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Current guidelines for the care of heart transplantation recipients recommend routine endomyocardial biopsy and invasive coronary angiography as the cornerstones in the surveillance for acute rejection (AR) and coronary allograft vasculopathy (CAV). Non-invasive tools, including coronary computed tomography angiography and cardiac magnetic resonance, have been introduced into guidelines without roles of their own as gold standards. These techniques also carry the risk of contrast-related kidney injury. There is a need to explore non-invasive approaches providing valuable information while minimizing risks and allowing their application independently of patient comorbidities. Echocardiographic examination can be performed at bedside, serially repeated, and does not carry the burden of contrast-related kidney injury and procedure-related risk. It provides comprehensive assessment of cardiac morphology and function. Advanced echocardiography techniques, including Doppler tissue imaging and strain imaging, may be sensitive tools for the detection of minor myocardial dysfunction, thus providing insight into early detection of AR and CAV. Stress echocardiography may offer a valuable tool in the detection of CAV, while the assessment of coronary flow reserve can unravel coronary microvascular impairment and add prognostic value to conventional stress echocardiography. The review highlights the role of Doppler echocardiography in heart transplantation follow-up, weighting advantages and limitations of the different techniques.
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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.
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Consejo , Cardiopatías Congénitas , Padres , Impresión Tridimensional , Ultrasonografía Prenatal , Humanos , Cardiopatías Congénitas/diagnóstico por imagen , Femenino , Padres/psicología , Embarazo , Consejo/métodos , Adulto , Ultrasonografía Prenatal/métodos , Proyectos Piloto , Corazón Fetal/diagnóstico por imagenRESUMEN
The field of prenatal cardiac imaging has revolutionized the way we understand and manage congenital heart diseases (CHD) in the developing fetus. In the prenatal period, cardiac imaging plays a pivotal role in the diagnostic pathway, from screening to classification and follow-up of CHD. The ability to visualize the fetal heart in utero allows healthcare professionals to detect abnormalities early, thus enabling timely interventions and informed decision-making processes for both the mother and the medical team. Early CHD detection improves preparation for delivery, postnatal care, and postnatal outcomes. Advancements in medical technology and imaging techniques have provided clinicians with insights into the fascinating workings of the fetal heart. Several imaging modalities have proven to be helpful in this field, with echocardiography undoubtedly representing the primary modality for evaluating the fetus. By providing detailed anatomical and functional information, fetal cardiac magnetic resonance (CMR) imaging contributes to better prenatal counseling and enhances the coordination of care between obstetricians, maternal-fetal medicine specialists, and pediatric cardiologists. Shortcomings of fetal CMR are due to technical concerns related to the intrauterine position of the fetus and subsequent challenges to following a standard scan protocol. The aim of this paper was to revise the current state-of-the-art in the field of fetal CMR and its clinical applications and to delve into methods, challenges, and future directions of fetal CMR in prenatal imaging.
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Congenital heart disease (CHD) affects approximately one in every one hundred infants worldwide, making it one of the most prevalent birth abnormalities globally. Despite advances in medical technology and treatment choices, CHD remains a significant health issue and necessitates specialized care throughout an individual's life. Childhood obesity has emerged as a novel global epidemic, becoming a major public health issue, particularly in individuals with lifelong conditions such as CHD. Obesity has profound effects on cardiac hemodynamics and morphology, emphasizing the importance of addressing obesity as a significant risk factor for cardiovascular health. Obesity-induced alterations in cardiac function can have significant implications for cardiovascular health and may contribute to the increased risk of heart-related complications in obese individuals. Moreover, while diastolic dysfunction may be less apparent in obese children compared to adults, certain parameters do indicate changes in early left ventricular relaxation, suggesting that obesity can cause cardiac dysfunction even in pediatric populations. As most children with CHD now survive into adulthood, there is also concern about environmental and behavioral health risk factors in this particular patient group. Addressing obesity in individuals with CHD is essential to optimize their cardiovascular health and overall quality of life. This review aims to succinctly present the data on the impact of obesity on CHD and to enhance awareness of this perilous association among patients, families, and healthcare providers.
