What do you see on a chest X-ray (Chest Radiograph) in someone with Congenital Heart Disease (CHD)?

Chest X-ray Findings in Congenital Heart Disease (CHD)

Chest X-ray findings in congenital heart disease vary based on the specific defect but typically include cardiomegaly, abnormal pulmonary vascularity patterns, and specific cardiac chamber enlargement that reflects the underlying hemodynamic abnormalities. 1

General Findings on Chest X-ray in CHD

• Chest X-ray quickly illustrates gross cardiac and mediastinal contours, pulmonary vascularity, pathologic calcification, and presence of indwelling metallic devices 1
• Assessment of cardiac size, cardiac configuration, and position of the aortic arch can be determined 1
• Situs of the abdomen and thorax can usually be determined, which is important in complex CHD 1
• Thoracic cage anomalies associated with CHD and postoperative changes may be detected 1

Specific Findings Based on Hemodynamic Effects

Left-to-Right Shunts (Increased Pulmonary Blood Flow)

• Cardiomegaly due to dilation of cardiac chambers 1, 2
• Increased pulmonary vascular markings (pulmonary plethora) 1, 2
• Pulmonary venous congestion may be present 1
• Examples include:
  • Atrial Septal Defect (ASD): Right atrial and ventricular enlargement, prominent pulmonary artery segment, increased pulmonary vascularity 1
  • Ventricular Septal Defect (VSD): Cardiomegaly with increased pulmonary vascular markings 2
  • Patent Ductus Arteriosus (PDA): Cardiomegaly, increased pulmonary vascularity 1

Pulmonary Hypertension

• In patients with long-standing left-to-right shunts who develop pulmonary arterial hypertension (PAH): 1
  • Prominent main pulmonary artery segment
  • Pruning (decreased caliber) of distal pulmonary vessels
  • In severe PAH with Eisenmenger syndrome, peripheral lung fields may appear oligemic 1

Obstructive Lesions

• Pulmonary Stenosis: Normal heart size unless severe, vascular fullness in left lung base greater than right (Chen's sign), post-stenotic dilation of main pulmonary artery 1
• Coarctation of Aorta: Rib notching (in older children/adults), "figure 3 sign" from post-stenotic dilation of the descending aorta 1

Complex Lesions

• Tetralogy of Fallot: Boot-shaped heart (coeur en sabot) due to RV hypertrophy with uplifted apex and concave pulmonary artery segment, decreased pulmonary vascularity 1
• Atrioventricular Septal Defect (AVSD): Cardiomegaly may be present due to dilation of right or left AV heart chambers, increased pulmonary vascular markings with significant left-to-right shunt 1

Findings in Pulmonary Venous Congestion

• Interstitial edema with Kerley B lines 1
• Pleural effusions may be present 1
• Pulmonary venous congestion may be seen with long-standing mitral regurgitation 1

Limitations of Chest X-ray in CHD

• Chest X-ray alone is insufficient for definitive diagnosis of CHD 1
• Cardiomegaly can be absent not only in acute but also in chronic heart failure 1
• Normal chest X-ray does not exclude significant CHD 1
• Additional imaging modalities such as echocardiography, cardiac MRI, or CT are typically required for comprehensive evaluation 1

Clinical Correlation

• Findings must be interpreted in context of clinical presentation and other diagnostic tests 1
• In adults with repaired CHD, chest X-ray may show postoperative changes and residual abnormalities 1
• Chest X-ray remains useful for initial assessment and follow-up of patients with surgically treated CHD 1

Common Pitfalls

• Over-reliance on chest X-ray alone for diagnosis of CHD 1
• Failure to recognize subtle findings that may indicate significant CHD 1
• Misinterpreting normal variants or non-cardiac abnormalities as CHD 1
• Not correlating radiographic findings with clinical presentation and other diagnostic tests 1