Attribute latencies causally shape intertemporal decisions.

Intertemporal choices - decisions that play out over time - pervade our life. Thus, how people make intertemporal choices is a fundamental question. Here, we investigate the role of attribute latency (the time between when people start to process different attributes) in shaping intertemporal preferences using five experiments with choices between smaller-sooner and larger-later rewards. In the first experiment, we identify attribute latencies using mouse-trajectories and find that they predict individual differences in choices, response times, and changes across time constraints. In the other four experiments we test the causal link from attribute latencies to choice, staggering the display of the attributes. This changes attribute latencies and intertemporal preferences. Displaying the amount information first makes people more patient, while displaying time information first does the opposite. These findings highlight the importance of intra-choice dynamics in shaping intertemporal choices and suggest that manipulating attribute latency may be a useful technique for nudging.

Evaluating the efficacy and safety of oral triple sequential combination therapy for treating patients with pulmonary arterial hypertension: A multicenter retrospective study.

This study aimed to evaluate the effectiveness and safety of an oral sequential triple combination therapy with selexipag after dual combination therapy with endothelin receptor antagonist (ERA) and phosphodiesterase-5 inhibitor (PDE5I)/riociguat in pulmonary arterial hypertension (PAH) patients. A total of 192 PAH patients from 10 centers had received oral sequential selexipag therapy after being on dual-combination therapy with ERA and PDE5i/riociguat for a minimum of 3 months. Clinical data were collected at baseline and after 6 months of treatment. The study analyzed the event-free survival at 6 months and all-cause death over 2 years. At baseline, the distribution of patients among the risk groups was as follows: 22 in the low-risk group, 35 in the intermediate-low-risk group, 91 in the intermediate-high-risk group, and 44 in the high-risk group. After 6 months of treatment, the oral sequential triple combination therapy resulted in reduced NT-proBNP levels (media from 1604 to 678 pg/mL), a decline in the percentage of WHO-FC III/IV (from 79.2% to 60.4%), an increased in the 6MWD (from 325 ± 147 to 378 ± 143 m) and a rise in the percentage of patients with three low-risk criteria (from 5.7% to 13.5%). Among the low-risk group, there was an improvement in the right heart remodeling, marked by a decrease in right atrium area and eccentricity index. The intermediate-low-risk group exhibited significant enhancements in WHO-FC and tricuspid annular plane systolic excursion. For those in the intermediate-high and high-risk groups, there were marked improvements in activity tolerance, as reflected by WHO-FC and 6MWD. The event-free survival rate at 6 months stood at 88%. Over the long-term follow-up, the survival rates at 1 and 2 years were 86.5% and 86.0%, respectively. In conclusion, the oral sequential triple combination therapy enhanced both exercise capacity and cardiac remodeling across PAH patients of different risk stratifications.

Plasma-induced nitrogen vacancy-mediated ammonia synthesis over a VN catalyst.

Plasma catalysis has recently been recognized as a promising route for artificial N2 reduction under mild conditions. Here we report a highly active VN catalyst for plasma-catalytic NH3 synthesis via the typical Mars-van Krevelen (MvK) mechanism. Our results indicate that NH3 synthesis occurs through the continuous regeneration and elimination of nitrogen vacancies on the VN surface. With this strategy, the VN catalyst achieves a superhigh NH3 yield of 143.2 mg h-1 gcat.-1 and a competitive energy efficiency of 1.43 gNH3 kW h-1.

Impact of different sequential triple oral combination therapies based selexipag on outcomes in pulmonary arterial hypertension.

BACKGROUND: While the GRIPHON study and others have confirmed the efficacy and safety of selexipag with single, dual, and initial triple combination therapy for patients with pulmonary arterial hypertension (PAH), multicenters studies concerning diverse triple oral combination therapies based on selexipag are limited. HYPOTHESIS: This study was conducted to evaluate the effects of various sequential triple oral combination therapies on PAH outcomes. METHODS: A retrospective study was carried out involving 192 patients from 10 centers, who were receiving sequential triple oral combination therapy consisting of an endothelin receptor antagonist (ERA), a phosphodiesterase 5 inhibitor (PDE5i)/riociguat and selexipag. Clinical parameters, event-free survival, and all-cause survival were assessed and analyzed at baseline and posttreatment. RESULTS: Among the 192 patients, 37 were treated with ERA + riociguat + selexipag, and 155 patients received ERA + PDE5i + selexipag. Both sequential triple oral combination therapies improved the World Health Organization functional class and raised the count of low-risk parameters. As a result of the larger patients' population in the ERA + PDE5i + selexipag group, these individuals exhibited significant increases in 6-minute walking distance (6MWD), pulmonary arterial systolic pressure, pulmonary arterial pressure, right ventricle, and eccentricity index, and significant decreases in N-terminal probrain natriuretic peptide after 6 months of treatment. Nevertheless, both sequential triple oral combination therapy groups demonstrated similar shifts in these clinical parameters between baseline and 6 months. Baseline 6MWD and mean pulmonary arterial pressure were independent predictors of survival in patients undergoing ERA + PDE5i + selexipag therapy. Importantly, no significant differences were found in 6-month event-free survival and all-cause survival between two groups. CONCLUSIONS: Different oral sequential triple combination therapies based on selexipag could comparably improve outcomes in patients with PAH.

