Diseases Commons^™

Associated Symptoms In The Ten Days Before Diagnosis Of Kawasaki Disease., Annette L. Baker, Minmin Lu, L Luann Minich, Andrew M. Atz, Gloria L. Klein, Rosalind Korsin, Linda Lambert, Jennifer S. Li, Wilbert Mason, Elizabeth Radojewski, Victoria L. Vetter, Jane W. Newburger, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: To describe common associated symptoms within the 10 days before diagnosis in subjects enrolled in the Pediatric Heart Network's trial of steroid therapy in Kawasaki disease (KD).

STUDY DESIGN: Patients with acute KD were enrolled between days 4 and 10 of illness at 8 centers between 2002 and 2004. We defined common associated symptoms as those occurring in >or=10% of patients. Principal clinical criteria for KD were not included in this analysis.

RESULTS: Among 198 patients, irritability was reported in 98 (50%), vomiting in 88 (44%), decreased food/fluid intake in 73 (37%), cough in 55 (28%), diarrhea in 52 …

Universal Screening For Extracardiac Abnormalities In Neonates With Congenital Heart Disease., Javier H. Gonzalez, Girish S. Shirali, Andrew M. Atz, Sarah N. Taylor, Geoffrey A. Forbus, Sinai C. Zyblewski, Anthony M. Hlavacek

Manuscripts, Articles, Book Chapters and Other Papers

Extracardiac or genetic abnormalities (EGA) represent a factor in the morbidity of patients with congenital heart disease. We evaluated the way neonates with CHD are screened at our institution and determined the yield for the screening tests. We reviewed the charts of 223 neonates with structural CHD. Subjects were categorized into 6 groups: univentricular, left-sided obstructive lesions, right-sided obstructive lesions, septal defects, conotruncal defects (CTD), and other. We reviewed which patients underwent cranial ultrasonogram (CUS), abdominal ultrasonogram (AUS), and/or genetic studies (GS) as well as their results. There was a high prevalence of EGA in each group by CUS (32% …

Rationale And Design Of A Trial Of Angiotensin-Converting Enzyme Inhibition In Infants With Single Ventricle., Daphne T. Hsu, Seema Mital, Chitra Ravishankar, Renee Margossian, Jennifer S. Li, Lynn A. Sleeper, Richard V. Williams, Jami C. Levine, Brian W. Mccrindle, Andrew M. Atz, Darlene Servedio, Lynn Mahony, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Angiotensin converting enzyme (ACE) inhibitors are known to improve clinical outcome and ventricular function in adults with heart failure. Infants with single-ventricle physiology show abnormalities in ventricular function as well as poor growth. The ability of an ACE inhibitor to preserve ventricular function and improve growth in these infants is unknown.

METHODS: The Pediatric Heart Network designed a randomized, double-blind trial to compare outcomes in infants with single-ventricle physiology receiving enalapril or placebo. Neonates < or =45 days old were eligible. The primary outcome is weight-for-age Z-score at 14 months of age. Secondary outcomes include other measures of somatic growth, laboratory and functional measures of heart failure, developmental indices, measures of ventricular size and function, and the relationship of the renin-angiotensin-aldosterone system genotype to the response to enalapril. The incidence and spectrum of adverse events will also be compared between treatment groups.

RESULTS: A total of 1,245 neonates were screened and 533 (43%) were eligible. The consent rate was 43%; 230 subjects were enrolled. Parental reluctance to …

Sequential Segmental Analysis., Robert H. Anderson, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Design And Rationale Of A Randomized Trial Comparing The Blalock-Taussig And Right Ventricle-Pulmonary Artery Shunts In The Norwood Procedure., Richard G. Ohye, J William Gaynor, Nancy S. Ghanayem, Caren S. Goldberg, Peter C. Laussen, Peter C. Frommelt, Jane W. Newburger, Gail D. Pearson, Sarah Tabbutt, Gil Wernovsky, Lisa M. Wruck, Andrew M. Atz, Steve D. Colan, James Jaggers, Brian W. Mccrindle, Ashwin Prakash, Michael D. Puchalski, Lynn A. Sleeper, Mario P. Stylianou, Lynn Mahony, Pediatric Heart Network Investigators, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: The initial palliative procedure for patients born with hypoplastic left heart syndrome and related single right ventricle anomalies, the Norwood procedure, remains among the highest risk procedures in congenital heart surgery. The classic Norwood procedure provides pulmonary blood flow with a modified Blalock-Taussig shunt. Improved outcomes have been reported in a few small, nonrandomized studies of a modification of the Norwood procedure that uses a right ventricle-pulmonary artery shunt to provide pulmonary blood flow. Other nonrandomized studies have shown no differences between the two techniques.

METHODS: The Pediatric Heart Network designed a randomized clinical trial to compare outcomes for …

Rhabdomyosarcoma Embryonal Type: In A Three And A Half Year Old Girl, Ameer A Khowaja, Saqib Ali Gowani, Asmatullah Khan, Saulat H. Fatimi

Department of Biological & Biomedical Sciences

Rhadomyosarcoma is the most common tumour of the soft tissues in infants and children. We report a case of a 3 1/2 year old girl who presented with a swelling over the neck. Swelling was diagnosed as Rhabdomyosarcoma (embryonal type). Tumour mass was found to be unresectable initially. The patient was subjected to multiple courses of checmotherapy, which shrunk the tumour significantly. There was simultaneous development of cystic lesions in upper lobe of right lung. Excision of the remnant rhabdomyosarcoma mass and biopsy of right lung with cystectomy of right upper lobe cystic lesion was performed. The patient fully recovered …

Functional Status, Heart Rate, And Rhythm Abnormalities In 521 Fontan Patients 6 To 18 Years Of Age., Andrew D. Blaufox, Lynn A. Sleeper, David J. Bradley, Roger E. Breitbart, Allan Hordof, Ronald J. Kanter, Elizabeth A. Stephenson, Mario Stylianou, Victoria L. Vetter, J Philip Saul, Pediatric Heart Network Investigators

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Our objective was to determine the relationship between functional outcome and abnormalities of heart rate and rhythm after the Fontan operation.

METHODS: The National Heart, Lung, and Blood Institute Pediatric Heart Network conducted a cross-sectional analysis of patients who had undergone a Fontan procedure at the 7 network centers. Analysis was based on 521 patients with an electrocardiogram (n = 509) and/or bicycle exercise test (n = 404). The Child Health Questionnaire parent report and the oxygen consumption at the anaerobic threshold were used as markers of functional outcome.

RESULTS: Various Fontan procedures had been performed: intracardiac lateral tunnel …

Three Dimensional Echocardiography In Congenital Heart Defects., Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

Three dimensional echocardiography (3DE) is a new, rapidly evolving modality for cardiac imaging. Important technological advances have heralded an era where practical 3DE scanning is becoming a mainstream modality. We review the modes of 3DE that can be used. The literature has been reviewed for articles that examine the applicability of 3DE to congenital heart defects to visualize anatomy in a spectrum of defects ranging from atrioventricular septal defects to mitral valve abnormalities and Ebstein's anomaly. The use of 3DE color flow to obtain echocardiographic angiograms is illustrated. The state of the science in quantitating right and left ventricular volumetrics …

Left Ventricular Noncompaction Mimicking Peripartum Cardiomyopathy., Chetan Patel, Girish S. Shirali, Naveen Pereira

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Survival After Bidirectional Cavopulmonary Anastomosis: Analysis Of Preoperative Risk Factors., Mark A. Scheurer, Elizabeth G Hill, Nagavardhan Vasuki, Scott Maurer, Eric M. Graham, Varsha Bandisode, Girish S. Shirali, Andrew M. Atz, Scott M. Bradley

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVE: Prognostic factors for survival after bidirectional cavopulmonary anastomosis for functionally single ventricle are not well defined. We analyzed preoperative hemodynamic and echocardiographic data to determine risk factors for death or transplantation at least 1 year after bidirectional cavopulmonary anastomosis.

METHODS: Data for all patients who underwent bidirectional cavopulmonary anastomosis before 5 years of age at our institution from September 1995 through June 2005 were analyzed. Available preoperative echocardiograms and catheterizations were reviewed. Survivors were compared with those who died or underwent transplantation. Bivariable associations between demographic and clinical risk factors and survival status (alive without transplantation vs dead or …

Incidence And Outcome Of Cardiopulmonary Resuscitation In Patients With Shunted Single Ventricle: Advantage Of Right Ventricle To Pulmonary Artery Shunt., Eric M. Graham, Geoffrey A. Forbus, Scott M. Bradley, Girish S. Shirali, Andrew M. Atz

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Cardiac Transplant Following Failed Fontan Or Glenn Procedures., Naveen L. Pereira, Girish S. Shirali

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Ischemic Stroke Subtypes In Pakistan: The Aga Khan University Stroke Data Bank, N. A. Syed, B. A. Khealani, S. Ali, A. Hasan, H. Brohi, T. Mozaffar, N. Ahmed, A. Hameed, S. M. Baig, M. Wasay

Department of Medicine

Objective: Frequency of ischemic stroke subtypes is influenced by ethnic and geographic variables. Our objective was to identify various stroke subtypes and its determinants at a tertiary care hospital.

