DOCSLIB.ORG

Common Cardiac Anomalies

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Common Cardiac Anomalies 1/7/2020 MATERNAL/NEWBORN & NICU FELLOWSHIP Presented by COMMON CONGENITAL ANOMALIES Peg Peterson APRN CONGENITAL-EXISTENCE AT OR BEFORE BIRTH Congenital anomalies are also known as: • Birth Defects • Congenital Disorders • Congenital Malformations Structural/Anatomical • Heart Defects • Club Foot • Cleft Lip or Palate Functional • Metabolic Disorders Chromosomal • Down’s Syndrome KEY FACTS •An estimated 303,000 newborns die within the first 4 weeks of birth every year, worldwide, due to congenital anomalies •Congenital anomalies can contribute to long term disability, which may have significant impacts on families, individuals, health care systems and societies •The most common severe congenital anomalies are heart defects, neural tube defects and Downs syndrome •Congenital anomalies maybe the result of one or more genetic, infectious, nutritional, or environmental factor •Difficult to identify the exact cause 1 1/7/2020 CAUSES AND RISK FACTORS 50% of all congenital anomalies cannot be linked to a specific cause •Genetic Factors • Inherited genes that code for a specific anomaly • Result of a mutation • Consanguinity • Increases genetic anomalies, doubles risk of neonatal and childhood death • Ethnic • Ashkenazi Jews and Finns-Cystic Fibrosis, Hemophilia C • Socioeconomic Factors •Environmental Factors •Infections •Maternal Nutritional Status PREVENTION Some congenital anomalies can be prevented • Vaccinations • Congenital rubella • Heart problems, microcephaly, premature birth, stillborn, miscarriage • Adequate intake of folic acid • Neural tube defects • Adequate iodine intake • Neural development • Adequate antenatal care • STD treatment • Pre-conception counseling • Zika Virus • Environmental chemicals/pesticides • Work related CONGENITAL HEART DEFECTS •Occur in about 1 in 110 births •An Anatomical Abnormality •Prenatal Diagnosis • Cardiac development happens at 8 weeks gestation •Defects are classified as Cyanotic/Non Cyanotic •Most common anomaly is Ventral Septal Defect (VSD) •28% of children with CHD have another recognized anomaly (trisomy 21, 13, 18) •Children can become symptomatic any time in first year of life 2 1/7/2020 CONGENITAL HEART DEFECTS Symptoms •Tachycardia •Tachypnea •Murmur •Failed Congenital Heart Disease Screening •Cyanosis •Cool clammy skin •Metabolic Acidosis •Failure to Thrive CONGENITAL HEART DEFECTS • PDA Increased • ASD Pulmonary Blood Flow • VSD * most common • AV Canal • PS Classification Decreased • TOF Pulmonary of CHD Blood Flow • TGV • Truncus Decreased • AS Systemic • HLHS Blood flow • CoA NORMAL HEART 3 1/7/2020 PATENT DUCTUS ARTERIOSUS (PDA) Failure to close after birth (usually closes first 12 hours after birth) Signs/Symptoms Asymptomatic is ductus small Can present with murmur on day 3 – 4 Wide pulse pressures Bounding pulses Medical Management O2 administration closes the PDA Assess for murmur on day 3 -4 Monitor pulses, fluid, and respiratory status May need addition PEEP Indomethacin, Ibuprofen Surgical ligation UNIVERSITY OF MICHIGAN HEALTH SYSTEMS ATRIAL SEPTAL DEFECT (ASD) Hole in the muscular wall (30% will close on their own within 6 months) Signs/Symptoms Usually asymptomatic May hear murmur Wide pulse pressures Bounding pulses Medical Management Assess for murmur on day 3 -4 Monitor pulses, fluid, and respiratory status Restrict fluids, increase calories Treat CHF if developed (rare) Surgical repair done at school age UNIVERSITY OF MICHIGAN HEALTH SYSTEMS VENTRAL SEPTAL DEFECT (VSD) Hole between ventricles (*most common CHD) Signs/Symptoms Asymptomatic is small Produces left to right shunt Murmur May have signs of CHF and FTT Medical