Congenital heart defects: Eisenmenger's syndrome

Eisenmenger syndrome, is a rare complication of a congenital heart defect, will affect the hole that connects the heart chambers or major blood vessels

This will result in abnormal circulation between the heart and lung and pulmonary hypertension. If the pressure in the arteries is excessively high, the blood will reverse its flow and oxygen-depleted blood will circulate in the body.

It may also lead to sudden death, especially in pregnant women.

Eisenmenger’s syndrome manifests itself in prepubertal age, but symptoms may also occur in adolescence or adulthood

Life expectancy following diagnosis can range from around 20 to 60 years.

The highest mortality rate occurs in pregnant women, leading to their hospitalisation as early as the 20th week.

Symptoms include difficulty breathing, chest pain, fatigue, exhaustion, drowsiness, syncope, irregular heartbeat, cyanosis, abdominal pain, hippocratic and therefore enlarged fingers, especially the last phalanx, and a cough with blood.

The cyanosis will be a consequence of the low oxygen content of the blood, which will have to produce a greater quantity of red blood corpuscles, with the consequent increased risk of blood clots and resulting pathologies.

Heart failure, cerebral embolisms and arrhythmias causing cardiac arrest, endocarditis and kidney problems may also occur.

As mentioned earlier, one of the main causes of Eisenmenger’s syndrome will be the presence of a shunt, a hole, present from birth, that connects blood vessels or cardiac chambers.

If there is a ventricular septal defect, the hole will connect the two ventricles; if there is an atrial septal defect, the hole will be between the two atria; if there is patency of the ductus arteriosus, there will be communication between the aorta and the pulmonary artery; if there is an atrioventricular canal defect, the hole in the centre of the heart will make all the heart chambers communicate with each other.

If the congenital malformation is discovered before pulmonary hypertension manifests itself, it will be possible to prevent the syndrome through specific surgery.

If, on the other hand, the malformation is discovered when the blood flow has already been reversed, it will be necessary to intervene with a lung and heart transplant.

Pharmacological treatment will only allow a slight reduction in symptoms.

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