Stephens EH, Eltayeb O, Kennedy C, Rigsby CK, Rastatter JC, Carr MR, Mongé MC, Backer CL . Ann Thorac Surg. 2021 Jan 29:S0003-4975(21)00124-7. doi: 10.1016/j.athoracsur.2021.01.025. PMID: 33524348
Take Home Points:
- There has been an increasing frequency of fetal diagnosis of vascular rings. Increasing frequency of fetal diagnosis was noted in the past 10 years.
- Patients with a fetal diagnosis were significantly younger at the time of surgery and there were no differences in postoperative complications or length-of-stay for fetal diagnosis vs. postnatal diagnosis.
- Fetal diagnosis leads to the potential for expectant management of vascular ring patients and may lead to improved long-term outcomes.
Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch:
Background: With increasing and improving fetal imaging, vascular rings are more frequently being diagnosed prior to birth. However, how best to manage these patients remains undetermined. Previously patients with vascular rings were diagnosed postnatally and underwent repair only after symptoms occurred.
Patients and Methods: A retrospective review was performed of all pediatric patients undergoing primary vascular ring repair from 1/1/2000 to 6/1/2019. The study cohort consisted of 190 patients operated on for a vascular ring between January 2000 and June 2019, with 15% (n=29) having a fetal diagnosis. The most common anatomic variant of vascular ring was right aortic arch ± aberrant left subclavian artery.
Results: Patients with a fetal diagnosis were significantly younger at time of surgery (13.1 months [20.6] vs. 24.0 months [87.0], p=0.029). The length-of-stay for the total cohort who did not undergo cardiopulmonary bypass was 3 days  and was not significantly different between fetal and postnatal diagnosis (3 days  for fetal diagnosis vs. 4 days  for postnatal diagnosis, p=0.50). There was no operative mortality.
Author’s recommendations for the management of a patient with a fetal diagnosis of vascular ring:
- Getting an echocardiogram at birth in all patients to evaluate for intracardiac pathology and confirm the fetal diagnosis.
- If the patient is asymptomatic, we recommend a CT scan at 2-4 months of age when general anesthesia is not required. Post-scan 3D reconstruction is then performed for an extremely precise depiction of the anatomic features of the vascular ring.
- Significant external tracheal compression is an indication for operative intervention even in an asymptomatic patient.
- If the patient has a double aortic arch we recommend operative intervention at 6-9 months of age even if they have minimal or no symptoms.
- In the postnatal diagnosis group symptoms began to appear at 6 months of age. For patients with a right aortic arch, if Kommerell diverticulum resection and left subclavian artery transfer are indicated, operation is recommended at 12-18 months of age. Left subclavian artery transfer is eminently feasible at this age with an anastomosis of a vessel 3.0-3.5 mm in diameter. The timely removal of the Kommerell diverticulum will prevent known late complications of aneurysm formation, and aortic dissection.
- For vascular ring patients who become symptomatic prior to 2-4 months of age we perform CT imaging followed by surgical repair at the time of symptom onset.
- In the special case of pulmonary artery sling we perform CT imaging prior to hospital discharge to rule out complete tracheal rings and tracheal stenosis. Median age at pulmonary artery sling repair was 3 months of age.
- Fetal diagnosis of vascular ring allows the opportunity for optimizing the timing of diagnostic tests and surgical intervention.
- Symptomatic infants should have a CT scan for diagnosis followed by surgical repair.
- Asymptomatic infants should have CT scan at 2 – 4 months of age to plan for appropriate elective surgical repair.
- In our experience we have found that earlier intervention, as detailed above, results in excellent outcomes while also potentially minimizing complications related to longstanding airway and esophageal compression.