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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 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 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 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 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 DEVELOPMENT ABERRANT RIGHT SUBCLAVIAN ARTERY 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