Congenital Module 3

Jatene procedure

- arterial switch method - Involves transection of the aorta and PAs about the valve sinuses (including removal of the coronaries) - great arteries are switched. - The coronaries are sewn into the new aorta (formerly the PA) - Very difficult. - Advantages: LV is systemic ventricle, and no conduit to go bad.

Transannular Patch Repair

- relieve PS/sub PS - RVOT incision - pericardial/synthetic patch - enlarged outflow tract - results in free PR

D-Transposition

1 of the 2 most common cyanotic CHD

large unrestrictive VSD, RVOT obstruction, large, overriding aorta, RV hypertrophy

4 characteristics of Tetralogy of Fallot

right aortic arch, secundum ASD, LSVC, coronary artery abnormalities

4 most common associated cardiac defects with Truncus arteriosus

valvular

80-90% of patients with RVOTO at this level

Tet Spell

A child with Tetralogy of Fallot (TOF) responds to this by squatting, which will increase blood flow back to the heart. It is a period of cyanosis. hold baby in knee-chest position

D-Transposition of the Great Arteries

An anomaly where the RA connects to the RV but the RV connects to the aorta and the LA connects to the LV but the LV connects to the pulmonary artery - lethal congenital malformation

double-chambered RV

Can occur when infundibular stenosis is so severe that the RV is divided into a low-pressure (distal or infundibulum and apical) and a high-pressure chamber (proximal or inflow) - usually with a membranous VSD

VSD (most common), coronary artery anomalies, PS or subpulmonic outflow tract obstruction, AS or subaortic stenosis, coarctation of the aorta

D-TGA associated lesions (most common is this)

McGoon & Rastelli

Truncus Arteriosus surgical repair: VSD closure, detach PA's and repair aorta, possible trruncal valve repair, possible aortic arch repair

Noonan syndrome

Webbed neck Pectus excavatum Short stature shield chest Pulmonary stenosis - 25% - thickened, myxomatous PV - DO NOT respond well to balloon angioplasty

L-looping

abnormal twisting of the heart tube to the left and results in the RV to the left

thickened leaflets, fusion along free edge cusp, domed, fixed central orifice, dysplastic valve, hypoplastic annulus, bicuspid pulmonary valve

anatomic features seen on valvular RVOTO

anterior and leftward

aorta orientation in CCTGA

PFO/ASD, additional VSD, AV canal defect, persistent LSVC, right Ao arch, anomalous coronary arteries

associated lesions with TOF

sinus node dysfunction, RV dysfunction, TR, LVOTO

atrial switch complications

Senning procedure

atrial switch procedure that uses native atrial tissue

Mustard procedure

atrial switch procedure that uses prosthetic material

double switch

atrial-level re-routing procedure (Mustard or Senning) and either an arterial switch or a Rastelli type ventricular level re-routing procedure

PLAX

best view to assess aortic override, aortic root size, infundibular and pulmonary valve narrowing

PSAX

best view to assess relationship of VSD to inlet, outlet, and trabecular septum, RVOTO, PV morphology, coronary arteries, pulmonary arteries

dyspnea, fatigue, tachy/brady arrhythmias/ complete HB, CHF

clinical features of CCTGA

VSD, LVOTO, subpulmonary stenosis, unusual anatomical location of AV node and Bundle of His (complete heart block)

common associated anomalies with CCTGA

higher risk in young child, transannular patch to enlarge RVOT results in increase PR leading to RVE and heart failure

cons of early surgical repair of TOF

infundibular septum

displacement of _________ _________ crowds RVOT and fails to occupy space above ventricular septum resulting in VSD shunt

truncus arteriosus

failure of septum formation, resulting in a single vessel that comes off of the ventricles - uncommon CHD - conotruncal abnormalitiy

common arterial trunk, single truncal valve, outlet VSD, pulmonary artery anatomy, aortic arch, coronary arteries

features and assessment of truncus arteriosus

relieve RVOTO and close VSD

goals of complete surgical TOF repair

discordance

in CCTGA there is atrioventricular and ventriculoarterial _________

corrected

in CCTGA there is hemodynamically ______ circulation

right and anterior

in D-TGA the aorta is described as

parallel

in D-TGA the great arteries are _______ to one another

concordant, discordant

in D-TGA there are ________ atrioventricular connections and ________ ventriculoarterial connections resulting in 2 parallel circuits

coronary arteries, pulmonary arteries, systemic arteries

in truncus arteriosus the single vessel coming off of the ventricle gives rise to this arteries

asymptomatic, dyspnea, fatigue

mild PS clinical features

cyanosis, CHF, syncope, sudden death

moderate-severe PS clinical features

- TV inserts more apically - TV/RV has chrodal attachments to ventricular septum - RV has moderator band, pyramidal shape, trabeculated endocardial surface

morphological TV/RV will have these anatomic qualities

Tetralogy of Fallot

most common cyanotic congenital heart defect

severe pulmonary regurgitation (symptoms, RVE/dysfunction)

most common reason for repeat surgical repair

RVOT obstruction

obstruction of blood flow from the RV to the pulmonary artery (valvular, subvalvular, supravalvular)

systemic, pulmonic

post-op assessment of atrial switch looks at ventricular function. The RV is the _______ ventricle and LV is the _______ ventricle

decrease RVH, myocardial fibrosis and frequency of late arrhythmia

pros of early surgical repair in TOF

atrial switch

pulmonary veins rerouted to right heart which is connected to Aorta and body. SVC/IVC rerouted to left heart which is connected to PV and lungs

cyanosis

right-to-left shunting at ventricular level through VSD and aorta rather than to the lungs - timing of presentation and degree is dependent on severity of obstruction

parallel

semilunar valves are in ______ and seen in same imaging plane (aorta typically anterior and rightward)

tricuspid

single (semilunar) truncal valve has this anatomy

infundibular

subvalvular RVOTO also referred to as this

pulmonary atresia

the absence of the pulmonary valve, which in turn prohibits blood flow from the right ventricle into the pulmonary artery and essentially to the lungs

PA trunk, PA bifurcation, proximal right and left branches

three locations of supravalvular RVOTO

repair or replace left AV valve, afterload reduction - RV dysfunction, transplant

treatment for CCTGA

regurgitation

truncal valve will more commonly have this problem with it

conotruncal septation

truncus arteriosus anomaly results from the failure of this to form

subpulmonary, infundibulum stenosis

type of RVOTO that is almost ALWAYS present in TOF

congenitally corrected transposition of the great arteries

uncommon, ventricles are switched - morphologic RV is the systemic ventricle on the left side of heart - morphologic LV is the sub pulmonary ventricle on right side of heart

central cyanosis

usually occurs due to right-left shunt through foramen ovale or small ASD

2/3

when RV pressure is _____ systemic = surgery

asymptomatic, no cyanosis, not diagnosed until late adulthood

without any associated lesions the patient is usually these things