prior authorization review panel mco policy …...b. fetal surveillance (e.g., congenital heart...

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(https://www.aetna.com/) Fetal Echocardiography and Magnetocardiography Clinical Policy Bulletins Medical Clinical Policy Bulletins Number: 0106 *Please see amendment for Pennsylvania Medicaid at the end of this CPB. I. Aetna considers fetal echocardiograms, Doppler and color ow mapping medically necessary after 12 weeks gestation for any of the following conditions: A. A mother with insulin dependent diabetes mellitus or systemic lupus erythematosus; or B. As a screening study in families with a first-degree relative of a fetus with congenital heart disease; or C. Fetal nuchal translucency measurement of 3.5 mm or greater in the rst trimester; or D. 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 E. For ductus arteriosus dependent lesions and/or with other known complex congenital heart disease; or F. For pregnancies conceived by in vitro fertilization (IVF) or intra-cytoplasmic sperm injection (ICSI); or G. In cases of persistent right umbilical vein; or H. In cases of single umbilical artery; or Last Review 03/18/2019 Effective: 05/08/1996 Next Review: 01/23/2020 Review History Definitions Additional Clinical Policy Bulletin Notes www.aetna.com/cpb/medical/data/100_199/0106.html#dummyLink2 Proprietary 1/27

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Page 1: Prior Authorization Review Panel MCO Policy …...B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis of Sjögren’s syndrome. 1. Frequency of

(https://www.aetna.com/)

Fetal Echocardiography andMagnetocardiography

Clinical Policy Bulletins Medical Clinical Policy Bulletins

Number: 0106

*Please see amendment for Pennsylvania Medicaid at the end of this CPB.

I. Aetna considers fetal echocardiograms, Doppler and color flow mapping medically

necessary after 12 weeks gestation for any of the following conditions:

A. A mother with insulin dependent diabetes mellitus or systemic lupus erythematosus; or

B. As a screening study in families with a first-degree relative of a fetus with congenital heart

disease; or

C. Fetal nuchal translucency measurement of 3.5 mm or greater in the first trimester; or

D. 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

E. For ductus arteriosus dependent lesions and/or with other known complex congenital

heart disease; or

F. For pregnancies conceived by in vitro fertilization (IVF) or intra-cytoplasmic sperm

injection (ICSI); or

G. In cases of persistent right umbilical vein; or

H. In cases of single umbilical artery; or

Last Review

03/18/2019

Effective: 05/08/1996

Next

Review: 01/23/2020

Review

History

Definitions

Additional

Clinical Policy

Bulletin

Notes

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Page 2: Prior Authorization Review Panel MCO Policy …...B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis of Sjögren’s syndrome. 1. Frequency of

I. In cases of suspected or known fetal chromosomal abnormalities; or

J. In suspected or documented fetal arrhythmia: to define the rhythm and its significance, to

identify structural heart disease and cardiac function; or

K. In members with autoimmune antibodies associated with congenital cardiac

anomalies [anti-Ro (SSA)/anti-La (SSB)]; or

L. In members with familial inherited disorders associated with congenital cardiac

abnormalities (e.g., Marfan syndrome); or

M. In cases with monochorionic twins; or

N. In cases of multiple gestation and suspicion of twin-twin transfusion syndrome; or

O. In members with seizure disorders, even if they are not presently taking anti-seizure

medication; or

P. In cases with non-immune fetal hydrops or unexplained severe polyhydramnios; or

Q. 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

Lithium; or

Paroxetine (Paxil); or

Retinoids; or

R. When other structural abnormalities are found on ultrasound; or

II. Aetna considers repeat studies of fetal echocardiograms medically necessary for any of

the following:

A. When the initial screening study indicates any of the following:

1. A ductus arteriosus dependent lesion; or

2. Structural heart disease with a suggestion of hemodynamic compromise;or

3. Tachycardia other than sinus tachycardia or heart block; or

B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis

of Sjögren’s syndrome.

1. Frequency of testing: Doppler fetal echocardiography may be repeated every 1 to

2 weeks starting at 16 weeks gestation continuing through 28 weeks gestation,

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Page 3: Prior Authorization Review Panel MCO Policy …...B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis of Sjögren’s syndrome. 1. Frequency of

then every other week until 32 weeks gestation to detect fetal (congenital) heart

block.

III. Aetna considers fetal echocardiograms experimental and investigational for all other

indications including the following (not an all-inclusive list) because their effectiveness

for these indications has not been established.

As a screening test in advanced maternal age; or

Pregnant women receiving selective serotonin reuptake inhibitors (except

paroxetine); or

Suspected cystic fibrosis.

IV. Aetna considers fetal magnetocardiography experimental and investigational because its

effectiveness has not been established.

Definition of fetal cardiac structures is currently possible at 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.

According to the American Institute for Ultrasound in Medicine (AIUM), fetal echocardiography is

commonly performed between 18 and 22 weeks’ gestational age. Some forms of congenital

heart disease may even be recognized during earlier stages of pregnancy (AIUM, 2013). Newer

technology including endovaginal transducers can obtain images of the heart as early as 12

weeks gestation (AHA, 2018).

Hutchinson et al. (2017) states that early fetal echocardiography (FE), performed at 12 to 16

weeks' gestational age (GA), can be used to screen for fetal heart disease similar to that

routinely performed in the second trimester; however, the efficacy of FE at earlier GAs has not

been as well explored, particularly with recent advances in ultrasound technology. Pregnant

women were prospectively recruited for first-trimester FE. All underwent two-dimensional (2D)

cardiac imaging combined with color Doppler (CD) assessment, and all were offered second-

trimester fetal echocardiographic evaluations. Fetal cardiac anatomy was assessed both in real

time during FE and additionally offline by two separate reviewers. Very early FE was performed

in 202 pregnancies including a total of 261 fetuses, with 92% (n = 241) being reassessed at

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greater than or equal to 18 weeks' GA. Transabdominal scanning was used in all cases, and

transvaginal scanning was used additionally in most at less than 11 weeks' GA (n = 103 of 117

