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AIUM Practice Guideline
for the Performance of
Fetal Echocardiography




                                                       ®




      © 2010 by the American Institute of Ultrasound in Medicine




                                                                   127
The American Institute of Ultrasound in Medicine (AIUM) is a multidiscipli-
      nary association dedicated to advancing the safe and effective use of
      ultrasound in medicine through professional and public education, research,
      development of guidelines, and accreditation. To promote this mission,
      the AIUM is pleased to publish in conjunction with the American College of
      Obstetricians and Gynecologists (ACOG) and the Society for Maternal-Fetal
      Medicine (SMFM) this AIUM Practice Guideline for the Performance of Fetal
      Echocardiography. Additionally, this guideline is endorsed by the American
      College of Radiology (ACR).


      The AIUM represents the entire range of clinical and basic science interests
      in medical diagnostic ultrasound, and, with hundreds of volunteers, this
      multidisciplinary organization has promoted the safe and effective use of
      ultrasound in clinical medicine for more than 50 years. This document and
      others like it will continue to advance this mission.


      Practice guidelines of the AIUM are intended to provide the medical ultra-
      sound community with guidelines for the performance and recording of
      high-quality ultrasound examinations. The guidelines reflect what the
      AIUM considers the minimum criteria for a complete examination in each
      area but are not intended to establish a legal standard of care. AIUM-
      accredited practices are expected to generally follow the guidelines with
      recognition that deviations from these guidelines will be needed in some
      cases, depending on patient needs and available equipment. Practices are
      encouraged to go beyond the guidelines to provide additional service and
      information as needed.


      The clinical aspects contained in specific sections of this practice guideline
      (Introduction, Indications, Specifications of the Examination, and Equipment
      Specifications) were developed collaboratively by the American Institute of
      Ultrasound in Medicine (AIUM), American College of Radiology (ACR),
      American College of Obstetricians and Gynecologists (ACOG), and Society
      for Maternal-Fetal Medicine (SMFM). Recommendations for physician require-
      ments, written request for the examination, documentation, and quality
      control vary among these organizations and are addressed by each separately.




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                                             ®
                                                     Laurel, MD 20707-5906 USA
                                                     800-638-5352 • 301-498-4100
                                                     www.aium.org


                               Cite this guideline as follows:
      American Institute of Ultrasound in Medicine. AIUM practice guideline for the
      performance of fetal echocardiography. J Ultrasound Med 2011; 30:127–136.

            Original copyright 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography




128                                                                                   J Ultrasound Med 2011; 30:127–136
I. Introduction                                                           Fetal Indications
Congenital heart disease is a leading cause of infant mor-                •    Abnormal cardiac screening examination;
bidity and mortality from birth defects, with an estimated                •    Abnormal heart rate or rhythm;
incidence of 6 per 1000 live births for moderate to severe
forms.1,2 Accurate prenatal diagnosis offers potential clini-             •    Fetal chromosomal anomaly;
cal benefit with regard to infant outcomes, especially in                 •    Extracardiac anomaly;
those cases that are likely to require prostaglandin infu-
sion to maintain patency of the ductus arteriosus.3–5 Fetal               •    Hydrops;
echocardiography is broadly defined as a detailed sono-                   •    Increased nuchal translucency;
graphic evaluation that is used to identify and characterize
                                                                          •    Monochorionic twins; and
fetal heart anomalies before delivery. This specialized
diagnostic procedure is an extension of the “basic” and                   •    Unexplained severe polyhydramnios.
“extended basic” fetal cardiac screening guidelines that
have been previously described for the 4-chamber view
and outflow tracts.6,7 It should be performed only when                IV. Written Request for the Examination
there is a valid medical reason, and the lowest possible
                                                                       The written or electronic request for an ultrasound
ultrasonic exposure settings should be used to gain the
                                                                       examination should provide sufficient information to
necessary diagnostic information. In some cases, addi-
                                                                       allow for the appropriate performance and interpretation
tional or specialized examinations such as the inclusion
                                                                       of the examination.
of color Doppler sonography may be necessary. While it
is not possible to detect every abnormality, adherence to              A request for the examination must be originated by a
the following guideline will maximize the probability of               physician or other appropriately licensed health care
detecting most cases of clinically significant congenital              provider or under their direction. The accompanying
heart disease.                                                         clinical information should be provided by a physician or
                                                                       other appropriate health care provider familiar with the
                                                                       patient’s clinical situation and should be consistent with
II. Qualifications and Responsibilities of                             relevant legal and local health care facility requirements.
    Personnel
See the AIUM Official Statement Training Guidelines
for Physicians Who Evaluate and Interpret Diagnostic                   V. Specifications of the Examination
Ultrasound Examinations and the AIUM Standards and                     The following section details recommended and optional
Guidelines for the Accreditation of Ultrasound Practices.              elements for fetal echocardiography.

