Situs inversus is a rare congenital anomaly reported to occur in 1 in 8000 to 1 in 25,000 patients [
3]. No racial predilection exists for situs inversus. The male-to-female incidence is 1:1. The arrangements of the position of the abdominal viscera in dextrocardia may be normal (situs solitus), reversed (situs inversus), and indeterminate (situs ambiguous or isomerism) in 32 to 35%, 35 to 39% and 26 to 28% of cases respectively [
4].
In our patient situs inversus was associated with asplenia and dextrocardia. Cardiac anomalies identified on echocardiography were ASD, VSD, AR, PS and TGA. Both her kidneys were normal. In the vascular anomaly IVC and aorta were both on her right side. Dextrocardia with a normal abdominal situs has a high incidence of associated congenital cardiac anomalies including among others, transposition of the great vessels and ASDs [
5] and VSDs [
6] in 90 to 95% of cases. However, dextrocardia with situs inversus is associated with a lower incidence of congenital heart disease (0 to 10%) as was the case in our patient. Presentation of cause varies depending on associated malformation [
1‐
3]. Situs inversus may be associated with other congenital anomalies such as duodenal atresia, asplenism, multiple spleens, ectopic kidney, horseshoe kidney and various pulmonary and vascular abnormalities. Situs inversus totalis that is associated with primary ciliary dyskinesia is known as Kartagener syndrome [
7,
8]. Patients with primary ciliary dyskinesia have repeated sinus and pulmonary infections [
7,
9]. Frequent pulmonary infections often result in bronchiectasis, which predominantly affects the lower lungs. Typically, persons having situs inversus with dextrocardia without other congenital anomaly have a normal life expectancy and have a similar risk of getting acquired disease as that of other persons of the same age and sex group. In the rare instances of cardiac anomalies, life expectancy is reduced, depending on the severity of the defect [
10]. The recognition of situs inversus is also important for preventing surgical mishaps that result from the failure to recognize reversed anatomy or an atypical history. For example, in a patient with situs inversus, cholecystitis typically causes left upper quadrant pain, and appendicitis causes left lower quadrant pain. Cardiac situs is determined by the atrial location. In situs inversus, the morphologic right atrium is on the left, and the morphologic left atrium is on the right. The normal pulmonary anatomy is also reversed so that the left lung has three lobes and the right lung has two lobes. In addition, the liver and gallbladder are located on the left, whereas the spleen and stomach are located on the right. The remaining internal structures are also a mirror image of the normal. In a study of 111 cases, Merklin and Varano classified cases of situs inversus into: (a) complete situs inversus; (b) dextrocardia with situs solitus; (c) partial situs inversus; (d) dextroposition of the heart; and (e) levocardia [
11]. Although the exact cause is unknown, dextrocardia has been linked with several factors including autosomal recessive gene with incomplete penetrance, maternal diabetes, cocaine use, and conjoined twinning [
12‐
14]. Diagnosis of dextrocardia is usually confirmed by several modalities which include chest radiography, ECG, echocardiography, computed tomography, magnetic resonance imaging and abdominal ultrasonography. Echocardiography is one of the modalities for making the diagnosis. Of interest, this patient had situs inversus totalis with multiple cardiac lesions ASD, VSD, AR, PS and TGA. This case is reported because of the situs inversus, dextrocardia and asplenia with early symptomatic presentation due to complex pattern of cardiac malformation.