patient amy in flower fieldNo pediatric cardiac condition is simple – neither from the perspective of the patient and their family, nor from that of the medical team. When a heart condition is even more rare than usual, the challenges become significantly greater. To navigate and overcome these challenges, a combination of three key elements is often essential: courage, confidence and precision.

Fortunately, for Amy, she benefitted from all three.

Courage: For the patient, to face overwhelming odds with bravery; for the medical team, to embrace the complex challenges with resolve and determination. Courage is the foundation upon which many medical battles are fought and won.

Confidence: For the patient, to trust in the process and face each step with strength; for the care team, to execute each procedure with the utmost confidence in their skills. This mutual confidence is crucial in turning challenging situations into successful outcomes.

Precision: For the patient, to carefully engage in their own care, asking questions and understanding each step of the treatment process; for the surgical team, to perform with accuracy, where there is no room for error and no time to spare. This collaboration helps enable high standards of care and positive outcomes.

For Amy Simpson, a charming 17-year-old diagnosed with Carney Stratakis Syndrome – a rare genetic condition that led to the development of a life-threatening tumor pressing against her heart – these three elements came together to pave the way to recovery.

Amy’s successful medical journey was made possible thanks to the experience and dedication of her team at the Children’s Heart Institute at Children’s Memorial Hermann Hospital, including Jorge Salazar, MD, affiliated pediatric heart surgeon and executive director of the Children’s Heart Institute, and Avichal Aggarwal, MD, a pediatric cardiologist affiliated with Children's Memorial Hermann Hospital, and the dynamic, multidisciplinary team at the Children's Heart Institute.

Together, Amy and her team navigated through complex medical and emotional challenges with a level of courage, confidence and precision that saved her life and revitalized her future.

Facing the unthinkable: 400 cardiac events in six days

Even at a young age, Amy was no stranger to medical treatments and hospitalizations. As an 11-year-old living in Colorado, she began experiencing dizzy spells and stomach pains that led to hospitalizations and treatment for endocrine issues. She frequently became pale and passed out, with regular hospital visits becoming a part of her adolescence.

As a teenager now living in the Rio Grande Valley in South Texas, about a six-hour drive from Houston, the symptoms of her childhood returned with alarming intensity. After visiting a local hospital, she was fitted with a heart monitor. Less than one week later, the monitor picked up on some major irregularities with her heart. The local hospital staff recognized the severity of her condition and advised her to seek specialized care immediately at Children's Memorial Hermann Hospital. The seriousness of this situation was evident just 10 days after Amy’s initial appointment at her local hospital.

From fear to hope

“I felt like my body was betraying me all over again, and the thought of another hospitalization was daunting,” Amy shared, reflecting on her fears. “I was really scared, but I held onto a glimmer of hope that somewhere out there, there were doctors who could truly help me.”

Upon reviewing Amy’s case, Dr. Aggarwal quickly realized the urgency of the situation and immediately got her transferred to Children’s Memorial Hermann Hospital for further care. "Amy's case was one of the most severe I've encountered. She had more than 400 cardiac asystolic events in just a six-day period where her heart would stop beating from anywhere between 4 and 25 seconds at a time. Any pauses over three seconds could cause serious damage,” said Dr. Aggarwal. “This is equivalent to having 70 heart attacks a day. She was a ticking time bomb because every time her heart stopped, there was a chance it wouldn’t start again. The tumor was compressing the main blood and oxygen supply to her heart. Her survival up to that point was nothing short of miraculous, and it highlighted the need for immediate and decisive medical intervention."

Despite the dire circumstances, Amy's perspective began to shift from fear to hope. "Everything changed when I met the team at Children's Memorial Hermann Hospital. Their confidence and readiness to take on my case head-on transformed my outlook. For the first time in a long while, I felt hopeful about overcoming this," Amy said.

As she prepared for surgery, this new-found optimism was a turning point for the teenager who had already been through so much.

