Learn about the history behind the Glaucomflecken name, the inspiration for Glaucomflecken videos as well as the advocacy work they are doing. Make sure to also check out their new podcast, Knock, Knock, Hi!
Meet our Guests
Kristin Flannary completed her undergraduate work at Texas Tech University and obtained a graduate degree from Dartmouth after training in cognitive neuroscience and social psychology. She has worked as an advocate and educator for survivors of critical illness, and for the families and partners of survivors. She co-founded Glaucomflecken with Will and has received multiple awards for her work in resuscitation science, including the AHA 2022 Resuscitation Champion award.
Will Flannary completed his undergraduate at Texas Tech University, and his MD at Dartmouth. He completed his residency in ophthalmology at the University of Iowa, and currently is an Ophthalmologist at EyeHealth Northwest in Portland, Oregon. During his education and training he was diagnosed with testicular cancer, a recurrence, and then suffered a cardiac arrest, for which Kristin, quickly acted. After surviving all of this with tenacity and a strong sense of humor, he has transformed his hilarity into multiple successful comedic initiatives about medical training
We are extremely excited to be hosting this episode in collaboration with CardioNerds! We have known Amit and Dan for many years, and they have been huge supporters of Pulm PEEPs, so it is an honor to address a topic we’re all interested in together.
We are joined by experts in the field today to discuss acute, decompensated right ventricle failure in patients with Pulmonary Arterial Hypertension (PAH). This topic can be quite intimidating, so we hope this will serve as a valuable guide for anyone who encounters a patient like this in the ICU.
Meet Our Guests
Leonid “Leon” Mirson is an internal medicine resident at the Johns Hopkins Hospital Osler Medical Residency and an Associate Editor here at Pulm PEEPs. He was born in Ukraine and moved to Philadelphia in early childhood with his family. He received his undergraduate degree from the University of Pittsburgh where he studied biomedical engineering and received his medical degree from the University of Pittsburgh School of Medicine. His current interests include pulmonary and critical care medicine with a focus on pulmonary hypertension as well as medical education. He is a rising PCCM fellow at the University of Pennsylvania.
Bhavya Varma completed her medical school at the University of Pittsburgh, her internal medicine residency at Johns Hopkins, and is a rising Cardiology fellow at NYU. She is interested in medical education and has done work with CardioNerds during her residency.
Mardi Gomberg-Maitland is a Professor of Medicine at George Washington University. She serves as the Medical Director of the Pulmonary Hypertension Program at George Washington Hospital. She completed her medical degree at Albert Einstein College of Medicine, completed her residency at the Weill-Cornell Medical Center, and completed her fellowship in cardiovascular diseases at Mount Sinai Medical Center. Her research focus is on understanding the epidemiology of pulmonary hypertension and the development of novel therapeutics and biomarkers. Dr. Gomberg-Maitland is internationally known for her work, she has had extensive grant funding and has published over 150 articles, abstracts, reviews, and chapters.
Rachel Damico is a pulmonologist and an Associate Professor of Medicine at Johns Hopkins Hospital, where she is also the Associate Director of the physician-scientist training program. Dr. Damico received her medical degree and doctoral degree in Molecular and Cellular biology from the University of Pennsylvania. She completed her residency in the Osler Internal Medicine training program and continued on as a PCCM fellow at Johns Hopkins. She has quickly achieved an international reputation in the field of pulmonary vascular biology and both basic and translational research, as well as clinical excellence, in Pulmonary Arterial Hypertension.
Patient Presentation
A 21-year-old woman with a past medical history notable for congenital heart disease (primum ASD and sinus venosus with multiple surgeries) complicated by severe PAH on home oxygen, sildenafil, ambrisentan, and subcutaneous treprostinil is presenting with palpitations, chest pain, and syncope. She presented as a transfer from an outside ED where she arrived in an unknown tachyarrhythmia and had undergone DCCV due to tachycardia into the 200s and hypotension. On arrival at our hospital, she denied SOB but did endorse nausea, leg swelling, and poor medication adherence. Her initial vitals were notable for a BP of 80/50, HR 110, RR 25, and saturating 91% on 5L O2. On exam, she was uncomfortable appearing but mentating well. She had cool extremities with 1-2+ LE edema. Her JVP was 15cm H2O. She has an RV Heave and 2/6 systolic murmur. Her lungs were clear bilaterally. Her labs were notable for Cr 2.0, an anion gap metabolic acidosis (HCO3 = 11), elevated lactate (4.1), elevated troponin to 14, and a pro-BNP of ~5000. Her CBC was unremarkable. Her EKG demonstrated 2:1 atrial flutter at a rate of 130.
