Total Aortic Arch and Neoaortic Root Replacement Following Stage III Palliation for Hypoplastic Left Heart Syndrome.

Progressive dilatation of the neoaortic root and reconstructed aortic arch is a serious complication after the Norwood procedure. There are no clear guidelines on the management of this complex anatomy in the setting of single ventricle physiology, and the surgical treatment of such an entity remains anecdotal. We describe a successful surgical repair in a 15-year-old girl presenting with a severely dilated neoaortic root and aortic arch causing compression and narrowing of the left pulmonary artery after successful three-stage palliation for hypoplastic left heart syndrome.

Ventricular Septal Defect Exposure by Tricuspid Valve Chordal Detachment-A Retrospective Matched Study.

Background: Transatrial approach is the standard method in repairing ventricular septal defects (VSD) in the pediatric population. However, the tricuspid valve (TV) apparatus might obscure the inferior border of the VSD risking the adequacy of repair by leaving residual VSD or heart block. Detachment of the TV chordae has been described as an alternative technique to TV leaflet detachment. The aim of this study is to investigate the safety of such a technique. Methods: Retrospective review of patients who underwent VSD repair between 2015 and 2018. Group A (n = 25) had VSD repair with TV chordae detachment were matched for age and weight to group B (n = 25) without tricuspid chordal or leaflet detachment. Electrocardiogram (ECG) and echocardiogram at discharge and at 3 years of follow-up were reviewed to identify new ECG changes, residual VSD, and TV regurgitation. Results: Median ages in groups A and B were 6.13 (IQR 4.33-7.91) and 6.33 (4.77-7.2) months. New onset right bundle branch block (RBBB) was diagnosed at discharge in 28% (n = 7) of group A versus 56% (n = 14) in group B (P = .044), while the incidence dropped to 16% (n = 4) in group A versus 40% (n = 10) in group B (P = .059) in the 3 years follow-up ECG. Echocardiogram at discharge showed moderate tricuspid regurgitation in 16% (n = 4) in group A and 12% (n = 3) in group B (P = .867). Three years of follow-up echocardiography revealed no moderate or severe tricuspid regurgitation and no significant residual VSD in either group. Conclusion: No significant difference in operative time was observed between the two techniques. TV chordal detachment technique reduces the incidence of postoperative RBBB without increasing the incidence of TV regurgitation at discharge.

Urgent Excision of Primary Intracardiac Burkitt Lymphoma in a Child.

A 9-year-old girl presented with a recent history of shortness of breath, fatigue, visual disturbances, and gastrointestinal symptoms. Echocardiography demonstrated three large intracardiac masses in the right and left atria protruding into the mitral and tricuspid valve orifices causing bilateral inflow obstruction. She underwent urgent surgical excision of the masses. Histology revealed rare intracardiac Burkitt's Lymphoma.

Contemporary outcomes of aortic arch hypoplasia and coarctation repair in a tertiary paediatric cardiac surgery centre.

OBJECTIVES: There are several studies reporting the outcomes of hypoplastic aortic arch and aortic coarctation repair with combination of techniques. However, only few studies reported of aortic arch and coarctation repair using a homograft patch through sternotomy and circulatory arrest with retrograde cerebral perfusion. We report our experience and outcomes of this cohort of neonates and infants. METHODS: We performed retrospective data collection for all neonates and infants who underwent aortic arch reconstruction between 2015 and 2020 at our institute. Data are presented as median and inter-quartile range (IQR). RESULTS: The cohort included 76 patients: 49 were males (64.5%). Median age at operation was 16 days (IQR 9-43.25 days). Median weight was 3.5 kg (IQR 3.10-4 kg). There was no 30 days mortality. Three patients died in hospital after 30 days (3.95%), neurological adverse events occurred in only one patient (1.32%) and recurrent laryngeal nerve injury was noted in four patients (5.26%). Only three patients required the support of extracorporeal membrane oxygenation (ECMO) with a median ECMO run of 4 days. Median follow-up was 35 months (IQR 18.9-46.4 months); 5 years survival was 93.42% (n = 71). The rate of re-intervention on the aortic arch was 9.21% (n = 7). CONCLUSION: Our experience shows excellent outcomes in repairing aortic arch hypoplasia with homograft patch under moderate to deep hypothermia with low in-hospital and 5 years mortality rates.

COVID-19 and congenital heart disease: an insight of pathophysiology and associated risks.

