Mechanical atrioventricular valve replacement in patients with single ventricle palliation.

OBJECTIVES: Atrioventricular valve (AVV) replacements in patients with single-ventricle circulations pose significant surgical risks and are associated with high morbidity and mortality. METHODS: From 1997 to 2021, 16 consecutive patients with functionally single-ventricle physiology underwent mechanical AVV replacement. Primary outcome was transplant-free survival. Secondary outcomes included major postoperative morbidity. RESULTS: The median age of AVV replacement was 2 years old (interquartile range 0.6-3.8 years). All AVV replacements were performed with a St. Jude Medical mechanical valve, median 24 mm (range, 19-31mm). Extracorporeal membrane oxygenation (ECMO) was required in 4 patients. Operative mortality was 38% (6/16). There were 2 late deaths and 3 transplants. Transplant-free survival was 50% at 1 year, 37.5% at 5 years, and 22% at 10 years. Transplant-free survival was higher for patients with preserved ventricular function (P = 0.01). Difference in transplant-free survival at 1 year was 75% vs 25%, at 5 years was 62.5% vs 12.5% and at 10 years was 57% vs 0%. Three (19%) patients had complete heart block requiring permanent pacemaker insertion. 6 of 13 patients (46%) patients reached Fontan completion (3 patients operated at/after Fontan). Significant bleeding events occurred in 8 patients (50%) with 3 patients suffering major cerebrovascular accidents. There were 6 events of valve thrombosis in 5 patients, resulting in 2 deaths and 2 heart transplants. CONCLUSIONS: Mechanical valve replacement carries significant morbidity and mortality risk. While it successfully salvages about half of patients with preserved ventricular function, careful consideration of alternative options should be made before embarking upon mechanical valve replacement.

Melody Mitral Valve Is a Promising Alternative to Mechanical Valve Replacement for Young Children.

PURPOSE: The purpose of this study was to compare outcomes of Melody mitral valve to mechanical mitral valve replacement (MVR) for young children. DESCRIPTION: Children who underwent Melody MVR from 2014 to 2020 were case-matched to mechanical MVR patients. Transplant-free survival and cumulative incidence of reintervention were compared. A subanalysis was performed for infants aged < 1 year (9 Melody MVRs and their matches). EVALUATION: Twelve children underwent Melody MVR. Two children (17%) salvaged from mechanical support died. Five of 10 survivors (50%) had subsequent MVR. At 1 and 3 years, transplant-free survival (Melody: 83%, 83%; mechanical: 83%, 67%; P = .180) and reintervention (Melody: 9%, 39%; mechanical: 0%, 18%; P = .18) were equivalent between groups. For children < 1 year of age, Melody MVR had a modest survival benefit (Melody: 89%, 89%; mechanical: 80%, 60%; P = .046), while rate of reintervention remained equivalent (Melody: 13%, 32%; mechanical: 0%, 22%; P = .32). CONCLUSIONS: For patients < 1 year old, Melody MVR offers a promising alternative and is a reasonable bridge to mechanical MVR, which can be performed safely at an older age. Further studies are necessary to corroborate these findings.

Patients with repaired tetralogy of Fallot and the HIF1A1744C/T variant have increased imaging markers of diffuse myocardial fibrosis.

