Displaying publications 1 - 20 of 35 in total

Abstract:
Sort:
  1. Jin QW, Jeswant Dillon J, Tjen Jhung L, Isman Rusani B
    Eur Heart J Case Rep, 2020 Dec;4(6):1-5.
    PMID: 33442636 DOI: 10.1093/ehjcr/ytaa441
    Background : Sinus of Valsalva aneurysm (SoVA) is a rare anomaly and can be divided into acquired and congenital forms, the latter being commonly associated with ventricular septal defects (VSDs). Rupture is a catastrophic complication with high mortality without urgent surgical intervention. We would like to highlight the use of echocardiography in an emergency setting for diagnosis and surgical intervention in a critically ill patient.

    Case summary : We report a 27-year-old female with history of conservatively managed VSD known since childhood. She presented with acute decompensated cardiac failure requiring intubation and inotropic support. Bedside echocardiography performed in the emergency department suggested a ruptured SoVA at the right coronary cusp with underlying supracristal VSD. Despite the patient being critically ill with multi-organ failure, surgery was performed as it was the patient's best chance for survival. Intraoperative findings tallied with the early echocardiographic results. She recovered gradually and was eventually discharged despite a stormy post-operative period.

    Discussion : This case report highlights the importance of prompt recognition of SoVA rupture by using bedside echocardiography. Surgical intervention needs to be early despite ongoing sepsis in view of acute mechanical failure. This case was unique as it illustrates a successful management of an acutely ill patient with multi-organ failure through early diagnosis, intensive perioperative stabilization, and surgical intervention.

    Matched MeSH terms: Heart Septal Defects, Ventricular
  2. Ram SP, Noor AR, Ariffin WA, Ariffin NA
    Singapore Med J, 1994 Apr;35(2):205-7.
    PMID: 7939823
    A 37-week gestation male boy was born to a gravida seven para six mother by spontaneous vertex delivery at home. The baby cried at birth. On day 3 of life, he was admitted for respiratory distress. Physical examination revealed ectrodactyly, thin dry skin, anomalous tear duct with cardiomegaly. X-ray revealed absent radii, cardiomegaly and hemivertebra at L1. Echocardiogram revealed perimembranous type of ventricular septal defect. A diagnosis of Ectodermal Dysplasia Ectrodactyly Clefting Syndrome with ventricular septal defect was made. He was managed conservatively in the nursery. However, he expired on day 27 of life following short spell of fever apnoeic episode due to neonatal sepsis.
    Matched MeSH terms: Heart Septal Defects, Ventricular/genetics*
  3. Chua C, Then K, Mohd Khalid KF
    Cardiol Young, 2023 May;33(5):827-828.
    PMID: 36511131 DOI: 10.1017/S1047951122002931
    Mycotic pseudoaneurysm secondary to infective endocarditis is an uncommon complication in CHD with conduit placement. We report a case of late presentation of bacterial infective endocarditis with pseudoaneurysm in an 8-year-old girl with underlying pulmonary atresia with ventricular septal defect, post Rastelli procedure done at the age of 3 years old.
    Matched MeSH terms: Heart Septal Defects, Ventricular*
  4. Ahzad Hadi Ahmad, Rabiatul Basria S.M.N. Mydin, Nur Ain Nisrina Roan, Abdul Rahman Azhari, Narazah Mohd Yusoff
    MyJurnal
    Advanced parental age is a risk factor for chromosomal abnormalities in their offspring. Trisomy X or Triple X syn- drome has previously been reported with advanced maternal age. Here we report two (2) cases of Trisomy X with paternal age as risk factor. Generally, Trisomy X individuals show variable physical and psychological manifesta- tions. However, both cases reported here have advanced paternal age as a risk factor; 55 years old (46 years old at conception) for Case 1 with patient having right eye squint, beaked nose, Posterior Misalignment Type Ventricular Septal Defect (PMVSD) and small Patent Ductus Arteriosus (PDA) with failure to thrive and 49 years old (45 years old at conception) for Case 2 with speech delay and protruding tongue. In view of that, advanced paternal age could possibly contribute the accumulation of de novo mutations in germ line mosaicism.

