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Graphical abstract, Highlights Anomalous left common carotid artery is a unique and rare abnormality. Most aortic arch anomalies can be explained by the totipotential aortic arch. Rare anomalies may not fit traditional arch regression models. Echocardiography is key for arch anomaly diagnosis and monitoring. Identification of this very rare anomaly carries significant clinical implications.

A case is presented of a 10-year-old girl with refractory leukemia with poor prognosis and chemotherapy-induced heart failure. She is evaluated for a ventricular assist device (VAD), but the pediatric heart failure team views VAD as clinically inappropriate due to her active oncologic problems. This article examines ethical concerns that arise in deciding whether to offer and use this technology.

Purpose We sought to evaluate the association between early-onset cardiac allograft vasculopathy (CAV) [ Methods Data from the Pediatric Heart Transplant Society (PHTS) for pediatric ( Results Over a median follow-up period of 4.1 (IQR 1.3 - 8.3) years, angiographic evidence of CAV was identified in 17% (885/5,075) of patients, 28% (252/885) of which were early-onset CAV. Compared to those who developed CAV >10 years post HT, patients with early-onset CAV were older at transplant (8.3 +/- 6.2 vs. 3.8 +/- 4.8 years, p Conclusion Following diagnosis, the progression of CAV & graft survival was similar for patients with early-onset CAV compared with CAV at other time points. These findings suggest that early-onset CAV does not necessarily represent a more aggressive phenotype of disease.

This article considers complexities of shared decision making in pediatric heart transplantation and suggests that decisions about pediatric heart transplantation should be shared between a clinical team and parents. This article also considers goals of shared decision making involving Public Health Service increased-risk donors and recommends policy changes to strengthen decision sharing.

BACKGROUND: The clinical spectrum of pediatric acute myocarditis ranges from minimal symptoms with intact hemodynamics to rapid cardiovascular collapse and death. We sought to identify factors on initial presentation associated with subsequent hemodynamic compromise. METHODS: We performed a retrospective cohort study of patients with acute myocarditis at a freestanding pediatric hospital from 2007 to 2016. We defined 2 cohorts: high-acuity patients with hemodynamic compromise defined as requiring inotropic or vasoactive medications, cardiopulmonary resuscitation, extracorporeal membrane oxygenation, ventricular assist devices, or transplant or who died and low-acuity patients without these interventions. We collected the first recorded set of vital signs, symptoms, laboratory values, and chest radiograph, electrocardiogram, and echocardiography results. Univariate analysis was performed, and 2 multivariable logistic regression models were created to discriminate between cohorts. RESULTS: A total of 74 patients were included: 33 high acuity and 41 low acuity. There were significant differences in demographics, symptoms, and physical examination, laboratory, electrocardiogram, and echocardiography findings between high- and low-acuity cohorts. Multivariable logistic regression models were highly discriminate in predicting those in the high-acuity cohort. The first model included presence of tachycardia, tachypnea, creatinine, and cardiomegaly on chest radiograph (area under the curve = 0.913). The second model added the presence of pericardial effusion to the above variables (area under the curve = 0.964). CONCLUSIONS: Models based on factors available at initial presentation with acute myocarditis are predictive of subsequent hemodynamic compromise. If our results can be validated in a multicenter study, these models may help disposition patients with suspected acute myocarditis (with those who meet model criteria being admitted to centers capable of rapidly providing extracorporeal membrane oxygenation, ventricular assist devices, and heart transplant evaluation).