BACKGROUND: Right ventricular fibrotic remodeling has been identified pre- and postoperatively in patients with tetralogy of Fallot (ToF) and linked to adverse outcomes. Polymorphisms of hypoxia inducible factor-1-alpha (HIF1A) have been associated with the fibrotic burden by cardiac magnetic resonance (CMR) late gadolinium enhancement imaging. Their association with diffuse fibrotic myocardial remodeling is unknown. We sought to determine whether polymorphisms in HIF1A are related to CMR markers of diffuse myocardial fibrosis. METHODS: Patients with repaired ToF who had undergone CMR with T1 mapping as well as whole genome sequencing were included. Myocardial native T1 was quantified using a modified Look-Locker inversion recovery sequence and measured in the left ventricular free wall, the interventricular septum, and the right ventricular free wall. Patients who had at least one functioning allele of HIF1A were compared to those who did not using the Mann Whitney U test for continuous variables and chi-square or the Fischer test for discrete variables. RESULTS: 46 patients had both CMR and whole genome sequencing. Only one HIF1A variant was identified in the cohort and present in 13 patients. There were no significant differences in demographics, surgical variables, right or left ventricular volumes or function between patients with and without the variant. Despite a trend towards a lower age at the time of CMR (11.3 vs 13.7 years; p = 0.07), patients with HIF1A variants had higher native T1 values (1094 vs. 1050; p = 0.027) in the right ventricular outflow tract myocardium, reflecting increased diffuse interstitial ventricular fibrosis in them. CONCLUSION: Hypoxia-inducible factor is associated with imaging markers of increased diffuse right ventricular fibrosis late after repair of tetralogy of Fallot.

Challenges in diagnosis and management of coarctation of the aorta.

PURPOSE OF REVIEW: Coarctation of the aorta remains a controversial topic with uncertainties in long-term outcomes. RECENT FINDINGS: Recent advances in fetal imaging including echocardiography and MRI offer novel opportunities for better detection and prediction of the need for neonatal intervention.New imaging techniques are providing novel insights about the impact of arch geometry and size on flow dynamics and pressure gradients. The importance of arch size rather than shape for optimal hemodynamics has been identified. Long-term outcome data suggest a significant increase in mortality risk in coarctation patients beyond the third decade when compared with the general population. Hypertension is highly prevalent not only in adult patients following repair of coarctation but also in normotensive patients presenting with LV diastolic dysfunction and adverse remodelling, indicating that abnormal vascular properties are important. Patients with coarctation undergoing neonatal repair are at risk for adverse neurodevelopmental outcomes and patients could benefit from timely neurocognitive evaluation and intervention. SUMMARY: Optimizing aortic arch size, prevention and aggressive treatment of hypertension and vascular stiffening are important to improve long-term outcomes.

Risk of dispersion or aerosol generation and infection transmission with nasopharyngeal and oropharyngeal swabs for detection of COVID-19: a systematic review.

OBJECTIVES: SARS-CoV-2-related disease, referred to as COVID-19, has emerged as a global pandemic since December 2019. While there is growing recognition regarding possible airborne transmission, particularly in the setting of aerosol-generating procedures and treatments, whether nasopharyngeal and oropharyngeal swabs for SARS-CoV-2 generate aerosols remains unclear. DESIGN: Systematic review. DATA SOURCES: We searched Ovid MEDLINE and EMBASE up to 3 November 2020. We also searched the China National Knowledge Infrastructure, Chinese Medical Journal Network, medRxiv and ClinicalTrials.gov up to 29 March 2020. ELIGIBILITY CRITERIA: All comparative and non-comparative studies that evaluated dispersion or aerosolisation of viable airborne organisms, or transmission of infection associated with nasopharyngeal or oropharyngeal swab testing. RESULTS: Of 7702 citations, only one study was deemed eligible. Using a dedicated sampling room with negative pressure isolation room, personal protective equipment including N95 or higher masks, strict sterilisation protocols, structured training with standardised collection methods and a structured collection and delivery system, a tertiary care hospital proved a 0% healthcare worker infection rate among eight nurses conducting over 11 000 nasopharyngeal swabs. No studies examining transmissibility with other safety protocols, nor any studies quantifying the risk of aerosol generation with nasopharyngeal or oropharyngeal swabs for detection of SARS-CoV-2, were identified. CONCLUSIONS: There is limited to no published data regarding aerosol generation and risk of transmission with nasopharyngeal and oropharyngeal swabs for the detection of SARS-CoV-2. Field experiments to quantify this risk are warranted. Vigilance in adhering to current standards for infection control is suggested.

High-flow nasal cannula for acute hypoxemic respiratory failure in patients with COVID-19: systematic reviews of effectiveness and its risks of aerosolization, dispersion, and infection transmission.

