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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2935_Библиотеки_им_академика_М_И_Перельмана
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Pulmonary Hypertension196
https://t.me/med1917
HRCT:
•
• Absence of underlying lung disease.
• Centrilobular ground glass nodules can be evident.
CTPA/VQ scan:
•
• Assessment for presence of chronic thromboembolic disease (See
Figure 12.2).
Figure 12.2 CTPA of a patient with pulmonary hypertension.
PFT:
•
• Mild restrictive or obstructive pattern in some patients.
• Reduced T.
Abdominal ultrasound:
•
• Screen for portal hypertension.
Right heart catheter:
•
Respiratory Medicine
• Mean pulmonary artery pressure of more than 20 mmHg.
• Pulmonary wedge pressure less than 15 mmHg.
12.2 Idiopathic Pulmonary Arterial Hypertension (IPAH)
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• Normal or reduced cardiac output.
• Patients can also undergo vasodilator response test to assess likely
response to calcium channel blockers during the procedure.
6. MANAGEMENT
General measures:
•
• Diuretics.
• Oxygen therapy.
• Oral anticoagulation (no clear guideline, must be individualised).
• Influenza and pneumococcal vaccination.
• Correction of anaemia.
Supportive therapy:
•
• Psychosocial support
• Avoid excessive physical activity
• Rehabilitation.
Vasoreactive response on right heart catheter:
•
• Calcium channel blockers:
– Patients must be followed up to confirm they are long-term
responders.
– Examples include nifedipine and diltiazem.
Non-vasoreactive response on right heart catheter:
•
• Prostacyclin analogues:
– Acts as a vasodilator and inhibits platelet aggregation.
– Can be given as continuous IV infusion, subcutaneous infusion or
nebulised.
– Examples include epoprostenol and iloprost.
• Endothelin receptor antagonists:
– Counteracts vasoconstriction of vascular smooth muscle by
endothelin.
– Examples include bosentan and ambrisentan.
• Phosphodiesterase-5 inhibitors:
– Increases vasodilatory effects of nitric oxide.
– Examples include sildenafil and tadalafil.
Balloon atrial septostomy:
•
• Atrial septostomy creates a right to left shunt within the atria.
• is decompresses the right heart, increases left ventricular preload and
improves cardiac output.
• Can be used in patients awaiting transplantation, right heart failure
refractory to medical therapy or with syncopal symptoms.
Transplantation:
•
• Indicated when there is inadequate response to other therapies espe-
cially in context of poor prognosis profile.
• 5-year survival is 45- 50%.
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Pulmonary Hypertension198
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7. PROGNOSIS
Prognosis is indicated by WHO functional classification (see Table 12.2).
•
• Median survival of untreated patients with WHO functional class IV is
6 months.
• Medial survival of untreated patients with WHO functional class III is
2.5 years.
Table 12.2 WHO functional classification of PH.
CLASS WHO FUNCTIONAL CLASSIFICATION
I
II
III
IV
Data from: Rich, S. Primary pulmonary hypertension: executive summary. Evian, France.
World Health Organization, 1998.
Patients with pulmonary hypertension but without resulting
limitations of physical activity. Ordinary physical activity does not
cause undue fatigue or dyspnoea, chest pain, or heart syncope.
Patients with pulmonary hypertension resulting in slight limitation
of physical activity. ey are comfortable at rest. Ordinary physical
activity results in undue fatigue or dyspnoea, chest pain, or heart
syncope.
Patients with pulmonary hypertension resulting in marked limita-
tion of physical activity. ey are comfortable at rest. Less than
ordinary physical activity causes undue fatigue or dyspnoea, chest
pain, or heart syncope.
Patients with pulmonary hypertension resulting in inability to carry
on any physical activity without symptoms. ese patients
manifest signs of right heart failure. Dyspnoea and/or fatigue may
be present even at rest. Discomfort is increased by physical activity.
