Peds Cardiac

Front 1

Approach to peds radiograph looking for heart disease

Back 1

-Pulmonary vascularity
-Cardiac size
-Situs
-Position of aortic arch

Front 2

Vascularity

Back 2

-Normal, increased (PA flow), decreased, centralized, or failure (increased PV flow) (cephalization)
-Increased PA flow: pulmonary arteries are larger than the adjacent bronchi. If the descending interlobar pulmonary artery is larger than the trachea. Distinct with well defined borders.
-Increased venous flow: very indistinct and poorly defined.
-In many things such as L to R shunts, there is both increased PAs (due to shunt) and increased PVs (due to heart failure).
-Decreased vascularity: paucity of arterial structures throughout the lung

Front 3

Cardiac size

Back 3

-specific chamber emlargement is not possible to determine in infants
-lateral view more helpful in infants than frontal view if there is cardiomegaly. On lateral, if posterior aspect of the cardiac silhouette extends over the vertebral bodies, cardiomegaly is present.
-Cardiac axis: if the apex points superiorly or inferiorly.
-If it points superiorly, right sided cardiac enlargement is suggested, and if it points inferiorly, left sided cardiac enlargement is suggested.

Front 4

Situs

Back 4

-The relationship of asymmetry!
-BEST = bronchial branching pattern!!!
-Next best: atrial situs (RA = receives the IVC), ventricular situs (LV = fibrous continuity of the aortic and mitral valves)
-situs solitus (normal): 0.6% incidence of CHD
-situs inversus: 3-5% incidence of CHD
-situs ambiguous: asplenia/polysplenia. Heterotaxy (left atrial isomerism or right atrial isomerism)
-Asplenia is associated with complex, cyanotic CHD. Prone to infxn with encapsulated bacteria due to abscense of a spleen. Also: malrotation, microgastria, midline gallbladder, midline liver, bilateral right sided bronchi, decreased PA flow, azygous continuation of the IVC, etc.
-Polysplenia: less complex acyanotic CHD, usually L to R shunts. Also: azygous continuation of IVC, bilateral SVC, malrotation, and absent gallbladder. Abscense of IVC shadow, prominent azygous vein, midline liver, and increased PA flow
-much greater chance of CHD if the stomach bubble and cardiac apex are on opposite sides.

Front 5

Aortic arch position

Back 5

-Right sided aortic arch: a red flag for CHD.
-in infants, the aortic knob may not be seen. Secondary findings include the position of the descending aorta, tracheal displacement, and tracheal indentation.

Front 6

Blue, decreased flow, normal to mildly increased C/T ratio (R to L shunts)

Back 6

-TOF: Most common type of cyanotic CHD. Usually diagnosed by 3 mos of age. 1. RV outflow tract obstruction 2. VSD 3. Overriding aorta 4. RV hypertrophy. Uplifting of cardiac apex, main PA segment is concave, boot-shaped heart.
-Pulmonary atresia with a VSD (most severe form of TOF): large bronchial arteries supply the lungs.
-Tricuspid atresia with PS and VSD

Front 7

Blue, decreased flow, massively increased C/T radio (also R to L shunts)

Back 7

-Ebstein anomaly: redundancy of the tricuspid valve, which is displaced into the RV causing atrialization of part of the RV. Massive dilatation of the RA and the atrialized portion of the RV.
-Pulmonary atresia with an intact ventricular septum: NO forward flow from the right heart. Massive dilatation of the RA and to a lesser extent the RV. Radiographic appearance can be identical to ebstein anomaly.

Front 8

Blue, increased pulmonary arterial flow

Back 8

-Admixture lesions: systemic and pulmonary circulations are mixed
-5 T's:
-Truncus arteriosus: failure of division of primitive truncus into an aorta and a pulmonary artery. One vessel arises from heart. There is always an associated VSD. Classified into how the pulmonary arteries arise from the primitive truncus. Increased blood flow. Right aortic arch in 1/3. Usually moderate cardiomegaly and superimposed pulmonary venous congestion.
-TAPVR: pulmonary venous return does NOT connect to the left atrium, but instead connects to systemic venous structures such as the SVC, RA, or portal vein. Divided into supracardiac, cardiac, or infracardiac. Supracardiac is most common: pulmonary veins converge and form a left vertical vein that runs superiorly and connects into the innominate vein. With infracardiac, the pulmonary veins penetrate the diaphragm and connect to the IVC below the diaphragm. These veins may become obstructed, and present with pulmonary edema! If not obstructed, cardiomegaly and increased pulmonary artery flow. Supracardiac TAPVR has a snowman appearance: dilated left vertical vein and dilated SVC form the superior part of the snowman, and the cardiac silhouette forms the inferior portion.
-Transposition. D-TGA: most common CHD presenting with cyanosis in the first 24 hrs of life. Aorta and pulmonary arteries are transposed. Blood flows in two parallel circuits. Survival depends on PFO, ASD, VSD, or PDA. Now, arterial switch procedures occur during the first 2 weeks of life, so increased flow is rarely seen. Most common CXR in a child born with D-TGA is normal. But, narrowing of superior mediastinum due to decreased thymic tissue and AP stacking of great vessels: egg on a string.
-Tricuspid Atresia (without RVOT obstruction): RA markedly enlarged, causing marked cardiomegaly. Radiograph can vary greatly.
-Tingles (single ventricle/atrium)

Front 9

Pink, increased pulmonary arterial flow

Back 9

-Left to right shunts
-ASD, VSD, PDA, atrioventricular canal (endocardial cushion defect), partial anomalous pulmonary venous return, and aortopulmonary window.
-Particular chamber enlargement is a clue to which type of shunt is present:
-ASD: RA and RV are enlarged
-VSD: RV, LA, and LV are enlarged
-PDA: LA, LV, aorta are enlarged

-Or, age at presentation: Very large shunts (VSDs or ECDs present in infancy). ASDs present later in childhood or in early adulthood. PDAs occur most commonly in premature infants. ECDs mostly in children with T21.

