Hybrid palliation versus nonhybrid management for a multi-institutional cohort of infants with critical left heart obstruction.

OBJECTIVE: To compare patient characteristics and overall survival for infants with critical left heart obstruction after hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) versus nonhybrid management (eg, Norwood, primary transplantation, biventricular repair, or transcatheter/surgical aortic valvotomy). METHODS: From 2005 to 2019, 1045 infants in the Congenital Heart Surgeons' Society critical left heart obstruction cohort underwent interventions across 28 institutions. Using a balancing score propensity analysis, 214 infants who underwent hybrid palliation and 831 infants who underwent nonhybrid management were proportionately matched regarding variables significantly associated with mortality and variables noted to significantly differ between groups. Overall survival between the 2 groups was adjusted by applying balancing scores to nonparametric estimates. RESULTS: Compared with the nonhybrid management group, infants who underwent hybrid palliation had lower birth weight, smaller gestational age, and higher prevalence of in-utero interventions, noncardiac comorbidities, preoperative mechanical ventilation, absent interatrial communication, and moderate or severe mitral valve stenosis (all P values < .03). Unadjusted 12-year survival after hybrid palliation and nonhybrid management, was 55% versus 69%, respectively. After matching, 12-year survival after hybrid palliation versus nonhybrid management was 58% versus 63%, respectively (P = .37). Among matched infants born weighing <2.5 kg, 2-year survival after hybrid palliation versus nonhybrid management was 37% versus 51%, respectively (P = .22). CONCLUSIONS: Infants born with critical left heart obstruction who undergo hybrid palliation have more high-risk characteristics and anatomy versus infants who undergo nonhybrid management. Nonetheless, after adjustment, there was no significant difference in 12-year survival after hybrid palliation versus nonhybrid management. Mortality remains high, and hybrid palliation confers no survival advantage, even for lower-birth-weight infants.

Time-Related Risk of Pulmonary Conduit Re-replacement: A Congenital Heart Surgeons' Society Study.

BACKGROUND: Patients receiving a right ventricle to pulmonary artery conduit (PC) in infancy will require successive procedures or replacements, each with variable longevity. We sought to identify factors associated with time-related risk of a subsequent surgical replacement (PC3) or transcatheter pulmonary valve insertion (TPVI) after a second surgically placed PC (PC2). METHODS: From 2002 to 2016, 630 patients from 29 Congenital Heart Surgeons' Society member institutions survived to discharge after initial valved PC insertion (PC1) at age ≤ 2 years. Of those, 355 underwent surgical replacement (PC2) of that initial conduit. Competing risk methodology and multiphase parametric hazard analyses were used to identify factors associated with time-related risk of PC3 or TPVI. RESULTS: Of 355 PC2 patients (median follow-up, 5.3 years), 65 underwent PC3 and 41 TPVI. Factors at PC2 associated with increased time-related risk of PC3 were smaller PC2 Z score (hazard ratio [HR] 1.6, P < .001), concomitant aortic valve intervention (HR 7.6, P = .009), aortic allograft (HR 2.2, P = .008), younger age (HR 1.4, P < .001), and larger Z score of PC1 (HR 1.2, P = .04). Factors at PC2 associated with increased time-related risk of TPVI were aortic allograft (HR: 3.3, P = .006), porcine unstented conduit (HR 4.7, P < .001), and older age (HR 2.3, P = .01). CONCLUSIONS: Aortic allograft as PC2 was associated with increased time-related risk of both PC3 and TPVI. Surgeons may reduce risk of these subsequent procedures by not selecting an aortic homograft at PC2, and by oversizing the conduit when anatomically feasible.

Partial Anomalous Pulmonary Venous Connection Repair: Customized Approach and Outcomes.

