Interventions on residual lesions in patients with heterotaxy syndrome following orthotropic heart transplantation: A single-center experience.

BACKGROUND: Heterotaxy syndrome (HS) is a defect in lateralization which often results in complex intra and extracardiac abnormalities. Orthotropic heart transplantation (OHT) in HS involves intricate and individualized modifications to surgical technique. Post-OHT outcomes are worse in patients with HS, however, the impact of post-OHT residual lesions has not yet been characterized. METHODS: Patients with HS who underwent OHT at Ann & Robert H. Lurie Children's Hospital of Chicago between January 2012 and June 2023 were identified. Patients were excluded if follow-up data was not available due to follow up at a different institution of early mortality. Pre-OHT clinical data, surgical data, and post-OHT surgical and catheterization data were collected. RESULTS: Two early mortalities were excluded from analysis, leaving 15 patients in the study cohort. Median age at OHT was 3.7 years (range: 0.7-15.4). Nine out of 15 patients were diagnosed with residual lesions requiring intervention at a median of 188 days post transplantation. All interventions on residual lesions occurred via catheterization. Overall mortality rate was 27% (4/15) with all deaths occurring in patients with residual lesions (4/9 patients, 44%). 83% (10/12) of lesions were diagnosed via catheterization, and 83% (10/12) of lesions of occurred in the first year after transplant. CONCLUSIONS: Patients with HS are at high risk for residual lesions after OHT, which may contribute to increased mortality. Comprehensive invasive diagnostics were required to diagnose residual lesions, which were all addressed percutaneously.

Histopathology, mRNA expression profile, and donor-derived cell-free DNA for assessment of rejection in pediatric heart transplantation.

BACKGROUND: The relationship between histopathologic and molecular ("MMDx"®) assessments of endomyocardial biopsy (EMB) and serum donor-derived cell-free DNA (ddcfDNA) in acute rejection (AR) assessment following pediatric heart transplantation (HT) is unknown. METHODS: EMB sent for MMDx and histopathology from November 2021 to September 2022 were reviewed. MMDx and histopathology results were compared. DdcfDNA obtained ≤1 week prior to EMB were compared with histopathology and MMDx results. The discrimination of ddcfDNA for AR was assessed using receiver-operating curves. FINDINGS: In this study, 177 EMBs were obtained for histopathology and MMDx, 101 had time-matched ddcfDNA values. MMDx and Histopathology displayed moderate agreement for T-cell-mediated rejection (TCMR, Kappa = 0.52, p < .001) and antibody-mediated rejection (ABMR, Kappa = 0.41, p < .001). Discordant results occurred in 24% of cases, most often with ABMR. Compared with no AR, ddcfDNA values were elevated in cases of AR diagnosed by both histopathology and MMDx (p < .01 for all). Additionally, ddcfDNA values were elevated in injury patterns on MMDx, even when AR was not present (p = .01). DdcfDNA displayed excellent discrimination (AUC 0.83) for AR by MMDx and/or histopathology. Using a threshold of ≥0.135%, ddcfDNA had a sensitivity of 90%, specificity of 63%, PPV of 52%, and NPV of 94%. CONCLUSIONS: Histopathology and MMDx displayed moderate agreement in diagnosing AR following pediatric HT, with most discrepancies noted in the presence of ABMR. DdcfDNA is elevated with AR, with excellent discrimination and high NPV particularly when utilizing MMDx. A combination of all three tests may be necessary in some cases.

Tricuspid Atresia with Absent Pulmonary Valve with Nearly Discontinuous Branch Pulmonary Arteries.

Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making.

Modifying the trajectory of epicardial leads can substantially reduce MRI-induced RF heating in pediatric patients with a cardiac implantable electronic device at 1.5T.

