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1.
Curr Opin Pulm Med ; 29(5): 340-347, 2023 09 01.
Article in English | MEDLINE | ID: mdl-37461845

ABSTRACT

PURPOSE OF REVIEW: Chronic thromboembolic disease (CTED) is distinct from chronic thromboembolic pulmonary hypertension (CTEPH) and is defined by dyspnea on exertion after acute pulmonary embolism with the presence of residual perfusion defects and absence of resting pulmonary hypertension. Here, we review clinical features and diagnostic criteria for CTED and summarize treatment options. RECENT FINDINGS: The optimal management for CTED is unclear as the long-term outcomes of conservative vs. invasive treatment for this disease have not been reported. There are a few studies evaluating outcomes of pulmonary thromboendarterectomy and balloon pulmonary angioplasty (BPA) in CTED, concluding that these procedures are safe and effective in select patients. However, these trials are small nonrandomized observational studies, reporting outcomes only up to 1 year after the intervention. Conservative management of CTED with observation, pulmonary hypertension-targeted therapy, or cardiopulmonary rehabilitation has not been studied. It is unknown whether these treatments are as effective or superior to pulmonary thromboendarterectomy or BPA in CTED. SUMMARY: The management of CTED is individualized and based on symptoms and exercise limitations. Early referral of patients with CTED to a specialized CTEPH center is recommended to determine if watchful waiting, BPA, or pulmonary thromboendarterectomy is most beneficial.


Subject(s)
Angioplasty, Balloon , Hypertension, Pulmonary , Pulmonary Embolism , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Pulmonary Embolism/complications , Pulmonary Embolism/diagnosis , Pulmonary Embolism/therapy , Lung , Dyspnea , Angioplasty, Balloon/methods , Chronic Disease , Pulmonary Artery
2.
Radiographics ; 43(2): e220078, 2023 02.
Article in English | MEDLINE | ID: mdl-36525366

ABSTRACT

Management of chronic thromboembolic pulmonary hypertension (CTEPH) should be determined by a multidisciplinary team, ideally at a specialized CTEPH referral center. Radiologists contribute to this multidisciplinary process by helping to confirm the diagnosis of CTEPH and delineating the extent of disease, both of which help determine a treatment decision. Preoperative assessment of CTEPH usually employs multiple imaging modalities, including ventilation-perfusion (V/Q) scanning, echocardiography, CT pulmonary angiography (CTPA), and right heart catheterization with pulmonary angiography. Accurate diagnosis or exclusion of CTEPH at imaging is imperative, as this remains the only form of pulmonary hypertension that is curative with surgery. Unfortunately, CTEPH is often misdiagnosed at CTPA, which can be due to technical factors, patient-related factors, radiologist-related factors, as well as a host of disease mimics including acute pulmonary embolism, in situ thrombus, vasculitis, pulmonary artery sarcoma, and fibrosing mediastinitis. Although V/Q scanning is thought to be substantially more sensitive for CTEPH compared with CTPA, this is likely due to lack of recognition of CTEPH findings rather than a modality limitation. Preoperative evaluation for pulmonary thromboendarterectomy (PTE) includes assessment of technical operability and surgical risk stratification. While the definitive therapy for CTEPH is PTE, other minimally invasive or noninvasive therapies also lead to clinical improvements including greater survival. Complications of PTE that can be identified at postoperative imaging include infection, reperfusion edema or injury, pulmonary hemorrhage, pericardial effusion or hemopericardium, and rethrombosis. ©RSNA, 2022 Online supplemental material is available for this article.


Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Humans , Hypertension, Pulmonary/diagnostic imaging , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Pulmonary Embolism/complications , Pulmonary Embolism/diagnostic imaging , Pulmonary Embolism/surgery , Endarterectomy/adverse effects , Endarterectomy/methods , Angiography/methods , Radiologists , Chronic Disease
3.
Crit Care Med ; 47(12): 1751-1758, 2019 12.
Article in English | MEDLINE | ID: mdl-31567351