Key Learning Points
Diagnosing RV failure in patients with PH:
RV dysfunction and RV failure are two separate entities. RV dysfunction can be measured on echocardiography, but RV failure can be thought of as a clinical syndrome where there is evidence of RV dysfunction and elevated right sided filling pressures.
RV failure is a spectrum and can present with a range of manifestations from evidence of R sided volume overload and markers of organ dysfunction, all the way to frank cardiogenic shock. Most patients with RV failure are not in overt shock.
One of the first signs of impending shock in patients with RV failure is the development of new or worsening hypoxemia. Patients with decompensated RV failure approaching shock often do not present with symptoms classic for LV low flow state. Instead, hypoxia 2/2 VQ mismatching may be the first sign and they can be otherwise well appearing. Particularly because patients with PH tend to be younger, they can often appear compensated until they rapidly decompensate.
Causes of decompensation for patients with RV dysfunction and PH:
Iatrogenesis (inadvertent cessation of pulmonary vasodilators by providers, surgery if providers are not familiar with risks of anesthesia), non-adherence to pulmonary vasodilators (either due to affordability issues or other reasons), infections, arrhythmias (particularly atrial arrhythmias), and progression of underlying disease.
Patients with atrial arrhythmias (atrial flutter or atrial fibrillation) and pulmonary hypertension do not tolerate the loss of the atrial kick well as it contributes a significant amount to their RV filling and impacts their cardiac output. It is often difficult to determine if the atrial arrhythmia is a cause or effect of decompensated RV failure, but its presence is associated with a worse prognosis. Efforts should be made to re-establish normal sinus rhythm in patients with decompensated RV failure and atrial arrhythmias.
A patient’s home PH medications should never be stopped for any reason upon admission unless on the basis of recommendations by a pulmonary hypertension provider as this is often a cause of decompensation inpatient
Interpreting findings on echocardiogram:
Echo is a useful screening tool. When interpreting evidence of RV dysfunction, it is important to look at the global picture and not just one measurement.
RVSP, though commonly reported, may be of limited value when evaluating for decompensation. It’s a function of blood pressure, heart rate, and cardiac output. RVSP may even decline as shock worsens.
TAPSE is useful as a marker of RV dysfunction if it is reduced, but it is difficult to follow over time and only gives information about cardiac function around the annulus; it may be normal even when apical RV function is depressed. RV fractional area of change may be more useful for global RV function. It is important to pay attention to the RV size overall, the degree of TR, and the presence of effusion all of which are associated with RV dysfunction.
Tips regarding the interpretation of invasive hemodynamics:
Cardiac output by thermodilution is the standard way to calculate PVR. Despite the degree of TR that is typically present, it is thought to be a better representation of cardiac output than the estimated Fick calculation.
Our experts agree that routine monitoring of invasive hemodynamics for acute decompensated RV failure is likely not helpful and has significant risks. A good external volume exam or CVP off a central venous catheter + central venous saturation will likely be all you need to navigate a patient with shock secondary to RV failure. A right heart catheterization (should be only done under fluoroscopy for patients with large RVs) may be helpful if the etiology of shock is unclear.
Management of decompensated RV failure in patients with pulmonary hypertension
Managing preload is of utmost importance, perhaps the most important tenant of management of decompensated RV failure. The overwhelming majority of patients with PH and decompensation are volume overloaded, it is exceptionally rare that someone would be dry. Furthermore, the myth that the RV is “preload responsive” is only true in the setting of acute RV injury (eg. RV infarction) and not so in patients with acute on chronic RV dysfunction. It is important to optimize preload in someone in decompensated RV failure and it is safe to do this more rapidly than traditionally taught. Exact goals varied between our experts, but anywhere from 2-4L net negative per day is reasonable especially if the patient is hemodynamically tolerating the fluid removal. If the patient is not responding to diuretics, hemodialysis with ultrafiltration may be necessary to optimize the patient.
Afterload is the next tenant of management. Optimizing the following parameters will reduce the patient’s pulmonary vascular resistance and reduce afterload to the right ventricle.
— Avoiding hypoxic pulmonary vasoconstriction, liberalize the patient’s O2 goal
— Avoid permissive hypercapnia and academia in this patient population
— Do not withhold a patient’s pulmonary vasodilator until discussion with the PH team. If stopped inadvertently, restart this medication immediately. For patients with malfunctioning pumps, there is a phone number on the back that you can call for rapid troubleshooting. Sildanefil can be given IV if a patient is NPO.
