PulmPEEPs

This week on Pulm PEEPs, we are continuing our Top Consults series with a discussion on the work-up and diagnosis of Pulmonary Hypertension. See our prior Radiology Rounds on signs of PAH on CT scan, and listen to our follow-up episode on right heart catheterizations for some background before this episode… or dive right in! We’ll cover everything from history and physical, to recent guideline changes in the definition of PH, and much, much more! Meet Our Guests Erika Berman Rosenzweig is a Professor of Pediatrics and the Director of the Pulmonary Hypertension Center and CTEPH Program at Columbia University Medical Center / New-York Presbyterian Hospital. She is an active member of the Pulmonary Hypertension Association, was the Editor-in-Chief of Advances in Pulmonary Hypertension and is on the Scientific Board of the World Symposium on PH. Catherine Simpson is an Assistant Professor of Medicine at Johns Hopkins Hospital and is one of the faculty members in our Pulmonary Hypertension group. Her clinical and research areas of expertise are in pulmonary vascular disease and right heart function. Her research is focused on novel biomarker discovery and metabolomics in pulmonary vascular disease. Cyrus Kholdani is an Instructor in Medicine at Beth Israel Deaconess Medical Center and Harvard Medical School. He is also the director of the Pulmonary Hypertension Program at BIDMC, and is actively involved in clinical care and clinical research in a variety of pulmonary vascular disease domains. Consult Patient Ms. Pamela Harris (PH) is a 47-year-old woman with PMH of migraines, obesity s/p gastric sleeve (BMI now 33), and a history of remote DVT in her 20s while on OCP s/p 6 months of AC who is referred to pulmonary hypertension clinic for evaluation of dyspnea on exertion. She has actually had dyspnea for some time and previously it has been attributed to her weight. Based on this, she pursued a gastric sleeve and has lost 55 pounds, but continues to have shortness of breath. She has no cough, and does not get dyspnea at rest, but notes that after 1 flight of stairs, or 2-3 blocks on flat ground she has shortness of breath. She saw her PCP and had basic labs, basic spirometry, and an echocardiogram. He did not note anything significant on examination in the notes. The labs had no anemia, and normal renal and liver function. Her serum bicarbonate was 25 and there was no blood gas. Spirometry showed an FVC 82% predicted, FEV1 83% predicted, and FEV1/FVC was 99% predicted. The echocardiogram had normal LVEF, mild LVH, normal RV size and function qualitatively. There was mild TR with tricuspid valve peak regurgitant velocity of 3.4 m/sec. The estimated PASP + RA pressure (based on normal IVC diameter 2.1 cm) was 46 mmHg. RHC: Systemic BPs 140s/90s, with O2 saturations 97-98% on RA throughout. RA mean pressure was 9, RV was 48 with an RVEDP of 17, PA was 48/27 with mean of 34, and PCWP mean was 11. CO/CI by Fick was 5.56 / 2.42, and by thermo was similar, 5.8 / 2.52. Her PA sat was 62%, and PVR was 3.97 WU. Key Learning Points History Understand the constellation of symptoms and the functional limitationThe goal is to assign a WHO functional class by the end of the visitEvaluate the time course and evolution of the symptomsConcerning symptoms that need to be addressedPalpitationsPre-syncopeSyncopeChest painLE edemaEvaluate for risk factors to explain or contribute to pulmonary hypertensionSigns or symptoms of OSASigns or symptoms of auto-immune diseaseRaynaudsSkin changesFamily historyHeritable lung diseaseClotting disordersAuto-immune diseaseSocial historyExposure historySmoking Physical Exam Look for signs that confirm PHLoud P2Accentuated with elevated PVRCan hear pretty early on. Could be one of the earliest findingsTR murmur – pansystolic murmur at RUSBDiastolic murmur if severe pulmonary insufficiencyLook for signs of right heart failureJVDS4 gallop – later in courseRV heave – later in coursePeripheral edemaPulsatile liver or hepatosplenomegalyLook for signs of other secondary causes of PHMitral regurgitation or aortic stenosis murmurAsymmetric lower extremity edemaPulmonary edemaSkin findings concerning for auto-immune disease or liver diseaseArthritis Work up for etiology of PH CBC with diff – myeloproliferative and hemolytic anemiaCMP – renal function, liver functionSerologies – lupus, scleroderma, vasculitis – broad evaluationHIV, hepatitisLiver duplex if concernedECHO with bubbleConsider cardiac MRIHistory of toxin and anorexigenic useCT scan of the chestPFTs including lung volumes and DLCO to evaluate for lung diseasePulse oximetry at rest and with exerciseA sleep study or nocturnal oximetryV/Q scan for all patients References and links for further reading Bonno EL, Viray MC, Jackson GR, Houston BA, Tedford RJ. Modern Right Heart Catheterization: Beyond Simple Hemodynamics. Advances in Pulmonary Hypertension. 2020;19(1):6-15. doi:10.21693/1933-088X-19.1.6Augustine DX, Coates-Bradshaw LD, Willis J, et al. Echocardiographic assessment of pulmonary hypertension: a guideline protocol from the British Society of Echocardiography. Echo Res Pract. 2018;5(3):G11-G24. doi:10.1530/ERP-17-0071Callan P, Clark AL. Right heart catheterisation: indications and interpretation. Heart. 2016;102(2):147-157. doi:10.1136/heartjnl-2015-307786Chokkalingam Mani B, Chaudhari SS. Right Heart Cardiac Catheterization. In: StatPearls. StatPearls Publishing; 2022. Accessed April 18, 2022. http://www.ncbi.nlm.nih.gov/books/NBK557404/D’Alto M, Dimopoulos K, Coghlan JG, Kovacs G, Rosenkranz S, Naeije R. Right Heart Catheterization for the Diagnosis of Pulmonary Hypertension: Controversies and Practical Issues. Heart Failure Clinics. 2018;14(3):467-477. doi:10.1016/j.hfc.2018.03.011Galiè N, McLaughlin VV, Rubin LJ, Simonneau G. An overview of the 6th World Symposium on Pulmonary Hypertension. European Respiratory Journal. 2019;53(1). doi:10.1183/13993003.02148-2018Rosenkranz S, Preston IR. Right heart catheterisation: best practice and pitfalls in pulmonary hypertension. European Respiratory Review. 2015;24(138):642-652. doi:10.1183/16000617.0062-2015

