karagoz ◽  
...  

We evaluated whether updated pulmonary hypertension definitive criteria proposed in sixth World Symposium on Pulmonary Hypertension had an impact on diagnosis of overall pulmonary hypertension and pre-capillary and combined pre- and post-capillary phenotypes as compared to those in European Society of Cardiology/European Respiratory Society 2015 pulmonary hypertension Guidelines. Study group comprised the retrospectively evaluated 1300 patients (age 53.1 ± 18.8 years, female 807, 62.1%) who underwent right heart catheterization with different indications between 2006 and 2018. Mean pulmonary arterial pressure ≥25 mmHg (European Society of Cardiology) and PAMP (mean pulmonary arterial pressure) >20 mmHg (World Symposium on Pulmonary Hypertension) right heart catheterization definitions criteria were used, respectively. For pre-capillary pulmonary hypertension, pulmonary artery wedge pressure ≤15 mmHg and pulmonary vascular resistance ≥3 Wood units criteria were included in the both definitions. Normal mean pulmonary arterial pressure (<21 mmHg), borderline mean pulmonary arterial pressure elevation (21–24 mmHg), and overt pulmonary hypertension (≥25 mmHg) were documented in 21.1, 9.8, and 69.1% of the patients, respectively. The pre-capillary and combined pre- and post-capillary pulmonary hypertension were noted in 2.9 and 1.1%, 8.7 and 2.5%, and 34.6 and 36.6% of the patients with normal mean pulmonary arterial pressure, borderline, and overt pulmonary hypertension subgroups, respectively. The World Symposium on Pulmonary Hypertension versus European Society of Cardiology/European Respiratory Society definitions resulted in a net 9.8% increase in the diagnosis of overall pulmonary hypertension whereas increases in the pre-capillary pulmonary hypertension and combined pre- and post-capillary pulmonary hypertension diagnosis were only 0.8 and 0.3%, respectively. The re-definition of mean pulmonary arterial pressure threshold seems to increase the frequency of the overall pulmonary hypertension diagnosis. However, this increase was mainly originated from those in post-capillary pulmonary hypertension subgroup whereas its impact on pre-capillary and combined pre- and post-capillary pulmonary hypertension was negligible. Moreover, criteria of pre-capillary pulmonary vascular disease and combined pre- and post-capillary phenotypes were still detectable even in the presence of normal mean pulmonary arterial pressure. The obligatory criteria of pulmonary vascular resistance ≥3 Wood units seems to keep specificity for discrimination between pre-capillary versus post-C pulmonary hypertension after lowering the definitive mean pulmonary arterial pressure threshold to 20 mmHg.


2019 ◽  
Vol 21 (1) ◽  
Author(s):  
Zixuan Pan ◽  
Alberto M. Marra ◽  
Nicola Benjamin ◽  
Christina A. Eichstaedt ◽  
Norbert Blank ◽  
...  

Abstract Objective The objective of this randomized, placebo-controlled, double-blind, parallel group, trial was to assess the effect of ambrisentan on mean pulmonary arterial pressure (mPAP) in patients with systemic sclerosis (SSc) and mildly elevated pulmonary hypertension (PH). Methods Thirty-eight SSc patients with mildly elevated mPAP at rest between 21 and 24 mmHg and/or > 30 mmHg during low-dose exercise were randomly assigned to treatment with either ambrisentan 5–10 mg/day or placebo. Right heart catheterization and further clinical parameters were assessed at baseline and after 6 months. The primary endpoint was the difference of mPAP change at rest between groups. Results After 6 months, the two groups did not differ in the primary endpoint (ambrisentan mPAP − 1 ± 6.4 mmHg vs. placebo − 0.73 ± 3.59 mmHg at rest, p = 0.884). However, three patients from the placebo group but none of the ambrisentan group progressed to SSc-associated pulmonary arterial hypertension. Furthermore, ambrisentan treatment showed significant improvements in the secondary endpoints cardiac index (CI) and pulmonary vascular resistance (PVR) at rest (CI 0.36 ± 0.66 l/min/m2 vs. − 0.31 ± 0.71 l/min/m2, p = 0.010; PVR − 0.70 ± 0.78 WU vs. 0.01 ± 0.71 WU, p = 0.012) and during exercise (CI 0.7 ± 0.81 l/min/m2 vs. − 0.45 ± 1.36 l/min/m2, p = 0.015; PVR − 0.84 ± 0.48 WU vs. − 0.0032 ± 0.34 WU, p < 0.0001). Conclusion This is the first randomized, double-blind, placebo-controlled study testing the effect of ambrisentan in patients with mildly elevated mPAP and/or exercise PH. The primary endpoint change in mPAP did only tendentially improve in the ambrisentan group, but the significant improvement of other hemodynamic parameters points to a possible benefit of ambrisentan and will be helpful to design future trials. Trial registration www.ClinicalTrials.gov, unique identifier NCT: NCT02290613, registered 14th of November 2014.


