Table of ContentsView AllTable of ContentsAnatomyLong-Term EffectsBirth vs. Later in LifeTreatment
Table of ContentsView All
View All
Table of Contents
Anatomy
Long-Term Effects
Birth vs. Later in Life
Treatment
Situs inversusis a rare ailment in which a person’s internal organs are arranged in a mirror image (a reversal) of normal organ placement. This problem iscongenital(present at birth) and linked to several genetic mutations. Situs inversus, or status inversusviscerum, describes the broader issue in which the soft organs (viscera) are reversed.
Situs inversus is described in one of two subtypes. Situs inversus withdextrocardiais the most common form. It happens when your organs are flipped, and your heart’s apex (tip) points toward the right side of your chest. Situs inversus withlevocardiaoccurs when the apex of yourheartpoints to the left side of your chest, which is normal, but other organs are flipped.
Many people with situs inversus can lead normal lives without symptoms. As a result, they do not know they have the problem until it is found during an imaging study for another issue. However, some people havelung damage,infertility, and other health problems with situs inversus based on the placement of their organs.
Explaining Situs Inversus Anatomy
Organ placement is established early in fetal development. In normal anatomy, the organs in your chest and abdomen are left-right asymmetrical. The word “situs” describes the position or orientation of these organs.
The normal anatomy of your organs is described as situs solitus. In this configuration, the normal placement of your heart,spleen, andpancreasis on the left side of your body, while most of your liver is on the right.
Situs inversus totalis describes a condition in which the organs in your chest and abdomen have a complete position reversal. This total organ reversal allows your organs to function normally, though on the opposite side of your body.
Between the extremes of situs solitus (normal organ placement) and situs inversus totalis (total organ position reversal), exists situs ambiguous (intermediate abnormal positioning of organs). Situs ambiguous, or heterotaxy syndrome, includes a spectrum of disorders that involve any other abnormality in left-to right organ placement though not in a defined pattern.
The 11 Organ Systems of the Body and How They Work
Long Term, Is Situs Inversus Serious?
The severity and prognosis for situs inversus depends on the type of situs inversus present. Situs inversus totalis usually does not impair organ function since all the organs are intact and exist in the position required to work together, though on the opposite side of your body.
Conditions that can occur with situs inversus include respiratory problems, infertility, and heart defects.
Respiratory Symptoms
Respiratory symptoms linked to situs inversus are often related to a condition calledprimary ciliary dyskinesia, a rare inherited lung disease. About 50% of people with this condition have situs inversus totalis. The combination of situs inversus totalis and primary ciliary dyskinesia is calledKartagener syndrome. An additional 12% of people with primary ciliary dyskinesia have situs ambiguous.
Primary ciliary dyskinesia occurs due to defects in the cilia. These microscopic hairlike structures sit atop cells lining your respiratory tract, which extends from your nose to the airways of your lungs.
Malfunctioning cilia do not beat rhythmically, so they fail to remove mucous,bacteria, and other substances that travel through the respiratory tract. As a result, these substances get stuck in yourlungs, sinuses, and ears, leaving these areas vulnerable to frequent infections.
Respiratory symptoms of primary ciliary dyskinesia include:
Infertility Symptoms
Symptoms ofinfertility(inability to conceive naturally) in situs inversus are often linked with primary ciliary dyskinesia. While it does not lead to sterility (the inability to procreate due to unviableeggsorsperm), infertility symptoms that occur with primary ciliary dyskinesia can make it difficult to becomepregnant.
Symptoms of infertility that occur with situs inversus include:
Heart Defects
Congenital heart defects occur in about 5% to 10% of people affected with situs inversus.
The defects described with situs inversus typically include conotruncal heart malformations.These heart defects include a heterogeneous group of structural abnormalities of the pathway in which the blood leaves the heart and circulates to your body or lungs.
Some of the most common conotruncal heart malformations linked with situs inversus include:
Ventricular Septal Defect
This defect is an opening, or hole, in the septum (wall) separating the two lower chambers of your heart (ventricles). Symptoms can vary based on the size of the opening.
A small hole can be asymptomatic, only causing a loudmurmur(noise heard with a stethoscope). A large hole can interfere with normal breathing, feeding, and growth rate. The condition can lead to increased lung pressure as more blood than normal is pumped there. Permanent damage to the lung blood vessels can occur over time.
