1) The document describes various types of congenital heart diseases including acyanotic and cyanotic defects with left-to-right or right-to-left shunts. 2) Tetralogy of Fallot and transposition of the great arteries are discussed in detail as common cyanotic congenital heart diseases with decreased or increased pulmonary blood flow respectively. 3) Other cyanotic defects described include pulmonary atresia, Ebstein's anomaly, tricuspid atresia, and truncus arteriosus.

1. CARDIOPATIAS CONGENITAS
2018
Dr. Edgar A. Napa Felix
Medico Radiólogo

2. Cava
superior
Cava
inferior
Vena pulmonar superior
izquierda
Vena pulmonar inferior
izquierda
VISTA
ANTERIOR
Corazón y
grandes vasos
www.mirevistamedica.net

3. ACIANOTICAS CIANOTICAS
SHUNT I-D
• CIV (20%)
• CIA (10%)
• PCA (10%)
• Defecto septal atrioventricular (2-5%)
• Ventana Aortopulmonar (raro)
LESIONES OBSTRUCTIVAS DEL LADO IZQUIERDO
• Coartación de aorta (10%)
• Estenosis de aorta congénita (10%)
• Interrupción de arco aórtico (1%)
• Estenosis mitral (raro)
SHUNT D-I
• Tetralogía de Fallot (10%)
• Estenosis pulmonar (10%)
• Atresia pulmonar con CIV (5%)
• Atresia pulmonar sin CIV
• Atresia tricúspide (3%)
• Anomalía de Ebstein (raro)
DEFECTOS DE MEZCLA COMPLEJOS
• TGA (5-8%)
• DVPAT (2%)
• Truncus arterioso (3%)
• SDCH (2%)
Mavroudis, Backer. PEDIATRIC CARDIAC SURGERY. 4th Edition. WILEY-BLACKWELL
Clasificación de las cardiopatías congénitas

4. PERSISTENCIA DEL CONDUCTO
ARTERIOSO
CONDUCTO ARTERIOSO PERMEABLE

5. www.mirevistamedica.net
AORTA
Tronco
pulmonar

7. CARDIOPATIAS CONGENITAS CIANOTICAS CON FLUJO
PULMONAR DISMINUIDO
TETRALOGIA DE FALLOT
4 componentes descritos por primera vez en 1888
 CIV
 Estenosis pulmonar (infundibular)
 Cabalgamiento aórtico
 Hipertrofia VD (pacientes con largo tiempo de evolución)
10-11% de Todos los casos de C.C
Hallazgos en RX de Tórax
Corazón en forma de ´´bota´´
RadioGraphics 2007
Figure 5. (c) Drawing depicts the pattern of blood flow
(arrows) with the characteristic ventricular septal defect (1),
infundibular pulmonary stenosis (2), overriding aorta (3), and
right ventricular hypertrophy (4). The oxygen-rich blood in the
left side of the heart (5) mixes with oxygen-poor blood in the
right side of the heart (6) before it proceeds to the aorta (7).

8. Figure 5. Tetralogy of Fallot. (a, b) Chest radiograph obtained in an infant with a right-sided aortic arch (b the same as a with a superimposed drawing) shows
the characteristic boot-shaped sign produced by upturning of the cardiac apex because of right ventricular hypertrophy and by the concavity of the main
pulmonary artery.
RadioGraphics 2007

9. CARDIOPATIAS CONGENITAS CIANOTICAS CON FLUJO PULMONAR DISMINUIDO
ATRESIA PULMONAR CON CIV
Evaluación en pacientes con Concordancia A-V y V-Arterial
 Comportamiento tipo FALLOT EXTREMO
Ausencia de Flujo Pulmonar
Evaluación morfológica de la arteria Pulmonar: Planeamiento Qx
 Puede estar presente o Ausente
Forma de Flujo Pulmonar
 Multifocal
 PDA
 Vasos colaterales Aorto-Pulmonares
 Afectación de vascular y parenquimal pulmonar.
RadioGraphics 2007

10. Figure 19. Pulmonary atresia with VSD. LPA left pulmonary artery, RPA right pulmonary artery. (a) CT scan shows the confluent portion of the
central pulmonary artery (arrowheads). (b) CT scan shows absence of the confluent portion of the central pulmonary artery (*). Each pulmonary
artery was supplied by a corresponding PDA (not shown).
RadioGraphics 2007

11. CARDIOPATIAS CONGENITAS CIANOTICAS CON FLUJO
PULMONAR DISMINUIDO (CON CARDIOMEGALIA)
ANOMALIA DE EBSTEIN
Descrita por primera vez en 1866
 0.5-0.7 % de todos los casos de cardiopatía
congénita
Fuerte Asociación entre la ingesta de LITIO y la
presentación de A. Ebstein
Característica Importante
 Aorta y T. Pulmonar: Ambos disminuidos de calibre
 Desplazamiento caudal de las hojas de la V.
Tricuspide.
 Formación de cámara V-A común derecha:
REGURGITACIÓN TRICUSPIDEA
 Insuficiencia Tricuspidea: Dilatación del Tracto de
Salida V.D
 Incremento de la cámara auricular derecha
 Cianosis: SHUNT D-I a través de CIA
RadioGraphics 2007
(d) Drawing shows the pattern of blood flow (arrows) caused by downward
displacement of the tricuspid valve (1), with resultant formation of a common
chamber (3) consisting of the right ventricle (2) and the dilated right atrium
(4), and by the right to- left shunt of blood through a defect at the atrial level
(5). 6 left atrium, 7 left ventricle, 8 aorta, 9 pulmonary artery.

