Nature Decides Survival

Abstract

A 76-year-old man with congenitally corrected transposition of great arteries (CCTGA) presented with acute inferior wall myocardial infarction and underwent primary angioplasty. Coronary anatomic variations and challenges are discussed. (Level of Difficulty: Advanced.)

Graphical abstract

A 76-year-old man with congenitally corrected transposition of great arteries (CCTGA) presented with acute inferior wall myocardial infarction…

Presentation

A 76-year-old man with a known case of CCTGA presented with acute retrosternal chest pain for 3.5 h. He was active and asymptomatic before this event and was able to walk for 1 h daily without symptoms. On presentation, he was stable hemodynamically. Cardiac examination revealed a prominent aortic component of second heart sounds and grades 3/6 pansystolic murmur at the apex.

Learning Objectives

• •To understand the natural history of CCTGA, a rare congenital heart disease, usually with a poor prognosis because of several associated anomalies.
• •Morbidity and mortality in this disease is predominantly due to systemic atrioventricular valve regurgitation and systemic right ventricle failure, which usually occurs in subjects in their 40s and 50s. Natural survival beyond age 70 is rare.
• •Atherosclerotic coronary artery disease in CCTGA is uncommon, and acute myocardial infarction is rarely reported.
• •Coronary intervention in CCTGA is difficult and may be challenging due to anatomic variations.

Online Video 1.

Download video file ^{(1.8MB, mp4)}

Right coronary angiogram (RAO cranial view) pre-PTCA. Right coronary artery: subtotal occlusion in mid-RCA (open arrow). Note TIMI-I antegrade flow.

Online Video 2.

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Right coronary angiogram (PA caudal view) post-PTCA. TIMI-III flow in dominant RCA after angioplasty and stenting.

Medical history

He was nondiabetic and nonhypertensive and was a nonsmoker with no adverse family history.

Investigations

Electrocardiography (ECG) showed normal sinus rhythm, a PR interval of 202 ms, absent septal Q-wave in lead V₆, presence of QS in V₁, and evidence of acute inferior wall myocardial infarction (MI) (Figure 1A).

Figure 1.

12-Lead ECG on Presentation

(A) 12-lead ECG on presentation. Normal sinus rhythm, PR interval of 202 ms, and absent septal Q-wave in V₆ and Q_S in V₁ due to right-to-left septal activation in CCTGA. ST-segment elevation in leads II, III, and aVF was suggestive of acute inferior MI. ST-segment elevation in lead III>II with reciprocal ST-segment depression in leads I and aVL suggested involvement of inferoseptal area due to RCA occlusion. (B) Echocardiogram (apical 4-chamber view). Features of CCTGA included atrioventricular discordance with dilated morphological right ventricle. Note the more apical insertion of the left AV valve (tricuspid valve) and moderator band; markers are MRV. (C) Echocardiogram color Doppler (apical 4-chamber view). Moderate left atrioventricular valve regurgitation (TR). (D) Echocardiogram (apical 5-chamber view). PA arises from the MLV ventriculoarterial discordance. AV = atrioventricular; CCTGA = congenitally corrected transposition of great arteries; ECG = electrocardiogram; MI = myocardial infarction; MLV = morphological left ventricle; MRA = morphological right atrium; MRV = morphological right ventricle; PA = pulmonary artery; RCA = right coronary artery; TR = tricuspid regurgitation.

Echocardiography (Figures 1B to 1D), which had been performed in the past, had revealed situs solitus, levocardia, atrioventricular and ventriculoarterial discordance suggestive of CCTGA, and moderate left (tricuspid) atrioventricular valve regurgitation. There was no associated structural anomaly: it was uncomplicated CCTGA. Ejection fraction (EF) of the systemic ventricle (morphological right ventricle) was 45% and the left ventricular EF (LVEF) was 55%.

Management

He was given aspirin, 350 mg, ticagrelor, 180 mg, and atorvastatin, 80 mg, and underwent coronary angiography with the intention of proceeding for primary angioplasty for acute ST-segment elevation MI. In CCTGA, the aorta is left and anterior to the pulmonary artery, and coronary arteries arise from posterior facing sinuses, making selective engagement of coronary arteries difficult.

Employment of a right radial arterial approach and a 5-F Tiger catheter (Terumo Medical, Japan), selective angiography revealed moderate stenosis in the left anterior descending artery (LAD) (Figure 2A) and subtotal occlusion of the right coronary artery (RCA) after large conal-right ventricle (RV) branch with thrombolysis in myocardial infarction-I antegrade flow (Figure 2B). Small left circumflex artery was normal. Primary percutaneous transluminal coronary angioplasty of the RCA was performed uneventfully by using a 6-F Judkins right 4 catheter, floppy-tip coronary guidewire, and a 2.75- × 18-mm drug-eluting stent (Figures 2C to 2E). Angina subsided, and ST-segment elevation reverted toward the baseline immediately after angioplasty (Figure 3). The patient had an uneventful recovery and was discharged after 36 h with guideline-directed medical therapy for secondary prevention. LVEF on echocardiography was 60%, and there was no wall motion abnormality.

Figure 2.

