Anatomic Repair of Left Main Coronary Artery Atresia: Coronary Ostioplasty With Autologous Pulmonary Artery.

BACKGROUND: Left main coronary arterial (LMCA) atresia is a rare coronary arterial anomaly with extremely limited data on the optimal management. We aimed to report our single-surgeon experience of the ostioplasty in patients with LMCA atresia. METHODS: From July 2018 to December 2019, pediatric patients who presented with LMCA atresia and subsequently underwent surgical coronary ostioplasty were recruited into this retrospective study. Concomitant mitral repair was applied when the regurgitation was moderate or more severe. RESULTS: A total of 9 patients diagnosed with LMCA atresia were included. Mitral regurgitation was found in all of them, including 6 (66.7%) severe, 1 (11.1%) moderate, and 2 (22.2%) mild. In addition to ischemic lesions, which were found in 7 (77.8%) patients, structural mitral problems were also common (presented in 7 [77.8%] patients). All the patients underwent coronary ostioplasty with autologous pulmonary arterial patch augmenting the anterior wall of the neo-ostium. Mean aortic cross clamp time and cardiopulmonary bypass time was 88.1 ± 18.9 and 124.6 ± 23.6 minutes, respectively. During a median of 10.9 (range: 3.3 to 17.2) months' follow-up, there was only 1 death at 5 months after surgery. All survivors were recovered uneventfully with normal left-ventricular function; however, with 4 (50.0%) having significant recurrence of mitral regurgitation. CONCLUSIONS: With favourable surgical outcomes, coronary ostioplasty for LMCA atresia may be an option of revascularization. Structural mitral problems presented in majority patients, resulting in the requirement of concomitant mitral repair. However, the optimal technique of mitral repair remains unclear.

Intracoronary Imaging‒Guided Percutaneous Coronary Intervention for Woven Coronary Artery Disease With Chronic Total Occlusion.

Woven coronary artery disease is a relatively rare congenital anomaly with unexplained etiology. Herein we presented the first unusual woven case involving all coronary arteries with chronic total occlusion lesion of the left anterior descending artery; describe intracoronary imaging‒guided percutaneous coronary intervention for woven coronary artery disease with chronic total occlusion lesion of the left anterior descending artery; and discuss the complexities involved in intervening with such lesions.

The role of the fractional flow reserve in the coronary steal phenomenon evaluation caused by the coronary-pulmonary fistulas: case report and review of the literature.

BACKGROUND: Congenital coronary-pulmonary fistulas (CPFs) are commonly unilateral; however, bilateral and multilateral fistulas are relatively rare. The steal phenomenon aroused from bilateral or multilateral CPFs, and was uncertain and seldom reported. We possess a new tool to assess the hemodynamic significance of coronary artery fistulas. This study aimed to describe the clinical presentation, diagnostic modalities, and management of the coincidentally detected congenital bilateral CPFs. CASE PRESENTATION: A case of a 52 year-old female with 10 years history of typical palpitations and chest tightness was presented. The selective coronary arteriography showed a right dominant coronary circulation without significant stenosis; however, with anomalous vessels originating from the proximal right and left anterior descending coronary arteries, draining into the pulmonary artery through a plexus of small vessels. We introduced the fractional flow reserve (FFR) to evaluate the hemodynamic significance of CPFs. The patient was successfully treated with coil embolization. CONCLUSIONS: We presented the case of a female with typical palpitations and chest tightness due to the steal phenomenon that aroused from bilateral CPFs. The fistulas were safely and successfully closed by coil embolization. We showed a new tool for the sophisticated evaluation of the hemodynamic significance of CPFs using FFR measurement and temporary occlusion of the fistula with a standard balloon. FFR could be a promising means for the treatment of decision making of the CPFs.

Clinical characteristics in patients with rest angina and hypoplastic right coronary artery.

