Today we’re back with another stop on our Fellows’ Case Files journey. We’re at Boston University and Boston Medical Center with an outstanding fellow, the program director, and an expert guest to dive into a new case. Tune in to hear about this fascinating case and learn some key pulmonary points along the way.
Meet Our Guests
Lauren Kearney is a research fellow at Boston University. She completed her internal medicine residency and chief residency at BU. She is pursuing a research career in health equity and community-based participatory research to improve outcomes for patients with lung cancer and other pulmonary conditions.
Chris Reardon is a Clinical Professor of Medicine at Boston University, where she is also the Fellowship Training Program Director for Pulmonary and Critical Care Medicine. She is additionally the Director of Respiratory Care Services at Pappas Rehab Hospital for Children.
Katie Steiling is an Assistant Professor of Medicine at Boston University and a Member of the Bioinformatics Graduate Program. She founded the Lung Nodule Clinic at Boston Medical Center and co-chairs the Lung Cancer Screening Steering Committee. She is dedicated to improving the equitable treatment of patients with and at risk for lung cancer.
Case Presentation
A 44-year-old man who is undomiciled and a current smoker presents with three days of intermittent, progressively worsening pleuritic chest pain with a cough productive of blood-tinged sputum. This is in the setting of 1-2 months of fatigue and anorexia, and 2 weeks of bilateral calf swelling.
Key Learning Points
Factors that may lead to initial hypercoagulability testing in first unprovoked PE: young age, thrombosis in multiple or unusual vascular beds, history of warfarin-induced skin necrosis, arterial thrombosis.
When lung cancer is suspected, identification of metastases to identify the highest radiologic stage and optimal biopsy site is essential.
Given that pleural fluid sensitivity is only ~46%, negative cytology should not assuage further malignancy work-up in a high-risk patient.
Black individuals in the US are at higher risk of developing and dying of lung cancer- the reasons for which are complex & multifactorial. Yet, gaps remain in the targeted identification of these patients, adequate lung cancer screening, and connection to diagnosis & treatment.
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.
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
A 40-year-old patient s/p allogeneic stem cell transplant for AML 6 months prior presents with progressive dyspnea. The exam is unrevealing and imaging is obtained.
The patient’s CT reveals mosaic attenuation. Mosiac attenuation is a pattern of scattered regions of the lung with differing densities. The abnormal portions can be those that appear white or black.
Tip: Inspiratory and expiratory films can help identify the cause!
The patient had PFTs that showed severe obstruction, and significant change from PFTs prior to the stem cell transplant. Inspiratory and expiratory CT confirmed significant areas of gas trapping. She was diagnosed with bronchiolitis obliterans secondary to chronic GVHD
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 limitation
The goal is to assign a WHO functional class by the end of the visit
Evaluate the time course and evolution of the symptoms
Concerning symptoms that need to be addressed
Palpitations
Pre-syncope
Syncope
Chest pain
LE edema
Evaluate for risk factors to explain or contribute to pulmonary hypertension
Signs or symptoms of OSA
Signs or symptoms of auto-immune disease
Raynauds
Skin changes
Family history
Heritable lung disease
Clotting disorders
Auto-immune disease
Social history
Exposure history
Smoking
Physical Exam
Look for signs that confirm PH
Loud P2
Accentuated with elevated PVR
Can hear pretty early on. Could be one of the earliest findings
TR murmur – pansystolic murmur at RUSB
Diastolic murmur if severe pulmonary insufficiency
Look for signs of right heart failure
JVD
S4 gallop – later in course
RV heave – later in course
Peripheral edema
Pulsatile liver or hepatosplenomegaly
Look for signs of other secondary causes of PH
Mitral regurgitation or aortic stenosis murmur
Asymmetric lower extremity edema
Pulmonary edema
Skin findings concerning for auto-immune disease or liver disease
Arthritis
Work up for etiology of PH
CBC with diff – myeloproliferative and hemolytic anemia
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
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.
For Radiology Rounds this week we’ll be looking at multiple imaging modalities to help solve the case. Please follow along on Twitter for our live polls and for ongoing discussion of the case.