Atrial Septal Defect
This defect is an opening or hole in the septum that separates the right and left atrium, the top two chambers of your heart.
An atrial septal defect can allow blood to travel through the hole from the left upper heart chamber (left atrium) to the right upper heart chamber (right atrium) and into the lung arteries. Having excess blood pumped into the lung arteries makes your heart and lungs work harder, resulting in damaged lung arteries.
Transposition of the Great Arteries
This complex congenital defect is a reversal of the two main arteries that carry blood away from your heart, theaortaandpulmonary artery. The arteries are transposed from their normal positions in that the aorta begins at the right ventricle instead of the left and the pulmonary artery begins at the left ventricle instead of the right. The disorder creates a situation in which the organs of your body do not get the oxygen they need.
Situs Inversus Diagnosis at Birth vs. Older Age
Diagnosis at birth can occur in infants whoseprenatal ultrasoundsindicate abnormal organ position before birth. Babies undiagnosed during the prenatal stage may be born with symptoms that affect their respiratory or cardiac function. Imaging studies to diagnose these types of complications can reveal the disorder at birth. However, babies born without symptoms are not routinely screened for the disorder.
Situs inversus can remain asymptomatic throughout a lifetime. It may not be found until a diagnostic imaging study, such as anX-ray,computed tomography (CT) scan, ormagnetic resonance imaging (MRI), is performed for another reason.
Cross-sectional imaging like CT scans provides the exact anatomical variations in the chest and abdomen. CT can demonstrate the mirror-image location of the solid organs and a right-sided cardiac apex. In addition to showing a mirror image of the solid organs, a CT scan also can identify a reversed positioning of the bowel and mesenteric vessels.
Situs Inversus Genes and Risk FactorsOver 100 genes have been linked to situs inversus defects. These genes include:ZIC3CCDC11CFAP52NME7PKD1L1Situs inversus is inherited in an autosomal recessive pattern with multiple gene mutations. This means that people who have the disorder must inherit one copy of a mutated gene from each parent, though their parents remain unaffected. Risk factors for situs inversus include:Family history of heart defectsFamily history of noncardiac anomaliesMaternaldiabetesAntitussive usePaternal smokingLow socioeconomic status
Situs Inversus Genes and Risk Factors
Over 100 genes have been linked to situs inversus defects. These genes include:ZIC3CCDC11CFAP52NME7PKD1L1Situs inversus is inherited in an autosomal recessive pattern with multiple gene mutations. This means that people who have the disorder must inherit one copy of a mutated gene from each parent, though their parents remain unaffected. Risk factors for situs inversus include:Family history of heart defectsFamily history of noncardiac anomaliesMaternaldiabetesAntitussive usePaternal smokingLow socioeconomic status
Over 100 genes have been linked to situs inversus defects. These genes include:
Situs inversus is inherited in an autosomal recessive pattern with multiple gene mutations. This means that people who have the disorder must inherit one copy of a mutated gene from each parent, though their parents remain unaffected. Risk factors for situs inversus include:
Treatment for Situs Inversus Complications
Treatment of situs is based on symptoms. Procedures are performed to facilitate and support people in living a normal life without debilitating symptoms or discomfort. Specific procedures and treatments, along with changes in your prognosis, vary based on the complications present.
Treatments used for common situs inversus complications include:
Primary Ciliary Dyskinesia
Treatment centers on improving lung function and limiting disease progression. This can involve the use ofantibioticsto treat lung or sinus infections. Airway clearance methods and chest physical therapy can help keep your lungs clear.Oxygen therapymay be needed for severe disease, with the option oflung transplantationa rare necessity.
Infertility
There is no consensus on the optimal treatment of infertility due to primary ciliary dyskinesia in situs inversus. The use of assisted reproductive technology, such asin vitro fertilization(IVF), seems to benefit both males and females with primary ciliary dyskinesia, though research indicates that success rates vary.
Treatment may not be needed for a small hole. Small ventricular septal defects can close on their own without treatment. However, a large hole may require repair with open heart surgery.
