Impact of age over 70 years in the new allocation system on the outcomes of heart transplantation in the US.

BACKGROUND: United Network for Organ Sharing (UNOS) allocation criteria changed in 2018 to accommodate the increased prevalence of patients on a ventricular assist device as a bridge to heart transplant and prioritize sicker people in anticipation of a heart graft. We aimed to assess the impact of patient age in the new allocation policy on mortality following heart transplantation. Secondary outcomes included the effect of age ≥70 on post-transplant events, including stroke, dialysis, pacemaker, and rejection requiring treatment. METHODS: The UNOS Registry was queried to identify patients who underwent heart transplants alone in the US between 2000 and 2021. Patients were divided into groups according to their age (over 70 and under 70 years old). RESULTS: Patients aged over 70 were more likely to require dialysis during follow-up, but less likely to experience rejection requiring treatment, compared with patients aged <70. Age ≥70 in the new allocation system was a significant predictor of 1-year mortality (adjusted HR: 1.41; 95% CI: 1.05-1.91; p = .024), but its effect on 5-year mortality was not significant after adjusting for potential confounders (adjusted HR: 1.27; 95% CI:.97-1.66; p = .077). Undergoing transplantation under the new allocation policy vs the old allocation policy was not a significant predictor of mortality in patients over 70 years old. CONCLUSIONS: Age ≥70 is a significant predictor of 1-year mortality following heart transplantation, but not at 5 and 10 years; however, the new allocation does not seem to have changed the outcomes for this group of patients.

Nationwide hospitalizations of patients with down syndrome and congenital heart disease over a 15-year period.

Down syndrome is one of the most common genetic diseases, generally associated with an increased probability of congenital heart diseases. This increased risk contributes to escalated levels of morbidity and mortality. In this study, we sought to analyze nationwide data of pediatric and adult patients with Down syndrome and congenital heart disease over a 15-year period. Data obtained from the hospital discharge form between 2001 and 2016 of patients diagnosed with Down syndrome in Italy and at least one congenital heart disease were included. Information on 12362 admissions of 6527 patients were included. Age at first admission was 6.2 ± 12.8 years and was a predictor of mortality (HR = 1.51, 95% CI 1.13-2.03, p = 0.006). 3923 (60.1%) patients underwent only one admission, while 2604 (39.9%) underwent multiple (> 1) admissions. There were 5846 (47.3%) admissions for cardiac related symptoms. Multiple admissions (SHR: 3.13; 95% CI: 2.99, 3.27; P < 0.01) and cardiac admissions (SHR: 2.00; 95% CI: 1.92, 2.09; P < 0.01) were associated with an increased risk of additional potential readmissions. There was an increased risk of mortality for patients who had cardiac admissions (HR = 1.45, 95% CI: 1.08-1.94, p = 0.012), and for those who underwent at least 1 cardiac surgical procedure (HR = 1.51, 95% CI 1.13-2.03, p = 0.006). CONCLUSIONS: A younger age at first admission is a predictor for mortality in patients with Down syndrome and congenital heart disease. If patients undergo more than one admission, the risk of further readmissions increases. There is a pivotal role for heart disease in influencing the hospitalization rate and subsequent mortality. WHAT IS KNOWN: • Down syndrome individuals often face an increased risk of congenital heart diseases. • Congenital heart diseases contribute significantly to morbidity and mortality in Down syndrome patients. WHAT IS NEW: • This study analyzes nationwide data covering a 15-year period of pediatric and adult patients in Italy with Down syndrome and congenital heart disease. • It identifies a younger age at first admission as a predictor for mortality in these patients, emphasizing the criticality of early intervention. • Demonstrates a correlation between multiple admissions, particularly those related to cardiac issues, and an increased risk of further readmissions, providing insights into the ongoing healthcare needs of these individuals.

Enhancing parental understanding of congenital heart disease through personalized prenatal counseling with 3D printed hearts.

OBJECTIVES: In addition to a correct prenatal diagnosis of congenital heart disease (CHD), comprehensive parental counseling is crucial to ensure that parents are well-informed about the condition of the fetus. This study aims to investigate whether there is a significant difference in the information acquired by parents through traditional counseling, utilizing 2-dimensional (2D) illustrations and images, compared to an advanced approach utilizing personalized three-dimensional (3D) printed models of the fetal heart developed from 3D ultrasound imaging. METHODS: This study, designed as a pilot randomized control trial, enrolled pregnant women with gestational ages greater than 18 weeks, whose fetuses were diagnosed with CHD and referred to our center between November, 2020 and June, 2021. Two groups of patients were included in the study. The first group received standard medical counseling with 2D images and illustrations, while the second group underwent advanced counseling with 3D-printed patient-specific heart models. Both groups were then required to complete the same survey in which the knowledge of the CHD was investigated. The 3D models were created from 3D ultrasound imaging and printed using resin materials in both 1:1 and 5:1 scale. RESULTS: A comparison of the scores obtained from the two groups revealed that 3D visualization of the fetus's heart has the potential to increase parental knowledge about CHD and the required surgical procedures. Furthermore, all couples expressed interest in receiving a 1:1 scale model of their baby's heart. CONCLUSION: Personalized prenatal counseling with 3D-ultrasound-based heart models positively impacts parents' understanding of CHD. The use of 3D models provides a more comprehensive and accessible representation of the condition, contributing to an increased knowledge gain, and potentially helping to support informed decisions regarding their child's care.

Intracorporeal LVAD implantation in pediatric patients: A single-center 10 years' experience.

BACKGROUND: Mechanical cardiac support is currently an effective strategy to reduce morbidity and mortality in pediatric patients. However, solid evidence regarding the feasibility of intracorporeal devices in children still needs to be provided. We report our 10-year experience with intracorporeal left ventricular assist devices (LVAD) in children. MATERIALS AND METHODS: We included all patients undergoing intracorporeal, continuous-flow LVAD implantation between 2012 and 2022. Baseline and postoperative data were collected from the institutional database. RESULTS: Seven HeartWare and 4 HeartMate3 were implanted in 11 patients (median age 13.9 years, median body surface area - BSA - 1.42 m2, IQR 1.06-1.68). The most frequent indication to LVAD implant was dilated cardiomyopathy (72.7%). All candidates underwent a thorough preoperative advanced imaging. Three-dimensional reconstructions and implant fit simulation were performed when BSA was <1.2 m2, weight <30 kg, or internal transverse thoracic diameter <20 cm. There was no operative death. The most common postoperative complication was surgical re-exploration due to bleeding (27.3%). One patient died of severe neurological complications after about 3 months of hospitalization. No late deaths or unplanned re-hospitalizations occurred in the remaining 10, 6 of whom were discharged home. There were no major complications at the follow-up. All survivors underwent successful heart transplantation. CONCLUSIONS: Intracorporeal LVAD implantation proved to be a potentially feasible and safe option in young teenagers and children whose BSA was >1.0 m2. In borderline cases, the 3D reconstruction with implant fit simulation can effectively help to identify those patients who can safely undergo intrathoracic LVAD implantation.

Impact of new allocation system on length of stay following heart transplantation in the United States.

BACKGROUND: United Network for Organ Sharing (UNOS) allocation criteria changed in 2018 to accommodate the increased prevalence of ventricular assist device use as a bridge to heart transplant, which consequently prioritized sicker patients. We aimed to assess the impact of this new allocation policy on the length of stay following heart transplantation. Secondary outcomes include other risk factors for prolonged hospitalization and its effect on mortality and postoperative complications. METHODS: The UNOS Registry was queried to identify patients who underwent isolated heart transplants in the United States between 2001 and 2023. Patients were divided into quartiles according to their respective length of stay. RESULTS: A total of 57 020 patients were included, 15 357 of which were allocated with the new system. The median hospital length of stay was 15 days (mean 22.7 days). Length of stay was longer in the new allocation era (25 ± 30 vs. 22 ± 27 days, p < .001). The longer length of stay was associated with increased 5-year mortality in the new allocation system (aHR: 1.18; 95% CI: 1.15, 1.20; p-value: < .001). CONCLUSION: Longer hospital stays and associated observed increased risk for mortality in the era after the allocation criteria change reflect the rationale of this shift which was to prioritize heart transplants for sicker patients. Further studies are needed to track the progress of surgical and perioperative management of these studies over time.

Hybrid approach for end-stage heart failure treatment in a 6-month-old baby.

A 6-month-old baby girl, with a history of 2 months of intensive care management and two episodes of cardiac arrest, was transferred from another European country to initiate the "Giessen approach" for end-stage heart failure in children. At the admission, left ventricular ejection fraction was 20%. Severe mitral valve regurgitation and severe left atrial dilatation were present. Right ventricular function was preserved, and tricuspid valve regurgitation was mild. As a result, the patient underwent surgical pulmonary artery banding. Additionally, unloading of the left atrium was achieved by implanting an 8 mm atrial flow regulator device through a hybrid per-atrial approach. Two months after the procedure, the patient was progressively weaned from the inotropes and transferred to the ward.

3D Reconstruction for a Complex Pediatric Redo Aortic Arch Surgery in a 9-Year-Old Ukrainian Refugee.

Redo aortic surgery poses significant challenges, particularly in complex scenarios involving congenital heart conditions that have been previously operated on several years prior. The integration of three-dimensional (3D) reconstruction and printing holds immense potential to greatly improve surgical precision, particularly in critical situations.

Three-Dimensional Printing Application in a Challenging Case of Type II Endoleak.

PURPOSE: To highlight the importance of 3-dimensional (3D) arterial printing in a case of type II endoleak (EL) embolization. CASE REPORT: An 81-year-old patient, previously treated with endovascular aortic repair (EVAR), developed a type II EL requiring treatment. The EL's main origin was the median sacral artery (MSA). Initial attempts in embolization via a transsealing and transarterial approach were unsuccessful owing to extremely tortuous arterial communications between the left hypogastric artery and the MSA. The construction of a clear resin 3D model of the aorta and iliolumbar arteries improved anatomy understanding and moreover allowed a preoperative simulation. The subsequent transarterial attempt in embolization was resolutive, significantly reducing total procedural time and radiation dose. CONCLUSION: Printing of clear resin 3D arterial models facilitates type II EL transarterial embolization, improving anatomy understanding and allowing simple fluoroscopy-free simulations. CLINICAL IMPACT: The aim of our work is to highlight the additional value of three-dimensional (3D) printing during preoperative planning of challenging endovascular cases. To our best knowledge, this is the first report about 3D printing use in a case of type II endoleak (EL). We believe that realizing life-size aortic models in selected cases where a complex type II EL embolization procedure is indicated, could lead to a better understanding of arterial anatomy, thus allowing to increase procedural success and reduce operative and most importantly fluoroscopy time.

Rescue ross procedure and mitral valve repair on a low birth weight preterm neonate.

Although mid- and long-term outcomes after the Ross procedure for aortic valve disease have been increasingly improving over the years, this is still a rather challenging operation in neonates and small children. This is particularly true for patients with associated congenital heart defects and critical clinical conditions. Herein we describe the application of this procedure as a rescue operation in emergency circumstances in a low-birth-weight neonate with severe aortic stenosis, aortic regurgitation and mitral regurgitation after a previous aortic coartectomy.

Effectiveness of Repair of Aortic Coarctation in Neonates: A Long-Term experience.

To evaluate early and long-term results of surgical treatment of aortic coarctation (CoAo) in neonates. This is a retrospective clinical review of neonates with CoAo, who underwent surgery between 1995 and 2019. Data were retrieved from our institutional database, to identify preoperative and postoperative characteristics. Statistical analysis was performed by means of relative risk ratio and Cox and logistic multivariate analysis. 218 consecutive neonates (M/F: 129/89, median age 11 days, IQR 7-17 days) were included; 202 (92.7%) had a left thoracotomy; 178 underwent extended end-to-end anastomosis (EEEA, 81.6%). Hypoplastic aortic arch (HAA) was present in 102 patients (46.8%); complex cardiac anomalies in 85 (39%). Significant postoperative complications occurred in 20 (9.2%). Thirty-day mortality was 2.3% (most in complex types). At a median follow-up of 10.4 years (IQR 5.6-15.0 years; FU completeness 95.9%), there were 8 late deaths (3.7%), all associated to complex CoAo. Among 196 survivors, 177 (93.2%) were in NYHA class I; re-interventions on aortic arch occurred in 9.2% (2.0% were surgical). Freedom from mortality and re-intervention on aorta at 10 years were 94.3% and 96.7%, respectively. Surgical repair of CoAo in newborns without CPB in our series was safe and low-risk, with excellent early and late outcomes.

Anomalous left coronary artery from pulmonary artery repair: Outcomes from the European Congenital Heart Surgeons Association Database.

INTRODUCTION: We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database. MATERIALS AND METHODS: From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS). RESULTS: The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001). CONCLUSIONS: ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients.

