USE OF POINT-OF-CARE ULTRASONOGRAPHY (POCUS) TO PREVENT DILTIAZEM-INDUCED CARDIOGENIC SHOCK

SESSION TITLE: Using Imaging for Diagnosis Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Point of care ultrasonography (POCUS) uses an ultrasound technique that helps physicians augment physical examination findings and guide clinical decision-making at the bedside. We present a case that became a watershed moment for internal medicine residents at Abington Jefferson Hospital to use POCUS for every patient with atrial flutter/fibrillation with RVR prior to initiating diltiazem drip. CASE PRESENTATION: A 73-year-old male presented to the emergency department with complaints of palpitations. He was tachycardic with a heart rate in the 150s, and his rhythm was irregular. His basic labs were normal;an electrocardiogram investigation showed that he was experiencing an atrial flutter with 2:1 and 3:1 blocks. Chest X-ray was clear. He was given IV metoprolol 10 mg twice without achieving rate control and then started on a diltiazem drip, which initially improved his heart rate to 70s with rhythm changing to atrial flutter with 4:1 block. However, he started to become hypoxic, requiring intubation and then hemodynamically unstable, requiring initiation of pressors. Postintubation CXR indicated bilateral diffuse pulmonary edema and vascular congestion. Subsequently, he had Pulseless electrical activity (PEA) arrest. Return of spontaneous circulation (ROSC) was achieved after 3 minutes of chest compression and one round of epinephrine injection. Transthoracic echocardiogram showed an ejection fraction of 10%. He had a right heart catheterization which showed a CI of 1.7 and elevated PCWP and RVP. He was started on milrinone for ionotropic support and needed norepinephrine, vasopressin and phenylephrine to sustain his blood pressure. DISCUSSION: Atrial flutter and fibrillation are routinely seen arrhythmias in hospital settings. Patients with irregular rhythm who are in rapid ventricular rate and normotensive are often given IV metoprolol few times and then started on a diltiazem drip if RVR continues. Diltiazem not only decreases heart rate (negative chronotropic) but also decreases ventricular squeeze (negative ionotropic). It is contraindicated in patients with reduced ejection fraction. Patients’ ejection fraction values are not always known, especially if they have never had a transthoracic echocardiogram in the past or prior records are not available. POCUS helps physicians and residents to access and estimate LV function quickly and augments clinical decision making at the bedside. CONCLUSIONS: Internal Medicine Residents at Abington Hospital have made it a part of their protocol to always perform bedside ultrasonography in patients with atrial flutter/fibrillation with rapid ventricular rate before initiating diltiazem drip to prevent further avoidable cardiogenic shocks. Reference #1: Fey H, Jost M, Geise AT, Bertsch T, Christ M. Kardiogener Schock nach bradykardisierender Therapie bei tachykardem Vorhofflimmern : Fallvorstellung einer 89-jährigen Patientin [Cardiogenic shock after drug therapy for atrial fibrillation with tachycardia : Case report of an 89-year-old woman]. Med Klin Intensivmed Notfmed. 2016 Jun;111(5):458-62. German. doi: 10.1007/s00063-015-0089-9. Epub 2015 Oct 6. PMID: 26440099. Reference #2: Bitar ZI, Shamsah M, Bamasood OM, Maadarani OS, Alfoudri H. Point-of-Care Ultrasound for COVID-19 Pneumonia Patients in the ICU. J Cardiovasc Imaging. 2021 Jan;29(1):60-68. doi: 10.4250/jcvi.2020.0138. PMID: 33511802;PMCID: PMC7847790. Reference #3: Murray A, Hutchison H, Popil M, Krebs W. The Use of Point-of-Care Ultrasound to Accurately Measure Cardiac Output in Flight. Air Med J. 2020 May-Jun;39(3):218-220. doi: 10.1016/j.amj.2019.12.008. Epub 2020 Jan 14. PMID: 32540116. DISCLOSURES: No relevant relationships by Fnu Aisha No relevant relationships by Lucy Checchio No relevant relationships by Ans Dastgir No relevant relationships by Shravya Ginnaram No relevant relationships by Syeda Hassan No relevant relationships by Chaitra Janga No relev nt relationships by Rameesha Mehreen No relevant relationships by Rahat Ahmed Memon No relevant relationships by Binod Poudel No relevant relationships by Shreeja Shah

