Chronic thromboembolic pulmonary hypertension

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Chronic thromboembolic pulmonary hypertension
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Epidemiology and Pathophysiology

• Present late in the course of the disease.
• Early natural history of the condition is not
  completely known.
• Can occur without symptoms.
• May remain asymptomatic for months or years.
• May involve recurrent thromboembolism.
• May involve in situ pulmonary-artery thrombosis.
• Vascular remodeling.
• Hypertensive pulmonary arteriopathy.
• Inadequate anticoagulation.

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Predisposing factors

• Defective fibrinolytic systems
• Presence of lupus-like anticoagulant
• Deficiency of protein C, protein S, and
  antithrombin III
• Malignancy
• Atrial septal defects
• Indwelling venous catheters

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Several lines of evidence

• Low correlation between the extent of central
  anatomical obstruction and the degree of
  pulmonary hypertension.
• Documented hemodynamic progression in the
  absence of recurrent embolic events.
• Evidence of in situ pulmonary-artery thrombosis.
• Histopathological evidence of arteriopathic
  changes in the resistance vessels

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Selected etiologic conditions giving rise 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.

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Clinical Manifestation

• Progressive exertional dyspnea
• Exercise intolerance
• Pulmonic component of the second heart sound
• Chest pain on exertion
• Presyncope
• Syncope
• Inability of a compromised right ventricle to
  meet the bodys demands for CO

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Clinical Manifestation

• Loud second heart sound,
• Tricuspid regurgitation murmurs
• Engorged liver and neck veins
• Elevated jugular pressure with a positive
  hepatojugular reflex
• The presence of peripheral oedema
• Peripheral and central cyanosis
• Prominent right ventricular impulse

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honeymoon period
The existence of a honeymoon period during
which time pulmonary hypertension is present but
the subject exhibits few symptoms, if any. It is
during this time that compensatory hypertrophy of
the right ventricle occurs in an effort to
maintain cardiac output in the presence of
increased pulmonary vascular resistance (PVR).
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The pathophysiological events in the progression
of pulmonary hypertension during this period have
not been well defined.
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Diagnostic Approach

• To establish the presence and extent of pulmonary
  hypertension.
• PASP gt 35 mmHg, PADP gt 15 mmHg
• PVR gt 300 dynes/s/cm
• To determine its cause
• To determine whether it is amenable to surgical
  correction

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Laboratory Tests

• Standard laboratory tests are nonspecific.
• Transthoracic echocardiography is usually the
  first study to suggest that a abnormality of the
  pulmonary vasculature is present.

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TEE

• Abnormal right ventricular systolic function
• Tricuspid regurgitation
• Leftward displacement of the interventricular
  septum
• Decreased left ventricular size
• Abnormal left ventricular systolic and diastolic
  function
• Right atrial and right ventricular enlargement

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Patients with PVR greater than
300/dynes/s/cm5 are good candidates for PTE
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The procedure

• Median sternotomy
• Cardiopulmonary bypass
• Periods of cardioplegia
• Hypothermia
• Circulatory arrest
• Post bypass

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

• Arrhythmias
• Haemodynamic instability
• Electrolyte imbalance
• Pericardial effusion
• Pericarditis
• Infection
• Persistence of pulmonary hypertension
• Reperfusion pulmonary oedema

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Primary Objectives

• Preserve adequate hemodynamic function
• Preserve RV function
• Maintain of adequate coronary perfusion pressure
• Avoid excessive ischemia during By pass
• Prevention of pulmonary oedema reperfusion
  injury
• Cerebral protection
• Preserve of the main functions of the organism

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Premedication

• Anxyolithyc or sedative night before PTE
• Morphine 10 mg SC 1hr before transporting the
  patient unto the operating block.

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Mortality Risk Factors

• PVR gt 1100 dynes/s/cm5
• PAP gt 50 mmHg

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Monitoring

• Pulse Oximetry
• ECG (5 leads)
• NIBP (when arrive into OR)
• IBP
• Capnography
• TEE (if severe deterioration of VD)
• PA catheter
• Thermometers

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Induction of the Anaesthesia

• Good preoxygenation
• Sufentanyl 25? bolus
• Midazolam 0.5 mg bolus
• Etomidate 0.2 0.3 mg/kg
• Pancuronium bromide 0.3 0.4mg/kg

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Maintenance

• Ventilation VT 10 ml/kg
• FR 12 c/min
• FiO2 0.6 ( air/oxygène)
• PEEP 5 mmHg ( At the and of by pass)
• Sufentanyl 7.5 ?/kg
• Midazolam 0.1mg/kg/hr

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Objectives of the Anaesthetist

• Respect hemodynamic stability (CO,CI)
• Prevention of the factor which aggravate CTEPH
  (hypoxia, acidosis, hypercania, hypothermia,
  pulmonary hyperinflation, PEEP, a adrenergetic
  stimulation, histamine liberation)
• Participation in cerebral protection

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Pharmacological agents that diminish CMRO2

• Halogen compounds anesthetics
• Pentothal
• Propofol
• BZD
• Etomidate
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Conclusion

• Long term of survival 85 in 3 years 80 in 5
  years
• Continuing anticoagulant treatment
• Relative complications
• hemorrhages lt 5
• neurologicals lt 3

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Eugene Yevstratov MD
Phone 0054111540682712 (ARG) Private
0030372236344 / 0030372231698(UKr) Fax 001 775
796 2780 (USA) Email ostlandfox_at_yahoo.de /
ostlandfox_at_medscape.com Link http//myprofile.co
s.com/eugenefox
Thanks