                                                                           A. General Considerations
III. Indications                                                           Fetal echocardiography is commonly performed
Indications for fetal echocardiography are often based on a                between 18 and 22 weeks’ gestational age. Some
variety of parental and fetal risk factors for congenital heart            forms of congenital heart disease may even be rec-
disease.8,9 However, most cases are not associated with                    ognized during earlier stages of pregnancy.10
known risk factors. Common indications for a detailed                      Optimal views of the heart are usually obtained
scan of the fetal heart include but are not limited to:                    when the cardiac apex is directed toward the anteri-
                                                                           or maternal wall. Technical limitations (eg, maternal
  Maternal Indications                                                     obesity or prone fetal position) can make a detailed
                                                                           heart evaluation very difficult because of acoustic
  •   Autoimmune antibodies, anti-Ro (SSA)/anti-La (SSB);
                                                                           shadowing, especially during the third trimester.
  •   Familial inherited disorders (eg, Marfan syndrome);                  It may be necessary to examine the patient at a
                                                                           different time if the heart is poorly visualized. The
  •   First-degree relative with congenital heart disease;
                                                                           examiner can optimize sonographic images by
  •   In vitro fertilization;                                              appropriate adjustment of technical settings, such
  •   Metabolic disease (eg, diabetes mellitus and                         as acoustic focus, frequency selection, signal gain,
      phenylketonuria); and                                                image magnification, temporal resolution, harmon-
                                                                           ic imaging, and Doppler-related parameters (eg,
  •   Teratogen exposure (eg, retinoids and lithium).                      velocity scale, frequency wall filter, and frame rate).



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J Ultrasound Med 2011; 30:127–136                                                                                                129
B. Cardiac Imaging Parameters: Basic Approach                                •    Pulmonary veins;
    The fetal echocardiogram is a detailed evaluation of                         •    Foramen ovale;
    cardiac structure and function. This method typically
                                                                                 •    Atrioventricular valves;
    involves a sequential segmental analysis of 3 basic
    areas that include the atria, ventricles, and great                          •    Atrial and ventricular septa;
    arteries and their connections.11–13 A segmental
                                                                                 •    Aortic and pulmonary valves;
    analysis includes an assessment of the following
    connections and their relationships:                                         •    Ductus arteriosus; and
    •   Atrial arrangement (situs);                                              •    Aortic arch.
    •   Atrioventricular junction between the atria and
        ventricles;
                                                                                 E. M-Mode Echocardiography (Optional but
    •   Ventriculoarterial junction between the ventricle                           Recommended for Cardiac Rate or Rhythm
        and arterial outflow tracts.                                                Abnormalities)
    Each anatomic segment can be further evaluated for                           M-mode echocardiography displays moving struc-
    associated anomalies such as cardiac malposition,                            tures along a thin sampling line over time. The high
    atrial isomerism, aortic override, an atrial septal                          temporal resolution makes it useful for the assess-
    defect, a ventricular septal defect, myocardial hyper-                       ment of ventricular contractility. Cardiac rhythm
    trophy, abnormal systemic and pulmonary venous                               disturbances can be characterized by establishing
    connections, a restricted foramen ovale mechanism,                           an atrial rate, ventricular rate, and their relationship
    ventricular disproportion, coarctation, and abnormal                         to each other. Alternative approaches, based on
    development of mitral or tricuspid valves.                                   pulsed wave or tissue Doppler sonography, have also
                                                                                 been used for evaluating fetal heart dysrhythmias.24

    C. Gray Scale Imaging (Recommended)
                                                                                 F. Cardiac Biometry (Optional but Can Be
    Key scanning planes can provide useful diagnostic
                                                                                    Considered in the Presence of Structural
    information about the fetal heart (Figures 1–3).14–19
                                                                                    Anomalies)
    The following cardiac images should be obtained:
                                                                                 Normal ranges for fetal cardiac measurements have
                                                                                 been published as percentiles and z scores that are
    •   Four-chamber view;                                                       based on gestational age or fetal biometry. Individual
                                                                                 measurements can be determined from M-mode or
    •   Left ventricular outflow tract;
                                                                                 2-dimensional images, and they include the follow-
    •   Right ventricular outflow tract;                                         ing parameters22,25–31:
    •   Three-vessel and trachea view;                                           •    Aortic and pulmonary artery diameters at the
                                                                                      level of the valve annulus;
    •   Short-axis views (“low” for ventricles and
        “high” for outflow tracts);                                              •    Aortic arch and isthmus diameter measurements;
    •   Aortic arch;                                                             •    End-diastolic ventricular dimensions just inferior
                                                                                      to the atrioventricular valve leaflets; and
    •   Ductal arch;
                                                                                 •    Thickness of the ventricular free walls and
    •   Superior vena cava; and
                                                                                      interventricular septum just inferior to the
    •   Inferior vena cava.                                                           atrioventricular valves.
                                                                                 •    Additional measurements may be taken if
                                                                                      warranted, including:
    D. Doppler Sonography (Optional but
       Recommended for Suspected Cardiac                                         •    Systolic dimensions of the ventricles;
       Flow Abnormalities)
                                                                                 •    Transverse dimensions of the atria; and
    Spectral, continuous wave, color, and/or power
                                                                                 •    Diameter of branch pulmonary arteries.
    Doppler sonography can be used to evaluate the
    following structures for potential flow or rhythm
    disturbances20–23:

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130                                                                                                          J Ultrasound Med 2011; 30:127–136
G. Complementary Imaging Strategies (Optional)                     VIII. Quality Control and Improvement,
   Other adjunctive imaging modalities, such as 3- and                      Safety, Infection Control, and Patient
   4-dimensional sonography, have been used to evalu-                       Education
   ate anatomic defects and to quantify fetal hemody-                 Policies and procedures related to quality control,
   namic parameters, such as cardiac output. Doppler                  patient education, infection control, and safety should
   sonography and speckle-tracking technologies have                  be developed and implemented in accordance with the
   also been described for ventricular strain and                     AIUM Standards and Guidelines for the Accreditation of
   myocardial performance index measurements.32–39                    Ultrasound Practices.