ABSTRACT

OBJECTIVES: To assess the efficacy of ramelteon in preventing delirium, an acute neuropsychiatric condition associated with increased morbidity and mortality, in the perioperative, ICU setting. DESIGN: Parallel-arm, randomized, double-blinded, placebo-controlled trial. SETTING: Academic medical center in La Jolla, California. PATIENTS: Patients greater than or equal to 18 years undergoing elective pulmonary thromboendarterectomy. INTERVENTIONS: Ramelteon 8 mg or matching placebo starting the night prior to surgery and for a maximum of six nights while in the ICU. MEASUREMENTS AND MAIN RESULTS: Incident delirium was measured twice daily using the Confusion Assessment Method-ICU. The safety outcome was coma-free days assessed by the Richmond Agitation-Sedation Scale. One-hundred twenty participants were enrolled and analysis completed in 117. Delirium occurred in 22 of 58 patients allocated to placebo versus 19 of 59 allocated to ramelteon (relative risk, 0.8; 95% CI, 0.5-1.4; p = 0.516). Delirium duration, as assessed by the number of delirium-free days was also similar in both groups (placebo median 2 d [interquartile range, 2-3 d] vs ramelteon 3 d [2-5 d]; p = 0.181). Coma-free days was also similar between groups (placebo median 2 d [interquartile range, 1-3 d] vs ramelteon 3 d [2-4 d]; p = 0.210). We found no difference in ICU length of stay (median 4 d [interquartile range, 3-5 d] vs 4 d [3-6 d]; p = 0.349), or in-hospital mortality (four vs three deaths; relative risk ratio, 0.7; 95% CI, 0.2-3.2; p = 0.717), all placebo versus ramelteon, respectively. CONCLUSIONS: Ramelteon 8 mg did not prevent postoperative delirium in patients admitted for elective cardiac surgery.


Subject(s)
Delirium/prevention & control , Endarterectomy , Indenes/therapeutic use , Postoperative Complications/prevention & control , Adult , Aged , Double-Blind Method , Elective Surgical Procedures , Female , Humans , Male , Middle Aged
4.
J Card Surg ; 34(5): 312-317, 2019 May.
Article in English | MEDLINE | ID: mdl-30908728

ABSTRACT

BACKGROUND AND OBJECTIVES: Atrial arrhythmias (AAs) are common after cardiac surgeries including pulmonary thromboendarterectomy (PTE). This study was done to identify patients at highest risk of developing post-PTE AA and their length of stay (LOS). METHODS: We reviewed 521 consecutive patients referred to University of California San Diego (UCSD) for PTE and examined their demographics as well as their baseline pulmonary hemodynamics to determine risk factors for AA. RESULTS: Overall, 24.2% of patients developed an AA after PTE. Patients who developed AA had a significantly longer Intensive Care Unit (ICU) LOS (median: 5 vs 3 days, P < 0.001) and postoperative LOS (median: 14 vs 9 days; P < 0.001). Patients who developed AA were more frequently male (63.2% male, P = 0.003), older (mean age 60.8 vs 50.7 years, P < 0.001), had a prior history of atrial fibrillation (80.2% of those who developed AA) and were more likely to have undergone concomitant Coronary Artery Bypass Graft (12.7% vs 6.6%, P = 0.028). Compared to those who did not develop AA, the cardiopulmonary bypass time was longer among those who developed AA (261.6 vs 253.8 minutes, P = 0.027). In a multivariate logistic regression model, the preoperative variables that predicted AA were age (odds ratio [OR], 1.058 per year, 95% confidence interval [CI]: 1.038-1.078), male sex (OR, 1.68, 95% CI: 1.06-2.64), prior AA (OR, 2.52, 95% CI: 1.23-5.15) and baseline right atrial pressure (OR, 1.039 per mm Hg, 95% CI: 1.000-1.079). While mortality rates were similar, patients who developed AA had more bleeding complications and more postoperative delirium. CONCLUSIONS: AA is common after PTE surgery. The strongest risk factors for AA after PTE included the previous history of AA, age and male sex. Development of AA was associated with longer lengths of stay and more postoperative complications.


Subject(s)
Arrhythmias, Cardiac/epidemiology , Endarterectomy , Postoperative Complications/epidemiology , Pulmonary Embolism/surgery , Adult , Age Factors , Aged , Arrhythmias, Cardiac/etiology , Atrial Fibrillation/complications , Cardiopulmonary Bypass , Female , Humans , Length of Stay , Male , Middle Aged , Operative Time , Postoperative Hemorrhage/epidemiology , Postoperative Hemorrhage/etiology , Risk , Risk Factors , Sex Factors
5.
Crit Care Nurs Q ; 42(1): 96-105, 2019.
Article in English | MEDLINE | ID: mdl-30507670

