Managing Bilateral Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy.

BACKGROUND: We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. METHODS: We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair. RESULTS: Median age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11). CONCLUSIONS: Successful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.

Long-Term Outcomes of Ascending Sliding Arch Aortoplasty.

Background: Coarctation of the aorta can be associated with significant hypoplasia of the aortic arch. In contrast to patch aortoplasty, ascending sliding arch aortoplasty uses viable autologous tissue for potential growth in children. We reviewed the mid- to long-term outcomes of this technique. Methods: Between 2002 and 2023, 28 patients underwent ascending sliding arch aortoplasty for the patients with coarctation of the aorta (n = 22) and interrupted aortic arch (n = 2). Four patients underwent previous surgical coarctation repair at other institutions. The median patient age and body weight were 28.5 months (3 weeks to 15.6 years) and 13.4 kg (3.7-70 kg), respectively. Results: Although one patient had a recurrent nerve injury postoperatively, there were no other major morbidities or mortalities. The last follow-up echocardiography demonstrated that the mean peak velocity improved from 3.9 ± 0.6 to 0.9 ± 0.8 m/s, and the pressure gradient improved from 63.6 ± 21.5 to 7.1 ± 7.7 mm Hg. The postoperative diameters of the ascending aorta, proximal arch, distal arch, and isthmus all increased significantly. The mean postoperative length of stay was 5.9 ± 2.1 days, and the median follow-up time was 7.3 years (10 days to 20.5 years). No reoperation or catheterization-based intervention was performed for residual coarctation. Conclusions: Ascending sliding arch aortoplasty is safe and effective for treating coarctation of the aorta with aortic arch hypoplasia. This technique is applicable for children ranging in size from neonates to older children (or adolescents), recurrent coarctation cases, and provides complete relief of narrowing by utilizing viable native aortic tissue.

Use of Berlin EXCOR cannulas in both venovenous and venoarterial central extracorporeal membrane oxygenation configurations overcomes the problem of cannula instability while bridging infants and young children to lung transplant.

Objectives: Infants and young children awaiting lung transplantation present challenges that often preclude successful extracorporeal membrane oxygenation support as a bridge to transplantation. Instability of neck cannulas often results in the need for intubation, mechanical ventilation, and muscle relaxation creating a worse transplant candidate. With the use of Berlin Heart EXCOR cannulas (Berlin Heart, Inc) in both venoarterial and venovenous central cannulation configurations, 5 pediatric patients were successfully bridged to lung transplant. Methods: We performed a single-center retrospective case review of central extracorporeal membrane oxygenation cannulation used as a bridge to lung transplantation cases performed at Texas Children's Hospital between 2019 and 2021. Results: Six patients, 2 with pulmonary veno-occlusive disease (15-month-old male and 8-month-old male), 1 with ABCA3 mutation (2-month-old female), 1 with surfactant protein B deficiency (2-month-old female), 1 with pulmonary arterial hypertension in the setting of D-transposition of the great arteries after repair as a neonate (13-year-old male), and 1 with cystic fibrosis and end-stage lung disease, were supported for a median of 56.3 days on extracorporeal membrane oxygenation while awaiting transplantation. All patients were extubated after initiation of extracorporeal membrane oxygenation, participating in rehabilitation until transplant. No complications due to central cannulation and use of the Berlin Heart EXCOR cannulas were observed. One patient with cystic fibrosis developed fungal mediastinitis and osteomyelitis resulting in discontinuation of mechanical support and death. Conclusions: Novel use of Berlin Heart EXCOR cannulas for central cannulation eliminates the problem of cannula instability allowing extubation, rehabilitation, and bridge to lung transplant for infants and young children.

Improved Outcomes for Infants and Young Children Undergoing Lung Transplantation at Three Years of Age and Younger.