Mitochondrial transplantation by intra-arterial injection for acute kidney injury.

Acute kidney injury is a common clinical disorder and one of the major causes of morbidity and mortality in the postoperative period. In this study, the safety and efficacy of autologous mitochondrial transplantation by intra-arterial injection for renal protection in a swine model of bilateral renal ischemia-reperfusion injury were investigated. Female Yorkshire pigs underwent percutaneous bilateral temporary occlusion of the renal arteries with balloon catheters. Following 60 min of ischemia, the balloon catheters were deflated and animals received either autologous mitochondria suspended in vehicle or vehicle alone, delivered as a single bolus to the renal arteries. The injected mitochondria were rapidly taken up by the kidney and were distributed throughout the tubular epithelium of the cortex and medulla. There were no safety-related issues detected with mitochondrial transplantation. Following 24 h of reperfusion, estimated glomerular filtration rate and urine output were significantly increased while serum creatinine and blood urea nitrogen were significantly decreased in swine that received mitochondria compared with those that received vehicle. Gross anatomy, histopathological analysis, acute tubular necrosis scoring, and transmission electron microscopy showed that the renal cortex of the vehicle-treated group had extensive coagulative necrosis of primarily proximal tubules, while the mitochondrial transplanted kidney showed only patchy mild acute tubular injury. Renal cortex IL-6 expression was significantly increased in vehicle-treated kidneys compared with the kidneys that received mitochondrial transplantation. These results demonstrate that mitochondrial transplantation by intra-arterial injection provides renal protection from ischemia-reperfusion injury, significantly enhancing renal function and reducing renal damage.

Mitochondrial transplantation enhances murine lung viability and recovery after ischemia-reperfusion injury.

The most common cause of acute lung injury is ischemia-reperfusion injury (IRI), during which mitochondrial damage occurs. We have previously demonstrated that mitochondrial transplantation is an efficacious therapy to replace or augment mitochondria damaged by IRI, allowing for enhanced muscle viability and function in cardiac tissue. Here, we investigate the efficacy of mitochondrial transplantation in a murine lung IRI model using male C57BL/6J mice. Transient ischemia was induced by applying a microvascular clamp on the left hilum for 2 h. Upon reperfusion mice received either vehicle or vehicle-containing mitochondria either by vascular delivery (Mito V) through the pulmonary artery or by aerosol delivery (Mito Neb) via the trachea (nebulization). Sham control mice underwent thoracotomy without hilar clamping and were ventilated for 2 h before returning to the cage. After 24 h recovery, lung mechanics were assessed and lungs were collected for analysis. Our results demonstrated that at 24 h of reperfusion, dynamic compliance and inspiratory capacity were significantly increased and resistance, tissue damping, elastance, and peak inspiratory pressure (Mito V only) were significantly decreased (P < 0.05) in Mito groups as compared with their respective vehicle groups. Neutrophil infiltration, interstitial edema, and apoptosis were significantly decreased (P < 0.05) in Mito groups as compared with vehicles. No significant differences in cytokines and chemokines between groups were shown. All lung mechanics results in Mito groups except peak inspiratory pressure in Mito Neb showed no significant differences (P > 0.05) as compared with Sham. These results conclude that mitochondrial transplantation by vascular delivery or nebulization improves lung mechanics and decreases lung tissue injury.

Mitochondrial transplantation ameliorates acute limb ischemia.

