Frequency of Pulmonary Hypertension and Its Associated Risk Factors in End-Stage Renal Disease (ESRD) Patients on Maintenance Hemodialysis.

Introduction Pulmonary hypertension (PH) is a recognized complication in patients with end-stage renal disease (ESRD undergoing maintenance hemodialysis (MHD). PH is commonly found in patients with chronic kidney disease (CKD) and ESRD. PH is associated with increased morbidity and mortality in patients with CKD. Methodology This cross-sectional study aimed to assess the prevalence of PH and its associated risk factors in MHD patients. A total of 220 ESRD patients on MHD patients at The Kidney Center, Karachi, Pakistan, aged 18-70 were included. Patients with chronic obstructive lung disease, valvular heart disease, and obstructive sleep apnea were excluded, as these conditions can be responsible for PH. PH was evaluated by echocardiography (ECHO), which was performed by a cardiologist. Results The mean age was 50.65 ± 14.4 years, with 131 (59.5%) males and 89 (40.5%) females. The average duration on hemodialysis was 5.3 ± 2.8 years. Hypertension (89.5%) and ischemic heart disease (24.1%) were prominent comorbidities. Hypertensive nephropathy (42.7%) was the leading cause of ESRD. Left ventricular hypertrophy was mild in most cases (85.5%), whereas regional wall motion abnormality (RWMA) was common (67.3%). The average pulmonary artery pressure was 35.2 ± 15.3 mmHg. Out of 220 patients, 109 patients (49.8%) of them had mild PH, nine patients (4.1%) had severe PH, and 72 patients (32.7%) had moderate PH. Associations between PH and various factors were examined. RWMA, left ventricular hypertrophy, and left ventricular ejection fraction were significantly associated with PH (p < 0.001). Serum calcium and albumin levels were also associated with PH severity (p < 0.05). Other demographic and laboratory parameters did not show a significant association. Conclusion This study highlights the prevalence of PH in MHD patients and identifies associated risk factors. Understanding these associations can aid in better managing PH in ESRD patients.

Pulmonary artery vasodilators for treatment of pulmonary hypertension complicating fibrosing mediastinitis.

Fibrosing mediastinitis (FM) is a heterogeneous disease characterized by sclerosing fibrosis of mediastinal structures. Pulmonary hypertension (PH) may complicate the course of the disease and can contribute significantly to the morbidity of FM. Due to the rarity and complexity of the disease, evidence-based guidelines are not currently available, and the optimal treatment approach is unknown. Management approaches should be individualized, and current techniques are often unsatisfactory. Here, we present two cases of PH complicating FM that were managed using pulmonary artery vasodilator therapy with excellent hemodynamic response.

Digital Spatial Profiling Identifies Distinct Molecular Signatures of Vascular Lesions in Pulmonary Arterial Hypertension.

RATIONALE: Idiopathic Pulmonary Arterial Hypertension (IPAH) is characterized by extensive pulmonary vascular remodeling due to plexiform and obliterative lesions, media hypertrophy, inflammatory cell infiltration, and alterations of the adventitia. OBJECTIVE: Test the hypothesis that microscopic IPAH vascular lesions express unique molecular profiles, which collectively are different from control pulmonary arteries. METHODS: We used digital spatial transcriptomics to profile the genome-wide differential transcriptomic signature of key pathological lesions (plexiform, obliterative, intima+media hypertrophy, and adventitia) in IPAH lungs (n= 11) and compared these data to the intima+media and adventitia of control pulmonary artery (n=5). RESULTS: We detected 8273 transcripts in the IPAH lesions and control lung pulmonary arteries. Plexiform lesions and IPAH adventitia exhibited the greatest number of differentially expressed genes when compared with intima-media hypertrophy and obliterative lesions. Plexiform lesions in IPAH showed enrichment for (i) genes associated with TGFß-signaling and (ii) mutated genes affecting the extracellular matrix and endothelial-mesenchymal transformation. Plexiform lesions and IPAH adventitia showed upregulation of genes involved in immune and interferon signaling, coagulation, and complement pathways. Cellular deconvolution indicated variability in the number of vascular and inflammatory cells between IPAH lesions, which underlies the differential transcript profiling. CONCLUSIONS: IPAH lesions express unique molecular transcript profiles enriched for pathways involving pathogenetic pathways, including genetic disease drivers, innate and acquired immunity, hypoxia sensing, and angiogenesis signaling. These data provide a rich molecular-structural framework in IPAH vascular lesions that inform novel biomarkers and therapeutic targets in this highly morbid disease.

