We report clinical, CT, invasive coronary angiography and intra-operative findings of a sympto- matic elderly man with anomalous origin of left coronary artery from pulmonary artery (ALCAPA). ALCAPA is a rare syndrome with anomalous origin of left main coronary artery (LMCA) from main pulmonary artery (MPA). Survival into adulthood is rare and our case is probably the oldest sur- vivor who has undergone two coronary system repairs for this anomaly. The unique features of our case include: 1) Absence of “q” wave myocardial infarction on ECG; 2) Absence of significant mitral regurgitation; 3) Demonstration of stress induced perfusion defects in nuclear imaging which as per our knowledge has been done for the first time in the literature; 4) A unique tech- nique of closing anomalous LMCA origin by a pericardial patch, sutured on the internal aspect of MPA is the hallmark of this case.
11 Read more
Anomalous origin of the right coronary artery is a rare congenital anomaly, but is associated with sudden death. Originating from the opposite sinus of Valsalva, an interarterial course and an intramural course are especially considered as the risk factor for fatal cardiac events. Surgical indication remains controversial be- cause many patients are asymptomatic. A 52-year-old man with anomalous ori- gin of the right coronary artery with an interarterial course concomitant with the left main trunk disease was resuscitated from cardiopulmonary arrest. It was likely to be attributed to the left main trunk disease, but anatomical structure of the right coronary artery suggests its possible involvement. Prophylactic bypass grafting for the right coronary artery was performed using saphenous vein graft without ligating native vessel to prevent future cardiac events, as well as revascu- larization of the left main trunk disease. All grafts were patent in one-year fol- low-up coronary angiography. Any cardiac event has not occurred.
Anomalous origin of coronary arteries may be encountered coincidentally in the presence of unrelated pathology or when these are affected directly. This may directly be responsible for affecting the procedure or outcome. Various types of anomalies of origin, as well as course of coronary arteries, have been classified in the past. Here we report 3 cases of anomalous origin of coronary arteries in different scenarios. First case had anomalous coronary with bicus- pid aortic valve with dilated ascending aorta for which Bentall’s procedure was done, while the second and third cases were anomalous coronaries with coronary artery disease for which coronary artery bypass grafting was done.
7,115 patients, who underwent 64-slice CT (So- matom Sensation 64 Cardiac, Siemens, Erlangen, Germany) or dual-source CT (Somatom Definition, Siemens, Erlangen, Germany) in the years 2005–2011 due to suspected coronary artery disease or in order to evaluate congenital heart disease were also screened for the presence of anomalous origin of the coronary artery. The exclusion criteria were: prior revascula- risation therapy including percutaneous coronary interventions and coronary artery bypass grafting (concerned 1,063 patients); contraindications to per- form cardiac CT including respiratory failure, severe heart failure, irregular heart rate, pregnancy, known allergy to iodinated contrast agent, impaired renal function (serum creatinine ≥ 1.5 mg/dL) and thyroid disorders .
We present the case of a 15-year-old boy with clinical features of an acute myocardial infarction. Angiography revealed a complete obstruction of the left main coronary artery. A coronary-aorto-bypass graft was undertaken immediately. Cardiac computed tomography demonstrated an anomalous origin of the left main coronary artery from the right coronary sinus of the aorta. A thrombophilic state with a heterozygote genotype for prothrombin G20210 mutation, a C677T methylenetetrahydrofolate reductase gene mutation, and a protein C type 1 deficiency was detected. No other embolic source could be identified. The patient recovered with persistent left ventricular dysfunction. He is now taking the anticoagulant warfarin. Combined prothrombotic defects in combination with additional risk factors such as coronary anomalies can lead to myocardial infarction even in children and adolescents.
Copyright © 2018 Junji Matsuda et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Congenital coronary artery anomalies, including anomalous origin of a coronary artery, can manifest as life-threatening conditions, such as myocardial infarction or arrhythmia, and may even lead to sudden death associated with speciﬁc con- genital anatomical features. Such arteries can also develop atherosclerotic lesions. This report describes the case of a 75-year-old man who was admitted to our hospital due to exertional dyspnea. The right coronary artery was found to originate from the left coronary sinus and exhibit tight stenosis due to atherosclerosis, causing eﬀort angina pectoris. This case highlights the fact that coronary artery anomalies can cause angina pectoris via both atherosclerotic and nonatherosclerotic eﬀects, and successful revascularization was achieved noninvasively via percutaneous coronary angioplasty.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual clinical course is severe left sided heart failure and mitral valve insufficiency presenting during the first months of life. However, in some cases collateral blood supply from the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA. We report a case, where a 39-year old woman presented with ventricular fibrillation during phycial exertion. Coronary angiography and CT-angiography revealed an anomalous origin of the left coronary artery, and an aortic reimplantation of the left coronary artery was performed followed by ICD implantation. A review of the literature on ALCAPA is presented along with CT images before and after surgery.
