- Email: [email protected]
Mirror image dextrocardia with situs inversus!
Visual Journal of Emergency Medicine 8 (2017) 53–56
Contents lists available at ScienceDirect
Visual Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/visj
Visual Case Discussion
Mirror image dextrocardia with situs inversus!
MARK
⁎
Abhijit S. Nair , Asiel Christopher, Basanth Kumar Rayani Department of Anaesthesiology, Basavatarakam Indo-American Cancer Hospital and Research Institute, Road No. 10, Banjara Hills, Hyderabad, Telangana, India
A R T I C L E I N F O Keywords: Dextrocardia Situs inversus Electrocardiogram Heart
During pre-anaesthesia evaluation of an 18 year old boy for palatoplasty, we encountered his postero-anterior view chest radiograph in which the cardiac shadow was seen in the right hemithorax (Image 1). The gastric bubble was also seen on the right side. The boy was asymptomatic and was physically active with no dyspnea on exertion. His resting heart rate was 80 beats per minute and his blood pressure was 124/80 mm of mercury. His heart sounds were audible on auscultation on right side and the apical impulse was also palpable on the right. We requested a 12 lead electrocardiogram (12 lead ECG) to evaluate any conduction abnormalities, a 2-dimensional echocardiogram (2D ECHO) to know biventricular function and presence of any structural abnormalities and an ultrasonography( USG) of abdomen to know whether the dextrocardia is associated with situs inversus. 12 lead ECG with standard lead placement revealed inverted T waves in lead I and aVL, deep S wave in lead I,tall R wave in V1, a reversed R wave progression from V1 to V6 leads and flattenned T waves in V4-V6 (Image 2). We requested a 12 lead ECG with reverse lead placement. The findings were RSR in lead V1, right axis deviation with left ventricular hypertrophy by voltage which was normal for his age (Image 3). The 2D ECHO findings showed normal biventricular function with an ejection fraction of 60%, no septal defects, no regional wall motion abnormalities and no pericardial effusion. The USG findings were consistent with situs inversus (liver and gall bladder on left side and stomach, spleen on right side). Dextrocardia is the presence of heart in the right hemithorax which is not due to extracardiac abnormalities. Cardiac dextroposition is due
⁎
Corresponding author. E-mail address: [email protected] (A.S. Nair).
http://dx.doi.org/10.1016/j.visj.2017.04.018 Received 8 April 2017; Accepted 22 April 2017 2405-4690/ © 2017 Elsevier Inc. All rights reserved.
to displacement of heart to the right secondary to conditions like hypoplastic lung,post right pneumonectomy or a diaphragmatic hernia. In cardiac dextroversion, there is abnormal rotation of the apex of the heart. This is usually associated with conditions like septal defects, anamolous venous return, tetralogy of fallot, coarctation of aorta, pulmonary stenosis.1 Dextrocardia is of several types. In situs solitus, the dextrocardia is associated with normal orientation of great arteries. In situs inversus, the great vessels are usually inversely placed in the thoracic cavity. In situs ambiguous where there is asplenia or polysplenia, the presence and origin of great vessels from ventricles are variable.2 Isolated dextrocardia is usually associated with other malformations of heart like septal defects, single outlet ventricles or tricuspid atresia. Mirror image dextrocardia with situs inversus is when the heart and the abdominal organs are placed in opposite side.3 To know this is important so as to diagnose and treat abdominal pathologies. Kartagener's syndrome is found in 25% patients with mirror image dextrocardia with situs inversus. In this syndrome, there is a triad of situs inversus, paranasal sinusitis and bronchiectasis. Appendix A. Supplementary material Supplementary data associated with this article can be found in the online version at doi:10.1016/j.visj.2017.04.018.
Visual Journal of Emergency Medicine 8 (2017) 53–56
A.S. Nair et al.
Image 1. The PA view chest radiograph shows heart shadow in right hemithorax along with gastric bubble on the right side.
Image 2. A 12 lead ECG with normal lead placement shows abnormalities in lead I, aVL and V1-V6 not consistent with an 18 yr old aysmptomatic patient.
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Visual Journal of Emergency Medicine 8 (2017) 53–56
A.S. Nair et al.
Image 3. A 12 lead ECG with reverse lead placement which is essentially normal.
References 1 Gonçalves LFG, Souto FMS, Faro FN, et al. Dextrocardia with situs inversus associated with non-compaction cardiomyopathy. Arq Bras Cardiol. 2013;101(2):e33–e36. 2 Maldjian PD, Saric M. Approach to dextrocardia in adults: review. Am J Roentgenol. 2007;188(Suppl. 6) [S39-49; quiz S35-8]. 3 Garg N, Agarwal BL, Modi N, Radhakrishnan S, Sinha N. Dextrocardia: an analysis of cardiac structures in 125 patients. Int J Cardiol. 2003;88(2–3) [143-55; discussion 155-6].
Answers 1. False. Explanation: Theoretically, there are many types of dextrocardia. With situs solitus, one may have dextrocardia with normally related great arteries. With situs inversus, one may have dextrocardia with inversely related great arteries. With situs ambiguous (either polysplenia or asplenia), one may have dextrocardia with any of the preceding relationships between the ventricles and great vessels.
Questions 1. Dextrocardia always presents with abnormal great arteries? a. True b. False
2. True. Explanation: Kartagener's syndrome occurs in 25% of individuals who have mirror-image dextrocardia. This disorder is characterized by the triad of situs inversus, paranasal sinusitis, and bronchiectasis. The incidence is estimated to be 1–2/30,000. The primary abnormality is termed “immotile cilia syndrome” or “primary ciliary dyskinesia” and stems from defective ciliary motility due to genetically determined structural abnormalities. They present with recurrent lung infections, bronchiectasis, chronic sinusitis, and otitis media due to impaired mucociliary clearance. Ciliary dysfunction also causes reduced fertility in women and sterility in men from reduced motility of spermatozoa.
2. Kartagener's syndrome is associated with mirror image dextrocardia? a. True b. False 3. In dextrocardia, there are no other structural cardiac anomalies? a. True b. False 4. Dextrocardia and dextroposition are the same entities? a. True b. False
3. False. Explanation: Incidence of congenital cardiac anomalies in dextrocardia with situs inversus is low as compared to congenital cardiac anomalies in isolated dextrocardia.Incidence of Total situs inversus is around1:10,000. Dextrocardia can also be associated with more complex cardiac malformations, such as single ventricle, double-outlet or double-inlet ventricles, and tricuspid atresia.
5. Dextroversion and dextrocardia are different entities? a. True b. False
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Visual Journal of Emergency Medicine 8 (2017) 53–56
A.S. Nair et al.
incidence of additional cardiac malformations like anomalous pulmonaryvenous return, tetralogy of Fallot, septal defects, pulmonic stenosis, coarctation of the aorta, and corrected transposition of great arteries. Isolated dextroversion, without associated congenital cardiac deformities,is rare; but such patients are usually asymptomatic, and discovery of the condition is delayed till they get evaluated for acquired cardiac disease.
4. False. Explanation: Dextrocardia is different from cardiac dextroposition, which is the displacement of the heart to the right secondary to extracardiac causes such as right lung hypoplasia,right pneumonectomy, or diaphragmatic hernia. 5. True. Explanation: In dextroversion, there is abnormal rotation of the apex into the right hemithorax. In dextroversion, there is a 90%
56
Contents lists available at ScienceDirect
Visual Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/visj
Visual Case Discussion
Mirror image dextrocardia with situs inversus!
MARK
⁎
Abhijit S. Nair , Asiel Christopher, Basanth Kumar Rayani Department of Anaesthesiology, Basavatarakam Indo-American Cancer Hospital and Research Institute, Road No. 10, Banjara Hills, Hyderabad, Telangana, India
A R T I C L E I N F O Keywords: Dextrocardia Situs inversus Electrocardiogram Heart
During pre-anaesthesia evaluation of an 18 year old boy for palatoplasty, we encountered his postero-anterior view chest radiograph in which the cardiac shadow was seen in the right hemithorax (Image 1). The gastric bubble was also seen on the right side. The boy was asymptomatic and was physically active with no dyspnea on exertion. His resting heart rate was 80 beats per minute and his blood pressure was 124/80 mm of mercury. His heart sounds were audible on auscultation on right side and the apical impulse was also palpable on the right. We requested a 12 lead electrocardiogram (12 lead ECG) to evaluate any conduction abnormalities, a 2-dimensional echocardiogram (2D ECHO) to know biventricular function and presence of any structural abnormalities and an ultrasonography( USG) of abdomen to know whether the dextrocardia is associated with situs inversus. 12 lead ECG with standard lead placement revealed inverted T waves in lead I and aVL, deep S wave in lead I,tall R wave in V1, a reversed R wave progression from V1 to V6 leads and flattenned T waves in V4-V6 (Image 2). We requested a 12 lead ECG with reverse lead placement. The findings were RSR in lead V1, right axis deviation with left ventricular hypertrophy by voltage which was normal for his age (Image 3). The 2D ECHO findings showed normal biventricular function with an ejection fraction of 60%, no septal defects, no regional wall motion abnormalities and no pericardial effusion. The USG findings were consistent with situs inversus (liver and gall bladder on left side and stomach, spleen on right side). Dextrocardia is the presence of heart in the right hemithorax which is not due to extracardiac abnormalities. Cardiac dextroposition is due
⁎
Corresponding author. E-mail address: [email protected] (A.S. Nair).
http://dx.doi.org/10.1016/j.visj.2017.04.018 Received 8 April 2017; Accepted 22 April 2017 2405-4690/ © 2017 Elsevier Inc. All rights reserved.
