scholarly journals Diagnostic performance of 320 cardiac MDCT angiography in assessment of PDA either isolated or associated with duct dependent congenital heart disease

2021 ◽  
Vol 52 (1) ◽  
Author(s):  
Samar Abdelatty Gad ◽  
Ekhlas Abdelmonem Shaban ◽  
Mohammed Mahmoud Dawoud ◽  
Mohamed Ahmed Youssef
Keyword(s):  
Aortic Arch ◽  
Congenital Heart ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Morphological Type ◽  
Mdct Angiography ◽  
Transposition Of Great Arteries ◽  
Cardiac Anomalies ◽  
Cardiac Mdct

Abstract Background Patent ductus arteriosus (PDA) is one of most common congenital heart defects, it's a unique vascular structure that provides direct communication between pulmonary and systemic circulation. MDCT angiography is a good imaging modality for evaluation of the PDAs and detection of their exact morphological type; course and diameters, which is important before percutaneous closure or stenting procedure of the PDA, also for selection of closure hardware. The aim of this study was to assess the role of MDCT angiography in qualitative and quantitative evaluation of PDA and associated cardiac and\or extracardiac anomalies. Results Echocardiography detected PDA in 28\30 cases while cardiac MDCT detected PDA in all studied 30 cases confirmed by cardiac catheterization and/or operation. MDCT angiography had sensitivity 100% and specificity 100% for PDA detection. PDA originated from aortic isthmus in 15 cases, inferior surface of aortic arch in 11 cases and innominate artery in 4 cases. The most common morphological type of PDA was type A (cone\46.67%) followed by type C (tubular\23.3%), type D (complex\10%), type E (elongated\13.33%) and type B (window\6.67%). The spearman correlation coefficient test demonstrated poor correlation between size of aortic end and MPA (P = 0.75), and between size of pulmonary end and diameter of MPA (P = 0.99) and also demonstrated fair correlation between length of PDA and MPA (P = 0.018). PDA was isolated in 4\30 cases and associated with cardiac and\or extra cardiac anomalies in 26\30 cases included; ASD (n = 18), VSD (n = 16), pulmonary atresia (n = 7), transposition of great arteries (n = 5), teratology of Fallot (n = 4), aortic coarctation (n = 4), persistent truncus arteriosus (n = 3), tricuspid atresia (n = 3), anomalous of pulmonary venous return (n = 3), hypoplastic segment of aorta (n = 2), Ebstein's anomaly (n = 1), bicuspid aortic arch (n = 1) and left hypoplastic heart syndrome (n = 1). Conclusion Cardiac MDCT angiography was superior to Echocardiography in detection, quantitative and qualitative evaluation of PDA either isolated or associated with congenital cardiac and\or extracardiac anomalies and was superior to Echocardiography in detection of associated extracardiac anomalies rather than associated intra cardiac anomalies.

scholarly journals Veleszületett szívhibák sebészi kezelése 60 éves kor felett

2016 ◽  
Vol 157 (21) ◽  
pp. 820-824
Author(s):  
István Hartyánszky ◽  
Sándor Varga ◽  
László Csepregi ◽  
Barna Babik ◽  
Judit Simon ◽  
...  
Keyword(s):  
Heart Disease ◽  
Valve Replacement ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Multiorgan Failure ◽  
Ductus Arteriosus ◽  
Right Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Transposition Of Great Arteries

