A CASE of 51/MALE Chest CT Scan: With BRONCHIECTASIS; S/P PTB (Incidental Finding Of Situs Inversus)
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Dextrocardia with Situs Inversus is a rare heart condition characterized by abnormal positioning of the heart. In this condition, the tip of the heart (apex) is positioned on the right side of the chest. Additionally, the position of the heart chambers as well as the visceral organs such as the liver and spleen is reversed (situs inversus). However, most affected individuals can live a normal life without associated symptoms or disability.
Causes
Dextrocardia with Situs Inversus, a rare condition that is present at birth, is transmitted by autosomal recessive genes. The primitive loop in the embryo moves into the reverse direction of its normal position during fetal development, causing displacement of organs.
Human traits including the classic genetic disorders are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In recessive disorders, the condition does not appear unless a person inherits the same defective gene for the same trait from each parent. If an individual receives one normal gene and one gene for the disease, the person will be a carrier for the disease, but usually will not show symptoms. The risk of transmitting the disease to the children of a couple, both of whom are carriers for a recessive disorder, is 25 percent. Fifty percent of their children risk being carriers of the disease, but generally will not show symptoms of the disorder. Twenty-five percent of their children may receive both normal genes, one from each parent, and will be genetically normal (for that particular trait). The risk is the same for each pregnancy.
Affected Populations
Dextrocardia with Situs Inversus is present at birth. The condition affects males and females in equal numbers.
Related Disorders
Dextroversion of the heart means that the location of the heart is abnormally positioned in the right half of the chest. The left ventricle remains on the left, but lies in front of the right ventricle. The heart is rotated to the right. The waves representing the heart beat on the electrocardiogram will indicate an abnormality.
Dextroposition of the heart is a displacement of the heart to the right. It is usually caused by acquired disease of the lungs, the membrane around the lungs (pleura), or diaphragm. The electrocardiogram is usually normal.
Kartagener Syndrome is a combination of Dextrocardia with chronic dilatation of the bronchi, difficulty breathing, recurrent respiratory infection (bronchiectasis) and infection of the sinuses (sinusitis). Clubbed fingers and bluish discoloration of the skin (cyanosis) may also be present.
Standard Therapies
Treatment of Dextrocardia with Situs Inversus is symptomatic and supportive when needed. In most cases, affected individuals can live a normal life without any symptoms or discomfort. If the condition is associated with other more serious heart malformations, the prognosis and treatment will vary. Genetic counseling may be helpful for affected families.
CT Result:
CLINICAL INFORMATION: Bronchiectasis T/C PTB. Situs inversus
COMPARISON: None
TECHNIQUE: Multislice (16 MDCT) axial sections of the Chest and Mediastinum without and with contrast with
coronal and sagittal reformations.
Lungs are aerated. Mild bronchiectatic changes prominent in the lower lungs and the right upper lung. Fibrotic densities
in the right upper lung with associated pleural thickening. Subcentimeter calcified nodules in the right upper lung with
largest of 0.31 cm. Confluence of tiny air cystic lesions in the periphery of the left upper lung and more in the periphery
of the lower lungs with patch of ground glass densities more in the right.
The heart is not enlarged with the apex seen directed to the right side of the chest consistent with dextrocardia. No
pericardial effusion is seen. Aortic arch is right sided with note of intimal calcification in the thoracic and imaged
abdominal aorta. No evidence of hilar or mediastinal lymphadenopathy.
Thyroid gland is normal in size, attenuation and enhancement with no demonstrable lesion. No calcification is seen.
The liver is left sided with no evidence of enhancing mass, unusual density or calcification. Bile ducts are not dilated.
There are 4 dense stones in the left side oriented gallbladder with largest of 0.93 cm. The common bile duct is not
dilated.
The spleen is right-sided with no enhancing lesion or calcification. A 3.3 cm splenule is seen in the superior aspect. No
discrete lesion in imaged right side oriented pancreatic tail and the left side oriented pancreatic head.
The stomach is right sided and is not distended. Non distended imaged small and large bowel loops seen.
No discrete lesion in the imaged upper portion of the kidneys and the adrenal glands.
Mild thoracic spine osteophytes are seen.
IMPRESSION:
- Bronchiectasis with features of paraseptal emphysema - lower lung interstitial fibrosis and concurrent pneumonitis
- Right upper lung fibrosis with apical pleural thickening likely residual of previous infection with tiny (subcentimeter)
granulomas - Dextrocardia - situs inversus
- Atherosclerotic aorta.
- Cholelithiases
- Mild thoracic spondylosis