Correction of a large hole withopen heart surgeryis usually reserved for later in childhood. A temporary operation called pulmonary artery banding may be used to reduce blood flow to the lungs and postpone open heart surgery.
A small atrial septal defect doesn’t make your heart work harder, so treatment may be unnecessary. Some small holes may close on their own. A large atrial septal defect may be corrected with open heart surgery orcardiac catheterizationin early childhood to prevent complications.
Surgical correction of this defect is always needed. Medication and temporary procedures can help stabilize babies born with this defect in the short term, but arterial switch surgery is typically performed within the first weeks of life.
Adjustments Necessary When Situs Inversus Is PresentDue to the rarity of this condition, most healthcare providers are not experienced in treating people with situs inversus. Since most surgeons are right-handed, surgical procedures can cause difficulties if they have to adjust to handling instruments with their left hand or maneuvering pedals with their left foot. Organ transplants, especiallyliver transplants, can be a significant challenge.Before a scheduled surgery, imaging methods like a CT scan or MRI can be used to plan creative approaches to take in surgery. However, undiagnosed situs inversus can pose problems in emergency situations and even routine health maintenance.
Adjustments Necessary When Situs Inversus Is Present
Due to the rarity of this condition, most healthcare providers are not experienced in treating people with situs inversus. Since most surgeons are right-handed, surgical procedures can cause difficulties if they have to adjust to handling instruments with their left hand or maneuvering pedals with their left foot. Organ transplants, especiallyliver transplants, can be a significant challenge.Before a scheduled surgery, imaging methods like a CT scan or MRI can be used to plan creative approaches to take in surgery. However, undiagnosed situs inversus can pose problems in emergency situations and even routine health maintenance.
Due to the rarity of this condition, most healthcare providers are not experienced in treating people with situs inversus. Since most surgeons are right-handed, surgical procedures can cause difficulties if they have to adjust to handling instruments with their left hand or maneuvering pedals with their left foot. Organ transplants, especiallyliver transplants, can be a significant challenge.
Before a scheduled surgery, imaging methods like a CT scan or MRI can be used to plan creative approaches to take in surgery. However, undiagnosed situs inversus can pose problems in emergency situations and even routine health maintenance.
Summary
Situs inversus is a rare congenital condition that occurs in an autosomal recessive pattern. It involves the mirror-image transposition of the chest and belly organs.
The problem does not reduce lifespan except in the rare cases of certain heart defects. However, situs inversus can cause symptoms that affect your breathing, fertility, heart, and lungs. Treatment centers on fixing malfunctions and reducing symptoms.
Since situs inversus can occur without symptoms, you can have this problem without knowing it. However, it can be found during prenatal ultrasounds or at birth when breathing problems require imaging.
Talk to your healthcare provider about your risk of situs inversus. A family history of the problem or certain health issues can increase your risk. While you cannot reduce your risk of having this disease, you may decide to pursue genetic testing before getting pregnant.
