Cardiac Flashcards
Diagnosis?
Association?
Left atrial myxoma. 75% in the LA, 20% in the RA, rarely ventricular. Usually originate from the fossa ovalis. Can be heterogeneous on MR, T1 low, enhance (helps distinguish from thrombus). Can have calcifications.
Mostly sporatic, but can be associated with Carney complex (rare MEN syndrome)- cardiac & extracardiac myxoma, skin pigmentation (blue nevi), pituitary adenoma, psammomatous melanotic schwannoma, testicular tumors.
Diagnosis?
What is this?
Papillary fibroelastoma.
Benign, rare gelatinous tumors derived from the endocardium, primarily occuring on the left-sided cardiac valves. Potential for emoblization.
Small round or oval polypoid lesion (<2cm). Homogeneous echotexture. Usually mobile, attached to the commisure fo a cardiac valve by a small stalk.
Diagnosis?
Why is this problematic?
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA, aka Bland-White-Garland syndrome).
Flow in the left coronary artery is reversed, and is towards the pulmonary artery due to lower pressure in the MPA. This results in a steal phenomenon, with blood diverted from the heart. Very high mortality rate in children. In those that survive untreated to adulthood, there is usually significant intercoronary collateral circulation with dilated and tortuous coronaries.
The right coronary version (ARCAPA) is very rare, may be asymptomatic.
What are the most common causes of constrictive pericarditis?
Imaging features?
Treatment?
Prior surgery or radiation. Also infectious pericarditis (viral, TB), collagen vascular disease, uremia.
The affected pericardium may be normal, but is often thickened (>4 mm). May be calcified, especially at the AV groove. Small ventricles, dilated atrai, dilated IVC.
Surgery (pericardiectomy or pericardial stripping).
Important to differentiate from restrictive cardiomyopathy.
What is Shone’s complex?
A complex of obstructive lesions including mitral valve stenosis or parachute deformity, bicuspid aortic valve, and coarctation of the aorta.
Diagnosis?
Other form?
Associations?
Discrete subaortic stenosis. Fibrous narrowing proximal to the aortic valve. This type is most common. Typically present around 20 years.
Muscular- more diffuse narrowing.
Aortic regurgitation seen in 80%. Associated with bicuspid aortic valve, VSD, Shone’s complex.
What are the types of ASD?
What is supravalvular aortic stenosis?
Association?
Imaging features?
A rare condition- focal or diffuse narrowing af the aorta above the level of the aortic valve, resulting in LV outflow obstruction. Defect in the elastin gene on chromosome 7, can be familial (AD) or sporatic.
Associated with Williams-Beuren syndrome (also see elfin facies, hypercalcemia, pulmonary artery stenosis, mitral valve prolapse) and congenital heart disease.
Can see narrowing of other vessels besides the aorta: peripheral pulmonary arteries, arch vessels, renal. Can get valve thickening, dilated coronaries.
Diagnosis?
Imaging features?
Cardiac amyloidosis. Deposition of amyloid protein in the myocardium. Usually primary amyloidosis (AL type). Cardiac involvement in secondary (AA type) is rare, and usually causes restrictive cardiomyopathy.
MRI: LV wall thickening, patchy and heterogeneous enhancement throughout the myocardium, difficult to suppress the signal of the myocardium on delayed gadolinium images.
What MRI findings can you see with cardiac sarcoidosis?
Seen in ~25% of patients with sarcoid, but only a minority are symptomatic. Get sarcoid granulomas in the myocardium, can lead to arrhythmias (heart block most common). Findings are nonspecific, but look for:
Mid or epicardial delayed enhancement in a noncoronary distribution. Often involves the interventricular septum, and sometimes the LV wall. Myocardial thickening, contraction abnormalities.
What are the types of endoleaks?
Most likely congenital cardiac defect in this patient?
Associated anomalies?
Dextro-transposition of the great arteries. Because of the anterior & right position of the aorta in relation to the pulmonary artery.
“Complete transposition-“ aorta arises from RV, pulmonary artery arises from LV. Complete separation of the systemic and pulmonary circulation, so there must be a communication to survive (ductus arteriorsus, PFO most common).
Most often occurs without associated cardiac anomalies (unilke L-TGA, which is often associated with other congenital heart anomalies). Arterial switch- most common repair procedure (over “atrial switch” aka Mustard procedure).
Diagnosis?
How is this diagnosis made?
Cause?
Arrhythmogenic right ventricular dysplasia (AVRD, aka AVRC). Fatty and fibrous replacement of RV > LV myocardium. See hyperintense fat on spin echo and PD images, fat sats out. Fibrous tissue enhances with gad. May get right ventricular thinning and dilatation.
MRI is suggestive but not diagnostic of AVRD. Made based on major and minor criteria which include family history, biopsy, MRI, and ECG.
Autosomal dominant, with involvement of chromosomes 1, 2, 14, and 17.
What is the mode of inheritance of hypertrophic obstructive cardiomyopathy?
Typically autosomal dominant with variable penetrance.
What is the cause of hypoplastic left heart syndrome, and what is seen?
Mistral valve stenosis limits flow of blood into the LV. Without blood flow, the LV, and subsequently the aortic valve and ascending aorta, are not stimulated to grow and are small.
Perfusion of the coronary arteries is “ductal dependent,” and is from the pulmonary artery retrograde through the ascending aorta via a PDA.
There must be an ASD for pulmonary vein flow into the LA to shunt to the RA. If the ASD is small (restrictive), can see severe pulmonary hypertension.
Diagnosis?
What is this?
Genetics?
Noncompaction of the left ventricle.
Arrested development of organized compaction of the myocardium. Markedly prominent trabeculation, decreased ventricular function, may form endocardial thrombus.
May be sporatic or X-linked, anomalies of q28 region of the X chromosome.
