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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5537_Библиотеки_им_академика_М_И_Перельмана.pdf
344
J. Kim and T. J. Harkin
and can predispose the patient to atypical infections. Rheumatologic flares
in CTD–ILD and sarcoidosis are also fairly common and should be treated
appropriately.
Patients with DPLDs commonly present with decompensated cardiopulmonary symptoms. A CT angiogram of the chest may be helpful in
ruling out pulmonary embolism in immobile patients and in determining
if changes in the lung parenchyma represent infection, heart failure, or
worsening of underlying lung disease. An infectious workup often
requires early bronchoscopy for prompt diagnosis and treatment of atypical lung infections due to poor functional reserve in this population.
Appropriate noninvasive testing for typical viral diseases and bacterial
pneumonias (viral washings, sputum cultures, legionella urine antigen,
mycoplasma studies, and blood cultures) should always be pursued prior
to bronchoscopy. It should also be recognized that any extrapulmonary
stressor can increase cardiopulmonary demands and be mistaken as a primary cardiopulmonary insult (e.g. urosepsis, pancreatitis).
IPF patients can present with an “acute IPF exacerbation.” Proposed
diagnostic criteria for acute exacerbation include subjective worsening of
breathing over 30 days or less, new bilateral radiographic opacities, and
the absence of infection or another identifiable etiology for respiratory
decline.
24,26
This is a diagnosis of exclusion which may require extensive
testing to rule out other etiologies.
24
Acute IPF exacerbations are associated with increased inpatient mortality, which can possibly be reduced
with the use of early anticoagulation and possibly increased immunosuppression.
26
Similar exacerbations are described less commonly but can
also be seen in other DPLDs. IPF patients should be promptly referred for
lung transplantation due the high mortality associated with this disease.
25
References
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Evaluation and Treatment of Diffuse Parenchymal Lung Disease
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Diagnosis and treatment. International consensus statement.
American Thoracic Society (ATS) and European Respiratory Society
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BC Decker, London.
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14. Petri M. (2005) Review of classification criteria for systemic lupus
erythematosus. Rheum Dis Clin North Am 31(2): 245–254, vi.
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erythematosus. Lancet 369(9561): 587–596.
16. Dalakas MC. (1991) Polymyositis, dermatomyositis and inclusionbody myositis. N Engl J Med 325(21): 1487–1498.
17. Mastaglia FL, Phillips BA. (2007) Idiopathic inflammatory myopathies:
epidemiology, classification, and diagnostic criteria. Rheum Dis Clin
North Am 28(4): 723–741.
18. Iannuzzi MC, Rybicki BA, Teirstein AS. (2007) Sarcoidosis. N Engl
J Med 357(21): 2153–2165.
19. McCormack FX. (2008) Lymphangioleiomyomatosis: A clinical
update. Chest 133(2): 507–516.
20. Johnson SR. (2006) Lymphangioleiomyomatosis. Eur Respir J 27(5):
1056–1065.
21. Allen TC. (2008) Pulmonary Langerhans cell histiocytosis and other
pulmonary histiocytic diseases: Areview. Arch Pathol Lab Med 132(7):
1171–1181.
22. Kinder BW, Shariat C, Collard HR, et al. (2010) Undifferentiated connective tissue disease–associated interstitial lung disease: Changes in
lung function. Lung 188(2): 143–149.
23. Kinder BW, Collard HR, Koth L, et al. (2007) Idiopathic nonspecific
interstitial pneumonia: Lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med 176(7): 691–697.
24. Hunninghake GW, Zimmerman MB, Schwartz DA, et al. (2001)
Utility of a lung biopsy for the diagnosis of idiopathic pulmonary
fibrosis. Am J Respir Crit Care Med 164(2): 193–196.
25. Collard HR, Moore BB, Flaherty KR, et al. Idiopathic Pulmonary
Fibrosis Clinical Research Network Investigators. (2007) Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care
Med 176(7): 636–643.
26. Orens JB, Estenne M, Arcasoy S, et al. Pulmonary Scientific Council
of the International Society for Heart and Lung Transplantation.
(2006) International guidelines for the selection of lung transplant
candidates: 2006 update — a consensus report from the Pulmonary
Scientific Council of the International Society for Heart and Lung
Transplantation. J Heart Lung Transplant. 25(7): 745–755.
