Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview

Porres‐Aguilar, Mateo; Altamirano, José; Torre-Delgadillo, Aldo; Charlton, Michael; Duarte‐Rojo, Andrés

doi:10.1183/09059180.00007211

Cited by 105 publications

(136 citation statements)

References 99 publications

Supporting

Mentioning

124

Contrasting

Unclassified

Order By: Relevance

“…The pathophysiology of each is characterized by distinct changes in the pulmonary vasculature due to the combined effects of angiogenesis and multiple vasoactive substances, including nitric oxide, vascular endothelial growth factor, platelet-derived growth factor, and endothelin-1, among others. 1,2 The presence of intrapulmonary vascular dilatations (IPVDs) is the sine qua non for a diagnosis of HPS, and it results in ventilation-perfusion mismatching, diffusion limitation, and intrapulmonary shunting. Classic clinical features include arterial hypoxemia, platypnea, and orthodeoxia.…”

mentioning

confidence: 99%

Intrapulmonary vascular dilatations are common in portopulmonary hypertension and may be associated with decreased survival

et al. 2015

View full text Add to dashboard Cite

MinnesotaHepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are pulmonary vascular complications of portal hypertension with divergent clinicopathologic features and management. The presence of intrapulmonary vascular dilatations (IPVDs), detected by agitated saline contrast-enhanced transthoracic echocardiography (cTTE), is an essential feature of HPS but is not typically characteristic of POPH. Although IPVDs have been reported rarely in POPH, the prevalence and significance of this finding have not been systematically studied. We conducted a retrospective chart review of 80 consecutive patients diagnosed with POPH from January 1, 2002 to June 30, 2014 with documentation of cTTE findings, pulmonary hemodynamics, oxygenation, and survival. A total of 34 of the 80 patients (42%) underwent cTTE during initial diagnosis of POPH. IPVDs were detected in 20/34 patients (59%); intracardiac shunting was detected in 9/34 patients (26%; 4 also had IPVDs); and 9 patients (26%) had negative cTTE with no evidence of IPVD or intracardiac shunting. Patients with IPVD had decreased survival as compared to those without IPVD (P 5 0.003), a trend that persisted after exclusion of liver transplant recipients (P 5 0.07). The IPVD group had a trend toward higher Model for End-Stage Liver Disease score with and without incorporating sodium (MELD or MELD-Na; P 5 0.05 for both). The right ventricular index of myocardial performance (RIMP) was lower in the IPVD group (median, 0.4 versus 0.6; P 5 0.006). Patients with moderate or large IPVDs (n 5 6) had worse oxygenation parameters (partial pressure of arterial oxygen, diffusing capacity of the lung for carbon monoxide, and alveolar-arterial oxygen gradient) as compared to the rest of the cohort. Unexpectedly, IPVDs were frequently documented in POPH and associated with decreased survival. To further understand this observation, we recommend screening for IVPD in all patients with POPH. Liver Transpl 21:1355-1364, 2015. V C 2015 AASLD.Received March 25, 2015; accepted June 11, 2015. Abbreviations: A1AT, alpha-1-antitrypsin; A-a, alveolar-arterial; AIH, autoimmune hepatitis; ALD, alcoholic liver disease; ARDS, acute respiratory distress syndrome; ATN, acute tubular necrosis; CO, cardiac output; cTTE, contrast-enhanced transthoracic echocardiography; DLCO, diffusing capacity of the lung for carbon monoxide; DPG, diastolic pulmonary pressure gradient; ESLD, end-stage liver disease; HCC, hepatocellular carcinoma; HCV, hepatitis C virus; HPS, hepatopulmonary syndrome; INR, international normalized ratio; IPVD, intrapulmonary vascular dilatation; IQR, interquartile range; LT, liver transplantation; MELD, Model for End-Stage Liver Disease; MELD-Na, Model for End-Stage Liver Disease incorporating sodium; MPAP, mean pulmonary artery pressure; N/A, not available; Na, sodium; NASH, nonalcoholic steatohepatitis; PA, pulmonary artery; PAH, pulmonary arterial hypertension; PaO 2 , partial pressure of arterial oxygen; PCWP, pulmonary capillary wedge pressure; PEA, pulseless elect...

show abstract

mentioning

confidence: 99%

Intrapulmonary vascular dilatations are common in portopulmonary hypertension and may be associated with decreased survival

et al. 2015

View full text Add to dashboard Cite

show abstract

“…Aetiology of pulmonary vascular dilatation in cirrhosis is thought to relate to an increase in pulmonary NO by means of both endothelial and inducible NO synthase (eNOS and iNOS) [2][3][4].…”