[88%]). There was stepwise improvement in image resolution of the fetal heart in those

pregnancies that presented at later gestation for assessment. CD assisted with definition of

cardiac anatomy at all GAs. A four-chambered heart could be identified in 52% of patients in the

eighth week (n = 12 of 23), improving to 80% (n = 36 of 45) in the 10th week and 98% (n = 57 of

58) by the 11th week. The inferior vena cava was visualized by 2D imaging in only 4% (n = 1 of

23) in the eighth week, increasing to 13% (n = 6 of 45) by the 10th week and 80% (n = 25 of 31)

by the 13th week. CD improved visualization of the inferior vena cava at earlier GAs to greater

than 80% (n = 37 of 45) from 10 weeks. Pulmonary veins were not visualized by either 2D

imaging or CD until after the 11th week. Both cardiac outflow tracts could be visualized by 2D

imaging in the minority from 8+0 to 10+6 weeks (n = 18 of 109 [16%]) but were imaged in most

from 11+0 to 13+6 weeks (n = 114 of 144 [79%]). CD imaging improved visualization of both

outflow tracts to 64% (n = 29 of 45) in the 10th week. On 2D imaging alone, both the aortic and

ductal arches were seen in only 29% of patients in the 10th week (n = 13 of 45), increasing to

58% when CD was used (58% [n = 26 of 45]) and to greater than 80% (n = 47 of 58) using CD in

the 11th week. The authors concluded that very early FE, from as early as 8 weeks, can be used

to assess cardiac structures; however, the ability to image fetal heart structures between 6 and 8

weeks is currently nondiagnostic. The use of CD significantly increases the detection of cardiac

structures on early FE. The ideal timing of complete early FE, excluding pulmonary vein

assessment, appears to be after 11 weeks' GA.

Ventriglia et al. (2016) state that there is a growing body of evidence that most of the major

cardiac abnormalities can be diagnosed from 12-16 weeks of gestation (compared with the usual

18-22 weeks). Furthermore, the reason for performing early fetal echocardiography (EFEC) is

that "the combined EFEC-NT (nuchal translucency) approach (11th-13th week) gives a 60-70%

increase in detection rate for CHD. Combined EFE-NT analysis is also justified by the high CHD

frequency in genetic syndromes and the similarity of anatomic relations between cardiac

structures at 11-13 wks GA and those of the second trimester.” “The technical limits of EFEC are

CRL < 50 mm, an increase of maternal Body mass index (BMI), unfavorable fetal position and a

possible progression of cardiac disease especially in outflow obstructions. This means that the

pregnant women should be informed about the limits of early screening and also recommended

to have a further scan as from 18 weeks for a more complete diagnosis.”

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,

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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.

The umbilical cord normally contains two arteries and one vein embedded in Wharton's jelly.The

umbilical cord "achieves its final form by the 12th week of gestation". Initially during umbilical

cord development, there are two umbilical arteries and two umbilical veins, in which the two

veins (left and right) converge into one. Obliteration of the right umbilical vein by the end of the

6th week of gestation results in a single persisting left umbilical vein (Spurway et al,

2012). However, persistence of the right umbilical vein in the fetus is a variant of the intra-

abdominal umbilical venous connection. The estimated prevalence of an intrahepatic persistent

right umbilical vein is 1 per 786 births; which may be an underestimated calculation in

populations that do not undergo targeted sonographic examinations. In addition, the variation in

anatomy can be subtle (Lide et al, 2016).

Lide et al (2016) provided a comprehensive review of the current data surrounding an intra-

hepatic persistent right umbilical vein in the fetus, including associated anomalies and outcomes,

to aid practitioners in counseling and management of affected pregnancies. These investigators

performed a Medline, Embase, Cochrane Central Register of Controlled Trials, and Northern

Light database search for articles reporting outcomes on prenatally diagnosed cases of a

persistent right umbilical vein. Each article was independently reviewed for eligibility by the

investigators. Thereafter, the data were extracted and validated independently by 3

investigators. A total of 322 articles were retrieved, and 16 were included in this systematic

review. The overall prevalence of an intra-hepatic persistent right umbilical vein was found to be

212 per 166,548 (0.13 %). Of the 240 cases of an intra-hepatic persistent right umbilical vein

identified, 183 (76.3 %) were isolated. The remaining cases had a co-existing abnormality,

including 19 (7.9 %) cardiac, 9 (3.8 %) central nervous system, 15 (6.3 %) genito-urinary, 3 (1.3

%) genetic, and 17 (7 %) placental/cord (predominantly a single umbilical artery). The authors

concluded that a persistent right umbilical vein is commonly an isolated finding but may be

associated with a co-existing cardiac defect in 8 % of cases. Therefore, consideration should be

given to fetal echocardiography in cases of a persistent right umbilical vein.

Canavan et al (2016) stated that a fetal persistent intrahepatic right umbilical vein has been

linked to anomalies and genetic disorders but can be a normal variant. These researchers

conducted a retrospective review to determine other sonographic findings that can stratify

fetuses for further evaluation. A total of 313 fetuses had a persistent intra-hepatic right umbilical

vein identified on 17- to 24-week sonography. The outcome was any major congenital anomaly

or an adverse neonatal outcome, which was defined as aneuploidy, fetal demise, or neonatal

death. A total of 217 patients (69.3 %) had a normal neonatal outcome; 69 patients (22.0 %)

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were lost to follow-up; 5 fetuses (2.1 %) had aneuploidy; 4 of the 5 had additional sonographic

findings, and 1 had an isolated persistent intra-hepatic right umbilical vein; 24 fetuses had a

major anomaly in association with the persistent right umbilical vein; 26 additional fetuses had

soft sonographic markers associated with karyotypic abnormalities but were chromosomally

normal. Of those with adverse neonatal outcomes, 12 had a congenital heart defect (57 %). An

additional sonographic finding with a persistent intra-hepatic right umbilical vein was predictive of

a congenital anomaly or an adverse neonatal outcome (p < 0.001), with a positive predictive

value of 44.0 % (95 % confidence interval[ CI]: 30.0 % to 58.7 %). An isolated persistent intra-

hepatic right umbilical vein had a 0.4 % risk for a congenital anomaly or an adverse neonatal

outcome. The authors concluded that a persistent intra-hepatic right umbilical vein should

prompt an extended anatomic survey and a fetal cardiac evaluation. If the survey and cardiac

anatomy are reassuring, no further follow-up is needed. If additional findings are identified,

genetic counseling and invasive testing should be considered.