27. Kubo H, Nakayama K, Yanai M, et al. (2005) Anticoagulant therapy
for idiopathic pulmonary fibrosis. Chest 128(3): 1475–1482
28. Argiriadi PA, Mendelson DS. (2009) High resolution computed
tomography in idiopathic interstitial pneumonias. Mt Sinai J Med
76(1): 37–52.
29. Flaherty KR, Andrei AC, Murray S, et al. (2006) Idiopathic
pulmonary fibrosis: Prognostic value of changes in physiology and
six-minute-walk test. Am J Respir Crit Care Med 174(7): 803–809.
30. Fisher MR, Forfia PR, Chamera E, et al. (2009) Accuracy of Doppler
echocardiography in the hemodynamic assessment of pulmonary
hypertension. Am J Respir Crit Care Med 179(7): 615–621.
31. Shim HS, Park MS, Park IK. (2010) Histopathologic findings of transbronchial biopsy in usual interstitial pneumonia. Pathol Int 60(5):
373–377.
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Evaluation and Treatment of Diffuse Parenchymal Lung Disease
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Pulmonary Hypertension
Ajith P. Nair*
Key Pearls
• Pulmonary hypertension (PH) is defined by a mean pulmonary artery
(mPA) pressure greater than 25 mmHg. Pulmonary arterial hypertension (PAH) is specified by a mPA > 25 mmHg, pulmonary capillary
wedge pressure < 15 mmHg and pulmonary vascular resistance
greater than 3 Woods Units.
• PH can be divided into five categories.
• Echocardiography and right heart catheterization with vasoreactivity
testing are mandatory for the evaluation of PAH.
• PAH requires further investigation and specific therapy. PH from leftsided heart disease or lung disease requires treatment of the underlying disorder.
• Standard therapy for PAH includes diuretics, digoxin, anticoagulation, and supplemental oxygen. Calcium channel blockers are appropriate for patients who demonstrate vasoreactivity. Targeted therapy
includes endothelin receptor antagonists, prostacyclin analogues and
phosphodiesterase inhibitors.
Introduction
Pulmonary hypertension is characterized by increased resistance across
the pulmonary circulation, which can lead to right ventricular (RV) failure
349
*Mount Sinai School of Medicine, New York, NY, USA.
30
Chapter
through increased afterload. The pulmonary vascular abnormalities can
result from a number of disorders, including cardiac, pulmonary or collagen vascular disease. Most hospitalized patients will have pulmonary
hypertension secondary to left-sided heart disease (heart failure with preserved or abnormal systolic function). Further investigation is warranted
for pulmonary hypertension that is due to pulmonary vascular disease or
pulmonary arterial hypertension.
Definition
Pulmonary hypertension (PH) can be generally defined as an elevation in
systolic pulmonary pressures greater than 35 mmHg or mean pulmonary
artery pressures greater than 25 mmHg.
1
The disease can be pre-capillary,
post-capillary or due to high cardiac output. Post-capillary pulmonary
hypertension results from pulmonary venous congestion, which may
result from left ventricular dysfunction or mitral valve disease. Pulmonary
arterial hypertension (PAH ) results from pre-capillary disease and is
defined by a pulmonary arterial pressure > 25 mmHg, a pulmonary capillary wedge pressure of < 15 mmHg and pulmonary vascular resistance
(PVR) of greater than 3 Woods Units.
The term cor pulmonale is used when pulmonary hypertension and
cardiac dysfunction result from lung disease (e.g. chronic obstructive lung
disease, connective tissues disease, and interstitial lung disease).
Eisenmenger’s syndrome occurs when right to left shunts associated with
congenital heart disease reverse due to increasing pulmonary pressures.
Classification
Pulmonary hypertension can be acute or chronic, and the acute form usually
results from pulmonary thromboembolic disease. The chronic forms of pulmonary hypertension can divided into five major categories (see Table. 1).