Experts Jessica Bon, Michael Lester, and Niru Putcha discuss severe COPD, lung volume reduction surgery, comorbidities, and advanced treatments. They explore assessing and managing COPD patients, focusing on personalized care, surgical interventions, pulmonary rehab, and innovative therapies like lung transplant evaluation and bronchial reoplasty.

We are thrilled here @PulmPEEPS to have our first episode with one of our new Associate Editors Luke Hedrick, and our first nephrology consultant Jeff William. Luke will walk us through an interesting case presentation, and we will discuss an approach to severe weakness in our patient in the ICU. Meet Our Guests Jeff William is an Assistant Professor of Medicine at Harvard Medical School and Beth Israel Deaconess Medical Center, where he is also the Associate Director of the Nephrology Fellowship Program. He completed a Medical Education Research Fellowship at Harvard Medical School, and is very involved in residency, fellowship and medical student education. Patient Presentation We have a man in his 40s with a past medical history of asthma, hypertension, and acid reflux who was brought in by EMS with back pain and profound proximal lower extremity weakness. He reports mild weakness in his legs which started 2 days ago, but this morning his weakness acutely worsened to the point that he can’t lift his legs out of the bed. He also has some cramping pain in his thighs. He additionally has had mild shortness of breath and yesterday went to an urgent care where he was given steroids and swabbed for COVID (which was negative). Key Learning Points **Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below Although our patient’s etiology of severe hypokalemia was thought to be secondary to thiazide diuretic use, it is important to be familiar with hypokalemic periodic paralysis. References Knochel JP, Schlein EM. On the mechanism of rhabdomyolysis in potassium depletion. J Clin Invest. 1972 Jul;51(7):1750-8. doi: 10.1172/JCI106976.Wang X, Han D, Li G. Electrocardiographic manifestations in severe hypokalemia. J Int Med Res. 2020 Jan;48(1):300060518811058. doi: 10.1177/0300060518811058. Venance SL, Cannon SC, Fialho D, Fontaine B, Hanna MG, Ptacek LJ, Tristani-Firouzi M, Tawil R, Griggs RC; CINCH investigators. The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain. 2006 Jan;129(Pt 1):8-17. doi: 10.1093/brain/awh639.Lin SH, Lin YF, Halperin ML. Hypokalaemia and paralysis. QJM. 2001 Mar;94(3):133-9. doi: 10.1093/qjmed/94.3.133. Lin SH, Lin YF, Chen DT, Chu P, Hsu CW, Halperin ML. Laboratory tests to determine the cause of hypokalemia and paralysis. Arch Intern Med. 2004 Jul 26;164(14):1561-6. doi: 10.1001/archinte.164.14.1561.