Management Depends on severity Digoxin / diuretics Increased calories Most close spontaneously at school age Surgical repair if not spontaneously closed by school age UNIVERSITY OF MICHIGAN HEALTH SYSTEMS 4 1/7/2020 ENDOCARDIAL CUSHION DEFECT (AV CANAL) ASD, high VSD, and anomalies to tricuspid and mitral valves (may be partial or complete) 30 – 40% have Downs Syndrome Signs/Symptoms Murmur FTT CHF at about 4 -12 weeks Medical Management Treat CHF Surgical repair at 3 – 6 months UNIVERSITY OF MICHIGAN HEALTH SYSTEMS PULMONARY STENOSIS (PS) Obstructed RV outflow tract Signs/Symptoms Asymptomatic if mild Murmur Desaturations Medical Management Monitor pulmonary status May require prostaglandins Balloon valvuloplasty Surgical repair at 1 – 4 years UNIVERSITY OF MICHIGAN HEALTH SYSTEMS TETRALOGY OF FALLOT CYANOTIC - DECREASED PULMONARY BLOOD FLOW Four different defects (*most common cyanotic heart defect) Signs/Symptoms 1/3 of infants are cyanotic (“pink” vs “blue” depends on severity of PS Harsh murmur Increased RR, decreased pules, mottling of skin, polycythemia, metabolic acidosis Often see boot-shaped heart on CXR Medical Management Knee to chest position to increase SVR and venous return May require prostaglandins (‘blue” tets) Surgical repair not before 6 months UNIVERSITY OF MICHIGAN HEALTH SYSTEMS 5 1/7/2020 TRANSPOSITION OF GREAT ARTERIES CYANOTIC - DECREASED PULMONARY BLOOD FLOW Most common cause of cyanosis in the newborn period (1:3300 US births) Signs/Symptoms Will become symptomatic when ductus closes (between 1 day – 1 week) *need mixing 50% do not have murmur Cyanosis with crying Tachypnea CHF Medical Management Prostaglandins Monitor O2, too much will close ductus Maintain NTE Surgical repair done in first two weeks of life UNIVERSITY OF MICHIGAN HEALTH SYSTEMS TRUNCUS ARTERIOSUS (TA) Persistence of fetal structure (single outlet). There is always a large VSD Signs/Symptoms May be cyanotic CHF Medical Management Monitor and treat CHF Surgical repair UNIVERSITY OF MICHIGAN HEALTH SYSTEMS AORTIC STENOSIS (AS) Obstruction of aortic valve (blood not getting out to the body) Signs/Symptoms Murmur Decreased pulses CHF Medical Management Monitor and treat CHF May require immediate surgical repair UNIVERSITY OF MICHIGAN HEALTH SYSTEMS 6 1/7/2020 HYPOPLASTIC LEFT HEART SYNDROME CYANOTIC - MIXED BLOOD FLOW Includes varying degrees of MV atresia, hypoplastic LV, AS, and hypoplasia of the aorta. (*most common cause of death from cardiac defects in the first month of life) Signs/Symptoms Murmur Cyanosis, tachypnea, tachycardia, poor peripheral perfusion, metabolic acidosis Medical Management 3 options – Norwood, transplant, or no treatment Prostaglandins 3-stage surgical repair UNIVERSITY OF MICHIGAN HEALTH SYSTEMS COARCTATION OF THE AORTA (COA) Narrowing of aorta (diminished blood to lower extremities) Signs/Symptoms Murmur Upper extremity BP greater than lower Pulse discrepancies Decreased urine output CHF Medical Management If suspected – 4 extremity BP Prostaglandins Monitor and treat CHF May do balloon angioplasty Surgical repair UNIVERSITY OF MICHIGAN HEALTH SYSTEMS CRITICAL CONGENITAL HEART DISEASE SCREENING (CCHD) CCHD Screens for 7 different types of CHD •If baby is transferred back to M/B prior •Hypoplastic Left Heart Syndrome to 24 hrs of age, screening done by M/B •Pulmonary Atresia •In M/B – done between 36 – 48 hours of life •Tetralogy of Fallot •In NICU – done prior to discharge •Total Anomalus Pulmonary Venous Return •Failed test needs echocardiogram •Transposition of Great Arteries •Tricuspid Atresia •Truncus Arteriosus 7 1/7/2020 CRITICAL CONGENITAL HEART DISEASE SCREENING (CCHD) Negative Screen NORMAL Positive Screen ABNORMAL •O2 saturation ≥ 95% in right hand •O2 saturation < 90% in right