PURPOSE: After epicardial cardiac implantable electronic devices are implanted in pediatric patients, they become ineligible to receive MRI exams due to an elevated risk of RF heating. We investigated whether simple modifications in the trajectories of epicardial leads could substantially and reliably reduce RF heating during MRI at 1.5 T, with benefits extending to abandoned leads. METHODS: Electromagnetic simulations were performed to assess RF heating of two common 35-cm epicardial lead trajectories exhibiting different degrees of coupling with MRI incident electric fields. Experiments in anthropomorphic phantoms implanted with commercial cardiac implantable electronic devices confirmed the findings. Both electromagnetic simulations and experimental measurements were performed using head-first and feet-first positioning and various landmarks. Transfer function approach was used to assess the performance of suggested modifications in realistic body models. RESULTS: Simulations (head-first, chest landmark) of a 35-cm epicardial lead with a trajectory where the excess length of the lead was looped and placed on the inferior surface of the heart showed an 87-fold reduction in the 0.1 g-averaged specific absorption rate compared with the lead where the excess length was looped on the anterior surface. Repeated experiments with a commercial epicardial device confirmed this. For fully implanted systems following low-specific absorption rate trajectories, there was a 16-fold reduction in the average temperature rise and a 28-fold reduction for abandoned leads. The transfer function method predicted a 7-fold reduction in the RF heating in 336 realistic scenarios. CONCLUSION: Surgical modification of epicardial lead trajectory can substantially reduce RF heating at 1.5 T, with benefits extending to abandoned leads.

Donor-specific antibodies after heart transplantation for Fontan-associated protein-losing enteropathy.

BACKGROUND: Despite ubiquitous exposure to sensitizing events, most Fontan PLE patients have low panel reactive antibodies (PRA). To assess whether they are at risk for donor-specific antibody (DSA) memory response following heart transplantation (HT) when their PLE resolves, DSA profiles, incidence of rejection, and graft outcomes in Fontan recipients with and without PLE were compared. METHODS: Patient characteristics, appearance of newly detected DSA (nDSA), and graft outcomes were compared between patients with and without PLE using Wilcoxon rank-sum and Chi-squared tests. DSA burden was quantified using titers and time to nDSA, incidence of rejection, and graft outcomes were compared using Kaplan-Meier curves and the log-rank test. RESULTS: Characteristics of patients with and without PLE were similar. Lymphocyte and albumin levels were lower in the PLE group, and flow PRA were comparable. Graft failure, CAV, and ACR were similar between the two groups, but AMR occurred more frequently in the PLE group (p = .03). Nearly 50% of PLE patients experienced class II nDSA by 1-year post-HT, compared to 30% of non-PLE patients, but this difference was statistically not significant. Antibody burden did not differ between groups. CONCLUSIONS: In this cohort, PLE was associated with AMR within the first-year post-HT, despite no significant difference in nDSA. Small patient numbers limited statistical comparison of nDSA in this cohort. PLE may be a risk factor for AMR post-HT, and the possibility of a clinically important DSA memory response remains. Larger studies are necessary to better understand these preliminary findings.

Variability in clinical decision-making for ventricular assist device implantation in pediatrics.

BACKGROUND: Minimal data exist on clinical decision-making in VAD implantation in pediatrics. This study aims to identify areas of consensus/variability among pediatric VAD physicians in determining eligibility and factors that guide decision-making. METHODS: An 88-item survey with clinical vignettes was sent to 132 pediatric HT cardiologists and surgeons at 37 centers. Summary statistics are presented for the variables assessed. RESULTS: Total respondents were 65 (72% cardiologists, 28% surgeons) whose centers implanted 1-5 (34%), 6-10 (40%), or >10 (26%) VADs in the past year. Consensus varied by patients' age, diagnosis, and Pedimacs profile. Highest agreement to offer VAD (97%) was a mechanically ventilated teenager with dilated cardiomyopathy. Patients stable on inotropes were less likely offered VAD (11%-25%). SV infant with Pedimacs profile 2 had the most varied responses: 37% offered VAD; estimated survival ranged from 15% to 90%. Variables considered for VAD eligibility included mild developmental delays (100% offered VAD), moderate-severe behavioral concerns (46%), cancer in remission >2 years (100%), active malignancy with good prognosis (68%) or uncertain prognosis (36%), and BMI >35 (74%) or <15 (69%). Most respondents (91%) would consider destination therapy VADs in pediatrics, though not currently feasible at 1/3 of centers. Factors with greatest influence on decision-making included HT candidacy, families' goals of care, and risks of complications. CONCLUSIONS: Significant variation exists among pediatric VAD physicians when determining VAD eligibility and estimating survival, which can lead to differences in access to emerging technologies across institutions. Further work is needed to understand and mitigate these differences.

Bridge to Transplantation With Long-Term Mechanical Assist Devices in Adults With Transposition of the Great Arteries.