Methods: We prospectively collected data on ischemic stroke subtypes admitted to The Aga Khan University Hospital in Karachi.

Results: A total of 596 patients were enrolled in 22 months in the Aga Khan Universtiy Stroke Registry. These included 393 patients with Ischemic stroke, 126 patients with intracerebral hemorrhage, 50 patients with subarachnoid hemorrhage and others. The ischemic stroke group was classified according to the TOAST criteria and comprised of lacunar 168/393 …

Risk Factors For Cardiovascular Disease In School Children--A Pilot Study., A. K. Khuwaja, Zafar Fatmi, W. B. Soomro, N. K. Khuwaja

Community Health Sciences

OBJECTIVE:

To assess the frequencies of risk factors for cardiovascular disease in school children. The information may help in designing interventions aimed at modifying unhealthy lifestyle in children, which may reduce the later incidence of cardiovascular disease in adults.

METHODS:

A cross-sectional study was conducted on 206 students (ages 14-18 years), enrolled in higher secondary school. Students were interviewed about their lifestyles, family history of cardiovascular disease and its risk factors. Moreover, they were assessed for height, weight and blood pressure.

RESULTS:

Twenty nine percent of the children were physically inactive, 31% were taking unhealthy diet daily, 21% were overweight …

Probucol Prevents Early Coronary Heart Disease And Death In The High-Density Lipoprotein Receptor Sr-Bi/Apolipoprotein E Double Knockout Mouse, Anne Braun, Songwen Zhang, Helena E. Miettinen, Shamsah Ebrahim, Teresa M. Holm, Eliza Vasile, Mark J. Post

Dartmouth Scholarship

Mice with homozygous null mutations in the high-density lipoprotein receptor SR-BI (scavenger receptor class B, type I) and apolipoprotein E genes fed a low-fat diet exhibit a constellation of pathologies shared with human atherosclerotic coronary heart disease (CHD): hypercholesterolemia, occlusive coronary atherosclerosis, myocardial infarctions, cardiac dysfunction (heart enlargement, reduced systolic function and ejection fraction, and ECG abnormalities), and premature death (mean age 6 weeks). They also exhibit a block in RBC maturation and abnormally high plasma unesterified-to-total cholesterol ratio (0.8) with associated abnormal lipoprotein morphology (lamellar/vesicular and stacked discoidal particles reminiscent of those in lecithin/cholesterol acyltransferase deficiency and cholestasis). Treatment …

Is Dynamic Atrial Overdrive Pacing Anti-Arrhythmic Or Proarrhythmic: A Case Report, Ngai-Yin Chan, Miu-Fong Kwok, Chi-Chung Choy

Journal of the Hong Kong College of Cardiology

No abstract provided.

Respiratory Arrest In A Child After Flushing Of Pancuronium From The Dead Space Of Intravenous Cannula, Robyna Irshad Khan, Fazal Hameed Khan, Hamid Iqil Naqvi

Department of Anaesthesia

It is common practice for patients admitted to Intensive Care Units to have indwelling intravenous cannulae. Sometimes these cannulae are used only for the administration of drugs and are closed otherwise. Residual drugs in the deadspace of these intravenous cannulae as well as connected stoppers or 3-way stopcocks can later be flushed unknowingly. We would like to report a case in which inadvertent flushing of residual pancuronium caused complete muscle paralysis and respiratory arrest in a young child.

Drug Eluting Stent: A Major Advance In Fighting Coronary Artery Restenosis, Yuk-Kong Lau

Journal of the Hong Kong College of Cardiology

Since the first use of coronary angioplasty in 1977, there have been major growth and development in percutaneous coronary intervention (PCI). Due to the advances in techniques, experiences, devices, and newer potent antiplatelet and antithrombotic medications, PCI is increasingly performed in highly complex and high risk patients with a reasonably low complication rate. Coupled with reduced discomfort, shorter hospital stay and recovery time, it has far outnumbered the volume of coronary artery bypass surgery. Currently, 1.7 million PCI procedures are performed annually worldwide with increases by 15-20% per year the last decade.

