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