OBJECTIVE: Acute limb ischemia (ALI), the most challenging form of ischemia-reperfusion injury (IRI) in skeletal muscle tissue, leads to decreased skeletal muscle viability and limb function. Mitochondrial injury has been shown to play a key role in skeletal muscle IRI. In previous studies, we have demonstrated that mitochondrial transplantation (MT) is an efficacious therapeutic strategy to replace or to augment mitochondria damaged by IRI, allowing enhanced muscle viability and function in cardiac tissue. In this study, we investigated the efficacy of MT in a murine ALI model. METHODS: C57BL/6J mice (male, 10-12 weeks) were used in a model of ALI. Ischemia was induced by applying a tourniquet on the left hindlimb. After 2 hours of ischemia, the tourniquet was released, and reperfusion of the hindlimb was re-established; either vehicle alone (n = 15) or vehicle containing mitochondria (n = 33) was injected directly into all the muscles of the hindlimb. Mitochondria were delivered at concentrations of 1 × 106 to 1 × 109 per gram wet weight to each muscle, and the animals were allowed to recover. Sham mice received no ischemia or injections but were anesthetized for 2 hours and allowed to recover. After 24 hours of recovery, limb function was assessed by DigiGait (Mouse Specifics Inc, Boston, Mass), and animals were euthanized; the gastrocnemius, soleus, and vastus medialis muscles were collected for analysis. RESULTS: After 24 hours of hindlimb reperfusion, infarct size (percentage of total mass) and apoptosis were significantly decreased (P < .001, each) in the gastrocnemius, soleus, and vastus medialis muscles in MT mice compared with vehicle mice for all mitochondrial concentrations (1 × 106 to 1 × 109 per gram wet weight). DigiGait analysis at 24 hours of reperfusion showed that percentage shared stance time was significantly increased (P < .001) and stance factor was significantly decreased (P = .001) in vehicle compared with MT and sham mice. No significant differences in percentage shared stance time (P = .160) or stance factor (P = .545) were observed between MT and sham mice. CONCLUSIONS: MT ameliorates skeletal muscle injury and enhances hindlimb function in the murine model of ALI.

Modified extracardiac Fontan with a fenestrated pericardial patch.

Extracardiac Fontan is a preferred treatment strategy in many centers treating patients with single ventricle physiology, and many of these centers regularly include a fenestration between the extracardiac conduit and the common atrium. Spontaneous closure of the fenestration is a common complication of this technique and is independently associated with increased morbidity and mortality. Recently, we introduced a novel technique for fenestration of the extracardiac conduit wherein a pericardial patch is utilized at the fenestration point with excellent outcomes in the midterm fenestration patency rates, thus reducing the risk of acute post-Fontan complications.

A Multi-Mode System for Myocardial Functional and Physiological Assessment during Ex Situ Heart Perfusion.

Ex situ heart perfusion (ESHP) has proven to be an important and valuable step toward better preservation of donor hearts for heart transplantation. Currently, few ESHP systems allow for a convenient functional and physiological evaluation of the heart. We sought to establish a simple system that provides functional and physiological assessment of the heart during ESHP. The ESHP circuit consists of an oxygenator, a heart-lung machine, a heater-cooler unit, an anesthesia gas blender, and a collection funnel. Female Yorkshire pig hearts (n = 10) had del Nido cardioplegia (4°C) administered, excised, and attached to the perfusion system. Hearts were perfused retrogradely into the aortic root for 2 hours before converting the system to an isovolumic mode or a working mode for further 2 hours. Blood samples were analyzed to measure metabolic parameters. During the isovolumic mode (n = 5), a balloon inserted in the left ventricular (LV) cavity was inflated so that an end-diastolic pressure of 6-8 mmHg was reached. During the working mode (n = 5), perfusion in the aortic root was redirected into left atrium (LA) using a compliance chamber which maintained an LA pressure of 6-8 mmHg. Another compliance chamber was used to provide an afterload of 40-50 mmHg. Hemodynamic and metabolic conditions remained stable and consistent for a period of 4 hours of ESHP in both isovolumic mode (LV developed pressure: 101.0 ± 3.5 vs. 99.7 ± 6.8 mmHg, p = .979, at 2 and 4 hours, respectively) and working mode (LV developed pressure: 91.0 ± 2.6 vs. 90.7 ± 2.5 mmHg, p = .942, at 2 and 4 hours, respectively). The present study proposed a novel ESHP system that enables comprehensive functional and metabolic assessment of large mammalian hearts. This system allowed for stable myocardial function for up to 4 hours of perfusion, which would offer great potential for the development of translational therapeutic protocols to improve dysfunctional donated hearts.

Minimally Invasive Implantation of Continuous Flow Left Ventricular Assist Devices: The Evolution of Surgical Techniques in a Single-Center Experience.