Correction: Strengthening Pt/WOx interfacial interactions to increase the CO tolerance of Pt for hydrogen oxidation reaction.

Correction for 'Strengthening Pt/WOx interfacial interactions to increase the CO tolerance of Pt for hydrogen oxidation reaction' by Daojun Long et al., Chem. Commun., 2023, https://doi.org/10.1039/d3cc03990k.

Strengthening Pt/WOx interfacial interactions to increase the CO tolerance of Pt for hydrogen oxidation reaction.

Here, the modulation of the Pt electronic structure by the formation of an amorphous WOx overlayer on Pt nanoparticles is proposed. The resulting Pt/WOx@NC electrode shows exceptional CO oxidation potential (0.24 V vs. RHE) in aqueous test, and the corresponding membrane electrode assembly (MEA) steadily generates power in fuel cells fed with H2 gas containing 1000 ppm CO.

Percutaneous Left Atrial Appendage Closure With a Novel LAA Occluder for Stroke Prevention in Atrial Fibrillation.

Background: More than 90% of thromboses originate from the left atrial appendage (LAA) in patients with nonvalvular atrial fibrillation (NVAF). Objectives: This study was designed to investigate the safety and efficacy of LAA closure with the Leftear device (Pulse Scientific) in NVAF patients. Methods: A prospective, multicenter, registry-based study was conducted in 200 NVAF patients with CHA2DS2-VASc (congestive heart failure, hypertension, age, diabetes, previous stroke/transient ischemic attack, vascular disease, female sex) scores ≥2. The primary safety endpoint was defined as any serious adverse events. Efficacy was assessed by a primary composite endpoint of hemorrhagic or ischemic stroke, systemic embolism, and cardiac or unexplained death at 1 year of follow-up. Results: The device was implanted in 196 patients, with 1-stop LAA closure combined with atrial fibrillation ablation implemented in 133 patients. The immediate success rate was 100%. There were serious adverse events in 9 patients (4.5%; 95% CI: 1.6%-7.4%), which mainly occurred in 1-stop LAA closure. All pericardial tamponades occurred in 6 patients with 1-stop LAA closure. No patient experienced a major bleeding event or acute device-related thrombus. During the 12-month follow-up period, the risk of the primary composite endpoint was 1.6% (95% CI: 0.3%-4.5%), and statistical noninferiority was achieved (the upper bound of 95% CI: 4.5% < the prespecified maximum annual incidence of 8.0%). Ischemic stroke occurred in 1 patient, 3 patients had incomplete LAA sealing, and no delayed device-related thrombus was found. Conclusions: LAA closure with the novel disc-like occluder shows high procedural success, satisfactory safety, and encouraging efficacy for stroke prevention in patients with NVAF. Compared with 1-stop LAA closure, single LAA closure may be more tolerable. (A multicenter, single-arm clinical trial to evaluate the efficacy and safety of left atrial appendage system for left atrial appendage occlusion in patients with non-valvular atrial fibrillation; ChiCTR1900023035).

Left atrial appendage closure in nonvalvular atrial fibrillation patients with percutaneous coronary intervention.