Hypoplastic coronary artery disease is a rare congenital abnormality reported to be associated with myocardial infarction and sudden cardiac death. Provoked positive spasm in the left circumflex coronary artery (LCX) with pharmacological spasm provocation tests was remarkably lower than other coronary arteries. We sometimes encountered patients with rest angina and hypoplastic right coronary artery (H-RCA). Among 5953 patients with diagnostic and follow-up coronary arteriography, we found 93 patients (1.6%) with H-RCA. During the same period, we could perform spasm provocation tests in 564 patients with rest angina including 13 patients with H-RCA and 249 patients with effort angina including 10 patients with H-RCA. Pharmacological spasm provocation tests were performed in 51 of 93 patients including 34 patients with ischemic heart disease (IHD) and 17 patients with non-IHD. Provoked spasm incidence in patients with IHD was higher than in those with non-IHD but not significant (52.9% vs. 29.4%, p = 0.1114). Provoked positive spasm in the LCX in patients with rest angina and H-RCA was significantly higher than that in those without H-RCA (69.2% vs. 23.4%, p < 0.001). Provoked spasm on both left anterior descending artery and LCX in patients with rest angina and H-RCA was also remarkably higher than in those without H-RCA (53.8% vs. 3.1%, p < 0,001). There were no clinical differences between patients with and without H-RCA rest angina. Two-vessel spasm (61.5% vs. 0%, p < 0.01) and LCX-provoked spasm (69.2% vs. o%, p < 0.01) were significantly higher in patients with H-RCA and rest angina than that in those with H-RCA and effort angina. In patients with rest angina and H-RCA, LCX-positive spasm was significantly higher and these patients may have a potential of high disease activity in the clinic as a coronary spastic angina.

Congenital coronary collateral.

Intercoronary communications are a very rare congenital coronary artery anomalies. We report a case of a man who underwent elective coronary angiography that showed a bidirectional direct intercoronary communication between right coronary and left circumflex arteries.

[Pediatric coronary artery surgery. A new area in cardiac surgery?] / Coronariasebészet csecsemo- és gyermekkorban. A szívsebészet új ága?

Introduction and aim: Pediatric coronary artery surgery for congenital heart disease has become increasingly important in newborns and infants. It is life-saving in unsuccessful coronary transfer surgery, injury of coronary artery during surgery, in pediatric coronary artery bypass surgery (PCABS) due to acquired Kawasaki disease. Method: We review the current surgical role of congenital and acquired coronary artery diseases in newborns and infants. Results: The 7 main challenges are: 1) anomalous origin of the left coronary artery from the pulmonary artery; 2) different variations of the origins of the coronary arteries in transposition of the great arteries (TGA); 3) acute and late coronary events in the arterial switch operation for TGA; 4) complications after coronary transfer procedures: Ross-, Nikaidoh operation; 5) inadvertent coronary artery injury during heart surgery; 6) Kawasaki disease; 7) coronary artery procedures in premature infants. Direct coronary reimplantation in most, surgical angioplastic procedures in selected patients is the first choice; however, PCABS with internal thoracic artery (ITA) grafts can be life-saving in emergency or severe myocardial hypoperfusion conditions. Since the patency of saphenous vein grafts is poorer than that of ITA grafts, their use should be avoided in growing children. The procedures can be performed safely in neonates and infants, using high-power magnifying glasses or a surgical microscope. Conclusion: Pediatric coronary artery surgery technique is now established as the standard safety surgical choice, as a new area in cardiac surgery. Congenital heart surgeons after proper training are able to use coronary transfer and bypass surgery safely even in premature babies. Orv Hetil. 2019; 160(49): 1935-1940.

Single right coronary artery with congenital absence of left coronary system.

Single coronary artery (SCA) is a very rare coronary anomaly. The accurate diagnosis of the entity requires multimodality imaging of the coronary anatomy. SCA is often incidentally diagnosed when patients are investigated for symptoms of suspected coronary artery disease with invasive or non-invasive coronary angiography. There are no established diagnostic electrocardiographic or echocardiographic criteria to identify the presence of SCA, which makes the diagnosis a far-reaching fruit. We present a young male patient presenting with a non-ST elevation myocardial infarction. He was found to have SCA on invasive coronary angiography, which was subsequently confirmed by CT coronary angiography.

Unusual Association of Three Types of Congenital Coronary Artery Diseases.

Congenital coronary artery disease (CAD) is commonly asymptomatic and often benign. Investigation of potential myocardial ischemia is also necessary. We report an original observation of abnormal origin and course of the right coronary artery (RCA), abnormal left main artery, and agenesis of the left anterior descending (LAD) artery. This is the first report of an association of 3 types of congenital coronary artery anomaly according to the modified Angelini's classification. This high-risk condition was successfully treated surgically.