— Inhaled nitric oxide can improve oxygenation and reduce afterload
— Intubation and mechanical ventilation greatly increase PVR and are poorly tolerated. Exacting care must be taken to titrate PEEP and tidal volume, and avoid intubation when possible.
— Starting a new systemic pulmonary vasodilator in decompensated RV failure may be considered under close guidance from the pulmonary hypertension team
Management of atrial arrhythmias:
As above, patients with severe pulmonary hypertension do not tolerate loss of sinus rhythm well. If they are decompensated, every effort should be made to re-establish normal sinus rhythm.
Management of RV perfusion:
Unlike the LV, the RV is perfused during BOTH systole and diastole. Maintaining effective coronary perfusion to the RV is essential in RV failure. For this reason, the systemic systolic pressure (as well as the mean arterial pressure) should be kept high enough to ensure that the RV is able to perfuse. There is no great body of evidence as to which pressor works best. Norepinephrine, vasopressin, and even phenylephrine are all reasonable choices to maintain appropriate perfusing blood pressure.
Inotropy:
Patients in shock and RV failure do not always require inotropes, but if they do it’s often a sign of a grim prognosis. Either dobutamine or milrinone is reasonable, but the negative effects of these drugs (arrhythmias, tachycardia, and systemic hypotension) may limit their uses.
Mechanical circulatory support:
Limited options are available. Balloon pumps and Impella devices have limited roles except in expert centers, and ECMO remains the standard of care. ECMO (either V-V or V-A) may have utility as a bridge to recovery if a reversible cause is identified, or a bridge to transplant if the patient is on the transplant list.
Goals of care:
The prognosis of a patient admitted to the ICU with acute on chronic decompensated RV failure is guarded, with very high mortality rates even if not in shock
It is important for the patient’s longitudinal pulmonary hypertension provider to discuss the prognosis and goals of care ahead of time but this is not always possible. If they are admitted, early discussions regarding code status and prognosis are essential. It may be helpful to bring in the patient’s longitudinal pulmonary hypertension doctor into these discussions if possible.
We’re podcasting again today from #ATS2023! Yesterday, we heard all about the conference in general, with some great recaps and previews of the remaining sessions. Today, we are coming to you in collaboration with the Critical Care Assembly to preview one of the symposiums they are hosting. This session is about Cardiac Arrest: New Science and Changing Guidelines and is happening today (the day of podcast release, 5/23/23)! If you’re interested in hearing more after the episode then please plan to attend at 2:15 PM in Room 150 A-B.
For those of you who aren’t at the conference, or couldn’t make this session, we’ll talk about some of the key points and discussion topics. We will additionally be talking to the chairs of other symposiums over the next coming months so stay tuned for some more highlights from ATS 2023.
Meet Our Guest
Ari Moskowitz is an Assistant Professor of Medicine at Montefiore Medical Center / Albert Einstein College of Medicine. He is additionally the MICU Director at Montefiore Einstein and Director of Critical Care Quality Improvement. He is an NIH-funded researcher and has conducted research in cardiac arrest care, sepsis, and ARDS.
Meet our Collaborators
The American Thoracic Society Critical Care Assembly is the largest Assembly in the American Thoracic Society. Their members include a diverse group of intensivists and care providers for both adult and pediatric critically ill patients. The primary goal of the Critical Care Assembly is to “improve the care of the critically ill through education, research, and professional development.”
We are thrilled today here at Pulm PEEPs to be coming to you live from the American Thoracic Society 2023 Annual Meeting. We are joined by three fantastic speakers, and ATS leaders to discuss the highlights and events of the conference, and to share some great learning points along the way. The episode is being released immediately after recording today, Monday 5/22/23, so if you’re at the conference now make sure to listen for some extremely timely recommendations. If you’re not here in DC, we’ve highlighted some learning points that you can take away and some wisdom on how to maximize your conference experience!
We will be coming out with another episode tomorrow highlighting one of the ATS sessions, and we will continue to review symposiums from the conference throughout the year with ATS members and organizers.
Meet The Guests
Debra Boyer is a Pediatric Pulmonologist at Nationwide Children’s Hospital and has devoted much of her career to her patients and numerous trainees. Debra has been extensively involved with ATS as a prior chair of the Education Committee, and the Assembly on Pediatrics Planning Committee, and is currently on the Board of Directors and current Co-Chair of the ATS International Conference Committee.
