Andrada R. Popescu

Intramural Coronary Length Correlates With Symptoms in Patients With Anomalous Aortic Origin of the Coronary Artery

BACKGROUND Anomalous aortic origin of a coronary artery (AAOCA) from the opposite sinus of Valsalva is a rare congenital anomaly with the potential for myocardial ischemia and sudden death. This review evaluated our series of AAOCA patients, who underwent coronary artery unroofing, to test our hypothesis that the intramural length of the anomalous coronary artery correlates with symptoms. METHODS A retrospective analysis of symptoms, preoperative imaging (computed tomography and magnetic resonance imaging), intraoperative assessment, perioperative course, and follow-up were reviewed. RESULTS From 2005 to 2010, 27 patients (70% male) underwent surgical AAOCA repair. Mean age was 14.3±12 (range, 6 to 52) years. In 25 patients with right AAOCA, 14 had chest pain and 4 had syncope. Both patients with left AAOCA had chest pain. AAOCA unroofing was done in 25 and side-to-side anastomosis in 2. The intramural coronary artery length measured intraoperatively correlated with preoperative symptoms (symptoms=10±3.58 mm, no symptoms=5.2±1.5 mm, p<.002), as did preoperative imaging measurements (symptoms=7.8±2.8 mm, no symptoms=5.3±0.8 mm, p<.001). Preoperative imaging strongly predicted the intraoperative measurement (r=0.81, p=0.00001). There were no deaths, significant morbidity, or recurrence of symptoms. CONCLUSIONS Coronary unroofing for AAOCA is a safe method of enlarging the coronary orifice and eliminating the intramural course. Symptomatic patients had a longer intramural course than asymptomatic patients, as assessed by preoperative imaging and intraoperative measurements. These results may have important clinical implications in determining indications for operation.

Safety of Blood Pool Contrast Agent Administration in Children and Young Adults

OBJECTIVE The objective of our study was to determine the adverse reaction rate associated with the administration of blood pool contrast material in children and young adults. MATERIALS AND METHODS A review of the MRI and pharmacy databases identified all patients who received gadofosveset trisodium from October 1, 2011, to June 30, 2014. Patients were classified as having been anesthetized or not anesthetized for the MRI examinations. A review of the electronic medical records identified adverse reactions recorded within 24 hours of contrast administration. The adverse reactions were graded as mild, moderate, or severe. Risk ratios were calculated between the adverse reaction rate experienced by anesthetized patients and that experience by nonanesthetized patients. RESULTS During the study period, 626 patients (mean age, 11.7 years) received 711 doses of gadofosveset trisodium; 137 adverse reactions were recorded, which yields a 19.3% (137/711) adverse reaction rate. There were 115 adverse reactions experienced by 367 anesthetized patients (31.3%): 93.0% (107/115) were mild and 7.0% (8/115) were moderate. The remaining 22 adverse reactions were experienced by 344 (6.4%) nonanesthetized patients, and 90.9% (20/22) were mild. Three nonanesthetized patients had allergiclike reactions; of these allergiclike reactions, one was mild and two were severe for a severe allergiclike reaction rate of 0.28% (2/711). Severe allergiclike reactions were treated without any adverse outcomes. Anesthetized patients were 5.7 times more likely to experience an adverse event than nonanesthetized patients; most reactions in anesthetized patients were seen after the administration of anesthesia alone. CONCLUSION Most reactions after gadofosveset trisodium administration in children and young adults are mild; however, severe allergiclike reactions occur, so policies must be in place to treat patients with adverse reactions when using this contrast agent. These data may be useful to centers considering administering gadofosveset trisodium to pediatric patients.

Reoperation after Vascular Ring Repair

The majority of patients having surgical intervention for a vascular ring have resolution of their symptoms. However, 5% to 10% of these patients develop recurrent symptoms related either to airway or esophageal compression and may require reoperation. In our series of 300 patients with vascular rings, we performed a reoperation on 26 patients, not all of whom were originally operated on at our institution. The four primary indications for reoperation were Kommerell diverticulum (n = 18), circumflex aorta (n = 2), residual scarring (n = 2), and tracheobronchomalacia requiring aortopexy (n = 4). All patients undergoing reoperation have had preoperative evaluation with bronchoscopy and computed tomographic scanning (CT) with 3-dimensional reconstruction. Patients with dysphagia have had a barium esophagram and esophagoscopy. Patients with a Kommerell diverticulum have undergone resection of the diverticulum and transfer of the left subclavian artery to the left carotid artery. The aortic uncrossing procedure has been used in patients with a circumflex aorta. Aortopexy has been used to treat anterior compression of the trachea by the aorta. Results of these reinterventions have been successful in nearly all cases. Lessons learned from these reoperations can be applied to prevent the need for reoperation by properly selecting the correct initial operation. A dedicated team caring for these children consisting of medical imaging, otolaryngology, cardiovascular-thoracic surgery, and critical care is imperative.

