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The lung is the most common organ affected by sarcoidosis. Multiple tools are available to assist clinicians in assessing lung disease activity and in excluding alternative causes of respiratory symptoms. Improving outcomes in pulmonary sarcoidosis should focus on preventing disease progression and disability, and preserving quality of life, in addition to timely identification and management of complications like fibrotic pulmonary sarcoidosis. While steroids continue to be first-line therapy, other therapies with fewer long-term side-effects are available and should be considered in certain circumstances. Knowledge of common clinical features of pulmonary sarcoidosis and specific pulmonary sarcoidosis phenotypes is important for identifying patients who are more likely to benefit from treatment.Copyright © ERS 2022.
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Background: Patients with coronavirus disease 2019 (COVID-19) and severe acute respiratory distress syndrome (ARDS) need in 10.5 to 15% veno-venous ECMO (V-V ECMO) therapy. The worldwide mortality in COVID-19 patients on ECMO has been described as extremely high with a mortality rate of 40 to 70%. Method(s): We collected data from 56 patients with severe ARDS who received V-V ECMO in 2020 to January 2022 at the University Hospital Magdeburg due to COVID-19 infection. We recorded demographic, pre-, intra-, and posttreatment data retrospectively. We divided the patients into two groups (survivors and nonsurvivors) to build the final prediction model based on our statistic and to detect relevant mortality risk factors. Result(s): Only 39.3% of patients survived the intensive care unit. Compared groups didn't differ in associated diseases. Most of the non-survivors were male (14 [63.6%] vs. 28 [82.4%], p = 0.114). Nonsurvivors showed a higher incidence of bleeding complications (10 [45.5%] vs. 23 [67.6%], p = 0,099), especially hemothorax (1 [4.5%] vs. 7 [20.6%], p = 0.094) and endobronchial bleeding (0 vs. 5 [14.7%], p = 0.059) as well as a higher incidence of bacterial superinfection (9 [40.1%] vs. 22 [64.7%], p = 0.080). Moreover, groups differed concerning the incidence of acute kidney injury without dialysis (1 [4.5% vs. 9 [26.5%], p = 0.036), and acute liver failure (1 [4.5%] vs. 7 [20.6%], p = 0.094). According to the results of bivariate regression analysis, male sex (odd ratio [OR]: 2.66;95% confidence interval [CI]: 0.773-9.194;p = 0.120), major bleeding events (OR: 2.50;95% CI: 0.831-7.574;p = 0.103), bacterial superinfection (OR: 2.65;95% CI: 0.879-7.981;p = 0.084), acute kidney injury without dialysis (OR: 7.56;95% CI: 0.884-64.636;p = 0.065), and acute liver failure (OR: 5.44;95% CI: 0.621-47.756, p = 0.126) were tendentious significant predictors of death. Subsequently, according to the results of multivariate analysis, the most significant factors of mortality were major bleeding events (OR: 3.27;95% CI: 0.888-12.047, p = 0.075) and the bacterial superinfection (OR: 2.81;95% CI: 0.800-9.888, p = 0.107). The mortality prediction model explained 31.8% (Nagelkerke R2) of the variance in-hospital mortality and correctly classified 71.4% of the cases. Conclusion(s): Major bleeding events and bacterial superinfection might be relevant mortality factors in COVID-19 patients on V-V ECMO therapy. Especially prevention of superinfection and strictly anticoagulation management might result in lower mortality rates.
