Pulmonary Hypertension in the CICU

Pulmonary HypertensionPulmonary Hypertensionin the CICUin the CICU

Plato J Alexander, MDPlato J Alexander, MD

CICUCICU

Miami Children’s HospitalMiami Children’s Hospital

Pulmonary Hypertension (PH)Pulmonary Hypertension (PH)

• Systolic pulmonary arterial pressure (PAP) Systolic pulmonary arterial pressure (PAP) > 35 mm Hg, > 35 mm Hg, oror

• A mean PAP > 25 mm HgA mean PAP > 25 mm Hg• Can also be described as a fraction of Can also be described as a fraction of

SBP, i.e. half systemic, 2/3 systemic, SBP, i.e. half systemic, 2/3 systemic, systemic, or suprasystemic PA pressures systemic, or suprasystemic PA pressures

Taylor et al, PCCM 2010

Pulmonary Hypertension Pulmonary Hypertension

• Usually thought of as a disease of Usually thought of as a disease of increased pulmonary vessel vascular toneincreased pulmonary vessel vascular tone

• That tone can be increased all of the time, That tone can be increased all of the time, or have episodes of severe elevationor have episodes of severe elevation

• Much of the time the disease is “reactive”, Much of the time the disease is “reactive”, (ie, responsive to iNO challenge), which (ie, responsive to iNO challenge), which prognosticates and helps us guide our prognosticates and helps us guide our therapytherapy

Pulmonary HypertensionPulmonary Hypertension

• ““Fixed” pulmonary hypertension implies Fixed” pulmonary hypertension implies that the tone is irreversibly elevated and that the tone is irreversibly elevated and not remedied by therapynot remedied by therapy

• In the CICU usually seen after months or In the CICU usually seen after months or years of exposure to high blood pressuresyears of exposure to high blood pressures

• Host of other causes including BPD, Host of other causes including BPD, sarcoidosis, PVOD, HIV, chronic sarcoidosis, PVOD, HIV, chronic reflux/aspiration, airway obstruction, etcreflux/aspiration, airway obstruction, etc

QuickTime™ and a decompressor

are needed to see this picture.

Hagworth Arch Dis Child 2008

Acute Pulmonary Hypertensive Acute Pulmonary Hypertensive CrisisCrisis

1.1. Elevated PAP leads to RV pressure overload Elevated PAP leads to RV pressure overload and dysfunction, and dysfunction, causingcausing

2.2. Decreased pulmonary blood flow and Decreased pulmonary blood flow and increased RV pressure and RV Failure, increased RV pressure and RV Failure, causingcausing

3.3. Deviation of interventricular septum into LV, Deviation of interventricular septum into LV, causingcausing

4.4. Diminished LV function, Diminished LV function, contributing tocontributing to

5.5. Low cardiac output and death. Low cardiac output and death.


PH Crisis TriggersPH Crisis Triggers

1.1. HypoxiaHypoxia

2.2. AcidosisAcidosis

3.3. HypercapneaHypercapnea

4.4. Increased autonomic toneIncreased autonomic tone

5.5. HypothermiaHypothermia

Clinical signs to watch forClinical signs to watch for

• Elevated HRElevated HR• - Or - Or bradycardia with pallor (ominous)bradycardia with pallor (ominous)• Decreased saturations (especially in patients Decreased saturations (especially in patients

with cardiac shunts)with cardiac shunts)• HypotensionHypotension• Increasing gap between EtCO2 and PaCO2Increasing gap between EtCO2 and PaCO2• Elevations in RA, RV or PA intracardiac linesElevations in RA, RV or PA intracardiac lines• Increasing acidosis and/or lactatesIncreasing acidosis and/or lactates• Or, Or, NONE OF THE ABOVENONE OF THE ABOVE!!!!!!


Echo: The Good…Echo: The Good…

Quick, non-invasive way of checking for Quick, non-invasive way of checking for “right-sided” pressures“right-sided” pressures

Can estimate PA pressures if TR jet is Can estimate PA pressures if TR jet is presentpresent

Can estimate RV pressure based upon Can estimate RV pressure based upon septal deviationseptal deviation

Visualizes RV function and degree of Visualizes RV function and degree of hypertrophyhypertrophy

Echo: The BadEcho: The Bad

However, not always accurateHowever, not always accurate Little data about RV pressure obtained if Little data about RV pressure obtained if

no TR jetno TR jet Can be misleadingCan be misleading Cath is considered the gold standardCath is considered the gold standard



