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.

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.

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.

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.

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.

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.

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.

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.

Post-cardiotomy Rescue Extracorporeal Cardiopulmonary Resuscitation in Neonates with Single Ventricle After Intractable Cardiac Arrest: Attrition After Hospital Discharge and Predictors of Outcome.

Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We reviewed outcomes of neonates with functional single ventricle (FSV) surviving post-cardiotomy ECPR after hospital discharge. Fifty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2011) were identified. Forty-one were neonates. Survival analysis was conducted. Of 41 neonates receiving post-cardiotomy ECMO, 32 had FSV. Twenty-one had ECPR. Fourteen underwent Norwood operation (NO) for hypoplastic left heart syndrome (HLHS). Seven had non-HLHS FSV. Four (of 7) underwent modified NO/DKS with systemic-to-pulmonary shunt (SPS), 2 SPS only and 1 SPS with anomalous pulmonary venous connection repair. Mean age was 6.8 ± 2.1 days. ECMO median duration was 7 days [interquartile range (IQR25-75: 4-18)]. Survival to ECMO discontinuation was 72% (15 of 21 patients) and at hospital discharge 62% (13 of 21 patients). The most common cause of late attrition was cardiac. At last follow-up (median: 22 months; IQR25-75: 3-36), 47% of patients were alive. Duration of ECMO and failure of lactate clearance within 24 h from ECMO deployment determined late survival after hospital discharge (p < 0.05). Rescue post-cardiotomy ECMO support in neonates with FSV carries significant late attrition. ECMO duration and failure in lactate clearance after deployment are associated with unfavorable outcome. Emphasis on CPR quality, refinement of management directives early during ECMO and aggressive early identification of patients requiring heart transplantation might improve late survival.

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.

Fate of ventricular and valve performance following early bidirectional Glenn procedure after Norwood operation controlled for hypoplastic left heart syndome anatomic subtype.

The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is reportedly associated with early hemodynamic advantage. Shunt strategy has been implicated in ventricular function. Outcomes after NRVPA compared with classic procedure as part of a strategy involving early bidirectional Glenn (BDG) procedure were analyzed with reference to RV, tricuspid, and neoaortic valve performance. Between January 2005 and December 2010, 128 neonates with hypoplastic left heart syndrome (HLHS) underwent NO. Controlled for aortic/mitral stenosis (AS-MS) subtype, 28 patients underwent NRVPA (group A), and 26 patients had classic procedure (group B). The patients with a non-HLHS single-ventricle anatomy and those who had undergone a hybrid approach for HLHS were excluded from the study. The mean age at NO was 6.8 ± 3.5 days in group A and 6.9 ± 3.6 days in group B. Transthoracic echocardiographic evaluation (TTE) after NO (TTE-1) at the midinterval between NO and BDG (TTE-2), before BDG (TTE-3), before Fontan (TTE-4), and at the last follow-up evaluation (TTE-5) was undertaken. Cardiac catheterization was used to assess hemodynamic parameters before the Glenn and Fontan procedures. The operative, interstage, and pre-Fontan survival rates for AS-MS after NO were respectively 88.1 % (90.3 % in group A vs. 84.7 % in group B; p = 0.08), 82.5 % (82.7 % in group A vs. 81.8 % in group B; p = 0.9), and 80.7 % (79.5 % in group A vs. 81.8 % in group B; p = 0.9). The median follow-up period was 39.6 months (interquartile range 2.7-4.9 months). The RV global function, mid- and longitudinal indexed dimensions, fractionated area change before BDG (TTE-1, TTE-2, TTE-3) and after BDG (TTE-4, TTE-5), and right ventricular end-diastolic pressure did not differ statistically between the groups (p > 0.05). No statistically significant difference in tricuspid or neoaortic intervention was found between the groups (p > 0.05). Controlled for the AS-MS HLHS subtype, shunt strategy showed no midterm survival or hemodynamic (ventricular or valve) impact. At midterm, the follow-up need for neoaortic or tricuspid valve surgical intervention was not affected by shunt selection. The structural ventricular adaptation after reversal of shunt physiology was irrespective of shunt strategy.