hand and foot and foot NNP to assess / PCP notified and • • Echocardiogram ordered •O2 saturation ≤ 3% difference between right hand and foot If O2 sat 90% - 95% and > 3% difference – repeat screening 1 hour after initial screen No further follow needed OMPHALOCELE Failure of the intestine to return from the umbilical cord into the abdominal cavity • Covered in thin sac • 1:5500 - 800 per year in US • Look for midline defects Contributing Factors • Alcohol/Tobacco/SSRI use • Obesity Medical Management • Protect organs, decompression of the gut, adequate hydration • Thermoregulation • Surgical repair vs scarring of sac OMPHALOCELE 8 1/7/2020 GASTROSCHISIS Defect in abdominal wall allowing bowel outside of abdominal cavity • Protrudes thru hole beside umbilicus • 2000 cases in US a year • Poor abdominal growth Contributing Factors Medical Management • Protect organs, decompression of the gut, adequate hydration • Thermoregulation • May require staged repair depending on size of defect (silo) GASTROSCHISIS TRACHEOESOPHAGEAL FISTULA (TEF) Failure of trachea to differentiate and separate from the esophagus S/S • Maternal polyhydramnios • Need for frequent oral suctioning • Inability to pass ng/og • Choking, spitting, cyanosis Medical Management • At risk for aspiration pneumonia • Endotracheal suctioning • Surgical anastomosis 9 1/7/2020 TRACHEOESOPHAGEAL FISTULA (TEF) DUODENAL ATRESIA Absence or complete closure of a portion of the lumen of the duodenum • 1 in 6,000 – 10,000 live births • Nearly half are premature or low birth weight • 60 – 70% have associated anomalies (trisomy 21, malrotation, TE, CHD, imperforated anus, VACTERL, renal anomalies) S/S • Maternal polyhydramnios (significant risk factor) • Bilious vomiting • Failure to pass meconium • Double bubble on xray Medical Management • Gastric decompression (NG suction), hydration, management of electrolytes • Surgical repair DUODENAL ATRESIA 10 1/7/2020 IMPERFORATE ANUS Cause unknown, occurs 1 in every 20,000 live births • Broadly classified as high or low depending on relationship to the distal
Load more

Recommended publications
  • Usefulness Ofcontinuous Positive Airway Pressure in Differential
    Arch Dis Child: first published as 10.1136/adc.53.6.456 on 1 June 1978. Downloaded from Archives of Disease in Childhood, 1978, 53, 456-460 Usefulness of continuous positive airway pressure in differential diagnosis of cardiac from pulmonary cyanosis in newborn infants P. SYAMASUNDAR RAO, BRENDA L. MARINO, AND ALEX F. ROBERTSON III From the Department of Pediatrics, Sections of Pediatric Cardiology and Neonatology, Medical College of Georgia, Augusta, Georgia, USA SUMMARY Differential diagnosis of cyanosis in the neonate is difficult and cardiac catheterisa- tion may be required for a correct diagnosis. It has been suggested that the response of Pao2 to continuous positive airway pressure (CPAP) with 100% oxygen may be useful. The purpose of this study was to test further this hypothesis by studying all neonates investigated for cyanosis with a Pao2 -50 torr in 0 8 to 1 .0 F1o2. Arterial blood samples were obtained in an F1o2 of 0 21-0 .4 and 0 8-1 .0, and in an F1O2 of 0 8-1 0 with 8-10 cm CPAP, and were analysed for Pao2, Paco2, and pH, bicarbonate being calculated. The final diagnoses were congenital heart disease (CHD) 21 cases, pulmonary parenchymal disease (PD) 10 cases, and persistent fetal circulation (PFC) 3 cases. No significant difference in pH, bicarbonate, or Paco2 was observed among the three groups or with CPAP. In the CHD and PFC infants CPAP produced no significant change in Pao2. In the PD babies Pao2 increased by an average of 33 torr (P<0 05). Despite thus attaining statistical significance 2 PD infants had no increase in Pao2 with CPAP.
