chronic thromboembolic pulmonary hypertension. epidemiology and pathophysiology present late in the...

Chronic Chronic thromboembolic thromboembolic

pulmonary pulmonary hypertensionhypertension

Epidemiology and PathophysiologyEpidemiology and Pathophysiology• PPresent late in the course ofresent late in the course of the disease.the disease.• EEarly natural historyarly natural history of the condition is not of the condition is not

completely known.completely known.• CCan occur withoutan occur without symptomssymptoms..• MMay remainay remain asymptomatic for months or years.asymptomatic for months or years.• MMay involve recurrent thromboembolism.ay involve recurrent thromboembolism.• MMay involveay involve in situ pulmonary-artery in situ pulmonary-artery

thrombosis.thrombosis.• VVascular remodeling.ascular remodeling.• HHypertensive pulmonary arteriopathyypertensive pulmonary arteriopathy..• Inadequate anticoagulation.Inadequate anticoagulation.

Predisposing factorsPredisposing factors

• DDefective fibrinolytic systemsefective fibrinolytic systems

• PPresence of lupus-like anticoagulantresence of lupus-like anticoagulant

• DeficiencyDeficiency of of protein C, protein S, andprotein C, protein S, and antithrombin IIIantithrombin III

• MMalignancyalignancy

• AAtrial septal defectstrial septal defects

• IIndwelling venous cathetersndwelling venous catheters

SSeveral lines of evidenceeveral lines of evidence

• LLow correlation between the extent of ow correlation between the extent of central anatomicalcentral anatomical obstruction and the obstruction and the degree of pulmonary hypertensiondegree of pulmonary hypertension..

• DDocumented hemodynamic progression inocumented hemodynamic progression in the absence of recurrent embolic eventsthe absence of recurrent embolic events..

• Evidence of in situ pulmonary-artery Evidence of in situ pulmonary-artery thrombosis.thrombosis.

• Histopathological evidence of arteriopathic Histopathological evidence of arteriopathic changes in the resistance vesselschanges in the resistance vessels

Selected etiologic conditions giving Selected etiologic conditions giving rise to pulmonary hypertensionrise to pulmonary hypertension

• 1. Pulmonary Arterial hypertension

• 2. Pulmonary Venous hypertension

• 3. Pulmonary hypertension associated with disorders of the respiratory system and/or hypoxaemia

• 4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease

• 4.1. Thromboembolic obstruction of proximal pulmonary arteries.

• 4.2. Obstruction of distal pulmonary arteries

• a). Pulmonary embolism (thrombus, tumour, ova and/or parasites, foreign material)

• b). In-situ thrombosis

• c). Sickle cell disease

• 5.Pulmonary hypertension due to disorders directly affecting the pulmonary vasculature.

Clinical ManifestationClinical Manifestation

• PProgressive exertional dyspnearogressive exertional dyspnea

• EExercise intolerancexercise intolerance

• PPulmoniculmonic component of the second heart component of the second heart soundsound

• CChest pain on exertionhest pain on exertion

• PresyncopePresyncope

• SyncopeSyncope

• IInabilitynability of a compromised right ventricle to of a compromised right ventricle to meet the body’smeet the body’s demands for demands for COCO

Clinical ManifestationClinical Manifestation

• LLoud second heart sound,oud second heart sound,• TTricuspid regurgitation murmursricuspid regurgitation murmurs• EEngorged liver and neck veinsngorged liver and neck veins• EElevated jugular pressurelevated jugular pressure with a with a

positive hepatojugular reflexpositive hepatojugular reflex• TThe presence of peripheral oedemahe presence of peripheral oedema• PeripheralPeripheral and central cyanosisand central cyanosis• PProminent right ventricular impulserominent right ventricular impulse

““honeymoon period”honeymoon period”

TThe existence of a “honeymoon he existence of a “honeymoon period” during which time pulmonaryperiod” during which time pulmonary hypertension is present but the subject hypertension is present but the subject exhibits few symptoms, if any. It is exhibits few symptoms, if any. It is during thisduring this time that compensatory time that compensatory hypertrophy of the right ventricle hypertrophy of the right ventricle occurs in an effort to maintainoccurs in an effort to maintain cardiac cardiac output in the presence of increased output in the presence of increased pulmonary vascular resistance (PVR).pulmonary vascular resistance (PVR).

The pathophysiologicalThe pathophysiological events in the progression of events in the progression of pulmonary hypertensionpulmonary hypertension during this period have not during this period have not been well defined.been well defined.

Diagnostic ApproachDiagnostic Approach

• TTo establish theo establish the presence and extent of presence and extent of pulmonary hypertensionpulmonary hypertension..

• PASP PASP > 35 mmHg, PADP > 15 mmHg> 35 mmHg, PADP > 15 mmHg

• PVR > 300 PVR > 300 dynes/s/cm*dynes/s/cm*

• ToTo determine its causedetermine its cause

• TTo determine whether ito determine whether it is amenable to is amenable to surgical correctionsurgical correction

LLaboratory aboratory TTestsests

• SStandard laboratory tests are tandard laboratory tests are nonspecificnonspecific..

• Transthoracic echocardiography Transthoracic echocardiography is usually the firstis usually the first study to study to suggest that asuggest that a abnormality of the abnormality of the pulmonarypulmonary vasculature is vasculature is presentpresent.

