Cyanotic Heart Disease With Decreased Vascularity
Robert Fleck, MD
DIFFERENTIAL DIAGNOSIS
Common
Tetralogy of Fallot (TOF)
Pulmonary Atresia (PA)
Tricuspid Atresia
Less Common
Tricuspid Stenosis
Ebstein Anomaly
Transposition Complexes
Rare but Important
Truncus Arteriosus
Severe Pulmonary Artery Stenosis with R to L Shunt
Total Anomalous Pulmonary Venous Return (TAPVR)
Single Ventricle
DORV with Pulmonic Stenosis
Uhl Disease (Parchment RV)
Isolated RV Hypoplasia
ESSENTIAL INFORMATION
Helpful Clues for Common Diagnoses
Tetralogy of Fallot (TOF)
Most common cyanotic CHD in childhood
Tetrad includes
RV outflow obstruction
RV hypertrophy
VSD
Aorta overriding VSD/interventricular septum
Variants
Trilogy: PA stenosis with RV hypertrophy
Pentalogy: Tetrad plus ASD
Pink tetralogy: Tetralogy with mild pulmonic stenosis
Radiographic appearance
Boot-shaped heart with upturned apex
Concave PA segment
Right arch (25%)
Treatment
Reconstruction of RV outflow tract by muscle resection and enlargement
Closure of VSD
Pulmonary Atresia (PA)
Radiographically similar to TOF
Reticular pulmonary vascularity
Tricuspid Atresia
No direct communication between RA and RV
ASD or patent foramen ovale are always present
Transposition of great arteries in 30% has different appearance
VSD is common
Radiographic appearance
Convex left cardiac border, upturned apex
Concave PA segment
Straight right heart border
↑ vascularity if TGA in (30%)
Helpful Clues for Less Common Diagnoses
Tricuspid Stenosis
Isolated tricuspid stenosis is usually congenital, but carcinoid can cause stenosis
Rheumatic heart disease
Ebstein Anomaly
Displacement of tricuspid leaflets into RV inflow
Effectively “atrializes” portion of RV
Mostly regurgitant lesion but can be obstructive
Associated with right bundle branch block and arrhythmias
Radiographic appearance
Convex right heart border
Cardiomegaly (box-shaped heart)
Treatment includes valve reconstruction and pacemaker placement
Transposition Complexes
Most common CHD with early cyanosis
Narrow superior mediastinum
If PS or PA, then decreased vascularity
Aorta is at right of MPA
Anterior and to right in d-TGA
More to right and less anterior in DORV
Superimposed, parallel great vessels give rise to narrow mediastinum “string”
RA and RV enlargement has “egg-on-side” appearance
Thus “egg-on-a-string”
Pulmonary vascularity increases as pulmonary resistance decreases
Increased pulmonary blood flow is “classic” pattern
Decreased pulmonary blood flow is normal early in life
Decreased pulmonary blood flow when pulmonic stenosis is present
Treatment
Early treatment is aimed at providing mixing of blood and involves atrial balloon septostomy
Jatene arterial switch procedure is current standard of care
Helpful Clues for Rare Diagnoses
Truncus Arteriosus
Always large VSD present
Type 1: Single main PA originates from truncus as common artery
Type 2: Right and left PA arise separately from truncus
Type 3: Right and left PA arise from clearly separate origins on truncus
Type 4: “Pseudotruncus,” arteries supplying lungs arise from descending aorta
Radiographic appearance
Right aortic arch in 35%
Cardiomegaly often at birth
Pulmonary vascularity can be increased, normal, or decreased
Enlarged aorta (truncus)
Severe Pulmonary Artery Stenosis with R to L Shunt
Radiographically similar to TOF
Total Anomalous Pulmonary Venous Return (TAPVR)
Typically described as variable or increased vascularity but can be initially decreased due to elevated pulmonary resistance in newborn period
Single Ventricle
Variable radiographic appearance
Pulmonic stenosis: Normal heart size, decreased vascularity
No pulmonic stenosis: Cardiomegaly, large MPA, ↑ pulmonary vascularity, and congestive heart failure
Usually, RV is underdeveloped
Treatment is staged repair
Ultimately leads to Fontan
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