PURPOSE: We conducted two World Health Organization-commissioned reviews to inform use of high-flow nasal cannula (HFNC) in patients with coronavirus disease (COVID-19). We synthesized the evidence regarding efficacy and safety (review 1), as well as risks of droplet dispersion, aerosol generation, and associated transmission (review 2) of viral products. SOURCE: Literature searches were performed in Ovid MEDLINE, Embase, Web of Science, Chinese databases, and medRxiv. Review 1: we synthesized results from randomized-controlled trials (RCTs) comparing HFNC to conventional oxygen therapy (COT) in critically ill patients with acute hypoxemic respiratory failure. Review 2: we narratively summarized findings from studies evaluating droplet dispersion, aerosol generation, or infection transmission associated with HFNC. For both reviews, paired reviewers independently conducted screening, data extraction, and risk of bias assessment. We evaluated certainty of evidence using GRADE methodology. PRINCIPAL FINDINGS: No eligible studies included COVID-19 patients. Review 1: 12 RCTs (n = 1,989 patients) provided low-certainty evidence that HFNC may reduce invasive ventilation (relative risk [RR], 0.85; 95% confidence interval [CI], 0.74 to 0.99) and escalation of oxygen therapy (RR, 0.71; 95% CI, 0.51 to 0.98) in patients with respiratory failure. Results provided no support for differences in mortality (moderate certainty), or in-hospital or intensive care length of stay (moderate and low certainty, respectively). Review 2: four studies evaluating droplet dispersion and three evaluating aerosol generation and dispersion provided very low certainty evidence. Two simulation studies and a crossover study showed mixed findings regarding the effect of HFNC on droplet dispersion. Although two simulation studies reported no associated increase in aerosol dispersion, one reported that higher flow rates were associated with increased regions of aerosol density. CONCLUSIONS: High-flow nasal cannula may reduce the need for invasive ventilation and escalation of therapy compared with COT in COVID-19 patients with acute hypoxemic respiratory failure. This benefit must be balanced against the unknown risk of airborne transmission.

RéSUMé: OBJECTIF: Nous avons réalisé deux comptes rendus sur commande de l'Organisation mondiale de la santé pour guider l'utilisation de canules nasales à haut débit (CNHD) chez les patients ayant contracté le coronavirus (COVID-19). Nous avons synthétisé les données probantes concernant leur efficacité et leur innocuité (compte rendu 1), ainsi que les risques de dispersion des gouttelettes, de génération d'aérosols, et de transmission associée d'éléments viraux (compte rendu 2). SOURCE: Des recherches de littérature ont été réalisées dans les bases de données Ovid MEDLINE, Embase, Web of Science, ainsi que dans les bases de données chinoises et medRxiv. Compte rendu 1 : nous avons synthétisé les résultats d'études randomisées contrôlées (ERC) comparant les CNHD à une oxygénothérapie conventionnelle chez des patients en état critique atteints d'insuffisance respiratoire hypoxémique aiguë. Compte rendu 2 : nous avons résumé sous forme narrative les constatations d'études évaluant la dispersion de gouttelettes, la génération d'aérosols ou la transmission infectieuse associées aux CNHD. Pour les deux comptes rendus, des réviseurs appariés ont réalisé la sélection des études, l'extraction des données et l'évaluation du risque de biais de manière indépendante. Nous avons évalué la certitude des données probantes en nous fondant sur la méthodologie GRADE. CONSTATATIONS PRINCIPALES: Aucune étude éligible n'incluait de patients atteints de COVID-19. Compte rendu 1 : 12 ERC (n = 1989 patients) ont fourni des données probantes de certitude faible selon lesquelles les CNHD réduiraient la ventilation invasive (risque relatif [RR], 0,85; intervalle de confiance [IC] 95 %, 0,74 à 0,99) et l'intensification de l'oxygénothérapie (RR, 0,71; IC 95 %, 0,51 à 0,98) chez les patients atteints d'insuffisance respiratoire. Les résultats n'ont pas démontré de différences en matière de mortalité (certitude modérée), ni de durée du séjour hospitalier ou à l'unité des soins intensifs (certitude modérée et faible, respectivement). Compte rendu 2 : quatre études évaluant la dispersion de gouttelettes et trois évaluant la génération et la dispersion d'aérosols ont fourni des données probantes de très faible certitude. Deux études de simulation et une étude croisée ont donné des résultats mitigés quant à l'effet des CNHD sur la dispersion des gouttelettes. Bien que deux études de simulation n'aient rapporté aucune augmentation associée concernant la dispersion d'aérosols, l'une a rapporté que des taux de débit plus élevés étaient associés à des régions à densité d'aérosols élevée plus grandes. CONCLUSION: Les canules nasales à haut débit pourraient réduire la nécessité de recourir à la ventilation invasive et l'escalade des traitements par rapport à l'oxygénothérapie conventionnelle chez les patients atteints de COVID-19 souffrant d'insuffisance respiratoire hypoxémique aiguë. Cet avantage doit être soupesé contre le risque inconnu de transmission atmosphérique.

