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Objectives The study aimed to identify factors influencing the severity of primary immune thrombocytopenia (ITP) during pregnancy, develop a predictive model for treatment response, and report maternal and neonatal outcomes associated with severe ITP. Design A retrospective analysis was conducted on 155 pregnancies with severe ITP between January 2018 and April 2023 at a tertiary critical maternity referral center in Shanghai, China. Participants/Materials The study included 155 pregnancies diagnosed with severe ITP, divided into groups based on the lowest platelet count (<30×109/L vs. 30-50×109/L) and first-line treatment response (Non-response vs. Response). Setting The study was conducted at Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, a tertiary critical maternity rescue referral center. Methods Clinical characteristics and outcomes were compared between groups. A multivariable logistic regression model was used to identify factors associated with the severity of ITP. A prediction model for treatment response was established using LASSO-logistic regression and internally validated. Results ITP severity was found to be correlated with low maximum amplitude (MA) of thrombelastography (TEG) (OR 5.43, 95% CI 1.48 to 16.00, p=0.002), bleeding events (OR 4.91, 95% CI 1.62 to 14.86, p=0.005), and low reticulocytes (OR 2.40×10-7, 95% CI 1.06×10-13 to 0.55, p=0.04). Of the 118 patients who received first-line therapy, 52 (44%) responded. The dataset was randomly split into a training (N=99) and test (N=23) set with a ratio of 8:2. A predictive nomogram was created and internally validated showing good discrimination. The model yielded an area under receiver operating characteristic curve (ROC) of 0.78 (0.69 to 0.87) and 0.85 (0.67 to 1.00) in the training and validation cohort, respectively. Earlier delivery and high rate of NICU admission occurred with severe ITP and treatment failure. Limitations The study was limited by a relatively small sample size and the retrospective observational design, which imposed limitations on the assessment of treatment efficacy. Conclusions We identified clinical predictors of ITP severity and treatment resistance during pregnancy. A nomogram predicting first-line response was validated. These findings can facilitate clinical decision-making and counseling regarding this challenging pregnancy complication.
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BACKGROUND: Intestinal pseudo-obstruction (IPO) is a rare disease, and its clinical manifestations can resemble mechanical intestinal obstruction leading to unnecessary and potentially harmful surgery. Certain autoimmune diseases have been associated with IPO, however, cases secondary to Sjögren's syndrome (SjS) are especially rare. CASE PRESENTATION: We described the first case of SjS-associated acute IPO in pregnancy, which was successfully treated with combined immunosuppressive therapy and resulted in an uneventful caesarean delivery. CONCLUSIONS: Women with SjS is likely to experience more complications during pregnancy, and IPO rather than the classic symptoms could be the first sign of SjS flares. IPO should be suspected in patients with unrelenting symptoms of small bowel obstruction, and a multidisciplinary approach can provide optimal management of such high-risk pregnancies.
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Doenças Autoimunes , Pseudo-Obstrução Intestinal , Síndrome de Sjogren , Gravidez , Humanos , Feminino , Síndrome de Sjogren/complicações , Pseudo-Obstrução Intestinal/etiologia , Pseudo-Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/tratamento farmacológicoRESUMO
High-risk PE can be complicated by the presence of a patent foramen ovale (PFO), which can lead to paradoxical systemic embolization, including cerebral embolism ultimately leading to acute ischemic stroke (AIS). Acute management is challenging given the competing benefits and risks of systemic thrombolysis. Herein, we aim to provide a review of clinical presentations, diagnostic findings, and treatment and outcome from the available literature, with the hopes of providing insight into treatment options. We followed the guidelines outlined by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). A systematic literature search using PubMed/MEDLINE database, Cochrane Library, and Google Scholar for all reported cases/case series of concomitant high-risk PE and paradoxical ischemic stroke was conducted from inception to July 2019. Twenty-nine cases from 27 articles (26 single case reports, 1 case series of 3 patients) were included. There were 10 men and 19 women, ranging in age from 29 to 81 years (mean 56.1 ± 13.5 years). PFO was diagnosed in 89.7% of patients, mostly by transesophageal echocardiography. Treatment modalities included systemic thrombolysis (40%), anticoagulation alone (36%), surgical thrombectomy (16%), and percutaneous thrombectomy (8%). Overall mortality rate was 31%. Patients receiving thrombolysis and surgical thrombectomy had the most favorable outcome. Survival to discharge was 90% (9 out of 10), 100% (5 out of 5), and 50% (4 out of 8) in the systemic thrombolysis, surgical thrombectomy, and anticoagulation alone groups respectively. In the setting of combined high-risk PE and ischemic stroke, PFO can be detected in 90% of published cases. Thrombolysis and surgical thrombectomy seem to be effective management, but further studies are needed for validation.