Section: Discussionmentioning

confidence: 99%

Liver disease masquerading as primary cardiopulmonary disease: Hepatopulmonary syndrome as a result of idiopathic cirrhosis

Aravinda¹,

Ratnatilaka²,

Sadikeen³

2017

IJCRI

View full text Add to dashboard Cite

“…On the other hand, liver patients with PVH have a PAWP > 15 mm Hg. (1,4) To further differentiate between the 2 forms of PVH, patients with elevated pulmonary artery pressures due to high CO tend to have a high normal to mildly elevated PAWP compared to states of elevated blood volume due to intercompartmental shifts. We have described 3 patients with PoPH who at varying durations after their initial diagnosis were noted to have a change in hemodynamic profile to PVH, an entity not routinely seen in patients with other forms of group I PH.…”

Section: Discussionmentioning

confidence: 99%

“…Notably, a majority of these patients have concomitant left heart disease, commonly diastolic dysfunction. (4) Vigorous diuresis is often limited by azotemia inherent in managing this group of patients.…”

mentioning

confidence: 99%

Novel role of midodrine in pulmonary hypertension in liver transplant candidates

Rajaram¹,

Spivey

Fisher³

2016

Liver Transpl

View full text Add to dashboard Cite

Pulmonary hypertension (PH) in liver transplant candidates is associated with high morbidity and mortality in the pre-and post-liver transplantation period. There is a high incidence of acute right heart failure and death at the time of reperfusion when PH is not wellcontrolled preoperatively.(1,2) Portopulmonary hypertension (PoPH) is a rare form of pulmonary arterial hypertension (PAH) in patients with portal hypertension, with or without end-stage liver disease. The official definition includes the presence of portal hypertension with mean pulmonary arterial pressure (mPAP) 25 mm Hg, pulmonary artery wedge pressure (PAWP) 15 mm Hg, and pulmonary vascular resistance (PVR) > 3 Wood units (240 dyne/seconds/cm 25 ) as measured during right heart catheterization (RHC). Although the exact pathophysiology continues to be debated, the current understanding faults the shear stress acting on the pulmonary arteries by the high cardiac output (CO) state as one of the culprits for the vascular remodeling.(1,3)Pulmonary venous hypertension (PVH), increasingly recognized in liver transplant candidates, has a hemodynamic profile consistent with mPAP 25 mm Hg and PAWP > 15 mm Hg on RHC. The etiology of PVH in this population has been attributed either to high CO states or increased blood volume due to fluid shifts. Notably, a majority of these patients have concomitant left heart disease, commonly diastolic dysfunction.(4) Vigorous diuresis is often limited by azotemia inherent in managing this group of patients.The above-mentioned entities are thought to exist independent of each other. However, we have noted instances where interchange in the hemodynamic profiles between PoPH and PVH are seen in liver transplant candidates, particularly during periods of high CO. We describe 3 patients illustrating the novel role of midodrine in patients with this phenomenon. Patient 1A 47-year-old male with a history of cirrhosis presumed secondary to fatty liver disease presented with an echocardiogram showing a normal left ventricular ejection fraction (LVEF) and size, a severely dilated right ventricle (RV), a severely reduced RV function, and septal flattening. He was diagnosed with PoPH after RHC (Table 1). He was listed for liver transplantation after a decrease in mPAP < 35 mm Hg with 20 mg of sildenafil 3 times a day (tid), 5 mg of ambrisentan daily, and intravenous (IV) epoprostenol of 6 ng/ kg/minute.During his routine 3-month RHC while being listed, an increase in his PAWP to 23 mm Hg with an elevated CO (9.6 L/minute) was noted, which is consistent with PVH (Table 1). No significant hemodynamic changes were observed with increased diuresis, which necessitated the use of 10 mg of midodrine tid to increase systemic vascular resistance (SVR) in an effort to decrease his CO. Three weeks after initiation of midodrine, his hemodynamic profile returned to one more consistent with PoPH (Table 1). Despite an Abbreviations: CO, cardiac output; IV, intravenous; LVEF, left ventricular ejection fraction; mPAP, mean pulmonary arterial pressu...

show abstract

Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview

Cited by 105 publications

References 99 publications

Intrapulmonary vascular dilatations are common in portopulmonary hypertension and may be associated with decreased survival

Intrapulmonary vascular dilatations are common in portopulmonary hypertension and may be associated with decreased survival

Liver disease masquerading as primary cardiopulmonary disease: Hepatopulmonary syndrome as a result of idiopathic cirrhosis

Novel role of midodrine in pulmonary hypertension in liver transplant candidates

Contact Info

Product

Resources

About