Aggressive treatment of hydrops fetalis results in positive outcome

James Strainic, MD

Director, Fetal Heart Program, UH Rainbow Babies & Children’s Hospital , Assistant Professor of Pediatrics, Case Western Reserve University School of Medicine

The diagnosis and management of hydrops fetalis have improved in recent years, thanks to advances in prenatal diagnostic and therapeutic interventions and advances in neonatal intensive care. However, fetal hydrops still carries high risk. When associated with a heart arrhythmia, the mortality rate for the condition is more than 50 percent. 

Doctors at University Hospitals Rainbow Babies & Children’s Hospital and the Congenital Heart Collaborative recently faced this challenging reality, diagnosing hydrops in an 18-week-old fetus. The abnormality was first detected during the young mother's routine second-trimester scan, which showed signs of tachycardia with a fetal heart rate between 200 and 300 beats per minute. The ultrasonographer also identified signs of fluid accumulation in the scans.

Pediatric cardiologist James Strainic, MD, Director of the Fetal Heart Program at UH Rainbow Babies & Children's Hospital, attributed these signs to atrial tachycardia, which can be difficult to treat. He enlisted an interdisciplinary team of obstetricians, fetal ultrasonographers, electrophysiologists, pediatric and adult cardiologists, and maternal fetal medicine specialists to collaborate with the mother to develop an effective treatment plan. 

M-mode imaging of the heart showing tachycardia with a ventricular heart rate of 220 beats per minute.

This case was unusual, Dr. Strainic says, both because fetal arrhythmias often present later in the pregnancy, in the third trimester. “Plus, it usually takes a while for the hydrops to show itself,” he says. 

The clinicians admitted the mother to the hospital and, because of the presence of hydrops at this gestation, treated her with digoxin and sotalol to slow the heart rate. “This combination is often effective in resolving tachycardia, but it wasn't in this case,” Dr. Strainic says.

Dr. Strainic's team began going through their protocols for fetal heart arrhythmias, discontinuing digoxin and starting the mother on the anti-arrhythmic flecainide. However, after three days, flecainide wasn't working, either. The team discontinued all the mother’s medications for a few days to let them clear her system and then considered a more aggressive approach – one that involved more clinicians. 

“We enlisted the help of multiple services at University Hospitals to try to manage it,” says Dr. Strainic.

The team’s proposed solution was amiodarone, a class III drug with serious side effects that is typically used only in patients with life-threatening situations, and the mother consented. The team enlisted adult cardiologists to monitor the mother. Maternal fetal medicine specialists kept an eye on fetal growth, and Dr. Strainic managed the medications for the fetus and the fetal tachycardia.

After 24 hours, the tachycardia had subsided, and the heart was beating normally. Within 48 hours, the fluid around the heart, chest and belly was gone. The mother continued with the medication throughout her pregnancy, and a few months later, the infant born was born at term. He didn't need a breathing tube and he remained with his mother for a few hours after birth before going to the pediatric cardiovascular intensive care unit for a day of evaluation.

The infant, now six months old, shows no signs of arrhythmia. Because the hydrops occurred so early in the pregnancy – and because amiodarone can interfere with developmentally important iodine, neurologists will continue to monitor the child.

Dr. Strainic credits the positive outcome to the cooperation of the interdisciplinary team. By identifying the problem as early as possible, and by monitoring mother and fetus throughout the process, the team was able to bring about a great outcome for both mother and child. 

They also gained valuable experience with a little-used medication. “Our treatment of hydrops was atypical because we turned to amiodarone so early in the pregnancy,” Dr. Strainic says. “It's pretty rare to need to use this medication. We don't like to use it because of its side effects, but when you have to use it, you do.” 

For more details about this case, email James.Strainic@UHhospitals.org