scholarly journals Chest radiographic findings in children with asplenia syndrome

2010 ◽  
Vol 4 (4) ◽  
pp. 585-594
Author(s):  
Panruethai Trinavarat ◽  
Kullana Tantiprawan ◽  
Apichai Khongphatthanayothin
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Pulmonary Atresia ◽  
Chest Radiographs ◽  
Pulmonary Vasculature ◽  
Ipsilateral Side ◽  
Radiographic Findings ◽  
Asplenia Syndrome

Abstract Background: Early diagnosis of asplenia syndrome is important because prophylactic antibiotic and proper vaccination will prevent serious infection. Most children with asplenia syndrome present with symptoms of congenital heart disease. Chest X-ray is usually the first line imaging modality. Objective: Define useful findings in chest radiograph that could suggest the diagnosis of asplenia syndrome. Methods: Chest radiographs of pediatric patients who had asplenia syndrome diagnosed between January 1, 2002 and June 30, 2008 in a single institute were retrospectively reviewed for the positions of the visceral organs in the chest and abdomen. Results: Three hundred seventy one chest radiographs of 30 patients were reviewed. The mean age at diagnosis was 3 years old. Asplenia was diagnosed by ultrasound in 27 patients, by CT scan in two patients, and by damaged red blood cell scintigraphy in one patient. Six important findings detected from chest radiographs were, 1) bilateral minor fissures, 16 cases (53%), 2) bilateral eparterial bronchi, 16 cases (53%), 3) ipsilateral side of stomach and liver, 12 cases (40%), 4) ipsilateral side of minor fissure or eparterial bronchus and stomach, 10 cases (33%), 5) symmetrical transverse lie of the liver, nine cases (30%), and 6) contralateral side of minor fissure or eparterial bronchus and the liver in seven cases (23%). All except two patients (93%) had at least one of the above findings. All patients had congenital heart diseases. Most of the heart diseases were pulmonary atresia or pulmonary stenosis (88%) and single ventricle (85%). Conclusion: Chest radiographs have high sensitivity in suggesting the diagnosis of asplenia syndrome, when detecting one or more of the above findings, particular in patients with congenital heart disease and decreased pulmonary vasculature.

scholarly journals Personalized Genetic Diagnosis of Congenital Heart Defects in Newborns

2021 ◽  
Vol 11 (6) ◽  
pp. 562
Author(s):  
Olga María Diz ◽  
Rocio Toro ◽  
Sergi Cesar ◽  
Olga Gomez ◽  
Georgia Sarquella-Brugada ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Malformations ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Genetic Diagnosis ◽  
Single Nucleotide Variants ◽  
Personalized Approach

Congenital heart disease is a group of pathologies characterized by structural malformations of the heart or great vessels. These alterations occur during the embryonic period and are the most frequently observed severe congenital malformations, the main cause of neonatal mortality due to malformation, and the second most frequent congenital malformations overall after malformations of the central nervous system. The severity of different types of congenital heart disease varies depending on the combination of associated anatomical defects. The causes of these malformations are usually considered multifactorial, but genetic variants play a key role. Currently, use of high-throughput genetic technologies allows identification of pathogenic aneuploidies, deletions/duplications of large segments, as well as rare single nucleotide variants. The high incidence of congenital heart disease as well as the associated complications makes it necessary to establish a diagnosis as early as possible to adopt the most appropriate measures in a personalized approach. In this review, we provide an exhaustive update of the genetic bases of the most frequent congenital heart diseases as well as other syndromes associated with congenital heart defects, and how genetic data can be translated to clinical practice in a personalized approach.

scholarly journals Prediction of Pulmonary to Systemic Flow Ratio in Patients With Congenital Heart Disease Using Deep Learning–Based Analysis of Chest Radiographs

2020 ◽  
Vol 5 (4) ◽  
pp. 449 ◽  
Author(s):  
Shuhei Toba ◽  
Yoshihide Mitani ◽  
Noriko Yodoya ◽  
Hiroyuki Ohashi ◽  
Hirofumi Sawada ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Deep Learning ◽  
Congenital Heart ◽  
Chest Radiographs ◽  
Flow Ratio

scholarly journals Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Heart Defects ◽  
Descriptive Analysis ◽  
Atrioventricular Septal Defect

