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Dextrocardia

Dextrocardias

Dextrocardia is a congenital cardiac defect. People born with dextrocardia have their heart positioned on the right side of the thorax.


Presentation

Although dextrocardia does not necessarily lead to the appearance of symptoms and dysfunction, some individuals may start to experience shortness of breath, cyanosis (lips and skin), fatigue and a hindered growth. In cases where dextrocardia is isolated, the heart is located on the right side of the thoracic cavity and other cardiac irregularities may be present as well, such as a septal defect, pulmonary hypoplasia, etc [4]. Another type of dextrocardia, dextrocardia with situs inversus, involves the misplacement not only of the heart but of the rest of the viscera as well - the heart is a mirror image of a normal heart and the lungs, diaphragm and other internal organs are mirrored as well. If all of the organs are mirrored, the condition is referred to as dextrocardia with situs inversus totalis.

In general, situs inversus implies the mirror-like reversion of the internal organs in the body and can either accompany a normal heart, or a heart with dextrocardia [5] [6] [7] [8] [9] [10] [11] [12]. In general, the term dextrocardia means that the cardiac apex is directed to the right when a child is born, but provides no other information concerning the location and arrangement of the atria or ventricles. Situs inversus more frequently occurs when a person is born with a cardiac apex directed to the right [13]; individuals born with levocardia (left orientation of the apex) and situs inversus most often exhibit congenital heart disease as well [14] [15] [16] [17] [18] [19] [20].

People born with dextrocardia often have their great arteries in a reversed position as well.

Abdominal Pain
  • A 32 years old gentleman, presented in emergency department, with complaints of sudden onset of severe upper abdominal pain, associated with nausea and vomiting. He was a known case of acid peptic disease.[ncbi.nlm.nih.gov]
  • A 55-year-old man with dextrocardia who suffered from abdominal pain was suspected to have multiple arterial thromboembolisms, which might have originated from left atrium thrombosis since he had atrial fibrillation.[ncbi.nlm.nih.gov]
Constipation
  • Patients with diaphragmatic hernia either traumatic or nontraumatic may initially have no symptoms or signs to suggest an injury to the diaphragm.Here, we report a case of a 75-year-old woman diagnosed with irritable bowel syndrome -associated dominant constipation[ncbi.nlm.nih.gov]
Upper Abdominal Pain
  • A 32 years old gentleman, presented in emergency department, with complaints of sudden onset of severe upper abdominal pain, associated with nausea and vomiting. He was a known case of acid peptic disease.[ncbi.nlm.nih.gov]
Heart Disease
  • We report herein for the first time, a case of fetal dextrocardia and situs solitus with complex congenital heart disease in which the FINE method was invaluable in diagnosing multiple abnormalities and defining complex anatomic relationships.[ncbi.nlm.nih.gov]
  • Dextrocardia remains a rare finding in adults, even in a highly select group of patients with known congenital heart disease.[ncbi.nlm.nih.gov]
  • We report a case of a patient with dextrocardia, situs solitus, rheumatic heart disease, severe mitral regurgitation, moderate pulmonary artery hypertension, and severe left ventricular dysfunction who underwent mitral valve replacement using a two-stage[ncbi.nlm.nih.gov]
  • Surgical correction of valvular heart disease in patients with dextrocardia is extremely rare. We report a surgical case of mitral valve repair in a patient with acquired dextrocardia.[ncbi.nlm.nih.gov]
  • Congenital heart disease in infancy and childhood. In: Braunwald E, ed. Heart Disease. 3rd ed. Philadelphia, Pa., W. B. Saunders Co; 1988:963. McAleer E; Kort S; Rosenzweig BP; Katz ES; Tunick PA; Phoon CK; Kronzon I.[rarediseases.org]
Left Ventricular Dysfunction
  • We report a case of a patient with dextrocardia, situs solitus, rheumatic heart disease, severe mitral regurgitation, moderate pulmonary artery hypertension, and severe left ventricular dysfunction who underwent mitral valve replacement using a two-stage[ncbi.nlm.nih.gov]
  • BACKGROUND Poorly controlled ventricular rate associated with atrial fibrillation (AF) leads to tachycardia-induced left ventricular dysfunction.[ncbi.nlm.nih.gov]
Thrombosis
  • A 55-year-old man with dextrocardia who suffered from abdominal pain was suspected to have multiple arterial thromboembolisms, which might have originated from left atrium thrombosis since he had atrial fibrillation.[ncbi.nlm.nih.gov]
Fracture
  • Coats plus syndrome is an autosomal recessive, pleiotropic, multisystem disorder characterized by retinal telangiectasia and exudates, intracranial calcification with leukoencephalopathy and brain cysts, osteopenia with predisposition to fractures, bone[ncbi.nlm.nih.gov]
Salpingitis
  • A mummy of a young woman, who died due to tuberculous peritonitis and salpingitis, is conserved in the Pathological Anatomy Museum of the University of Padua.[ncbi.nlm.nih.gov]