Introduction: The population with congenital heart disease is increasing and ageing. Aim: The aim of the authors was to examine the outcome of surgical management of congenital heart diseases beyond the age of 60 years. Method: Between 2013 and 2015, 77 adults were operated (36 younger, and 41 older than forty years, including 12 patients aged over 60 years. The numbers of procedures were as follows (in brackets the number of operations in the three age groups): Ross surgery 5 (3, 2, 0); aortic valve replacement 19 (12, 1, 6); subaortic membrane resection 1 (0, 0, 1); Bentall/ascending aortic plasty 8 (4, 3, 1); myectomy with or without mitral valve replacement in left ventricular outflow obstruction 5 (0, 3, 2); aortic coarctation 1 (1, 0, 0); ligation of ductus arteriosus 2 (1, 1, 0); reconstruction of right ventricular outflow tract with biological valve 4 (0, 3, 1); homograft 5 (5, 0, 0); BioValsalva graft 1 (0, 1, 0); primary reconstruction of complete atrioventricular septum defect 3 (1, 2, 0); valve replacement 2 (1, 1, 0); ventricular septum defect 10 (4, 6, 0); atrial septum defect closure 5 (2, 3, 0); total cavopulmonal anastomosis 1 (1, 0, 0); valve replacement in congenital transposition of great arteries 1 (0, 1, 0), Ebstein operation with valve plasty 2 (0, 1, 1); valve replacement 2 (1, 0, 1). Results: There was no operative mortality, while early mortality occurred in one patient with total cavopulmonal anastomosis due to multiorgan failure. Conclusions: Congenital heart defects can be operated beyond the age of 60 years with good results in a tertiary heart centre having great experience in the management of congenital and acquired heart disease. Orv. Hetil., 2016, 157(21), 820–824.

Congenital heart disease in adults

2018 ◽  
Author(s):  
Liz Orchard
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Left Ventricular ◽  
Interventional Treatment ◽  
Transposition Of Great Arteries ◽  
Complex Lesions

Congenital heart disease is the most common congenital abnormality, affecting 0.8% of births. There have been major advances in both the surgical and interventional treatment of congenital heart disease, with about 85% of patients now surviving into adulthood. Congenital heart disease can be divided into simple and complex lesions. Simple lesions include atrial septal defect, ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, and left ventricular outflow tract lesions. Complex lesions include tetralogy of Fallot, complete transposition of great arteries, single ventricle/Fontan physiology, pulmonary atresia, and Ebstein’s anomaly of the tricuspid valve. This chapter reviews the clinical features and management of those congenital cardiac disorders seen in adolescents and adults.

Transition to Ductal Stenting for Single Ventricle Patients Led to Improved Survival: An Institutional Case Series

2021 ◽  
Vol 12 (4) ◽  
pp. 518-526 ◽  
Author(s):  
Neel K. Prabhu ◽  
Alexander Zhu ◽  
James M. Meza ◽  
Kevin D. Hill ◽  
Gregory A. Fleming ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Case Series ◽  
Stage Ii ◽  
Shunt Revision ◽  
High Morbidity ◽  
Mechanical Ventilation Duration ◽  
Hospital Morbidity

Background: The use of systemic-to-pulmonary shunts (SPS) in neonates with single ventricle heart defects and ductal-dependent pulmonary blood flow (ddPBF) was historically associated with high morbidity and mortality at our center. As a result, we transitioned to the preferential use of ductus arteriosus stents (DS) when feasible. This report describes our initial results with this strategy. Methods: A single-center study of single ventricle patients that received DS or SPS from 2015 to 2019 was performed to assess whether DS was associated with decreased in-hospital morbidity and increased survival to stage II palliation. Results: A total of 34 patients were included (DS = 11; SPS = 23). Underlying cardiac anomalies were similar between groups and included pulmonary atresia, unbalanced atrioventricular septal defect, and tricuspid atresia. Procedure success was similar between groups (82% vs 83%). Two DS patients were converted to SPS, due to ductal vasospasm or pulmonary artery obstruction, and four SPS patients required surgical shunt revision. In DS patients, postprocedure mechanical ventilation duration was shorter (one vs three days, P = .009) and fewer required postprocedure extracorporeal membrane oxygenation (9% vs 39%, P = .11). A higher proportion of DS patients survived to stage II palliation (100% vs 64%, P = .035), and the probability of one-year survival was higher in DS patients (100% vs 61%, P = .02). Conclusions: At our center, patients with single ventricle heart defects and ddPBF that received DS experienced reduced in-hospital morbidity and increased survival to stage II palliation compared to SPS.