OBJECTIVE: We aimed to examine the literature to determine if both paediatric and adult patients diagnosed with congenital heart disease (CHD) are at a higher risk of poor outcomes if they have the coronavirus disease 2019 (COVID-19), compared to those without CHD. METHODS: A systematic review was executed using the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines. To identify articles related to COVID-19 and CHD, an extensive literature search was performed on EMBASE, Medline, Scopus, and Global Health databases using keywords and MeSH terms. RESULTS: A total of 12 articles met the inclusion criteria and were included for analysis in this systematic review. Two themes were identified for data extraction: evidence supporting higher risks in CHD patients and evidence against higher risks in CHD patients. After combining the data, there were 99 patients with CHDs out of which 12 required admissions to ICU. CONCLUSION: This systematic review suggests that CHD may increase the risk of poor outcomes for those with COVID-19, but also highlights the necessity for more research with larger sample sizes in order to make a more justified conclusion, as the majority of papers that were analysed were case series and case reports. Future research should aim to quantify the risks if possible whilst accounting for various confounding factors such as age and treatment history.

Unifocalization cannot rely exclusively on native pulmonary arteries: the importance of recruitment of major aortopulmonary collaterals in 249 cases†.

OBJECTIVES: We sought to define the early and late outcomes of unifocalization based on a classification of the native pulmonary artery (nPA) system and major aortopulmonary collateral arteries (MAPCAs) with a policy of combined recruitment and rehabilitation and to analyse the role of unifocalization by leaving the ventricular septal defect (VSD) open with a limiting right ventricle-pulmonary artery (RV-PA) conduit in borderline cases. METHODS: An analysis of 271 consecutive patients assessed for unifocalization at a single institution between 1988 and 2016 was performed. Patients were classified according to the pulmonary blood supply: group A, unifocalization based on nPA only; group B, based on nPA and MAPCAs; group C, MAPCAs only (absent nPAs). RESULTS: Unifocalization was achieved in 249 (91.9%) cases with an early mortality of 2.8%. Group A included 72 (28.9%) patients, group B 119 (47.8%) patients and group C 58 (23.3%) patients with no difference in early survival between groups. Survival at 5, 10 and 15 years was 90.0% (85.9-94.3), 87.2% (83.5-91.2) and 82.3% (75.2-89.9), respectively. Late survival in groups A and B was similar but 10- and 15-year survival in group C decreased to 79.2% (68.2-92.1) and 74.3% (61.1-90.4) (P = 0.02), respectively. A mean of 1.9 (±0.6) MAPCAs were recruited per patient (range 0-6). The VSD was left open with a limiting RV-PA conduit in 97 (39.0%) cases, but subsequently closed in 48 patients, giving a total of 200 (80.3%) patients achieving VSD closure (full repair). Delaying VSD closure was not associated with increased risk for early or late survival. A central shunt to rehabilitate the nPAs was used in 56 (22.5%) cases. This was associated with a reduction in the number of MAPCAs recruited, but still required a mean of 1.8 (±0.5) MAPCAs recruited per patient to achieve unifocalization. In multivariate risk analysis, those suitable for single-stage full repair had the best long-term outcomes. Group C anatomy was associated with poor late survival compared to groups A and B (hazard ratio 2.7). CONCLUSIONS: Survival is maximized by a combined approach of rehabilitation and recruitment. MAPCAs should always be recruited if they supply areas with absent nPA supply. A strategy of leaving the VSD open with a limiting RV-PA conduit is a safe and effective way of managing borderline cases.

Fate of the Left Ventricular Outflow Tract After Rastelli With Selective Infundibular Muscle Resection.

BACKGROUND: The Rastelli procedure has been criticized for a high rate of left ventricular outflow tract obstruction (LVOTO) and translocation procedures have been proposed as an alternative. Infundibular resection can be performed during Rastelli to optimize the outflow tract. This study examines whether a selective policy to enlarge the outflow tract improves Rastelli outcomes across all morphological variants. METHODS: Single institution 29-year experience in 105 patients. Patients were classified into transposition of the great arteries with ventricular septal defect, congenitally corrected transposition of the great arteries with ventricular septal defect, and double outlet right ventricle morphology groups. The infundibular muscle was routinely resected if prominent, in 28 cases. RESULTS: Early mortality was 1.9% (2 of 105) and actuarial survival was 95.4% (95% confidence interval [CI], 89% to 99%) at 1 year, 92.9% (95% CI, 85% to 97%) at 5 years, and 84.5% (95% CI, 74% to 92%) at 10 years. The cumulative freedom from LVOTO was 99% (95% CI, 96% to 100%), 97% (95% CI, 92% to 99%), and 90% (95% CI, 88% to 96%) at 1, 5, and 10 years, respectively. The incidence was similar in all morphological groups and those undergoing infundibular resection were not at higher risk of late LVOTO. Eleven patients required surgical reoperation on the left ventricular outflow tract over a median follow-up period of 8.5 years, with no mortality, although 2 of these patients developed complete heart block. Left ventricular function was well preserved in 98.1% of all cases, including all of those requiring left ventricular outflow tract reoperation. CONCLUSIONS: The Rastelli is a safe procedure that can be applied in a variety of morphological variants. LVOTO remains a late complication of Rastelli, but can be minimized by concomitant infundibular muscle resection. Late reoperation is safe and ventricular function is well preserved in greater than 95% of cases at late follow-up. The operation has stood the test of time and avoids many of the risks of translocation procedures.

