Cardiology overview pulmonary hypertension
|
Cardiology Overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Causes
- In general, obstructive sleep apnea does not cause sufficiently elevated pulmonary pressures to cause PAH
(In Alphabetical Order)
- High Altitude(chronically)
- Alveolar capillary dysplasia with misalignment of pulmonary veins
- Atrial Septal Defects
- Bronchiectasis
- Bronchopulmonary dysplasia
- Cholesterol ester storage disease
- Chronic hemolytic anemia
- Chronic obstructive pulmonary disease
- Chronic renal failure on dialysis
- Churg-Strauss syndrome
- Coal workers' pneumoconiosis
- Cor triatriatum
- Cystic fibrosis
- Diastolic dysfunction.
- Diethylpropion
- Fallot tetralogy
- Fetal circulation, persistent
- Fibrosing mediastinitis
- Gaucher disease
- Glycogen storage diseases
- Idiopathic pulmonary haemosiderosis
- Idiopathic spinal scoliosis
- Indian familial childhood cirrhosis
- Interstitial Lung Disease
- Langerhans cell histiocytosis
- Mitral valve insufficiency
- Mitral valve stenosis
- Monocrotaline poisoning
- Myeloproliferative disorders
- Neurofibromatosis
- Obstructive sleep apnea
- Paroxysmal nocturnal haemoglobinuria
- Phentermine poisoning
- Pickwickian syndrome
- Portal hypertension
- Polycythemia vera
- Pulmonary alveolar microlithiasis
- Pulmonary capillary hemangiomatosis
- Pulmonary embolism
- Pulmonary fibrosis
- Pulmonary veno-occlusive disease
- Sarcoidosis
- Schistosoma japonicum
- Schistosoma mansoni
- Sickle cell disease
- Splenectomy
- Systemic lupus erythematosus
- Systolic dysfunction
- Tropical pulmonary eosinophilia
- Vasculitis
- Ventricular septal defect
Diagnosis
- In primary pulmonary hypertension the pulmonary artery diastolic pressure is much higher than the pulmonary capillary wedge pressure.
- If the pulmonary hypertension is due to left-sided heart failure, and the pulmonary artery diastolic pressure in the pulmonary capillary wedge pressure will be similar.
- In the cardiac catheterization laboratory, inhaled nitric oxide is administered to determine if the pulmonary vasculature is still reactive or if the obstructionist fixed.
- It is to get dangerous to give nifedipine IV as a test. This could lead to a dangerous episode of hypotension.
Treatment
Pregnancy
- Patients with pulmonary hypertension should avoid pregnancy
General anesthesia
- Epidural rather than general anesthesia should be administered for surgical procedures given the potential for hypotension
Oxygen
- In-flight oxygen should be considered for those patients who have a resting oxygen pressure less than 60 mm Hg.
Anticoagulation
- Anticoagulation should be considered in patients with pulmonary hypertension in the absence of hemoptysis
- If the pulmonary hypertension is due to chronic thromboembolic pulmonary hypertension, and thrombectomy may be indicated.
Diuretics
- Diuretics are used to treat right ventricular failure and edema
First-Line Therapies
- First-line therapies or type I pulmonary hypertension or world of organization class to pulmonary hypertension include bosentan, ambrisentan and Viagra
Calcium Channel Blockade
- Nifedipine can be used in these patients, but should not be given to patients who are not reactive to inhaled nitric oxide as it will not work in that scenario. Calcium channel blocker should not be used in patients with Eisenmenger syndrome.
Advanced Pulmonary Hypertension
- The prostaglandins can be useful in these patients and include epoprosterol and iloprost.