Portopulmonary hypertension

and, Rodrigo Cartin-Ceba; Krowka, Michael J.

doi:10.1002/9781118412640.ch19

Cited by 7 publications

(18 citation statements)

References 81 publications

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“…Despite the current availability of 12 Food and Drug Administration–approved PAH medications for the treatment of PAH, there are no controlled studies to guide POPH treatment selection. POPH treatment is, therefore, based on case reports and small case series . In 6 of 7 POPH patients, Khaderi et al successfully used 24/7 intravenous epoprostenol as their pre‐LT agent to reduce the mean pulmonary artery pressure to less than 35 mm Hg and the pulmonary vascular resistance to less than 400 dyne·s·cm −5 .…”

Section: Optimal Poph Therapy?mentioning

confidence: 99%

“…Early in the Mayo Clinic experience with transplantation for POPH patients, intravenous epoprostenol was an attractive option because of its success (improved hemodynamics and survival) with other forms of PAH . Subsequently, several investigators have reported the effectiveness of intravenous prostacyclin (epoprostenol or treprostinil) before LT …”

Section: Optimal Poph Therapy?mentioning

confidence: 99%

“…In addition to the current oral phosphodiesterase type 5 inhibitors (sildenafil and tadalafil) and endothelin receptor antagonists (bosentan and ambrisentan) used for POPH, we will likely see 3 recently Food and Drug Administration–approved agents tried in POPH patients: macitentan (an oral dual endothelin receptor antagonist), riociguat (an oral guanylate cyclase stimulator), and extended‐release treprostinil (an oral form of prostacyclin) …”

Section: Optimal Poph Therapy?mentioning

confidence: 99%

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Portopulmonary hypertension: Formidable dual threat versus hopeful dual therapy

Krowka

Cartin‐Ceba

2014

Liver Transpl

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Portopulmonary hypertension (POPH) poses a clinically important dual threat. It is defined as pulmonary arterial hypertension (PAH) in association with portal hypertension. Pretransplant mortality in POPH patients has been equally distributed between complications of liver disease (gastrointestinal bleeding, infections, and multiorgan failure) and cardiac issues (right heart failure). In studies involving more than 400 POPH patients, the 5-year POPH survival rate ranged from 40% to 68% for those treated with PAHspecific therapy and from only 2% to 14% for those not treated at all. [1][2][3][4] For those POPH patients being considered for liver transplantation (LT) and treated with PAH-specific drugs, 3 pertinent questions arise: Is there an optimal pre-LT medical therapy for POPH? What is the risk for LT in the presence of treated POPH? Will POPH be resolved if LT is performed? Although a small series, this contribution by Khaderi et al. 5 provides a single-institution, long-term experience for addressing these questions. OPTIMAL POPH THERAPY?Despite the current availability of 12 Food and Drug Administration-approved PAH medications for the treatment of PAH, there are no controlled studies to guide POPH treatment selection. POPH treatment is, therefore, based on case reports and small case series. 6 In 6 of 7 POPH patients, Khaderi et al. 5 successfully used 24/7 intravenous epoprostenol as their pre-LT agent to reduce the mean pulmonary artery pressure to less than 35 mm Hg and the pulmonary vascular resistance to less than 400 dyneÁsÁcm 25 . Certainly other PAH-specific agents can attain similar results, but the intravenous prostacyclin approach was the first very successful "kid on the block" to demonstrate significant improvements in pulmonary hemodynamics and survival in the PAH world. Early in the Mayo Clinic experience with transplantation for POPH patients, intravenous epoprostenol was an attractive option because of its success (improved hemodynamics and survival) with other forms of PAH. 7 Subsequently, several investigators have reported the effectiveness of intravenous prostacyclin (epoprostenol or treprostinil) before LT. 6 In addition to the current oral phosphodiesterase type 5 inhibitors (sildenafil and tadalafil) and endothelin receptor antagonists (bosentan and ambrisentan) used for POPH, 6 we will likely see 3 recently Food and Drug Administration-approved agents tried in POPH patients: macitentan (an oral dual endothelin receptor antagonist), 8 riociguat (an oral guanylate cyclase stimulator), 9 and extended-release treprostinil (an oral form of prostacyclin). 10 Reports of the effects of these newer medications (as well as others) on portal hypertension per se and the outcomes of POPH from controlled, multicenter studies will be welcomed by those managing POPH. RISK FOR LT?Moderate-to-severe POPH (mean pulmonary artery pressure > 35 mm Hg) has been associated with intraoperative mortality and death due to right heart failure during transplant hospitalization. 11 However, those experiences w...