Fontan Failure and Death in Contemporary Fontan Circulation: Analysis From the Last Two Decades.

BACKGROUND: We sought to evaluate the incidence of Fontan failure or complication and its relation to death in patients having contemporary Fontan strategies over 2 decades. METHODS: Five hundred patients who underwent Fontan completion (extracardiac, n = 326; lateral tunnel, n = 174) from 1985 to 2012 were reviewed. Patient characteristics, modes of Fontan failure/complication and death, and predictors for Fontan failure/complication and death were analyzed. RESULTS: There were 23 early deaths (4.6%) and 17 late deaths (3.4%), with no early death since 2000. Survival has improved over time (p < 0.001). Twenty-three of 40 patients who died were identified as Fontan failure before death, including ventricular dysfunction (n = 14), pulmonary vascular dysfunction (n = 4), thromboembolism (n = 2), and arrhythmia (n = 4). Mode of death was circulatory failure (n = 18), multiorgan failure (n = 6), pulmonary failure (n = 3), cerebral/renal (n = 5), and sudden death (n = 4). Modes of failure/complication were directly (65%) or conceivably (10%) related to death in 30 of 40 patients (75%). Forty-eight percent of survivors had late Fontan complication(s). Five-year freedom from late Fontan complication was lower among patients who died compared with patients who survived (29.4% versus 53.3%, p < 0.001). Ventricular dysfunction (p = 0.001) and higher pulmonary artery pressures (p < 0.001) after Fontan were predictors for death. Longer cardiopulmonary bypass time (p = 0.032) and reinterventions (p < 0.001) were predictors for late Fontan complication. CONCLUSIONS: Early death in the early era has been overcome. Yet the incidence and causes of late death remain unchanged. There was a strong causative relationship between the mode of Fontan failure/complication and death, indicating the importance of early recognition and treatment of Fontan failure/complication.

Successful Treatment of Pediatric Ventricular Assist Device Thrombosis.

Pump thrombosis represents a significant cause of morbidity and mortality in patients on continuous flow ventricular assist devices (CF-VAD). Pump thrombosis in the pediatric CF-VAD population has been reported between 11% and 44%, with the largest reported series from the PediMACS registry reporting a rate of approximately 15%. We report our early experience with four pediatric patients who developed pump thrombosis on a CF-VAD. Our limited experience suggests that the treatment of pediatric VAD thrombosis can be approached with similar principles to the adult population. Our current strategy includes:i. Initiating treatment with bivalirudin for an isolated rise in lactate dehydrogenase (LDH) with no corresponding rapid rise in plasma-free hemoglobin which may prevent further progression.ii. Treatment with a low-dose systemic tissue plasminogen activator (TPA) protocol as opposed to targeted therapy via catheter intervention if bivalirudin fails.iii. If there are concerns with respect to the impact of hemolysis on kidney function or the patient is close to a previous surgery, device exchange can be considered.The balance between achieving appropriate anticoagulation/antiplatelet therapy in the face of bleeding/hemorrhagic complications remains a challenge. There is a need for larger studies in the pediatric population to outline an algorithm for the definitive management of VAD thrombosis.