ABSTRACT

Prevention of burnout is a national imperative, and blame-free investigations of clinical events are advocated. Reflective inquiry techniques are helpful in processing adverse events while minimizing blame. The purpose of this project was to develop an interprofessional peer review program (Case Study Investigation) to process emotions, improve teamwork, and optimize patient outcomes. This evidence-based practice project was conducted in a 12-bed intensive care unit using reflective inquiry techniques to perform peer case review across disciplines. Significant improvements were seen in percent strongly agree to the 2 satisfaction questions asked: "I feel free to speak up regarding issues that may affect patient care" (increased from 37% to 73%, χ = 6.19, P < .05), and "There is open communication between physicians and nurses" (rose from 33% to 73%, χ = 7.53, P < .05). In total, 95% perceived improvement in interprofessional teamwork. Burnout decreased significantly at 6 months (n = 22, M = 18.40, SD = 3.36) from baseline scores (n = 27, M = 21.96, SD = 4.47), F2,72 = 4.48, P < .02. Central line-associated infections decreased from 3.6 per 1000 to 0 per 1000 catheter-days. Catheter-related urinary tract infections decreased from 2 per 1000 to 0 per 1000 patient-days. Both were sustained below benchmark. Reflective inquiry decreases nurse burnout while improving perceived interprofessional teamwork and employee satisfaction, and measurements of patient safety.


Subject(s)
Burnout, Professional/prevention & control , Interprofessional Relations , Job Satisfaction , Organizational Case Studies , Patient Safety , Communication , Humans , Intensive Care Units , Nursing Staff, Hospital/psychology , Quality Improvement
6.
Article in English | MEDLINE | ID: mdl-28807909

ABSTRACT

Widespread antibiotic use in clinical medicine and the livestock industry has contributed to the global spread of multidrug-resistant (MDR) bacterial pathogens, including Acinetobacter baumannii We report on a method used to produce a personalized bacteriophage-based therapeutic treatment for a 68-year-old diabetic patient with necrotizing pancreatitis complicated by an MDR A. baumannii infection. Despite multiple antibiotic courses and efforts at percutaneous drainage of a pancreatic pseudocyst, the patient deteriorated over a 4-month period. In the absence of effective antibiotics, two laboratories identified nine different bacteriophages with lytic activity for an A. baumannii isolate from the patient. Administration of these bacteriophages intravenously and percutaneously into the abscess cavities was associated with reversal of the patient's downward clinical trajectory, clearance of the A. baumannii infection, and a return to health. The outcome of this case suggests that the methods described here for the production of bacteriophage therapeutics could be applied to similar cases and that more concerted efforts to investigate the use of therapeutic bacteriophages for MDR bacterial infections are warranted.


Subject(s)
Acinetobacter Infections/therapy , Acinetobacter baumannii/drug effects , Anti-Bacterial Agents/therapeutic use , Bacteriophages/classification , Pancreatic Pseudocyst/therapy , Pancreatitis, Acute Necrotizing/therapy , Phage Therapy/methods , Acinetobacter Infections/microbiology , Acinetobacter baumannii/isolation & purification , Acinetobacter baumannii/virology , Aged , Drug Resistance, Multiple, Bacterial , Gallstones/pathology , Humans , Male , Minocycline/therapeutic use , Pancreatic Pseudocyst/microbiology , Pancreatitis, Acute Necrotizing/microbiology
7.
Pulm Circ ; 14(2): e12367, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38646413

ABSTRACT

Current predictors of clinical outcomes after pulmonary thromboendarterectomy (PTE) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) are largely limited to preoperative clinical characteristics. N-terminal-pro-brain natriuretic peptide (NT-pro-BNP), a biomarker of right ventricular dysfunction, has not yet been well described as one such predictor. From 2017 to 2021, 816 patients with CTEPH referred to the University of California, San Diego for PTE were reviewed for differences in NT-pro-BNP to predict preoperative characteristics and postoperative outcomes up to 30 days post-PTE. For analysis, NT-pro-BNP was dichotomized to less than/equal to or greater than 1000 pg/mL based on the mean of the study population. Mean NT-pro-BNP was 1095.9 ±1783.4 pg/mL and median was 402.5 pg/mL (interquartile range: 119.5-1410.8). Of the 816 patients included, 250 had NT-pro-BNP > 1000 pg/mL. Those with NT-pro-BNP > 1000 pg/mL were significantly more likely to have worse preoperative functional class (III-IV) and worse preoperative hemodynamics. Patients with NT-pro-BNP > 1000 pg/mL also tended to have more postoperative complications including reperfusion pulmonary edema (22% vs. 5.1%, p < 0.001), airway hemorrhage (8.4% vs. 4.9%, p = 0.075), residual pulmonary hypertension (11.9% vs. 3.1%, p < 0.001), and 30-day mortality (4.8% vs. 1.1%, p = 0.001). Even after adjusting for confounders, patients with NT-pro-BNP > 1000 pg/mL had a 2.48 times higher odds (95% confidence interval: 1.45-4.00) of reaching a combined endpoint that included the above complications. Preoperative NT-pro-BNP > 1000 pg/mL is a strong predictor of more severe preoperative hemodynamics and identifies patients at higher risk for postoperative complications.