-CXR: increased PA flow, a variable amount of increased PV flow, and cardiomegaly. Marked hyperinflation can be seen (air trapping secondary to peribronchial edema).
-

Front 10

Pink with increased pulmonary venous flow, normal to increased C/T ratio

Back 10

-essentially a patient with CHF (this list is also a DDx of CHF in a newborn)
-Two major categories of disease:
1. Anatomic left sided obstruction:
a)LV outflow obstruction:
AS
Coarctation
Inturrupted aortic arch
Hypoplastic left heart
LV dysfunction: cardiomyopathies, glycogen storage disease, ALCAPA, birth asphyxia (shock myocardium), infants of diabetic mothers, hypoplastic left heart syndrome
b) LV inflow obstruction:
obstructed TAPVR (i.e. infracardiac)
Cor triatriatum
Pulmonary vein atresia
Congenital MV stenosis
2. Systemic causes: anemia, polycythemia, sepsis, high output failure resulting from a peripheral AVM (VOGM, hepatic hemangioendothelioma)

Front 11

hypoplastic left heart syndrome

Back 11

combination of hypoplasia or aplasia of the ascending aorta, aortic valve, left ventricle, and mitral valve
-present with CHF at birth
-dependent on a PDA
-systemic flow goes from the main PA to the descending aorta via the PDA
-flow to coronary arteries and cranial areas is retrograde via the hypoplastic ascending aorta
-Treatment is the norwood procedure

Front 12

Pink, normal pulmonary arterial flow

Back 12

-obstructive lesions, extrinsic airway compression, and congenital heart disease with increased or decreased pulmonary arterial flow that has been surgically corrected.
-Obstructive lesions: AS, PS, and coarctation of the aorta
-AS: biscuspid aortic valve or previous rheumatic disease. May be dilatation of ascending aorta. Ascending aorta should never be identified in a normal child on frontal radiography!!! Another cause: aneurysm such as with Marfan syndroma. With AS: may also see LV enlargement due to hypertrophy. Williams syndrome: supravalvular aortic stenosis, peripheral pulmonary artery stenosis, and other sx such as mental retardation.
-PS: dilatation of main PA secondary to the jet effect.
-Coarctation of aorta: diffuse or localized (more common, usually just beyond the left subclavian artery in the vicinity of the ductus arteriosus). Leftward border of superior mediastinum = 3. Sup portion: prestenotic dilatation of the aorta above the coarctation, narrow part is coarctation, and the inferior part is the poststenotic dilatation of the descending aorta. Rib notching occurs at 4th-8th ribs. Increased association with bicuspid aortic valve. Tx is balloon dilatation and stent placement.

Front 13

Conotruncal rotation abnormalities

Back 13

-Primitive truncus is an anterior midline structure, that divides into the aorta and pulmonary artery, which rotates clockwise 150 degrees.
-Normal PA: Superior, Anterior, and to the Left of the aorta.
-Abnormal division or rotation of this primitive truncus may result in a number of diseases, including L-TGA, D-TGA, truncus arteriosus, double outlet RV, and situs inversus.
-The cross sectional anatomic relationship between the Ao and PA at the level of the semilunar valves is characteristic of these abnormalities!!
-Truncus arteriosus: failure of division of the primitive truncus into a separate aorta and pulmonary artery. A single large artery gives rise to the coronary, systemic, and pulmonary arterial circulation.
-L-TGA (congenitally corrected TGA), AV and VA discordance. The morphologic RV is in the position of and serves as the anatomic LV. May be isolated, commonly occurs as part of a complex CHD. Ao is anterior and leftward to the PA.
-D-TGA: ventriculo-arterial disconcordance (ao from RV, PA from LV). Ao is anterior and to the right of the PA. Arterial switch = Jatene procedure (PA draped over Ao anteriorly), occurs after interatrial baffle.
-Double outlet RV: Only outlet for LV is a VSD. Often assoc with other complex CHD. Ao and PA are side by side, with the aorta on the right.
-Situs inversus: Ao, PA completely opposite of normal.

Front 14

Kawasaki disease

Back 14

-mucocutaneous lymph node syndrome
-inflammatory disease of unknown cause
-fever, rash, conjunctivitis, erythema of the lips and oral cavity, and cervical lymphadenopathy
-generalized vasculitis
-Acute myocarditis which can lead to CHF.
-Delayed complications include development of coronary artery aneurysms, coronary artery stenoses.
-rarely, the coronary artery aneurysms can calcify
-CXRs show CHF when myocarditis is severe
-Gallbladder hydrops (on U/S)
-tx is gamma globulin

Front 15

Cardiac masses

Back 15

-By far, the most common type is a rhabdomyoma
-seen in TS
-typically involute over time and are usually treated conservatively
-Other masses: angiosarcoma, fibroma, teratoma, hemangioma.
-Most common cause of cardiac tumor associated with a pericardial effusion is a hemangioma