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.

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.

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.

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.

Surgical palliation strategy does not affect interstage ventricular dysfunction or atrioventricular valve regurgitation in children with hypoplastic left heart syndrome and variants.

BACKGROUND: All 3 palliation strategies, Norwood, Sano, and Hybrid, currently used for hypoplastic left heart syndrome pose a risk of myocardial injury at different times and through different mechanisms. We sought to compare these strategies to understand longitudinal differences in interstage ventricular dysfunction and their subsequent impact on transplant-free survival and atrioventricular valve regurgitation (AVVR) as well as the relationship between adverse events and ventricular function. METHODS AND RESULTS: Serial echocardiographic reports and clinical data were reviewed for 138 children with hypoplastic left heart syndrome who underwent stage I surgical palliation (Sano: 11; Norwood: 73; Hybrid: 54) between 2004 and 2011. Stage II palliation was achieved in 92 (67%) patients (Sano: 7; Norwood: 51; Hybrid: 34). Interstage transplant-free survival, ventricular dysfunction, and AVVR were equivalent among palliation strategies. Patients with preserved ventricular function had a higher rate of transplant-free survival and freedom from AVVR, regardless of palliation strategy. Patients who had cardiac arrest, cardiopulmonary resuscitation, or extracorporeal membrane oxygenation (adverse events) experienced more transient and persistent ventricular dysfunction compared to those without adverse events. Surgical palliation strategies were not identified as risk factors for ventricular dysfunction or AVVR. CONCLUSIONS: Surgical palliation strategy does not affect mortality, interstage ventricular function, or interstage AVVR in children with hypoplastic left heart syndrome. Therefore, the different timing and mechanisms of myocardial injury among palliation strategies do not affect outcomes. Ventricular dysfunction adversely affects transplant-free survival and atrioventricular valve function. Adverse events are associated with the development of ventricular dysfunction. To improve outcomes, interstage treatment should focus on the preservation of ventricular function.

Surgical Melody Mitral Valve: A Paradigm Shift for Infants with Unrepairable Mitral Valve Disease.

BACKGROUND: Melody valve (Melody) for mitral valve replacement (MVR)(MelodyMVR) has been an effective strategy to treat unrepairable mitral valve disease in small children. The purpose of this study is to analyze survival, durability, and complications of MelodyMVR strategy. METHODS: Patients who underwent MelodyMVR between 2014 and 2023 were included. Transplant-free survival was analyzed with Kaplan-Meier analysis. Fine and Gray sub-distribution method was applied to quantify the cumulative incidence. RESULTS: Twenty-five patients underwent MelodyMVR. Median age and weight were 6.3 (4.4-15.2) months and 6.36 (4.41-7.57) kg. 60% had congenital mitral valve disease and 52% had dominant mitral regurgitation. The median diameter of the implanted Melody was 16 (14-18) mm. Mortality at 6 months, 1 year, and 5 years was 8.3% (95% CI, 2.2%-29.4%), 12.5% (4.2%-33.9%), and 17.6% (7.0%-40.7%), respectively. Two (8%) hospital survivors required early Melody replacement. Competing risk analysis showed that approximately 50% of patients underwent mechanical MVR by 3.5 years after MelodyMVR. Freedom from bleeding and thrombosis at 4 years was 87.5% (95%CI, 74.2%-100%). Eleven patients underwent mechanical MVR with no mortality. One (9%) required pacemaker implantation after mechanical MVR. CONCLUSIONS: MelodyMVR provides reasonable early and medium-term survival in small children and a high rate of successful bridge to mechanical MVR. MelodyMVR is associated with minimal pacemaker requirement, bleeding, and thrombosis. Early Melody functional deterioration necessitates early re-MVR, which can be achieved with minimal mortality and morbidity.

Hybrid versus Norwood strategies for single-ventricle palliation.

BACKGROUND: Hybrid and Norwood strategies differ substantially in terms of stage II palliative procedures. We sought to compare these strategies with an emphasis on survival and reintervention after stage II and subsequent Fontan completion. METHODS AND RESULTS: Of 110 neonates with functionally single-ventricle physiology who underwent stage I palliation between 2004 and 2010, 75 (69%) infants (Norwood, n=43; hybrid, n=32) who subsequently underwent stage II palliation were studied. Survival and reintervention rates after stage II palliation, anatomic and physiologic variables at pre-Fontan assessment, and Fontan outcomes were compared between the groups. Predictors for reintervention were analyzed. Freedom from death/transplant after stage II palliation was equivalent between the groups (Norwood, 80.4% versus hybrid, 85.6% at 3 years, P=0.66). Hybrid patients had a higher pulmonary artery (PA) reintervention rate (P=0.003) and lower Nakata index at pre-Fontan evaluation (P=0.015). Aortic arch and atrioventricular valve reinterventions were not different between the groups. Ventricular end-diastolic pressure, mean PA pressure, and ventricular function were equivalent at pre-Fontan assessment. There were no deaths after Fontan completion in either group (Norwood, n=25, hybrid, n=14). CONCLUSIONS: Survival after stage II palliation and subsequent Fontan completion is equivalent between the groups. The hybrid group had a higher PA reintervention rate and smaller PA size. Both strategies achieved adequate physiology for Fontan completion. Evolution of the hybrid strategy requires refinement to provide optimal PA growth.

Evolution of technology, establishment of program, and clinical outcomes in pediatric extracorporeal membrane oxygenation: the "sickkids" experience.

Technological development has had a tremendous impact on the management of patients who require extracorporeal membrane oxygenation (ECMO). Team development and education are a vital component of a successful extracorporeal life support (ECLS) Program to reduce complications and subsequently improve clinical outcomes. We sought to review the evolution in technology, importance of team development and training, and report our experience at The Hospital for Sick Children, Toronto. There were a total of 576 ECMO runs in 534 patients (42 repeat ECMO runs) between January 1988 and June 2012. The use of ECMO for cardiac disease has increased in the last decade due to an expanded indication for ECMO in patients with single-ventricle physiology. Cardiac ECMO still remains a challenge in terms of survival (177/392, 45%). Although development of an ECLS program and team education facilitated extracorporeal cardiopulmonary resuscitation, clinical outcomes were not satisfactory (survival, 33%). The most common complications were hemorrhagic (13.8%), followed by renal (10.6%) and pulmonary dysfunction (6.9%). Advances in technology made management during ECMO safer, and the mechanical complications related to the ECMO system were 6.1%, including circuit changes due to thrombus formation, cannula repositioning, or optimization of size.

Left atrial decompression during venoarterial extracorporeal membrane oxygenation for left ventricular failure in children: current strategy and clinical outcomes.

From 2005 to 2011, 23 of 178 (12.9%) patients with venoarterial (VA) extracorporeal membrane oxygenation (ECMO) had left atrial (LA) decompression to help improve left ventricular (LV) function, LA/LV dilatation, and/or lung edema. LA decompression was achieved with LA cannulation (n = 16), surgically created adjustable atrial septal defect (n = 3), or balloon atrial septostomy (n = 4). Sixteen (70%) patients had LA decompression at the time of ECMO initiation and all had LA decompression within 12 hours of ECMO initiation. ECMO duration was 5.9 ± 4.5 days and 16 (70%) patients were successfully decannulated. Subsequent intensive care unit and hospital survival was achieved in 13 (57%) and 12 (52%) patients, respectively. Earlier timing of LA decompression appeared to be associated with a high probability of weaning from ECMO and reasonable LV functional recovery.

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.

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.

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.

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.

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.