Experts share insights on managing pneumothorax cases, including assessment, urgent vs. emergent situations, needle decompression, chest tube insertion, post-procedure management, classification, use of 100% oxygen, avoiding non-invasive methods, varying levels of care, managing chest tubes, and strategies for secondary spontaneous pneumothorax.

We are thrilled here at Pulm PEEPs to have our first episode with our new Associate Editor Tess Litchman. Tess will walk us through an interesting case presentation of hemoptysis and we’ll use the approach from our Top Consults episode on hemoptysis to come to a key pulmonary and critical care diagnosis. Meet Our Guests Tess Litchman is a second-year internal medicine resident at Beth Israel Deaconess Medical Center. She received her undergraduate degree from Wesleyan University in Middletown, CT where she studied neuroscience and internal relations. She attended medical school at the Yale School of Medicine in New Haven, CT. She is currently completing her internal medicine residency at BIDMC. She is interested in medical education and pulmonary and critical care medicine. Patient Presentation A young man in his 20s presented to the emergency department with one week of cough and small volume hemoptysis. He has been experiencing several episodes of hemoptysis per day during this time. He says he coughs up about 1/4 cup of blood with each episode. He also adds that for the past 2 weeks he also has noticed worsening nausea, vomiting, headaches, and fatigue. He saw his primary care doctor and he was diagnosed with new hypertension and started on clonidine 0.1 mg three times a day, and provided cough medication. However, his symptoms continued. Given the increasing frequency of the hemoptysis and worsening nausea, he presented to the emergency department. Key Learning Points **Spoilers Ahead** If you want to think through the case on your own we advise listening to the episode first before looking at the infographics below References and links for further reading Radchenko C, Alraiyes AH, Shojaee S. A systematic approach to the management of massive hemoptysis. J Thorac Dis. 2017;9(Suppl 10):S1069-S1086. doi:10.21037/jtd.2017.06.41Lara AR, Schwarz MI. Diffuse Alveolar Hemorrhage. CHEST. 2010;137(5):1164-1171. doi:10.1378/chest.08-2084Gallagher H, Kwan JTC, Jayne DRW. Pulmonary renal syndrome: A 4-year, single-center experience. American Journal of Kidney Diseases. 2002;39(1):42-47. doi:10.1053/ajkd.2002.29876Sanders JSF, Rutgers A, Stegeman CA, Kallenberg CGM. Pulmonary-Renal Syndrome with a Focus on Anti-GBM Disease. Semin Respir Crit Care Med. 2011;32(3):328-334. doi:10.1055/s-0031-1279829Hudson BG, Tryggvason K, Sundaramoorthy M, Neilson EG. Alport’s syndrome, Goodpasture’s syndrome, and type IV collagen. N Engl J Med. 2003;348(25):2543-2556. doi:10.1056/NEJMra022296McAdoo SP, Pusey CD. Anti-Glomerular Basement Membrane Disease. Clin J Am Soc Nephrol. 2017;12(7):1162-1172. doi:10.2215/CJN.01380217Maxwell AP, Nelson WE, Hill CM. Reversal of renal failure in nephritis associated with antibody to glomerular basement membrane. BMJ. 1988;297(6644):333-334. doi:10.1136/bmj.297.6644.333

Dr. Brad Drummond and Dr. Allison Lambert discuss COPD exacerbations, emphasizing severity, triggers, and management strategies. They highlight the importance of patient awareness, antibiotic use, and outpatient therapy for better outcomes in COPD patients. Insights on non-invasive ventilation benefits and the need for tailored care approaches are also shared.

