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Prenatal echocardiographic diagnosis of cardiac right/left axis and malpositions according to standardized Cordes technique/Standart Cordes teknigine gore kalbin sag/sol ekseninin ve malpozisyonlarinin prenatal ekokardiyografik tanisi.

Introduction

Fetal cardiac malpositions are difficult to diagnose in routine screening ultrasound. Determining the fetal right/left axis is essential for the diagnosis. Constantly variable fetal position within the uterus can confuse distinguishing the right/left side of the fetus. Variability among echocardiographers regarding image acquisition adds to this confusion. If there is confusion regarding the right/left axis, then atrial and visceral situs, cardiac position and cardiac segmental anatomy cannot be evaluated correctly. There are some recommended techniques and images required for a standard fetal echocardiogram (1-6).

In this study, the 20 cases among 1536 cases diagnosed as cardiac malposition using standardized technique recommended by Cordes et al. (2) for assignment of fetal right / left axis were evaluated. The accuracy of prenatal diagnosis was compared with postnatal echocardiographic diagnosis and autopsy findings.

Methods

Study patients

We studied retrospectively 1536 cases whose fetal echocardiographic examinations were performed between 1999 and 2006 in prenatal cardiology unit. Among these, cardiac malposition was determined in 20 cases and these cases performed our study group. These cases were referred to our center either from the departments of obstetrics and gynecology of our institution or from other centers by obstetricians or by pediatric cardiologist. Some were siblings of our patients with congenital heart defects and fetuses of mothers with congenital heart defects. Data of 20 cases with cardiac malposition were evaluated in respect to gestational week, maternal age, maternal and familial medical histories, previous obstetric history, fetal and postnatal echocardiographic examinations, and autopsy findings.

Fetal echocardiography

The prenatal and postnatal echocardiographic examinations were performed using a Trinitron GE Vivid Five performance echocardiographic scanner with 2.5-5 MHz transducers (Cardiovascular Ultrasound Systems, General Electric, Horten, Norway). All echocardiographic examinations were performed by the same pediatric cardiologist and all studies were recorded on videotape. The fetal examination included the standard positions used in fetal heart scanning technique (7). The cardiac axis and position were determined according to the technique described by Cordes et al. (2).

Cordes technique

In this technique the fetal head and sagittal plane of fetal body are then located. The transducer is oriented so that it is parallel to the fetal sagittal plane, with the fetal head on the right side of the video screen. When the transducer is aligned parallel to the fetal cranial-caudal axis this way, the side of the transducer toward the fetal head can be designated the "top" of the transducer. The transducer is then rotated clockwise (from the perspective of the echocardiographer) 90 degrees, to visualize optimally a transverse image of the fetal thorax. In this transverse image, the fetus' left side is on the right of the video screen, and the fetus' right side is on the left of the video screen (Fig.1)(2).

A systematic approach was used based on segmental anatomy (8, 9). Viscero-atrial situs was determined as situs solitus, inversus, or ambiguous. In this study, the type of cardiac malposition was determined by the cardiac base-apex axis as dextrocardia, mesocardia and levocardia.

[FIGURE 1 OMITTED]

Situs solitus was defined as liver on the right side and the stomach on the left side, with the right inferior caval vein and the superior caval vein connecting to the systemic right atrium on the right side. Situs inversus was defined as a mirror-image configuration so that the liver was on the left side and the stomach was on the right, with the left inferior caval vein and superior connecting to the systemic right atrium on the left. Situs ambiguous was defined as the liver on the midline position and indeterminate stomach situs, abnormal inferior caval vein connection, or relations with the descending aorta. Situs ambiguous was divided into two major subtypes: left atrial isomerism and right atrial isomerism. These were categorized based on the following echocardiographic criteria: a diagnosis of left atrial isomerism was made if there was an interrupted inferior caval vein with azygous continuation and anterior located descending aorta according to azygous vein, and a diagnosis of right atrial isomerism was made if the inferior caval vein and aorta was both located on the right or left side of the spine in parallel anteroposterior orientation (8,9).