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. Molly has been extensively involved with ATS as well and has served on the Education Committee, currently as a vice chair, chairs the Core Curriculum for the conference, is the chair for the Subcommittee on Education in Critical Care for the Critical Care Assembly’s Programming Committee, and has been very involved in the Section of Medical Education for the last several years.
Julianna Ferreria is an Associate Professor of Pulmonary and Critical Care Medicine at the University of Sao Paulo Medical School in Brazil, and her work focuses on mechanical ventilation. She has made a significant impact on global health initiatives and serves as the Co-Director of the MECOR program in Latin America for ATS. Julianna has also been extremely involved with ATS Critical Care Assembly Program Committee and was just awarded the Inaugural Philip Hopewell Prize for her commitment to research in low and middle-income countries.
This week we are joined by one of our Associate Editors, Tess Litchman, as well as two guest experts to discuss two disease states that involve both the liver and the lung. Join us as we go through how to differentiate portopulmonary hypertension and hepatopulmonary syndrome.
Meet the Guests
Tess Litchman is a senior resident at Beth Israel Deaconess Medical Center and is one of the Associate Editors for PulmPEEPs. Tess will be continuing her training as a Pulmonary and Critical Care Medicine fellow at Brigham and Women’s Hospital next year.
Tyler Peck is an Instructor in Medicine at Beth Israel Deaconess Medical Center in the Division of Pulmonary and Critical Care Medicine. Tyler’s clinical and research interests are in pulmonary vascular disease and pulmonary hypertension.
Michael Curry is an Associate Professor of Medicine at Beth Israel Deaconess Medical Center and Section Chief of the Hepatology Department at BIDMC.
Further Readings and References
Rodríguez-Roisin R, Krowka MJ. Hepatopulmonary syndrome–a liver-induced lung vascular disorder. N Engl J Med. 2008 May 29;358(22):2378-87. doi: 10.1056/NEJMra0707185. PMID: 18509123
Krowka MJ, Fallon MB, Kawut SM, et al. International Liver Transplant Society Practice Guidelines: Diagnosis and Management of Hepatopulmonary Syndrome and Portopulmonary Hypertension. Transplantation 2016; 100:1440.
Peppas, S., Nagraj, S., Koutsias, G., Kladas, M., Archontakis-Barakakis, P., Schizas, D., Giannakoulas, G., Palaiodimos, L., & Kokkinidis, D. G. (2022). Portopulmonary Hypertension: A Review of the Current Literature. Heart, lung & circulation, 31(9), 1191–1202. https://doi.org/10.1016/j.hlc.2022.04.056
This week on Pulm PEEPs we are thrilled to share a collaboration with the American Thoracic Society Assembly on Respiratory Cell & Molecular Biology. We are joined by two expert members of the ATS RCMB Assembly who have done basic and translational research in respiratory biology and lung disease. We explore the topic of Short Telomeres and their role in lung disease. With the annual ATS Conference just around the corner, this is a great intro episode for everyone from aspiring researchers and clinical pulmonologists.
Meet The Guests
Mark Snyder is an Assistant Professor of Medicine at the University of Pittsburgh Medical Center, and a member of the Graduate Program in Microbiology and Immunology there. He does research on the role of the adaptive immune system’s role in chronic rejection after lung transplantation and has received both a Parker B Francis Foundation award and an NIH K23 grant for this work.
Jonathan Alder is an Assistant Professor of Medicine at the University of Pittsburgh. His research focuses on telomeres and their role in human health and disease. He is an accomplished researcher, was a Parker B Francis fellow, and now has an NIH RO1 studying Telomere-mediated Lung disease.
Join us as we head to Indiana University! Listen in as we discuss another great case and hear teaching points from our amazing guests.
Meet our Guests
Parth Savsani is currently an internal medicine resident at Indiana University School of Medicine. He received his undergraduate degree from the University of Wisconsin-Madison and his medical degree from the University of Illinois College of Medicine. He enjoys medical education and was selected to be the VA chief resident next year.
Maria Srour is a Pulmonary and Critical Care Fellow at Indiana University School of Medicine. She completed her internal medicine residency at Saint Louis University where she was also a chief resident, and received her medical degree from IU. She works in global health to improve care for sepsis patients in low resources settings, and is currently pursuing her MPH.