Patent Ductus Arteriosus and Lung MRI Phenotype in Moderate and Severe Bronchopulmonary Dysplasia-Pulmonary Hypertension.

RATIONALE: A hemodynamically significant patent ductus arteriosus (hsPDA) in premature infants has been associated with bronchopulmonary dysplasia (BPD) and pulmonary hypertension (PH). However, these associations remain incompletely understood. OBJECTIVES: The aim was to assess the association between hsPDA duration with clinical outcomes, PH, and phenotypic differences on lung MRI. METHODS: This retrospective cohort study identified all infants with BPD <32 weeks gestation who also underwent a research lung MRI <48 weeks postmenstrual age (PMA) from 2014-2022. Clinical echocardiograms were reviewed for hsPDA, and categorized into no hsPDA, hsPDA 1-60 days, and hsPDA >60 days. Outcome variables included BPD severity, PH at 36 weeks PMA, PH after 36 weeks PMA in the absence of shunt (PH-PVD), tracheostomy or death, and lung phenotype by MRI via modified Ochiai score, indexed total lung volume (TLVI), and whole lung hyperdensity (WLH). Logistic regression and ANOVA analysis were used. MEASUREMENTS AND MAIN RESULTS: In total, 133 infants born at 26.2 ± 1.9 weeks and 776 ± 276g were reviewed (47 no hsPDA, 44 hsPDA 1-60 days, 42 hsPDA >60 days). hsPDA duration >60 days was associated with BPD severity (p<0.01), PH at 36 weeks PMA (aOR 9.7 [95% CI: 3.3-28.4]), PH-PVD (aOR 6.5 [95% CI: 2.3-18.3]), and tracheostomy or death (aOR 3.0 [95% CI: 1.0-8.8]). Duration of hsPDA > 60 days was associated with higher Ochiai score (p=0.03) and TLVI (p=0.01), but not WLH (p=0.91). CONCLUSIONS: In infants with moderate or severe BPD, prolonged exposure to hsPDA is associated with BPD severity, PH-PVD, and increased parenchymal lung disease by MRI.

Management of Pulmonary Hypertension in the Context of Heart Failure with Preserved Ejection Fraction.

PURPOSE OF REVIEW: To review the current evidence and modalities for treating pulmonary hypertension (PH) in heart failure with preserved ejection fraction (HFpEF). RECENT FINDINGS: In recent years, several therapies have been developed that improve morbidity in HFpEF, though these studies have not specifically studied patients with PF-HFpEF. Multiple trials of therapies specifically targeting the pulmonary vasculature such as phosphodiesterase (PDE) inhibitors, prostacyclin analogs, endothelin receptor antagonists (ERA), and soluble guanylate cyclase stimulators have also been conducted. However, these therapies demonstrated lack of consistency in improving hemodynamics or functional outcomes in PH-HFpEF. There is limited evidence to support the use of pulmonary vasculature-targeting therapies in PH-HFpEF. The mainstay of therapy remains the treatment of the underlying HFpEF condition. There is emerging evidence that newer HF therapies such as sodium-glucose transporter 2 inhibitors and angiotensin-receptor-neprilysin inhibitors are associated with improved hemodynamics and quality of life of patients with PH-HFpEF. There is also a growing realization that more robust phenotyping PH and right ventricular (RV) function may hold promise for therapeutic strategies for patients with PH-HFpEF.