                                                                      Equipment performance monitoring should be in
VI. Reporting and Documentation                                       accordance with the AIUM Standards and Guidelines
                                                                      for the Accreditation of Ultrasound Practices.
Adequate documentation is essential for high-quality
patient care. There should be a permanent record of the
fetal echocardiographic examination and its interpreta-
tion. Images of all appropriate areas, both normal and                IX. ALARA Principle
abnormal, should be recorded. Variations from normal                  The potential benefits and risks of each examination
size should be accompanied by measurements. Images                    should be considered. The ALARA (as low as reasonably
should be labeled with the patient identification, facility           achievable) principle should be observed when adjusting
identification, examination date, and side (right or left) of         controls that affect the acoustic output and by considering
the anatomic site imaged. An official interpretation (final           transducer dwell times. Further details on ALARA may be
report) of the diagnostic findings should be included in              found in the AIUM publication Medical Ultrasound Safety.
the patient’s medical record. Retention of the ultrasound
examination should be consistent both with clinical needs
and with relevant legal and local health care facility
requirements. Reporting should be in accordance with
                                                                      Acknowledgments
the AIUM Standard for Documentation of an Ultrasound                  This guideline was developed by a Fetal Echocardiography
Examination.40–42                                                     Task Force under the auspices of the AIUM Clinical
                                                                      Standards Committee (David M. Paushter, MD, Chair) in
                                                                      collaboration with the American College of Obstetricians
VII. Equipment Specifications                                         and Gynecologists (ACOG) and Society for Maternal-Fetal
                                                                      Medicine (SMFM) according to the process described in
A sonographic examination of the fetal heart should be                the AIUM Clinical Standards Committee Manual. The
conducted using a real-time scanner. Sector, curvilinear,             American College of Radiology (ACR) has endorsed this
and endovaginal transducers are used for this purpose.                document. Appreciation is particularly extended to Kathi
The transducer or scanner should be adjusted to operate               Keaton Borok, BS, RDMS, RDCS, for administrative assis-
at the highest clinically appropriate frequency, realizing            tance during the development of this document and to
that there is a trade-off between resolution and beam                 Victoria Webster, MA, RT(MR), CNMT, RDMS, for develop-
penetration. With modern equipment, fetal imaging                     ment of the illustrations.
studies performed from the anterior abdominal wall can
usually use frequencies of 3.5 MHz or higher, while scans
performed from the vagina should be performed using
frequencies of 5 MHz or higher. Acoustic shadowing and                Fetal Echocardiography Task Force
maternal body habitus may limit the ability of higher-
                                                                          Task Force Chair
frequency transducers to provide greater anatomic detail
for the fetal heart.                                                      Wesley Lee, MD

                                                                          AIUM
                                                                          Julia Drose, BA, RT, RDMS, RVT, RDCS
                                                                          Joseph Wax, MD




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                                        Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography                3




J Ultrasound Med 2011; 30:127–136                                                                                               131
ACOG                                                                           5.    Berning RA, Silverman NH, Villegas M, Sahn DJ, Martin GR,
      James D. Goldberg, MD                                                                Rice MJ. Reversed shunting across the ductus arteriosus or
                                                                                           atrial septum in utero heralds severe congenital heart dis-
      Isabelle A. Wilkins, MD                                                              ease. J Am Coll Cardiol 1996; 27:481–486.
                                                                                     6.    Lee W. Performance of the basic fetal cardiac ultrasound
      ACR                                                                                  examination [published erratum appears in J Ultrasound
      Carol Benson, MD                                                                     Med 1998; 17:796]. J Ultrasound Med 1998; 17:601–607.
      Mary C. Frates, MD                                                             7.    International Society of Ultrasound in Obstetrics and
                                                                                           Gynecology. Cardiac screening examination of the fetus:
                                                                                           guidelines for performing the “basic” and “extended basic”
      SMFM                                                                                 cardiac scan. Ultrasound Obstet Gynecol 2006; 27:107–
      Mary Norton, MD                                                                      113.
      Lynn Simpson, MD                                                               8.    Small M, Copel JA. Indications for fetal echocardiography.
                                                                                           Pediatr Cardiol 2004; 25:210–222.
      AIUM Clinical Standards Committee                                              9.    Olson CK, Kepper-Noreuil KM, Romitti PA, et al. In vitro
      David M. Paushter, MD, Chair                                                         fertilization is associated with an increase in major birth
                                                                                           defects. Fertil Steril 2005; 84:1308–1315.
      Leslie Scoutt, MD, Vice Chair
      Susan Ackerman, MD                                                             10. Lee W, Allan L, Carvalho JS, et al. ISUOG consensus state-
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      Mert O. Bahtiyar, MD
      Harris L. Cohen, MD                                                            11. Anderson RH, Becker AE, Freedom RM, et al. Sequential
                                                                                         segmental analysis of congenital heart disease. Pediatr
      Jude Crino, MD                                                                     Cardiol 1984; 5:281–287.
      William L. Diacon, MD, RDMS, RPVI
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      Judy Estroff, MD
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      Kimberly D. Gregory, MD, MPH                                                       1999; 9:430–444.
      Charlotte Henningsen, MS, RT, RDMS, RVT
                                                                                     13. Carvalho JS, Ho SY, Shinebourne EA. Sequential segmental
      Charles Hyde, MD                                                                   analysis in complex fetal cardiac abnormalities: a logical
      Christopher Moore, MD, RDMS, RDCS                                                  approach to diagnosis. Ultrasound Obstet Gynecol 2005;
      Olga Rasmussen, RDMS                                                               26:105–111.
      Carl Reading, MD                                                               14. Comstock CH. Cardiac axis and position. Obstet Gynecol
      Daniel Skupski, MD                                                                 1987; 70:255–259.
      Jay Smith, MD                                                                  15. Allan LD. A practical approach to fetal heart scanning. Semin
      Joseph Wax, MD                                                                     Perinatol 2000; 24:324–330.
                                                                                     16. Viñals F, Heredia F, Giuliano A. The role of the three vessels
                                                                                         and trachea view (3VT) in the diagnosis of congenital heart
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                                              Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography                               5