Geometry and growth of the reconstructed aorta in patients with hypoplastic left heart syndrome and variants.

OBJECTIVE: The interdigitating technique in aortic arch reconstruction in hypoplastic left heart syndrome and variants (HLHS) reduces the recoarctation rate. Little is known on aortic arch growth characteristics and resulting clinical impact. METHODS: A total of 139 patients with HLHS underwent staged palliation between 2007 and 2014; 73 patients underwent arch reconstruction. Dimensions of ascending aorta, transverse arch, interdigitating anastomosis, and descending aorta in pre-stage II and pre-Fontan angiograms were measured. Aortic arch dimensions were analyzed. Ventricular and atrioventricular valve function were assessed. RESULTS: Diameters increased in all segments between pre-stage II and pre-Fontan (P < .0005). The z scores remained unchanged in all segments but the descending aorta that was significantly larger pre-Fontan (P = .039). Dimensions and z scores between pre-stage II and pre-Fontan correlated in proximal segments, but not at and distal to the interdigitating anastomosis. Pronounced tapering occurred between the transverse arch and the interdigitating anastomosis. Arch intervention of any type was performed in 7 (9.6%), and intervention for recoarctation in 3 (4.1%) patients. CONCLUSIONS: The aortic arch after reconstruction with the interdigitating technique differs from normal. Growth was proportional with no further geometrical distortion. Recoarctation and reintervention rate is low. Further improvement may be achieved by optimizing patch configuration and material.

Pulmonary flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

BACKGROUND: We hypothesized that mean pulmonary artery pressure (PAP) detected on a pulmonary flow study may predict medium-term survival and right ventricular systolic pressure (RVSP) in patients with pulmonary atresia (PA), ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs). METHODS: Fifty patients with PA/VSD/MAPCAs underwent unifocalization between 2000 and 2013, and 40 of these patients had a pulmonary flow study since 2003. Predictability of the mean PAP on VSD status, medium-term survival, reintervention, and RVSP were analyzed. RESULTS: Forty-seven of the 50 patients (94%) had complete unifocalization at a median age of 11 months (range, 1-194 months), and 37 patients (74%) achieved VSD closure. Among the 40 patients who underwent a pulmonary flow study, the VSD was closed in 34 (85%), with salvage VSD fenestration in 4 (10%), and was intentionally left open in 6 (15%). Survival was 85.5% at 1 year and 78.5% at 5 years. A mean PAP ≥25 mm Hg was associated with worse survival (P = .011). Cox regression analysis identified a mean PAP ≥25 mm Hg as the sole predictor for death (P = .037). Patients with an open VSD had an increased risk of reoperation (P = .001) and pulmonary artery reintervention (P = .010), and had a trend toward increased risk of death (P = .059), compared with those with a closed VSD. CONCLUSIONS: PAP obtained from the intraoperative pulmonary flow study is associated with medium-term survival and late RVSP in patients with PA/VSD/MAPCAs. VSD closure for patients with a mean PAP ≥25 mm Hg on a flow study is considered high risk, and sensible judgment and a low threshold for VSD fenestration are required.

Geometry of the Aortic Arch After Initial Hybrid or Norwood Palliation.

