Mended Little Hearts Open Forum

Hello- my boy has been diagnosed with ToF at 25 weeks gestation. I am trying to understand if the ToF is being complicated with other issues suck as Pulmonary Atresia or MAPCAS or other, and I'm not sure I'm able to interpret the notes correctly.  We are also sending off amnio fluid for 22 deletion testing.

Can someone tell me if this is "run-of-the-mill" Tetralogy of Fallot with no further complications, or otherwise? Thank you so much. This has been difficult for us. We are looking for a surgeon in St. Louis.

"Single gestation with fetal lie in cephalic presentation. All four cardiac chambers were visualized. The cardiac chambers were balanced in size. A moderate to large outlet perimembranous ventricular septal defect was seen. The aortic valve was overriding the ventricular septal defect. The pulmonary valve annulus was hypoplastic. The main pulmonary artery branches were moderately hypoplastic. These findings were consistent with tetralogy of Fallot.. A stretched patent foramen ovale seen .. No pericardial effusion was seen. No pleural effusion seen. No ascites seen. No mass is seen around the heart. The heart appeared to be located in the normal position in the chest. Pulmonary venous drainage, systemic venous drainage, and aortic arch could not be well visualized but most likely normal No diaphragmatic hernias seen. Normal 3 vessel cord."

"Tetralogy of Fallot with moderately hypoplastic pulmonary valve annulus, main pulmonary artery and branch pulmonary arteries."

Hello Ray,

I'm sorry that I'm just seeing this now so please forgive my delay in responding.  I was born with Tetralogy of Fallot  57 years ago.  Yes, Pulmonary stenosis (narrowing) or pulmonary atresia (closure) is one of the 4 defects of Tetralogy of Fallot.  I had to look up MAPCA but it seems that developed out of need to get blood flow to the lungs an alternative way since the pulmonary valve was blocked - atresia.  This seems it was in response to a severe case of Tetralogy of Fallot.  I had pulmonary stenosis (narrowing) but did have an extra flap of skin that would close over my pulmonary valve.  For this reason, I needed palliative surgery at 14 months old which was a BT shunt that held me over until I could have my total repair at the age of 6.  I would get Tet spells and turn blue and have difficulty breathing.  However, this shunt is no longer needed as technology has made it possible for cardiologists to perform surgery on the tiniest of hearts.  Every situation is different but some surgeries for this congenital defect - Tetralogy of Fallot can be done in utero or shortly after birth.  Children don't have to wait for surgery like I did 50 years ago.  I am glad to report that I fully recovered from my surgery and went on to live a very full life with dancing lessons, roller skating, piano lessons and I was able to deliver both of my children naturally without complications.  I still go to Children's Hospital for check ups at their Adult Congenital Heart Clinic ACHC and only recently developed an arrhythmia that meds are keeping in control.  I do have a leaky valve the doctors are watching but I haven't needed it to be changed yet.

I know outcomes are even better now but I feel I was given a second chance to live a great life!  There is so much HOPE!  I will hold you and your baby boy in prayer.  Please keep me posted and take care of yourself too!  This is a lot of stress for parents.

Gratefully,

Tina Marshall