Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.

BACKGROUND AND AIMS: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI. METHODS: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries. RESULTS: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively. CONCLUSIONS: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs.

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.

Percutaneous approach to residual pulmonary bifurcation stenosis in conotruncal diseases.

Residual stenosis after right ventricle outflow tract surgery represents a major issue to manage in the children and adult patient with conotruncal defects. Despite a detailed multimodality imaging, the anatomy of distal pulmonary trunk and pulmonary artery bifurcation may be challenging in these patients.The aim of this study was to analyse retrospectively the outcome of the percutaneous transcatheter treatment in children with post-surgical stenosis of pulmonary artery bifurcation.We enrolled 39 patients with a median age of 6.0 years. Standard high-pressure balloon dilation was attempted in 33 patients, effective in 5 of them. Pulmonary branch stenting was performed in 10 patients, effective in 6. A kissing balloon approach was chosen in 17 patients (6 after angioplasty or stenting failure), and this technique was effective in 16 cases. Finally, a bifurcation stenting was performed in 10 patients (second step in 9 cases), effective in all the cases. None of the patients approached by kissing balloon needed a bifurcation stenting.In conclusion, standard balloon angioplasty and standard stenting might be ineffective in post-surgical stenosis involving pulmonary artery bifurcation. In this population, kissing balloon or bifurcation stenting, followed by side branch de-jailing, may be more effective in relieving the gradient.

The impact of dominant ventricle morphology and additional ventricular chamber size on clinical outcomes in patients with Fontan circulation.

OBJECTIVES: The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes. METHODS: Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed. Clinical, cardiac MRI, and cardiopulmonary exercise test data from the last follow-up were analysed. RESULTS: Fifty patients were analysed: left dominance was present in 29 patients (58%, median age 20 years, interquartile range:16-26). At a median follow-up after the Fontan operation was 16 years (interquartile range: 4-42), NYHA classes III and IV was present in 3 patients (6%), 4 (8%) underwent Fontan conversion, 2 (4%) were listed for heart transplantation, and 2 (4%) died. Statistical analysis showed that the additional ventricular chamber was larger (>20 mL/m2) in patients with a right dominant ventricle (p = 0.01), and right dominance was associated with a higher incidence of post-operative low-cardiac output syndrome (p = 0.043). Left ventricular dominance was associated with a better ejection fraction (p = 0.04), less extent of late gadolinium enhancement (p = 0.022), higher metabolic equivalents (p = 0.01), and higher peak oxygen consumption (p = 0.033). A larger additional ventricular chamber was associated with a higher need for post-operative extracorporeal membrane oxygenation support (p = 0.007), but it did not influence functional parameters on cardiac MRI or cardiopulmonary exercise test. CONCLUSIONS: In Fontan patients, left ventricular dominance correlated to better functional outcomes. Conversely, a larger additional ventricular chamber is more frequent in right ventricular dominance and can negatively affect the early post-Fontan course.

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.

Systemic sclerosis sine scleroderma in children.

OBJECTIVE: Juvenile systemic sclerosis (JSSc) is a rare condition in childhood and its variety with no skin involvement, sine scleroderma (ssJSSc), is anecdotal. We report the first case series of patients with ssJSSc. METHODS: Demographic, clinical and laboratory data of patients with JSSc followed at our centre were retrospectively collected. Patients with no skin involvement but with all of the features RP, positive ANA, intestinal dysmotility and/or interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH) and/or cardiac or renal involvement typical of scleroderma were defined as having ssJSSc and compared with those with classic JSSc (cJSSc). RESULTS: Among 52 JSSc patients seen in 20 years, five (9.6%) presented with ssJSSc. Their clinical features and those of the only two patients reported in the literature so far were compared with classic JSSc with available complete data. Six patients had cardiac involvement as presenting feature, three primary cardiomyopathy, three secondary to PAH. Two patients died after a brief disease course and one rapidly underwent heart transplantation. In comparison with cJSSc, ssJSSc showed a significantly longer diagnostic delay (20.1 vs 8.3 months, P = 0.017), higher frequency of cardiac involvement (85.7 vs 15.6%, P = 0.001) and worse outcome, intended as mortality or end-stage organ failure rates (42.9% vs 6.2%, P < 0.001). CONCLUSION: Cardiac involvement represents the most important characteristic of ssJSSc and carries a high morbidity and mortality rate. The longer delay in diagnosis underlines the need for a comprehensive rheumatological work-up in patients with isolated cardiomyopathy or PAH/ILD.