Workup

A safe method in order to definitively confirm suspicion of dextrocardia, with or without situs inversus, is a computed tomography scan (CT), since it enables the physician to accurately pinpoint the position of the internal organs, the position and alignment of the cardiac apex and various other information. An electrocardiogram can also outline inverted waves, that can raise suspicion of the condition. An ultrasonographic scan or a plain X-ray can also reveal signs possibly associated with dextrocardia, but do not suffice in order to establish a diagnosis [21] [22] [23] [24] [25] [26] Should further information be required after performing a CT scan, a magnetic resonance imaging scan can also be carried out [27] [28] [29] [30] .

Pneumoperitoneum
  • X-ray chest showed pneumoperitoneum, with dextrocardia. Ultrasound showed situs inversus. Exploration confirmed the diagnosis of perforated ulcer and situs inversus. Grahm's patch repair of perforation was done. His postoperative recovery was smooth.[ncbi.nlm.nih.gov]
Suppression
  • […] syndrome is an autosomal recessive, pleiotropic, multisystem disorder characterized by retinal telangiectasia and exudates, intracranial calcification with leukoencephalopathy and brain cysts, osteopenia with predisposition to fractures, bone marrow suppression[ncbi.nlm.nih.gov]
Flattened T Wave

Treatment

Dextrocardia cannot be corrected in itself, since the heart and viscera are located in the opposite positions, in comparison to their normal locations. Patients born with it receive solely symptomatic treatment when their condition interferes with vital functions and leads to health problems; however, the majority of the people born with dextrocardia do not face any symptoms and lead normal and healthy lives.

It is important to bear in mind that, should there be a need for an ECG on a person with dextrocardia, the positions of the leads should be reversed in order to obtain a valid result, in terms of cardiac function. Defibrillation should likewise be conducted with the pads positioned in reverse locations (upper left - lower right). Given that individuals with dextrocardia are often simultaneously affected by other cardiac irregularities, such as septal defects, the latter can also be surgically addressed. Other comorbidities are treated also symptomatically: a malfunctioning spleen or the absence of a spleen, often observed in dextrocardia, render the administration of precautionary antibiotics necessary in some occasions.

Prognosis

In general, many people with dextrocardia lead their lives unaffected by the condition. Severer cases, however, are followed by increased morbidity, surgeries to repair the defect and frequent hospitalizations.

Etiology

There are five primary types of dextrocardia, which have been outlined following the observation and analysis of fifty cases of the condition. Every case of dextrocardia that is a result of the abnormal growth of the primitive heart tube is termed intrinsic, whereas a cardiac displacement that occurs due to the anomalous positioning of other organs (lungs, diaphragm etc) is termed extrinsic:

  • Type I (intrinsic): Otherwise referred to as "mirror-image" dextrocardia, it involves the mirror-like displacement of the heart in such a way, that the right ventricle and atrium is situated to the left of the left ventricle and atrium; the latter are in their normal location.
  • Type II (intrinsic): The right ventricle and atrium are positioned to the right of the left chambers, as in every healthy individual. Depending on the degree of dextroversion present (complete, incomplete or mesoversion) the apex of the heart is in an abnormal position. In cases of complete dextroversion, for instance, the apex maintains an anterior position in the right side.
  • Type III (intrinsic): In these cases, the atria or ventricles are inverted. The condition is termed "mixed" dextrocardia, since the positioning resembles the one seen in mirror-image dextrocardia or cardioversion.
  • Type IV (intrinsic): The fourth type involves a displacement of the heart as an organ.The chambers are all arranged normally, but the organ is placed in the middle of the thorax.
  • Type V (extrinsic): The heart is positioned in a different location due to the misplacement of other thoracic structures, which is referred to as situs inversus. Its exact position may vary between an anterior switch (dextroposition, simple) or followed by a rotation as well.

Epidemiology

Dextrocardia is a relatively rare condition, with 1 child in every 12,000 live births being born with the condition. There is no ethnic or gender-related predilection [1].

Sex distribution
Age distribution

Pathophysiology

From an embryologic point of view, the heart develops from the primitive heart tube, which is formed after the two endocardial tubes are conjoined. The primitive heart tube lacks mobility and plasticity on both its ends; the middle region is the structure that loops, in order to give shape to the atria, ventricles, sinus venosus, bulbus cordis and truncus arteriosus. The right and left atrium are the first to be formed in an embryo, with their situs being in correspondence to the visceral situs, for this reason, an displacement of the atrium is usually attributed to an anomaly affecting the visceral situs [2].

