Primary Angioplasty and Later Elective Multivessel Stenting in a Patient with Dextrocardia: A Case Report and Literature Review

ABSTRACT: Dextrocardia is a rare congenital anomaly with a prevalence of 1 in 10,000 births.1 The incidence of coronary artery disease in such patients is thought to be similar to that of the general public,2 however, patients are seldom seen during routine clinical practice and at the cardiac catheterization laboratory. Patients with this condition and acute myocardial ischemia may pose challenges at presentation, clinical and diagnostic findings and at cardiac catheterization. In this report, a male patient presenting with acute inferior myocardial infarction and three-vessel coronary artery disease was managed successfully with emergency primary angioplasty and later elective multi-vessel stenting. This is the first combined primary and later multi-vessel stenting to be reported in a single patient with dextrocardia and situs inversus. The clinical findings, details of the procedures and literature review will be discussed.

J INVASIVE CARDIOL 2010;22:E125–E128

Key words: Dextrocardia, acute MI, emergency angioplasty, inferior MI, multivessel stenting, three-vessel disease    Case Report. A 55-year-old diabetic Asian male previously diagnosed with dextrocardia, presented with acute right-sided chest pain. He was a lifelong non-smoker with normal serum lipids and no family history of premature coronary artery disease.    He described the right-sided chest pain as severe and crushing and radiating into the right arm and shoulder. Initially, he was seen at a local facility where a left-sided 12 lead ECG was obtained (Figure 1). This showed negative P wave in leads 1 and aVL and reversed R wave progression across the anterior leads. There was minimal ST depression and T wave inversion in the anterior leads.    Loading doses of aspirin and sub-lingual nitrate were given with no relief of symptoms. He was then transferred to our service with a working diagnosis of unstable angina. Upon arrival, he was hemodynamically and electrically stable with pain severity of 8/10. Immediately a 12 lead right-sided ECG with limb leads reversal was obtained (Figure 2). This showed ST-segment elevation of the inferior leads, reciprocal ischemic changes in the lateral leads, positive P waves in leads 1 and AVL and normal progression of R waves across the anterior leads. A portable chest X-ray (Figure 3) showed a right-sided cardiac shadow and aortic knuckle. Gastric air was seen below the right hemi-diaphragm. A diagnosis of acute inferior myocardial infarction was made and the patient was emergently transferred to the cardiac catheterization laboratory for primary angioplasty. 600 mg clopidogrel was loaded.    Angioplasty procedure and technique. The procedure was done through the right femoral approach with a 6 French system (Fr) and a biplane Siemens image intensifier (Siemens Healthcare, Mountain View, California). The right-sided, morphologically left coronary artery was engaged in the antero-posterior position with a Judkins left (JL4) catheter.    Initially, spontaneous catheter engagement failed and the ostium was then successfully engaged with clockwise rotation of the catheter at the level of the ostium. This revealed significant stenosis of the mid segment of the left anterior descending artery (LAD). The left circumflex artery (LCX) in the grove, recognized by localizing the coronary sinus at the late venous phase of contrast injection, was small and moderately diseased. The obtuse marginal (OM) was a descent caliber vessel with significant stenosis at its proximal part (Figures 4 and 5).    The left-sided anatomically right coronary artery was then negotiated in the right anterior oblique (RAO 40°) angle view using a Judkins Right guiding catheter (JR4). Gradual withdrawal with counter-clockwise rotation was successful to engage the ostium of the vessel. The vessel was totally occluded from its proximal part (Figure 6). We proceeded to angioplasty, heparin was loaded and a Balanced Middle Weight wire (BMW, Abbott Vascular, Santa Clara, California) was steered through the lesion flipping between the simultaneous biplane RAO and LAO views to monitor its progress through the anatomical path of the vessel. The RAO view, however, was the best to show the full profile of the vessel. The wire was then secured to the distal vessel. Multiple balloon dilatations uncovered a long segment of disease extending from the proximal vessel to just before the bifurcation (Figure 7). Two overlapping 2.75 x 18 (distal) and 3.0 x 18 mm (proximal) Endeavor drug-eluting stents (Medtronic, Santa Rosa, California) were deployed with the distal stent the first to be deployed. The area of overlap was taken up by a bigger size non-compliant balloon with excellent radiological result (Figure 8). The procedure was well tolerated and subsequent hospital stay was unremarkable. The patient was readmitted after couple of months for elective stenting of the other two coronaries.    