Pulmonary Hypertension

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Pulmonary Hypertension By Dr Sajid Ali MO/PGR Pulmonology SZMC/H RYK

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A 24 year old girl from Quetta presented with 6 months history of exertional dyspnoea and chest pain.a cardiologist ruled out cardiac cause for the dyspnoea and reffered to you because echocardiography showed markely elevated pulmonary arterial pressure, What two furhter investigation would you like to do in this patient. Name five medicine which are valuable in the management of pulmonary hypertention.

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Two investigations HRCT with contrast Right cardiac cathetrization

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Five valuable drugs. Diuretics Calcium channel blockers Prostcycline antalogs(Epoprostenol (Flolan)., Treprostinil, Iloprost) Endothelin receptor antagonists(Bosentan) Phoshodiesterase inhibitors(sildenafil)

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Pulmonary Hypertension It is de?ned as a mean pulmonary artery pressure greater than 25mmHg at rest, or greater than 30mmHg with exercise.

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CorPulmonale It is an enlargement of the right ventricle due to derangements in the structure or function of the respiratory system. Cor pulmonale is a complication of pulmonary hypertension.

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Clinical Classi?cation of Pulmonary Hypertension Group 1. Pulmonary arterial hypertension (PAH) Idiopathic PAH Familial PAH Associated with (APAH): Collagen vascular disease Congenital systemic to pulmonary shunts (large, small, repaired, or non repaired) Portal hypertension HIV infection Drugs and toxins Other (glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies,myeloproliferative disorders, splenectomy) Associated with signi?cant venous or capillary involvement Pulmonary veno-occlusive disease Pulmonary capillary hemangiomatosis

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Clinical Classi?cation of Pulmonary Hypertension Group 2. Pulmonary venous hypertension Left-sided atrial or ventricular heart disease Left-sided valvular heart disease Group 3. Pulmonary hypertension associated with hypoxemia COPD Interstitial lung disease Sleep-disordered breathing Alveolar hypoventilation disorders Chronic exposure to high altitude

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Clinical Classi?cation of Pulmonary Hypertension Group 4. Pulmonary hypertension due to chronic thrombotic and/or embolic disease Thromboembolic obstruction of proximal pulmonary arteries Thromboembolic obstruction of distal pulmonary arteries Pulmonary embolism (tumor, parasites, foreign material) Group 5.Miscellaneous Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, ?brosing mediastinitis)

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Functional Classi?cation of Pulmonary hypertention Class I: Patients with PH but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near syncope Class II: Patients with PH resulting in slight limitation of physical activity. They are comfortable at rest.Ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near syncope.

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Functional Classi?cation of Pulmonary hypertention Class III: Patients with PH resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnea or fatigue, chest pain, or near syncope. Class IV: Patients with PH with inability to carry out any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may even be present at rest

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Risk Factors of Pulmonary Hypertension Drugs and toxins De?nite Aminorex Fen?uramine Dexfen?uramine Toxic rapeseed oil Very likely Amphetamines L-Tryptophan Possible Meta-amphetamines Cocaine Chemotherapeutic agents Unlikely Antidepressants Oral contraceptives Estrogen therapy Cigarette smoking

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Risk Factors of Pulmonary Hypertension Demographic and medical conditions De?nite Gender Possible Pregnancy Systemic hypertension Unlikely Obesity

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Risk Factors ofPulmonary Hypertension Diseases De?nite HIV infection Very likely Portal hypertension/liver disease Collagen vascular diseases Congenital systemic-pulmonary-cardiac shunts Possible Thyroid disorders

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Proposed pathogenesis of PAH.

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FIGURE Pulmonary arteriole showing the medial hypertrophy and concentric intimal narrowing that occur as a result of vascular remodeling in pulmonary arterial hypertension.

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Examination Cyanosis clubbing Raised JVP, with giant V waves Right ventricular heave and tapping apex beat Wide splitting of S2 with loud P2 Murmur of tricuspid regurgitation Hepatomegaly Ascites Peripheral oedema.