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

Non-fluoroscopic cardiac ablation of neonates with CHD

2016 ◽  
Vol 27 (3) ◽  
pp. 592-596 ◽  
Author(s):  
Amee M. Bigelow ◽  
Brandon S. Arnold ◽  
Gregory C. Padrutt ◽  
John M. Clark
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Catheter Ablation ◽  
Supraventricular Tachycardia ◽  
Tricuspid Valve ◽  
Congenital Heart ◽  
Current Practice ◽  
Pulmonary Atresia ◽  
Intact Ventricular Septum ◽  
Cardiac Ablation

AbstractIn current practice, children with anatomically normal hearts routinely undergo fluoroscopy-free ablations. Infants and children with congenital heart disease (CHD) represent the most difficult population to perform catheter ablation without fluoroscopy. We report two neonatal patients with CHD in whom cardiac ablations were performed without fluoroscopy. The first infant had pulmonary atresia with intact ventricular septum with refractory supraventricular tachycardia, and the second infant presented with Ebstein’s anomaly of the tricuspid valve along with persistent supraventricular tachycardia. Both patients underwent uncomplicated, successful ablation without recurrence of arrhythmias. These cases suggest that current approaches to minimising fluoroscopy may be useful even in challenging patients such as neonates with CHD.

Pediatric Cardiology

PEDIATRICS ◽  
1958 ◽  
Vol 21 (1) ◽  
pp. 165-165
Author(s):  
PAUL R. LURIE
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Rheumatic Fever ◽  
Harvard Medical School ◽  
Congenital Heart ◽  
Pediatric Cardiology ◽  
Heart Diseases ◽  
Medical Center ◽  
Rapid Development ◽  
Laboratory Diagnosis

Many books on rheumatic fever, congenital heart disease, laboratory diagnosis of heart diseases, and lengthy sections on cardiologic subjects in pediatric textbooks have been written but until now there is no comprehensive textbook in the field of pediatric cardiology. It is fortunate that the first book to appear to fill this real need is an excellent one. Dr. Nadas, of the Children's Medical Center in Boston and Harvard Medical School, is a recognized authority in the field of cardiology and has contributed much to its recent rapid development.

scholarly journals Increased arterial stiffness in children with congenital heart disease

2017 ◽  
Vol 25 (1) ◽  
pp. 103-109 ◽  
Author(s):  
Anna-Luisa Häcker ◽  
Barbara Reiner ◽  
Renate Oberhoffer ◽  
Alfred Hager ◽  
Peter Ewert ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Arterial Stiffness ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Organ Damage ◽  
Mean Difference ◽  
Cardiac Damage ◽  
Need For Treatment ◽  
Reference Cohort

Objective Central systolic blood pressure (SBP) is a measure of arterial stiffness and strongly associated with atherosclerosis and end-organ damage. It is a stronger predictor of cardiovascular events and all-cause mortality than peripheral SBP. In particular, for children with congenital heart disease, a higher central SBP might impose a greater threat of cardiac damage. The aim of the study was to analyse and compare central SBP in children with congenital heart disease and in healthy counterparts. Patients and methods Central SBP was measured using an oscillometric method in 417 children (38.9% girls, 13.0 ± 3.2 years) with various congenital heart diseases between July 2014 and February 2017. The test results were compared with a recent healthy reference cohort of 1466 children (49.5% girls, 12.9 ± 2.5 years). Results After correction for several covariates in a general linear model, central SBP of children with congenital heart disease was significantly increased (congenital heart disease: 102.1 ± 10.2 vs. healthy reference cohort: 100.4 ± 8.6, p < .001). The analysis of congenital heart disease subgroups revealed higher central SBP in children with left heart obstructions (mean difference: 3.6 mmHg, p < .001), transpositions of the great arteries after arterial switch (mean difference: 2.2 mmHg, p = .017) and univentricular hearts after total cavopulmonary connection (mean difference: 2.1 mmHg, p = .015) compared with the reference. Conclusion Children with congenital heart disease have significantly higher central SBP compared with healthy peers, predisposing them to premature heart failure. Screening and long-term observations of central SBP in children with congenital heart disease seems warranted in order to evaluate the need for treatment.

