Acute coronary syndrome in congenitally corrected transposition of the great arteries

Abstract

DESCRIPTIONThe congenitally corrected transposition of the great arteries (TGA) is a rare condition accounting for less than 1% of all congenital heart diseases, usually combined with other congenital heart anomalies such as a ventricular septal defect or pulmonary outflow tract obstruction. Very often they present with abnormalities of the left-sided systemic tricuspid valve. In rare cases (<5%), some patients have no associated cardiac abnormalities and they can go unnoticed until adulthood.1 A 53-year-old man presented to the emergency department complaining of chest pain suggestive of angina. Medical history was positive for hypercholesterolaemia, smoking and arterial hypertension. The patient also revealed a positive family history of coronary heart disease. The admission ECG showed repolarisation abnormalities of the anterolateral wall (inverted T waves at leads I, aVL, V1-V5) consistent with ischaemia and suggestive of a possible non-ST elevation myocardial infarction (figure 1). The blood pressure on admission was 150/85 mm Hg and the patient was haemodynamically stable. The primary differential diagnosis at the time of admission was coronary ischaemia. A bedside transthoracic echocardiogram was performed and the aorta and the pulmonary artery were shown to be transposed. The left and right ventricles were also transposed. Systolic function of both ventricles was normal and the ejection fraction of the right (functionally left) ventricle was estimated to be approximately 60%. There were no identifiable regional wall motion abnormalities of either the left or right ventricle. It needs to be emphasised that a number of limitations hamper the evaluation of the right ventricle by transthoracic echocardiography. However, imaging was performed by an experienced consultant, and the quantitative methods for the evaluation of the right ventricle including tricuspid annular plane systolic excursion and systolic tissue Doppler imaging velocity of the tricuspid annulus were also commensurate with normal function.Following the early stabilisation phase, cardiac troponin I, creatine kinase and creatine kinase MB mass tests were found marginally positive (troponin levels at 0.28 mg/dl with a laboratory cut-off value of 0.10 mg/dl and minor increases of creatine kinase and creatine kinase-MB). The patient was prescribed standard acute coronary syndrome medical treatment as this was the most likely explanation for his clinical condition, including acetylsalicylic acid, clopidogrel, metoprolol, atorvastatin and enalapril. Low molecular weight heparin was also administered. He was promptly referred to the catheterisation laboratory for a coronary angiography and aortography that showed anomalous origin of the coronary arteries with the left coronary artery originating from the right sinus of Valsalva and the right coronary artery originating from the left sinus of Valsalva, a finding typical of congenitally corrected TGA where each coronary artery follows the course of its respective ventricle.Both coronaries were free of ...

Conventional Repair of an Intraoperatively Diagnosed Congenitally Corrected Transposition of the Great Arteries in an Adult

“…Even without associated lesions, the survival of ccTGA is poor, with only 50% at 40 years [Murtuza 2011]. In some cases, patients are asymptomatic and the diagnosis can go unrecognized until adulthood [Baumgartner 2010;Lampropoulos 2013]. The incidence of congestive heart failure and SRV dysfunction increases with age and by the age of 45 years, clinical heart failure is found on 67% of patients with associated abnormalities [Hornung 2010].…”

Case report: new onset heart failure in congenitally corrected transposition of the great arteries, dextrocardia, and situs inversus in an octogenarian

“…As a result, not much data exist regarding the role common adult acquired secondary causes of systemic ventricular failure, such as hypertensive heart disease, diabetes mellitus, or coronary artery disease, may play in systemic RV failure in ccTGA. Only a handful of published cases described acute coronary syndrome (ACS) in ccTGA patients who were diagnosed with varying degrees of coronary artery disease [12][13][14]. The degree to which coronary artery disease may contribute to systemic RV dysfunction and long-term outcomes has yet to be established in the aging population with ccTGA.…”

A great imitator in adult cardiology practice: congenitally corrected transposition of the great arteries

“…These patients might develop other acquired diseases, such as coronary artery disease in this patient, which might be their initial presentation. Cardiac arrest or acute coronary syndrome also may be caused by coronary anomalies, of which the most common is a single coronary arising from the right‐facing sinus. Thus, interventional cardiologists should be aware of such varying coronary artery anatomy, and angiography in patients with ccTGA should be performed with vigilance because of the possibility of significant abnormalities that could complicate the procedure .…”

“…Cardiac arrest or acute coronary syndrome also may be caused by coronary anomalies, of which the most common is a single coronary arising from the right‐facing sinus. Thus, interventional cardiologists should be aware of such varying coronary artery anatomy, and angiography in patients with ccTGA should be performed with vigilance because of the possibility of significant abnormalities that could complicate the procedure . This challenge was particularly faced in this patient; the interventional cardiologist had difficulty engaging the coronaries and performing PCI, especially in the emergent situation.…”