pulmonary hypertension clinical classifications
- related: pulmonary hypertension
- tags: #literature #pulmonology
Group 1
Pulmonary arterial hypertension (PAH) (WHO group 1): PAH can be related to certain autoimmune conditions (eg, rheumatoid arthritis, scleroderma). However, isolated pulmonary vascular involvement is exceedingly rare; patients usually have evidence of rheumatism or connective tissue disease (eg, sclerodactyly, Raynaud disease).
- TIPS can cause portopulmonary hypertension
- pulmonary hypertension associated medications
- sickle cell disease and pulmonary hypertension
Group 2
- Left heart disease (group 2): The most common cause of PH in the United States is left-sided heart disease (eg, mitral stenosis, diastolic dysfunction). Significant left-sided heart dysfunction is usually evident on echocardiography.
- ESRD patients with AV fistula can cause high cardiac output heart failure and pulmonary hypertension
Group 3
- Lung disease/hypoxemia (group 3): Patients typically have a preexisting history of lung disease (eg, abnormal spirometry, parenchymal abnormalities on imaging) and/or signs of sleep-disordered breathing (eg, daytime sleepiness, snoring, arousals), which can be evaluated by polysomnography.
- COPD patients have proportional pulmonary hypertension
Group 4
Chronic thromboembolic PH (CTEPH) (group 4): In approximately 2% of patients with acute pulmonary embolism (as in this patient with a history of postoperative pulmonary embolism), the clots become scarred into the vessel wall, leading to chronic vascular obstructions. If present, pulmonary bruits are a very specific sign of CTEPH, reflecting turbulent flow through these irregularly narrowed arteries.
Because pulmonary thromboendarterectomy surgery is potentially curative, a correct diagnosis is essential. Ventilation-perfusion scanning is the test of choice for CTEPH because it is the most sensitive method for detecting distal subsegmental perfusion defects. CT pulmonary angiography is more accurate for visualization of fresh segmental pulmonary emboli but is often obtained in patients with confirmed CTEPH to identify those with favorable operative disease.
Patients with CTEPH have a history of venous thromboembolism that may be in the distant past and may even have recurrent emboli. Unresolved thromboembolism (eg, despite a course of anticoagulation) is likely due to an underlying hypercoagulable state coupled with failure of endogenous fibrinolysis. Therefore, patients should also receive lifelong anticoagulation.2
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pulmonary hypertension pre and post capillary pressures
- Patients with elevated PCWP belong to group 2 (left heart disease) or group 5 (mixed or unclear), different from other groups that generally have decreased PCWP (pulmonary hypertension clinical classifications). Use diastolic pulmonary gradient (PAP Diastolic - PCWP) to get accurate measurement of precapillary PH in patients who are volume up, after diuresis: