Characteristics and Outcomes of a Single-Centre Cohort of Adult Congenital Heart Disease Patients Referred for Heart Transplant.

BACKGROUND: Adult congenital heart disease (ACHD) services increasingly encounter heart failure (HF) in the ageing ACHD population. Optimal timing of referral for heart transplant (HTx) evaluation in this heterogeneous population is complex and ill-defined. We aim to outline the characteristics and outcomes of ACHD patients referred for HTx from a large Australian ACHD centre. METHOD: Retrospective review of ACHD patients referred for HTx from a primary ACHD centre (1992-2021). Database analysis of patient demographics, characteristics, wait-listing, and transplantation outcomes was performed. RESULTS: A total of 45 patients (mean age 37±9.9 years old; 69% male) were referred for HTx with a mean follow-up of 5.9±6.3 years. Of these, 22 of 45 (49%) were listed and transplanted, including one heart-lung transplant. The commonest diagnosis was dextro-transposition of the great arteries (13/45, 29%). Most patients, 33 of 45 (73.3%) had undergone at least one cardiac surgery in childhood. Indications for HTx referral included HF in 34 of 45 (75%), followed by pulmonary hypertension in 7 of 45 (11%). Median transplant wait-list time was 145 days (interquartile range, 112-256). Of the 23 patients not wait-listed, the reasons included clinical stability in 13 of 45 (29%), psychosocial factors in 2 of 45 (4.4%) and prohibitive surgical risk, including multiorgan dysfunction, in 8 of 45 (17.7%). Transplant was of a single organ in most, 21 of 22 (95.5%). Overall mortality was 5 of 22 (22.7%) in those after HTx, and 14 of 23 (60.9%) in those not listed (p=0.0156). CONCLUSIONS: Increasingly, ACHD patients demonstrate the need for advanced HF treatments. HTx decision-making is complex, and increased mortality is seen in those not wait-listed. Ultimately, the referral of ACHD patients for HTx is underpinned by local decision-making and experience, wait-list times and outcomes.

Fontan Hepatopathy-Managing Unknowns.

BACKGROUND AND AIMS: How to best monitor Fontan-associated liver disease (FALD) remains unclear. We describe results from a prospective liver care pathway in adults (n=84) with a Fontan circulation. METHODS: Routine assessment of the liver, by acoustic radiation force frequency and ultrasound was undertaken. Results, including liver biochemistry, systemic ventricular function (echocardiography), functional class, medication use and clinical endpoints (varices, hepatocellular carcinoma, heart transplantation and death) were collated. RESULTS: Most individuals returned a cirrhotic range acoustic radiation force impulse imaging (ARFI) result. ARFI values were greater in the proportion of individuals with hepatic nodularity (p=0.024). Univariate analysis demonstrated moderate correlation with platelet number (Spearmans rho= -0.376, p=0.049). Patients with clinical endpoints had lower platelets (p=0.012) but only a trend to hepatic nodularity (p=0.057). Clinical endpoints were more common in those with ventricular dysfunction (p=0.011). Multivariate analysis revealed that age at Fontan and being on angiotensin converting enzyme inhibitors (ACEI) predicted ARFI score (ß=0.06 [95% CI 0.01-0.09], p=0.007 and ß=0.53 [95% CI 0.17-0.89], p=0.005, respectively). However, these associations were not significant once adjusted for Fontan type, age at ARFI, systemic ventricle morphology, ventricle function, or Model for End-stage Liver Disease (MELD-XI) excluding international normalised ratio (INR) (p>0.05 for all). CONCLUSIONS: Ideal FALD monitoring remains unclear. ARFI has utility as a binary non-invasive indicator of cirrhosis, highlighting individuals who may need more frequent ongoing monitoring for hepatocellular carcinoma. However, no definite advantage to serial ARFI, once cirrhotic range ARFI results are present, has been identified.

Impact of Telehealth on Failure to Attend Rates and Patient Re-Engagement in Adult Congenital Heart Disease Clinic.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic paved the way for telehealth consultations. We aimed to determine the impact of telehealth on rates of failure to attend (FTA) in adult congenital heart disease (ACHD) clinics and whether telehealth could re-engage patients with previous FTA face-to-face appointments. METHODS: This was a retrospective audit of a tertiary ACHD clinic over a 12-month pre-telehealth (26 March 2019-17 March 2020) and 12-month post-telehealth implementation period (24 March 2020-16 March 2021). Patients with one or more FTAs during the 24-month study period were included. Our ACHD clinic is run three times per month. Patients with ACHD are offered lifelong follow-up and reviewed annually on average. Re-engagement was defined as two or more consecutive face-to-face FTAs immediately before the telehealth period with subsequent attendance of their telehealth appointment. RESULTS: A total of 359 patients with a total of 623 FTAs were included. Complexity of congenital heart disease was moderate in 56% (202/359) and severe in 19% (69/359) of patients. Overall FTA rate was 18% (623/3,452). FTA rate was significantly lower in the post-telehealth period (15%, 257/1,664) compared with the pre-telehealth period (20%, 366/1,788) (p<0.00001). At study conclusion, 1% of patients had died (5/359). Of the 354 remaining patients, 42% (150/354) were considered lost to follow-up (two or more FTAs including telehealth), 37% (132/354) missed only one clinic appointment, and 20% (72/354) previously considered lost to follow-up had re-engaged in the telehealth period. CONCLUSIONS: Rates of FTA in a tertiary ACHD clinic significantly reduced after the introduction of telehealth consultation. A fifth of patients considered lost to follow-up were re-engaged with telehealth. Additional strategies to further reduce FTA should be explored.

Left bundle branch area pacing in congenitally corrected transposition of great arteries - The obvious choice?

Patients with congenitally corrected transposition of the great arteries (CCTGA) are susceptible to acquired atrioventricular conduction disease in early life. Emerging studies propose that conduction system pacing either by His bundle pacing or bundle branch pacing is advantageous in this population. The anatomical abnormality of CCTGA conveniently positions the left bundle branches on the easily accessible right ventricular septal side. We present a case of a young female CCTGA patient with exercise-induced heart block who received left bundle branch area pacing with the ventricular lead implanted at the conventional right ventricular septal approach without the need for 3-dimensional electroanatomical mapping.

Remote Device Interrogation Kiosks (ReDInK) - Pharmacy Kiosk Remote Testing of Pacemakers and Implantable Cardioverter-Defibrillators for Rural Victorians. A Novel Strategy to Tackle COVID-19.

BACKGROUND: In the era of COVID-19, travel restrictions and social distancing measures have changed the landscape for device interrogations of pacemakers and defibrillators for rural Victorians. Previously, device checks were performed infrequently in large volume, face-to-face rural clinics by visiting cardiologists and technicians. Access to remote areas and social distancing restrictions have made these clinics unfeasible to operate. The Cardiac Society of Australia and New Zealand (CSANZ) and Heart Rhythm Society (HRS) COVID-19 consensus statements have suggested the utilisation of remote monitoring to minimise the potential spread of COVID-19 infections between clinicians and high-risk patients. A novel solution to this challenge was the implementation of a remote device interrogation (RI) service located in two kiosks at two rural pharmacies. This service was termed Remote Device Interrogation Kiosks (ReDInK). AIM: This cross-sectional observational study aimed to describe the set-up process, safety and efficacy of RI and customer satisfaction of the ReDInK program. METHODS: Two-hundred-and-ninety-two (292) rurally located patients with implantable cardiac devices were identified via the cardiology department database. Of these, 101 (44%) were enrolled into the ReDInK program across two rurally located pharmacies between April and July 2020. RI was performed and download outcomes were reviewed. A customer satisfaction survey assessed attitudes towards the program and explored options of ongoing service application. RESULTS: Of 101 patients enrolled into ReDInK, 96 (95%) resulted in satisfactory device checks. Four (4) individuals failed-to-attend and one individual experienced technical download issues. Of the 96 satisfactory device checks, three required in-person follow-up for reasons including battery replacement, lead repositioning and in-person programming. No adverse events were reported. A satisfaction telephone survey was conducted with 81 (83%) participants enrolled in ReDInK. Seventy-one (71) individuals (88%) of those surveyed expressed satisfaction and 73 (90%) labelled the process as efficiently conducted. Sixty-nine (69) (85%) participants felt reassured that this service was established during the pandemic. However 47 (58%) participants reported they would still feel comfortable to undergo in-person reviews despite social distancing recommendations. CONCLUSIONS: With the COVID-19 pandemic posing restrictions to social distancing and reducing unnecessary in-person interaction, the ReDInK program emerges as an efficacious and safe solution for patients in rural Victoria. The program's widely positive reception and successful conduction in rural Victoria invites further opportunity for a wider application of similar programs, expanding its role to metropolitan areas.