In this work we aimed to evaluate the evolution of our surgical experience with the implantation of a continuous flow left ventricular assist device (LVAD), from the original full sternotomy approach to less invasive surgical strategies including mini-sternotomy and/or mini-thoracotomies. We reviewed all consecutive patients implanted with a continuous flow LVAD at our Institute. To exclude the possible bias related to the device used, out of 91 collected LVADs implants, we selected only those patients (n = 42) who received, between 2012 and 2015, the HeartWare HVAD. The analysis focused on the surgical approach used for the LVAD implant. Most of the patients (95%) were affected by dilated or ischemic cardiomyopathy, with an INTERMACS class I-II in the majority of cases (77%). The LVAD implant was performed through a full sternotomy in 10 patients (24%); the remaining 32 cases (76%) were managed with minimally invasive procedures. These were left mini-thoracotomy with upper mini-sternotomy (20 patients, 62%), right and left mini-thoracotomy (7 patients, 22%), and a recently developed left mini-thoracotomy with outflow graft anastomosis to the left axillary artery (5 patients, 16%). The most common adverse event on device was right heart failure (26%). Eighteen patients (43%) were transplanted. Overall estimated 24 months survival (on device or after transplant) was 68 ± 7%. The causal analysis, adjusted by propensity score weighting baseline data and sample size, showed that left mini-thoracotomy with outflow anastomosis to the left axillary artery resulted in a significantly reduced rate of post implant right heart failure (P < 0.01), and mechanical ventilation time (P = 0.049). To conclude, in our series, by applying mini-invasive implant techniques in the majority of cases, mid-term survival of continuous flow LVADs in severely compromised patients was satisfactory. In the adjusted analysis, the left anterior mini-thoracotomy with outflow anastomosis to the left axillary artery showed the most favorable results.

Pulmonary arteriopexy to prevent pulmonary artery kinking in orthotopic heart transplantation.

Heart transplantation is still the best treatment for patients with end-stage heart failure unresponsive to medical therapy or those treated with mechanical circulatory support. The surgical technique for heart transplantation is fraught with potential complications. One of these potential complications, kinking of the pulmonary artery after anastomosis of the recipient and donor pulmonary arteries, has been reported as a cause of acute right ventricular failure. We describe a technique to ensure proper configuration of the pulmonary artery after heart transplantation via a pulmonary arteriopexy to restore a physiologically appropriate angle of the great vessels.

The natural history and surgical outcome of patients with scimitar syndrome: a multi-centre European study.

Aims: Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status. Methods and results: We collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs). Median age at last follow-up was 11.6 years (interquartile range 4-22 years). Overall survival probability at 30 years of age was 88% [85-92% confidence intervals (CI)] and was lower in patients with associated congenital heart disease (CHD) (P < 0.001) and pulmonary hypertension (P < 0.001). Most patients were asymptomatic at last follow-up (279/451, 62%); STPs were more frequently asymptomatic than CMPs (73% vs. 47%, P < 0.001), with fewer cardiac [odds ratio (OR) 0.42, 95% CI 0.22-0.82] and respiratory symptoms (OR 0.08, 95% CI 0.02-0.28). Many STPs (63/254, 25%) had stenosis/occlusion of the scimitar drainage, and this was associated with a younger age at surgery (OR 0.4, CI 0.21-0.78). Conclusion: Patients with SS have a high overall survival. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. Surgical treatment of SS is beneficial in reducing symptoms, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic evaluation and to the patient's age.

Long-term outcomes following transatrial versus transventricular repair on right ventricular function in tetralogy of Fallot.

BACKGROUND AND AIM OF THE STUDY: Outcomes after repair of tetralogy of Fallot (TOF) are good with either a transventricular (TV) or transatrial (TA) approach. We sought to determine if there is a relationship between the TV or TA approach and right ventricular (RV) function, and the role of residual pulmonary regurgitation (PR) on the long-term outcomes. METHODS: This was a retrospective cohort multicentric study on survivors after surgical repair of TOF (TA versus TV approach, ±transannular patch) between 1990 and 2004. All patients underwent magnetic resonance imaging to assess RV volume, function, and PR. Patients were matched for length of follow-up and age. Clinical adverse events were retrieved from institutional databases. RESULTS: Seventy-nine patients (TA/TV = 37/42, median age 0.3 and 1.0 yrs, respectively) were included. At a median follow-up of 16.6 years (12.5-20.3), there were no differences in freedom from reintervention (either catheter or surgical), RV volumes, function, and PR between the TA and TV groups. Pulmonary valve (PV) replacement was significantly less frequent in the TA subgroup (P = 0.033) and patients with a preserved PV showed significantly lower RV volumes and less adverse events at follow-up. CONCLUSIONS: There is no significant difference in RV volumes and function between the TA and TV. However, the TA approach seems to be protective against PV replacement in the long-term. When PV is not preserved at repair, residual pulmonary regurgitation is a significant cause of late RV dysfunction and dilation, and is associated with a higher rate of late adverse events.