Coronary Artery-Left Ventricular Fistula and Takotsubo Cardiomyopathy - An Association or an Incidental Finding? A Case Report.

BACKGROUND A coronary artery-left ventricular fistula is an anomalous communication between the coronary arteries and the cardiac chambers and is a rare congenital coronary anomaly that is often small and asymptomatic. Takotsubo cardiomyopathy, on the other hand, is a syndrome characterized by transient regional systolic dysfunction of the left ventricle, mimicking myocardial infarction, but in the absence of angiographic evidence of obstructive coronary artery disease or acute plaque rupture. We present the case of an elderly woman who presented with Takotsubo cardiomyopathy and who was incidentally discovered to have an associated coronary artery-left ventricular fistula. CASE REPORT We report the case of a 68-years-old woman with a family history of premature cardiac diseases who presented with ischemic chest pain and elevated troponin levels. Her EKG and troponins were suggestive of non-ST-elevation myocardial infarction (NSTEMI), for which she was initially treated medically and later underwent coronary angiography. Unexpectedly, the angiography revealed patent coronary arteries, and we discovered evidence of coronary artery to left ventricular fistula in the addition to angiographic evidence of Takotsubo cardiomyopathy. A working diagnosis of Takotsubo was made, for which she was treated medically with resulting improvement of her symptoms and later in the imaging findings. CONCLUSIONS This described case illustrates a rare association between coronary artery fistulas and Takotsubo cardiomyopathy. It is unclear if this association has played a role in the pathogenesis or perhaps is just an incidental finding. More similar cases are needed to expand the clinical presentation of both conditions and add to the literature.

Outcomes after Balloon Pulmonary Valvuloplasty for Critical Pulmonary Stenosis and Incidence of Coronary Artery Fistulas.

Outcome data for neonates with critical pulmonary stenosis (PS) is limited. We aimed to review the outcomes after balloon pulmonary valvuloplasty (BPV) for neonates with critical PS at our institution. All neonates with critical PS who underwent BPV from 1990 to 2017 were included. A total of 44 neonates underwent BPV for critical PS. Nonright ventricular dependent coronary artery fistulas was seen in 6/44 (13.6%) patients. Tricuspid valve z-scores were -1.9 (interquartile range [IQR] -3.04, -0.48) in those with coronary artery fistulas as compared with -0.27 (IQR -0.5, 0.8) in those without (p = 0.03). Fifteen of forty-four subjects (34.1%) patients underwent reintervention with 10 patients (22.7%) requiring an alternate source of pulmonary blood flow (3 patients subsequently underwent right ventricular overhaul, 2 underwent Glenn operations, and 1 underwent repeat BPV). Five patients underwent reintervention for right ventricular outflow tract obstruction. Pulmonary valve annulus z-score was significantly smaller in those who needed reintervention -2.4 (IQR -2.9 to -0.95) versus -0.59 (IQR -1.3, -0.15); p = 0.02. At a median follow-up of 8.2 (IQR 3.4 to 13.1) years, moderate or severe pulmonary regurgitation was seen in 22/42 (53.7 %) patients with biventricular circulation, 3 requiring pulmonary valve repair/replacement. In conclusion, coronary artery fistulas occur in a significant number of patients with critical pulmonary stenosis, occurring more frequently in patients with small tricuspid valves. Reintervention is required for 1/3 of patients. Patients with small pulmonary valve annuli are more likely to undergo reintervention for right ventricular outflow tract obstruction. Significant pulmonary regurgitation is common and may require eventual pulmonary valve replacement.

[Late discover of a coronaro-pulmonary fistula]. / Une fistule coronaro-pulmonaire de découverte tardive.

Anomalies of the coronary arteries mainly concern a pediatric population, bringing together a wide range of defects. In adults, the evolution is linked to the hemodynamic consequences of fistula. Several therapeutic options have been proposed such as surgery or embolization. We report the case of a 55 years old patient addressed because of dyspnea secondary to aortic insufficiency. The preoperative assessment shown the coexistence of coronary abnormality corresponding to a coronaro-pulmonary fistula. This type of coronary anomaly is rarely described in the adult population, because of its consequences secondary to the closure of the foramen ovale, resulting in angina symptoms in childhood. Without treatment, mortality from this type of malformation is important (90%).