An unusual case of tetralogy of Fallot with an absent pulmonary valve associated with a retro-aortic innominate vein in a patient with a 16p12.2 microdeletion

2021 ◽  
pp. 1-2
Author(s):  
Niall Linnane ◽  
Andrew Green ◽  
Colin J. McMahon
Keyword(s):  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Unusual Case ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Innominate Vein ◽  
Absent Pulmonary Valve ◽  
Rare Association

Abstract 16p12.2 microdeletion has been associated with congenital heart defects and developmental delay. In this case, we describe the rare association between tetralogy of Fallot with an absent pulmonary valve a right-sided aortic arch and a retro-aortic innominate vein associated with a 16p12.2 microdeletion and epilepsy.

Lecompte Procedure for Relief of Severe Airway Compression in Children With Congenital Heart Disease

2019 ◽  
Vol 10 (5) ◽  
pp. 558-564
Author(s):  
Christina L. Greene ◽  
Richard D. Mainwaring ◽  
Douglas Sidell ◽  
Michal Palmon ◽  
Frank L. Hanley
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Surgical Procedure ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Heart Defects ◽  
Pulmonary Atresia ◽  
Absent Pulmonary Valve ◽  
Airway Compression ◽  
Absent Pulmonary Valve Syndrome

Purpose: Children with congenital heart disease may present with severe airway compression prior to any surgical procedure or may develop airway compression following their surgical procedure. This combination of congenital heart defect and airway compression poses a significant management challenge. The purpose of this study was to review our experience with the Lecompte procedure for relief of severe airway compression. Methods: This was a retrospective review of ten patients who underwent a Lecompte procedure for relief of severe airway compression over the past nine years (2010-2018). Three patients with absent pulmonary valve syndrome presented with severe symptoms prior to any surgical procedure. Seven patients presented with symptoms of airway compression following repair of their congenital heart defects (one with absent pulmonary valve syndrome, three patients had repair of pulmonary atresia with ventricular septal defect, and three patients had undergone aortic arch surgery). The median age at presentation was two years (range: one day to seven years). Results: The ten patients underwent a Lecompte procedure without any significant complications or operative mortality. The median interval between the surgical procedure and extubation was 9.5 days. No patients have required any further interventions for relief of airway obstruction. Conclusions: The Lecompte procedure is a surgical option for young children who present with severe airway compression. The patients in this series responded well to the Lecompte procedure as evidenced by clinical relief of airway compression.

scholarly journals Congenital Heart Defects Are Rarely Caused by Mutations in Cardiac and Smooth Muscle Actin Genes

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Tatiana Khodyuchenko ◽  
Anna Zlotina ◽  
Tatiana Pervunina ◽  
Dmitry Zverev ◽  
Anna Malashicheva ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Smooth Muscle Actin ◽  
Ductus Arteriosus ◽  
Control Group ◽  
Missense Mutations ◽  
Muscle Actin ◽  
Cardiac Actin

Background. Congenital heart defects (CHDs) often have genetic background due to missense mutations in cardiomyocyte-specific genes. For example, cardiac actin was shown to be involved in pathogenesis of cardiac septum defects and smooth muscle actin in pathogenesis of aortic aneurysm in combination with patent ductus arteriosus (PDA). In the present study, we further searched for mutations in humanα-cardiac actin (ACTC1) and smooth muscleα-actin (ACTA2) genes as a possible cause of atrial septum defect type II (ASDII) and PDA.Findings. Total genomic DNA was extracted from peripheral blood of 86 individuals with ASDs and 100 individuals with PDA. Coding exons and flanking intron regions ofACTC1(NM_005159.4) andACTA2(NM_001613) were amplified by PCR with specific primers designed according to the corresponding gene reference sequences. PCR fragments were directly sequenced and analyzed. Sequence analysis ofACTC1andACTA2did not identify any nucleotide changes that altered the coding sense of the genes. InACTC1gene, we were able to detect one previously described nucleotide polymorphism (rs2307493) resulting in a synonymous substitution. The frequency of this SNP was similar in the study and control group, thus excluding it from the possible disease-associated variants.Conclusions. Our results confirmed that the mutations inACTC1gene are rare (at least <1%) cause of ASDII. Mutations inACTA2gene were not detected in patients with PDA, thus being excluded from the list of frequent PDA-associated genetic defects.

