Pulmonary Hypertension

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WHO Classification of Pulmonary hypertension
Group 1: Pulmonary arterial hypertension
Idiopathic primary
Familial and related disorders collagen vascular disease, congenital systemic to pulmonary shunts, portal hypertension, HIV, drugs and toxins, thyroid disorders, glycogen storage disease, Gaucher’s disease, hereditary hemorrhagic telangiectasia, hemoglobinopa thies, myeloproliferative disorders, splenectomy.
Associated with significant venous or capillary involvement pulmonary veno occlusive diease, pulmonary capillary hemangiomatosis. Persistent pulmonary hypertension of newborn group II.  Pulmonary venous hypertension left sided atrial or ventricular heart disease, left sided valvular heart disease.
Group III. Pulmonary hypertension associated with hypoxemia COPD, interstitial lung disease, sleep disordered breathing, alveolar hypoventilation disorders, chronic exposure to high altitude, developmental abnormalities.
Group IV. Pulmonary hypertension due to chronic thrombotic disease, Embolic disease or both: Thromboembolic obstruction of proximal pulmonary arteries, thromboembolic obstruction of distal pulmonary arteries, pulmonary embolism (tumor, parasites, foreign material).
Group V. Miscellaneous sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangiomatosis, compression of pulmonary vessels (adenopathy, tumor, fibrosing mediastinitis).


Pathophysiology
Definition of pulmonary hypertension mean pulmonary arterial pressure (PAP) >25 mmHg at rest or mean PAP >30 mmHg with exercise mea sured with right heart catheterization
Clinical Features
History unexplained dyspnea on exertion, cough, chest pain, hemoptysis, dizziness, syncope, hoarseness, past medical history (cardiac and respiratory diseases, thromboembolic diseases, HIV, cirrhosis, autoimmune and rheumatologic dis orders), medications (amphetamine, diet pill such as dexfenfluramine)
Physical vitals (tachypnea, tachycardia, atrial fibrillation, hypoxemia), peripheral cyanosis, small pulse volume, elevated JVP (prominent a wave or absent if atrial fibrillation, large v wave), right ventri cular heave, palpable P2, narrowly split or paradoxi cally split S2, right sided S4, tricuspid regurgitation  murmur, Graham Steell murmur (high pitched, decrescendo diastolic rumble over LUSB), crackles, congestive liver, ascites, ankle edema
Investigations
Basic
LABS CBCD, lytes, urea, Cr, AST, ALT, ALP, bilir ubin, INR, albumin, ANA, RF, anti CCP, anti SCL 70, anticentromere antibody, ESR, HIV serology, TSH
Imaging CXR, CT chest, V/Q scan or CT chest PE protocol, echocardiogram
ECG
Overnight Polysomnography if suspect OSA
ABG
PFT
Special
Right heart catheterization
Management
Symptom of control O2, calcium channel blockers if positive vasoreactivity test (high doses), vasodilators (prostacyclin, sildenafil, bosentan, NO), anticoagulation
Treatment underlying cause
Atrial septostomy
Lung transplant
Specific Entitis
Eisenmenger Syndrome left to right shunt leading to pulmonary hypertension and eventually right to left shunt
Thyrotoxic associated pulmonary hypertension pulmonary artery hypertension and isolated right sided heart failure are associated with hyperthyroidism. Restoration to a euthyroid state may reverse pulmonary hypertension.

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