Prior to the widespread adoption of the arterial switch operation, patients with transposition of the great arteries (TGA) commonly underwent atrial switch operation (Mustard or Senning). It is not uncommon for these patients to progress to end stage heart failure and increasingly ventricular assist devices (VADs) are used to support these patients as a bridge to transplantation, though there is limited experience with this worldwide. A retrospective review of our institution's VAD database was undertaken and revealed seven adult patients with a history of TGA and subsequent systemic ventricular failure were implanted with a VAD: four of whom received the VAD as a bridge to transplantation (BTT) at the time of implantation, two who were initially designated as destination therapy secondary to severe pulmonary hypertension, and one who was designated as destination therapy secondary to a high risk of life-threatening non-compliance. Seven patient cases who received a VAD for severe systemic ventricular failure were included in this study. The mean age of the patients was 40 years and the majority of patients were male (6/7, 85%). Five of the patients (71.4%) had previously undergone an atrial switch operation and all of these were Mustard procedures. Two of the seven patients (28.5%) had congenitally corrected transposition of the great arteries (CC-TGA). Two of the seven patients (28.5%) had supra-systemic pulmonary pressures before VAD implantation and were designated as destination therapy (DT). One of these patients was later designated as BTT as an improvement in his pulmonary vascular resistance was observed, and subsequently underwent heart transplantation. Because of anatomic considerations, four of the patients (57%) underwent redo-sternotomy with outflow cannula anastomosis to the ascending aorta, one patient underwent VAD implantation via a left subcostal incision with anastomosis of the outflow graft to the descending thoracic aorta, and two patients (28.5%) underwent VAD implantation via a left thoracotomy and anastomosis of the outflow cannula to the descending thoracic aorta. Six of the seven patients had a HeartWare HVAD VAD implanted; one received a Thoratec Heartmate II VAD. Two patients underwent VAD explant and orthotopic heart transplant, 222 days and 444 days after VAD implant, respectively. One patient died on postoperative day 17 after complications from recurrent VAD thrombosis despite multiple pump exchanges. Four patients remain on VAD support, three of these patients are awaiting transplantation at last follow-up (mean days on support, 513 days). Bridge to transplantation with a durable VAD is technically feasible and relatively safe in patients with TGA. Multiple redo-sternotomies can be avoided with a left posterior thoracotomy approach and outflow graft anastomosis to the descending thoracic aorta after careful anatomic considerations.

Successful Bridge-to-Transplant of Functionally Univentricular Patients With a Modified Continuous-Flow Ventricular Assist Device.

A continuous flow extracorporeal ventricular assist device (VAD) was modified to support functionally univentricular infants and children awaiting heart transplantation. A centrifugal VAD, designed to flow from 1.5 to 8 L/min, was used as a bridge-to-transplant in four patients with functionally univentricular circulation. A variable restrictive recirculation shunt permitted lower flow ranges in small patients. In hypoxic patients, an oxygenator was incorporated into the circuit. From 2012 to 2015, the modified VAD was placed in four patients with Glenn physiology. Age ranged from 0.97 to 6.98 years (median = 2.2 yrs). Body surface area ranged from 0.41 to 0.84 m2 (median = 0.54 m2 ). One patient was on extracorporeal membrane oxygenation prior to VAD. A recirculation shunt was used in three patients. Three patients required temporary use of an oxygenator for 4, 10, and 27 days. Median time on the VAD was 32.3 days (range = 23-43 days). A decrease in the cavopulmonary pressure was noted in all patients, as was a fall in the B-type natriuretic peptide. Three patients survived transplant and were discharged at 28-82 days post-transplantation. One patient died after 35 days of support. Two patients experienced major bleeding events. Two patients experienced cerebrovascular accidents, one major and one minor. The centrifugal VAD successfully supported palliated functionally univentricular patients awaiting heart transplantation. The modified recirculation shunt facilitated the successful support of patients in whom optimal flows were substantially lower than that recommended by the manufacturer. The continuous-flow VAD effectively decompressed the cavopulmonary system. The design allowed placement of an in-line oxygenator in hypoxic patients. Further investigation is required to decrease the thromboembolic events, and associated morbidity, in patients supported with this device.

Risk Factors for Cardiac Arrest or Mechanical Circulatory Support in Children with Fulminant Myocarditis.

