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Background A right aortic arch is present in 18% of patients with congenital heart disease, and is usually associated with Tetralogy of Fallot or truncus arteriosus. Its incidence in D-TGA ranges from <1% to 12% in the literature. An isolated left subclavian, defined as a left subclavian artery that is disconnected from the aorta and arises from a branch pulmonary artery, is a rare right aortic arch anomaly. It is typically associated with Tetralogy of Fallot and truncus arteriosus, and exceedingly rare in cases of D-TGA. Case A full-term neonate with prenatal diagnosis of D-TGA with VSD was born at our institution. At birth, the baby was vigorous but with low oxygen saturation in the right hand (60s). The baby was started on continuous positive airway pressure at 100% FiO2 as well as a prostaglandin infusion and transferred to the NICU. Our initial echocardiogram confirmed the diagnosis of D-TGA with moderate-size muscular VSD as well as a right-sided aortic arch. On echocardiogram there appeared to be an aberrant left subclavian artery that could not be imaged definitively. The baby was taken to the catheterization lab the next day for balloon atrial septostomy and angiograms to better delineate arch anatomy. Angiography showed a left subclavian artery arising from the left pulmonary artery Decision-making In the case of our patient, the typical practice of measuring pre- and post-ductal saturations in the right and left hands, respectively, did not yield the "classic" picture of reverse differential cyanosis. We opted to monitor pre-ductal saturations on the right earlobe to better monitor saturations. Conclusion It is important to consider variability in arch sidedness and aortic arch anatomy when evaluating a newborn with D-TGA. We recommend checking for a pre-ductal saturation on one of the ears, if feasible, as this data is more representative of the arterial saturation of the cerebral blood vessels. Another important clinical consideration with an isolated subclavian artery with or without TGA is the subclavian steal phenomenon. When pulmonary vascular resistance drops, flow will preferentially go from the systemic circulation into the pulmonary circulation, reducing flow in vertebral arteries.
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Partial anomalous pulmonary venous return (PAPVR) comprises a group of congenital cardiovascular anomalies associated with pulmonary venous flow directly or indirectly into the right atrium. Scimitar syndrome is a variant of PAPVR in which the right lung is drained by right pulmonary veins connected anomalously to the inferior vena cava. Surgery is the definitive treatment for scimitar syndrome. However, it is not always necessary as many patients are asymptomatic, have small left-to-right shunts, and enjoy a normal life expectancy without surgery. We report multimodality imaging in four adults with scimitar syndrome and the implications for management of this rare syndrome.

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.