Rationale: Since its inception, older children and adolescents have predominated in pediatric lung transplantation. Most pediatric lung transplant programs around the world have transplanted few infants and young children. Early mortality after lung transplantation and inadequate donor organs have been perceived as limitations for success in lung transplantation at this age. Objectives: Our aim was to describe our experience in a large pediatric lung transplant program with respect to lung transplantation in infants and young children, focusing on diagnosis, waitlist, and mortality. Methods: We performed a retrospective review of infants and young children under 3 years of age at the time of transplant in our program from 2002 through 2020. Results: The patient cohort represented a severely morbid recipient group, with the majority hospitalized in the intensive care unit on mechanical ventilation just before transplantation. There was a marked heterogeneity of diagnoses distinct from diagnoses in an older cohort. Waitlist time was shorter than in older age cohorts. There was a decrease in early mortality, lower incidence of allograft rejection, and satisfactory long-term survival in this age group compared with the older cohort and published experience. Severe viral infection was an important cause of early mortality after transplant. Nonetheless, survival is comparable to older patients, with better enduring survival in those who survive the early transplant period in more recent years. Conclusions: Carefully selected infants and young children with end-stage lung and pulmonary vascular disease are appropriate candidates for lung transplantation and are likely underserved by current clinical practice.

Decellularized vs Non-decellularized Allogeneic Pulmonary Artery Patches for Pulmonary Arterioplasty.

We studied pulmonary artery size, reinterventions, and panel reactive antibodies in patients with single-ventricle physiology who underwent a pulmonary arterioplasty with decellularized (DAPAP) and non-decellularized allogeneic pulmonary artery patches (non-DAPAP). Retrospective review identified 59 patients with single-ventricle physiology who underwent pulmonary arterioplasty from 2008 to 2017: 28 patients underwent arterioplasty with DAPAP and 31 patients with non-DAPAP. Demographic and operative variables were similar between groups. Among patients who underwent a Norwood procedure, a right ventricle to pulmonary artery shunt was more commonly used in the DAPAP group (12/20, 60%) and a modified Blalock-Taussig shunt was more commonly used in the non-DAPAP group (17/22, 77%). On multivariable analysis, the use of DAPAP was associated with higher pre-Fontan angiography Z-scores in right (estimate = 0.17, standard error = 0.04, P = 0.0005) and left pulmonary arteries (estimate = 0.12, standard error = 0.05, P = 0.01). No areas of calcification, discrete coarctation, or pulmonary dilation were noted in any of the pulmonary arteries. On multivariable analysis, the use of DAPAP was associated with higher freedom from pulmonary artery reinterventions (Hazard ratio = 0.36, 95% confidence interval = 0.13-0.9, P = 0.04). The median value for Class I panel reactive antibodies was 0% (IQR 0, 4) in the DAPAP and 23% (IQR 14, 36) in the non-DAPAP group. The median value for Class II panel reactive antibodies was 15% (IQR 0, 17) in the DAPAP and 21% (IQR 10, 22) in the non-DAPAP group. Pulmonary arterioplasty with DAPAP was associated with higher pre-Fontan pulmonary artery Z-scores and higher freedom from pulmonary artery reinterventions.

Right Ventricle to Pulmonary Artery Conduit Size Is Associated with Conduit and Pulmonary Artery Reinterventions After Truncus Arteriosus Repair.

