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Cardiovascular magnetic resonance (CMR) and computed tomography (CCT) are advanced imaging modalities that recently revolutionized the conventional diagnostic approach to congenital heart diseases (CHD), supporting echocardiography and often replacing cardiac catheterization. This is the second of two complementary documents, endorsed by experts from the Working Group of the Italian Society of Pediatric Cardiology and the Italian College of Cardiac Radiology of the Italian Society of Medical and Interventional Radiology, aimed at giving updated indications on the appropriate use of CMR and CCT in different clinical CHD settings, in both pediatrics and adults. In this article, support is also given to radiologists, pediatricians, cardiologists, and cardiac surgeons for indications and appropriateness criteria for CMR and CCT in the most referred CHD, following the proposed new criteria presented and discussed in the first document. This second document also examines the impact of devices and prostheses for CMR and CCT in CHD and additionally presents some indications for CMR and CCT exams when sedation or narcosis is needed.
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Consenso , Cardiopatías Congénitas , Humanos , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/terapia , Italia , Tomografía Computarizada por Rayos X/normas , Cardiología/normas , Imagen por Resonancia Magnética/normas , Niño , Valor Predictivo de las Pruebas , Adulto , Sociedades Médicas/normasRESUMEN
BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) shows a significant overlap of symptoms with other hyper-inflammatory diseases such as Kawasaki disease (KD), but the real difference of the two conditions is still matter of debate. Coronary artery lesions (CAL) are the most relevant complication in KD. Nonetheless, CAL, myocarditis, pericarditis, arrhythmia are the main cardiovascular complications in MIS-C. A close clinical assessment is mandatory, both at the diagnosis and during the follow-up, by ECG and echocardiography. Cardiac magnetic resonance (MRI) adds important data to ultrasound findings. However, cardiac MRI studies in MIS-C are limited to a small number of cohorts. METHODS: We enrolled 20 children (age:1-16 years; 11 F; 9 M) with cardiac involvement secondary to MIS-C, all evaluated by cardiac MRI. RESULTS: 8 children showed pathological cardiac MRI: 2 showed pericardial effusion; 2 showed myocardial oedema; 1 showed aortic insufficiency; 3 showed delayed enhancement (one for acute myocarditis with oedema; 2 for myocardial fibrosis). Delayed enhancement was reduced significantly 5.6-9 months after the first MRI evaluation. 25% of patients with pathological MRI had CAL associated with valvular insufficiency of 2 valves. 17% of patients with normal MRI had CAL, associated with valvular insufficiency of 1 valve in 1 patient. The correlations between haematological, clinical, cardiologic parameters, treatment, did not reach the statistical significance. 4 patients were treated with anakinra. Among those, 2 patients showed a normal cardiac MRI. Cardiac lesions resolved in all the patients during the follow-up. Some patients with pathological cardiac MRI could not underwent a control with MRI, for the low compliance. However, echocardiography and ECG, documented the resolution of the pathological data in these cases. CONCLUSIONS: A higher risk of CAL was documented in patients with an association of other cardiac lesions. Cardiac MRI is difficult to perform routinely; however, it is useful for evaluating the acute myocardial damage and the outcome of patients with MIS-C.
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COVID-19/complicaciones , Síndrome de Respuesta Inflamatoria Sistémica , Humanos , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico por imagen , Niño , Masculino , Femenino , Adolescente , Preescolar , Lactante , Imagen por Resonancia Magnética , Ecocardiografía , SARS-CoV-2 , Imagen por Resonancia Cinemagnética/métodosRESUMEN
Background: Managing repaired tetralogy of Fallot (TOF) patients is still challenging despite the fact that published studies identified prognostic clinical or imaging data with rather good negative predictive accuracy but weak positive predictive accuracy. Heterogeneity of the initial anatomy, the surgical approach, and the complexity of the mechanism leading to dilation and ventricular dysfunction explain the challenge of predicting the adverse event in this population. Therefore, risk stratification and management of this population remain poorly standardized. Design: The CMR/CT WG of the Italian Pediatric Cardiology Society set up a multicenter observational clinical database of repaired TOF evaluations. This registry will enroll patients retrospectively and prospectively assessed by CMR for clinical indication in many congenital heart diseases (CHD) Italian centers. Data collection in a dedicated platform will include surgical history, clinical data, imaging data, and adverse cardiac events at 6 years of follow-up. Summary: The multicenter repaired TOF clinical database will collect data on patients evaluated by CMR in many CHD centers in Italy. The registry has been set up to allow future research studies in this population to improve clinical/surgical management and risk stratification of this population.
