Introduction
Anomalous left coronary artery from the pulmonary artery, previously known as Bland-White-Garland syndrome, is a rare congenital coronary anomaly first reported in 1933. The incidence rate is stated as 1 in 300,000 live births, Reference Schwerzmann, Salehian, Elliot, Merchant, Siu and Webb1 accounting for 0.25–0.5% of all CHD cases. Reference Yuan, Hu, Zeng, Zhou and Chen2 We report a rare case of two sudden cardiac arrests resuscitated in a 36-year-old male, who was diagnosed with anomalous left coronary artery from the pulmonary artery in childhood and underwent successful surgical correction years before implantation of implantable cardioverter-defibrillator in secondary prevention, followed by cardiac resynchronisation therapy with a defibrillator later in life.
Case report
A 36-year-old male who underwent a surgical correction of anomalous left coronary artery from the pulmonary artery in early childhood with a history of multiple electrotherapeutic devices implanted over the years (first as a 16-year-old) has suffered sudden cardiac arrest twice. Sudden cardiac arrests were reported in the 16th and in the 27th year of the patient’s life, with successful cardiorespiratory resuscitations performed in both cases. In early childhood, in the 6th year of life, along with the surgical anomalous left coronary artery from the pulmonary artery correction, the patient underwent a mitral valve annuloplasty, followed by its replacement with a mechanical valve 8 years later. Aged 16 years, the patient underwent CARTO 3D ablation twice due to recurrent atrial flutter. In the same year, he suffered from the first sudden cardiac arrest. After the incident the patient was provided with an implantable cardioverter-defibrillator device. No further episodes within the following 7 years were reported. Aged 24 years, the patient was referred back to the hospital with an abrupt electrical storm episode. He was then upgraded with a cardiac resynchronisation therapy with a defibrillator (Figure 1). Just three years later, he suffered from a ventricular fibrillation episode, which led to the second sudden cardiac arrest. The device along with damaged leads was replaced. Transthoracic echocardiography showed dilatation and severe biventricular heart failure with left ventricular ejection fraction of 29% and a properly functioning mitral valve prosthesis. At the age of 33, another two electrical storms were recorded. In left ventricle multi-field mapping, an area of slowed conduction in the lateral apical left ventricle area was detected and ablation of that area was performed. After the procedure, no ventricular arrhythmia was induced with ventricular pacing. Cardiac implantable device infective endocarditis was excluded at any time that an electrical storm occurred. Subsequently, due to device battery depletion, the cardiac resynchronisation therapy with a defibrillator device was once again replaced with a new one. Apart from the details mentioned, supraventricular arrhythmias, paroxysmal atrial fibrillation, and nonsustained ventricular tachycardia episodes were also reported. The patient has also been treated for superior vena cava syndrome due to venous thrombosis, presumably related to heart failure and presence of multiple pacemaker leads.
Fluoroscopic view of CRT-D leads and mechanical mitral valve prosthesis.
Latest transthoracic echocardiography, in a 33-year-old patient, revealed: dilated cardiomyopathy with all 4 chambers enlarged, notably dilated left atrium, left ventricular global systolic dysfunction with left ventricular ejection fraction of 29%, mechanical mitral valve prosthesis with increased diastolic gradient and pulmonary hypertension.
As a thirty-six-year-old, the patient was admitted once again to the hospital with an infective endocarditis affecting mitral valve prosthesis and endocardial leads. Further diagnosis revealed an ischaemic stroke of the central nervous system (Supplementary Video S.1, Supplementary Video S.2). At the time of submitting this article, the 36-year-old man is in stable condition, under regular follow-up at the outpatient clinic (Figure 2).
Patient’s chest X-ray after replacement of multiple leads and implantable devices. Mechanical prosthesis in the position of mitral valve.
Discussion
Anomalous left coronary artery from the pulmonary artery is a rare congenital anomaly with two possible clinical presentations: the infant type and the adult type. The disease predominantly presents itself during infancy with congestive heart failure and myocardial infarction. Reference Peña, Nguyen, Merchant and Dennie3 Unless treated with a surgical intervention, approximately 90% of patients die in the first year of life. Reference Wesselhoeft, Fawcett and Johnson4 In some anomalous left coronary artery from the pulmonary artery patients, a well-developed collateral circulation from right coronary artery to left coronary artery permits the patient to reach adulthood, occasionally without a diagnosis. Reference Danel, Suchodolski, Szulik and Głowacki7 If the collateral blood supply from right coronary artery is inadequate, patients may present with dyspnoea, chest pain, arrhythmias, heart failure, or sudden cardiac death. Reference Holst, Helvind and Andersen5
The patient presented in this case report underwent surgical intervention in early childhood. Despite successful surgery, he has survived two sudden cardiac arrests in his early adolescence and adulthood. Apart from sudden cardiac arrests mentioned, multiple supraventricular, and ventricular arrhythmias treated with either implantable devices or electrophysiological procedures have been reported.
In adult anomalous left coronary artery from the pulmonary artery patients potential origin of ventricular arrhythmias could be myocardial scar. Its visualisation via late gadolinium enhancement could provide relevant information for evaluation of ICDs’ implantation indications. Reference Prandi, Zaidi and LaRocca6,Reference Danel, Suchodolski, Szulik and Głowacki7
Electrotherapy should be considered in adults with anomalous left coronary artery from the pulmonary artery when indicated by current guidelines, as other cardiovascular episodes, secondary to anomalous left coronary artery from the pulmonary artery, may eventually lead to sudden cardiac arrest or death. When a myocardial scar is detected, consideration of an ICD implantation is of great importance as the scarring itself is a potential source of ventricular arrhythmias and cardiac arrest, even in patients with preserved ejection fraction and full revascularisation conducted. Reference Prandi, Zaidi and LaRocca6
This study also has some limitations. Unfortunately, due to reasons beyond the authors’ capabilities, a cardiac magnetic resonance examination could not be and was not performed in the patient. Regarding the results of the coronary angiography, due to the remote date of the detection of anomalous left coronary artery from the pulmonary artery, it was not possible to include a detailed description of it in this paper.
To the authors’ knowledge this is the first case of this coronary anomaly resulting in two aborted sudden cardiac deaths during adulthood followed by the patient being discharged in a good physical condition.
Conclusion
Early diagnosis and surgical treatment of anomalous left coronary artery from the pulmonary artery followed with regular patient evaluation provide a good long-term prognosis. Conditions of younger patients should be regularly monitored due to increased risk of other cardiovascular complications secondary to the primary anomaly. Electrotherapy should be considered in anomalous left coronary artery from the pulmonary artery patients with an increased risk of sudden cardiac arrest. Establishment of specific criteria for including patients in electrotherapy prevention could be beneficial for the long-term prognosis in this patient group. Visualising myocardial scarring via late gadolinium enhancement could possibly become one of the criteria mentioned above.
Supplementary material
The supplementary material for this article can be found at https://doi.org/10.1017/S104795112511086X.
Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Competing interests
The authors declare they have no conflict of interest.
Ethical standard
This research does not involve any human and/or animal experimentation.
Open access