The ductus arteriosus and associated cardiac anomalies in interruption of the aortic arch

1982 ◽  
Vol 2 (3) ◽  
pp. 185-193 ◽  
Author(s):  
Arentje Oppenheimer-Dekker ◽  
Adriana C. Gittenberger-de Groot ◽  
Henk Roozendaal
Keyword(s):  
Aortic Arch ◽  
Ductus Arteriosus ◽  
Cardiac Anomalies

scholarly journals Head growth in fetuses with isolated congenital heart defects: lack of influence of aortic arch flow and ascending aorta oxygen saturation

2016 ◽  
Vol 48 (3) ◽  
pp. 357-364 ◽  
Author(s):  
F. A. R. Jansen ◽  
E. W. van Zwet ◽  
M. E. B. Rijlaarsdam ◽  
E. Pajkrt ◽  
C. L. van Velzen ◽  
...  
Keyword(s):  
Oxygen Saturation ◽  
Aortic Arch ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Defects ◽  
Ascending Aorta ◽  
Head Growth

scholarly journals Spectrum of associated congenital heart defects in patients with “Non-Cardiac Congenital defects at a tertiary care children hospital in Pakistan”

2021 ◽  
Vol 37 (3) ◽  
Author(s):  
Muhammad Sohail Arshad ◽  
Muhammad Aslam ◽  
Shahnab Ahmad ◽  
Muhammad Kashif
Keyword(s):  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Vascular Malformations ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Tertiary Care ◽  
Primary Diagnosis ◽  
Congenital Defects

Objectives: To assess the frequency and pattern of associated congenital heart disease (CHD) among patients with “non-cardiac congenital defects”. Methods: An observational study was done at Paediatric Cardiology Department, The Children’s Hospital and The Institute of Child Health, Multan, Pakistan, from December 2018 to November 2019. Children from birth to 15 years having non-cardiac congenital defects, referred for cardiac evaluation from surgical unit during the study period were enrolled. Echocardiography was done to confirm diagnosis of CHD by consultant pediatric cardiologist. Results: Out of a total of 323 cases, 176 (54.5%) were male. Out of 323 patients, 160 (49.5%) belonged to age one month to one year. Vascular malformations were the most frequent primary diagnosis among our cases, seen in 69 (21.4%) children followed by cleft lip and palate 55 (17.2%), cleft palate only 52 (16.1%), Cleft lip only 40 (12.4%) and ARM high variety 33 (10.2%). CHD was found among 42 (13.0%) children while patent ductus arteriosus (PDA) and VSD were the commonest finding seen in 14 (33.3%) and 6 (14.3%) children respectively. Conclusion: Frequency of associated CHD among patients with non-cardiac congenital defects was high (13.0%). Children with cleft lip and/or palate should be given more attention because of the high incidence of CHD in this group. Echocardiography must be advised for the timely identification of any possible type of CHD. doi: https://doi.org/10.12669/pjms.37.3.3604 How to cite this:Arshad MS, Aslam M, Ahmad S, Kashif M. Spectrum of associated congenital heart defects in patients with “Non-Cardiac Congenital defects at a tertiary care children hospital in Pakistan”. Pak J Med Sci. 2021;37(3):---------. doi: https://doi.org/10.12669/pjms.37.3.3604 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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