What is the recommended management and treatment for a patient with congenital heart disease?

Management and Treatment of Congenital Heart Disease

All patients with congenital heart disease require risk stratification by anatomic complexity (simple, moderate, or great complexity) and physiological stage (A through D) to determine the appropriate care setting, follow-up frequency, and treatment intensity. 1

Risk Stratification Framework

Anatomic Complexity Classification

Simple CHD (Type I) includes lesions that typically require minimal intervention: 1, 2

• Isolated small atrial septal defect (ASD)
• Isolated small ventricular septal defect (VSD) without associated lesions
• Mild isolated pulmonic stenosis
• Small patent ductus arteriosus
• Previously repaired secundum or sinus venosus ASD without residua
• Repaired VSD without residua

Moderate Complexity CHD (Type II) encompasses a broader spectrum requiring ongoing surveillance: 3, 1, 2

• Atrioventricular septal defects (partial or complete)
• Coarctation of the aorta
• Ebstein's anomaly
• Tetralogy of Fallot (repaired)
• Ostium primum ASD
• Patent ductus arteriosus (not closed)
• Moderate-to-severe pulmonary valve regurgitation or stenosis
• Sinus of Valsalva fistula/aneurysm
• VSD with absent valve, aortic regurgitation, coarctation, mitral disease, or right ventricular outflow tract obstruction

Great Complexity CHD (Type III) represents the most challenging lesions: 1, 2

• All cyanotic congenital heart defects
• Fontan procedure patients
• Single ventricle physiology
• Transposition of the great arteries
• Truncus arteriosus
• Pulmonary atresia
• Double-outlet ventricle

Physiological Stage Classification

The physiological staging system guides treatment intensity: 1

• Stage A (asymptomatic, normal function): Observation with periodic surveillance
• Stage B (mild symptoms, mild hemodynamic sequelae): Close monitoring and medical management
• Stage C (moderate-severe symptoms, significant valvular disease): Active medical management and often surgical or catheter-based intervention
• Stage D (end-stage, severe complications): Advanced heart failure therapies and consideration for heart transplantation

Care Delivery Model

Specialized Center Requirements

Patients with moderate and great complexity CHD must receive care at regional Adult Congenital Heart Disease (ACHD) centers with integrated multidisciplinary services. 3, 1

Required capabilities at ACHD centers include: 3, 1

• Specialized echocardiography with CHD expertise
• CHD-specific diagnostic and interventional catheterization
• Electrophysiology services for arrhythmia management
• Advanced cardiac imaging (MRI, CT)
• Cardiac anesthesiology with CHD experience
• Surgical teams with CHD expertise

Follow-Up Frequency

Simple CHD patients require cardiac follow-up at a regional ACHD center at least once to formulate future needs, then can be managed in the general medical community. 3

Moderate complexity CHD patients require follow-up at a regional ACHD center every 12 to 24 months. 3

Great complexity CHD patients require follow-up at a regional ACHD center every 6 to 12 months at minimum. 3

Diagnostic Surveillance Strategy

Primary Imaging Modalities

Electrocardiography is recommended for serial assessment to detect: 1

• Sinus node dysfunction
• Intra-atrial re-entry tachycardia
• Atrioventricular block progression

Echocardiography serves as the primary imaging modality for initial and serial assessment of all CHD patients. 1

Cardiac MRI is specifically recommended for: 1

• Quantitative assessment of right ventricular size and function
• Serial assessment based on anatomic complexity and clinical status
• Evaluation of thoracic aortic aneurysms extending beyond echocardiographic windows

Procedural Requirements

All diagnostic and interventional procedures for moderate and complex CHD must be performed at regional ACHD centers with appropriate personnel and equipment working as part of a team with expertise in surgical and transcatheter management. 3

This includes: 3

• Advanced imaging (echocardiography, MRI, CT)
• Cardiac catheterization
• Electrophysiology procedures

Treatment Modalities

Interventional and Surgical Approaches

Treatment ranges from simple defect closure to complex reconstructive procedures depending on lesion type: 1

• Catheter-based closure for appropriate defects
• Valve repair or replacement
• Staged palliation for complex lesions
• Fontan completion for single ventricle physiology

All surgical procedures requiring general anesthesia or conscious sedation in moderate or complex CHD patients must be performed at regional ACHD centers with anesthesiologists familiar with ACHD patients. 3

Pharmacotherapy

Medical management addresses specific complications: 1

• Heart failure medications for ventricular dysfunction
• Antiarrhythmic therapy for rhythm disturbances
• Pulmonary vasodilators for pulmonary hypertension
• Anticoagulation for thromboembolic risk

Critical Management Caveats

Acute Care Transfer Protocol

Stabilized adult CHD patients requiring urgent or acute cardiac care should be transferred to a regional ACHD center, except in specific circumstances after consultation with the patient's primary ACHD specialist. 3

Adult patients with complex or high-risk CHD should be transferred to an ACHD center even for urgent or acute noncardiac problems. 3

Special Considerations

Minimize radiation exposure during imaging by choosing non-ionizing modalities (echocardiography, MRI) whenever appropriate, as CHD patients require lifelong serial imaging. 1

Assess renal function before prescribing medications, as patients with complex CHD may have end-organ dysfunction that affects drug metabolism and clearance. 1

Patients with cyanotic CHD require special consideration for any medication due to altered physiology, particularly avoiding agents that impair renal function. 1

Common Pitfalls to Avoid

The primary caregiver or cardiologist without specific ACHD training should not attempt to manage moderate or complex CHD independently—these patients achieve better outcomes when managed in integrated, collaborative, multidisciplinary ACHD programs rather than general cardiology settings. 3, 1

Failing to recognize that CHD patients may have multiple types of heart disease simultaneously (congenital plus acquired) can lead to inadequate management, as can overlooking acquired comorbidities in the adult CHD population. 2