TOF/PA with well developed central pulmonary arteries

Primary complete repair

Redmond P. BurkeChief, Division of Cardiovascular Surgery

The Congenital Heart InstituteMiami Children’s Hospital and Arnold Palmer Hospital

Cara GuentherUndergraduate Fellow

www.pediatricsurgery.com

What is TOF/PA? Children with TOF/PA illustrate the

following four characteristics: A hole in the ventricular septum (VSD) An anterior shift of the aorta, resulting in an aorta that

lies above the VSD (“overriding aorta”) Obstruction of right ventricular outflow (pulmonary

atresia) – smaller pulmonary artery, and muscular narrowing below the valve

Thickening of the right ventricle (ventricular hypertrophy) since the heart has to pump harder to compensate for the pulmonary atresia

Info from: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Tetralogy-of-Fallot_UCM_307038_Article.jsp

Normal Heart vs. Heart with TOF/PA

Normal Heart Heart with TOF/PA

*Please note the intact ventricular septum, the location of the aorta, the size of and flow through the pulmonary artery, and the thickness of the right ventricular muscle

*Please note 1) pulmonary atresia, 2) right ventricular hypertrophy 3) overriding aorta and 4) VSD

Info from: http://kardiol.com/?p=263 and http://www.nhlbi.nih.gov/health/dci/Diseases/tof/tof_what.html

How does TOF/PA affect my baby’s heart and health?

As a result of the obstruction to the pulmonary artery, poorly developed tricuspid valve, and muscular narrowing near the pulmonary valve, your child has decreased blood flow to the lungs, which causes:

The appearance of blue or purplish skin (cyanosis) Bulging nailbeds in fingers and toes (clubbing) Shortness of breath Fatigue

Information from: http://www.healthscout.com/ency/68/711/main.html

Conventional Approaches

The approach used in most centers begins with an echo diagnosis, followed by diagnostic Catheterization, shunt, repeat catheterization, and finally a repair when the child is several years of age.

In some programs, these surgeries are performed on circulatory arrest.

We believe this approach results in significant cumulative trauma to the patient and family.

Miami Children’s Hospital Approach to TOF/PA

We reduce cumulative trauma by performing early complete repair. This reduces number of operations and

hospitalizations• Avoiding the shunt eliminates an entire operation

and a thoracotomy incision We perform the repair on bypass and

avoid circulatory arrest. This method reduces the potential for

neurologic injury.

Our Program Philosophy

To treat each patient with the most effective and least traumatic approach available

Case Report: Baby Girl 35 week with placenta previa (a condition

in which the placenta is located in the lower uterus) and chromosomal analysis 46XX, apgars 7/8, weight 2.9kg

Echo dx of TOF/PA, no collaterals, normal coronary arteries, good sized confluent branch pulmonary arteries with pulmonary atresia

No catheterization Planned complete repair

Images from https://irounds.mch.com/

Preoperative Image

Images from https://irounds.mch.com/

Surgery: Steps 1-4

Images from https://irounds.mch.com/

Pulmonary artery

Duct

• Open chest, harvest pericardium, place patient on heart-lung bypass, and locate duct dependent pulmonary artery

Surgery: Step 5

Images from https://irounds.mch.com/

• Dissect the duct

Duct

Pulmonary artery

Surgery: Step 7

Images from https://irounds.mch.com/

• Measure central pulmonary artery segment (one cm in this case)

Divided duct

Central pulmonary artery segment

Surgery: Step 8• Close VSD via right ventricle on cardiopulmonary

bypass with bicaval cannulation at 25C

VSD

Images from https://irounds.mch.com/

Right Ventricular Incision

Surgery: Step 9• Complete distal anastomosis with an aortic homograft (9mm in this

case) We also use pulmonary homografts and Contegra™ grafts.

Aortic homograft

AortaIncision in pulmonary artery confluence

Images from https://irounds.mch.com/

Day 1 (Operative day): Cross clamp 50 min. Elective open chest, first lactate in CICU

was 6.1, Dopa 5, Milrinone 1.

Images from https://irounds.mch.com/

Day 3 (POD 2): Chest closed, Lactate 1.2

Images from https://irounds.mch.com/

Day 6 (POD 5): Baby is extubated

Images from https://irounds.mch.com/

Day 17 (POD 16): Feeding is improved

Images from https://irounds.mch.com/

Long Term follow-up of patients with neonatal RV-PA conduits

Courtesy of Anthony Rossi MD

Age at conduit explant versus size at implant – up to 15 years of age

Courtesy of Anthony Rossi MD

Age at conduit explant versus conduit size – up to 20 years of age

Courtesy of Anthony Rossi MD

Conclusions

Diagnosis and operative plan can be based on echocardiography alone.

MRI is emerging as a powerful tool for mapping collaterals.

Complete neonatal repair can be accomplished without circulatory arrest.

Mid term outcomes are excellent, and conduits last almost 2 years.

Early complete repair may decrease cumulative trauma.

Dr. Anthony Rossi’s Paper Neonatal Complete Repair of Congenital Heart Disease Requiring a Right Ventricle to Pulmonary

Artery Conduit Plato Alexander, Anthony Rossi, Robert Hannan, Juan Bolivar, Redmond Burke Introduction: The physiologic repair of complex congenital heart problems results in early restoration of

normal cardiovascular physiology. Repair in some patients has been postponed if the need for continuity between the right ventricle and pulmonary arteries required placement of a conduit (RV-PA conduit). We report our experience of attempted neonatal physiologic repair in patients requiring RV-PA conduits.

Methods: We reviewed our surgical database for all neonates who received RV-PA conduits from June 1995-September 2008. We excluded patients undergoing single ventricle palliations.

Results: There were 44 pts undergoing RV-PA conduit repairs at an average age of 10.5 days (range 2-29 days) and weight of 3.1 kg (2-4.7 kg). There were 22 pts with a diagnosis of truncus arteriosus and 19 with VSD pulmonary atresia. Conduits were constructed from valved homografts in 39 pts (pulmonary15, aortic 24) and bovine heterografts in 2 pts (not described in 3 pts). Median size of homografts was 10 mm (7-19 mm). Cardiopulmonary bypass times averaged 176.5 mins (86-331) and aortic cross clamp time averaged 96.8 mins (19-188 mins). Deep hypothermic circulatory arrest was required in only 10 pts (21%). Delayed sternal closure (DSC) in the CICU was used in 23 pts. DSC was performed at an average of 2.5 days (1-5 days). Conduit size did not determine use of DSC (DSC mean conduit size: 10.1 mm, OR sternal closure 10.3 mm). There were 2 hospital deaths (hospital survival 95%) and no late deaths. Pts were ventilated for a median of 6 days postoperatively (2-50 days). Median postoperative stay was 16.5 days (9-144 days) and hospital stay 26 days (3-140 days). Postoperative cardiac complications included JET, SVT, atrial flutter (1 pt each) and ECMO in 3 pts. One pt requiring postoperative ECMO was placed on ECMO preoperatively. There was no heart block. There have been 16 pts who have undergone conduit replacements at a mean of 1.7 yrs (9 mth-4.6 yrs).

Conclusions:

Early physiologic repair of congenital heart disease requiring an RV-PA conduit is possible in most patients and associated with low early mortality and morbidity.

Deep hypothermic circulatory arrest can be avoided in most pts.

The long-term fate of the neonatal RV-PA conduit needs to be determined.