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Table of Contents
CASE REPORT
Year : 2021  |  Volume : 11  |  Issue : 1  |  Page : 32-36

Working in the mirror: A case series on interventions in children having dextrocardia with congenital heart disease


Department of Pediatric Cardiology, NH RTIICS, Kolkata, West Bengal, India

Date of Submission05-Apr-2020
Date of Decision15-May-2020
Date of Acceptance11-Jun-2020
Date of Web Publication18-Feb-2021

Correspondence Address:
Dr. Mahua Roy
931 Jawpur Road, Kolkata - 700 074, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JICC.JICC_21_20

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  Abstract 


This case series comprises of five children having dextrocardia with congenital heart disease who underwent percutaneous interventional procedures. Four of them underwent percutaneous device closure and one had percutaneous balloon coarctoplasty. There are a few reports of percutaneous coronary intervention in adults with dextrocardia, however barring a few isolated case reports, literature on intervention in children with dextrocardia is limited?.

Keywords: Congenital heart disease, dextrocardia, interventions


How to cite this article:
Roy M, Gangopadhyay D, Patel P. Working in the mirror: A case series on interventions in children having dextrocardia with congenital heart disease. J Indian coll cardiol 2021;11:32-6

How to cite this URL:
Roy M, Gangopadhyay D, Patel P. Working in the mirror: A case series on interventions in children having dextrocardia with congenital heart disease. J Indian coll cardiol [serial online] 2021 [cited 2021 Mar 6];11:32-6. Available from: https://www.joicc.org/text.asp?2021/11/1/37/309618




  Introduction Top


Dextrocardia is a rare entity with an estimated prevalence of 0.83 in 10,000. Percutaneous interventions in dextrocardia are challenging due to abnormal orientation of the heart. Interventions in dextrocardia require modifications of imaging angles and in the catheter maneuvering techniques. There are very limited number of case reports about cardiac interventions in dextrocardia with congenital heart disease (CHD). Due to the unusual orientation of the heart (difference in morphology) and consequent different orientation of the common defects and the conduction system, there are unique challenges during interventions in catheterization laboratory. The series of cases presented here provide an insight into the various challenges faced during percutaneous interventions in CHD with dextrocardia and tricks to overcome them.


  Case Reports Top


Case 1

A 14-year-old boy was referred to us for incidental detection of murmur on the right side of the chest. Clinical examination detected an apparently healthy adolescent boy weighing 77 kg with apex beat on the right 5th intercostal space, liver dullness on the left 5th intercostal space, and stomach tympanicity on the right upper abdomen. His first heart sound (S1) was normal, and the second heart sound was fixed (S2) and widely split with Grade III/VI ejection systolic murmur (ESM) on the right 2nd intercostal space. A 12-lead electrocardiogram (ECG) pointed out situs inversus (SI) and dextrocardia with volume overload of left-sided right atrium (RA) and right ventricle (RV); chest X-ray detected SI and dextrocardia, cardio-thoracic ratio of 55', and mild increase in pulmonary vascular markings. Transthoracic echocardiography (TTE) confirmed SI, dextrocardia, normally related great arteries (I, L, and I), right aortic arch with mirror image branching pattern with moderate ostium secundum atrial septal defect (OS-ASD), and dilated RA and RV with mild tricuspid regurgitation (TR) with pressure gradient of 25 mmHg. For better assessment of ASD rims, transesophageal echocardiography (TEE) was done which detected 16 mm, OS-ASD with deficient retro-aortic rim, and other rims were adequate. Percutaneous device closure was advised, and written consent was taken from parents. The procedure was performed under general anesthesia in view of the expected prolonged procedure time due and also to have a better control of the hemodynamics in case of any untoward event. Right heart catheterization was done, and Qp/Qs obtained was 1.8:1. The defect was crossed with a 5 French (F) multipurpose catheter on anteroposterior (AP) projection, and the catheter was kept in the pulmonary vein [Figure 1]. An 18-mm Amplatzer septal occluder (ASO) (AGA Medical Corporation, Golden Valley, MN, USA) was delivered through an 8 (F) Amplatzer ASD delivery sheath (AGA Medical Corporation, Golden Valley, MN, USA) and positioned across the defect under fluoroscopy [Figure 2]. Device position was assessed by TEE and fluoroscopy on right lateral (15°) and right anterior oblique (RAO), cranial (RAO 30°/cranial 30°) views. The device was released under fluoroscopic guidance. The patient tolerated the procedure well, was extubated on table, and was shifted to the pediatric intensive care unit (PICU). He was discharged after 48 h with oral aspirin (5 mg/kg/day). He has been on follow-up for the last 2 years and has remained well.
Figure 1: Deployment of atrial septal defect device in dextrocardia from the pulmonary vein

