CONGENITAL AORTIC DISEASE

EMBRYOLOGYHYPOTHETICAL DOUBLE AORTIC ARCH

(Edward JE)

NORMAL LEFT AORTIC ARCHDEVELOPMENT

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 ANOMALIESPRESENTATION

• VASCULAR RING– STRIDOR, WHEEZE, INFECTION, DYSPHAGIA

• ASSOCIATED CARDIAC DISORDERS– CYANOSIS, CONGESTIVE HEART FAILURE

RIGHT AORTIC ARCHMOST COMMON TYPES

• MIRROR IMAGE

• ABERRANT LEFT SUBCLAVIAN

• ISOLATED LEFT SUBCLAVIAN

MIRROR IMAGERIGHT 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 SUBCLAVIANRIGHT 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 ARCHANEURYSM 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 SUBCLAVIANRIGHT AORTIC ARCH

ISOLATED LEFT SUBCLAVIAN RIGHT AORTIC ARCH

DEVELOPMENT

ISOLATED LEFT SUBCLAVIAN ARTERYRIGHT 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 ARCHCHILD WITH RECURRENT PNEUMONIA

DOUBLE AORTIC ARCHCHILD 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 ARCHRIGHT SUBCLAVIAN FROM DIVERTICULUM

RIGHT CERVICAL ARCHABERRANT 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 KNOBPRE AND POST STENOTIC DILATION

COARCTATION“FIGURE 3” AORTA KNOB

COLLATERAL VESSELS

COARCTATION

COARCTATIONLEFT VENTRICULAR ENLARGEMENT

CHEST RADIOGRAPHDILATED LEFT SUBCLAVIAN ARTERY

RIB NOTCHING

W???

34-YEAR-OLD MANAUTOMOBILE 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 REPAIRAORTOBRONCHIAL 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

ABERRANTRIGHT 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 ARTAORTIC DISSECTION

LIGAMENTUM ARTERIOSUM

BOVINE AORTIC ARCH

THE END

PATENT DUCTUS ARTERIOSIS