Pregnancy Outcomes in Women After the Arterial Switch Operation.

BACKGROUND: Pregnancy outcomes after the arterial switch operation (ASO) are rare. We sought to determine outcomes of ASO survivors who underwent pregnancy. METHODS: Female patients who had an ASO and underwent pregnancy were identified from the congenital heart disease pregnancy clinic at The Royal Melbourne Hospital. All follow-up data were collected retrospectively by medical record review. RESULTS: Eleven (11) women were identified as having undergone medical care during pregnancy, from the adult congenital database, at The Royal Melbourne Hospital. There were 17 successful pregnancies, and nine women have been followed post pregnancy. Of the 17 successful deliveries, eight were delivered by Caesarean section, seven were vaginal deliveries and two were instrumented vaginal deliveries. Of the eight Caesarean sections, five were emergency and three were elective. The indications for emergency Caesarean section were obstructed labour (n = 2), abnormal cardiotocography (n = 1), obstructed labour and abnormal cardiotocography (n = 1) and congestive cardiac failure (n = 1). There was one neonatal complication (respiratory distress requiring intubation) in a child born at 31 weeks. There were maternal obstetric complications in 10 patients. There were two maternal cardiac complications during pregnancy (heart failure and rapid atrial fibrillation/flutter). There was no change in left ventricular function post-pregnancy. There was progression of severity of neo-aortic valve regurgitation in two patients post pregnancy (trivial to mild and moderate-severe to severe respectively). CONCLUSION: Pregnancy post ASO appears to be safe in the majority of women. Maternal cardiac complications are uncommon in patients without residual significant haemodynamic lesions, although maternal obstetric complications may be common.

Learning Curve and Initial Experience With Implementation of a His-Bundle Pacing Program in an Australian Setting.

BACKGROUND: His-bundle pacing (HBP) has emerged as a promising technique to avoid pacing complications associated with dyssynchrony from right ventricular pacing, but data are limited to experienced operators and centres. We aimed to evaluate the implementation and outcomes of an HBP program in an Australian setting. METHODS: Data were retrospectively collected on 140 consecutive HBP procedures attempted at three centres from March 2018 to September 2019. The cohort was divided into three groups (early: procedures 1-47, middle: 48-94, late: 95-140) to determine changes in procedural success in relation to operator experience. RESULTS: Median age was 76 years (IQR 68-80 yrs); 69% were male. Atrial fibrillation was present in 59%, left ventricular ejection fraction (LVEF) ≤40% in 25%, and left and right bundle branch blocks present in 23% and 16% respectively, and atrioventricular (AV) block was present in 26%. Overall procedural success was 87%, median implant threshold 0.8V@1 ms, and QRS duration improved in 64% of procedures. Procedural success (early 83%, middle 89%, late 89%, p=0.58) was not different, while median procedural time (early 98 mins, middle 83 mins, late 70 mins, p<0.001) improved across operator experience groups. Lower success rates were identified for patients with AV block (73% vs. 92%, p<0.01), a previous device (69% vs. 89%, p=0.02), moderate-severe TR (69% vs. 88%, p=0.04), and when right-sided access was required (25% vs. 89%, p<0.01). CONCLUSIONS: His-bundle pacing is a feasible procedure with continued improvement in procedural measures of success after an early learning period. The presence of AV block, a previous device, significant tricuspid regurgitation, or right-sided access may affect procedural success.

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement.

The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.

Adult Congenital Heart Disease in Australia and New Zealand: A Call for Optimal Care.

