A nonsystematic literature review regarding these conditions has been performed.Īneurysm bicuspid aortic valve coarctation of the aorta computed tomography angiogram interrupted aortic arch.Ĭopyright: © 2020 Indian Journal of Radiology and Imaging. She underwent a computed tomography angiogram showing interruption of the aortic arch, distal to left subclavian artery origin, large bilateral collateral vessels connecting subclavian arteries to descending aorta with multiple voluminous aneurysms, a bicuspid aortic valve, dilatated tubular segment of ascending thoracic aorta, and a suspected atrial septal defect. We present a case of a 52-year-old woman accessed to the emergency department for chest and right upper limb pain that increased in the last days. Most of them are classified as type A interrupted aortic arch and differential diagnosis is associated with severe chronic coarctation. A list of contributors can be seen here.Interrupted aortic arch diagnosed in adult age is a rare entity, with only a few cases published in the literature. 2009 Apr 35(4):565-8: Kobayashi et al: Outcomes following surgical repair of aortic arch obstructions with associated cardiac anomaliesĪll Marc’s PICU cardiology FOAM can be found on PICU Doctor and can be downloaded as a handy app for free on iPhone or Android. Critical Heart Disease in Infants and Children 2nd ed, Nichols et al: Interrupted Aortic Arch Long term survival after 10 yrs: 94% (IAA and VSD) 72% (IAA and TGA). Long term antihypertensive treatment required in 30%. Neurological injury due to deep hypothermic circulatory arrest.Spinal cord injury (spinalis anterior injury): 0.4-1.5%.PHT if high PBF was preceding (VSD or ASD).Postcoarctectomy syndrome: hypertension, abdominal pain, ileus (2-3 days post repair).Acute hypertension (increase of noradrenaline release due to sympathetic stimulation during repair): SNP or Esmolol infusion.Fluid restriction: 1ml/kg/hr, careful trophic feeds.Haemodynamics: age adjusted, in neonates: SBP >60 mmHg but 40 mmHg prevent hypertension (SNP or Esmolol infusion).Keep intubated, ventilated, sedated and paralysed for 24 hours for patients with preceding high PBF elective cases can be extubated earlier.Preferred single stage repair with end-to-end or end-to-side anastomosis, patch augmentation, subclavian-flap aortoplasty or extended resection with primary anastomosis and also VSD closure. Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates. Calcium infusion if there is Di-George Syndrome (see inotropes)ĮCG, CXR, CUS, FBE, xlotting, UECs, FISH, PRBC(4), FFP (2), platelets (2), cryoprecipitate (2).Careful fluid resuscitation – this is an obstructive lesion, not hypovolaemic!. Dopamine (5-10 mcg/kg/min), dobutamine (5-10 mcg/kg/min) or adrenaline (0.02-0.1 mcg/kg/min) may be required to stabilise for a low CO.Balanced circulation with PDA open and/or VSD present (aim SpO2 75-85%).Hypoventilation to higher the PVR and to lower the SVR. Intubate and sedate to lower the oxygen consumption. Commence Prostaglandin E1 (20ng/kg/min) to maintain systemic perfusion.Hypertension of upper limbs is usually not present before Day 5, but usually after PDA closure with signs of CCF of various degree.ĬXR: cardiomegaly and pulmonary congestion.Ĭardiac catheterisation (diagnostic and interventional) This in turn causes increased PBF and severe CCF with systemic hypotension. This leads to CCF (in extreme: myocardial ischemia) and shunt reversal along the PFO (and VSD if present). With PDA closure there is an acute increase in LV afterload, a decreased CO, increased LVEDP. Genetic association to Di-George-Syndrome. Most of them are associated with a VSD or other defects. Type C (2%): proximal to left carotid artery. Type B (78%): between left subclavian and left carotid artery. Three smaller blood vessels branch off the aorta to supply the head and arms with. The aorta rises upwards out of the heart, then curves down to the body (aortic arch). Type A (20%): IAA distal to left subclavian artery. Interrupted aortic arch is when the large blood vessel that takes blood away from the heart to the rest of the body (aorta) is not fully developed. Definition: obstructive anomaly of the aortic arch.
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |