Aetna considers fetal echocardiograms, Doppler and color flow mapping medically necessary for any of the following conditions:
A mother with insulin dependent diabetes mellitus or systemic lupus erythematosus; or
As a screening study in families with a first-degree relative with a history of congenital heart disease; or
Fetal nuchal translucency measurement of 3.5 mm or greater in the first trimester; or
Following an abnormal or incomplete cardiac evaluation on an anatomic scan, 4-chamber study.
(Note: When the 4-chambered view is adequate and there are no other indications of a cardiac abnormality, a fetal echocardiogram is not considered medically necessary); or
For ductus arteriosus dependent lesions and/or with other known complex congenital heart disease; or
For pregnancies conceived by in vitro fertilization (IVF) or intra-cytoplasmic sperm injection (ICSI); or
In cases of single umbilical artery; or
In cases of suspected or known fetal chromosomal abnormalities; or
In suspected or documented fetal arrhythmia: to define the rhythm and its significance, to identify structural heart disease and cardiac function; or
In members with autoimmune antibodies associated with congenital cardiac anomalies [anti-Ro (SSA)/anti-La (SSB)]; or
In members with familial inherited disorders associated with congenital cardiac abnormalities (e.g., Marfan syndrome); or
In cases with monochorionic twins or multiple gestation and suspicion of twin-twin transfusion syndrome; or
In members with seizure disorders, even if they are not presently taking anti-seizure medication; or
In cases with non-immune fetal hydrops or unexplained severe polyhydramnios; or
When members' fetuses have been exposed to drugs known to increase the risk of congenital cardiac abnormalities including but not limited to:
Anti-seizure medications; or
Excessive alcohol intake; or
Paroxetine (Paxil); or
When other structural abnormalities are found on ultrasound; or
Aetna considers repeat studies of fetal echocardiograms medically necessary when the initial screening study indicates any of the following:
A ductus arteriosus dependent lesion; or
Structural heart disease with a suggestion of hemodynamic compromise; or
Tachycardia other than sinus tachycardia or heart block.
Aetna considers fetal echocardiograms experimental and investigational for all other indications (e.g., suspected cystic fibrosis, as a screening test in advanced maternal age) because their effectivenes for these indications has not been established.
Definition of fetal cardiac structures is currently possible at 10 to 12 weeks of gestation with the use of vaginal probes with high-resolution transducers. With current technologies, accurate segmental analysis of cardiac structures and blood flow across valves, shunts, and the ductus arteriosus is possible with a conventional transabdominal approach by 16 to 18 weeks of gestation.
Patients are referred for fetal echocardiography because of an abnormality of structure or rhythm noted on ultrasound examination or because the patient is in a high-risk group for fetal heart disease. Treatment of the patient is facilitated by the early recognition of the exact nature of the cardiac problem in the fetus. The correct diagnosis may be difficult because of fetal physiology, the effect on flow across defects and valves, inability to see the fetus for orientation reference, and inability to examine the fetus for clinical findings. For these reasons, fetal echocardiography should be performed only by trained fetal echocardiographers.
A single umbilical artery (SUA) is present in 0.2 % to 0.6 % of live births, occurring more frequently in twins and in small for gestational age and premature infants. In infants with SUA, there is an increased rate of chromosomal and other congenital anomalies. Studies have shown that 20 % to 30 % of neonates with SUA had major structural anomalies, frequently involving multiple organs (Palazzi and Brandt, 2009; Thummala et al, 1998). The most commonly affected organ is the heart. Single umbilical artery is an isolated finding in the remaining 70 % to 80 % of infants.
Conception by in vitro fertilization (IVF) or intra-cytoplasmic sperm injection (ICSI) has been associated with an increased incidence of fetal heart defects. A meta-analyses on the prevalence of birth defects in infants conceived following IVF and/or ICSI compared with spontaneously conceived infants reported a 30 % to 40 % increased risk of birth defects associated with IVF and/or ICSI (Hansen et al, 2005). Researchers have reported that infants conceived with the use of IVF and/or ICSI have a 2-to-4-fold increase of heart malformations compared with naturally conceived infants.