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Embolia Paradoxal/diagnóstico por imagem , Forame Oval Patente/diagnóstico por imagem , AVC Isquêmico/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Diagnóstico Diferencial , Embolia Paradoxal/terapia , Forame Oval Patente/terapia , Humanos , AVC Isquêmico/terapia , Embolia Pulmonar/terapiaRESUMO
OBJECTIVE: Major progress in reperfusion strategies has substantially improved the short-term outcomes of patients with pulmonary embolism (PE), however, up to 50% of patients report persistent dyspnea after acute PE. METHODS: A retrospective study of the PE response team registry and included patients with repeat imaging at 3 to 12 months. The primary outcome was to determine the incidence of residual pulmonary vascular obstruction following acute PE. Secondary outcomes included the development of PE recurrence, right ventricular (RV) dysfunction, chronic thromboembolic pulmonary hypertension, readmission, and mortality at 12 months. RESULTS: A total of 382 patients were included, and 107 patients received reperfusion therapies followed by anticoagulation. Patients who received reperfusion therapies including systemic thrombolysis, catheter-directed thrombolysis, and mechanical thrombectomy presented with a higher vascular obstructive index (47% vs 28%; P < .001) and signs of right heart strain on echocardiogram (81% vs 43%; P < .001) at the time of diagnosis. A higher absolute reduction in vascular obstructive index (45% vs 26%; 95% confidence interval, 14.0-25.6; P < .001), greater improvement in RV function (82% vs 65%; P = .021), and lower 12-month mortality rate (2% vs 7%; P = .038) and readmission rate (33% vs 46%; P = .031) were observed in the reperfusion group. No statistically significant differences were found between groups in the development of chronic thromboembolic pulmonary hypertension (8% vs 5%; P = .488) and PE recurrence (8% vs 6%; P = .646). CONCLUSIONS: We observed a favorable survival and greater improvement in clot resolution and RV function in patients treated with reperfusion therapies.
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Hipertensão Pulmonar , Embolia Pulmonar , Trombose , Humanos , Terapia Trombolítica/efeitos adversos , Estudos Retrospectivos , Hipertensão Pulmonar/complicações , Resultado do Tratamento , Embolia Pulmonar/terapia , Trombose/etiologia , Reperfusão/métodosRESUMO
Background: Residual Pulmonary Vascular Obstruction (RPVO) is an area of increasing focus in patients with acute pulmonary embolism (PE) due to its association with long-term morbidity and mortality. The predictive factors and the effect catheter-directed therapies (CDT) have on RPVO are still under investigation. Methods: This is a single-center retrospective review between April 2017 and July 2021. Patients with intermediate risk of PE were included. Patient variables associated with RPVO were analyzed and the degree of clot burden was quantified using the Qanadli score. Results: A total of 551 patients with acute PE were identified, 288 were intermediate risk and 53 had RPVO based on CT or V/Q scan three months post-PE. Baseline clot burden was higher in patients who received CDT compared to those who received anticoagulation alone (Qanadli score 45.88% vs. 31.94% p < 0.05). In univariate analysis, treatment with CDT showed a HR of 0.32 (95% CI 0.21-0.50, p < 0.001) when compared with anticoagulation alone. Patient variables including intermediate-high risk, sPESI ≥ 1, elevated biomarkers, RV dysfunction on imaging, malignancy, history of or concurrent DVT were also significantly associated with development of RPVO in univariate analysis. In multivariable analysis, only baseline Qanadli score (HR 13.88, 95% CI 1.42-135.39, p = 0.02) and concurrent DVT (HR 2.53, 95% CI 1.01-6.40, p = 0.04) were significantly associated with the development of RPVO. Conclusions: Catheter-directed therapy may be associated with a reduced risk of RPVO at 3 months; however, quantitative clot burden scores, such as the Qanadli score, may be stronger predictors of the risk of developing RPVO at 3 months. Further prospective studies are required.
