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Van Praagh (VP) A3 variant of truncus arteriosus (or common arterial trunk) is defined by only one pulmonary artery (usually the right) originating from the common trunk, while the other lung is supplied either by collaterals or a pulmonary artery arising from the aortic arch. This report describes a staged approach to manage a VP-A3 variant truncus arteriosus with ductal origin of the left pulmonary artery (LPA), a hypoplastic right pulmonary artery, and cyanosis. Initially, the ductal portion of the proximal LPA was stented with a Resolute Onyx drug-eluting stent. The pulmonary arteries grew and at four months of age had an acceptable McGoon ratio and Nakata index. The patient then underwent repair which included unifocalization of the branch pulmonary arteries, closure of the ventricular septal defect, and placement of a right ventricle-to-pulmonary artery homograft conduit.

Graphical abstract, Highlights • Abnormal right coronary artery origin and course are demonstrated by FE. • Color flow Doppler is essential for visualizing fetal coronary artery anatomy. • Color-compare images help identify coronary artery origin and course.

Objective We sought to examine the concordance of prenatally diagnosed congenital cardiac disease with postnatal echocardiography. Study design Patients who underwent fetal echocardiograms performed by pediatric cardiologists at a single referral center from January to December 2014 were reviewed. Fetal echocardiography exams were performed in accordance with guidelines by the American Institute of Ultrasound in Medicine (AIUM) and the American Heart Association (AHA) guidelines (2013 and 2014, respectively). The concordance of prenatal diagnosis was compared to postnatal echocardiograms. Result One hundred and six patients were included. Overall, the prenatal diagnosis precisely matched the postnatal diagnosis in 69.8%, minor discrepancies were seen in 14.2% and major differences were seen in 16% of cases. Three cases with major differences resulted in a more guarded postnatal prognosis; all three were fetuses with complex disease where the additional finding of total anomalous pulmonary venous return (TAPVR) was missed. Conclusion Fetal echocardiograms performed in our specialized fetal cardiology program are in high agreement with postnatal diagnosis of congenital cardiac disease. A worse postnatal prognosis is uncommon. Emphasis on pulmonary venous imaging in complex congenital heart disease is vital.

National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13–20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.

Kawasaki disease (KD) is the leading cause of acquired coronary disease in children and may lead to subsequent myocardial ischemia and infarction. Because coronary computed tomographic angiography (CTA) is the most sensitive noninvasive test in patients with atherosclerosis, the aim of this study was to retrospectively evaluate coronary CTA performed in patients with KD for aneurysm, stenosis, and calcified and noncalcified coronary artery disease (CAD). Clinical histories and prior stress and imaging test results were reviewed. Thirty-two patients underwent coronary CTA for KD, and 385 coronary segments were evaluated. Twenty-three of 32 patients had ≥1 diseased coronary segment. There were 20 aneurysms, 7 lesions, and 75 segments (20%) with nonobstructive CAD (16% noncalcified, 2% calcified, and 2% mixed). All nonobstructive and obstructive CAD was in patients with histories of acute-phase coronary artery dilatation or aneurysm (echocardiographic z score 4 to 44), and were almost always associated with normal stress imaging test results on follow-up. No lesion or CAD was found in coronary computed tomographic angiographic studies performed in a control group referred for other indications (n = 32, 422 segments evaluated). The median coronary computed tomographic angiographic dose-length product was 59 mGy cm (interquartile range 32 to 131), the median unadjusted radiation dose was 0.8 mSv (interquartile range 0.4 to 1.8), and the median age- and size-adjusted radiation dose was 1.3 mSv (interquartile range 0.7 to 2.3). In conclusion, high-risk patients with histories of KD had nonobstructive and obstructive CAD not visualized by other noninvasive imaging tests. In properly selected high-risk patients with KD, coronary CTA may identify a subset at increased risk for future coronary pathology who may benefit from medical therapy.