Congenital heart disease in adults: Quantitative and qualitative evaluation of IR FLASH and IR SSFP MRA techniques using a blood pool contrast agent in the steady state and comparison to first pass MRA

OBJECTIVES To evaluate magnetic resonance angiography sequences during the contrast steady-state (SS-MRA) using inversion recovery (IR) with fast low-angle shot (IR-FLASH) or steady-state free precession (IR-SSFP) read-outs, following the injection of a blood-pool contrast agent, and compare them to first-pass MR angiography (FP-MRA) in adults with congenital heart disease (CHD). MATERIALS AND METHODS Twenty-three adult patients with CHD who underwent both SS-MRA and FP-MRA using a 1.5-T scanner were retrospectively identified. Signal-to-noise and contrast-to-noise ratios were obtained at eight locations within the aorta and pulmonary vessels.. Image quality and the presence of artifacts were subjectively assessed by two radiologists. The presence of pathology was noted and given a confidence score. RESULTS There was no difference in vessel dimensions among the sequences. IR-SSFP showed better image quality and fewer artifacts than IR-FLASH and FP-MRA. Confidence scores were significantly higher for SS-MRA compared to FP-MRA. Seven cases (30.4%) had findings detected at SS-MRA that were not detected at FP-MRA, and 2 cases (8.7%) had findings detected by IR-SSFP only. CONCLUSION SS-MRA of the thoracic vasculature using a blood pool contrast agent offers superior image quality and reveals more abnormalities compared to standard FP-MRA in adults with CHD, and it is best achieved with an IR-SSFP sequence. These sequences could lead to increased detection rates of abnormalities and provide a simpler protocol image acquisition.

Clinical utility of cardiac magnetic resonance T2 mapping for acute myocardial edema

Objective To evaluate the potential clinical utility of T2 quantitative mapping for myocardial edema in acute disease pathology - myocarditis, myocardial infarction, TakoTsubo cardiomyopathy, and transplant rejection. Background Edema is ag eneric tissue response to acute myocardial injury and, therefore; a potential marker of impending tissue damage. Currently in clinical use, T2 weighted imaging provides a qualitative technique in assessing myocardial edema. We hypothesize that quantitative T2 mapping in patients with suspected cases of myocarditis, myocardial infarction (AMI), and cardiac transplant rejection will provide a more sensitive and specific diagnostic prediction than with T2W imaging, and add to other imaging techniques. Method

Time-resolved MR venography of the pulmonary veins precatheter-based ablation for atrial fibrillation.

To evaluate time‐resolved magnetic resonance angiography (TR‐MRA) of the pulmonary venous circulation using the time‐resolved angiography with interleaved stochastic trajectories (TWIST) method and compare it with the more commonly used conventional contrast‐enhanced magnetic resonance angiography (CE‐MRA) approach in atrial fibrillation patients referred for preablation pulmonary vein mapping.

Transient partial response to sorafenib treatment in an adolescent patient with MEN2B syndrome and end-stage medullary thyroid carcinoma.

Metastatic medullary thyroid carcinoma (MTC) is an aggressive malignancy with an extremely poor prognosis. Currently no effective conventional systemic therapies exist to treat pediatric MTC. We describe an adolescent female with newly diagnosed MEN2B syndrome who presented with advanced stage metastatic MTC and demonstrated a partial transient response to sorafenib monotherapy. This clinical result supports further research into the use of sorafenib in the treatment of pediatric MTC. Pediatr Blood Cancer 2012; 58: 98–100.

Right pulmonary artery to left atrial fistula in a neonate: case report and review of the literature

A communication between the right pulmonary artery (RPA) and the left atrium is a rare congenital anomaly that presents with cyanosis and heart failure. We describe the surgical repair of an RPA to left atrial fistula using cardiopulmonary bypass in a neonate. Advanced imaging (computed tomography scan) guided the surgical approach. Although previous reports have associated a patent ductus arteriosus with high neonatal mortality, in our case, the ductus arteriosus was actually important for maintaining enough total pulmonary blood flow.