HYDROXYCHLOROQUINE CAN BREAK YOUR HEART

SESSION TITLE: The Cardiac Intensivist 2 SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 12:25 pm - 01:25 pm INTRODUCTION: Hydroxychloroquine and chloroquine are medications derived from aminoquinoline. They are disease-modifying antirheumatic drugs used in the treatment of systemic lupus erythematosus (SLE). Although well tolerated, they do have side effects such as retinopathy, vacuolar myopathy, neuropathy, and as seen in our patient, cardiotoxicity. CASE PRESENTATION: Patient is a 48 year old female with a past medical history significant for chronic kidney disease secondary to autosomal dominant polycystic kidney disease, SLE on hydroxychloroquine who presented to the emergency department complaining of weakness. On arrival the patient was found to be in cardiogenic shock. Her transthoracic echocardiogram revealed a reduced ejection fraction of 37% and a large pericardial effusion concerning for tamponade physiology. Her COVID-19 PCR test was positive. She was taken for emergent pericardiocentesis which revealed 300cc of exudative fluid. Patient’s right heart catheterization revealed mean pulmonary capillary wedge pressure of 23 mmHg, pulmonary artery pressures of 44 mmHg/24 mmHg, mean 31mmHg, cardiac index 1.1L/min/m² by thermodilution, 1.7 L/min/m² by Fick. Following right heart catheterization and intra aortic balloon pump placement, the patient was admitted to the medical intensive care unit (MICU) and placed on intravenous inotropic and vasopressor support. Shortly after arrival to the MICU, patient had an increase in vasopressor requirements. Bedside ultrasound revealed cardiac tamponade. Patient had approximately 400cc of bloody pericardial fluid removed from her pericardial drain. The decision was made for emergent venoarterial extracorporeal membrane oxygenation (ECMO) to be initiated. Endomyocardial biopsy was performed which revealed vacuolization in the cytoplasm of several myocytes as well as lymphocytes in the interstitium of the endocardium. The vacuoles found in the cardiac myocytes were PAS positive. These biopsy results are consistent with hydroxychloroquine cardiotoxicity. The patient’s hydroxychloroquine was discontinued. In addition to hemodynamic support, she also received intravenous immunoglobuluin and systemic steroids. After a prolonged hospitalization she was successfully discharged. DISCUSSION: Cardiotoxicity is a rare adverse reaction seen with hydroxychloroquine. A 2018 systematic review revealed 127 cases of cardiac toxicity associated with the use of hydroxychloroquine or chloroquine. Most patients had been treated with the medication for a prolonged period of time and the toxicity is dose dependent. The mechanism behind hydroxychloroquine and chloroquine induced cardiomyopathy is believed to be secondary to lysosomal dysfunction as a result of toxic phospholipid accumulation in cardiomyocytes. CONCLUSIONS: In patients with new onset cardiomyopathy, a detailed medication reconciliation should be conducted to evaluate for toxins such as hydroxychloroquine and chloroquine. Reference #1: Della Porta, A., Bornstein, K., Coye, A., Montrief, T., Long, B., & Parris, M. A. (2020). Acute chloroquine and hydroxychloroquine toxicity: A review for emergency clinicians. The American Journal of Emergency Medicine. Reference #2: Abbi, B., Patel, S., Kumthekar, A., Schwartz, D., & Blanco, I. (2020). A Case of Cardiomyopathy With Long-term Hydroxychloroquine Use. JCR: Journal of Clinical Rheumatology, 26(8), e300. Reference #3: Chatre, C., Roubille, F., Vernhet, H., Jorgensen, C., & Pers, Y. M. (2018). Cardiac complications attributed to chloroquine and hydroxychloroquine: a systematic review of the literature. Drug safety, 41(10), 919-931. DISCLOSURES: no disclosure on file for Joseph Adams;no disclosure on file for Suliman Alradawi;No relevant relationships by George Kalapurakal No relevant relationships by Mohammed Siddiqui