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Outbreak of coronavirus disease 2019 (COVID-19) with its overwhelming morbidity and mortality has created a significant challenge for health systems worldwide. Although peripheral ground-glass opacities are the most frequent radiologic feature of COVID-19 described in the literature, long term rare complications such as cavitations, pneumatocele, pneumothorax, empyema or hemothorax are occasionally reported. Pneumatoceles are defined as thin-walled air-filled cyst in the lung interstitium, caused by ischemia related damage of alveolar wall and check valve mechanism, which lead to air trapping. We describe a case series of eight patients, diagnosed with COVID-19, confirmed by the reverse transcription polymerase chain reaction (RT-PCR) for SARS-CoV-2 of nasopharyngeal swab and complicated with pneumatocele and pneumothorax, the largest group at present. Among these patients seven were males, only one had a history of a previous lung disease, six had a smoking history. One out of these eight patients suffered from acute respiratory distress syndrome (ARDS) requiring mechanical ventilation. During the hospitalization three patients received surgical intervention with resection of pneumatocele, others were successfully treated conservatively with antibiotic therapy. Reason for surgical intervention included non-resolving pneumothorax, superinfection of pneumatocele non responding to antibiotic therapy and hemothorax. According to our study not every pneumatocele requires surgical intervention. Conservative management or surgical approach in those complications should be decided individually.
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Background: The conduct of clinical trials during COVID-19 created challenges to ensuring access to clinical trials, healthy clinical teams, and patients. Innovative methods such as remote patient follow-up and monitoring visits can lead to successful completion of device trials. Objective(s): The INGEVITY+ Active Fixation Pace/Sense Lead Study examined the safety/ efficacy of the INGEVITY+ Lead in a prospective, non-randomized, multicenter, post-approval study. Method(s): Subjects met indications for a pacing device with INGEVITY+ leads implanted in the RA and RV. The primary safety endpoint was the 3-month lead-related complication-free (LRC-free) rate. The primary efficacy endpoint evaluated the 3-month pacing capture threshold with the secondary efficacy endpoint evaluating the other lead-related electrical parameters. Implanter experience was assessed. Telephone visits with LATITUDE remote transmission of lead measurement data and remote monitoring visits were allowed. Result(s): A total of 201 leads were evaluated in 101 subjects, 46.8% female with a mean age of 73.2 years, at 13 centers in the United States. The study took 6.8 months, with a median follow-up of 3.4 months. The 3-month follow-up was completed by 98% of subjects, of which 36% were remote. A total of 98% of monitoring visits were done remotely. The study met all primary and secondary endpoints. The LRC-free rate through 3-months was 98.5% (lower CL 95.4%). Three leads were repositioned and reimplanted;two were RA dislodgements, and one was a RA perforation and hemothorax requiring thoracentesis. The pacing capture threshold was <= 2V in 98.9% of subjects with a mean of 0.77 V at 0.4-ms pulse width, mean pacing impedance of 712 ohms, and a median P-wave amplitude of 4.3 mV, and median R-wave amplitude of 15.9 mV at 3- months. No helix performance issues were reported. The mean turn count for helix extension was 6.7 and retraction was 7.6. More than 85% of physicians rated the lead as 'very good' or higher on all aspects of lead handling. Conclusion(s): The INGEVITY+ Lead was found to be safe and effective with a very favorable implant experience. This study was entirely conducted successfully during COVID-19 with remote follow-up and monitoring visits, and nearly half of the subjects were females.