We studied conduit-related risk factors for mortality, conduit reintervention, conduit replacement, and pulmonary artery (PA) reinterventions after truncus repair. Patients who underwent truncus repair at our institution between 1995 and 2019 were studied. Cox proportional hazards modeling evaluated variables for association with mortality, time to conduit reintervention, time to conduit replacement, and time to PA reintervention. Truncus was repaired in 107 patients at median age of 17 days (IQR 9-45). Median follow-up time was 7 years. Aortic homografts were implanted in 57 (53%) patients, pulmonary homograft in 40 (37%), and bovine jugular conduit in 10 (9%). Median conduit size was 11 mm (IQR 10-12) and median conduit Z-score was 1.71 (IQR 1.08-2.34). At 5 years, there was 87% survival, 21% freedom from conduit reinterventions, 37% freedom from conduit replacements, and 55% freedom from PA reinterventions. Conduit size (HR 0.7, 95%CI 0.4-1.4, p=.41) and type (aortic homograft reference; bovine jugular vein graft HR 0.6, 95% CI 0.08-5.2, p=.69; pulmonary homograft HR 0.7, 95% CI 0.2-2.3, p=.58) were not associated with mortality. On multivariate analysis, the hazard for conduit reintervention, conduit replacement, and PA reintervention decreased with increasing conduit Z-score values of 1 to 2.5 (non-linear relationship, p<.01), with little additional reduction in hazard beyond this range. Implantation of a larger conduit within Z-score values of 1 and 2.5 is associated with a decreased hazard for conduit reintervention, conduit replacement, and PA reintervention after truncus repair. The type and size of the conduits did not impact mortality.

Outcomes in anomalous aortic origin of a coronary artery after surgical reimplantation.

OBJECTIVE: Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We compared outcomes data of patients who underwent transection and reimplantation (TAR) and patients who underwent an unroofing. METHODS: Patients who presented to the Coronary Artery Anomalies Program were evaluated and managed following a standardized approach. Anatomy was determined using computed tomography angiography, myocardial perfusion using advanced stress imaging, and surgical intervention according to anatomic features. RESULTS: Sixty-one patients underwent surgical repair of AAOCA between 2012 and 2019: 16 (26%) patients underwent TAR of the anomalous coronary without an aortic button and 45 (74%) patients underwent coronary unroofing. Compared with patients who underwent an unroofing, patients who underwent TAR had similar intramural length (5 mm with interquartile range of 4-7.7 vs 6 mm with interquartile range of 5-7; P = .6). One patient with an anomalous right coronary underwent coronary artery bypass grafting after TAR because of persistent postoperative ischemic changes. One patient with unroofing of an anomalous left coronary artery presented with recurrent aborted sudden cardiac death and underwent subsequent TAR, without further events. At last follow-up, 15 of 16 patients (94%) who underwent TAR and 42 of 45 (93%) patients who underwent an unroofing were released to unrestricted exercise activities. CONCLUSIONS: Coronary artery TAR is a useful surgical alternative for AAOCA when there is a course below the commissure, when unroofing does not relocate the ostium to the appropriate sinus, or when unroofing results in compression by the intercoronary pillar.

Smaller right pulmonary artery is associated with longer survival time without scimitar vein repair.

INTRODUCTION: The optimal management of scimitar syndrome remains incompletely defined. We (1) evaluated the impact of aortopulmonary collateral (APC) occlusion, (2) compared outcomes according to surgical approach for patients who underwent surgery, and (3) identified anatomic factors associated with longer survival time without scimitar vein repair. METHODS: We conducted a single center, retrospective study of 61 patients diagnosed with scimitar syndrome between 1995 and 2019. Right pulmonary artery to total pulmonary artery cross-sectional area (RPA:PA CSA) quantitatively assessed right pulmonary artery size. Anatomical features were analyzed for association with longer survival time without scimitar vein repair. RESULTS: Median follow-up time was 6 years (Q1-Q3, 2-12), with 96% 5-year survival. Twenty-three patients underwent APC occlusion, which significantly decreased symptoms of overcirculation (100%-46%; p = .001) and systolic pulmonary artery pressure (median, 34-29 mmHg; p = .004). Twenty-three patients underwent scimitar vein repair; 5-year freedom from scimitar vein stenosis was 90% among patients who underwent a reimplantation compared with 42% in patients with baffle repair (p = .1). Three patients underwent surgery before the first year of age, with lower 5-year freedom from scimitar vein stenosis (0% vs. 84%; p < .001). On multivariate analysis, a lower RPA:PA CSA was associated with longer survival time without scimitar vein repair (p = .003). CONCLUSIONS: APC occlusion improves the clinical status of young and hemodynamically unstable patients. Repair at an early age is associated with an increased risk of scimitar vein stenosis. Scimitar vein repair might be avoided in patients with a smaller right pulmonary artery.

