Non-immune hydrops fetalis represents the end-stage status of a variety of diseases, including metastatic tumors. We report a case of non-immune hydrops fetalis associated with multiple disseminated echogenic nodular lesions detected by ultrasound and confirmed by magnetic resonance. Cordocentesis demonstrated anemia and thrombopenia.
View Article and Find Full Text PDFResidual postoperative pneumoperitoneum (RPP) can be confused with postoperative complications. Our aim was to study RPP characteristics in pediatric patients. Prospective study in children with noncomplicated appendicitis, from July to December 2019, divided into open appendectomy (OA) or laparoscopic appendectomy (LA).
View Article and Find Full Text PDFJ Cardiovasc Echogr
October 2021
Background: While the short- and median-term survival has improved considerably in patients with Fontan circulation, cardiac function and exercise capacity are still reduced and may deteriorate over time. Cardiac magnetic resonance (CMR) is the gold standard for the assessment of ventricular volume and function. Speckle-tracking echocardiography (STE) is a myocardial deformation technique to assess ventricular function, with promising results.
View Article and Find Full Text PDFBackground: Atrial tachyarrhythmias (ATs) represent the major late complications of congenital heart diseases (CHDs) following surgery. Little is known about the association between echocardiographic parameters and AT.
Aims: This study aimed to investigate a potential correlation among clinical, echocardiographic, and electrocardiographic parameters and AT as well as to analyze outcomes in adults with CHD and AT.
Scimitar syndrome is a rare congenital disorder which is associated with right lung developmental abnormalities, mainly hypoplastic right lung. We present the case of a neonate with a retrocardiac lung lobe that, to the best of our knowledge, has not been described yet. Multimodality imaging can establish the diagnosis and enable a correct description of this anomaly and its anatomical varieties.
View Article and Find Full Text PDFThe development of multidetector computed tomography has triggered a revolution in the study of the aorta and other large vessels and has replaced angiography in the diagnosis of congenital anomalies of the aortic arch, particularly vascular rings. The major advantage of multidetector computed tomography is that it permits clear 3-dimensional assessment of not only vascular structures, but also airway and esophageal compression. The current update aims to summarize the embryonic development of the aortic arch and the developmental anomalies leading to vascular ring formation and to discuss the current diagnostic and therapeutic role of multidetector computed tomography in this field.
View Article and Find Full Text PDFCortical hyperostosis is a very uncommon side-effect of prolonged prostaglandin therapy with distinctive radiological signs that may be accompanied by painful swelling of the limbs and responds rapidly to withdrawal of therapy.
View Article and Find Full Text PDFIntroduction And Objectives: Although congenital heart defects are the most common major congenital abnormalities, the associated mortality has been decreasing due to improvements in their diagnosis and treatment. We assessed the usefulness of 64-multidetector computed tomography in the diagnosis and management of these patients.
Methods: This 5-year observational, analytical, retrospective, cohort study included a total of 222 tomographic studies of patients with congenital heart disease.
Mutations conferring loss of function at the FLNA (encoding filamin A) locus lead to X-linked periventricular nodular heterotopia (XL-PH), with seizures constituting the most common clinical manifestation of this disorder in female heterozygotes. Vascular dilatation (mainly the aorta), joint hypermobility and variable skin findings are also associated anomalies, with some reports suggesting that this might represents a separate syndrome allelic to XL-PH, termed as Ehlers-Danlos syndrome-periventricular heterotopia variant (EDS-PH). Here, we report a cohort of 11 males and females with both hypomorphic and null mutations in FLNA that manifest a wide spectrum of connective tissue and vascular anomalies.
View Article and Find Full Text PDFAneurysmal disease is uncommon in children, and its presence often leads to suspicion of genetic disorders (Loeys-Dietz syndrome, Marfan syndrome, Ehlers-Danlos syndrome, tuberous sclerosis), trauma, and infection. We describe the case of a newborn boy with generalized left lower limb hypoplasia associated with diffuse areas of arteriectasis combined with areas of stenosis and fusiform aneurysms of the iliac, femoral, and popliteal arteries. No additional vascular territories were affected.
View Article and Find Full Text PDFChagas' disease is caused by Trypanosoma Cruzi. It is considered as endemic in central and South America and is transmitted by several species of triatomic bedbug. However, there are other important ways of transmission between humans: vertical transmission and, above all, through blood products and transplants.
View Article and Find Full Text PDFPrimary cardiac tumors are infrequent, less than 15-20% are malignant, and most of them are sarcomas. Primary recidivant cardiac osteosarcomas are extremely rare, only a few cases have been reported, and the prognosis is ominous. We report a case of a primary cardiac osteosarcoma in a 70-year-old woman who was admitted to the hospital for evaluation of congestive heart failure.
View Article and Find Full Text PDFPersistence of the primitive hepatic venous plexus (PPHVP) is an uncommon anomaly that can present associated with complex congenital heart disease and inferior vena cava (IVC) development anomalies and complicate endovascular and surgical procedures. We report a case of PPHVP in a patient with complex congenital heart disease with unexplained persistent arterial desaturation after Kawashima operation and describe magnetic resonance angiography (Angio-MRI) findings leading to the diagnosis. We recommend that IVC-Angio-MRI be performed in preoperative evaluation of patients with complex congenital heart disease and left isomerism when redirection of systemic venous blood to the pulmonary arterial circulation is considered.
View Article and Find Full Text PDFPurpose: To prospectively evaluate accuracy of gadolinium-enhanced three-dimensional (3D) magnetic resonance (MR) angiography with gadodiamide and gadopentetate dimeglumine (0.1 mmol/kg), with intraarterial DSA as reference standard, for imaging abdominal and iliac arterial stenoses.
Materials And Methods: The study was approved by all institutional review boards; informed consent was obtained from each subject before procedures.
Improvements in the diagnosis and surgical treatment of congenital heart disease during infancy and childhood have resulted in an outstanding increase in the prevalence of these entities during adulthood. Congenital heart disease in the adult represents a new diagnostic challenge to the consultant cardiologist, unfamiliar with the anatomical and functional complexities of cardiac malformations. Assessment of adult congenital heart disease with imaging techniques can be as accurate as in children.
View Article and Find Full Text PDF