In this study, dextrocardia was defined as the location of the heart in the right hemithorax with the apex pointing to the right. Dextrocardia was divided into two subgroups: Isolated dextrocardia and situs inversus. Dextrocardia was defined as isolated dextrocardia, occurring in conjunction with situs solitus and situs ambiguous. Mesocardia was defined as location of the heart with the cardiac base-apex axis directed to the midline of the thorax or with ventricular apices equally directed to both right and left sides. Levocardia was defined as the location of the heart in the left hemithorax with the apex pointing to the left. Levocardia as a cardiac malposition was also defined as isolated levocardia, occurring in conjunction with situs inversus and situs ambiguous (8). In this study, the pathologic displacement of the heart into the right or left thorax by extracardiac malformations was defined as dextroposition, mesoposition and levoposition (8).

All cases were followed-up by serial fetal echocardiograms until birth or intrauterine death occurred. In cases of intrauterine death, an autopsy was performed. After birth, physical and echocardiographic examinations were done and prenatal and postnatal diagnoses were compared.

Statistical analysis

All statistical analyses were performed using the SPSS 15.0 statistical software (Chicago, IL, USA). Quantitative variables are expressed as mean [+ or -] standard deviation, and qualitative variables are given as frequency and percentage. Reliability of fetal echocardiography for diagnose of cardiac malpositions was evaluated by sensitivity and specificity formulas.

Results

Of 1536 fetal echocardiograms performed, 144 revealed congenital heart diseases (9.4%), among these cases, 20 were diagnosed with cardiac malposition. Of these cases, 16 had congenital heart disease, and 4 had extracardiac malformation (Table 1). All the other cases (124 cases) had situs solitus, levocardia and left cardiac axis between 60[degrees] and 90[degrees]. The mean gestational age and the maternal age at the time of first examination was 28[+ or -]5 weeks (range 20-37 weeks) and 30.0[+ or -]3.6 years (range 24-35 years), respectively.

The indications for referral for fetal echocardiographic examination were suspected congenital heart disease, fetal arrhythmia, and fetal hydrops on routine obstetric ultrasound. Seven of 20 cases were referred for fetal echocardiography after a preliminary diagnosis of cardiac malposition. One case, referred for fetal echocardiography after a preliminary diagnosis of dextrocardia on obstetric ultrasound and magnetic resonance imaging examination, had normal cardiac anatomy and position. Except the cases 10 and 18, maternal and familial medical histories, and previous obstetric history of all cases were unremarkable. Case 10 had a sibling with situs inversus totalis and transposition of the great arteries, and the mother of the case 18 had congenital heart disease.

Of 20 cases with cardiac malposition, 16 cases had congenital heart disease, four cases had extracardiac malformation. There were six cases of isolated dextrocardia, three cases of situs inversus totalis, and six cases of situs ambiguous, and one case of situs inversus with levocardia (Fig. 2A-B) (Table 1). Of six cases with situs ambiguous, five cases had left atrial isomerism and levocardia (Fig. 3A-B) and one case had right atrial isomerism and dextrocardia (Fig. 4). Of four cases with cardiac malposition caused by extracardiac congenital malformation,two cases had mesoposition dueto pleural effusion, one case had dextroposition due to left-sided congenital diaphragmatic hernia, and one case had an extreme levoposition due to cystic adenomatoid malformation in the right lung (Table 1). All of them had normal cardiac anatomy.

In follow-up, in six cases (case 10, 11, 13, 14, 16, and 19) pregnancy was terminated (Table 1). The autopsy findings of these cases were the same as their prenatal echocardiographic findings. The remaining cases were born and have survived the neonatal period.

