Superior Vena Cava Inflow Following Repair for Anomalous Right Pulmonary Venous Drainage in Children.

Risk of superior vena cava (SVC) obstruction following repair of anomalous right upper pulmonary veins in children is unclear. The incidence and outcome of subclinical obstruction remained unknown. Retrospective single institutional study (07/1993-02/2017) in a pediatric population (N = 42, median age 3.9-year, range 0.1-15.3 years). 33 (79%) children had repair without SVC translocation ("non-Warden") and 9 (21%) had Warden-type surgery. Echocardiographic SVC obstruction was defined as (I) turbulent flow across SVC and (II) continuous flow pattern without return to baseline velocity (0 m/s); severe obstruction was defined as loss of distinct biphasic profile ± mean gradient ≥ 5 mmHg. 3 (7%) patients required intra-operative revision due to obstruction (non-Warden: 1, Warden: 2). After discharge, 2 (5%) patients required reintervention (3 and 6-month post-op) for severe symptomatic obstruction (non-Warden: 1, Warden-type: 1). Both patients responded to balloon angioplasty with symptomatic resolution (one required repeat catheter reintervention). 10 (24%) patients had subclinical echocardiographic obstruction (2, 22% Warden vs. 8, 24% non-Warden; p = 1.0; 8 of 10 patients had mild gradient), which resolved and remained well without reintervention. At follow-up (mean 7.2-year, range 0-23 years), all patients were alive. Freedom from SVC reintervention at 10 and 20-year is 95% (97% at 10, 20-year in non-Warden and 89% at 5, 8-year in Warden-type group; log-rank p = 0.34). Surgical repair for anomalous right upper pulmonary veins is associated with risk of SVC obstruction in children. The need for reintervention for severe obstruction is rare at late follow-up. Patients with subclinical obstruction remain asymptomatic and demonstrate echocardiographic improvement.

The impact of surgical repair on left ventricular outflow tract in atrioventricular septal defect with common atrioventricular valve orifice.

Objective: Although a narrow left ventricular outflow tract in atrioventricular septal defect is related to its intrinsic morphology, the contribution from the repair technique remains to be quantified. Methods: A total of 108 patients with an atrioventricular septal defect with a common atrioventricular valve orifice were divided into 2 groups: 2-patch (N = 67) and modified 1-patch (N = 41) repair. The left ventricular outflow tract morphometric was analyzed by quantifying the degree of disproportion between subaortic and aortic annular dimensions (disproportionate morphometrics ratio was defined as ≤ 0.9). Z-scores (median, interquartile range) were further analyzed in a subset of 80 patients with immediate preoperative and postoperative echocardiography. A total of 44 subjects with ventricular septal defects served as controls. Results: Before repair, 13 patients (12%) with an atrioventricular septal defect had disproportionate morphometrics (vs 6 [14%] ventricular septal defect P = .79), but the subaortic Z-score (-0.53, -1.07 to 0.06) was lower than the ventricular septal defect (0.07, -0.57 to 1.17; P < .001). After repair, both 2-patch (8 [12%] preoperatively vs 25 [37%] postoperatively; P = .001) and modified 1-patch (5 [12%] vs 21 [51%], P < .001) procedures showed a greater degree of disproportionate morphometrics. Both 2-patch (postoperatively -0.73, -1.56 to 0.08 vs preoperatively -0.43, -0.98 to 0.28; P = .011) and modified 1-patch (-1.42, -2.63 to -0.78 vs -0.70, -1.18 to -0.25; P = .001) procedures also demonstrated lower subaortic Z-scores postrepair. The postrepair subaortic Z-scores were lower in the modified 1-patch group (-1.42 [-2.63 to -0.78]) compared with the 2-patch group (-0.73 [-1.56 to 0.08]; P = .004). Low postrepair subaortic Z-scores (<-2) were observed in 12 patients (41%) in the modified 1-patch group and 6 patients (12%) in the 2-patch group (P = .004). Conclusions: Surgical correction resulted in greater disproportionate morphometrics seen immediately postrepair. The impact on the left ventricular outflow tract was observed in all repair techniques, with a greater burden seen after modified 1-patch repair. Video Abstract: This morphometric study in AVSD with common atrio-ventricular valve orifice confirmed further derangements of LV outflow tract morphometrics immediately after surgical repair.

The role of abnormal subaortic morphometry as a substrate for left ventricular outflow tract obstruction following atrioventricular septal defect repair.

