eMedicine Specialties > Radiology > Pediatrics

Situs Inversus

Annamaria Wilhelm, MD, Staff Physician, Department of Radiology, Mayo Clinic
John M Holbert, MD, Professor, Department of Radiology, Scott and White Memorial Hospital and Clinic

Updated: Feb 24, 2009

Introduction

Background

Marco Severino first recognized dextrocardia in 1643. More than a century later, Matthew Baillie described the complete mirror-image reversal of the thoracic and abdominal organs in situs inversus.

Schematic drawings illustrate the standard anatomy of situs solitus (A) and the mirror image of situs inversus (B). The right lung (RL), left lung (LL), right atrium (RA), and left atrium (LA) are shown.

Pathophysiology

Situs describes the position of the cardiac atria and viscera.^{1,2,3,4,5,6,7,8} Situs solitus is the normal position, and situs inversus is the mirror image of situs solitus (see Image 1). 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 3 lobes and the right lung has 2 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.

Schematic drawings illustrate the standard anatomy of situs solitus (A) and the mirror image of situs inversus (B). The right lung (RL), left lung (LL), right atrium (RA), and left atrium (LA) are shown.

When situs cannot be determined, the patient has situs ambiguous or heterotaxy.^{1,2,3,4,5,6,7,8} In these patients, the liver may be midline, the spleen absent or multiple, the atrial morphology unclear, and the bowel malrotated. Often, normally unilateral structures are duplicated or absent. The 2 primary subtypes of situs ambiguous include the following^{1,2,3,4,5,6,7} : (1) right isomerism, or asplenia syndrome, and (2) left isomerism, or polysplenia syndrome.

In classic right isomerism, or asplenia, bilateral right-sidedness occurs. These patients have bilateral right atria, a centrally located liver, and an absent spleen, and both lungs have 3 lobes. The descending aorta and inferior vena cava are on the same side of the spine. In left isomerism, or polysplenia, bilateral left-sidedness occurs. These patients have bilateral left atria and multiple spleens, and both lungs have 2 lobes. Interruption of the inferior vena cava with azygous or hemiazygous continuation is often present.

The features of situs ambiguous are inconsistent; therefore, situs ambiguous cases are challenging and require thorough evaluation of the viscera.^{1,2,3,4,5,6,7,9} The location and relationships of the following should be reviewed carefully: abdominal viscera, hepatic veins, superior vena cava, inferior vena cava, coronary sinus, pulmonary veins, cardiac atria, atrioventricular connections and valves, cardiac ventricles, position of the cardiac apex, and aortic arch and great vessels.

Frequency

United States

Situs inversus is present in 0.01% of the population.

Mortality/Morbidity

Situs inversus totalis that is associated with primary ciliary dyskinesia is known as Kartagener syndrome.^{10,11,12,13,14} Patients with primary ciliary dyskinesia have repeated sinus and pulmonary infections.^10,15,16 Frequent pulmonary infections often result in bronchiectasis, which predominantly affects the lower lungs.

Typically, patients with situs inversus have a normal life expectancy. In the rare instances of cardiac anomalies, life expectancy is reduced, depending on the severity of the defect.¹⁷ Patients with Kartagener syndrome have a normal life expectancy if the bronchiectasis is treated adequately.¹¹

Race

No racial predilection exists for situs inversus.

Sex

The male-to-female incidence is 1:1.

Age

Situs abnormalities are congenital.

Presentation

Situs inversus occurs more commonly with dextrocardia.¹⁸ A 3-5% incidence of congenital heart disease is observed in situs inversus with dextrocardia, usually with transposition of the great vessels. Of these patients, 80% have a right-sided aortic arch. Situs inversus with levocardia is rare¹⁹ ; this condition occurs in 1 case per 22,000, and it is almost always associated with congenital heart disease.^{20,21,22,23,24,25}  

Kartagener syndrome affects 20% of patients with situs inversus. Kartagener syndrome is a subgroup of disorders of primary ciliary dyskinesia in which the cilia have abnormal or no movement, most commonly because of absent or irregular dynein arms. The inheritance pattern of Kartagener syndrome is autosomal recessive with no male or female predominance.