Ruptured sinus of Valsalva found incidentally in a patient with tetralogy of Fallot.

Ruptured sinus Valsalva aneurysm (SVA) in a patient with tetralogy of Fallot is a very rare cardiac complication. This report describes the case of a 10-year-old Hispanic girl who presented to her cardiologist for a routine transthoracic echocardiography (TTE), which showed a ruptured sinus of Valsalva, with left-to-right shunting noted from the right cusp to the right ventricular outflow tract and pulmonic insufficiency. The patient underwent right and left heart catheterization with transesophageal echocardiography guidance and closure of the ruptured sinus of Valsalva with an Amplatzer muscular ventricular septal defect (VSD) device. Later that day, after the procedure, it was noted that the patient had a recurrence of continuous murmur. The TTE did not visualize the Amplatzer VSD device, and chest X-ray showed that the device had embolized to the right pulmonary artery. The device was successfully retrieved percutaneously. The patient returned 2 weeks later for surgical repair of a ruptured SVA and pulmonary valve replacement, which went well. This case report is the first to describe congenital rupture of a Valsalva aneurysm in a patient with tetralogy of Fallot. The conventional treatment for a ruptured sinus of Valsalva is surgical, although transcatheter approaches have been used increasingly in recent years.

Tetralogy of Fallot with atrioventricular septal defect: surgical strategies for repair and midterm outcome of pulmonary valve-sparing approach.

Repair for tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) has been reported with good early and intermediate outcomes. Morbidity, however, remains significantly high. To date, repair of CAVSD/TOF using a pulmonary valve-sparing technique (PVS) and freedom from valve reoperation are not well defined. A study was undertaken to investigate outcomes. This study was conducted in as a retrospective investigation. Between January 1988 and December 2008, 13 consecutive patients with CAVSD/TOF were identified, and their records were reviewed retrospectively. Of these 13 patients, 9 had Rastelli type C CAVSD. Trisomy 21 was present in 9 cases (69 %; 7 with type C). Five patients had received a systemic-to-pulmonary shunt (SPS) before complete repair at a mean age 1.7 ± 0.6 months. All the patients survived until complete repair. At complete CAVSD/TOF repair, AVSD was corrected with a two-patch technique in all patients. For eight patients (61.5 %), PVS was used. The remaining five patients had transannular patch (TAP) repair. The mean age at complete repair was 6.3 ± 2.4 months. At complete repair, the mean cardiopulmonary bypass time was 173.5 ± 30.6 min, and the cross-clamp time was 134.7 ± 28.8 min. There was one hospitalization and no late deaths. The median follow-up period was 9.2 years [interquartile range (IQR), 4.7-13.3 years]. The actuarial survival was 90.0 ± 9.5 % at 1 year, 90 ± 9.5 % at 5 years, and 90 ± 9.5 % at 8 years. Of the 12 survivors, 6 had some reintervention during the follow-up period. Within the first 11 years after complete repair, two patients underwent left atrioventricular (AV) valve repair, and one patient had right AV valve repair. Two patients had residual VSD closure. Four patients underwent the first right ventricular outflow tract (RVOT) reintervention for critical insufficiency or stenosis at a mean interval of 6 ± 21) months. One patient had a second RVOT reoperation. Findings showed that CAVSD/TOF with PVS was related to significantly higher freedom from RVOT reintervention (100 % at 1, 5, and 8 years compared with 80 ± 17.9 % at 1 year, 60 ± 21.9 % at 5 years, and 40 ± 21.9 % at 8 years for CAVSD/TOF using TAP; P < 0.05). No patient who underwent PVS had left ventricular outflow tract obstruction requiring reoperation. Overall freedom from any reintervention was 90.9 ± 8.6 % at 1 year, 71.6 ± 14.0 % at 5 years, and 53.7 ± 8.7 % at 8 years in this group of patients. Correction of TOF with CAVSD can be performed at low risk with favorable intermediate-term survival and satisfactory freedom from reoperation. Use of TAP can be avoided in almost two thirds of patients and may influence freedom from early RVOT reintervention.