Since its first English language description by Dr. Tomisaku Kawasaki in 1974, Kawasaki disease (KD) has become the leading cause of acquired heart disease among children in the United States [1] . The pathogenesis of KD involves a marked immune response to an unknown stimulus, resulting in high levels of cytokines, endothelial cell activation, and arteritis of the coronary arteries and occasionally other medium-sized muscular arteries. Patients in whom coronary ectasia, dilation, or aneurysm occur are at risk for coronary artery thrombosis, angina, myocardial infarction, and sudden death. Early in the illness, therapy is designed to reduce immune perturbations and severity of vasculitis. Ultimately, the aim of therapy is reducing the incidence of coronary sequelae. Aspirin therapy is initiated in the acute phase of KD at a dose of 80–100 mg/kg/day, divided into four daily doses. Many centers use high-dose aspirin only until the patient has been afebrile for 48 h, after which the dose is decreased to 3–5 mg/kg daily. Intravenous immune globulin (IVIG) has been shown to decrease the incidence of coronary artery aneurysms (CAA). A single high-dose (2 g/kg) is given at time of diagnosis and repeated if the patient is still febrile 36 h after initial infusion. There is a suggestion that pulsed-dose methylprednisolone, in addition to aspirin and IVIG as primary therapy results in faster resolution of fever, more rapid improvement in inflammatory markers, and shorter length of hospitalization. The effect, if any, on coronary artery abnormalities is not well established. A number of other therapies are also discussed in this review including plasma exchange, serine protease inhibitors, pentoxifylline, cytotoxic agents, tumor necrosis factor, and platelet glycoprotein IIb/IIIa.

Background— Coronary artery (CA) dilatation on echocardiography is a criterion for treatment with intravenous immunoglobulin for incomplete Kawasaki disease (KD). However, CA dimensions for febrile children are unknown. We compared CA dimensions in children with febrile illnesses other than KD to those of normal afebrile children and to KD patients. Methods and Results— We performed echocardiograms in 43 patients who met the following inclusion criteria: (1) age 3 months to 18 years, (2) daily fever >38°C for ≥96 hours, and (3) a diagnosis other than KD. These subjects had mean CA z scores greater than normative values (left main CA=0.66±0.75, P P =0.03; left anterior descending CA=0.35±1.0, P =0.03). Maximum CA z score >2 was found in 2 subjects (osteomyelitis, Mycoplasma pneumonia). Among demographic and laboratory measures, only higher platelet count was associated with greater left anterior descending CA z scores ( P =0.004) and maximum CA z score ( P =0.03). Non-KD febrile subjects, compared with 144 KD patients, had smaller CA z scores ( P =0.04, P P P z score cutoff of 2.0 had specificity of 95% (95% confidence interval, 84%–99%) and sensitivity of 32% (95% confidence interval, 25%–41%) in distinguishing non-KD febrile from KD patients; for maximum CA z score of 2.5, specificity was 98% and sensitivity was 20%. Conclusions— This pilot study found that mean CA dimensions in children with non-KD febrile illnesses are larger than those in normative afebrile subjects but smaller than dimensions in patients with KD. Future studies should augment the available data on CA dimensions in children with more severe febrile illnesses.

The arterial switch operation is the surgical correction of choice for patients born with d-transposition of the great arteries (d-TGA) and an intact ventricular septum. However, prematurity and very low birth weight present both technical and physiologic challenges to this approach. Furthermore, in the setting of d-TGA and an intact ventricular septum, delaying intervention results in deconditioning of the left ventricle, rendering the patient a poor candidate for the arterial switch operation. The report presents an infant born at 27 weeks gestation weighing 1.01 kg who as a newborn underwent a successful urgent balloon atrial septostomy, pulmonary artery banding, and a central shunt on day of life (DOL) 82 and the arterial switch operation on DOL 93.

We report the successful long-term use of a left ventricular assist device (Berlin EXCOR) as a bridge to recovery in a patient with fulminant parvovirus B19 myocarditis. The use of this device allowed time for myocardial recovery, avoiding the need for cardiac transplantation.

OBJECTIVE: To assess parental knowledge regarding lifelong congenital cardiac care (LLCCC). BACKGROUND: National guidelines recommend that nearly 50% of adult survivors with congenital heart disease (CHD) receive LLCCC; the number of adults who receive such care seems far less. Inadequate parental knowledge of LLCCC might contribute to care interruption. METHODS: In this multicenter study, we administered a questionnaire to parents of children with moderate and complex CHD to assess knowledge of LLCCC. RESULTS: A total of 500 parents participated; the median age of their children was 10 years (range: 2–18 years). Most parents (81%) understood that their child would need LLCCC, but only 44% recognized that their child's cardiology care should be guided by an adult congenital heart specialist in adulthood. More than half (59%) of the parents stated that their current cardiology team had never spoken to them about LLCCC, but 96% wished to learn more. Variables associated with parental LLCCC knowledge included previous discussions regarding LLCCC, underlying cardiac surgical diagnosis, and level of parental education. CONCLUSIONS: A substantial number of parents of children with moderate and complex CHD lack knowledge about LLCCC, but almost all of them have a desire to learn more about the care their child will need as an adult.