Kartagener syndrome is typified by bronchiectasis, sinusitis, and situs inversus, but only 50% of patients with Kartagener syndrome have situs inversus.^10,12,13,14 In addition to this classic triad of abnormalities, patients may also have nasal polyps, an impaired sense of smell, recurrent otitis media, hearing loss, chronic respiratory infections, obstructive lung disease, reduced fertility (females), and/or infertility (males).^10,13 Each of these conditions is related to impaired cilia or flagella. Abnormal sperm motility makes affected males completely infertile. Although ciliary dyskinesia of the fallopian tubes impairs female fertility, the tubes are not obstructed, and women with Kartagener syndrome can conceive.

The recognition of situs inversus is 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. A trauma patient with evidence of external trauma over the ninth to eleventh ribs on the right side is at risk for splenic injury. If surgery is planned on the basis of radiographic findings in a patient with situs inversus, the surgeon should pay careful attention to image labeling to avoid errors such as a right thoracotomy for a left lung nodule. 

Preferred Examination

Situs abnormalities may be recognized first by using radiography or ultrasonography.^{9,26,27,28,29} However, computed tomography (CT) scanning is the preferred examination for the definitive diagnosis of situs inversus with dextrocardia. CT scanning provides good anatomic detail for confirming visceral organ position, cardiac apical position, and great vessel branching. Magnetic resonance imaging (MRI) is usually reserved for difficult cases or for patients with associated cardiac anomalies.^30,31

Limitations of Techniques

Most patients with situs inversus with levocardia require additional imaging to evaluate the associated cardiac anomalies. When radiation exposure is a concern, MRI or ultrasonography may be preferred.

Differential Diagnoses

Appendicitis: Surgical Perspective
Asplenia/Polysplenia
Congenital Coronary Abnormalities: Surgical Perspective
Sinusitis
Ventricular Septal Defect

Other Problems to Be Considered

Kartagener Syndrome

Heterotaxy (See also the eMedicine articles Heterotaxy, Asplenia and Heterotaxy, Polysplenia [in the Pediatrics section].)

Left isomerism (ie, Ivemark syndrome) (See also the eMedicine article Asplenia/Polysplenia.)

Right isomerism (ie, asplenia syndrome) (See also the eMedicine article Asplenia.)

Situs solitus

Transposition of the Great Arteries  (in the Pediatric section)

Radiography

Posteroanterior chest radiograph in a 40-year-old man with situs inversus and dextrocardia. This image shows that the cardiac apex (*) points to the right. A right-sided aortic arch (A) is associated with slight deviation of the trachea (T) to the left. The stomach (S) bubble is visible in the right upper quadrant.

Posteroanterior chest radiograph in a 55-year-old woman with Kartagener syndrome and situs inversus. This image shows a right-sided aortic arch (A) with slight leftward deviation of the trachea (T), dextrocardia (*), and a stomach bubble (S) in the right upper quadrant of the abdomen. Subtle bronchiectasis is also present in the lung bases (see Image 4 in Multimedia).

Magnified view of the left lower lobe in a 55-year-old woman with Kartagener syndrome and situs inversus (same patient as in Image 3 in Multimedia). This image shows bronchiectasis (arrows).

Radiograph of the upper abdomen from a barium enema examination in a 40-year-old man with situs inversus and dextrocardia. This image shows the liver (L) in the left upper quadrant of the abdomen. The positions of the splenic flexure (SF) and hepatic flexure (HF) are reversed. (See also Images 6-9 in Multimedia.)

Radiograph of the lower abdomen from a barium enema examination in a 40-year-old man with situs inversus and dextrocardia (same patient as in Images 5 and 7-9 in Multimedia). This image shows the sigmoid colon (SC) on the right and the cecum (C) on the left.

Findings

In most patients with situs inversus, chest radiography shows dextrocardia, with the cardiac apex pointing to the right and the aortic arch and stomach bubble located on the right as well (see Image 2). Confirming a mirror-image position of the atria allows confident diagnosis of situs inversus if the viscera are also reversed. The atrial morphology cannot be discerned on chest radiographs, but it can be determined indirectly by evaluating the bronchi.²⁹ In almost every patient, the side of the morphologic bronchus corresponds to the side of the morphologic atrium; therefore, situs inversus is confirmed if the bronchus intermedius is on the left because the morphologic right atrium is also on the left. If a minor fissure can be identified, by inference, an eparterial bronchus and morphologic right atrium exist on that side.

