Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards). 1st Edition.

These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care.

Quality care close to home: Objectives and early outcomes of a second paediatric heart transplant service in Australia.

AIM: We describe the experience of a new paediatric heart transplant (HT) centre in Australia. New South Wales offers quaternary paediatric cardiac services including comprehensive care pre- and post-HT; however, perioperative HT care has previously occurred at the national paediatric centre or in adult centres. Internationally, perioperative HT care is highly protocol-driven and a majority of HT occurs in low volume centres. Establishing a low volume paediatric HT centre in New South Wales offers potential for quality HT care close to home. METHODS: Retrospective review of programme data for the first 12 months was undertaken. Patient selection was audited against the programme's intended initiation criteria. Longitudinal patient data on outcomes and complications were obtained from patient medical records. RESULTS: The programme's initial phase offered HT to children with non-congenital heart disease and no requirement for durable mechanical circulatory support. Eight patients met criteria for HT referral. Three underwent interstate transfer to the national paediatric centre. Five children (13-15 years, weight 36-85 kg) underwent HT in the new programme. Individual predicted 90-day mortality was 1.3-11.6%, with increased risk for recipients transplanted from veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and with restrictive/hypertrophic cardiomyopathies. Survival at 90 days and for duration of follow-up is 100%. Observed programme benefits include mitigation of family dislocation and improved continuity of care within a family-centred programme. CONCLUSION: Audit of the first 12 months' activity of a second paediatric HT centre in Australia demonstrates adherence to proposed patient selection criteria and excellent 90-day patient outcomes. The programme demonstrates feasibility of care close to home, providing continuity for all patients including those requiring increased rehabilitation and psychosocial support post-transplantation.

In patients supported with peripheral veno-arterial extracorporeal membrane oxygenation, what factors are associated with the development of spinal cord ischaemia?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'in patients supported with peripheral veno-arterial extracorporeal membrane oxygenation, what factors are associated with the development of spinal cord ischaemia'? Altogether, more than 22 papers were found using the reported search, of which 10 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Of the 28 patients reported by included studies, the thoracic spinal cord was most commonly affected. Twenty patients (71%) survived to hospital discharge and 7 (25%) were reported to have neurological recovery. Potential confounders included coronary angiography, cardiac arrest requiring chest compressions and concomitant intra-aortic balloon pump. Consequently, all papers highlighted the likely multifactorial aetiology of spinal cord infarction in these patients. We propose that close neurological observation, particularly in patients who have received chest compressions, and management of potential aetiological factors is crucial to aid in timely diagnosis and potential prevention of this rare complication. Limiting sedation and neuromuscular blockade to enable neurologic assessment of the lower limbs may allow more timely diagnosis.

Long-term outcomes following the Ross procedure in neonates and infants: A multi-institutional analysis.

OBJECTIVES: For neonates and infants with aortic valve pathology, the Ross procedure historically has been associated with high rates of morbidity and mortality. Data regarding long-term durability are lacking. METHODS: The international, multi-institutional Ross Collaborative included 6 tertiary care centers. Infants who underwent a Ross operation between 1996 and 2016 (allowing a minimum 5 years of follow-up) were retrospectively identified. Serial echocardiograms were examined to study evolution in neoaortic size and function. RESULTS: Primary diagnoses for the 133 patients (n = 30 neonates) included isolated aortic stenosis (14%, n = 19), Shone complex (14%, n = 19), and aortic stenosis plus other (excluding Shone complex; n = 95, 71%), including arch obstruction (n = 55), left ventricular hypoplasia (n = 9), and mitral disease (moderate or greater stenosis or regurgitation, n = 31). At the time of the Ross procedure, median age was 96 days (interquartile range, 36-186), and median weight was 4.4 kg (3.6-6.5). In-hospital mortality occurred in 13 of 133 patients (10%) (4/30 [13%] neonates). Postdischarge mortality occurred in 10 of 120 patients (8%) at a median of 298 days post-Ross. Post-Ross neoaortic dilatation occurred, peaking at 4 to 5 SDs above normal at 2 to 3 years before returning to near-baseline z-score at a median follow-up of 11.5 [6.4-17.4] years. Autograft/left ventricular outflow tract reintervention was required in 5 of 120 patients (4%) at a median of 10.3 [4.1-12.8] years. Freedom from moderate or greater neoaortic regurgitation was 86% at 15 years. CONCLUSIONS: Neonates and infants experience excellent postdischarge survival and long-term freedom from autograft reintervention and aortic regurgitation after the Ross. Neoaortic dilatation normalizes in this population in the long-term. Increased consideration should be given to Ross in neonates and infants with aortic valve disease.