Kurinczuk and Bower (1997) examined the prevalence of birth defects on 420 liveborn infants who were conceived after ICSI in Belgium compared with 100,454 liveborn infants in Western Australia delivered during the same period. Infants born after ICSI were twice as likely as Western Australian infants to have a major birth defect [odds ratio (OR) 2.03, 95 % confidence interval (CI): 1.40 to 2.93); p = 0.0002] and nearly 50 % more likely to have a minor defect (OR 1.49 (0.48 to 4.66); p = 0.49). Secondary data-led analyses found an excess of major cardiovascular defects (OR 3.99).
Koivurova et al (2002) evaluated the neonatal outcome and the prevalence of congenital malformations in children born after IVF in northern Finland in a population-based study with matched controls. Children born after IVF (n = 304) in 1990 to1995 were compared with controls (n = 569), representing the general population in proportion of multiple births, randomly chosen from the Finnish Medical Birth Register (FMBR) and matched for sex, year of birth, area of residence, parity, maternal age and social class. Plurality matched controls were randomly chosen from the FMBR and analyzed separately. Additionally, IVF singletons were compared with singleton controls. The prevalence of heart malformations was four-fold in the IVF population than in the controls representing the general population (OR 4.0, 95 % CI: 1.4 to 11.7).
Reefhuis et al (2009) analyzed data from the National Birth Defects Prevention Study, a population-based, multi-center, case-control study of birth defects. Included were mothers of fetuses or live-born infants with a major birth defect (case infants) and mothers who had live-born infants who did not have a major birth defect (control infants), delivered during the period October 1997 to December 2003. Mothers who reported IVF or ICSI use were compared with those who had unassisted conceptions. Among singleton births, IVF or ICSI use was associated with septal heart defects (adjusted odds ratio [aOR] = 2.1, 95 % CI: 1.1 to 4.0).
As fetal heart disease is typically associated with structural abnormalities and consequent aberrant blood flow through the heart, it is necessary to perform Doppler studies and color flow mapping when such abnormalities are detected with 2D fetal echocardiography.
The American College of Obstetricians and Gynecologists' Committee Opinion on the treatment with selective serotonin reuptake inhibitors during pregnancy (ACOG, 2006) noted that paroxetine use among pregnant women and women planning pregnancy should be avoided, if possible. Fetal echocardiography should be considered for women who were exposed to paroxetine in early pregnancy.
In a practice bulletin on screening for fetal chromosomal anomalies, ACOG (2007) has stated that patients who have a fetal nuchal translucency measurement of 3.5 mm or greater in the first trimester, despite a negative result on an aneuploidy screen, normal fetal chromosomes, or both, should be offered a targeted ultrasound examination, fetal echocardiogram, or both, because such fetuses are at a significant risk for non-chromosomal anomalies, including congenital heart defects, abdominal wall defects, diaphragmatic hernias, and genetic syndromes.
Twin-twin transfusion syndrome (TTTS) is a severe complication of monochorionic (1 placenta) twin pregnancies, characterized by the development of unbalanced chronic blood transfer from one twin, defined as donor twin, to the other, defined as recipient, through placental anastomoses. If left untreated, TTTS is associated with very high peri-natal mortality and morbidity rates; and fetuses who survive are at risk of severe cardiac, neurological, and developmental disorders.
The American Society of Echocardiography's guidelines and standards for performance of the fetal echocardiogram (Rychik et al, 2004) stated that multiple gestation and suspicion of TTTS is an indication of fetal echocardiography.