to displacement of heart to the right secondary to conditions like hypoplastic lung,post right pneumonectomy or a diaphragmatic hernia. In cardiac dextroversion, there is abnormal rotation of the apex of the heart. This is usually associated with conditions like septal defects, anamolous venous return, tetralogy of fallot, coarctation of aorta, pulmonary stenosis.1 Dextrocardia is of several types. In situs solitus, the dextrocardia is associated with normal orientation of great arteries. In situs inversus, the great vessels are usually inversely placed in the thoracic cavity. In situs ambiguous where there is asplenia or polysplenia, the presence and origin of great vessels from ventricles are variable.2 Isolated dextrocardia is usually associated with other malformations of heart like septal defects, single outlet ventricles or tricuspid atresia. Mirror image dextrocardia with situs inversus is when the heart and the abdominal organs are placed in opposite side.3 To know this is important so as to diagnose and treat abdominal pathologies. Kartagener's syndrome is found in 25% patients with mirror image dextrocardia with situs inversus. In this syndrome, there is a triad of situs inversus, paranasal sinusitis and bronchiectasis. Appendix A. Supplementary material Supplementary data associated with this article can be found in the online version at doi:10.1016/j.visj.2017.04.018.
Visual Journal of Emergency Medicine 8 (2017) 53–56
A.S. Nair et al.
Image 1. The PA view chest radiograph shows heart shadow in right hemithorax along with gastric bubble on the right side.
Image 2. A 12 lead ECG with normal lead placement shows abnormalities in lead I, aVL and V1-V6 not consistent with an 18 yr old aysmptomatic patient.
54
Visual Journal of Emergency Medicine 8 (2017) 53–56
A.S. Nair et al.
Image 3. A 12 lead ECG with reverse lead placement which is essentially normal.
References 1 Gonçalves LFG, Souto FMS, Faro FN, et al. Dextrocardia with situs inversus associated with non-compaction cardiomyopathy. Arq Bras Cardiol. 2013;101(2):e33–e36. 2 Maldjian PD, Saric M. Approach to dextrocardia in adults: review. Am J Roentgenol. 2007;188(Suppl. 6) [S39-49; quiz S35-8]. 3 Garg N, Agarwal BL, Modi N, Radhakrishnan S, Sinha N. Dextrocardia: an analysis of cardiac structures in 125 patients. Int J Cardiol. 2003;88(2–3) [143-55; discussion 155-6].
Answers 1. False. Explanation: Theoretically, there are many types of dextrocardia. With situs solitus, one may have dextrocardia with normally related great arteries. With situs inversus, one may have dextrocardia with inversely related great arteries. With situs ambiguous (either polysplenia or asplenia), one may have dextrocardia with any of the preceding relationships between the ventricles and great vessels.
Questions 1. Dextrocardia always presents with abnormal great arteries? a. True b. False
2. True. Explanation: Kartagener's syndrome occurs in 25% of individuals who have mirror-image dextrocardia. This disorder is characterized by the triad of situs inversus, paranasal sinusitis, and bronchiectasis. The incidence is estimated to be 1–2/30,000. The primary abnormality is termed “immotile cilia syndrome” or “primary ciliary dyskinesia” and stems from defective ciliary motility due to genetically determined structural abnormalities. They present with recurrent lung infections, bronchiectasis, chronic sinusitis, and otitis media due to impaired mucociliary clearance. Ciliary dysfunction also causes reduced fertility in women and sterility in men from reduced motility of spermatozoa.
2. Kartagener's syndrome is associated with mirror image dextrocardia? a. True b. False 3. In dextrocardia, there are no other structural cardiac anomalies? a. True b. False 4. Dextrocardia and dextroposition are the same entities? a. True b. False
3. False. Explanation: Incidence of congenital cardiac anomalies in dextrocardia with situs inversus is low as compared to congenital cardiac anomalies in isolated dextrocardia.Incidence of Total situs inversus is around1:10,000. Dextrocardia can also be associated with more complex cardiac malformations, such as single ventricle, double-outlet or double-inlet ventricles, and tricuspid atresia.
5. Dextroversion and dextrocardia are different entities? a. True b. False
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Visual Journal of Emergency Medicine 8 (2017) 53–56
A.S. Nair et al.
incidence of additional cardiac malformations like anomalous pulmonaryvenous return, tetralogy of Fallot, septal defects, pulmonic stenosis, coarctation of the aorta, and corrected transposition of great arteries. Isolated dextroversion, without associated congenital cardiac deformities,is rare; but such patients are usually asymptomatic, and discovery of the condition is delayed till they get evaluated for acquired cardiac disease.
4. False. Explanation: Dextrocardia is different from cardiac dextroposition, which is the displacement of the heart to the right secondary to extracardiac causes such as right lung hypoplasia,right pneumonectomy, or diaphragmatic hernia. 5. True. Explanation: In dextroversion, there is abnormal rotation of the apex into the right hemithorax. In dextroversion, there is a 90%
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