BACKGROUND: Adult congenital heart disease (ACHD) is a relatively new subspecialty in the cardiology field. The prevalence of ACHD is estimated at ∼ 3,000 per million adult population. The ACHD patient group is estimated to grow at ∼ 5% per year and in the next decade it is forecast that 1 in 150 young adults will carry some form of ACHD diagnosis. These estimates translate to ∼ 72,000 ACHD patients in Australia and ∼ 14,000 in New Zealand, although no current numbers are available. The Cardiac Society of Australia and New Zealand (CSANZ) has recently published Recommendations for Standards of Care for Adult Congenital Heart Disease (ACHD) in 2016. There is currently no long-term plan or proposal to address this huge health care burden within the federal government. This document details the size of the problem insofar as it is known and recommends solutions to be implemented. METHODS: This document was developed by the Adult Congenital Heart Disease Working Group of the Paediatric and Congenital Council (the Congenital Heart Alliance of Australia and New Zealand) as a response to the chronic under resourcing in this area, the risk this poses to patients and clinicians, and the clear need for long-term planning to develop safe care pathways. RESULTS: These issues were raised with the CSANZ Board in December 2015 and the document was developed in response to the Board's request for more information. The current iteration was finalised on 14 November 2017. CONCLUSIONS: The authorship group comprised participants in the CSANZ adult CHD standards of care recommendations from 2013 with the inclusion of some newly trained ACHD cardiologists, who represented most states and territories across ANZ. None of the authors has any academic or professional conflict of interest.

Extracardiac Conduit Fontan - Outcome Data in Early Adulthood.

BACKGROUND: To describe the survival and health outcome status of young adults with an extracardiac Fontan procedure performed either as a primary or conversion (secondary) Fontan surgery. METHODS: The database of the Adult Congenital Heart disease service at the Royal Melbourne Hospital was interrogated to identify all adults who had undergone a primary extracardiac conduit Fontan (n=29) or a Fontan conversion with this procedure (n=8). We then determined vital status, age, original anatomy and functional status in early adulthood in both groups. RESULTS: Adults with an ECC Fontan procedure report reasonable NYHA functional class (84% NYHA I or II) though, objectively, exercise testing demonstrates a reduced exercise capacity, and desaturation on exertion is frequent. The majority (86%) have completed secondary education. Most (78%) are managed on warfarin and there is a preponderance of ACE inhibition use (62%). Atrial arrhythmias have been documented in 5 of the 29 primary ECC groups (17%); in 3 patients this preceded primary ECC and 2 patients developed post primary ECC (between 6 and 14 years postoperatively). At a lesser time of follow-up [median 4.5 years (IQR 3.3-6)], conversion to an ECC as a secondary Fontan procedure has successfully treated atrial arrhythmias in the 7 (of 8) patients where this was the surgical indication for conversion. CONCLUSIONS: Though long-term data will require decades to establish, in young adulthood the functional outcomes of a primary ECC Fontan operation are encouraging. Secondary ECC conversion successfully mitigates atrial arrhythmias in the short to medium term.

A Contemporary Phone-Based Cardiac Coaching Program: Evolution and Cross Cultural Utility.

BACKGROUND: The Hospital Admission Risk Program (HARP) Cardiac Coach Program at Royal Melbourne Hospital has evolved to include a Greek and Italian service, developed in response to the diverse local community and supported by evidence that Culturally and Linguistically Diverse (CALD) groups both perceive health and respond to health care services and information, differently. This paper aims to evaluate if a phone-based cardiac coaching program can be adapted to the Greek and Italian populations using the English cohort as a comparator. METHODS: We retrospectively analysed cardiovascular risk profiles at recruitment into and at discharge from the program. Patients (n=383) were recruited after an acute coronary event or intervention between June 2011 and June 2013. Recruitment was into the English (n=301 patients (79%)) Greek (40 (10%)) or Italian (42 (11%)) model. Data was collected on demographic information and risk factor status at entry and discharge from the program: waist circumference, weight, height, lipid profile, HbA1c, smoking status and physical activity. A comparison of the proportion of patients meeting the defined targets across the English, Italian and Greek cohorts was performed, with multivariate logistic regression analysis applied to adjust for differences in baseline variables. RESULTS: There were baseline differences in age, smoking history, total cholesterol and cholesterol fractions, diastolic blood pressure, weight and physical activity between the cohorts. At discharge, the proportion of patients meeting targets within each cohort were similar. CONCLUSION: A phone-based integrated disease management program can be adapted to CALD patients, achieving comparable outcomes as compared with an English-speaking cohort. Health services need to respond to their local needs and be flexible in program delivery in order to benefit as many patients as possible.