OBJECTIVES: Nonvalvular atrial fibrillation (NVAF) concomitant with coronary artery disease (CAD) may increase the risk of thromboembolism. Antithrombotic therapy for NVAF patients with percutaneous coronary intervention (PCI) remains contradictory and challenging. This study aimed to assess the safety and efficacy of left atrial appendage closure (LAAC) in a cohort of patients with NVAF and PCI. METHODS: A total of 109 patients undergoing LAAC procedures between March 2017 and December 2020 were categorized into 2 groups, Group I included 36 patients with PCI while group II included 73 patients without. Peri-procedural and long-term complications, as well as ischemia and bleeding events, were retrospectively analyzed. RESULTS: Group I had more diabetes mellitus (55.6% vs. 26.0%; p = 0.003), higher CHA2DS2-VASc scores (5.44 ± 1.85 vs. 4.22 ± 1.64; p = 0.002) and HAS-BLED scores (3.39 ± 0.93 vs. 2.74 ± 1.05; p = 0.003) compared to Group II. Procedure-related complications within 7 days were similar in both groups (8.3% vs. 8.2%; P = 1.000). Over a median follow-up period of 20.9 months, there were no significant differences between two subgroups with regard to cardiovascular death (2.8% vs. 0%, p = 0.330), stroke/transient ischemic attack (2.8% vs. 5.5%, p = 1.000), major bleeding (0% vs. 2.7%, p = 1.000) and device-related thrombus (8.3% vs. 1.4%, p = 0.104). The observed annualized thromboembolic and major bleeding events determined by Kaplan-Meier analysis decreased by 82.4% and 100% in group I, 55.9% and 75.8% in group II, respectively. CONCLUSION: LAAC is a safe and effective option for stroke prevention in NVAF patients with PCI.

A Study of the Efficacy and Safety of Aerobic Exercise Training in Pulmonary Arterial Hypertension (the Saturday Study): Protocol for a Prospective, Randomized, and Controlled Trial.

Background: Patients with pulmonary arterial hypertension (PAH) have reduced exercise capacity and poor quality of life. Exercise-based rehabilitation in PAH results in clinically relevant improvements in exercise capacity and hemodynamics. To clarify the mechanism, we will evaluate the effect of aerobic exercise training rehabilitation on right ventricular (RV) remodeling and function as determined measured by cardiac magnetic resonance imaging (CMR). Methods: We will conduct a 26-week multicenter randomized controlled trial. Patients on stable and unchanged PAH-targeted medication are randomly assigned (1:1) to the control and training groups. The primary endpoint is the RV stroke volume (RVSV) change from baseline to Week 26, determined by CMR. Comprehensive RV function is also performed using CMR. Other characteristics of the RV and left ventricle, World Health Organization functional class, 6-min walk distance, and N-terminal pro-B-type natriuretic peptide are included in secondary endpoints. We also investigate the proteomic, metabolomic, and transcriptomic changes after exercise training as exploratory endpoints. Ethics and Dissemination: The study and protocol were approved by the Ethics Committee of Shanghai Pulmonary Hospital (Approved No. of ethics committee: L20-17). The results will be disseminated at medical conferences and in journal publications. All participants will sign written informed consent. Trial Registration Number: ChiCTR2000031650.

Angry Women Are More Trusting: The Differential Effects of Perceived Social Distance on Trust Behavior.

Accumulating evidence suggests that anger can have a strong impact on discrete trust behaviors. However, the mechanisms underlying how anger influences trust are still unclear. Based on the appraisal tendency framework, we hypothesized that perceived social distance would positively mediate the effect of anger on trust, and that gender would moderate this mediation. In Study 1, a 2 (Anger vs. Control) × 2 (Men vs. Women) factorial design was used to investigate this hypothesis. Results supported our predictions that anger drove women, but not men, to perceive smaller social distance, and thus sent more money to their counterparts in a trust game as compared to controls. In Study 2, social distance was manipulated, and a 2 (Low social distance vs. Control) × 2 (Men vs. Women) factorial design was used to critically test the causal role of the mediator, namely to examine the effect of perceived social distance on trust. Results showed that women, but not men, sent more money to their counterparts in the low social distance condition than in the control condition. Results of both studies indicate that the high certainty, higher individual control, and approach motivation associated with anger could trigger optimistic risk assessment, and thus more trust toward others in women, via perceiving smaller social distance to others.

Transcatheter closure for patent ductus arteriosus in patients with Eisenmenger syndrome: to do or not?