The bulboventricular region is the part of the primitive heart tube that is curved, in order to produce the aforementioned structures. Its precise direction and bending style is responsible for the potential misplacement of the apex and dextrocardic phenomena. If it is bent in the style of a "D", the cardiac apex is moved from the right side of the thoracic cavity to the left one quite early during gestation. Should the bulboventricular region form the shape of an "L", however, the cardiac apex moves to the left side of the chest. In some cases, the heart's apex does not migrate to the left side of the thorax, even though there is a D-loop, and the resulting condition is known as dextroversion. Quite similarly, an abnormal L-loop of the bulboventricular region also leads to dextrocardia. Large vessels may also accompany a heart that is affected by dextrocardia, alongside other manifestations, including the existence of only one ventricle, atresia of the tricuspid valve, etc [3].

Prevention

Dextrocardia is a congenital defect and there is no substantial advice concerning its prevention. However, it is believed that, during pregnancy, avoiding drugs such as cocaine and following a correct therapeutic scheme in cases of diabetes mellitus can prevent some cases of dextrocardia. Families can receive genetic counseling if there is a family history of the condition.

Summary

Dextrocardia is a congenital defect that affects the heart. It involves the malpositioning of the heart, which, instead of the left hemithorax, is located on the right hemithorax. Cardiac chambers are also malpositioned. The condition typically leads to no severe complications or morbidity. It is important to draw attention to the fact that the alteration in the heart's location induces respective alterations on the morphology of the electrocardiogram, when performed on such a patient. Individuals affected by dextrocardia exhibit the following unique electrocardiographic morphology:

  • Negative P wave in lead I
  • Negative QRS complex in lead I
  • R wave, which is tallest in V1 lead and gradually shortens, with the shortest wave being in v6 lead
  • Inverted R wave progression

The aforementioned changes are attributed to the reversed positioning of the cardiac structures. Even though the organ functions normally, in affected individuals depolarization is initiated on the left and is transmitted to the right, since the chambers and atria are located on the opposite of the normal side. A subcategory of dextrocardia is dextrocardia with situs inversus. This condition is characterized not only by the abnormal position of the heart itself, but some or all other viscera (spleen, liver, lungs) are also in a reversed location.

Patient Information

Dextrocardia is a congenital condition. This means that a person cannot develop it during the course of their lives, but it is born with it. Dextrocardia actually means a "right heart": people born with it have their heart in an abnormal position, either on the right side of the trunk, instead of the left, or with the lower frontal part being directed to the right, instead of to the left. Dextrocardia is often accompanied by other abnormalities. Many times it is not the only organ to be positioned in the wrong location, but the rest of the internal organs, such as the lungs, spleen or diaphragm are reversed as well (dextrocardia with situs inversus). The condition is diagnosed with an electrocardiogram, an ultrasonographic scan and a CT scan. Patients most of the times do not need any treatment and experience no symptoms or health problems; in more severe cases, however, surgical treatment, the administration of antibiotics and other measures are taken in order to ensure that the individual can be fully functional.