On postnatal echocardiographic examination these cases had the same echocardiographic findings with prenatal diagnosis. There were not false negative and false positive results in the cases with cardiac malposition, in terms of fetal echocardiographic diagnosis. The sensitivity of the prenatal echocardiographic examination in diagnosing cardiac malpositions was calculated as 100% and specificity was 100%.

Discussion

Recently, advances in ultrasound technology and increased experience in fetal echocardiography have led to increased sensitivity and specificity of fetal echocardiography in the accuracy of diagnose of congenital heart disease (10-12). However, the prenatal diagnosis of cardiac malpositions is difficult. Therefore, cardiac malpositions are usually diagnosed by postnatal echocardiographic examination or autopsy. The current methods to distinguish the right side of the fetus from the left side on the transabdominal ultrasound examination rely on several parameters including maternal position, fetal position and transducer orientation. In present study, visceroatrial situs, cardiac position and cardiac segmental anatomy were evaluated according to the technique described by Cordes et al. (2). Since 1998, in our prenatal cardiology unit this technique has been preferred because it is easily applied, and it reduces confusion significantly relating to fetal right/left axis. This study using Cordes technique showed that there was no difference between the type of cardiac malposition diagnosed prenatally and post-natally (sensitivity and specificity 100%).

[FIGURE 2 OMITTED]

[FIGURE 3 OMITTED]

[FIGURE 4 OMITTED]

There are a few published studies about malpositions (13-16). Walmsley et al. (16) retrospectively reviewed the fetal echocardiographic diagnosis of dextrocardia in large series. In this study, 85 cases of dextrocardia were diagnosed from 5539 fetal echocardiograms and thirty-three of these cases had been referred for fetal echocardiography after a preliminary diagnosis of dextrocardia on routine ultrasound examination. In present study, only seven of 20 cases were referred for fetal echocardiography after diagnosis of cardiac malposition on routine obstetric ultrasound, and the cases whose preliminary diagnosis of dextrocardia on obstetric ultrasound and magnetic resonance imaging had normal cardiac anatomy and position on pre-postnatal echocardiogram. These studies have shown that fetal cardiac malpositions are difficult to diagnose on routine obstetric ultrasound.

Fetal cardiac malposition is caused by either intrinsic congenital heart diseases or extracardiac malformations. Generally, intrinsic congenital heart diseases cause abnormal cardiac axis (17, 18). In addition, extracardiac malformations are often responsible for the abnormal location of the heart in the thorax due to mediastinal shift (19). In our study among 20 cases with cardiac malposition, 16 had congenital heart disease, and four had extracardiac malformation.

Most of the cases with congenital heart disease had abnormal cardiac axis. Shipp et al. (20) reported that 44% of fetal heart defects were associated with left-sided heart greater than 57 degrees. Smith et al. (17) also reported that left-sided heart greater than 75 degrees correlated with a positive predictive value of 76% for a heart defect in fetuses. In most of our cases with levocardia and dextrocardia, cardiac axis was greater than 57 degrees (Table 1).

Of six cases with situs ambiguous, five had left atrial isomerism and one right atrial isomerism. This study demonstrated significant predominance of fetuses diagnosed with left atrial isomerism. Some studies have noted that fetuses with left atrial isomerism appear to be more common in utero because of an increased rate of very early death of fetuses with right atrial isomerism (15). All cases with situs ambiguoushad severe complex congenital heart disease. It was stated that the cardiac anomalies in right atrial isomerism tend to be more severe than those in left atrial isomerism (18). This study also indicated that the prognosis of cases with left atrial isomerism was better than case with right atrial isomerism.

Of four cases with cardiac malposition due to extracardiac anomalies, one had congenital diaphragmatic hernia and other had cystic adenomatoid malformation. Although previous studies were reported that these anomalies could be associated with cardiac defects and other anomalies, our cases had normal cardiac anatomy (18, 21,22).

Conclusion

The fetal right/left side and axis must be determined correctly for the accurate diagnosis of cardiac malpositions. Therefore, we recommend that Cordes technique provides a simple and reliable determination of the fetal right/left side and fetal situs.

doi:10.5152/akd.2011.033

Conflict of interest: None declared.