Noncoding RNA Lipotherapeutics: A Promising Breakthrough in Pulmonary Hypertension Treatment.

Pulmonary Hypertension (PH) is a complex cardiovascular disorder characterized by elevated blood pressure in the pulmonary arteries. Current therapeutic approaches for PH have limitations in addressing the underlying molecular mechanisms. This article explores the potential of noncoding RNAs (ncRNAs), including microRNAs (miRNAs), long noncoding RNAs (lncRNAs), and circular RNAs (circRNAs), delivered through Lipid-Based Nanoparticles (LNPs) as a novel treatment strategy. These ncRNAs play critical roles in regulating vascular function and are implicated in PH pathogenesis. LNPs provide a promising method for the efficient and targeted delivery of ncRNAs. Advances in LNP technology, including the incorporation of R8 peptide modification, have shown promise in enhancing the delivery and efficacy of ncRNAs in PH models. Challenges such as biocompatibility, toxicity, and precise targeting must be addressed as these therapies move toward clinical application. The potential of personalized medicine and the integration of artificial intelligence in LNP design are discussed as prospects. In conclusion, ncRNA lipotherapeutics delivered via LNPs offer a transformative approach to treating PH, potentially leading to more effective management and improved patient outcomes in the future. However, continued research and clinical trials are necessary to fully realize their therapeutic potential in the field of PH treatment.

Sleep-disordered breathing and nocturnal hypoxemia in chronic thromboembolic pulmonary disease.

BACKGROUND AND AIMS: Sleep-disordered breathing (SDB) and nocturnal hypoxemia were known to be present in patients with chronic thromboembolic pulmonary hypertension (CTEPH), but the difference between SDB and nocturnal hypoxemia in patients who have chronic thromboembolic pulmonary disease (CTEPD) with or without pulmonary hypertension (PH) at rest remains unknown. METHODS: Patients who had CTEPH (n = 80) or CTEPD without PH (n = 40) and who had undergone sleep studies from July 2020 to October 2022 at Shanghai Pulmonary Hospital were enrolled. Nocturnal mean SpO2 (Mean SpO2) <90% was defined as nocturnal hypoxemia, and the percentage of time with a saturation below 90% (T90%) exceeding 10% was used to evaluate the severity of nocturnal hypoxemia. Logistic and linear regression analyses were performed to investigate the difference and potential predictor of SDB or nocturnal hypoxemia between CTEPH and CTEPD without PH. RESULTS: SDB was similarly prevalent in CTEPH and CTEPD without PH (P = 0.104), both characterised by obstructive sleep apnoea (OSA). Twenty-two patients with CTEPH were diagnosed with nocturnal hypoxemia, whereas only three were diagnosed with CTEPD without PH (P = 0.021). T90% was positively associated with mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance in patients with CTEPH and CTEPD without PH (P < 0.001); T90% was also negatively related to cardiac output in these patients. Single-breath carbon monoxide diffusing capacity, sex and mPAP were all correlated with nocturnal hypoxemia in CTEPH and CTEPD without PH (all P < 0.05). CONCLUSION: Nocturnal hypoxemia was worse in CTEPD with PH; T90%, but not SDB, was independently correlated with the hemodynamics in CTEPD with or without PH.

Endothelial phosphodiesterase 4B inactivation ameliorates endothelial-to-mesenchymal transition and pulmonary hypertension.