    [Show full text]
  • Pharmacy Policy Statement
    PHARMACY POLICY STATEMENT Ohio Medicaid DRUG NAME Synagis (palivizumab) BILLING CODE 90378 (1 unit = 1 vial) BENEFIT TYPE Medical SITE OF SERVICE ALLOWED Office/Outpatient Hospital/Home COVERAGE REQUIREMENTS Prior Authorization Required (Preferred Product) QUANTITY LIMIT— 1 vial per month (max 5 during respiratory syncytial virus season) LIST OF DIAGNOSES CONSIDERED NOT Click Here MEDICALLY NECESSARY Synagis (palivizumab) is a preferred product and will only be considered for coverage under the medical/pharmacy benefit when the following criteria are met: Members must be clinically diagnosed with one of the following disease states and meet their individual criteria as stated. PREVENTION OF RESPIRATORY TRACT DISEASE CAUSED BY RESPIRATORY SYNCYTIAL VIRUS (RSV) For initial authorization: 1. Request must be made during the RSV season (November 1st through March 31st) AND initiation of injections should be timed with the onset of laboratory confirmed cases of RSV activity in the community, no earlier than November 1, 2017; AND 2. Member is < 12 months old at the beginning of the RSV season AND meet one of the following criteria (chart notes must be provided to support evidence): a) Member was born < 29 weeks, 0 days’ gestation; b) Member has Chronic Lung Disease (CLD) of prematurity (defined as gestational age <32 weeks, 0 days and a requirement for >21% oxygen for at least the first 28 days after birth); c) Member has hemodynamically significant Congenital Heart Disease (CHD) with one or more of the following: i) Acyanotic heart disease (e.g. atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), etc.), AND member is receiving medication to control congestive heart failure (CHF) AND will require cardiac surgical procedures; ii) Moderate to severe pulmonary hypertension; iii) Cyanotic heart defect (e.g.
    [Show full text]
  • Review Article Congenital Heart Diseases
    KYAMC Journal Vol. 9, No.1, April 2018 Review Article Congenital heart diseases: A review of echocardiogram records Md. Saiful Islam1, Md. Moniruzzaman2 Abstract Congenital heart defect (CHD) means an anatomic malformation of the heart or great vessels which occurs during intrauterine development, irrespective of the age at presentation. They can disrupt the normal blood flow through the heart. The blood flow can slow down, go in the wrong direction or to the wrong place, or be blocked completely. Broadly congenital heart defects can be acyanotic and cyanotic. We have reviewed retrospectively from echocardiogram record nearly two years of period & collected total 404 patients with congenital heart defects. Among them 329 (81.43%) was acyanotic and 75 (18.57%) was cyanotic congenital defects with variety of diagnosis. Ventricular septal defect was the most common acyanotic heart defect and Tetralogy of Fallot was the most common cyanotic heart defect. There was no significant gender deference. Keywords: Acyanotic, Congenital heart disease, Cyanotic. Date of received: 11. 11. 2017 Date of acceptance: 05. 01. 2018 Introduction known. The majority of the defects can be explained by Congenital heart defects (CHD) are reported in almost 1% of multifactorial inheritance hypothesis which states that a live births, and about half of these children need medical or predisposed fetus, when exposed to a given environmental surgical management in infancy1. In the first decade, a further trigger, to which the fetus is sensitive during the critical period 25% require surgery to maintain or improve their life1. Only of cardiac morphogenesis may develop the disease5. A variety 10% survive to adolescence without specific treatment.
    [Show full text]
  • Pulmonary-Atresia-Mapcas-Pavsdmapcas.Pdf
    Normal Heart © 2012 The Children’s Heart Clinic NOTES: Children’s Heart Clinic, P.A., 2530 Chicago Avenue S, Ste 500, Minneapolis, MN 55404 West Metro: 612-813-8800 * East Metro: 651-220-8800 * Toll Free: 1-800-938-0301 * Fax: 612-813-8825 Children’s Minnesota, 2525 Chicago Avenue S, Minneapolis, MN 55404 West Metro: 612-813-6000 * East Metro: 651-220-6000 © 2012 The Children’s Heart Clinic Reviewed March 2019 Pulmonary Atresia, Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries (PA/VSD/MAPCAs) Pulmonary atresia (PA), ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCAs) is a rare type of congenital heart defect, also referred to as Tetralogy of Fallot with PA/MAPCAs. Tetralogy of Fallot (TOF) is the most common cyanotic heart defect and occurs in 5-10% of all children with congenital heart disease. The classic description of TOF includes four cardiac abnormalities: overriding aorta, right ventricular hypertrophy (RVH), large perimembranous ventricular septal defect (VSD), and right ventricular outflow tract obstruction (RVOTO). About 20% of patients with TOF have PA at the infundibular or valvar level, which results in severe right ventricular outflow tract obstruction. PA means that the pulmonary valve is closed and not developed. When PA occurs, blood can not flow through the pulmonary arteries to the lungs. Instead, the child is dependent on a patent ductus arteriosus (PDA) or multiple systemic collateral vessels (MAPCAs) to deliver blood to the lungs for oxygenation. These MAPCAs usually arise from the de- scending aorta and subclavian arteries. Commonly, the pulmonary arteries are abnormal, with hypoplastic (small and underdeveloped) central and branch pulmonary arteries and/ or non-confluent central pulmonary arteries.