Cardiac sarcoma presenting as paraneoplastic arthritis and clubbing: a case report and literature review.

BACKGROUND: Cardiac tumours are rare, and clinical manifestations depend on the anatomical location. Symptoms can be the result of cardiac outflow anomalies, constitutional features such as fever, loss of weight, and/or paraneoplastic manifestations such as arthritis. To date, there has only been one other case report in the literature of cardiac sarcoma presenting as paraneoplastic arthropathy. CASE PRESENTATION: A 52-year-old woman presented with acute onset corticosteroid-resistant inflammatory polyarthralgia, clubbing and a systolic murmur. Transthoracic echocardiogram revealed a dilated left atrium with an echogenic mass and brain magnetic resonance imaging revealed multiple embolic infarcts. Histopathology following emergency resection showed a Grade 3 left atrial intimal sarcoma. The polyarthralgia and clubbing resolved soon after tumour removal. The patient went on to receive chemotherapy and remains in remission. CONCLUSIONS: This case highlights the rare paraneoplastic association of cardiac sarcoma and arthropathy.

Mid- and long-term outcomes after surgical correction of subaortic stenosis: a 27-year experience.

OBJECTIVES: We reviewed the mid- and long-term surgical outcomes of patients with subaortic stenosis (SAS). METHODS: Patients operated for SAS from April 1990 to August 2016 were reviewed retrospectively. Patients with major associations such as aortic arch obstruction were excluded. Time to reintervention and predictors of recurrence were assessed using Kaplan-Meier analysis, log-rank test and uni/multivariable Cox regression. RESULTS: 120 patients at a median age of 4.7 years (interquartile range 2.9, 8.1) underwent primary operation (median peak preoperative left ventricular outflow tract gradient 52.5 mmHg, interquartile range 40, 70) involving fibrous tissue excision (n = 120) with septal myectomy (93%; n = 112) as the procedure of choice.At median follow-up of 13 years (interquartile range 7, 18), freedom from reintervention at 1, 3, 5 and 10 years was 99% (95% confidence interval 94%, 99%), 94% (87%, 97%), 93% (86%, 96%) and 90% (82%, 94%), respectively. Recurrence occurred in 18% (n = 20) with 15 patients undergoing reinterventions, 13 of whom required radical reoperation. Multivariable analysis revealed higher preoperative peak left ventricular outflow tract gradient (hazard risk 1.06, confidence interval 1.03, 1.09, P < 0.001), and presence of bicuspid aortic valve (hazard risk 14.13, confidence interval 3.32, 60.1, P < 0.001) as predictors for reintervention. Mild/moderate aortic regurgitation occurred in 49% (n = 55) of patients at the most recent follow-up. CONCLUSIONS: Reintervention for recurrent SAS is common, predicted by higher preoperative peak left ventricular outflow tract gradient, and presence of bicuspid aortic valve, and frequently involves a radical procedure. Aortic regurgitation is a major consequence of SAS, but its severity usually remains low. CLINICAL REGISTRATION NUMBER: SCHN HREC reference number 2019/ETH02729, approved on 09 July 2019.

Technique of Coronary Button Transfer Has no Impact on Neoaortic Root Size in Simple Transposition.