In situs inversus, the longer hyparterial bronchus is on the right side and passes under the pulmonary artery; the shorter eparterial bronchus is on the left side and passes over the pulmonary artery. A left bronchus and right bronchus of equal length suggests isomerism. Because 1 of 5 patients with situs inversus has Kartagener syndrome, evaluate the chest radiographs carefully for evidence of bronchiectasis (see Images 3-4).

Upper and lower gastrointestinal examinations are usually not performed for the diagnosis of situs inversus. However, situs inversus may be found incidentally during such examinations. In an upper gastrointestinal examination in a patient with situs inversus, the stomach is on the right, with the C loop of the duodenum curving to the left. The liver and spleen are also in mirror-image locations compared with their normal position. In a barium enema examination, the sigmoid colon curves to the right, leading to a right-sided descending colon and terminating in a left-sided cecum (see Images 5-6).

Degree of Confidence

The degree of confidence of radiographs is high. CT scan findings can be used to resolve any remaining questions.

False Positives/Negatives

The most common cause of false-positive results is the technologist's or radiologist's inattention to proper labeling. This problem occasionally occurs when a technologist prepares for posteroanterior imaging of the chest and labels the image, but then, the patient is seated and imaged in an anteroposterior projection (eg, because of patient debility); as a result, the correct labeling is reversed.

The most common cause of a false-negative diagnosis of situs inversus also results from inattention to labeling. The technologist may incorrectly revise a properly labeled radiograph in a patient with situs inversus because the anatomy is reversed compared with the normal anatomy. A radiologist may incorrectly display an image so that it fits a mental template of what is normal without consciously noting the left or right marker. If a question of proper labeling exists, consult the technologist. If the projection of the image is known, the positioning of the name blocker can usually be used to reconstruct the correct labeling of the image. Alternatively, radiography may be repeated with supervision or special instructions to verify correct left-sided and right-sided labeling.

Most fluoroscopic machines have a button that electronically reverses the image. An experienced radiologist recognizes this reversal as soon as the table is moved to the left or right because the expected direction of table travel is opposite to that observed on the image intensifier. An inexperienced operator can be confused by this apparent reversal of normal anatomy. Conceivably, a patient with situs inversus can be examined with a fluoroscopy machine, and the image can be reversed electronically in a misguided attempt to correct the mirror-image anatomy.

Computed Tomography

Chest computed tomography scan obtained at the level of the origins of the great vessels in a 40-year-old man with situs inversus and dextrocardia (same patient as in Images 5-6 and 8-9 in Multimedia). This image demonstrates mirror-image branching of the great vessels (*) and a left-sided superior vena cava (+).

Chest computed tomography scan obtained at the level of the aortic outflow tract in a 40-year-old man with situs inversus and dextrocardia (same patient as in Images 5-7 and 9 in Multimedia.) This image shows reversal of the normal cardiac anatomy. The left atrium (LA), right atrium (RA), left ventricle (LV), and right ventricle (RV) are shown. The descending aorta (DA) is on the right.

Computed tomography scan of the upper abdomen in a 40-year-old man with situs inversus and dextrocardia (same patient as in Images 5-8 in Multimedia). This image shows reversal of the normal anatomy. The spleen (SP), stomach (ST), and liver (L) are shown. The descending aorta (DA) is on the right.

Findings

CT scanning demonstrates the mirror-image anatomy of the viscera in situs inversus (see Images 7-9):

• The heart and great vessels are a mirror image of their normal anatomy.
• The left hemithorax contains a trilobed lung, whereas the right hemithorax contains a bilobed lung.
• The liver and gallbladder are on the left side, and the spleen and stomach are on the right side.

Degree of Confidence

The degree of confidence with CT scanning is high.

False Positives/Negatives

In preparing for CT scanning, the technologist records the patient's position—prone or supine—and whether the patient is moved into the scanner head first or feet first. If the orientation is specified incorrectly, the left-right orientation is displayed incorrectly, and situs inversus is simulated.