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OBJECTIVE: The short and long-term outcomes of children with anti-Ro/La-related congenital heart block treated with a combined maternal-neonatal therapy protocol were compared with those of controls treated with other therapies. STUDY DESIGN: Sixteen mothers were treated during pregnancy with a therapy consisting of daily oral fluorinated steroids, weekly plasma exchange and fortnightly intravenous immunoglobulins and their neonates with intravenous immunoglobulins (study group); 19 mothers were treated with fluorinated steroids alone or associated to intravenous immunoglobulins or plasma exchange (control group). RESULT: The combined-therapy children showed a significantly lower progression rate from 2nd to 3rd degree block at birth, a significant increase in heart rate at birth and a significantly lower number of pacemaker implants during post-natal follow-up with respect to those treated with the other therapies. CONCLUSION: The combined therapy produced better short and long term outcomes with respect to the other therapies studied.
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Bloqueo Cardíaco , Inmunoglobulinas Intravenosas , Betametasona , Niño , Femenino , Bloqueo Cardíaco/congénito , Bloqueo Cardíaco/terapia , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Recién Nacido , Embarazo , Esteroides FluoradosRESUMEN
Background: Although most children may experience mild to moderate symptoms and do not require hospitalization, there are little data on cardiac involvement in COVID-19. However, cardiac involvement is accurately demonstrated in children with MISC. The objective of this study was to evaluate cardiac mechanics in previously healthy children who recovered from severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in a long-term follow-up by means of two-dimensional speckle-tracking echocardiography (STE). Methods: We analyzed a cohort of 157 paediatric patients, mean age 7.7 ± 4.5 years (age range 0.3−18 years), who had a laboratory-confirmed diagnosis of SARS-CoV-2 infection and were asymptomatic or mildly symptomatic for COVID-19. Patients underwent a standard transthoracic echocardiogram and STE at an average time of 148 ± 68 days after diagnosis and were divided in three follow-up groups (<180 days, 180−240 days, >240 days). Patients were compared with 107 (41 females38%) age- and BSA-comparable healthy controls (CTRL). Results: Left ventricular (LV) global longitudinal strain (post-COVID-19: −20.5 ± 2.9%; CTRL: −21.8 ± 1.7%; p < 0.001) was significantly reduced in cases compared with CTRLs. No significant differences were seen among the three follow-up groups (p = NS). Moreover, regional longitudinal strain was significantly reduced in LV apical-wall segments of children with disease onset during the second wave of the COVID-19 pandemic compared to the first wave (second wave: −20.2 ± 2.6%; first wave: −21.2 ± 3.4%; p = 0.048). Finally, peak left atrial systolic strain was within the normal range in the post-COVID-19 group with no significant differences compared to CTRLs. Conclusions: Our study demonstrated for the first time the persistence of LV myocardial deformation abnormalities in previously healthy children with an asymptomatic or mildly symptomatic (WHO stages 0 or 1) COVID-19 course after an average follow-up of 148 ± 68 days. A more significant involvement was found in children affected during the second wave. These findings imply that subclinical LV dysfunction may also be a typical characteristic of COVID-19 infection in children and are concerning given the predictive value of LV longitudinal strain in the general population.
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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.
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Cardiomiopatía Dilatada/cirugía , Insuficiencia Cardíaca/cirugía , Corazón Auxiliar , Complicaciones Posoperatorias , Niño , Preescolar , Femenino , Cardiopatías Congénitas/cirugía , Trasplante de Corazón/estadística & datos numéricos , Humanos , Lactante , Masculino , Arteria Pulmonar/cirugía , Estudios Retrospectivos , Análisis de Supervivencia , Resultado del Tratamiento , Disfunción Ventricular Izquierda/cirugíaRESUMEN
The role of immunosuppressive therapy (IT) in paediatric autoimmune/immune-mediated myocarditis remains poorly defined. To explore its role, we present a series of three consecutive paediatric patients with biopsy-proven, virus negative, autoimmune/immune-mediated myocarditis, with distinct clinical and pathological features, who have been successfully treated with IT, a 14-year-old boy with Loeffler's fibroblastic parietal endomyocarditis, a 6-year-old child with celiac disease with chronic active lymphocytic myocarditis, and a 13-year-old boy with long-standing heart failure and active lymphocytic myocarditis. Patients started IT and entered follow-up between July 2017 and September 2019; the first patient completed IT. IT was associated with a substantial and sustained recovery of cardiac function in our patients, regardless of their heterogeneous clinical and pathological features. Combination IT was well tolerated and enabled tapering and weaning off steroids.