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.

Early detection of ventricular dysfunction in juvenile systemic sclerosis by speckle tracking echocardiography.

OBJECTIVE: Cardiac involvement is the most important cause of mortality in juvenile systemic sclerosis (JSSc). Recent reports in adult patients underline that traditional techniques of imaging are inadequate to assess the subclinical cardiac involvement, while speckle tracking echocardiography (STE) is able to identify ventricular dysfunctions in the early stages. The aim of our study was to assess the role of STE in JSSc. METHODS: Demographic, clinical and laboratory data were collected from patients with JSSc. Cardiac investigations performed at baseline (T0) and 18 (T18) and 36 months (T36) follow-up included electrocardiography, conventional echocardiography with measurement of the ejection fraction (EF) and STE with assessment of left and right ventricular global longitudinal strain (LV-GLS and RV-GLS). Cardiac parameters have been compared with demographic characteristics and disease severity, assessed by the Juvenile Systemic Sclerosis Severity Score (J4S). RESULTS: A total of 18 patients, 12 (67%) females, entered the study. At T0, electrocardiography was abnormal in three patients, EF was reduced in one, LV-GLS was abnormal in three (16.7%) and RV-GLS was abnormal in five (27.8%). At T18, EF remained stable while at T36 the result decreased in seven of nine patients. At the same time, LV-GLS also worsened (from -21.6% to -18.2%, P = 0.01). LV-GLS and RV-GLS at baseline showed a significant correlation with J4S (P = 0.012 and P = 0.02, respectively). CONCLUSION: STE is more sensitive than standard echocardiography to identify cardiac involvement in JSSc. Over time, we observed a gradual worsening of LV-GLS, a sign of left ventricular dysfunction, that anticipated by several months the decrease of EF.

Surgical strategies for the management of end-stage heart failure in infants and children: A 15-year experience with a patient-tailored approach.

End-stage heart failure (ESHF) in pediatric age is an ongoing challenge. Heart transplantation is the final option, but its long-term outcomes are still suboptimal in children. An alternative patient-tailored surgical protocol to manage ESHF in children is described. Retrospective, single-center analysis of pediatric patients admitted to our institution between April 2004 and February 2021 for ESHF. Our current protocol is as follows: (a) Patients <1 year with isolated left ventricular dysfunction due to dilated cardiomyopathy underwent pulmonary artery banding (PAB). (b) Patients <10 years and <20 kg, who did not meet previous criteria were managed with Berlin Heart EXCOR. (c) Patients >10 years or >20 kg, underwent placement of intracorporeal Heartware. Primary outcomes were survival, transplant incidence, and postoperative adverse events. A total of 24 patients (mean age 5.3 ± 5.9 years) underwent 26 procedures: PAB in 6 patients, Berlin Heart in 11, and Heartware in 7. Two patients shifted from PAB to Berlin Heart. Overall survival at 1-year follow-up and 5-year follow-up was 78.7% (95%CI = 62%-95.4%) and 74.1% (95%CI = 56.1%-92.1%), respectively. Berlin Heart was adopted in higher-risk settings showing inferior outcomes, whereas a PAB enabled 67% of patients to avoid transplantation, with no mortality. An integrated, patient-tailored surgical strategy, comprehensive of PAB and different types of ventricular assist devices, can provide satisfactory medium-term results for bridging to transplant or recovery. The early postoperative period is critical and requires strict clinical vigilance. Selected infants can benefit from PAB that has demonstrated to be a safe bridge to recovery.

A case of atrial septal defect associated with anomalous sinoatrial node artery in pulmonary atresia with intact ventricular septum.

An 11-year-old boy affected by pulmonary atresia with intact ventricular septum (AP-IVS) was listed for percutaneous pulmonary valvuloplasty and closure of multi-fenestrated atrial septal defect (ASD). Intra-procedural transesophageal echocardiography arose the suspect of abnormal coronary artery pattern while selective angiography documented a single sinoatrial node artery (SANa) with an unusual retro-aortic course. As consequence, we proceeded to effectively close the defects with a not self-centering device placed in the most central side hole. This case supports the hypothesis that sometimes arrhythmic complication during ASD closure procedures might be due to unrecognized injury of the SANa.