In children with fulminant myocarditis (FM), we sought to describe presenting characteristics and clinical outcomes, and identify risk factors for cardiac arrest and mechanical circulatory support (MCS). A retrospective review of patients with FM admitted at our institution between January 1, 2004, and June 31, 2015, was performed. We compared characteristics and outcomes of FM patients who received cardiopulmonary resuscitation (CPR) and/or were placed on MCS (CPR/MCS group) to those who did not develop these outcomes (Control group). There were 28 patients who met criteria for FM. Median age was 1.2 years (1 day-17 years). Recovery of myocardial function occurred in 13 patients (46%); 6 (21%) had chronic ventricular dysfunction, 6 (21%) underwent heart transplantation, and 3 (11%) died prior to hospital discharge (including one death following heart transplant). Of the 28 FM patients, 13 (46%) developed cardiac arrest (n = 11) and/or received MCS (n = 8). When compared to controls, patients in the CPR/MCS group had a higher peak b-type natriuretic peptide (BNP) levels (p = 0.03) and peak inotropic scores (p = 0.02). No significant differences were found between groups in demographics; chest radiograph, electrocardiogram, or echocardiogram findings; or initial laboratory values including BNP, troponin, C-reactive protein, lactate, and creatinine (p > 0.05 for all). Children with FM are at high risk of cardiovascular collapse leading to the use of CPR or MCS. Aside from peak BNP levels and inotropic scores, the most presenting characteristics were not helpful for predicting these outcomes. FM patients should ideally receive care in centers that provide emergent MCS.

Shunt Lesions Part I: Patent Ductus Arteriosus, Atrial Septal Defect, Ventricular Septal Defect, and Atrioventricular Septal Defect.

OBJECTIVES: This review summarizes the current understanding of the pathophysiology and perioperative management of patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect. DATA SOURCE: MEDLINE and PubMed. CONCLUSIONS: The four congenital cardiac lesions that are the subject of this review, patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect, are the most commonly found defects causing a left-to-right shunt. These defects frequently warrant transcatheter or surgical intervention. Although the perioperative care is relatively straightforward for many of these patients, there are a number of management strategies and complications associated with each intervention. The treatment outcomes for all of these lesions are very good in the current era.

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.

The aortic uncrossing procedure.

OBJECTIVE: Cross-sectional imaging allows identification of rare patients with a vascular ring and circumflex aorta. The key diagnostic feature is crossing of the transverse aortic arch from right to left posterior to the trachea and superior to the carina in a patient with a right aortic arch. We evaluated our patients who received an aortic uncrossing procedure. METHODS: We reviewed all patients who underwent aortic uncrossing from 2002 to 2022. All patients received preoperative computed tomography imaging and bronchoscopy. RESULTS: Eleven patients ranging in age from 1.5 to 10 years (median 4 years) underwent aortic uncrossing. Two patients had prior left ligamentum division, and 3 patients had prior left aortic arch division. All had significant clinical symptoms. Eight patients had deep hypothermic circulatory arrest (mean 34 minutes), and 3 patients had antegrade cerebral perfusion (median, 28 minutes). Patch material was not used for aortic augmentation, and no patient underwent a posterior tracheopexy or rotational esophagoplasty. Postoperative length of stay ranged from 4 to 31 days (median, 5 days). One patient required a temporary tracheostomy for bilateral recurrent laryngeal nerve paresis, which recovered. One patient required an aortic extension graft to alleviate esophageal compression from an unusual ectatic esophageal course. All patients had relief of airway symptoms and dysphagia. CONCLUSIONS: In properly selected patients with a right aortic arch and circumflex aorta, aortic uncrossing is a safe and effective therapy to treat airway and esophageal compression. The procedure can be conducted with deep hypothermic circulatory arrest or antegrade cerebral perfusion. Careful attention to the location of the esophagus and recurrent laryngeal nerves is required.

Cumulative radiation dose from medical imaging in paediatric congenital heart disease patients with epicardial cardiac implantable electronic devices.