Today we have a special edition of Pulm PEEPs! We are revisiting our Radiology Rounds from 4 weeks ago to dive further into Right Heart Catheterizations and how to interpret them. We are joined by two experts in the field, Allison Tsao and Stephen Mathai. For a reminder, in that Radiology Rounds, we met a woman in her 50s with GERD, Raynaud’s, and multiple positive auto-antibodies (+ ANA 1:2560, + RNA pol III, + SSA, + anti-centromere) who presented with progressive dyspnea and was found to be hypoxemic. Her workup revealed severe pulmonary hypertension, and RV dysfunction on TTE with right to left shunting. Meet Our Guests Dr. Steve Mathai is an Associate Professor of Medicine at Johns Hopkins Hospital and the Director of the Inpatient Pulmonary Service. He specializes in Pulmonary Hypertension and his research focus is on scleroderma-associated PAH. Dr. Allison Tsao is an Instructor in Medicine at Harvard Medical School and is an interventional cardiologist working at the Boston VA and Brigham and Women’s Hospital. She specializes in adult congenital heart disease and is the assistant director of the Translational Discovery Lab at BWH. Key Learning Points References and links for further reading Bonno EL, Viray MC, Jackson GR, Houston BA, Tedford RJ. Modern Right Heart Catheterization: Beyond Simple Hemodynamics. Advances in Pulmonary Hypertension. 2020;19(1):6-15. doi:10.21693/1933-088X-19.1.6Callan P, Clark AL. Right heart catheterisation: indications and interpretation. Heart. 2016;102(2):147-157. doi:10.1136/heartjnl-2015-307786Chokkalingam Mani B, Chaudhari SS. Right Heart Cardiac Catheterization. In: StatPearls. StatPearls Publishing; 2022. Accessed April 18, 2022. http://www.ncbi.nlm.nih.gov/books/NBK557404/D’Alto M, Dimopoulos K, Coghlan JG, Kovacs G, Rosenkranz S, Naeije R. Right Heart Catheterization for the Diagnosis of Pulmonary Hypertension: Controversies and Practical Issues. Heart Failure Clinics. 2018;14(3):467-477. doi:10.1016/j.hfc.2018.03.011Galiè N, McLaughlin VV, Rubin LJ, Simonneau G. An overview of the 6th World Symposium on Pulmonary Hypertension. European Respiratory Journal. 2019;53(1). doi:10.1183/13993003.02148-2018Rosenkranz S, Preston IR. Right heart catheterisation: best practice and pitfalls in pulmonary hypertension. European Respiratory Review. 2015;24(138):642-652. doi:10.1183/16000617.0062-2015

Dr. Bob Wise and Dr. Wassim Labaki discuss COPD classification and management strategies. They cover GOLD 2022 guidelines, COPD diagnosis, severity assessment, biomarkers, digital monitoring, and practical management options including medications, exercise, and inhaler techniques.