    Matched MeSH terms: Heart Septal Defects, Ventricular
  5. Shamsuddin AM, Wong AR, Anderson RH, Corno AF
    PMID: 26795906 DOI: 10.1177/2150135115603330
    A neonate with cyanosis at birth was found to have a rare type of totally anomalous pulmonary venous connection. The pulmonary veins entered a confluence posterior to the left atrial wall, which drained into the right superior cavoatrial junction. There were no other major structural defects and no evidence of isomerism. Because of the severe cyanosis, and the restrictive nature of the interatrial communication, we performed balloon atrioseptostomy to improve oxygenation. We then achieved successful surgical repair when the baby was aged 7 months.
    Matched MeSH terms: Heart Septal Defects, Atrial/surgery*
  6. Ahmad F, Sadiq MA, Chee KH, Mahmood Zuhdi AS, Wan Ahmad WA
    J Coll Physicians Surg Pak, 2014 Jun;24(6):441-3.
    PMID: 24953923 DOI: 06.2014/JCPSP.441443
    Pulmonary hypertension is frequently associated with atrial septal defect and various connective tissue disorders. This case describes a 74-year-old woman who presented with symptoms of heart failure and concomitant involvement of salivary glands and keratoconjunctivitis. An echocardiogram demonstrated ostium secundum atrial septal defect with left to right shunt and severe pulmonary hypertension. Laboratory investigations confirmed the diagnosis of Sjögren's syndrome (SS) with positive anti-nuclear factor and centromere SS-A/Ro pattern. Anti-Ro (SS-A) was found positive. Atrial septal defect was closed through transcatheter route with significant improvement in clinical outcome. This case report suggests a possible association of atrial septal defect with primary Sjögren's syndrome in an adult patient.
    Matched MeSH terms: Heart Septal Defects, Atrial/complications*; Heart Septal Defects, Atrial/therapy; Heart Septal Defects, Atrial/ultrasonography
  7. Talib N, Sayuti KA, Abdullah MS, Mohd Zain MR
    BMJ Case Rep, 2018 Mar 05;2018.
    PMID: 29507009 DOI: 10.1136/bcr-2017-221074
    Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect, and patients are usually symptomatic at a very young age. Survival to adulthood without surgical correction is extremely rare. We report a 33-year-old woman with a heart murmur and a history of a successful pregnancy. Echocardiogram revealed a large atrial septal defect with suspicious pulmonary vein anomaly. Chest radiograph demonstrated classical 'snowman' configuration. Cardiac catheterisation was consistent with anomalous pulmonary venous drainage. Cardiac CT confirmed supracardiac TAPVR, whereby all the pulmonary veins drain into the anomalous vein and finally to the superior vena cava. She remained asymptomatic and underwent a successful surgical repair.
    Matched MeSH terms: Heart Septal Defects, Atrial/complications*; Heart Septal Defects, Atrial/surgery
  8. Leong MC, Alwi M
    Cardiol Young, 2021 May 31.
    PMID: 34053471 DOI: 10.1017/S1047951121001980
    Device occlusion of perimembranous ventricular septal defect is gaining popularity with the emergence of newer, softer occluders and improved technical know-how. We report a 26-year-old lady with a moderate size perimembranous ventricular septal defect who had a new onset of bundle branch block shortly after device closure. The patient subsequently developed a complete atrio-ventricular heart block.
    Matched MeSH terms: Heart Septal Defects, Ventricular
  9. Moghaddam HM, Esfehani RJ, Panah NY, Esfehani AJ
    Ann Saudi Med, 2014 6 5;34(2):147-52.
    PMID: 24894784 DOI: 10.5144/0256-4947.2014.147
    BACKGROUND AND OBJECTIVES: The rate of consanguineous marriage is high in Middle Eastern countries such as Iran. The relationship between consanguineous marriage and congenital heart disease is discussed in some studies, but there is not much data for relationship between atrial septal defect (ASD) and consanguineous marriage. The aim of this study was to evaluate the relationship between consanguineous marriage and ASD echocardiographic characteristics.

    DESIGN AND SETTINGS: This was a cross-sectional study approved by Mashhad University of Medical Sciences ethics committee and took place in Mashhad, Iran, for a period of 3 years from August 2008 till September 2011.

    METHODS: In this cross-sectional study, 113 ASD patients participated and they were categorized into 3 groups on the basis of family relationship between their parents: first group-"no relationship," second group- "third degree relationship," and third group- "far relationship."

