Pulmonary vascular disease

بسم الله الرحمن الرحيم

IN THE NAME OF ALLAH THE MOST MERCIFULL.
7/2/2021

Pulmonary vascular disease

1- Pulmonary hypertension.
2-Pulmonary venous thromboembolism.
3-Pulmonary vasculitis.

Pulmonary hypertension

Objectives:

Causes of pulmonary hypertension

1-Pulmonary vascular disorders
Acute pulmonary thromboembolism (rarely tumour emboli)
Primary pulmonary hypertension
2-Diseases of the lung and parenchyma
COPD
3-Musculoskeletal disorders (causing chronic underventilation)
Kyphoscoliosis

4-Disturbance of respiratory control

Obstructive sleep apnoea
5-Cardiac disorders
Mitral stenosis
Left ventricular failure
6-Miscellaneous
Appetite-suppressant drugs.


Primary pulmonary hypertension (PPH)
Present spontaneously with no apparent underlying disease
occur in association with other disease processes.

Primary pulmonary hypertension

Clinical presentation
Clinical signs of pulmonary hypertension

Chest X-ray ,ECG , Echocardiography .
The diagnosis can be confirmed by CT pulmonary angiography.

Treatment

Warfarin and oxygen
oral calcium-channel blockers.
oral endothelin receptor antagonists (bosentan).
Prostanoid analogues.
Prognosis
Poor.

Electrocardiogrm demonstrating sinus rhythm, right axis deviation, and right ventricular hypertrophy with a strain pattern.




Posterior-anterior (A) andLateral (B) chest radiographs demonstratingenlarged proximal pulmonary arteries andright ventricular enlargement

Chronic Cor Pulmonale

enlargement of the right ventricle because of increase in afterload .

Pathophysiology

mechanism varies according to the cause of cor pulmonale.

Hypoxia impairs right ventricular function and, as it develops, left ventricular function is also depressed.

Clinical features

Chest pain.
exertional dyspnoea.
syncope .
fatigue .
sudden death .
symptoms due the pulmonary hypertension.


physical examination :
signs of pulmonary hypertension.
signs right ventricular enlargement and failure.
Treatment
Diuretic .
oxygen therapy.

Pulmonary Venous Thromboembolism

Essentials of Diagnosis
venous thrombosis, usually of the lower extremities.
dyspnea, chest pain, hemoptysis, syncope.
Tachypnea.
CT scan of the chest.
pulmonary angiogram is diagnostic.

General Considerations

pulmonary embolism (PE), is a common, serious, and potentially fatal.( Massive)
200,000 deaths each year in the U.S.A.
The third leading cause of death among hospitalized patients.
Cause of death in PE.



Substances that embolize to the pulmonary circulation:

Air (during neurosurgery, from central venous catheters),

Amniotic fluid (during active labor),
Fat (long bone fractures),
Foreign bodies (talc in injection drug users),
Parasite eggs (schistosomiasis),
Septic emboli (acute infectious endocarditis),
Tumor cells (renal cell carcinoma).

The most common embolus is thrombus, which may arise anywhere in the venous circulation or heart but most often originates in the deep veins of the lower extremities.

PE and DVT are two manifestations of the same disease.

The risk factors for PE and DVT : (Virchow's triad).
1-venous stasis.
2- injury to the vessel wall.
3- hypercoagulability.

1-Venous stasis increases

immobility (bed rest—especially postoperative—obesity, stroke).
hyperviscosity (polycythemia).
increased central venous pressures (low cardiac output states, pregnancy)


2-Vessels may be damaged by
prior episodes of thrombosis.
orthopedic surgery.
trauma.

3-Hypercoagulability can be caused by

medications (oral contraceptives, hormonal replacement therapy)
disease (malignancy, surgery)
pregnancy.
inherited gene defects.
• 1-activated protein C, also known as factor V Leiden.
• 2-deficiencies or dysfunction of
• - protein C.
-protein S.
-antithrombin III.