9.
Eur Respir J ; 41(3): 735-42, 2013 Mar.
Article in English | MEDLINE | ID: mdl-23143539

ABSTRACT

It is likely that chronic thromboembolic pulmonary hypertension (CTEPH) is more prevalent than currently recognised. Imaging studies are fundamental to decision making with respect to operability. All patients with suspected CTEPH should be referred to an experienced surgical centre. Currently, there is no risk scoring stratification system to guide operability assessment and it is predominantly based on surgical experience. The aim of pulmonary endarterectomy (PEA) is the removal of obstructive material to immediately reduce pulmonary vascular resistance. PEA affords the best chance of cure, but is difficult to perfect. Recognition and clearance of distal segmental and subsegmental disease is the main problem. The basic surgical techniques include: median sternotomy incision, cardiopulmonary bypass, arteriotomy incisions within pericardium, and a true endarterectomy with meticulous full distal dissection. Deep hypothermic circulatory arrest is recommended as the best means of reducing blood flow in the pulmonary artery to allow a clear field for dissection. In the recent PEACOG (PEA and COGnition) trial there was no evidence of cognitive impairment post-PEA. Reperfusion pulmonary oedema and residual pulmonary hypertension are unique post-operative complications post-PEA and are associated with increased mortality. However, in-hospital mortality is now <5% in experienced centres.


Subject(s)
Endarterectomy/methods , Hypertension, Pulmonary/surgery , Hypertension, Pulmonary/therapy , Thromboembolism/surgery , Vascular Surgical Procedures/methods , Angiography/methods , Brain/pathology , Circulatory Arrest, Deep Hypothermia Induced/methods , Cognition Disorders/prevention & control , Endarterectomy/adverse effects , Humans , Postoperative Complications/prevention & control , Pulmonary Artery/surgery , Pulmonary Edema/surgery , Pulmonary Embolism/surgery , Risk , Vascular Surgical Procedures/adverse effects
10.
ATS Sch ; 4(4): 528-537, 2023 Dec.
Article in English | MEDLINE | ID: mdl-38196677

ABSTRACT

Background: Medical schools have used holistic review in admissions to increase mission-aligned enrollment of students from backgrounds underrepresented in medicine. Graduate medical education programs have increasingly followed suit. However, there is a paucity of literature regarding holistic review at the fellowship level. Objective: Here, we share our experience implementing the Association of American Medical Colleges core principles of holistic review during the 2021 recruitment cycle. Methods: We used a partially asynchronous and online learning strategy to train division members on the principles of holistic review. Following the match, we conducted a survey of faculty members and fellows to understand their opinions on our holistic review training and implementation. Results: Although few of our colleagues clearly understood holistic review before the training, they were able to identify broad-based criteria that aligned with our division's mission and balanced applicants' experiences, attributes, competencies, and metrics. These were viewed as better selection criteria than traditional measures and were incorporated into the individualized consideration of applicants. Our survey had a 41.5% response rate, with 10 of 22 fellows and 24 of 60 faculty members responding. Most faculty members and fellows agreed that holistic review decreases socioeconomic disparities in fellowship recruitment (79.2% and 80.0%, respectively) and promotes inclusion and diversity (83.3% and 90.0%, respectively). Faculty members appeared more confident than fellows that our training efforts had influenced recruitment. All respondents agreed that it would be critical for such training to be repeated yearly. Conclusion: Although this was a single-institution experience, implementing holistic review was feasible and well received by faculty and fellows.

