Diagnosis & Classification of Pulmonary Hypertension

Classification & Diagnosis of Pulmonary Hypertension

Pulmonary Hypertension Overview Defined as mean PA pressure > 25mm Hg at rest or >30mm Hg with exercise. Originally categorized by WHO in 1973 into just 2 categories. Primary vs. secondary pulmonary hypertension. In 1998, WHO reclassified disease into 5 specific sub-categories based on specific disease process. In 2003, categories slightly modified and updated to current Venice Classification System.

Clinical Classification of Pulmonary Hypertension (Venice 2003) 1. Pulmonary arterial hypertension (PAH) 1.1. Idiopathic (IPAH) 1.2. Familial (FPAH) 1.3. Associated with (APAH): 1.3.1. Collagen vascular 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 (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy) 1.4. Associated with significant venous or capillary involvement 1.4.1. Pulmonary veno-occlusive disease (PVOD) 1.4.2. Pulmonary capillary hemangiomatosis (PCH) 1.5. Persistent pulmonary hypertension of the newborn

Clinical Classification of Pulmonary Hypertension (Venice 2003) Cont… 2. Pulmonary hypertension 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 diseases and/or hypoxemia 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

Clinical Classification of Pulmonary Hypertension (Venice 2003) Cont… 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 4.3. Non-thrombotic pulmonary embolism (tumor, parasites, foreign material) 5. Miscellaneous Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis)

When to suspect pulmonary hypertension Any case of breathlessness without overt signs of specific heart and lung disease Increased breathlessness unexplained by the underlying disease itself Symptoms and/or physical signs of pulmonary hypertension in presence of co-morbid conditions like CTD / ILD, CHD with systemic to pulmonary shunt, portal hypertension, HIV infection, sleep apnea syndromes, deep vein thrombosis Incidental suspicion via abnormal ECG , CXR echocardiographic finding .

ECG Supportive evidence for pulmonary hypertension includes Right Ventricular Hypertrophy +/- strain Right atrial enlargement P wave> 2.5 mm in lead II is associated with 2.8 fold greater risk of death over 6 year period . Each additional 1 mm of P wave amplitude in lead II correspond with 4.5 fold increased risks of death. Poor senstivity (55%) & specificity (70%) Normal ECG dose not exclude the presence PH .

Chest X- ray May disclose abnormal anatomic feature due to pulmonary hypertension Enlarged main hilar pulmonary arterial shadows and/ or Attenuation of peripheral pulmonary vascular markings (pruning). Right ventricular enlargement.

Chest X- ray May provide clue to coexisting conditions Pulmonary venous congestion Pulmonary venous hypertension Pulmonary veno-occlusive disease Pulmonary capillary hemangiomatosis Hyperinflation COPD Kyphosis Restrictive ventilatory disease Mosaic oligemia CTEPH

No correlation appear to exist between the extent of radiographic abnormalities and the degree of pulmonary hypertension

Identification of pulmonary hypertension clinical class

Echocardiography Excellent non invasive screening test for suspected pulmonary hypertension. Allows for differential diagnosis of possible causes Valvular heart diseases Myocardial diseases CHD with systemic to pulmonary shunt Transthorasic echocardiography (TEE) is sufficient for most cases.

Echocardiography cont… TEE estimates pulmonary artery systolic pressure (PASP) using tricuspid jet velocity PASP = RVSP = 4V ² + RAP IVC findings estimated RAP Small & Collapse 00-05 mmHg Normal & normal ↓ in size 05-10 mmHg Normal & abnormal ↓ in size 10-15 mmHg Dilated & abnormal ↓ in size 15-20 mmHg Dilated & no change in size >20 mmHg

Echocardiography cont… Severity of PH PASP Mild 35-45 mmHg Moderate 46-60 mmHg Severe >60 mmHg

PFT & ABG Helps in identification of underlying airway/ parenchymal lung disease. Oxymetry & polysomnography For assessment of sleep related breathing disorders , if indicated clinically.

Ventilation & perfusion (V/Q) scan Helpful in diagnosis of chronic thrombo embolic pulmonary hypertension (CTEPH), when perfusion image shows segmental defect while ventilation image is normal. Similar changes can occur in pulmonary veno occlusive disease. In parenchymal lung disease,the perfusion defect are matched by ventilation defects.

HRCT of lung Provides detailed overview of the lung parenchyma and facilitate the diagnosis of ILD & emphysema. Provides clue to Pulmonary veno-occlusive disease / Pulmonary capillary hemangio-matosis . Poorly defined centrilobular nodular opacities Thickened septal lines Mediastinal adenopathy

CT- Pulmonary Angiography Indicated in patients with perfusion defect & normal ventilation on V/Q scan. It may show complete occlusion of pulmonary arteries or eccentric filling defects. Should not be sued to exclude CTEPH.

Conventional pulmonary angiography Indicated in case of inconclusive CT- angiography in patients with clinical or V/Q scan suspicion of CTEPH.

Pulmonary arterial hypertension evaluation

Additional investigations Routine biochemistry Hemogram Thrambophilia screen including anti-phospholipid antibodies An autoimmune screen HIV serology Abdominal ultrasound scan

Right heart catheterization Required to To confirm the diagnosis of PAH, To assess the severity of the haemodynamic impairment and To test the vasoreactivity of the pulmonary circulation. PAH is defined by a mean PAP >25 mmHg at rest or >30 mmHg with exercise, by a PWP ≤ 15 mmHg and by PVR >3 mmHg/l/min (Wood units). Left heart catheterization is required in the rare circumstances in which a reliable PWP cannot be measured.

Acute vasodilator testing Recommended to be done by using short acting selective pulmonary vasodilators like epoprostenol, Adenosine and inhaled nitric oxide. A positive response is defined as a reduction of mean PAP ≥ 10mmHg to reach an absolute value of mean PAP ≤ 40mmHg with an increase or unchanged cardiac output.

Assessment of functional capacity six-minute walk test (6MWT) A technically simple and inexpensive test. It is predictive of survival in IPAH an also correlates inversely with NYHA functional status. Traditional ‘‘primary’’ end point for the great majority of controlled clinical trials performed in PAH

Summary History+ physical examination+ ECG+ chest x-ray Evidence of PAH Evidence of PAH No further evaluation Echo- Doppler Structural cardiac abnormality Pulmonary function test Normal Arterial blood gas analysis No further evaluation Yes No Yes No Primary cardiac disease Restrictive pattern Evaluate for ILD Obstructive pattern COPD Yes No

Arterial blood gas analysis No hypercapnia Is any evidence of pulmonary thromboembolism Cardiac catheterization Hypercapnia Evaluate for hypoventilation syndrome Yes No Thromboembolic PAH Definitive diagnosis Acute vasodilator challenge Summary cont….

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Diagnosis & Classification of Pulmonary Hypertension

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Diagnosis & Classification of Pulmonary Hypertension