Michael’s Resilient Journey: Overcoming the Challenges of Scimitar Syndrome
During a routine medical procedure for a gallbladder infection, SanJuanita Rivera received unexpected news.
I was 8 weeks pregnant,
SanJuanita said. The doctors removed my gallbladder, and that’s when I discovered I was expecting my fourth baby. I had no early symptoms, so it was a complete shock.
SanJuanita’s pregnancy progressed smoothly under the care of her OB-GYN in San Antonio.
However, antibodies were identified in SanJuanita’s blood that could potentially cause anemia in her baby, Michael. This prompted a referral to a Maternal-Fetal Medicine, or MFM specialist.
Although the initial ultrasounds showed no issues, the presence of the antibodies meant that SanJuanita needed to be closely monitored throughout her pregnancy.
Pediatric Fetal Care
Fetal Care Center at CHRISTUS Children's
The Fetal Care Center connects pediatric specialists to pregnant women and their families, regardless of where they plan to deliver their baby. Our Fetal Care Center is committed to patient- and family-centered care.
Unexpected diagnosis: Discovery of a Heart Condition
During her third trimester at 29 weeks, a routine ultrasound revealed what appeared to be a congenital diaphragmatic hernia (CDH), a condition where the baby’s abdominal organs protrude into the chest cavity through an abnormal opening in the diaphragm.
Doctors first thought it might be CDH after spotting a shadow during the ultrasound scan.
Later, an MRI revealed the mass seen on the ultrasound was part of Michael’s heart, and a portion of his lung was receiving insufficient oxygen,
SanJuanita said. I was told he had a heart condition, but I wasn’t sure of the specific diagnosis at that time.
In addition to regular OB-GYN and MFM appointments, SanJuanita was referred to Dr. Monesha Gupta, a pediatric cardiologist at CHRISTUS Children’s, who performed regular fetal echocardiograms to closely monitor Michael's heart in the months before his birth.
Dr. Gupta first evaluated Michael at 38 weeks of pregnancy. She detected a pulmonary venous anomaly and recommended hospitalization in the neonatal intensive care unit (NICU) with further cardiac evaluation after birth.
Monitoring Michael after Scimitar Syndrome Diagnosis
On February 10, 2024, SanJuanita and her husband welcomed their son Michael into the world, weighing a healthy nine pounds. He was quickly transferred to the neonatal intensive care unit (NICU) at CHRISTUS Children’s, where his early days were spent under close monitoring by his medical team.
While he was being evaluated in the NICU, I learned that my baby had Scimitar syndrome,
SanJuanita said. I had never heard of it before, and knowing it was such a rare condition made me feel nervous and overwhelmed.
Soon after birth, Dr. Gupta performed a cardiac CT angiogram and diagnosed the baby with pulmonary venous anomaly and Scimitar syndrome.
CHRISTUS Children's The Heart Center
The Heart Center at CHRISTUS Children's provides comprehensive care to children with heart problems. Our integrated group of specialists bring together a multidisciplinary approach from many specialties to care for children with heart defects.
What is Scimitar Syndrome?
Scimitar syndrome is a rare and complex congenital heart defect that presents unique challenges for patients. This complex disease is characterized by an:
- Underdeveloped right lung: The right lung is smaller than normal and may not function properly.
- Anomalous pulmonary veins: Pulmonary veins from the right lung drain into the inferior vena cava instead of the left atrium, causing a mismatch in blood flow.
- Heart and lung challenges: This abnormality can lead to significant respiratory issues, recurrent lung infections, and heart complications.
Treatment for Scimitar Syndrome
Treatment for Scimitar syndrome typically involves a multidisciplinary team approach, including pediatric cardiologists, cardiothoracic surgeons, pulmonologists, and other specialists. The primary goals are to improve heart function, optimize lung development, and alleviate symptoms such as shortness of breath and recurrent lung infections. This comprehensive approach may include:
- Monitoring and management: Regular check-ups with pediatric cardiologists and pulmonologists to monitor heart and lung function.
- Surgical intervention: Surgery may be necessary to reroute the anomalous pulmonary veins and correct any associated heart defects.
- Supportive care: Managing symptoms such as shortness of breath and preventing lung infections through medication and other therapies.
Early diagnosis and comprehensive care are crucial for optimizing outcomes and ensuring a better quality of life for those affected by Scimitar syndrome.
A Rare Diagnosis
Michael's case of Scimitar syndrome is extremely rare and complicated. According to Dr. Gupta, he had an unusual issue where a major vein on the right side of his heart (right superior vena cava) was missing, and some of the veins that should return blood to the heart were incorrectly connected to another vein (left superior vena cava). This specific combination has not been described in medical literature before.
Despite facing challenges, including aspiration issues unrelated to Scimitar syndrome that required a G-tube for feeding, Michael showed remarkable resilience during his 40-day NICU stay.
He was on oxygen support for a few hours, but he soon was strong enough to breathe on his own. The doctors and nurses at CHRISTUS Children’s monitored his progress and adjusted treatments as needed.
Follow-up Care for Scimitar Syndrome
Although Michael does not need immediate surgery for Scimitar syndrome, he will continue to have regular cardiology check-ups with Dr. Gupta. This ongoing care is essential to monitor his heart condition, manage any potential complications, and ensure his overall well-being.
Monitoring conditions like Scimitar syndrome is crucial, especially given its rarity and complexity,
Dr. Gupta said. Regular echocardiograms allow us to track the heart's development in real-time, identify any complications early, and plan the necessary interventions. This proactive approach is essential to ensure the best outcomes and to tailor the care plan to the unique needs of each patient.
If patients continue to experience lung problems with Scimitar syndrome, such as frequent infections, or lung damage, then a procedure by cardiac catheterization may be needed. Only rarely a surgery to remove the affected lung segment is required, however, the child will require heart surgery when older.
Beating the Odds
At four months old, Michael continues to surprise everyone with how well he's doing. His bright smile and cheerful attitude demonstrate his resilience and hopefulness. His mom says he’s just like any other baby—bright, engaging, and surrounded by so much love. While the road ahead will include ongoing medical management and may involve eventual surgery, SanJuanita remains optimistic.
The compassionate care of the CHRISTUS staff has been instrumental in helping Michael thrive against all odds,
SanJuanita said. They love Michael. They've been so attentive and focused in his progress, ensuring he receives the best possible care. Their support gives me hope and strength for the journey.
Learn more about the NICU or the Heart Center at CHRISTUS Children's.