BACKGROUND: Aortic arch reconstruction is a challenging technical step in the Norwood operation or the comprehensive stage II operation. This study sought to analyze differences in aortic arch geometry and dimensions in patients undergoing Norwood or hybrid palliation. METHODS: Retrospective data collection included all patients who underwent Norwood or hybrid palliation at the Hospital for Sick Children in Toronto between 2007 and 2014; 139 patients were analyzed. Lateral angiograms obtained before stage II or comprehensive stage II and Fontan completion were measured at the level of the ascending aorta, transverse arch, isthmus, and descending aorta. Reintervention rate and type were assessed. RESULTS: Before stage II or comprehensive stage II hybrid procedures, patients had significantly larger descending aorta z-scores. Patients undergoing Norwood operations showed a significant increase in descending aorta z-scores before Fontan completion. Comparable dimensions (absolute and z-scores) were found at all points of measurements before Fontan completion. Geometry was similar in both groups but significantly different compared with normal dimensions. Reduction in aortic arch diameter happened almost solely in the segment between the transverse arch and the isthmus. Stent inclusion in patients undergoing hybrid procedures led to similar dimensions. Reintervention rates were very low (Norwood 9.6% vs hybrid 7.6%). Reintervention for excessive dimensions was as common as for recoarctation. CONCLUSIONS: Aortic arch geometry and growth is not altered by palliation type. The increase in descending aorta diameter seen in patients undergoing Norwood operations is in accord with physiologic changes and may reflect catch-up growth. Reintervention rates are low and are not related to recoarctation alone.

Impact of Norwood versus hybrid palliation on cardiac size and function in hypoplastic left heart syndrome.

OBJECTIVE: The hybrid approach for hypoplastic left heart syndrome (HLHS) could theoretically result in better preservation of right ventricular (RV) function then the Norwood procedure. The aim of this study was to compare echocardiographic indices of RV size and function in patients after Norwood and hybrid throughout all stages of palliation. METHODS: 76 HLHS patients (42 Norwood, 34 hybrid) were retrospectively studied. Echocardiography was obtained before stage I, before and after stage II, and before and after Fontan. Median follow-up was 4.9 years (range 1.1-8.5). RESULTS: Baseline characteristics before stage I were similar. Hybrid patients demonstrated a significant decrease in RV fractional area change (FAC) between baseline and pre-stage II (36±9% vs 27±6%; p<0.01); Norwood patients remained stable (32±10% vs 32±7%; p=0.21). At pre-stage II, moderate/severe tricuspid valve (TV) regurgitation was found in nine Norwood (33%) and four hybrid (18%) patients (p=0.19). After stage II, the difference in FAC became insignificant (29±7% vs 25±8%, p=0.08) and moderate/severe TV regurgitation (TR) was found in 13 Norwood (48%) and four hybrid patients (19%) (p=0.18). At pre-Fontan, RV FAC was similar after Norwood and hybrid (34±5% vs 33±6%, p=0.69), which remained unchanged after Fontan. After Fontan, one Norwood and one hybrid patient had moderate TR. RV and TV size were similar for both groups at each time point. CONCLUSIONS: Patients after Norwood and hybrid procedures had equivalent indices of RV size, and systolic and diastolic function throughout all stages of palliation. Small differences in individual RV and TV indices are likely to be explained by differences in physiology or surgical timing rather than by intrinsic differences in myocardial and valve function.

Outcomes in Patients with Persistent Ventricular Dysfunction After Stage I Palliation for Hypoplastic Left Heart Syndrome.

We sought to describe the clinical course for patients with hypoplastic left heart syndrome and persistent ventricular dysfunction and identify risk factors for death or transplantation before stage II palliation. 138 children undergoing stage I palliation from 2004 to 2011 were reviewed. Twenty-two (16 %) patients (seven Hybrid, 15 Norwood) with two consecutive echocardiograms reporting at least moderate dysfunction were included and compared to case-matched controls. Eleven of the 22 patients with dysfunction (50 %) underwent stage II, seven (32 %) were transplanted, and four (18 %) died prior to stage II. Of the patients who survived to hospital discharge (n = 17) following stage 1, 14 (82 %) required readmission for heart failure (HF) compared to only two (10 %) for controls (p < 0.001). Among patients with ventricular dysfunction, there was an increased use of ACE inhibitors or beta-blockers (82 vs. 25 %; p = 0.001), inotropes (71 vs. 15 %; p = 0.001), ventilation (58 vs. 10 %; p = 0.001), and ECMO (29 vs. 0 %; p = 0.014) for HF management post-discharge when compared to controls. There was a lower heart transplant-free survival at 7 months in patients with dysfunction compared to controls (50.6 vs. 90.9 %; p = 0.040). ECMO support (p = 0.001) and duration of inotropic support (p = 0.04) were significantly associated with death or transplantation before stage II palliation. Patients with ventricular dysfunction received more HF management and related admissions. Longer inotropic support should prompt discussion regarding alternative treatment strategies given its association with death or transplant.

