Eisenmenger syndrome is a cyanotic heart defect characterized by a long-standing intracardiac shunt (caused by ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) that eventually reverses to a right-to-left shunt.
Symptoms: Bluish lips, fingers, toes, and skin (cyanosis) Chest pain. Coughing up blood. Dizziness. Fainting. Feeling tired. Shortness of breath. Stroke.
Diagnosis: It is mainly detected by: Increased pulmonary arterial flow - Atrial septal defect (ASD), systemic arteriovenous fistulae, total anomalous pulmonary venous return.
Treament: If the hole in the heart is identified before it causes pulmonary hypertension, it can normally be repaired through surgery preventing the disease .After pulmonary hypertension, a heart,lung transplant or a lung transplant with repair of the heart is a possible option. Transplantation is the final therapeutic option and only for patients with poor prognosis and quality of life. Various medicines and therapies for pulmonary hypertension are under investigation for treatment of the symptoms. Causes: A number of congenital heart defects can cause Eisenmenger syndrome, including atrial septal defects, ventricular septal defects, patent ductus arteriosus, and more complex types of acyanotic heart disease.
Epidemology: Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood. Patients in underdeveloped countries are more likely to present late with uncorrected congenital cardiac lesions and a markedly elevated pulmonary vascular resistance (PVR). They are more likely to be inoperable secondary to Eisenmenger physiology.