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Figure 2: Atrial septal defect device closure in anteroposterior projection

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Case 2

A 6-year-old girl referred for incidental detection of murmur on the right side of the chest. Clinical examination detected SI and dextrocardia. On cardiovascular examination, apex beat was on the right 5th intercostal space, S1 was normal, and S2 was wide and fixed split with Grade III/IV ESM audible on right parasternal region. ECG and chest X-ray were consistent with SI and dextrocardia. Echocardiography confirmed SI, dextrocardia with 19 mm of OS-ASD with adequate rims, and dilated RA and RV with mild TR with a pressure gradient of 18 mmHg. Transcatheter device closure was planned, and consent for the procedure was obtained from the parents. The procedure was done under conscious sedation. A 20-mm Amplatzer septal occlude (AGA Medical Corporation, Golden Valley, MN, USA) was deployed under fluoroscopic guidance on AP projection and RAO views. Device position was confirmed by TTE and was deployed under fluoroscopic guidance. The patient remained well during and after the procedure, and there were no immediate complications. She was discharged after 48 h with oral aspirin (5 mg/kg/day) and is well on follow-up for the last 3 years.

Case 3

A 15-year-old male weighing 43.6 kg with dextrocardia was referred for cardiac evaluation. Clinical examination detected SI, dextrocardia with cardiomegaly, and left ventricular (LV) type of apex. Apex beat was palpable on the 6th intercostal space just outside the right mid-clavicular line. Systolic thrill was palpable on the right parasternal region. S1 was normal, whereas S2 was wide and variably split; a LV third heart sound (S3) and a soft mid-diastolic murmur were audible over the right-sided apical area. Grade IV/VI pansystolic murmur was detected on the right parasternal region. Chest X-ray showed SI and dextrocardia with cardiomegaly. ECG was supportive of SI, dextrocardia, and LV volume overload. TTE confirmed SI, dextrocardia, normally related great arteries (I, L, and I), 6-mm peri-membranous ventricular septal defect (VSD) with muscular extension with LV to RV flow with a pressure gradient of 65 mmHg, dilated left atrium (LA), and LV with right aortic arch and mirror image branching. Judging the defect suitable for catheter closure, we proceeded for the same with written consent from the parents He was electively intubated for the procedure and intraprocedure TEE assessment. The measured Qp: Qs was 2:1. We performed LV angiogram in AP, shallow RAO cranial (30°/30?°), and steep RAO cranial (60?°/30°) views [Figure 3]. VSD was crossed with 0.32 × 260 angulated glide wire by wire-only technique from the aortic end on 30°/30° RAO cranial projection and was kept inside the RV cavity. A 6 (F) Amplatzer patent ductus arteriosus (PDA) delivery system was taken over the wire and kept in the RV cavity. A 7-mm Lifetech Cera Muscular VSD occluder device (Lifetech Scientific Shenzen Co Ltd, Lifetech Scientific Corporation, Hi-tech Industrial Park, Nanshan district, Shenzen, PRC) was deployed from the aortic side [Figure 4] and the position was confirmed by TEE and also levophase of RV angiogram. After confirmation of the device position, it was deployed under fluoroscopic and TEE guidance. He was extubated on the table and shifted to the PICU. He remained in normal sinus rhythm and was discharged after 48 h with oral aspirin. He has been well on follow-up with normalization of LA and LV sizes and sinus rhythm.
Figure 3: Left ventricular angiogram with pigtail catheter showing the location of ventricular septal defect in dextrocardia

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Figure 4: Deployment of muscular ventricular septal defect device by retrograde approach