19 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Nardella G, Candela MA, Di Pianella V,et al.Situs inversus viscerum and renal agenesis in a newborn.Archives of Disease in Childhood.2017;102:A58.Pandey S, Singh S, Passey J, Singh R, et al.Complete situs inversus: a variation in left-right asymmetry during embryogenesis.Italian Journal of Anatomy and Embryology. 2018;123(3):263-276. doi:10.13128/IJAE-25418Eitler K, Bibok A, Telkes G.Situs inversus totalis: a clinical review.Int J Gen Med. 2022 Mar 3;15:2437-2449. doi:10.2147/IJGM.S295444MedlinePlus.Herotaxy syndrome.Situs inversus. In: Bissonnette B, Luginbuehl I, Engelhardt T. eds.Syndromes: Rapid Recognition and Perioperative Implications, 2e. McGraw-Hill Education; 2019.MedlinePlus.Primary ciliary dyskinesia.Children’s Hospital of Philadelphia (CHOP).Primary ciliary dyskinesia.Primary Ciliary Dyskinesia Foundation.Reproductive health in PCD.National Organization for Rare Diseases (NORD).Situs inversus.Bamshad Laboratory, Department of Pediatrics, University of Washington.Birth defects of the heart.American Heart Association (AHA).Ventricular septal defect (VSD).American Heart Association (AHA).Atrial septal defect (ASD).Cincinnati Children’s.Transposition of the great arteries.Devera J, Licandro F, Ramos J, Taymoorian HT, Yap LG.Situs inversus rotalis in the neonatal setting.Cureus. 2021 Feb 23;13(2):e13516. doi:10.7759/cureus.13516Ramavathu KVM.Imaging findings in a case of situs inversus totalis. BJR Case Rep. 2021 Mar 4;7(4):20200202. doi:10.1259/bjrcr.20200202Postema, M.C., Carrion-Castillo, A., Fisher, S.E.et al.The genetics of situs inversus without primary ciliary dyskinesia.Sci Rep.2020;10:3677. doi:10.1038/s41598-020-60589-zNational Organization for Rare Diseases.Dextrocardia with situs inversus.American Lung Association.Treating and managing primary ciliary dyskinesia.Newman L, Chopra J, Dossett C, Shepherd E, Bercusson A, Carroll M, Walker W, Lucas JS, Cheong Y.The impact of primary ciliary dyskinesia on female and male fertility: a narrative review.Hum Reprod Update. 2023 May 2;29(3):347-367. doi:10.1093/humupd/dmad003
19 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Nardella G, Candela MA, Di Pianella V,et al.Situs inversus viscerum and renal agenesis in a newborn.Archives of Disease in Childhood.2017;102:A58.Pandey S, Singh S, Passey J, Singh R, et al.Complete situs inversus: a variation in left-right asymmetry during embryogenesis.Italian Journal of Anatomy and Embryology. 2018;123(3):263-276. doi:10.13128/IJAE-25418Eitler K, Bibok A, Telkes G.Situs inversus totalis: a clinical review.Int J Gen Med. 2022 Mar 3;15:2437-2449. doi:10.2147/IJGM.S295444MedlinePlus.Herotaxy syndrome.Situs inversus. In: Bissonnette B, Luginbuehl I, Engelhardt T. eds.Syndromes: Rapid Recognition and Perioperative Implications, 2e. McGraw-Hill Education; 2019.MedlinePlus.Primary ciliary dyskinesia.Children’s Hospital of Philadelphia (CHOP).Primary ciliary dyskinesia.Primary Ciliary Dyskinesia Foundation.Reproductive health in PCD.National Organization for Rare Diseases (NORD).Situs inversus.Bamshad Laboratory, Department of Pediatrics, University of Washington.Birth defects of the heart.American Heart Association (AHA).Ventricular septal defect (VSD).American Heart Association (AHA).Atrial septal defect (ASD).Cincinnati Children’s.Transposition of the great arteries.Devera J, Licandro F, Ramos J, Taymoorian HT, Yap LG.Situs inversus rotalis in the neonatal setting.Cureus. 2021 Feb 23;13(2):e13516. doi:10.7759/cureus.13516Ramavathu KVM.Imaging findings in a case of situs inversus totalis. BJR Case Rep. 2021 Mar 4;7(4):20200202. doi:10.1259/bjrcr.20200202Postema, M.C., Carrion-Castillo, A., Fisher, S.E.et al.The genetics of situs inversus without primary ciliary dyskinesia.Sci Rep.2020;10:3677. doi:10.1038/s41598-020-60589-zNational Organization for Rare Diseases.Dextrocardia with situs inversus.American Lung Association.Treating and managing primary ciliary dyskinesia.Newman L, Chopra J, Dossett C, Shepherd E, Bercusson A, Carroll M, Walker W, Lucas JS, Cheong Y.The impact of primary ciliary dyskinesia on female and male fertility: a narrative review.Hum Reprod Update. 2023 May 2;29(3):347-367. doi:10.1093/humupd/dmad003