Evaluation of the Effectiveness of a Phone Based Care Coordination Pilot on Hospital Utilisation and Costs for Patients With Chest Pain.

BACKGROUND: A small percentage of the population represents a disproportionate number of attendances at emergency departments (ED). "Frequent presenters" to ED with chest pain do not always fit into established pathways for acute myocardial events. With accelerated "rule out" protocols, patients are often discharged from the ED after short lengths of stay. This research will evaluate the effectiveness of a phone based care-coordination pilot designed to meet the needs of patients attending ED with cardiac and non-cardiac chest pain. METHODS: A longitudinal, single-arm interventional study with retrospectively recruited control group. Ninety-five patients were enrolled as the intervention group; 97 patients were retrospectively identified as controls. These patients had re-presented with chest pain within 6 months of a cardiac event, or attended hospital within 12 months two or more times with chest pain and/or complex needs. Intervention group patients were holistically assessed then phone-coached to support self-management of chest pain over 6 months. Following descriptive and univariate analysis, multivariate analysis was conducted to adjust for noted differences between the intervention and control groups. RESULTS: Thirty-day representation to ED was significantly less for the intervention group (14.1%) compared to controls (27.7%). After adjusting for baseline differences, intervention patients were more than two-fold less likely to re-present compared to controls (OR=0.42, 95%CI: 0.19-0.96). After adjustment for baseline differences, the savings in subsequent inpatient costs was $1588 per person, as a result of intervention, patients were less likely to have inpatient readmissions (16.3%) compared to controls (20.2%), although this was not statistically significant (p=0.588). CONCLUSION: A phone based care-coordination pilot with targeted interventions has the potential to reduce ED presentations and hospital readmissions among patients representing with chest pain.

Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand.

BACKGROUND: The life expectancy of patients undergoing a Fontan procedure is unknown. METHODS AND RESULTS: Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975-1995), 271 lateral tunnels (1988-2006), and 532 extracardiac conduits (1997-2010). The proportion with hypoplastic left heart syndrome increased from 1/173 (1%) before 1990 to 80/500 (16%) after 2000. Survival at 10 years was 89% (84%-93%) for AP and 97% (95% confidence interval [CI], 94%-99%) for lateral tunnels and extracardiac conduits. The longest survival estimate was 76% (95% CI, 67%-82%) at 25 years for AP. AP independently predicted worse survival compared with extracardiac conduits (hazard ratio, 6.2; P<0.001; 95% CI, 2.4-16.0). Freedom from failure (death, transplantation, takedown, conversion to extracardiac conduits, New York Heart Association III/IV, or protein-losing enteropathy/plastic bronchitis) 20 years after Fontan was 70% (95% CI, 63%-76%). Hypoplastic left heart syndrome was the primary predictor of Fontan failure (hazard ratio, 3.8; P<0.001; 95% CI, 2.0-7.1). Ten-year freedom from failure was 79% (95% CI, 61%-89%) for hypoplastic left heart syndrome versus 92% (95% CI, 87%-95%) for other morphologies. CONCLUSIONS: The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure. Large, comprehensive registries such as this will further improve our understanding of late outcomes after the Fontan procedure.

Efficacy and feasibility of a tele-health intervention for acute coronary syndrome patients with depression: results of the "MoodCare" randomized controlled trial.

BACKGROUND: Depression is common after a cardiac event, yet there remain few approaches to management that are both effective and scalable. PURPOSE: We aimed to evaluate the 6-month efficacy and feasibility of a tele-health program (MoodCare) that integrates depression management into a cardiovascular disease risk reduction program for acute coronary syndrome patients with low mood. METHODS: A two-arm, parallel, randomized design was used comprising 121 patients admitted to one of six hospitals for acute coronary syndrome. RESULTS: Significant treatment effects were observed for Patient Health Questionnaire 9 (PHQ9) depression (mean difference [change] = -1.8; p = 0.025; effect size: d = 0.36) for the overall sample, when compared with usual medical care. Results were more pronounced effects for those with a history of depression (mean difference [change] = -2.7; p = 0.043; effect size: d = 0.65). CONCLUSIONS: MoodCare was effective for improving depression in acute coronary syndrome patients, producing effect sizes exceeding those of some face-to-face psychotherapeutic interventions and pharmacotherapy. ( TRIAL REGISTRATION NUMBER: ACTRN1260900038623.).