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Acute clinical manifestations of COVID-19 are generally less severe in childhood, however a proportion of them can develop a severe systemic hyperinflammatory syndrome after SARS-CoV-2 infection, known as the multisystem inflammatory syndrome (multisystem inflammatory syndrome in children, MIS-C). Cardiovascular manifestations in MIS-C are frequent (34-82%), including myocardial dysfunction, coronary artery dilation or aneurysms, arrhythmias, conduction abnormalities, pericarditis and valvulitis. The most affected cases can develop cardiogenic shock needing intensive care unit admission, inotropic support and sometimes even mechanical circulatory support. The elevation of myocardial necrosis markers, the frequently transient left ventricular systolic dysfunction and the presence of changes on magnetic resonance imaging, support the hypothesis of an immune-mediated post-viral pathogenesis similar to myocarditis. Although MIS-C shows excellent short-term survival, further studies are needed to demonstrate complete reversibility of residual subclinical heart damage.
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COVID-19 , Aneurisma Coronario , Niño , Humanos , COVID-19/complicaciones , SARS-CoV-2 , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , CorazónRESUMEN
BACKGROUND: In Sicily, the first wave of COVID-19 showed a low epidemic impact in paediatric population, while the second and the third waves had a higher impact on clinical presentation of COVID-19 in children and a significantly higher severe outcome in patients with multisystem inflammatory syndrome in children (MIS-C), with a frequent life-threatening progression. METHODS: We describe a cohort of 22 Sicilian children (11 M; 11 F; age: 1.4-14 years), presenting with clinical features compatible with MIS-C. Patients with negative swab had a history of recent personal or parental infection. RESULTS: The following diagnostic criteria were detected: fever (100%); cheilitis and/or pharyngeal hyperaemia (86%); latero-cervical lymphadenitis (82%); rash (73%); abdominal pain and/or vomiting and/or diarrhoea (64%); conjunctivitis (64%); hands and feet oedema (18%). 59% showed cardiac involvement (6 pericardial effusion; 8 mitral valve insufficiency; 4 insufficiency of two valves; 3 coronary artery lesions (CAL)). In all the patients, treatment was started within 72 h after the admission, with intravenous immunoglobulins (IVIG) (2 g/Kg/dose), methylprednisolone (2 mg/Kg/day in 73% of patients; 30 mg/Kg/day for 3 days, followed by 2 mg/Kg/day in 27% of patients). Two patients were treated with enoxaparin. Two patients with shock, were additionally treated with vasoactive drugs, albumin, diuretics. Cardiac involvement evolved into the complete resolution of lesions in most of the patients. All the patients were included in a follow-up, to investigate on clinical outcome and resolution of organ involvement. Cardiac valve insufficiency persisted only in 18% of children, CAL persisted only in 33% of children with coronary involvement, however without the evolution into aneurisms. CONCLUSIONS: The preferred treatment strategy was more aggressive at the diagnosis of MIS-C, to block the cytokine cascade. Most of our patients, in fact, received a first-line treatment with IVIG and steroids. This approach could explain the favourable prognosis, the rapid restoring of cardiac function also in patients with MAS or shock, and the good outcome during the 10 months follow-up in all the patients.