    RESULTS: Among the 54 male and 59 female ASD patients, the most prevalent type of ASD was ASD secundum (85.0%) followed by sinus venosus (8.8%). A total of 56% patients were present in the first group and 15% and 29% in the second group and the third group, respectively." The relationship between consanguinity and type of ASD (P < .001) and gender (P < .001 each) was observed. The relationship between the age of onset of disease and consanguinity (P=.003) was also observed.

    CONCLUSION: Considering the fact that there is a high prevalence of ASD and consanguineous marriage in Iran and bearing in mind the results of the present study, we recommend educating couples about the outcomes of consanguineous marriage in pre-marriage counseling.

    Matched MeSH terms: Heart Septal Defects, Atrial/classification; Heart Septal Defects, Atrial/epidemiology*
  10. Zainudin AR, Tiong KG, Mokhtar SA
    Ann Pediatr Cardiol, 2012 Jan;5(1):92-4.
    PMID: 22529613 DOI: 10.4103/0974-2069.93725
    Cor triatriatum dexter is a rare congenital heart anomaly where the right atrium is divided into two chambers by a membrane. We report a boy who had persistent mild cyanosis and diagnosed to have cor triatriatum dexter with secundum atrial septal defect by transoesophageal echocardiography. Interestingly, he had persistent mild cyanosis despite insignificant obstruction to the right ventricular inflow and normal pulmonary artery pressure. The pathophysiology, approach to the diagnosis, and mode of treatment are also discussed.
    Matched MeSH terms: Heart Septal Defects, Atrial
  11. Majid AA
    J Cardiovasc Surg (Torino), 1991 Nov-Dec;32(6):837-9.
    PMID: 1836465
    This paper reports a case of double orifice mitral valve which was treated conservatively. A new scheme of classification of this entity linked to the accepted lines of surgical treatment is introduced.
    Matched MeSH terms: Heart Septal Defects, Atrial/pathology*; Heart Septal Defects, Atrial/surgery
  12. Leong MC, Ahmed Alhassan AA, Sivalingam S, Alwi M
    Ann Thorac Surg, 2019 09;108(3):813-819.
    PMID: 30998905 DOI: 10.1016/j.athoracsur.2019.03.045
    BACKGROUND: Ductal stenting is performed to retrain involuted left ventricles (LVs) in patients with d-transposition of the great arteries and intact ventricular septum (TGA-IVS). However, its efficacy is largely unknown. This study aimed to determine the safety and efficacy of ductal stenting in retraining of the involuted LV in patients with TGA-IVS.

    METHODS: This was a single-center, retrospective study. Echocardiographic assessment of the LV geometry, mass, and free wall thickness was performed before stenting and before the arterial switch operation. Patients then underwent the arterial switch operation, and the postoperative outcomes were reviewed.

    RESULTS: There were 11 consecutive patients (male, 81.8%; mean age at stenting, 43.11 ± 18.19 days) with TGA-IVS with involuted LV who underwent LV retraining by ductal stenting from July 2013 to December 2017. Retraining by ductus stenting failed in 4 patients (36.3%). Two patients required pulmonary artery banding, and another 2 had an LV mass index of less than 35 g/m2. Patients in the successful group had improved LV mass index from 45.14 ± 17.91 to 81.86 ± 33.11g/m2 (p = 0.023) compared with 34.50 ± 10.47 to 20.50 ± 9.88 g/m2 (p = 0.169) and improved LV geometry after ductal stenting. The failed group was associated with an increased need for extracorporeal support (14.5% vs 50%, p = 0.012). An atrial septal defect-to-interatrial septum length ratio of more than 0.38 was associated with failed LV retraining.

    CONCLUSIONS: Ductal stenting is an effective method to retrain the involuted LV in TGA-IVS. A large atrial septal defect (atrial septal defect-to-interatrial septum length ratio >0.38) was associated with poor response to LV retraining.