The accuracy of chest radiographs in the detection of congenital heart disease and in the diagnosis of specific congenital cardiac lesions

2006 ◽  
Vol 36 (7) ◽  
pp. 677-681 ◽  
Author(s):  
Bernard F. Laya ◽  
Marilyn J. Goske ◽  
Stuart Morrison ◽  
Janet R. Reid ◽  
Leonard Swischuck ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Chest Radiographs ◽  
Cardiac Lesions

scholarly journals Role of TEE in Improving Diagnostic Accuracy of Congenital Heart Disease

2014 ◽  
Vol 2 (2) ◽  
pp. 68-70
Author(s):  
Sandeep Singh Rana ◽  
Balbir Kumar ◽  
Sethu Madhavan J
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Diagnostic Accuracy ◽  
Multimodal Imaging ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Associated Lesions ◽  
Cardiac Lesions ◽  
Intraoperative Period

ABSTRACT Complex congenital heart diseases (CHD) often present as multiple cardiac lesions. The presence of one anomaly should stimulate the physician to perform a comprehensive assessment and look for other associated anomalies.1 Multimodal imaging may be necessary to diagnose such associated lesions as single imaging may occasionally miss them. Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) are complimentary to each other. Routine use of TEE during intraoperative period may help us to refine diagnosis, detect the missing components and guide effective surgical repair. We present one such case where diagnosis and management were optimized by multimodal imaging. How to cite this article Kumar B, Madhavan JS, Puri GD, Rana SS. Role of TEE in Improving Diagnostic Accuracy of Congenital Heart Disease. J Perioper Echocardiogr 2014;2(2):68-70.

scholarly journals Stenting of modified Blalock–Taussig shunt in adult with palliated pulmonary atresia and ventricular septal defect: a case report

2019 ◽  
Vol 3 (4) ◽  
pp. 1-4
Author(s):  
Julia Illner ◽  
Holger Reinecke ◽  
Helmut Baumgartner ◽  
Gerrit Kaleschke
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Congenital Heart ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Complex Congenital Heart Disease ◽  
Bare Metal

Abstract Background Adults with complex congenital heart disease palliated with systemic-to-pulmonary artery shunts have become rare and represent a particularly challenging patient group for the cardiologist. One of the complications and causes of severe clinical deterioration during long-term follow-up are progressive obstruction or total occlusion of the shunt. The risk for surgical intervention is frequently high and catheter intervention may be complicated by complex anatomy and shunt calcification. Case summary We report the case of a 47-year-old man with uncorrected (palliated) pulmonary atresia and ventricular septal defect who presented with progressive cyanosis (oxygen saturation 69%) and decreasing exercise capacity. Computed tomography revealed a totally occluded modified left Blalock–Taussig (BT) shunt and a severely stenosed central shunt (Waterston–Cooley) in a patient with confluent but hypoplastic pulmonary arteries and multiple major aortic pulmonary collaterals. Due to a high operative risk, an interventional, percutaneous approach was preferred to re-do surgery. From a radial access the calcified BT shunt could be crossed with a hydrophilic guidewire. Then, a rotational thrombectomy, balloon dilatation, and bare-metal stenting at the proximal and distal anastomoses were performed. Post-interventionally, peripheral oxygen saturation increased from 69% to 82%. Clopidogrel was administered for 1 month after bare-metal stenting. At 1-year follow-up, the BT shunt was still patent on echocardiography and exercise tolerance markedly improved. Discussion This case highlights the benefit of percutaneous rotational thrombectomy followed by stenting of chronically occluded systemic-to-pulmonary artery shunts for further palliation in adult patients with complex congenital heart disease not suitable for surgical repair.

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