J Ultrasound Med 2011; 30:127–136                                                                                                                 133
Figure 1. Standardized transverse scanning planes for fetal echocardiography include an evaluation of the 4-chamber view (1), arterial outflow
tracts (2 and 3), and the 3-vessel and trachea view (4). Ao indicates descending aorta; Asc Ao, ascending aorta; LA, left atrium; LV, left ventricle;
PA, pulmonary artery; RA, right atrium; RV, right ventricle; and Tra, trachea.




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134                                                                                                            J Ultrasound Med 2011; 30:127–136
Figure 2. Sagittal views of the superior and inferior vena cava (1), aortic arch (2), and ductal arch (3). The scan angle between the ductal arch
and thoracic aorta ranges between 10° and 19° during pregnancy, as illustrated by the 4-chamber view diagram (Espinoza J, Gotsch F, Kusanovic
JP, et al. Changes in fetal cardiac geometry with gestation: implications for 3- and 4-dimensional fetal echocardiography. J Ultrasound Med 2007;
26:437–443). Ao or Desc Ao indicates descending aorta; Ao Root, aortic root; DA, ductus arteriosus; IVC, inferior vena cava; LA, left atrium; LV,
left ventricle; PV, pulmonary valve; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle; and SVC, superior vena cava.




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                                              Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography                       7




J Ultrasound Med 2011; 30:127–136                                                                                                            135
Figure 3. Low and high short-axis views of the fetal heart. Ao indicates aortic valve; DA, ductus arteriosus; LV, left ventricle; PA, pulmonary
artery; RA, right atrium; and RV, right ventricle.




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AIUM Practice Guideline for the Performance of Fetal Echocardiography