Kumar et al (2016) appraised the incidence and significance of persistent right umbilical vein

(PRUV), the most common fetal venous aberration. Based on a South Indian antenatal cohort,

these researchers identified 23 cases of PRUV amongst 20,452 fetuses of consecutive

pregnancies, from 2009 to 2014, yielding an incidence of 1 in 889 total births (0.11 %). The

median maternal age was 24 (inter-quartile range [IQR], 22 to 26) years, and median gestational

age at diagnosis was 23 (IQR, 22 to 24) weeks. Intra-hepatic drainage of PRUV was seen in

91.3 % cases. In 3 cases (13 %), ductus venosus was absent. In 52.2 % of the cases,

additional major abnormalities were observed - predominantly cardiovascular (39.1 %). The

common minor marker was single umbilical artery (SUA; 13 %). The karyotype was found to be

normal in 6 cases (26 %) that underwent invasive testing. When associated anomalies were

inconsequential or absent, the post-natal outcome was good, which reflected in 60.9 % of these

cases. Fetal echocardiography was one of the keywords listed in this study.

In a prospective, observational study, Hill et al (1994) reviewed their experience with antenatal

detection and subsequent neonatal outcome of fetuses with a persistent right umbilical vein. A

total of 33 cases of persistent right umbilical vein were detected during 15,237 obstetric

ultrasound examinations performed after 15 weeks' gestation. Persistent right umbilical vein was

detected at a rate of 1 per 476 obstetric ultrasound examinations; 6 of 33 (18.2 %) fetuses with a

persistent right umbilical vein had additional important congenital malformations. The authors

concluded that careful 2nd- and 3rd-trimester ultrasound examinations can detect a persistent

right umbilical vein. When this particular anomaly is detected, a thorough fetal anatomic survey,

including echocardiography, should be performed to rule out more serious congenital

malformations.

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Wolman et al (2002) conducted a prospective evaluation of the incidence and neonatal outcome

of fetuses with persistent right umbilical vein. This condition had traditionally been considered to

be extremely rare and to be associated with a very poor neonatal prognosis, but later evidence

has raised some doubts about the veracity of these contentions. Between August 1995 and

November 1998, a total of 8,950 low-risk patients were prospectively evaluated at 2 medical

centers. The sonographic diagnosis of a persistent right umbilical vein was made in a transverse

section of the fetal abdomen when the portal vein was curved toward the stomach, and the fetal

gall bladder was located medially to the umbilical vein. Persistent right umbilical vein was

detected in 17 fetuses during the study; 4 of them had additional malformations, of which 3 had

been detected antenatally. The authors established that the incidence of persistent right

umbilical vein in a low-risk population was 1:526. They believed that the sonographic finding of

this anomaly was an indication for conducting targeted fetal sonography and echocardiography.

When the persistent right umbilical vein was connected to the portal system and other anomalies

were ruled out, the prognosis can generally be expected to be favorable.

Martínez et al (2012) described the ultrasound findings, maternal and perinatal variables in

cases with a prenatal diagnosis of persistence of right umbilical vein. This was a descriptive

analysis of cases with prenatal diagnosis of persistence of right umbilical vein in the Fetal

Medicine Unit, Department of Obstetrics and Gynecology, Hospital Universitario Severo Ochoa.

These investigators described ultrasound findings, maternal and perinatal variables. They

examined 9,198 fetuses and 6 cases (0.06 %) were diagnosis prenatally of persistent right

umbilical vein, between 20 and 29 weeks of gestation. The male/female ratio was 1/1. Ductus

venosus was presented in all cases; 2 fetuses (33 %) were proved to have other structural

anomalies and their parents opted for termination of the pregnancy. All cases had no

chromosomal anomaly associated and after birth, neonatal developments were favorable. The

authors concluded that based on these findings and a literature review, after prenatal diagnosis

of persistent right umbilical vein, an exhaustive morphological study, which included a fetal

echocardiography, is mandatory in order to rule out other structural malformations. Indication for

fetal karyotype study has to be individualized considering persistence right umbilical vein type

and other ultrasound findings.

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.

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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.

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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

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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.

Pregnant Women Receiving Selective Serotonin Reuptake Inhibitors

Reefhuis and colleagues (2015) followed up on previously reported associations between peri­

conceptional use of selective serotonin reuptake inhibitors (SSRIs) and specific birth defects

using an expanded dataset from the National Birth Defects Prevention Study. These researchers

performed a Bayesian analysis combining results from independent published analyses with data

from a multi-center population based case-control study of birth defects. A total of 17,952

mothers of infants with birth defects and 9,857 mothers of infants without birth defects, identified

through birth certificates or birth hospitals, with estimated dates of delivery between 1997 and

2009 were included in this analysis; exposures were citalopram, escitalopram, fluoxetine,

paroxetine, or sertraline use in the month before through the 3rd month of pregnancy. Posterior

OR estimates were adjusted to account for maternal race/ethnicity, education, smoking, and pre­

pregnancy obesity. Main outcome measure was 14 birth defects categories that had

associations with SSRIs reported in the literature. Sertraline was the most commonly reported

SSRI, but none of the 5 previously reported birth defects associations with sertraline was

confirmed. For 9 previously reported associations between maternal SSRI use and birth defect

in infants, findings were consistent with no association. High posterior ORs excluding the null

value were observed for 5 birth defects with paroxetine (anencephaly 3.2, 95 % CI: 1.6 to 6.2;

atrial septal defects 1.8, 95 % CI: 1.1 to 3.0; right ventricular outflow tract obstruction defects 2.4,

95 % CI: 1.4 to 3.9; gastroschisis 2.5, 95 % CI: 1.2 to 4.8; and omphalocele 3.5, 95 % CI: 1.3 to

8.0) and for 2 defects with fluoxetine (right ventricular outflow tract obstruction defects 2.0, 95 %

CI: 1.4 to 3.1 and craniosynostosis 1.9, 95 % CI: 1.1 to 3.0). The authors concluded that these

data provided reassuring evidence for some SSRIs; but suggested that some birth defects

occurred 2 to 3.5 times more frequently among the infants of women treated with paroxetine or

fluoxetine early in pregnancy.