Pulmonary hypertension (PH) is a fatal disorder characterized by pulmonary vascular remodeling and obstruction. The phosphodiesterase 4 (PDE4) family hydrolyzes cyclic AMP (cAMP) and is comprised of four subtypes (PDE4A-D). Previous studies have shown the beneficial effects of pan-PDE4 inhibitors in rodent PH; however, this class of drugs is associated with side effects owing to the broad inhibition of all four PDE4 isozymes. Here, we demonstrate that PDE4B is the predominant PDE isozyme in lungs and that it was upregulated in rodent and human PH lung tissues. We also confirmed that PDE4B is mainly expressed in the lung endothelial cells (ECs). Evaluation of PH in Pde4b wild type and knockout mice confirmed that Pde4b is important for the vascular remodeling associated with PH. In vivo EC lineage tracing demonstrated that Pde4b induces PH development by driving endothelial-to-mesenchymal transition (EndMT), and mechanistic studies showed that Pde4b regulates EndMT by antagonizing the cAMP-dependent PKA-CREB-BMPRII axis. Finally, treating PH rats with a PDE4B-specific inhibitor validated that PDE4B inhibition has a significant pharmacological effect in the alleviation of PH. Collectively, our findings indicate a critical role for PDE4B in EndMT and PH, prompting further studies of PDE4B-specific inhibitors as a therapeutic strategy for PH.

Dedifferentiated early postnatal lung myofibroblasts redifferentiate in adult disease.

Alveolarization ensures sufficient lung surface area for gas exchange, and during bulk alveolarization in mice (postnatal day [P] 4.5-14.5), alpha-smooth muscle actin (SMA)+ myofibroblasts accumulate, secrete elastin, and lay down alveolar septum. Herein, we delineate the dynamics of the lineage of early postnatal SMA+ myofibroblasts during and after bulk alveolarization and in response to lung injury. SMA+ lung myofibroblasts first appear at ∼ P2.5 and proliferate robustly. Lineage tracing shows that, at P14.5 and over the next few days, the vast majority of SMA+ myofibroblasts downregulate smooth muscle cell markers and undergo apoptosis. Of note, ∼8% of these dedifferentiated cells and another ∼1% of SMA+ myofibroblasts persist to adulthood. Single cell RNA sequencing analysis of the persistent SMA- cells and SMA+ myofibroblasts in the adult lung reveals distinct gene expression profiles. For instance, dedifferentiated SMA- cells exhibit higher levels of tissue remodeling genes. Most interestingly, these dedifferentiated early postnatal myofibroblasts re-express SMA upon exposure of the adult lung to hypoxia or the pro-fibrotic drug bleomycin. However, unlike during alveolarization, these cells that re-express SMA do not proliferate with hypoxia. In sum, dedifferentiated early postnatal myofibroblasts are a previously undescribed cell type in the adult lung and redifferentiate in response to injury.

Recovery from kidney failure associated with chronic thromboembolic pulmonary hypertension following pulmonary thomboendarterectomy.

The occurrence of renal failure in pulmonary hypertension (PH) is an ominous sign and implies excessive adverse hemodynamic factors. Pharmacologic agents to treat the PH are the mainstay of management, whereas diuretics assist in management of fluid overload. However, when such measures fail, dialysis and ultrafiltration (UF) become necessary to manage progressive azotemia and hypervolemia. Reversal of PH is essential to interrupt this vicious cycle of multisystem failure; otherwise, the need for renal replacement therapy would be permanent.

Tadalafil in Neonates and Infants With Pulmonary Hypertension Secondary to Bronchopulmonary Dysplasia.

OBJECTIVES: The primary outcome of this study was to describe the dosing regimen of tadalafil in neonates and infants diagnosed with pulmonary hypertension (PH) secondary to bronchopulmonary dysplasia (BPD). Secondary outcomes included tolerability, efficacy, adverse events, discontinuation of therapy, and changes in echocardiography. METHODS: This was a single-center, retrospective review of neonates and infants <1 year of age at initiation of tadalafil for PH secondary to BPD from January 2010 to November 2021. Data collected from the electronic medical record included patient demographics, tadalafil dosing, oxygen support, mechanical ventilation, concomitant PH medications, adverse events, and echocardiography information. RESULTS: Forty-two patients-4 neonates and 38 infants-met the inclusion criteria. The postnatal and post-menstrual age (median, IQR) at diagnosis were 121 (35.5-153.5) days and 42.6 (40.6-47.6) weeks, respectively. The initial and highest tadalafil doses (median, range) were 1 (0.25-2) and 1 (0.5-2) mg/kg/day. Only 1 patient experienced pulmonary overcirculation and required tadalafil to be discontinued. Over half (57.1%) of the patients in this study discontinued tadalafil therapy owing to improvements in pulmonary artery pressures. CONCLUSIONS: Tadalafil 1 mg/kg/day was the most commonly used dose regimen in neonates and infants. Tadalafil at this dose of 1 mg/kg/day appears well tolerated in neonates and infants with PH secondary to BPD and correlates with improvements in pulmonary artery pressures. Further studies evaluating tadalafil in comparison to other phosphodiesterase-5 inhibitors in neonates with PH secondary to BPD are warranted.