11.
Ann Thorac Surg ; 116(1): 121-128, 2023 Jul.
Article in English | MEDLINE | ID: mdl-36368350

ABSTRACT

BACKGROUND: Airway hemorrhage is a known complication of pulmonary thromboendarterectomy (PTE) in patients with chronic thromboembolic pulmonary hypertension. Predisposing factors for postoperative airway hemorrhage have not been well described. The aims of this study were to determine the incidence and outcomes of airway hemorrhage after PTE and to identify potential risk factors. METHODS: This was a retrospective chart review of subjects undergoing PTE between 2015 and 2019. Postoperative airway hemorrhage was defined as significant endobronchial bleeding requiring withholding anticoagulation, bronchial blocker placement, and/or extracorporeal membrane oxygenation (ECMO). RESULTS: Of 877 subjects who underwent PTE, 58 subjects (6.6%) developed postoperative airway hemorrhage. Subjects with hemorrhage were more likely to be women (60% vs 45%, P = .03), to be older (57.8 vs 54.0 years, P = .04), and to have a higher incidence of preoperative hemoptysis (19.0% vs 7.6%, P = .006) compared with control subjects (subjects without airway hemorrhage). Those with hemorrhage had significantly higher preoperative right atrial pressure (P = .002) and pulmonary vascular resistance (P < .001) and a higher incidence of residual pulmonary hypertension (P = .005). Airway hemorrhage management included ECMO with bronchial blocker (n = 2), bronchial blocker without ECMO (n = 26), or withholding anticoagulation alone until bleeding subsided (n = 30). Mortality was significantly higher in those with airway hemorrhage compared with control subjects (13.8% vs 1.2%, P < .001). CONCLUSIONS: The incidence of postoperative airway hemorrhage is low but associated with significant mortality. Older age, female sex, preoperative hemoptysis, and worse preoperative pulmonary hypertension were associated with an increased risk of developing postoperative airway hemorrhage.


Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Humans , Female , Male , Pulmonary Embolism/surgery , Pulmonary Embolism/complications , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Retrospective Studies , Hemoptysis/etiology , Risk Factors , Postoperative Hemorrhage/etiology , Anticoagulants/therapeutic use , Endarterectomy/adverse effects
12.
J Thorac Cardiovasc Surg ; 166(6): 1512-1519.e2, 2023 12.
Article in English | MEDLINE | ID: mdl-37032250

ABSTRACT

OBJECTIVE: Chronic thromboembolic pulmonary hypertension is potentially curable via pulmonary thromboendarterectomy. A minority of patients experience recurrence of their symptoms and are eligible for repeat pulmonary thromboendarterectomy. However, little data exist regarding risk factors and outcomes for this patient population. METHODS: We performed a retrospective review of the University of California San Diego chronic thromboembolic pulmonary hypertension quality improvement database, including all patients who underwent pulmonary thromboendarterectomy from December 2005 to December 2020. Of the 2019 cases performed during this period, 46 were repeat pulmonary thromboendarterectomy procedures. Demographics, preoperative and postoperative hemodynamics, and surgical complications were compared between the repeat pulmonary thromboendarterectomy group and 1008 first pulmonary thromboendarterectomy group. RESULTS: Repeat pulmonary thromboendarterectomy recipients were more likely to be younger, to have an identified hypercoagulable state, and to have higher preoperative right atrial pressure. Etiologies of recurrent disease include incomplete initial endarterectomy, discontinuation of anticoagulation (noncompliance or for medical reasons), and anticoagulation treatment failure. Patients who received repeat pulmonary thromboendarterectomy had significant hemodynamic improvement, but less pronounced compared with patients who received first pulmonary thromboendarterectomy. Repeat pulmonary thromboendarterectomy was associated with an increased risk of postoperative bleeding, reperfusion lung injury, residual pulmonary hypertension, and increased ventilator, intensive care unit, and hospital days. However, hospital mortality was similar between the groups (2.2% vs 1.9%). CONCLUSIONS: This is the largest reported series of repeat pulmonary thromboendarterectomy surgery. Despite an increase in postoperative complications, this study demonstrates that repeat pulmonary thromboendarterectomy surgery can result in significant hemodynamic improvement with acceptable surgical mortality in an experienced center.


Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Reperfusion Injury , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Pulmonary Embolism/surgery , Pulmonary Embolism/complications , Retrospective Studies , Reperfusion Injury/complications , Endarterectomy , Anticoagulants , Chronic Disease
13.
J Heart Lung Transplant ; 42(8): 1112-1119, 2023 08.
Article in English | MEDLINE | ID: mdl-37024310

