A Brief Insight into Pulmonary Hypertension

Pulmonary Hypertension

Most of us are well aware of Hypertension, as it is one of the major non-communicable disease burdening today’s world and is usually defined as blood pressure of more than 140/90mmHg, the values signifying the systolic blood pressure and the diastolic blood pressure respectively. This entity is better termed as Systemic Hypertension.

But at some point of time, you may have come across the term Pulmonary Hypertension. Lets get a brief insight of this disease.

Definition

Pulmonary Hypertension is a rare disease spectrum that occurs due to increased pressures particularly in the lung vasculature. It is defined as mean Pulmonary Artery Pressure of more than 25mmHg or systolic Pulmonary Artery pressure more than 36mmHg.

Causes, Associations and Risk Factors

• Idiopathic, or where no cause can be identified for the disease.
• It may occur as a result of left sided heart disease
• It may be associated with lung diseases like chronic obstructive lung diseases, interstitial lung diseases, obstructive sleep apnoea.
• Chronic thrombosis in lung vasculature or pulmonary embolism.
• Blood disorders like Sickle Cell Anemia, Hemolytic Anemia
• Other risk factors would include Liver Diseases, Connective Tissue Diseases (like systemic lupus erythematosus, scleroderma), infections (like HIV, schistosomiasis), etc.

Clinical Presentation or Symptoms

The disease may initially present with symptoms like fatigue, shortness of breath, chest pain, or syncope.

But as the disease progresses, it leads to right sided heart failure, and the symptoms like edema of feet and ankles, distension of abdomen, swelling of neck veins may become more evident.

Diagnosis

As the disease presents with non-specific symptoms, an absolute clinical diagnosis is not possible.

• One of the most important investigation to screen for the disease is a Cardiac Ultrasound, also known as Echocardiography.
• Lung function tests and chest imaging also serve as tools for diagnosing pulmonary hypertension secondary to lung diseases.
• HRCT (high resolution-CT) may also play a significant role.
• Other supportive investigations include ECG, V/Q (ventilation-perfusion) Scan.
• However, for a definitive diagnosis, invasive techniques for monitoring of the pulmonary pressures is often required. A diagnostic procedure known as Right Heart Catheterization is performed to confirm the diagnosis and assess the severity of the disease.

Management

On diagnosis, the disease warrants treatment as without treatment, the prognosis may not be favourable.

In most of the cases, the treatment of the disease is done by treating the underlying cause of the disease. For example: Oxygen therapy, Diuretics, Anticoagulants, etc.

There is no definitive cure for Pulmonary Hypertension. However, few medications have been approved for the treatment of the disease, which mainly aim at dilating the vessels in the pulmonary vasculature. As per studies, these medications have proven to reduce morbidity, and in some cases, even the mortality.

There are three main group of medications being used:

• Prostanoids
• Endothelin Receptor Antagonists
• Phosphodiesterase V Inhibitors

Complications

• Cor Pulmonale – which is right sided heart failure or enlargement.
• Irregular heartbeats or Arrythmias
• Hemoptysis or coughing up of blood due to bleeding in the lungs
• Thrombosis (formation of blood clots)
• Congestive Liver Disease as a result of right sided heart failure.