Faculty Bibliography

Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV). Six patients with complex anatomy and uncertainty of the optimal management strategy were included in this study. The models were subsequently used to guide management decisions, and the outcomes reviewed. 3D models clearly demonstrated the complex intra-cardiac anatomy in all six patients and were utilized to guide management decisions. In the three patients with CMVSD, one underwent successful endovascular device closure following a prior failed attempt at transcatheter closure, and the other two underwent successful primary surgical closure with the aid of 3D models. In all three cases of DORV, the models provided better anatomic delineation and additional information that altered or confirmed the surgical plan. Patient-specific 3D heart models show promise in accurately defining intra-cardiac anatomy in CHD, specifically CMVSD and DORV. We believe these models improve understanding of the complex anatomical spatial relationships in these defects and provide additional insight for pre/intra-interventional management and surgical planning.

Fluid overload (FO) is a common complication for pediatric patients in the intensive care unit. When conventional therapy fails, hemodialysis or peritoneal dialysis is classically used for fluid removal. Unfortunately, these therapies are often associated with cardiovascular or respiratory instability. Ultrafiltration, using devices such as the Aquadex system (Baxter Healthcare, Deerfield, IL, USA), is an effective tool for fluid removal in adult patients with congestive heart failure. As compared to hemodialysis, ultrafiltration can be performed using smaller catheters, and the extracorporeal volume and minimal blood flow rates are lower. In addition, there is no associated abdominal distension as is seen in peritoneal dialysis. Consequently, ultrafiltration may be better tolerated in critically ill pediatric patients. We present three cases of challenging pediatric patients with FO in the setting of congenital heart disease in whom ultrafiltration using the Aquadex system was successfully utilized for fluid removal while cardiorespiratory stability was maintained.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly present in approximately one in 300,000 live births. Here, we present a unique ALCAPA variant identified in a neonate. The left anterior descending artery originated posterolaterally on the main pulmonary artery, and the circumflex originated separately from the distal right pulmonary artery. doi: 10.1111/jocs.12079 (J Card Surg 2013;28:306-308).

ABSTRACT: OBJECTIVE: Behcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation. We present a case report of a teenager girl who developed recalcitrant life-threatening Behcet's vasculitis, involving both small and large venous and arterial systems including a giant coronary aneurysm. CASE REPORT: De-identified data were collected retrospectively in case report format. Although our sixteen year old female with Behcet's vasculitis had resolution of many arterial aneurysms, she had persistent venous thrombosis of large vessels, as well as persistent, giant arterial aneurysms requiring intra-arterial coiling of a lumbar artery and coronary bypass grafting despite intensive immunosuppression including glucocorticoids, cyclophosphamide, infliximab, methotrexate, azathioprine and intravenous immunoglobulin. CONCLUSIONS: Vascular manifestations may be seen in Behcet's syndrome, including asymptomatic coronary aneurysm, which may be refractory to immunosuppression and ultimately require surgical intervention. Increased awareness is essential for prompt diagnosis and management.

OBJECTIVE: The purpose of this study was to compare combined cine gradient-recalled echo MRI and MR angiography with conventional angiography in the evaluation of the pulmonary vascular supply in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. MATERIALS AND METHODS: Eleven patients who underwent both MRI and conventional angiography were retrospectively reviewed. Contiguous 2D cine gradient-recalled echo images (TR range/TE, 30-80/4.8; flip angle, 20 degrees or 30 degrees ) and 3D MR angiographic images (TR range/TE range, 3.8-5.0/1.3-2.0; acquisition time, 13-32 sec) using gadopentetate meglumine (0.1-0.2 mmol/kg) were obtained. The presence, size, and course of the pulmonary arteries (main, right, left) and major aortopulmonary collateral arteries (>/= 5 mm) were determined. Presence of minor collateral arteries (< 5 mm) was also noted. Results were compared with findings at conventional angiography. RESULTS: MRI showed all main (n = 4) and branch (n = 17) pulmonary arteries found at conventional angiography and showed the pulmonary confluence in five of six cases. MRI showed all major aortic collaterals (n = 22) with a highly significant correlation between MRI and conventional angiography measurements (r = 0.84, p < 0.001 [95% confidence interval, -0.35 to 0.40]). One coronary artery collateral was not shown on MRI examination. At MRI, 12 of 14 major and four of seven minor brachiocephalic artery collaterals were shown. MRI showed more minor aortic collaterals than angiography (22 vs 18 vessels, respectively). CONCLUSION: Combined cine gradient-recalled echo MRI and MR angiography is a reliable method for imaging pulmonary vascular supply in patients with these disorders. Additional prospective studies comparing MRI and conventional angiography may determine whether routine preoperative conventional angiography is required

Programmed capillary regression occurs during normal development of the eye and serves as a useful model for assessing the forces that drive vascular involution. Using a combination of S-phase labeling and liposome-mediated macrophage elimination, we show that during regression, macrophages induce apoptosis of both pericytes and endothelial cells in a cell cycle stage-dependent manner. Target cells are signaled to die by macrophages approximately 15 hours after S-phase labeling and this corresponds to a point in mid-G1 phase of the cell cycle. The tight correlation between the restriction point of the cell cycle and the point where the macrophage death signal is received suggests that the mitogen, matrix and cytoskeletal signals essential for cell-cycle progression may be inhibited by macrophages as a means of inducing cell death. Furthermore, these experiments show that cells from two distinct lineages are induced to die as a consequence of macrophage action, and this provides evidence that macrophage-induced cell death may be a general phenomenon during development and homeostasis