Proteomic analysis of the serum in dogs with pulmonary hypertension secondary to myxomatous mitral valve disease: the preliminary study.

Background: Pulmonary hypertension (PH) is a common complication in dogs with myxomatous mitral valve disease (MMVD), characterized by elevated blood pressure in pulmonary artery. Echocardiography is a reliable technique for PH diagnosis in veterinary medicine. However, it is limited to use as an early detection method. Liquid chromatography-tandem mass spectrometry (LC-MS/MS) has found extensive application in the discovery of serum protein biomarkers for various diseases. The objective of this study was to identify serum proteins in healthy control dogs and MMVD dogs both with and without PH using LC-MS/MS. Materials and methods: In this research, a total of 81 small-breed dogs participated, and they were categorized into three groups: the control (n = 28), MMVD (n = 24) and MMVD+PH (n = 29) groups. Serum samples were collected and analyzed by LC-MS/MS. Results: Differentially expressed proteins were identified, and the upregulated and downregulated proteins in MMVD+PH group including Myomesin 1 (MYOM1) and Histone deacetylase 7 (HDAC7), Pleckstrin homology domain containing M3 (PLEKHM3), Diacylglycerol lipase alpha (DAGLA) and Tubulin tyrosine ligase like 6 (TTLL6) were selected as proteins of interest in MMVD dogs with PH. Conclusion: Different types of proteins have been identified in healthy dogs and MMVD dogs with and without PH. Additional studies are needed to investigate the potential of these proteins as biomarkers for PH in dogs with MMVD.

NGF increases Connexin-43 expression and function in pulmonary arterial smooth muscle cells to induce pulmonary artery hyperreactivity.

AIMS: Pulmonary hypertension (PH) is characterised by an increase in pulmonary arterial pressure, ultimately leading to right ventricular failure and death. We have previously shown that nerve growth factor (NGF) plays a critical role in PH. Our objectives here were to determine whether NGF controls Connexin-43 (Cx43) expression and function in the pulmonary arterial smooth muscle, and whether this mechanism contributes to NGF-induced pulmonary artery hyperreactivity. METHODS AND RESULTS: NGF activates its TrkA receptor to increase Cx43 expression, phosphorylation, and localization at the plasma membrane in human pulmonary arterial smooth muscle cells, thus leading to enhanced activity of Cx43-dependent GAP junctions as shown by Lucifer Yellow dye assay transfer and fluorescence recovery after photobleaching -FRAP- experiments. Using both in vitro pharmacological and in vivo SiRNA approaches, we demonstrate that NGF-dependent increase in Cx43 expression and activity in the rat pulmonary circulation causes pulmonary artery hyperreactivity. We also show that, in a rat model of PH induced by chronic hypoxia, in vivo blockade of NGF or of its TrkA receptor significantly reduces Cx43 increased pulmonary arterial expression induced by chronic hypoxia and displays preventive effects on pulmonary arterial pressure increase and right heart hypertrophy. CONCLUSIONS: Modulation of Cx43 by NGF in pulmonary arterial smooth muscle cells contributes to NGF-induced alterations of pulmonary artery reactivity. Since NGF and its TrkA receptor play a role in vivo in Cx43 increased expression in PH induced by chronic hypoxia, these NGF/Cx43-dependent mechanisms may therefore play a significant role in human PH pathophysiology.

