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Dergi Kimliği

Online ISSN (İngilizce)
1305-3124

Basılı ISSN (Türkçe)
1300-5251

Online ISSN (Türkçe)
1305-3132

Kuruluş
1993

Editör
Cihat Şen

Yardımcı Editörler
Murat Yayla, Oluş Api

Isolated levocardia with situs inversus without cardiac abnormality in fetus: Prenatal diagnosis and management

Mucize Eriç Özdemir, Oya Demirci

Künye

Isolated levocardia with situs inversus without cardiac abnormality in fetus: Prenatal diagnosis and management. Perinatoloji Dergisi 2020;28(0):- DOI: 10.2399/prn.20.0281004

Yazar Bilgileri

Mucize Eriç Özdemir,
Oya Demirci

  1. Health Science University, Zeynep Kamil Women and Children's Diseases Training and Research Hospital Perinatology Department Istanbul TR
Yazışma Adresi

Mucize Eriç Özdemir, Health Science University, Zeynep Kamil Women and Children's Diseases Training and Research Hospital Perinatology Department Istanbul TR, ozdemir.mucize@gmail.com

Yayın Geçmişi

Gönderilme Tarihi: 15 Ocak 2020

Kabul Edilme Tarihi: 17 Şubat 2020

Erken Baskı Tarihi: 17 Şubat 2020

Çıkar Çakışması

Çıkar çakışması bulunmadığı belirtilmiştir.

Amaç
Isolated levocardia is a situs abnormality that the heart is in the normal levo position, but the abdominal viscera are in dextro position. Most cases are accompanied by structural heart anomalies. In this case, we aimed to present a fetus with isolated levocardia without cardiac abnormality. 
 
Olgu
The mother was referred to our clinic with a suspicion of fetal dextrocardia at 22 weeks of gestation. When detailed examination was planned by ultrasonography isolated levocardia was detected in fetus. There were no cardiac abnormalities in fetal echocardiography. Fetus was followed up until delivery and newborn was examined again at postnatal period. No problem was detected until the age of 2 years. 
Sonuç
Fetal situs should be assessed by ultrasonography in all pregnant women. 
Anahtar Kelimeler

Fetus, levocardia, ultrasonography

Giriş
Isolated levocardia (IL) is a kind of situs anomaly that the heart is in the normal levo position, but the abdominal viscera are in dextro position [1]. The vast majority of the cases have major cardiac anomalies [2]. This condition is rare when it is associated with a normal cardiac structure [3] and the incidence of this anomaly in fetuses is not known [1]. Most of IL patients who do not have cardiac and intestinal abnormalities survive unwittingly. So, the prenatal incidence of IL is difficult to estimate [4]. According to reports, the incidence of IL is approximately 1 per 22,000 births [5], and it represents 0.4-1.2 % of all congenital heart disease (CHD) [6]. In this article, we report a case of IL with structurally normal heart and no rhythm abnormalities.
 
Olgu
A 28-year-old woman gravida 3 para 2 with no family history of congenital anomalies, having consanguineous marriage and a normal medical history had routine prenatal ultrasound with suspicion of dextrocardia. The patient rejected to have any of aneuploidy screening tests. The patient was referred for fetal echocardiography at 22 weeks of gestation. On ultrasound, the fetus had a left-sided heart and a right-sided stomach (Figure 1). The gallbladder was located at right side to the umbilical vein in the midline. Fetal echocardiography showed situs solitus of the atria, the ventricles, and the main arteries. The inferior vena cava (IVC) and aorta were both on the left, and the main portal vein was on the right side. The IVC runs on the left side, returns to the anterior right side of the diaphragm (Figure 2). Portal venous structures and ductus venosus were normal. Sonographic evaluation of the fetus revealed no additional abnormalities. With these findings, the case was considered as IL. Amniocentesis showed normal male karyotype. At 38 weeks of gestation, a planned cesarean section was performed secondary to fetal macrosomia. A male infant was born with a birth weight of 4,100 g and an Apgar score of 9 at 1 min and 8 at 5 min. The neonate’s electrocardiogram showed normal sinus rhythm and postnatal echocardiography confirmed the prenatal diagnosis. Abdominal ultrasonography of the baby after birth revealed right-sided spleen and stomach and confirmed left-sided IVC. The baby was in good condition and oral feeding was started. The boy was stable and discharged home after a week. He was regularly followed up by a pediatrician and there were no major problems recorded with his health at 2 years.
 