Minimal data exists on clinical practice and decision-making in the use of ventricular assist devices (VADs) in pediatrics. This study aims to identify areas of consensus/dissension among pediatric VAD providers in determining eligibility and factors that guide decision-making. An 88-item survey

The association of cardiorenal syndrome (CRS) with mortality in children with dilated cardiomyopathy (DCM) is unknown.With a modified Schwartz formula, we estimated glomerular filtration rates (eGFR) for children ≥1 year of age with DCM enrolled in the Pediatric Cardiomyopathy Registry at the time of DCM diagnosis and annually thereafter. CRS was defined as an eGFR of90 mL/min/1.73 m(2). Children with and without CRS were compared on survival and serum creatinine concentrations (SCr). The association between eGFR and echocardiographic measures was assessed with linear mixed-effects regression models.Of 285 eligible children with DCM diagnosed at ≥1 year of age, 93 were evaluable. CRS was identified in 57 of these 93 children (61.3%). Mean (standard deviation) eGFR was 62.0 (22.6) mL/min/1.73 m(2) for children with CRS and 108.0 (14.0) for those without (P 0.001); median SCr concentrations were 0.9 and 0.5 mg/dL, respectively (P 0.001). The mortality hazard ratio of children with CRS versus those with no CRS was 2.4 (95% confidence interval 0.8-7.4). eGFR was positively correlated with measures of left ventricular function and negatively correlated with age.CRS in children newly diagnosed with DCM may be associated with higher 5-year mortality. Children with DCM, especially those with impaired left ventricular function, should be monitored for renal disease.

Background For neonates with atrioventricular septal defect and aortic arch obstruction including coarctation of the aorta, we sought to determine whether a difference in outcomes exists after a primary neonatal versus staged surgical repair (neonatal arch repair with delayed intracardiac repair). Methods This retrospective cohort study included consecutive neonates with atrioventricular septal defect and aortic arch obstruction who underwent cardiac surgery before 28 days of age at six centers from 1990 to 2009. Characteristics and outcomes between patients undergoing neonatal versus staged repair were compared. Results Of 66 study patients, 31 (47%) underwent primary neonatal repair and 35 (53%) underwent staged repair. At baseline echocardiogram, a greater percentage of neonatal repair patients had relative unbalanced ventricular size (56% versus 35%, p = 0.02). There were no other differences in demographic characteristics, cardiac anatomical or functional details, or surgical technique. Those undergoing neonatal repair tended to be more likely to have at least moderate left atrioventricular valve regurgitation early after repair (42% versus 19%, p = 0.05) and to have at least one major in-hospital complication (42% versus 20%, p = 0.06). After the initial cardiac operation, compared with the neonatal repair group, patients undergoing staged repair had greater survival (87% versus 57% at 6 years, log-rank p = 0.02) and freedom from the first unplanned cardiac reoperation (69% versus 45% at 6 years, log-rank p = 0.005). Conclusions For neonates with atrioventricular septal defect and aortic arch obstruction, when compared with neonatal repair, a staged approach was associated with improved survival and lower morbidity.

Background A continuous-flow "adult" ventricular assist device (VAD) was modified to support infants and children waiting for heart transplantation. Methods A centrifugal VAD, designed to flow at 1.5 to 8 L/min, was used as a bridge to transplantation in pediatric patients. In smaller children and infants, a modified recirculation shunt permitted lower flow ranges. In hypoxic patients, an oxygenator was spliced into the circuit. Results From 2010 to 2015, the VAD was placed in 13 consecutive patients. Age ranged from 0.9 to 16 years (median, 7 years). Body surface area (BSA) ranged from 0.4 to 2.1 m 2 (median, 0.8 m 2 ). Ten patients had a BSA less than 1.0 m 2 . Four patients were receiving extracorporeal membrane oxygenation (ECMO) before VAD. Three patients had single-ventricle physiology. Five patients had a recirculation shunt and 3 underwent insertion of an oxygenator. Median time on the VAD was 20 days (range, 2–140 days). In patients with a recirculation shunt, mean patient flow was 1.5 L/min (mean flow/BSA, 2.7 L/min/m 2 ), with mean total VAD flow of 3.4 L/min. Twelve patients underwent transplantation, and 1 patient underwent VAD explantation. All patients survived and were discharged at a median of 26 days (range, 17–83 days) after transplantation. Three patients experienced major bleeding events. There were 2 cerebrovascular accidents. VAD mortality dropped from 33% (3 of 9) during 2007 to 2010 to 0% (0 of 13) between 2011 and 2015 ( p = 0.05). Wait-list mortality dropped from 10% (5 of 52) to 4% (4 of 91) for these periods ( p = 0.29). Conclusions The centrifugal VAD successfully supported pediatric patients awaiting heart transplantation. The modified recirculation shunt facilitated the successful support of patients in whom optimal flows were substantially lower than those recommended by the manufacturer. The design allows placement of an in-line oxygenator. Compared with pulsatile devices, use of this VAD was associated with a trend toward decreased mortality associated with VAD use.

