Eisenmenger’s syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2], Priyamavada Singh, MBBS [3];Kristin Feeney, B.S. [4]
Overview
Eisenmenger's syndrome or Eisenmenger's reaction is defined as the process in which a left-to-right shunt in the heart causes increased flow through the pulmonary vasculature, which leads to pulmonary hypertension, which finally causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt. This right to left shunt causes the patient to become cyanotic. Thus, Eisenmenger's syndrome is said to develop when there is a pulmonary artery disease, right-to-left heart shunting and cyanosis.
Historical Perspective
Victor Eisenmenger first described the syndrome in 1897 in a patient who presented with dyspnea and cyanosis since infancy. Since then, advances in the medical treatment of pulmonary hypertension improved the survival of Eisenmenger's syndrome.
Classification
Pathophysiology
Causes
Eisenmenger's syndrome is causes by cardiac defects that shunt blood heading to the systemic circulation back into the pulmonary circulation. These defects include ventricular septal defect, atrial septal defect, atrioventricular canal defect, patent ductus arteriosus, and truncus arteriosus.
Differentiating Eisenmenger's syndrome from Other Diseases
Epidemiology and Demographics
The incidence and prevalence of Eisenmenger's syndrome has been decreasing gradually over the years. In the general population, the prevalence decreased from 24.6 to 11.9/million inhabitants in 2012. About 8% of patents with congenital heart diseases develop Eisenmenger's syndrome. The mortality rate of Eisenmenger's syndrome is about 27%. The risk of Eisenmenger's syndrome increases in patients with large cardiac defects or who live in developing countries due to poor healthcare access.
Risk Factors
The common risk factors for Eisenmenger's syndrome include genetic mutations, exposure to rubella virus, and drug and alcohol abuse during pregnancy. Less common risk factors include poor healthcare access and patients in developing countries.
Screening
Natural History, Complications, and Prognosis
The complications of Eisenmenger's syndrome include intracranial hemorrhage, stroke, congestive heart failure, angina pectoris, hyperviscosity syndrome, infection (cerebral abscess), renal failure, and sudden death.
Diagnosis
Diagnostic Study of Choice
Echocardiography is the first line diagnostic modality of Eisenmenger's syndrome. Findings on an echocardiography suggestive of Eisenmenger's syndrome include underlying cardiac lesion responsible for the shunt, direct of the shunt, and elevated right ventricular systolic and pulmonary artery diastolic pressures.
History and Symptoms
Patients with Eisenmenger's syndrome pass through the following stages: An underlying heart defect that initially allows a left-to-right shunt between the left and right sides of the heart, then the development of pulmonary hypertension, then polycythemia, an increase in the number of red blood cells, and finally, a reversal of the left-to-right shunt so that there is a right-to-left shunt. The common symptoms of Eisenmenger's syndrome include dyspnea, syncope, hemoptysis, chest pain, dizziness, fatigue, and dyspnea.
Physical Examination
The general examination of patients with Eisenmenger's syndrome may show cyanosis, dermal changes, clubbing, peripheral edema, and abdominal swelling. Cardiac examination may show A wave dominant jugular venous pulse, right ventricular heave, high-pitched early diastolic murmur of pulmonary insufficiency, right-sided S4, pulmonary ejection click, single palpable S2, and loud P2.
Laboratory Findings
The laboratory findings of patients with Eisenmenger's syndrome include erythrocytosis, iron deficiency, increased bleeding time, increased blood uric acid and conjugated bilirubin concentrations, as well as mixed respiratory and metabolic acidosis.
Electrocardiogram
Eisenmenger's patients may show some ECG findings, including right axis deviation (ventricular hypertrophy), ST changes (Right ventricular or biventricular hypertrophy), P Pulmonale (Right atrial hypertrophy), tall R wave in V1 and deep S wave in V6, and microvolt T-wave alternans.
X-ray
Chest X-ray of patients with Eisenmenger's syndrome may show cardiomegaly, right ventricular or biventricular enlargement, right atrial or biatrial enlargement, and pulmonary vascular plethora.
Echocardiography and Ultrasound
Echocardiography is the first line diagnostic modality of Eisenmenger's syndrome. Findings on an echocardiography suggestive of Eisenmenger's syndrome include underlying cardiac lesion responsible for the shunt, direct of the shunt, and elevated right ventricular systolic and pulmonary artery diastolic pressures.
CT scan
Computed tomography can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive. The findings on CT scan may include large main pulmonary artery, ventricular septal defect, and right ventricular hypertrophy.
MRI
Magnetic resonance imaging can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive. The following can be observed on MRI examination of Eisenmenger's syndrome patients: Magnitude and direction of the cardiac shunt, reduced systolic function of the cardiac ventricles, and brain diffusion changes on brain MRI.
Other Imaging Findings
Cardiac catheterization is useful in patients with Eisenmenger's syndrome in terms of assessing the severity of pulmonary hypertension, coexisting congenital anomalies, degree and direction of shunting, ventricular function, pulmonary artery pressure and flow, and calculation of pulmonary vascular resistance.
Other Diagnostic Studies
There are no other diagnostic studies associated with Eisenmenger's syndrome.
Treatment
Medical Therapy
If surgical intervention is not available, treatment is mostly palliative. It includes anticoagulants, pulmonary vasodilators such as bosentan, PGE 5 inhibitor, prostacyclin, antibiotic prophylaxis to prevent endocarditis, phlebotomy to treat polycythemia, and maintaining proper fluid balance.
Surgery
Congenital heart defects should undergo surgical repair before progression to Eisenmenger's syndrome. Once the condition develops into Eisenmenger's syndrome, no surgical cure is available except for heart and lung transplantation. However, palliative interventions (as creation of an artificial ASD) may prolong the lifespan and improve the quality of life.