RESUMO
Generalised arterial calcification of infancy, an autosomal recessive disorder characterised by abnormal calcification of medium and large-sized arteries, represents a rare cause of dilated cardiomyopathy. We present the case of a two-month-old girl diagnosed posthumously with dilated cardiomyopathy. Studies suggest that early initiation of treatment can improve prognosis in generalised arterial calcification of infancy, so clinicians should be alert to the condition, especially in patients displaying generalised narrowing of medium and large-sized arteries.
RESUMO
IntroductionDiagnostic and interventional catheter angiography of the heart is frequently used in paediatric cardiology. It is also possible to detect urinary system anomalies with cineurography images that may be obtained during angiocardiography. In this study, the aim was to determine the frequency, distribution, and properties of urinary system anomalies accompanying heart diseases, and to find out the effectiveness of cineurographic images in detecting the urinary system anomalies. METHODS: The cineurographic images of 2022 children who had undergone angiocardiography between 1995 and 2015 were retrospectively examined. RESULTS: Urinary system anomalies were detected in 261 of the 2022 cases (12.9%). Of these 261 cases, 148 were males (56.7%), whereas 113 were females (43.3%). Among the heart diseases, the group most accompanied by urinary system anomalies was the non-cyanotic left-to-right shunted heart diseases, which was detected in 120 (39.1%) patients. Pelvicalyceal ectasia was the most common urinary system anomaly encountered and was detected in 89 patients (34.1%). Of the urinary system anomalies cases, 94 detected by cineurography were determined to be clinically severe. When the effectiveness of the cineurography was evaluated using the ultrasonography records of the patients, it was found to have 63.8% accuracy. CONCLUSION: Many of the patients with CHDs concomitantly have urinary system anomalies. It is possible to determine asymptomatic urinary system anomalies using cineurographic imaging during angiocardiography. This may lead to earlier treatments and improved prognosis for the patients, thus making it possible to prevent potential future problems.
Assuntos
Anormalidades Múltiplas , Angiocardiografia/métodos , Cardiopatias Congênitas/diagnóstico , Ultrassonografia/métodos , Sistema Urinário/anormalidades , Anormalidades Urogenitais/epidemiologia , Urografia/métodos , Adolescente , Doenças Assintomáticas , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/epidemiologia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos , Estados Unidos/epidemiologia , Sistema Urinário/diagnóstico por imagem , Anormalidades Urogenitais/diagnósticoRESUMO
This study aims to share the results of critically ill newborn cases with interrupted aortic arch (IAA) and Left ventricular outflow tract (LVOT) obstruction (LVOTO) who underwent the hybrid approach, which consists of bilateral pulmonary artery banding and/or patent ductus arteriosus stenting, as first-line treatment. This retrospective study includes the results of high-risk term newborns whom we applied a hybrid approach due to IAA and LVOTO in our clinic between January 1, 2021 and December 31, 2021. The demographic characteristics, hybrid approach methods and results of the cases were evaluated. Nine cases underwent hybrid approach during the study period. The mean age and weight at interventions were 7 days (3-16 days) and 3280 g (2700-4300 g). Six of the patients were diagnosed with type B IAA, 2 with type A, and one with type C. LVOTO was present in 7 patients. The success rate for the procedures was 100%. No patients died during the procedure or within the first 5 days after the procedure or from reasons related to the procedure. The median length of the hospital stay after stent placement was 28 days (22-35 days) for discharged patients. Three patients died in interstage period, and 6 patients underwent total corrective surgery after a median of 7 months (4-10 months). The average LVOT diameter was increased from 3.1 mm to 4.8 mm before total repair surgery. The hybrid approach should be kept in mind for treating high risk newborns with IAA with LVOTO and high-risk newborns who are not suitable for single stage total corrective surgery.