Cyanotic Heart Disease With Variable or Increased Vascularity
Robert Fleck, MD
DIFFERENTIAL DIAGNOSIS
Common
Transposition of Great Arteries (d-TGA)
Less Common
Total Anomalous Pulmonary Venous Return (TAPVR)
Atrioventricular Septal Defect (AVSD)
Truncus Arteriosus
Rare but Important
Single Ventricle
Tricuspid Atresia
Eisenmenger Physiology
Double Outlet Right Ventricle (DORV)
ESSENTIAL INFORMATION
Key Differential Diagnosis Issues
Normal chest radiograph does not exclude congenital heart disease (CHD)
Pulmonary vascularity is variable
Many lesions are known prenatally or evaluated soon after birth due to cyanosis
Helpful Clues for Common Diagnoses
Transposition of Great Arteries (d-TGA)
Most common CHD with early cyanosis
Aorta is anterior and right of main pulmonary artery (MPA)
Superimposed, parallel great vessels give rise to narrow mediastinum (“string”)
Right atrium (RA) and right ventricle (RV) enlargement has “egg-on-side” appearance
Thus, creates “egg-on-a-string”
Pulmonary vascularity increases as pulmonary resistance decreases
Increased pulmonary blood flow is “classic” pattern
Decreased pulmonary blood flow in pulmonic stenosis
Early treatment tries to provide mixing of right and left blood
Rashkind procedure is atrial septostomy with balloon catheter
Prostaglandin E1 to keep patent ductus arteriosus (PDA)
Later treatment is related to correcting blood flow
Jatene arterial switch procedure is current standard of care
Atrial switch (baffles) used in past include Mustard or Senning procedures
Helpful Clues for Less Common Diagnoses
Total Anomalous Pulmonary Venous Return (TAPVR)
Supracardiac type (50%); infrequently obstructed
Cardiac type (30%)
Infracardiac type (15%); most obstructed
Mixed type (5%)
Appearance depends on degree of obstruction to pulmonary venous return
Obstructed: Small or normal heart size with severe pulmonary edema
Unobstructed: Increased pulmonary vascularity and “snowman” silhouette or pretracheal density (newborn)
MR or CT to define anatomy
Improved pre- and perioperative care along with better surgical techniques has resulted in low mortality
Infracardiac and those with complex cardiac lesion have increased mortality
Postoperative course is complicated by obstruction of venous return
Atrioventricular Septal Defect (AVSD)
40% have Down syndrome
Radiographic appearance
Cardiomegaly: Moderate to marked
Prominent pulmonary artery segment
Increased pulmonary vascularity
Skeletal findings of Down syndrome
Frequently present in asplenia and polysplenia
Angiographically shows “goose neck” deformity of left ventricular outflow
Patch closure of ASD/VSD with reconstruction of left AV valve
Truncus Arteriosus
Failure of division of truncus to form aorta and PA
Always large VSD
Type 1: Single main PA originates from truncus
Type 2: Right and left PA arise separately from truncus
Type 3: Right and left PA arise from clearly separate origin on truncus
Type 4: “Pseudotruncus,” arteries supplying lungs arise from descending aorta
Radiographic appearance
Right aortic arch (35%)
Cardiomegaly: Often at birth
Pulmonary vascularity increases as pulmonary resistance decreases
Enlarged aorta (truncus)
Treatment
Usually early with small valved allographs for RV to PA reconstruction
Helpful Clues for Rare Diagnoses
Single Ventricle
Variable radiographic appearance
Pulmonic stenosis: Normal heart size, decreased vascularity
No pulmonic stenosis: Cardiomegaly, large MPA, ↑ pulmonary vascularity, congestive heart failure
Imaging is important in identifying single ventricle as left or right ventricle
Usually RV is underdeveloped
Treatment is staged repair
Ultimately leads to Fontan
Tricuspid Atresia
TGA is common (30%)
Radiographic appearance with TGA
Cardiomegaly
Increased pulmonary vascularity
Narrow pedicle if TGA
Radiographic appearance with normally related great vessels
Normal to small heart
Elevated apex
Concave PA segment and ↓ vascularity
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