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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
نسخه الکترونیک
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Clinical features of pulmonary veno-occlusive disease

Clinical features of pulmonary veno-occlusive disease
Signs and symptoms

Clinical signs and symptoms of pulmonary hypertension (eg, dyspnea on exertion, lethargy, lower extremity swelling)

Onset following respiratory tract infection*

Risk factors specific for PVOD (eg, chemotherapy, stem cell transplantation, family history of PVOD)*
Genetic EIF2AK4 gene mutations*
Computed tomographic

Signs of pulmonary hypertension (eg, enlarged central pulmonary arteries, right ventricular, hypertrophy, dilation, intraventricular septal bowing)

Signs of venous congestion, eg, pulmonary edema, centrilobular ground glass opacities, septal thickening*

Mediastinal lymphadenopathy*

Pleural effusion

Normal-sized left atrium and central pulmonary veins
Pulmonary function tests

Severe reduction in single breath diffusion of carbon monoxide (<55% predicted)*

Normal or minimal abnormalities in spirometry and lung volumes
Arterial blood gas and six-minute walk test Severe hypoxemia (partial pressure of oxygen <65 mmHg [8.7 kPa]); low nadir on six-minute walk testing (<85%)
Echocardiography

Signs of right ventricular (RV) strain (eg, RV hypertrophy, RV dilatation, septal bowing)

Normal left-sided cardiac function*
Right heart catheterization

Mean pulmonary artery pressure >20 mmHg*

Pulmonary artery wedge pressure ≤15 mmHg*

Pulmonary vascular resistance ≥3 Woods units (≥240 dynes/sec/cm-5)
Bronchoscopy Hemosiderin-laden macrophages on bronchoalveolar lavage
PVOD: pulmonary veno-occlusive disease; PAH: pulmonary arterial hypertension; mPAP: mean pulmonary artery pressure.
* Patients with PVOD typically have hemodynamic findings of PAH (an elevated mPAP >20 mmHg, elevated pulmonary vascular resistance ≥3 Woods units, and a normal pulmonary artery wedge pressure <15 mmHg). Suspicion for PVOD is increased when PAH is severe (mPAP >45 mmHg). Patients with PVOD also have signs of venous congestion in the absence of having left-sided heart disease. The constellation of such clinical, computed tomographic, physiologic, and hemodynamic findings as indicated by the * are highly suggestive of PVOD and may obviate the need for biopsy.
¶ Bronchoscopy is only performed by some, not all, experts. Hemosiderin-laden macrophages may indicate occult hemorrhage that may be more common in patients with PVOD than in patients with PAH.
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