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Nardella G, Candela MA, Di Pianella V,et al.Situs inversus viscerum and renal agenesis in a newborn.Archives of Disease in Childhood.2017;102:A58.Pandey S, Singh S, Passey J, Singh R, et al.Complete situs inversus: a variation in left-right asymmetry during embryogenesis.Italian Journal of Anatomy and Embryology. 2018;123(3):263-276. doi:10.13128/IJAE-25418Eitler K, Bibok A, Telkes G.Situs inversus totalis: a clinical review.Int J Gen Med. 2022 Mar 3;15:2437-2449. doi:10.2147/IJGM.S295444MedlinePlus.Herotaxy syndrome.Situs inversus. In: Bissonnette B, Luginbuehl I, Engelhardt T. eds.Syndromes: Rapid Recognition and Perioperative Implications, 2e. McGraw-Hill Education; 2019.MedlinePlus.Primary ciliary dyskinesia.Children’s Hospital of Philadelphia (CHOP).Primary ciliary dyskinesia.Primary Ciliary Dyskinesia Foundation.Reproductive health in PCD.National Organization for Rare Diseases (NORD).Situs inversus.Bamshad Laboratory, Department of Pediatrics, University of Washington.Birth defects of the heart.American Heart Association (AHA).Ventricular septal defect (VSD).American Heart Association (AHA).Atrial septal defect (ASD).Cincinnati Children’s.Transposition of the great arteries.Devera J, Licandro F, Ramos J, Taymoorian HT, Yap LG.Situs inversus rotalis in the neonatal setting.Cureus. 2021 Feb 23;13(2):e13516. doi:10.7759/cureus.13516Ramavathu KVM.Imaging findings in a case of situs inversus totalis. BJR Case Rep. 2021 Mar 4;7(4):20200202. doi:10.1259/bjrcr.20200202Postema, M.C., Carrion-Castillo, A., Fisher, S.E.et al.The genetics of situs inversus without primary ciliary dyskinesia.Sci Rep.2020;10:3677. doi:10.1038/s41598-020-60589-zNational Organization for Rare Diseases.Dextrocardia with situs inversus.American Lung Association.Treating and managing primary ciliary dyskinesia.Newman L, Chopra J, Dossett C, Shepherd E, Bercusson A, Carroll M, Walker W, Lucas JS, Cheong Y.The impact of primary ciliary dyskinesia on female and male fertility: a narrative review.Hum Reprod Update. 2023 May 2;29(3):347-367. doi:10.1093/humupd/dmad003
Nardella G, Candela MA, Di Pianella V,et al.Situs inversus viscerum and renal agenesis in a newborn.Archives of Disease in Childhood.2017;102:A58.
Pandey S, Singh S, Passey J, Singh R, et al.Complete situs inversus: a variation in left-right asymmetry during embryogenesis.Italian Journal of Anatomy and Embryology. 2018;123(3):263-276. doi:10.13128/IJAE-25418
Eitler K, Bibok A, Telkes G.Situs inversus totalis: a clinical review.Int J Gen Med. 2022 Mar 3;15:2437-2449. doi:10.2147/IJGM.S295444
MedlinePlus.Herotaxy syndrome.
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MedlinePlus.Primary ciliary dyskinesia.
Children’s Hospital of Philadelphia (CHOP).Primary ciliary dyskinesia.
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Bamshad Laboratory, Department of Pediatrics, University of Washington.Birth defects of the heart.
American Heart Association (AHA).Ventricular septal defect (VSD).
American Heart Association (AHA).Atrial septal defect (ASD).
Cincinnati Children’s.Transposition of the great arteries.
Devera J, Licandro F, Ramos J, Taymoorian HT, Yap LG.Situs inversus rotalis in the neonatal setting.Cureus. 2021 Feb 23;13(2):e13516. doi:10.7759/cureus.13516
Ramavathu KVM.Imaging findings in a case of situs inversus totalis. BJR Case Rep. 2021 Mar 4;7(4):20200202. doi:10.1259/bjrcr.20200202
Postema, M.C., Carrion-Castillo, A., Fisher, S.E.et al.The genetics of situs inversus without primary ciliary dyskinesia.Sci Rep.2020;10:3677. doi:10.1038/s41598-020-60589-z
National Organization for Rare Diseases.Dextrocardia with situs inversus.
American Lung Association.Treating and managing primary ciliary dyskinesia.
Newman L, Chopra J, Dossett C, Shepherd E, Bercusson A, Carroll M, Walker W, Lucas JS, Cheong Y.The impact of primary ciliary dyskinesia on female and male fertility: a narrative review.Hum Reprod Update. 2023 May 2;29(3):347-367. doi:10.1093/humupd/dmad003
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