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.
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.
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.
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).
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.
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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.
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.
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%.
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%).
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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).
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.
Conflict of interest: None declared.
Accepted Date/Kabul Tarihi: 01.09.2010 Available Online Date Cevrimici Yayin Tarihi: 08.02.2011
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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: email@example.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|
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