Outcomes Following Norwood Procedures: Analysis of a "Small Volume" Program.

BACKGROUND: Institutional survival following Norwood procedures is traditionally correlated with a center's surgical volume. Multiple single and multi-institutional studies conducted at large-volume centers have recently demonstrated improved survival following Norwood procedures. We report both short- and long-term outcomes at a single, small-volume institution and comment on factors potentially influencing outcomes at this institution. METHODS: All patients undergoing Norwood procedures from January 1, 2005, to January 1, 2020, at our institution were included in this study. Kaplan-Meier survival and Cox regression risk factor analyses were performed in addition to first interstage risk factor scoring to compare observed versus expected survival. RESULTS: The cohort included 113 patients. Kaplan-Meier freedom from death or transplant was 88%, 80%, and 76% at 1, 5, and 10 years, respectively. Freedom from death following hospital discharge after Norwood procedures was 94%, 87%, and 83% at 1, 5, and 10 years, respectively. The presence of genetic syndromes was a significant risk factor for mortality. First interstage observed-to-expected mortality following discharge was 0.57 (P = .04). Postoperative length of stay was comparable to that reported for the period 2015 to 2018 in the Society of Thoracic Surgeons Database. CONCLUSIONS: Survival outcomes at this single, small-volume institution were similar to those reported by large-volume centers and multi-institutional collaborative studies. These results may be related to structural and functional features that have been demonstrated to influence outcomes in other studies. These factors are achievable by small-volume programs with sufficient resource allocation.

Outcomes after anomalous aortic origin of a coronary artery repair: A Congenital Heart Surgeons' Society Study.

OBJECTIVES: It remains unclear when sudden cardiac event risk outweighs surgical risk for patients with anomalous aortic origin of a coronary artery. The Congenital Heart Surgeons' Society sought to characterize the surgical risks by determining the techniques, complications, and outcomes of repair. METHODS: Between January 2000 and September 2018, 682 patients with anomalous aortic origin of a coronary artery aged 30 years or less were enrolled. Demographic, morphologic, operative, imaging, and ischemia-related data were analyzed. RESULTS: There were 395 of 682 (57%) surgical patients (45 centers, median follow-up 2.8 years). In addition to primary repair (87% unroofing, 26% commissural manipulation), 13 patients had 15 coronary-related reoperations. Of 358 patients with pre/postoperative aortic insufficiency assessment, 27 (8%) developed new mild or greater aortic insufficiency postoperatively, and 7 (2%) developed new moderate or greater aortic insufficiency. Freedom from mild aortic insufficiency differed in those with versus without commissural manipulation (85%/91% at 6 months, 83%/90% at 1 year, and 77%/88% at 3 years, respectively) (P = .05). Of 347 patients with preoperative/postoperative ejection fraction, 6 (2%) developed new abnormal ejection fraction (<50%) within 30 days of surgery which persisted. Although 64 of 395 patients (16%) had preoperative ischemia, after surgery 51 of 64 patients (80%) no longer had ischemia (13 = new postoperative ischemia, P < .0001). Four patients died postoperatively (preoperatively 2 asymptomatic, 1 symptomatic, 1 in extremis). Composite surgical adverse event rates were 7% to 13% in the entire cohort (increasing/decreasing by presentation/anatomy/repair strategy). CONCLUSIONS: Anomalous aortic origin of a coronary artery surgery may relieve ischemia with low mortality; however, it can result in a variety of important morbidities, varying by the group evaluated. Strategies avoiding commissural manipulation may decrease the risk of developing aortic insufficiency. Understanding these risks should inform surgical decision-making and support the need for standardized assessment and management.

Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease.

OBJECTIVE: To develop a risk score to predict mortality or transplant in the interstage period. BACKGROUND: The "interstage" period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease. METHODS: This was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015. The primary composite endpoint was interstage mortality or transplant. Multivariable logistic regression and classification and regression tree analysis were performed on two-thirds of the patients ("learning cohort") to build a risk score for the composite endpoint, that was validated in the remaining patients ("validation cohort"). RESULTS: In the 2128 patients analyzed in the registry, the overall event rate was 9% (153 [7%] deaths, 42 [2%] transplants). In the learning cohort, factors independently associated with the composite endpoint were (1) type of Norwood; (2) postoperative ECMO; (3) discharge with Opiates; (4) No Digoxin at discharge; (5) postoperative Arch obstruction, (6) moderate-to-severe Tricuspid regurgitation without an oxygen requirement, and (7) Extra Oxygen required at discharge in patients with moderate-to-severe tricuspid regurgitation. This model was used to create a weighted risk score ("NEONATE" score; 0-76 points), with >75% accuracy in the learning and validation cohorts. In the validation cohort, the event rate in patients with a score >17 was nearly three times those with a score ≤17. CONCLUSIONS: We introduce a risk score that can be used post-stage 1 palliation to predict freedom from interstage mortality or transplant.

Pulmonary artery banding in complete atrioventricular septal defect.