Long term follow-up after aortic valve replacement (Ross procedure): echocardiographic determinants of ventricular recovery.

AIMS AND METHODS: This prospective cohort study aimed to assess LV recovery post aortic valve replacement, stratified according to pre-operative valve lesion (aortic stenosis (AS), mixed disease (AS/AR) or aortic regurgitation (AR)), as well as define predictors of persistent LV dilatation post operation. We prospectively followed all patients post Ross procedure performed between 1992 and 2009 by a single surgeon. Echocardiography was performed pre-operatively, at approximately one year post operation then second yearly thereafter. RESULTS: 265 patients were followed for a mean of 6.4 years (range 1-14 years, total 1702 patient-years). Seventy percent were male and mean age was 38.8 ± 12.6 years. The indication for surgery was AS in 44.5% (118), AS/AR in 23.4% (62), and AR in 32.1% (85). Overall mortality was 1.8% and 80% of deaths were non-cardiac. Morbidity was low and the need for pacing was less than 1%. Ninety-nine percent of patients were NYHA class 1 at one year follow up. The indexed LV end diastolic diameter (LVEDDi) decreased significantly post-operation in the AR (3.34 ± 0.39-2.66 ± 0.32 cm/m(2), p<0.001) and mixed (2.85 ± 0.38-2.65 ± 0.30 cm/m(2), p = 0.01) groups, whilst the indexed systolic LV dimension decreased significantly post-operation in the AR group (2.26 ± 0.34-1.87 ± 0.27, p<0.001). At five years post operation, independent predictors for a larger LVEDDi were female gender, a pre-operative LVEDDi >3.0 cm/m(2) and the presence of mild aortic regurgitation at one year post-operation. Pre-operative valve lesion was not a predictor. The only independent predictor of a lesser reduction of LVEDDi at five year follow-up was mild post-operative AR, whilst predictors of a lesser reduction in indexed left ventricular end systolic diameter (LVESDi) at five year follow-up included mild post-operative AR and a larger pre-operative LVEDDi. LV wall thickness decreased significantly the AS and AS/AR groups within one year post operation, whilst the neo-aortic root size remained stable throughout follow-up. CONCLUSIONS: Recovery of LV size post Ross procedure is influenced predominantly by the pre-operative LV size, in particular the indexed LV end diastolic diameter. The pre-operative valve lesion was not predictive of larger ventricular dimensions post AVR, but independent predictors of a larger ventricular dimensions post operation included female gender, enlarged pre-operative LVEDDi and the presence of mild AR in the first post operative year. Those with mild post-operative AR did not have progressive LV enlargement, thus the clinical significance of this finding remains unclear.

Favourable anatomy after end-to-side repair of interrupted aortic arch.

OBJECTIVE: To evaluate cardiovascular outcomes in patients with aortic arch repair and their possible correlation with arch geometry. METHODS: Ten patients who underwent end-to-side repair for aortic arch interruption (IAA), older than 10 years were compared to a cohort of 10 post coarctation (CoA) repair patients matched for age, sex and age at repair. Mean age at operation was 9.7±6.5 days. Patients underwent a resting and 24 h blood pressure measurements, exercise study, MRI, transthoracic echocardiography and vascular studies. RESULTS: Seven patients developed hypertension, two from IAA group and five from CoA group. Nine patients (45%) had gothic arch geometry, three from IAA group and six from CoA group. Despite differences in arch geometry, both groups had normal LV mass, LV function and vascular function. CONCLUSION: No differences in functional or morphologic outcomes could be demonstrated between the end-to-side repair of the arch by sternotomy and the conventional coarctation repair by thoracotomy. A favourable arch geometry can be achieved after the end-to-side repair of the aortic arch. In the present study, we could not correlate adverse arch geometry with any adverse cardio-vascular outcomes. After neonatal arch repair, the contributive role of aortic arch geometry to late hypertension remains uncertain.