Carolinas Case Conference - Dr. S. Pecevich

Pulmonary Hypertension

• WHO classifies 5 types of Pulmonary Hypertension:

1. Pulmonary Arterial Hypertension
2. Left Heart Failure
3. Cor Pulmonale
4. Chronic Embolic Disease
5. Miscellaneous (Sarcoid, etc)

• Generally, Class 1 is a type that will be prescribed and also potentially respond to vasodilators including prostacyclins, endothelin receptor antagonists, and other vasodilators--sildenafil, tadalafil. Other types need to have underlying cause addressed
• Withdrawing from a pulmonary hypertension drug infusion can be LIFE THREATENING. It is imperative to restart an infusion, for example epoprostenol, if there is any concern about port compromise or malfunction
• Respect Pulmonary Hypertension as these patients decompensate rapidly! 
• Respect the RV! -- pulmonary hypertension, and subsequent RV failure, is difficult to manage. Most patients are volume overloaded yet also preload dependent. If you overload them, this can cause worsened LV function (septum flattening) and subsequent decreased cardiac output. 
• Even the slightest systemic illness can lead to rapid hemodynamic compromise
• Call for help -- generally cardiologists manage these complicated cases 
• In the acute setting, consider 250cc crystalloid boluses and perhaps more importantly, use norepinephrine as your pressor. Phenylephrine & Dobutamine = Bad.

 
Hemorrhagic Shock in a Jehovah's Witness

• Elevated lactate does not necessarily mean sepsis, it can imply malperfusion
• Consider your types of shock and then frame your differential accordingly
• Elevated Cr with active bleeding? Push the contrast for the CT, fix the kidneys later
• Be wary of INR with novel anticoagulants. This is not specific or sensitive for anticoagulation status on NOACs.
• If administering K-Centra for these medications, do it ONCE. NOAC's are inhibitors, they do not disrupt clotting factor synthesis like Warfarin. Therefore, there is concern for hypercoaguable state with multiple doses of K-Centra, particularly once the NOAC wears off
• Jehovah's Witnesses have the same HIPPAA rights as any other individual. You should inform him or her of the right to privacy were he or she to accept blood products
• Not all blood products contain human factors. Have a discussion with the patient about what types of blood products he or she might be willing to accept. For instance, patients are occasionally willing to undergo autologous transfusions or receive plasma
• Generally with children, your decision as a physician to medically treat a patient, especially in emergent circumstance, supersedes the desire of a parent for his or her child not to receive blood products

        Tenants of this concept:

1. Immediate interests of child and state generally > that of the parents' wishes
2. Parents do not have right to life/death over another individual who lacks capacity
3. Parents do not hold right to refuse medical treatment for this patient

• These are ethically challenging cases. If uncomfortable, consult with another physician or risk management/ethics board, if appropriate 

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