Champion Circulation 2009

PH in the CICU - 2 GroupsPH in the CICU - 2 Groups

• Patient with New Onset: Need to Avoid Patient with New Onset: Need to Avoid triggerstriggers• Group is more likely to have severe Group is more likely to have severe

acute, life-threatening exacerbationsacute, life-threatening exacerbations

• Patient with Chronic condition: Need to Patient with Chronic condition: Need to Support RVSupport RV• More likely group to have RV ischemiaMore likely group to have RV ischemia• Also likely to have arrhythmiasAlso likely to have arrhythmias

Patients with Chronic ConditionPatients with Chronic Condition

• Frequently due to pulmonary vascular Frequently due to pulmonary vascular bed being exposed to:bed being exposed to:• High Pressures orHigh Pressures or• High VolumesHigh Volumes• Combination of bothCombination of both

• Usually involves some hypertrophy of Usually involves some hypertrophy of the RVthe RV

• Depending on duration, may result in Depending on duration, may result in irreversibility of the diseaseirreversibility of the disease

Principles of PAH ManagementPrinciples of PAH Management

• Pre-OpPre-Op• Identify patients at RiskIdentify patients at Risk• Early surgeryEarly surgery• Pre-Op TherapiesPre-Op Therapies

• Intra-OpIntra-Op• Modified ultrafiltrationModified ultrafiltration

• Post-OpPost-Op• What we care about in the CICU….What we care about in the CICU….

Post-Op ManagementPost-Op Management

• Proper ventilationProper ventilation• Proper Sedation (pre-emptive) for Proper Sedation (pre-emptive) for

suctioning and painful stimulationsuctioning and painful stimulation• Proper InotropesProper Inotropes• Proper PH MedicationsProper PH Medications



Shukla Pediatr Crit Care Med 2010

Does being on the ventilator Does being on the ventilator help or hurt a patient with help or hurt a patient with

severe pulmonary severe pulmonary hypertension?hypertension?

VentilatorVentilator



Vent Management in PH patientsVent Management in PH patients

• Balance betweenBalance between• Optimized gas exchangeOptimized gas exchange• Maximized cardiac outputMaximized cardiac output• Optimized systemic oxygen deliveryOptimized systemic oxygen delivery

Adopted from Nieves, 2010

Encourage Vent Factors which Encourage Vent Factors which PVRPVR

• Mild Hypocarbia (PaCO2 of 30-35mmHg)Mild Hypocarbia (PaCO2 of 30-35mmHg)• Mild Alkalosis (pH of 7.45- 7.50)Mild Alkalosis (pH of 7.45- 7.50)• Hypoxemia (maintain PaO2 > 100mmHg)Hypoxemia (maintain PaO2 > 100mmHg)• Appropriate FRC and tidal volumes that Appropriate FRC and tidal volumes that

avoid hyperinflation and atelectasisavoid hyperinflation and atelectasis• Minimize suctioning, and then only after Minimize suctioning, and then only after

generous premedication with sedationgenerous premedication with sedation


Avoid Vent Factors which Avoid Vent Factors which PVRPVR

• HypercarbiaHypercarbia• AcidosisAcidosis• HypoxemiaHypoxemia• Atelectasis (causing hypoxic vasoconstriction)Atelectasis (causing hypoxic vasoconstriction)• Hyperinflation (compress intraalveolar vessels)Hyperinflation (compress intraalveolar vessels)• Elevated intrathoracic pressureElevated intrathoracic pressure• Agitation (eg, during suctioning)Agitation (eg, during suctioning)• Ventilator-induced lung injury and pulmonary Ventilator-induced lung injury and pulmonary

edemaedema


Therapy: Transitioning out of CICUTherapy: Transitioning out of CICU

Goals of TherapyGoals of Therapy

• Improve hemodynamicsImprove hemodynamics• Improve exercise capacityImprove exercise capacity• Improve Functional ClassImprove Functional Class• Improve survivalImprove survival• Improve quality of lifeImprove quality of life

