How should a newborn with coarctation of the aorta and patent ductus arteriosus be initially managed and definitively treated?

Management of Newborn with Coarctation of the Aorta and Patent Ductus Arteriosus

Immediately initiate prostaglandin E1 infusion (0.05–0.1 mcg/kg/min) to maintain ductal patency and restore lower body perfusion, then proceed to urgent surgical repair once the infant is stabilized. 1, 2

Initial Resuscitation and Stabilization

The patent ductus arteriosus is life-sustaining in neonatal coarctation and must be kept open pharmacologically until definitive repair. 1, 3

• Neonates with coarctation present in clinical distress with significant metabolic acidosis and respiratory failure at the time of ductal closure. 1
• The ductus provides critical lower body perfusion by allowing blood to bypass the coarctation site; when it constricts, severe heart failure, poor systemic perfusion, and acidemia rapidly develop. 2, 4
• Prostaglandin E1 infusion effectively dilates the ductus arteriosus in 10 of 11 infants with coarctation, increasing descending aortic blood pressures and improving left ventricular failure. 2
• Do not discontinue prostaglandin infusion even if echocardiography shows predominantly left-to-right ductal flow—the ductus prevents constriction that would worsen the coarctation and precipitate acute left heart failure. 4

Diagnostic Evaluation Before Surgery

Transthoracic echocardiography is the first-line imaging modality and may be sufficient for surgical planning in simple neonatal coarctation. 1

• Echocardiography confirms the coarctation location (typically juxtaductal, just distal to the left subclavian artery), assesses arch hypoplasia, identifies associated intracardiac defects, and evaluates left ventricular function. 1
• Measure blood pressure in both arms and one lower extremity to document the inter-limb gradient. 1, 5, 6
• The pathognomonic finding is brachial-femoral pulse delay with decreased amplitude or absent femoral pulses. 1, 6
• If echocardiography does not fully visualize the aortic arch, coarctation segment, or branching vessels, obtain cardiac MRI or CT before surgery—but do not delay urgent repair in a critically ill neonate. 1
• Screen for bicuspid aortic valve, which occurs in 50–85% of coarctation patients. 1, 6

Definitive Surgical Treatment

Surgical repair with extended resection and end-to-end anastomosis is the gold standard for neonatal coarctation and should be performed urgently once the infant is stabilized on prostaglandin. 1

• Surgical methods include subclavian flap repair, patch aortoplasty, and simple or extended end-to-end anastomosis. 1
• Extended end-to-end anastomosis is preferred because it removes ductal tissue and addresses associated arch hypoplasia, which commonly accompanies neonatal coarctation. 1
• The patent ductus arteriosus is ligated at the time of coarctation repair. 1, 7
• Surgery should be performed by surgeons with training and expertise in congenital heart disease. 1

When Balloon Angioplasty May Be Considered

Balloon angioplasty without stenting may be used as a palliative bridge in critically ill neonates with severely depressed ventricular function or extenuating circumstances, but surgical repair remains definitive. 1

• Balloon angioplasty for native coarctation in infants <6 months carries higher recurrence rates and a small but important risk of aneurysm formation. 1
• Young patients (1–6 months) with discrete narrowing and no arch hypoplasia may benefit from balloon angioplasty, but this applies to relatively few neonates. 1
• Stent implantation is not appropriate in neonates because available stents cannot be expanded to adult size (minimum 2 cm diameter). 1

Critical Pitfalls to Avoid

Never discontinue prostaglandin E1 before definitive repair, even if the ductus appears to have left-to-right flow on echocardiography—ductal constriction will precipitate acute decompensation. 2, 4

• The central role of the patent ductus is to prevent ductal constriction, which markedly worsens the coarctation and causes acute left heart failure. 4
• Do not delay surgical consultation while obtaining advanced imaging in a critically ill neonate—echocardiography provides sufficient anatomic detail for urgent repair. 1
• Recognize that arch hypoplasia commonly accompanies neonatal coarctation and requires extended surgical repair rather than simple resection. 1

Postoperative Management and Long-Term Surveillance

All coarctation patients require lifelong cardiology follow-up with at least yearly evaluations, even after successful repair. 1, 5, 6

• Monitor closely for residual or recurrent coarctation, aneurysm formation at the repair site, and systemic hypertension. 1, 5
• Perform cardiac MRI or CT every 3–5 years (or more frequently if anatomic concerns arise) to detect restenosis, aneurysm formation, or dissection. 1, 5, 6
• Treat hypertension aggressively with beta-blockers, ACE inhibitors, or angiotensin-receptor blockers after excluding recoarctation. 1, 5, 6
• Surveillance for bicuspid aortic valve dysfunction and ascending aortic dilation is required throughout life. 1, 5, 6
• Screen for intracranial aneurysms (present in 10% of coarctation patients) by MRA or CTA, particularly if family history or neurologic symptoms develop. 1, 6

Balloon angioplasty is the treatment of choice for recurrent coarctation after surgical repair, with intervention indicated when the transcatheter systolic gradient is >20 mmHg. 1