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CONGENITAL AORTIC DISEASE. EMBRYOLOGY HYPOTHETICAL DOUBLE AORTIC ARCH (Edward JE)

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Presentation on theme: "CONGENITAL AORTIC DISEASE. EMBRYOLOGY HYPOTHETICAL DOUBLE AORTIC ARCH (Edward JE)"— Presentation transcript:

1 CONGENITAL AORTIC DISEASE

2 EMBRYOLOGY HYPOTHETICAL DOUBLE AORTIC ARCH (Edward JE)

3 NORMAL LEFT AORTIC ARCH DEVELOPMENT

4 AORTIC ANOMALIES

5 AORTIC ARCH ANOMALIES RIGHT ARCH DOUBLE ARCH CERVICAL ARCH COARCTATION INTERRUPTED AORTIC ARCH PSEUDOCOARCTATION ABERRANT RIGHT SUBCLAVIAN ARTERY PATENT DUCTUS ARTERIOSIS

6 AORTIC ARCH ANOMALIES PRESENTATION VASCULAR RING –STRIDOR, WHEEZE, INFECTION, DYSPHAGIA ASSOCIATED CARDIAC DISORDERS –CYANOSIS, CONGESTIVE HEART FAILURE

7 RIGHT AORTIC ARCH MOST COMMON TYPES MIRROR IMAGE ABERRANT LEFT SUBCLAVIAN ISOLATED LEFT SUBCLAVIAN

8 MIRROR IMAGE RIGHT AORTIC ARCH

9 MIRROR IMAGE RIGHT AORTIC ARCH DEVELOPMENT

10 MIRROR IMAGE RIGHT AORITIC ARCH IN ASYMPTOMATIC ADULT

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12 MIRROR IMAGE RIGHT AORTIC ARCH CYANOTIC HEART DISEASE IN 98% TETRALOGY, TRUNCUS, TRANSPOSITION,TRICUSPID ATRESIA RARE... VASCULAR RING & NO HEART DEFECT INDENTS RIGHT SIDE OF TRACHEA & ESOPHAGUS

13 ABERRANT LEFT SUBCLAVIAN RIGHT AORTIC ARCH

14 ABERRANT LEFT SUBCLAVIAN RIGHT AORTIC ARCH DEVELOPMENT

15 ABERRANT LEFT SUBCLAVIAN RIGHT AORTIC ARCH ASYMPTOMATIC ADULT

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17 AB LEFT SUBCLAVIAN RIGHT ARCH ANEURYSM OF LEFT SUBCLAVIAN ARTERY

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19 ABERRANT LEFT SUBCLAVIAN RIGHT AORTIC ARCH MOST COMMON RIGHT ARCH RARELY CAUSES SYMPTOMS RARELY FORMS A VASCULAR RING THAT AFFECTS ESOPH, TRACHEA DIVERTICULUM OF KOMMERELL INDENTS POSTERIOR ESOPHAGUS

20 ISOLATED LEFT SUBCLAVIAN RIGHT AORTIC ARCH

21 ISOLATED LEFT SUBCLAVIAN RIGHT AORTIC ARCH DEVELOPMENT

22 ISOLATED LEFT SUBCLAVIAN ARTERY RIGHT AORTIC ARCH SIGNS AND SYMPTOMS OF LEFT ARM ISCHEMIA

23 ISOLATED LEFT SUBCLAVIAN RIGHT AORTIC ARCH RARE CAUSE OF ARM ISCHEMIA –DECREASED PULSE, FATIGUE, DISCOMFORT SUBCLAVIAN STEAL SYNDROME

24 DOUBLE AORTIC ARCH

25 DOUBLE AORTIC ARCH DEVELOPMENT

26 DOUBLE AORTIC ARCH CHILD WITH RECURRENT PNEUMONIA

27 DOUBLE AORTIC ARCH CHILD WITH DYSPHAGIA

28 64-YEAR-OLD WOMAN BROUGHT TO ER AFTER MVA

29 ASYMPTOMATIC ADULT

30 DOUBLE AORTIC ARCH MOST COMMON COMPLETE VASCULAR RING STRIDOR, WHEEZE, INFECTION, DYSPHAGIA CONGENITAL HEART DISEASE IS RARE INDENTATION OF BOTH SIDES ESOPHAGUS