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Anti-synthetase syndrome (ASS) is a rare inflammatory myopathy with a wide variety of clinical presentations. ASS-related interstitial lung disease (ASS-ILD) presents with rapid onset and progression, which could often be confused with other more common acute processes such as pneumonia, especially when ILD can be the sole manifestation. A woman in her 50s presented with recurrent dyspnoea for 2 months requiring multiple hospital admissions, and each time, she was diagnosed with multifocal pneumonia and treated with antibiotics. On admission, the evaluation revealed a markedly elevated creatine kinase level at 3258 U/L and a CT scan of the chest revealed worsening scattered ground-glass opacities. Given the concern for ILD as the cause of antibiotic failure, she underwent bronchoscopy with bronchoalveolar lavage which revealed non-specific interstitial pneumonia. A subsequent myositis panel revealed a positive anti-Jo-1 antibody, and she was diagnosed with ASS-ILD. She completed a course of intravenous immunoglobulin and methylprednisolone and experienced significant clinical improvement with the resolution of hypoxaemia and improved polyarthralgia.ASS could often be misdiagnosed as other more common acute lung processes, as a clinically subtle course can escape detection given its rarity, as well as its non-specific and highly variable presentations. This case highlights the importance of early suspicion and consideration of performing specific autoantibody testing when evaluating patients with a suspicion of undifferentiated autoimmune condition.
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Doenças Pulmonares Intersticiais , Miosite , Pneumonia , Feminino , Humanos , Autoanticorpos , Ligases , Pulmão , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Miosite/diagnóstico , Miosite/tratamento farmacológico , Miosite/complicações , Pneumonia/complicaçõesRESUMO
Pulmonary embolism (PE) is the third leading cause of cardiovascular death in the United States. Black Americans have higher incidence, greater clot severity, and worse outcomes than White Americans. This disparity is not fully understood, especially in the context of the advent of PE response teams (PERT), which aim to standardize PE-related care. This retrospective single-center cohort study compared 294 Black and 131 White patients from our institution's PERT database. Primary objectives included severity and in-hospital management. Secondary outcomes included length of stay, 30-day readmission, 30-day mortality, and outpatient follow-up. Clot (p = 0.42), acute treatment (p = 0.28), 30-day mortality (p = 0.77), 30-day readmission (p = 0.50), and outpatient follow-up (p = 0.98) were similar between races. Black patients had a lower mean household income ($35,383, SD 20,596) than White patients ($63,396, SD 32,987) (p < 0.0001). More Black patients (78.8%) had exclusively government insurance (Medicare/Medicaid) compared to White patients (61.8%) (p = 0.006). Interestingly, government insurance patients had less follow-up (58.3%) than private insurance patients (79.7%) (p = 0.001). Notably, patients with follow-up had fewer 30-day readmissions. Specifically, 12.2% of patients with follow-up were readmitted compared to 22.2% of patients without follow-up (p = 0.008). There were no significant differences in PE severity, in-hospital treatment, mortality, or readmissions between Black and White patients. However, patients with government insurance had less follow-up and more readmissions, indicating a socioeconomic disparity. Access barriers such as health literacy, treatment cost, and transportation may contribute to this inequity. Improving access to follow-up care may reduce the disparity in PE outcomes.