MICRO-print
Indicators of poor prognosis in IPAH:
Clinical evidence of right heart failure.
•
Rapid progression of symptoms.
•
Syncope.
•
WHO functional class IV.
•
6-minute walk test of less than 300m.
•
Peak O2 consumption of cardio-pulmonary exercise test less than
•
12 mL/min/kg.
Elevated or rising BNP levels.
•
Respiratory Medicine
12.3 Pulmonary Hypertension due to Other Causes
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12.3 PULMONARY HYPERTENSION DUE TO
OTHER CAUSES
1. GROUP 2: PULMONARY HYPERTENSION DUE TO LEFT
HEART DISEASE
is can be caused by systolic, diastolic or valvular dysfunction.
•
Passive backwards transmission of pressure increases pulmonary artery
•
pressure.
Pulmonary vascular resistance is increased by increased vasomotor tone and
•
structural remodelling in the pulmonary arteries.
Diagnosis is made on right heart catheterisation with the following criteria:
•
• Mean pulmonary arterial pressure ≥20 mmHg.
• Pulmonary wedge pressure ≥15 mmHg.
• Normal/reduced cardiac output.
Left atrial enlargement on echocardiogram is a useful marker for chronic left
•
atrial hypertension.
Treatment should be aimed at the underlying heart disease. Drugs specific to
•
the treatment of PAH are not recommended.
2. GROUP 3: PULMONARY HYPERTENSION DUE TO LUNG
DISEASE AND/OR HYPOXIA
As this group covers a wide variety of causes, there are multiple factors in the
•
development of PH including:
• Hypoxic vasoconstriction
• Mechanical stress from hyperinflation
• Loss of capillaries
• Inflammation
• Smoking.
PH is a poor prognostic factor in COPD and ILD.
•
Echo can be used to screen for PH but diagnosis should be confirmed with
•
right heart catheter.
Management should be with treatment of the underlying lung disease and
•
long-term oxygen therapy for hypoxic patients.
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Respiratory Medicine
3. GROUP 4: CHRONIC THROMBOEMBOLIC PULMONARY
HYPERTENSION (CTEPH)
Resolution of clot in PE occurs within 30 days in 90% of patients.
•
In CTEPH there is abnormal organisation of thromboemboli into endotheli-
•
alised fibrotic lesions that cause either occlusion or stenosis of the vessels.
Peripheral vascular changes similar to those in IPAH occur in the peripheral
•
vessels.
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Neovascularisation occurs via collateral vessels.
•
Suspected cases should undergo V/Q scan. A normal scan rules out CTEPH.
•
Diagnosis is confirmed with right heart catheter.
•
Patients should have lifelong anticoagulation and be referred for consider-
•
ation of pulmonary endarterectomy or balloon pulmonary angioplasty.
Medical therapies shown to reduce pulmonary vascular resistance and
•
improve exercise capacity include oral riociguat, and subcutaneous
treprostinil.
4. GROUP 5: PULMONARY HYPERTENSION WITH UNCLEAR/
MULTIFACTORIAL MECHANISM
is is a diverse group of patients who do not clearly fit into one of the other
•
four WHO classifications.
e causes of PH in this group include systemic diseases, haematological
•
disorders and metabolic diseases.
MICRO-case
You are working as a respiratory physician in a district general hospital when
a 60-year-old man is referred to you by one of your colleagues. The patient
has noticed progressive shortness of breath over the last few months, and
as part of his work-up, underwent an echocardiogram. He has been referred
to yourself as his estimated pulmonary artery systolic pressure (PASP) in
60 mmHg. You note that there is no evidence of left heart disease on the
echocardiogram but there is right ventricular hypertrophy.
An HRCT is performed to exclude any underlying disease (there is
none) and a CTPA demonstrates no thromboembolic disease. There is
no evidence of any underlying systemic disease on clinical assessment or
blood tests.