    [Show full text]
  • Track 5: Cardiology and the Imaging Revolution
    TRACK 5: CARDIOLOGY AND THE IMAGING REVOLUTION Volume 10 • Number 1 Abstract no: 1 Summer 2013 Real time 3-D echocardiographic characteristics of left ventricle and left atrium in normal children Bao Phung Tran Cong, Nii Masaki, Miyakoshi Chihiro, Yoshimoto Jun, Kato Atsuko, Ibuki Keichiro, Kim Sunghae, Mitsushita Norie, Tanaka Yasuhiko and Ono Yasuo Cardiac Department, Shizuoka Children’s Hospital, Shizuoka, Japan Background: The accurate assessment of left atrial (LA) and/or left ventricular (LV) volume and contractility is crucial for the management of patients with congenital heart disease. The real time 3-dimensional echocardiography (RT3-DE) is reported to show better correlation with magnetic resonance imaging (MRI) in estimating LV and LA volume than conventional 2-dimensional echocardiography (2-DE). On the other hand, the volume measurement in RT3-DE is also reported to be significantly smaller than those in MRI, necessitating the establishment of normal values of RT3-DE itself. Aim: To identify the normal values of LV and LA volume measured by RT3-DE in Japanese children. Methods: Sixty four normal school students (age: median 9.6 years; range (5.5 - 14.5); male 26, female 38) were enrolled in this study. End-diastolic and end- systolic LV and LA volumes were analysed using M-mode in short-axis view, 2-D biplane method, and RT3-DE. We used IE-33 (PHILIPS) with matrix probe X7 and X4. Off-line assessment to calculate LA and LV volume was done using QLAB 8.1 (Philips). Results: Forty nine children (age: median 9.1 years, range (6 - 14); male 21, female 28) had adequate RT3-DE data sets and were analysed.
    [Show full text]
  • Clinical Presentations of Critical Cardiac Defects in the Newborn: Decision Making and Initial Management
    Review article DOI: 10.3345/kjp.2010.53.6.669 Korean J Pediatr 2010;53(6):669-679 Clinical presentations of critical cardiac defects in the newborn: Decision making and initial management Jae Young Lee, M.D. The risk of mortality and morbidity of patients with congenital heart defects (CHDs) is highest during neonatal period and increases Department of Pediatrics, College of Medicine, the when diagnosis and proper management are delayed. Neonates Catholic University of Korea, Seoul, Korea with critical CHDs may present with severe cyanosis, respiratory distress, shock, or collapse, all of which are also frequent clinical presentations of various respiratory problems or sepsis in the newborn. Early diagnosis and stabilization and timely referral to a tertiary cardiac center are crucial to improve the outcomes in Received: 7 May 2010, Accepted: 17 May 2010 neonates with CHDs. In this review, the clinical presentation of Corresponding author: Jae Young Lee, M.D. critical and potentially life-threatening CHDs is discussed along with Department of Pediatrics, College of Medicine, the Catholic University of Korea, 505, Banpo-dong, Seocho-gu, Seoul 137- brief case reviews to help understand the hemodynamics of these 701, Korea defects and ensure proper decision-making in critically ill patients. Tel: +82-2258-6189, Fax: +82-2-537-4544 E-mail: [email protected] Key Words: Congenital heart defect, Ductal-dependent lesions, Copyright © 2010 by The Korean Pediatric Society Cyanosis, Shock Introduction in these critically ill patients. In this review, clinical presentations of potentially life-threatening CHDs in neonates were discussed Congenital heart defects (CHDs) are the most common birth along with brief case reviews to help pediatricians understand the defects with an incidence of approximately 6-8 in 1,000 live births, hemodynamics of these defects and to facilitate correct decision- accounting for 6-10% of all infant deaths and 20-40% of all infant making in these patients.