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Acute encephalopathy is a change in the level of consciousness where the underlying etiology can be difficult to diagnose, and thus, difficult to treat, especially in the context of multi-organ diseases. We report a case of acute encephalopathy in a patient with end-stage renal disease (ESRD) on hemodialysis, chronic hypotension, and a recent diagnosis of colon cancer who presented shortly after initiation of FOLFOX, a chemotherapy regimen for treatment of colorectal cancer comprised of folinic acid (leucovorin), fluorouracil (5-FU), and oxaliplatin (eloxatin). We present a systematic approach to elucidate ambiguous causes of toxic-metabolic encephalopathy.
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Background: To evaluate the occurrence of malnutrition in pulmonary embolism (PE)-related hospitalisations and assess the impact of malnutrition on the outcomes of patients with PE. Methods: A retrospective observational study using data extracted from the Nationwide Inpatient Sample from 2016 to 2018. Hospitalisations with a principal diagnosis of PE were obtained using International Classification of Diseases, Tenth Revision codes and divided into groups based on a secondary diagnosis of malnutrition. Results: Of 563 135 PE hospitalisations, 30 495 (5.4%) had malnutrition. PE patients with malnutrition were older (mean age±SD, 69.1±14.5 vs 62.3±16.6, p<0.001) and with higher Charlson Comorbidity Index score (3 to 5, 24.8% vs 12.9%, p<0.001). Concurrent malnutrition was associated with higher adjusted OR (aOR) of in-hospital mortality (aOR 2.43, 95% CI 2.18 to 2.70, p<0.001), acute kidney injury (aOR 1.56, 95% CI 1.45 to 1.67, p<0.001), sepsis (aOR 4.37, 95% CI 3.79 to 5.03, p<0.001), shock (aOR 2.52, 95% CI 2.25 to 2.81, p<0.001), acidosis (aOR 2.55, 95% CI 2.34 to 2.77, p<0.001) and mechanical ventilation (aOR 2.95, 95% CI 2.61 to 3.33, p<0.001). Patients with PE and malnutrition had an increased mean length of stay (adjusted difference 3.39 days, 95% CI 3.14 to 3.65, p<0.001), hospital charges (adjusted difference US$34 802.11, 95% CI US$31 005.01 to US$38 599.22, p<0.001) and costs (adjusted difference US$8 332.01, 95% CI US$7489.09 to US$9174.94, p<0.001). Conclusion: Concurrent PE and malnutrition were associated with worse outcomes. The study highlights the importance of identifying malnutrition in patients with PE to improve outcomes and reduce healthcare utilisation.
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Pregnancy in patients with Eisenmenger syndrome (ES) is associated with high maternal mortality rates of 30%â50%, or even up to 65% in the case of a cesarean section (Yuan, 2016). Here, we report a case of term pregnancy complicated with ES and severe pulmonary artery hypertension (PAH), which was managed by a multidisciplinary team (MDT) and resulted in an uncomplicated delivery via elective cesarean section. The goal of this study is to emphasize the importance of multidisciplinary approach in the management of pregnancy with ES, which can profoundly improve maternal and infant outcomes.
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Complexo de Eisenmenger , Hipertensão Pulmonar , Complicações Cardiovasculares na Gravidez , Feminino , Humanos , Gravidez , Cesárea , Complexo de Eisenmenger/complicações , Complexo de Eisenmenger/terapia , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/terapia , Mortalidade Materna , Complicações Cardiovasculares na Gravidez/terapia , Resultado da GravidezRESUMO
Objectives: Cerebral venous thrombosis (CVT) in early pregnancy is extremely rare and evidence limited to only a few published reports. This study aims to present our experience and summarize the available literature to further elucidate the clinical manifestations, treatment, and outcomes of CVT in early pregnancy. Methods: A retrospective case series of seven patients diagnosed with CVT in early pregnancy (<12 weeks of gestations) in a tertiary referral center (2018-2021), along with a review of published literature. Results: All the patients presented with nausea, vomiting, headaches, and neurological symptoms including aphasia (n = 5, 71.4%), limb weakness (n = 4, 57.1%), seizures (n = 2, 28.6%), altered mental status (n = 3, 42.9%), and blurred vision (n = 2, 28.6%). All the patients were diagnosed with CVT by neuroimaging, which revealed various extents of sinus involvement, with the transverse sinus being the most common site (n = 7, 100%) followed by the sigmoid sinus (n = 5, 71.4%). All the patients received subcutaneous low-molecular-weight heparin once the diagnosis was confirmed. Two patients with rapid deterioration underwent venous thrombectomy, and one patient subsequently underwent decompressive craniotomy but died despite the above interventions. All