1983 ◽  
Vol 54 (1) ◽  
pp. 262-266 ◽  
Author(s):  
B. Nemery ◽  
W. Wijns ◽  
L. Piret ◽  
F. Cauwe ◽  
L. Brasseur ◽  
...  

In the human upright lung the downward increase in lung perfusion reverses in the lower third, thus giving rise to a zone of reduced basal perfusion (zone 4). The flow in zone 4 is regulated by the extra-alveolar vessels, the diameter of which is determined by lung volume, perivascular interstitial pressure, and vasomotor tone. To estimate the role of pulmonary vascular tone in the formation of zone 4, we infused nitroprusside (NTP), a potent pulmonary vasodilator, in six normal seated subjects. We measured their regional perfusion distribution using 133Xe in control conditions and at two dose levels of NTP (20.8 and 52.1 micrograms/min). Regional perfusion distribution was measured similarly and according to the same protocol in six subjects receiving only a placebo solution. In four of the six subjects receiving NTP, right-heart catheterization allowed simultaneous estimations of cardiac output and pulmonary arterial pressure to be made. NTP slightly decreased the perfusion of the nondependent parts of the lungs and markedly increased the perfusion of the lung bases, thus reducing the extent of zone 4. No changes were observed in the placebo experiments. Cardiac output and indices of ventilation and gas exchange did not change significantly. Peripheral and pulmonary arterial pressure fell slightly but significantly during NTP infusion. We attribute the observed changes in basal perfusion to the vasodilatory effects of NTP on the extra-alveolar vessels. Our findings thus support the hypothesis that in normal subjects zone 4 is partly created by the pulmonary vascular tone.


2000 ◽  
Vol 278 (1) ◽  
pp. H8-H15 ◽  
Author(s):  
Hunter C. Champion ◽  
Douglas J. Villnave ◽  
Allen Tower ◽  
Philip J. Kadowitz ◽  
Albert L. Hyman

The present study employed a new right-heart catheterization technique to measure pulmonary arterial pressure, pulmonary arterial wedge pressure, and pulmonary vascular resistance in anesthetized intact-chest, spontaneously breathing mice. Under fluoroscopic guidance, a specially designed catheter was inserted via the right jugular vein and advanced to the main pulmonary artery. Cardiac output was determined by the thermodilution technique, and measured parameters were stable for periods of ≤3 h. Pressure-flow curves in vivo were curvilinear, with mean pulmonary arterial pressure increasing more rapidly at low pulmonary blood flows of 5–10 ml/min and less rapidly at higher blood flow rates. The pressure-flow relationship was shifted to the left by the nitric oxide synthase inhibitor nitro-l-arginine methyl ester (l-NAME) at higher blood flow levels, whereas the cyclooxygenase inhibitor sodium meclofenamate was without effect. The increase in pulmonary arterial pressure in response to acute hypoxia (fractional inspired O2 10%) was augmented byl-NAME but unaltered by sodium meclofenamate. The present results demonstrate that the right-heart catheterization technique can be used to measure pulmonary vascular pressures and responses in the mouse. This is, to our knowledge, the first report of a right-heart catheterization technique to measure pulmonary vascular pressures and responses in the intact-chest, spontaneously breathing mouse and should prove useful for the investigation of pulmonary vascular responses in transgenic mice.


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