Complex Case: Cardiac Anomaly including Dextrocardia

The baby was at 30 weeks gestation when echocardiographic examination of the fetus revealed that the fetal heart was formed abnormally.

The cardiac anomaly consisted of the following defects 1) dextrocardia — ​the heart was located in the right chest, with the apex pointing rightward, 2) L-malposition of the aorta, 3) pulmonary atresia, 4) L-looped ventricles, 5) large conoventricular septal defect, and 6) aorta arising leftward and anteriorly, overriding the ventricular septum and committed more toward the leftward morphologic right ventricle.

At NYU Langone’s Hassenfeld Children’s Hospital, Achiau Ludomirsky, MD, the Andrall E. Pearson Professor of Pediatric Cardiology and director of the Division of Pediatric Cardiology, and Ralph S. Mosca, MD, the George E. Reed Professor of Cardiac Surgery and chief of the Division of Pediatric and Adult Congenital Cardiac Surgery, became the primary clinicians leading a team of pediatric cardiac surgeons and pediatric cardiologists in monitoring and planning for the case. In collaboration with NYU Langone’s maternal-fetal medicine specialists, they monitored the mother’s pregnancy from 30 weeks’ gestation through delivery and together developed a treatment plan that would begin at the patient’s birth and evolve over time.

The baby was born via a normal vaginal delivery. The first step in the care would be to secure blood supply to the lungs.

When the baby was three days old, Dr. Mosca performed a modified Blalock–Taussig shunt, a palliative procedure to redirect blood from the systemic circulation to the lungs. The baby recovered in the Congenital Cardiovascular Care Unit and was discharged home after five days.

Over the next four months, the pediatric cardiology team followed the baby closely. After four months, the baby’s cardiac condition was reevaluated in order to establish the next step in her care. The dilemma was whether to pursue a one- or a two-ventricle repair. After reviewing data from new diagnostic studies, the team agreed that a bidirectional Glenn procedure that would not preclude a two-ventricle repair in the future would be the most appropriate treatment at this stage.

Dr. Mosca performed a bidirectional Glenn procedure, connecting the superior vena cava to the right pulmonary artery and a patch, enlarging the branch pulmonary arteries. The child had an uneventful postoperative course, was discharged from the hospital within a week, and was then followed by the team for the next two years.

When the child was three years old, imaging studies indicated that the left pulmonary artery was narrowed. At that time, NYU Langone congenital and structural interventional cardiologist, Doff B. McElhinney, MD, professor of pediatrics, medicine and cardiothoracic surgery, and director of interventional cardiology for congenital and structural heart disease, implanted a stent in the child’s left pulmonary artery, using a transcatheter technique in order to overcome the obstruction, with excellent results.

Frank Cecchin, MD, professor of pediatrics and medicine, and a pediatric electrophysiologist, also addressed the child’s electrophysiological condition, Wolff–Parkinson–White syndrome, a po­tential cause of atrial arrhythmias. Dr. Cecchin recommended that cardiac ablation be done during the next surgery.

When the child was three and a half years old, the team agreed that the child’s heart was ready for the most desirable repair solution — ​a two-ventricle repair.

The surgery was challenging because of the complexity of the lesion and the location of the heart in the right chest. During the repair, Dr. Mosca patched the ventricular septal defect toward the aorta, established a communication between the morphologic right ventricle and the pulmonary artery via a valved conduit, and performed a hemi-Mustard operation (an intra-atrial switch) and a cardiac ablation.

As a result of the close collaboration and expertise of this dedicated multidisciplinary team and the ability to perform complex procedures and provide specialized preoperative and postoperative intensive care in the hospital’s Congenital Cardiovascular Care Unit, the child has not only survived, but is thriving. The team has been able to give her an excellent quality of life and an optimistic long-term outlook.