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RespiRatoRy  ` RESPIRATORY—PAThOlOgY                     RespiRatoRy  ` RESPIRATORY—PAThOlOgY         seCtioN iii      679




                  Sleep apnea            Repeated cessation of breathing > 10 seconds during sleep Ž disrupted sleep Ž daytime
                                          somnolence. Diagnosis confirmed by sleep study.
                                         Nocturnal hypoxia Ž systemic/pulmonary hypertension, arrhythmias (atrial fibrillation/flutter),
                                          sudden death.
                                         Hypoxia Ž  EPO release Ž  erythropoiesis.
                   Obstructive sleep     Respiratory effort against airway obstruction. Normal Pao  during the day. Associated with obesity,
                                                                                        2
                    apnea                 loud snoring, daytime sleepiness. Caused by excess parapharyngeal tissue in adults, adenotonsillar
                                          hypertrophy in children. Treatment: weight loss, CPAP, dental devices.
                   Central sleep apnea   Impaired respiratory effort due to CNS injury/toxicity, HF, opioids. May be associated with
                                          Cheyne-Stokes respirations (oscillations between apnea and hyperpnea). Think 3 C’s:  Congestive
                                          HF, CNS toxicity, Cheyne-Stokes respirations. Treat with positive airway pressure.


                   Obesity               Obesity (BMI ≥ 30 kg/m ) Ž hypoventilation Ž  Paco  during waking hours (retention);  Pao
                                                             2
                                                                                      2
                                                                                                                      2
                    hypoventilation       and  Paco  during sleep. Also known as Pickwickian syndrome.
                                                   2
                    syndrome
                  Pulmonary             Normal mean pulmonary artery pressure = 10–14 mm Hg; pulmonary hypertension ≥ 25 mm Hg
                  hypertension           at rest. Results in arteriosclerosis, medial hypertrophy, intimal fibrosis of pulmonary arteries,
                                         plexiform lesions. Course: severe respiratory distress Ž cyanosis and RVH Ž death from
                                         decompensated cor pulmonale.
                   ETIOlOgIES
                   Pulmonary arterial    Often idiopathic. Heritable PAH can be due to an inactivating mutation in BMPR2 gene (normally
                    hypertension          inhibits vascular smooth muscle proliferation); poor prognosis. Pulmonary vasculature endothelial
                                          dysfunction results in  vasoconstrictors (eg, endothelin) and  vasodilators (eg, NO and
                                          prostacyclins).
                                         Other causes include drugs (eg, amphetamines, cocaine), connective tissue disease, HIV infection,
                                          portal hypertension, congenital heart disease, schistosomiasis.
                   Left heart disease    Causes include systolic/diastolic dysfunction and valvular disease.
                   Lung diseases or      Destruction of lung parenchyma (eg, COPD), lung inflammation/fibrosis (eg, interstitial lung
                    hypoxia               diseases), hypoxemic vasoconstriction (eg, obstructive sleep apnea, living in high altitude).
                   Chronic               Recurrent microthrombi Ž  cross-sectional area of pulmonary vascular bed.
                    thromboembolic
                   Multifactorial        Causes include hematologic, systemic, and metabolic disorders, along with compression of the
                                          pulmonary vasculature by a tumor.
































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