    Matched MeSH terms: Heart Septal Defects, Atrial/surgery*
  13. Leong MC, Latiff HA, Hew CC, Mazlan SL, Osman H
    Echocardiography, 2013 Feb;30(2):E33-5.
    PMID: 23134298 DOI: 10.1111/echo.12044
    An inverted left atrial appendage is a rare phenomenon post cardiac surgery. The lesion presents as an additional mass in the left atrium, which would trigger unnecessary concerns and frequently, a battery of tests. The lesion can be easily diagnosed using echocardiography. We report a case of inverted left atrial appendage in a patient post repair of common arterial trunk. Echocardiographic pictures and features which help to identify this lesion as well as to differentiate it from other possible left atrial mass are described. This article aimed to improve the awareness of sonographers toward this rare but possible post operative lesion.
    Matched MeSH terms: Heart Septal Defects/surgery
  14. Leong MC, Kandavello G, Husin A, Perumal D, Kaur Khelae S
    Pacing Clin Electrophysiol, 2020 11;43(11):1252-1257.
    PMID: 32845014 DOI: 10.1111/pace.14049
    BACKGROUND: Elderly patients with atrial septal defect (ASD) often present with chronic atrial fibrillation and large left to right shunt. This study reports the experience of left atrial appendage (LAA) and ASD closure in patients with significant ASD and chronic atrial fibrillation.

    METHODS: We report six consecutive elderly patients with chronic atrial fibrillation and significant ASD who underwent LAA and fenestrated ASD closure from January 1, 2014 until December 31, 2019. All periprocedural and long-term (>1 year) outcomes were reported.

    RESULTS: Six patients (male: 33.3%; mean age: 66.8 ± 3.3 years) were included. Mean CHADS2 , CHA2 DS2 -VASc , and HAS-BLED scores were 2.33 ± 0.82, 3.83 ± 0.75, and 1.83 ± 0.75. Four patients underwent simultaneous procedure, while two patients underwent a staged procedure. Procedural success was achieved in all patients. Total occlusion was achieved during LAA occlusion without device embolization prior to ASD closure. Patients who underwent simultaneous procedure had a shorter total hospital stay and lower total hospital stay. During a follow-up period of 32.8 ± 19.4 months, both the devices were well seated. No device-related thrombosis or erosion reported. All patients remained in atrial fibrillation. No patients experienced any thromboembolic stroke or transient ischemic attack.

    CONCLUSIONS: LAA and ASD closure is feasible and can be safely performed in the same seating in elderly patients with a significant ASD.