Repair Type Influences Mode of Pulmonary Vein Stenosis in Total Anomalous Pulmonary Venous Drainage.

BACKGROUND: We hypothesized that primary sutureless (SL) repair of total anomalous pulmonary venous drainage (TAPVD) may have a lower incidence of postrepair pulmonary vein obstruction (PVO) and different modes of PVO compared with standard repair (SR). METHODS: One hundred ninety-five patients who underwent TAPVD repair (1990 to 2012) with the exception of congenital pulmonary vein stenosis, isomerism, and single-ventricle anomalies were included. Survival, reintervention, incidence, degree of PVO were compared between groups. The mode of PVO was expressed as central or peripheral. The Mann-Whitney test, Kaplan-Meier analysis, and Cox regression were used. RESULTS: The SL group had more infracardiac or mixed TAPVD (p = 0.02) and preoperative PVO (p = 0.07). There were no differences between SR and SL groups in survival (5-year survival, 83.1% versus 82.5%, respectively; p = 0.73) and composite outcome (death, intervention, PVO, 5-year survival, 76.4% versus 80.7%, respectively; p = 0.225). The SL group had a lower incidence of PVO of moderate or greater degree (SR, 11.3% versus SL, 2.9%; p = 0.05) than the SR group, especially in the infracardiac and mixed TAPVD cohort (p = 0.011), with a lower pulmonary vein score (SR, 8 versus SL, 4; p = 0.01). The SL group had peripheral PVO exclusively (100%), whereas the SR group predominantly had central PVO (76.4%; p = 0.005). There was a trend toward less reoperation in the SL group (SR, 10.4% versus SL, 2.9%; p = 0.08). Survival after reoperation was comparable to primary TAPVD repair types as well as reoperation repair types. CONCLUSIONS: Primary SL appeared to be associated with a lower incidence and severity of PVO. The primary SL repair eliminated the risk of developing central PVO, although a relatively benign type of peripheral PVO could occur.

Longer- Versus Shorter-Duration Dual-Antiplatelet Therapy After Drug-Eluting Stent Placement: A Systematic Review and Meta-analysis.

BACKGROUND: The appropriate duration of dual-antiplatelet therapy (DAPT) after drug-eluting stent (DES) placement remains controversial. PURPOSE: To summarize data on clinical outcomes with longer- versus shorter-duration DAPT after DES placement in adults with coronary artery disease. DATA SOURCES: Ovid MEDLINE and EMBASE, 1996 to 27 March 2015, and manual screening of references. STUDY SELECTION: Randomized, controlled trials comparing longer- versus shorter-duration DAPT after DES placement. DATA EXTRACTION: Two reviewers screened potentially eligible articles; extracted data on populations, interventions, and outcomes; assessed risk of bias; and used the Grading of Recommendations Assessment, Development and Evaluation guidelines to rate overall confidence in effect estimates. DATA SYNTHESIS: Among 1010 articles identified, 9 trials including 29,531 patients were eligible; data were complete for 28,808 patients. Moderate-quality evidence showed that longer-duration DAPT decreased risk for myocardial infarction (risk ratio [RR], 0.73 [95% CI, 0.58 to 0.92]) and increased mortality (RR, 1.19 [CI, 1.04 to 1.36]). High-quality evidence showed that DAPT increased risk for major bleeding (RR, 1.63 [CI, 1.34 to 1.99]). LIMITATION: Confidence in estimates were decreased owing to imprecision for most outcomes (particularly myocardial infarction), risk of bias from limited blinding in 7 of 9 studies, indirectness due to variability in use of first- and second-generation stents, and off-protocol use of DAPT in some studies. CONCLUSION: Extended DAPT is associated with approximately 8 fewer myocardial infarctions per 1000 treated patients per year but 6 more major bleeding events than shorter-duration DAPT. Because absolute effects are very small and closely balanced, decisions regarding the duration of DAPT therapy must take into account patients' values and preference. PRIMARY FUNDING SOURCE: None.