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Background: HIT is an intensely procoagulant disorder and in patients on ECMO it is associated with a high thrombotic morbidity and mortality, thus is crucial to intervene early. Aim(s): To present a case of a COVID-19 patient on ECMO who was diagnosed HIT. To describe evolution on Rivaroxaban treatment due to the unavailability of parenteral non-heparin anticoagulants in our country. Method(s): Not applicable. Result(s): A 60-year- old female patient was admitted to our hospital for ECMO support. She was diagnosed with SARS-CoV2, her condition quickly worsened with pneumothorax and refractory hypoxemia hence she was referred to our Institution. ECMO and hemodialysis were required. Unfractionated heparin (UFH) was given to achieve goal anti-Xa 0.3-0.7 units/mL. Platelet count (PLT) was 278,000 / mm3 and D dimer 1844 ng/mL FEU. Next day UFH was stopped because of haemothorax. Five days after restart anticoagulation an increased transmembrane pressure and a trombi was observed in the system. PLT were 12,000/mm3 and Ddimer 8500 ng/mL FEU. HIT was suspected;4Ts score = 7 and anti-FP4 antibodies positive (5.7 UA/mL). UFH was stopped and due to inaccessibility to other intravenous anticoagulant rivaroxaban 15 mg twice daily was started. Rivaroxaban calibrated anti-Xa assay was twice daily performed to monitor trough and peak levels, 30 mg twice daily was necessary to achieve therapeutic plasma concentration. Patient's PLT recovered after 7 days. No thrombotic event was recorded during rivaroxaban use. After ECMO was weaning-off rivaroxaban dose was reduced to 10 mg/day. Ten days later she had an upper gastrointestinal bleeding because gastric ulcer and rivaroxaban was stopped. With clinical stability and non evidence of bleeding, thromboprophylaxis with rivaroxaban was restarted and continued until discharged. Conclusion(s): Our case highlights the difficulties on management of HIT in patients on ECMO support and the need for consented guidelines in this specific situation, particularly for countries without access to parenteral non-heparin anticoagulants.
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SESSION TITLE: Pneumothorax, Chylothorax, and Pleural Effusion Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Chest tube placement is generally done to drain air (Pneumothorax) or fluid (Effusion or Hemothorax) from the pleural cavity. The incidence of complications related to such intervention varies between 1 to 6 percent (1), and includes but not limited to malposition, injuring chest wall structures, injuring intrathoracic structures, bleeding, and infection. In this case we present an unusual complication to surgical chest tube placement. CASE PRESENTATION: Our patient is a 59-year-old male, long term resident of a nursing facility with past medical history of alcohol use disorder in remission, alcoholic cirrhosis, seizure disorder, protein-calorie malnutrition and a recent COVID-19 infection. He presented with worsening shortness of breath and was admitted with acute hypoxemic respiratory failure. Initial CT scan showed fibrotic, reticular and cystic changes, traction bronchiectasis and diffuse bilateral ground glass opacities. He was admitted to the medical ICU;he was treated initially with broad spectrum antibiotics and diuresis with minimal response. Eventually steroid therapy was started for Covid related organizing pneumonia, and he improved. Later in his hospital state he developed bilateral small pneumothoraxes that enlarged overtime and a surgical chest tube was placed on the right side. Post procedure chest x ray showed that the tube was kinked, and the pneumothorax was still present. A follow up CT chest confirmed the presence of an extra-pleural hematoma with the tube kinked inside it. CT angiography of the chest was done and showed active extravasation of contrast into the extra-pleural space likely from the intercostal arterial branches. Interventional radiology took the patient to see if they could cauterize the bleeding vessel but they were unable to identify the source of bleeding. Thoracic surgery was also consulted and was planning to take the patient to the OR, remove the tube, evacuate the hematoma and control the bleeding. However, the patient