Role Of Signal-Averaged Ecg In Predicting Results Of Flecainide Provocation Test Used In Family Screening For Brugada Syndrome, Ngai-Shing Mok, Chi-Chung Choy, Ngai-Yin Chan, Amy Ho

Journal of the Hong Kong College of Cardiology

Brugada syndrome (BS) is an inherited arrhythmogenic disease with an autosomal dominant mode of inheritance. Flecainide provocation test (FPT) has been shown to be highly sensitive and specific in unmasking the Brugada ECG pattern in affected subjects. We sought to test if late potential (LP) in signal averaged electrocardiogram (SAECG) is helpful in predicting the results of FPT used in family screening for BS. The study included 17 asymptomatic Chinese subjects from 8 families (M:F 10:7, mean age 24.8±11.4 years) who have undergone family screening for BS. All screened subjects had a normal 12-lead ECG at baseline. None had structural …

The Post-Madit Ii Era: Icd For All Post-Infarct Patients With Moderate To Severe Left Ventricular Dysfunction?, Kathy Lai-Fun Lee, Chu-Pak Lau

Journal of the Hong Kong College of Cardiology

Device therapy has become the preferred treatment for patients who have survived sudden cardiac death. Throughout the last decade, large-scale randomized clinical trials have provided consistent evidence on the clinical efficacy of device therapy. ICD is proven to be superior to anti-arrhythmic drugs in patients with structural heart diseases who suffer from haemodynamically significant ventricular arrhythmias. The role of ICD in primary prevention of sudden death in selected patients with coronary artery disease and left ventricular dysfunction is also widely established after the Multicenter Automatic Defibrillator Implant Trial (MADIT) and the Multicenter Unsustained Tachycardia Trial (MUSTT).

Percutaneous Revascularisation In Diabetic Patients With Coronary Artery Disease, Wai-Hong Chen, Chu-Pak Lau

Journal of the Hong Kong College of Cardiology

Diabetic patients with coronary artery disease are associated with an unfavourable long-term outcome. Surgical revascularisation on top of medical therapy has been shown to confer survival benefit in patients with mutivessel and left main disease. With the emergence of the percutaneous approach of revascularisation, several clinical trials have been conducted to compare these two modalities of treatment. Consistent results were demonstrated in the diabetic subgroup that coronary artery bypass grafting is superior to balloon angioplasty in preventing late adverse cardiac event. With the advancement of percutaneous technique using coronary stenting as the predominant mode of catheter based revascularisation, the latest …

Typical Symptoms Are Predictive Of Acute Coronary Syndromes In Women, Kerry A. Milner, Marjorie Funk, Amy L. Arnold, Viola Vaccarino

Nursing Faculty Publications

Background: Previous research suggests that the presentation of acute coronary syndromes (ACS) may differ in women and men. No study has prospectively evaluated the role of a comprehensive set of typical and atypical symptoms and whether different symptoms on presentation predict ACS diagnosis in women and men. Methods and Results: We directly observed 246 women and 276 men seen in the emergency department with symptoms suggestive of ACS and documented their symptoms verbatim. ACS was eventually diagnosed in 89 (36%) women and 124 (45%) men on the basis of standard electrocardiogram and cardiac enzyme criteria. Presence of typical symptoms (chest …

The Long-Term Clinical Prognosis In Patients With Anterior Wall And Non-Anterior Wall Acute Myocardial Infarction Referred To Primary Percutaneous Transluminal Coronary Angioplasty, Mingzhong Zhao, Dayi Hu, Tianchang Li, Sanqing Jia

Journal of the Hong Kong College of Cardiology

Objective: To investigate the long-term clinical prognosis in patients with anterior wall acute myocardial infarction (AW-AMI) and non AW-AMI (NAW-AMI) referred to primary percutaneous transluminal coronary angioplasty (P-PTCA). Methods: 287 patients with AMI who underwent P-PTCA were divided into AW-AMI group (142 cases) and NAW AMI group (145 cases) according to different sites of AMI. The baseline characteristics and coronary artery lesions of patients were analysed. The primary end points were in-hospital mortality and the major cardiovascular events (MACE) during a mean 17.3±9.8 months follow-up including the occurrences of non-fatal myocardial infarction, non-fatal congestive heart failure, revascularization of target vessels …

Appropriate Use Of Clopidogrel, Cyrus R Kumana, Bernard Man-Yung Cheung, Ij Lauder

Journal of the Hong Kong College of Cardiology

Clopidogrel (Plavix®) is an orally administered pro-drug, whose pharmacology and therapeutic role has been extensively reviewed in recent publications.1-5 In essence, it results in selective and irreversible antagonism of ADP-induced platelet aggregation. Such aggregation normally results in the surface expression of platelet glycoprotein (GP) IIb/IIIa receptors, which facilitate fibrinogen binding as well as consequential further platelet aggregation – the final common pathway of vascular occlusion. Specific cytochrome P450 enzymes are thought to generate the responsible active metabolite (believed to persist transiently). However, dosage adjustment is not recommended in hepatic impairment (unless severe) and drug-drug interactions and co-administration with meals are …

