Pulmonary Hypertension

Pulmonary hypertension is not as well known as systemic hypertension. When people think about Hypertension, what readily comes to mind is Systemic Hypertension, the one that can be diagnosed by tying a cuff around one’s arm, and checking the blood pressure with the aid of a sphygmomanometer. The reason for this is not far-fetched, as systemic hypertension is relatively common, and have far-reaching effects.

The other type of vascular hypertension– Pulmonary Hypertension, doesn’t enjoy the same popularity, maybe because there is as few as 100,000 cases per year, in Nigeria. As a matter of fact, I’m sure that not up to 10% of non-medical personnels reading this have heard of pulmonary hypertension before now. For the medical personnels, only the doctors, and perhaps nurses, may know its full implications. Of these, only a fraction may perhaps be able to recognize it when it presents, especially where its symptoms could mimic that of heart failure, and we don’t have Swan Ganz catheters, ECGs, and Echo– things that could aid speed of diagnosis– lying around in our hosoitals here in Nigeria.

pulmonary hypertension
pulmonary hypertension Credit: Designua/Shutterstock.com

The irony? Pulmonary hypertension is as deadly– heck I dare say even deadlier– than systemic hypertension. While, if untreated, systemic hypertension, on the average, would usually take upto 5 to 10 years, to cause significant organ damage– which may be amenable to treatment, and would not necessarily translate to death; without treatment, the average duration of survival for pulmonary hypertension is 2 to 5 years!

To make matters worse, pulmonary hypertension, like systemic hypertension, has no cure, but unlike systemic hypertension, it has a point of no return: once remodeling of the blood vessels in the lungs takes place, you’re better off sweeping the Sahara. Unfortunately, diagnosis is usually late, especially here in Africa. By the time it is made, the therapy that could improve symptoms and prolong life– such as long term oxygen therapy, advanced vasodilatory therapy, and lung transplantation– is either not available, or too expensive and can’t be afforded by the patient.

Causes of pulmonary Hypertension

The causes are classified into 5 groups:

  1. Pulmonary Arterial Hypertension. Eg includes HIV, connective tissue disease, congenital heart disease, sickle cell disease, etc.
  2. Diseases of the left side of the heart. Eg mitral stenosis.
  3. The disease of the lungs, itself, or hypoxemia. Eg COPD.
  4. Chronic Pulmonary Embolism, which is basically blocking of arteries in the lungs by a blood clot.
  5. Others. This includes myeloproliferative disorders [eg. polycythemia (Rubra) bera], systemic disorders [eg. sarcoidosis], and metabolic disorders [eg. glycogen storage diseases].

Symptoms of pulmonary Hypertension

Symptoms are non-specific, and include:

  • Fatigue
  • Difficulty with breathing, when one exerts himself.
  • Difficulty with breathing, when lying down.
  • Neck pulsation
  • Chest pain
  • Pain in the right-hand corner of the upper tummy, just below the ribcage.
  • Undue awareness of heartbeat: palpitations
  • Leg swelling
  • Fainting episodes, especially when one exercises
  • Rarely: cough, hoarseness of voice, and bleeding into the lungs.

As the symptoms are similar to that of heart failure, and other conditions, it could be mistaken for those conditions. And without ECG, Echo, and Cardiac catheterization– things that are usually not readily available in most hospitals in Nigeria, it may be misdiagnosed as any of these.

Complications of pulmonary Hypertension

  • Respiratory failure
  • Cor pulmonale
  • Failure of the right side of the heart
  • Abnormalities of heart rhythm
  • Increased susceptibility for forming clots in the lungs
  • Polycythemia
  • Sudden death

Treatment of pulmonary Hypertension

  • Oxygen therapy, including long-term supplemental oxygen, is key. If started early, it could even help stall remodelling of the lung vessels, as it eliminates one of the major driving forces–hypoxia.
  • Routine drugs for heart failure, when indicated. Example diuretics– to relieve systemic congestion, in the event of failure of the right heart; and cardiac glycosides– in the event of arrhythmias.
  • Routine vasodilatory medication, for instance, high-dose calcium channel blockers
  • Advanced vasodilatory therapy, using Endothelin inhibitors, Prostacyclin analogues, and Phosphodiesterase-5 inhibitors, such as Sildenafil.
  • Lung transplant for advanced cases not amenable to medical therapy.
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