2
Group I pulmonary arterial hypertension is characterized by disease of
the small pulmonary arteries. Included are idiopathic pulmonary arterial
hypertension (IPAH), which was formerly termed primary pulmonary
350
A. Nair
351
Pulmonary Hypertension
Table 1. Classification of Pulmonary Hypertension (Dana Point Clinical
Classification of PH, 2008)
Group 1 Pulmonary Arterial Hypertension
• Idiopathic PAH (“primary pulmonary hypertension”)
• Heritable pulmonary arterial hypertension (HPAH):
BMPR2, ALK1, endoglin
• Drug- and toxin-induced
• Associated pulmonary arterial hypertension (APAH):
Connective tissue diseases, HIV infection, Portal hypertension
• Congenital heart disease
• Schistosomiasis
• Chronic hemolytic anemia
• Persistent pulmonary hypertension of the newborn
Group 2 Left-sided Heart Disease
• Systolic dysfunction
• Diastolic dysfunction
• Valvular disease
Group 3 Lung Disease or Hypoxemia
• Chronic obstructive pulmonary disease
• Interstitial lung disease
• Other pulmonary diseases with mixed restrictive and
obstructive pattern
• Sleep-disordered breathing
• Alveolar hypoventilation disorders
• Chronic exposure to high altitude
• Developmental abnormalities
Group 4 Chronic thrombotic and/or embolic disease
Group 5 Miscellaneous
• Hematologic disorders: myeloproliferative disorders,
splenectomy
• Systemic disorders: sarcoidosis, pulmonary Langerhans cell
• Metabolic disorders: glycogen storage disease, Gaucher
disease, thyroid disorders
• Others: tumoral obstruction, fibrosing mediastinitis,
chronic renal failure on dialysis
hypertension; associated pulmonary arterial hypertension (APAH ) from
collagen vascular diseases; HIV; and portopulmonary hypertension; and
heritable pulmonary arterial hypertension (HPAH ). Mutations in bone
morphogenetic protein receptor type 2 (BMPR2) have been identified as
the primary cause of heritable PAH (HPAH). Drugs, including appetite
suppressants containing aminorex, dexfenfluramine and fenfluramine,
have been associated with an increased risk of PAH.
Secondary pulmonary hypertension usually results from cardiac or respiratory diseases. Increases in pulmonary blood flow through shunts or high
output (e.g. dialysis fistulas or hepatic failure) or through venous congestion can lead to elevated pulmonary pressures. Lung disease can result in
hypoxic vasoconstriction and the loss of pulmonary vasculature due to
fibrosis. Pulmonary pressures can increase “out of proportion” to the
degree of left-heart failure or lung disease, which may be due to intimal
proliferation and increases in PVR.
Chronic thromboembolic pulmonary hypertension (CTEPH) is important
to exclude as a cause of pulmonary hypertension as it can be potentially
cured via thromboendarterectomy. Ventilation-perfusion scan has been
shown to be a more sensitive test for detection of CTEPH than CT angiography. Embolic occlusion of the pulmonary vascular bed may also result
from tumor metastases, schistosomiasis, filariasis, or talc or fiber
embolism from intravenous drug use.
Clinical Presentation
The presentation of pulmonary hypertension is similar despite the multiple different etiologies of the disease. Dyspnea, fatigue, edema and exertional syncope are classic symptoms. RV ischemia may lead to angina.
Patients with collagen vascular disease commonly have concomitant
Raynaud’s disease.
Physical examination findings include an increased intensity of the P2
component of the second heart sound, a right ventricular heave, a right-sided
fourth sound, and murmurs of tricuspid and pulmonic valve regurgitation.
352
A. Nair
Distension of the jugular veins, a pulsatile liver, ascites, and peripheral
edema usually indicate advanced right-sided failure.
Findings on chest radiograph are enlargement of the pulmonary trunk
and hilar vessels and right-heart enlargement. The electrocardiogram may
demonstrate right axis deviation, T wave inversions in the anterior
precordial leads, right atrial enlargement, and RV hypertrophy.
Evaluation (see Fig. 1)
Echocardiography is essential to the diagnosis of pulmonary hypertension. The RV systolic pressure can be estimated via Doppler. It is important, however, to distinguish between PAH and PH from left-sided
disease. Isolated RV enlargement and dysfunction and right atrial enlargement with normal LV function can be key indicators of PAH. Diastolic
and systolic bowing of the septum are significant for RV volume and pressure overload, respectively.
353
Pulmonary Hypertension
Fig. 1. Evaluation of suspected PH.