OBJECTIVES: Although left ventricular outflow tract (LVOT) obstruction is a recognized risk after atrioventricular (AV) septal defect (AVSD) repair, quantitative assessments to define the substrate of the obstruction are lacking. METHODS: Morphometric analyses were based on measurements from early 2-dimensional echocardiographic scans (within 3 months postoperatively) for 117 patients (82 CAVVO = common AV valve; 35 SAVVO = separate AV valve orifices), which were compared to 50 age/weight matched controls (atrial septal defect/ventricular septal defect). Late echocardiographic analyses were performed in 57 patients with AVSD (follow-up range, 1.2-10.7 years). RESULTS: Adequate z scores (above -2.5) were observed in 109 (93%) patients with AVSD at the aortic annulus and in 89 (76%) with AVSD in the subaortic area. Compared to the control group, patients with AVSD had lower median z scores at the aortic annulus (-0.64 vs 0.60; P < 0.001) and the subaortic areas (-1.48 vs 0.59; P < 0.001), disproportionate subaortic/aortic annulus ratio <1.00 (67% vs 22%; P < 0.001), narrower annuloaortic-septal angle (94.0 vs 104.0; P < 0.001) and annuloaortic left AV valve angle (78.0 vs 90.0; P < 0.001). Compared to patients with CAVVO, those with SAVVO had narrower annuloaortic-septal angles (P = 0.022) that persisted at late analysis, with lower subaortic/aortic annular ratios (P = 0.039). In patients with CAVVO, lower early postoperative subaortic z scores were found following modified single-patch repairs (median -2.12 vs -1.02 in two-patch repairs; P = 0.004). A total of 6/117 (5%) patients (4 CAVVO, 5% and 2 SAVVO, 6%) required reoperations for LVOT obstruction (mean 6.9 years postoperatively), with no difference in morphology or types of operations. CONCLUSIONS: Despite having adequate z scores, patients with AVSD demonstrated abnormal LVOT morphometrics early postoperatively. Besides intrinsic morphology, repair techniques may have an impact on postoperative LVOT morphometrics and requires further evaluation.

Remote ALCAPA Repair-Total Arterial Reconstruction Using Free Aortic Graft Technique.

A late presenting anomalous left coronary artery from pulmonary artery (ALCAPA) with remote origin may be associated with several technical caveats due to distance for coronary transfer and inadequate autologous tissues for reconstruction. A technique using full circumferential aortic wall as a free graft that is sutured as a posterior hood on an anterior pulmonary arterial flap is used to achieve reconstruction of a neo-left coronary that is tension free, with laminar flow and without the use of any prosthetic material. The technique with potential modifications described could potentially be applied to any variant of ALCAPA to achieve total arterial reconstruction to yield an optimal long-term outcome.

Surgical Approach for Hypoplastic Mirror Circumflex Aortic Arch.

Surgical approach for the mirror-form of circumflex aortic arch with a long-segment hypoplasia is described. Instead of "uncrossing" the arch, posterior aortic translocation of the aorta is required for the mirror-form. These surgical strategies addressed all the associated patho-mechanisms: (1) relief of vascular ring, (2) removal of posterior arch compression on the trachea, and (3) correction of any arch obstruction. Posterior tracheobronchopexy was further required to address severe underlying tracheobronchomalacia.

Restrictive Cardiomyopathy or Constrictive Pericarditis: An Unresolved Conundrum.

Cardiomyopathy may have a variety of causes and may lead to significant morbidity. Often, there is no "perfect" treatment. New investigative techniques may add insight but retain the possibility of uncertainty. The distinction between restrictive cardiomyopathy and pericardial constriction may be challenging, particularly when considering the incidence of these entities. This distinction may significantly impact patient management and this is becoming increasingly important in the context of donor organ austerity. We present a case of a 17-year-old male to illustrate the overlap highlighting this debate and our subsequent management.

Extracorporeal membrane oxygenation in pediatric cardiac surgery: A retrospective review of trends and outcomes in Scotland.

INTRODUCTION: Around 3.2%-8.4% of patients receive venoarterial (VA) extracorporeal membrane oxygenation (ECMO) support after pediatric cardiac surgery. The desired outcome is "bridge-to-recovery" in most cases. There is no universally agreed protocol, and given the associated costs and complications rates, the decisions as of when and when not to institute VA ECMO are largely empirical. METHODS: A retrospective review of the ECMO database at the Scottish Pediatric Cardiac Services (SPCS) was undertaken. Inclusion criterion encompassed all children (<16 years of age) who were supported with VA ECMO following cardiac surgery between January 2011 and October 2016. The timing of ECMO support was divided into three distinct phases: "endofcase" or intheatre ECMO for patients unable to effectively wean from cardiopulmonary bypass (CPB), ECMO for cardiopulmonary resuscitation ("ECPR"), and Intensive Care Unit ECMO for "failing maximal medial therapy" following cardiac surgery. The patients were analyzed to identify survival rates, adverse prognostic indicators, and complication rates. RESULTS: We identified 66 patients who met the inclusion criterion. 30day survival rate was 45% and survival rate to hospital discharge was 44% (the difference represents one patient). On followup (median: 960 days, range: 42-2010 days), all survivors to hospital discharge were alive at review date. "End-of-case" ECMO showed a trend toward better survival of the three subcategories ("end of case," ECPR, and ECMO for "failing maximal medical therapy" survival rates were 47%, 41%, and 37.5%, respectively, P = 0.807). The poorest survival rates were in the younger children (<6 months, P = 0.502), patients who had prolonged CPB (P = 0.314) and aortic crossclamp times (P = 0.146), and longer duration of ECMO (>10 days, P = 0.177). CONCLUSIONS: Allcomers VA ECMO following pediatric cardiac surgery had survival to discharge rate of 44%. Elective "end-of-case" ECMO carries better survival rates and therefore ECMO instituted early maybe advantageous. Prolonged ECMO support has a direct correlation with mortality.