opted against this. DISCUSSION: Extra-pleural hematoma is a rare complication of surgical chest tube placement. It is usually seen after blunt trauma or rib fracture, but can still occur after subclavian vein central line placement or chest tube placement. Bleeding is usually arterial in origin and treatment is often surgical. Radiological characteristics include biconvex shape and the extra-pleural fat sign (2,3,);hypodense rim medial to the hematoma due to the inward displacement of the extra-pleural fat by the hematoma. CONCLUSIONS: Chest tube placement remains a routine procedure that is done in emergency departments and hospital wards. Generally, a safe intervention but clinicians should be aware of the possible complications and their management including extra-pleural hematomas. Reference #1: Pleural procedures and thoracic ultrasound: British Thoracic Society pleural disease guideline 2010 Tom Havelock1, Richard Teoh2, Diane Laws3, Fergus Gleeson4 on behalf of the BTS Pleural Disease Guideline Group. Correspondence to Dr Tom Havelock, Wellcome Trust Clinical Research Facility, Southampton General Hospital, Southampton SO16 6YD, UK;t.havelock@soton.ac.uk Reference #2: Journal of Trauma and Injury 2017;30(4): 202-205. Published online: December 30, 2017 DOI: https://doi.org/10.20408/jti.2017.30.4.202 Traumatic Extrapleural Hematoma Mimicking Hemothorax Yong Seon Choi, M.D., Soon Jin Kim, M.D., Sang Woo Ryu, Seung Ku Kang Department of Thoracic and Cardiovascular Surgery, Mokpo Hankook Hospital, Mokpo, Korea Correspondence to: Soon Jin Kim, M.D., Department of Thoracic and Cardiovascular Surgery, Mokpo Hankook Hospital, 483 Yeongsan-ro, Mokpo 58643, Korea, Tel: +82-61-270-5574, Fax: +82-61-277-0199, E-mail : innocent-blood@hanmail.net Reference #3: The Journal of Emergency Medicine Volume 51, Issue 2, August 2016, Pages 159-163 Nonoperative Management of a Large Extrapleural Hematom after Blunt Chest Trauma LuisGorospe MD, María Ángeles Fernández-Méndez MD, AnaAyala-Carbonero MD, AlbertoCabañero-Sánchez MD, Gemma MaríaMuñoz-Molina MD, PhD DISCLOSURES: No relevant relationships by Ahmad Allaham No relevant relationships by Elyce Sheehan
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SESSION TITLE: Lessons Learned from Critical Care Cases SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 12:25 pm - 01:25 pm INTRODUCTION: Air outside the lungs, bowel, or paranasal cavities suggests critical pathology. Pneumoperitoneum is a classic example in which free abdominal air may signify hollow viscus injury and the need for emergent surgical management. Ectopic gas can also be secondary to barotrauma secondary to mechanical ventilation and concurrent lung injury;the latter being findings often observed in COVID pneumonia (1,2,3). Our case of extensive intramedullary gas in the setting of COVID pneumonia is an example of extensive dissecting air related to barotrauma, but also illustrates how it mimics dire cases of pneumoperitoneum. Therefore, it is an imaging finding that intensivists caring for COVID pneumonia patients should be aware of. CASE PRESENTATION: A 52-year-old male with mild restrictive lung disease from congenital scoliosis developed COVID pneumonia and hypoxic respiratory failure requiring intubation. Clinical course was complicated by renal failure, deep venous thromboses, and radial artery occlusion. CT evaluation revealed large volume upper abdominal pre-peritoneal gas, pneumoperitoneum, soft tissue and intramedullary gas within bilateral ribs and multiple vertebral bodies. Despite reassuring abdominal exams, the patient deteriorated. The patient was placed on comfort care and expired. DISCUSSION: Intramedullary gas refers to the presence of air within the cortical or trabecular bone, bone marrow, or medullary cavity. It is an exceedingly rare imaging finding which was first described in ischemic vertebral collapse and osteomyelitis (4,5). Differential diagnosis includes infection, trauma, degenerative and iatrogenic causes (5,6). Embryologically, fascial layers of the thorax and periosteal coverings of the thoracic osseous structures are derived from the mesoderm, thus creating a continuum between the lungs, surrounding soft tissues, peritoneum, and surrounding osseous structures, and therefore allowing gas