BACKGROUND: Patent ductus arteriosus (PDA) complicated by Eisenmenger syndrome (ES) remains to be a major cause of morbidity and mortality worldwide. Giving increasing evidences of benefit from targeted therapies, ES patients once thought to be inoperable may have increasing options for management. This study aims to explore whether PDA in patients with ES can be treated with transcatheter closure (TCC). METHODS: Between August 2014 and July 2016, four of fifteen PDA-ES patients whose Qp/Qs improved significantly and Qp/Qs > 1.5 after acute vasodilator testing with 100% oxygen were selected to receive TCC and pulmonary vasodilator therapy. PAH-targeted drugs were prescribed before and after occlusion for all. Trial occlusion was performed before permanent closure. RESULTS: The first TCC failed after initiation of PAH-targeted drugs for 6 months in four patients. After the medication was adjusted and extended to 12 months, TCC was performed for all without hemodynamic intolerances during perioperative period. Pulmonary artery systolic pressure (PASP) was significantly decreased (≥ 40%) immediately after TCC. During a mean follow-up of 48 ± 14.70 months, there were a further decrease of PASPs in two patients, the other two showed improved pulmonary vascular resistance, WHO functional class and six-minute walking distance despite deteriorated PASP. CONCLUSION: Some selected PDA-ES patients might benefit from TCC and combined PAH-targeted drugs play a crucial role.

Impact of twice- or three-times-weekly maintenance hemodialysis on patient outcomes: A multicenter randomized trial.

AIM: Maintenance hemodialysis (MHD) frequency is associated with survival and complication rates. Achieving the optimal balance between healthcare, quality of life (QOL), and medical costs is challenging. We compared complications, inflammatory status, nutritional status, and QOL between patients with different MHD frequencies. MATERIAL AND METHODS: This was a multicenter randomized trial of patients treated between May 2011 and August 2017 at 3 tertiary hospitals in Wenzhou. Patients were grouped according to their treatment schedule over 1 year: twice-weekly or 3-times-weekly. Complications, biochemistry parameters, and QOL (KDQOL-SFTM 1.3 scale) were assessed. RESULTS: One hundred forty patients were included aged 29 to 68 years (mean age, 50.9 ±â€Š4.3 years). There were no significant differences in infection, heart failure, or cerebral hemorrhage complications between the 2 groups (P = .664). Pre-dialysis hemoglobin, high-sensitivity C-reactive protein, serum albumin, total cholesterol, triglyceride, calcium, phosphate, parathyroid hormone, and ejection fraction were similar in both groups (P > .05). After 1 year of MHD, both groups exhibited significant improvements in these parameters (all P < .05) with no significant differences between groups. Serum creatinine, blood urea nitrogen (BUN), and weekly standard hemodialysis treatment adequacy did not improve after treatment (all P > .05), although a difference in BUN was observed between the 2 groups (P < .001). QOL was superior in the twice-weekly group than in the 3-times-weekly group (all P < .05), except for social support, which was slightly better in the 3-times-weekly group than in the twice-weekly group. CONCLUSIONS: Twice- and 3-times-weekly MHD resulted in comparable inflammatory and nutritional clinical outcomes and adverse events. QOL was better for the twice-weekly schedule. Even for patients with economic constraints, twice- or 3-times-weekly MHD should be selected with caution after consideration of BUN levels at baseline.

Acute peripheral pulmonary embolism attributed to autoimmune haemolytic anaemia: a case report.

BACKGROUND: PE (pulmonary embolism) is a life-threatening complication rarely seen in the AIHA (autoimmune haemolytic anaemia) patients. Herein we reported a rare and serious AIHA-PE patient characterised by extensive peripheral pulmonary embolism on CTPA. CASE PRESENTATION: A 59-year-old woman presented to our ED (emergency department) complaining of acute chest pain and dyspnea. During her presentation in ED she experienced a sudden syncope and soon developed CA (cardiac arrest). Laboratory studies showed a increase of CK-MB,troponin T,myoglobin and D-dimer. Computed tomography pulmonary angiography (CTPA) showed no large central or segment pulmonary emboli but increased RV (right ventricle)size,enlarged main pulmonary artery and invisible peripheral pulmonary artery. She was diagnosed with acute PE and alteplase was delivered intravenously. After thrombolytic therapy she remained hypotension and developed worsening anaemia. Detailed examination for anaemia revealed AIHA. She was discharged in a stable condition after 5 weeks with methylprednisolone and warfarin. Hb, D-dimer and transthoracic echocardiography showed complete recovery at 3-months follow up. CONCLUSION: PE attributed to AIHA is characterized by subsegment and distal pulmonary artery embolism which is easily neglected but always life-threatening. This case also highlights the PE as a secondary diagnosis should be evaluated comprehensively in order to identify the underlying pathogenesis.

The Differential Effects of Anger on Trust: A Cross-Cultural Comparison of the Effects of Gender and Social Distance.

