Lectures 12 and 13 Pulmonary Arterial Hypertension Flashcards
What is the average mean pulmonary arterial pressure (mPAP)?
25mmHg
What causes increases mPAP in pulmonary HT?
shunting of systemic blood pressure
thrombus formation
sustained pulmonary vasoconstriction
pulmonary vascular remodelling
What is the consequence of high mPAP in pulmonary HT?
right ventricular hypertrophy
right heart failure
high morbidity and death (~2.8 years)
What is the most common form of pulmonary HT?
schistosomiasis
What can other conditions have pulmonary HT as a secondary condition?
HIV and autoimmune diseases
diet pills
substance abuse
What are symptoms of increased PAH?
exertional breathlessness
chest pains
How is high PH diagnosed?
ECG - right heart in particular
hypertrophy of lungs, PAs and heart
lung function test
pulmonary artery systolic pressure
How is the mean pressure calculated?
2/3 diastolic pressure + 1/3 systolic pressure
How is the pulmonary vascular resistance calculated?
(mPAP-PAWP)/cardiac output
PAWP - pulmonary artery wedge pressure
What is the clinical thresholds for mPAP, PAWP and PVR (pulmonary vascular resistance)?
mPAP - 25mmHg
PAWP - 25mmHg (proposed lowered to 20mmHg)
PVR - 2 Woods Units
What are the types of treatable pulmonary hypertension?
idiopathic PAH
PAH - pulmonary veno-occlusive disease; pulmonary capillary haemoglomatosis
chronic thromboembolic PH
What are the causes of idiopathic PAH?
heritable (genetic) drugs connective tissue disease HIV/autoimmune disease portal HT congenital heart disease schistosomiasis haemolytic anaemia
What are the types of untreatable pulmonary hypertension?
PH-lung disease/hypoxia - COPD; interstitial lung disease; alveolar hypoventilation
PH-left heart - systolic dysfunction; diastolic dysfunction; valvular disease
haematological - myeloproliferative; splenectomy
system disorders - multifactorial/unclear
metabolic disorders - multifactorial/unclear
tumour obstruction
fibrosing mediastinitis
chronic renal failure
What are type of untreatable PH system disorders?
sarcoidosis Langerhans cell histiocytosis lymphangioleiomyomatosis neurofibromatosis vasculitis
What are type of untreatable PH metabolic disorders?
glycogen storage disease
Gaucher’s disease
thyroid disorder
Give examples of diet pills that led to PAH?
fenfluramine
phentamine
What are risk factors of PAH?
more common in females