Alternative options for the correction of partial anomalous pulmonary venous connection (PAPVC) have been proposed. Each can be associated with variable risk for dysrhythmias, caval or pulmonary venous (PV) obstruction. A selective customized strategy to address PAPVC taking into account atrial shunt (AS) and growth potential was pursued. Between September 2014 and August 2018 21 PAPVC patients were identified. Two levels of reference determined the chosen repair strategy; azygous vein (AzV) and cavoatrial junction (CAJ). Six (Group-A) with PAPVC entering SVC cephalad to AV underwent a combined in-situ cavoatrial autologous reconstruction with atrial appendage advancement flap (CARAF). PAPVC entering caudally to AzV (Group-B) underwent alternative repair (caval division/Warden-type or intraatrial rerouting) (n = 15). Age was 8.3 (IQR:4.2-18.5) years for Group-A (vs 11.9; IQR:8.8-34.7 in Group-B) (p = 0.07). In Group-A 5(83%) had AS (vs 12[80%] Group-B; p = 0.9). None had left SVC in Group-A (vs 1 in Group-B; p = 0.9). Preoperative advanced imaging and echocardiographic hemodynamic evaluation was undertaken. Follow-up was complete (median 2.9; IQR:1.2-3.7 years). Freedom from atrial dysrhythmias, caval or PV obstruction was assessed. There were no early or late deaths. ICU and hospital length of stay were 1.8 ± 1.1 and 3.2 ± 0.5 days, respectively. No atrial dysrhythmias occurred postoperatively in Group-A (vs 1 in Group-B; p = 0.9). No permanent pacemaker was implanted. All patients remained in normal sinus rhythm. There were no early or late caval/PV obstruction. A customized approach reserves the advantages of each technique tailored to patient's needs. Expanding surgical capacity with favorable outlook for all PAPVC variations, irrespective of association with AS, can maximize efficiency and reproducibility paired with the lowest morbidity.

Myocardial Ischemia and Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery in the Adult: Management Implications and Follow-Up.

Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a very rare and potentially fatal congenital heart defect due to myocardial ischemia secondary to coronary steal phenomenon. We present a case of an adult presenting with a myocardial infarction who was found to have ARCAPA. Three-dimensional (3D) reconstructed computed tomography angiography (CTA) was utilized preoperatively for surgical planning. Surgical technique as well as pre- and postoperative 3D CTA are described and literature reviewed.

Central Vascular Thrombosis in Neonates with Congenital Heart Disease Awaiting Cardiac Intervention.

Central vascular thrombosis (CVT) in critically ill neonates carries significant clinical implications. Neonates with congenital heart disease (CHD) awaiting cardiac intervention might be associated with increased risk of thrombosis. Outcome analysis was undertaken. An analysis of 77 neonates with CHD who were admitted to the NICU prior to cardiac intervention between January 2015 and December 2016 was undertaken. Patients requiring extracorporeal life support prior to any cardiac intervention, or receiving prophylactic anticoagulation not related to central vascular catheter (CVC) were excluded. Diagnosis of CVT was provided based on clinical indication and verified with imaging that warranted anticoagulation therapy. Location of CVC and extent of CVT along with treatments, outcomes, and vascular access types and durations were assessed. Logistic regression multivariate analysis was used to assess predictors of outcome. Neonates with CHD were complicated with CVT in 10.4%. Longer duration of CVC was also associated with thrombosis in neonates with CHD (72.7 days vs. 29.3 days, p < 0.001). Independent predictors of outcome included lower gestational age and CHD with single-ventricle (SV) anatomy (p < 0.05). In neonates with CHD awaiting cardiac intervention risk of CVT is substantial. Duration CVC, lower gestational age and SV anatomy are risk determinants of outcome. Standardized development of customized surveillance protocols tailored to this unique subsets of neonates and adherence to quality guidelines can influence outcome.

Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.

Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results.

Right Ventricular Outflow Tract Reintervention in the Transcatheter Era: Outcomes and Cost Analysis.

Surgical pulmonary valve insertion (SPVI) for re-entry right ventricular outflow tract intervention (RVOTI) remains an established and reproducible approach. Fast-track in patients undergoing RVOTI of the comprehensive valve program targets early ICU and hospital discharge (Hd). Feasibility study for outcome and cost analysis was undertaken. Between January 2015 and December 2016, 34 patients underwent re-entry RVOTI. Seventeen had SPVI and 17 transcatheter PVI (TPVI). Surgical perioperative fast-track protocol was used. Echocardiographic evaluation preoperatively (TTE-1), after RVOTI (TTE-2), at hospital discharge (TTE-3), and follow-up (TTE-4) were obtained. Cost Analysis included procedural and hospital costs. Mean follow-up period was 11.3 ± 6.9 months. All patients were extubated prior to ICU arrival. Mean age was 8.5 ± 7.8 for SPVI [vs 28.5 ± 8.6 years for TPVI] (p < 0.05). There was no hospital mortality or 30-day readmission for SPVI (versus 1 for TPVI).Mean hospital length of stay (LOS) was 4.1 ± 1.1 days for SPVI [vs 1.1 ± 0.7 days for TPVI] (p < 0.05). Number of prior sternal re-entry had no influence on outcome. RV systolic pressure referenced to LVSP (rRVSP, %) and diastolic dimension (RVEDDi, z score) showed sustainable improvement (TTE-2, TTE-3, TTE-4) in both groups compared to TTE-1 (p < 0.05). Mean total hospital cost was $5475.86 ± 2503.91 lower after SPVI (p = 0.09), 21.7% procedural cost reduction. Patients undergoing RVOTI can be safely stratified, based on a customized concept, towards SPVI or TPVI. Standardized strategy can advocate a fast-track path. SPVI is associated with comparable mid-term outcomes to TPVI although SPVI is delivered in younger patients. Despite longer LOS SPVI is associated with reduced hospital cost. Multisite studies might help determine suitability for each strategy on cost containment/quality of life basis.

