Jean-Paul (Hypoplastic Left Heart Syndrome) - Louisiana Pediatric Cardiology Foundation

Jean-Paul (Hypoplastic Left Heart Syndrome)

Imagine being 25 weeks pregnant and hearing the words, “Your baby boy has a rare congenital heart defect and will die soon after birth.” Those are the words my husband and I heard after going in for a routine sonogram, having had till then a healthy pregnancy. The date was July 28, 2003. As we tried to absorb the words we had just heard, we tearfully looked at each other and we both went numb. The doctor continued to talk, no doubt, but we didn’t really hear much beyond, “We’re setting up an appointment with some specialists…”

Over the next 6 weeks, we met with 4 different specialists to narrow-down the diagnosis, to discuss our options, and to try and answer all of the questions we had on variations of Hypoplastic Left Heart Syndrome. We went about living our everyday lives as normal as we could for Jean-Luc, our then 3-year-old little boy, although the pain in our hearts was sometimes unbearable. We prayed every day and had our families, friends, and friends of friends also praying. We needed a miracle!

We had 3 options: (1) Abort (which was not an option we even considered); (2) multiple open-heart surgeries over a few years (which we researched endlessly); and (3) comfort care (which we ultimately decided on). One statement from one of the specialists still sticks with me today. While discussing the surgeries our baby boy would have to endure, he said, “I do feel like we would be doing something TO the child instead of FOR the child.” Even though our decision for comfort care was made, as the parents of one healthy little boy and my carrying in-utero another precious little boy with a broken heart, we both prayed for peace with our decision and placed our baby in God’s hands!

We met with our parish priest for a heart-to-heart, and we also met with Hospice to discuss the role they would play once we brought our little boy home from the hospital – he was not expected to live beyond a week. My husband also met with our church to discuss funeral arrangements. As you can imagine, this was a time of unspeakable sadness.

jean-paul-1Fast forward to November 12, 2003, the delivery date. By then, we had been blessed with God’s peace, knowing within that everything was in His hands and in His time! After the delivery, the doctors took our precious Jean-Paul to evaluate his heart. Several long hours later, Dr. Hixon, our pediatric cardiologist, came to my room and said the new imaging studies showed Jean-Paul “has a few more heart parts to work with” and he thought the staged surgeries would work well for him! Our miracle had been answered! We understood that God doesn’t always give you what you want, but He gives you what you need. In our case – or rather Jean-Paul’s – He gave him enough “heart parts” to work with.

The next morning he was air-lifted to Ochsner in New Orleans. Tears of joy and tears of uncertainty rolled down my face while kissing him goodbye, then watching the helicopter disappear to a dot, then nothing, in the sky.

jean-paul-2On November 18, 2003, at 6 days old, Jean-Paul had his first open-heart surgery. It was lengthy, yet all seemed to go well. But, as he was being wheeled up to NICU, his little heart stopped…and went into cardiac arrest! An emergency surgery was immediately done in NICU, as there was no time to get him to the O.R. to remove the clot! He had more complications and set backs over the next few weeks, which required 2 additional surgeries, now totaling 4 open-heart surgeries, but he was finally discharged on December 22nd after a 6-week hospital stay, and on our older son’s 4th birthday, AND home for Christmas!!

His next staged surgery was performed on May 21, 2004, at 6 months of age. After the doctors corrected a few complications, he was discharged 11 days later.

jean-paul-3Jean-Paul continued to thrive, but in July of 2007, at 3 years of age, it was time for his final staged surgery. His cardiovascular surgeon had left New Orleans after Hurricane Katrina, so plans were made for his surgery at Boston Children’s Hospital. After an 18-day stay, a few scares, and a blocked SVC that miraculously “opened” overnight, we arrived home hoping this is his final open-heart surgery!

Today, Jean-Paul is only taking a few oral medications and sees Dr. Hixon about every 3 to 4 months or so. He literally does most everything all other 4-year-old little boys like to do! We have been truly blessed to be Jean-Paul’s parents. He is our miracle baby!

We thank Dr. Hixon and his team at Pediatric Cardiology Associates for their guidance, special care, and friendship. We also especially wish to thank Sr. Dulce, our family, and friends for their many prayers and support. Further, we are both proud and grateful to be a part of the LPCF and its dual mission to promote public awareness and to support families whose children are born with congenital heart defects and heart disease.

Summary of Jean-Paul’s medical issues

  • Single ventricle (no left ventricle)
  • Mitral atresia*
  • Transposition of the great arteries
  • Pulmonary atresia*
  • Atrial septal defect
  • Left branch pulmonary artery stenosis/atresia*
  • Tricuspid atresia*
  • Bilateral superior vena cavae
  • Patent ductus arteriosis, resolved
  • Supraventricular tachycardia, resolved
  • Congestive heart failure, controlled

* absent

Summary of major surgeries/procedures

  • November 18, 2003: Surgery #1, Stage 1: Modified right Blalock-Taussig shunt, PDA ligation and atrial septectomy
  • November 18, 2003: Surgery #2 due to complications
  • December 8, 2003: Surgery #3 due to complications
  • December 9, 2003: Surgery #4 due to complications
  • December 31, 2003: Pulmonary flow scan
  • April 14, 2004: Heart cath — pre-op
  • May 21, 2004: Surgery #5, Stage 2: Bilateral bi-directional Glenn and PA angioplasty
  • May 24, 2004: Heart cath due to severe central/RPA stenosis (dilation)
  • August 24, 2004: Heart cath due to occluded PA and SVC (dilation and stent)
  • April 27, 2007: EP study
  • July 30, 2007: Heart cath — pre-op
  • August 3, 2007: Surgery #6, Stage 3: Fenestrated Fontan, pericardial patch augmentation of CPA and atrial septectomy
  • August 10, 2007: Sedated echocardiogram due to obstructed SVC… but heart cath cancelled as clot disappeared!