Tamponade by an expanding left ventricular pseudoaneurysm: A unique presentation.

Left ventricular free wall rupture secondary to myocardial infarction is an uncommon but catastrophic event requiring emergency surgery. We describe a unique presentation of left ventricular free wall rupture as delayed tamponade caused by a gradually expanding pseudoaneurysm compressing the left atrium, leading to pulmonary congestion that required increasing respiratory support to maintain oxygenation, and necessitated emergency surgery. We discuss the options available to treat pseudoaneurysms due to left ventricular free wall rupture.

Mechanical complications of acute myocardial infarction.

Acute myocardial infarction (AMI) can result in ischemic, mechanical, arrhythmic, embolic, or inflammatory complications. The development of mechanical complications following AMI is associated with a significantly reduced short-term and long-term survival. Since the introduction of primary percutaneous coronary intervention as the principal reperfusion strategy following acute ST-elevation myocardial infarction, the incidence of mechanical complications, including rupture of the left ventricular free wall, papillary muscle, and ventricular septum, has reduced significantly to less than 1%. Despite high operative mortality, the lack of an effective medical alternative makes surgical repair the mainstay of current management for these patients.

Use of centrifugal left ventricular assist device as a bridge to candidacy in severe heart failure with secondary pulmonary hypertension.

OBJECTIVES: Raised pulmonary artery pressure (PAP), trans-pulmonary gradient (TPG) and pulmonary vascular resistance (PVR) are risk factors for poor outcomes after heart transplant in patients with secondary pulmonary hypertension (PH) and may contraindicate transplant. Unloading of the left ventricle with an implantable left ventricular assist device (LVAD) may reverse these pulmonary vascular changes. We studied the effect of implanting centrifugal LVADs in a cohort of patients with secondary PH as a bridge to candidacy. METHODS: Pulmonary haemodynamics on patients implanted with centrifugal LVADs at a single unit between May 2005 and December 2010 were retrospectively reviewed. RESULTS: Twenty-nine patients were implanted with centrifugal LVADs (eight HeartWare ventricular assist device (HVAD), HeartWare International, USA and 21 VentrAssist, Ventracor Ltd., Australia). Seventeen were ineligible for transplant by virtue of high TPG/PVR. All the patients were optimized with inotrope/balloon pump followed by LVAD insertion. Four required temporary right VAD support. Thirty-day mortality post-LVAD was 3.4% (1 of 29) with a 1-year survival of 85.7% (24 of 28). Thirteen patients have been transplanted to date: 30-day mortality was 7.7% (1 of 13) and 1-year survival was 91% (10 of 11). Baseline and post-VAD pulmonary haemodynamics were significantly improved: systolic PAP (mmHg), mean PAP, TPG (mmHg) of 57 ± 9.5, 42 ± 4.4 and 14 ± 3.9 reduced to 32 ± 7.5, 18 ± 5.5 and 9 ± 3.3, respectively. PVR reduced from 5 ± 1.5 to 2.1 ± 0.5 Wood units (P < 0.05). CONCLUSIONS: In selected heart failure patients with secondary PH, use of centrifugal LVAD results in significant reductions in PAP, TPG and PVR, which are observed within 1 month, reaching a nadir by 3 months. Such patients bridged to candidacy have post-transplant survival comparable with those having a heart transplant as primary treatment.

A meta-analysis of minimally invasive versus traditional open vein harvest technique for coronary artery bypass graft surgery.

The long saphenous vein remains the most commonly used conduit in coronary artery bypass surgery. Vein harvest is a critical component of this operation with significant morbidity associated with large leg wounds from open techniques. Here, we analyse the available literature comparing minimally invasive techniques vs. the traditional open method for vein harvest. A systematic literature search of Medline, Embase and Cochrane databases was performed using the following terms; 'saphenous vein', 'coronary artery bypass', 'tissue and organ harvesting' and 'endoscopic'. Relevant papers were then analysed using Statsdirect software. There was significantly reduced leg wound infection, leg wound haematoma and postoperative pain in the minimally invasive group. There was no statistical difference between the groups for vein harvest time, length of hospital stay and incidence of vein injury. There was a significantly reduced long-term graft patency in veins harvested by a minimally invasive technique. The results of this meta-analysis demonstrate the operative advantages of minimally invasive techniques for the purposes of vein harvest in coronary artery bypass surgery. However, further studies are required to look at long-term graft patency following minimally invasive vein harvest as this remains a major concern.