![]() For an infant with an interrupted aortic arch, a patent (open) ductus arteriosus allows for blood to bypass the "interruption," without which blood will be unable to reach the lower half of the body. Death occurs due to increased blood flow from the left side of the heart (oxygenated blood) to the right side (deoxygenated blood), inducing heart failure pulmonary edema and eventual closing of the ductus arteriosus. Prognosis įailure to treat the condition yields a mortality rate of 90% at a median age of 4 days. After successful treatment, the patient is monitored for the rest of their life by a specialist to ensure that problems do not occur. Awaiting surgery, prostaglandin can be administered to keep the ductus arteriosus open, thereby allowing blood flow to the lower body. Recent research has revealed that an initial single-stage repair using direct anastomoses and repair of any existing cardiac defects is the preferred surgical technique, as opposed to a two-stage surgical repair. Often, a synthetic patch is used to recreate the lost section of aorta. Surgery Ĭurative treatment consists of open heart surgery soon after birth, preferably immediately after diagnosis. However, the diagnosis may go undetected, delaying treatment until closure of the ductus arteriosus produces symptoms. Prostaglandin therapy is performed via a continuous infusion, due to how quickly prostaglandins are metabolized in the body. If the diagnosis is made prenatally, prostaglandin E1 (PGE 1) is started after birth to avoid closure of the ductus arteriosus. However, these subgroups do not affect how the disease is diagnosed or treated. Įach class can be divided into two subgroups, based upon whether the right subclavian artery originated in a normal, anatomical position (subgroup 1) or if it originated distal to the left subclavian artery and continues behind the esophagus (subgroup 2). This is the least common form of the condition. Type C: The aortic arch is interrupted between the innominate artery and the left common carotid artery.This is the most common form of the condition, and is the classification most often associated with DiGeorge syndrome. Type B: The aortic arch is interrupted between the left common carotid artery and the left subclavian artery.Type A: The aortic arch is interrupted after the left subclavian artery.There are three primary classifications for an interrupted aortic arch, on the basis of the specific, anatomic anomaly. Intestinal injury: signs of necrotizing enterocolitis, such as bloody stoolsĬHARGE syndrome, a specific, rare pattern of genetic abnormalities, commonly features conotruncal and aortic arch heart defects, which can include an interrupted aortic arch.Kidney injury: elevated serum creatinine. ![]() Liver injury: elevated serum glutamic oxaloacetic transaminase (SGOT) (also known as aspartate transaminase, AST) and lactic acid dehydrogenase (LDH).Signs of ischemia due to interrupted aortic arch can be separated by the organ system involved: Rarely, an interrupted aortic arch can be associated with an intracranial aneurysm. The pattern of pulse abnormalities is dependent upon the classification e.g., for type B interrupted aortic arch, the right brachial pulse will be palpable and the left brachial and femoral pulses will be impalpable due to closure of the ductus arteriosus. If the condition progresses, the infant may turn pale, feel cold in the lower half of the body, and have a weak pulse due to insufficient blood flow. ![]() Patients will have a loss of appetite, appear tired and weak, and exhibit rapid breathing and a rapid heart rate. The diagnosis can also be made prior to birth via ultrasound. An echocardiogram can also aid in classifying the type of defect. It can be diagnosed with a standard echocardiogram. Around 50% of patients have DiGeorge syndrome. It is thought that an interrupted aortic arch occurs through excessive apoptosis in the developing, embryonic aorta. Patients with an interrupted aortic arch usually have symptoms from birth, with nearly all presenting symptoms within two weeks (when the ductus arteriosus is usually closed). Interrupted aortic arch (especially Type B) is often associated with DiGeorge syndrome. There are three types of interrupted aortic arch, with type B being the most common. Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus. In a sense it is the complete form of a coarctation of the aorta. There is a gap between the ascending and descending thoracic aorta. Interrupted aortic arch is a very rare heart defect (affecting 3 per million live births) in which the aorta is not completely developed. Medical condition Interrupted aortic arch
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