Evolution and trends in a pediatric cardiac magnetic resonance imaging program in a tertiary hospital over a 14-year period. / Evolución y tendencias de un programa de resonancia magnética cardiaca pediátrica en un hospital terciario durante 14 años.

OBJECTIVES: 1. To review the activity in our hospital's pediatric cardiac magnetic resonance imaging (cMRI) program from its inception to the present. 2. To evaluate changes in the number of patients, in the number of studies done under anesthesia, in the number of studies done with contrast material (magnetic resonance angiography (MRA) and delayed enhancement), and in representative diseases studied. 3. To estimate trends in the parameters evaluated in objective 2. MATERIAL AND METHODS: The pediatric cMRI program at our hospital started on February 14, 2005. We assessed cMRI studies done between the inception of the program and December 31, 2018. The cases were entered in a calculation table that included sex, date of birth, date of examination, clinical presentation, radiologic diagnosis, sequences done, and anesthesia. For each year, we obtained data about patients' age, studies done under anesthesia, contrast-enhanced MRA, delayed enhancement studies, and postoperative studies. We also evaluated the evolution of the number of patients studied for a group of representative diseases (coarctation of the aorta; tetralogy of Fallot; dextro-transposition of the great arteries; corrections of univentricular heart; hypoplastic left heart syndrome; anomalous pulmonary venous return; and cardiomyopathy). We analyzed these data with bar graphs, evolutions of means, and logarithmic trend curves. RESULTS: A total of 2606 cases were included. The number of cases per year increased gradually. The mean age of all patients was 12.5 years, and the age of the patients studied also increased during the 14-year period. Anesthesia was used in 42%. Contrast-enhanced MRA was done in 57.6% and delayed enhancement in 42.13%. The most common condition was aortic coarctation (16.39%), although the frequency of aortic coarctation and hypoplastic left heart syndrome decreased slightly during the period. By contrast, the frequency of cardiomyopathy (7.25% of cases) increased slightly, to the point where it represented 9.35% in 2018. CONCLUSION: During the 14-year period in which pediatric cMRI has been done at our hospital, the conditions studied, the type of patients, and the techniques used has varied; the number of patients and patients' age has increased, where as the frequency of MRA studies has decreased. The prevalence of the different conditions studied has also changed.

What radiologists need to know about the pulmonary-systemic flow ratio (Qp/Qs): what it is, how to calculate it, and what it is for.

Cardiac magnetic resonance imaging (cMRI) provides abundant morphological and functional information in the study of congenital heart disease. The functional information includes pulmonary output and systemic output; the ratio between these two (Qp/Qs) is the shunt fraction. After birth, in normal conditions the pulmonary output is practically identical to the systemic output, so Qp/Qs = 1. In patients with « shunts ¼ between the systemic and pulmonary circulations, the ratio changes, and the interpretation of these findings varies in function of the location of the shunt (intracardiac or extracardiac) and of the associated structural or postsurgical changes. We review the concept of Qp/Qs; the methods to calculate it, with special emphasis on cMRI; and the meaning of the results obtained. We place special emphasis on the relevance of these findings depending on the underlying disease and the treatment the patient has undergone.

[Quality of 3D magnetic resonance imaging of coronary arteries in patients with D-transposition of the great arteries after the Jatene switch procedure]. / Calidad de la coronariografía 3D por resonancia magnética en los pacientes con D-transposición de las grandes arterias intervenidos con switch arterial de Jatene.

OBJECTIVES: To evaluate the quality of images obtained with 3D balanced fast-field echo whole heart (WH3D) MRI sequences for assessing the coronary anastomosis and coronary stenosis in patients with D-transposition of the great arteries who have undergone the Jatene switch procedure. MATERIAL AND METHODS: We retrieved 100 WH3D studies done in 83 patients who had undergone the Jatene switch procedure from our pediatric cardiac MRI database; 84 of these studies fulfilled the criteria for inclusion in the study. We evaluated coronary stenoses on WH3D MR images and their correlation with coronary CT or angiography images. We retrospectively studied the quality of the images of the proximal coronary arteries using a four-point scale and correlating the findings with age, heart rate, and heart size. RESULTS: Of the 84 studies, 4 (4.8%) were of a quality considered «insufficient for diagnosis¼, 7 (8.3%) were considered «fair¼, 23 (27.4%) «good¼, and 50 (59.5%) «excellent¼. The quality of the image of the coronary arteries was significantly correlated with heart rate. MRI detected stenosis in the origin of the coronary arteries in 9 (10.7%) studies. CONCLUSION: Images obtained with the WH3D MRI sequence in patients who had undergone the Jatene procedure were of diagnostic quality in most cases and were better in patients with lower heart rates. In 10.7%, stenosis in the origin of the coronary arteries that required new studies was detected.