Development of a Comprehensive, Integrated Patient Data Repository of US Pulmonary Arterial Hypertension (PAH) Patients to Assess the Impact of Clinical Care on Patient Outcomes

RATIONALE: Advances in PAH management and well-established treatment guidelines have improved the prognosis for patients. However, the extent to which guidelines are implemented in real-world practice and the relationship between guidelines and real-world patient outcomes remain in question. To assess real-world treatment and outcomes, a new type of comprehensive, integrated patient data repository (CIPDR) was created. Here, we describe the process to create this repository to enable interpretation of the collected data. METHODS: The TRIO CIPDR was created with guidance from six pulmonologists who have experience in design of and/or participation in PH registries (e.g. REVEAL). The CIPDR includes data elements of demographics, disease, comorbidities, laboratory data, pulmonary function testing, functional status, PAH treatment, reasons for treatment discontinuation/switch, hospitalizations, and death, which are collected through HIPAA-secure online forms. To minimize entry errors, participating sites received form training and ongoing support, and each form contained logic to identify improbable entries. All data were deidentified prior to storage in secure, redundant servers. The site engagement, data collection forms, data storage, and data output processes were all designed to allow both retrospective and prospective data collection and for near-immediate repository expansion through addition of other PAH-treating centers. Eleven Pulmonary Hypertension Association-certified care centers initially contributed to the CIPDR though two centers were unable to continue participation due to COVID19 impact. Central IRB approval was obtained though many sites independently received approval for the repository protocol by their IRBs. To facilitate enrollment, specialty pharmacy data corresponding to each site were used to identify potential patients and pre-populate qualification forms. Each site reviewed and qualified patients who met repository criteria: age >18 years, prescribed PAH-specific medications, and confirmation of PAH diagnosis by right heart catheterization (mean Pulmonary Arterial Pressure ≥25mmHg, Pulmonary Capillary Wedge Pressure ≤15 mmHg, and Pulmonary Vascular Resistance ≥3.0 Wood Units at rest). The initial data collection included care encounters between Jan 2019 and Dec 2020 and data concerning diagnosis, onset of symptoms, procedures, and laboratory values closest to enrollment. After completion of data collection, all data were reviewed by Trio Health and adjudicated with each site. RESULTS: Of 3200 patients identified as potentially qualified, 1009 were initially enrolled and their retrospective data encompassing 4489 visits collected. Descriptive measures of the repository are presented in the TABLE. CONCLUSION: The Trio CIPDR is an important step forward to uniquely characterize the patient journey ,treatment patterns, and outcomes for patients with PAH.

Prolonged ECMO Support of an Adult with SARS-CoV-2 ARDS and Right Heart Failure in a Children's Hospital