Bahtiyar et al (2007) noted that congenital heart defects (CHDs) affect approximately 0.5 % of all neonates. Recent literature points to a possible increase in the CHD prevalence among monochorionic/diamniotic (MC/DA) twin gestations. These researchers hypothesized that MC/DA twin pregnancy is a risk factor for CHD. A systematic review of all published English literature was conducted on MEDLINE (Ovid and PubMed) from January 2000 through April 2007 using the medical subject heading terms "congenital heart defect" and "monozygotic twins". Four observational studies were included in the final analysis. Published historical data were used for the population background risk of CHD. Relative risk (RR) estimates with 95 % confidence intervals (CIs) were calculated by fixed and random effect models. These investigators included a total of 40 fetuses with CHDs among 830 fetuses from MC/DA twin gestations. Compared with the population, CHDs were significantly more prevalent in MC/DA twins regardless of the presence of TTTS (RR, 9.18; 95 % CI: 5.51 to 15.29; p < 0.001). Monochorionic/diamniotic twin gestations affected by TTTS were more likely to be complicated by CHDs than those that did not have TTTS (RR, 2.78; 95 % CI: 1.03 to 7.52; p = 0.04). Ventricular septal defects were the most frequent heart defects. Pulmonary stenosis and atrial septal defects were significantly more prevalent in pregnancies complicated with TTTS. The authors concluded that MC/DA twin gestation appears to be a risk factor for CHDs. Conditions that lead to abnormal placentation may also contribute to abnormal heart development, especially in MC/DA twin pregnancies complicated with TTTS. Fetal echocardiography may be considered for all MC/DA twin gestations because ventricular septal defects and pulmonary stenosis are the most common defects.
The Royal College of Obstetricians and Gynaecologists' clinical practice guidelines on "Management of monochorionic twin pregnancy" (RCOG, 2008) stated that a fetal echocardiographic assessment should be considered in the assessment of severe TTTS.
Documentation Requirements for Fetal Echocardiography:
According to guidelines from the American Institute for Ultrasound in Medicine (AIUM), fetal echocardiography should include the following cardiac images:
Inferior vena cava;
Left ventricular outflow tract;
Right ventricular outflow tract;
Short-axis views (“low” for ventricles and “high” for outflow tracts);
Superior vena cava; and
Three-vessel and trachea view.
According to the 2010 AIUM's practice guideline on "Performance of Fetal Echocardiography", indications for fetal echocardiography are often based on a variety of parental and fetal risk factors for congenital heart disease. However, most cases are not associated with known risk factors. Common indications for a detailed scan of the fetal heart include but are not limited to:
Maternal Indications Associated with Congenital Heart Disease:
First-degree relative with congenital heart disease
Metabolic disease (e.g., diabetes mellitus and phenylketonuria)
Teratogen exposure (e.g., lithium and retinoids)
Abnormal cardiac screening examination
Abnormal heart rate or rhythm
Fetal chromosomal anomaly
Increased nuchal translucency
Unexplained severe polyhydramnios
This AIUM practice guideline was published in conjunction with the American College of Obstetricians and Gynecologists (ACOG) and the Society for Maternal-Fetal Medicine (SMFM). Furthermore, this practice guideline was endorsed by the American College of Radiology (ACR).