Ausencia de realce tardío por resonancia magnética en la no compactación del ventrículo izquierdo en lactantes y niños pequeños / Lack of MR late-enhancement in left ventricular non-compaction in infants and young children

Objetivo: La miocardiopatía no compactada o la no compactación del ventrículo izquierdo (NCVI) es una enfermedad rara y probablemente infradiagnosticada. El diagnóstico es ecográfico, si bien la resonancia magnética (RM) aporta importante información morfológica y funcional. El realce tardío es un hallazgo importante que refleja fibrosis o infarto en las zonas afectadas, y se refiere en adultos y niños mayores de forma generalizada. El objetivo de este trabajo es revisar el realce tardío en esta enfermedad en lactantes y niños pequeños. Material y métodos: En nuestra base de datos de RM cardíaca encontramos 5 pacientes (rango de edad: un mes a 5 años; media de 29,4 meses). Se revisaron los hallazgos morfológicos y funcionales, incluyendo el realce tardío de contraste. Resultados: Todos los pacientes se diagnosticaron previamente por ecocardiografía. Los hallazgos morfológicos y el cociente miocardio no compactado/miocardio compactado fueron compatibles con NCVI. Ninguno de los casos mostró realce tardío poscontraste. Conclusiones: Al contrario que en adultos y niños mayores, ninguno de nuestros pacientes mostró realce tardío. Este hallazgo podría reflejar la naturaleza evolutiva de la fibrosis subendocárdica en estos pacientes (AU)

Objective: Non compaction cardiomyopathy or left ventricular non compaction is a rare disease that is probably underdiagnosed. The diagnosis is reached by echocardiography, although MRI provides additional morphological and functional information. Late MRI enhancement is a hallmark of the disease that reflects fibrosis or infarction of affected areas in adults and older children. We aimed to review the presence of late enhancement in left ventricular non compaction in infants and young children. Material and methods: We found five very young patients (mean age, 29.4 months; range 1 month to 5 years) with left ventricular non compaction in our cardiac MRI database. We reviewed the morphological and functional findings, including late enhancement after the administration of contrast material. Results: All patients had been previously diagnosed by echocardiography. At MRI, the morphological findings and the ratio of non compacted myocardium to compacted myocardium were compatible with left ventricular non compaction. None of the cases showed late enhancement after the administration of contrast material. Conclusions: Unlike in adults and older children, none of the infants and young children we studied had late enhancement. This finding might reflect the natural history of the disease, with subendocardial fibrosis developing over time (AU)

[Lack of MR late-enhancement in left ventricular non-compaction in infants and young children]. / Ausencia de realce tardío por resonancia magnética en la no compactación del ventrículo izquierdo en lactantes y niños pequeños.

OBJECTIVE: Noncompaction cardiomyopathy or left ventricular noncompaction is a rare disease that is probably underdiagnosed. The diagnosis is reached by echocardiography, although MRI provides additional morphological and functional information. Late MRI enhancement is a hallmark of the disease that reflects fibrosis or infarction of affected areas in adults and older children. We aimed to review the presence of late enhancement in left ventricular noncompaction in infants and young children. MATERIAL AND METHODS: We found five very young patients (mean age, 29.4 months; range 1 month to 5 years) with left ventricular noncompaction in our cardiac MRI database. We reviewed the morphological and functional findings, including late enhancement after the administration of contrast material. RESULTS: All patients had been previously diagnosed by echocardiography. At MRI, the morphological findings and the ratio of noncompacted myocardium to compacted myocardium were compatible with left ventricular noncompaction. None of the cases showed late enhancement after the administration of contrast material. CONCLUSIONS: Unlike in adults and older children, none of the infants and young children we studied had late enhancement. This finding might reflect the natural history of the disease, with subendocardial fibrosis developing over time.