Tartışma
The fetal side and axis must be examined carefully for diagnosis of cardiac malpositions. Ozkutlu et al. recommended that Cordes technique provides a simple determination of the fetal situs [7]. Situs anomalies are the least common forms of CHD [8].  ‘Situs’ defines the arrangement of the viscera, atria, and vessels within the body. ‘Situs solitus’ refers to normal arrangement; ‘situs inversus’ refers to inverted arrangement; and situs ambiguous (heterotaxy) refers to abnormalities in arrangements that can neither be described as solitus nor inversus [9]. Univentricular physiology, transposition of the great arteries were more common in fetuses with levocardia, whereas abnormal pulmonary venous connection, double outlet of right ventricle, left ventricle outlet obstruction were more frequent in fetuses with dextrocardia [10]. IL is a subtype of situs inversus that was first defined by Taussing in 1947 [11].  Then, Van Praagh et al. [2] classified this condition as a partial situs inversus in which the heart remains in levocardia. In 95% of the cases, major cardiac anomalies [2] are detected and only 5-13 % of the cases survive for longer than 5 years [12,13].  IL is one of the heterotaxy syndromes and should be carefully evaluated in terms of associated organ anomalies. Splenic abnormalities like asplenia and polysplenia are usually encountered with this anomaly [2]. Vaccination against Streptococcus pneumonia and Neisseria meningitidis and daily antibiotic prophylaxis can be suggested for asplenic patients to prevent serious infection[9].  In some cases, IL may be complicated by an intestinal malrotation and intestinal obstruction due to peritoneal band, intraluminal web and an aberrant vessel, commonly the superior mesenteric artery. These cases cannot be detected for years until an abdominal surgery [14,15]. Interrupted inferior vena cava (IVC) with azygous continuation can be detected in cases of IL [16] and intestinal malrotation is generally related to interrupted IVC [3].  Also, double inferior vena cava (DIVC) has been reported in some IL cases.  DIVC may be important for a retroperitoneal surgery and management of a thromboembolic disease [17].  Rhythm abnormalities such as atrioventricular node re-entry tachycardia and sick sinus syndrome are reported in cases of IL. Therefore, long-term monitorization is recommended for these patients against new-onset arrhythmias [18].  Since there were no asplenia detected in our case, the neonatologist did not recommend vaccination and daily antibiotherapy. In the neonatal period there was no sign of primary ciliary dyskinesia and the change in respiratory sounds was associated with gastroesophageal reflux. Findings of intestinal malrotation and obstruction were not observed, there was no pathology associated with IVC. Cardiac arrhythmia did not develop during the neonatal period and subsequent follow-ups. 
The pathogenesis of IL is not entirely clear but it is associated with the abnormalities in the embryological development process. Genetic studies do not define a specific gene mutation, but there are studies investigating the primer ciliary dyskinesia (PCD) gene. The lack of the PCD gene is thought to be resulting in IL when it is absent in the regions associated with the development of abdominal visceral organs [4].  Heterotaxic syndromes are more common in diabetic pregnancies, first trimester cocaine use, and in those with family history of CHD [19].  In our case there were no risk factors underlying IL. Prognosis of IL mostly depends on the severity of associated cardiac anomalies [9]. In addition, intestinal malrotation and associated obstructions may be life-threatening, requiring immediate surgical intervention [4].  The IL patients with no CHD and no intestinal malrotation are considered healthy and may not be diagnosed until later ages. It is important to diagnose fetuses with IL prenatally. Although accompanying no cardiac anomalies, it is important to follow the neonate carefully for the complications like intestinal malrotation and bowel obstruction may develop during postnatal period. Families with the baby prenatally diagnosed with IL syndrome can be better informed about the prognosis. In prenatally suspected cases, a more comprehensive examination can be performed with postnatal MR (magnetic resonance imaging). MR provides more detailed information about the anatomy of the abdominal organs and provides a more detailed examination of bowel problems [20]. Newborns with IL should be followed-up closely especially after feeding. The families of uncomplicated cases should be also warned in terms of gastrointestinal pathologies and cardiac arrhythmias that may develop in future. For the abdominal surgeries that may be encountered in the future, it is important to inform the surgical team about the anatomy in advance.
 
Sonuç
In the absence of associated cardiac anomaly and chromosomal abnormality, IL cases have low risk for morbidity and mortality. Anatomical investigation and careful observation may improve the outcome of IL.
 
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Dosya / Açıklama
Figure 1
On ultrasound, the fetus has a left appearing heart and a right-sided stomach.
Figure 2
The vena cava inferior (VCI) returns to the anterior right side of the diaphragm.