Pulmonary Hypertension in the CICU

Plato J Alexander, MD CICU Miami Childrens Hospital

Pulmonary Hypertension (PH)

Systolic pulmonary arterial pressure (PAP) > 35 mm Hg, or A mean PAP > 25 mm Hg Can also be described as a fraction of SBP, i.e. half systemic, 2/3 systemic, systemic, or suprasystemic PA pressures

Taylor et al, PCCM 2010

Pulmonary Hypertension

Usually thought of as a disease of increased pulmonary vessel vascular tone That tone can be increased all of the time, or have episodes of severe elevation Much of the time the disease is reactive, (ie, responsive to iNO challenge), which prognosticates and helps us guide our therapy

Pulmonary Hypertension

Fixed pulmonary hypertension implies that the tone is irreversibly elevated and not remedied by therapy In the CICU usually seen after months or years of exposure to high blood pressures Host of other causes including BPD, sarcoidosis, PVOD, HIV, chronic reflux/aspiration, airway obstruction, etc

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Hagworth Arch Dis Child 2008

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Acute Pulmonary Hypertensive Crisis

1. 2.

3.
4.

Elevated PAP leads to RV pressure overload and dysfunction, causing Decreased pulmonary blood flow and increased RV pressure and RV Failure, causing Deviation of interventricular septum into LV,
causing

5.

Diminished LV function, contributing to Low cardiac output and death.

Taylor et al, PCCM 2010

PH Crisis Triggers
1. 2. 3. 4. 5.

Hypoxia Acidosis Hypercapnea Increased autonomic tone Hypothermia

Clinical signs to watch for

Elevated HR - Or bradycardia with pallor (ominous) Decreased saturations (especially in patients with cardiac shunts) Hypotension Increasing gap between EtCO2 and PaCO2 Elevations in RA, RV or PA intracardiac lines Increasing acidosis and/or lactates Or, NONE OF THE ABOVE!!!
Taylor et al, PCCM 2010

Echo: The Good

Quick, non-invasive way of checking for right-sided pressures Can estimate PA pressures if TR jet is present Can estimate RV pressure based upon septal deviation Visualizes RV function and degree of hypertrophy

Echo: The Bad

However, not always accurate Little data about RV pressure obtained if no TR jet Can be misleading Cath is considered the gold standard

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Champion Circulation 2009

PH in the CICU - 2 Groups

Patient with New Onset: Need to Avoid triggers

Group is more likely to have severe acute, life-threatening exacerbations

Patient with Chronic condition: Need to Support RV

More likely group to have RV ischemia Also likely to have arrhythmias

Patients with Chronic Condition

Frequently due to pulmonary vascular bed being exposed to:

High Pressures or High Volumes Combination of both

Usually involves some hypertrophy of the RV Depending on duration, may result in irreversibility of the disease

Principles of PAH Management

Pre-Op
Identify patients at Risk Early surgery Pre-Op Therapies

Intra-Op
Modified ultrafiltration

Post-Op
What we care about in the CICU.

Post-Op Management

Proper ventilation Proper Sedation (pre-emptive) for suctioning and painful stimulation Proper Inotropes Proper PH Medications

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Shukla Pediatr Crit Care Med 2010

Does being on the ventilator help or hurt a patient with severe pulmonary hypertension?

Ventilator

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Vent Management in PH patients

Balance between

Optimized gas exchange Maximized cardiac output Optimized systemic oxygen delivery

Adopted from Nieves, 2010

Encourage Vent Factors which PVR

Mild Hypocarbia (PaCO2 of 30-35mmHg) Mild Alkalosis (pH of 7.45- 7.50) Hypoxemia (maintain PaO2 > 100mmHg) Appropriate FRC and tidal volumes that avoid hyperinflation and atelectasis Minimize suctioning, and then only after generous premedication with sedation
Adopted from Nieves, 2010

Avoid Vent Factors which PVR

Hypercarbia Acidosis Hypoxemia Atelectasis (causing hypoxic vasoconstriction) Hyperinflation (compress intraalveolar vessels) Elevated intrathoracic pressure Agitation (eg, during suctioning) Ventilator-induced lung injury and pulmonary edema
Adopted from Nieves, 2010

Therapy: Transitioning out of CICU

Goals of Therapy

Improve hemodynamics Improve exercise capacity Improve Functional Class Improve survival Improve quality of life

Rationale for Early Treatment

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Stenmark Pediatr Crit Care Med 2010

Schultze-Nieke Europ Resp Review 2010

IV Therapy

Milrinone Epoprostinol

Epoprostinol

Gold standard for severe PAH Prostacyclin analogue Potent pulmonary vasodilator Half life of 3-5 minutes: NEVER interrupt the infusion Common side effects: jaw pain, diarrhea, infection, pulmonary edema

Epoprostinol

Requires long-term catheter Prone to infection Unstable, needs refrigeration and good only for 24 hours

Treprostinil

Prostacyclin analogue Half life of 4 hours Can be considered when stable epoprostinil dose has been determined Fewer side effects

Oral Therapy

Sildenafil Bosentan

Sildenafil

Phosphodiesterase inhibitor type 5 inhibitor Raises intracellular cGMP levels Inhibits breakdown into 5-GMP Causes vasodilation
Fraisse Pediatr Crit Care Med 2010

Sildenafil

Contraindicated in postcapillary hypertension Side effects: dizziness, tachycardia, hypotension, hearing loss, erythema, drowsiness, prolonged erection, optic ischemia, priapism Can also be given iv

Tadalafil

2cnd generation PDE Once daily dosing Used not uncommonly in teenage patients for compliance No good pediatric studies

Bosentan

Non selective endothelin antagonist Drug interactions occur Induces CYP3A4 and CYP2C9 When given with Sildenafil, requires dose-adjustment

Bosentan

Liver monitoring monthly Teratogenic - requires contraception and monthly pregnancy tests Other: headache, flushing, edema, anemia

Miscellaneous Oral Therapies

Digoxin Diuretics Anticoagulants Calcium Channel Blockers

Inhaled Therapy

iNO Oxygen Inhaled iloptrost

Limitations of iNO

Non-responders Partial responders and tachyphylaxis Severe rebound hypertension on discontinuation Availability, Complexity, Cumbersome, Cost Difficult to administer in controlled way if not intubated

Iloprost

Prostacyclin analogue 25 minute half life Delivered directly to the pulmonary vascular bed Less side effects

Iloprost

Complex delivery device Most kids unable to utilize before 7 years of age Can be given via nebulization therapy through the vent

Nursing Questions to ask

Is there a cardiac shunt or pop off? Is the patient on a muscle relaxant? What are the targeted PaO2/saturations, pH and PaCO2 levels? What is the current EtCO2-PaCO2 gradient? Is there an ET leak that interferes with ventilation? Presence of atelectasis vs hyperinflation? Do we need iNO at the bedside? When can I wean the FiO2?

Thank You!