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Prenatal diagnosis of congenital heart disease

Prenatal diagnosis of congenital heart disease using four-dimensional spatio-temporal image correlation (STIC) telemedicine via an Internet link: a pilot study

F. Viñals 1 *, L. Mandujano 2, G. Vargas 3, A. Giuliano 1
1Centro AGB Ultrasonografía, Clinica Sanatorio Alemán, Concepción, Chile – 2Centro Medico El Bosque, Punta Arenas, Chile
3Hospital La Serena, La Serena, Chile

email: F. Viñals (fvinals55@hotmail.com)

*Correspondence to F. Viñals, Avda Sanhueza 55. 403 A, Concepción, Chile

Keywords
4D • congenital heart disease • prenatal diagnosis • spatio-temporal image correlation • STIC • telemedicine

Abstract

Objective
To assess whether the spatio-temporal image correlation (STIC) acquisition technique can be taught to a general obstetrician by e-mail; whether STIC volume datasets can be transmitted over the Internet; and whether STIC volume datasets analyzed offline at a remote setting can be used to confirm or exclude major cardiac defects (TELE-STIC).

Methods
This was a prospective study involving 50 pregnant women with gestational ages ranging between 20 and 36 weeks. These patients were selected by two general obstetricians (operators) working in geographically remote areas of Chile. Although both obstetricians were users of equipment capable of four-dimensional (4D) ultrasound with STIC, they lacked skill in the performance of fetal cardiac examination. A dedicated web disk was created to upload the acquired volume datasets using an Internet broadband connection. Offline analysis was performed by a single investigator experienced in fetal echocardiography (the administrator).

Results
A telemedicine link via the Internet was possible in all cases. Seventy-seven volume datasets were sent to the web server. A complete cardiac examination according to set criteria was achieved by the administrator in 86% of the cases scanned by one operator and 95% of the cases scanned by the other operator. Three patients had cardiac defects confirmed postnatally, two fetuses had extracardiac anomalies and one fetus had a suspected cardiac defect unconfirmed by second-opinion TELE-STIC. There were two isolated major congenital heart defects. Both patients were given advice by e-mail and teleconference using a web camera about the likely outcome and benefits of scheduling in utero transport to a tertiary care center.

Conclusions
STIC volumes can be obtained by operators inexperienced in fetal echocardiography, transmitted via the Internet, and their analysis enables recognition of most of the structures and views necessary to assess fetal cardiac anatomy. The preliminary use of TELE-STIC allowed us to demonstrate that some intracardiac anomalies can be ruled out and others confirmed, allowing perinatal management to be tailored accordingly.

Ultrasound in Obstetrics and Gynecology Volume 25, Issue 1

Copyright © 2004 ISUOG. Published by John Wiley & Sons, Ltd.

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Double-Orifice Mitral Valve

Double-Orifice Mitral Valve with Intact Atrioventricular Septum: An
Echocardiographic Study With Anatomic and Functional Considerations
Bibhuti B. Das, MD, Linda B. Pauliks, MD, Ole A. Knudson, Jr, RDCS, Scott Kirby, RDCS,
Kak-Chen Chan, MD, Lilliam Valdes-Cruz, MD, and Raul O. Cayre, MD,
Denver, Colorado; Boston, Massachusetts; and Corrientes, Argentina

We identified 18 patients with double-orifice mitral
valve (DOMV) and intact atrioventricular (AV) septum
out of 40,179 echocardiographic studies performed
between 1997 and 2002 at Children’s Hospital,
Denver, CO. In this study we describe (1) the
anatomic characteristics of the DOMV in the absence
of AV septal defect, (2) the function of the mitral
valve by spectral and color Doppler flow mapping,
and (3) associated lesions. The topographic location
of the orifices in the leaflets suggests possible embryologic
mechanisms of DOMV. In this series,
DOMV was most commonly associated with leftsided
obstructive lesions (in 39% of patients). Spectral
and color Doppler interrogation demonstrated a
normal flow profile in most cases; only 2 patients
had significant mitral regurgitation or stenosis.
Therefore, due to the uncertain natural history of
this lesion and the potential need for endocarditis
prophylaxis, careful imaging of the mitral valve is
recommended, particularly in the presence of leftsided
obstructive lesions.

(J Am Soc Echocardiogr
2005;18:231-6.)