OBJECTIVES: To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function. METHODS: PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months. The median duration of follow-up was 2.1 years. Atrioventricular valve function was assessed by review of pre-PAB and predischarge echocardiograms (median, 9 days postoperatively). Competing-risks methodology was used to analyze the risks for biventricular repair, univentricular repair, and death. RESULTS: At 2 years, the proportions of patients who underwent biventricular repair, univentricular repair, and death were 68%, 13%, and 12%, respectively, with 8% awaiting definitive repair. After PAB, atrioventricular valve regurgitation decreased in 14 infants and increased in 10, but the distribution of regurgitation severity did not change significantly in the total cohort or subgroups. The intended management plan at PAB was deferred biventricular/univentricular decision (23 infants), 2-stage biventricular repair (24 infants), and univentricular repair (3 infants). Among the 24 infants intended for biventricular repair, 23 achieved biventricular repair and 1 died before repair. Survival at 4 years after biventricular repair among patients with previous PAB (93%) was similar to the 4-year survival of the patients who underwent primary biventricular repair (91%; n = 333). CONCLUSIONS: PAB is a successful strategy in complete AVSD to bridge to biventricular repair and has similar post-biventricular repair survival to primary biventricular repair. Changes in atrioventricular valve regurgitation after PAB were variable.

Pre-intervention morphologic and functional echocardiographic characteristics of neonates with critical left heart obstruction: a Congenital Heart Surgeons Society (CHSS) inception cohort study.

AIMS: The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions. METHODS AND RESULTS: Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%). Only 17% of patients with AS/MS had an apex-forming left ventricle, compared with 0% of those with AA/MA and AA/MS (P < 0.0001). Aortic arch hypoplasia and coarctation were common across all four groups, while those with AA/MA and AA/MS had the smallest ascending aorta diameters. Flow in the ascending aorta was retrograde in 43% and 10% of the patients with AS/MS and iAS, respectively. The right ventricle was apex forming in 100% of patients with AA/MA and AA/MS, 96% with AS/MS and 70% with iAS (P < 0.0001). Moderate to severe tricuspid regurgitation was present in 13% of all patients. CONCLUSION: This large multi-institutional study generates insight into the distribution of the functional and morphologic spectrum in patients with critical left-sided heart disease and identifies differences in these functional and morphologic characteristics between the main anatomic subtypes of critical left heart obstruction.

Surgical Palliation of Right Aortic Arch With an Isolated Innominate Artery From the Left Ductus Arteriosus.

A right aortic arch with an isolated left innominate artery from the left patent ductus arteriosus is a rare arch anomaly, and establishing continuity between the innominate artery and aorta can be challenging. We describe repair of this lesion in a three-week-old male using an autologous pedicle flap of ascending aorta as well as a homograft patch as the roof to recreate continuity between the aorta and left innominate artery.

Fontan Fenestration Closure Prior to Posterior Spinal Fusion in Patients With Single-Ventricle Heart Disease.

STUDY DESIGN: Case series. OBJECTIVE: To describe transcatheter closure of the Fontan fenestration prior to posterior spinal fusion in two children to prevent paradoxical venous air embolism during the operation. SUMMARY OF BACKGROUND DATA: Scoliosis is common among patients with single-ventricle congenital heart disease who have undergone Fontan operation and spinal surgery can offer physiologic benefits. Venous air embolism is a rare, but important reported complication during spinal surgery performed in the prone position. Patients with Fontan circulation can have significant right to left shunting via a patent Fontan fenestration that can increase the risk of paradoxical systemic embolization of any entrained venous air. METHODS: We retrospectively reviewed the charts of two patients with single-ventricle congenital heart disease who had undergone fenestrated Fontan operation and underwent transcatheter fenestration closure prior to spinal fusion. RESULTS: Two patients with Fontan circulation underwent successful transcatheter fenestration closure with Amplatzer Ductal Occluder II devices. Five to 6 months after closure, both underwent uncomplicated posterior spinal fusion. CONCLUSION: Transcatheter closure of the Fontan fenestration prior to spinal fusion in two with Fontan circulation and scoliosis is a rare, but important indication for fenestration closure that warrants emphasis. LEVEL OF EVIDENCE: N/A.

Digoxin Use Is Associated With Reduced Interstage Mortality in Patients With No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease.

BACKGROUND: Interstage mortality (IM) remains significant after stage 1 palliation (S1P) for single-ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post-S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC). METHODS AND RESULTS: From June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM. Arrhythmia during S1P hospitalization or discharge on antiarrhythmic medications were exclusions (n=270); 2 patients were lost to follow-up. Two analyses were performed: (1) propensity-score adjusted logistic regression with IM as outcome and (2) retrospective cohort analysis for patients discharged on digoxin versus not, matched for surgical site and other established IM risk factors. Of 544 study patients, 119 (21.9%) were discharged on digoxin. Logistic regression analysis with propensity score, site-size group, and digoxin use as predictor variables showed an increased risk of IM in those not discharged on digoxin (odds ratio, 8.6; lower confidence limit, 1.9; upper confidence limit, 38.3; P<0.01). The retrospective cohort analysis for 60 patients on digoxin (matched for site of care, type of S1P, post-S1P ECMO use, genetic syndrome, discharge feeding route, ventricular function, tricuspid regurgitation, and aortic arch gradient) showed 0% IM in the digoxin at discharge group and an estimated IM difference between the 2 groups of 9% (P=0.04). CONCLUSIONS: Among SVD infants in the NPCQIC database discharged post-S1P with no history of arrhythmia, use of digoxin at discharge was associated with reduced IM.