A 61-year-old woman was emergently admitted for chest pain and diagnosed with acute myocardial infarction. Cardiac catheterization revealed that patient had triple vessel disease concomitant with ARCA (Figure 1B), and chest CT confirmed ARCA. An emergent off-pump cor- onary artery bypass was performed. The internal thor- acic arteries (ITAs) and the right gastroepiploic artery (RGEA) were prepared. Then, the resected right ITA was anastomosed to in situ the left ITA in a “Y” config- uration. The distal anastomoses were constructed in the following sequence: the distal left ITA to the left anterior descending artery (LAD) and the right ITA to the two obtuse marginal branches sequentially. Subsequently, the distal RCA was revascularized with the in situ RGEA (Figure 2B). The postoperative course was uneventful, and she remains asymptomatic for 14 months.
After 2.0-mm semi-compliant balloon inflation from the mid RCA through to the proximal AV branch (Fig. 2e), we attempted to advance an Eagle Eye® Plat- inum intravascular ultrasonography (IVUS) catheter (Volcano Corp., CA, USA) to the lesion. However, resist- ance to its insertion was so marked that we could not entirely advance the IVUS catheter over the first curve of the JL4 inside the GuideLiner (Fig. 2f ). We tried to change the position of the GuideLiner, but ultimately failed to deliver the IVUS catheter into the RCA. From the angiographical findings, we predicted the vessel size and initially deployed Xience Xpedition® (Abbott Labora- tories, IL, USA) 2.5/28mm by crossing over the PD artery. We additionally deployed Xience Xpedition® 3.0/ 38 mm slightly overlapping the proximal edge of the first drug-eluting stent. Final angiography demonstrated fa- vorable dilatation from the mid RCA through to the
The current recommendation of management is to operate at any age when the diagnosis is made, due to the risk for ventricular arrhythmias and sudden death. Current surgical procedures are directed at establishing revascularization by creating a two- coronary artery system via either (i) Intra pulmonary Tunnel Operation i.e Takeuchi procedure (creation of an aorto pulmonary window and an intrapulmonary tunnel extending from the anomalous ostium to the window) (ii) Left coronary artery implantation, (iii) Subclavian artery to left coronary artery anastomosis, or (iv) Tashirorepair. By establishing a patent two- coronary artery system, most patients experience normalization of LV systolic function and improving long-term survival. 19,20 Based on clinical suspicion
The anomalous origin of the left coronary artery from the pulmonary artery - known as Bland-White-Garland syndrome - is a rare congenital malformation that affects 1 in 300,000 live births. Most patients die in infancy without any surgical treatment. Some patients who survive past childhood often have varying symptoms such as myocardial ischemia, impaired left ventricular function, mitral regurgitation, and progressive heart failure, depending on the development collateral circulation. In the present report, we describe a procedure wherein the left coronary artery ostium was translocated through the transverse sinus of the pericardium in a 43-year-old mother with Bland-White-Garland syndrome and concomitant mitral regurgitation and report on the associated midterm results.
Coronary artery anomalies (CAA) are a diverse group of congenital disorders with highly variable clinical rele- vance and pathophysiological mechanisms. No single institution has collected a large enough series to clarify the natural prognosis of each entity. This contributes to ongoing controversy in relation to incidence and clinical identification of these lesions, especially the ones that could lead to angina or sudden cardiac death. Screening and management strategies have caused and are still causing debate among protagonists and antagonists.
An 89 year old Caucasian female, with medical history significant for hypertension and no previous reported history of coronary artery disease (CAD), presented with dyspnea on exertion and worsening bilateral lower extremity edema. Social history was negative for any smoking or alcohol intake. Vital signs were stable. Cardiac examination showed regular rhythm and controlled rate without murmurs, gallops, or rubs. Chest X ray showed no evidence of pulmonary edema. EKG showed new T wave inversion in inferior and lateral chest leads. Troponin was mildly elevated. Echocardiogram showed normal Ejection Fraction (EF) with no wall motion abnormalities, dilated right ventricle with volume and pressure overload. Pulmonary embolism was excluded with CT chest.
