Prognosis for Pulmonary Arterial Hypertension

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Median survival after diagnosis of patients with idiopathic pulmonary arterial hypertension is 2.8 years without any treatment. The leading causes of death are right heart failure (63%), sudden death (7%) and infections such as pneumonia (7%).    

On the other hand, good treatment follow-up ensures a better evolution of the disease, which is why the patient should commit to the therapy and be aware of the medications to take, as well as their dosage and adverse effects. Follow-up requires a multidisciplinary team at the referral centre and good coordination with the local unit or primary care.

The main complications of drug treatment are:  

  • Endothelin receptor antagonists. Alteration of liver enzymes, fluid retention and risk of hypotension (low blood pressure).  
  • Phosphodiesterase-5 inhibitors. Headaches and discomfort in the pit of the stomach in the form of gastritis (inflammation).
  • Prostacyclins. Hypotension, lower jaw pain, joint pain, diarrhoea or constipation, abdominal cramping pain.  
  • Parenteral prostanoids (injected, not oral). According to the administration system, the main adverse effects are related to the malfunction, infection or obstruction of the catheter. For iloprost, coughing is the main effect related to inhalation. For treprostinil, pain at the infusion site may cause treatment to be discontinued.

Heart failure is often an acute complication. The right side of the heart in a patient with pulmonary hypertension increases in size and becomes insufficient to pump blood to the lungs. To compensate for this and increase the blood flow, the heart increases the size of the walls of the right ventricle and the right atrium. This overload, however, is a temporary measure, because it can end up causing the heart to fail.  

Sudden symptoms of heart failure can be due to increased physical activity, increased fluid and salt intake, fever, infections, anaemia, irregular heartbeats (arrhythmias), overactive thyroid gland and even kidney disease.  

The signs and symptoms of this heart failure are:  

-    Swelling of the ankles and legs due to fluid accumulation (malleolar and/or lower extremity oedema).  

-    Increased nocturnal urination.  

-    Marked prominence of the jugular vein at the level of the neck (jugular engorgement).  

-    Palpitations.  

-    Irregular heartbeat.  

-    Fatigue, weakness, tiredness.  

-    Fainting or dizziness.  

-    Sudden weight gain.  

-    Enlargement of the liver.

The main chronic complication of pulmonary hypertension is heart failure. The disease has no cure, but many episodes of heart failure can be controlled with medication and the correction of the disorder producing it. It can turn into a chronic disease and become worse with infections or other factors.  

Cardiac function can also worsen if the person stops taking the medications needed to control the underlying disease, or due to the natural progression of the disease.

Substantiated information by:

Isabel Blanco Vich
Joan A. Barberà Mir

Published: 23 November 2021
Updated: 1 December 2021

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