Second elective procedure. The diagnostic part of this was similar to the first procedure. A re-look at the previously stented RCA demonstrated a widely patent vessel and stents (Figure 9). Then our attention was focused on the left system.    An Extra Back-up (EBU) guiding catheter (Medtronic) was used to engage the left system with clockwise rotation in the AP (antero-posterior) view. Due to vessel overlap, we have to stent flipping between the LAO 10 Caudal 40 view (which shows the proximal end of the lesion)(Figure 4) and the AP cranial 40 (which shows the distal end of the lesion) (Figure 5). Using these two views, the mid LAD lesion was crossed and dilated and then stented with 3.0 x 24 mm Endeavor stent with excellent radiographic outcome (Figure 11).    The LCX/OM was stented in the LAO 10 Caudal 40 with provisional stenting as the intended plan. Two BMW wires were passed into the LCX and OM (Figure 10). A small balloon was passed with difficulty to dilate the LCX and then the OM was pre-dilated and stented with a 2.75 x 18 Endeavor stent. No plaque shift was observed in the LCX and its wire was then pulled out. The stent was further post dilated with higher pressure noncompliant balloon with excellent result (Figure 11). The procedure was uncomplicated. The patient was discharged on aspirin 75 mg, atorvastatin 80 mg, an ACE Inhibitor and a beta-blocker. A long course of clopidogrel 75 mg was also prescribed. The patient remained symptom free at 6-month follow up.    Discussion. Dextrocardia is referred to as the deviation of the heart to the right hemi-thorax. There are several classifications of the condition with classification according to the viscero-atrial situs (the position of the viscera, atria and great vessels) the simplest and the most commonly adopted one.³ According to this, three types of the condition are recognized: dextrocardia with situs solitus (also called dextroversion), dextrocardia with situs inversus (mirror image dextrocardia) and dextrocardia with situs ambiguous or indeterminate. In all 3 types the atria are located dorsal to the ventricles attached to the great vessels. In both the solitus and inversus types the whole heart is shifted to the right hemi-thorax and the visceral situs is concordant to the atrial situs, while in dextrocardia ambiguous, the heart location is variable and there is no consistency of the viscero-atrial situs. In situs soiltus, the viscera (the stomach and liver) are located in their normal anatomical position, the anatomical left atrium is located to the left of the anatomical right atrium and the origins of the coronary arteries are close to their normal position. The aorta is left sided as in normal anatomy. The reverse is true in dextrocardia with situs inversus, where the anatomical location of the viscera, atria, the aorta and the coronaries are reversed. This configuration makes engagement technique during cardiac catheterization different than the usual technique used when heart anatomy is normal.    Clinical aspects of dextrocardia. In dextrocardia with situs inversus, the incidence of other cardiac malformations is around 3%.⁴ These patients have a relatively normal life span and low morbidity unless their condition is associated with the rare immotile cilia syndrome (Kartagener’s Syndrome) where repeated chest infections can be associated with significant morbidity. The incidence of Kartagener’s syndrome in dextrocardia with situs inversus is estimated at 15%.⁴ The incidence of congenital heart disease in patients with dextrocardia and situs solitus is quite common and is associated with significant mortality and morbidity and shortening of life span.    The prevalence of coronary artery disease in dextrocardia patients is thought to be similar to the general population,² however, unlike normal patients the ischemic chest pain is commonly right sided and may radiates to the right upper arm and shoulder.^6–14 This may pose difficulty in early detection of the nature and origin of chest pain.    