Thursday, January 26, 2012

Hypoplastic Right Heart Syndrome

Hypoplastic right heart syndrome (HRHS) refers to underdevelopment of the right sided structures of the heart. These defects cause inadequate blood flow to the lungs and thus, a blue or cyanotic infant. The major problem is pulmonary valve atresia (absence). This valve normally opens and closes to let blood flow to the pulmonary artery. Secondary problems include a very small (hypoplastic) right ventricle (lower chamber which normally pumps blood to the lungs); a small tricuspid valve (this valve allows blood to flow into the right ventricle) and a small (hypoplastic) pulmonary artery. Also, the blood flow into the coronary arteries may be abnormal causing damage to the heart muscle.

The infant is born with two connections that help blood flow. These are a foramen ovale (hole between the atria) and patent ductus arteriosus (or PDA, a blood vessel between the aorta and pulmonary artery). As these connections begin to close, the infant becomes critically ill.

Because the blue blood cannot pass through the right side of the heart to get to the lungs, it crosses into the left atrium and mixes with red blood returning from the lungs. This mixed blood is pumped out of the aorta. The only way in which blood gets to the lungs is through the PDA. The PDA must be maintained open with medicine (PGE1). Surgery is usually performed shortly after starting PGE1 to create an artificial connection (shunt) between the aorta and the pulmonary artery to deliver blood to the lungs.

Sunday, January 22, 2012

Congenital Heart Defects

I mentioned in my first blog that I would explain in greater detail Baby Hartwell's heart defects and the surgeries he/she will need. I’m thinking I will explain each defect over the next several days. It is all very overwhelming so I thought I would start off by explaining what complex congenital heart defects are before describing each individual defect.

So here it goes.....

Congenital heart defects are problems with the heart's structure that are present at birth. These defects can involve the interior walls of the heart, valves inside the heart, or the arteries and veins that carry blood to the heart or out to the body. Congenital heart defects change the normal flow of blood through the heart.

There are many different types of congenital heart defects. They range from simple defects with no symptoms to complex defects with severe, life-threatening symptoms.

Congenital heart defects are the most common type of birth defect, affecting 8 of every 1,000 newborns. Each year, more than 35,000 babies in the United States are born with congenital heart defects. Most of these defects are simple conditions that are easily fixed or need no treatment.

A small number of babies are born with complex congenital heart defects that need special medical attention soon after birth. Over the past few decades, the diagnosis and treatment of these complex defects has greatly improved.

As a result, almost all children with complex heart defects grow to adulthood and can live active, productive lives because their heart defects have been effectively treated.

Most people with complex heart defects continue to need special heart care throughout their lives. They may need to pay special attention to certain issues that their condition could affect, such as health insurance, employment, pregnancy and contraception, and preventing infection during routine health procedures. Today in the United States, about 1 million adults are living with congenital heart defects.

Tuesday, January 17, 2012

Baby Hartwell- Our Heart Hero

Michael and I are beyond excited to welcome a new member to our family this spring. Baby Hartwell is scheduled to join us around April 15, 2012. We've decided to keep it's gender a surprise, and can't wait to find out if it's a boy or girl! We've recently learned that Baby Hartwell has a chronic heart disorder, and we will be using this blog to keep all of our amazing friends and family up to date.
On December 7th, Mike and I went to my regular 21 week appointment, thrilled to get to see our baby for the first time on the ultrasound. The tech was very nice, and mentioned that the baby was being stubborn, and she couldn't get a good picture of the right side of the heart. My obgyn Dr. De Vries then came in, and also tried to get some additional pictures. I received a phone call the next day from Dr. De Vries when he informed me that after re-looking at a few of the pictures, he thought the right side of the baby's heart looked a bit "small" and had gone ahead and set up an appointment with us to meet with Dr. Danford, a pediatric cardiologist specialist at Childrens Hospital Omaha on January 6th. Mike and I tried to not assume the worst, but just that they couldn't get a "good" picture.
We met with Dr. Danford on the 6th and learned that Baby Hartwell has Pulmonary Atresia and Hypoplastic Right Heart Syndrome. More or less, blood is unable to pass through the pulmonary artery due to lack of pulmonary valvues and an underdeveloped right ventricle of it's heart. We've learned that HRHS is rare, and most information and cases are usually Hypoplastic LEFT Heart Syndrome. Dr. Danford was able to draw us a "map" of Baby Hartwell's heart, and also describe the surgeries he/she will have.
At this point, we've been told I'll have a "normal" delivery at Methodist Women's Hospital (plus about 20+ people, specialists, etc in the room I'm guessing LOL). The baby will be immediately stabilized and transferred to Children's Hospital. Mike will go with the baby until I can be released. It will  be given a drug to keep one of it's valves open temporarily. It will undergo open heart surgery within it's first week. This procedure is to put in a shunt, which will direct blood to the lungs.
Baby Hartwell will undergo it's second open heart surgery at age 4-6 months. At this time they will be doing a procedure called a "Glenn" which will replace the shunt that the baby will have out-grown.
It's third open heart surgery will be around age 2, where they will finalize the process by doing a "Fontan" operation.
Each of these procedures is incredibly scary. Baby Hartwell will never have a "normal" heart, but instead function on a single ventricle heart, where the left side will do all of the pumping for both sides. The first two surgeries are designed to temporarily relieve blood flow to and from the lungs. The third surgery is to improve overall circulation. The procedures do not cure the defects, but rather re-routes the blood flow around the defective areas. 
As unlucky as this may all seem, we are very lucky for several reasons. One of those is the fact that we live so close to a town where we have access to a wonderful team of physicians who are dedicated to the care of patients with heart disease. We are also very lucky that the Children’s Hospital in Omaha has surgeons who perform surgeries on babies born with heart defects. So, with all of this said, I have decided to start a blog. We feel so grateful to have our family and friends. It is during times like this, we realize how truly blessed we are. We can’t thank each of you enough for your prayers and support thus far. My hope is for this blog to keep each of you updated on my pregnancy and updated on Baby Hartwell's progress. I'll update with additional posts that explain the exact defects Baby Hartwell has.