TEETEE

•AAbnormal right ventricularbnormal right ventricular systolic systolic functionfunction•TTricuspid regurgitationricuspid regurgitation•LLeftwardeftward displacement of the displacement of the interventricular septuminterventricular septum•DDecreasedecreased left ventricular sizeleft ventricular size•AAbnormal left ventricular systolicbnormal left ventricular systolic and and diastolic diastolic functionfunction•RRight atrial and rightight atrial and right ventricular ventricular enlargementenlargement

Patients with PVR greater than Patients with PVR greater than 300/dynes/s/cm300/dynes/s/cm55

are good candidates for are good candidates for PTEPTE

The procedureThe procedure

• MedianMedian sternotomysternotomy

• CCardiopulmonary bypassardiopulmonary bypass

• PPeriods of cardioplegiaeriods of cardioplegia

• HHypothermiaypothermia

• CCirculatory arrestirculatory arrest

• Post bypassPost bypass

Surgical success depends on Surgical success depends on having a bloodless field sohaving a bloodless field so cardiopulmonary bypass with cardiopulmonary bypass with periods of circulatory arrest is periods of circulatory arrest is essential to preventessential to prevent bronchial bronchial arterial flow flooding the arterial flow flooding the surgical field.surgical field.

Probable ComplicationsProbable Complications

• ArrhythmiasArrhythmias• HHaemodynamic instabilityaemodynamic instability• EElectrolyte imbalancelectrolyte imbalance• PPericardial effusionericardial effusion• PPericarditisericarditis• IInfectionnfection• PPersistence of pulmonary hypertensionersistence of pulmonary hypertension• RReperfusioneperfusion pulmonary oedemapulmonary oedema

Primary ObjectivesPrimary Objectives• Preserve adequate hemodynamic functionPreserve adequate hemodynamic function• Preserve RV functionPreserve RV function• Maintain of adequate coronary perfusion Maintain of adequate coronary perfusion

pressurepressure• Avoid excessive ischemia during By passAvoid excessive ischemia during By pass• Prevention of pulmonary oedema reperfusion Prevention of pulmonary oedema reperfusion

injuryinjury• Cerebral protectionCerebral protection• Preserve of the main functions of the organismPreserve of the main functions of the organism

PremedicationPremedication

• Anxyolithyc or sedative night Anxyolithyc or sedative night before PTEbefore PTE

• Morphine 10 mg SC 1hr before Morphine 10 mg SC 1hr before transporting the patient unto the transporting the patient unto the operating block.operating block.

Mortality Risk FactorsMortality Risk Factors

• PVR PVR > 1100 dynes/s/cm> 1100 dynes/s/cm5 5

• PAP > 50 mmHgPAP > 50 mmHg

MonitoringMonitoring• Pulse OximetryPulse Oximetry• ECG (5 leads)ECG (5 leads)• NIBP (when arrive into NIBP (when arrive into

OR)OR)• IBPIBP• CapnographyCapnography• TEE (if severe TEE (if severe

deterioration of VD)deterioration of VD)• PA catheterPA catheter • ThermometersThermometers

Induction of the AnaesthesiaInduction of the Anaesthesia

• Good preoxygenationGood preoxygenation

• Sufentanyl 25Sufentanyl 25γγ bolus bolus

• Midazolam 0.5 mg bolusMidazolam 0.5 mg bolus

• Etomidate 0.2 – 0.3 mg/kgEtomidate 0.2 – 0.3 mg/kg

• Pancuronium bromide 0.3 – 0.4mg/kgPancuronium bromide 0.3 – 0.4mg/kg

MaintenanceMaintenance

• Ventilation VT 10 ml/kgVentilation VT 10 ml/kg

• FR 12 c/minFR 12 c/min

• FiO2 0.6 ( air/oxygène)FiO2 0.6 ( air/oxygène)

• PEEP 5 mmHg ( At the and of by pass)PEEP 5 mmHg ( At the and of by pass)

• Sufentanyl 7.5 Sufentanyl 7.5 γγ/kg/kg

• Midazolam 0.1mg/kg/hrMidazolam 0.1mg/kg/hr

Objectives of the AnaesthetistObjectives of the Anaesthetist

• Respect hemodynamic stability (CO,CI)Respect hemodynamic stability (CO,CI)

• Prevention of the factor which Prevention of the factor which aggravate CTEPH (hypoxia, acidosis, aggravate CTEPH (hypoxia, acidosis, hypercania, hypothermia, pulmonary hypercania, hypothermia, pulmonary hyperinflation, PEEP, hyperinflation, PEEP, αα adrenergetic adrenergetic stimulation, histamine liberation)stimulation, histamine liberation)

• Participation in cerebral protectionParticipation in cerebral protection

Pharmacological agents that diminish CMRO2

• Halogen compounds anestheticsHalogen compounds anesthetics• PentothalPentothal• PropofolPropofol• BZDBZD• EtomidateEtomidate

ConclusionConclusion

• Long term of survival 85% in 3 years Long term of survival 85% in 3 years 80% in 5 years80% in 5 years

• Continuing anticoagulant treatmentContinuing anticoagulant treatment

• Relative complications:Relative complications:

hemorrhages hemorrhages < 5%< 5%

neurologicals < 3%neurologicals < 3%

Eugene Yevstratov MDEugene Yevstratov MD

Phone: 0054111540682712 (ARG)Private: 0030372236344 / 0030372231698(UKr)Fax: 001 775 796 2780 (USA)Email: ostlandfox@yahoo.de / ostlandfox@medscape.com

Link: http://myprofile.cos.com/eugenefox

ThanThanksks