pulmonary hypertension 1 31 2023 lecture

  • 1035531

  • usually presenting with exertional dyspnea
  • edema, JVD
  • RVH, RBBB, RV straining
  • PVOD
  • echo can look for shunt
  • PFT: isolated low DLCO

  • ESRD and CKD can also cause PHTN.
  • HHT are at high risk for PHTN
  • pulmonary capillary hemangiomatosis: can have worse outcome on vasodilators

Symptoms and signs

  • mostly exertional dyspnea, goes back months
  • low CO: weakness, fatigue, syncope
  • dilated PA can compress left main coronary: chest pain
  • 2nd heart sound should be loudest at base, but if loud at apex just as loud as first heart sound, it's abnormal

Labs

  • connective tissue panels not necessary unless they have physical findings
    • muscle weakness
    • rash
    • joints
    • skin changes
    • thyroid problems

EKG

  • presence of qR complex in V1 and RVH (tall R in V2 and S in V5)
  • p wave > 25mm in II, p pulmonone
  • ST depression, T wave inversion in inferior and anterior leads

Echo

  • RV dysfunction can impair LV filling, usually indeterminate

  • pericardial effusion poor prognosis

  • stop using RVSP as cut off

  • use TR jet as better measurement

  • Increasing suspicion based on velocity

  • TR > 3.4 m/s, confirms PHTN. RHC then only tells which type.

  • TAPSE: how RV adapting to high pressure. Lower TAPSE = higher mortality

CT

  • index PA diameter to size of aorta
  • dilated pulmonary arteries more distally
  • dilated IVC
  • regurgitated contrast
  • PA dilation on CXR

Pathophysiology

  • after PVR gets to certain point, PAP goes down
  • RAP and CO more important

Case

  • hepatic AVM: L to R shunt
  • hepatopulmonary syndrome: R to L shunt
  • high output cardiac failure causing PHTN due to AVMS with HHS.
  • ESRD patients with AV fistula could have similar problem
  • A: vasodilate can cause worsening hypotension
  • PA pressure can go up with either increased CO or PVR
  • high PCWP can actually be beneficial

Treatment

  • oral drugs not great because of GI side effects