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A 2015 study by the Centers for Disease Control and Prevention (CDC) used new data to

examine previous reported links between use of specific SSRIs medications just before or during

early pregnancy and the occurrence of certain birth defects. Researchers looked at links with 5

different SSRI medications: citalopram, escitalopram, fluoxetine, paroxetine, and sertraline.

Although the new data confirmed the risks seen with paroxetine, it did not appear to suggest that

the risk is across the board with all SSRIs. Therefore, fetal echocardiography is still

recommended for women exposed to paroxetine, but there doesn’t seem to be enough evidence

to recommend coverage of fetal echocardiograms for all pregnant members receiving any SSRI.

The study concluded that despite the increased risks for certain birth defects from some SSRIs

found in this study, the actual risk for a birth defect among babies born to women taking one of

these medications is still very low. Because these specific types of birth defects are rare, even

doubling the risk still results in a low absolute risk. For example, the risks for heart defects with

obstruction of the right ventricular outflow tract could increase from 10 per 10,000 births to about

24 per 10,000 births among babies of women who are treated with paroxetine early in

pregnancy.

Fetal Magnetocardiography

Fetal magnetocardiography (fMCG) is a new, non-invasive technique used to monitor the

spontaneous electrophysiological activity of the fetal heart. Hrtankova and associates (2015)

reviewed the evidence on the clinical value of fMCG. These investigators performed an analysis

of the literature using database search engines PubMed, and SCOPE in field of fMCG.

Compared to cardiotocography and fetal electrocardiography, fMCG is a more effective method

with a higher resolution. The signal obtained from the fetal heart is sufficiently precise and the

quality allows an assessment of PQRST complex alterations, and to detect fetal arrhythmia.

Thanks to early diagnosis of fetal arrhythmia, there is the possibility for appropriate therapeutic

intervention and the reduction of unexplained fetal death in late gestation. These investigators

also noted that fMCG with high temporal resolution also increased the level of clinical trials that

recorded fetal heart rate (FHR) variability. According to the latest theories, FHR variability is a

possible indicator of fetal status and enabled the study of the fetal autonomic nervous system

indirectly. The authors concluded that fMCG is an experimental method that requires expensive

equipment; it has yet to be shown in the future if this method will get any application in clinical

practice.

Eswaran and colleagues (2017) stated that fMCG provides the requisite precision for diagnostic

measurement of electrophysiological events in the fetal heart. Despite its significant benefits,

this technique with current cryogenic based sensors has been limited to few centers, due to high

cost of maintenance. In this study, these researchers demonstrated that a less expensive non­

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cryogenic alternative, optically pumped magnetometers, can provide similar electrophysiological

and quantitative characteristics when subjected to direct comparison with the current technology.

They concluded that further research can potentially increase its clinical use for fMCG.

Furthermore, an UpToDate review on “Overview of the general approach to diagnosis and

treatment of fetal arrhythmias” (Levine and Alexander, 2017) states that “Magnetocardiography

shifts the electrical signals into an evoked magnetic signal that can be processed to create a

beat-to-beat magnetocardiogram that looks much like a traditional electrocardiogram (ECG).

Continuous recordings can be performed for relatively sustained periods and have permitted

elegant demonstration of arrhythmia onset/offset and more direct observation of mechanisms.

The equipment is not widely available, requires careful shielding and requires skilled technical

support, so the technology remains investigational”.

Fetal Surveillance in Sjögren’s Syndrome

Gupta and Gupta (2017) state that studies show a high incidence of poor fetal outcomes for

women with Sjögren’s syndrome; however pregnancy outcomes in these women have not been

extensively studied. The authors conducted a literature review to evaluate Sjögren’s syndrome

and pregnancy. Gupta and Gupta found that well-known fetal outcomes in Sjögren syndrome-

complicated pregnancies include neonatal lupus and congenital heart block (CHB), of which

CHB is the most severe fetal complication. CHB is thought to occur because of damage to the

atrioventricular node by anti-SS-A or anti-SS-B antibodies, or both. The reported prevalence of

CHB in the offspring of an anti-SS-A-positive woman is 1% to 2%. The recurrence rate in a

patient with antibodies, who has a previous child affected, is approximately 10 times higher.

Based on Gupta’s review, frequent surveillance by serial echocardiograms and obstetric

sonograms between 16 to 20 weeks of gestation and thereafter is required for at-risk

pregnancies, with the goal of early diagnosis and early treatment of incomplete CHB, thus

improving the outcome for the fetus.

Although there are no formal guidelines for type or frequency of testing to detect fetal heart

block, it is recommended that pregnant women with Sjögren’s syndrome receive weekly pulsed

Doppler fetal echocardiography from the 18th through the 26th week of pregnancy and then

every other week until 32 weeks. “The most vulnerable period for the fetus is during the period

from 18 to 24 weeks gestation. Normal sinus rhythm can progress to complete block in seven

days during this high-risk period. New onset of heart block is less likely during the 26th through

the 30th week, and it rarely develops after 30 weeks of pregnancy” (eviCore, 2018).

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A scientific statement from the American Heart Association by Donofrio et al. (2014) states that

maternal factors of Sjögren’s syndrome are associated with the absolute risk of 1 to 5 percent of

live births that will have congenital heart block (CHB), risk increases to 11 to 19 percent for prior

affected child with CHB or neonatal lupus. It is recommended that fetal echocardiography be

performed at 16 weeks, then weekly or every other week to 28 weeks. The authors state that

studies have suggested that high SSA values (≥50 U/mL) correlate with increased fetal risk, and

that concern for late myocardial involvement may justify additional assessments in the third

trimester. In addition to abnormalities in the conduction system, up to 10% to 15% of SSA-

exposed fetuses with conduction system disease may also develop myocardial inflammation,

endocardial fibroelastosis, or atrioventricular (AV) valve apparatus dysfunction. “Although the

value of serial assessment for the detection of the progression of myocardial inflammation or

conduction system disease from first-degree block (PR prolongation) to CHB has not been

proved, serial assessment at 1- to 2-week intervals starting at 16 weeks and continuing through

28 weeks of gestation is reasonable to perform because the potential benefits outweigh the risks.