We studied the effect of various coronary transfer techniques (CTT) on neo-aortic root size after an arterial switch operation (ASO) in simple transposition by excluding the impact of recognized predisposing factors. One hundred and seventy-eight patients with simple transposition were reviewed retrospectively (January 2004-December 2018) and grouped as Punch Hole (n = 83/178), Nonpunch Hole (n = 65/178; Trapdoor or Standard) and Mixed (n = 30/178). Factors predicting the neo-aortic root z-scores- annulus, mid-sinus, and sinotubular junction (STJ) were analyzed by uni/multivariable linear regression. Follow-up was 6 years, Interquartile range (IQR) 3.4,10.6. Preoperative aortic (7.4 mm, IQR 6.9,8) and pulmonary annulus (7.5 mm, IQR 6.8,8.1) sizes were identical (P = 0.831). The changes in preoperative, postoperative, and latest median z-scores for neo-aortic annulus (-0.2, IQR -1.2,0.9; 0.0, IQR -0.9,0.9; 0.9, IQR -0.4,2.6; P < 0.001), mid-sinus (1.1, IQR-0.1,2; 2.6, IQR 1.6,3.7; 2.9, IQR 1.8,4.3; P < 0.001) and STJ (-0.1, IQR -0.8,1.1; 2.1, IQR 0.7,3; 2.4, IQR 1,3.5; P < 0.001) were significant. On multivariate analysis, preoperative pulmonary annulus z-score predicted the latest neo-aortic annulus z-score [Beta estimate (BE) = 0.32, 95% confidence interval (CI) = 0.03,0.62; P = 0.03] and STJ z-score (BE= 0.45, 95% CI= 0.20,0.70; P < 0.0001). CTT did not predict any of the latest neo-aortic z-scores (all P > 0.05). Mild plus neo-aortic regurgitation (neoAR) was not significantly different across CTT groups [punch hole 20% (n = 15/74), mixed 37% (n = 11/30), nonpunch hole 21% (n = 13/62); Fisher-exact P = 0.186], one patient required valve replacement for severe neoAR. The neo-aortic root enlarges significantly over time at all 3 levels following an ASO in simple transposition, however, this is not significantly influenced by the CTT utilized.

Does leaving native antegrade pulmonary blood flow at the time of the superior cavopulmonary connection impact long-term outcomes after the Fontan?

Objectives: Antegrade pulmonary blood flow (APBF) may be left or eliminated at the time of the superior cavopulmonary connection (SCPC). Our aim was to assess the impact of leaving native APBF at the SCPC on long-term Fontan outcomes. Methods: In the Australia and New Zealand Fontan Registry (1985-2021), 587 patients had pre-existing native APBF at the SCPC. At the SCPC, 302 patients had APBF eliminated (APBF-) and 285 patients had APBF maintained (APBF+). The incidence of Fontan failure (composite end point of Fontan takedown, transplant, plastic bronchitis, protein losing enteropathy and death) and atrioventricular (AV) valve repair/replacement post SCPC was compared between the 2 groups. Results: Sex, predominant-ventricle morphology, isomerism, primary diagnosis, and age/type of Fontan were similar between groups. APBF- versus APBF+ had a higher incidence of arch obstruction/coarctation (17% vs 7%) and previous pulmonary artery band (54% vs 45%) and a lower rate of Fontan fenestration (27% vs 41%). The risk of Fontan failure was similar between the 2 groups (hazard ratio [HR], 1.01; 95% confidence interval [CI], 0.58-1.78; P = .96). The risk of AV-valve repair/replacement was greater in APBF+ versus APBF- (HR, 2.32; CI, 1.13-4.75; P = .022). The risk of AV-valve repair/replacement remained after adjustment for arch obstruction/coarctation, previous pulmonary artery band and Fontan fenestration (HR, 2.27; CI, 1.07-4.81; P = .033). Conclusions: Maintaining APBF at the time of the SCPC does not impact the risk of Fontan failure but may increase the incidence of AV-valve repair and/or replacement post-SCPC.

Correlation of ventricular septal defect height and outcomes after complete atrioventricular septal defect repair.

