9: Pulmonary vascular disorders

Published on 02/03/2015 by admin

Filed under Internal Medicine

Last modified 02/03/2015

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TOPIC 9 Pulmonary vascular disorders

Topic Contents

Pulmonary hypertension 89

Definition 89
Incidence 89
Causes and Classification (ESC Guidelines 2004) 89
Investigations 90
Commonly used vaso-active dugs for vasodilator study drug 91
Algorithm for investigation suspected pulmonary hypertension 91
Management 91
Pulmonary thromboembolism 93

Risk factors 93
Investigations 94
Treatment 94

Pulmonary hypertension

Definition

Mean pulmonary artery pressure (PAP):

> 25 mmHg at rest
> 30 mmHg with exercise.

Incidence

Estimated at 500–1000 new cases annually
Approximately 2–3 per million per year with a prevalence of 15 per million
Occurs in men, women and children of all ages
Most common in females between 20 and 40 years old (M:F 1:2)
Rare in children, but sometimes seen in infants born with valvular defects.

Causes and Classification (ESC Guidelines 2004)

1. Pulmonary arterial hypertension (PAH)

1.1. Idiopathic pulmonary arterial hypertension (IPAH)
1.2. Familial pulmonary arterial hypertension (FPAH)
1.3. Associated with APAH

1.3.1. Connective tissue disease
1.3.2. Congenital systemic to pulmonary shunts
1.3.3. Portal hypertension
1.3.4. HIV infection
1.3.5. Drugs and toxins
1.3.6. Other

1.4. Associated with significant venous or capillary involvement

1.4.1. Pulmonary veno-occlusive disease (PVOD)
1.4.2. Pulmonary capillary haemangiomatosis

1.5. Persistent pulmonary hypertension of the newborn (PPHN)

2. Pulmonary hypertension associated with left heart disease

2.1. Left-sided atrial or ventricular heart disease
2.2. Left-sided valvular heart disease

3. Pulmonary hypertension associated with lung respiratory disease/hypoxia

3.1. Chronic obstructive pulmonary disease
3.2. Interstitial lung disease
3.3. Sleep disordered breathing
3.4. Alveolar hypoventilation disorders
3.5. Chronic exposure to high altitude
3.6. Developmental abnormalities

4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease

4.1. Thromboembolic obstruction of proximal pulmonary arteries
4.2. Thromboembolic obstruction of distal pulmonary arteries
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