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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.
Diagnosis
To identify Eisenmenger syndrome, your healthcare provider conducts a physical examination and inquires about your medical history.
Diagnostic tests for Eisenmenger syndrome may encompass:
- Blood tests. A thorough blood cell count is frequently performed, with a potential elevation in red blood cell count observed in Eisenmenger syndrome. Additionally, blood tests are conducted to assess kidney and liver function, as well as the levels of iron.
- Electrocardiogram (ECG).This rapid and painless examination gauges the electrical activity of the heart. In an ECG, sensors (electrodes) are affixed to the chest, and occasionally to the arms or legs. Wires link the sensors to a machine that displays or prints the results. An ECG can indicate the rate of the heartbeats, revealing whether it is beating at a fast or slow rate.
- Chest X-ray. A chest X-ray aids in revealing the status of the heart and lungs.
- Echocardiogram. An echocardiogram is a heart ultrasound that utilizes sound waves to generate detailed, moving images of the heart. This test provides visual representation of blood flow throughout the heart.
- Computerized Tomography (CT) scan. A CT scan employs X-rays to produce cross-sectional images of targeted body areas. This procedure is performed to obtain additional information about the lungs and lung arteries. Intravenous dye (contrast) may be administered to enhance the visibility of blood vessels in the images. CT scan images offer more comprehensive details compared to conventional X-rays.
- Magnetic resonance imaging (MRI) scan of the lungs. This examination utilizes magnetic fields and radio waves to generate intricate images of the blood vessels within the lungs.
- Cardiac catheterization. A slender, flexible tube known as a catheter is inserted into a blood vessel, typically in the groin or wrist, and guided to the heart. Dye is then injected through the catheter into the arteries, enhancing their visibility on X-ray images and videos. Throughout this procedure, the doctor may measure blood pressure in the blood vessels and chambers of the heart. Additionally, cardiac catheterization can reveal the size of any existing heart defects and provide insights into blood flow in the region.
- Walking test. This exercise tolerance assessment, lasting six minutes, is conducted to evaluate your capacity to withstand a mild level of physical activity.
Treatment
The objectives of treating Eisenmenger syndrome include managing symptoms, enhancing quality of life, and averting severe complications. Upon diagnosis, individuals with Eisenmenger syndrome will be directed to a cardiologist, a specialist in heart conditions. Regular health checkups, scheduled at least annually, constitute a crucial aspect of Eisenmenger syndrome management. It is advisable to seek out a cardiologist with expertise in treating individuals with congenital heart defects.
Medications
Medications serve as the primary approach for treating Eisenmenger syndrome. While they cannot cure the condition, they play a crucial role in enhancing your quality of life. Regular medical checkups are essential when using medications, during which your doctor monitors changes in blood pressure, pulse rate, and fluid levels.
Common medications for Eisenmenger syndrome comprise:
- Heart rhythm drugs. These drugs, known as anti-arrhythmics, assist in regulating heart rhythm and preventing irregular heartbeats.
- Aspirin or blood-thinning medications. In the event of a stroke, blood clot, or specific irregular heart rhythms, your doctor might suggest aspirin or a blood thinner like warfarin. It’s important to note that these medications can elevate the risk of bleeding, so do not take them without your doctor’s guidance. Always seek your doctor’s advice regarding the safety of pain medications, such as ibuprofen.
- Drugs for pulmonary arterial hypertension. A drug named bosentan may be employed to enhance blood supply to the lungs. If you are on bosentan, regular monthly liver tests are necessary due to the potential for liver damage caused by the medication.
- Sildenafil and tadalafil. Sildenafil and tadalafil are occasionally prescribed for managing elevated blood pressure in the pulmonary arteries associated with Eisenmenger syndrome. These medications facilitate the dilation of blood vessels in the lungs, promoting easier blood flow.
- Iron supplements. Your doctor may recommend these if your iron levels are insufficient. Refrain from initiating iron supplementation without consulting your doctor beforehand.
- Antibiotics. Certain dental and medical procedures have the potential to introduce bacteria into the bloodstream. Depending on your health condition, it may be necessary to take antibiotics before undergoing such procedures. This precautionary measure aims to prevent a heart infection, specifically endocarditis. If you have any form of congenital heart disease, it is advisable to discuss your risks with your dentist and other doctors, determining whether preventive antibiotics are warranted.
Surgeries or other procedures
Doctors do not advise surgical intervention to correct the hole in the heart once Eisenmenger syndrome has manifested. Surgical or procedural interventions that might be considered for managing symptoms or complications of Eisenmenger include:
- Phlebotomy or withdrawal of blood. If an elevated red blood cell count leads to symptoms like headaches, difficulty concentrating, or vision problems, your doctor may suggest this procedure. Phlebotomy should not be a routine practice and should only be conducted after consulting with a congenital heart disease specialist. During this procedure, it is advisable to receive intravenous fluids to compensate for the lost fluids.
- Transplant. Individuals with Eisenmenger syndrome may require surgery to replace the heart or lungs if alternative treatments prove ineffective.
When seeking a procedure or surgery for Eisenmenger syndrome, it is advisable to seek care at a medical center where healthcare providers have expertise in congenital heart diseases.