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Insuficiencia Cardíaca , Miocarditis , Adolescente , Biopsia , Niño , Humanos , Inmunosupresores , Masculino , Miocarditis/diagnóstico , Miocarditis/tratamiento farmacológico , MiocardioRESUMEN
Background: Pulmonary artery banding (PAB) is reported as an innovative strategy for children with end-stage heart failure (ESHF) to bridge to transplantation or recovery. We report our early experience with PAB to evaluate outcomes, indications, and limitations. Materials and Methods: This is a single-center prospective clinical study, including infants and children admitted for ESHF owing to dilated cardiomyopathy (DCM) with preserved right ventricular function after failure of maximal conventional therapy. All patients underwent perioperative anticongestive medical therapy with ACE inhibitor, beta blocker, and spironolactone. Post-operatively, all patients underwent echocardiographic follow-up to assess myocardial recovery. Results: We selected five patients (four males) who underwent PAB at a median age of 8.6 months (range 3.9-42.2 months), with preoperative ejection fraction (EF) <30%. Sternal closure was delayed in all. One patient did not improve after PAB and underwent Berlin Heart implantation after 33 days, followed by heart transplant after 13 months. Four patients were discharged home on full anticongestive therapy. However, 2 months after discharge, one patient experienced severe acute heart failure secondary to pneumonia, which required mechanical circulatory support, and the patient underwent a successful heart transplant after 21 days. The remaining three patients are doing well at home, 22.4, 16.9, and 15.4 months after PAB. They all underwent elective percutaneous de-banding, 18.5, 4.8, and 10.7 months after PAB. EF increased from 17.7 ± 8.5% to 63.3 ± 7.6% (p = 0.03), and they have all been delisted. Conclusion: Use of PAB may be an effective alternative to mechanical support in selected infants for bridging to transplant or recovery. Better results seem to occur in patients aged <12 months. Further experience and research are required to identify responders and non-responders to this approach.
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Trastorno Autístico/diagnóstico , Trastorno Autístico/fisiopatología , Síndrome de QT Prolongado/diagnóstico , Síndrome de QT Prolongado/fisiopatología , Fenotipo , Sindactilia/diagnóstico , Sindactilia/fisiopatología , Adolescente , Niño , Preescolar , Electrocardiografía , Humanos , MasculinoRESUMEN
Congenital heart block (CHB) is an autoantibody mediated disorder presumably caused by placental transmission of maternal autoantibodies to Ro/SSA 52 kd, p200, Ro/SSA 60 kd, La/SSB ribonucleoproteins. This study investigated the clinical significance of isolated anti-Ro/SSA 52 kd, anti-p200, anti-Ro/SSA 60 kd, and anti-La/SSB antibodies in positive pregnant patients. One hundred sixty-three pregnant women positive to anti-Ro/SSA 52 kd and/or anti-Ro/SSA 60 kd and/or anti-La/SSB antibodies were prospectively enrolled in the study. Anti-Ro52, anti-Ro60, anti-p200, and anti-La antibodies were assayed using home-made ELISA assays. Isolated antibody positivity was found in 25 women (15.3%), while multiple antibody positivity in 138 (84.7%). Twenty-four developed CHB, and the 139 had a favorable pregnancy outcome. The prevalence of isolated anti-Ro/SSA 60 kd antibodies was significantly higher (p < 0.046) as the prevalence of lower mean antibody titers (p < 0.0001) in the later group. Confirmation of these results by large-scale studies could lead clinicians to recommend less stringent fetal echocardiography monitoring in women with isolated anti-Ro/SSA 60 kd antibodies.
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Anticuerpos Antinucleares/inmunología , Autoanticuerpos/inmunología , Bloqueo Cardíaco/congénito , Complicaciones Cardiovasculares del Embarazo , Adulto , Anticuerpos Antinucleares/sangre , Autoanticuerpos/sangre , Ensayo de Inmunoadsorción Enzimática , Femenino , Estudios de Seguimiento , Bloqueo Cardíaco/sangre , Bloqueo Cardíaco/epidemiología , Bloqueo Cardíaco/inmunología , Humanos , Incidencia , Recién Nacido , Italia/epidemiología , Embarazo , Resultado del Embarazo , Estudios Prospectivos , Factores de TiempoRESUMEN
OBJECTIVE: To evaluate the performance of Fetal Intelligent Navigation Echocardiography (FINE) applied to spatiotemporal image correlation (STIC) volume datasets of the normal fetal heart in generating standard fetal echocardiography views. METHODS: In this prospective cohort study of patients with normal fetal hearts (19-30 gestational weeks), one or more STIC volume datasets were obtained of the apical four-chamber view. Each STIC volume successfully obtained was evaluated by STICLoop™ to determine its appropriateness before applying the FINE method. Visualization rates for standard fetal echocardiography views using diagnostic planes and/or Virtual Intelligent Sonographer Assistance (VIS-Assistance®) were calculated. RESULTS: One or more STIC volumes (total n = 463) were obtained from 246 patients. A single STIC volume per patient was analyzed using the FINE method. In normal cases, FINE was able to generate nine fetal echocardiography views using: (1) diagnostic planes in 76-100% of the cases, (2) VIS-Assistance® in 96-100% of the cases, and (3) a combination of diagnostic planes and/or VIS-Assistance® in 96-100% of the cases. CONCLUSION: FINE applied to STIC volumes can successfully generate nine standard fetal echocardiography views in 96-100% of cases in the 2nd and 3rd trimesters. This suggests that the technology can be used as a method of screening for congenital heart disease.
