March Ch. 12 p (459 – 512 PULMONARY DISEASES OF VASCULAR ORIGIN

VASCULAR PULMONARY DISEASES PULMONARY EMBOLISM (usually WITHOUT infarction) PULMONARY HYPERTENSION, leading to cor pulmonale HEMORRHAGIC SYNDROMES –GOODPASTURE SYNDROME –WEGENER GRANULOMATOSIS – HEMOSIDEROSIS, idiopathic

Pulmonary Embolism Usually secondary to debilitated states with immobilization, or following surgery Usually (95%) deep leg and deep pelvic veins (DVT), NOT superficial veins Follows Virchow’s triad.

The effects of pulmonary emboli Depend on: size of the embolus, & the cardiopulmonary status of the patient Usually do NOT infarct, usually ventilate When they DO infarct, the infarct is hemorrhagic Risk of recurrence is high. RX: short term heparin, then long term coumadin

The majority (60% to 80%) of emboli are clinically silent, A minority (5%) cause acute cor pulmonale, shock, or death (typically from large “saddle emboli”), And the remaining cause pulmonary infarction & HT. This depend on the size of the emboli & the state of the lung & the circulation Pulmonary Embolism

This CT scan shows a darkly attenuated saddle pulmonary embolus ( ) with extension into the right pulmonary artery. Brightly attenuated contrast fills the vasculature Pulmonary embolism, CT image

Pulmonary embolism, angiogram A thoracic CT angiogram show pulmonary thromboemboli ( ). There should be contrast material filling pulmonary Ars into the periphery. Risk factors included older age, smoking, & immobilization. Clinical findings include dyspnea, tachypnea, tachycardia, cough, fever, & chest pain

R L Pulmonary embolism, gross; A Saddle embolus that bridges the pulmonary artery trunk as it divides into right and left main pulmonary arteries. A common lab test is an increased plasma D-dimer

Pulmonary infarct, gross

Pulmonary infarcts About ¾ of all infarcts affect the lower lobes, and more than ½ are multiple.  The lungs are oxygenated by; Pulmonary Ars, bronchial Ars, & air in the alveoli. So, infarction occurs (in only 10% of patients with emboli). if there is HF, or low bronchial circulation, or underventilated lung.

Pulmonary embolism, microscopic A small pulmonary artery thromboembolus with hemorrhagic infarct, marked by many RBCs within alveolar spaces. A partial recanalization ( ) of this blocked Ar. Such small embolus probably would not cause dyspnea or pain hemorrhagic infarct

Emboli usually resolve by endogenous fibrinolytic activity. Patients with pulmonary embolism are given anticoagulation therapy & Massive pulmonary embolism are candidates for thrombolytic therapy.

PULMONARY HYPERTENSION COPD, C”I”PD (vicious cycle) CHD (Congenital HD, increased left atrial pressure) Recurrent PEs Autoimmune, e.g., PSS (Scleroderma), i.e., fibrotic pulmonary vasculature

VERY thickened arteriole in pulmonary hypertension NORMAL pulmonary arteriole

HEMORRHAGIC SYNDROMES GOODPASTURE Syndrome: Ab’s to the alpha-3 chains of collagen IV, GBM deposits too! IDIOPATHIC PULMONARY HEMOSIDEROSIS, to be differentiated from chronic CHF WEGENER GRANULOMATOSIS; vasculitis & granulomatous inflammation. ANCA are present 95% of cases

CHF, CHRONIC IDIOPATHIC PULMONARY HEMOSIDEROSIS