    [Show full text]
  • Growth, Development, and Quality of Life in Children with Congenital Heart Disease
    Scientific Foundation SPIROSKI, Skopje, Republic of Macedonia Open Access Macedonian Journal of Medical Sciences. 2020 Aug 20; 8(B):613-618. https://doi.org/10.3889/oamjms.2020.4047 eISSN: 1857-9655 Category: B - Clinical Sciences Section: Cardiology Growth, Development, and Quality of Life in Children with Congenital Heart Disease Sri Maya1*, Eka Gunawijaya2, N. P. Veny Kartika Yantie2, I. G. A. Trisna Windiani2 1Department of Child Health, Sondosia General Hospital, Bima, West Nusa Tenggara, Indonesia; 2Department of Child Health, Faculty of Medicine, Udayana University, Denpasar, Indonesia Abstract Edited by: Sinisa Stojanoski BACKGROUND: Despite the advances in medical and surgical care have improved the survival rates of children Citation: Maya S, Gunawijaya E, Yantie NPVK, Windiani IGAT. Growth, Development, and Quality of Life with congenital heart disease (CHD), they still remain risky for nutritional, cognitive problems, and quality of life. in Children with Congenital Heart Disease. Open Access Those impacts vary according to the severity of heart lesions and still manifested years after surgery. Maced J Med Sci. 2020 Aug 20; 8(B):613-618. https://doi.org/10.3889/oamjms.2020.4047 AIM: The objective of this study was to compare growth, development, and quality of life between cyanotic and Keywords: Congenital heart disease; Cognitive; Quality of life acyanotic CHD. *Correspondence: Sri Maya, Department of Child Health, Sondosia General Hospital, Bima, West Nusa Tenggara, METHODS: The study was performed on 52 patients aged 24–69 months old from June to January 2018 in Sanglah Indonesia. E-mail: [email protected] Pediatric Cardiology clinic used WHO Anthro software, The Mullen Scales of Early Learning and PedsQL Cardiac Received: 11-Nov-2019 Revised: 18-Jul-2020 module.
    [Show full text]
  • Dyspnea in Adult Patient with Corrected Tetralogy of Fallot
    Dyspnea in adult patient with corrected Tetralogy of Fallot F. Mut, M. Beretta Nuclear Medicine Service, Asociacion Española Montevideo, Uruguay Clinical history • Male 51 y.o. • Tetralogy of Fallot (TF) - acyanotic form. • Operated 16 years before • Dyspnea. • EKG: LV, RV hypertrophy, repolarization changes. • Echo: LVH, RVH with preserved systolic function of both ventricles, mild pulmonary valve stenosis. • MPS was indicated to rule out associated CAD. Clinical history • Exercise/rest MPS with 99mTc-MIBI was performed. • 90% of maximum predicted heart rate was achieved. • No ECG changes, no chest pain. • Dyspnea. Myocardial perfusion study STRESS REST STRESS REST STRESS REST Myocardial perfusion (enlarged stress images) The MPI results indicate: a) Normal study. b) LV & RV hypertrophy, anterior ischemia. c) LV & RV hypertrophy, transient dilation. d) LV & RV hypertrophy, inferior ischemia. The MPI results indicate: a) Normal study. b) LV & RV hypertrophy, anterior ischemia. c) LV & RV hypertrophy, transient dilation. d) LV & RV hypertrophy, inferior ischemia. • There is very marked LV & RV hypertrophy. • No perfusion defects are observed. • The LV cavity seems to be larger in the post-stress study as compared to rest, indicating transient ischemic dilation (TID). The 4 features typical of Tetralogy of Fallot include: a) Right ventricular outflow tract obstruction, ventricular septal defect, dextroposition of the aorta, and right ventricular hypertrophy. b) Right ventricular outflow tract obstruction, atrial septal defect, dextrocardia, and left ventricular hypertrophy. c) Mitral valve prolapse, ventricular septal defect, dextroposition of the aorta, and left ventricular hypertrophy. d) Right ventricular outflow tract obstruction, atrial septal defect, dextrocardia, and right hypertrophy. The 4 features typical of Tetralogy of Fallot include: a) Right ventricular outflow tract obstruction, ventricular septal defect, dextroposition of the aorta, and right ventricular hypertrophy.