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Ecocardiografía Tetradimensional/métodos , Corazón Fetal/diagnóstico por imagen , Cardiopatías Congénitas/diagnóstico por imagen , Femenino , Humanos , Embarazo , Segundo Trimestre del Embarazo , Tercer Trimestre del Embarazo , Estudios Prospectivos , Ultrasonografía Prenatal/métodosRESUMEN
OBJECTIVES: At the moment there are no standard guidelines for the treatment of autoimmune congenital heart block (CHB). We set out to carry out a prospective cohort study to evaluate the benefits, limits, and safety of a combined therapy protocol to treat antibody-related CHB. METHODS: Twelve consecutive pregnant patients positive to anti-SSA/Ro ± anti-SSB/La antibodies in whom CHB was detected were prospectively evaluated from 2009 to 2014. The treatment protocol consisted of: weekly plasmapheresis, fortnightly intravenous immunoglobulins (IVIG), and daily 4 mg betamethasone from CHB detection until delivery; IVIG was administered to the neonates soon after birth. RESULTS: At the time CHB was detected, six of the foetuses presented atrioventricular blocks of 2(nd) degree type and six of 3(rd) degree type. Two of the foetuses with a 2(nd) degree block reverted to a 1st degree block and one to a normal atrioventricular conduction. The condition was stable throughout the pregnancy in the other three cases of 2(nd) degree block. All six 3(rd) degree blocks were stable during pregnancy and confirmed at birth. After a mean of 37.6 months ± 19.6 SD post-birth, the infants with 1st, normal sinus rhythm, and 2(nd) degree blocks at birth were all found to be stable. During the follow-up (29 months ± 19.8 SD), pacemakers were implanted in three of the six infants with 3(rd) degree blocks. CONCLUSIONS: This combined therapy seems to be effective and safe in treating 2(nd) degree CHB, while its efficacy in treating 3rd degree CHB remains to be established.
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Enfermedades Autoinmunes/terapia , Betametasona/administración & dosificación , Glucocorticoides/administración & dosificación , Bloqueo Cardíaco/congénito , Inmunoglobulinas Intravenosas/administración & dosificación , Plasmaféresis , Adulto , Anticuerpos Antinucleares/sangre , Enfermedades Autoinmunes/sangre , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/inmunología , Betametasona/efectos adversos , Biomarcadores/sangre , Terapia Combinada , Esquema de Medicación , Quimioterapia Combinada , Ecocardiografía Doppler , Femenino , Edad Gestacional , Glucocorticoides/efectos adversos , Bloqueo Cardíaco/sangre , Bloqueo Cardíaco/diagnóstico , Bloqueo Cardíaco/inmunología , Bloqueo Cardíaco/terapia , Humanos , Inmunoglobulinas Intravenosas/efectos adversos , Recién Nacido , Masculino , Plasmaféresis/efectos adversos , Embarazo , Estudios Prospectivos , Factores de Tiempo , Resultado del Tratamiento , Ultrasonografía Prenatal/métodosRESUMEN
BACKGROUND: Conflicting data were reported about normalization of sizes of right chambers, systolic and diastolic function and prevalence of arrhythmias after ostium secundum atrial septal defect closure. We sought to compare surgical and percutaneous approaches in terms of arrhythmias, right chamber volumes, and function at long-term follow-up. METHODS: In all, 107 patients were enrolled, all corrected at pediatric age. Forty-four of them were treated surgically with a right thoracotomy approach and 63 were treated percutaneously. All patients underwent a standard echocardiogram and electrocardiographic Holter examinations. RESULTS: No difference was detected between the two groups regarding right atrial or ventricular volumes. The global right ventricular function assessed by fractional area change was similar between the two groups. However, the longitudinal function and the diastolic function were significantly impaired in the surgical group (tricuspid annulus peak systolic excursion 23.7 ± 4.5 mm versus 18.7 ± 3.5 mm, p < 0.001; S' wave 13.7 ± 3.1 cm/s versus 9.8 ± 2.4 cm/s, p < 0.001; E/E' 4.7 ± 1.7 versus 7.1 ± 2.9, p < 0.001). There was a low incidence of supraventricular couples or runs, but slightly higher in the surgical group (6.8% versus 1.6%), although not statistically significant. No echocardiographic variable related to ventricular or supraventricular arrhythmic events. CONCLUSIONS: Either surgical or percutaneous closure of atrial septal defect have a similar efficacy on the volume normalization of the right chamber. Modern surgical techniques have a limited impact on the systolic and diastolic function as well as on the arrhythmic risk; however, the right ventricular longitudinal and diastolic function seems to be better preserved in the percutaneous group.