Aims: To determine whether paediatric congenital heart disease (CHD) patients with epicardial cardiac implantable electronic devices (CIEDs) receive high cumulative effective doses (CEDs) of ionizing radiation from medical imaging tests. Methods and results: We compared 28 paediatric CHD patients with epicardial CIEDs (cases) against 40 patients with no CIED matched by age at operation, sex, surgical era, and CHD diagnosis (controls). We performed a retrospective review of radiation exposure from medical imaging exams between 2006 and 2022. Radiation dose from computed tomography (CT) and X-ray radiography was calculated using the National Cancer Institute Radiation Dosimetry Tool. We performed univariate analysis to compare the CED between the two groups. In the case subgroup, we convened experts' review to adjudicate the prevalence of CT exams that should have been performed with magnetic resonance imaging (MRI) in the absence of a CIED. Children (median age 2.5 years at implant) with CIEDs received significantly higher median CED compared with matched controls (6.90 vs. 1.72 mSv, P = 0.0018). In cases, expert adjudication showed that 80% of the CT exams would have been performed with MRI in the absence of a CIED. This resulted, on average, a five-fold increase in the effective dose (ED) from post-lead implant CTs. Conclusion: Paediatric CHD patients with CIED received four times higher CED than matched controls. Improved access to medical imaging tests without ionizing radiation, such as MRI, could potentially reduce the ED in CIED patients by up to five times.

HeartMate 3 VAD in a Single-Ventricle Patient Palliated With a Superior Cavopulmonary Anastomosis.

We describe a 9-year-old, 25-kg child with failing single-ventricle palliation with a superior cavopulmonary anastomosis successfully supported with a HeartMate 3 ventricular assist device (Abbott) as a bridge to transplantation.

Interventricular Septal Hematoma Complicating Ventricular Assist Device Placement in an Infant: Case Report and Review of the Literature.

Interventricular septal hematoma is a rare and life-threatening complication of pediatric cardiac surgery. Commonly seen following ventricular septal defect repair, it has also been associated with ventricular assist device (VAD) placement. Although conservative management is usually successful, operative drainage of interventricular septal hematoma occurring in pediatric patients undergoing VAD implantation should be considered.

Association Between Prenatal Diagnosis and Age at Surgery for Noncritical and Critical Congenital Heart Defects.

BACKGROUND: The relationship between the prenatal diagnosis of congenital heart defects (CHDs) and age at CHD surgery is poorly understood, despite the known relationships between age at surgery and long-term outcomes. The objective of this study was to determine the associations between prenatal diagnosis of CHD and age at surgery, and whether these associations differ for critical and noncritical CHDs. METHODS: This is a cohort analysis of patients aged 0 to 9 years who received their initial cardiac surgery at Ann & Robert H. Lurie Children's Hospital of Chicago between 2015 and 2021 with prenatal diagnosis as the exposure variable. All data were obtained from the locally maintained Society of Thoracic Surgeons Congenital Heart Surgery Database at Lurie Children's Hospital. We used multivariable fixed effects regression models to estimate the strength of the association of prenatal diagnosis with age at surgery among patients with critical (surgery ≤60 days) and noncritical (surgery >60 days) CHDs. RESULTS: Of 1131 individuals who met inclusion criteria, 532 (47%) had a prenatal diagnosis, 428 (38%) had critical CHDs, 533 (47%) were female, and the median age at surgery was 119 days (interquartile range, 11-309 days). After controlling for demographics, comorbidities, and surgical complexity, the mean age at surgery was significantly younger in those with prenatally versus postnatally diagnosed critical CHD (7.1 days sooner, P<0.001) and noncritical CHDs (atrial septal defects [12.4 months sooner, P=0.037], ventricular septal defects [6.0 months sooner, P<0.003], and noncritical coarctation of the aorta [1.8 months sooner, P=0.010]). CONCLUSIONS: Younger age at CHD surgery, which is associated with postsurgical neurodevelopmental and physical outcomes, is significantly associated with prenatal CHD diagnosis. This relationship was identified for both critical and noncritical CHDs.

Expanding use of the HeartMate 3 ventricular assist device in pediatric and adult patients within the Advanced Cardiac Therapies Improving Outcomes Network (ACTION).

