Basic Description of Congenital Abnormalities Flashcards

1
Q

Supravalvular Mitral Stenosis Ring

A

Rare
characterized by an abnormal ridge of connective tissue on the atrial side of the MV which can lead to MS
(frequently part of Shone Complex)

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2
Q

Shone Complex

A

Multiple levels of left heart obstruction are present

Includes Aortic Arch Hypoplasia or Coarctation, AV Stenosis, or Subaortic Stenosis

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3
Q

Eisenmengers

A

With significant L to R shunting exaggerated volumes over time to lunges cause irreversible damage
R pressure exceeds L pressures and shunt changes direction R to L
Results in decreased oxygen saturation and cyanosis

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4
Q

Situs Inversus

A

Organs of the chest and abdomen are arranged in a perfect mirror image reversal of normal positions

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5
Q

Aortic Coarctation

A

Narrowing of the proximal descending thoracic aorta just upstream from the entry of the ductus arteriosus

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6
Q

Marfans Syndrome

A

Gene defect in the coding of fibrillin a part of elastic fibers

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7
Q

Sinus of Valsalva Aneurysm

A

Aneurysm of one of the sinus; appears as a thin dilated area that projects into adjacent cardiac structures often within a fistula

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8
Q

Kawasaki Disease

A

A febrile illness that can dilate the proximal coronary arteries and cause LV dysfunction in children

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9
Q

Myxomatous MV Disease

A

Thick and redundant leaflets and apparatus, often presents as MVP

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10
Q

Patent Foramen Ovale (PFO)

A

Failure of foramen ovale to close (25% incidence) results in PFO

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11
Q

Partial Anomalous Pulmonary Venous Return (PAPVR)

A

One or more PV drain into RA or SVC or IVC

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12
Q

Total Anomalous Pulmonary Venous Return (TAPVR)

A

Diagnosed in children

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13
Q

Patent Ductus Arteriosus

A

Ductus (fetal communication between descending Ao and PA) remains open after birth

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14
Q

Ventricular Inversion

AKA CCTGA

A

CCTGA: Congenitally Corrected Transposition of the Great Arteries
AKA L- Transposition
Ventricles Switch Positions
Anatomic RV (with TV) = systemic ventricle
Anatomic LV (with MV) = venous ventricle

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15
Q

Transposition of the Great Arteries

A

AKA D- Transposition

Ao and PA have switched places and a shunt sustains life (Ao from RV, PA from LV)

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16
Q

Double Outlet RV (AKA Partial Transposition)

A

Great vessels exit RV

17
Q

Pulmonary Atresia

A

No PV or permanently closed PV

18
Q

Tricuspid Atresia

A

No TV with small RV

19
Q

Hypoplastic Left Heart Syndrome

A

LV and Aorta are hypoplastic

20
Q

Double Inlet LV

A

Typically ventricle and arteries have switched, both AV valves open into LV; RV hypoplastic

21
Q

Truncus Arteriosus

A

One great vessel exits the heart and splits into Aorta and PA

22
Q

Tetralogy Of Fallout

A

VSD, Pulmonary Stenosis, Overriding Aorta, RVH