For women who have had a previously affected child, more frequent serial assessment, at least

weekly, is recommended.”

DocumentationRequirementsforFetalEchocardiography

According to guidelines from the American Institute for Ultrasound in Medicine (AIUM), fetal

echocardiography should include the following cardiac images:

Aortic arch;

Ductal arch;

Four-chamber view;

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 2013 AIUM's practice parameter for the "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

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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

Autoimmune antibodies [anti-Ro (SSA)/anti-La (SSB)]

Familial inherited disorders (e.g., 22q11.2 deletion syndrome)

In-vitro fertilization

Metabolic disease (e.g., diabetes mellitus and phenylketonuria)

Teratogen exposure (e.g., lithium and retinoids)

Fetal Indications

Abnormal cardiac screening examination

Abnormal heart rate or rhythm

Fetal chromosomal anomaly

Extra-cardiac anomaly

First-degree relative of a fetus with congenital heart disease

Hydrops

Increased nuchal translucency

Monochorionic twins

This AIUM (2013) practice parameter was published in conjunction with the American College of

Obstetricians and Gynecologists (ACOG), and the Society for Maternal-Fetal Medicine (SMFM),

and the American Society of Echocardiography (ASE). Furthermore, this practice parameter

was endorsed by the American College of Radiology (ACR).

Source: AIUM Practice Parameter – Fetal Echocardiography (2013).

CPT Codes / HCPCS Codes / ICD-10 Codes

Information in the [brackets] below has been added for clarification purposes. Codes requiring a 7th character are represented by "+":

Code Code Description

CPT codes covered if selection criteria are met:

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76825 Echocardiography, fetal, cardiovascular system, real time with image documentation

(2D), with or without M-mode recording;

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Code Code Description

76826 follow-up or repeat study

76827 Doppler echocardiography, fetal, cardiovascular system, pulsed wave and/or

continuous wave with spectral display; complete

76828 follow-up or repeat study

+93325 Doppler echocardiography color flow velocity mapping (List separately in addition to

codes for echocardiography)

CPT codes not covered for indications listed in the CPB:

0475T Recording of fetal magnetic cardiac signal using at least 3 channels; patient

recording and storage, data scanning with signal extraction, technical analysis and

result, as well as supervision, review, and interpretation of report by a physician or

other qualified health care professional

0476T patient recording, data scanning, with raw electronic signal transfer of data and

storage

0477T signal extraction, technical analysis, and result

0478T review, interpretation, report by physician or other qualified health care

professional

0541T - 0542T Myocardial imaging by magnetocardiography (MCG) for detection of cardiac

ischemia, by signal acquisition using minimum 36 channel grid, generation of

magnetic-field time-series images, quantitative analysis of magnetic dipoles,

machine learning–derived clinical scoring, and automated report generation

Other HCPCS codes related to the CPB:

Q9950 Injection, sulfur hexafluoride lipid microspheres, per ml

Maternal ICD-10 codes covered if selection criteria are met:

B97.10, B97.89 Unspecified viral infection

D68.61 Antiphospholipid syndrome

E10.10 - E13.9 Diabetes mellitus [do not report for gestational diabetes]

F10.20 - F10.29 Alcohol dependence

G40.001 - G40.919 Epilepsy and recurrent seizures

I34.0 - I37.9 Mitral valve disorders, aortic valve disorders, tricuspid valve disorders and

pulmonary valve disorders, specified as nonrheumatic,

I42.3 Endomyocardial (eosinophilic) disease

I42.4 Endocardial fibroelastosis

I42.6 Alcoholic cardiomyopathy

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Code Code Description

I50.1 - I50.9 Heart failure

I51.7 Cardiomegaly

I78.0 Hereditary hemorrhagic telangectasia

L93.0 - L93.2 Lupus erythematosus

M05.40 - M06.9 Rheumatoid arthritis

M32.0 - M32.9 Systemic lupus erythematosus

M34.0 - M34.9 Systemic sclerosis [scleroderma]

M35.00 - M35.09 Sicca syndrome [Sjögren]

M35.9, M36.8 Unspecified diffuse connective tissue disease

O24.011 - O24.019,

O24.111 - O24.119

O24.311 - O24.319,

O24.811 -O24.819

O24.911 -O24.919

Diabetes mellitus in pregnancy [pre-existing, excludes gestational diabetes]

O30.001 - O30.93 Multiple gestation

O36.8310

O36.8399

Maternal care for abnormalities of the fetal heart rate or rhythm ­

O98.411 - O98.419,

O98.511 - O98.519

Viral hepatitis and other viral diseases complicating pregnancy

O98.611 - O98.619,

O98.711 - O98.719

O98.811 - O98.819,

O99.830

Other specified infectious and parasitic diseases complicating pregnancy

O98.911 - O98.93 Unspecified maternal infectious and parasitic diseases complicating pregnancy,

childbirth and the puerperium

099.111 - O99.119 Other diseases of the blood and blood-forming organs and certain disorders

involving the immune mechanism complicating pregnancy with brackets stating

[Antiphospholipid syndrome]

O99.350 - O99.353 Diseases of the nervous system complicating pregnancy [epilepsy]

O99.411 - O99.419 Diseases of the circulatory system complicating pregnancy

O99.89 Other specified diseases and conditions complicating pregnancy, childbirth and the

puerperium [Systemic lupus erythematosus (SLE)]

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Code Code Description

Q20.0 - Q28.9 Congenital malformations of the circulatory system

Q79.6 Ehlers-Danlos syndrome

Q87.40 - Q87.43 Marfan's syndrome

Q89.3 Situs inversus

Q89.7 Multiple congenital malformations, not elsewhere classified

Q90.0 - Q90.9 Down syndrome

Q91.0 - Q91.3 Trisomy 18 [Edward's syndrome]

R56.1 Post traumatic seizures

R56.9 Unspecified convulsions [seizure disorder NOS]

