Pulmonary Hypertension

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WHO Classification of Pulmonary Hypertension
Group I. 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 venoocclusive disease, 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 Diseases or Both thromboembolic obstruction of proximal pulmonary arteries, thromboembolic obstruction of distal pulmonary arteries, pulmonary embolism (tumor, parasites, foreign material)
Group V. Mi8scellaneous 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 measured 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, electrolytes, urea, Cr, AST, ALT, ALP, bilirubin, 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 Catherization
Management
Symptoms of Control O2, calcium channel blockers if positive vasoreactivity test (high doses), vasodilators (prostacyclin, sildenafil, bosentan, NO), anticoagulation
Treatment Treatments include conventional medical therapies and oral, Depending on the severity of PH, heart or lung transplant may also be an option
Atrial Septostomy
Lung Transplant
Specific Entities
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

Comments

  1. Billy Hall28 November 2020 at 10:51

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