Pulmonary Hypertension 

In morning report today we discussed the case of an elderly patient who presents with dyspnea. Specific points include an echocardiogram that showed a normal LV, with no significant diastolic dysfunction, but reported a very elevated RVSP (over 100). 

Given the absence of obvious left sided heart disease as the cause and suggestions of high right sided pressures, the diagnosis of pulmonary hypertension was raised. 

Approach to pulmonary hypertension

An anatomical approach is useful and from this come the 5 major categories of disease.

1. PAH (pulmonary  artery hypertension)

-idiopathic, hereditary ((bmpr2, alk (alk mutations, not the ones that cause cancer, can lead to PAH, and HHT))

-secondary to CTD (scleroderma more often CREST, anticentromere…whereas anti-scl70 is systemic, causes ILD), HIV, 

-hemoglobinopathies (ie sickle cell, anything with hemolysis leads to binding of NO that prevents vasodilation), drugs (amphetamines, cocaine, fen-phen)

1 prime: PVOD (pulmonary veno-occlusive disease)

PVOD vs PAH: one defining feature is that if PVOD receives PAH treatment, they will get significantly worse.

2. Cardiac: CHF, LV dysfunction, AS, MR, diastolic

3. Lung disease: Chronic hypoxemia eg COPD, OSA, hypoventilation syndrome or parenchymal lung disease eg ILD

4. CTEPH: treated with endarterectomy (surgery)

5. Miscellaneous: sarcoid, langerhan’s, splenectomy, mpd, glycogen storage diseases

Workup

History, physical 

-looking for consequences of and causes of pulmonary hypertension:

O/E

-Elevated JVP, CV, loud P2, palpable P2, RV heave, SOA, pulsatile liver.

PFT’s (also 6 minute walk test for prognostication, not diagnostic)

- looking for lung disease

- normal pft but isolated dlco would be hint to IPAH

ECHO

-Normal RVSP is 35

-RV dilatation, RV hypokinesis, pericard effusion

V/Q scan

-looking for CTEPH

-CT is good for acute, but not chronic. If V/Q positive, CT normal, then think of CTEPH

High res CT with contrast

-for parenchymal lung disease. Contrast is to help look for PE’s

Sleep study

BW: 

-CTD, HIV, Hgb electrophoresis etc.

Right heart catheterization

-mPAP > 25 with PCWP < 15

-Trans pulm grad is mPAP – PCWP  If > 12 then abnormal

Treatment considerations include:

- Oxygen 

- Diuretics 

- Anticoagulation (controversial)

Condition specific treatments include: 

For CTEPH: endarterectomy, Riociguat (recent NEJM link is here)

PAH: endothelin antagonist such as bosentan, vasodilators such as PDE5 inhibitors, IV epoprostenol

Lastly is transplant.

For more check out this BMJ review article on pulmonary hypertension, also where the above figure comes from.

A rash diagnosis

Morning report discussed a case of a patient who presented with non specific symptoms such as nausea, vomiting, subjective fevers, and back pain.

Based on this, proposed diagnoses based on symptom/syndrome recognition included pyelonephritis, vertebral osteomyelitis, epidural abscess. 

If the patient was delirious, how would your consideration change?
Perhaps age factors into this and would be appropriate if this were a patient who was elderly with vascular injury in the past. 

Delirium might invoke CNS infections, or may be secondary to the primary process. 

Physical examination revealed a vesicular rash, in a dermatomal distribution. 

These lesions were de-roofed and sent for analysis, which came back as VZV.

VZV:

Primary infection with VZV = Chicken Pox. Latency develops in dorsal nerve roots.

Reactivation then occurs: 

-Can be with rash = shingles, dermatomal distribution

-Can be without rash = zoster sine herpete

-Reactivation can present with or without visceral involvement eg. pancreatitis, hepatitis

Age is the biggest risk factor on a population basis for reactivation.

Neurologic complications of VZV include:

-         Zoster opthalmicus

-         Bell’s Palsy

-         Ramsay Hunt

-         Immunocompetent: can develop

o       Transverse myelitis

o       Granulomatous angiitis – can present as stroke

-         Immunocompromised:

o       Transverse myelitis

o       Small vessel vasculopathy – present as encephalitis

-         POST HERPETIC NEURALGIA

o       Post herpetic neuralgia defined as pain > 90 days after onset of rash. Age again  is RF.

Zoster associated pain comprises phase of acute neuritis and post herpetic neuralagia

 Antivirals

-         Acyclovir, Famcyclovir, Valcyclovir. (FCV,VCV may be considered over ACV because of activity, dosing)

-         Decrease rash by 0.5 day, decrease fever by 0.5 day, decrease acute neuritis phase

-         Treatment should be within 72 hours of rash. Exceptions include zoster opthalmicus, immunocompromised, one could argue for Bell's Palsy.

Post Herpetic Neuralgia treatment

-         gabapentin, TCA’s...no clear direction over which to choose, which is better. Acetaminophen, NSAIDs may help.

Other points in management include getting an ophtho assessment if V1 is involved. The role of steroids is controversial.

Zostavax

-         Indicated for greater than 60 yo

-         50% reduction in shingles

-         67% reduction in zoster associated pain

-         Similar results greater than 50 yo

-         Cannot give if on immunosupression. Should wait at least 6 months

-         Not clear when to give after acute zoster infection…

For more details see this NEJM review article on Zoster

...and this NEJM article on neurologic complications of Zoster