the other patients proceeded with induced abortion after stabilization and were discharged on oral anticoagulation for 1 year. On the 12-month follow-up, the MRI/magnetic resonance venography (MRV) revealed recanalization of sinuses and resolution of thrombi. Conclusions: Cerebral venous thrombosis (CVT) in early pregnancy represents a diagnostic challenge given its rarity and nonspecific overlapping clinical features with nausea and vomiting of pregnancy/hyperemesis gravidarum (NVP/HG), which could lead to delay in diagnosis and result in rapid deterioration. Persistent or aggravating headaches combined with other focalizing neurological symptoms in NVP/HG patients could be an initial sign of CVT. Urgent MRI/MRV remains the cornerstone for diagnosis, and immediate anticoagulation is the key for disease prognosis. Glasgow coma scale (GCS) evaluation on admission is probably correlated with the prognosis. Early pregnancy combined with CVT is not a contraindication of continued pregnancy.
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OBJECTIVES: Accurate prenatal diagnosis of placenta accrete spectrum disorder (PAS) remains a challenge, and the reported diagnostic value of ultrasonography (US) and magnetic resonance imaging (MRI) varies widely. This study aims to systematically evaluate the diagnostic accuracy of US as compared with MRI in the detection of PAS within the identical patient population. METHODS: Medline, EMBASE, Google scholar and Cochrane library were searched. Pooled sensitivity, specificity, diagnostic odds ratio (DOR) and the area under the summary receiver operating characteristic (SROC) curve were calculated. Subgroup analysis was also performed to elucidate the heterogeneity of results. RESULTS: A total of 18 articles comprising 861 pregnancies were included in the study. The overall diagnostic accuracy of US for identification of PAS was as follows: sensitivity [0.90 (0.86-0.93)], specificity [0.83 (0.79-0.86)], DOR [39.5 (19.6-79.7)]. The overall diagnostic accuracy of MRI for identification of PAS was as follows: sensitivity [0.89 (0.85-0.92)], specificity [0.87 (0.83-0.89)], DOR [37.4 (17.0-82.3)]. The pooled sensitivity (p = 0.808) and specificity (p = 0.413) between US and MRI are not significantly different. SROC analysis revealed that there was no statistical difference (p = 0.552) in US and MRI for the overall predictive accuracy of PAS. Furthermore, in the subgroup analysis of between retrospective and prospective studies, between earlier and most recent studies, there was no statistical difference (p > 0.05) in diagnostic accuracy of US and MRI for the detection of PAS. CONCLUSIONS: Both ultrasonography (US) and magnetic resonance imaging (MRI) showed comparable accuracy in the prenatal diagnosis of placenta accrete spectrum disorder (PAS). Routine employment of MRI with relatively high expense in the prenatal identification of PAS should not be recommended.
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OBJECTIVE: Pulmonary infarction is a common clinical and radiographic finding in acute pulmonary embolism (PE), yet the clinical relevance and prognostic significance of pulmonary infarction remain unclear. The study aims to investigate the clinical features, radiographic characteristics, impact of reperfusion therapy and outcomes of patients with pulmonary infarction. DESIGN, SETTING AND PARTICIPANTS: A retrospective cohort study of 496 adult patients (≥18 years of age) diagnosed with PE who were evaluated by the PE response team at a tertiary academic referral centre in the USA. We collected baseline characteristics, laboratory, radiographic and outcome data. Statistical analysis was performed by Student's t-test, Mann-Whitney U test, Fischer's exact or χ2 test where appropriate. Multivariate logistic regression was used to evaluate potential risk factors for pulmonary infarction. RESULTS: We identified 143 (29%) cases of pulmonary infarction in 496 patients with PE. Patients with infarction were significantly younger (52±15.9 vs 61±16.6 years, p<0.001) and with fewer comorbidities. Most infarctions occurred in the lower lobes (60%) and involved a single lobe (64%). The presence of right ventricular (RV) strain on CT imaging was significantly more common in patients with infarction (21% vs 14%, p=0.031). There was no significant difference in advanced reperfusion therapy, in-hospital mortality, length of stay and readmissions between groups. In multivariate analysis, age and evidence of RV strain on CT and haemoptysis increased the risk of infarction. CONCLUSIONS: Radiographic evidence of pulmonary infarction was demonstrated in nearly one-third of patients with acute PE. There was no difference in the rate of reperfusion therapies and the presence of infarction did not correlate with poorer outcomes.
