Eisenmenger syndrome

Overview

Eisenmenger syndrome arises as a persistent complication of an uncorrected congenital heart defect present from birth, posing a life-threatening condition.

The syndrome is characterized by disrupted blood flow in the heart and lungs, leading to the stiffening and narrowing of blood vessels in the lungs. This, in turn, results in elevated blood pressure in the pulmonary arteries, causing pulmonary arterial hypertension. The persistent impact of Eisenmenger syndrome leads to permanent damage to the lung’s blood vessels.

Timely diagnosis and correction of congenital heart defects typically serve as a preventive measure against the development of Eisenmenger syndrome. In instances where it does manifest, the treatment approach involves regular medical check-ups and the administration of medications aimed at alleviating symptoms.

Symptoms

Signs and symptoms of Eisenmenger syndrome encompass:

• Edema in the abdomen, legs, ankles, or feet
• Headaches
• Heart palpitations
• Dizziness or fainting
• Exhaustion or lethargy
• Coughing up blood
• Chest pain or tightness
• Shortness of breath and tiring during physical exertion
• Shortness of breath during periods of rest
• Skin appearing blue or grayish due to reduced oxygen levels (cyanosis)
• Clubbing wherein the fingernails or toenails becomes large and rounded
• Sensation of numbness or tingling in fingers or toes

If you experience any symptoms of Eisenmenger syndrome, consult with your doctor. Schedule an appointment, even if you have not received a previous diagnosis of a heart issue. Symptoms like shortness of breath or chest pain warrant prompt medical attention.

Causes

Eisenmenger syndrome commonly results from an unaddressed opening (shunt) between the main blood vessels or chambers within the heart. A shunt refers to a congenital heart defect present at birth. To comprehend the impact of Eisenmenger syndrome on the heart and lungs, it is beneficial to grasp the usual functioning of the heart.

The heart’s function

The heart consists of chambers, specifically, two upper chambers known as atria and two lower chambers known as ventricles.

• The right side of the heart transports blood to the lungs via blood vessels, namely the pulmonary arteries.
• Within the lungs, blood acquires oxygen and subsequently travels back to the left side of the heart through the pulmonary veins.
• The left side of the heart then propels the oxygenated blood through the aorta, distributing it to the entire body.

The flow of blood into and out of the heart chambers is regulated by heart valves. These valves open to facilitate the movement of blood to the next chamber or into one of the arteries, and subsequently close to prevent the backward flow of blood.

Eisenmenger syndrome develops

Congenital heart defects, which are heart problems present from birth and can lead to Eisenmenger syndrome, encompass:

• Ventricular septal defect. This represents the predominant cause of Eisenmenger syndrome. It involves the presence of a hole (shunt) in the tissue wall that separates the main pumping chambers (ventricles) on the right and left sides of the heart.
• Atrial septal defect. This is a hole in the tissue wall that separates the upper chambers of the heart (atria) into right and left sides.
• Patent ductus arteriosus. This is a passage connecting the pulmonary artery, which transports oxygen-poor blood to the lungs, with the artery responsible for carrying oxygen-rich blood to the rest of the body (aorta).
• Atrioventricular canal defect. This refers to a significant hole located at the heart’s center, where the walls between the upper chambers (atria) and lower chambers (ventricles) intersect. Additionally, there may be issues with the proper functioning of certain heart valves.

In these heart conditions, blood follows an unusual flow pattern. Consequently, there is an elevation in pressure within the pulmonary artery. Over time, this heightened pressure leads to the damage of the smaller blood vessels in the lungs. The impaired blood vessel walls impede the efficient pumping of blood to the lungs.

In Eisenmenger syndrome, there is an elevation in blood pressure on the side of the heart carrying oxygen-poor blood (blue blood). The blue blood traverses through the hole (shunt) in the heart or blood vessels, resulting in the mingling of oxygen-rich and oxygen-poor blood. This mixing leads to diminished levels of oxygen in the bloodstream.

Risk factors

A familial background of congenital heart defects amplifies the likelihood of similar cardiac issues in an infant. If you have received a diagnosis of Eisenmenger syndrome, discuss with your doctor the prospect of screening other family members for congenital heart defects.