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Case 4

A 5-year-old boy weighing 17 kg with SI and dextrocardia was referred for cardiac evaluation. Clinical examination revealed cardiomegaly on right side of precordium with features of LV type of apex and systolic thrill and pansystolic murmur in right parasternal area. ECG and chest X-ray documented LV volume overload. Echocardiography detected a moderate-sized peri-membranous VSD (4.5 mm) with dilated LA and LV. He was taken up for device closure following written consent by the parents. After angiography in different RAO cranial (30°/30° and 60°/30°) views, VSD was crossed from the aortic side with 0.32 × 260 angulated glide wire that was kept in the left pulmonary artery. A 6F guiding Judkins right catheter was taken over the wire and was kept in the RV apex. A 5 mm × 4 mm Amplatzer duct occluder II (AGA Medical Corporation, Golden Valley, MN, USA) was deployed by retrograde approach. The device position was assessed by TTE, and the absence of aortic regurgitation (AR) was also confirmed. The device was deployed under fluoroscopic guidance. The patient remained stable with sinus rhythm maintained during the procedure with no rhythm disturbances during or after the procedure. He is on regular follow-up, the device is perfectly in place, and no AR has been documented by echocardiography and sinus rhythm by ECG.

Case 5

A 45-day-old male baby weighing 3.7 kg attended the outpatient department from a remote area with poor feeding, lethargy, and fast breathing. Echo revealed situs ambiguous (SA), dextrocardia, interrupted inferior vena cava, bilateral superior vena cava, L-looped ventricle, bicuspid aortic valve, no aortic stenosis or AR with severe juxta ductal coarctation of aorta (COA), moderate-sized PDA with right-to-left shunt (R-L) with severe biventricular dysfunction, and severe pulmonary arterial hypertension. The narrowest CoA segment (2 mm) and? descending thoracic aorta (DTA) at the level of diaphragm (6 mm) were measured. In view of severe biventricular dysfunction, balloon coarctoplasty was considered as a bailout procedure. The baby was electively intubated before being shifted to the catheterization lab. The right femoral artery was accessed with 4 (F) short sheath (Cordis, Santa Clara, CA, USA), and the CoA segment was crossed with a 0.18 straight tip glide wire (Boston Scientific Corporation, Marlborough, Massachusetts, US) and 4 (F) end-hole multipurpose (MPA1) (Cordis, Santa Clara, CA, USA) catheter. Hand angiogram was done in shallow right lateral view (RAO 15°), which did not delineate COA segment properly [Figure 5]. Then, one ventricular angiogram was done in AP projection with pigtail catheter that delineates COA segment in a better way. A 0.14” coronary wire (Cordis, Santa Clara, CA, USA) was kept inside the ventricle, and a 6 × 2 mini Tyshak balloon (B. Braun Interventional Systems Inc., Bethlehem, Pennsylvania, US.) was taken over the wire [Figure 6]. The CoA segment was dilated twice with good result. He was shifted to the PICU and was extubated on the next day. Subsequently, the cardiac function was improved, but the baby succumbed to septicemia later.
Figure 5: Ventricular angiogram in anteroposterior projection delineated coarctation of the aorta

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Figure 6: Confirmation of position of mini Tyshak balloon in anteroposterior projection

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  Discussion Top


Dextrocardia is a rare congenital cardiac malformation with an incidence of 0.83/10,000 births, characterized by displacement of the cardiac apex to the right of the thoracic midline.[1],[2] Dextrocardia with SI, also known as mirror-image dextrocardia, accounts for nearly 40' of all dextrocardia cases and is characterized by heart chambers located exactly opposite to their normal positions. Dextrocardia with SI is the third most common type of dextrocardia in a series of autopsied cases,[3] Situs solitus and SA were more common in that series. The most common subtype of this group is the SI of the viscera and atria (I), L-loop ventricles (L), and normally related great arteries of the inverted type (I), i.e., I, L, and I. This kind of right-sided heart can be structurally normal; however, CHD is the rule occurring in five out of seven autopsies. Associated cardiac anomalies include tetralogy of Fallot of the inverted type, VSD, and ASD. Hakim et al. in 1998 described a successful transcatheter ASD device closure in a dextrocardia patient by ASO device.[4] The difficulties encountered in this patient were due to heart position which was mirror image of normal, therefore, the operators had to get used to this situation during the procedure. The first instance of diagnostic cardiac catheterization in a patient with dextrocardia was reported in 1973 by Hynes. Fourteen years later, Moreyra et al. performed the first balloon angioplasty in coronary artery disease with dextrocardia.[5] The first stent deployment in a patient with dextrocardia in SI was reported in Italy by Patti et al.[6] There are reports of percutaneous coronary interventions and pacemaker implantations in patients with dextrocardia. Ergene et al. published a first case report of VSD device closure in a 23-year-old male in 2009.[7] However, to the best of our knowledge, no case series has been reported in children with CHD associated with dextrocardia undergoing transcatheter procedures. Performing percutaneous interventions in patients with dextrocardia presents several challenges to the interventional cardiologist due to unfamiliarity with the different radiological orientation of the heart and the need for modified views.