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.

Hypoplastic left heart syndrome and aortic atresia-mitral stenosis variant: role of myocardial protection strategy and impact of ventriculo-coronary connections after stage I palliation.

Aortic atresia-mitral stenosis (AA-MS) has been implicated as a determinant of outcome after Stage-1 palliation (S1P) in hypoplastic left heart syndrome (HLHS).Studies evaluating the association of AA-MS with ventriculo-coronary connections (VCC) and mortality report conflicting results. The significance of VCC, myocardial protection, and shunt strategy after S1P has yet to be determined. Between January 2005 and July 2009, 100 neonates with HLHS underwent S1P. Mitral and aortic valves and presence of VCC were assessed. Antegrade continuous cold blood cardioplegia was administered throughout the vast extent of the neo-aortic reconstruction. A right ventricle-to-pulmonary shunt was used for an ascending aortic diameter of 0.6 mm/kg or less. Survival analysis was performed to determine predictors and assess impact of AA-MS and VCC on hospital and interstage mortality. Twenty-seven (of 100) patients had AA-MS. The mean age and weight at S1P were 6.5 ± 2.8 days and 3.09 ± 0.47 kg, respectively. VCC were found in 56% of AA-MS. Twenty-two had Norwood-Sano, 3 had classic Norwood, and 2 had hybrid S1P. VCC were associated with AA-MS, endocardial fibroelastosis, and ascending aortic size <2 mm (P < 0.05) but not higher mortality (P = ns). Operative and interstage survival for AA-MS after S1P was 85.2 and 71%, respectively (not statistically different compared to all other subtypes; P = ns). Actuarial survival after S1P at 1, 3, 6, 12, and 36 months was 92.9 ± 4.9, 78.6 ± 7.8, 75 ± 8.2, 71.3 ± 8.3, and 71.3 ± 8.3%, respectively. Intact atrial septum and post-S1P renal dysfunction (P < 0.05) were independent predictors of hospital and interstage mortality. In patients with HLHS, AA-MS carries no survival disadvantage after S1P during the hospital and interstage period regardless of VCC. Intact atrial septum and post-S1P renal dysfunction predict early and interstage mortality. Myocardial protection and shunt strategy might influence the outcome in this HLHS variant.

Technical description of the use of selective perfusion techniques during the Norwood procedure for hypoplastic left heart syndrome.

Since the introduction of the Norwood procedure for surgical palliation of hypoplastic left heart syndrome in 1983, refinements have been made to the original procedure to improve patient outcomes while still accomplishing the original goals of the procedure. One of these refinements has been the introduction of regional selective perfusion to limit the duration of circulatory arrest times and optimize the regional flow distribution. In this paper we describe our technique for performing selective cerebral and lower body perfusion during the Norwood procedure.

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.

Congenital anomalous chordae tendinae of the mitral valve: an unusual presentation of mitral insufficiency in children.

Anomalous chordae tendinae (CT) originating from mitral valve leaflet is a rare congenital mitral valve anomaly. Our case report is unique as this anomaly is extremely rare in this pediatric age group. The anomalous CT extended from anterior mitral leaflet to the atrial septum (AS). Surgical repair in the form of anomalous CT excision, anterior leaflet chordoplasty, and posterior mitral annuloplasty was successfully performed. Congenital mitral valve (MV) leaflet or chordae anomalies are rare. In anomalous CT from MV leaflet to the AS, the surgical experience is extremely limited and only reported in adults and adolescents. We describe an unusual presentation of severe mitral insufficiency (MR) associated with anomalous CT from the anterior mitral valve leaflet (AMVL) to the AS that prompted successful repair during childhood.