12. Figure 9. Ebstein anomaly. (a, b) Frontal (a) and lateral (b) views obtained with chest radiography in an infant show massive cardiomegaly with
decreased pulmonary flow. (c) Frontal view (same as a with a superimposed drawing) best depicts the box-shaped heart, an appearance caused
by enlargement of the right atrium and hypoplasia of the pulmonary trunk.
RadioGraphics 2007

13. Figure 9. Ebstein anomaly. (a, b) Frontal (a) and lateral (b) views obtained with chest radiography in an infant show massive cardiomegaly with
decreased pulmonary flow. (c) Frontal view (same as a with a superimposed drawing) best depicts the box-shaped heart, an appearance caused
by enlargement of the right atrium and hypoplasia of the pulmonary trunk.
RadioGraphics 2007

14. CARDIOPATIAS CONGENITAS CIANOTICAS CON FLUJO PULMONAR
AUMENTADO
TRANSPOSICIÓN DE GRANDES VASOS
Cardiopatía congénita cianótica mas frecuente en
NEONATOS
5-7% de las Cardiopatías congénitas
Mas común en hijos de madres con D.M
90% se presenta de forma aislada
CARACTERISTICAS
 DISCORDANCIA VENTRICULO-ARTERIAL
 Borde Auricular Derecho
 Aurícula Izquierda Agrandada
PRESENCIA COMUNICACIÓN
 Foramen Oval
 CIA
 CIV
FLUJO PULMONAR: NORMAL AL NACIMIENTO (D.A)
RadioGraphics 2007
(d) Drawing shows the pattern of blood flow (arrows) through the heart with
transposition of the great arteries. The aorta (1) arises from the right ventricle (2), and
the pulmonary artery (3) arises from the left ventricle (4). Communication between the
systemic and the pulmonary circulation—an interatrial septal defect (5), an
interventricular septal defect (6), or both—sustains life by allowing oxygenated blood
from the left atrium (7) to mix with deoxygenated blood from the right atrium (8) before it
flows via the right ventricle to the aorta and via the left ventricle to the pulmonary artery.

15. Figure 1. Transposition of the great arteries (a) Chest radiograph obtained in a neonate shows narrowing of the superior mediastinum, enlargement of the cardiac
silhouette with abnormal convexity of the right atrial border, and increased vascular flow—typical features of transposition of the great arteries. (b) Same image as a with
a superimposed drawing shows the characteristic cardiomediastinal silhouette: the egg-on-a-string sign.
RadioGraphics 2007

17. CARDIOPATIAS CONGENITAS CIANOTICAS CON
FLUJO PULMONAR AUMENTADO
ATRESIA TRICUSPIDEA
Aurícula Derecha no tiene comunicación con
V.D.
Dos Tipos
 Tipo I
Ausencia de Comunicación Atrio-
ventricular.
 Tipo II
Válvula Tricúspide Atrésica esta
presente.
Figure 21. Tricuspid atresia. CT scan shows fatty tissue (arrowheads)
between the right atrium (RA) and the right ventricle (RV), which prevents any
direct connection between the two compartments. This case represents the
more common type of tricuspid atresia.

18. CARDIOPATIAS CONGENITAS CIANOTICAS CON FLUJO PULMONAR AUMENTADO
TRONCO ARTERIOSO
• Poco frecuente pero con alta tasa de letalidad
• Periodo Neonatal e Infancia temprana
• Origen común de arteria Aorta y Pulmonar
• Tipos
• Tipo I (Mas común : 48%)
• Tipo II
• Tipo III
• Tipo IV
FIGURE 45-1 Collett and Edwards classification:
type I, a pulmonary trunk connects the common
arterial trunk to the branch pulmonary arteries; type
II, branch pulmonary arteries arise closely from the
common arterial trunk; type III, branch pulmonary
arteries arise far apart from the common arterial
trunk; type IV, branch pulmonary arteries arise from
the descending aorta. LPA, left pulmonary artery;
RPA, right pulmonary artery.
Cardiovascular Imaging - Vincent B. Ho,
MD, MBA, FAHA

19. FIGURE 45-3
ECG gated CT scan of a Van Praagh and Van Praagh type 2
truncus arteriosus.
Volume rendered image shows the left pulmonary artery
(LPA) and the right pulmonary artery (RPA) arising
separately from the common arterial trunk (TA).
The LPA is typically positioned above the RPA. The topmost
branch is the left carotid artery (LCA).
Cardiovascular Imaging - Vincent B. Ho, MD, MBA, FAHA

20. FIGURE 45-4
ECG gated CT scan of a Van Praagh and Van Praagh type 3
truncus arteriosus.
Volume rendered image shows the right pulmonary artery (RPA)
arising from the common arterial trunk (TA).
The left pulmonary artery is connected by a major
aorticopulmonary collateral artery (MAPCA) to the descending
aorta (DA).
Cardiovascular Imaging - Vincent B. Ho, MD, MBA, FAHA