TranspositionsTranspositions of the Heartof the Heart

D-TGA & Congenitally Corrected TGA

………………..……………………………………………………………………………………………………………………………………..

Columbus Ohio Adult Congenital Heart ProgramNationwide Children’s Hospital &

Wexner Medical Center at The Ohio State University

………………..……………………………………………………………………………………………………………………………………..

Central Ohio Congenital Heart Walkg

Sunday 09/30/121 Mile Walk & 5K Run/Walk

yScioto Audubon Metro Park

8:30am Registrationg10:00am Start

www.congenitalheartwalk.org

Transpositions of the HeartTranspositions of the Heart

• D-transposition of the Great Arteries (D-TGA)p ( )

• Senning Procedure/Mustard Procedure (Atrial Switch)• Arterial Switch

• Congenitally Corrected Transposition of the Great Vessels (L-TGA)

………………..……………………………………………………………………………………………………………………………………..

History – D-TGAAlfred Blalock

History D TGA• 1797- TGV was first described by Matthew

BaillieBaillie• 1950 - First surgical palliation occurred by

Drs. Alfred Blalock & C. Rollins Hanlon• Hole created between top two chambers of the heart

• 1951 - Second type of palliative repair by Müller & Dammann

• Bands on lung artery to prevent too much blood to lungs

………………..……………………………………………………………………………………………………………………………………..

C. Rollins Hanlon

History Åke Senning

• 1957 – More long-term surgical palliation developed by Åke Senning

• Surgical creation of baffles/pathways outside the heart to direct blood flow to the right places. Baffle within atria with native tissues. Very technically demanding

• 1963 – Mustard Procedure developed by William Mustard• Used pericardial tissue (own heart tissue from the sac p (that surrounds the heart) to perform operation similar to Senning

When asked to comment on use of an artificial material for atrial baffle, Mustard replied jokingly: “Use pericardium! The only excuse for using Dacron is if you drop the pericardium on the floor”

………………..……………………………………………………………………………………………………………………………………..

William Mustard

HistoryHistory

• 1982 – First successful Arterial Switch procedure performed by p p yAdib Jatene

………………..……………………………………………………………………………………………………………………………………..

Normal Blood Flow Through the Heart

Blood Flow Through the Heart – YouTubehttp://www.youtube.com/watch?v=FCimR_P9ID0………………..……………………………………………………………………………………………………………………………………..

p y

D-TGAD TGA

• D-TGA (Dextro Transposition) or “swapped” ( p ) pparteries (aorta and pulmonary artery)

• This is also sometimes called complete transposition of the great arteries

• It is called a cyanotic (blue baby) congenital heart defect (CHD) because the newborn baby turns blue from lack of oxygen.

………………..……………………………………………………………………………………………………………………………………..

Mustard ProcedureMustard Procedure

http://www.youtube.com/watch?v=O83cYwKOKtI………………..……………………………………………………………………………………………………………………………………..

Arterial Switch ProcedureArterial Switch Procedure

………………..……………………………………………………………………………………………………………………………………..

………………..……………………………………………………………………………………………………………………………………..

Long Term Complications…

D-TGA Mustard/Senning Procedure

• Arrhythmias

• Baffle Leaks

• Baffle Obstruction

• Heart Failure

………………..……………………………………………………………………………………………………………………………………..

Long Term Complications…D-TGA• Arrhythmias

•Electrical system of heart in the atrium (upper chamber of heart) is disturbed by stitches used during operation

•Problems with abnormal heart rhythms from the atria

•Patients own “pacemaker” often damaged during operation causing slow heart rates

•Artificial pacemaker may be required to keep your heart rate normal

………………..……………………………………………………………………………………………………………………………………..

PacemakersPacemakers

………………..……………………………………………………………………………………………………………………………………..

Long Term Complications D-TGALong Term Complications…D TGA• Baffle Leaks

• Small hole/leak that can develop / pwithin the previous surgical repair

• These leaks are important• These leaks are important because this can lower your blood oxygen levels (mixing of bl e and red blood)blue and red blood)

• These leaks put you at an increased risk for a blood clot or stroke

• Often can be repaired in the cath lab vs needing to have surgery

………………..……………………………………………………………………………………………………………………………………..g g y

Long Term Complications…D-TGA• Baffle Obstructions

• Narrowing/scarring of surgical repairs (baffles to redirect blood flow) causing(baffles to redirect blood flow) causing decreased blood flow (obstruction)

• Can result in decreased blood flow to the lungs or blood coming back to the heart

• This can often times be repaired in the cath lab with a stent. Some repairs maycath lab with a stent. Some repairs may require surgery.

………………..……………………………………………………………………………………………………………………………………..

Long Term Complications D-TGALong Term Complications…D TGA• Heart Failure

• The blood flow of the heart hasThe blood flow of the heart has been repaired by surgery, although this does not mean your heart is normalnormal• The right ventricle (pumping chamber) is designed to pump to h l h i i ithe lungs, however it is pumping

to the body• Over years, the right pumping chamber wears out and becomes large, weak, and less effective

………………..……………………………………………………………………………………………………………………………………..

Long Term Complications…D-TGAg p

• Arterial Switch ProcedureP l A S i ( i )• Pulmonary Artery Stenosis (narrowing)

• Coronary artery abnormalities• Compressionp• Thickening• Narrowing

• Aortic Root Dilation (enlargement)• May be related to pulmonary artery tissue that is now part of the aortap

• Aortic Insufficiency (leaky valve) • Related to other surgical repairs at time of switch or

ld r t th tim f r p ir r rti r t dil ti n………………..……………………………………………………………………………………………………………………………………..older age at the time of repair, or aortic root dilation

Pulmonary Artery Stenosis (Narrowing)Pulmonary Artery Stenosis (Narrowing)

………………..……………………………………………………………………………………………………………………………………..