Accepted Date/Kabul Tarihi: 01.09.2010 Available Online Date Cevrimici Yayin Tarihi: 08.02.2011

References

(1.) Bronshtein M, Gover A, Zimmer EZ. Sonographic definition of the fetal situs. Obstet Gynecol 2002; 99:1129-30.

(2.) Cordes TM, O'Leary PW, Seward JB, Hagler DJ. Distinguishing right from left: a standardized technique for fetal echocardiography. J Am Soc Echocardiogr 1994; 7: 47-53.

(3.) DeVore G. The prenatal diagnosis of congenital heart disease; a practical approach for the fetal sonoggrapher. J Clin Ultrasound 1985; 13: 229-45.

(4.) Silverman NH, Golbus M. Echocardiographic techniques for assessing normal and abnormal fetal cardiac anatomy. J Am Coll Cardiol 1985; 5: 20-9.

(5.) Cyr DR, Guntheroth WG, Mack LA, Shuman WP A systematic approach to fetal echocardiography using real-time/two-dimensional sonography. J Ultrasound Med 1986; 5: 343-50.

(6.) Reed K. Fetal echocardiography. Semin Ultrasound CT MR 1991; 12:2-10.

(7.) Drose JA. Scanning: Indication and Technique. In: Drose JA, editor. Fetal Echocardiography. Philadelphia: W.B. Saunders Company; 1998. p.15-59.

(8.) Hagler DJ, O'Leary PW. Cardiac malpositions and abnormalities of atrial and visceral situs. In: Allen HD, Gutgesell HP Clark EB, Driscoll DJ (eds): Moss and Adams. Heart Disease in Infants, Children and Adolescents Including the Fetus and Young Adult, 6th ed. Philadelphia:Williams and Wilkins; 2001. p. 1151-64.

(9.) Rabih Chaoui. Cardiac malpositions and syndromes with right or left atrial isomerism. In: Yagel S, Silverman NH, Gembruch U, Cohen SM, editors. Fetal Cardiology. London and New York: Martin Dunitz; 2003. p 173-82.

(10.) Allan LD, Crawford DC, Anderson RH, Tynan M. Spectrum of congenital heart disease detected echocardiographically in prenatal life. Br Heart J 1985; 54:523-6.

(11.) Ozkutlu S, Elshershari H, Akcbren Z, Onderoglu LS, Tekinalp G. Visceroatrial situs solitus with atrioventricular alignment discordance double outlet right ventricle and superoinferior ventricles: fetal and neonatal echocardiographic findings. J Am Soc Echocardiogr 2002; 15: 749-52.

(12.) Ozkutlu S, Ayabakan C, KaragbzT, Onderoglu L, Deren 0, Caglar M, et al. Prenatal echocardiographic diagnosis of congenital heart disease: comparison of past and current results. Turk J Pediatr 2005; 47: 232-8.

(13.) Allan LD, Sharland GK, Milburn A, Lochart SM, Groves AM, Anderson RH, et al. Prospective diagnosis of 1.006 consecutive cases of congenital heart disease in the fetus. J Am Coll Cardiol 1994; 23: 1452-8.

(14.) Comstock CH, Smith R, Lee W, Kirk JS. Right fetal cardiac axis: clinical significance and associated findings. Obstet Gynecol 1998;91:495-9.

(15.) Phoon CK, Villegas MD, Ursell PC, Silverman NH. Left atrial isomerism detected in fetal life. Am J Cardiol 1996; 77: 1083-8.

(16.) Walmsley R, Hishitani T, Sandor GG, Lim K, Duncan W, Tessier F, et al. Diagnosis and outcome of dextrocardia diagnosed in the fetus. Am J Cardiol 2004; 94:141-3.