OBJECTIVES: There are limited data available on the height of the ventricular component of the septal deficiency (VSD) in patients undergoing complete atrioventricular septal defect (CAVSD) repair. VSD height may influence optimal choice of repair strategy with potential consequences for long-term outcomes. We aimed to measure VSD height using 2-dimensional echocardiography and review its association with postoperative outcomes. METHODS: We retrospectively reviewed the preoperative echocardiograms of 45 consecutive patients who underwent CAVSD repair between May 2010 and December 2015 at a single centre. VSD height and left ventricular length on the four-chamber view were measured. Demographic details and early and late outcomes including reoperation and long-term survival were studied. RESULTS: Twenty patients underwent modified single-patch repair and 25 patients underwent double-patch repair of CAVSD. VSD height in the modified single-patch group ranged from 4.2 to 11.7 mm and in the double-patch group ranged from 5.1 to 14.9 mm. Nine patients had a deep 'scoop' with a VSD height of >10 mm, (7 double patch, 2 modified single patch). VSD height did not correlate with a specific Rastelli classification. There was no significant difference in the VSD height (P = 0.51) or the VSD height-to-left ventricular length ratio (P = 0.43) between the 2 repair groups. There was no 30-day mortality. Eight patients required reoperation; however, VSD height was not a significant predictor of reoperation (hazard ratio 0.95, 95% confidence interval 0.69-1.33; P = 0.08). CONCLUSIONS: There was no correlation between VSD height and risk of reoperation after CAVSD repair. A deep ventricular scoop is uncommon in CAVSD patients.

A Simplified Approach to Predicting Reintervention in the Arterial Switch Operation.

We investigated patients with transposition anatomy suitable for the arterial switch operation (ASO) to evaluate a simplified approach to prediction of reintervention. A retrospective review was performed of 180 consecutive patients who underwent ASO from 2009 to 2018. Patients were classified as Category I (n = 122) d-transposition of great arteries (dTGA) + intact ventricular septum, Category II (n = 28) dTGA + ventricular septal defect (VSD) and Category III (n = 30) dTGA + Aortic arch obstruction (AAO) +/- VSD or Taussig-Bing Anomaly (TBA) +/- AAO. Outcomes included reintervention-free survival (using Kaplan-Meier estimates) and predictors of reintervention. Median follow up was 3.3 (interquartile range 1.7-5.8) years with no difference between categories(P = 0.082). There were 3 mortalities- 2 early (one each in Category I and II) and one late (in Category I). Reintervention-free survival for the whole group at 1, 3, 5 and 8 years was 94%, 91%, 90% and 86% respectively. Conventional criteria predicting reintervention included the presence of TBA(P = 0.0054) and AAO(P = 0.027). Low birth weight did not predict reintervention(P = 0.2). When analyzed by category, multivariable analysis showed that patients in Category III carried a high risk of reintervention [Hazard risk (HR) = 7.43, 95% confidence interval (CI)=(2.39, 23.11), P < 0.001], but so did those in Category II [HR=6.90, 95% CI = (2.19, 21.75, P < 0.001] when compared to Category I. Conventional risk factors for technical difficulty may not be the best predictors of reintervention. A simplified approach highlights Category II patients (dTGA + VSD) as being at substantial risk of re-intervention, and not part of a low risk cohort.

The Ross/Ross-Konno procedure in infancy is a safe and durable solution for aortic stenosis.

OBJECTIVE: The objective of this study was to characterize early and midterm outcomes after the Ross/Ross-Konno procedure performed in infancy for severe aortic valve disease. METHODS: Between January 1995 and December 2018, 35 infants younger than 1 year (13 neonates) underwent a Ross/Ross-Konno procedure. Patients were followed up to a median of 4.1 years (interquartile range [IQR], 2.6-9.5). Primary outcome measures were survival, early morbidity, freedom from reintervention and long-term functional and echocardiographic status. RESULTS: Median age at operation was 49 days (IQR, 17-135) and weight was 4 kg (IQR, 3.4-5.2). Thirty-one (89%) had undergone a previous procedure, including balloon valvuloplasty in 26 (74%). Thirty (86%) required annular enlargement (Konno incision). Five required concomitant aortic arch surgery (2 neonates, 3 infants). There were no early deaths, and 1 late death at 18 months. Freedom from reoperation was 85% (95% confidence interval [CI], 68%-93%) at 1 year, 76% (95% CI, 54%-88%) at 5 years, and 62% (95% CI, 36%-79%) at 10 years. One modified Konno was performed at 5 years after a Ross in infancy. Ten right ventricle to pulmonary artery conduits have required reintervention (2 percutaneous pulmonary valve implantations). One child required a permanent pacemaker for complete heart block. At latest follow-up, 32 (94%) of 34 survivors were asymptomatic. There was no significant change in neoaortic Z-scores between 6 weeks and latest follow-up. CONCLUSIONS: The neonatal and infant Ross/Ross-Konno procedure can be performed with low mortality and achieves a stable left ventricular outflow tract. Significant early morbidity reflects the preoperative condition of the patients but definitive surgery of this type can be considered as a primary approach.