Successful HeartWare Bridge to Recovery in a 3-Year Old: A Game Changer?

We report a 3-year-old boy weighing 13.5 kg who presented with intractable cardiac failure resulting from myocarditis and was treated by implantation of a HeartWare (HVAD) device. He was discharged home with the device. His cardiac function subsequently recovered, and the device was decommissioned. We believe this to be the youngest HVAD recipient and the only child to have recovered and had the device decommissioned.

An Extended Role of Continuous Flow Device in Pediatric Mechanical Circulatory Support.

BACKGROUND: Mechanical circulatory support in the pediatric population is currently limited to pulsatile ventricular assist devices (VAD). In recent years, the use of durable, newer generation, continuous flow devices have increased substantially among adults with end-stage heart failure. We examined the extended role of this device in the pediatric population (aged less than 18 years). METHODS: Between 2010 and 2015, 12 patients (median age 7.1 years; range, 3.7 to 17.0; one third of patients were aged 5 years or less) received a HeartWare ventricular assist device (HVAD; HeartWare, Framingham, MA), 11 for cardiomyopathy and 1 for posttransplant rejection. Right VAD support (n = 5; 42%) was provided by a short-term device (Levitronix, Zurich, Switzerland). RESULTS: Overall, 1 patient died (day 638), 8 patients (67%) underwent transplantation, 1 patient (8.3%) recovered, and 2 patients (17%) remain on HVAD. The mean length of support was 150 days (range, 16 to 638). Four patients (33.3%) were discharged home (all left VAD). In the left VAD group (n = 7), 3 patients subsequently received transplants (days 185, 201, and 234, respectively), 1 recovered (day 149), 1 died (day 638), 1 remained on HVAD (day 198), and 1 needed conversion to biventricular assist device (BIVAD [day 73]). In the BIVAD group (n = 5), right VAD was weaned in 3 (60%), all subsequently received transplants, and 2 remained on BIVAD support until transplant (days 16 and 17, respectively). One BIVAD patient required conversion to central cannulation for longer-term support. Four BIVAD patients (80%) were in Interagency Registry for Mechanically Assisted Circulatory Support level 1 before VAD compared with 2 (29%) in the left VAD group (p = not significant). The actuarial survival rate was 100% at 1 year with no neurologic events. CONCLUSIONS: The third-generation, continuous flow device can provide durable support in the pediatric population. The selection strategy for patients who benefit most from the device continues to evolve. It is anticipated that a smaller design in the future will benefit an even wider pediatric population with heart failure.

Epicardial implantation of a transvenous implantable cardioverter-defibrillator system in Ebstein's malformation with tricuspid valve repair.

In this case report, we describe the implant of a transvenous implantable cardioverter-defibrillator lead on the left ventricular epicardial surface of a young patient with Ebstein's anomaly, facilitating effective defibrillation and anti-tachycardia pacing. The difficulties of conventional placement in Ebstein's malformation as well as the technique and outcome of epicardial lead implantation of a transvenous defibrillator-pacer system are discussed. .

Three-Sinus Repair of Elastin Arteriopathy Associated Supravalvar Pulmonary Stenosis With Bilateral Branch Pulmonary Artery Involvement.

Elastin arteriopathy-associated supravalvar pulmonary stenosis is characterized by a thick intraluminal ringlike obstruction at the sinotubular junction of the pulmonary valve. Extension of disease into the branch pulmonary arteries is common. A three-sinus pulmonary trunk augmentation combined with bilateral branch pulmonary augmentation is presented. This approach normalizes the pulmonary trunk and allows optimal augmentation of the central pulmonary arteries.

Training the novice to become cardiac surgeon: does the "early learning curve" training compromise surgical outcomes?