Congenital cardiovascular disorders complicating pregnancy, antepartum condition or complication
Other cardiovascular diseases complicating pregnancy, antepartum condition or complication
Hydrocephalic fetus causing disproportion, antepartum condition or complication
Central nervous system malformation in fetus affecting management of mother, antepartum condition or complication
Known or suspected chromosomal abnormality in fetus affecting management of mother, antepartum condition or complication
Hereditary disease in family possibly affecting fetus affecting management of mother, antepartum condition or complication
Suspected damage to fetus from viral disease in mother, antepartum condition or complication
Suspected damage to fetus from other disease affecting management of mother, antepartum condition or complication
Suspected damage to fetus from drugs affecting management of mother, antepartum condition or complication
Other known or suspected fetal abnormality, not elsewhere classified, antepartum condition or complication
Unspecified known or suspected fetal abnormality, not elsewhere classified, affecting management of mother, antepartum condition or complication
Fetal-maternal hemorrhage affecting management of mother, antepartum condition or complication
Rhesus isoimmunization affecting management of mother, antepartum condition or complication
Isoimmunization from other and unspecified blood-group incompatibility affecting management of mother, antepartum condition or complication
Polyhydramnios, antepartum condition or complication
Abnormality in fetal heart rate or rhythm, antepartum condition or complication
Absence or hypoplasia of umbilical artery
Pregnancy resulting from assisted reproductive technology
ICD-9 codes not covered for indications listed in the CPB (not all-inclusive):
648.80 - 648.84
Abnormal glucose tolerance complicating pregnancy
659.50 - 659.53
Elderly primigravida [screening]
659.60 - 659.63
Elderly multigravida [screening]
Special screening for endocrine, nutritional, metabolic and immunity disorders [screening for cystic fibrosis]
Other ICD-9 codes related to the CPB:
Other specified complications of pregnancy, antepartum condition or complication
Drug dependence complicating pregnancy, antepartum condition or complication
Mental disorders complicating pregnancy, antepartum condition or complication
Noxious influences affecting fetus via placenta or breast milk, antimetabolic agents [retinoids]
Noxious influences affecting fetus via placenta or breast milk, other [Lithium]
Other nonspecific abnormal findings on radiological and other examination of body structure
Abnormal finding on antenatal screening [cardiac]
Family history of other cardiovascular diseases [first degree relative with history of congenital heart disease]
Screening for malformation using ultrasonics [when reported alone - indicates routine screen without signs or symptoms]
Other specified antenatal screening [when reported alone - indicates routine screen without signs or symptoms]
Long-term (current) use of insulin
Long-term (current) use of other medications [Lithium]
The above policy is based on the following references:
Cheitlin MD, Alpert JS, Armstrong WF, et al. ACC/AHA Guidelines for the clinical application of echocardiography: Executive summary. A report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (Committee on Clinical Application of Echocardiography). Developed in collaboration with the American Society of Echocardiography. J Am Coll Cardiol. 1997;29(4):862-879.
Winsberg F. Echocardiography of the fetal and newborn heart. Invest Radiol. 1972;3:152-158.
Copel JA, Pilu G, Kiemman CS. Congenital heart disease and extracardiac anomalies: Associations and indications for fetal echocardiography. Am J Obstet Gynecol. 1986;541:1121-1132.
Kiemman CS, Donnerstein RY, DeVore OR. Fetal echocardiography for evaluation of in utero congestive cardiac failure: A technique for study of non-immune hydrops. N Engl J Med. 1982;306:568-575.
DeVore OR, Donnerstein RL, Klemman CS, et al. Fetal echocardiography. II. The diagnosis and significance of a pericardial effusion in the fetus using real-time-directed M-mode ultrasound. Am J Obstet Gynecol. 1982;144:693-700.
Nora JJ, Nora AH. The evolution of specific genetic and environmental counseling in congenital heart diseases. Circulation. 1978;57:205-213.
Cyr DR, Guntheroth WO, Mack LA, et al. A systematic approach to fetal echocardiography using real-time/A two-dimensional sonography. J Ultrasound Med. 1986;5(6):343-350.
Silverman NH, Golbus MS. Echocardiographic techniques for assessing normal and abnormal fetal cardiac anatomy. J Am Coll Cardiol. 1985;5(Suppl 1):20S-29S.
Sahn DJ. Resolution and display requirements for ultrasound/Doppler evaluation of the heart in children, infants and unborn human fetus. J Am Coll Cardiol. 1985;5(Suppl 1):12S-19S.
DeVore OR, Platt LD. The random measurement of the transverse diameter of the fetal heart: A potential source of error. J Ultrasound Med. 1985;4:335-341.
