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Pulmonary Hypertension
Learning Objectives: 1‐Definition of pulmonary hypertension 2‐Mechanism & etiology of pulmonary hypertension 3‐ Complications of pulmonary hypertension 4‐Diagnosis of pulmonary hypertension( Clinical &Investigations) 5‐Lines of therapy of pulmonary hypertension Definition: PA systolic pressure >30 mmHg Mean PAP >20 mmHg Etiology (Types): I. Primary (Idiopathic = essential) pulmonary hypertension: The exact etiology is not known, however there are many theories: a) Recurrent occult systemic venous thrombosis with pulmonary embolism. b) Thrombosis in situ. c) Congenital persistent fetal pulmonary arterial architecture. d) Familial primary pulmonary hypertension (autosomal dominant inheritance). e) Vasculitis. II. Secondary pulmonary hypertension: (A) Increased resistance to pulmonary venous drainage: 1.↑ Left ventricular end‐diastolic pressure: a) Left ventricular failure. b) Restrictive cardiomyopathy. c) Constrictive pericarditis. 2. Left atrial hypertension: a) Mitral valve disease. b) LA myxoma. 3. Pulmonary venous obstruction: a) Congenital stenosis of pulmonary veins. b) Mediastinal fibrosis. (B) Increased resistance to flow through pulmonary vascular bed: 1. Decreased cross sectional area of pulmonary vascular bed:
a) Chronic obstructive pulmonary disease. b) Restrictive lung disease.
c) Congenital pulmonary hypoplasia. d) Eisenminger syndrome.
2.Persistent fetal circulation in newborn. 3.Schistosomiasis. (C) Increased resistance to flow through large pulmonary arteries: 1. Pulmonary thromboembolism. 2. Unilateral absence or stenosis of pulmonary artery. (D) Hypoventilation: 1. Obesity hypoventilation syndrome. 2. Sleep apnoea hypoventilation. 3. Neuromuscular disorders.
Effects of pulmonary hypertension 1. Decrease cardiac output (low fixed COP). 2. Dilatation of pulmonary artery and its main branches. 3. Right ventricular hypertrophy. 4. Right ventricular failure. Clinical features: Symptoms: 1. Dyspnoea. 2. Easy fatigue. 3. Effort syncope. 4. Angina. Signs: I. Low cop→ a) Small pulse volume, tachycardia. b) Pale cold extremities. c) Peripheral cyanosis. II. Pulmonary arterial dilatation and hypertension → a) Pulsations and dullness in second left intercostal space. b) Diastolic shock. c) Second H.S. = close splitting, loud P2 d) Ejection systolic murmur and systolic pulmonary ejection click. e) Graham steel murmur. III. Hypertrophy and dilatation of right ventricle and right atrium: a) Left parastemal lift. b) Epigastric pulsations. c) Dullness to right of sternum.
d) RV.S4
IV. Right ventricular failure: a) Increase jugular venous pressure. b) Enlarged tender liver. c) Edema of lower limb. d) Tricuspid incompetence. Pulmonary plain radiography: 1) Dilatation of main pulmonary artery and its proximal branches. 2) Narrowing of peripheral branches of pulmonary arteries which become invisible in peripheral one third
of lung fields. 3) Signs of right ventricular hypertrophy. 4) Right atrial dilatation. Electrocardiogram 1. Clockwise rotation. 2. P‐pulmonale. 3. Right ventricular hypertrophy.
Treatment of pulmonary hypertension
I. Remove cause when possible: 1. Surgical correction of mitral stenosis. 2. Closure of cardiac shunt.
II. Pulmonary vasodilators: 1. Vasodilators: Hydralazine, Nitroglycerine. 2. Diazoxide. 3. Calcium channel blockers :Nifedipine, Verapamil. 4. Prostacyclin therapy : Intravenous prostacyclin 5. Endothelin‐receptor antagonists 6‐Sildenafil III. Anticoagulants For recurrent pulmonary embolism or pulmonary veno‐occlusive disease. IV. Heart‐Lung Transplantation: For irreversible pulmonary vascular disease and primary pulmonary hypertension.
Cor‐ pulmonale
Learning Objectives: 1‐ Definition of Cor‐pulmonale 2‐ Etiological classification of Cor pulmonale 3‐ Differentiation between Vascular & hypoxic Cor pulmonale Definition: Right ventricular hypertrophy or dilatation secondary to pulmonary hypertension resulting from diseases affecting lung vessels or lung parenchyma and not from left heart lesions. Causes and types: I. Acute cor pulmonale: 1. Massive pulmonary embolism. 2. Extensive lung disease:
a) Spontaneous pneumothorax. b) Massive atelectasis.
3. Acute spontaneous mediastinal emphysema. II. Subacute cor pulmonale: 1. Recurrent showers of minute pulmonary embolization. 2. Lymphangitic carcinomatosis of lung. 3. Collagen vascular disorders (systemic lupus erythmatosis, polyarteritis nodosa). III. Chronic cor pulmonale: (A) Vascular cor pulmonale: (non hypoxic) 1. Schistosomal cor pulmonale. 2. Primary pulmonary hypertension. (B) Parenchymatous cor pulmonale (Hypoxic) 1. Obstructive airway disease. 2. Extensive lung fibrosis. 3. Bronchiectasis. 4. Interstitial fibrosis of the lung. 5. Defective movement of chest wall. Vascular cor pulmonale
(e.g. schistosomiasis) Parenchymatous cor pulmonale (e.g. emphysema)
Age Young adult Old Complaint G.I.T or urinary symptoms
of schistosomal infection Chest symptoms (Dyspnoea, cough)
Examination 1.Cyanosis &Clubbing Rare Always present 2.Chest Quite, free Hyperinflated, restricted
movement 3. Heart a) R.V. HYPERTROPHY b) H.S2 c) GRAHAM STEEL MURMUR d) Tricuspid regurge e) PA DILATATION f) Pulm. Hypertesion
Severe and obvious Loud, closely split Common Common ++ ++, static not affected by O2
Masked by emphysema Faint Very rare Very rare + +, decrease by 100% O2
4. X‐ ray Lung is ischemic (Autum tree appearance)
Ischemic at periphery
5. ECG Pulm. Hypertension ,RVH Less marked 6. Blood gases Normal PO2, CO2 ↓PO2, ↑CO2 7. Prognosis
(heart failure) Late, irreversible Early, recurrent partially
reversible