Mapping And Ablation Of Complex Cardiac Arrhythmia Guided By A Novel Three Dimensional Non-Contact Endocardial Activation Mapping System, Wing-Hong Fung, Chi-Kin Chan, Kam-Sang Woo, Wilson Wai-Man Chan

Journal of the Hong Kong College of Cardiology

Conventional cardiac activation mapping requires a sequentially-based method of positioning a catheter in contact with the endocardium to generate intracardiac electrograms at single points during sustained arrhythmia. Significant limitation of such point by point mapping may be encountered when applying to complex arrhythmia e.g. non-sustained arrhythmia or haemodynamically unstable arrhythmia. A novel non-contact mapping system (Ensite 3000 system) providing a three-dimensional simultaneous display of arrhythmia activation may offer a solution. The non-contact mapping system consists of a specially designed balloon catheter (Ensite EP catheter) with 64 insulated wires over the surface of the balloon for sensing electrical potentials on the …

The Use Of Wallstent And Smart Stents Incarotid Stent-Supported Angioplasty, Steven Siu-Lung Li

Journal of the Hong Kong College of Cardiology

In the endovascular approach of carotid stenosis treatment, stent-supported angioplasty is current state-of-the-art technique. Self-expandable stents are preferable to balloon-expandable stents due to the potential risk of deformation of the latter by external pressure. Wallstents and SMART stents are the more commonly used self-expandable stents. They appear to be of similar efficacy and safety but due to their different characteristics, their applications may be different according to different lesion characteristics.

Presentation And Symptom Predictors Of Coronary Heart Disease In Patients With And Without Diabetes, Marjorie Funk, Janice B. Naum, Kerry A. Milner, Deborah Chyun

Nursing Faculty Publications

The aims of this prospective, observational study were to compare: (1) symptom presentation of coronary heart disease (CHD) between patients with and without diabetes and (2) symptom predictors of CHD in patients with and without diabetes. We directly observed 528 patients with symptoms suggestive of CHD as they presented to the ED of a 900-bed cardiac referral center in the northeastern United States. There were no significant differences in symptom presentation of CHD between patients with and without diabetes, although patients with diabetes were slightly more likely to present with shortness of breath (P =.056). Patients with diabetes reported …

Arrhythmogenic Right Ventricular Cardiomyopathy, Wing-Hong Fung, Chi-Kin Chan, John E. Sanderson

Journal of the Hong Kong College of Cardiology

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a familial form of cardiomyopathy predominantly affecting the right ventricle but may progress to involve the left ventricle as well. At the late stage of the disease, it is sometimes difficult to differentiate with other forms of dilated cardiomyopathy. In most affected families, the mode of inheritance of the disease is autosomal dominant. Lately, the gene accounting for the autosomal recessive form of ARVC, which was associated with palmoplantar keratosis, was identified. ARVC can present with sudden cardiac death without any preceding symptoms in the young population. Therefore, ARVC must be included in the …

Association Of Viral Genome With Graft Loss In Children After Cardiac Transplantation., Girish S. Shirali, J Ni, R E. Chinnock, J K. Johnston, G L. Rosenthal, N E. Bowles, J A. Towbin

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: The survival of recipients of cardiac allografts is limited by rejection, lymphoproliferative disease, and coronary vasculopathy. The purpose of this study in children who had received heart transplants was to evaluate the cardiac allografts for myocardial viral infections and to determine whether the presence of viral genome in the myocardium correlates with rejection, coronary vasculopathy, or graft loss.

METHODS: We enrolled heart-transplant recipients 1 day to 18 years old who were undergoing evaluation for possible rejection and coronary vasculopathy. Endomyocardial-biopsy specimens were evaluated for evidence of rejection with the use of standard criteria and were analyzed for the presence …

Thrombolytic Therapy For Acute Myocardial Infarction In The Elderly, Bernard Bun-Lap Wong, Wai-Hong Chen

Journal of the Hong Kong College of Cardiology

Acute myocardial infarction (MI) is a leading cause of morbidity and mortality in developed countries. Patients ≥75 years constitute up to 30% of the acute MI population and are associated with a 50% in-hospital mortality. Thrombolytic therapy was shown in a metaanalysis of 9 largest randomized controlled trials to reduce 35-day mortality. However, the benefit was observed mainly among patients < 75 years old. For patients ≥ 75 years, the 35-day mortality was 24.3% in those treated with thrombolytic therapy and 25.3% in the placebo group, a difference not reaching statistical significance.