Surgical Techniques in Management of Supravalvular Aortic Stenosis in Children.

BACKGROUND: Multiple techniques exist for the repair of supravalvular aortic stenosis (SVAS), but given the lesion's rarity, analyses comparing the efficacy of each repair have been limited. METHODS: A retrospective review of all children at a single institution who underwent repair of SVAS from June 1995 to May 2019 was performed. Anatomic and physiologic measurements across time points were compared between 2 predominant surgical techniques. Time-to-event outcomes were compared using the log-rank test. RESULTS: SVAS was repaired in 89 patients, by using a single-patch in 31 (35%) and the Doty repair in 58 (65%). Median age at operation was 2.5 years (interquartile range [IQR], 1.0 to 6.8 years), with median follow-up of 5.8 years (IQR, 1.8 to 10.7 years). Reoperation was required in 8 (9%) patients at a median of 1.5 years postoperatively (IQR, 0.3 to 4.8 years). There was 1 death after multiple reinterventions. The change from the preoperative to the postoperative sinotubular junction z-score was greater for patients after Doty repair (median change +2.5; IQR, 1.5, 4.1) than for patients after single-patch repair (median change +0.8; IQR, -0.1, 2.1; P = .001). Freedom from reoperation was longer for patients after Doty repair than after the single-patch technique (P = .008). CONCLUSIONS: The Doty repair provides longer freedom from reoperation after supravalvular aortic stenosis repair compared with a single-patch technique, likely through a greater increase in the sinotubular junction at the time of initial operation.

Mechanical Mitral Valve Replacements in the Pediatric Population.

BACKGROUND: We evaluated the range of prosthetic size-to-weight ratio to optimize valve survival in small children. METHODS: A single-institution retrospective review of mechanical mitral valve replacements from 1995 to 2019 was performed. Prosthetic valve size-to-weight ratio was calculated as the prosthetic valve diameter divided by the patient's operative weight in children less than or equal to 35 kg. Patient death or reoperation on the valve was analyzed by size-to-weight ratio. Identifying a U-shaped distribution of events, patients were stratified as being in the nadir of the distribution or on the edges. RESULTS: Mechanical mitral valve replacements were performed in 56 (75%) children weighing less than or equal to 35 kg. Median follow-up time was 3.7 (interquartile range, 0.46-12) years. Median size-to-weight ratio was 1.5 (interquartile range, 1.0-2.0). A second replacement was required in 15 (27%) patients. Death occurred in 6 (11%) patients, including 3 after reoperation. The nadir of U-shaped distribution of events by size-to-weight ratio was bounded by a ratio from 1 to 2, which included 29 (52%) patients. A size-to-weight ratio from 1 to 2 provided optimal outcomes regardless of patient age. Reoperation-free survival at 5 years was 96% for patients with a ratio from 1 to 2 and 46% for patients with a ratio less than 1 or greater than 2. Patients with size-to-weight ratio 1 to 2 had longer reoperation-free survival than patients with a ratio less than 1 or greater than 2 (P < .001). CONCLUSIONS: Regardless of patient age, in patients less than or equal to 35 kg, optimal reoperation-free survival after prosthetic mitral valve replacement can be obtained by placing a prosthetic valve whose diameter is between 1 and 2 times the patient's weight in kilograms.

Coronary Artery Anomalies Are Associated With Increased Mortality After Truncus Arteriosus Repair.