You refer the patient for right heart catheterisation which confirms
pulmonary arterial hypertension and demonstrates a pulmonary capillary wedge pressure <15 mmHg. The test also demonstrates a positive
response to calcium channel blockers, so he is commenced on diltiazem.
Key Points
The definitive diagnosis of PH requires right heart catheterisation,
•
demonstrating a mean pulmonary artery pressure of >20 mmHg.
To diagnose IPAH, other causes of pulmonary hypertension must be
•
excluded.
Individuals with IPAH may demonstrate a vasoactive response on right
•
heart catheterisation, in which case calcium channel blockers are the
mainstay of treatment.
If there is no vasoactive response, then treatment is with prostacyclin
•
Respiratory Medicine
analogues, endothelin receptor antagonists or phosphodiesterase-5
inhibitors.
13
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Fungal Diseases
of the Lung
13.1 ALLERGIC BRONCHOPULMONARY
ASPERGILLOSIS (ABPA)
1. DEFINITION
A chronic hypersensitivity reaction that occurs in response to an Aspergillus
•
fungus, most commonly Aspergillus fumigatus.
Diagnostic criteria:
•
• History of asthma.
• Positive skin prick test to Aspergillus antigen.
• Elevated precipitating antibodies to Aspergillus species.
• Elevated serum total IgE concentration >417 IU/mL (>1000 ng/mL).
• Blood eosinophilia > 500 / mm
• Lung infiltrates on chest radiograph or chest high-resolution computed
tomography (HRCT).
• Central bronchiectasis on chest computed tomography (CT).
• Elevated specific serum IgE and IgG to A. fumigatus.
ABPA-S (seropositive) disease is defined by the first four criteria in the
•
absence of central bronchiectasis.
e minimal criteria to diagnose ABPA are:
•
• History of asthma.
• Immediate skin test reactivity to A. fumigatus.
• Elevated serum total IgE.
• Central bronchiectasis.
• Elevated specific serum IgE and IgG to A. fumigatus.
In CF the diagnosis can be more difficult. Proposed definition:
•
• Deterioration in symptoms.
• Serum IgE >1000iu / ml .
3
.
DOI: 10.1201/9781315113937-14
Fungal Diseases of the Lung202
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• Immediate skin prick test positivity to Aspergillus or elevated specific
IgE to Aspergillus.
• IgG Aspergillus or precipitating antibodies.
• New chest radiology changes.
2. EPIDEMIOLOGY
Patients usually have a history of asthma or cystic fibrosis (CF).
•
• e disease is estimated to affect around 2% of asthmatics and up to
15% of those with CF.
3. AETIOLOGY AND RISK FACTORS
Atopy is an important risk factor for developing ABPA.
•
4. PATHOPHYSIOLOGY
e true mechanisms are not clearly understood.
•
Aspergillus fumigatus colonises the bronchi. Airborne spores reach the alveoli
•
where they germinate leading to inflammation caused by IgE- and IgGmediated reactions.
Underlying airway disease such as asthma or CF causes mucus hypersecretion
•
and impairs mucociliary clearance.
• is leads to increased trapping of spores and decreased clearance.
• Increased germination of spores within the lung induces an immune
response.
• e proximal bronchi become dilated and filled with mucus plugs
containing eosinophils and fungal hyphae.
• Bronchial obstruction, inflammation and mucoid impaction can lead to
fibrosis and respiratory compromise.
MICRO-print
Chronic aspergillosis can lead to complications:
Chronic cavitary aspergillosis – cavities form in the lung without the
•
presence of aspergilloma.
Chronic fibrosing pulmonary aspergillosis – if aspergillosis remains
•
untreated, it causes scarring of the lung tissue that is irreversible.
Respiratory Medicine
13.1 Allergic Bronchopulmonary Aspergillosis (ABPA)
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5. CLINICAL FEATURES
Almost exclusively in those with a history of asthma or CF.
•
Typically presents from teenage years until the fourth decade.
•
Symptoms:
•
• History of atopy
• Cough
• Wheeze
• Purulent mucus production
• Pleuritic chest pain.