Multidisciplinary management of a rare case of mixed total anomalous pulmonary venous connection.

INTRODUCTION: Mixed total anomalous pulmonary venous connection (TAPVC) is a extremely rare congenital heart disease. METHODS: We report the initial management of a case of Mixed total anomalous pulmonary venous connection associated to right extralobar bronchopulmonary sequestration (BPS). RESULTS: Mixed TAPVC associated to right extra-lobar BPS was diagnosed at birth in a full-term newborn. At one month of age, the patient underwent embolization of the BPS, complicated by coil entrapment in the right common iliac artery requiring urgent laparotomy. Few days later, the congenital cardiac repair was accomplished uneventfully. At 12-months follow-up, the patient did not have pulmonary hypertension, but presented a moderate stenosis of the right femoral artery, which was effectively treated with anticoagulation therapy. CONCLUSIONS: The multidisciplinary approach allowed a successful treatment of these complex anomalies and the related potential complications.

Severe scoliosis and percutaneous atrial septal defect closure, a word of caution.

In the presence of complex septal morphology or extra-cardiac factors like severe scoliosis or diaphragm paralysis, the procedure may become more challenging, and pre-formed sheaths and devices thought to facilitate the implantation might turn into a problem to address. Similarly to Herron and Kobayashi, we have experienced a similar issue during atrial septal defect closure with Gore Cardioform atrial septal defect device® in a 12-year-old female patient with severe scoliosis. After the conformation of the locking loop and the removal of the safety cord, the Gore Cardioform atrial septal defect device prolapsed in the right atrium.

Rituximab for rapidly progressive juvenile systemic sclerosis.

OBJECTIVE: Juvenile systemic sclerosis (JSSc) with rapidly progressive course is a life-threatening condition associated with a poor prognosis. Recently, rituximab (RTX) has been shown to be a promising treatment for adult patients with SSc. We present a series of four patients with rapidly progressive JSSc successfully treated with RTX. METHODS: Clinical, laboratory and functional parameters were collected from four patients with rapidly progressive JSSc treated with RTX for at least 1 year. All patients underwent four yearly courses of i.v. RTX 375 mg/m2 on day 0 and 14, at 3-month intervals. Low dose oral prednisone and MMF were also administered. Data were recorded at baseline and every 6 months and included pulmonary and myocardial function parameters, muscular, vascular and skin changes. The Juvenile Systemic Sclerosis Severity Score (J4S) estimated the overall disease severity over time. RESULTS: Four patients (three males, one female), aged 8-17 years, entered the study. Three patients presented with prevalent cardiac involvement, one with severe pulmonary involvement. After 1 year of RTX treatment, all patients showed significant improvement of J4S, Raynaud's phenomenon and cutaneous involvement. Among those with prevalent cardiac involvement, two showed an improvement of the myocardial function (left ventricular ejection fraction [EF] +37% and +19%, respectively) and in the third arrhythmias disappeared. The patient with severe pulmonary involvement showed a significant improvement of the respiratory function (forced vital capacity +46%, forced expiratory volume in 1 s +33%, diffusing capacity of the lung for carbon monoxide [DLCO] +30%). No major side effects were reported. CONCLUSIONS: Our data suggest that a combination of RTX and MMF is effective in arresting the rapid progression of JSSc.

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 case of AndraStent® fracture in a patient with aortic coarctation: a review of the literature.

Percutaneous treatment of aortic coarctation is based on angioplasty and/or stenting of the isthmus. We report a case of a 28-year-old girl suffering from aortic coarctation syndrome (coarctation + ventricular septal defect + bicuspid aorta). She underwent coarctectomy with subclavian flap and pulmonary bandage followed by ventricular septal defect closure and bandage removal in her first year of life. When she was 27 years old, a follow-up echocardiography detected an isthmic pressure gradient and a demodulated Doppler in abdominal aorta. A cardiac catheterisation confirmed the diagnosis of aortic re-coarctation. An AndraStent® XL 48 mm was implanted with a resolution of the isthmic gradient. One year later, because of the reappearance of demodulated Doppler in abdominal aorta, a chest X-ray was performed, which showed a stent third-grade fracture. The fracture was corrected by positioning a covered stent cheatham platinum 45 mm through the fragments. The rarest complication after stenting procedures is the fracture of the device with an incidence between 0.01% and 0.08%. Pressure overload beyond the elastic threshold of the material and the pulsatile tension exerted by the blood flow on the walls of the stent are the main mechanisms at the base of the fracture, together with the compliance of the tissue. A vessel that underwent multiple surgical rearrangements could interfere with and complicate the physiopathology at the basis of the fracture. In conclusion, stenting is a safe technique to treat aortic coarctation; stent fracture is a rare event, and different anatomical and haemodynamic factors are related to this complication.

Surgical repair of aortic coarctation in pediatric age: A single center two decades experience.

BACKGROUND: To evaluate early and long-term results of surgical treatment of aortic coarctation (CoAo) in infants and children. METHODS: A retrospective clinical review of patients less than 18 years with CoAo, undergoing surgery between 1995 and 2015. Data were retrieved from our institutional database, to identify preoperative and postoperative characteristics. Statistical analysis was performed by bivariate, Cox's, and logistic regression analysis. RESULTS: Three hundred forty-one consecutive patients (male/female: 192/149; the median age at surgery of 25 days; interquartile range [IQR], 10-143 days) were included; 187 patients were less than 1 month (54.9%); 276 underwent extended end-to-end anastomosis (EEEA) (80.9%). Hypoplastic aortic arch (HAA) occurred in 34.6% and bicuspid aortic valve in 21.1%. The isolated type was present in 249 (73.0%). Significant postoperative complications occurred in 5.6%. Thirty-day mortality was 1.4%. At a median follow-up of 10.2 years (IQR, 6-15 years; FU completeness, 91.2%), there were eight late deaths (2.6%), most in the complex type. Among 298 survivors, 284 (95.3%) were in NYHA class I; 10 (3.0%) were on antihypertensive treatment. Reinterventions on aortic arch occurred only in 4.5%, being HAA a significant risk factor for reoperation ( P = 0.00173). Freedom from mortality and reintervention on aorta at 21 years were 93.5% and 93.6%, respectively. CONCLUSIONS: Surgical repair of CoAo by EEEA without CPBP is a safe and low-risk procedure, concerning either early or late outcomes, despite the presence of HAA and neonatal age can influence recoarctation. Most patients are clinically well in the long-term, and only a few require antihypertensive therapy.

Arterial switch operation for transposition of the great arteries: A single-centre 32-year experience.

OBJECTIVE: Arterial switch operation (ASO) is nowadays the standard of care for neonates with D-transposition of the great arteries (D-TGA). We evaluated our early and late results with ASO for D-TGA. MATERIALS AND METHODS: We collected data on 267 patients with D-TGA, either with intact ventricular septum (D-TGA/IVS; n = 182, 68%) and with other associated congenital heart malformations (complex D-TGA; n = 85, 32%) that underwent ASO between January 1987 and July 2018. RESULTS: Median age at ASO was 8 days (interquartile range [IQR], 6-12 days). Fifteen patients (5.6%) died in hospital (6/182 with D-TGA/IVS, 3.3% and 9/85 with complex D-TGA, 11%; P = 0.02). Median follow-up time was 10.2 years (IQR, 3.7-18 years). There were 2 (0.8%) late sudden deaths. Overall survival at 10 and 20 years was 94% and 93%, respectively. Thirty-five patients (14%) required either reoperations or reinterventions, mainly for right ventricular outflow tract obstruction (n = 28, 11%). Freedom from reoperation/reintervention at 10 and 20 years was 87% and 78%, respectively. All patients were in NYHA I at latest clinical examination. Six over 173 patients (3.4%) who underwent a postoperative evaluation of their coronaries presented acquired anomalies. Forty-four patients (17%) who performed a cardiopulmonary exercise testing (CPET) have a predicted VO 2 comparable to normal peers. CONCLUSIONS: The results of ASO for D-TGA are excellent, with a fairly low mortality and reoperation/reintervention rate. Functional capacity evaluated with CPET is comparable to normal peers. Continuous follow-up for detecting asymptomatic acquired coronary artery disease is mandatory. A reassessment of competitive sport eligibility criteria for specific D-TGA patients should be considered.