    Matched MeSH terms: Heart Septal Defects, Atrial
  15. Alwi M
    Catheter Cardiovasc Interv, 2006 May;67(5):679-86.
    PMID: 16572430 DOI: 10.1002/ccd.20672
    Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable morphologic variability, affecting not only the pulmonary valve but also the tricuspid valve, the RV cavity and coronary arteries. With advances in interventional techniques and congenital heart surgery, the management of PAIVS continues to evolve. This review is an attempt at providing a practical approach to the management of this disease. The basis of our approach is morphologic classification as derived from echocardiography and angiography. Group A, patients with good sized RV and membranous atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often it is the only procedure required in this group with the most favourable outcome. Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts with single ventricle physiology. The treatment at presentation is patent ductus arteriosus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6-12 months later followed by Fontan completion after a suitable interval. Patients in Group B, the intermediate group, are those with borderline RV size, usually with attenuated trabecular component but well developed infundibulum. The treatment at presentation is RF valvotomy and PDA stenting +/- balloon atrial septostomy. Surgical re-interventions are not uncommonly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (11/2 - ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar obstruction or small pulmonary annulus. Catheter based interventions viz. repeat balloon dilatation or device closure of patent foramen ovale (PFO) may also be required in some patients.
    Matched MeSH terms: Heart Septal Defects/diagnosis; Heart Septal Defects/pathology; Heart Septal Defects/therapy*
  16. Looi LM
    Histopathology, 1981 Nov;5(6):615-22.
    PMID: 7319480
    Nineteen out of 121 consecutive cardiac biopsies from 107 patients were found to contain amyloid deposits on routine Congo red screening. Seventeen were left atrial appendages removed during mitral valvotomy for chronic rheumatic mitral valve disease while the remaining two were right atrial appendages excised during surgical repair of atrial septal defects. The distribution of amyloid deposits within the atria and their tinctorial characteristics are described. The high prevalence of atrial amyloidosis observed could not be attributed to generalized or senile amyloidosis. The possibility that this is a distinctive localized form of amyloidosis secondary to chronic heart disease is discussed.
    Matched MeSH terms: Heart Septal Defects, Atrial/complications
  17. Maran S, Ee R, Faten SA, Sy Bing C, Khaw KY, Erin Lim SH, et al.
    PLoS One, 2020;15(4):e0230982.
    PMID: 32315303 DOI: 10.1371/journal.pone.0230982
    Atrial septal defect (ASD) is one of the most common congenital heart defects diagnosed in children. Sarcomeric genes has been attributed to ASD and knockdown of MYH3 functionally homologues gene in chick models indicated abnormal atrial septal development. Here, we report for the first time, a case-control study investigating the role of MYH3 among non-syndromic ASD patients in contributing to septal development. Four amplicons which will amplifies the 40 kb MYH3 were designed and amplified using long range-PCR. The amplicons were then sequenced using indexed paired-end libraries on the MiSeq platform. The STREGA guidelines were applied for planning and reporting. The non-synonymous c. 3574G>A (p.Ala1192Thr) [p = 0.001, OR = 2.30 (1.36-3.87)] located within the tail domain indicated a highly conserved protein region. The mutant model of c. 3574G>A (p.Ala1192Thr) showed high root mean square deviation (RMSD) values compared to the wild model. To our knowledge, this is the first study to provide compelling evidence on the pathogenesis of MYH3 variants towards ASD hence, suggesting the crucial role of non-synonymous variants in the tail domain of MYH3 towards atrial septal development. It is hoped that this gene can be used as panel for diagnosis of ASD in future.
    Matched MeSH terms: Heart Septal Defects, Atrial/genetics*
  18. Haranal M, Hew CC, Dillon JJ
    World J Pediatr Congenit Heart Surg, 2019 11;10(6):793-795.
    PMID: 31701824 DOI: 10.1177/2150135119872202
    Interventricular septal hematoma following congenital cardiac surgery is an uncommon entity. Literature search reveals few cases of interventricular septal hematoma complicating pediatric cardiac surgery. We report a case of interventricular septal hematoma following patch closure of ventricular septal defect, with associated myocardial necrosis and myocardial rupture.
    Matched MeSH terms: Heart Septal Defects, Ventricular/surgery*
  19. Qureshi AU, Latiff HA, Sivalingam S
    Cardiol Young, 2014 Aug;24(4):756-9.
    PMID: 24016801 DOI: 10.1017/S1047951113001200
    Incomplete involution of valve of systemic venous sinus can present across a spectrum of anatomical lesions ranging from eustachian valve to division of right atrium (cor triatriatum dexter) with overlapping features. We present the case of a neonate presenting with cyanosis, having persistent valve of systemic venous sinus with anatomical details of the redundant tissue in right atrium suggesting an intermediate form between Chiari network and division of right atrium.
    Matched MeSH terms: Heart Septal Defects, Atrial/complications; Heart Septal Defects, Atrial/ultrasonography*
  20. Suhaimi Hussain, Wong A. Rahim, Ibrahim W. Pauzi W., Naing, N.N.
    MyJurnal
    Introduction: There are many factors that determine the survival of patients with VSD. Among these include size of VSD, position, pulmonary hypertension, bacterial sepsis, valvular involvement, associated anomalies with VSD, associated syndromes and age at first diagnosis. There has been no published local data as far as we know and this information will be useful especially for consultation with parents. Even though VSD in general has a good prognosis, whenever they have added risks for example pulmonary hypertension then they are at risk of further morbidity and mortality. Objective: To determine the factors that are associated with survival of patients with VSD. Design: Retrospective cohort. Materials and methods: All cases of isolated VSDs admitted to HUSM from 1996 to 2003 were reviewed. Results: Univariate Cox regression of survival time of patients with VSDs revealed that 4 factors had prognostic significance namely bacterial sepsis (HR= 287.7, 95% CI 51.1, 1618.5, P < 0.001), Down syndrome (HR = 14.89, 95% CI 3.00, 73.92, P = 0.001), pulmonary hypertension (HR=14.58, 95% CI 1.69, 125.7, P=0.015) and large VSDs (HR=8.23, 95% CI 1.5, 45, P=0.015). Bacterial sepsis was the only significant prognostic factors for the survival of patients with VSDs using the multivariate Cox proportional hazard model. Conclusion: Bacterial sepsis, pulmonary hypertension, large VSD and Down syndrome were the significant prognostic factors from Univariate Cox analysis, however bacterial sepsis was the only significant prognostic factor from Multivariate Cox analysis.
    Matched MeSH terms: Heart Septal Defects, Ventricular
Filters
Contact Us

Please provide feedback to Administrator (afdal@afpm.org.my)

External Links