Magnetic Resonance Imaging

Findings

MRI is a valuable adjunct to echocardiography and angiography in demonstrating abnormalities of congenital heart disease and in aiding surgical planning. This imaging modality is particularly helpful in diagnosing atrial situs. The morphologic right atrium contains the ostium of the coronary sinus; a connection to the suprahepatic inferior vena cava; a large, wide-based, pyramidal atrial appendage; the crista terminalis; and pectinate muscles. The morphologic left atrium has the ostia for the pulmonary veins and an atrial appendage with a narrow base and a tubular, hooked shape.³²

Degree of Confidence

The degree of confidence with MRI is high.

False Positives/Negatives

As with CT scanning, if the technologist incorrectly records whether the patient is moved head first or feet first into the bore or whether the patient is prone or supine, the image is reversed, and incorrect situs anatomy is simulated.

Ultrasonography

Findings

Echocardiography demonstrates the morphologic left and right atria. The morphologic right atrium has connections to the superior and inferior vena cava and a wide atrial appendage. The morphologic left atrium has a narrow left atrial appendage. Ultrasonography demonstrates the mirror-image anatomy of the abdominal viscera. Fetal ultrasonography can be used to detect situs inversus in utero; detection of this condition in utero alerts the physician to the possibility of congenital heart disease, which then warrants a careful cardiac evaluation.

Degree of Confidence

The degree of confidence with ultrasonography is high.

False Positives/Negatives

Although it is possible to switch the left and right sides of the ultrasonographic displays by holding the transducer backwards or electronically reversing the image, this error is expected only with inexperienced users. False-positive or false-negative diagnoses with ultrasonography are unlikely.

Nuclear Imaging

Findings

Any nuclear medicine study that is used to evaluate the heart or viscera can be influenced by the presence of situs inversus. These studies include cardiac, pulmonary, hepatobiliary, splenic, and gastrointestinal imaging. For example, on a ventilation-perfusion pulmonary scan, the photopenic defect from the heart is reversed in cases of situs inversus with dextrocardia. The technologist must be able to recognize situs inversus anatomy because nonstandard camera positioning is often necessary for optimal imaging.

Degree of Confidence

The degree of confidence with most nuclear medicine studies is moderate because of the limited anatomic detail.

False Positives/Negatives

Recording the anterior and posterior projections incorrectly reverses the left and right labeling. As with other digital images, the nuclear medicine image can be reversed electronically.

Angiography

Findings

Angiography is unnecessary for the diagnosis of situs inversus. In fact, noninvasive methods are preferred. Although the atrial morphology can be analyzed to determine atrial situs, angiography is usually reserved for the evaluation of congenital heart disease.

Degree of Confidence

The degree of confidence with angiography is high.

False Positives/Negatives

The false-positive and false-negative angiographic findings are similar to those of fluoroscopy.

Intervention

If radiologic intervention is to be performed in a patient with situs inversus, the condition should be known from earlier diagnostic imaging. A question of improper image labeling must be resolved before any procedure is initiated.³³

Medicolegal Pitfalls

• Failure to recognize situs inversus before performing a radiologic procedure may result in intervention on the incorrect side in the patient.
• Attention to the left and right sides of the patient and the left and right labeling of images is helpful to prevent mistakes in diagnosis and/or surgical intervention.

Special Concerns

• Discordance between the direction of the cardiac apex and the abdominal situs suggests congenital heart disease.
• Situs ambiguous and situs inversus with levocardia have this discordance between the direction of the cardiac apex and the abdominal situs; thus, further imaging is usually needed.

Multimedia

Media file 1: Schematic drawings illustrate the standard anatomy of situs solitus (A) and the mirror image of situs inversus (B). The right lung (RL), left lung (LL), right atrium (RA), and left atrium (LA) are shown.

Media file 2: Posteroanterior chest radiograph in a 40-year-old man with situs inversus and dextrocardia. This image shows that the cardiac apex (*) points to the right. A right-sided aortic arch (A) is associated with slight deviation of the trachea (T) to the left. The stomach (S) bubble is visible in the right upper quadrant.