Background Surgical techniques for repair of supravalvular aortic stenosis (SVAS) include McGoon's one-patch, Doty's two-patch, and Brom's three-patch method. In this review we evaluated mid-term clinical outcomes of these techniques at our institution. Methods Our cardiac surgery database identified patients with SVAS repair from 1990 to 2008. Follow-up records, reintervention and reoperation data, and most recent echocardiograms were obtained. Results From 1990 to 2008, 20 patients (70% male) underwent surgery for SVAS. Mean age was 3.6 ± 5.6 years. In chronological sequence, 8 patients had single-patch aortoplasty, 4 had the Doty procedure, and 8 received Brom's symmetric three-patch aortoplasty. Of the Brom patients, 6 had Williams syndrome. Aortic cross-clamp times were 40.1 ± 13.6 minutes (one-patch), 60.3 ± 38.8 minutes (Doty), and 104 ± 20.5 minutes (Brom). Perioperative mortality was 5.0% (1 patient in one-patch group). Mean postoperative length of stay was 10 ± 10.6 days. Follow-up data were available for all survivors (mean follow-up, 6.3 ± 6.0 years; range, 6 months to 16 years). There were no late deaths. Follow-up echocardiograms revealed a peak Doppler gradient across the aortic outflow tract of 33 ± 18.0 mm Hg (one-patch), 10 ± 1 mm Hg (Doty), and 18 ± 12 mm Hg (Brom). All patients in the Doty and Brom groups had less than moderate aortic insufficiency. Reoperations were required in 5 of 8 one-patch patients (62%) for residual aortic stenosis (n = 3), aortic insufficiency (n = 1), and subvalvar stenosis (n = 1). No Doty or Brom patient has required aortic reoperations, which was nearly statistically associated with freedom from reoperation ( p = 0.06). Subvalvar stenosis was the only risk factor associated with reoperation ( p = 0.0028). Conclusions Despite a longer cross-clamp time, SVAS repair by Doty or Brom aortoplasty restores normal hemodynamics and reduces the need for reoperation when compared with the classic one-patch technique. Our current preference for SVAS repair is the Brom three-patch symmetric aortoplasty.

Background Patients with failing Fontan circulation are at high risk for complications after heart transplantation (HTx) because of multiple prior operations, elevated panel reactive antibody, hepatic dysfunction, coagulopathy, protein-losing enteropathy (PLE), and poor nutrition. The purpose of this review was to evaluate the outcome of HTx for these patients, including those who are status post-Fontan conversion. Methods Of 206 heart transplants at Ann & Robert H. Lurie Children's Hospital of Chicago from 1990 to 2012, 22 patients had a failing Fontan. Median age at HTx was 12.2 years, median interval from initial Fontan to HTx was 7.1 years. Potential preoperative risk factors included PLE (n = 15), mechanical ventilation (n = 8), prior Fontan conversion (n = 7), renal failure (n = 3), and plastic bronchitis (n = 2) Median number of prior operations was 3. Donor branch pulmonary arteries were used in 17 patients. Results There were 5 early deaths (23%), due to graft failure (1), pulmonary hypertension (1), and infection (3). There were 3 late deaths (13%) at 1, 5, and 8 years. Two of 3 patients with preoperative renal failure died. Survivors who had preoperative PLE (n = 11) and preoperative plastic bronchitis (n = 2) experienced complete resolution of these pathological conditions after heart transplantation. Median length of stay was 30 days. Five of 7 Fontan conversion patients survived, and 6 of 8 preoperative ventilator-dependent patients survived. One-, 5-, and 10-year survival was 77%, 66%, and 45%, respectively. Conclusions The operative mortality of HTx for patients with a failing Fontan is high. Using the donor branch pulmonary arteries greatly facilitated the transplant. Because infection caused the majority of early deaths, lower intensity initial immunosuppression may be warranted. Transplantation was successful in treating PLE in all survivors. Prior Fontan conversion was not a risk factor. Preoperative mechanical ventilation was not a risk factor. Preoperative renal failure may be a relative contraindication. Earlier referral of failing Fontan patients may improve results.