(17.) Smith RS, Comstock CH, Kirk JS, Lee W. Ultrasonographic left cardiac axis deviation: a marker for fetal anomalies. Obstet Gynecol 1995; 85:187-91.

(18.) Russ DR, Weingard JP Cardiac Malposition. In: Drose JA (ed). Fetal Echocardiography. Philadelphia: WB. Saunders Company;1998. p. 59-76.

(19.) Allan LD, Lockhart S. Intrathoracic cardiac position in the fetus. Ultrasound Obstet Gynecol 1993; 3: 93-6.

(20.) Shipp TD, Bromley B, Hornberger LK, Nadel A, Benacerraf BR. Levorotation of the fetal cardiac axis: a clue for the presence of congenital heart disease. Obstet Gynecol 1995; 85: 97-102.

(21.) Cunniff C, Jones KL, Jones MC. Patterns of malformation in children with congenital diaphragmatic defects. J Pediatr 1990; 116: 258-61.

(22.) Adzick NS, Harrison MR, Glick PL, Golbus MS, Anderson RL, Mahony BS, et al. Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg 1985; 20: 483-8.

Suheyla Ozkutlu, Ozlem Mehtap Bostan (1), Ozgur Deren *, Lutfu Onderoglu *, Gulsev Kale **, Safak Gucef *, Diclehan Orhan *

From Departments of Pediatric Cardiology, * Obstetrics and Gynecology and ** Pediatric Pathology; Faculty of Medicine, Hacettepe University, Ankara, 'Department of Pediatric Cardiology, Faculty of Medicine, Uludag University, Bursa, Turkey

Address for Correspondence/Yazisma Adresi: Dr. Siiheyla Ozkutlu, Department of Pediatric Cardiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey Phone: +90 312 305 11 57-58 Fax: +90 312 309 02 20 E-mail: suheylaozkutlu@yahoo.com This work was presented at the Second Annual Congress on Update in Cardiology and Cardiovascular Surgery, 20-24 September2006, Bodrum/Turkey
Table 1. Diagnoses and features of the cases with cardiac malposition

           Gestational
             age at
          presentation,        Visceroatrial          Cardiac apex
Cases         weeks                situs              orientation

Case 1         27                Ambiguous              Isolated
                          (left atrial isomerism)      Levocardia

Case 2         30                Ambiguous              Isolated
                          (left atrial isomerism)      Levocardia

Case 3         32                Ambiguous              Isolated
                          (left atrial isomerism)      Levocardia

Case 4         28                Ambiguous              Isolated
                          (left atrial isomerism)      Levocardia

Case 5         30                 Inversus              Isolated
                                                       Levocardia

Case 6         37                Ambiguous            Dextrocardia
                          (right atrial isomerism)

Case 7         34                 Solitus               Isolated
                                                      Dextrocardia

Case 8         23                 Solitus               Isolated
                                                      Dextrocardia

Case 9         21                Ambiguous              Isolated
                          (left atrial isomerism)      Levocardia

Case 10        24                 Inversus            Dextrocardia

Case 11        24                 Solitus               Isolated
                                                      Dextrocardia

Case 12        34                 Inversus            Dextrocardia

Case 13        19                 Solitus               Isolated
                                                      Dextrocardia

Case 14        18                 Solitus               Isolated
                                                      Dextrocardia


Case 15        37                 Inversus            Dextrocardia

Case 16        24                 Solitus               Isolated
                                                      Dextrocardia

Case 17        22                 Solitus             Mesoposition

Case 18        33                 Solitus            Dextroposition

Case 19        24                 Solitus               Extreme
                                                      Levoposition

Case 20        20                 Solitus             Mesoposition

             Cardiac                 Congenital
Cases         Axis                  heart disease

Case 1      Left axis                   AVSD
          77 [degrees]                  DORV
                                         PS
                            hemiazygous vein continuation

Case 2      Left axis                   AVSD
          75 [degrees]        azygous vein continuation