In addition to bibasilar opacities, the CXR shows bilateral enlarged pulmonary arteries.
The patient was hypoxemic on room air and desatted into the 70s with ambulation. Given her possible connective tissue disease a CT chest was obtained.
Although no ILD was revealed on the CT scan, let’s take a look at the mediastinal windows:
There are multiple signs of pulmonary hypertension, and right ventricular dilation and strain on this CT scan. Let’s review them:
To help explain the patient’s hypoxemia, an ECHO with bubble study was ordered next
Since pulmonary hypertension can only truly be diagnosed on right heart catheterization, this was performed next and revealed severe pre- and post- (mostly pre)-capillary pulmonary hypertension
Pulm PEEPs hosts, Kristina Montemayor and David Furfaro, bring our first episode in our Top Consults series. In this series, we will bring in experts to work through the most common pulmonary and critical care consults. Whether you are the consulting physician, or a pulmonologist responding to the page, these episodes are geared to give you all the information you need to care for your patients!
Today, we are joined by Chris Kapp and Matthew Schimmel, two interventional pulmonologists, to discuss hemoptysis. Chris and Matt will help us work through two hemoptysis consults, and together we’ll provide a framework for thinking about hemoptysis, outline some key components of the evaluation, and delve into treatment options.
Key Learning Points
Hemoptysis Evaluation
Hemoptysis Management
Life-Threatening or Large Volume Hemoptysis
Stabilize the patient! Make sure the airway is protected either by the patient coughing themselves, or intubation if needed. Provide hemodynamic support with IVF, blood products, and pressors if needed. If it is known which lung has the bleeding the patient can be positioned so the lung with the bleeding is down. This protects the non-bleeding lung.
Correct any bleeding diathesis If the patient is on anti-coagulation, or has any reversible bleeding diathesis, these should be corrected immediately to reduce further bleeding.
Localize the bleed If the patient is stable, they should undergo a CTA to localize the bleeding. If they are not stable to make it to a CT scan, a bronchoscopy should be performed.
Bronchoscopic treatment In addition to clearing blood from the airway, bronchoscopy can localize the bleeding. With available expertise, bronchoscopic treatments can be performed such as ice saline, topical epinephrine, or balloon tamponade to isolate the bleed.
Definitive therapy with arteriography and embolization Patients with life-threatening hemoptysis should ultimately undergo arteriography and embolization of any bleeding vessel. If this is not possible, then surgery can be needed in some cases.
A note on diffuse hemoptysis If there is not one distinct bleeding lesion, then localizing and treating the bleed becomes more difficult. For diffuse alveolar hemorrhage, evaluation should be performed for if it is primary, and due to an immunologic cause and capillaritis, or secondary to a systemic disease and / or bleeding diathesis. These investigations will guide available treatment options. Capillaritis from an immunologic cause, such as lupus or vasculitis, can be treated with systemic glucocorticoids and an additional immunosuppressive agent such as cyclophosphamide or rituximab.
Non-life-threatening or Small Volume Hemoptysis
Monitor for clinical worsening Patient’s should be monitored, either in the in-patient or out-patient setting, for increased volume or frequency of hemoptysis and for any clinical worsening, such as desaturations or decreased ability to clear the airway.
Correct any bleeding diathesis If the patient is on anti-coagulation, or has any reversible bleeding diathesis, these should be corrected immediately to reduce further bleeding. In pattients with non-life-threateneing hemoptysis this requires careful consideration of balancing the risk of bleeding vs the benefits for continuing anti-coagulation.
Evaluate for underlying cause Patient’s should undergo imaging and evaluation for the underlying cause of the hemoptysis. This may be evidence of an underlying infection, a pulmonary embolism, or new lung lesions making the patient at risk. If the source can’t be found on non-invasive imaging, and there is no clear systemic source such as an infection, a bronchoscopy is warranted. Any underlying cause should be treated and investigated further.
Inhaled Tranexamic Acid Nebulized tranexamic acid is well tolerated and can help resolve hemopytysis without invasive procedures.
