The History

Situs inversus with dextrocardia and a structurally normal heart is usually discovered by chance in a chest x-ray which is often considered normal because the film is inadvertently reversed when first read. There is a reported tendency for left handedness in complete situs inversus,22 but Matthew Baillie wrote: "The person seems to have used his right hand in preference to his left . . . which was readily discovered by the greater bulk and hardness of that hand as well as the greater fleshiness of the arm."14 Baillie's conclusion has been confirmed.74'131

Important are investigations of the human brain which is midline but asymmetric in both structure and func-tion.74'131 The developmental factors that determine functional asymmetry of the brain independently recognize laterality (asymmetry) in visceral situs.131 Developmental factors that determine anatomic asymmetry of the brain are distinct from those that determine visceral asymmetry and lateralization of language.74

Situs inversus with dextrocardia is the malposition most likely to occur with an otherwise structurally normal heart and with normal longevity. Symptoms caused by coexisting acquired cardiac or noncardiac disease may lead to the discovery of hitherto unsuspected situs inversus.19'39'66,68,69,76 The pain of ischemic heart disease is located in the right anterior chest with radiation to the right shoulder and right arm.66,68,76 The pain of appendicitis is referred to the left lower quadrant,93 and the pain of biliary colic presents in the left upper quadrant (Fig. 3-13).

In 1933, Kartagener called attention to the association of sinusitis, bronchiectasis and situs inversus,72 a combination subsequently called Kartagener syndrome or triad.16'51'73'80'92'132 In the first English language publication of the syndrome (1937), as many as one fifth of patients with situs inversus had bronchiectasis, underscoring that the association was not fortuitous.5 In 1986, a blinded controlled study of cilia ultrastructure in Kartagener syndrome found a widespread inherited ciliary disorder40,95,98,133 that included the upper and lower respiratory tracts54,73,133 (bronchitis, bronchiectasis, sinusitis) and the testis4,6,7,49,103 (immobile sperm, male infertility). Situs inversus is common in infertile men, an observation that contributed to the identification of a generalized disorder of ciliary motil-ity6,49 Respiratory symptoms are a significant part of the history and may lead to the discovery of situs inversus. The connection between abnormal cilia and laterality remains enigmatic.7 Familial situs inversus has been reported,23,135,159 and Kartagener syndrome is sometimes familial.40,95,133 One family of six siblings included two cases of Kartagener syndrome and two cases of isolated bronchiectasis.16

Figure 3-9

A, Right ventriculogram (anteroposterior) in a two-month-old female in situs solitus with dextrocardia and no associated congenital heart disease. The morphologic right ventricle (RV) occupies the apex on the right and gives rise to the pulmonary trunk (PT). The hemidiaphragm is lower on the side of the apex. B, The morphologic left ventricle (LV) is in a medial position, and gives rise to a normally positioned ascending aorta (Ao) and a left-sided descending aorta (DA).

Figure 3-9

A, Right ventriculogram (anteroposterior) in a two-month-old female in situs solitus with dextrocardia and no associated congenital heart disease. The morphologic right ventricle (RV) occupies the apex on the right and gives rise to the pulmonary trunk (PT). The hemidiaphragm is lower on the side of the apex. B, The morphologic left ventricle (LV) is in a medial position, and gives rise to a normally positioned ascending aorta (Ao) and a left-sided descending aorta (DA).

Situs solitus with dextrocardia occasionally occurs without coexisting congenital heart disease and escapes recognition. A routine chest x-ray may provide the first evidence (see Fig. 3-10). As a rule, accompanying congenital cardiac malformations bring the patient to medical attention. Situs inversus with levocardia (see Fig. 3-11) invariably occurs with coexisting congenital heart disease that leads to the discovery of the cardiac malposition.

Physical Appearance, the Arterial Pulse, and the Jugular Venous Pulse

These features are determined by coexisting congenital heart disease rather than the cardiac malposition. The left testicle in the normal upright male is lower than the right testicle, whereas the opposite is the case in situs inversus.