Case 3      Left axis                 Sup.lnf.
          65 [degrees]                Ventricle
                                       VSD+ASD
                           Ventriculo-arterial discordance
                              azygous vein continuation

Case 4      Left axis                   DORV
           70[degrees]               Subpulmonic
                                         VSD
                                         PS
                            hemiazygous vein continuation

Case 5      Left axis                   DIRV
           80[degrees]            Pulmonary atresia
                                         ASD
                                        MAPCA

Case 6     Right axis       Common inlet right ventricle
           75[degrees]      Large primum and secundum ASD
                          (common atrium), MGA+Hypoplastic
                                  pulmonary artery

Case 7     Right axis                   DORV
           60[degrees]                   VSD

Case 8     Right axis                Primum ASD
           60[degrees]      Common inlet single ventricle
                                         MGA

Case 9      Left axis                    VSD
           72[degrees]            Sinus venosus ASD
                              azygous vein continuation

Case 10    Right axis                    TGA
           68[degrees]                   VSD

Case 11    Right axis       Common inlet single ventricle
           60[degrees]              Common atrium

Case 12    Right axis                    VSD
           70[degrees]

Case 13    Right axis                Primum ASD
           60[degrees]         Dimunitive RV+lnlet VSD
                                  (small)+INCVM+PA

Case 14    Right axis                Outlet VSD
           60[degrees]

Case 15    Right axis                Primum ASD
           58[degrees]            Common ventricle

Case 16    Riqht axis                Primum ASD
           30[degrees]            Common ventricle

Case 17    0[degrees]                     -

Case 18     Left axis                     -
           45[degrees]

Case 19     Left axis
           30[degrees]

Case 20    0[degrees]                     -

            Extracardiac
Cases         anomalies          Outcome

Case 1            -               Alive,
                               Following-up

Case 2            -               Alive,
                               Following-up

Case 3                            Alive,
                               Followinq-up

Case 4            -               Alive,
                               Following-up

Case 5            -               Alive,
                               Following-up

Case 6                         Intrauterine
                               fetal death
                               and autopsy
                              was performed

Case 7            -               Alive,
                               Following-up

Case 8            _               Alive,
                               Following-up

Case 9                            Alive,
                               Following-up

Case 10                         Pregnancy
                               termination
                               and autopsy

Case 11           -             Pregnancy
                               termination
                               and autopsy

Case 12           -               Alive,
                               Following-up

Case 13           -             Pregnancy
                               termination
                               and autopsy

Case 14     Gastroschisis       Pregnancy
                               termination
                               and autopsy

Case 15           _               Alive,
                               Following-up

Case 16           _             Preqnancy
                               termination
                               and autopsy

Case 17   Pleural effusion        Alive,
                               Following-up

Case 18     Diaphragmatic       Repair of
               hernia          diaphragmati
                               c hernia and
                                  alive

Case 19        Cystic           Pregnancy
             adenomatoid       termination
            malformation       and autopsy

Case 20   Pleural effusion        Alive
                               Following-up

ASD - atrial septal defect, AVSD - atrioventricular septal defect,
DIRV - double inlet right ventricle, DORV - double-outlet right
ventricle, INCVM - Isolated non-compaction of the ventricular
myocardium, MAPCA - multiple aortopulmonary collateral arteries,
MGA - malposition of the great arteries, PA - pulmonary atresia,
PS - pulmonary stenosis, RV - right ventricle, Sup.
Inf - Superioinferior, TGA - Transposition of the great arteries,
VSD - ventricular septal defect
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Title Annotation:Original Investigation/Ozgun Arastirma
Author:Ozkutlu, Suheyla; Bostan, Ozlem Mehtap; Deren, Ozgur; Onderoglu, Lutfu; Kale, Gulsev; Gucer, Safak;
Publication:The Anatolian Journal of Cardiology (Anadolu Kardiyoloji Dergisi)
Date:Mar 1, 2011
Words:3234
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