Can myocardial remodeling be a useful surrogate predictor of myocardial iron load? A 3D echocardiographic multicentric study.

The relationship between myocardial iron load and eccentric myocardial remodeling remains an under-investigated area; it was thought that remodeling is rather linked to fibrosis. This study aims to determine whether or not measures of remodeling can be used as predictors of myocardial iron. For this purpose, 60 patients with thalassemia were studied with 3D echocardiography and myocardial relaxometry (T2*) by Cardiac MRI. 3D derived sphericity index was significantly higher in patients with myocardial iron load. It was correlated with T2* with a 100% sensitivity and specificity (cut-off value of 0.34) to discriminate between patients with and without myocardial iron overload.

Early modifications of cardiac function in preterm neonates using speckle tracking echocardiography.

BACKGROUND AND AIMS: Preterm newborns undergo hemodynamic challenges in the postnatal period. The aim of this study was to investigate myocardial mechanics changes in the postnatal period in preterm infants using speckle tracking echocardiography (STE). MATERIAL AND METHODS: Thirty-nine preterm infants ≤34 weeks' gestation underwent cardiac ultrasound evaluation during the first 96 hours of life. A repeated echocardiogram at 3 weeks of age was performed. Echocardiographic assessment involved left ventricular ejection fraction, mitral E/A ratio, S' and E' velocities, E/E' ratio, tricuspid annular plane systolic excursion (TAPSE), left atrium-to-aorta ratio, ductal diameter and ductal shunt pattern. Left ventricular longitudinal, circumferential and radial strain, apex-basal rotation and twist were measured from the apical 4-chamber and short-axis views using STE. RESULTS: The mean gestational age was 30 ± 2.7 weeks with a mean birth weight of 1318 ± 485 g. Apical segments demonstrated higher longitudinal strain than basal and mid-ones. In all gestational ages, endocardial longitudinal strain was higher than the epicardial. Epicardial longitudinal strain significantly increased during the first 3 weeks, resulting in the change in basal rotation from counterclockwise to clockwise and thus in the acquisition of twist. Deformation parameters were higher in infants with a hemodynamic significant patents ductus arteriosus. CONCLUSIONS: Echocardiographic assessment of myocardial deformation parameters is feasible in preterm infants. Our data suggest that the maturational process of the myocardium is due to the development of the epicardial layer after birth, which allows the acquisition of the twist.

Speckle Tracking in ALCAPA Patients After Surgical Repair as Predictor of Residual Coronary Disease.

Surgical re-implantation of the left coronary artery (LCA) is the treatment of choice in anomalous origin of left coronary artery from pulmonary artery (ALCAPA). Despite normalization of left ventricular function after surgery, residual coronary lesions or myocardial fibrosis may be found. The aim of this study was to detect regional left ventricular dysfunction predictive of coronary lesions or residual myocardial fibrosis using speckle tracking. We enrolled ten patients treated with surgical re-implantation of LCA for ALCAPA. All patients were asymptomatic, and ejection fraction (EF) was normal. Using S-SR imaging, we studied longitudinal, radial, and circumferential function. A cardiac MRI was performed to assess myocardial fibrosis and the anatomy of the coronaries. In case of suspected coronary stenosis, a coronary angiography was performed. Finally, 20 normal subjects were enrolled as control group. Median age at surgery was 188 days, and mean follow-up was 8.7 ± 4.7 years. Longitudinal and circumferential functions were reduced in LCA territory compared to RCA territory and normal. MRI showed LCA stenosis in three of ten patients, confirmed by coronary angiography: these patients had the lowest longitudinal strain values in LCA territories (-11.7, -14.7 and -14.8%). Radial strain was preserved (Normal 45.6 ± 12.1, ALCAPA 43.5 ± 10.7%, p = ns), while circumferential strain was mildly depressed (-23.5 ± 3.8 vs. -20.3 ± 2.0%, p < 0.05). After LCA re-implantation, ALCAPA patients showed a residual sub-endocardial damage in LCA territories. Despite a normal systolic and diastolic function, the prevalence of residual coronary lesions was high. A mean longitudinal strain >-15% in LCA territories was able to identify those patients.