Signs:
•
• Fever
• Finger clubbing
• Weight loss
• Cyanosis (late feature).
6. INVESTIGATIONS
Investigation findings in ABPI are listed in Table 13.1.
•
Table 13.1 Investigations in patients with suspected ABPI.
INVESTIGATIONS RESULT
Aspergillus fumigatus skin Can be used as an initial screening test. Positive
test wheal and flare reaction (not specific). Further
serological and radiographic tests are needed, so
often excluded in favour of serology.
Serum total IgE IgE > 400 IU/mL if skin prick test not positive, is
highly suggestive of ABPA.
Specific IgE to Aspergillus IgE RAST positivity evident.
Full blood count (FBC) Peripheral blood eosinophilia.
Sputum culture and Macroscopic mucus plugs may be expectorated. A.
microscopy fumigatus may be cultured and eosinophils seen on
microscopy. ese findings, however, are non-specific.
Chest X-ray Flitting consolidation due to mucus plugging can
be seen +/− bronchiectatic change.
Chest computed to CT describes central proximal bronchiectasis (see
mography (CT) Figure 13.1).
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Fungal Diseases of the Lung204
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Figure 13.1 Chest CT of a patient with ABPA; the CT shows bilateral bronchiectasis
and an aspergilloma in the left lung.
Diagnosis of ABPI is separated clinically into 5 stages (see Micro-Facts box).
•
MICRO-facts
Stages of allergic bronchopulmonary aspergillosis (they are not necessarily consecutive):
Stage 1: acute
Fever, cough, chest pain, haemoptysis, sputum.
•
Infiltrates on CXR: upper or middle lobe.
•
Serum IgE: markedly elevated.
•
Stage 2: remission
Respiratory Medicine
Asymptomatic/stable asthma.
•
13.1 Allergic Bronchopulmonary Aspergillosis (ABPA)
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No infiltrates on CXR when patient off prednisolone >6 months.
•
Serum IgE: elevated or normal.
•
Stage 3: exacerbation
Symptoms mimicking acute stage, or asymptomatic.
•
Infiltrates on CXR: upper or middle lobe.
•
Serum IgE: markedly elevated.
•
Stage 4: corticosteroid-dependent asthma
Persistent severe asthma.
•
Infiltrates on CXR: absent or only intermittent.
•
Serum IgE: elevated or normal.
•
Stage 5: end-stage fibrosis
Cyanosis and dyspnoea.
•
Fibrotic, bullous, or cavitating lesions on CXR.
•
7. MANAGEMENT
Prednisolone commenced at 30–40 mg with tapering over 3–6 months.
•
Prolonged corticosteroid therapy will require bone protection, e.g.
•
bisphosphonates.
Itraconazole 200mg can be used as a steroid sparing agent:
•
• Eliminates potential risk of adrenal suppression.
• Affects the cytochrome PA450 34pathway.
• Metabolism of inhaled budesonide and fluticasone can be reduced and
so dose should be reduced.
205
MICRO-facts
Chronic pulmonary aspergillosis consists of five current consensus
definitions:
Chronic cavitatory pulmonary aspergillosis (CCPA) is the most com-
•
mon form: defined by one or more cavities, with or without a fungal
ball present.
Simple aspergilloma: Single fungal ball growing in a cavity.
•
Aspergillus nodules: An unusual form of CPA, non-cavity forming,
•
mimicking carcinoma of the lung or metastases and can only be definitively diagnosed using histology.
Chronic fibrosing pulmonary aspergillosis (CFPA): Late-stage CCPA.
•
Subacute invasive aspergillosis (SAIA) is very similar to CCPA but
•
occurs in mildly immunocompromised or debilitated patients (e.g.
diabetes mellitus, malnutrition, alcoholism, advanced age, prolonged
steroid administration, COPD). Histology can often show tissue invasion. Progression is more rapid than with CCPA.
Respiratory Medicine
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