Predictors of reoperation and mortality after complete atrioventricular septal defect repair.

OBJECTIVES: Previous studies investigating risk factors associated with reoperation or mortality after repair of complete atrioventricular septal defect (CAVSD) often have not included sizeable cohorts undergoing modified single-patch repair. Both double patch and modified single-patch techniques have been widely used in Australia since the 1990s. Using a large multi-institutional cohort, we aimed to identify risk factors associated with reoperation or mortality following CAVSD repair. METHODS: Between January 1990 and December 2015, a total of 829 patients underwent biventricular surgical repair of CAVSD in Australia at 4 centres. Patients with associated tetralogy of Fallot and other conotruncal abnormalities were excluded. Demographic details, postoperative outcomes including reoperation and survival, and associated risk factors were analysed. RESULTS: Fifty-six patients (6.8%) required early reoperation (≤30 days) for significant left atrioventricular valve regurgitation or residual septal defects. Freedom from reoperation at 10, 15 and 20 years was 82.7%, 81.1% and 77%, respectively. Patients without Down syndrome and moderate left atrioventricular valve regurgitation on postoperative echocardiogram were found to be independent risk factors for reoperation. Operative mortality was 3.3%. Overall survival at 10, 15 and 20 years was 91.7%, 90.7% and 88.7%, respectively. Prior pulmonary artery banding was a predictor for mortality, while later surgical era (2010-2015) was associated with a reduction in mortality risk. CONCLUSIONS: Improved survival in the contemporary era is in keeping with improvements in surgical management and higher rates of primary CAVSD repair over time. The presence of residual moderate left atrioventricular valve regurgitation on postoperative echocardiography is an important factor associated with reoperation and close surveillance is essential to allow timely reintervention. Primary CAVSD repair at age <3 months should be preferenced to palliation with pulmonary artery banding due to the association of pulmonary artery banding with mortality in the long-term.

Rapidly Enlarging Aortic Root Pseudoaneurysm in a Child With Endocarditis and Repaired Congenital Heart Disease.

A child with repaired double outlet right ventricle presented with Staphylococcus aureus bacteremia. Despite unsuspecting echocardiography on admission and clinical improvement on antibiotics, repeat routine echocardiography detected an aortic pseudoaneurysm, requiring a Ross-Konno operation. In repaired congenital heart defects with bacteremia, close echocardiographic surveillance is required to detect aortic pseudoaneurysm. (Level of Difficulty: Intermediate.).

Modified pulmonary artery banding: A novel strategy for balancing pulmonary blood flow with transposed great arteries.