DeVore OR, Siassi B, Platt LD. Fetal echocardiography. IV. M-mode assessment of ventricular size and contractility during the second and third trimesters of pregnancy in the normal fetus. Am J Obstet Gynecol. 1984;150:981-988.
DeVore OR, Donnerstein RL, Kiemman CS, et al. Fetal echocardiography. I. Normal anatomy as determined by real-time-directed M-mode ultrasound. Am J Obstet Gynecol. 1982;144:249-260.
St. John Sutton MG, Oewitz MH, Shah B, et al. Quantitative assessment of growth and function of the cardiac chambers in the normal human fetus: A prospective longitudinal echocardiographic study. Circulation. 1984;69(4):645-654.
Azancot A, Caudell TP, Allen HD, et al. Analysis of ventricular shape by echocardiography in normal fetuses, newborns, and infants. Circulation. 1983;68:1201-1211.
Allan LD, Joseph MC, Boyd EG, et al. M-mode echocardiography in the developing human fetus. Br Heart J. 1982;47(6):573-583.
DeVore OR, Siassi B, Platt LD. Fetal echocardiography. V. M-mode measurements of the aortic root and aortic valve in second and third trimester normal human fetuses. Am J Obstet Gynecol. 1985;152:543-550.
Shime J, Gresser CD, Rakowski H. Quantitative two-dimensional echocardiographic assessment of fetal growth. Am J Obstet Gynecol. 1986;154:290-300.
Driggers RW, Spevak PJ, Crino JP, et al. Fetal anatomic and functional echocardiography: A 5-year review. J Ultrasound Med. 2003;22(1):45-51.
Simpson LL. Indications for fetal echocardiography from a tertiary-care obstetric sonography practice. J Clin Ultrasound. 2004;32(3):123-128.
Forbus GA, Atz AM, Shirali GS. Implications and limitations of an abnormal fetal echocardiogram. Am J Cardiol. 2004;94(5):688-689.
Cheitlin MD, Armstrong WF, Aurigemma GP, et al. ACC/AHA/ASE 2003 guideline update for the clinical application of echocardiography--summary article: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/ASE Committee to Update the 1997 Guidelines for the Clinical Application of Echocardiography). J Am Coll Cardiol. 2003;42(5):954-970.
McAuliffe FM, Trines J, Nield LE, et al. Early fetal echocardiography--a reliable prenatal diagnosis tool. Am J Obstet Gynecol. 2005;193(3 Pt 2):1253-1259.
Randall P, Brealey S, Hahn S, et al. Accuracy of fetal echocardiography in the routine detection of congenital heart disease among unselected and low risk populations: A systematic review. BJOG. 2005;112(1):24-30.
Friedman AH, Copel JA, Kleinman CS. Fetal echocardiography and fetal cardiology: Indications, diagnosis and management. Semin Perinatol. 1993;17(2):76-88.
American College of Obstetricians and Gynecologists (ACOG), Committee on Obstetric Practice. ACOG Committee Opinion No. 354: Treatment with selective serotonin reuptake inhibitors during pregnancy. Obstet Gynecol. 2006;108(6):1601-1603.
Johnson B, Simpson LL. Screening for congenital heart disease: A move toward earlier echocardiography. Am J Perinatol. 2007;24(8):449-456.
Yacobi S, Ornoy A. Is lithium a real teratogen? What can we conclude from the prospective versus retrospective studies? A review. Isr J Psychiatry Relat Sci. 2008;45(2):95-106.
Marques Carvalho SR, Mendes MC, Poli Neto OB, Berezowski AT. First trimester fetal echocardiography. Gynecol Obstet Invest. 2008;65(3):162-168.
Thummala MR, Raju TN, Langenberg P. Isolated single umbilical artery anomaly and the risk for congenital malformations: A meta-analysis. J Pediatr Surg. 1998;33(4):580-585.
Rinehart BK, Terrone DA, Taylor CW, et al. Single umbilical artery is associated with an increased incidence of structural and chromosomal anomalies and growth restriction. Am J Perinatol. 2000;17(5):229-232.
