Heart defects and surgical repairs

Transposition of the great arteries (D-type)

the aorta and pulmonary artery artery are connected to the wrong ventricles. Ao and PA will arise parallel to each other. May have a PDA, ASD, and/or VSD.

Most common associated defect with TGA D type

anomalous coronary arteries 33%

Transposition of the great arteries (L-TGA) or corrected

The aorta and pulmonary artery are transposed and the right and left ventricles are inverted.

Associated conditions with L-TGA

• VSD (70-80%)

• pulmonary stenosis

• abnormal TV

Jatene procedure (arterial switch)

• The preferred surgical procedure for DTGA.

• usually performed within the first 2 weeks

• switches the AO and PA into their normal position and moving the coronaries to the new aorta.

Mustard and senning procedures (artrial switch)

• intra-aterial surgical repair used in the treatment of DTGA before the Jatene procedure.

• both operations involve creating a baffle within the atria to redirect blood.

Double switch procedure

• For patients with LTGA

• If pulmonary stenosis is not present, then you can so the atrial and arterial switch

• is pulmonary stenosis is present then the arterial switch cannot be performed but rather an atrial switch and a Rastelli conduit.

coarctation of the aorta

• narrowing of the proximal portion portion of the descending

Coarctation the aorta surgical correction

• resection with end-to-end anastomosis

• synthetic patch aortoplasty

• left subclavian artery patch

Tricuspid atresia surgical repair

• BT shunt

  • usually performed at 4-6 weeks

  • a systemic venous-to-pulmonary connection is established from the BA or RSA to the RPA through a homograft conduit.

• Glenn procedure

  • usually performed at 4-6 months as a bridge to fontan completion

  • BT shunt will be disconnected from the RPA and removed

  • SVC is connected to the RPA

• Fontan procedure

  • usually performed at 2-3yrs

  • ivc connected to the RPA

  • ASD is closed

Truncus Arteriosus

• A single great artery with a common trunk overriding a large VSD

• The Ao and PV are fused into a large semilunar valve with multiple leaflets called the truncal valve

Type 1 truncus arteriosus

a single pulmonary trunk arises from the common arterial trunk, with left and right pulmonary arteries originating from the pulmonary trunk.

Type 2 truncus arteriosus

• The left and right pulmonary arteries arise separately form the posterior portion of the common arterial trunk in close proximity to one another.

• A right aortic arch is common.

Type 3 truncus arteriosus

• The left and right pulmonary arteries arise as separated vessels from the common trunk.

• This type is generally considered to be a form of Pulmonary Atresia with VSD or TOF.

Rastelli procedure

• PDA is excised

• proximal and distal ends of the aorta are anastomosed

• the VSD is closed with a patch

• the PA is separated from the arterial trunk

Tetralogy of Fallot

4 abnormalities

• VSD

• RVOT obstruction or pulmonary stenosis

• overriding aorta

• right ventricular hypertrophy

What is the most common cyanotic heart defeat among infants?

TOF

What causes a Persistent left SVC?

results when the embryologic left sided svc (cardinal vein) fails to degenerate normally and remains patent

What are the 2 types of PLSVC?

• PLSVC drain directly into the coronary sinus (90% of cases)

• PLSVC connects to the LA

What happens when the PLSVC drains into the coronary sinus?

• CS will be enlarged

• RA and RV might be dilated

What defines an endocardial cushion defect or AV canal?

Characterized by having an ASD, VSD and mitral and tricuspid valve malformations

What is a partial AV canal?

• typically, only the right and left atria involved.

• A primum atrial septal defect will be present with left to right shunting

• Cleft mitral valve and MR are common

What is an “incomplete AV canal"?

Defined the combination of partial AV canal anatomy with outlet VSD

What defines a complete AV canal defect?

Involves all 4 chambers and the AV valves

• A large inlet VSD located in the posterior portion of the IVS will typically present

• A large primum ASD in the anterior portion of the interatrial septum will also be present

• The MV and TV are merged, forming a single AV valve that crosses the ASD and VSD

What are the different types of coarctation repairs?

• end-to-end anastomosis

• extended end-to-end anastomosis

• subclavian patch or reverse subclavian patch

what is ebsteins anomaly?

cyanotic abnormality of the TV characterized by the downward displacement of the valves posterior and septal leaflets into the RV.

What is the Warden procedure?

• SVC is transected above the anomalous pulmonary veins

• the lower SVC stump is oversewn and an intracardiac baffle redirects pulmonary venous flow through an ASD

• The upper SVC is anastomosed to the right atrial appendage to restore systemic venous return from the upper body

What makes up Shones complex?

• supra-valvar mitral ring or membrane

• parachute mitral valve

• sub-aortic membrane 

• coarctation of the aorta.