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Pathology presentation by Heather Spencer, Leah Shaver, Rachel Nottrodt, Julia Inneo

Heather Spencer

on 30 October 2012

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Transcript of Dextrocardia

Congenital Dextrocardia
with Situs Inversus Leah, Rachel, Julia and Heather Developmental Pathogenesis Diagnosis and Treatment Diagnosis Treatment Potential Contributing Factor? Embryological Development What is Dextrocardia? Chamber Pathology Defining Terms Pathology of Dextrocardia "Dextro" - Right
"Cardia" - Heart
"Situs" - Position
"Inversus" - Asymmetric Dextrocardia is a congenital cardiac malformation http://nathansprayer.com/2009/03/26/dextrocardia/ Heart a MIRROR IMAGE
CONGENITAL cardiac malformation & malposition
INTRINSIC to heart
Apex points RIGHT What’s wrong with this picture? Screening Ultrasounds
Fetal Echocardiography X-rays
Bronchial Morphology
Surgical Procedures
ECG Autopsy Fetal Later in Life Dextrocardia
Fontan Procedure
Diaphragm hernia repair
Patent ductus ligation
Blalock-Taussig shunt
Ventricular septal defect closure
Ladd’s procedure
Lower lung lobectomy Dextrocardia with other Congenital Defects Fontan Prevalence and Prognosis Catherine O’hara Symptoms Absolutely Nothing!
Respiratory Distress
Systolic Murmur
Cardiac disease
Gastric symptoms Randy Foye Positive correlation exists between Dextrocardia and Situs Inversus Genetic Component? Cockayne (1938) suggested that situs inversus has Mendelian autosomal recessive inheritance
Generational distribution within family
Affected monozygotic twins http://www.nlm.nih.gov/ RL Kartagener’s Syndrome
Present in 25% of cases
Triad of situs inversus, sinusitis, and bronchiectasis
Widespread ciliary disorder
Ciliary action key to heart looping LL QUESTIONS? 3:1 Ration of women vs men
2.5:1 Ratio of men vs women
Estimated incidence in 1947 was between 1 in 35,000 to 1 in 6,000
In 2010 incidence was reported to be 1 in 10,000
In 5,539 fetal echocardiograms 85 cases of dextrocardia were seen. [1] [1,2] [3,4] [5] [6] [2] [2,3,7,8] [3] [3] [2] [7] [9] [3] [10] [2,5,11] [12] http://www.babycenter.ca/pregnancy/antenatalhealth/scans/secondtrimesterscans/ http://physics.aps.org/story/v4/st25 http://www.heart-valve-surgery.com/heart-surgery-blog/2007/09/03/danger-of-angiogram-risks-of-angiography/ http://remomd.com/training/tormented-learning-a-paradigm-shift.html [13,14] [2,12] http://drpinna.com/the-gold-standard-22948/gold-standard-2 [4] [15] [1] http://files.gamebanana.com/img/ico/sprays/absolutely_nothing.png http://remomd.com/training/tormented-learning-a-paradigm-shift.html http://www.turningnewsintoknowledge.com/2011/07/25/want-to-make-sense-of-social-media-ask-the-right-questions/question-mark/ http://en.wikipedia.org/wiki/Fontan_procedure [14] [14] [9] [16] [12] [4] http://hellogiggles.com/everything-i-need-to-know-i-learned-from-home-alone http://people.zap2it.com/p/catherine-ohara/1279?aid=paalca http://www.nba.com/playerfile/randy_foye/ 1. Bindra, S., Tabibiazar, R., Mazar, M., Dave, R. (2011). Dextrocardia with Situs Inversus: Through the Looking Glass with an ECG. UCLA Heathcare, 15, 1-3.
2. Perloff, J. K. (2011). The cardiac malpositions. American Journal of Cardiology, 108(9), 1352-1361. doi: 10.1016/j.amjcard.2011.06.055
3. Maldjian, P. D., & Saric, M. (2007). Approach to dextrocardia in adults: Review. American Journal of Roentgenology, 188(6), S39-S49. doi: 10.2214/AJR.06.1179
4. Evans, W. N., Acherman, R. J., Collazos, J. C., Castillo, W. J., Rollins, R. C., Kip, K. T., & Restrepo, H. (2010). Dextrocardia: Practical clinical points and comments on terminology. Pediatric Cardiology, 31(1), 1-6. doi: 10.1007/s00246-009-9516-0
5. Vanpraagh, R., Keith, J., Vlad, P., & Vanpraagh.S. (1964). Anatomic types of congenital dextrocardia - diagnostic + embryologic implications. American Journal of Cardiology, 13(4), 510-&. doi: 10.1016/0002-9149(64)90159-6
6. Radhika, D., Rekha, N., Mohan, K. (2011). Dextrocardia with situs inversus- a case report. International Journal of Anatomical Variation 4:88-89
7. Lev, M., Liberths, R.R., Eckner, F., & Arcilla, R. (1968). Pathologic anatomy of dextrocardia and its clinical implications. Circulation, 37(6), 979-999.
8. Grant, R. (1958). Syndrome of dextroversion of the heart. Circulation, 18(1), 25-36.
9. Rosenberg, H., & Rosenberg, I. (1949). Simultaneous association of situs inversus, coronary heart disease and hiatus hernia - report of a case and review of literature. Annals of Internal Medicine, 30(4), 851-859.
10. Cockayne, E. (1938). The genetics of transposition of the viscera. Quarterly Journal of Medicine, 7(27), 479-493.
11. Solzbach, U., Beuter, M., Hartig, B., & Haas, H. (2010). Isolated dextrocardia with situs solitus (dextroversion). Herz, 35(3), 207-210. doi: 10.1007/s00059-010-3279-5
12. Yildirim, S. V., Tokel, K., Varan, B., Aslamaci, S., & Ekici, E. (2007). Clinical investigations over 13 years to establish the nature of the cardiac defects in patients having abnormalities of lateralization. Cardiology in the Young, 17(3), 275-282. doi: 10.1017/S1047951107000479
13. Bohun, C. M., Potts, J. E., Casey, B. M., & Sandor, G. G. S. (2007). A population-based study of cardiac malformations and outcomes associated with dextrocardia. American Journal of Cardiology, 100(2), 305-309. doi: 10.1016/j.amjcard.2007.02.095
14. Walmsley, R., Hishitani, T., Sandor, G., Lim, K., Duncan, W., Tessier, F., . . . Potts, J. (2004). Diagnosis and outcome of dextrocardia diagnosed in the fetus. American Journal of Cardiology, 94(1), 141-143. doi: 10.1016/j.amjcard.2004.03.049
15. Jenkins, D & Gerred, S. (2011). ECGs by example. Toronto: Elsevier Churchill Livingstone.
16. Garg, N., Agarwal, B., Modi, N., Radhakrishnan, S., & Sinha, N. (2003). Dextrocardia: An analysis of cardiac structures in 125 patients. International Journal of Cardiology, 88(2-3), 143-155. doi: 10.1016/S0167-5273(02)00539-9 Bibliography
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