OBJECTIVE: It is unclear whether novice trainees can be taught safely to perform adult cardiac surgery without any impact on early or late outcomes. METHODS: All patients (n = 1305) data were obtained from an externally validated, mandatory institutional database (2003-2010). 'Novice' is defined as a trainee who required substantial assistance or supervision to perform part or whole of the specified procedure (Intercollegiate Surgical Curriculum Programme UK, Competency Level ≤2). Outcome measures were in-hospital mortality, composite score of in-hospital mortality-morbidities, mid-term survival and revascularisation rate after CABG. Follow-up up to 7 years (median 3.2 years) was determined. RESULTS: Some 39 % (n = 510) of the cases involved novice (28 %-part, 11 %-whole procedure), 12 % (n = 157) competent trainees and 49 % (n = 638) consultant. Median EuroSCORE was higher in consultant group (p < 0.001). Without risk adjustment, composite outcome score and mid-term mortality were higher in consultant group (p = 0.03). With adjustment using EuroSCORE and propensity scores, EuroSCORE was significantly predictive of in-hospital mortality [odd ratio (OR) 1.38, 95 %CI 1.20-1.57, p < 0.001], composite outcome (OR 1.26, 95 %CI 1.15-1.37, p < 0.001) and mid-term mortality (HR 1.24, 95 %CI 1.18-1.31, p < 0.001) but not the operator categories. Further analysis of subcohort undergoing first-time, isolated CABG (n = 1070) showed that EuroSCORE remained predictive of adjusted in-hospital mortality (OR 1.39, 95 %CI 1.13-1.71, p = 0.002), composite outcome (OR 1.33, 95 %CI 1.19-1.49, p < 0.001) and mid-term mortality (HR 1.22, 95 %CI 1.10-1.35, p < 0.001). The operator categories were not associated with adjusted outcome measures including revascularisation rate after CABG. CONCLUSION: Supervised training in adult cardiac surgery can be achieved safely at the early learning curve phase without compromising both early and mid-term clinical outcomes.

Durable ventricular assist device support for failing systemic morphologic right ventricle: early results.

BACKGROUND: The systemic morphologic right ventricle (RV) in congenitally corrected transposition of the great arteries or after atrial switch for transposition of the great arteries is associated with late ventricular failure. Although the role of the left ventricular assist device (LVAD) in supporting the failing LV is established, the indications and outcomes of using LVAD in a systemic RV remain unclear. We assessed the role of a third-generation LVAD for systemic RV support. METHODS: Seven patients (mean age, 36 years) received the HeartWare (HeartWare International Inc, Framingham, MA) VAD for systemic RV failure (congenitally corrected transposition of the great arteries in 1 and after atrial switch in 6). Four patients (57%) had severe subpulmonic LV failure, and aggressive perioperative diuresis with or without hemofiltration was used to offload the subpulmonic LV. The indications of VAD were (1) bridge to transplant in 3 and (2) bridge to decision for a high transpulmonary gradient in 4. Transplantation outcome was compared with systemic RV failure without VAD bridge in 19 patients (years 1989 to 2013). RESULTS: Systemic RV support alone was achieved in all patients, with no early deaths (≤30 days). Overall, 6 (86%) returned home, 3 (44%) received a transplant, 2 (28%) died of noncardiac causes, and 2 (28%) continue on VAD support (median support, 232 days). Repeat catheterization (n = 4) showed an improved median transpulmonary gradient in 3 patients (median 18.5 mm Hg pre-VAD vs 8.0 mm Hg post-VAD). Two bridge-to-decision patients received transplants at 640 and 685 days. The stroke rate on VAD support was 43% (2 thromboembolic and 1 hemorrhagic; 3 with satisfactory recovery). De novo aortic regurgitation was 29% (n = 2; 1 valve replacement). All patients (n = 3) survived transplantation (vs 10.5% early mortality without VAD bridge; p = 1.00) and were well at follow-up (range, 53 to 700 days). CONCLUSIONS: The third-generation VAD provides durable support for systemic RV failure as a bridge to transplant and as a strategy to reduce pulmonary vascular resistance. Although concomitant subpulmonic LV failure is common, systemic RV support alone was achieved in all patients.

Right ventricular outflow tract tumour: an unsuspected intracardiac ectopic thyroid mass.

Ectopic thyroid is a rare embryogenic anomaly that occurs during its migration from foramen caecum to its pretracheal position. An intracardiac ectopic location is even rarer and found most commonly in the right ventricular outflow tract in sporadic reports. While surgery in symptomatic patients seems appropriate, resection of non-neoplastic ectopic tissue remains a clinical equipoise. Its occurrence is often unsuspected by clinicians, but its possibility should be considered due to its typical location in the right ventricular outflow tract. Unlike true neoplastic intracardiac tumour which mandates surgical resection, both surgical and non-surgical approach may be considered for an intracardiac ectopic thyroid mass.