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Remodelación Atrial , Defectos del Tabique Interatrial/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , Niño , Preescolar , Ecocardiografía , Electrocardiografía Ambulatoria , Femenino , Defectos del Tabique Interatrial/diagnóstico por imagen , Defectos del Tabique Interatrial/fisiopatología , Humanos , Masculino , Factores de TiempoRESUMEN
BACKGROUND: Breath-holding spells (BHS) are benign non-epileptic paroxysmal events of infancy, rarely occurring with high frequency and complicated by prolonged syncope, convulsions and even status epilepticus. In these cases response to medical treatment is often unsatisfactory. Pacemaker implantation is a possible therapeutic option, but its indications, efficacy and complications have not been clarified yet. OBJECTIVE: To report a new case of BHS treated with pacemaker and to review its indications and efficacy in patients with severe BHS. METHODS: We extensively searched the literature in PubMed on cardiac pacing in patients with BHS and we described a new case. RESULTS: A previously healthy boy presented at the age of 4 months with frequent BHS inconstantly associated to prolonged syncope and post-anoxic non-epileptic and epileptic seizures. Parental reassurance, iron supplementation and piracetam were ineffective. After cardiac pacing at the age of 16 months, BHS and their complications disappeared. We identified 47 patients with BHS treated with pacemaker in the literature. Based on the available data, in all patients asystole or marked bradycardia were documented during BHS or stimulating maneuvers; syncope complicated BHS in 100% of cases and post-anoxic convulsions in 78.3%. Medical treatment before pacing, when administered, was ineffective or poorly tolerated. After pacing, BHS complications disappeared in 86.4% of cases, and decreased in 13.6%. Technical problems with the device were reported in 25.7% of patients and mild medical complications in 11.4%. CONCLUSIONS: Pacemaker could be reasonably considered in subjects with frequent and severe BHS, poor response to medications, and demonstration of cardioinhibition during spells.
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Contencion de la Respiración , Marcapaso Artificial , Humanos , Lactante , Masculino , Convulsiones/etiología , Síncope/etiologíaRESUMEN
Primary cardiac tumours in infants and children are extremely rare, with an estimated incidence of 0.2% according to echocardiographic studies. Owing to their rarity, there is very little literature available, and most knowledge is based on collections of case reports. Therefore, we reviewed retrospectively our 27 years of clinical experience on the overall management of cardiac tumours among children in order to improve not only our knowledge but also to provide others with information about the incidence, clinical presentation, management, and long-term outcome of this rare disease. Between April, 1982 and April, 2009, 52 children were diagnosed with cardiac tumours at our Institution. Medical records and follow-up echocardiographic evaluations were studied. The diagnosis was prenatal in 35% of the patients. The most frequent tumour types were rhabdomyomas (61.5%), fibromas (15.4%), and myxomas (5.8%). There were no cases of primary malignant tumours. All diagnoses were achieved using two-dimensional echocardiography, and for 12 patients a pathological analysis was carried out. A total of 41 patients (79%) were managed medically, whereas 11 (21%) patients underwent surgical treatment. At a mean follow-up of 7.2 ± 5.4 years, two patients died of complications related to cardiac transplantation; all the remaining patients are in excellent clinical condition. In conclusion, cardiac tumours in paediatric practice are usually clinically and histologically benign. Only a few cases need surgery. Up to one-third of the cardiac masses are detectable prenatally. Rhabdomyoma is the most common histotype, followed by fibroma and myxoma. The long-term prognosis is generally good.
