Pulmonary

Hypertension

Introduction
Pulmonary hypertension (PH) is an increase in mean pulmonary arterial pressure (mPAP) 25 mmHg at
rest as assessed by right heart catheterization.
Normal mPAP (at rest) -> 14+3 mmHg, with an upper limit of normal of 20 mmHg.
mPAP 21-24 mmHg is unclear, need further evaluation in epidemiological studies.
PH is a haemodynamic and pathophysiological state with multiple clinical conditions.
These have been classified into six clinical groups with specific characteristics.

Pathobiology of Pulmonary
Hypertension
Grup 1 (Pulmonary arterial hypertension)
Still unknown even if it is recognized
A multifactorial pathobiology that involves various
biochemical pathways and cell types.
The increase in pulmonary vascular resistance (PVR) is
related to vasoconstriction, proliferative and obstructive
remodelling of the pulmonary vessel wall, inflammation,
and thrombosis.

Pathobiology of Pulmonary
Hypertension
Grup 2 (PH due to left heart disease)
The mechanisms responsible are multiple, include the
passive backward transmission of the pressure elevation

Pathobiology of Pulmonary
Hypertension
Grup 3 (PH due to lung disease and/or hypoxia)
The mechanisms responsible are multiple, include:
Hypoxic vasoconstriction
Mechanical stress of hyperinflated lungs
Loss of capillaries
Inflammation
Toxic effects of cigarette smoke

Pathobiology of Pulmonary
Hypertension
Grup 4 (Chronic Obstructive Pulmonary Disease)
Acute embolic masses which later undergo fibrosis
leading to mechanical obstruction of pulmonary
arteries.
Pulmonary thromboembolism or in situ thrombosis may
be initiated or aggravated by abnormalities in either the
clotting cascade, endothelial cells, or platelets, all of
which interact in the coagulation process.

Pathobiology of Pulmonary
Hypertension
Grup 5 (PH with unclear and/or multifactorial
mechanisms)
The pathobiology in this group is unclear or
multifactorial.

Diagnosis
Requires a series of investigations intended to confirm
the diagnosis, clarify the clinical group of PH and the
specific etiology within the group, and evaluate the
functional and haemodynamic impairment.

Clinical Presentation
The symptoms are non-specific
Breathlessness
Fatigue
Weakness
Angina
Syncope
Abdominal distension

Clinical Presentation
Loud pulmonic valve closure sound (P2)
Murmur of tricuspid regurgitation (a systolic murmur
over the left lower sternal border)
Murmur of pulmonic insufficiency (a diastolic murmur
over the left sternal border)
Jugular venous distension
Peripheral edema
Hepatomegaly
Ascites

 Electrocardiogram
Chest radiograph
Pulmonary function tests and arterial blood gases
Ventilation/perfusion lung scan
High-resolution computed tomography, contrast-enhanced
computed tomography, and pulmonary angiography
Cardiac magnetic resonance imaging
Blood tests and immunology
Abdominal ultrasound scan
Right heart catheterization and vasoreactivity

Assessement disease
progression and response to
therapy (adaptation of the
NYHA system)

Thank You

Electrocardiogram
Sensitivity (55%) and specificity (70%) to be a screening
RV hypertrophy and strain
Right atrial dilatation
The absence of these findings does not exclude the
presence of PH nor does it exclude severe
haemodynamic abnormalities

Chest radiograph
central pulmonary arterial dilatation, which contrasts
with pruning (loss) of the peripheral blood vessels
Right atrium and RV enlargement (in more advanced
cases)
The chest radiograph allows associated moderate-tosevere lung diseases or pulmonary venous hypertension
due to left heart disease to be reasonably excluded.

Chronic Thromboembolic
Pulmonary Hypertension
acute pulmonary embolism showing signs of PH or RV
dysfunction (follow-up echocardiography after discharge
usually after 3 6 months)
The final diagnosis of CTEPH -> pre-capillary PH (mean
PAP 25 mmHg, PWP 15 mmHg, PVR >2 Wood units)
in patients with multiple chronic/organized occlusive
thrombi/emboli in the elastic pulmonary arteries (main,
lobar, segmental, subsegmental)

Chronic Obstructive Pulmonary

Disease
Patients with CTEPH should receive life-long
anticoagulation, usually with vitamin K antagonists
adjusted to a target INR between 2.0 and 3.0.