This week the Pulm PEEPs, David Furfaro and Kristina Montemayor, are joined by three outstanding critical care doctors and medical educators to discuss the evaluation of patients with undifferentiated shock. We cover everything from the basics about defining shock, to advanced POCUS techniques to clarify the etiology of shock. Listen today and let us know your favorite technique for evaluating shock in the ICU. Meet Our Guests Molly Hayes is an Assistant Professor of Medicine at Beth Israel Deaconess Medical Center and Harvard Medical School, the Director of the MICU at BIDMC, and the Director of External Education at the Carl J Shapiro Institute for Education and Research. She is also a course director for a yearly CME course on principles of critical care medicine run by BIDMC and HMS. Nick Mark is a Pulmonologist and Intensivist at Swedish Medical Center in Seattle, Washington. He is also the founder of ICU One Pager, which produces high yield critical care education one-page guides that have been downloaded by thousands of learners. Matt Siuba is an Assistant Professor of Medicine and intensivist at the Cleveland Clinic, where he is the associate program director for the Critical Care Medicine fellowship. He founded and runs the website Zentensivist.com, has his own associated podcast, and is a senior editor at CriticalCareNow.com. Key Learning Points Key graphics Courtesy of Nick Mark and ICU One Pager Courtesy of Matt Siuba Courtesy of Nick Mark and ICU One Pager Definition of shock – Shock is defined as inadequate oxygen delivery to meet the body’s needs. Decreased perfusion and oxygen delivery leads to cell injury and death – If you define just as hypotension, you will miss people who have cryptic shock, and categorize some people with shock who don’t have it – Cryptic shock = a patient with normal blood pressure (MAP > 65), but who still has shock based on inadequate O2 delivery – O2 delivery is broken down in to cardiac output and arterial oxygen content Causes of shock Shock can be divided into three large categories: 1) A pump problem – low cardiac output. This includes cardiogenic and obstructive shock. Make sure to remember to look for tamponade and valvulopathies. 2) A pipe problem – low systemic vascular resistance. This includes distributive shock. Distributive shock is most often due to sepsis but can be due to anaphylaxis, endocrinopathies, cirrhosis, or spinal shock. 3) A tank problem – low preload. This includes hypovolemic and hemorrhagic shock. Make sure to remember about high intrathoracic pressure, which can decrease effective preload. Examining a patient with undifferentiated shock – See if the patient is on the “Shock BUS” by examining their brain (mental status), urine output, and skin – Feel if their skin is warm vs cold and if it is mottled – Feel the patient’s pulses to see if they are bounding, normal, or thready Point of Care Ultrasound – “Ultrasound is the new stethoscope” – The first step is to always look at the heart and look for chamber size and function. You can then look for pericardial effusion – Point of care ultrasound then includes looking at the lungs for signs of fluid overload, consolidation, or pneumothorax – A complete ultrasound also involves looking at the abdomen and at the extremities for DVT – More specific ultrasound techniques include looking at: 1) IVC exam to estimate right atrial pressure. This test is often misused. It is most helpful in states when the patient has low stroke volume and trying to figure out if they have cardiac limitation to stroke volume vs if they are hypovolemic. 2) Velocity time index as a measure of cardiac output to trend with interventions References and links for further reading Vincent JL, De Backer D. Circulatory shock. N Engl J Med. 2013;369(18):1726-1734. doi:10.1056/NEJMra1208943Seymour CW, Liu VX, Iwashyna TJ, et al. Assessment of Clinical Criteria for Sepsis: For the Third International Consensus Definitions for Sepsis and Septic Shock (Sepsis-3). JAMA. 2016;315(8):762-774. doi:10.1001/jama.2016.0288Chukwulebe SB, Gaieski DF, Bhardwaj A, Mulugeta-Gordon L, Shofer FS, Dean AJ. Early hemodynamic assessment using NICOM in patients at risk of developing Sepsis immediately after emergency department triage. Scandinavian Journal of Trauma, Resuscitation and Emergency Medicine. 2021;29(1):23. doi:10.1186/s13049-021-00833-1Hernández G, Ospina-Tascón GA, Damiani LP, et al. Effect of a Resuscitation Strategy Targeting Peripheral Perfusion Status vs Serum Lactate Levels on 28-Day Mortality Among Patients With Septic Shock: The ANDROMEDA-SHOCK Randomized Clinical Trial. JAMA. 2019;321(7):654-664. doi:10.1001/jama.2019.0071Wang J, Zhou D, Gao Y, Wu Z, Wang X, Lv C. Effect of VTILVOT variation rate on the assessment of fluid responsiveness in septic shock patients. Medicine (Baltimore). 2020;99(47):e22702. doi:10.1097/MD.0000000000022702Sweeney DA, Wiley BM. Integrated Multiorgan Bedside Ultrasound for the Diagnosis and Management of Sepsis and Septic Shock. Semin Respir Crit Care Med. 2021;42(5):641-649. doi:10.1055/s-0041-1733896Yuan S, He H, Long Y. Interpretation of venous-to-arterial carbon dioxide difference in the resuscitation of septic shock patients. J Thorac Dis. 2019;11(Suppl 11):S1538-S1543. doi:10.21037/jtd.2019.02.79Volpicelli G, Lamorte A, Tullio M, et al. Point-of-care multiorgan ultrasonography for the evaluation of undifferentiated hypotension in the emergency department. Intensive Care Med. 2013;39(7):1290-1298. doi:10.1007/s00134-013-2919-7Perera P, Mailhot T, Riley D, Mandavia D. The RUSH exam: Rapid Ultrasound in SHock in the evaluation of the critically lll. Emerg Med Clin North Am. 2010;28(1):29-56, vii. doi:10.1016/j.emc.2009.09.010

We are extremely excited to introduce our new Pulm PEEPs Meet the Patients series. Teaching and learning medicine is an incredible journey, and the goal is always to be improving patient care. Patients are our best teachers about the diseases we encounter, so the goal of this series is to spend more time with patients with pulmonary disease and with those who have been critically ill. For our first episode, we are thrilled to be joined by Katie Fielding. Katie s an educator and spent 13 years teaching high school science. She now specializes in integrating technology into the classroom to enhance education. Katie was diagnosed with CF as an infant and has spent years as a patient advocate. She works closely with the Cystic Fibrosis Foundation and serves on the Adult Advocacy Council. Katie gives us an incredible perspective about what it is like to live with Cystic Fibrosis, how her life has changed with modern therapies, and how to be the best provider possible.