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Fibroma/terapia , Neoplasias Cardíacas/terapia , Mixoma/terapia , Regresión Neoplásica Espontánea , Neoplasias Primarias Múltiples/terapia , Rabdomioma/terapia , Teratoma/terapia , Adolescente , Arritmias Cardíacas/tratamiento farmacológico , Arritmias Cardíacas/etiología , Enfermedades Asintomáticas , Niño , Preescolar , Estudios de Cohortes , Ecocardiografía , Femenino , Fibroma/diagnóstico por imagen , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Trasplante de Corazón , Humanos , Lactante , Recién Nacido , Masculino , Mixoma/diagnóstico por imagen , Neoplasias Primarias Múltiples/diagnóstico por imagen , Embarazo , Estudios Retrospectivos , Rabdomioma/diagnóstico por imagen , Teratoma/diagnóstico por imagen , Ultrasonografía PrenatalRESUMEN
Limited options do exist for mechanical circulatory support as a bridge to transplantation in the pediatric population. This is especially true when it comes to intracorporeal technologies. We describe our successful experience with the use of HeartWare ventricular assist devices (HeartWare, Inc., Framingham, MA, USA) in three patients <16 years of age, as a successful and feasible bridge to transplant in patients weighing greater than 20 kg.
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Cardiomiopatía Dilatada/cirugía , Corazón Auxiliar , Adolescente , Anticoagulantes/uso terapéutico , Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Dilatada/terapia , Niño , Femenino , Trasplante de Corazón , Humanos , Masculino , Resultado del TratamientoRESUMEN
A 9-year-old boy was admitted to the emergency department for interscapular chest discomfort. Bidimensional echocardiography revealed a severe aortic root and sino-tubular junction dilatation with a suspicion of intimal flap at the aortic isthmus and a pseudo-aneurysm which was confirmed by a computerized tomography scan. The patient was immediately transferred to the operating room for an emergent replacement of the aortic isthmus. His recovery after surgery was uneventful, and he was discharged home 12 days later on beta blockers and angiotensin II receptor antagonist. A genetic recognition confirmed the diagnosis of Loeys-Dietz syndrome type I.
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Accidentes por Caídas , Disección Aórtica/etiología , Prótesis Vascular , Síndrome de Loeys-Dietz/complicaciones , Disección Aórtica/diagnóstico , Disección Aórtica/cirugía , Niño , Diagnóstico Diferencial , Ecocardiografía , Estudios de Seguimiento , Humanos , Imagenología Tridimensional , Síndrome de Loeys-Dietz/diagnóstico , Síndrome de Loeys-Dietz/cirugía , Masculino , Tomografía Computarizada por Rayos XRESUMEN
Congenital heart defects (CHDs) occur in 1% of live-born infants and frequently are associated with extracardiac malformations. This study aimed to assess the feasibility and accuracy of three-dimensional ultrasonography (3DUS) in fetuses with CHD and to investigate whether 3DUS can add information about the heart and general fetal morphology that shows other congenital malformations or suggests syndromes. For 30 fetuses affected by CHD, 3DUS was performed using a Sonos 7500 ultrasound machine with a cardiac 3D transducer. In 44% of the exams, 3DUS was completely diagnostic for the CHD, providing additional information in 28% of the exams. Furthermore, 3DUS showed 82% of associated malformations, providing the complete diagnosis in 57% of the cases and helping with recognition of syndromes in others. The diagnostic accuracy of 3DUS was superior, with a higher number of acquisitions per exam. Performance was better in fetuses younger than 24 weeks for general morphologic details and in fetuses older than 24 weeks for the heart morphology.
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Corazón Fetal/diagnóstico por imagen , Cardiopatías Congénitas/diagnóstico por imagen , Imagenología Tridimensional/métodos , Complicaciones Cardiovasculares del Embarazo/diagnóstico por imagen , Ultrasonografía Prenatal/estadística & datos numéricos , Anomalías Múltiples/diagnóstico por imagen , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Edad Gestacional , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/embriología , Humanos , Recién Nacido , Masculino , Embarazo , Resultado del Embarazo , Estudios Retrospectivos , SíndromeRESUMEN
Postoperative chylothorax represents one of the early and most serious complications after cardiothoracic operations. When conservative measures are not effective, an operation is mandatory. We describe a case of persistent chylothorax after a "one-and-a-half ventricle" repair in an infant with complex congenital heart disease, which was successfully treated with a transabdominal ligation of the cisterna chyli. At the 1-year follow-up, she is in excellent clinical condition, without recurrence of pleural effusion or the requirement for nutritional support. Ligation of cisterna chyli may represent a safe and effective ultimate treatment in children with persistent chylothorax.