Budorick NE, Kelly TF, Dunn JA, Scioscia AL. The single umbilical artery in a high-risk patient population: What should be offered? J Ultrasound Med. 2001;20(6):619-627.
Li M, Wang W, Yang X, et al. Evaluation of referral indications for fetal echocardiography in Beijing. J Ultrasound Med. 2008;27(9):1291-1296.
Geipel A, Germer U, Welp T, et al. Prenatal diagnosis of single umbilical artery: Determination of the absent side, associated anomalies, Doppler findings and perinatal outcome. Ultrasound Obstet Gynecol. 2000;15(2):114-117.
Pierce BT, Dance VD, Wagner RK, et al. Perinatal outcome following fetal single umbilical artery diagnosis. J Matern Fetal Med. 2001;10(1):59-63.
Prucka S, Clemens M, Craven C, McPherson E. Single umbilical artery: What does it mean for the fetus? A case-control analysis of pathologically ascertained cases. Genet Med. 2004;6(1):54-57.
Lubusky M, Dhaifalah I, Prochazka M, et al. Single umbilical artery and its siding in the second trimester of pregnancy: Relation to chromosomal defects. Prenat Diagn. 2007;27(4):327-331.
Cristina MP, Ana G, Inés T, et al. Perinatal results following the prenatal ultrasound diagnosis of single umbilical artery. Acta Obstet Gynecol Scand. 2005;84(11):1068-1074.
Granese R, Coco C, Jeanty P. The value of single umbilical artery in the prediction of fetal aneuploidy: findings in 12,672 pregnant women. Ultrasound Q. 2007;23(2):117-121.
Palazzi DL, Brandt ML. Care of the umbilicus and management of umbilical disorders. UpToDate [online serial]. Waltham, MA: UpToDate; 2009.
Kurinczuk JJ, Bower C. Birth defects in infants conceived by intracytoplasmic sperm injection: An alternative interpretation. BMJ. 1997;315(7118):1260-1265.
Koivurova S, Hartikainen AL, Gissler M, et al. Neonatal outcome and congenital malformations in children born after in-vitro fertilization. Hum Reprod. 2002;17(5):1391-1398.
Hansen M, Kurinczuk JJ, Bower C, Webb S. The risk of major birth defects after intracytoplasmic sperm injection and in vitro fertilization. N Engl J Med. 2002;346(10):725-730.
Hansen M, Bower C, Milne E, et al. Assisted reproductive technologies and the risk of birth defects -- a systematic review. Hum Reprod. 2005;20(2):328-338.
Reefhuis J, Honein MA, Schieve LA, et al; National Birth Defects Prevention Study. Assisted reproductive technology and major structural birth defects in the United States. Hum Reprod. 2009;24(2):360-366.
Hirata T, Osuga Y, Fujimoto A, et al. Conjoined twins in a triplet pregnancy after intracytoplasmic sperm injection and blastocyst transfer: Case report and review of the literature. Fertil Steril. 2009;91(3):933.e9-e12.
Friedberg MK, Silverman NH. Changing indications for fetal echocardiography in a University Center population. Prenat Diagn. 2004;24(10):781-786.
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Rychik J, Ayres N, Cuneo B, et al. American Society of Echocardiography guidelines and standards for performance of the fetal echocardiogram. J Am Soc Echocardiogr. 2004;17(7):803-810.
Bahtiyar MO, Dulay AT, Weeks BP, et al. Prevalence of congenital heart defects in monochorionic/diamniotic twin gestations: A systematic literature review. J Ultrasound Med. 2007;26(11):1491-1498.
Royal College of Obstetricians and Gynaecologists (RCOG). Management of monochorionic twin pregnancy. London, UK: Royal College of Obstetricians and Gynaecologists (RCOG); December 2008.
Copyright Aetna Inc. All rights reserved. Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.