Pulmonary Hypertension: Emma’s Story

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Emma was born with a heart defect and diagnosed with pulmonary hypertension at just 3 months old. Now 9, she is healthy and able to participate in the activities she loves, like swimming and dancing, thanks to the Cardiac Center at Children’s Hospital of Philadelphia. 

Emma swimming The first indications of Emma’s heart problems came during a 20-week ultrasound. The technician didn’t like the way her heart looked, and he was concerned that he couldn’t see her  stomach. An appointment was made for higher resolution imaging with fetal diagnosis specialists at Children’s Hospital of Philadelphia (CHOP).

The doctors from CHOP diagnosed Emma with tetralogy of the Fallot with pulmonary atresia (TOF/PA). She had a hole between the two bottom chambers of her heart, a problem known as a ventricular septal defect (VSD). And her pulmonary valve, which opens from the heart to the pulmonary artery, was shut, blocking deoxygenated blood from flowing to the lungs. She would need surgery soon after birth to unblock the valve. Doctors were also concerned with Emma’s small stomach.

Emma’s parents, Melissa and Matthew, arranged for delivery at the Hospital of the University of Pennsylvania (HUP), which is right next the CHOP, so that the newborn would have access to immediate medical care.

Today, babies like Emma who require specialized care before, during or after birth can be delivered in CHOP’s Garbose Family Special Delivery Unit, the first birth facility in a pediatric hospital designed for healthy mothers carrying babies with known birth defects. Moms can stay close, and babies are treated immediately without having to be transported.

As soon as she was born, Emma was taken to the Cardiac Intensive Care Unit (CICU) at CHOP. Her medical team determined that she would need immediate surgery, but not for the reason they expected. Emma’s trachea wasn’t connected to her esophagus, meaning that there wasn’t a pathway from her mouth to her stomach. The problem was corrected with a surgical procedure that constructed a pathway so that when she recovered, Emma could properly swallow.

A lifesaving procedure

Three weeks after she was born, Emma had the procedure to repair her heart and the circulation between her heart and lungs. The operation was performed by Thomas Spray, MD, Chief of the Division of Cardiothoracic Surgery. Dr. Spray closed the hole between the two ventricles (the VSD), and reconstructed the pulmonary valve to enable blood to flow from the heart to the lungs for oxygenation.

Before she was released, Emma also had a procedure called a cardiac catheterization. An interventional cardiologist used long flexible tubes to place stents in Emma’s pulmonary artery to keep it open to an appropriate width.

Emma went home after spending a month in CHOP’s CICU and her parents were trained in the special care she would require. She was on oxygen, and she needed to be fed at frequent intervals. She had no natural desire to eat and could not eat much at each feeding, so she needed to be prompted to eat and fed in small amounts.

During her first few months at home, Emma returned to the Hospital for regular follow-up appointments with her Cardiologist, Geoffrey Bird, MD.

A new diagnosis: pulmonary hypertension

When she was 3 months old, Dr. Bird diagnosed Emma with pulmonary hypertension, a condition in which the blood flowing from the heart to the lungs is being pumped at too high of pressure. Untreated, this can lead to a weakening of the heart. He prescribed a combination of medications, to be taken by mouth, to reduce the pressure by opening the artery between the heart and lungs.

Emma did well for the next year, but then battled with respiratory syncytial virus (RSV). She was admitted to the hospital three times for RSV, and her vital signs were particularly worrisome during her third visit — she was actually close to heart failure.

Emma’s team decided to put in a Broviac catheter, a tube inserted just under the collarbone and fed through a vein to the heart, as a way to deliver higher doses of the medication being used to treat Emma’s pulmonary hypertension. That worked to stabilize her condition, and once she had recovered from the RSV, the catheter was kept in place to administer the medications she required.

Monitoring Emma’s progress

Emma smiling Emma’s care continues with the Pulmonary Hypertension Program, and her team worked with the family to reduce the doses of Emma’s medication. The Broviac catheter was replaced with an IV line, and then eventually by oral medications — a significant breakthrough. This is how Emma now gets her treatment for pulmonary hypertension.

Now 9, Emma comes to CHOP every three months for appointments. At these visits, her heart and lung functions are checked with an electrocardiogram (EKG), echocardiogram and a six-minute walk test, and blood tests are done to monitor the effect of her medication. These tests are supplemented every two or three years by a cardiac catheterization to get an inside look at her heart.

Emma is able to participate in activities she enjoys. She’s on a swim team, and she takes dance classes — tap and jazz. Emma loves arts and crafts and playing with her dolls and stuffed animals.

Her parents are happy to see her enjoying life and thankful for the expert care that is helping her do what she loves. 

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