Persistent fenestration may be a marker for physiologic intolerance after Fontan completion.

BACKGROUND: We sought to evaluate the medium-term implications of fenestration status. METHODS: Between 1994 and 2012, 326 patients received an extracardiac Fontan (hospital mortality n = 6, 1.8%). A fenestration was routinely created (n = 306, 94%) unless there was technical difficulty. Three hundred patients discharged with an open fenestration were included. The primary end points were death and Fontan failure. Secondary outcomes were Fontan complications such as venovenous collaterals, protein-losing enteropathy, pacemaker requirement, and arrhythmias. RESULTS: The fenestration was closed in 260 patients: 185 as a catheter intervention (62%) and 75 (25%) spontaneously. Forty patients (13%) had the fenestration open at a median follow-up period of 5.05 years. Of these patients, catheter-based closure failed in 10 (3%). There was no statistically significant difference in pre-Fontan hemodynamic parameters, such as pulmonary artery pressure and pulmonary vascular resistance between the patients with open fenestration and the ones with closed fenestration. Patients with an open fenestration had significantly more late deaths (P < .001), Fontan failure (P = .021), and Fontan complications (P = .011) compared with those with a closed fenestration. Multivariable Cox regression revealed open fenestration (P < .001) and indeterminate ventricular morphology (P = .002) as risk factors for death/Fontan failure, and ventricular dysfunction (P = .014) and open fenestration (P = .009) as risk factors for Fontan complications. CONCLUSIONS: Persistent fenestration was a marker for physiologic intolerance as noted by increased rates of mortality and a higher incidence of Fontan failure/complications. The specificity of pre-Fontan physiologic data for fenestration status may not have the fidelity needed for long-term care and thus, the consequences of decision making regarding fenestration status may not be determined until well after the operation.

Fate of the hypoplastic proximal aortic arch in infants undergoing repair for coarctation of the aorta through a left thoracotomy.

BACKGROUND: Extended end-to-end anastomosis (EEEA) through a left thoracotomy for coarctation of the aorta (CoA) and tubular hypoplasia of the aortic arch (THAA) leaves an unaugmented hypoplastic proximal aortic arch (PAA) segment, which may increase late reintervention for PAA obstruction. We sought to assess PAA growth and reintervention for PAA obstruction after EEEA. METHODS: Preoperative and follow-up echocardiographic images of 140 patients who underwent EEEA for CoA from 2005 to 2012 were reviewed. Patients were divided into two groups on the basis of preoperative PAA z-scores: THAA group, z-score less than -3; non-THAA group, z-score greater than or equal to -3. RESULTS: Eighty (57%) patients were identified as having THAA. There were three surgical reinterventions (PAA in 2 patients and distal aortic arch in 1 patient) and nine catheter reinterventions (all related to anastomotic stenosis) during a median follow-up period of 18 months. Both patients who required PAA reintervention had preoperative PAA z-scores below -8. Freedom from reintervention at 3 years was comparable between the groups (THAA group, 90.0% vs non-THAA group, 87.9%, p = 0.483). Follow-up echocardiography revealed PAA catch-up growth in the THAA group (z-score, preoperative -4.63 vs follow-up -1.17, p < 0.001); however, there was a nonsignificant trend toward smaller PAA in the THAA group (z-score: THAA, -1.17 vs non-THAA, -0.55, p = 0.057). All but 2 patients with preoperative PAA z-scores above -6 did not have any PAA obstruction. CONCLUSIONS: The hypoplastic PAA segment in patients with CoA/THAA grew significantly after EEEA but remained smaller than in those without THAA. Our data support that CoA and PAA with z-scores as small as -6 can be repaired through a thoracotomy approach with a low risk of reintervention.