31 CERVICAL AORTIC ARCH

32 RIGHT AORTIC ARCH LEFT AORTIC ARCH

33 CERVICAL AORTIC ARCH

34 CERVICAL ARCH RIGHT SUBCLAVIAN FROM DIVERTICULUM

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37 RIGHT CERVICAL ARCH ABERRANT LEFT SUBCLAVIAN ARTERY

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39 CERVICAL AORTIC ARCH OFTEN NO SYMPTOMS TRACHEAL OR ESOPHAGEAL COMPRESSION WHEEZING, STRIDOR, COUGHING CHOKING, RECURRENT PNEUMONIA PALPABLE SUPRACLAVICULAR MASS CONGENITAL HEART DISEASE TEF, VSD, PDA COARCTATION OF DESCENDING AORTA

40 COARCTATION

41 5% OF CONGENITAL HEART DISEASE POSTDUCTAL ADULT TYPE COARCTATION DISTAL TO DUCTUS OR LIGAMENTUM ARTERIOSUM JUXTADUCTAL INFANTS AND CHILDREN HYPOPLASIA OF TRANSVERSE AORTIC ARCH AND AORTIC ISTHMUS. DUCTUS AT AREA OF COARCTATION AORTIC ARCH INTERRUPTION 95% HAVE PDA, 50% HAVE VSD

42 COARCTATION AORTIC KNOB FIGURE 3 AORTIC KNOB DILATED ARTERIES COLLATERALS ARTERIES LEFT SUBCLAVIAN ARTERY ASCENDING AORTA RIB NOTCHING LEFT VENTRICLE HYPERTROPHY ENLARGEMENT

43 FIGURE 3 AORTIC KNOB PRE AND POST STENOTIC DILATION

44 COARCTATION “FIGURE 3” AORTA KNOB

45

46 COLLATERAL VESSELS

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49 COARCTATION

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51 COARCTATION LEFT VENTRICULAR ENLARGEMENT

52 CHEST RADIOGRAPH DILATED LEFT SUBCLAVIAN ARTERY

53 RIB NOTCHING

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56 W???

57 34-YEAR-OLD MAN AUTOMOBILE ACCIDENT

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59 COARCTATION AORTIC VALVE –MAJORITY DEFORMED OR BICUSPID 10% HAVE CEREBRAL ANEURYMS AORTIC DISSECTION CHD HEART DISEASE COMMON ASSOCIATED ANOMALIES –TURNER SYNDROME,

60 HEMOPTYSIS 10 YEARS POST COARC REPAIR AORTOBRONCHIAL FISTULA

61 COARCTATION VERSUS PSEUDOCOARCTATION

62 PSEUDOCOARCTATION

63 KINK AT INSERTION SITE OF LIGAMENTUM ARTERIOSUM MINIMAL GRADIENT ACROSS DEFORMITY

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70 PSEUDOCOARCTATION ARCH BUCKLED AT LIG. ARTERIOSUM

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72 PSEUDOCOARCTATION MOST OFTEN DISCOVERED AS A MEDIASTINAL MASS ON PA CHEST RADIOGRAPH LOOK FOR KINK OF DESCENDING AORTA ON LATERAL CHEST RADIOGRAPH MEASUREMENT OF GRADIENT ACROSS KINK MAY BE NECESSARY TO DIFFERENTIATE FROM COARCTATION

73 ABERRANT RIGHT SUBCLAVIAN ARTERY

74 ABERRANT RIGHT SUBCLAVIAN ARTERY DEVELOPMEN T

75 ABERRANT RIGHT SUBCLAVIAN ARTERY

76 MOST COMMON ARTERIAL ANOMALY IN CHEST ALMOST ALWAYS AN INCIDENTAL FINDING OBLIQUE INDENTATION, POSTERIOR ESOPHAGUS CAN OCCUR WITH OTHER ANOMALIES

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80 ABERRANT RIGHT SUBCLAVIAN ART AORTIC DISSECTION

81 LIGAMENTUM ARTERIOSUM

82 BOVINE AORTIC ARCH

83 THE END

84 PATENT DUCTUS ARTERIOSIS

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