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

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Presentation transcript:

CONGENITAL AORTIC DISEASE

EMBRYOLOGY HYPOTHETICAL DOUBLE AORTIC ARCH (Edward JE)

NORMAL LEFT AORTIC ARCH DEVELOPMENT

AORTIC ANOMALIES

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

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

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

MIRROR IMAGE RIGHT AORTIC ARCH

MIRROR IMAGE RIGHT AORTIC ARCH DEVELOPMENT

MIRROR IMAGE RIGHT AORITIC ARCH IN ASYMPTOMATIC ADULT

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

ABERRANT LEFT SUBCLAVIAN RIGHT AORTIC ARCH

ABERRANT LEFT SUBCLAVIAN RIGHT AORTIC ARCH DEVELOPMENT

ABERRANT LEFT SUBCLAVIAN RIGHT AORTIC ARCH ASYMPTOMATIC ADULT

AB LEFT SUBCLAVIAN RIGHT ARCH ANEURYSM OF LEFT SUBCLAVIAN ARTERY

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

ISOLATED LEFT SUBCLAVIAN RIGHT AORTIC ARCH

ISOLATED LEFT SUBCLAVIAN RIGHT AORTIC ARCH DEVELOPMENT

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

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

DOUBLE AORTIC ARCH

DOUBLE AORTIC ARCH DEVELOPMENT

DOUBLE AORTIC ARCH CHILD WITH RECURRENT PNEUMONIA

DOUBLE AORTIC ARCH CHILD WITH DYSPHAGIA

64-YEAR-OLD WOMAN BROUGHT TO ER AFTER MVA

ASYMPTOMATIC ADULT

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

CERVICAL AORTIC ARCH

RIGHT AORTIC ARCH LEFT AORTIC ARCH

CERVICAL AORTIC ARCH

CERVICAL ARCH RIGHT SUBCLAVIAN FROM DIVERTICULUM

RIGHT CERVICAL ARCH ABERRANT LEFT SUBCLAVIAN ARTERY

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

COARCTATION

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

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

FIGURE 3 AORTIC KNOB PRE AND POST STENOTIC DILATION

COARCTATION “FIGURE 3” AORTA KNOB

COLLATERAL VESSELS

COARCTATION

COARCTATION LEFT VENTRICULAR ENLARGEMENT

CHEST RADIOGRAPH DILATED LEFT SUBCLAVIAN ARTERY

RIB NOTCHING

W???

34-YEAR-OLD MAN AUTOMOBILE ACCIDENT

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

HEMOPTYSIS 10 YEARS POST COARC REPAIR AORTOBRONCHIAL FISTULA

COARCTATION VERSUS PSEUDOCOARCTATION

PSEUDOCOARCTATION

KINK AT INSERTION SITE OF LIGAMENTUM ARTERIOSUM MINIMAL GRADIENT ACROSS DEFORMITY

PSEUDOCOARCTATION ARCH BUCKLED AT LIG. ARTERIOSUM

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

ABERRANT RIGHT SUBCLAVIAN ARTERY

ABERRANT RIGHT SUBCLAVIAN ARTERY DEVELOPMEN T

ABERRANT RIGHT SUBCLAVIAN ARTERY

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

ABERRANT RIGHT SUBCLAVIAN ART AORTIC DISSECTION

LIGAMENTUM ARTERIOSUM

BOVINE AORTIC ARCH

THE END

PATENT DUCTUS ARTERIOSIS