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Eisenmenger syndrome or Eisenmenger's syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) causes pulmonary hypertension [1] [2] and eventual reversal of the shunt into a cyanotic right-to-left shunt.
The ostium primum atrial septal defect is a defect in the atrial septum at the level of the tricuspid and mitral valves. This is sometimes known as an endocardial cushion defect because it often involves the endocardial cushion, which is the portion of the heart where the atrial septum meets the ventricular septum and the mitral valve meets the tricuspid valve.
Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria (upper chambers) of the heart.Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale; however, when this does not naturally close after birth it is referred to as a patent (open) foramen ovale (PFO).
Approximately 40-50% of fetuses diagnosed with AVCD have Down syndrome, and a further 15-20% are associated with other chromosomal abnormalities and syndromes, such as DiGeorge syndrome. [3] [10] The remaining 30-40% of cases are not linked to a syndrome, with AVCD observed without other major defects. AVCD is also linked with Noonan syndrome. [3]
If left untreated, the disease may progress from left-to-right shunt (acyanotic heart) to right-to-left shunt (cyanotic heart), called Eisenmenger's syndrome. Pulmonary hypertension is a potential long-term outcome, which may require a heart and/or lung transplant. Another complication of PDA is intraventricular hemorrhage. [citation needed]
Trilogy of Fallot is a combination of three congenital heart defects: pulmonary stenosis, right ventricular hypertrophy, and an atrial septal defect. [1]The first two of these are also found in the more common tetralogy of Fallot.
Less common signs/symptoms include non-productive cough and exercise-induced nausea and vomiting. [12] Coughing up of blood may occur in some patients, particularly those with specific subtypes of pulmonary hypertension such as heritable pulmonary arterial hypertension, Eisenmenger syndrome and chronic thromboembolic pulmonary hypertension. [16]
Since the 1950s, as the understanding of heart and lung disease has evolved, Eisenmenger's syndrome is the name given to the situation in which a cardiac defect causes too much blood flow to the lungs, which in turn causes changes to the blood vessels in the lungs (pulmonary hypertension) and a reversal of blood flow so that now there is ...