BACKGROUND: Truncus arteriosus is associated with coronary anomalies. We identified coronary artery lesions in patients undergoing repair of truncus arteriosus, defined the impact of lesions on mortality, and studied the effect of surgical intervention of coronary lesions. METHODS: A retrospective review identified 107 patients with truncus repair (1995-2019). Coronary lesions were categorized as ostial stenosis, intramural, juxtacommissural origin, and single coronary. Survival analysis characterized survival after truncus repair and studied the association of coronary lesions and mortality. RESULTS: Among 107 patients with truncus repair 34 patients had at least 1 coronary lesion. Median follow-up time was 7 years, with 85% 5-year survival. Coronary lesions including ostial stenosis, intramurality, and juxtacommissural origin were associated with increased mortality, whereas single coronaries did not impact survival. Eleven patients had 1 coronary lesion and 6 patients with 2 coronary lesions had similar (80% and 83%, respectively) 5-year survival. Eight patients with 3 coronary lesions had 24% 5-year survival (P = .0003). Among patients with 1 or 2 lesions, surgical intervention on the coronary lesions tended to be associated with longer 5-year survival (100% vs 62%, respectively; P = .06). All patients with 3 lesions underwent coronary artery intervention, with 24% 5-year survival. CONCLUSIONS: Impact of coronary lesions on mortality after truncus repair increases with the number of lesions. Coronary artery intervention may be associated with improved time-related survival among patients with 1 or 2 lesions. Patients with the most complex anomalies (3 lesions) have poor survival and warrant ongoing study of repair techniques.

Younger Age at Operation Is Associated With Reinterventions After the Warden Procedure.

BACKGROUND: Multiple techniques are available for repair of supracardiac partial anomalous pulmonary venous return (PAPVR); however, most series fail to compare the techniques in contemporary cohorts. This study aimed to describe outcomes of the Warden procedure with a single-patch repair cohort to serve as a control. METHODS: A retrospective cohort analysis of all patients at a single institution (Texas Children's Hospital, Houston, TX) included patients undergoing either the Warden procedure or single-patch repair from 1996 to 2019 for PAPVR. Reintervention was defined as any catheter or surgical procedure on the superior vena cava (SVC) or pulmonary veins. Subgroup analysis was performed within the Warden cohort to evaluate for association between an SVC patch and reintervention-free survival. RESULTS: In total, 158 patients (122 in the Warden group and 36 in the single-patch group) were identified. The median age at operation was younger for patients in the Warden cohort (5.4 years; interquartile range, 3.3 to 10.2 years) compared with patients in the single-patch cohort (13.3 years; interquartile range, 6.5 to 18.7 years; P < .001). One patient in each cohort died. One patient required reoperation after the Warden procedure for dehiscence of the intracardiac patch. Ten patients required transcatheter reinterventions. Reintervention-free survival was not different between patients in the Warden cohort and patients in the single-patch cohort (P = .54) or within the Warden cohort in patients with an SVC patch (P = .27). When controlling for repair type, older age at repair was associated with longer reintervention-free survival (hazard ratio, 0.81; 95% confidence interval, 0.71 to 0.93; P = .002). CONCLUSIONS: The Warden procedure is a viable option for younger patients requiring supracardiac PAPVR repair, although these younger patients are likely at greatest risk for reintervention regardless of surgical technique.

Predictors of Transplant-Free Survival After the Norwood Procedure.