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COVID-19 , Humanos , Niño , Lactante , Preescolar , Adolescente , COVID-19/epidemiología , SARS-CoV-2 , Inmunoglobulinas Intravenosas/uso terapéutico , Estudios de Seguimiento , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , Síndrome de Respuesta Inflamatoria Sistémica/tratamiento farmacológico , Síndrome de Respuesta Inflamatoria Sistémica/epidemiología , Hospitales PediátricosRESUMEN
Cardiovascular magnetic resonance (CMR) and computed tomography (CCT) are advanced imaging modalities that recently revolutionized the conventional diagnostic approach to congenital heart diseases (CHD), supporting echocardiography and often replacing cardiac catheterization. Nevertheless, correct execution and interpretation require in-depth knowledge of all technical and clinical aspects of CHD, a careful assessment of risks and benefits before each exam, proper imaging protocols to maximize diagnostic information, minimizing harm. This position paper, written by experts from the Working Group of the Italian Society of Pediatric Cardiology and from the Italian College of Cardiac Radiology of the Italian Society of Medical and Interventional Radiology, is intended as a practical guide for applying CCT and CMR in children and adults with CHD, wishing to support Radiologists, Pediatricians, Cardiologists and Cardiac Surgeons in the multimodality diagnostic approach to these patients. The first part provides a review of the most relevant literature in the field, describes each modality's advantage and drawback, making considerations on the main applications, image quality, and safety issues. The second part focuses on clinical indications and appropriateness criteria for CMR and CCT, considering the level of CHD complexity, the clinical and logistic setting and the operator expertise.
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Cardiología , Cardiopatías Congénitas , Adulto , Niño , Consenso , Cardiopatías Congénitas/diagnóstico por imagen , Humanos , Espectroscopía de Resonancia Magnética , Radiología Intervencionista , Tomografía Computarizada por Rayos XRESUMEN
The success of cardiac surgery over the past 50 years has increased numbers and median age of survivors with congenital heart disease (CHD). Adults now represent two-thirds of patients with CHD; in the USA alone the number is estimated to exceed 1 million. In this population, many affected women reach reproductive age and wish to have children. While in many CHD patients pregnancy can be accomplished successfully, some special situations with complex anatomy, iatrogenic or residual pathology are associated with an increased risk of severe maternal and fetal complications. Pre-conception counselling allows women to come to truly informed choices. Risk stratification tools can also help high-risk women to eventually renounce to pregnancy and to adopt safe contraception options. Once pregnant, women identified as intermediate or high risk should receive multidisciplinary care involving a cardiologist, an obstetrician and an anesthesiologist with specific expertise in managing this peculiar medical challenge. This document is intended to provide cardiologists working in hospitals where an Obstetrics and Gynecology Department is available with a streamlined and practical tool, useful for them to select the best management strategies to deal with a woman affected by CHD who desires to plan pregnancy or is already pregnant.
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The success of cardiac surgery over the past 50 years has increased numbers and median age of survivors with congenital heart disease (CHD). Adults now represent two-thirds of patients with CHD; in the United States alone the number is estimated to exceed 1 million.In this population many affected women reach reproductive age and wish to have children. While in many CHD patients pregnancy can be accomplished successfully, some special situations with complex anatomy, iatrogenic or residual pathology are associated with an increased risk of severe maternal and fetal complications. Pre-conception counseling allows women to come to truly informed choices. Risk stratification tools can also help high-risk women to eventually renounce to pregnancy and to adopt safe contraception options. Once pregnant, women identified as intermediate or high-risk should receive multidisciplinary care involving a cardiologist, an obstetrician and an anesthesiologist with specific expertise in managing this peculiar medical challenge.This document is intended to provide cardiologists working in hospitals where an Obstetrics and Gynecology Department is available with a streamlined and practical tool, useful for them to select the best management strategies to deal with a woman affected by CHD who desires to plan pregnancy or is already pregnant.