  • 1. AIUM Practice Guideline for the Performance of Fetal Echocardiography ® © 2010 by the American Institute of Ultrasound in Medicine 127
  • 2. The American Institute of Ultrasound in Medicine (AIUM) is a multidiscipli- nary association dedicated to advancing the safe and effective use of ultrasound in medicine through professional and public education, research, development of guidelines, and accreditation. To promote this mission, the AIUM is pleased to publish in conjunction with the American College of Obstetricians and Gynecologists (ACOG) and the Society for Maternal-Fetal Medicine (SMFM) this AIUM Practice Guideline for the Performance of Fetal Echocardiography. Additionally, this guideline is endorsed by the American College of Radiology (ACR). The AIUM represents the entire range of clinical and basic science interests in medical diagnostic ultrasound, and, with hundreds of volunteers, this multidisciplinary organization has promoted the safe and effective use of ultrasound in clinical medicine for more than 50 years. This document and others like it will continue to advance this mission. Practice guidelines of the AIUM are intended to provide the medical ultra- sound community with guidelines for the performance and recording of high-quality ultrasound examinations. The guidelines reflect what the AIUM considers the minimum criteria for a complete examination in each area but are not intended to establish a legal standard of care. AIUM- accredited practices are expected to generally follow the guidelines with recognition that deviations from these guidelines will be needed in some cases, depending on patient needs and available equipment. Practices are encouraged to go beyond the guidelines to provide additional service and information as needed. The clinical aspects contained in specific sections of this practice guideline (Introduction, Indications, Specifications of the Examination, and Equipment Specifications) were developed collaboratively by the American Institute of Ultrasound in Medicine (AIUM), American College of Radiology (ACR), American College of Obstetricians and Gynecologists (ACOG), and Society for Maternal-Fetal Medicine (SMFM). Recommendations for physician require- ments, written request for the examination, documentation, and quality control vary among these organizations and are addressed by each separately. 14750 Sweitzer Ln, Suite 100 ® Laurel, MD 20707-5906 USA 800-638-5352 • 301-498-4100 www.aium.org Cite this guideline as follows: American Institute of Ultrasound in Medicine. AIUM practice guideline for the performance of fetal echocardiography. J Ultrasound Med 2011; 30:127–136. Original copyright 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography 128 J Ultrasound Med 2011; 30:127–136
  • 3. I. Introduction Fetal Indications Congenital heart disease is a leading cause of infant mor- • Abnormal cardiac screening examination; bidity and mortality from birth defects, with an estimated • Abnormal heart rate or rhythm; incidence of 6 per 1000 live births for moderate to severe forms.1,2 Accurate prenatal diagnosis offers potential clini- • Fetal chromosomal anomaly; cal benefit with regard to infant outcomes, especially in • Extracardiac anomaly; those cases that are likely to require prostaglandin infu- sion to maintain patency of the ductus arteriosus.3–5 Fetal • Hydrops; echocardiography is broadly defined as a detailed sono- • Increased nuchal translucency; graphic evaluation that is used to identify and characterize • Monochorionic twins; and fetal heart anomalies before delivery. This specialized diagnostic procedure is an extension of the “basic” and • Unexplained severe polyhydramnios. “extended basic” fetal cardiac screening guidelines that have been previously described for the 4-chamber view and outflow tracts.6,7 It should be performed only when IV. Written Request for the Examination there is a valid medical reason, and the lowest possible The written or electronic request for an ultrasound ultrasonic exposure settings should be used to gain the examination should provide sufficient information to necessary diagnostic information. In some cases, addi- allow for the appropriate performance and interpretation tional or specialized examinations such as the inclusion of the examination. of color Doppler sonography may be necessary. While it is not possible to detect every abnormality, adherence to A request for the examination must be originated by a the following guideline will maximize the probability of physician or other appropriately licensed health care detecting most cases of clinically significant congenital provider or under their direction. The accompanying heart disease. clinical information should be provided by a physician or other appropriate health care provider familiar with the patient’s clinical situation and should be consistent with II. Qualifications and Responsibilities of relevant legal and local health care facility requirements. Personnel See the AIUM Official Statement Training Guidelines for Physicians Who Evaluate and Interpret Diagnostic V. Specifications of the Examination Ultrasound Examinations and the AIUM Standards and The following section details recommended and optional Guidelines for the Accreditation of Ultrasound Practices. elements for fetal echocardiography. A. General Considerations III. Indications Fetal echocardiography is commonly performed Indications for fetal echocardiography are often based on a between 18 and 22 weeks’ gestational age. Some variety of parental and fetal risk factors for congenital heart forms of congenital heart disease may even be rec- disease.8,9 However, most cases are not associated with ognized during earlier stages of pregnancy.10 known risk factors. Common indications for a detailed Optimal views of the heart are usually obtained scan of the fetal heart include but are not limited to: when the cardiac apex is directed toward the anteri- or maternal wall. Technical limitations (eg, maternal Maternal Indications obesity or prone fetal position) can make a detailed heart evaluation very difficult because of acoustic • Autoimmune antibodies, anti-Ro (SSA)/anti-La (SSB); shadowing, especially during the third trimester. • Familial inherited disorders (eg, Marfan syndrome); It may be necessary to examine the patient at a different time if the heart is poorly visualized. The • First-degree relative with congenital heart disease; examiner can optimize sonographic images by • In vitro fertilization; appropriate adjustment of technical settings, such • Metabolic disease (eg, diabetes mellitus and as acoustic focus, frequency selection, signal gain, phenylketonuria); and image magnification, temporal resolution, harmon- ic imaging, and Doppler-related parameters (eg, • Teratogen exposure (eg, retinoids and lithium). velocity scale, frequency wall filter, and frame rate). ® Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography 1 J Ultrasound Med 2011; 30:127–136 129
  • 4. B. Cardiac Imaging Parameters: Basic Approach • Pulmonary veins; The fetal echocardiogram is a detailed evaluation of • Foramen ovale; cardiac structure and function. This method typically • Atrioventricular valves; involves a sequential segmental analysis of 3 basic areas that include the atria, ventricles, and great • Atrial and ventricular septa; arteries and their connections.11–13 A segmental • Aortic and pulmonary valves; analysis includes an assessment of the following connections and their relationships: • Ductus arteriosus; and • Atrial arrangement (situs); • Aortic arch. • Atrioventricular junction between the atria and ventricles; E. M-Mode Echocardiography (Optional but • Ventriculoarterial junction between the ventricle Recommended for Cardiac Rate or Rhythm and arterial outflow tracts. Abnormalities) Each anatomic segment can be further evaluated for M-mode echocardiography displays moving struc- associated anomalies such as cardiac malposition, tures along a thin sampling line over time. The high atrial isomerism, aortic override, an atrial septal temporal resolution makes it useful for the assess- defect, a ventricular septal defect, myocardial hyper- ment of ventricular contractility. Cardiac rhythm trophy, abnormal systemic and pulmonary venous disturbances can be characterized by establishing connections, a restricted foramen ovale mechanism, an atrial rate, ventricular rate, and their relationship ventricular disproportion, coarctation, and abnormal to each other. Alternative approaches, based on development of mitral or tricuspid valves. pulsed wave or tissue Doppler sonography, have also been used for evaluating fetal heart dysrhythmias.24 C. Gray Scale Imaging (Recommended) F. Cardiac Biometry (Optional but Can Be Key scanning planes can provide useful diagnostic Considered in the Presence of Structural information about the fetal heart (Figures 1–3).14–19 Anomalies) The following cardiac images should be obtained: Normal ranges for fetal cardiac measurements have been published as percentiles and z scores that are • Four-chamber view; based on gestational age or fetal biometry. Individual measurements can be determined from M-mode or • Left ventricular outflow tract; 2-dimensional images, and they include the follow- • Right ventricular outflow tract; ing parameters22,25–31: • Three-vessel and trachea view; • Aortic and pulmonary artery diameters at the level of the valve annulus; • Short-axis views (“low” for ventricles and “high” for outflow tracts); • Aortic arch and isthmus diameter measurements; • Aortic arch; • End-diastolic ventricular dimensions just inferior to the atrioventricular valve leaflets; and • Ductal arch; • Thickness of the ventricular free walls and • Superior vena cava; and interventricular septum just inferior to the • Inferior vena cava. atrioventricular valves. • Additional measurements may be taken if warranted, including: D. Doppler Sonography (Optional but Recommended for Suspected Cardiac • Systolic dimensions of the ventricles; Flow Abnormalities) • Transverse dimensions of the atria; and Spectral, continuous wave, color, and/or power • Diameter of branch pulmonary arteries. Doppler sonography can be used to evaluate the following structures for potential flow or rhythm disturbances20–23: ® 2 Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography 130 J Ultrasound Med 2011; 30:127–136
  • 5. G. Complementary Imaging Strategies (Optional) VIII. Quality Control and Improvement, Other adjunctive imaging modalities, such as 3- and Safety, Infection Control, and Patient 4-dimensional sonography, have been used to evalu- Education ate anatomic defects and to quantify fetal hemody- Policies and procedures related to quality control, namic parameters, such as cardiac output. Doppler patient education, infection control, and safety should sonography and speckle-tracking technologies have be developed and implemented in accordance with the also been described for ventricular strain and AIUM Standards and Guidelines for the Accreditation of myocardial performance index measurements.32–39 Ultrasound Practices. Equipment performance monitoring should be in VI. Reporting and Documentation accordance with the AIUM Standards and Guidelines for the Accreditation of Ultrasound Practices. Adequate documentation is essential for high-quality patient care. There should be a permanent record of the fetal echocardiographic examination and its interpreta- tion. Images of all appropriate areas, both normal and IX. ALARA Principle abnormal, should be recorded. Variations from normal The potential benefits and risks of each examination size should be accompanied by measurements. Images should be considered. The ALARA (as low as reasonably should be labeled with the patient identification, facility achievable) principle should be observed when adjusting identification, examination date, and side (right or left) of controls that affect the acoustic output and by considering the anatomic site