ECMO has become a widely recognized support modality for patients with severe cardiac or respiratory failure, and has been increasingly utilized in the ongoing severe acute respiratory syndrome due to coronavirus-2 (SARS-CoV-2) pandemic. Long-term support on ECMO for acute respiratory distress syndrome (ARDS) is also becoming more commonplace with eventual lung recovery, obviating the need for lung transplantation. However, long-term ECMO support has not been well studied for SARS-CoV-2 ARDS patients. We report the case of a 39-year-old female with severe SARS-CoV-2-induced ARDS successfully supported on venovenous ECMO (V-V ECMO) for 5,208 hours (217 days) in a high ECMO-volume, quaternary care children's hospital in 2021. At the time of this writing, this is the longest reported patient successfully supported on ECMO for SARS-CoV-2 ARDS. Our patient was initially cannulated at our children's hospital with dual-site V-V ECMO, via the right internal jugular vein (RIJ) and right common femoral vein. Bedside tracheostomy was performed on ECMO day 40, for early mobility, oral feeding, interaction, and pulmonary rehabilitation planning. Unfortunately, during her course she suffered multiple complications including bacterial co-infections, bleeding requiring anticoagulant changes from unfractionated heparin (UFH) to bivalirudin, multiple ECMO circuit changes due to blood product consumption and coagulopathy, and pneumothoraces requiring thoracostomy tube placements. Approximately 1.5 months into her ECMO course, she suffered acute hypoxemia and echocardiography revealed indirect evidence of pulmonary hypertension with right heart failure. Initial right heart catheterization while on V-V ECMO revealed elevated right ventricular end-diastolic pressure (RVEDP=15-20 mmHg) and severe systemic desaturation with main pulmonary artery (MPA) pressure of 30 mmHg. Pulmonary hypertension and right heart support was initiated in the form of inhaled nitric oxide (iNO), inotropes, phosphodiesterase inhibitors, nitrates, angiotensin-converting enzyme inhibitors, and diuresis. Ultimately, due to necessity of right-heart decompression and support, on ECMO day 86 she was transitioned to single-site V-V ECMO utilizing a 31 Fr dual-lumen venovenous cannula (ProtekDuo (LivaNova, UK)) placed via her RIJ through her right atrium (RA) into the MPA in the cardiac catheterization laboratory. Subsequent heart catheterization more than 2 months later revealed severe right ventricular (RV) diastolic dysfunction (RVEDP=35 mmHg) and moderate left ventricular (LV) diastolic dysfunction (pulmonary capillary wedge pressure (PCWP=24 mmHg)). During her course, multiple trials off ECMO were attempted with varying lengths of time off ECMO support, but ultimately required ongoing ECMO support. She developed evidence of end-organ dysfunction from her cor pulmonale, including oliguric renal failure requiring renal replacement therapy (RRT), hepatic injury with elevated transaminases and hyperammonemia leading to depressed mental state, feeding intolerance, and coagulopathy. Additionally, due to long-term nasogastric enteral tube placement for caloric supplementation and enteral medication administration, she developed concerns for invasive sinusitis with erosion into ethmoid and maxillary sinuses. As she was an adult patient being cared for in a free-standing academic children's hospital, multiple adult medicine consultants were involved in her care. Specifically, adult nephrology, cardiology, cardiothoracic surgery (for ProtekDuo cannula placement), and gastroenterology/ hepatology were integral into her care, along with our pediatric critical care medicine and ECMO teams. Notably, this was the first patient supported on ECMO to receive tracheostomy, RA-MPA dual-lumen VV cannula, and full autonomous mobility outside of the ICU at our well-established ECMO program, which has served as the index patient leading to future advances in the care of our ECMO patients. Over time and with multiple therapies to alleviate her cor pulmonale, the patient's echocardiograms evealed improved, half-systemic right-sided cardiac pressures. She was ultimately decannulated from ECMO at our center before being transferred back to the referring adult facility, and discharged to home 8 months after her initial presentation with acute respiratory failure.

TRANSESOPHAGEAL ECHOCARDIOGRAM GUIDED PULMONARY ARTERY CANNULATION FOR VENO-PULMONARY ARTERY EXTRACORPOREAL MEMBRANE OXYGENATION

Background: Pulmonary arterial (PA) cannulation for veno-pulmonary artery extracorporeal membrane oxygenation (V-Pa ECMO) is a treatment for critically ill patients in respiratory and right ventricular (RV) failure. Conventional fluoroscopic guided PA cannulation does not provide direct visualization of the catheter, PA and pulmonic valve. Complications of sub-optimal catheter placement include PA perforation and inadequate hemodynamic support. The following case study uses transesophageal echocardiogram (TEE) and right heat catheterization to simultaneously assess hemodynamics and directly visualize PA catheter placement to optimize support. Case: A 28 year old male with COVID acute respiratory distress syndrome and multisystem organ failure was placed on venovenous ECMO. He subsequently developed RV dysfunction necessitating PA cannulation for RV support via V-Pa ECMO. Right heart catheterization demonstrated an elevated central venous pressure (CVP), normal pulmonary capillary wedge pressure (PCWP) and elevated CVP/PCWP ratio consistent with RV dysfunction. A Protek Duo catheter was placed with fluoroscopic and TEE guidance. Decision-making:. Conclusion: The TEE clearly demonstrated the outflow cannula was in the main PA and proximal to the PA bifurcation. Fenestrations were observed distal to the pulmonic valve and mechanical flow was observed at the distal tip of the catheter (figure 1). Pulsatile pulmonic regurgitation without mechanical regurgitation was also observed. [Formula presented]