Hybrid approach to the comprehensive stage II operation in a subset of single-ventricle variants.

OBJECTIVE: The objective of a hybrid approach to staged palliation of single-ventricle anomalies is designed to minimize the trauma of the first stage. However, the second stage is a complex procedure that may negate the advantages of the first stage. We sought to devise a "hybrid" approach to the second stage when aortic outflow is expected to remain unobstructed. METHODS: The procedure involves a simple incision into the main pulmonary artery, dilation/stenting of the ductal continuation, formation of a stented baffle between the branch pulmonary arteries' orifices, and a bidirectional Glenn connection. It avoids dissection of the distal arch and ductal continuation and obviates the need for a Damus-Kaye-Stansel connection. We carried out this procedure in 2 patients, one with unbalanced atrioventricular canal and the other with mitral atresia. RESULTS: Both patients underwent an uncomplicated operative procedure. Both patients were successfully weaned from the ventilator, with no clinically evident neurologic injury. The first patient died of complications related to thrombosis of the left pulmonary artery before initiation of anticoagulation. The second patient is alive and well 1 year postoperation with no obstruction to either systemic or pulmonary flow and no baffle leak and good right ventricle function. CONCLUSIONS: This hybrid comprehensive stage II operation appears feasible and technically simpler than the conventional comprehensive stage II procedure. It is applicable to a subset of single-ventricle cases in which aortic outflow is anticipated to remain unobstructed. We recommend early postoperative anticoagulation to avoid early left pulmonary artery thrombosis.

New imaging modalities in the catheterization laboratory.

PURPOSE OF REVIEW: To provide an overview of the various modalities that have emerged in the last years as adjunctive tools to provide detailed imaging of the cardiovascular system in the catheterization laboratory. RECENT FINDINGS: Completion angiography after cardiac surgery for congenital heart disease using a dedicated hybrid operating cardiac suite may aid in early diagnosis and therapy of postsurgical abnormalities. The application of three-dimensional rotational digital angiography results in a more precise delineation of the three-dimensional anatomy of vascular structures outside the heart with rapid image processing and reduction of the amount of contrast used and ionizing radiation dose. Combined catheterization/magnetic resonance (MR) hybrid laboratories have the potential to accurately assess changes in the anatomy and physiology pretranscatheter and posttranscatheter interventions. Fully MR-guided transcatheter procedures have become possible in animal models. Real-time three-dimensional transesophageal echocardiography (TEE) has helped to guide transcatheter procedures in septal and valvar structures. Miniaturization of probes has expanded the applications of TEE for smaller neonates. The use of intravascular ultrasound has helped to better understand the mechanisms of vascular diseases and transcatheter treatments. SUMMARY: Knowledge and proper application of new imaging modalities during interventions in the catheterization laboratory may improve the outcomes of patients with congenital and acquired structural heart disease.

Results of the U.S. multicenter pivotal study of the HELEX septal occluder for percutaneous closure of secundum atrial septal defects.

OBJECTIVES: This study sought to compare the safety and efficacy of the HELEX septal occluder (HSO) with surgical repair of atrial septal defect (ASD). BACKGROUND: The HSO is a low-profile, double-disk occluder device for percutaneous closure of secundum ASD. METHODS: Patients were enrolled (HSO arm prospectively, surgery arm prospectively/retrospectively) from 14 U.S. sites and followed up for 12 months postprocedure. Investigator-reported outcomes were evaluated, including closure success (no or clinically insignificant residual shunt) and the incidence of adverse events. The first 3 HSO patients at each site were considered training cases and were excluded from analysis. RESULTS: Between March 2001 and April 2003, 119 nontraining cases received an HSO and 128 had surgical repair. The groups were similar with statistical but clinically unimportant differences in median age, weight, and preprocedural echocardiographic defect size. Anesthesia time and hospital stay were significantly shorter in the HSO group. Closure success, defined as complete closure or a clinically insignificant residual shunt, was similar in both groups. Major and minor adverse events rates were not statistically different. The most common major adverse events for the HSO group was device embolization requiring catheter retreival (1.7%), and in the surgery group was postpericardiotomy syndrome (6.3%), including one death because of tamponade. The primary end point, clinical success, a composite of closure success and no major adverse events at 12 months, satisfied the noninferiority hypothesis comparing device closure with surgery. CONCLUSIONS: Closure of ASD with the HELEX septal occluder is safe and effective when compared with surgical repair, with reduced anesthesia time and hospital stay. (U.S. Multicenter Pivotal Study of the HELEX Septal Occluder for Percutaneous Closure of Secundum Atrial Septal Defects; this study was approved by the Food and Drug Administration before the National Institutes of Health website was active, so there is not a URL or registration number.).