R93.1, R93.8 Abnormal findings on diagnostic imaging of heart and coronary circulation and other

body structures

T42.1x5+, T42.5x5+

T42.6x5+, T42.75x+

Adverse effects of other and unspecified anticonvulsants

Z3A.13 - Z34.49 13 - 49 Weeks of gestation of pregnancy

Z82.79 Family history of other congenital malformations, deformations and chromosomal

abnormalities

Z98.89 Other specified postprocedural states

Fetal ICD-10 codes covered if selection criteria are met:

O09.811 - O09.819 Supervision of pregnancy resulting from assisted reproductive rechnology

O33.6xx0

O33.6xx9

Maternal care for disproportion due to hydrocephalic fetus ­

O35.0xx0

O35.0xx9

­ Maternal care for (suspected) central nervous system malformation in fetus

O35.1xx0

O35.1xx9

­ Maternal care for (suspected) chromosomal abnormality in fetus

O35.2xx0

O35.2xx9

­ Maternal care for (suspected) hereditary disease in fetus

O35.3xx0

O35.3xx9

­ Maternal care for (suspected) damage to fetus from viral disease in mother

O35.4xx0

O35.4xx9

­ Maternal care for (suspected) damage to fetus from alcohol

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Code Code Description

O35.5xx0 ­

O35.5xx9

Maternal care for (suspected) damage to fetus from drugs

O35.8xx0 ­

O35.8xx9

Maternal care for (suspected) fetal abnormality and damage

O35.9xx0 ­

O35.9xx9

Maternal care for (suspected) fetal abnormality and damage, unspecified

O36.0110 ­

O36.0999

Maternal care for rhesus isoimmunization

O36.1110 ­

O36.1999

Maternal care for other isoimmunization

O36.20x0 ­

O36.23x9

Maternal care for hydrops fetalis

O36.8310 ­

O03.8399

Maternal care for abnormalities of the fetal heart rate or rhythm

O40.1XX0 ­

O40.3XX9

Polyhydramnios

O43.011 - O43.029 Placenta transfusion syndromes

Q27.0 Congenital absence and hypoplasia of umbilical artery

ICD-10 codes covered if selection criteria are met:

O09.811 - O09.819 Supervision of pregnancy resulting from assisted reproductive rechnology

O24.011 - O24.019,

O24.111 - O24.119

O24.311 - O24.319,

O24.811 - O24.819

O24.911 - O24.919

Diabetes mellitus in pregnancy [pre-existing, excludes gestational diabetes]

O33.6xx0

O33.6xx9

Maternal care for disproportion due to hydrocephalic fetus ­

O35.0xx0

O35.0xx9

­ Maternal care for (suspected) central nervous system malformation in fetus

O35.1xx0

O35.1xx9

­ Maternal care for (suspected) chromosomal abnormality in fetus

O35.2xx0

O35.2xx9

­ Maternal care for (suspected) hereditary disease in fetus

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Code Code Description

O35.3xx0 ­

O35.3xx9

Maternal care for (suspected) damage to fetus from viral disease in mother

O35.4xx0 ­

O35.4xx9

Maternal care for (suspected) damage to fetus from alcohol

O35.5xx0 ­

O35.5xx9

Maternal care for (suspected) damage to fetus from drugs

O35.8xx0 ­

O35.8xx9

Maternal care for (suspected) fetal abnormality and damage

O35.9xx0 ­

O35.9xx9

Maternal care for (suspected) fetal abnormality and damage, unspecified

O36.0110 ­

O36.0999

Maternal care for rhesus isoimmunization

O36.1110 ­

O36.1999

Maternal care for other isoimmunization

O40.1xx0 ­

O40.1xx9

Polyhydramnios

O43.011 - O43.029 Placenta transfusion syndromes

O76 Abnormality in fetal heart rate and rhythm complicating labor and delivery

O98.411 - O98.419,

O98.511 - O98.519

Viral hepatitis and other viral diseases complicating pregnancy

O98.611 - O98.619,

O98.711 - O98.719

O98.811 - O98.819,

O99.830

Other specified infectious and parasitic diseases complicating pregnancy

O98.911 - O98.919 Unspecified maternal infectious and parasitic diseases complicating pregnancy

O99.411 - O99.419 Diseases of the circulatory systerm complicating pregnancy

Q27.0 Congenital absence and hypoplasia of umbilical artery

ICD-10 codes not covered for indications listed in the CPB (not all-inclusive):

O09.511 - O09.519 Supervision of elderly primigravida

O09.521 - O09.529 Supervision of elderly multigravida

O99.810 - O99.815 Abnormal glucose complicating pregnancy, childbirth and the puerperium

Z13.228 Encounter for screening for other metabolic disorders

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1. 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.

2. Winsberg F. Echocardiography of the fetal and newborn heart. Invest Radiol.

1972;3:152-158.

3. 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.

4. 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.

5. 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.

6. Nora JJ, Nora AH. The evolution of specific genetic and environmental counseling in

congenital heart diseases. Circulation. 1978;57:205-213.

7. 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.

8. Silverman NH, Golbus MS. Echocardiographic techniques for assessing normal and

abnormal fetal cardiac anatomy. J Am Coll Cardiol. 1985;5(Suppl 1):20S-29S.

9. 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.

10. 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.

11. 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.

12. 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.

13. 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.

www.aetna.com/cpb/medical/data/100_199/0106.html#dummyLink2 Proprietary 20/27

Page 21: Prior Authorization Review Panel MCO Policy …...B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis of Sjögren’s syndrome. 1. Frequency of

14. 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.

15. Allan LD, Joseph MC, Boyd EG, et al. M-mode echocardiography in the developing

human fetus. Br Heart J. 1982;47(6):573-583.

16. 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.

17. Shime J, Gresser CD, Rakowski H. Quantitative two-dimensional echocardiographic

assessment of fetal growth. Am J Obstet Gynecol. 1986;154:290-300.

18. Huhta JC, Strasburger JF, Carpenter RJ, et al. Pulsed Doppler fetal echocardiography. J

Clin Ultrasound. 1985;13:247-254.

19. Maulik D, Nanda NC, Saini VD. Fetal Doppler echocardiography: Methods and

characterization of normal and abnormal hemodynamics. Am J Cardiol. 1984;53:572

578.