CATEGORISATION OF PULMONARY THROMBOEMBOLI

AM- Acute Massive PE
SM-Acute small/medium PE
C- Chronic PE

Pathophysiology

AM-Major haemodynamic effects: ↓ cardiac output; acute right heart failure

SM-Occlusion of segmental pulmonary artery → infarction ± effusion

C-Chronic occlusion of pulmonary microvasculature, right heart failure

Symptoms

AM-Faintness or collapse, central chest pain, apprehension, severe dyspnoea
SM-Pleuritic chest pain, restricted breathing, haemoptysis
C- Exertional dyspnoea. Late symptoms of pulmonary hypertension or right heart failure

Signs

AM- Major circulatory collapse: tachycardia, hypotension, ↑ JVP, right ventricular gallop rhythm, split P2 Severe cyanosis ↓ Urinary output
SM- TachycardiaPleural rub, raised hemidiaphragm, crackles, effusion (often blood-stained)Low-grade fever
C- May be minimal early in diseaseLater-RV heave, loud, split P2Terminal-right heart failure


Chest X-ray
AM-Usually normal.
May be subtle
oligaemia
SM-Pleuropulmonary opacities, pleural effusion, linear shadows, raised hemidiaphragm
C-Enlarged pulmonary artery trunk, enlarged heart, prominent RV

ECG

AM- S1 Q3 T3 anterior T-wave inversion Right bundle branch block (RBBB).

SM- Sinus tachycardia.

C- RV hypertrophy and strain.

Arterial blood gases

AM-Markedly abnormal with ↓ PaO2 and

↓ PaCO2. Metabolic acidosis
SM- May be normal or ↓ PaCO2
C-Exertional ↓ PaO2 or desaturation on formal exercise testing


Alternative diagnoses

AM-Myocardial infarction; pericardial tamponade; aortic dissection

SM-Pneumonia, pneumothorax, musculoskeletal chest pain
C-Other causes of pulmonary hypertension









Spiral computed tomographic image of acute pulmonary emboli in both main pulmonary arteries in a postoperative patient with the sudden onset of dyspnea, hypoxemia, and hypotension.

High-probability ventilation-perfusion scan.

Laboratory Findings

Arterial blood gases.
Plasma D-dimer, .
Serum troponin I, troponin T, and plasma beta-natriuretic peptide (BNP) levels.
Lung scanning
CT
Helical CT pulmonary angiography sensitive and very specific.

Venous thrombosis studies

venous ultrasonography, the test of choice to detect proximal DVT
impedance plethysmography.
contrast venography.
Pulmonary angiography
MRI

Prevention

Management


Managment

General measures

Oxygen should be given to all hypoxaemic patients
Opiates may be necessary to relieve pain and distress
hypotension should be treated by giving intravenous fluid or plasma expander.
Resuscitation by external cardiac massage .

Anticoagulation

'Low molecular weight heparins.'
Continued using oral warfarin.
The international normalised ratio (INR) is greater than 2.
Duration of warfarin therapy :variable 3 months- for life.

Thrombolytic therapy …INDICATIONS

Caval filters : INDICATIONS.

Plmonary Vasculitis

Wegener's granulomatosis
diagnosis
serologic testing and biopsy of lung .
Allergic angiitis and granulomatosis (Churg-Strauss syndrome)
an idiopathic multisystem vasculitis of small and medium-sized arteries that occurs in patients with asthma.
Treatment : corticosteroids and cyclophosphamide.


Alveolar Hemorrhage Syndromes
immune and nonimmune disorders.
Hemoptysis, alveolar infiltrates on chest radiograph, anemia, dyspnea, and occasionally fever are characteristic.
immune alveolar hemorrhage
Nonimmune causes diffuse hemorrhage
coagulopathy.
mitral stenosis.
Idiopathic pulmonary hemosiderosis .









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