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Section: Optimal Poph Therapy?mentioning

confidence: 99%

Section: Optimal Poph Therapy?mentioning

confidence: 99%

Section: Optimal Poph Therapy?mentioning

confidence: 99%

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Portopulmonary hypertension: Formidable dual threat versus hopeful dual therapy

Krowka

Cartin‐Ceba

2014

Liver Transpl

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“…Moderate to severe pulmonary hypertension, defined as a mean pulmonary arterial pressure (mPAP) on RHC of 35-45 mmHg should be evaluated further with vasodilator challenge. Positive response to vasodilator therapy is favorable and associated with improved outcomes after OLT (7). The presence of PoPHTN is estimated between 5% and 9% of all patients evaluated to undergo liver transplant (8,9).…”

Section: Introductionmentioning

confidence: 99%

Acute right ventricular failure after orthotopic liver transplantation

Goswami

Khouzam

2018

Ann. Transl. Med.

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The interdependence between the heart and liver in maintaining hemodynamic stability during the perioperative period of either orthotopic heart (OHT) or liver (OLT) transplantation is important. The pre-transplant hemodynamic changes that occur in patients with end-stage liver disease (ESLD) can include decreased systemic vascular resistance, poor ventricular response to stress and increased cardiac output (CO). Concomitant pulmonary disorders are often present in ESLD. Portopulmonary hypertension (PoPHTN) is an important marker for increased mortality in liver transplant patients. The pathophysiologic mechanisms specific to PoPHTN have been compared with other known forms of pulmonary hypertension, including primary pulmonary hypertension, and has been found to fall within a spectrum of disorders related to factors both due to intrinsic liver failure [with resultant portal hypertension and hepatopulmonary syndrome (HPS)] as well as pulmonary vascular remodeling. We present a 47-year-old Caucasian female with ESLD secondary to non-alcoholic steatohepatitis and HPS. Our current case demonstrates the difficulty in managing patients with acute pulmonary hypertension after OLT. Review of the contemporary literature demonstrated a total of eight case reports of post-transplant severe pulmonary hypertension thought to be due to a combination of either HPS or PoPHTN. This case highlights the complexities of patient management in the acute setting after OLT. Furthermore, it demonstrates the intricate role of careful preoperative evaluation and screening in patients undergoing workup for solid organ transplantation.

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“…Whereas the ultimate objective of PoPH treatment is to improve right ventricular function, the immediate goal is to restore pulmonary arterial flow. Although liver transplantation (LT) may lead to resolution of PoPH, ( 2 ) advanced stages may jeopardize safe and successful LT. ( 3 ) Hence, the concept of PoPH being a primary indication for LT remains controversial. ( 4 ) More specifically, cases with mPAPs exceeding 35 mm Hg are known to have increased mortality risk, ( 5 ) and inability to medically reduce mPAP to <35 mm Hg disqualifies patients for being granted a Model for End‐Stage Liver Disease (MELD) standard exception.…”

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confidence: 99%

Vasomodulators and Liver Transplantation for Portopulmonary Hypertension: Evidence From a Systematic Review and Meta‐Analysis

et al. 2020

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Background and Aims Untreated portopulmonary hypertension (PoPH) carries a poor prognosis. Previous reports have described vasomodulator (VM) therapy and liver transplantation (LT) as treatment options. We aimed to provide summary estimates on the endpoints of pulmonary hemodynamics and survival in patients with PoPH, treated with different modalities. Approach and Results We performed a systematic review with meta‐analysis of mainly observational and case‐control studies describing no treatment, VM, LT, or VM + LT in patients with PoPH. Twenty‐six studies (1,019 patients) were included. Both VM and VM + LT improve pulmonary hemodynamics. A substantial proportion of patients treated with VM become eligible for LT (44%; 95% confidence interval [CI], 31‐58). Pooled estimates for 1‐, and 3‐year postdiagnosis survival in patients treated with VM were 86% (95% CI, 81‐90) and 69% (95% CI, 50‐84) versus 82% (95% CI, 52‐95) and 67% (95% CI, 53‐78) in patients treated with VM + LT. Of note, studies reporting on the effect of VM mainly included Child‐Pugh A/B patients, whereas studies reporting on VM + LT mainly included Child‐Pugh B/C. Seven studies (238 patients) included both patients who received VM only and patients who received VM + LT. Risk of death in VM‐only–treated patients was significantly higher than in patients who could be transplanted as well (odds ratio, 3.5; 95% CI, 1.4‐8.8); however, importantly, patients who proceeded to transplant had been selected very strictly. In 50% of patients, VM can be discontinued post‐LT (95% CI, 38‐62). Conclusions VM and VM + LT both improve pulmonary hemodynamics and prognosis in PoPH. In a strictly selected subpopulation of cases where LT is indicated based on severe liver disease and where LT is considered safe and feasible, treatment with VM + LT confers a better prognosis. Considering successful VM, 44% can proceed to LT, with half being able to postoperatively stop medication.

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Portopulmonary hypertension

Cited by 7 publications

References 81 publications

Portopulmonary hypertension: Formidable dual threat versus hopeful dual therapy

Portopulmonary hypertension: Formidable dual threat versus hopeful dual therapy

Acute right ventricular failure after orthotopic liver transplantation

Vasomodulators and Liver Transplantation for Portopulmonary Hypertension: Evidence From a Systematic Review and Meta‐Analysis

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