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Evaluation of Patients with Pulmonary Hypertension

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Diagnosis Electrocardiogram. Abnormal in85% of patients with established PAH. Right-axis deviation with evidence of right ventricular and/or right atrial hypertrophy and right ventricular strain.

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Chest Radiography Shows abnormalities in more than 90% of patients with idiopathic PAH. Prominence of the main pulmonary arteries (90%) Enlargement of the hilar vessels (80%) Peripheral pruning (51%)

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FIGURE Chest radiograph in pulmonary arterial hypertension. Enlargement of the proximal pulmonary arteries .

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FIGURE Chest radiograph in pulmonary arterial hypertension. Enlargement of the proximal pulmonary arteries .

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High-Resolution Computed Tomography. exclude parenchymal lung disease as the cause of the PAH. HRCT is particularly important in the diagnosis of PVOD.

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Continuous Volume (Spiral) CT Pulmonary Angiography Pulmonary angiography is considered the “gold standard” for the investigation of patients with CTEPH

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Spiral computed tomography with contrast. A, Massive filling defect in the main pulmonary artery (arrow). B, Filling defects in lobar (large arrow) and segmental (small arrows) pulmonary arteries

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Spiral CT scan (2-mm collimation, pitch of 2, 24% iodinated contrast agent) obtained at the level of the right upper lobe in a 65-year-old patient with acute onset of dyspnea. Remy-Jardin M , Remy J Radiology 1999;212:615-636 ©1999 by Radiological Society of North America

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Pulmonary angiograms. A, Normal and, B, in pulmonary arterial hypertension, with marked pruning of peripheral vasculature.

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Echocardiography. The screening tool of choice for PAH. LV dysfunction/valve abnormalities Intracardiac shunt RVSP and RH function

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Severe right chamber dilation

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Ventilation and Perfusion ( V/Q) Lung Scanning FIGURE Normal ventilation scans. C and D, Multiple segmental (or larger) defects oncorresponding perfusion scans.

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Cardiac Magnetic Resonance Imaging (MRI) Most accurate than echocardiography . Accurate estimates of ventricular size and mass, as well as pulmonary blood flow. Cardiac MRI is now used to monitor disease-targeted therapy

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Biomarkers Brain natriuretic peptide (BNP) Elevated in idiopathic PAH and associated with interstitial lung disease, COPD, congenital heart disease, CTEPH, and scleroderma.

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Evaluation of Functional Class and Severity of PAH 6MWT. Cardiopulmonary Exercise Testing (CPET). Right Heart Catheterization

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Management

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General management. Education Long-term oxygen Diuretics and digoxin Anticoagulation

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vasodilator therapy Vasodilator responsiveness. Responders non-responders

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CalciumChannel Blockers CCBs are used in higher doses then systemic hypertention and coronary heart disease. 240 mg/day of nifedipine or 900 mg/day of diltiazem can be tolerated with careful monitoring. Verapamil has significant negative inotropic effects and should be avoided

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Disease-Targeted Therapies Prostacyclin (and analogs) Endothelin receptor antagonists Phosphodiesterase inhibitors

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Prostacyclin and Analogs PAH have deficient production of prostacyclin. very short circulating half-life, a continuous intravenous infusion is necessary, Epoprostenol (Flolan)., Treprostinil, Iloprost

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Endothelin Receptor Antagonist A Bosentan novel oral endothelin receptor A and B antagonist. Sitaxsentan,an ET-A receptor antagonist recently improved for class III patients.

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Phosphodiesterase Inhibitors Inhibition of type 5 PDEs with selective drugs, such as sildenafil, increase cyclic GMP and induce a fall in intracellular calcium concentration with consequent vasodilatation.

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Surgical treatments Balloon Atrial Septostomy . Lung Transplantation

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Pulmonary Arterial H...

TREATMENT OF HYPERTE...

Pulmonary Function T...

Hypertension

Recent Developments ...

Heart Disease, Hyper...

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