Thanks to Dr. Grock for today’s Morning Report!
The case:
20 yo male no pmhx, presents with pleuritic chest pain, dizziness, and worsening DOE x 3 days (class IV). No fevers, no toxic habits, no travel/sx/immobilizations/hx dvt or pe.
VS: tachycardic to 110s (up to 130s with minimal exertion), RR 30s, Sat 85% on RA. Afeb
PE: Benign. CTAB. Calves equal in size , no pedal edema. Talking in short sentences
EKG sinus tach, CXR unremarkable
Diagnosis???????
Well. I thought PE. The attending wanted me to order a D-Dimer. It came back at 6000 (upper limit of nml 500).
CTA showed………Enlarged Pulmonary Artery…
Dx: Pulmonary HTN!
On further hx, pt’s mom had severe pulmonary htn starting at a young age.
Pulmonary Htn:
Usually dx by: Rt heart cath. EKG can be sinus tach or signs RV hypertrophy/strain. TTE: RV enlargement
In ED? Help them breath (oxygen, noninvasive ventilation).
From: genetic, PE, L heart dx, Lung dx (COPD/ILD), drugs, idiopathic, inflammatory disorders
Present with: SOB/DOE, Chest pain, hemoptosis, syncope
Pathophys: “Muscularization of distal, normally nonmuscular arteries at alveolar duct and wall level”. BMPR2 gene involved (70% of hereditary PAH have mutation). BMPR2 defect leads to apoptosis and overproliferation of SMC’s
Prognosis: When do Rt heart cath, the cards gives the patient NO/prostacyclin/adenosine and see if PA pressure decreases. If so, they will respond well to calcium channel blocker and prognosis is much better.
Pulmonary HTN and PE have interesting relationship. Unknown which causes which, but they frequently occur together.
Meds if not Ca channel blocker responders:
- Prostacyclin analogues: epoprostenal, trepostinil, iloprost. All improved symptoms/mortality benefit.
- Endothelin Receptor Antagonist: bosentan, ambrisentan
- Phosphodiesterase type 5 inhibitor (Viagra!) also called sildenafil.
End Game: Lung transplant.
Lastly, CTA can miss PE. Can also get V/Q scan.
Jay Khadpe MD
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