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CONGENITAL AORTIC
DISEASE
EMBRYOLOGY
HYPOTHETICAL DOUBLE AORTIC ARCH
(Edward JE)
NORMAL LEFT AORTIC ARCH
DEVELOPMENT
AORTIC ANOMALIES
AORTIC ARCH ANOMALIES
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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
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