Accumulating empirical evidence suggests that anger elicited in one situation can influence trust behaviors in another situation. However, the conditions under which anger influences trust are still unclear. The present study addresses this research gap and examines the ways in which anger influences trust. We hypothesized that the social distance to the trustee, and the trusting person's gender would moderate the effect of anger on trust. To test this hypothesis, a study using a 2 (Anger vs. Control) × 2 (Low vs. High social distance) × 2 (Men vs. Women) factorial design was conducted in Germany (N = 215) and in China (N = 310). Results reveal that in both countries men's trust behavior was not influenced by the manipulations (i.e., anger and social distance). The pattern for women, however, differed by country. In Germany, women's trust to a stranger (i.e., high social distance) was increased by anger; while in China, women's trust to someone who they have communicated with (i.e., low social distance) was increased by anger. These results indicate that women's trust levels seem to be more context-sensitive than men's.

Biased sequential sampling underlies the effects of time pressure and delay in social decision making.

Social decision making involves balancing conflicts between selfishness and pro-sociality. The cognitive processes underlying such decisions are not well understood, with some arguing for a single comparison process, while others argue for dual processes (one intuitive and one deliberative). Here, we propose a way to reconcile these two opposing frameworks. We argue that behavior attributed to intuition can instead be seen as a starting point bias of a sequential sampling model (SSM) process, analogous to a prior in a Bayesian framework. Using mini-dictator games in which subjects make binary decisions about how to allocate money between themselves and another participant, we find that pro-social subjects become more pro-social under time pressure and less pro-social under time delay, while selfish subjects do the opposite. Our findings help reconcile the conflicting results concerning the cognitive processes of social decision making and highlight the importance of modeling the dynamics of the choice process.

[Efficacy and safety of ambrisentan therapy in Chinese patients with pulmonary hypertension].

OBJECTIVE: To explore the efficacy, safety and tolerance of ambrisentan, a high-selective endothelin receptor antagonist, in Chinese patients with pulmonary hypertension (PH). METHODS: Twenty-eight PH patients (Group 1+Group 4) came from Shanghai East Hospital, Zhongshan Hospital of Fudan University and Fifth People's Hospital of Shanghai were recruited into this open-label, prospective multi-center trial between August 2012 and February 2013. They received 2.5-5.0 mg ambrisentan once daily for 12 weeks. The primary endpoint was the change in exercise capacity showed by six-minute walk distance (6MWD) from baseline to 12 weeks. Secondary endpoints included the changes in World Health Organization (WHO) function class, N-terminal brain natriuretic peptide (NT-proBNP) and liver function test results. RESULTS: There were 9 males and 19 females with an average age of (35 ± 17) years. The value of 6MWD increased from (372 ± 86) m at baseline to (443 ± 96) m (P = 0.000) after 12 weeks. WHO functional class improved after a 12-week therapy compared to the baseline level (P = 0.000). NT-proBNP decreased from a median of 732 ng/L at baseline to 329 ng/L after 12 weeks (P = 0.046). The baseline liver function test was normal. And liver function test didn't significantly change after a 12-week therapy. CONCLUSION: Ambrisentan therapy is well-tolerated and it improves the exercise capacity and WHO function class in Chinese PH patients.

Atorvastatin prevents dehydromonocrotaline-induced pulmonary hypertension in beagles.

BACKGROUND: Pulmonary arterial hypertension is a life-threatening disease characterized by marked and sustained elevation of blood pressure in the lungs. Statins, 3-hydroxy-3-methylglutaryl CoA (HMG-CoA) reductase inhibitors, have been shown to attenuate the effects of pulmonary hypertension resulting from hypoxia, Monocrotaline exposure, or Monocrotaline exposure in the setting of pneumonectomy. In particular, the effects of Simvastatin have been well studied. Whether other statins, such as Atorvastatin, are capable of preventing dehydromonocrotaline-induced pulmonary hypertension in beagles has not been explored. METHODS: We used eighteen 3-month-old beagles of both genders, weighing 10.3 ± 3.2 kg. The experimental animals were randomized into one of 3 groups: the control group (n = 6), the dehydromonocrotaline (DHMC) + vehicle group (n = 5), and the DHMC + Atorvastatin group (n = 7). The beagles were injected with DHMC (n = 12) on day 1, and from day 5 to day 65 they received Atorvastatin (2 mg/kg, daily by gavage) or vehicle (0.9% saline, daily by gavage) treatment. We used the thermodilution method of hemodynamic measurements at baseline and at day 65 of treatment. At day 65, pulmonary tissue was sampled for morphometry and real-time quantitative PCR. RESULTS: After 65 days, DHMC increased mean pulmonary arterial pressure (mPAP), and this increase was prevented with Atorvastatin treatment (32 ± 11 mmHg vs. 15 ± 3 mmHg, P < .05). Hematoxylin and eosin staining demonstrated less pulmonary endothelium destruction and smooth muscle cell proliferation in the Atorvastatin-treated beagles, compared with the DHMC group. The eNOS mRNA expression was increased in the DHMC group, and this increase was prevented in the Atorvastatin-treated group. In addition, IL-1ß, prepro-ET-1, TNF-α, and VEGF (vascular endothelial growth factor) mRNA expression levels were increased in the lungs of the DHMC group, and these increases were reduced toward normal levels in the Atorvastatin-treated group. CONCLUSION: Atorvastatin prevents the effects of monocrotaline-induced pulmonary hypertension in beagles.