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Double inlet left ventricle

Original Articles
Anatomoechocardiographic correlation double inlet left ventricle

Luis Muñoz-Castellaños, MD – Nilda Espinola-Zavaleta, MD, PhD –
Candace Keirns, MD

Abstract

Double inlet left ventricle (LV) is a type of atrioventricular connection in which the morphologically LV receives more than 50% of the atrioventricular valves when they are separate, or more than 75% of a common atrioventricular valve. The aim of this study was to establish an anatomoechocardiographic correlation between the morphologic features of equivalent anatomic specimens and the echocardiographic images of patients to provide a means of interpreting the image correctly and a more precise diagnosis of the cardiac defect. Echocardiography was used to study 18 patients with LV double inlet who were seen in a congenital heart disease clinic. The morphology of 17 hearts with this malformation from the department of embryology was analyzed to compare the anatomic features with their echocardiographic images. Echocardiography proved to be a noninvasive diagnostic tool that allowed characterization of anatomic and functional aspects of double inlet LV.
Anatomoechocardiographic correlation double inlet left ventricle

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SYNCOPE


Available online
Task Force Report
Guidelines on management (diagnosis and treatment)
of syncope*

Task Force on Syncope, European Society of Cardiology†: M. Brignole (Chairman),
P. Alboni, D. Benditt, L. Bergfeldt, J. J. Blanc, P. E. Bloch Thomsen, J. G. van Dijk,
A. Fitzpatrick, S. Hohnloser, J. Janousek, W. Kapoor, R. A. Kenny, P. Kulakowski,
A. Moya, A. Raviele, R. Sutton, G. Theodorakis and W. Wieling


Table of contents

Preamble – Scope of the document 1256 – Method 1257

  • Part 1. Classification, epidemiology and prognosis
    Definition 1258
    Brief overview of pathophysiology of syncope 1258
    Classification 1259
    Epidemiological considerations 1259
    Prognostic stratification: identification of factors
    predictive of adverse outcome 1260

  • Part 2. Diagnosis
    Strategy of evaluation (flow chart) 1262
    Initial evaluation (history, physical examination,
    baseline electrocardiogram) 1264
    Echocardiogram 1266
    Carotid sinus massage 1266
    Tilt testing 1268
    Electrocardiographic monitoring (non-invasive
    and invasive) 1271
    Electrophysiological testing 1273
    ATP test 1277
    Ventricular signal-averaged electrocardiogram 1278
    Exercise testing 1278
    Cardiac catheterization and angiography 1279
    Neurological and psychiatric evaluation 1279
    Diagnostic yield and prevalence of causes
    of syncope 1282

  • Part 3. Treatment
    General principles 1282
    Neurally-mediated reflex syncopal syndromes 1283
    Orthostatic hypotension 1285
    Cardiac arrhythmias as primary cause 1286
    Structural cardiac or cardiopulmonary disease 1289
    Vascular steal syndromes 1289
    Metabolic 1290

  • Part 4. Special issues in evaluating patients with syncope
    Need for hospitalization 1290
    Syncope in the older adult 1290
    Syncope in paediatric patients 1292
    Driving and syncope 1293
    Glossary of uncertain terms 1293
    Preamble
    Scope of the document
    The purpose of this document is to provide specific
    recommendations on the diagnostic evaluation and
    management of syncope. The document is divided into
    four parts: (1) classification, epidemiology and prognosis;
    (2) diagnosis; (3) treatment; and (4) special issues
    in evaluating patients with syncope. Each part reviews
    background information and summarizes the relevant
    literature. The details of pathophysiology and mechanisms
    of various aetiologies were considered to lie
    outside the scope of this document. Although the document
    encompasses many of the important aspects of
    syncope, the panel recommendations focused on the
    following main questions:
    1. What are the diagnostic criteria for causes of
    syncope?
    2. What is the preferred approach to the diagnostic
    work-up in various subgroups of patients with
    syncope?
    3. How should patients with syncope be risk stratified?
    Correspondence: Michele Brignole, MD, FESC, Department of
    Cardiology and Arrhythmologic Centre, Ospedali Riuniti, 16033
    Lavagna, Italy.
    *This document has been reviewed by members of the Committee
    for Practice Guidelines (formerly Committee for Scientific and
    Clinical Initiatives) and by the members of the Board of the
    European Society of Cardiology (see Appendix 1), who approved
    the document on 8 March 2001. The full text of this document is
    available on the website of the European Society of Cardiology in
    the section ‘Scientific Information’, Guidelines.
    †For affiliations of Task Force members see Appendix 2.
    0195-668X/01/221256+51 $35.00/0  2001 The European Society of Cardiology
    4. When should patients with syncope be hospitalized?
    5. Which treatments are likely to be effective in preventing
    syncopal recurrences?
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    Fetal Echocardiography



    FETAL ECHOCARDIOGRAPHY

    Salvatore Gerboni M.D.
    University’ “G.d’Annunzio” – Chieti


    Introduction

      Fetal echocardiography is up to date the most valuable tool for the diagnosis of fetal heart diseases. The specificity is about 99 % and the sensibility is over 80 % depending from the experitise of the examiner. The examen is completely non invasive and harmless for the fetus and is the only tool that allows a complete cardiac structure and function evaluation and monitoring during fetal lifeFetal Ultrasound for Screening Examination.) It allows, also the studies of cardiac arrhithmias which are now managible with transplacentar therapy.