ABSTRACT

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is primarily managed by pulmonary thromboendarterectomy (PTE). As advanced surgical techniques permit resection at the segmental and subsegmental level, PTE can now be curative for CTEPH mostly involving the distal pulmonary arteries. METHODS: Between January 2017 and June 2021, consecutive patients undergoing PTE were categorized according to the most proximal level of chronic thrombus resection: Level I (main pulmonary artery), Level II (lobar), Level III (segmental) and Level IV (subsegmental). Proximal disease patients (any Level I or II) were compared to distal disease (Level III or IV bilaterally) patients. Demographics, medical history, preoperative pulmonary hemodynamics, and immediate postoperative outcomes were obtained for each group. RESULTS: During the study period, 794 patients underwent PTE, 563 with proximal disease and 231 with distal disease. Patients with distal disease more frequently had a history of an indwelling intravenous device, splenectomy, upper extremity thrombosis or use thyroid replacement and less often had prior lower extremity thrombosis or hypercoagulable state. Despite more use of PAH-targeted medications in the distal disease group (63.2% vs 50.1%, p < 0.001), preoperative hemodynamics were similar. Both patient groups exhibited significant improvements in pulmonary hemodynamics postoperatively with comparable in-hospital mortality rates. Compared to proximal disease, a lower percentage of patients with distal disease showed residual pulmonary hypertension (3.1% vs 6.9%, p = 0.039) and airway hemorrhage (3.0% vs 6.6%, p = 0.047) postoperatively. CONCLUSIONS: Thromboendarterectomy for distal (segmental and subsegmental) CTEPH is technically feasible and may result in favorable pulmonary hemodynamic outcomes, without increased mortality or morbidity.


Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Humans , Pulmonary Embolism/complications , Pulmonary Embolism/surgery , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Pulmonary Artery/surgery , Hemodynamics , Endarterectomy/methods , Chronic Disease
14.
Chest ; 163(1): 204-215, 2023 01.
Article in English | MEDLINE | ID: mdl-36087794

ABSTRACT

BACKGROUND: The prognosis and therapeutic responses are worse for pulmonary arterial hypertension associated with systemic sclerosis (SSc-PAH) compared with idiopathic pulmonary arterial hypertension (IPAH). This discrepancy could be driven by divergence in underlying metabolic determinants of disease. RESEARCH QUESTION: Are circulating bioactive metabolites differentially altered in SSc-PAH vs IPAH, and can this alteration explain clinical disparity between these PAH subgroups? STUDY DESIGN AND METHODS: Plasma biosamples from 400 patients with SSc-PAH and 1,082 patients with IPAH were included in the study. Another cohort of 100 patients with scleroderma with no PH and 44 patients with scleroderma with PH was included for external validation. More than 700 bioactive lipid metabolites, representing a range of vasoactive and immune-inflammatory pathways, were assayed in plasma samples from independent discovery and validation cohorts using liquid chromatography/high-resolution mass spectrometry-based approaches. Regression analyses were used to identify metabolites that exhibited differential levels between SSc-PAH and IPAH and associated with disease severity. RESULTS: From hundreds of circulating bioactive lipid molecules, five metabolites were found to distinguish between SSc-PAH and IPAH, as well as associate with markers of disease severity. Relative to IPAH, patients with SSc-PAH carried increased levels of fatty acid metabolites, including lignoceric acid and nervonic acid, as well as eicosanoids/oxylipins and sex hormone metabolites. INTERPRETATION: Patients with SSc-PAH are characterized by an unfavorable bioactive metabolic profile that may explain the poor and limited response to therapy. These data provide important metabolic insights into the molecular heterogeneity underlying differences between subgroups of PAH.


Subject(s)
Hypertension, Pulmonary , Scleroderma, Systemic , Humans , Familial Primary Pulmonary Hypertension , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/complications , Scleroderma, Systemic/drug therapy , Prognosis , Lipids/therapeutic use
15.
Respirology ; 17(1): 180-4, 2012 Jan.
Article in English | MEDLINE | ID: mdl-21899658

ABSTRACT

BACKGROUND AND OBJECTIVE: The management of chronic thromboembolic pulmonary hypertension (CTEPH) is dependent on the extent of pulmonary artery obstruction, which is usually evaluated by planar perfusion scanning and CT pulmonary angiography (CTPA). We previously reported that SPECT perfusion scanning is more sensitive than planar scanning for detecting vascular obstruction in CTEPH. The purpose of this study is to compare SPECT with CTPA for detecting segmental pulmonary artery obstruction in CTEPH. METHODS: SPECT and CTPA were carried out before pulmonary endarterectomy in 12 CTEPH patients. Field experts documented the anatomical distribution of perfusion defects disclosed by SPECT, the anatomical distribution of pulmonary arterial filling defects disclosed by CTPA and the segmental anatomy of the vascular obstructions based on a review of clinical and pathology records, without knowledge of scan results. RESULTS: Clinical/pathological evaluation disclosed 140 obstructed (15.5 ± 2.5 per patient) and 40 unobstructed lung segments. SPECT scanning identified 87/140 (62%) of the obstructed and 29/40 (72%) of the unobstructed segments. By comparison, CTPA identified 67/140 (47.8%) of the obstructed and 32/40 (80%) of the unobstructed segments. Sensitivity for detecting obstructed segments was significantly higher for SPECT compared with CTPA (62 ± 4.1% vs 47.8 ± 2.9%, respectively; P = 0.03). CONCLUSIONS: SPECT is more sensitive than CTPA for identifying obstructed segments in this small sample of CTEPH patients. However, even SPECT under-represents the extent of vascular obstruction from this disease.