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Embolia Pulmonar , Infarto Pulmonar , Disfunção Ventricular Direita , Adulto , Humanos , Estudos Retrospectivos , Embolia Pulmonar/complicações , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/epidemiologia , Pulmão , Fatores de Risco , Doença AgudaRESUMO
Hemostatic derangement is a hallmark in severe COVID-19. Markedly elevation of D-dimer and fibrinogen degradation product levels were observed in patients with severe COVID-19 higher and 71.4% of nonsurvivors met the International Society of Thrombosis and Haemostasis criteria of disseminated intravascular coagulation (DIC). Although the clinical and epidemiological features of COVID-19 have been well-described, the underlying mechanism influencing disease severity remains to be elucidated. Herein, the aim of this review article is to evaluate hemostasis in the pathogenesis of COVID-19 and its role in the management of this unprecedented pandemic.
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INTRODUCTION: Acute pulmonary embolism (PE) remains a common cause for morbidity and mortality in patients over 65 years. Given the increased risk of bleeding in the elderly population with the use of systemic thrombolysis, catheter-directed therapy (CDT) is being increasingly used for the treatment of submassive PE. Nevertheless, the safety of CDT in the elderly population is not well studied. We, therefore, aimed to evaluate the safety of CDT in our elderly patients. METHODS: We conducted a retrospective observational study of consecutive patients aged >65 years with a diagnosis of PE from our Pulmonary Embolism Response Team database. We compared the treatment outcomes of CDT versus anticoagulation (AC) in elderly. Propensity score matching was used to construct two matched cohorts for final outcomes analysis. RESULTS: Of 346 patients with acute PE, 138 were >65 years, and of these, 18 were treated with CDT. Unmatched comparison between CDT and AC cohorts demonstrated similar in-hospital mortality (11.1% vs 5.6%, p=0.37) and length of stay (LOS) (3.81 vs 5.02 days, p=0.5395), respectively. The results from the propensity-matched cohort mirrored results of the unmatched cohort with no significant difference between CDT and AC in-hospital mortality (11.8% vs 5.9%, p=0.545) or median LOS (3.76 vs 4.21 days, p=0.77), respectively. CONCLUSION: In this observational study using propensity score-matched analysis, we found that patients >65 years who were treated with CDT for management of acute PE had similar mortality and LOS compared with those treated with AC. Further studies are required to confirm these findings.
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Embolia Pulmonar , Terapia Trombolítica , Idoso , Catéteres , Fibrinolíticos/efeitos adversos , Humanos , Embolia Pulmonar/tratamento farmacológico , Fatores de TempoRESUMO
Acute pulmonary embolism (PE) is associated with significant morbidity and mortality. The management paradigm for acute PE has evolved in recent years with wider availability of advanced treatment modalities ranging from catheter-directed reperfusion therapies to mechanical circulatory support. This evolution has coincided with the development and implementation of institutional pulmonary embolism response teams (PERT) nationwide and internationally. Because most institutions are not equipped or staffed for advanced PE care, patients often require transfer to centers with more comprehensive resources, including PERT expertise. One of the unmet needs in current PE care is an organized approach to the process of interhospital transfer (IHT) of critically ill PE patients. In this review, we discuss medical optimization and support of patients before and during transfer, transfer checklists, defined roles of emergency medical services, and the roles and responsibilities of referring and receiving centers involved in the IHT of acute PE patients.