Hammock Mitral Valve Repair in Infancy: Operative Steps Toward a Customized Reconstruction After Preoperative Planning.

Hammock mitral valve (MV) repair is historically technically difficult with a guarded prognosis. Surgical experience is extremely limited and variable outcomes are reported. The perioperative strategy and technical details of hammock MV repair in an infant who presented with severe mitral stenosis are described and review of the existing literature was undertaken.

Standardized Approach to Intervention for Intestinal Malrotation in Single Ventricle Patients with Heterotaxy Syndrome: Impact on Interstage Attrition and Time to Superior Cavopulmonary Connection.

Heterotaxy syndrome (HS) is a significant determinant of outcome in single ventricle (SV) physiology. Attrition rate and time-related events associated with intestinal malrotation (IM) are, yet, to be determined. We sought to evaluate hospital and interstage outcomes in relation with operative intervention for IM (IMO). Twelve SV/HS patients, who underwent IMO, from January 2004 to December 2016, were studied. Early shunt failure, time to superior cavopulmonary connection (SCPC) and interstage attrition were assessed. Since September 2014, based on a comprehensive standardized protocol, IMO was performed at the time of hospitalization for stage-I palliation (S1P) irrespective of clinical manifestations. Patients were assigned to Group A (n = 8): expectant /symptoms-driven versus Group B (n = 4): protocol-driven. At S1P 7 had systemic-to-pulmonary shunt (SPS), 1 SPS with anomalous pulmonary venous return (APVR) repair (Group A) compared to 2 SPS, 1 SPS with APVR repair and 1 Norwood operation (Group B). Median duration from S1P to IMO was 82 days (range 57-336; Group A) compared to 14 days (range 11-31; Group B); p < 0.05. Median age at IMO was 87 days (range 8-345) [Group A: 99 days (range 68-345) vs Group B: 25 days (range 8-39)] (p < 0.05). Early SPS failure occurred in 25% (2 of 8) for Group A compared to none in Group B (p < 0.05). Hospital mortality following IMO was 25% [Group A: 37.5% (3 of 8) vs Group B: 0; p < 0.05]. Interstage survival was 67% [Group A: 50% (4 of 8) vs Group B: 100%; p < 0.05]. Time to SCPC following S1P was 186 days (range 169-218) for Group A compared to 118 days (range 97-161) (Group B); p < 0.05. Operative intervention for IM in SV/HS is associated with significant interstage attrition and might impact the time to SCPC. SPS is at risk for early failure after IMO. A comprehensive standardized concept can mitigate detrimental implications.

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.

OBJECTIVES: We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries. METHODS: We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded. RESULTS: We collected 156 surgical patients (median age 39.5 years, interquartile range 15-53) affected by anomalous right (67.9%), anomalous left (22.4%) and other anatomical abnormalities (9.6%). An interarterial course occurred in 86.5%, an intramural course in 62.8% and symptoms in 85.9%. The operations included coronary unroofing (56.4%), reimplantation (19.2%), coronary bypass graft (15.4%) and other (9.0%). Two patients with preoperative cardiac failure died postoperatively (1.3%). All survivors were discharged home in good clinical condition. At a median follow-up of 2 years (interquartile range 1-5, 88.5% complete), there were 3 deaths (2.2%), 9 reinterventions in 8 patients (5 interventional, 3 surgical); 91.2% are in New York Heart Association functional class ≤ II, but symptoms persisted in 14.2%; 48.1% of them returned to sport activity. On Kaplan-Meier analysis, event-free survival at follow-up was 74.6%. Morbidity was not significantly different among age classes, anatomical variants and types of surgical procedures. Furthermore, return to sport activity was significantly higher in younger patients who participated in sports preoperatively. CONCLUSIONS: Surgical repair of anomalous aortic origin of coronary arteries is effective and has few complications. Unroofing and coronary reimplantation are safe and are the most common procedures. The occurrence of late adverse events is not negligible, and long-term surveillance is mandatory. Most young athletes can return to an unrestrained lifestyle.