Subject(s)
Angiography/methods , Hypertension, Pulmonary/etiology , Pulmonary Artery/diagnostic imaging , Pulmonary Embolism/complications , Radiopharmaceuticals , Tomography, Emission-Computed, Single-Photon , Tomography, X-Ray Computed , Adult , Aged , California/epidemiology , Female , Humans , Hypertension, Pulmonary/diagnostic imaging , Male , Middle Aged , Perfusion Imaging , Pilot Projects , Pulmonary Embolism/diagnostic imaging , Reproducibility of Results , Sensitivity and Specificity , Tomography, Emission-Computed, Single-Photon/methods
16.
Am J Respir Crit Care Med ; 183(12): 1605-13, 2011 Jun 15.
Article in English | MEDLINE | ID: mdl-21330453

ABSTRACT

Over the past 4 decades, chronic thromboembolic pulmonary hypertension has evolved from an autopsy curiosity to a potentially correctable form of pulmonary hypertension. Advances in surgical techniques along with the introduction of pulmonary hypertension disease-modifying therapies provide a therapeutic option for the majority of patients afflicted with the disease. Approximately 5,000 thromboendarterectomy procedures have now been performed worldwide with mortality rates reported by established programs experienced in the management of this disease process falling to a range of 4 to 7%. A mortality rate of 1.3% has been reported in patients at low risk based on their preoperative hemodynamic profile. After a successful pulmonary thromboendarterectomy, substantial improvement and often normalization can be achieved in right ventricular function, gas exchange, exercise capacity, and quality of life. For patients not candidates for thromboendarterectomy, or for those with persistent post-thromboendarterectomy pulmonary hypertension, disease-modifying medical therapies have been demonstrated to stabilize and improve pulmonary hemodynamics, albeit not to the same extent as primary thromboendarterectomy. The current review focuses on the diagnostic approach to chronic thromboembolic pulmonary hypertension and the available surgical and medical therapeutic options. Additional research is necessary to more accurately predict postoperative hemodynamic outcome and to define the optimal therapeutic approach, especially in patients with involvement of the distal vasculature.


Subject(s)
Hypertension, Pulmonary/therapy , Pulmonary Embolism/therapy , Chronic Disease , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Pulmonary Embolism/complications , Pulmonary Embolism/diagnosis , Pulmonary Embolism/surgery , Treatment Outcome
17.
J Emerg Med ; 42(3): 275-8, 2012 Mar.
Article in English | MEDLINE | ID: mdl-19201139

ABSTRACT

BACKGROUND: Dog bites are the most common animal bite injuries occurring in the United States. Estimated infection rates range between 15% and 20%. Polymicrobial infections are most common. Capnocytophaga canimorsus (C. canimorsus) is a Gram-negative rod strongly associated with dog bites, and is known to cause life-threatening infection in humans. OBJECTIVES: 1) Outline epidemiology of dog bites in the United States; 2) Identify host factors associated with infection, and common pathogens; 3) Discuss microbiology of C. canimorsus; 4) Discuss common clinical manifestations of C. canimorsus infection; 5) Outline treatment options. CASE REPORT: A 42-year-old woman with a remote history of Hodgkin's lymphoma (treated with irradiation) and thyroid carcinoma, both of which were in remission, presented to the Emergency Department with fever, abdominal pain, and diarrhea. She was found to be in septic shock. She was aggressively resuscitated and administered broad-spectrum antibiotics. Blood cultures grew C. canimorsus in 2/4 bottles. The patient recalled being bitten by the family dog 48 h before her initial presentation. She made an uneventful recovery. She was felt to be "functionally hyposplenic" due to her prior irradiation. CONCLUSIONS: C. canimorsus is a rare pathogen strongly associated with dog bites. By eliciting a history of animal bite, clinicians may be able to alert the laboratory of suspected C. canimorsus infection. Prolonged laboratory incubation times may be necessary as the organism is fastidious. Predisposing conditions include, among others, prior splenectomy and alcoholism. The mortality rate from C. canimorsus sepsis is high, so treatment should be promptly initiated.


