Pulmonary Hypertension

-
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

Post a Comment

Popular posts from this blog

Pleural Effusion

-
Image
Pleural effusion , sometimes referred to as “water on the lungs,” is the build-up of excess  fluid  between the layers of the pleura  outside the lungs. The  pleura  are thin membranes that line the lungs and the inside of the chest cavity and act to lubricate and facilitate breathing. Differential Diagnosis Exudative malignancy, infections, connective tissue disease, pulmonary embolism, hemothorax, pancreatitis, chylothorax Trabsydatuve HF, hypoalbuminemia (GI losing enteropathy, cirrhosis, nephrotic syndrome, malnutrition), SVC obstruction, hepatohydrothorax, urinothorax, atelectasis, trapped lung, peritoneal dialysis, hypothyroidism, pulmonary embolism Note: pulmonary embolism, malignancy, and sar coidosis can present as either exudative or transudative effusions. HF following diuresis may become ‘‘pseudo exudative’’ (check albumin gradient) Clinical Features History dyspnea, cough, hemoptysis, chest pain, weight loss, fever, trauma, occup...
Read more

Pulmonary Embolism

-
Image
Pulmonary embolism  is the sudden blockage of a major blood vessel (artery) in the  lung , usually by a blood clot. In most cases, the clots are small and are not deadly, but they can damage the  lung . But if the clot is large and stops blood flow to the  lung , it can be deadly. Differential diagnosis of acute Dyspnea Respiratory Airway COPD exacerbation, asthma exacerbation, acute bronchitis, infectious exacerbation of bronchiectasis, foreign body obstruction Parenchyma pneumonia, cryptogenic organizing pneumonia, ARDS, acute exacerbation of interstitial lung disease Vascular pulmonary embolism, pulmonary hypertension Pleural pneumothorax, pleural effusion Cardiac Myocardial HF exacerbation, myocardial infarction Valvular aortic stenosis, acute aortic regurgitation, endocarditis Pericardial pericardial effusion, Tamponade Systemic sepsis, metabolic acidosis, anemia Others neuromuscular, psychogenic, anxiety Pathophysiology Virchow’...
Read more

FNAC of Salivary Gland Tumors

-
Image
Fine Needle Aspiration Cytology (FNAC) of Salivary Gland Tumors Introduction: Salivary gland neoplasms though relatively uncommon are by no means rare, accounting for 3 % of all head and neck tumors. Distribution wise 80% of the tumors occur in parotid gland, 15% in submandibular gland and less than 1% in sublingual glands. The World Health Organization (WHO) has classified the tumors of salivary gland as primary or secondary, malignant or benign and by the tissue of origin. The five broad – categories of salivary gland tumors are: Malignant tumors: e.g. Mucoepidermoid Carcinoma, Acinic cell Carcinoma and Adenoid Cystic Carcinoma, Benign tumors: e.g. Pleomorphic Adenoma, Warthin’s tumor, Myoepithelioma, Soft Tissue tumors: e.g. Hemangioma, Hematolymphoid tumors: e.g. Hodgkin’s Lymphoma & Secondary tumors. Of the malignant tumors Mucoepidermoid Carcinoma is the most common neoplasm occurring most frequently in the parotid gland. Exposure to radiation has been thought to play or...
Read more