Pulmonary Hypertension Flashcards
What is meant by pulmonary hypertension?
Pulmonary hypertension (PHTN) is defined as a resting mean pulmonary
artery pressure (mPAP) of 20 mm Hg or greater measured during right
heart catheterization.
What is the normal mPAP?
The normal mean pulmonary artery pressure is less than 20 mm Hg.
Untreated pulmonary hypertension leads to?
right ventricular failure and may
directly contribute to death.
pulmonary HTN is grouped into 5 categories and classifed based on similarities in mechanisms, hemodynamics, clinical presentation, and
approach to treatment?
- Pulmonary Arterial Hypertension
- Pulmonary Hypertension due to left heart disease
- Pulmonary Hypertension due to lung disease
- Pulmonary Hypertension due to blood clots in the lungs
- Blood and other rare disorders that lead to Pulmonary Hypertension
What is group 1 in pulmonary hypertension?
Pulmonary Arterial Hypertension (Group 1) pre-capillary
PAH specifically refers to this group. Diagnosis requires a heart catheterization and a battery of other tests to exclude left heart disease, lung disease and other rare disorders. We subdivide group 1 into four smaller groups.
PAH
- Idiopathic PAH
- Heritable (genetic causes that may be passed to your children)
a. BMPR2 (the most common cause of PAH that runs in families)
b. ALK1, endoglin, SMAD9, CAV1, KCNK3 (less common genetic causes of PAH that runs in families)
- Drug and Toxin induced
a. Amphetamines and Methamphetamines
b. Cocaine
c. Fenfluramine-Phentermine (prescription diet pills sold in the 1980’s and 1990’s)
- Associated with other systemic diseases
a. Connective tissue diseases such as scleroderma, systemic lupus erythematosis, mixed connective tissue disease and rheumatoid arthritis
b. HIV infection
c. Portal hypertension (a type of liver disease that leads to high pressures within the liver)
d. Congenital heart disease..
Also, These are due to they are responsive to treatment of type 1
1- pulmonary veno-occlusive disease (PVOD) and
2-pulmonary capillary hemangiomatosis (PCH).
What is the group 2 of pulmonary hypertension?
Pulmonary Hypertension due to Left Heart Disease (Group 2):
this group of disorders is characterized by problems on the left side of the heart. The pulmonary arteries are normal initially.
- Left ventricular systolic dysfunction (the squeeze of the left ventricle is impaired)
- Left ventricular diastolic dysfunction (stiffness of the left ventricle leads to pressure elevation in the pulmonary arteries)
- Left sided Valvular heart disease (AS, MS)
- Left heart inflow and outflow obstructions not due to valvular disease and congenital cardiomyopathies (problems with blood flowing into or out of the left side of the heart not due to a valve problem)
5- HFpEF or HFrEF
What is the group 3 of pulmonary hypertension?
Pulmonary Hypertension due to Lung Disease( hypoxia) (Group 3):
This large and diverse group of diseases share in common that the primary problem is within the lungs or the control of lung function
- Chronic obstructive pulmonary disease (COPD)
- Interstitial lung diseases (scarring and inflammation in the lungs)
- Sleep-disordered breathing (sleep apnea)
- Alveolar hypoventilation disorders (diseases that lead to inadequate breathing and increased levels of carbon dioxide in the blood)
- Chronic high altitude exposure
- Developmental abnormalities of the lung
7-mixed restrictive and obstructive lung disease like Lymphangioleiomyomatosis
What is the group 4 of PULMONARY HYPERTENSION?
Chronic Thromboembolic Pulmonary Hypertension (CTEPH) (Group 4):
1- chronic PE.
2- other pulmonary artery obstruction ( tumours, parasites, congenital stenosis).
3- thromboembolic obstruction of proximal pulmonary arteries.
What is the group 5 of PULMONARY HYPERTENSION?
Pulmonary Hypertension with Unclear or Multifactorial Mechanisms (Group 5)
There are a handful of rare disorders that lead to pulmonary hypertension. For most of the diseases in this group, the mechanisms of PH are poorly understood.
- Hematologic Disorders (sickle cell)
- Systemic Disorders that have lung involvement (Sarcoidosis, Langerhan cell histiocytosis, neurofibromatosis, vasculitis, and lymphangioleimyomatosis)
- Metabolic Disorders (rare diseases of impaired cell metabolism, thyroid disease)
- Other diseases not well classified elsewhere (such as chronic renal failure, extrinsic tumours obstructing the pulmonary arteries and other rare diseases)
What the signs and symptoms of pulmonary hypertension?
1- Dyspnea, exertional syncope (hypoxia, ↓ CO), exertional chest pain (RV ischemia).
2-Symptoms of right sided HF (eg,
peripheral edema, RUQ fullness,
abdominal distention).
3- By examination: Prominent P2, right
sided S4, RV heave, pulmonary
regurgitation (Graham Steell),
Tricuspid regurgitation.
4-Signs of RV failure: ↑ JVP,
hepatomegaly, ascites, peripheral
edema and sacral edema.
What is Needed Investigations
In pulmonary hypertension?
1-ECG: RAD(right axis deviation) , RAE( right atrial enlargement) , RVH, RBBB.
2- CXR: enlarged central pulmonary arteries, right ventricular enlargement or
peripheral hypovascularity.
3- PFT: DLCO usually reduced; volumes and flows normal.
4- ECHO (initial diagnostic modality): doppler assessment of right ventricular
systolic pressure.
5- Right heart catheterization: to confirm diagnosis through direct measurement of
mean pulmonary arterial pressure, pulmonary capillary wedge pressure,
pulmonary vascular resistance, and cardiac output.
6- CTA vs V/Q scan ± pulmonary angiogram to rule out thromboembolic disease.
7- Labs work to rule out underlying etiology.
What is the initial diagnostic modality for pulmonary hypertension?
Echo
Hot confirm the diagnosis of pulmonary hypertension with suspected chronic thromboembolic pulmonary hypertension( CTEPH)?
If there is v/q scan it’s the answer because it can detect small occlusion.
What is the treatment of group 2,3 and 5?
Therapy for patients with groups 2, 3, and 5 PH is directed at the underlying condition( treat the underlying disease)
What is the management of pulmonary hypertension.
1-General Principles:
Prevent & reverse vasoactive substance imbalance and vascular remodeling.
Prevent RV failure: ↓ wall stress (↓ PVR, PAP, RV diam); ensure adequate cardiac output.
2- Supportive measures:
Oxygen: maintain SaO2 >90–92% (reduces vasoconstriction).
Diuretics: ↓ RV wall stress and relieve RHF symptoms.
Digoxin: control AF, ? counteract negative inotropic effects CCB.
Anticoagulants: not routinely used; ?↓ VTE risk of Right HF, (AF).
Supervised exercise training.