to travel between the lung and intramedullary space (7). As cases of COVID pneumonia with ARDS increase, we are becoming aware of the increasing incidence of ectopic air, and the poor prognosis and increased mortality that results (1). Therefore, the finding of intramedullary gas is an important prognostic indicator that the clinician should be aware of. CONCLUSIONS: Several recent studies demonstrate increased mortality in COVID patients who develop ectopic gas as a result of barotrauma and/or acute lung injury (1,2,3). As such the clinician should be aware of these findings, which include pneumomediastinum, subcutaneous emphysema, pneumoperitoneum, pre-peritoneal air, and intramedullary gas for early recognition. Reference #1: 1. Lemmers DHL, Abu Hilal M, Bnà C, Prezioso C, Cavallo E, Nencini N, Crisci S, Fusina F, Natalini G. Pneumomediastinum and subcutaneous emphysema in COVID-19: barotrauma or lung frailty? ERJ Open Res. 2020 Nov 16;6(4):00385-2020. doi: 10.1183/23120541.00385-2020. PMID: 33257914;PMCID: PMC7537408. Reference #2: 2. Guven BB, Erturk T, Kompe Ö, Ersoy A. Serious complications in COVID-19 ARDS cases: pneumothorax, pneumomediastinum, subcutaneous emphysema and haemothorax. Epidemiol Infect. 2021 Jun 8;149:e137. doi: 10.1017/S0950268821001291. PMID: 34099076;PMCID: PMC8207553. Reference #3: 3. Tetaj N, Garotto G, Albarello F, Mastrobattista A, Maritti M, Stazi GV, Marini MC, Caravella I, Macchione M, De Angelis G, Busso D, Di Lorenzo R, Scarcia S, Farina A, Centanni D, Vargas J, Savino M, Carucci A, Antinori A, Palmieri F, D'Offizi G, Ianniello S, Taglietti F, Campioni P, Vaia F, Nicastri E, Girardi E, Marchioni L, Icu Covid-Study Group. Incidence of Pneumothorax and Pneumomediastinum in 497 COVID-19 Patients with Moderate-Severe ARDS over a Year of the Pandemic: An Observational Study in an Italian Third Level COVID-19 Hospital. J Clin Med. 2021 Nov 29;10(23):5608. doi: 10.3390/jcm10235608. PMID: 34884310;PMCID: PMC8658701. DISCLOSURES: Researc support relationship with 4D Medical Please note: March 2021 Added 04/04/2022 by Anu Brixey, value=Grant/Research Support No relevant relationships by raluca mccallum
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SESSION TITLE: Management of COVID-19-Induced Complications SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Rib fractures are an infrequent consequence of severe cough. In some patients, undetected rib fractures can lead to life-threatening outcomes. We bring to light a case of uncontrolled cough associated with COVID-19 infection resulting in multiple rib fractures that were missed in 2 hospital visits and subsequently diagnosed when the patient presented in acute distress and at high risk of decompensation from a hemothorax and a diaphragmatic hernia that required expedited evaluation, ICU admission, and surgical intervention. CASE PRESENTATION: This is a case of a 73 years old male who presented with shortness of breath and worsening dry cough of 4 weeks duration. He was seen in the ED 3 weeks previously for severe right-sided chest pain that was evaluated by a CT chest that did not reveal any acute findings. He was discharged home on pain medications. He had a repeat admission in 2 days and was diagnosed with acute hypoxic respiratory failure secondary to COVID-19 infection. CT abdomen was done for continued right upper quadrant pain which showed an upper abdominal wall hernia. He was discharged with instructions to follow up with outpatient surgery for hernia repair. In the interim, the patient continued to have excessive dry cough, progressively worsening shortness of breath, and bruising over the right abdominal wall and back. He returned to the ED where he was found to be hypoxic, and hypotensive. Imaging studies revealed a large right pleural effusion, fracture of the right sixth rib and seventh rib, and herniation of the hepatic flexure of the colon into the chest. He was subsequently admitted to the cardiothoracic ICU and underwent flexible bronchoscopy, right VATS, evacuation of hemothorax, complete decortication, and repair of diaphragmatic hernia. DISCUSSION: This case is an unusual presentation of an amalgamation of complications resulting from an unrelenting cough that prompted rapid recognition and swift action. Cough-induced