Ellen Kantor, SRNAUniversity of Pennsylvania

N746- Summer 2013

Pulmonary Hypertension

Let’s Review…From RVpulmonic (semilunar

valve)pulmonary arteryFrom pulmonary veinLALVsystemic

circulationPulmonary vessels

innervated by sympathetic nervous system

Alpha adrenergic- norepinephrine causes vasoconstriction

Beta adrenergic- such as isoproterenol causes vasodilation

Regulation of flow is mostly passive and controlled by OXYGEN.

Pulmonary Hypertension: Statistics

Average age at diagnosis: 36 years Women > Men10-15 million people/million diagnosed each year3 year survival at time of diagnosis: approximately

50%

Pulmonary HypertensionNORMAL PRESSURES:

Systolic: 15-30 mmHgDiastolic: 5-15 mmHgMean: 10-20 mmHg

HYPERTENSION:Defined as a sustained pulmonary artery

pressure of 40/20 mmHg and a mean pulmonary artery pressure of 25 mmHg.

Pulmonary HypertensionWorld Health Organization (WHO) classifies

into 5 groupsI: idiopathic/primary pulmonary arterial hypertension

II: pulmonary venous hypertension

III: lung disease or hypoxemia

IV: chronic thromboembolic disease

V: miscellaneous pulmonary vasculature diseases

What went wrong?

Patients have endothelial dysfunction where there is a decrease in synthesis of vasodilators such as NO and prostacyclin but an increase in thromboxane and vascular endothelial growth factor (vasoconstrictors).

Side note…Inhaled NO increased systemic O2 in

hypoxemic newborns with pulmonary hypertension.

Decreased need for ECMO

Presenting Signs and SymptomsSymptoms are usually nonspecific

DyspneaAnginaNonproductive coughClubbing

Ring a bell??

Rarely, patients present with syncope and hemoptysis, indicating severe disease.

Signs and Symptoms

While providers are struggling to diagnose a cause of the patient’s nonspecific symptoms, patients can experience acute decompensation from hypoxia, hypercarbia, vasocontrictors, increased sympathetic tone (ANXIETY), and increased pulmonary vascular resistance (PVR) all leading to right heart failure.

Systemic effects• Any abrupt

increase in PVR can lead to right heart failure or desaturation, further leading to decreased blood flow and oxygenation of the left heart, causing decreased cardiac output and ischemia.

Treatment OptionsSupplemental O2 at homeMedication management: diuretics,

anticoagulants, calcium channel blockers, phosphodiesterase inhibitors, prostanoids, endothelin receptor antagonists

Surgical managementLung transplantHeart/lung transplantPulmonary Thromboendarterectomy

Preoperative Management2D ECHO: gold standardEKGX RAYLabs: CBC, CMP (include LFTs)Spirometry (PFTs)Conduct an extensive ROS to identify

moderate-severe disease.Consider pre-operative Iloprost inhalation

***CONTINUE ALL MEDICATIONS ON DAY OF SURGERY

Preoperative Management

Intraoperative Management***CONTINUE ALL MEDICATIONS ON DAY OF SURGERYConsider regional anesthesia, MACConsider fiberoptic intubationStandard ASA monitors +/- ABG, a-line, central line,

foley***Any airway manipulation can cause activation of

sympathetic nervous system and cause a pulmonary hypertensive crisis.

MOST IMPORTANTLY: avoid pulmonary hypertensive crisis (hypoxemia, hypercarbia, metabolic acidosis, hypothermia, airway manipulation)

Postoperative ManagementInterdisciplinary challenge

Intermittent iloprost inhalationLong half life

Continuous monitoring- O2 sats, pain, vital signsConsider continous regional analgesia to

avoid high opioid doses

Case Report #1

Case Report #2

Case Report #3

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