54 yo M with pulmonary hypertension (Clinic Series)

Case by Tyra Fainstad, MD. Edits and figures by Yilin Zhang, MD.
Expert review by Peter Leary, MD (pulmonologist, pulmonary hypertension specialist).


OBJECTIVES


CASE

A 54-year-old man is noted to have a 3/6 pan-systolic murmur best heard over the right upper sternal border on a routine annual exam. He has no past medical history and takes no medications. He undergoes an echocardiogram (TTE) and presents for follow-up of the results.

His TTE showed signs of aortic sclerosis without significant stenosis and an estimated pulmonary artery systolic pressure (PASP) of 40 mmHg and mean pulmonary arterial pressure (mPAP) of 30 mmHg. He denies palpitations, shortness of breath, orthopnea, chest pain, leg swelling or significant weight gain. His vital signs were normal and the remainder of his cardiopulmonary exam is unremarkable.

What is normal range for pulmonary pressures? 

How do estimates of pulmonary pressures from a TTE compare to those determined by right heart catheterization (RHC)? 

Does he need a RHC? 


What is your differential for causes of PH? 


CASE CONTINUED

You obtain a 6 min walk (he does not desaturate) and an EKG (normal). What is the next step? 

What therapy can you recommend to the patient while he is waiting for a Pulmonary appointment? Would you diurese him?


CASE CONTINUED

What is his prognosis? What factors are associated with a poor prognosis?

How will you counsel him about end-of-life planning?  (optional, ~5 min) 


TAKE HOME POINTS


REFERENCES

  1. McLaughlin VV, et al. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension. Journal of American College of Cardiology. 2009; 53(17): 1573 – 1619.
  2. Parasuraman S, et al. Assessment of pulmonary artery pressure by echocardiography – a comprehensive review. IJC Heart & Vasculature. 2016; 12: 45-51.
  3. Janda S, et al. Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis. Heart. 2011 Apr; 97(8): 612-22.
  4. Fisher MR, et al. Accuracy of Doppler Echocardiography in the Hemodynamic Assessment of Pulmonary Hypertension. Am J Respir Crit Care Med. 2009; 179: 615-621.
  5. Brown LM, et al. Delay in recognition of pulmonary arterial hypertension: factors identified from the REVEAL Registry. CHEST. 2011;140(1):19.
  6. Hoeper MM, et al. Am J Respir Crit Care Med. 2002.