Anomalous aortic origin of coronary arteries: Early results on clinical management from an international multicenter study.

BACKGROUND: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies. METHODS: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment. Patients with isolated high coronary take off and associated major congenital heart disease were excluded. Preoperative, intraoperative, anatomical and postoperative data were retrieved from a common database. RESULTS: Among 217 patients, 156 underwent Surgical repair (median age 39 years, IQR: 15-53), while 61 were Medical (median age 15 years, IQR: 8-52), in whom AAOCA was incidentally diagnosed during screening or clinical evaluations. Surgical patients were more often symptomatic when compared to medical ones (87.2% vs 44.3%, p < 0.001). Coronary unroofing was the most frequent procedure (56.4%). Operative mortality was 1.3% (2 patients with preoperative severe heart failure). At a median follow up of 18 months (range 0.1-23 years), 89.9% of survivors are in NYHA ≤ II, while only 3 elderly surgical patients died late. Return to sport activity was significantly higher in Surgical patients (48.1% vs 18.2%, p < 0.001). CONCLUSIONS: Surgery for AAOCA is safe and with low morbidity. When compared to Medical patients, who remain on exercise restriction and medical therapy, surgical patients have a benefit in terms of symptoms and return to normal life. Since the long term-risk of sudden cardiac death is still unknown, we currently recommend accurate long term surveillance in all patients with AAOCA.

Selective Use of Temporary Epicardial Pacing Leads in Early Infancy Following Cardiac Surgery: Feasibility and Determinants of Clinical Application.

Use of temporary epicardial pacing (TEP) leads remains a routine perioperative strategy in congenital heart surgery. Selective use of TEP in neonates and infants undergoing cardiac intervention within the first 6 months of life has, yet, to be assessed. Outcome analysis was undertaken. From August 2014 to December 2016, 112 consecutive neonates and infants underwent cardiac intervention within the first 6 months of life. Patients with STS/EACTS Congenital Heart Surgery Mortality (STAT categories) 1-5 were prospectively followed from the index cardiac operation until hospital discharge and included in the study. Patients on permanent pacemaker (PPM) prior to the definitive cardiac intervention were excluded. Selective TEP placement was pursued if specific intraoperative indications were met. Determinants associated with the postoperative use of TEP were assessed. TEP leads were placed in 11 (9.8%) (GroupA). Nine was used for diagnostic and/or therapeutic purposes; two had no use (18%). From 101 patients without TEP (GroupB), one required treatment for postoperative dysrhythmia amenable to pacing. Vasoactive-inotrope score, ICU length of stay and time-to-negative balance was not statistically different between groups (p > 0.05). None of 112 patients required PPM insertion during hospital stay. Intraoperative need for cardioversion, attenuated ventricular function, and sustained sinus/AV node dysfunction or non-resolved elevated serum lactate at the time of operating room discharge were found to be predictors for TEP postoperative use. Selective placement of TEP leads is justified during early infancy for congenital heart surgery. Nearly 20% of those with TEP leads in place, even after its selective use, will not be required following surgery. Specific intraoperative parameters can guide the necessity and potential TEP postoperative use.

Neonatal Myocardial Perfusion in Right Ventricle Dependent Coronary Circulation: Clinical Surrogates and Role of Troponin-I in Postoperative Management Following Systemic-to-Pulmonary Shunt Physiology.

Right ventricle dependent coronary circulation (RVDCC) in pulmonary atresia with intact ventricular septum (PA/IVS) is associated with significant mortality risk in the immediate post-operative period following the initial stage of surgical palliation. Prognosis remains guarded during the interstage period towards conversion to the superior cavopulmonary shunt physiology. Current literature is scarce regarding this specific patient population. Cardiac troponin-I is widely used as a marker of coronary ischemia in adults, but its use for routine monitoring of neonatal myocardial tissue injury due to supply/demand perfusion mismatch is, yet to be determined. We sought to evaluate the clinical correlation of cTnl perioperative use in a PA/IVS RVDCC case and assess its interplay with established clinical, echocardiographic, and laboratory variables in guiding a real-time (dynamic) management strategy following systemic-to-pulmonary shunt palliation.

Restoring Fetal Circulation as a Means of Bridging Treatment Prior to Surgical Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta with Persistent Pulmonary Hypertension of the Newborn.

Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital anomaly. Even more rarely reported is its presence in conjunction with persistent pulmonary hypertension of the newborn (PPHN). We present a case of a full-term infant, initially thought to have PPHN and later found to have anomalous origin of the right pulmonary artery from the ascending aorta. We discuss our management concept which included use of PgE1 infusion to restore fetal circulation prior to surgical treatment in this unique clinical scenario.

The Challenge of Hammock Mitral Valve During Infancy: Precise Preoperative Advanced Imaging and Three-Dimensional Modeling Augments Customized Operative Valve Reconstruction.

Hammock mitral valve (MV), also known as anomalous mitral arcade, is a rare congenital anomaly. We report a case of a 10-month-old child who presented with congestive heart failure and was found to have severe mitral stenosis (MS) secondary to a hammock MV anomaly. Detailed advanced imaging with cardiac MRI and three-dimensional (3D) echocardiography favorably navigated a customized valve-sparing surgical reconstruction of the congenitally abnormal MV. Repair of a hammock MV is technically difficult with a guarded prognosis. Surgical experience is extremely limited and variable outcomes are reported. A rare case of severe MS secondary to hammock MV deformity with successful surgical repair and review of worldwide literature are presented.

Anomalous Origin of Pulmonary Artery From Aorta: Neonatal Autologous Aortopulmonary Reconstruction.

Anomalous origin of a branch pulmonary artery (PA) from the ascending aorta is rather rare within the spectrum of congenital cardiovascular anomalies. In the scarce subset of patients with anomalous origin of right PA (RPA) from the ascending aorta originating from the lateral aspect of the distal ascending aorta, early neonatal repair by employing native aortic tissue for RPA reconstruction combined with translocation of the aorta posteriorly to the RPA can be an alternative option in the surgical armamentarium.

Attrition in patients with single ventricle and trisomy 21: outcomes after a total cavopulmonary connection.

OBJECTIVES: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). METHODS: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27.6 ± 12.1 months. RESULTS: The initial procedure was pulmonary artery banding in 9 patients, systemic-to-pulmonary shunt in 2 and Damus-Kaye-Stansel/Norwood procedure in 2. Median follow-up was 69 months (interquartile range 25-75, 21-99). There was 1 death after a Damus-Kaye-Stansel/Norwood procedure and one interstage death after a bidirectional Glenn procedure. Nine (of 11) survivors underwent a Fontan operation. A fenestrated Fontan procedure was the predominate operation in 78%. One patient was deemed unsuitable for a Fontan operation. There was 1 takedown and 1 late death after the Fontan operation. Heterotaxy syndrome did not affect outcome ( P > 0.05). There was no statistical difference in the pre-Fontan McGoon ratio, hospital length of stay, duration of pleural drainage and Fontan-related adverse events between patients with a dominant right ventricle and those with a left ( P > 0.05). CONCLUSIONS: A TCPC in patients with T21 and an FSV is associated with reproducible, satisfactory outcomes. An assisted-Glenn procedure with pulsatile pulmonary blood flow and a fenestrated Fontan may be associated with attenuated perioperative morbidity and late attrition.

Transposition of Great Arteries with Complex Coronary Artery Variants: Time-Related Events Following Arterial Switch Operation.

Coronary artery anatomy represents a challenging and, often, determining predictor of outcome in an arterial switch operation (ASO). Impact of specific coronary artery variants, such as single, intramural and inverted, on time-related events following ASO, is, yet, to be determined. We sought to compare early and late outcomes within the group of nonstandard coronary artery variants. Patients who underwent ASO from January 1995 to October 2010 were reviewed. Patients with coronary artery variants other than L1Cx1R2 ("standard" by Leiden classification) were included. Patients with single, intramural and inverted coronary artery variants incorporated in group A. All other nonstandard coronary variants incorporated in group B. Demographics, perioperative variables, early and late outcomes were assessed. Of the 123 ASO, 24 patients (19.5%) with nonstandard coronary variant were studied. Thirteen were in group A and 11 in group B. There were two early deaths (1 in group A and 1 in group B) (p > 0.05). There is one death early after hospital discharge (group A). Mean follow-up was 59.4 ± 55.1 months. There was no structural coronary artery failure after hospital discharge following ASO. Freedom from any reintervention at 8 years was (78.3 ± 9.6%) (p 0.55) with no late neo-aortic or mitral valve intervention. ASO with single, intramural or inverted coronary artery course carries no added longitudinal risk for structural or flow impairment within the group of nonstandard coronary artery variants. There is an early hazard period with no late survival attrition. Aortic arch repair as part of staged strategy prior to ASO might influence early and late outcome.