[Surgical Repair of Coronary-pulmonary Artery Fistulae with Giant Coronary Aneurysm].

A 69-year-old woman was referred to our hospital due to dyspnea on exertion and a heart murmur. A chest X-ray showed a bulge at the left 3rd arch and chest computed tomography( CT) revealed a giant mass adjacent to the right ventricular outflow. Multidetector-row CT and coronary angiography showed a giant coronary aneurysm (55×45 mm) and fistulae arising from the left main coronary trunk and entering into the main pulmonary artery (PA). The pulmonary to systemic flow ratio was 1.84. She was diagnosed with coronary-pulmonary artery fistulae with giant coronary aneurysm. Aneurysmorrhaphy and closure of the fistula outlet from the PA were performed under cardiopulmonary bypass with cardiac arrest. After declamping the aorta, residual shunt flow was found at several sites of abnormal vessels in the right ventricular outflow using a Doppler flowmeter. The vessels were ligated with pledgeted sutures, but did not disappear completely. Postoperative coronary angiography and transthoracic echocardiogram showed a small residual shunt flow into the PA. The postoperative course was uneventful;she was discharged on postoperative day 18. Six months later, the residual shunt flow had disappeared. No recurrence has been detected for 7 years.

Coronary Artery Fistulas: Case Series and Literature Review.

Congenital coronary artery fistulas are rare anomalies. As coronary angiography and multidetector computed tomography have become more accessible, they have been increasingly used in the investigation of chest pain and heart failure. Coronary artery fistulas are often an incidental finding, which raises the question of how patients with this condition should be managed. Intervention with either transcatheter closure or surgical closure is often technically possible. Many patients are asymptomatic early after closure. However, follow-up studies have shown post-closure sequelae, such as residual leakage, thrombosis with or without myocardial infarction, and coronary stenosis. Therefore, there has been a shift from intervention towards watchful waiting in asymptomatic patients. In this article, we review the published literature on the natural history and treatment outcomes in individuals with coronary artery fistulas. We present case reports from our clinic and discuss the management of incidental findings of coronary artery fistulas.

Prevalence of congenital coronary artery anomalies as shown by multi-slice computed tomography coronary angiography: a single-centre study from Turkey.

Objective Coronary artery anomaly (CAA) is a remarkable etiological factor for sudden cardiac death in young adults. The incidence of CAA is unknown, with most reliable data available based on postmortem/angiography investigations. This study aimed to assess the prevalence of different forms of coronary anomalies, and to investigate the relationships between demographic data and occurrence of CAA. Methods A total of 2401 consecutive patients (1805 men; mean age, 56 ± 11.7 years), who were referred between January 2005 and December 2008 for noninvasive multi-slice computed tomography (MSCT) imaging, were retrospectively analysed. Results A total of 225 cases (191 men; mean age, 55.9 ± 12) of CAAs were identified (9.37%). Because 11 patients had multiple muscular bridges of the coronary arteries, 236 coronary artery anomalies were found in these 225 patients. Cases were classified into three groups: group 1, coronary anomalies of origin and distribution (n = 36, 1.5%); group 2, anomalies of intrinsic coronary arterial anatomy (n = 180, 7.49%); and group 3, anomalies of coronary termination (n = 9, 0.4%). Conclusion The prevalence of CAA was 9.37% in our single-centre study, which is consistent with previous research. A minimally invasive tool, such as MSCT angiography, should be used to identify CAA.

Peratrial Device Closure of a Congenital Coronary Artery Fistula through a Right Parasternal Approach: Innovative Use of Available Technology.

Current treatments for congenital coronary artery fistulas (CAFs) include surgical obliteration and transcatheter occlusion. However, surgical techniques involve significant trauma. Transcatheter occlusion is performed under fluoroscopy and angiography, in which radiation injury is inevitable. We present a patient, with a CAF from the left coronary artery to the right atrium, who underwent peratrial device closure of the CAF with a right parasternal approach under transesophageal echocardiography guidance. Complete occlusion was achieved by a symmetric ventricular septal occluder. We suggest that peratrial device closure of a congenital coronary artery fistula through a right parasternal approach may be a safe and effective option.