    [Show full text]
  • Pattern of Congenital Heart Disease at Liaquat University Hospital Hyderabad
    VOL. 40 NO. 1—2 JANUARY - JUNE 2007 PAKISTAN HEART JOURNAL PATTERN OF CONGENITAL HEART DISEASE AT LIAQUAT UNIVERSITY HOSPITAL HYDERABAD YASMEEN MEMON*, REHANA MAJEED**, FEROZ MEMON*** ABSTRACT OBJECTIVE: To find out the frequency of various Congenital heart disease among affected children from birth to 12 years of age at Liaquat University Hospital Hyderabad. Setting: Study was conducted in pediatric Department of Liaquat University Hospital Hyderabad. Study design: This descriptive study was conducted over a period of one year from April 2006 to March 2007. Patients and Method: Eighty Children up to 12 year of age with clinical suspicion of congenital heart disease were evaluated for type of lesion, gender and age at presentation. They were subjected to chest X-ray, ECG and detailed echocardiography which confirmed the final diagnosis .The results was analyzed on SPSS window version 10. Results: Out of eighty cases fifty were male (62.5%) and thirty were female (37.5%).Fifty eight (72.5%) children were having cyanotic heart disease. Among Acyanotic lesion VSD was present in 42 patients (52.5%), ASD were 7 (8.75%) 6 have secondum types. PDA was present in 6 (7.50%) patients. A total of 7 (8.75%) patients had the TOF and 3 (3.75%) had TGA in association with VSD in one and ASD in 2 patients .Severe pulmonary stenosis was seen in 3 (3.75%) patients 2 were in association with ASD. Single ventricle and dextrocardia were seen in 3 (3.75%) patients each. Complex cardiac lesion was seen in 2 (2.5%) patients. Conclusion: Majority of Congenital heart disease in children at Tertiary care Hospital are acynotic , VSD is the commonest acynotic lesion while TOF is the commonest cyanotic lesion.Early detection of these defect is important for proper management and the gold standard for diagnosis of these defect is 2D echocardiography with Doppler examination.
    [Show full text]
  • RNC Cardiac Review
    RNC cardiac review Elizabeth Rex, MS, NNP - BC NCC Cardiac Content ♥ Congestive Heart Failure ♥ Transition to extrauterine life ♥ Hypertension ♥ PDA ♥ Shock ♥ CV Assessment BP CVP ♥ Cardiac Tamponade EKG Monitoring ♥ Anomalies (Cyanotic / Acyanotic) Lines AV Canal ♥ Cyanosis Coarctation of aorta Central / Peripheral HLHS Cardiac / Pulmonary Pulmonary stenosis/atresia TOF ♥ Arrhythmias TGA TAPVR Fetal Circulation 3 fetal shunts Ductus venosus Foreman ovale Ductus arteriosus Embryonic Development Cardiac septation : begins middle of the 4 th week and complete by end of 5 th week Defects arising from problems in septation : VSD, ASD, endocardial cushion defect (AV canal), malformation of tricuspid and mitral valves Great Vessel Development Happens simultaneously with septation Defects that occur with great vessel development: Truncus Arteriosus TOF Pulmunary and Aortic valve malformations Transposition DORV Cardiovascular Transition ♥ 10 min = PaO2 50 mm hg ♥ 1 hr = PaO2 62 mm hg ♥ 2 days PaO2 75 - 85 mm hg 24 hours after birth: ♥ Oxygen consumption triples ♥ Significant increase in cardiac output ♥ Left ventricle must remodel and hypertrophy Respiratory Assessment ♥ Normal Rate: 30 - 60, easy effort ♥ Increased WOB: tachypnea, GFR, gasping ♥ S aturations: Pre and post Heart Rate Assessment ♥ Normal rate 120 - 160 (may range 80 - 200) ♥ Normal sinus rhythm Bradycardia Underlying causes ♥ Vagal response ♥ Apnea ♥ Hypoxemia ♥ Asphyxia ♥ H ypotension ♥ Acidosis ♥ Digoxin toxicity ♥ Central line in right atrium Evaluate for shock ♥ HR
    [Show full text]
  • Synagis 2019-2020 Auth Guidelines.08.28.19
