Hipertensión portopulmonar: Revisión actualizada

Rodríguez-Almendros, Nielzer Armando; Toapanta‐Yanchapaxi, Liz; Valadez, Jonathan Aguirre; Zavaleta, Nilda Espínola; Muñoz-Martínez, Sergio; García‐Juárez, Ignacio

doi:10.1016/j.acmx.2016.11.002

Cited by 3 publications

(21 citation statements)

References 83 publications

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“…The following data were obtained from medical records by well-trained rheumatologists: gender, age at entry, duration of SLE, involved organs, lupus disease activity, and laboratory data. Lupus disease activity was evaluated using the SLE disease activity index 2000 (SLE-DAI-2K), stratified to stable (<5), mild active (5)(6)(7)(8)(9), moderate active (10)(11)(12)(13)(14), and severe active (>14). The severity of liver diseases was evaluated with the Child-Pugh score.…”

Section: Clinical and Laboratory Datamentioning

confidence: 99%

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Clinical Characteristics of Systemic Lupus Erythematosus with Cirrhosis

You

Fei

Wang

et al. 2020

Journal of Immunology Research

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Aim. Cirrhosis is rare in systemic lupus erythematosus (SLE) patients with a poor prognosis. This study is aimed at retrospectively analyzing our single-center experience to explore the characteristics of cirrhosis in SLE patients. Methods. SLE patients with cirrhosis from 2012 to 2019 were enrolled. SLE diagnosis was rigorously confirmed by a medical record review according to the revised 1997 American College of Rheumatology classification criteria for SLE. The diagnosis of liver cirrhosis was based on a combination of clinical, laboratory, and imaging criteria features. We conducted a case-control study in SLE patients complicated with the cirrhosis group and the age-, sex-, and entry-time-matched noncirrhosis group. Results. A total of 21 patients with SLE cirrhosis were enrolled, 3 males and 18 females. The median age at the time of cirrhosis diagnosis was 47.3±4.0 years, and the mean disease duration of SLE before cirrhosis was 4.7±1.0 years. The most common initial presentation was the involvement of the hematological system in 9 patients and then skin and mucosal involvement in 5 patients, arthritis in 4 patients, and nephritis in 3 patients. Patients with cirrhosis had a significantly higher rate of hematological system involvement (thrombocytopenia and leukopenia) and worse liver function; a higher level of immune globulin G had higher mortality (p<0.05) than patients without cirrhosis. Conclusions. Cirrhosis is a rare and severe subtype of SLE with a poor prognosis. Those patients with hematological system involvement and impaired liver function should be paid more attention.

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Section: Clinical and Laboratory Datamentioning

confidence: 99%

“…The rate of PAH is also higher in the cirrhosis group. Portopulmonary hypertension (PPHT) is a respiratory complication of portal hypertension and is the 4th subtype in frequency in the group of pulmonary arterial hypertension [14]. The pathogenesis of PPHT may result from an imbalance between vasoconstrictor and vasodilating factors [15].…”

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confidence: 99%

Clinical Characteristics of Systemic Lupus Erythematosus with Cirrhosis

You

Fei

Wang

et al. 2020

Journal of Immunology Research

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“…Most patients are diagnosed between the fourth and fifth decade of life, unlike patients with idiopathic pulmonary arterial hypertension (IPAH) 1,12,13 . However, patients with an extrahepatic cause of PHT tend to be younger than those with liver cirrhosis as the underlying cause of POPH 14 .…”

Section: Epidemiologymentioning

confidence: 99%

“…In the literature, the term systolic pulmonary artery pressure (SPAP) is frequently used as the main echocardiographic measurement in the context of PH. But there is much data that can be obtained by TTE, including pulmonary regurgitation end-diastolic velocity, RV/left ventricular ratio, RV outflow tract acceleration time, tricuspid annular plane displacement in systole, RV isovolumetric relaxation time, RV wall thickness, inferior vena cava (IVC) inspiratory collapse degree 14 , RV performance index, RV tissue doppler index, eccentricity index, CO and LV diastolic function, valvular morphology/ function, cardiac defects, and right atrial area 3,14 .…”

Section: Diagnosismentioning

confidence: 99%

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Portopulmonar hypertension: Is there something new?

Toapanta‐Yanchapaxi¹,

Sánchez-Ávila²,

Rodríguez-Almendros³

et al. 2017

J Rare Dis Res Treat

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Portopulmonary hypertension (POPH) is a rare and life-threatening complication in patients with portal hypertension, with a prevalence of 3 -8%. It is characterized by pulmonary arterial hypertension (PAH) and results from obstruction to arterial flow in the pulmonary arterial bed, leading to the progressive deterioration of both the pulmonary circulation due to arterial remodeling and of the heart, as a result of right ventricular failure. Its diagnosis is based on hemodynamic findings based on a mean pulmonary arterial pressure (mPAP) ≥ 25mmHg, an increase in pulmonary vascular resistance (PVR) > 3 Wood Units or > 240 dynes/s/cm -5 , a pulmonary artery occlusion pressure (PAOP) ≤ 15mmHg or an elevated transpulmonary gradient (mPAP -PAOP: > 12 mmHg). Right heart catheterization (RHC) should be appropriately interpreted since management and MELD exception criteria depend on it. Although most therapeutic modalities have been inferred from patients with PAH, currently, new treatments are available and also various POPH clinical trials are ongoing, so further research data will soon be available. LT is a pivotal therapeutic option, but LT candidates require careful monitoring before, during and after the procedure.

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Portopulmonary Hypertension: An Updated Review

Jasso-Baltazar,

Peña-Arellano,

Aguirre-Valadez

et al. 2023

Transplantation Direct

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Portal hypertension may have major consequences on the pulmonary vasculature due to the complex pathophysiological interactions between the liver and lungs. Portopulmonary hypertension (PoPH), a subset of group 1 pulmonary hypertension (PH), is a serious pulmonary vascular disease secondary to portal hypertension, and is the fourth most common subtype of pulmonary arterial hypertension. It is most commonly observed in cirrhotic patients; however, patients with noncirrhotic portal hypertension can also develop it. On suspicion of PoPH, the initial evaluation is by a transthoracic echocardiogram in which, if elevated pulmonary pressures are shown, patients should undergo right heart catheterization to confirm the diagnosis. The prognosis is extremely poor in untreated patients; therefore, management includes pulmonary arterial hypertension therapies with the aim of improving pulmonary hemodynamics and moving patients to orthotopic liver transplantation (OLT). In this article, we review in detail the epidemiology, pathophysiology, process for diagnosis, and most current treatments including OLT and prognosis in patients with PoPH. In addition, we present a diagnostic algorithm that includes the current criteria to properly select patients with PoPH who are candidates for OLT.

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Hipertensión portopulmonar: Revisión actualizada

Cited by 3 publications

References 83 publications

Clinical Characteristics of Systemic Lupus Erythematosus with Cirrhosis

Clinical Characteristics of Systemic Lupus Erythematosus with Cirrhosis

Portopulmonar hypertension: Is there something new?

Portopulmonary Hypertension: An Updated Review

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