      The most frequent asked questions, from expectant mothers during obstretical consultations , are :

      Who must do Fetal ecocardiography
      When must be done Fetal ecocardiography
      Why must be done Fetal ecocardiography
      Where must be done Fetal ecocardiography

      must do the second level of Fetal Echocardiography

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    Internet Regitry CHD



    Internet Registry on CHD


    logo registry

    The incidence of congenital heart disease is about 8 for 1000 live births.
    We propose a register for continuous monitoring of congenital heart diseases from fetus to young adult in the world through Internet W.W.W.This register have opened since 03/30/1996 a new perspective study to evaluate the incidence and the spectrum of congenital heart diseases diagnosed either during fetal or postnatal life. The data entry was separated for Fetal and Neonatal CHD.
    The register is the most effective way to quickly compile and provides up to date informations on participating centers, summary and statistical data in DATA ENTRY , INSTITUTIONS and RESULTS pages and you will may send questions, suggestions and/or comments to improve this WWW service.

    We are very grateful to all Institutions, Pediatric Cardiologists, Pediatric Cardiac Surgeons and Anybody who are devoted in diagnosis of congenital malformations, that have the pleasure in adding theirs data in this multi-institutional, cooperative endeavour.
    All data collected will be presented up to date in these pages.


    Fetal Istitutions Fetal data entry Fetal results
    Neonatal Istitutions Neonatal data entry Neonatal results
    Send comments Fetal and Neonatal Total Results


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    Copyright © 1996 S.Gerboni M.D.

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    Fetal Arrhythmies



    fetal arrhythmies<br />


    Management of Fetal Arrhythmies

          The persistents major ipo or ipercinetic arrhythmies could constitute
          a condition of "cardiological fetal emergency" and if
          not in relief and promptly takes care of could conduct the fetus to heart
          failure and intrauterine death .The worse prognosis when the arrhythmy
          joins congenital heart disease (CHD).

          The most common form of ipercinetic arrhythmies is the supraventricular
          paroxysmal form that in the 10% of the cases join to structural CHD. The
          form that imposes a medical treatment the incessant one that it join to
          heart failure and/or CHD. Less frequent the atrial flutter or fibrillation
          or ventricular tachicardia.

          Between the ipocinetic arrhythmies the most remarkable is the complete
          a-v block whose prognosis tied to the ventricular frequency . When the
          ventricular frequency is < to 50 p.m. sever heart failure is the rule
          and having tried a trans-abdominal pacing without positive effect. This
          therapeutic approach would be desirable but for a prolonged stimulation
          the risk of infections fetal is very elevated . The literature brings again
          cases sporadic treatises without success with beta stimulating agent (
          isoproterenol,ritrodin etc)

          The most commonly drugs used are listed in the following chart.

          Transplacental Treatment of Fetal Arrhythmies
          Drugs ose Maintenance dose
          Digoxin g. os0.5 – 2 mg. e.v. 0.25 – 1 mg/die os
          Propranolol mg e.v. ( 0.04 mg/kg ) 80 – 160 mg/die os
          Verapamil e.v. 240 – 360 mg/die os
          Procainamid e.v. 3 – 4 gr/die os
          Quinidine os 1-2 gr/die os
          Flecainid e.v. 300 mg/die os
          Amiodarone os 600 mg/die os
          Betamimetics **) without effects.

          **) Fetal heart block may be treated with a loading test dose of Salbutamol e.v. 80 mg/L dextrose 4% solution starting
          with 4 micrograms/min and increased to 64 micrograms/min during the trial and
          followed by Salbutamol os 8 mg twice a day until delivery.(Groves and coll.Circ.92:3394:1995)

          In heart block with maternal anti-Ro and/or anti La proteins autoantibodies
          has been used Dexamethasone to the mother dosing 4 mg os once a day mantained until delivery,
          (Copel et al.:Am J obstet Gynecol 1384:173:1995)


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