imaged. An official interpretation (final transducer dwell times. Further details on ALARA may be report) of the diagnostic findings should be included in found in the AIUM publication Medical Ultrasound Safety. the patient’s medical record. Retention of the ultrasound examination should be consistent both with clinical needs and with relevant legal and local health care facility requirements. Reporting should be in accordance with Acknowledgments the AIUM Standard for Documentation of an Ultrasound This guideline was developed by a Fetal Echocardiography Examination.40–42 Task Force under the auspices of the AIUM Clinical Standards Committee (David M. Paushter, MD, Chair) in collaboration with the American College of Obstetricians VII. Equipment Specifications and Gynecologists (ACOG) and Society for Maternal-Fetal Medicine (SMFM) according to the process described in A sonographic examination of the fetal heart should be the AIUM Clinical Standards Committee Manual. The conducted using a real-time scanner. Sector, curvilinear, American College of Radiology (ACR) has endorsed this and endovaginal transducers are used for this purpose. document. Appreciation is particularly extended to Kathi The transducer or scanner should be adjusted to operate Keaton Borok, BS, RDMS, RDCS, for administrative assis- at the highest clinically appropriate frequency, realizing tance during the development of this document and to that there is a trade-off between resolution and beam Victoria Webster, MA, RT(MR), CNMT, RDMS, for develop- penetration. With modern equipment, fetal imaging ment of the illustrations. studies performed from the anterior abdominal wall can usually use frequencies of 3.5 MHz or higher, while scans performed from the vagina should be performed using frequencies of 5 MHz or higher. Acoustic shadowing and Fetal Echocardiography Task Force maternal body habitus may limit the ability of higher- Task Force Chair frequency transducers to provide greater anatomic detail for the fetal heart. Wesley Lee, MD AIUM Julia Drose, BA, RT, RDMS, RVT, RDCS Joseph Wax, MD ® Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography 3 J Ultrasound Med 2011; 30:127–136 131
  • 6. ACOG 5. Berning RA, Silverman NH, Villegas M, Sahn DJ, Martin GR, James D. Goldberg, MD Rice MJ. Reversed shunting across the ductus arteriosus or atrial septum in utero heralds severe congenital heart dis- Isabelle A. Wilkins, MD ease. J Am Coll Cardiol 1996; 27:481–486. 6. Lee W. Performance of the basic fetal cardiac ultrasound ACR examination [published erratum appears in J Ultrasound Carol Benson, MD Med 1998; 17:796]. J Ultrasound Med 1998; 17:601–607. Mary C. Frates, MD 7. International Society of Ultrasound in Obstetrics and Gynecology. Cardiac screening examination of the fetus: guidelines for performing the “basic” and “extended basic” SMFM cardiac scan. Ultrasound Obstet Gynecol 2006; 27:107– Mary Norton, MD 113. Lynn Simpson, MD 8. Small M, Copel JA. Indications for fetal echocardiography. Pediatr Cardiol 2004; 25:210–222. AIUM Clinical Standards Committee 9. Olson CK, Kepper-Noreuil KM, Romitti PA, et al. In vitro David M. Paushter, MD, Chair fertilization is associated with an increase in major birth defects. Fertil Steril 2005; 84:1308–1315. Leslie Scoutt, MD, Vice Chair Susan Ackerman, MD 10. Lee W, Allan L, Carvalho JS, et al. ISUOG consensus state- Lisa Allen, BS, RDMS, RDCS, RVT ment: what constitutes a fetal echocardiogram? Ultrasound Obstet Gynecol 2008; 32:239–242. Mert O. Bahtiyar, MD Harris L. Cohen, MD 11. Anderson RH, Becker AE, Freedom RM, et al. Sequential segmental analysis of congenital heart disease. Pediatr Jude Crino, MD Cardiol 1984; 5:281–287. William L. Diacon, MD, RDMS, RPVI 12. Yoo SJ, Lee YH, Cho KS, Kim DY. Sequential segmental Judy Estroff, MD approach to fetal congenital heart disease. Cardiol Young Kimberly D. Gregory, MD, MPH 1999; 9:430–444. Charlotte Henningsen, MS, RT, RDMS, RVT 13. Carvalho JS, Ho SY, Shinebourne EA. Sequential segmental Charles Hyde, MD analysis in complex fetal cardiac abnormalities: a logical Christopher Moore, MD, RDMS, RDCS approach to diagnosis. Ultrasound Obstet Gynecol 2005; Olga Rasmussen, RDMS 26:105–111. Carl Reading, MD 14. Comstock CH. Cardiac axis and position. Obstet Gynecol Daniel Skupski, MD 1987; 70:255–259. Jay Smith, MD 15. Allan LD. A practical approach to fetal heart scanning. Semin Joseph Wax, MD Perinatol 2000; 24:324–330. 16. Viñals F, Heredia F, Giuliano A. The role of the three vessels and trachea view (3VT) in the diagnosis of congenital heart References defects. Ultrasound Obstet Gynecol 2003; 22:358–367. 1. Hoffman JIE, Kaplan S. The incidence of congenital heart 17. Yagel S, Arbel R, Anteby EY, Raveh D, Achiron R. The three disease. J Am Coll Cardiol 2002; 39:1890–1900. vessels and trachea view (3VT) in fetal cardiac scanning. 2. Massaro AN, El-Dib M, Glass P, Aly H. Factors associated with Ultrasound Obstet Gynecol 2002; 20:340–345. adverse neurodevelopmental outcomes in infants with 18. Del Bianco A, Russo S, Lacerenza N, et al. Four-chamber view congenital heart disease. Brain Dev 2008; 30:437–446. plus three-vessel and trachea view for a complete evaluation 3. Bonnet D, Coltri A, Butera G, et al. Detection of transposi- of the fetal heart during the second trimester [published tion of the great arteries in fetuses reduces neonatal erratum appears in J Perinat Med 2006; 34:509]. J Perinat morbidity and mortality. Circulation 1999; 99:916–918. Med 2006; 34:309–312. 4. Tworetzky W, McElhinney DB, Reddy VM, Brook MM, 19. Pascal CJ, Huggon I, Sharland GK, Simpson JM. An echo- Hanley FL, Silverman NH. Improved surgical outcome after cardiographic study of diagnostic accuracy, prediction of fetal diagnosis of hypoplastic left heart syndrome. surgical approach, and outcome for fetuses diagnosed with Circulation 2001; 103:1269–1273. discordant ventriculo-arterial connections. Cardiol Young 2007; 17:528–534. ® 4 Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography 132 J Ultrasound Med 2011; 30:127–136
