Eisenmenger syndrome

Eisenmenger syndrome (or ES,Eisenmenger’s reaction or tardive cyanosis) is defined as the process in which a left to right shunt caused by a congenital heart defect in the fetal heart which causes increased flow through the pulmonary vasculature, causing pulmonary hypertension, which in turn causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt.

eisenmenger syndrome


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Age/sex prevalence of Eisenmenger's syndrome

Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood.

What Are The Causes Of Eisenmenger's syndrome?

There are number of causes that can lead to Eisenmenger syndrome
Congenital heart defects which cause Eisenmenger syndrome are as follows
• Atrial septal defects
• Ventricular septal defects
• Patent ductus arteriosus
• Acyanotic heart disease

Eisenmenger's syndrome Sign and symptoms

Symptoms of Eisenmenger Syndrome include
• Fainting
• Feeling tired
• Shortness of breath
• Stroke
• Swelling in the joints caused by too much uric acid
• Chest pain
• Coughing up blood
• Bluish lips, fingers, toes, and skin
• Dizziness

Clinically patient show the following symptoms –
• Cyanosis
• High red blood cell count
• Swollen or clubbed fingertips
• Fainting called syncope
• Heart failure
• Arrhythmia or irregular heart rhythms
• Bleeding disorders
• Coughing up blood
• Iron deficiency
• Kidney problems
• Gout
• Gallstones

Investigations To Be Done

Laboratory investigations to be done are as follows
• Complete blood count
• Increased conjugated bilirubin
• Increased uric acid
• Urea and creatinine are sometimes elevated
• Urinary biochemical analysis reveals proteinuria

Other findings to be done are as follows-
• Pulse oximetry
• Arterial blood gas
• Brain natriuretic peptide

Imaging studies to be done are as follows
• Chest radiography
• Magnetic resonance imaging
Cardiac catheterization

Treatment For Eisenmenger's syndrome?

Medications include are as follows
• Diuretics
• Cardiac glycosides
• Prostaglandins
• Iron products
• Endothelin antagonists
• Phosphodiesterase-5 enzyme inhibitors

Treatments procedures include
• Oxygen therapy
• Pulmonary vasodilation therapy
• Transplantation is done such as
Heart-lung transplantation
Bilateral lung transplantation

What Are The Complications of Eisenmenger's syndrome?

Possible complications are as follows
• Gout
• Heart attack
• Hyperviscosity
• Infection in the brain
• Kidney failure
• Poor blood flow to the brain
• Stroke
• Sudden death
• Bleeding in the brain
• Congestive heart failure

Prognosis of Eisenmenger's syndrome

How well the infant or child depends on another medical condition and the age at which high blood pressure develops in the lungs. Patients with this condition can live 20 to 50 years.

Differential diagnosis of Eisenmenger's syndrome

• Primary, Pulmonary hypertension
• Tetralogy of Fallot
• Idiopathic pulmonary hypertension
• Persistent newborn pulmonary hypertension
• Pulmonary infarction
• Respiratory failure
• Tetralogy of Fallot with absent pulmonary valve
• Tetralogy of Fallot with pulmonary atresia
• Total anomalous pulmonary venous connection
• Transposition of the great arteries
• Tricuspid atresia
• Truncus arteriosus
• Ventricular septal defect (VSD)
• Pulmonary atresia with VSD
• Single ventricle with pulmonary stenosis

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