The Effect of Pulmonary Hypertension on Mortality and Intensive Care Unit Admission in Patients With SARS-CoV-2 Infection: A Systematic Review and Meta-Analysis.

AIM: Severe COVID-19 can cause acute respiratory distress syndrome, hypoxia, systemic complications, and increased mortality. Pulmonary hypertension (PH) is a major global health issue associated with worsening symptoms and increased mortality. This systematic review aimed to assess the influence of PH onset among COVID-19 patients on all-cause mortality and intensive care unit (ICU) admission. METHOD: An unrestricted search of five databases up to June 2022 was undertaken. Pulmonary hypertension was assessed using transthoracic echocardiogram, computed tomography, or right heart catheterisation. After duplicate screening, data extraction, and risk of bias assessment, random effects meta-analyses of odds ratios (OR) and their 95% confidence intervals (CI) were performed for all-cause mortality and ICU admission. RESULTS: From the 26 studies that were included (3,373 patients, 76% males, median age 62.6 years), PH in COVID-19 patients was significantly associated with higher odds for all-cause mortality (26 studies; OR 3.89; 95% CI 2.85-5.31; p<0.001) and higher odds for ICU admission (six studies; OR 2.50; 95% CI 1.69-3.70; p<0.001). Meta-regression/subgroup analyses by patient demographics, comorbidities, or therapeutic regimens, and sensitivity analyses did not find any differences. CONCLUSION: Evidence from observational studies indicates that PH in COVID-19 patients is associated with increased odds of mortality and ICU admission.

Predictive value of red cell distribution width in children with pulmonary arterial hypertension associated with CHD.

BACKGROUND: Pulmonary arterial hypertension, a common consequence of untreated CHD, is associated with a significant morbidity and mortality. Recent researches have demonstrated that patients with clinically severe cardiovascular illnesses, including pulmonary hypertension, have a greater mortality risk when their red cell distribution width is high. This work aimed to assess the predictive value of red cell distribution width in children with pulmonary arterial hypertension-CHD and to correlate red cell distribution width with various clinical and echocardiographic data. SUBJECTS AND METHODS: Sixty patients with CHD associated with pulmonary arterial hypertension were enrolled as the patient group. Another 60 patients with CHD and no pulmonary arterial hypertension, matched for age and sex, were enrolled as the control group. Electrocardiography and echocardiographic evaluation were performed for all included children. Red cell distribution width as part of the complete blood count was also performed using a Coulter® LH 700 series haematology analyzer. RESULTS: The red cell distribution width was significantly higher in the pulmonary arterial hypertension-CHD group than in the CHD-only group (P < 0.05). There was a significant positive correlation between the red cell distribution width and mean pulmonary artery pressure. Red cell distribution width was an independent predictor of mortality in children with pulmonary arterial hypertension-CHD. The best red cell distribution width cut-off for predicting mortality in children with pulmonary arterial hypertension-CHD was ≥ 17.6%. CONCLUSION: Red cell distribution width was significantly higher in children with pulmonary arterial hypertension-CHD than in those without pulmonary arterial hypertension. Moreover, red cell distribution width could be a cheap easy predictive marker for mortality in children with pulmonary arterial hypertension-CHD.

Predominance of M2 macrophages in organized thrombi in chronic thromboembolic pulmonary hypertension patients.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a debilitating disease characterized by thrombotic occlusion of pulmonary arteries and vasculopathy, leading to increased pulmonary vascular resistance and progressive right-sided heart failure. Thrombotic lesions in CTEPH contain CD68+ macrophages, and increasing evidence supports their role in disease pathogenesis. Macrophages are classically divided into pro-inflammatory M1 macrophages and anti-inflammatory M2 macrophages, which are involved in wound healing and tissue repair. Currently, the phenotype of macrophages and their localization within thrombotic lesions of CTEPH are largely unknown. In our study, we subclassified thrombotic lesions of CTEPH patients into developing fresh thrombi (FT) and organized thrombi (OT), based on the degree of fibrosis and remodeling. We used multiplex immunofluorescence histology to identify immune cell infiltrates in thrombotic lesions of CPTEH patients. Utilizing software-assisted cell detection and quantification, increased proportions of macrophages were observed in immune cell infiltrates of OT lesions, compared with FT. Strikingly, the proportions with a CD206+INOS- M2 phenotype were significantly higher in OT than in FT, which mainly contained unpolarized macrophages. Taken together, we observed a shift from unpolarized macrophages in FT toward an expanded population of M2 macrophages in OT, indicating a dynamic role of macrophages during CTEPH pathogenesis.