Media file 3: Posteroanterior chest radiograph in a 55-year-old woman with Kartagener syndrome and situs inversus. This image shows a right-sided aortic arch (A) with slight leftward deviation of the trachea (T), dextrocardia (*), and a stomach bubble (S) in the right upper quadrant of the abdomen. Subtle bronchiectasis is also present in the lung bases (see Image 4 in Multimedia).

Media file 4: Magnified view of the left lower lobe in a 55-year-old woman with Kartagener syndrome and situs inversus (same patient as in Image 3 in Multimedia). This image shows bronchiectasis (arrows).

Media file 5: Radiograph of the upper abdomen from a barium enema examination in a 40-year-old man with situs inversus and dextrocardia. This image shows the liver (L) in the left upper quadrant of the abdomen. The positions of the splenic flexure (SF) and hepatic flexure (HF) are reversed. (See also Images 6-9 in Multimedia.)

Media file 6: Radiograph of the lower abdomen from a barium enema examination in a 40-year-old man with situs inversus and dextrocardia (same patient as in Images 5 and 7-9 in Multimedia). This image shows the sigmoid colon (SC) on the right and the cecum (C) on the left.

Media file 7: Chest computed tomography scan obtained at the level of the origins of the great vessels in a 40-year-old man with situs inversus and dextrocardia (same patient as in Images 5-6 and 8-9 in Multimedia). This image demonstrates mirror-image branching of the great vessels (*) and a left-sided superior vena cava (+).

Media file 8: Chest computed tomography scan obtained at the level of the aortic outflow tract in a 40-year-old man with situs inversus and dextrocardia (same patient as in Images 5-7 and 9 in Multimedia.) This image shows reversal of the normal cardiac anatomy. The left atrium (LA), right atrium (RA), left ventricle (LV), and right ventricle (RV) are shown. The descending aorta (DA) is on the right.

Media file 9: Computed tomography scan of the upper abdomen in a 40-year-old man with situs inversus and dextrocardia (same patient as in Images 5-8 in Multimedia). This image shows reversal of the normal anatomy. The spleen (SP), stomach (ST), and liver (L) are shown. The descending aorta (DA) is on the right.

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Keywords

situs inversus, situs inversus totalis, situs inversus with dextrocardia, situs inversus with levocardia, mirror image of situs solitus, mirror-image organs, situs solitus, situs ambiguous/ambiguus, Kartagener syndrome, Kartagener's syndrome, primary ciliary dysplasia, PCD, primary ciliary diskinesia

Contributor Information and Disclosures

Author

Annamaria Wilhelm, MD, Staff Physician, Department of Radiology, Mayo Clinic
Annamaria Wilhelm, MD is a member of the following medical societies: American College of Radiology
Disclosure: Nothing to disclose.

Coauthor(s)

John M Holbert, MD, Professor, Department of Radiology, Scott and White Memorial Hospital and Clinic
John M Holbert, MD is a member of the following medical societies: American College of Chest Physicians, American College of Radiology, American Roentgen Ray Society, and Radiological Society of North America
Disclosure: Amirsys Royalty Other

Medical Editor

Henrique M Lederman, MD, PhD, Consulting Staff, Department of Radiology, LeBonheur Children's Medical Center and St Jude Children's Research Hospital; Professor of Radiology and Pediatric Radiology, Chief, Division of Diagnostic Imaging in Pediatrics, Federal University of Sao Paulo, Brazil
Henrique M Lederman, MD, PhD is a member of the following medical societies: Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Marta Hernanz-Schulman, MD, FAAP, Professor, Radiology, Radiological Sciences, and Pediatrics, Director, Department of Pediatric Radiology, Radiologist-in-Chief, Director, Department of Diagnostic Imaging, Vanderbilt University Medical Center, Vanderbilt Children's Hospital
Marta Hernanz-Schulman, MD, FAAP is a member of the following medical societies: American Institute of Ultrasound in Medicine and American Roentgen Ray Society
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

John Karani, MBBS, FRCR, Consulting Staff, Department of Radiology, King's College Hospital, London
Disclosure: Nothing to disclose.

Related eMedicine topics

Kartagener Syndrome

Primary Ciliary Dyskinesia

Congenitally Corrected Transposition

Transposition of the Great Arteries

Partial Anomalous Pulmonary Venous Connection

Clinical studies

Genetic Study of Patients with Primary Ciliary Dyskinesia

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