complications are rare but can be life-threatening. Various imaging studies can be pursued to avoid a delay in diagnosis. CONCLUSIONS: Persistent cough is a common complication of COVID-19 infection. A clinician should have a high index of suspicion for rib fractures, diaphragmatic hernia, and hemothorax in a patient with persistent or progressive symptoms. Reference #1: Camarillo-Reyes LA, Marquez-Córdova RI, Surani S, Varon J. Hemothorax induced by severe cough: An unusual presentation. SAGE Open Med Case Rep. 2019;7:2050313X19846043. Published 2019 Apr 26. doi:10.1177/2050313X19846043 Reference #2: Daccache A, Haddad J, Ghanem A, Feghali EJ, El Osta B. Cough-induced rib fracture in a smoker: a case report. J Med Case Rep. 2020;14(1):147. Published 2020 Sep 5. doi:10.1186/s13256-020-02497-4 DISCLOSURES: No relevant relationships by Navya Akula No relevant relationships by Sanjana Chetana Shanmukhappa No relevant relationships by Muhammad Ahmed Malik No relevant relationships by Aqsa Malik No relevant relationships by Fahd Shaukat
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Introduction: Pulmonary artery aneurysms (PAAs) are exceedingly rare. Etiology includes congenital, idiopathic, and acquired. Bacterial and fungal infections are the most common acquired causes. Herein described is a patient with new-onset diabetes mellitus I (DM1) with COVID-19 infection complicated by PAA and mucormycosis. Case Description: A 17-year-old female with new-onset DM1 was admitted to the PICU with diabetic ketoacidosis, and COVID-19 infection complicated by multifocal necrotizing pneumonia. She was treated with remdesivir, antibiotics, systemic glucocorticoids, and discharged on inhaled glucocorticoids. Two weeks later she presented with hemoptysis. Chest computed tomography angiography (CTA) showed a resolving necrotizing pneumonia with a 16 mm aneurysmal dilatation of the proximal portion of the right inferior pulmonary artery (RIPA). Hemoptysis resolved, with no intervention required. One month later she presented again with hemoptysis. Repeat chest CTA demonstrated increasing aneurysmal dilatation, measuring 20 mm in diameter. Echocardiography showed no evidence of endocarditis, congenital heart defects, or elevated right ventricular pressures. A comprehensive infectious workup was negative (Table 1). Due to recurrent symptoms, progressive aneurysmal enlargement, and concerns for rupture, patient underwent RIPA occlusion by cardiac catheterization. Two months later hemoptysis recurred. Chest CTA revealed erosion of the occlusion device into the right inferior segmental bronchus. She underwent emergent right middle and lower lobectomy, and arterial bronchial fistula repair. Lung histology revealed non-septate hyphae with peribronchial and perivascular necrotizing granulomas concerning for mucormycosis (Figure 1). She was treated with amphotericin B and discharged on oral posaconazole. Discussion: The incidence of PAA in adults is estimated to be 1 in 14,000 patients. In adults, the upper limit of normal of an interlobar PA by CTA is 17mm. Our patient's RIPA was dilated up to 20 mm, for which she underwent occlusion of the RIPA. The proinflammatory state generated by COVID-19 can result in vascular inflammation and ultimately aneurysmal dilatation. Desnos et al. reported four cases of hemothorax secondary to PAA rupture in COVID-19 patients on ECMO for severe ARDS. The etiology for PAA formation in our patient had a complex interplay of factors including new-onset diabetes, COVID-19 vasculitis, exposure to systemic glucocorticoids, and an opportunistic infection with Mucor spp. Mucormycosis in diabetic patients with COVID-19 has a mortality of 31% in adults. We believe that the lobectomy performed for the management of PAA in our patient led to better outcomes since surgical debridement is a mainstay of mucormycosis treatment, along with antifungal therapy. Conclusion: PAA in children is uncommon. We describe a diabetic patient with COVID-19 pneumonia, complicated by PAA and mucormycosis. In patients with COVID-19 presenting with hemoptysis, it is important to have a high index of suspicion for PAA. Furthermore, diabetic patients with COVID-19 treated with systemic steroids can be at increased risk for mucormycosis. (Table Presented).