Left Coronary Angiogram (RAO Cranial View)

(A) Left coronary artery: moderate stenosis in proximal LAD. Small left circumflex artery. (B) RCA (RAO cranial view). RCA: subtotal occlusion in mid-RCA (white arrow). (C) PTCA of RCA (PA caudal view). Guide wire crossed, forward flow after pre-dilation (blue arrow is the site of the lesion). (D) PTCA of RCA (PA caudal view). Deployment of drug eluting stent in RCA. (E) PTCA of RCA (PA caudal view). Post-stent RCA angiogram showing fully open, dominant RCA (Videos 1 and 2). LAD = left anterior descending artery; PA = pulmonary artery; PTCA = percutaneous transluminal coronary angioplasty; RAO = right anterior oblique; RCA = right coronary artery.

Figure 3.

12-Lead ECG Post-PTCA

ST-segment elevation in leads II, III, and aVF reduced significantly, suggesting good tissue myocardial perfusion.

Follow-up

At 6 months, the patient remained asymptomatic.

Discussion

CCTGA was first described by Karl Von Rokitansky in 1875 (1). It is a rare disease with a prevalence of 0.5% of all congenital heart defects at birth. Only 1% to 10% of these patients have uncomplicated CCTGA. Associated anomalies include ventricular septal defect (in approximately 80%), pulmonary stenosis (in approximately 50%), and complete heart block (in approximately 30%). Progressive left atrioventricular valve regurgitation is the commonest association occurring in almost 90% of cases (1). In a multi-institutional study of retrospective reviews of 182 patients with CCTGA (2), two-thirds of patients were found to have heart failure by the age of 45 if they had associated lesions, whereas it was 25% if they had uncomplicated CCTGA. Morbidity and mortality in this disease is predominantly due to systemic AV valve regurgitation and systemic RV failure, usually occurring when subjects are in their 40s and 50s. The longest reported survival was an asymptomatic 83-year-old Italian man who had uncomplicated CCTGA (3). Only 9 living patients with CCTGA older than 70 years have been reported until 2014 (3). As survival beyond the fifth decade is uncommon, significant atherosclerotic coronary artery disease is rarely found and reported. On review of published articles, there are very few reported cases of MI in CCTGA, and there is only 1 reported case of primary angioplasty performed for MI in a 41-year-old patient (4). The 76-year-old patient in the present case, to the best of the authors’ knowledge, is the oldest in the world who has undergone primary angioplasty for acute ST-segment elevation MI.

Aorta is left and anterior to the pulmonary artery, and coronary arteries arise from both of the posterior-facing sinuses (rather than the normal origins from anterior sinuses) (Figures 4A and 4B). This leads to difficult engagement for selective coronary angiography. Coronary artery-ventricular concordance is the norm, but approximately 45% may have a coronary origin variation where single coronary artery and LAD from RCA are common.

Figure 4.

Normal Coronary Anatomy

(A) Normal pattern: PA is left and anterior to the aorta. Both coronary arteries arise from sinuses facing the PA. (B) Coronary anatomy in CCTGA. Aorta is left and anterior to the PA. Coronary arteries arise from sinuses facing the PA. Commonly, in cases of CCTGA, RCA arises from the posterior-facing sinus instead of the right coronary sinus, as normal, and the left coronary artery arises from the right anterior sinus instead of the left coronary sinus, as normal. CCTGA = congenitally corrected transposition of great arteries; LAD = left anterior descending; LCX = left circumflex; LMCA = left main coronary artery; PA = pulmonary artery; RCA = right coronary artery; RV = right ventricle.

Inferior MI due to proximal RCA occlusion before RV branch or conal branch will cause additional right ventricular MI, MI of systemic and already dysfunctional ventricles, which can be catastrophic. The present case had already been diagnosed previously as CCTGA, and technical difficulties were anticipated. Engagement of the left coronary artery, which arises from the right-facing sinus, may not be difficult using the Tiger catheter from a radial approach, but engagement of the RCA, which arises from the posterior sinus, may be difficult and may need an Amplatzer right or Judkin right catheter (for normal-sized aortic root) or an Amplatzer left catheter (for dilated aortic root) catheters. If the patient had no prior diagnosis of CCTGA at the time of initial presentation with chest pain, it may be suspected by ECG. Because the right and left conduction bundles relate anatomically to the respective ventricles, septal activation occurs in the opposite direction, namely from right to left. This leads to a mirror image pattern in the precordial leads leading to a reversal of the normal QRS morphology, with Q waves evident in V₁ and a dominant R-wave but no Q-wave in V₆ (5).

In the normal heart, occlusion of the dominant RCA causes ischemia predominantly in the inferoseptal area with an ST-segment vector pointing toward lead III. In case of left circumflex artery (LCx) occlusion, the ischemic area is located posterolaterally (leftward), resulting in an ST-segment vector-directed toward lead II. In CCTGA, with levocardia, IVS position is more or less the same, and ischemia of inferoseptal areas due to occlusion of RCA will have almost the same direction of the ST-segment vector as that seen in normal heart, resulting in more ST-segment elevation in lead III than in lead II (Figure 1A). Because the morphological left ventricle is on the right side of the morphologically right ventricle, occlusion of the LCx will have an ST-segment vector pointing posterolaterally (rightward) and in fact will be more away from lead II.

Conclusions

CCTGA is a rare form of congenital heart disease, and to have uncomplicated CCTGA is even rarer. Very few patients have survived beyond 70 years old. Acute MI and primary angioplasty in CCTGA are seldom reported. The patient in the present case, 76 years of age with uncomplicated CCTGA, was a natural survivor, asymptomatic, and successfully underwent primary angioplasty for acute inferior MI. To the best of the authors’ knowledge, he is probably the oldest reported survivor in the world after primary angioplasty. At 6 months follow-up, he has remained asymptomatic.