Cardiovascular magnetic resonance (CMR) angiography (CMRA) is an important non-invasive imaging tool for congenital heart disease (CHD) and aortopathy patients. The conventional 3D balanced steady-state free precession (bSSFP) sequence is often confounded by imaging artifacts. We sought to compare the respiratory navigated and electrocardiogram (ECG) gated modified Dixon (mDixon) CMRA sequence to conventional non-gated dynamic multi-phase contrast enhanced CMRA (CE-CMRA) and bSSFP across a variety of diagnoses. We included 24 patients with CHD or aortopathy with CMR performed between September 2017 to December 2017. Each patient had undergone CE-CMRA, followed by a bSSFP and mDixon angiogram. Patients with CMR-incompatible implants or contraindications to contrast were excluded. The studies were rated according to image quality at a scale from 1 (poor) to 4 (excellent) based on diagnostic adequacy, artifact burden, vascular border delineation, myocardium-blood pool contrast, and visualization of pulmonary and systemic veins and coronaries. Contrast-to-noise ratio (CNR), signal-to-noise ratio (SNR) and quantitative vascular measurements were compared between the two gated sequences. Bland-Altman plots were generated to compare paired measures. All scans were diagnostically adequate. Mean (SD) quality scores were 3.4 (0.7) for the mDixon, 3.2 (0.5) for the bSSFP and 3.4 (0.5) for the CE-CMRA. Qualitatively, the intracardiac anatomy and myocardium-blood pool definition were better in the bSSFP; however, mDixon images showed enhanced vessel wall sharpness with less blurring surrounding the anatomical borders distally. Coronary origins were identified in all cases. Pulmonary veins were visualized in 92% of mDixon sequences, 75% of bSSFP and 96% of CE-CMRA. Similarly, neck veins were identified in 92, 83 and 96% respectively. Artifacts prevented vascular measurement in 6/192 (3%) and 4/192 (2%) of total vascular measurements for the mDixon and bSSFP, respectively. However, the size of signal void and field distortion were significantly worse in the latter, particularly for flow and metal induced artifacts. In patients with congenital heart disease, ECG gated mDixon angiography yields high fidelity vascular images including better delineation of head and neck vasculature and pulmonary veins and fewer artifacts than the comparable bSSFP sequence. It should be considered as the preferred strategy for successful CHD imaging in patients with valve stenosis, vascular stents, or metallic implants.

Rare cardiovascular diseases (RCDs) have low incidence but major clinical impact. RCDs’ classification includes Class I—systemic circulation, Class II—pulmonary circulation, Class III—cardiomyopathies, Class IV—congenital cardiovascular diseases (CVD), Class V—cardiac tumors and CVD in malignancy, Class VI—cardiac arrhythmogenic disorders, Class VII—CVD in pregnancy, Class VIII—unclassified rare CVD. Cardiovascular Magnetic Resonance (CMR) is useful in the diagnosis/management of RCDs, as it performs angiography, function, perfusion, and tissue characterization in the same examination. Edema expressed as a high signal in STIRT2 or increased T2 mapping is common in acute/active inflammatory states. Diffuse subendocardial fibrosis, expressed as diffuse late gadolinium enhancement (LGE), is characteristic of microvascular disease as in systemic sclerosis, small vessel vasculitis, cardiac amyloidosis, and metabolic disorders. Replacement fibrosis, expressed as LGE, in the inferolateral wall of the left ventricle (LV) is typical of neuromuscular disorders. Patchy LGE with concurrent edema is typical of myocarditis, irrespective of the cause. Cardiac hypertrophy is characteristic in hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA) and Anderson–Fabry Disease (AFD), but LGE is located in the IVS, subendocardium and lateral wall in HCM, CA and AFD, respectively. Native T1 mapping is increased in HCM and CA and reduced in AFD. Magnetic resonance angiography provides information on aortopathies, such as Marfan, Turner syndrome and Takayasu vasculitis. LGE in the right ventricle is the typical finding of ARVC, but it may involve LV, leading to the diagnosis of arrhythmogenic cardiomyopathy. Tissue changes in RCDs may be detected only through parametric imaging indices.

Marfan syndrome (MFS) is an inherited autosomal-dominant connective tissue disorder with multiorgan involvement including musculoskeletal, respiratory, cardiovascular, ocular, and skin manifestations. Life expectancy in patients with MFS is primarily determined by the degree of cardiovascular involvement. Aortic disease is the major cardiovascular manifestation of MFS. However, non-aortic cardiac diseases, such as impaired myocardial function and arrhythmia, have been increasingly acknowledged as additional causes of morbidity and mortality. We present two cases demonstrating the phenotypical variation in patients with MFS and how CMR (Cardiovascular Magnetic Resonance) could serve as a “one stop shop” to retrieveS all the necessary information regarding aortic/vascular pathology as well as any potential underlying arrhythmogenic substrate or cardiomyopathic process.