BACKGROUND: Birth weight, preterm delivery, and size for gestational age are surrogate markers for development that are commonly used in congenital heart surgery. Understanding the associations of these variables with patient outcomes is of great importance. METHODS: This study included all patients with hypoplastic left heart syndrome who underwent a Norwood procedure at a single institution from 1995 to 2018. Low birth weight was defined as weight less than 2.5 kg, and preterm delivery occurred at less than 37 weeks' gestation. Overall and conditional analyses were performed to evaluate for association with outcomes after the Norwood procedure. Secondary analyses evaluated the association of development measures with postoperative length of stay and ventilator duration. RESULTS: In total, 303 neonates (60% male) underwent the Norwood procedure and were followed for a median of 3.9 years (interquartile range, 0.5 to 10.4 years). Median birth weight was 3.1 kg (interquartile range, 2.8 to 3.4 kg). Patients with low birth weight had decreased transplant-free survival compared with patients with a normal birth weight (hazard ratio, 1.7; 95% confidence interval, 1.03 to 2.82; P = .039). When conditioning on survival to second-stage palliation, patients born small for gestational age had decreased transplant-free survival compared with patients born at appropriate size for gestational age (hazard ratio, 2.8; 95% confidence interval, 1.31 to 6.09; P = .008). Patients delivered preterm had a longer hospital length of stay (median, 55 days vs 31 days; P = .02) and more ventilator days compared with patients delivered at term (median, 7 days vs 4 days; P = .004). CONCLUSIONS: Various developmental markers have differing prognostic importance for patients undergoing the Norwood procedure. Understanding these differences can help guide preoperative decision making and patient selection.

Intraseptal Anomalous Coronary Artery With Myocardial Infarction: Novel Surgical Approach.

Anomalous aortic origin of a coronary artery with an intraseptal course of the left coronary artery is a rare anomaly that can cause myocardial ischemia. Surgical technique known to improve outcome is lacking because of the challenging complexity of the anatomy. We herein describe a novel surgical approach in a young athlete with myocardial infarction and ongoing ischemia. Supraarterial myotomy of the intraseptal segment through a right ventriculotomy and direct reimplantation of the left coronary artery were performed successfully. After postoperative resolution of inducible myocardial hypoperfusion, the patient was allowed to return to competitive activities with no further symptoms.

Outcomes in Anomalous Aortic Origin of a Coronary Artery Following a Prospective Standardized Approach.

BACKGROUND: Anomalous aortic origin of a coronary artery (CA) is the second leading cause of sudden cardiac death in young athletes. Management is controversial and longitudinal follow-up data are sparse. We aim to evaluate outcomes in a prospective study of anomalous aortic origin of CA patients following a standardized algorithm. METHODS: Patients with anomalous aortic origin of a CA were followed prospectively from December 2012 to April 2017. All patients were evaluated following a standardized algorithm, and data were reviewed by a dedicated multidisciplinary team. Assessment of myocardial perfusion was performed using stress imaging. High-risk patients (high-risk anatomy-anomalous left CA from the opposite sinus, presence of intramurality, abnormal ostium-and symptoms or evidence of myocardial ischemia) were offered surgery or exercise restriction (if deemed high risk for surgical intervention). Univariate and multivariable analyses were used to determine predictors of high risk. RESULTS: Of 201 patients evaluated, 163 met inclusion criteria: 116 anomalous right CA (71%), 25 anomalous left CA (15%), 17 single CA (10%), and 5 anomalous circumflex CA (3%). Patients presented as an incidental finding (n=80, 49%), with exertional (n=31, 21%) and nonexertional (n=32, 20%) symptoms and following sudden cardiac arrest/shock (n=5, 3%). Eighty-two patients (50.3%) were considered high risk. Predictors of high risk were older age at diagnosis, black race, intramural course, and exertional syncope. Most patients (82%) are allowed unrestrictive sports activities. Forty-seven patients had surgery (11 anomalous left CA and 36 anomalous right CA), 3 (6.4%) remained restricted from sports activities. All patients are alive at a median follow-up of 1.6 (interquartile range, 0.7-2.8) years. CONCLUSIONS: In this prospective cohort of patients with anomalous aortic origin of a CA, most have remained free of exercise restrictions. Development of a multidisciplinary team has allowed a consistent approach and may have implications in risk stratification and long-term prognosis.

Prolonged cardiopulmonary resuscitation and low flow state are not contraindications for extracorporeal support.

