Outlines
Definition
Hemodynamic Classification
Clinical classification
Work up .
Risk stratification
Treatment.
PAH and pregnancy.
Clinical classification of pulmonary hypertension
1. PAH
2. PH due to left heart disease
3. PH due to lung disease and/or hypoxia
4. PH due to pulmonary artery obstructions
5. PH with unclear and/or multifactorial mechanisms
Updated Hemodynamic Definition of PH
Investigations
Blood workup : to identify possible causes and comorbidities.
CBC, electrolytes , renal function, liver parameters, iron studies , BNP/NT-proBNP ,ABG serological studies for hepatitis viruses and HIV, anti-nuclear antibodies, anti-centromere antibodies, and anti-Ro , markers of antiphospholipid syndrome .
Radiology : Chest x-ray , Abdominal ultrasound, Lung scintigraphy SPECT V/Q , Chest CT, cardiac MRI.
Echocardiography.
Genetic counselling and testing .
Chest x-ray
Central pulmonary arterial dilatation.
Pruning:loss of the peripheral blood vessels.
Right ventricular enlargement : decrease in the retrosternal space on the lateral image.
PVR = mean PAP- PCWP/ cardiac output
PAH Treatment Goal
 6 minutes walk distance more than 330 m
 Functional class I or II.
 Cardiopulmonary exercise testing:
 peak oxygen uptake more than 15 ml
 ventilatory equivalents for CO2 less than 45 l
 Hemodynamics: RAP less than 8 mmHg, C.I : 2.5-3 L
 Normal indexes of RV function: right atrial pressure less than 8 mm Hg and cardiac index more than 2.5 to 3.0 l/min/m2
 Normal BNP level.