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interrupted aortic arch

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interrupted aortic arch

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    1. Interrupted Aortic Arch Presented by Ri ???

    3. Incidence A rare form of congenital heart disease. 1% of all CHD.

    4. Background First described by Steidele in 1778 First known surgical correction of IAA by Samson in1955 Classified by Celoria and Patton in 1959 First repair in neonate by Sirak in 1968 First one-stage primary correction of IAA by Trusler and Izukawa in 1975

    5. Classifications: Type A(40%): The interruption is distal to the left subclavian artery Type B(>50%): The interruption is between the left carotid and left subclavian arteries Type C(<5%): The interruption is between the innominate and left carotid arteries

    7. Associated anomaly PDA and VSD (>90%) bicuspid aortic valve ( 60% ) subaortic stenosis (20% ) valve deformity (10%) persistent truncus arteriosus (10%)

    8. Aberrant right subclavian artery(2/3 of type B, 1/3 of typeA) Chromosome band 22q11.2 deletion (>50% of IAA type B)

    9. IAA.html

    10. The CATCH22 Syndrome Definition: Cardiac, Abnormal facies, Thymic Hypoplasia, Cleft palate, Hypocalcemia) includes the broad clinical spectrum of conditions with 22q11.2 deletions DiGeorge syndrome (hypocalcemia, thymic hypoplasia, mild facial anomalies )

    11. The velocardiofacial syndrome (VCFS) (abnormal facies, cleft palate) Conotruncal anomaly face syndrome (CTAFS)

    12. Pathogenesis Abnormal proliferation, function, migration, and maturation of neural crest cell (encoded by Ch.22q11) secondary to a decreased antegrade blood flow in the ascending aorta due to the constant presence of VSD and left ventricular outflow obstruction

    13. Clinical manifestations Tachypnea, poor feeding, lethargy Before ductus arteriosus closure: differential oxygen saturation between the right arm (normal saturation) and the legs (decreased) Reverse differential cyanosis may present in IAA with TGA

    14. differential systolic pressure between the right arm and the legs may or may not present After ductus arteriosus closure: severe congestive heart failure, lower extremity hypoperfusion, anuria, and shock

    15. Pulse oximetry arm and leg oxygen saturation as a screening test Left heart obstructive disease (coarctation of the aorta, interrupted aortic arch, hypoplastic left heart syndrome, and critical aortic stenosis) with right-to-left-shunt of ductus arteriosus

    16. Echocardiography Diagnostic for IAA. the ascending aorta follows a straight course to its branches without the normal continuous curvature to the descending aorta. The V sign: type B. The W sign: type A.

    18. Chest X ray Cardiomegaly increased pulmonary vascular markings, and pulmonary venous congestion or pulmonary edema Narrow upper mediastinum or increase cardiothymic silhouette: absence of the thymus

    19. ECG Right ventricular hypertrophy ST-T wave abnormalities QT prolong(CATCH22 syndrome related hypocalcemia)

    20. Cardiac catheterization Accurate diagnosis Site of interruption Size and type of VSD Whether the right subclavian artery is aberrant

    21. Medical treatment Prostaglandin E1 infusion (maintain the patency of ductus arteriosus) Intubation and oxygen administration diuretic and inotropic agents Monitoring and correction of abnormal blood gases and electrolytes

    22. PGE1(Alprostadil ) Effect:direct vasodilatation action on the ductus arteriosus and vascular smooth muscle Adverse effect: neonatal apnea, seizures, fever, hypotension, tachycardia, pulmonary overcirculation, Coadministration with heparin may increase aPTT

    23. Surgical management The prognosis is quite poor if uncorrected Early surgical repair is recommended Single-staged complete repair with primary anastomosis of the interruption should be the surgical goal

    24. Possible restenosis of the aortic arch can adequately be treated by percutaneous balloon dilatation or redo surgery if necessary long-term outcome is strongly influenced by the presence of subaortic obstruction

    26. Reference Nelson Textbook of Pediatrics, 17th, 2004 Prenatal diagnosis of interruption of the aortic arch and its association with deletion of chromosome 22q11 Ultrasound in Obstetrics & Gynecology Volume 20Issue 4Page 327 - October 2002 Oxygen saturation as a screening test for critical congenital heart disease: A preliminary study. Pediatric cardiology2002,23:403-409 Repair of interrupted aortic arch: results after more than 20 years.Ann Thorac Surg. 2000 Dec;70(6):1896-9; discussion 1899-900. Single-stage repair of aortic arch obstruction and associated intracardiac defects in the neonate.Am J Cardiol. 1995 Feb 15;75(5):370-3. Repair of aortic arch interruption by direct anastomosis Eur J Cardiothorac Surg. 1997 Jan;11(1):100-4. Results of primary and two-stage repair of interrupted aortic arch. Eur J Cardiothorac Surg. 1998 Sep;14(3):235-42. Deletion 22q11 in patients with interrupted aortic arch.Am J Cardiol. 1999 Aug 1;84(3):360-1.

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