10 Read more
Case presentation: A 70-year-old Caucasian man came to our hospital with slight limitation of physical activity (New York Heart Association class II). He was asymptomatic for angina, syncope, and palpitations. Cardiac magnetic resonance imaging was performed after echocardiography because a hypertrophic cardiomyopathy was suspected; a plausible coronary artery anomaly was demonstrated as collateral evidence. Subsequently, coronary computed tomographic angiography showed the anomalous origin of left coronary artery from the pulmonary artery; the coronary vessels appeared markedly dilated and tortuous. Dilated intercoronary vessels along the epicardial surface of the heart and dilated bronchial arteries, corresponding to collateral pathways, were observed. Left ventricular hypertrophy, delayed subendocardial enhancement, and mitral insufficiency were better evaluated on
mary artery or the radial artery . Reimplantation is technically difficult in adults because of stretching on the coronary repair and the friable nature of the dilated coro- nary artery. A simplified technique has recently described by Laks et al . According to this technique the left cor- onary artery and a cuff of pulmonary artery wall are excised as a button and transferred to the aorta. The defect in the pulmonary artery is replaced with a pericardial patch. The need to clamp the aorta in an ischemic myocar- dium probably contributes to the mortality with this pro- cedure. The intrapulmonary technique designed by Takeuchi et al is another solution but has the potential disadvantages of pulmonary artery stenosis, aortic regurgi- tation or tunnel stenosis . In our case an off pump LIMA to LAD anastomosis was carried out initially, fol- lowed by ligation of the anomalous origin of the left cor- onary artery and mitral valve replacement. It is very difficult to say whether the patient's clinical improvement was due to the mitral valve replacement or correction of the coronary artery abnormality or both.
Origin of the left coronary artery from right coronary sinus which runs anterior or posterior to pulmonary artery before it divides into circumflex and left anterior descending arteries. Such abnormal origin could cause compression of the vessel between pulmonary artery and aorta leading to sudden death. The branches of left coronary artery when directly arising from right coronary artery may be severed in operations to widen the right ventricular outflow tract as in Fallot’s tetralogy  .
Anomalous origin of a coronary artery from the pulmonary artery is a rare but potentially lethal condition. Anomalous origin of the left coronary artery (LCA) from the pulmonary artery typically presents during infancy, but anomalous ori- gin of the right coronary artery (RCA) from the pulmonary artery (ARCAPA) may go undetected for years. We report two cases of ARCAPA where the diagnosis was supplemented with cardiovascular computed tomography (CCT) and car- diovascular magnetic resonance (CMR). Surgical repair was achieved with ligation and coronary artery bypass in one case and direct reimplantation of the anomalous artery into the aorta in the other.
Myocardial bridging was the most frequent nor- mal variant observed in our study. The reported preva- lence of myocardial bridging varies from 40% to 80% in autopsy studies and from 0.5% to 16% in con- ventional angiography . The distinct difference between those 2 methods is due to the relatively low diagnostic sensitivity of conventional angiography in the visualisation of tunnelled segments of coronary arteries based only on systolic compression . Car- diac computed tomography is an alternative method for the identification of myocardial bridging, which is of clinical importance due to its potential clinical consequences: ischaemia, acute coronary syndro- Figure 4. A variant of the anomalous origin of the left main
Background: Direct re-implantation of an anomalous left coronary artery into the aorta is the preferred surgical option for creating a dual coronary arterial system in patients in whom the anomalous artery originated from the pulmonary trunk. This technique, however, is applicable only when the anomalous ar- tery arises from the left posterior pulmonary sinus. We report the successfully late follow-up of series of patients employing a new technique using combined autogenous aortic and pulmonary endotheliazed tube. Patients and Methods: We have treated 3 patients, aged 2 months, 6 months and 8 months respectively, who presented with anomalous origin of the left cor- onary artery from the left posterior pulmonary sinus and moderated mitral valve insufficiency by left ven- tricle dysfunction. We used a trapdoor like coronary artery elongation with autogenous aortic and pulmo- nary endotheliazed tube to connect the left coronary ostium to ascending aorta, avoiding the mitral valve intervention. Results: There was no early or late death. All patients are in functional class I, with good biventricular function and competent mitral valve at a median follow-up of 94 months, ranged from 108 to 132 months. Postoperative Computerized tomogram- phy of aorta in our 1 st patient showed good arterial flow, without any distortion. A local and distal steno- sis of the left pulmonary artery was observed and submitted to stent treatment. Conclusions: The poten- tial benefits of the trapdoor like and its modification technique are excellent operative exposure. The use of autogenous endothelized tube is a viable tissue capa- ble of further growth, avoidance of injury to the aor- tic and pulmonary valvar apparatus or production of obstruction within the right ventricular outflow tract.
In Saudi Arabia, radiology units in many centers are using regularly the sensitive cardiac-imaging technologies such as coronary CT angiography to screen and detect these coronary abnormalities, and several reports appeared recently from many parts of Saudi Arabia. 1,9 – 11 Consanguineous marriages are common in many Middle Eastern countries including Saudi Arabia, and with around 57.7% prevalence of consanguinity with ﬁ rst-cousin mar- riages being the most frequent, we expect higher preva- lence of congenital heart diseases in Saudi Arabia. 12,13 However, a recent retrospective analysis of 6 years of coronary CT angiography scans showed the coronary artery anomalies were similar to literature data. 9 However, this abnormality is not very common; few reported cases of a SCA with origin from a single ostium in the right sinus of Valsalva with an anomalous left coronary artery were reported by radiologists. We have searched in the PubMed database for these reported cases and summarize some of the reported cases of anomalous left coronary artery in Table 1.