OBJECTIVE: To study the outcomes of a novel modified pulmonary artery banding (mPAB) technique used for staged repair of a subset of patients with complex transposition physiology. METHODS: A total of 13 patients who underwent mPAB during their staged repair (biventricular repair [BVR], n = 6) or palliation (1-1/2 repair, n = 1; univentricular repair [UVR], n = 6) from 2004 to 2020 were studied retrospectively. A restrictive interposition graft was used to reconstruct the main PA between the pulmonary root and the distal pulmonary confluence, functioning as a mPAB. Twelve of the 13 patients (92.3%) underwent a concurrent arterial switch operation (ASO), of which 6 were palliative ASOs for 1-1/2 repair (n = 1) or UVR (n = 5). Patient weight and cardiac anatomy determined the size of interposition graft. RESULTS: The disease spectrum included dextro transposition of the great arteries (d-TGA) with multiple ventricular septal defects (VSDs) (n = 4), Taussig-Bing anomaly (n = 3), d-TGA with VSD and hypoplastic right ventricle (RV) (n = 3), double-inlet left ventricle with l-TGA (n = 2), and congenitally corrected TGA with double-outlet RV (n = 1). The Lecompte procedure was performed in 10 patients. Predischarge echocardiography revealed a band gradient of 61 mm Hg (interquartile range [IQR], 40-90 mm Hg) for BVR/1-1/2 ventricular repair (n = 7) and 49 mm Hg (IQR, 37-61 mm Hg) for UVR (n = 6). Survival was 100% at a median follow-up of 3.7 years (IQR, 2.6-4.0 years). CONCLUSIONS: The mPAB technique is effective and reproducible for staged BVR or UVR for patients with TGA. It effectively regulates pulmonary blood flow, may reduce neopulmonary root distortion, and eliminates complications associated with band migration in standard PAB.

Outcomes Following Heterotopic Placement of Right Ventricle to Pulmonary Artery Conduits.

BACKGROUND: We sought to evaluate the outcomes following right ventricle to pulmonary artery (RV-PA) conduit placement in pediatric patients, excluding those with a RV-PA conduit for the Ross procedure which is associated with improved conduit durability, partly related to its orthotopic position. METHODS: Outcomes for 119 patients who underwent RV-PA conduit placement at a single institution from January 2004 to December 2016 were reviewed. Primary outcome measures were reintervention-free survival (RFS) and overall survival. Survival analyses were performed using the Kaplan-Meier method, and risk factors associated with reintervention were evaluated. RESULTS: The median age at the time of conduit placement was 6 months (interquartile range, IQR: 1-14), and the median length of follow-up was 63 months (range: 0-156). During follow-up, 39 patients required conduit-related reintervention, while 6 patients died perioperatively with an overall survival of 90% at 10 years. Among the remaining 113 patients, the RFS at one, five, and ten years was 91% (84%-95%), 72% (60%-80%), and 33% (16%-50%), respectively. The median time to conduit replacement in the series was 43.5 months (IQR: 19.3-76.2). The use of a pulmonary homograft was associated with improved RFS (P = .03), and this was particularly pronounced in comparison with aortic homografts in neonates. Infection was the indication for replacement in only one patient. CONCLUSIONS: The majority of the conduits placed during the neonatal period required conduit replacement before the age of five years. Endocarditis was not a common indication for replacement. In neonates and infants, we prefer pulmonary homografts for most indications.

Bulky granulomatous disease resulting in constrictive tuberculous pericarditis requiring pericardiectomy: a case report.

BACKGROUND: We present a 23-year-old Nepalese migrant with mycobacterial tuberculosis (TB) pericarditis manifesting as effusive constrictive disease and subsequent rapid progression to constrictive pericarditis resulting from bulky granulomatous disease. CASE SUMMARY: Following initial presumptive diagnosis of TB pericarditis based on presence of moderate pericardial effusion and positive polymerase chain reaction on concurrent pleural aspirate, the patient was managed with standard empiric therapy. Despite treatment, he developed progressive heart failure with New York Heart Association (NYHA) class III symptoms and had confirmation of constrictive physiology on simultaneous left and right heart catheterization. He underwent pericardiectomy 4 months after his initial diagnosis, with debridement of large necrotizing granulomas and an associated immediate improvement clinical improvement. He remains well at 6-month follow-up with no residual heart failure symptoms off diuretic therapy. DISCUSSION: Tuberculous pericarditis accounts for 1-2% of presentations with TB infection, with progression to constrictive pericarditis in between 17 and 40% of cases. To date, pericardiectomy remains mainstay of treatment for constriction, albeit with high perioperative risk. In combination with anti-tuberculous therapy, prednisone and pericardiocentesis may reduce risk of progression to constriction, however, neither have shown mortality benefit. Our patient continued to progress, despite medical therapy and proceeded to pericardiectomy only 4 months after his initial diagnosis, with rapid improvement in symptoms, demonstrating the importance of close monitoring and revision of management strategy in these patients.