Laura Hinkle is a Indiana University die hard and has been there from her since medical school through residency and fellowship, and is now an Assistant Professor of Clinical Medicine and the Associate Program Director for the Pulmonary and Critical Care Medicine Fellowship. She will be taking over as the Program Director July 1, 2023. She is a dedicated educator and is the Key Clinical Educator for Pulmonary and Critical Care, and the Director of the Clinical Transitions Curriculum. Additionally, she is working on a Master’s Degree in Education through the University of Cincinnati.
Case Presentation
A male in her early 60s is transferred from a neighboring facility with a 1 week history of fatigue and lethargy. Three days prior to presentation he developed dyspnea and increased weakness with a near fall at home. HIs family also reported recent fevers, chills, dyspnea, and diarrhea. On his way to seek evaluation, he developed slurred speech without any other focal abnormalities.
Additional information is summarized as below:
Follow along our episode to hear the final diagnosis and key teaching points from the case!
Join us as we head to the Texas Medical Center and are joined by Dr. Bitar and Dr. Gotur from Houston Methodist Pulmonary and Critical Care Medicine.
Meet our Guests
Dr. Mohamad Bitar is a Pulmonary and Critical Care Fellow at the Houston Methodist Pulmonary and Critical Care Program in Houston, Texas. Dr. Bitar completed his medical school at Misr University for Science and Technology and his Internal Medicine residency at Good Samaritan Hospital.
Dr. Deepa Gotur is an Associate Professor of Clinical Medicine at Weill Cornell Medical College and an Adjunct Associate professor at Texas A&M. She is also the Pulmonary and Critical Care Program Director at Houston Methodist. She is a sepsis and ARDS researcher, as well as a dedicated educator.
Case Presentation
A 40s-year-old male presents with 3 months of progressive shortness of breath. He was diagnosed with COVID and given treatment with steroids, and antibiotics with no response.
He presented to the hospital 3 months later after not being able to carry out daily activities and was found to have low oxygen saturation
Admitted, initial CT Chest – showed “Non-specific patchy ground-glass bilateral pulmonary infiltrates with Coarsened interstitial markings that could be related to COVID-19/atypical infection”
References and further reading
1. Silva, C. Isabela S. MD, PhD; Müller, Nestor L. MD, PhD. Idiopathic Interstitial Pneumonias. Journal of Thoracic Imaging 24(4):p 260-273, November 2009.
2.Gruden JF, Naidich DP, Machnicki SC, Cohen SL, Girvin F, Raoof S. An Algorithmic Approach to the Interpretation of Diffuse Lung Disease on Chest CT Imaging: A Theory of Almost Everything. Chest. 2020 Mar;157(3):612-635.
3. Ryu JH, Daniels CE, Hartman TE, Yi ES. Diagnosis of interstitial lung diseases. Mayo Clin Proc. 2007 Aug;82(8):976-86.
We continue our Top Consult Series on Pleural Disease and bring you a dedicated episode on Parapneumonic effusions. We are joined by two guest experts, Dr. David Feller-Kopman and Dr. Mihir Parikh. Listen in as we discuss the spectrum of parapneumonic effusions, including simple parapneumonic effusions, complicated parapneumonic effusions, and empyema. You will hear what to look for on imaging, what tests to send with pleural drainage as well as discuss the need for surgical consultation.
Meet our Guests
Dr. Mihir Parikh is currently an Assistant Professor of Medicine and academic interventional pulmonologist at Beth Israel Deaconess Medical Center. He is a highly esteemed educator and has worked to incorporate simulation training to improve procedural training for trainees and is a master of pleural disease.
Dr. David Feller-Kopman is a Professor of Medicine and the Section Chief of Pulmonary and Critical Care Medicine at Darmouth whose clinical and research expertise span the field of interventional pulmonology. Dr. Feller-Kopman is a true master of pleural disease, and has authored more than 225 peer-reviewed manuscripts and has been a leader for both ATS and CHEST committees.
Today the PulmPEEPs are joined by two amazing educators as we start off our Top Consult series on Pleural Disease. Join us today as we go through cases to learn a systematic approach for evaluation and management of pleural effusions.
Meet our Guests
Dr. Mira John received her medical degree from Tulane University School of Medicine in New Orleans and completed internal medicine residency at Icahn School of Medicine at Mount Sinai. She is currently a second-year pulmonary and critical fellow at the University of Washington.
Dr. Ylinne Lynch completed her fellowship training at the University of Washington and is currently a Clinical Instructor at the UW. She is a great medical educator and spends her clinical time on the pulmonary consult service as well as in the ICU.