Outcomes of out-of-hospital cardiac arrest are poor irrespective of the patient age group and circumstances. Survival to discharge after out-of-hospital arrest in children is less than 10%. Use of extracorporeal cardiopulmonary resuscitation is increasing and has been shown to improve outcomes in some situations. However, the candidacy for such augmentation is based on patient selection, institutional practices, and availability of an extracorporeal membrane oxygenation center. Often, duration of resuscitation, low flow state, presenting pH, and circumstances of arrest dictate candidacy for extracorporeal membrane oxygenation. We present a case of extremely prolonged resuscitation for out-of-hospital arrest in a pediatric patient, and we describe the use of mechanical compression device and transition to extracorporeal membrane oxygenation. We present the case outcome as well as brief discussion about controversies in extracorporeal cardiopulmonary resuscitation. We hope the case provides an opportunity for further discussion regarding opportunities to improve selection, use of extracorporeal cardiopulmonary resuscitation, and impact outcomes.

Contemporary Midterm Outcomes in Pediatric Patients Undergoing Vascular Ring Repair.

BACKGROUND: This single-institution study assessed the midterm outcomes of patients undergoing complete vascular ring (CVR) repair and the need for reintervention. METHODS: The study included all patients who underwent surgical repair of an isolated CVR from 1996 to 2018 at our institution. Patients who underwent concomitant intracardiac repair were excluded. Data analysis included demographics, type of anomaly, other congenital heart disease, clinical symptomatology, operative technique, perioperative outcomes, reoperation rates, and mortality. RESULTS: CVR repair through open thoracotomy was performed in 148 patients (80 boys [54%]), median age, 1.04 years (interquartile range, 0.4-5.2 years), and median weight, 12.8 kg (interquartile range, 7.5-26.5 kg). The cohort included 72 patients with double aortic arch (DAA), 69 with right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum arteriosum (LLA), 5 with RAA with left descending aorta and LLA, and 2 with RAA with mirror-image branching and LLA. There was 1 outpatient perioperative death (0.7%) 15 days postoperatively. Perioperative complications occurred in 20 patients (14%): 18 (12%) with chylothorax (3 required reintervention), 1 pneumothorax, and 1 vocal cord paresis. Two of 36 patients (5.5%) without primary diverticulum resection required reoperation and subclavian reimplantation at 3 and 4 years, and 1 patient required aortic translocation 9 years later for persistent symptoms. CONCLUSIONS: Freedom from reoperation after CVR repair was 93% at 5 years and 86% at 10 years. A small proportion of patients who do not undergo diverticulum resection and aberrant left subclavian artery reimplantation at the time of CVR repair will require reintervention in the future.

Fibrinogen Concentrate as an Alternative to Cryoprecipitate in a Postcardiopulmonary Transfusion Algorithm in Infants Undergoing Cardiac Surgery: A Prospective Randomized Controlled Trial.