[Contrast-enhanced magnetic resonance angiography in congenital heart disease]. / Angiografía con contraste por resonancia magnética en las cardiopatías congénitas.

Contrast-enhanced MR angiography is one of the greatest achievements brought about by advances in body MRI. The noninvasive evaluation of arteries and veins can obviate heart catheterization, the administration of iodinated contrast, and exposure to ionizing radiation in many patients and spare them the risks associated with these factors. These gains are even more important in children with congenital heart disease, who will have to undergo numerous vascular studies in their lifetimes and are more susceptible to the effects of ionizing radiation. Contrast-enhanced MR angiography provides abundant information for diagnosis and postoperative follow-up in these patients, who reach advanced age thanks to advances in medical and surgical treatment and thus receive more and more imaging studies during their lifetimes. In this review, we analyze the contrast-enhanced MR angiography technique in these patients, the problems and precautions related to the use of gadolinium, the indications for the test, and the relevant imaging findings in patients with congenital heart disease.

Angiografía con contraste por resonancia magnética en las cardiopatías congénitas / Contrast-enhanced magnetic resonance angiography in congenital heart disease

El avance de la resonancia magnética (RM) de cuerpo ha visto en la angiografía con contraste por RM (ACRM) uno de sus mayores logros. La evaluación no invasiva de arterias y venas evita la realización de cateterismo, la administración de contraste yodado y la exposición a radiaciones ionizantes a un gran número de pacientes. Este hecho es, si cabe, de mayor relevancia en los niños con cardiopatías congénitas, al tratarse de pacientes a los que habrá que realizar numerosos estudios vasculares a lo largo de su vida, con los riesgos inherentes del cateterismo, los debidos al contraste yodado, y la mayor susceptibilidad de estos pacientes a los efectos de las radiaciones ionizantes. La ACRM aporta abundante información para el diagnóstico y seguimiento posquirúrgico en este grupo de pacientes que, con los avances terapéuticos médicos y quirúrgicos, hoy en día alcanzan edades avanzadas y reciben, cada vez más, numerosos estudios de imagen a lo largo de su vida. En esta revisión se analiza la técnica de la ACRM en estos pacientes, los problemas y precauciones relativas al uso del gadolinio, las indicaciones y los hallazgos radiológicos a los que hay que prestar especial atención en esta patología (AU)

Contrast-enhanced MR angiography is one of the greatest achievements brought about byadvances in body MRI. The non invasive evaluation of arteries and veins can obviate heartcatheterization, the administration of iodinated contrast, and exposure to ionizingradiation in many patients and spare them the risks associated with these factors. These gains are even more important in children with congenital heart disease, who will have toundergo numerous vascular studies in their life times and are more susceptible to theeffects of ionizing radiation. Contrast-enhanced MR angiography provides abundantinformation for diagnosis and postoperative follow-up in these patients, who reachadvanced age thanks to advances in medical and surgical treatment and thus receive moreand more imaging studies during their life times. In this review, we analyze the contrast-enhanced MR angiography technique in these patients, the problems and precautionsrelated to the use of gadolinium, the indications for the test, and the relevant imagingfindings in patients with congenital heart disease (AU)

Hipertensión arterial vasculorrenal en varón joven / Vasculo-renal arterial hypertension in a young male patient

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[Langerhans cell histiocytosis in the central nervous system: meningeal involvement]. / Histiocitosis de células de Langerhans en el sistema nervioso central: afectación meníngea.

The clinical manifestations of Langerhans cell histiocytosis in the central nervous system include not only the classical involvement of the hypothalamus-hypophyseal axis, but also of other anatomic locations, with the corresponding variability of symptoms. We report here two patients with meningeal lesions. In both patients, the onset of the disease was systemic, and neurological symptoms developed some years later. Imaging studies, particularly magnetic resonance, are very sensitive tools for the diagnosis and follow-up of these lesions. The natural history of this disease is still poorly understood. In this paper we related the clinical manifestations to the morphological changes observed in imaging studies.