­

20. Schulman H, Fleischer A, Stern W, et al. Umbilical velocity wave ratios in human

pregnancy. Am J Obstet Gynecol. 1984;148:985-990.

21. Chen HY, Lu CC, Cheng YT, et al. Antenatal measurement of fetal umbilical venous flow

by pulsed Doppler and B-mode ultrasonography. J Ultrasound Med. 1986;5:319-321.

22. Reed KL, Sahn DJ, Scagnelli S, et al. Doppler echocardiographic studies of diastolic

function in the human fetal heart: Changes during gestation. J Am Coll Cardiol.

1986;8:391-395.

23. Kiemman CS, Copel JA, Weinstein EM, et al. Treatment of fetal supraventricular

tachyarrhythmias. J Clin Ultrasound. 1985;13:265-273.

24. Allan LD, Anderson RH, Sullivan ID, et al. Evaluation of fetal arrhythmias by

echocardiography. Br Heart J. 1983;50(3):240-245.

25. Keinman CS, Hobbins JC, Jaffe CC, et al. Echocardiographic studies of the human fetus:

Prenatal diagnosis of congenital heart disease and cardiac dysrhythmias. Pediatrics.

1980;65:1059-1067.

26. Silverman NH, Enderlein MA, Stanger P, et al. Recognition of fetal arrhythmias by

echocardiography. J Clin Ultrasound. 1985;13:255-263.

27. McCue CM, Mantakas ME, Tinglestad JB, et al. Congenital heart block in newborns of

mothers with connective tissue disease. Circulation.1977;56:82-89.

28. Mapp T. Fetal echocardiography and congenital heart disease. Prof Care Mother Child.

2000;10(1):9-11.

29. Srinivasan S. Fetal echocardiography. Indian J Pediatr. 2000;67(7):515-521.

30. Tometzki AJ, Suda K, Kohl T, et al. Accuracy of prenatal echocardiographic diagnosis

and prognosis of fetuses with conotruncal anomalies. J Am Coll Cardiol.

1999;33(6):1696-1701.

www.aetna.com/cpb/medical/data/100_199/0106.html#dummyLink2 Proprietary 21/27

Page 22: Prior Authorization Review Panel MCO Policy …...B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis of Sjögren’s syndrome. 1. Frequency of

31. Frommelt MA, Frommelt PC. Advances in echocardiographic diagnostic modalities for

the pediatrician. Pediatr Clin North Am. 1999;46(2):427-439,xi.

32. Berghella V, Pagotto L, Kaufman M, et al. Accuracy of prenatal diagnosis of congenital

heart defects. Fetal Diagn Ther. 2001;16(6):407-412.

33. Gossett DR, Lantz ME, Chisholm CA. Antenatal diagnosis of single umbilical artery: Is

fetal echocardiography warranted? Obstet Gynecol. 2002;100(5 Pt 1):903-908.

34. Huggon IC, Ghi T, Cook AC, et al. Fetal cardiac abnormalities identified prior to 14

weeks' gestation. Ultrasound Obstet Gynecol. 2002;20(1):22-29.

35. 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.

36. Simpson LL. Indications for fetal echocardiography from a tertiary-care obstetric

sonography practice. J Clin Ultrasound. 2004;32(3):123-128.

37. Forbus GA, Atz AM, Shirali GS. Implications and limitations of an abnormal fetal

echocardiogram. Am J Cardiol. 2004;94(5):688-689.

38. 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.

39. McAuliffe FM, Trines J, Nield LE, et al. Early fetal echocardiography--a reliable prenatal

diagnosis tool. Am J Obstet Gynecol. 2005;193(3 Pt2):1253-1259.

40. 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.

41. Friedman AH, Copel JA, Kleinman CS. Fetal echocardiography and fetal cardiology:

Indications, diagnosis and management. Semin Perinatol. 1993;17(2):76-88.

42. 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.

43. Johnson B, Simpson LL. Screening for congenital heart disease: A move toward earlier

echocardiography. Am J Perinatol. 2007;24(8):449-456.

44. 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.

45. Marques Carvalho SR, Mendes MC, Poli Neto OB, Berezowski AT. First trimester fetal

echocardiography. Gynecol Obstet Invest. 2008;65(3):162-168.

46. 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.

www.aetna.com/cpb/medical/data/100_199/0106.html#dummyLink2 Proprietary 22/27

Page 23: Prior Authorization Review Panel MCO Policy …...B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis of Sjögren’s syndrome. 1. Frequency of

47. 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.

48. 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.

49. 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.

50. 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.

51. 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.

52. 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.

53. 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.

54. 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.

55. 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.

56. Palazzi DL, Brandt ML. Care of the umbilicus and management of umbilical disorders.

UpToDate [online serial]. Waltham, MA: UpToDate; 2009.

57. Kurinczuk JJ, Bower C. Birth defects in infants conceived by intracytoplasmic sperm

injection: An alternative interpretation. BMJ.1997;315(7118):1260-1265.

58. 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.

59. 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.

60. 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.

61. 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.

www.aetna.com/cpb/medical/data/100_199/0106.html#dummyLink2 Proprietary 23/27

Page 24: Prior Authorization Review Panel MCO Policy …...B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis of Sjögren’s syndrome. 1. Frequency of

62. 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.

63. American Institute for Ultrasound in Medicine (AIUM). AIUM practice guideline for the

performance of fetal echocardiography. Laurel, MD: AIUM; 2010. Available at:

https://www.smfm.org/attachedfiles/fetalEchoaiumsmfm.pdf. AccessedOctober25,

2010.

64. Friedberg MK, Silverman NH. Changing indications for fetal echocardiography in a

University Center population. Prenat Diagn. 2004;24(10):781-786.

65. ACOG Committee on Practice Bulletins. ACOG Practice Bulletin No. 77: Screening for

fetal chromosomal abnormalities. Obstet Gynecol.2007;109(1):217-227.

66. 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.

67. 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.

68. Royal College of Obstetricians and Gynaecologists (RCOG). Management of

monochorionic twin pregnancy. London, UK: Royal College of Obstetricians and

Gynaecologists (RCOG); December 2008.