    Synagis® (Palivizumab) 2019-2020 Authorization Guideline Age in Months Respiratory Syncytial Virus (RSV) Prophylaxis: Conditions Covered at (Follows American Academy of Pediatrics Recommendations) RSV Season Onset† Maximum Monthly Synagis Doses per RSV Season = 5 at 15 mg/kg per dose 0 to <12 12 to <24 Preterm Birth 1. Infants born before 29 weeks, 0 days’ gestation. Chronic Lung Disease (CLD) of Prematurity 2. Infants with CLD of prematurity‡. 3. Infants with both of thefollowing: • CLD of prematurity‡; • Continued requirement for supplemental oxygen, chronic systemic corticosteroid therapy, or diuretic therapy within 6 months of RSV season onset. Congenital Heart Disease (CHD) 4. Infants with hemodynamically significant CHD - any of the following: • Acyanotic heart disease if receiving medication to control congestive heart failure and will require a cardiac surgical procedure or if continues to need medication for congestive heart failure despite surgery; • Acyanotic heart disease with moderate to severe pulmonary hypertension; • Cyanotic heart defect if RSV prophylaxis is recommended by a pediatric cardiologist. 5. Infants undergoing cardiac transplantation or cardio pulmonary bypass during the current RSV season, and • Infants who continue to require RSV prophylaxis after cardio -pulmonary bypass should receive an additional Synagis dose as soon as possible after the procedure (even if sooner than a month from the previous dose). Thereafter, doses should be administered monthly as scheduled. 6. Infants who undergo cardiac transplantation during the RSV season. Anatomic Pulmonary Abnormalities and Neuromuscular Disorders 7. Infants with an anatomic pulmonary anomaly or neuromuscular disorder that impairs the ability to clear secretions from the upper airway due to ineffective cough.
    [Show full text]
  • Four FATCO Syndrome Cases: Clinical, Autopsy and Placental
    Journal of Medical Biomedical and Applied Sciences Volume 4 issue 12 2016 page no. 20-25 ISSN: 2349-0748 Four FATCO syndrome cases: clinical, autopsy and placental features with literature review update Rosete Nogueira, MD1,2,3*; Joaquim Sá, MD4; Catarina Varela, MSc1; Graça Amorim, MD5; Francisco Valente, MD6; Purificação Tavares, MD, PhD4 1Pathology Laboratory, CGC Genetics, Porto, Portugal 2Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal 3ICVS/3B’s - PT Government Associate Laboratory, Braga/Guimarães, Portugal 4Clinical Department, CGC Genetics, Porto, Portugal; 5Prenatal Diagnosis Unit, Centro Hospitalar Tâmega Sousa (EPE), Penafiel, Portugal 6Prenatal Diagnosis Unit, Hospital of Vila Nova de Gaia/Espinho (CHVNG/E), Porto, Portugal ARTICLE INFO ABSTRACT Corresponding Author: Fibular Aplasia-Tibial Campomelia-Oligosyndactyly, or FATCO, is a rare Rosete Nogueira, MD syndromic condition reported in 18 cases so far, from which only 3 were Director of Pathology Laboratory, diagnosed at prenatal stages. In this study we report comprehensive clinical, CGC Genetics Rua Sá da Bandeira, placental and autopsy findings of four additional prenatal cases of FATCO, 706-1º, 4000-432 Porto, Portugal with the aim of further delineating this syndromic condition. Understanding this disorder at prenatal stages will allow for an earlier diagnosis through the Keywords: FATCO syndrome, identification of key features, thus permitting an adequate parental prenatal diagnosis, placenta, fetal counselling about the pregnancy development. autopsy ©2016, JMBM, All Right Reserved INTRODUCTION Limb malformations in neonates occurs in needed in order to have a comprehensive understanding approximately 1 in 1,000 [1]. Fibular aplasia is the most of this disorder prenatally, allowing parents to make an common malformation amongst long bone deficiency informed decision about their pregnancy development.
    [Show full text]