A persistent left superior vena cava (SVC) is visualized in a 46-year-old woman with shortness of breath (SOB), suggesting the possibility of a previously unrecognized unroofed coronary sinus atrial septal defects (ASD). However, a confluence of right upper pulmonary veins entering the high right atrium/proximal right SVC is seen (see Fig. 14-1), consistent with a sinus venosum defect. The sinus venosum defect is seen in a coronal image depicting the entry site into the RA (arrow) and right SVC (chevron). The bottom left image is a 3D surface from the magnetic resonance angiography (MRA) of a 29-year-old woman in whom a murmur was heard and an echocardiography was performed demonstrating right ventricular (RV) enlargement, which prompted the CMR. A sinus venosus defect was seen for which she underwent surgical baffle from the SVC/RA to the LA redirecting pulmonary flow to the LA and venous return to the RA.

A 35-year-old woman presents with SOB. A CMR demonstrates a moderate-sized ostium secundum ASD (see Fig. 14-2). Various PVMs convincingly demonstrate the magnitude of the shunt which was measured as a Qp:Qs = 2.1 : 1 for which she underwent percutaneous placement of a closure device. SSFP and PVM were all that was necessary to accurately define the physiology. If an intracardiac shunt is suspected by SSFP imaging, confirmation can be obtained by switching to a gradient-recalled echo (GRE) sequence and increasing the echo time (TE) to 8.5 to 10 milliseconds. Further confirmation and quantification can be obtained using PVM as shown in the right panels. Often, a more dramatic visualization can be demonstrated through PVM than can be achieved by a dephasing artifact.

An 18-year-old black male football player presents with SOB. Routine echocardiography reveals markedly positive bubble study (left arm IV). TEE performed demonstrates no bubbles crossing. Repeat TTE performed again show copious bubble immediately crossing from the RA to the LA. Unknown diagnosis prompts CMR (see Fig. 14-3). On the basis of presentation, a coronal SSFP was performed (mid panel) and suggested the diagnosis. Temporal MRAfrom the left arm shows two mechanisms of venous return into the heart. Note the exquisite anatomic detail depicted, demonstrating both anatomy and physiology with the transit of contrast over time and space. This is diagnostic of a very rare anomalous left upper pulmonary vein draining into the cardinal vein (“Partially Partial Anomalous”).

This 32-year-old woman presents for evaluation of dyspnea. Routine CMR reveals an incidental finding of an interrupted inferior vena cava (IVC) (see Fig. 14-4). Note the multiple circular densities (see Fig. 14-4, left panel, arrows) indicative polysplenia, an often associated finding. This can be part of the Heterotaxy syndrome. Note the large SVC (middle panel) from the rerouted azygous return through which the mesenteric venous flow destined for the RA is rerouted. This is a benign variant for which, in the absence of imaging, she would be oblivious to her anomaly.

Axial-SSFP images revealing anomalous right upper pulmonary venous (RUPV) drainage into the mid SVC in a 41-year-old woman with palpitations and mild dyspnea on exertion (DOE) (see Fig. 14-5). A sinus venosus anomaly is differentiated, as the RUPV drains into the junction of the high RA and low SVC. Note the confluence of two pulmonary veins into SVC; which are very important for complete surgical repair, in addition to identification of any and all anomalous drainage into the SVC because the surgeon may only be expecting to correct one vein.

A 41-year-old white man with diagnosis of I-transposition since a child, and told he would never be normal. Multiple subsequent angiograms and TTEs and TEEs had been performed over the ensuing years, each reinforcing the last, saying that he had congenitally corrected transposition and eventually his heart would fail. Referred to us almost by accident, he underwent CMR (see Fig. 14-6). The RV moderator band is on the rightward and anterior trabeculated ventricle, whereas the leftward nontrabeculated ventricle AV valve plane is displaced atrially relative to the right AV valve plane (upper panels). There is no AV concordance (nor VA concordance). Although the cardia is markedly malrotated and superiorly displaced there is no transposition anatomy. The mother and son ask if they could hear the results of the study, stating how the grave diagnosis had affected her, her husband’s, and her son’s life, and how they wondered each day if they would wake up to see their son dead, day after day, year after year, and how it affected his grades, his plans for the future, and his relationships with others including his ability to marry as he got older. Is good news bad here? Indeed, good news can be catastrophic because they both realized how very different the last 41 years might have been.

