case review cardiac

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CXR being shown for congenital heart dz: 1) PULM BLOODFLOW

2) HEART SIZE
Group I criteria: ACYANOTIC

with

Increased pulmonary bloodflow

This means L to R shunt
What are the L to R shunt lesions: 1) Atrial level shunts -- Partial anomalous pulmonary venous return and ASD

2) VSD

3) PDA
Group II criteria: 1) CYANOTIC

2) Normal or decreased pulmonary bloodflow (mean the same thing in cyanotic patients)

3) Little or no cardiomegaly
Group II lesions: Tetralogy of Fallot

AND other lesions that look like tetralogy of Fallot

i.e. Pulm atresia with VSD

Some forms of tricuspid atresia -- those with a nonrestrictive intracardiac defect (large ASD or large VSD) which equalizes pressures across the 2 sides and stops cardiac enlargement from occurring

Basically, its TOF and lesions with severe restriction of pulmonary outflow with an unrestrictive defect within the heart.
Criteria for getting into group III: 1) CYANOSIS

2) Decreased or normal PBF

3) Cardiomegaly
Group III lesions: #1 diagnosis in this category: EBSTEIN'S. Also critical pulmonary stenosis and some forms of pulmonary atresia and tricuspid atresia.
Group IV criteria: 1) CYANOSIS

2) INCREASED PBF
Group IV lesions: "T" lesions, but not TOF, AND hypoplastic left heart

Transposition

Total anomalous pulm venous return

Truncus arteriosus

Tricuspid atresia

Tingle ventricle or atrium

Hypoplastic left heart

AND DORV, DOLV, which are just cousins of tets.
CXR meets criteria for group I: DIAGNOSTIC PATHWAY

2 stopsigns:

The first is to check for presence of left atrial enlargement. If the left atrium is not enlarged, its an atrial level shunt. This is because the blood is being allowed to cross back to the right side either prior to or via the left atrium, so the left atrium does not experience increased pressures.

If there is left atrial enlargement, then your shunt occurs distal to the atrial level, either a VSD or PDA.

The next stop sign is to look at the aortic arch. If the aortic arch is normal, its a VSD. If its enlarged, you have a PDA, which always results in enlargement of the aortic arch OUTSIDE of the infancy period.
Most common group IV lesion: transposition
Group IV lesion with narrow mediastinum: transposition, also has egg shaped heart (different appearance in corrected transpo)
Appearance of Transposition: on AP view, looks egg shaped, with very narrow mediastinum = egg on the straight appearance.
Group IV lesion with right aortic arch: truncus arteriosus, usually type I.
group IV lesion with widened mediastinum: supra cardiac type of total anomalous pulmonary venous connection.
AP radiograph of the chest demonstrates mild dextroposition of the heart and a linear structure which enlarges as it travels inferiorly towards the diaphragm: scimitar syndrome. Dextroposition of heart due to hypogenetic right lung. Linear structure is anomalous venous connection training right lung through scimitar vein.
Prominent convexity along upper left cardiac border: corrected transposition. Prominent convexity is ascending aorta, and there is absence of the ascending aorta in the normal position at the right cardiac border.
Large main pulmonary artery segment, and enlarged left pulmonary artery on plain film: pulmonic stenosis with poststenotic dilatation.
Long segment aortic Coarctation with enlarged proximal aorta: think of acquired pathology in adults, such as from Takayasu's.
Most common form of partial anomalous pulmonary venous connection: R upper lobe pulm vein to SVC
Another common form of PAPVC: Both upper lobe pulmonary veins drain into the SVC. The right is the same as the most common form, but on the left, the upper lobe pulmonary vein drains via a vertical vein, to enter the left brachiocephalic vein, and then into the superior vena cava. Very similar in appearance to supra cardiac total anomalous pulmonary venous return, however, this is partial so it only involves the upper lobe pulmonary veins.
Most likely lesion to be seen on MRI in cardiac section: tetralogy of fallot.
Components of tetralogy of fallot: unrestrictive membranous (high) ventricular septal defect, overriding aorta, infundibular pulmonary stenosis (always infundibular stenosis, sometimes narrowing at the annulus) , and right ventricular hypertrophy.
25% of tetralogy of fallot: right aortic arch
axial image of the heart demonstrating only one great vessel coming out of the heart: either pulmonary atresia or truncus arteriosus. In hypoplastic left heart with a atretic aorta, usually will see some remnant.
Patient with tetralogy of fallot and severe pulmonary atresia: how does the lung get perfused: ductus arteriosus doesn't help so much with severe atresia. Patient develops systemic to pulmonary collaterals to feed the pulmonary arteries past the atresia. They are not really bronchial arteries that are enlarged, because not all of them are bronchial arteries. The proper terminology is systemic to pulmonary collaterals. Now I see why we call it pseudo truncus, because when there's severe pulmonary atresia, it does look like a single vessel emerging from the heart.
Diagnostic of transposition: aorta in front of pulmonary artery. Only other thing-- double outlet right ventricle, not likely to be seen on boards.
Normal positions of great vessels: pulmonary valve is normally left, superior, and anterior to aortic.
Malignant coronary artery anomaly: anomalous coronary artery which passes inter arterial (between pulmonary artery and aorta). Outcome is sudden death during exercise.
how is acquired cardiac disease categorized: small heart and big heart heart disease
small heart heart disease: cardiothoracic ratio of less than 0.55
small heart heart disease lesions: pressure overload lesions and lesions which reduce ventricular compliance
big heart heart disease: cardiothoracic ratio greater than 0.55
big heart heart disease lesions: volume overload lesions or lesions in which early in the course of disease there is myocardial failure (i.e. cardiomyopathy)
cardiac disease with small heart: is there left atrial enlargement? if yes, that points to mitral valve disease. In small heart heart disease, suspect mitral stenosis. If no LAE , go to next signpost.
Cardiac disease with small heart, no left atrial enlargement: is there enlargement of the ascending aorta or aortic knob ? If yes, suspect aortic stenosis. If no, there are no signposts.
Cardiac disease with small heart, no left atrial enlargement or aortic enlargement: differential includes myocardial and pericardial disease such as acute M. I. , restrictive or hypertrophic cardiomyopathy, or constrictive pericarditis
cardiac disease with large heart: is there left atrial enlargement? if yes, that again points to mitral valve disease, which in big heart heart disease implies mitral regurgitation. If no LAE, go to next signpost.
Cardiac disease with large heart, no left atrial enlargement: is their ascending aorta or thoracic aortic enlargement? If yes, that again points to the aortic valve, but in big heart heart disease implies aortic regurgitation.
Cardiac disease with large heart, no signposts: again points to myocardial or pericardial disease, but this time things that make the myocardium floppy such as dilated cardiomyopathy, ischemic cardiomyopathy, or pericardial effusion. Also tricuspid regurgitation or right ventricular failure.
Determining left atrial enlargement: left atrial double density OR lack of concavity underneath main pulmonary artery, representing dilated left atrial appendage
dilated left atrial appendage: you see this, it implies a specific type of mitral valve disease, that caused by rheumatic heart disease. There are many causes of enlargement of the left atrium, but it is rheumatic heart disease that is specific for enlargement of the left atrial appendage.

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