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Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/terapia , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Complicaciones Cardiovasculares del Embarazo/terapia , Árboles de Decisión , Consejo Dirigido , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/fisiopatología , Humanos , Embarazo , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Medición de RiesgoRESUMEN
Cardiovascular disease (CVD) is the most important risk factor for morbidity and mortality in patients with chronic kidney disease (CKD). Aim of this study was to evaluate cardiac and vascular geometry in children with CKD stages 2, 3 and 4.Twenty-seven patients (18 males and 9 females) mean age 10.9 +/- 5.4 years with CKD and 30 children (control group) were enrolled with comparable age and sex. Weight, height, systolic and diastolic blood pressure were evaluated. We also analyzed biochemical assessments and proteinuria. We performed echocardiography with Philips iE33 and pulse wave velocity (PWV) with Vicorder PWS system. We documented significantly higher level of left ventricular mass index (LVMI) (30.3 +/- 7.6 g/m2.7) and PWV (4.7 +/- 1.6 m/sec) in CKD patients. Left ventricular hypertrophy (LVH) was present in 12 % and concentric remodelling in 36% of our patients. PWV values were significantly correlated with interventricular septal thickness (p<0.01) and with LVMI (p<0.05). In this study we documented the alterations of cardiac and vascular geometry since the early stages of CKD. PWV and echocardiographic measurements must be considered to assess cardiovascular risk in children with CKD stages 2-4.
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Enfermedades Cardiovasculares/etiología , Insuficiencia Renal Crónica/complicaciones , Enfermedades Cardiovasculares/epidemiología , Estudios de Casos y Controles , Niño , Femenino , Humanos , Masculino , Insuficiencia Renal Crónica/diagnóstico , Factores de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
We report a case of acute myocardial infarction in an 8-year-old boy with a history of Kawasaki disease and giant coronary aneurysms in the right and left coronary arteries. We performed coronary angiography and percutaneous coronary intervention 4 hours after the onset of symptoms. This case suggests that primary percutaneous coronary intervention might be safe and effective in the long-term treatment of acute myocardial infarction due to coronary sequelae of Kawasaki.
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Aneurisma Coronario/etiología , Síndrome Mucocutáneo Linfonodular/complicaciones , Infarto del Miocardio/terapia , Intervención Coronaria Percutánea , Niño , Aneurisma Coronario/diagnóstico , Angiografía Coronaria , Electrocardiografía , Humanos , Masculino , Síndrome Mucocutáneo Linfonodular/diagnóstico , Infarto del Miocardio/diagnóstico , Infarto del Miocardio/etiología , Resultado del TratamientoRESUMEN
OBJECTIVE: We evaluated the feasibility, safety, and diagnostic accuracy of noninvasive pacemaker stress echocardiography (PASE) test as a potential alternative to exercise or pharmacologic stress in patients with suspected or known coronary artery disease (CAD). BACKGROUND: Transesophageal atrial pacing echocardiography is an accurate test for detection of CAD, but its practical impact has been blunted by semi-invasiveness. In the expanding population of patients with permanent pacemakers (PMs), a pacing stress test can be administered noninvasively by external programming of the PM. METHODS: In a prospective, multicenter, international study design, transthoracic stress-pacing echocardiography was performed in 46 consecutive patients with a permanent PM (33 men, 13 women; age 66.6 +/- 11.1 years) with suspected or known CAD. All patients underwent noninvasive PM-stress test by external programming (10 beats/min increments up to ischemia or target heart rate). Coronary angiography was performed in all patients independently of test results. Significant CAD was defined as >/=50% visually assessed diameter reduction in at least one major epicardial coronary artery. All coronary angiograms were scored by Duke prognostic weight values. RESULTS: Fifteen patients were stimulated in atrial, and the remaining 31 in ventricular mode during stress. No significant side effects were observed. Echocardiographic images were interpretable in all patients. The average duration of stress was 8.9 +/- 3.5 min. Significant CAD was found in 27 patients. Sensitivity of PASE for identifying patients with significant CAD was 70%, specificity was 90%, and accuracy was 78%. When any abnormal wall motion at rest that remained unchanged at peak stress was regarded as a positive result of PASE, then the sensitivity, specificity, and accuracy levels for identifying patients with significant CAD were 85%, 84%, and 85%, respectively. Four of the eight patients with a false negative did not reach the target heart rate. The Duke values had significant correlation with values of wall motion score index at peak stress (r = 0.67) and with peak heart rate (r = -0.3). CONCLUSIONS: Noninvasive PASE is a simple, rapid, safe, and diagnostically efficient option for patients with permanent PM and suspected or known CAD.