Adapting nitric oxide: A review of its foundation, uses in austere medical conditions, and emerging applications.

Nitric oxide was first identified as a novel and effective treatment for persistent pulmonary hypertension of the newborn (PPHN), and has since been found to be efficacious in treating acute respiratory distress syndrome (ARDS) and pulmonary hypertension. Physicians and researchers have also found it shows promise in resource-constrained settings, both within and outside of the hospital, such as in high altitude pulmonary edema (HAPE) and COVID-19. The treatment has been well tolerated in these settings, and is both efficacious and versatile when studied across a variety of clinical environments. Advancements in inhaled nitric oxide continue, and the gas is worthy of investigation as physicians contend with new respiratory and cardiovascular illnesses, as well as unforeseen logistical challenges.

Is pulmonary hypertension protective against cardiac tamponade? A systematic review.

The presence of pulmonary hypertension (PH) may affect whether cardiac tamponade physiology develops from a pericardial effusion. Specifically, the increased intracardiac pressure and right ventricular hypertrophy associated with PH would seemingly increase the intrapericardial pressure threshold at which the right-sided chambers collapse. In this systematic review, we examined the impact of PH on the incidence, in-hospital and long-term mortality, and echocardiographic findings of patients with cardiac tamponade. Using the PRISMA guideline, a systematic search was conducted in PubMed, Academic Search Premier, Web of Science, Google Scholar, and the Cochrane Database for studies investigating PH and cardiac tamponade. The Newcastle-Ottawa Scale was used to analyze the quality of returned studies. Primary outcomes included the incidence of cardiac tamponade, as well as in-hospital and long-term mortality rates. Secondary outcomes were the presence or absence of echocardiographic findings of cardiac tamponade in patients with PH. Forty-three studies (9 cohort studies and 34 case reports) with 1054 patients were included. The incidence of cardiac tamponade was significantly higher in patients with PH compared to those without PH, 2.0% (95% CI 1.2-3.2%) vs. 0.05% (95% CI 0.05-0.05%), p < 0.0001, OR 40.76 (95% CI 24.8-66.9). The incidence of tamponade in patients with a known pericardial effusion was similar in those with and without PH, 20.3% (95% CI 12.0-32.3%) and 20.9% (95% CI 18.0-24.1%), p = 0.9267, OR 0.97 (95% CI 0.50-1.87). In patients with tamponade, those with PH demonstrated a significantly higher in-hospital mortality than those without PH, 38.8% (95% CI 26.4-52.8%) vs. 14.4% (95% CI 14.2-14.6%), p < 0.0001, OR 3.77 (95% CI 2.12-6.70). Long-term mortality in patients with tamponade was significantly lower in those with PH than in those without PH, 45.5% (95% CI 33.0-58.5%) vs. 59.1% (95% CI 54.7-63.4%), p = 0.0258, OR 0.576 (95% CI 0.33-1.01). However, after stratifying by non-malignant etiologies, the long-term mortality benefit for those with PH disappeared. In the studies that described specific echocardiographic findings of cardiac tamponade, only 10.5% of patients with PH and tamponade showed right atrial and right ventricular collapse. When evaluating patients with pericardial effusions, physicians must recognize the effects of underlying PH on the incidence, in-hospital and long-term mortality rates, and potentially atypical echocardiographic presentation of cardiac tamponade.