  • 7. 20. Chiba Y, Kanzaki T, Kobayashi H, Murakami M, Yutani C. 34. Molina FS, Faro C, Sotiriadis A, Daklis T, Nicolaides KH. Heart Evaluation of fetal structural heart disease using color flow stroke volume and cardiac output by four-dimensional ultra- mapping. Ultrasound Med Biol 1990; 16:221–229. sound in normal fetuses. Ultrasound Obstet Gynecol 2008; 32:181–187. 21. DeVore GR, Horenstein J, Siassi B, Platt LD. Fetal echocardio- graphy, VII: Doppler color flow mapping—a new technique 35. Valsky DV, Hamani Y, Verstandig A, Yagel S. Fetal cardiac for the diagnosis of congenital heart disease. Obstet ventricle volumetry in the second half of gestation assessed Gynecol 1987; 156:1054–1064. by 4D ultrasound using STIC combined with inversion mode. Ultrasound Obstet Gynecol 2007; 29:352–355. 22. DeVore GR. Assessing fetal cardiac ventricular function. Semin Fetal Neonat Med 2005; 10:515–541. 36. Larsen LU, Petersen OB, Norrild K, Sorensen K, Uldbjerg N, Sloth E. Strain rate derived for color Doppler myocardial 23. Chintala K, Tian Z, Du W, Donaghue D, Rychik J. Fetal imaging for assessment of fetal cardiac function. Ultrasound pulmonary venous Doppler patterns in hypoplastic left Obstet Gynecol 2006; 27:210–213. heart syndrome: relationship to atrial septal restriction. Heart 2008; 94:1446–1449. 37. DiSalvo G, Russo MG, Paladini D, et al. Quantification of regional left and right ventricular longitudinal function in 75 24. Api O, Carvalho JS. Fetal dysrhythmias. Best Pract Res Clin normal fetuses using ultrasound-based strain rate and strain Obstet Gynaecol 2008; 22:31–48. imaging. Ultrasound Med Biol 2005; 31:1159–1162. 25. Allen LD, Joseph MC, Boyd EG, Campbell S, Tynan M. 38. Espinoza J, Gonçalves LF, Lee W, Mazor M, Romero R. M-mode echocardiography in the developing human fetus. A novel method to improve prenatal diagnosis of abnormal Br Heart J 1982; 47:573–583. systemic venous connections using three- and four- 26. Devore GR, Siassi B, Platt LD. Fetal echocardiography, IV: dimensional ultrasonography and “inversion mode.” M-mode assessment of ventricular size and contractility dur- Ultrasound Obstet Gynecol 2005; 25:428–434. ing the second and third trimesters of pregnancy in the nor- 39. Falkensammer CB, Paul J, Huhta JC. Fetal congestive heart mal fetus. Am J Obstet Gynecol 1984; 150:981–988. failure: correlation of Tei index and cardiovascular score. 27. Cartier MS, Davidoff A, Warneke LA, et al. The normal J Perinat Med 2001; 29:390–398. diameter of the fetal aorta and pulmonary artery: echo- 40. American College of Radiology. ACR Practice Guideline for cardiographic evaluation in utero. AJR Am J Roentgenol Communication of Diagnostic Imaging Findings. Reston, VA: 1987; 149:1003–1007. American College of Radiology; 2005. 28. Sharland GK, Allan LD. Normal fetal cardiac measurements 41. American College of Radiology. ACR Practice Guideline for derived by cross-sectional echocardiography. Ultrasound Performing and Interpreting Diagnostic Ultrasound Obstet Gynecol 1992; 2:175–181. Examinations. Reston, VA: American College of Radiology; 29. Tan J, Silverman NH, Hoffman JIE, Villegas M, Schmidt KG. 2006. Cardiac dimensions determined by cross-sectional echo- 42. American Institute of Ultrasound in Medicine. AIUM practice cardiography in the normal human fetus from 18 weeks guideline for documentation of an ultrasound examination. to term. Am J Cardiol 1992; 70:1459–1467. J Ultrasound Med 2009; 28:110–113. 30. Pasquini L, Mellander M, Seale A, et al. Z-scores of the fetal aortic isthmus and duct: an aid to assessing arch hypoplasia [published erratum appears in Ultrasound Obstet Gynecol 2007; 30:366]. Ultrasound Obstet Gynecol 2007; 29:628– 633. 31. Schneider C, McCrindle BW, Carvalho JS, Hornberger LK, McCarthy KP, Daubeney PE. Development of z-scores for fetal cardiac dimensions from echocardiography. Ultrasound Obstet Gynecol 2005; 26:599–605. 32. Gonçalves LF, Lee W, Chaiworapongsa T, et al. Four- dimensional ultrasonography of the fetal heart with spatiotemporal image correlation. Am J Obstet Gynecol 2003; 189:1792–1802. 33. DeVore GR, Falkensammer P, Sklansky MS, Platt LD. Spatio- temporal image correlation (STIC): new technology for evaluation of the fetal heart. Ultrasound Obstet Gynecol 2003; 22:380–387. ® Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography 5 J Ultrasound Med 2011; 30:127–136 133
  • 8. Figure 1. Standardized transverse scanning planes for fetal echocardiography include an evaluation of the 4-chamber view (1), arterial outflow tracts (2 and 3), and the 3-vessel and trachea view (4). Ao indicates descending aorta; Asc Ao, ascending aorta; LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle; and Tra, trachea. ® 6 Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography 134 J Ultrasound Med 2011; 30:127–136
  • 9. Figure 2. Sagittal views of the superior and inferior vena cava (1), aortic arch (2), and ductal arch (3). The scan angle between the ductal arch and thoracic aorta ranges between 10° and 19° during pregnancy, as illustrated by the 4-chamber view diagram (Espinoza J, Gotsch F, Kusanovic JP, et al. Changes in fetal cardiac geometry with gestation: implications for 3- and 4-dimensional fetal echocardiography. J Ultrasound Med 2007; 26:437–443). Ao or Desc Ao indicates descending aorta; Ao Root, aortic root; DA, ductus arteriosus; IVC, inferior vena cava; LA, left atrium; LV, left ventricle; PV, pulmonary valve; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle; and SVC, superior vena cava. ® Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography 7 J Ultrasound Med 2011; 30:127–136 135
  • 10. Figure 3. Low and high short-axis views of the fetal heart. Ao indicates aortic valve; DA, ductus arteriosus; LV, left ventricle; PA, pulmonary artery; RA, right atrium; and RV, right ventricle. ® 8 Effective February 5, 2010—AIUM PRACTICE GUIDELINES—Fetal Echocardiography 136 J Ultrasound Med 2011; 30:127–136