Serum high-density lipoprotein cholesterol levels as a prognostic indicator in patients with idiopathic pulmonary arterial hypertension.

High-density lipoprotein (HDL) cholesterol levels are a strong, independent inverse predictor of cardiovascular disease. The present study aimed to determine whether serum HDL cholesterol levels correlated with disease severity and clinical outcomes in patients with idiopathic pulmonary arterial hypertension (IPAH). The serum HDL cholesterol levels were measured in 76 Chinese patients with IPAH and 45 healthy controls, together with other clinical variables. Univariate and multivariate Cox proportional hazards analysis was performed to assess the prognostic value of HDL cholesterol and event-free survival. Event-free survival was estimated using the Kaplan-Meier method. Serum HDL cholesterol levels were significantly decreased in patients with IPAH compared with controls (1.0 ± 0.3 vs 1.5 ± 0.3 mmol/L; p <0.001). The serum HDL cholesterol levels decreased in proportion to the severity of World Health Organization functional class. Compared to the high HDL cholesterol group, the low HDL cholesterol group demonstrated a significantly lower 6-minute walking distance, cardiac index, mixed venous saturation, and arterial carbon dioxide pressure but significantly greater pulmonary vascular resistance and serum uric acid levels. The serum HDL cholesterol levels correlated positively with the cardiac index (r = 0.42; p = 0.002) and negatively with the pulmonary vascular resistance (r = -0.25; p = 0.04). Serum HDL cholesterol was independently related to event-free survival on multivariate Cox proportional hazards analysis. Kaplan-Meier survival curves according to the median HDL cholesterol value showed that lower HDL cholesterol levels were associated with lower event-free survival. In conclusion, serum HDL cholesterol levels might serve as an indicator of disease severity and prognosis in patients with IPAH.

[Clinical characteristics and survival of patients with pulmonary veno-occlusive disease].

OBJECTIVE: To investigate the clinical presentation, diagnosis, treatment and outcome of patients with pulmonary veno-occlusive disease (PVOD). METHODS: Data from patients diagnosed as PVOD from May 2008 to May 2011 in Shanghai Pulmonary Hospital, Tongji University were retrospectively reviewed. RESULTS: During this period, 5 patients [4 female, aged from 12 to 42 (22 ± 12) years old] were diagnosed as PVOD. The durations from symptoms onset to PVOD diagnosis was 2 to 50 (16 ± 20) months and four of them were previously diagnosed as idiopathic pulmonary arterial hypertension. All patients at the time of PVOD diagnosis had a severely impaired WHO pulmonary hypertension functional class (3 in class III and 2 in class IV). Furthermore, all patients characterized by a typical sign of centrilobular ground-glass opacities in high-resolution computed tomography, a markedly reduction of diffusing capacity of the lung for carbon monoxide [(38 ± 12)% of predicted value] in pulmonary functional test and severely compromised cardio-pulmonary hemodynamics identified by right heart catheterization. All patients received conventional and pulmonary arterial hypertension specific therapies, and then followed-up regularly. Up to now, 4 out of 5 patients died due to refractory right heart failure. The durations from symptoms onset to death and from PVOD establish to death were 5 - 65 (27 ± 26) months and 1 - 16 (9 ± 9) months, respectively. CONCLUSIONS: PVOD is a rare and malignant cardio-pulmonary disorder that often be misdiagnosed as idiopathic pulmonary arterial hypertension. Given the poor responses to modern pulmonary arterial hypertension specific therapies, lung transplantation remains the treatment of choice.