Congenital coronary artery anomalies: a bridge from embryology to anatomy and pathophysiology--a position statement of the development, anatomy, and pathology ESC Working Group.

Congenital coronary artery anomalies are of major significance in clinical cardiology and cardiac surgery due to their association with myocardial ischaemia and sudden death. Such anomalies are detectable by imaging modalities and, according to various definitions, their prevalence ranges from 0.21 to 5.79%. This consensus document from the Development, Anatomy and Pathology Working Group of the European Society of Cardiology aims to provide: (i) a definition of normality that refers to essential anatomical and embryological features of coronary vessels, based on the integrated analysis of studies of normal and abnormal coronary embryogenesis and pathophysiology; (ii) an animal model-based systematic survey of the molecular and cellular mechanisms that regulate coronary blood vessel development; (iii) an organization of the wide spectrum of coronary artery anomalies, according to a comprehensive anatomical and embryological classification scheme; (iv) current knowledge of the pathophysiological mechanisms underlying symptoms and signs of coronary artery anomalies, with diagnostic and therapeutic implications. This document identifies the mosaic-like embryonic development of the coronary vascular system, as coronary cell types differentiate from multiple cell sources through an intricate network of molecular signals and haemodynamic cues, as the necessary framework for understanding the complex spectrum of coronary artery anomalies observed in human patients.

Transcatheter closure of medium and large congenital coronary artery fistula using wire-maintaining technique.

BACKGROUND: For medium and large coronary artery fistula (CAF), the initially selected device sometimes has to be exchanged by reconstruction of track wire loop due to the complexity of CAF. OBJECTIVES: We sought to evaluate the feasibility and safety of transcatheter closure of medium and large CAF by using the wire-maintaining technique (WMT). METHODS: A total of 18 patients aged 15-56 years with congenital CAF underwent percutaneous transcatheter closure by WMT between April 2006 and October 2012. The immediate and long-term outcomes were evaluated. RESULTS: Of the 18 patients (11 females), 16 (88%) underwent successful transcatheter closure of fistula using WMT. The CAFs originated from the right coronary artery (67%), the left circumflex coronary artery (28%), and the left anterior descending coronary artery (5%). The drainage sites were the right ventricle (56%), right atrium (22%), left ventricle (11%), and coronary sinus (11%). The mean diameter of fistulas was 9.5±1.71mm and mean size of the devices was 13.6±3.03mm. An angiogram following device deployment showed complete occlusion in 11 patients, mild residual shunt in 2 patients, and trivial residual shunt in 3 patients. One patient had transient ST-T wave changes, and one patient had hemolysis after the procedure. Follow-up ranged from 1 month to 54 months (median 39 months). Echocardiogram showed trivial residual shunt in 3 patients at 6-month follow-up and in 1 patient at 12-month follow-up. Coronary artery thrombosis was observed in 1 patient by multislice computed tomography at 12-month follow-up. CONCLUSION: For those patients with medium and large complex fistula, transcatheter closure of CAF can be performed by using the wire-maintaining technique.

[Pediatric case of congenital coronary artery fistula; surgical result and late changes in coronary artery aneurysm].

Congenital coronary artery fistula is an uncommon heart anomaly involving the coronary arteries. We report here a case of a 4-year-old boy who had a coronary fistula from the right coronary artery to the right ventricle, with a coronary aneurysm. He was asymptomatic, but the calculated ratio of pulmonary blood flow to systemic blood flow was shown to be high [pulmonary flow (Qp)/systemic flow(Qs)=1.78]. The coronary angiography showed that the right coronary artery was dilated beginning at the ostium and had an aneurysm at the acute marginal portion. A large spherical aneurysm approximately 20 mm in diameter was found to have been connected with coronary fistula opening into the right ventricle. Surgical repair by closure of the fistula under direct vision, partial resection and suture closure of the aneurysm was performed. Plication of the proximal portion of the right coronary artery was not performed, and the diffusely dilated artery was left untouched. After this operation, he recovered well under anticoagulant treatment with warfarin and aspirin. Postoperative angiography was performed 17 months after the surgery to evaluate morphological changes in the coronary artery. The angiography confirmed the closure of the fistula and the regression of coronary artery dilatation.