ECG in dextrocardia. The left-sided 12 lead ECG typically shows progressive loss of the height of the R wave from right towards the left located leads with or without loss of voltage. The P-wave axis is a reliable indicator of viscero-atrial situs with a negative P-wave axis in the left sided leads (leads 1 and AVL) in patients with situs inversus and positive deflection in normal individuals and patients with situs solitus.    The diagnosis of acute MI and the extent of myocardial ischemia can easily be missed or underestimated (as seen in this case and other cases),^11–14 unless a reversed right-sided ECG with reversed limb leads is obtained. This reversed leads technique will also correct for the P-wave axis and normalize the R-wave progression across the chest leads.    Coronary angiography and dextrocardia. Coronary angiography in patients with dextrocardia can be a challenge due to the abnormal anatomical location of the heart, coronary arteries and the aorta. These challenges involve choice of catheters, engagement technique, radiological orientation of anatomy and appropriate radiological angles and views.    The first cardiac catheterization in such patients was described by Richardson in 1974 in a patient with ventricular aneurysm prior to surgical repair.⁵ First angioplasty procedure was reported by Moreyra in 1987.⁶ Since then few cases of elective angioplasty and stenting were reported.^7–10 Three cases of primary angioplasty were reported in patients with dextrocardia and situs inversus^11–13 and one report of primary angioplasty in a patient with situs solitus.¹⁴ Two-vessel stenting was described by Hung-Shun Lo in 2004.⁹    To the best of our knowledge, this is the first successful combined primary angioplasty and later multi-vessel stenting to be reported in a single patient with mirror image dextrocardia. In 1987 Moreyra reported difficulty in using the regular Judkins catheters and advocated multi-purpose catheters; however in most following reports,^7–10 and in this case, regular Judkins catheters were used without difficulty. The only reason we chose to use an EBU guiding catheter in the second procedure was that we anticipated the need for catheter support dealing with the left system disease, however the engagement technique was the same. Most authors recommended reversed catheter rotation to engage the coronary arteries, i.e., counter-clockwise rotation for the RCA and clockwise rotation — if needed — for the LMS. This was the technique adopted for engagement of both vessels in this case.    Goel et al¹⁵ suggested a double inversion technique with left/right and horizontal reversal of the radiological image intensifier to correct for the unfamiliar mirror image views. In our experience we found this unnecessary and simple right /left reversal is enough for appropriate anatomical orientation. We found that the conventional RAO view (reversed LAO) and the straight lateral view were the best views for stenting the RCA. We recommend the straight lateral view to easily identify the anatomical LAD. In this view the LAD will appear anterior to the cardiac silhouette. The angle for stenting the LAD will depend on vessel overlap and the segment involved. We found it quite useful to visualize the coronary sinus by filming to the late venous phase of contrast injection to identify structures related to the atrioventricular grove.    Conclusion. In patients with dextrocardia and situs inversus the clinical presentation and findings may be different than those encountered in patients with left-sided hearts. The chest pain is typically right-sided and the conventional left-sided ECG may not demonstrate the true extent and nature of the disease. This can lead to misdiagnosis and considerable delay in swift triage and delivering the urgent and necessary treatment for these acutely sick patients.    Acute and multivessel stenting in patients with dextrocardia is feasible and can easily be performed with conventional catheters with appropriate reversed rotation. Radiological views with simple right/left reversal, is all that is required for proper anatomical orientation. We hope this case will add important information to the literature in this area. From Saad Specialist Hospital, Khobar, Saudi Arabia. The authors report no financial relationships or conflicts of interest regarding the content herein. Manuscript submitted November 10, 2009, provisional acceptance given November 30, 2009, final version accepted December 28, 2009. Address for correspondence: Elbagir Elbasheer, MRCP, Former Consultant Interventional Cardiologist at Saad Specialist Hospital, P.O. Box 30353, Al-Khobar 31952, Kingdom of Saudi Arabia. (currently Consultant Cardiologist at Sudan Heart Centre, Khartoum, Sudan) E-mail: elbagire@gmail.com References

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