Congenital Heart Disease Cxr

Figure 3-10

Chest x-ray from a 20-year-old male in situs solitus with dextrocardia and no associated congenital heart disease. The stomach (S) is on the left and the liver (L) is on the right. The base to apex axis points to the right, the cardiac shadow is chiefly to the right of midline, but the hemidiaphragms are at the same level. The ascending aorta and aortic knuckle (unmarked white arrows) are in their normal positions, and the descending thoracic aorta (DAo) is normally positioned along the left border of the vertebral column.

Figure 3-10

Chest x-ray from a 20-year-old male in situs solitus with dextrocardia and no associated congenital heart disease. The stomach (S) is on the left and the liver (L) is on the right. The base to apex axis points to the right, the cardiac shadow is chiefly to the right of midline, but the hemidiaphragms are at the same level. The ascending aorta and aortic knuckle (unmarked white arrows) are in their normal positions, and the descending thoracic aorta (DAo) is normally positioned along the left border of the vertebral column.

Levocardia Situs Inversus

Figure 3-11

X-ray from a 2-year-old female in situs inversus with levocardia. The stomach (S) is on the right and the liver (L) is on the left, but the heart (apex) is to the left of midline. The left hemidiaphragm is lower than the right hemidi-aphragm because the cardiac apex is on the left. The descending thoracic aorta (DAo) is on the right (concordant for situs inversus), but the position of the ascending aorta (AAo) indicates a discordant d-bulboventricular loop.

Figure 3-11

X-ray from a 2-year-old female in situs inversus with levocardia. The stomach (S) is on the right and the liver (L) is on the left, but the heart (apex) is to the left of midline. The left hemidiaphragm is lower than the right hemidi-aphragm because the cardiac apex is on the left. The descending thoracic aorta (DAo) is on the right (concordant for situs inversus), but the position of the ascending aorta (AAo) indicates a discordant d-bulboventricular loop.

Situs Psychosis

Figure 3-12

A, X-ray from a 16-year-old male in situs solitus with a midline heart (mesocardia) and no associated congenital heart disease. The stomach (S) is on the left, and the liver (L) is on the right. There is identical extension of the heart to the right and left of center (equal black arrows). The ascending aorta and aortic knuckle (unmarked white arrows) are in their normal positions. The cardiac silhouette is hump-shaped because the right atrium (RA) and right ventricle (RV) are superimposed (see angiogram, B). The right hemidiaphragm is lower than the left hemidiaphragm because the base to apex axis points to the right. B, The position of the right ventricle (RV) and the interventricular septal plane (IV septum) indicate that mesocardia resulted from a d-bulboventricular loop in which leftward pivoting stopped at the midline. RA, right atrium; PT, pulmonary trunk.

Figure 3-12

A, X-ray from a 16-year-old male in situs solitus with a midline heart (mesocardia) and no associated congenital heart disease. The stomach (S) is on the left, and the liver (L) is on the right. There is identical extension of the heart to the right and left of center (equal black arrows). The ascending aorta and aortic knuckle (unmarked white arrows) are in their normal positions. The cardiac silhouette is hump-shaped because the right atrium (RA) and right ventricle (RV) are superimposed (see angiogram, B). The right hemidiaphragm is lower than the left hemidiaphragm because the base to apex axis points to the right. B, The position of the right ventricle (RV) and the interventricular septal plane (IV septum) indicate that mesocardia resulted from a d-bulboventricular loop in which leftward pivoting stopped at the midline. RA, right atrium; PT, pulmonary trunk.

Poland syndrome, which is characterized by absence of a pectoralis major muscle (usually right-sided), ipsilateral syndactyly, brachydactyly, and hypoplasia of a hand, has been reported with situs solitus and dextrocardia46 (see Fig. 18-17), and the Goldenhar syndrome (oculoauricular vertebral dysplasia, hemifacial microsomia) has been reported with complete situs inversus.52

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