14 yo D-TGA s/p ASO with L-CxStretched at its Proximal CourseStretched at its Proximal Course

11 yo D-TGA s/p ASO with LCA Stretched at its Proximal CourseCStretched at its Proximal CourseC

Aortic Root DilationAortic Root Dilation

………………..……………………………………………………………………………………………………………………………………..

Definitions CCTGA (L-TGA)( )• Levo-TGA/CCTGA – Both arteries (aorta & pulmonary) &

both ventricles (pumping chambers) are swapped/switched

Normal Heart

CCTGA

………………..……………………………………………………………………………………………………………………………………..

………………..……………………………………………………………………………………………………………………………………..

CCTGACCTGA • Uncommon form of congenital heart disease

(CHD)• O i 0 5% f th it l h t di• Occurs in 0.5% of the congenital heart disease

population• The blood flow in the heart is normal• The pumping chambers of the heart are just• The pumping chambers of the heart are just

switched• Pulmonary artery displaced• Other associated problemsOther associated problems

• Ventricular Septal Defect (VSD)• Pulmonary Stenosis• Dextrocardia• Dextrocardia• Tricuspid valve problems

• Leaking• Ebsteins anomaly………………..……………………………………………………………………………………………………………………………………..• Ebsteins anomaly

Ventricular Septal Defect

Long Term ComplicationsLong Term Complications• Tricuspid valve leaking

Ab l h h h ( h h i )• Abnormal heart rhythms (arrhythmias)• Right ventricular enlargement• Heart Failure• Heart Failure

P waves

5 second pause………………..……………………………………………………………………………………………………………………………………..5 second pause

Tricuspid Valve LeakingTricuspid Valve Leaking

• Failure of the heart's tricuspidFailure of the heart s tricuspid valve to close properly during systole. As a result, with each heart beat some blood goes backward from normal flow

• To and Fro flow

………………..……………………………………………………………………………………………………………………………………..

Abnormal Heart RhythmsAbnormal Heart Rhythms• Slow heart beat

Normal Heart Conduction Systemhttp://video.about.com/heartdisease/Conduction-System.htm

………………..……………………………………………………………………………………………………………………………………..

AV Block- Slow Heart Beat (bradycardia)AV Block Slow Heart Beat (bradycardia)

P waves

5 second pausep

………………..……………………………………………………………………………………………………………………………………..

Right Ventricular Enlargement & Heart FailureRight Ventricular Enlargement & Heart Failure

• Although the heart has normal physiologic blood flow, anatomic abnormalities still exist. The right ventricle, which usually pumps blood , y p pto the low blood pressure lungs now pumps blood to the body (high pressure)pressure).

• This long term complication is just g p jlike that experienced by patients with D-TGA

………………..……………………………………………………………………………………………………………………………………..

Pregnancy & Transposition g y p• Patients with both D-TGA &

CCTGA can successfully undergo pregnancy & delivery

• Patients should plan pregnancy so that testing and cardiacso that testing and cardiac evaluations are up to date.

• Notify your cardiologist as soon y y gas you know you are pregnant

• You may require the care of a high risk OB along ith o rhigh risk OB along with your cardiologist

………………..……………………………………………………………………………………………………………………………………..

General Pregnancy Informationg y• Many medications are not safe during

pregnancy (harmful to baby)B d i k l f fl id• Be sure to drink plenty of fluid to prevent dehydration

• Have regular follow up with your OB AND your cardiologist

• Most women can have a normal delivery• In most cases a C-Section occurs relatedIn most cases a C-Section occurs related

to complications during labor rather than your heart diseaseAll h ld t k t l• All women should take pre-natal vitamins to prevent certain birth defects although NOT CHD

………………..……………………………………………………………………………………………………………………………………..

Pregnancy CHD & Your BabyPregnancy, CHD & Your Baby• If either parent has CHD there is an

increased risk of the fetus also having CHDg

• Risk for general population 1%

• Risk to fetus with a parent with CHD, 3-10%

• Routine ultrasounds in pregnancy can miss significant heart malformations

• Fetal ECHO recommended for baby

………………..……………………………………………………………………………………………………………………………………..

Fetal ECHO (Ultrasound)Fetal ECHO (Ultrasound)

• Fetal ECHO performed around 22-26 weeks pregnantpregnant

• Will have a consultation with a cardiologist the same day to reviewsame day to review the results

………………..……………………………………………………………………………………………………………………………………..

Keeping Your Self Healthy• Balanced Diet• Maintain a healthy weight• Taking your medications g y d c s• Drinking fluids• Engage in routine physical activity• Attending follow up appointments asAttending follow up appointments as

directed by your doctor• Complete requested testing• Notify us of symptomsy y p• Know & understand your type of heart

disease• Have a plan for emergenciesp g• Be prepared when you travel• Avoid Tobacco products• Alcohol is okay in moderation

………………..……………………………………………………………………………………………………………………………………..y

Growing Up My Pediatric Cardiologist

Got Me This Far…

………………..……………………………………………………………………………………………………………………………………..

Growing oldGrowing old

T h l i l h hTogether, our goal is not only to you them here…

But here and beyond…

………………..……………………………………………………………………………………………………………………………………..

37 year-old with d-TGA s/p Mustard repair26 year-old with d-TGA s/p Mustard repair

26 year-old with CoA and hypoplastic transverse arch 44 year-old with double-inlet LV s/p Fontan………………..……………………………………………………………………………………………………………………………………..

………………..……………………………………………………………………………………………………………………………………..

Questions?Questions?

www.nationwidechildrens.org/ACHD

THANK YOU!THANK YOU!………………..……………………………………………………………………………………………………………………………………..