Surgical strategy and execution

At the Children's Heart Institute, the affiliated medical team conducted a series of comprehensive tests on Amy, including an echocardiogram and a cardiac CT scan, that revealed the tumor was encircling her left main coronary artery, compressing it to a mere 10%-15% of its normal diameter. This constriction posed an immediate and grave threat to her cardiac function, escalating the urgency for a precise and strategic medical response.

The tumor, identified as a paraganglioma associated with Carney Stratakis Syndrome, was not just a cardiac risk but also a rarity in its presentation, complicating the prognosis and necessitating an aggressive and informed approach to treatment.

"Amy's tumor was compressing her left main coronary artery, essentially acting like a blockage in the gas line to a car's engine. Just as a car cannot run without gas, the heart cannot function without blood flow. This blockage posed a severe risk to her life, making immediate and decisive action crucial," said Dr. Salazar. "Any further growth of this tumor could have completely shut off her heart. In critical situations like this, every second counts."

A collaborative surgical strategy was swiftly put together by the Children’s Heart Institute team, involving affiliated pediatric anesthesiologists, cardiologists, oncologists, nurses and other specialists. This diverse team of specialists was essential in addressing the high-stakes intricacies of Amy's case, ensuring that her treatment was coordinated and executed with precision.

At Children’s Memorial Hermann Hospital, Amy's operation, which was meticulously planned and would last a total of six hours – with four crucial hours dedicated to the removal of the tumor – posed significant challenges. The tumor had deeply integrated with the coronary artery, complicating its removal. Dr. Salazar and the surgical team had to employ innovative techniques and adapt swiftly to the evolving situation. On-the-spot decisions led to the reconstruction of parts of Amy's heart, involving careful dissection and the preservation of as much heart tissue as possible while removing the tumor and reconstructing the affected areas.

Recovery and a new lease on life

Following the surgery, Amy was cared for in the Pediatric Heart Intensive Care Unit (ICU) at Children’s Memorial Hermann Hospital, where she began to show remarkable improvements. The heart ICU features the most advanced technology and equipment for babies and children in need of specialized cardiac care.

"It felt like a miracle. Within a short time, I could feel my energy returning, and I couldn't believe the difference," Amy shared, reflecting on the immediate effects of the surgery. This rapid improvement was an indication of the operation's success, considering her previous severe cardiac events.

Dr. Aggarwal, who was closely monitoring Amy's post-operative condition, added, "After the surgery, Amy was not experiencing any more pauses in her heart rhythm. A follow-up CT scan added to the good news, showing that the coronary artery previously compressed by the tumor had been restored to its full diameter, allowing proper blood flow and significantly reducing the risk of future cardiac events."

From Amy's perspective, the journey through her medical crisis was transformative. "The doctors and care team didn't just treat me; they saved my life. Dr. Salazar was like my own personal Superman," Amy said, highlighting the impact of her surgeons' confidence and support. "They gave me the strength to face this challenge and helped me trust in the recovery process."

A life ready for the future

Now back home in the Rio Grande Valley, Amy has rekindled her love for creating music, which has been helpful in her recovery. Able to engage in activities like weightlifting and exercise just like any other teenager, she has found new joy in her hobbies. According to Amy, the surgery helped her overcome not just her physical challenges but also the mental burdens that weighed on her. Currently, Amy is completing her junior year of high school and is looking forward to college.

Amy shared some heartfelt advice for others facing daunting health challenges. "The journey might feel overwhelming at times, and sometimes it might seem like there's no hope. But remember, you always have to keep pushing forward; not just for your friends and family, but for yourself. It's crucial to find courage and confidence within yourself, as I did during my treatment. These qualities can truly make a difference in your recovery. Keep going, because it will be worth it in the end."

At the Children's Heart Institute at Children’s Memorial Hermann Hospital, each patient's journey is more than a medical case; it is a shared mission to operate on the forefront of what is medically possible while nurturing the dreams of those we serve.

Find more information on innovative cardiac care and to read more inspiring stories like Amy's »

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