BACKGROUND: Infants undergoing cardiac surgery are at risk for bleeding and massive transfusion due to an immature coagulation system, complex surgeries, and cardiopulmonary bypass (CPB) effects. Hemodilution from CPB promotes an acquired hypofibrinogenemia that results in impaired fibrin formation, inadequate clot formation, and increased bleeding. In North America, the current standard of care to supplement fibrinogen is cryoprecipitate. An alternative option is the off-label use of fibrinogen concentrate (FC; RiaSTAP; CSL Behring, Marburg, Germany), a purified fibrinogen. Because perioperative allogenic transfusions are associated with increased morbidity and mortality, we sought to determine whether FC would be an acceptable alternative to cryoprecipitate in a post-CPB transfusion algorithm in infants undergoing open-heart surgery. METHODS: We randomized 60 infants (<12 months) undergoing nonemergent cardiac surgery with CPB at 2 tertiary care children's hospitals to receive either cryoprecipitate or FC in a post-CPB transfusion algorithm. Infants underwent a stratified randomization based on institution and surgical complexity. The primary outcome was the difference in number of intraoperative allogenic blood product transfusions. Secondary outcomes included 24-hour chest tube output (CTO), mechanical ventilation time, adverse events (AEs), intensive care unit (ICU) length of stay (LOS), hospital LOS, postoperative thrombosis, and death within 30 days of surgery. The primary analysis followed the intent-to-treat (ITT) principle and was performed using linear regression adjusted for institution and complexity of surgery. A per-protocol (PP) analysis was also performed. RESULTS: Between June 2016 and January 2018, we enrolled 60 patients with complete data available for 25 patients who received cryoprecipitate and 29 patients who received FC. Patients in the cryoprecipitate group (median age: 4 months [2-6 months]) received 5.5 (4.0-7.0) allogeneic blood units in the ITT analysis and 6.0 units (5.0-7.0 units) in the PP analysis. Patients in the FC group (median age: 4 months [2-5]) received 4 units (3.0-5.0 units) in the ITT analysis and 4.0 units (3.0-5.0 units) in the PP analysis. In the adjusted ITT analysis, the FC group received 1.79 units (95% confidence interval [CI], 0.64-2.93; P = .003) less than the cryoprecipitate group. In the adjusted PP analysis, the FC group received 2.67 units (95% CI, 1.75-3.59; P < .001) less than the cryoprecipitate group. There were no significant differences in secondary outcomes or AEs. CONCLUSIONS: Our findings suggest that FC may be considered as an alternative to cryoprecipitate for the treatment of hypofibrinogenemia in infants with bleeding after CPB. Although we found no significant differences between secondary outcomes or AEs, further studies are needed to assess safety.

Thoracic Aortic, Aortic Valve, and Mitral Valve Surgery in Pediatric and Young Adult Patients With Marfan Syndrome: Characteristics and Outcomes.

Patients with Marfan syndrome (MFS) often require surgical intervention on the mitral valve (MV), aortic root or valve (AV), or thoracic aorta (TA) during childhood and adolescence. We aim to utilize a national database to evaluate outcomes in pediatric and young adult patients with MFS undergoing MV, AV, and aortic surgical procedures, and describe factors associated with increased mortality. The Pediatric Hospital Information System (PHIS) database, a multi-institutional administrative database of 48 pediatric hospitals, was queried for patients less than 25 years of age with a diagnosis of MFS (ICD-9 759.82) who underwent MV, AV, or thoracic aortic surgery between January 2004 and October 2015. We assessed comorbidities and complications, and performed univariate analysis to evaluate factors associated with inpatient mortality. Included were 321 hospital encounters in 294 patients. Fifty-one patients underwent 54 MV surgeries, 213 patients underwent 224 aortic/AV surgeries, and 43 patients underwent both MV and aortic/AV surgery in the same encounter. Postoperative complications were common for all surgeries (46.3% for MV procedures and 45.5% for aortic/AV procedures). Overall in-hospital mortality was 2.2% (3.7% for MV procedures, 1.8% for AV/aortic procedures, and 2.3% in the combined MV and aortic/AV procedure group). Aortic dissection or rupture was reported in 3.4%, with no in-hospital mortalities. Death after MV as well as after aortic/AV surgery was associated with younger age. Postoperative complications are common in pediatric and young adult patients with MFS after intervention on the MV, AV, and TA, although mortality is relatively low.

Use of an Extracorporeal Centrifugal Pump with the Berlin Cannulas.

The Berlin EXCOR ventricular assist device has become a standard treatment in pediatric bridge to transplantation. Despite its increased use in pediatric heart failure, the patient population supported with this technology continues to suffer from hematologic issues such as postoperative hemorrhage and neurologic dysfunction secondary to thrombotic events. Finding a balance between hemorrhagic and thrombotic risks in the acute postoperative period poses a significant challenge. In this report, we describe our approach using a temporary centrifugal pump connected to cannulas designed for the Berlin EXCOR ventricular assist device. We believe that the interim use of an extracorporeal centrifugal pump allows time for hemodynamic stability, limits the hemorrhagic risks, and provides relevant hemodynamic information before conversion to the Berlin EXCOR.