Rationale for Early TreatmentRationale for Early Treatment



Stenmark Pediatr Crit Care Med 2010

Schultze-Nieke Europ Resp Review 2010

IV TherapyIV Therapy

• MilrinoneMilrinone• EpoprostinolEpoprostinol

EpoprostinolEpoprostinol

• Gold standard for severe PAHGold standard for severe PAH• Prostacyclin analogueProstacyclin analogue• Potent pulmonary vasodilatorPotent pulmonary vasodilator• Half life of 3-5 minutes: NEVER Half life of 3-5 minutes: NEVER

interrupt the infusioninterrupt the infusion• Common side effects: jaw pain, Common side effects: jaw pain,

diarrhea, infection, pulmonary edemadiarrhea, infection, pulmonary edema

EpoprostinolEpoprostinol

• Requires long-term catheterRequires long-term catheter• Prone to infectionProne to infection• Unstable, needs refrigeration and Unstable, needs refrigeration and

good only for 24 hoursgood only for 24 hours

TreprostinilTreprostinil

• Prostacyclin analogueProstacyclin analogue• Half life of 4 hoursHalf life of 4 hours• Can be considered when stable Can be considered when stable

epoprostinil dose has been epoprostinil dose has been determineddetermined

• Fewer side effectsFewer side effects

Oral TherapyOral Therapy

• SildenafilSildenafil• BosentanBosentan

SildenafilSildenafil

• Phosphodiesterase inhibitor type Phosphodiesterase inhibitor type 5 inhibitor5 inhibitor

• Raises intracellular cGMP levelsRaises intracellular cGMP levels• Inhibits breakdown into 5’-GMPInhibits breakdown into 5’-GMP• Causes vasodilationCauses vasodilation

Fraisse Pediatr Crit Care Med 2010

SildenafilSildenafil

• Contraindicated in postcapillary Contraindicated in postcapillary hypertensionhypertension

• Side effects: dizziness, tachycardia, Side effects: dizziness, tachycardia, hypotension, hearing loss, erythema, hypotension, hearing loss, erythema, drowsiness, prolonged erection, optic drowsiness, prolonged erection, optic ischemia, priapismischemia, priapism

• Can also be given ivCan also be given iv

TadalafilTadalafil

• 2cnd generation PDE2cnd generation PDE• Once daily dosingOnce daily dosing• Used not uncommonly in teenage Used not uncommonly in teenage

patients for compliancepatients for compliance• No good pediatric studiesNo good pediatric studies

BosentanBosentan

• Non selective endothelin Non selective endothelin antagonistantagonist

• Drug interactions occurDrug interactions occur• Induces CYP3A4 and CYP2C9Induces CYP3A4 and CYP2C9• When given with Sildenafil, When given with Sildenafil,

requires dose-adjustmentrequires dose-adjustment

BosentanBosentan

• Liver monitoring monthlyLiver monitoring monthly• Teratogenic - requires Teratogenic - requires

contraception and monthly contraception and monthly pregnancy testspregnancy tests

• Other: headache, flushing, Other: headache, flushing, edema, anemiaedema, anemia

Miscellaneous Oral TherapiesMiscellaneous Oral Therapies

• DigoxinDigoxin• DiureticsDiuretics• AnticoagulantsAnticoagulants• Calcium Channel BlockersCalcium Channel Blockers

Inhaled TherapyInhaled Therapy

• iNOiNO• OxygenOxygen• Inhaled iloptrostInhaled iloptrost

Limitations of iNOLimitations of iNO

• Non-respondersNon-responders• Partial responders and tachyphylaxisPartial responders and tachyphylaxis• Severe rebound hypertension on Severe rebound hypertension on

discontinuationdiscontinuation• Availability, Complexity, Cumbersome, Availability, Complexity, Cumbersome,

CostCost• Difficult to administer in controlled way if Difficult to administer in controlled way if

not intubatednot intubated

IloprostIloprost

• Prostacyclin analogueProstacyclin analogue• 25 minute half life25 minute half life• Delivered directly to the Delivered directly to the

pulmonary vascular bedpulmonary vascular bed• Less side effectsLess side effects

IloprostIloprost

• Complex delivery deviceComplex delivery device• Most kids unable to utilize before 7 Most kids unable to utilize before 7

years of ageyears of age• Can be given via nebulization therapy Can be given via nebulization therapy

through the ventthrough the vent

Nursing Questions to askNursing Questions to ask

• Is there a cardiac shunt or “pop off”?Is there a cardiac shunt or “pop off”?• Is the patient on a muscle relaxant?Is the patient on a muscle relaxant?• What are the targeted PaO2/saturations, pH and What are the targeted PaO2/saturations, pH and

PaCO2 levels?PaCO2 levels?• What is the current EtCO2-PaCO2 gradient?What is the current EtCO2-PaCO2 gradient?• Is there an ET leak that interferes with Is there an ET leak that interferes with

ventilation?ventilation?• Presence of atelectasis vs hyperinflation?Presence of atelectasis vs hyperinflation?• Do we need iNO at the bedside?Do we need iNO at the bedside?• When can I wean the FiO2?When can I wean the FiO2?

Thank You!Thank You!

Pulmonary Hypertension in the CICU

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