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Aortic Arch Interruption


Presentation

  • The presentation in adulthood with rupture of the aneurysm and the successful surgical treatment of both abnormalities are unusual.[ncbi.nlm.nih.gov]
  • Abstract We present a rare case of a patient with interrupted aortic arch with Ebstein's anomaly of the tricuspid valve and rheumatic mitral stenosis. This patient presented in early adulthood, which makes his case even more unusual.[ncbi.nlm.nih.gov]
  • Concomitant presentation of interrupted aortic arch and giant subclavian artery aneurysm is an unusual event in adulthood.[ncbi.nlm.nih.gov]
  • Abstract A case is described of a newborn infant, small for gestational age, with the features of the "fetal alcohol syndrome", who presented a murmur within the first few days of life, and died at age 7 days in heart failure.[ncbi.nlm.nih.gov]
  • Abstract We describe the treatment of an extremely rare case of interrupted aortic arch, annuloaortic ectasia, and aortic regurgitation in a 34-year-old man who presented with dyspnea and palpitation.[ncbi.nlm.nih.gov]
Death in Infancy
  • The unoperated natural history of TA patients is extremely poor, with death in infancy or early childhood due to severe heart failure, subsequent pulmonary vascular occlusive disease, endocarditis and cerebral abscess.[jcmr-online.biomedcentral.com]
Exertional Dyspnea
  • Here, we report a 19-year-old male with a 3-month history of exertional dyspnea.[ncbi.nlm.nih.gov]
Urinary Incontinence
  • A 14-year-old boy with a history of 2 years of flaccid paraplegia and rectal and urinary incontinence after two spontaneous subarachnoid hemorrhages was encountered in August 1993.[ncbi.nlm.nih.gov]

Treatment

  • Abstract A palliative operation is described for the treatment of infants with all types of aortic arch interruption without regard to anatomic variation.[ncbi.nlm.nih.gov]
  • A male infant, having a persistent fifth aortic arch and interruption of the aorta distal to the left subclavian artery, underwent successful surgical treatment.[ncbi.nlm.nih.gov]
  • Abstract We describe the treatment of an extremely rare case of interrupted aortic arch, annuloaortic ectasia, and aortic regurgitation in a 34-year-old man who presented with dyspnea and palpitation.[ncbi.nlm.nih.gov]
  • The presentation in adulthood with rupture of the aneurysm and the successful surgical treatment of both abnormalities are unusual.[ncbi.nlm.nih.gov]
  • For treatment, we believe that surgery should be directed to the hemodynamic abnormality of the aortic arch interruption.[ncbi.nlm.nih.gov]

Prognosis

  • MRI non-visualization of the portion of interruption great vessels may show a "V" configuration on coronal imaging 2 Treatment and prognosis If uncorrected, it carries a very poor prognosis with extrauterine survival being as little as a few days.[radiopaedia.org]
  • The prognosis of patients with TA lesions is poor without surgical intervention. Newborns often present with signs of florid heart failure and cyanosis.[jcmr-online.biomedcentral.com]

Etiology

  • He was then referred to an outpatient hypertension clinic for management and etiological study. Laboratory and imaging studies were performed with no apparent secondary causes of hypertension detected.[elsevier.pt]

Epidemiology

  • Interrupted aortic arch: an epidemiologic study. Teratology 2000 May;61(5):368-75. 6. Hsieh YC, Wu1 TJ, Wang KY, Liang KW, Lin WW, Chen YT.[oapublishinglondon.com]
  • Authors’ Affiliations (1) The Ohio State University Medical Center, Ross Heart Hospital, Columbus, Ohio, USA (2) Cleveland Clinic, Cleveland, Ohio, USA (3) Nationwide Children's Hospital, Columbus, Ohio, USA References Keith JD: Prevalence, incidence, and epidemiology[jcmr-online.biomedcentral.com]
  • Epidemiology Among patients with congenital heart disease, IAA occurs 1% of the time, with IAA type B being the most common form. Genetics Among patients with IAA, specifically type B, 50% have 22q11 deletion (DiGeorge syndrome).[pedecho.org]
Sex distribution
Age distribution

Pathophysiology

  • (Click picture to show/hide bloodflows) Pathophysiology All three forms of interrupted aortic arch require that there be a patent ductus arteriosus to supply blood to the lower half of the body.[vhlab.umn.edu]

Prevention

  • This prevents the flow of blood through the aorta. Because blood flow through the aorta is blocked, the blood supply to the lower body is compromised.[pediatriccardiacinquest.mb.ca]
  • Closure of the ductus a few days after birth prevents blood flow, leading to circulatory collapse and metabolic acidosis, both which threaten survival.[vhlab.umn.edu]
  • […] management: Keep intubated, ventilated, sedated and paralysed for 24 hours for patients with preceding high PBF; elective cases can be extubated earlier Inotropes: milrinone plus dopamine Haemodynamics: age adjusted, in neonates: SBP 60 mmHg but 40 mmHg; prevent[dontforgetthebubbles.com]
  • The blockage usually occurs just after the aorta has given off branches to supply the head and arms with blood, preventing normal circulation to the lower half of the body.[leedscongenitalhearts.com]
  • Patients who are treated for IAA usually have to take antibiotics for the rest of their lives in order to prevent endocarditis, an infection of the heart’s interior and valves. Following surgery for IAA, patients usually have a good outlook.[indiacardiacsurgerysite.com]

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