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Unexplained hypoxia in a pregnant patient is an alarming finding for patient and provider. The differential for hypoxia is broad, and many imaging techniques and procedures are contraindicated in pregnancy. Transient pulmonary arteriovenous malformations (AVMs) are a rare and relatively poorly studied cause of hypoxia in pregnancy. Our patient is a 27-year-old G1P0 female with a remote history of asthma who presented to clinic with slowly progressive exertional dyspnea, exertional tachycardia, and paroxysmal nocturnal dyspnea. She reported use of a home oximeter which read in the high 80s% during exertion. Prior to presentation, the patient was evaluated in the Emergency Department and noted to have an oxygen saturation of 86% on room air. A transthoracic echocardiogram, computed tomography angiography of chest, and basic laboratories including B-type natriuretic peptide, troponin, COVID-19, and hemoglobin were unremarkable. Her clinical timeline is presented in Figure 1. Further testing was obtained, including pulmonary function testing, bubble echocardiogram, nocturnal oximetry, and shunt study. Work-up revealed a 15-20% shunt, depending on calculation, and insignificant desaturations during nocturnal oximetry. Her symptoms progressed, and repeat nocturnal oximetry showed marked overnight desaturations requiring supplemental oxygen for the remainder of her pregnancy. She delivered a healthy baby girl via cesarean section without serious complication. Repeat testing in the post-partum period showed resolution of nocturnal desaturations and decreased shunt, and her exertional dyspnea and desaturations resolved spontaneously. This case illustrates the challenging diagnosis of transient pulmonary AVM in pregnancy. Case reports of this phenomenon have been published, but as in our case, no definitive diagnosis was made secondary to testing limitations in pregnancy and quick resolution of symptoms in the post-partum period. Some reports describe pre-existing pulmonary AVM becoming worse during pregnancy causing hemothorax, fetal demise and even death. While the mechanism is not known, theories include the vasodilatory effects of progesterone on vascular smooth muscle as well as other physiologic changes in pregnancy such as increased plasma volume. These AVM are thought to be like those seen in hepatopulmonary syndrome. Similar to our case, increasing positional hypoxia has been reported as the pregnancy progresses. Further research dedicated to early and accurate detection of pulmonary AVMs in pregnancy is necessary. (Figure Presented).
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Introduction: Isolated pauci-immune pulmonary capillaritis (IPIPC) is a rare disorder characterized by small vessel vasculitis limited to alveolar capillaries in the absence of systemic manifestations. There are very few case reports of this disorder in the medical literature. Case Report: A 37-yo male with no known history of autoimmune pathology who was admitted to the hospital for evaluation and treatment of dyspnea and thoracalgia. Peripheral blood cultures, serum studies to detect Legionella and Pneumococcus antigens, and a nasopharyngeal swab test for covid-19 were all negative. Chest imaging revealed bilateral pleural effusions from the base to the apices with concomitant atelectasis of the adjacent lung parenchyma. Although the results of an 18F-PET-CT scan revealed no pathological uptake, video-assisted thoracoscopy revealed diffusely edematous pleura with crater-like patches with new onset of venous vessel varicosities, intra-alveolar hemorrhages associated with disordered vascularization, suggesting small vessel vasculitis. Histologic findings included widespread intra-alveolar hemorrhage with organizing injury, hemosiderin-laden macrophages, scattered intra-arterial thrombi, and diffuse perivascular neutrophilic infiltrates consistent with a diagnosis of capillaritis. Conclusions: Given the negative immune studies (save for a weakly-positive lupus anticoagulant and no evidence for extra-pulmonary vasculitis, the diagnosis was Isolated pauci-immune pulmonary capillaritis. The patient recovered in response to immunosuppressive/anti-inflammatory therapy.
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Introduction: Massive bleeding on extracorporeal membrane oxygenation (ECMO) is associated with multiple coagulation defects, including depletion of coagulation factors and development of acquired von Willebrand syndrome (AVWS). The use of recombinant factors, in particular recombinant activated factor VII (rFVIIa, Novoseven), to treat severe refractory hemorrhage in ECMO has been described. However, the use of multiple recombinant factors has been avoided in large part due to concern for circuit complications and thrombosis. Here, we describe the safe and effective administration of rFVIIa and recombinant von Willebrand factor complex (vWF/ FVIII, Humate-P) via post-oxygenator pigtail catheter on VA-ECMO for the treatment of massive pulmonary hemorrhage. Case Description: A 21-month-old (13.4 kg) girl with a recent history of COVID-19 infection presented to an outside hospital with parainfluenza bronchiolitis resulting in acute refractory hypoxemic respiratory failure (oxygenation index 58), refractory septic shock, and myocardial dysfunction. She was cannulated to VA-ECMO and subsequently diagnosed with necrotizing pneumonia from Pseudomonas and herpes simplex infections. Her course was complicated by a large left-sided pneumatocele and bronchopleural fistula requiring multiple chest tubes. She also had right mainstem bronchus obstruction from necrotic airway debris and complete right lung atelectasis. She was noted to have prolonged episodes of mucosal and cutaneous bleeding (oropharynx, chest tube insertion sites, peripheral IV insertion sites) associated with absent high molecular weight von Willebrand multimers consistent with AVWS. Tranexamic acid infusion was initiated and bivalirudin anticoagulation was discontinued. VA-ECMO flows were escalated to 140-160 ml/kg/min to maintain circuit integrity and meet high patient metabolic demand in the absence of anticoagulation. On ECMO day 26, she underwent bronchoscopy to clear necrotic debris from her airway to assist with lung recruitment. The procedure was notable for mucosal bleeding requiring topical epinephrine and rFVIIa. Post-procedure, she developed acute hemorrhage from her right mainstem bronchus, resulting in significant hemothorax (estimated 950 ml) with mediastinal shift, increased venous pressures, desaturation and decreased ECMO blood flow rate, necessitating massive transfusion of 2,050 ml (150 ml/kg) of packed red blood cells, platelets, plasma and cryoprecipitate. An airway blocker was placed in the mid-trachea to control bleeding. In addition to transfusion of appropriate blood products and continuation of tranexamic acid infusion, she was given both rFVIIa (100mcg/kg) and vWF-FVIII (70 units vWF/kg loading dose on the day of hemorrhage, followed by 40 units vWF/kg every 12 hours for 3 additional doses). Both products were administered over 10 minutes through a post-oxygenator pigtail to allow the product to circulate throughout the patient prior to entering the ECMO circuit. The circuit was closely monitored during administration and no changes to circuit integrity were noted in the subsequent hours while hemostasis was achieved. The ECMO circuit remained without thrombosis for 9 days after the bleeding event. Discussion: Balancing anticoagulation and hemostasis is a central challenge in maintaining ECMO support, especially given the prevalence of acquired coagulopathies such as AVWS. For our patient, AVWS contributed to mucosal bleeding necessitating cessation of anticoagulation and utilization of a high ECMO blood flow strategy to minimize circuit clot burden. This was further complicated by absent native lung function and minimal myocardial function, resulting in complete dependence on ECMO. An acute massive pulmonary hemorrhage was treated with multiple recombinant factors (rFVIIa and vWF/FVIII), that are often avoided on ECMO. To minimize clotting risk to the circuit and to maximize transit of these factors to our patient, we added a post-oxygenator pigtail for administration. While this approach was the result of extreme circumstances, th use of a post-oxygenator pigtail for administration of recombinant factors may represent a viable strategy for refractory hemorrhage while on ECMO.