Pseudoaneurysm formation is a dangerous, but uncommon, complication after the Ross procedure. We describe a patient in whom two pseudoaneurysms formed at the proximal suture line of the pulmonary autograft. These were successfully treated percutaneously, one by placement of an Amplatzer Septal Occluder (AGA Medical, Golden Valley, MN), and the other with coil embolization.

A 4-year-old female with hypoplastic left heart syndrome and Fontan palliation presented with severe neurologic impairment from thrombosis of the superior vena cava (SVC). She underwent successful SVC thrombectomy with the X-SIZER® Thrombectomy Catheter System, followed by balloon angioplasty. She demonstrated rapid improvement in her neurologic deficits after the procedure. This represents the first published use of the X-SIZER in a child and its first published use for SVC thrombectomy. © 2006 Wiley-Liss, Inc.

Infants with hypoplastic left heart syndrome (HLHS) and restrictive or intact atrial septum (rAS) present with cyanosis, pulmonary edema, and are critically ill. A previous report from our institution on emergent Norwood for HLHS with rAS showed 10% survival. We hypothesized that transcatheter left atrial (LA) decompression in HLHS with rAS would safely and effectively relieve LA hypertension, improve oxygenation, and improve Norwood survival. Between 1996 and 2004, 30 patients with HLHS and rAS underwent cardiac catheterization for pre-Norwood intervention. Twenty-eight atrial septostomies were performed: 23 static balloon dilations, 4 Rashkind septostomies, and 1 intra-atrial stent. Two procedures were aborted due to perforation (n = 1) or inability to enter the LA (n = 1). Eight total patients required surgical septectomy, for a failure rate of 27%. There were no catheter-related mortalities, although two patients died within 36 hr of the procedure after surgical septectomy. Major complications occurred in three patients (10%)—atrial perforations requiring intervention. Mean atrial septal defect gradient fell from 16.7 ± 4.9 to 6.3 ± 3.4 mm Hg (P < 0.001; n = 18). Mean LA pressure dropped from 21.8 ± 5.5 to 13.1 ± 6.5 mm Hg (P < 0.001; n = 16). Mean PaO2 rose from 29.5 ± 9.1 to 36.5 ± 5.1 torr (P < 0.001; n = 23). Seventeen of 30 patients (57%) survived to discharge from Norwood. Thirteen have undergone hemi-Fontan and nine Fontan. Sixteen of 22 successful decompressions (73%) survived to discharge. Transcatheter decompression of the LA for patients with HLHS and rAS can be performed safely, reduces the transatrial gradient, and improves oxygenation. Catheter intervention improves survival compared to historical controls undergoing emergent Norwood. © 2006 Wiley-Liss, Inc.

ObjectiveSurgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome.MethodsOur cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990–2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0–4 years), and the median weight was 3.5 kg (range, 1.3–15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy.ResultsThere were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P

To the Editor: Plastic bronchitis (PB) is a rare condition characterized by formation of airway casts that obstruct the bronchial tree and cause respiratory failure. In the congenital cardiac population, PB occurs mostly in patients with single ventricle (SV) defects undergoing Fontan palliation.