Modified-Single Patch vs Double Patch Repair of Complete Atrioventricular Septal Defects.

Biventricular repair of complete atrioventricular septal defect (CAVSD) is largely achieved using the double-patch (DP) or modified single-patch (MSP) techniques in the current era; however, long-term results following MSP repair are not well defined. We aimed to compare long-term outcomes including reoperation and mortality after CAVSD repair using DP and MSP techniques, and identify the risk factors associated with adverse outcomes. A retrospective cohort study was performed including all patients who underwent CAVSD repair using DP and MSP techniques at our institution between 17 May 1990 and 14 December 2015. Demographic details, early (≤30 days) and late (>30 days) outcomes (reoperation, mortality) were studied. Competing risks analysis with cumulative incidence function was used for survival analyses. Overall, 273 consecutive patients underwent CAVSD repair (120 DP and 153 MSP) and 41 patients required reoperation during follow-up. Competing risks analysis showed no association between repair technique and reoperation (P = 1.0) or mortality (P = 0.9). Considering competing risks due to mortality, the cumulative incidence of reoperation at 5, 10, and 15 years was 14%, 17%, and 17% for DP and 12%, 13%, and 16% for MSP, respectively. Non-Down syndrome and moderate or greater left atrioventricular valve regurgitation were predictors for reoperation. Pulmonary artery banding was predictive of mortality, though strongly associated with earlier surgical era. Median follow-up duration was 8.0 years (interquartile range 3.9-20.8) for DP and 11.6 years (interquartile range 5.4-16.1) for MSP (P = 0.4). Event-free survival is similar after DP and MSP repair of CAVSD indicating either repair technique can be safely utilized.

Complete atrioventricular septal defect repair in Australia: Results over 25 years.

OBJECTIVES: To evaluate whether the long-term outcomes of modified-single-patch (MSP) repair of complete atrioventricular septal defect are equivalent to double-patch (DP) repair with respect to survival and risk of reoperation for left atrioventricular valve regurgitation or left ventricular outflow tract obstruction. METHODS: All patients who underwent biventricular repair of complete atrioventricular septal defect in Australia from 1990 to 2015 using either a MSP or DP technique were identified. Demographic characteristic details, operative data, and outcomes were analyzed. A propensity score analysis was performed to balance the 2 treatment groups according to several baseline covariates. Survival and freedom from reintervention between the 2 groups were compared using Kaplan-Meier curves and log-rank tests. RESULTS: A total of 819 patients underwent repair of complete atrioventricular septal defect (252 MSP and 567 DP) during the study period. There was no significant difference in unmatched survival (P = .85) and event-free survival (P = .49) between MSP and DP repair. Propensity score matching resulted in a total of 223 matched pairs. Matched analysis found no difference in overall survival (P = .59) or event-free survival (P = .90) between repair techniques, with an estimated event-free survival at 5, 10, and 15 years of 83%, 83%, and 74% for DP and 83%, 80%, and 77% for the MSP group, respectively. There was no significant difference between repair techniques in reoperation for left atrioventricular valve regurgitation or left ventricular outflow tract obstruction or need for permanent pacemaker. CONCLUSIONS: Overall and event free survival are similar following either MSP or DP repair of complete atrioventricular septal defect. There is no increased risk of reoperation for left ventricular outflow tract obstruction with the MSP technique.

Double drainage of total anomalous pulmonary venous connection: A rare variant of mixed total anomalous pulmonary venous connection.

Total Anomalous Pulmonary Venous Connection (TAPVC) is frequently classified based on the system proposed by Craig, Darling and Rothney. Occasionally variants are reported which do not fit into these classic forms. One such variant is the double drainage of TAPVC where the confluence has connections at two different levels. We report two infants with a double drainage pattern of TAPVC.