69. Wilson KL, Czerwinski JL, Hoskovec JM, et al. NSGC practice guideline: Prenatal

screening and diagnostic testing options for chromosome aneuploidy. J Genet Couns.

2013;22(1):4-15.

70. Desilets V, Audibert F; Society of Obstetrician and Gynaecologists of Canada.

Investigation and management of non-immune fetal hydrops. J Obstet Gynaecol Can.

2013;35(10):923-938.

71. Zhang YF, Zeng XL, Zhao EF, Lu HW. Diagnostic value of fetal echocardiography for

congenital heart disease: A systematic review and meta-analysis. Medicine (Baltimore).

2015;94(42):e1759.

72. Copel J. Fetal cardiac abnormalities: Screening, evaluation, and pregnancy

management. UpToDate [online serial]. Waltham, MA: UpToDate; reviewed December

2015.

73. Gomez O, Soveral I, Bennasar M, et al. Accuracy of fetal echocardiography in the

differential diagnosis between truncus arteriosus and pulmonary atresia with

ventricular septal defect. Fetal Diagn Ther. 2016;39(2):90-99.

74. Society for Maternal-Fetal Medicine (SMFM), Norton ME, Chauhan SP, Dashe JS. Society

for maternal-fetal medicine (SMFM) clinical guideline #7: Nonimmune hydrops fetalis.

Am J Obstet Gynecol. 2015;212(2):127-139.

www.aetna.com/cpb/medical/data/100_199/0106.html#dummyLink2 Proprietary 24/27

Page 25: Prior Authorization Review Panel MCO Policy …...B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis of Sjögren’s syndrome. 1. Frequency of

75. Reefhuis J, Devine O, Friedman JM, etal; and the National Birth Defects Prevention

Study. Specific SSRIs and birth defects: Bayesian analysis to interpret new data in the

context of previous reports. BMJ. 2015;351:h3190.

76. Hrtankova M, Biringer K, Sivakova J, et al. Fetal magnetocardiography: A promising way

to diagnose fetal arrhytmia and to study fetal heart rate variability?. Ceska Gynekol.

2015;80(1):58-63.

77. Eswaran H, Escalona-Vargas D, Bolin EH, et al. Fetal magnetocardiography using

optically pumped magnetometers: A more adaptable and less expensive alternative?

Prenat Diagn. 2017;37(2):193-196.

78. Levine JC, Alexander ME. Overview of the general approach to diagnosis and treatment

of fetal arrhythmias. UpToDate [online serial]. Waltham, MA: UpToDate; reviewed May

2017.

79. American Heart Association (AHA). Fetal echocardiogram test. Dallas, TX: AHA; 2018.

Available at: http://www.heart.org/en/health-topics/congenital-heart­

defects/symptoms--diagnosis-of-congenital-heart-defects/fetal-echocardiogram-test.

Accessed August 28, 2018.

80. American Institute for Ultrasound in Medicine (AIUM). AIUM practice parameter for the

performance of fetal echocardiography. Laurel, MD: AIUM; 2013. Available at:

https://www.aium.org/resources/guidelines/fetalecho.pdf. Accessed August 28, 2018.

81. Hutchinson D, McBrien A, Howley L, et al. First-trimester fetal echocardiography:

Identification of cardiac structures for screening from 6 to 13 Weeks' Gestational Age. J

Am Soc Echocardiogr. 2017;30(8):763-772.

82. Ventriglia F, Caiaro A, Giancotti A. et al. Reliability of early fetal echocardiography for

congenital heart disease detection: A preliminary experience and outcome analysis of

102 fetuses to demonstrate the value of a clinical flow-chart designed for at-risk

pregnancy management. Pediatrics & Therapeutics. 2016;6:270.

83. Hill LM, Mills A, Peterson C, Boyles D. Persistent right umbilical vein: Sonographic

detection and subsequent neonatal outcome. Obstet Gynecol. 1994;84(6):923-925.

84. Wolman I, Gull I, Fait G, et al. Persistent right umbilical vein: Incidence and significance.

Ultrasound Obstet Gynecol. 2002;19(6):562-564.

85. Martínez R, Gamez F, de Leon-Luis J, et al. Perinatal outcomes after prenatal ultrasound

diagnosis of persistence of right umbilical vein. Ginecol Obstet Mex. 2012;80(2):73-78.

86. Lide B, Lindsley W, Foster MJ, et al. Intrahepatic persistent right umbilical vein and

associated outcomes: A systematic review of the literature. J Ultrasound Med.

2016;35(1):1-5.

87. Canavan TP, Hill LM. Neonatal outcomes in fetuses with a persistent intrahepatic right

umbilical vein. J Ultrasound Med. 2016;35(10):2237-2241.

88. Kumar SV, Chandra V, Balakrishnan B, et al. A retrospective single centre review of the

incidence and prognostic significance of persistent foetal right umbilical vein. J Obstet

www.aetna.com/cpb/medical/data/100_199/0106.html#dummyLink2 Proprietary 25/27

Page 26: Prior Authorization Review Panel MCO Policy …...B. Fetal surveillance (e.g., congenital heart block) in mother with documented diagnosis of Sjögren’s syndrome. 1. Frequency of

Gynaecol. 2016;36(8):1050-1055.

89. Spurway J, Logan P, Pak S. The development, structure and blood flow within the

umbilical cord with particular reference to the venous system. Australasian Journal of

Ultrasound in Medicine. 2012;15(3):97-102.

90. Donofrio MT, Moon-Grady AJ, Hornberger LK et al. Diagnosis and treatment of fetal

cardiac disease: A scientific statement from the American Heart Association.

Circulation. 2014;129(21):2183-242.

91. eviCore Healthcare. OB ultrasound imaging policy. Clinical Guidelines, Version

20.0.2018. Bluffton, SC: eviCore; May 17, 2018.

92. Gupta S, Gupta N. Sjögren syndrome and pregnancy: A literature review. Perm J.

2017;21:16-047.

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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.

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AETNA BETTER HEALTH® OF PENNSYLVANIA

Amendment to Aetna Clinical PolicyBulletin Number: 0106 Fetal

Echocardiography and Magnetocardiography

There are no amendments for Medicaid.

www.aetnabetterhealth.com/pennsylvania annual 11/01/2019

Proprietary