What is the management approach for patients with aortic coarctation and ventricular septal defect (VSD) syndrome?

Management of Aortic Coarctation with Ventricular Septal Defect

Patients with aortic coarctation and ventricular septal defect should be managed with a staged approach, with initial repair of the coarctation followed by assessment of the VSD for potential closure or spontaneous resolution. 1, 2

Diagnostic Evaluation

Initial Assessment

• Comprehensive cardiovascular examination:
  • Simultaneous palpation of brachial and femoral pulses to detect "brachial-femoral delay" 1
  • Blood pressure measurements in both arms and lower extremities to detect gradient 1
  • Auscultation for systolic murmurs (VSD, coarctation)

Imaging Studies

• Echocardiography: First-line imaging modality 1

  • Assess coarctation severity and location
  • Determine VSD size, location, and hemodynamic significance
  • Evaluate for left ventricular volume overload (Qp:Qs ratio)
  • Assess pulmonary artery pressure
  • Rule out additional cardiac anomalies

• Cardiac MRI or CT: At least one comprehensive study is required 1

  • Complete evaluation of thoracic aorta anatomy
  • Assessment of arch hypoplasia
  • Evaluation of collateral vessels

• Cardiac Catheterization: Indicated in specific scenarios 1

  • To assess operability in patients with VSD and pulmonary arterial hypertension (PAH)
  • When noninvasive data are inconclusive
  • To measure peak-to-peak gradient across coarctation
  • To evaluate pulmonary vascular resistance

Management Algorithm

Step 1: Assess Severity and Hemodynamics

• Determine coarctation gradient (significant if peak-to-peak gradient ≥20 mmHg) 1
• Evaluate VSD size and hemodynamic impact:
  • Small VSD: Qp:Qs <1.5:1
  • Moderate VSD: Qp:Qs 1.5-2.0:1
  • Large VSD: Qp:Qs >2.0:1
• Assess pulmonary artery pressure and resistance

Step 2: Coarctation Repair

• Indications for coarctation repair: 1

  • Peak-to-peak gradient ≥20 mmHg
  • Significant coarctation with collateral flow even if gradient <20 mmHg
  • Hypertension attributable to coarctation

• Timing: Urgent repair in infants with heart failure; otherwise, elective repair once diagnosed 2, 3

• Surgical options:

  • End-to-end anastomosis
  • Subclavian flap aortoplasty
  • Patch augmentation
  • Percutaneous intervention (stenting) in older children/adults

Step 3: VSD Management Decision

• Small restrictive VSD (Qp:Qs <1.5:1):

  • Conservative management with monitoring for spontaneous closure 2, 1
  • Many VSDs (especially muscular) close spontaneously over time

• Moderate to large VSD (Qp:Qs ≥1.5:1):

  • Options include:
    1. Pulmonary artery banding at time of coarctation repair if infant is small/unstable 4, 5
    2. Delayed VSD closure after coarctation repair and patient stabilization 2
    3. Single-stage repair of both defects in selected cases 5, 3

• Indications for VSD closure: 1

  • Qp:Qs ≥2.0:1 with LV volume overload
  • Qp:Qs >1.5:1 with pulmonary artery pressure <2/3 systemic
  • History of infective endocarditis
  • VSD with aortic valve prolapse causing AR
  • Persistent heart failure symptoms

• Contraindications to VSD closure: 1

  • Severe irreversible PAH
  • Eisenmenger syndrome with right-to-left shunt
  • Exercise-induced desaturation

Approach Selection

Two-Stage Approach (Most Common)

• Stage 1: Coarctation repair ± pulmonary artery banding
• Stage 2: VSD closure if needed (many VSDs close spontaneously)
• Advantages: 4, 3
  • Lower operative complexity for initial repair
  • Avoids cardiopulmonary bypass in critically ill infants
  • Potential for spontaneous VSD closure (25-30% of cases) 2
• Best for: Neonates, small infants, hemodynamically unstable patients

Single-Stage Approach

• Options:
  1. One incision: Median sternotomy with CPB and circulatory arrest/regional perfusion
  2. Two incisions: Thoracotomy for coarctation repair followed by sternotomy for VSD closure
• Advantages: 5, 3
  • Complete repair in infancy
  • Avoids interim palliation period
  • Can address arch hypoplasia if present
• Best for: Older, stable infants; patients with arch hypoplasia

Follow-Up Management

• Lifelong cardiology follow-up is mandatory 1
• Yearly evaluation if residual defects are present 1
• Imaging of thoracic aorta every 3-5 years 1
• Monitor for:
  • Recoarctation (5-10% of cases)
  • Residual VSD
  • Development of aortic regurgitation
  • Hypertension
  • Ventricular dysfunction
  • Arrhythmias

Common Pitfalls and Complications

• Recoarctation: More common after neonatal repair
• Hypertension: May persist despite successful repair
• Residual VSD: May require later closure
• Aortic valve dysfunction: Monitor for development of AR
• Endocarditis risk: Prophylaxis indicated in high-risk patients
• Ventricular dysfunction: Can occur late after repair

The management of aortic coarctation with VSD requires expertise in congenital heart disease, and these patients should be managed at specialized centers with experience in adult congenital heart disease (ACHD) 1.