BACKGROUND: We report current outcomes in patients supported with the HeartMate 3 (HM3) ventricular assist device in a multicenter learning network. METHODS: The Advanced Cardiac Therapies Improving Outcomes Network database was queried for HM3 implants between 12/2017 and 5/2022. Clinical characteristics, postimplant course, and adverse events were collected. Patients were stratified according to body surface area (BSA) (<1.4 m2, 1.4-1.8 m2, and >1.8 m2) at device implantation. RESULTS: During the study period, 170 patients were implanted with the HM3 at participating network centers, with median age 15.3years; 27.1% were female. Median BSA was 1.68 m2; the smallest patient was 0.73 m2 (17.7 kg). Most (71.8%) had a diagnosis of dilated cardiomyopathy. With a median support time of 102.5days, 61.2% underwent transplantation, 22.9% remained supported on device, 7.6% died, and 2.4% underwent device explantation for recovery; the remainder had transferred to another institution or transitioned to a different device type. The most common adverse events included major bleeding (20.8%) and driveline infection (12.9%); ischemic and hemorrhagic stroke were encountered in 6.5% and 1.2% of patients, respectively. Patients with BSA <1.4 m2 had a higher incidence of infection, renal dysfunction, and ischemic stroke. CONCLUSIONS: In this updated cohort of predominantly pediatric patients supported with the HM3 ventricular assist device, outcomes are excellent with <8% mortality on device. Device-related adverse events including stroke, infection, and renal dysfunction were more commonly seen in smaller patients, highlighting opportunities for improvements in care.

Biventricular Assist Device Support for Intractable Arrhythmias From Histiocytoid Cardiomyopathy.

Histiocytoid cardiomyopathy (HICMP) is a rare mitochondrial cardiomyopathy associated with recurrent life-threatening arrhythmias and variable degrees of systolic dysfunction. Successful heart transplantation for HICMP has been described, but there has been no published experience with biventricular assist device (BiVAD) support for intractable arrhythmias in HICMP. We report a 13 month old girl with left ventricular noncompaction and preserved systolic function who presented in cardiogenic shock secondary to incessant ventricular arrhythmias. After failed attempts at chemical and electrical cardioversion, she underwent BiVAD implantation as bridge to transplantation. Her BiVAD course was complicated by mechanical inflow obstruction during sinus rhythm, necessitating left-sided cannulation revision from an apical to atrial inflow cannula. This maneuver resolved the obstruction and the patient was transitioned to Berlin EXCOR (Berlin Heart Inc, The Woodlands, TX) BiVADs. On Berlin pumps, she had intermittent pauses (no fill/no eject) while in sinus rhythm, felt to be due to competition from intrinsic ejection. Despite these pauses, the patient experienced an uneventful remainder of her BiVAD course (205 days total) with minimal fibrin deposition and no device-related complications. BiVAD can support pediatric patients with hemodynamically significant arrhythmias to transplantation. Atrial cannulation strategy may be preferred in cases of preserved systolic function, ventricular noncompaction, and frequent rhythm changes.

Influence of Fetal Diagnosis on Management of Vascular Rings.

BACKGROUND: Fetal diagnoses of vascular rings have been increasing. We compared management strategies and outcomes of infants with fetal diagnosis with those with postnatal diagnosis to inform recommendations regarding optimal management. METHODS: A retrospective review was performed of vascular ring operations from January 2000 to June 2019. Standard demographic data (preoperative clinical status, timing of diagnosis, cross-sectional imaging, operative and perioperative details, and clinical outcomes) were collected. Statistical analysis was performed to compare characteristics and outcomes of fetal versus postnatal diagnosis. RESULTS: Of 190 patients, 15% (n = 29) were diagnosed prenatally. Anatomic variants were double aortic arch (n = 66, 14 fetal diagnoses), right aortic arch, aberrant left subclavian artery (n = 94, 12 fetal diagnoses), circumflex aorta (n = 7, 1 fetal diagnosis), and pulmonary artery sling (n = 19, 2 fetal diagnoses). An increasing frequency of fetal diagnoses has been noted in the past 10 years. In 2012 1 of 9 patients (11%) had a fetal diagnosis, whereas in 2018 8 of 11 (72%) had a fetal diagnosis (P < .001). Patients with a fetal diagnosis were significantly younger at the time of surgery (13.1 months [interquartile range (IQR), 20.6] vs 24.0 months [IQR, 87.0], P = .029). There was no difference in postoperative complications or length of stay (3 days [IQR, 1] for fetal diagnoses vs 4 days [IQR, 3] for postnatal diagnoses, P = .50). CONCLUSIONS: Fetal diagnosis leads to the potential for expectant management of vascular ring patients. This has resulted in earlier time of intervention with no increase in postoperative morbidity. This may lead to improved long-term outcomes and potentially alter the natural history for these children.