A 27-year-old black woman status post Jatene arterial switch for d-transposition of the great vessels referred for evaluation of right ventricle morphology and function (see Fig. 14-7). Instead of a Senning or Mustard atrial baffle or reconstruction technique, the patient underwent the arterial switch (Jatene) surgery. In the left panel, arrow points to the reinsertion of the coronary arteries as a button graft from the native pulmonary artery (PA), as well as the anterior positioned RV with its moderator band delineating the morphologic RV as the original systemic ventricle.

A 45-year-old woman with known ventricular septal defect (VSD) diagnosed at early childhood. After many years being lost to follow-up she is referred for CMR (see Fig. 14-8). Her examination no longer reveals a murmur of VSD but a harsh systolic ejection sound with loss of P2 and a prominent RV S4. Examination reveals hypertrophied RV free wall without VSD. In its place there is a prominent RV high septal muscle band creating dramatic subpulmonic stenosis (right panel) with a high velocity jet radiating into and across the pulmonic valve without frank pulmonic valvar stenosis. Spontaneous closure of VSDs has been shown to occur by several means, one of which is hypertrophy of the adjacent myocardial tissue, but on occasion is overzealous converting a volume overload situation into a pressure overload state, as in this case. The patient underwent myotomy with relief of the 98 mm Hg gradient and relief of symptoms.

Standard SSFP images performed in a 32-year-old woman referred for reasons unrelated to her final diagnosis. In Fig. 14-9 left panel, standard two-chamber view provides the rationale for why the large FOV is a valuable commodity for CMR, where a moderate-sized patent ductus arteriosum is seen. Using PVM, the Qp:Qs was quantified as 1.3:1 and she underwent uneventful percutaneous coil closure and went home the following day.

A 35-year-old man presents with a harsh pan-cyclic murmur, heard most prominently along his back, with symmetric pulse in his upper extremities but diminished pulses bilaterally in his lower extremities. A coarctation was suspected, as confirmed on the 20-second MRA acquisition (see Fig. 14-10, arrow). Note the severe coarctation and poststenotic dilatation. Additionally, an indication of the collateral circulation to the lower extremities can be gleaned from the widely dilated right internal mammary artery (chevron).

A 36-year-old man with a history of Tetralogy of Fallot and large left bronchial collaterals has undergone several repairs since age of 6 years. He presents with a 6-day history of chest pain and progressive dyspnea on exertion. His admitting electrocardiogram (ECG) reveals a right bundle branch block, and negative cardiac enzymes. The patient continued to deteriorate, requiring mechanical ventilation, whereupon a transesophageal echocardiography was performed which suggested an ascending aortic aneurysm with possible focal dissection and normal left and right sized ventricle. To further delineate the anatomy, a CMR was performed which revealed gargantuan left and right ventricles measuring 98 mm and 54 mm in enddiastole, respectively (see Fig. 14-11). The left ventricle was extensively compressed from the right ventricle, resulting in severe distortion in the superior-inferior direction. The left atrium was markedly dilated and tubular, due to marked posterior dilation and displacement of the ascending aortic aneurysm. The right ventricle was markedly dilated and hypertrophied with severe pulmonic, tricuspid, and AR. The sinus of Valsalva was markedly dilated measuring 61 mm whereas the proximal ascending aorta formed a gigantic aneurysm measuring 93 × 83 mm, terminating cranially into a nearly normal size aortic arch. Most importantly, there was a focal dissection in the anterior and right lateral wall of the aortic aneurysm. Additionally, several aorta-topulmonary (bronchial) collaterals were seen, with many communicating directly with the left atrium.