Pulmonary Hypertension

Question 1

Phtn

Answer 1

Pulmonary Hypertension (PH) is a condition characterized by high blood pressure in the arteries of the lungs, leading to strain on the right side of the heart. This condition can be life-threatening if left untreated.

Question 2

Types

Answer 2

Types

PH is classified into five groups based on the underlying cause:

1. Pulmonary Arterial Hypertension (PAH) - caused by narrowing or stiffening of the small pulmonary arteries.
2. PH due to Left Heart Disease - related to heart conditions such as left ventricular failure or valvular disease.
3. PH due to Lung Diseases or Hypoxia - associated with chronic obstructive pulmonary disease (COPD), interstitial lung disease, or sleep apnea.
4. Chronic Thromboembolic Pulmonary Hypertension (CTEPH) - caused by blood clots in the pulmonary arteries.
5. PH with Unclear or Multifactorial Mechanisms - includes conditions like sarcoidosis or hematological disorders.

Question 3

Symptoms

Answer 3

Symptoms

Shortness of breath, especially during exercise.

Fatigue.

Chest pain or pressure.

Dizziness or fainting (syncope).

Swelling in the ankles, legs, or abdomen (edema).

Rapid heartbeat or palpitations.

Cyanosis (bluish discoloration of lips or skin).

Question 4

Causes

Answer 4

Causes

Genetic mutations (inherited PAH).

Connective tissue diseases (e.g., scleroderma, lupus).

Congenital heart defects.

Chronic lung diseases like COPD.

Blood clots in the lungs (pulmonary embolism).

Liver disease (portal hypertension).

Drug or toxin exposure (e.g., appetite suppressants, methamphetamines).

Question 5

Diagonosis

Answer 5

Diagnosis

1. Clinical History and Physical Examination:

Listening for heart murmurs or signs of heart failure.

2. Diagnostic Tests:

Echocardiography: Initial test to estimate pulmonary artery pressure.

Right Heart Catheterization: Gold standard for confirming diagnosis and measuring pressures.

Chest X-ray: Enlarged pulmonary arteries or heart.

Electrocardiogram (ECG): Right heart strain patterns.

Pulmonary Function Tests and CT scans: Evaluate lung function and structure.

Ventilation-Perfusion (V/Q) Scan: Screen for chronic clots.

Question 6

Treatment

Answer 6

Treatment

Treatment depends on the cause and type of PH:

1. Medications:

Vasodilators (e.g., prostacyclins, endothelin receptor antagonists).

Phosphodiesterase-5 Inhibitors (e.g., sildenafil, tadalafil).

Anticoagulants (for CTEPH or risk of clots).

Diuretics (to reduce fluid overload).

Oxygen therapy (for hypoxemia).

2. Lifestyle Modifications:

Low-sodium diet.

Avoiding strenuous physical activity.

Quitting smoking.

3. Surgical Options:

Balloon Atrial Septostomy: Palliative procedure to reduce right heart pressure.

Lung Transplantation: For severe, refractory cases.

Pulmonary Endarterectomy (PEA): For CTEPH.