The “mirror image technique” which involves reversing the required RAO/left anterior oblique (LAO) angles keeping the cranial/caudal tilts unchanged was used for ease of approach in such patients (our series). The catheters can be passed using the standard technique, except that the catheters are rotated in the opposite direction compared to the patients with normal cardiac anatomy. Note that with the Image inversion technique involves right left image inversion as seen in a plain mirror. However it still requires counter directional torqueing techniques and also reversal of angiographic angles which lead to difficulty in interpretation of angiographic structures. To overcome this, double inversion technique is used which involves right/left image reversal using horizontal sweep reverse button on the machine and reversal of angiographic angle. As a result, final images obtained resemble the normal angiographic images obtained in a levocardia patient. In “double inversion technique,”[8] there is artificial reversal of visualization of responses of catheters and wires to normal manipulation. A combination of right–left reversal of image on the monitor using the “horizontal sweep reverse” function during acquisition and a reversed RAO/LAO angle selection can help in better localization. The images acquired simulate normal cardiac anatomy and make the interpretation easy and avoid error. The advantage of “double inversion technique” is correction of unfamiliar angulated pictures in dextrocardia into the familiar conventional angiographic pictures of a normally located heart. This technique requires a specialized software to be integrated [Table 1].
Table 1: Summary of patient details, hardware, and angiographic views used

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In our case series, we had used reversed imaging after first angiogram on PA view and following that a corresponding view on right lateral or right oblique view. Simultaneous assessment on echocardiography is also important. TEE preoperatively or intraoperatively was done when TTE views were not adequate.


  Conclusion Top


Percutaneous intervention for CHD is challenging in cases of dextrocardia. Challenges are mainly due to the different orientation of the heart and the unfamiliarity of the operator with the radiological views in such cases. In our series, we observed that PA projection worked best for ASD interventions. While in case of VSDs, simple reversal of angulation of the image intensifier was sufficient to give us a good understanding of the defect position. One has to be aware of the different orientation of catheters and device inside the heart. Echocardiography plays a major role in such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Garg N, Agarwal BL, Modi N, Radhakrishnan S, Sinha N. Dextrocardia: An analysis of cardiac structures in 125 patients. Int J Cardiol 2003;88:143-55.  Back to cited text no. 1
    
2.
Bohun CM, Potts JE, Casey BM, Sandor GG. A population-based study of cardiac malformations and outcomes associated with dextrocardia. Am J Cardiol 2007;100:305-9.  Back to cited text no. 2
    
3.
Van Praagh R, Weinberg PM, Smith SD, Foran RB, Van Praagh S. Malpositions of the heart. In: Adams FA, Emmanouilides GC, Riemenschneider TA, editors. Heart Disease in Infants, Children, and Adolescents. 4th ed.. Baltimore: Williams & Wilkins; 1989. p. 554-5.  Back to cited text no. 3
    
4.
Hakim F, Madani A, Samara Y, Ata IA, Hiari A, Goussous Y, et al. Transcatheter closure of secundum atrial septal defect in a patient with dextrocardia using the Amplatzer septal occluder. Cathet Cardiovas Diagn 1998;43:291-4.  Back to cited text no. 4
    
5.
Moreyra AE, Saviano GJ, Kostis JB. Percutaneous transluminal coronary angioplasty in situs inversus. Cathet Cardiovasc Diagn 1987;13:114-6.  Back to cited text no. 5
    
6.
Patti G, D'Ambrosio A, Di Sciascio G. Coronary stenting in patients with situs viscerum inversus. G Ital Cardiol 1999;29:304-7.  Back to cited text no. 6
    
7.
Ergene O, Nazli C, Kocabas U, Duygu H, Akyildiz ZI, Hijazi ZM. Percutaneous closure of perimembranous ventricular septal defect with an Amplatzer Duct Occluder in a dextrocardia patient. Int J Cardiol 2011;150:e77-80.  Back to cited text no. 7
    
8.
Goel PK. Double-inversion technique for coronary angiography viewing in dextrocardia. Catheter Cardiovasc Interv 2005;66:281-5.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
 
 
    Tables

  [Table 1]



 

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