Comparison of 4D flow and 2D PC MRI blood flow quantification in children and young adults with congenital heart disease

Background Echocardiography (echo) is the primary imaging modality for assessment of aortic and pulmonary blood flow velocities. 2D phase contrast (PC) MRI provides better access to all segments of the aortic and pulmonary system and is considered the standard for evaluating blood flow. Both techniques are limited by velocity analysis in 2D planes and by single-direction velocity measurement which may be inadequate to characterize the complex 3D hemodynamics in congenital heart disease (CHD). 4D flow MRI provides simultaneous assessment of 3D blood flow characteristics of all vessels within a 3D volume and offers the ability to retrospectively quantify blood flow parameters at selectable regions of interest. The aim of this study is to test the potential of 4D flow for accuracy of quantification of aortic and pulmonary flow parameters compared to the reference standards echo and 2D PC MRI in children and young adults with CHD. Methods 32 patients with CHD who underwent simultaneous 4D flow and 2D PC MRI and echo within 9 months of MRI were retrospectively included. 2D PC MRI flow quantification in the aortic root (Ao), pulmonary trunk (PT), and right and left pulmonary arteries (RPA, LPA) was analyzed using Medis (Medis, Leiden, The Netherlands). 4D flow data analysis included calculation of a 3D-PC-angiogram which was used to position analysis planes in the Ao, PT, LPA and RPA (EnSight, CEI, Apex, NC) for quantification of net flow, regurgitant fraction, Qp:Qs, and peak velocities. Ao peak velocities were assessed by echo. Linear regression analysis was performed. Pearson’ sc orrelation coefficient (r) was calculated. A correlation with p<0.05 was considered significant. Results

Steady state imaging of the thoracic vasculature using inversion recovery FLASH and SSFP with a blood pool contrast agent

Background Although contrast-enhanced first-pass MRA (FP-MRA) is frequently used to visualize the thoracic vasculature, it may not be ideal for the assessment of the aortic root due to poor image quality and motion artifact. Bloodpool contrast agents remain within the intravascular space for several hours, allowing vessels to be imaged longer and therefore improving spatial resolution. The purpose of this study is to compare steady-state magnetic resonance angiography (SS-MRA) following injection of a blood-pool contras agent to first-pass MR angiography (FP-MRA) in adults with thoracic aortic disease. Methods 25 patients (14 men, 11 women) with suspect thoracic aortic disease disease underwent MRA on a 1.5 T scanner (Magnetom Aera and Avanto; Siemens Medical Solutions). The MRA protocol consisted of FP-MRA followed by SS-MRA after intravenous injection of gadofosveset trisodium (Ablavar, Lantheus Medical Imaging). FP-MRA consisted of a breath-held ECG-gated FLASH acquisition in a sagittal oblique orientation with the following imaging parameters: TR/TE: 2.8/1.0, flip angle 25°, FOV 343x500 mm, matrix 264x512, slice thickness 1.5 mm, voxel size 1.3 x 1.0 x 1.0 mm, GRAPPA x 2, 20 second acquisition. 0.03 mmol/kg of gadofosveset was injected intravenously at 1cc/sec in an antecubital vein. Contrast bolus timing was achieved using care bolus technique. SS-MRA consisted of free-breathing ECGgated IR-FLASH and IR-SSFP in a sagittal oblique orientation. IR-FLASH had the following parameters: TR/TE/TI: 3.5/1.5/260, flip angle 18°, and IR-SSFP had: TR/TE/TI: 3.3/1.5/260, flip angle 70°. Both sequences had FOV 326x380, matrix 440x512, slice thickness 1.5 mm, voxel 0.7 x 0.7 x 1.0 mm, GRAPPA x 2, and 3 minute acquisition. Respiratory gating was achieved using a navigator acquisition with an average acceptance window of 35%. For quantitative analysis, orthogonal dimensions of the thoracic aorta were measured at several locations. Signal-to-noise ratio (SNR) was also measured for both techniques by placing regions of interest in the aortic root and the ascending aorta. For qualitative analysis, two independent reviewers evaluated both FP-MRA and SS-MRA images separately. The aortic root and the ascending aorta were scored on an image quality scale of 1-4.