What is Eisenmenger Syndrome?
Eisenmenger’s Syndrome is characterized by congenital heart abnormalities that cause blood to circulate improperly in the heart and lungs of the patient. When blood does not flow correctly through your lungs. The blood vessels in your lungs become rigid and narrow. Raising the pressure in the arteries of your lungs (pulmonary arterial hypertension). The blood vessels in your lungs are permanently damaged as a result of this.
In those who were born with an uncorrected congenital cardiac defect. Eisenmenger (I-sun-meng-uhr) syndrome occurs as a long-term complication of the condition (congenital).
Moreover, developing Eisenmenger syndrome necessitates close medical supervision in the event that it occurs. Medications can help to alleviate symptoms and improve the prognosis.
How the heart functions
Your heart is divided into four chambers. Two of which are on the right and two of which are on the left side. The right side of your body is responsible for moving blood into the veins that lead to your lungs. Furthermore, the oxygen in your lungs enriches your blood. Which then circulates to the left side of your heart. The left side of your heart pumps blood into a big channel known as the aorta, which then circulates the blood throughout the rest of your body, including your brain.
Additionally, these valves open to allow blood to pass to the next chamber or to one of the arteries and then seal to prevent blood from going backward.
The progression of Eisenmenger syndrome
Defect in the atrioventricular canal. This heart defect is characterized by a big hole in the center of the heart where the walls separating the upper chambers (atria) and lower chambers (ventricles) meet. Causing the heart to beat irregularly. It is also possible that some of the valves in your heart are not functioning properly.
Additionally, an atrial septal defect is a hole in the middle of the heart. An unusually large shunt in the wall of tissue that separates the right and left sides of your heart’s upper chambers is known as an atrial septal defect (atria).
Also, the ductus arteriosus is patent. This heart defect is characterized by an opening between the pulmonary artery. Which transports oxygen-poor blood to the lungs, and the artery. Which transports oxygen-rich blood to the rest of your body (aorta).
Moreover, the ventricular septal defect is a defect in the septum of the heart. Having a shunt in the wall of tissue that splits your heart’s main pumping chambers (ventricles) between the right and left sides is the most prevalent cause of Eisenmenger syndrome, according to the American Heart Association.
The blood is flowing in a way that it would not ordinarily, which increases the pressure in your pulmonary artery when you have any of these problems. Over time, the increasing pressure in your lungs causes damage to the smaller blood vessels that supply oxygen to your body. Due to weakened blood artery walls, it is difficult to circulate blood through the body and into the lungs.
Symptoms and signs of a condition
Person to person, the specific symptoms of Eisenmenger’s syndrome might vary substantially in intensity. Eisenmenger syndrome with cyanosis is a congenital heart problem that manifests itself from birth. It most commonly manifests itself around puberty. But it might manifest itself earlier or later depending on the location and severity of the congenital heart defect. Low oxygen levels in the blood, and high pressures in the lungs.
Also, individuals suffering from Eisenmenger’s syndrome experience cyanosis. Which manifests itself most prominently on the lips, fingers, and toes, and is more noticeable after physical exertion. Patients with erythrocytosis (an increase in the number of red blood cells or erythrocytes) that transport oxygen to the body often have an increased number of red blood cells or erythrocytes. Which compensates for the insufficient oxygen delivery to tissues.
This is an invasive procedure that measures pressures in the heart and lungs to rule out other medical conditions. Pulse oximetry (which measures the amount of oxygen in the blood). Chest radiography, electrocardiogram (ECG), lung function test. Iron levels, and a complete blood count (CBC). In order to diagnose a cardiac abnormality and to rule out the possibility of excessive pressures in the lungs, echocardiography is essential.
Eisenmenger Syndrome Treatment
Transplantation of the cardiopulmonary system is curative in the treatment of Eisenmenger’s syndrome. However, this is not feasible in the majority of situations. The use of pharmaceuticals has reduced symptoms, but not mortality. Diuretics, antiarrhythmics, and anticoagulants are all potential medications for the treatment of Eisenmenger syndrome. And they may be necessary for some patients.
Moreover, vasodilator therapy may present an opportunity for a clinical study. And studies have demonstrated some symptomatic improvement as a result of their use. The Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) trial found that endothelin antagonists increased exercise capacity and clinical improvement in patients with ASD, VSD, and PDA who also had Eisenmenger syndrome. Further research has revealed that sildenafil has a protective effect on mortality as well.
In theory, anticoagulation may be able to alleviate some of the pathology associated with in situ thrombosis. Which is a characteristic of the disease. The evidence for this practice, on the other hand, is primarily based on observational research.
It is possible that in individuals who have developed PAH as a result of an untreated CHD, the defect itself is serving as a protective measure, by preventing worsening of the pulmonary vascular resistance in the face of growing right ventricular pressure. Post-surgical problems such as right heart failure increased pulmonary hypertension, and hypertensive crises are more likely to occur in children with the development of pulmonary vascular disease (more than 6 Woods units/m2) and inadequate vasodilator response.
Aside from that, there are just a few case reports that suggest benefits for the closure of the anatomical defect, even in the presence of clinical response to vasodilator therapy for the pulmonary arterial hypertension component. The evidence suggests that cardiac surgery, in conjunction with attentive peri- and postoperative control of pulmonary vascular resistance using modern PAH medications, may increase safety and minimize downstream complications in patients with heart failure