References

Article

  1. Bohun CM, Potts JE, Casey BM et al. A population-based study of cardiac malformations and outcomes associated with dextrocardia. Am. J. Cardiol. 2007; 100 (2): 305-9. doi:10.1016/j.amjcard.2007.02.095.
  2. Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: imaging of the heterotaxy syndrome. RadioGraphics 1999; 19:837-852.
  3. Elliott LP, Jue KL, Amplatz K. A roentgen classification of cardiac malpositions. Invest Radiol 1966; 1:17-28.
  4. Maldjian PD, Saric M. Approach to dextrocardia in adults: review. AJR Am J Roentgenol. 2007; 188 (6): S39-49. doi:10.2214/AJR.06.1179.
  5. Cotran RS, Kumar V, Robbins SL. Robbins Pathologic Basis of Disease. 4th ed. Philadelphia, Pa: WB Saunders Co; 1989. 777.
  6. Fraser RS, Muller NL, Colman NC, Pare PD. Fraser and Pare's Diagnosis of Diseases of the Chest. 4th ed. Philadelphia, Pa: WB Saunders Co; 1999. Vol 3: 2281-3.
  7. Gutgesell HP. Cardiac malposition and heterotaxy. Garson AG Jr, Fisher DJ, Neish SR, eds. Science and Practice of Pediatric Cardiology. 2nd ed. Baltimore, Md: Williams & Wilkins; 1998. Vol 2: 1539-61.
  8. Hagler DJ, O'Leary PW. Cardiac malpositions and abnormalities of atrial and visceral situs. Emmanouilides GC, Riemenschneider TA, Allen HD, Gutgesell HP, eds. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 5th ed. Baltimore, Md: Williams & Wilkins; 1995. Vol 2: 1307-36.
  9. Higgins CB. Essentials of Cardiac Radiology and Imaging. Philadelphia, Pa: JB Lippincott Co; 1992. 283-331.
  10. Jefferson K, Rees S. Clinical Cardiac Radiology. 2nd ed. London, UK: Butterworths; 1980. 9-67.
  11. Perloff JK. Clinical Recognition of Congenital Heart Disease. 4th ed. Philadelphia, Pa: WB Saunders Co; 1994.
  12. Winer-Muram HT. Adult presentation of heterotaxic syndromes and related complexes. J Thorac Imaging. 1995. 10(1):43-57.
  13. Maldjian PD, Saric M. Approach to dextrocardia in adults: review. AJR Am J Roentgenol. 2007 Jun. 188(6 suppl):S39-49; quiz S35-8.
  14. Gindes L, Hegesh J, Barkai G, Jacobson JM, Achiron R. Isolated levocardia: prenatal diagnosis, clinical importance, and literature review. J Ultrasound Med. 2007 Mar. 26(3):361-5.
  15. Fung TY, Chan DL, Leung TN, Leung TY, Lau TK. Dextrocardia in pregnancy: 20 years' experience. J Reprod Med. 2006 Jul. 51(7):573-7.
  16. Douglas YL, Jongbloed MR, den Hartog WC, Bartelings MM, Bogers AJ, Ebels T, et al. Pulmonary vein and atrial wall pathology in human total anomalous pulmonary venous connection. Int J Cardiol. 2008 Dec 29.
  17. Xu BP, Shen KL, Hu YH, Feng XL, Li HM, Lang ZQ. [Clinical characteristics of primary ciliary dyskinesia in children]. Zhonghua Er Ke Za Zhi. 2008 Aug. 46(8):618-22.
  18. Schrott-Fischer A, Rieger G, Morass B, Bitsche M, Horak E, Riechelmann H, et al. [Diagnostics of primary ciliary dyskinesia]. Laryngorhinootologie. 2008 Nov. 87(11):809-20; quiz 821-5.
  19. Van Mierop LH, Eisen S, Schiebler GL. The radiographic appearance of the tracheobronchial tree as an indicator of visceral situs. Am J Cardiol. 1970 Oct. 26(4):432-5.
  20. Palumbo E. [Neonatal diagnosis of primary ciliary dyskinesia. Recent advances]. Recenti Prog Med. 2008 Apr. 99(4):207-9.
  21. Lee SE, Kim HY, Jung SE, et al. Situs anomalies, and gastrointestinal abnormalities. J Pediatr Surg. 2006 Jul. 41(7):1237-42.
  22. Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: imaging of the heterotaxy syndrome. Radiographics. 1999 Jul-Aug. 19(4):837-52; discussion 853-4.
  23. Silverman NH. An ultrasonic approach to the diagnosis of cardiac situs, connections, and malpositions.Cardiol Clin. 1983 Aug. 1(3):473-86.
  24. Tonkin IL, Tonkin AK. Visceroatrial situs abnormalities: sonographic and computed tomographic appearance. AJR Am J Roentgenol. 1982 Mar. 138(3):509-15.
  25. Partridge J. The radiological evaluation of atrial situs. Clin Radiol. 1979 Jan. 30(1):95-103.
  26. Kashiwagi S, Ishikawa T, Onoda N, Kawajiri H, Takashima T, Hirakawa K. Laparoscopic adrenalectomy in a patient with situs inversus. JSLS. 2013. 17(3):487-90.
  27. Bartram U, Fischer G, Kramer HH. Congenitally interrupted inferior vena cava without other features of the heterotaxy syndrome: report of 5 cases and characterization of a rare entity. Pediatr Dev Pathol. 2007 Mar 22. 1 [epub ahead of print.
  28. Yoo SJ, Kim YM, Choe YH. Magnetic resonance imaging of complex congenital heart disease. Int J Card Imaging. 1999 Apr. 15(2):151-60.
  29. Oui H, Kim J, Bae Y, Oh J, Park S, Lee G, et al. Computed Tomography Angiography of Situs Inversus, Portosystemic Shunt and Multiple Vena Cava Anomalies in a Dog. J Vet Med Sci. 2013 Jul 9.
  30. Kashiwagi S, Ishikawa T, Onoda N, Kawajiri H, Takashima T, Hirakawa K. Laparoscopic adrenalectomy in a patient with situs inversus. JSLS. 2013. 17(3):487-90.

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Last updated: 2017-08-09 17:28