Subject(s)
Bites and Stings/complications , Capnocytophaga/isolation & purification , Dogs , Gram-Negative Bacterial Infections/diagnosis , Shock, Septic/microbiology , Adult , Animals , Bites and Stings/microbiology , Female , Gram-Negative Bacterial Infections/microbiology , Humans
18.
Pulm Circ ; 12(3): e12110, 2022 Jul.
Article in English | MEDLINE | ID: mdl-35874854

ABSTRACT

Patients with chronic thromboembolic pulmonary hypertension (CTEPH) require lifelong anticoagulant therapy. The safety and efficacy of direct oral anticoagulant (DOAC) in the chronic and transitional management of CTEPH has not been investigated. We performed a retrospective analysis of 405 consecutive pulmonary endarterectomy (PEA) cases at the University of California, San Diego, from July 2015 through July 2017. PEA specimen was reviewed for the presence of acute or subacute thrombotic material distinct from the expected chronic disease removed at the time of PEA by two investigators blinded to the patient information. Of 405 PEA cases, 166 patients (41.0%) were anticoagulated with one of three available DOACs; 239 (59.0%) presented on either oral vitamin-K antagonist or chronic injectable therapy. There were no significant differences in baseline characteristics between DOAC and non-DOAC groups. Evidence of recent thrombus was observed in 22 (13.3%) in the DOAC group versus 16 (6.7%) within the non-DOAC group. The odds ratio of DOACs usage and evidence of recent thrombus was 2.34 (95% confidence interval: 1.1-5.0, p = 0.03) after adjusting for age, gender, race, body mass index, and history of antiphospholipid antibody syndrome. CTEPH patients referred for PEA while on DOAC therapy were twice as likely to have associated acute or subacute thrombi present at the time of surgery compared with those on more traditional, non-DOAC anticoagulant therapies. This raises questions of the safety and efficacy of DOACs in the chronic management of CTEPH.

19.
Pulm Circ ; 12(4): e12148, 2022 Oct.
Article in English | MEDLINE | ID: mdl-36325508

ABSTRACT

Chronic thromboembolic pulmonary hypertension (CTEPH) is the result of incompletely resolved pulmonary emboli (PE) that lead to chronic right heart failure. The two mechanical treatment options are pulmonary thromboendarterectomy (PTE) and balloon pulmonary angioplasty (BPA). There are no formal criteria for BPA patient selection and treatment decisions vary according to a center's experience with BPA and PTE. We performed a retrospective review of consecutive patients treated with PTE and BPA at UCSD from March 2015 to 2021. Clinical and hemodynamic data were collected. Patients were categorized according to the rationale for BPA. One hundred fifty three patients underwent 643 BPA sessions, and 1104 patients underwent PTE. Patients selected for PTE had worse baseline hemodynamics with mean pulmonary artery pressure 41.1 ± 11.7 versus 34.6 ± 11.2 mmHg, p < 0.001. 59% of patients selected for BPA had surgically inaccessible disease, 21% had residual CTEPH after PTE, 10% had a discordance between disease burden and symptoms/hemodynamics, 7% had comorbidities that prevented PTE and 3% refused PTE surgery. 28% of patients who underwent PTE had exclusively level III or IV disease based on surgical specimen. There were no BPA procedure-related mortalities and minor pulmonary vascular complication rates during BPA were 9.2%. The most common reason for BPA selection was surgically inaccessible disease followed by residual CTEPH after PTE. Almost one third of patients who underwent PTE had exclusively distal disease by surgical criteria and might have been directed to BPA at a less experienced surgical center.

20.
Pulm Circ ; 12(1): e12004, 2022 Jan.
Article in English | MEDLINE | ID: mdl-35506111

ABSTRACT

Inflammatory pseudotumor (IPT), also known as plasma cell granuloma, is a rare lesion of unknown etiology that occurs in many organs, especially in the lung. Here we report five cases of IPT arising in pulmonary artery mimicking chronic thromboembolic disease, not previously documented in the literature. Those cases were identified at our institute among over 2500 pulmonary endarterectomy (PEA) specimens acquired from 2000 to 2017. The cohort included three men and two women with a median age of 41 years (range: 23-54). All patients presented with dyspnea and radiologic findings of pulmonary artery thromboembolism, some concerning for intimal sarcoma. The duration between disease onset and PEA ranged from 6 months to approximately 3 years. Histologically, all cases showed proliferation of spindle cells with marked inflammatory infiltrates composed predominantly of plasma cells, histiocytes, and small lymphocytes. Ancillary studies were performed in each case and ruled out other possibilities, such as sarcoma, lymphoma, plasmacytoma, IgG4-related disease, and infection. IPT arising in pulmonary artery presenting clinically as acute or chronic thromboembolic disease is very unusual, in which clinical data, radiographic findings, and histopathologic features have to be integrated for reaching the proper diagnosis.

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