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.

[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.

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.

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.

[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.

Pediatric coronary artery revascularization: a European multicenter study.

BACKGROUND: We sought to evaluate the hospital and midterm results of different surgical revascularization techniques in pediatric patients within the European Congenital Heart Surgeons Association. METHODS: From 1973 to 2011, 80 patients from 13 European Congenital Heart Surgeons Association centers underwent 65 pediatric coronary artery bypass grafting (PCABG) and 27 other coronary artery procedures (OCAP; 12 patients had combined PCABG and other coronary artery procedures). Excluded were patients with Kawasaki disease. Median age at the time of coronary procedure was 2.3 years (range, 2 days to 16.9 years); 33 patients (41.2%) were younger then 12 months. An emergency procedure was necessary in 34 patients (42.5%). RESULTS: Twelve patients (15%) died in the hospital; age at surgery (p=0.02) and the need for an emergent procedure (p=0.0004) were related to hospital mortality. Median follow-up time was 7.6 years (range, 0.9 to 23 years). There were 3 late cardiac deaths, all after a median time of 4 years (range, 9 months to 8.8 years) after PCABG. Fourteen patients (20.5%) presented with symptoms, including congestive heart failure (n=10) and angina (n=4), that were significantly associated with a low ejection fraction (p<0.001) and the presence of moderate or severe mitral valve regurgitation (p=0.0003). Six patients underwent a reintervention for impaired myocardial perfusion; all of them had a stenotic or atretic PCABG (p=0.001), and the majority were symptomatic (5 of 6 patients; 83.3%; p=0.001). CONCLUSIONS: Both PCABG and other coronary artery procedures are suitable surgical options in pediatric patients with impaired myocardial perfusion, which increases operative and midterm survival. Such population of patients needs to be followed for life to prevent and treat any possible cause of further myocardial ischemia.

Coronary artery fistula associated with mitral valve endocarditis.

Coronary artery fistulas are rare, and the further development of mitral valvular insufficiency and endocarditis is even more uncommon. We report a case of endocarditis secondary to a congenital coronary artery fistula arising from the right coronary artery and draining into the left ventricle. Vegetations were found on the mitral valve leaflet. The fistula was successfully treated with surgery, and the endocarditis, with antibiotic therapy. Surgical repair is the optimal treatment for coronary artery fistula, even in asymptomatic patients.

Cyanotic congenital heart disease the coronary arterial circulation.

BACKGROUND: The coronary circulation in cyanotic congenital heart disease (CCHD) includes the extramural coronary arteries, basal coronary blood flow, flow reserve, the coronary microcirculation, and coronary atherogenesis. METHODS: Coronary arteriograms were analyzed in 59 adults with CCHD. Dilated extramural coronaries were examined histologically in six patients. Basal coronary blood flow was determined with N-13 positron emission tomography in 14 patients and in 10 controls. Hyperemic flow was induced by intravenous dipyridamole pharmacologic stress. Immunostaining against SM alpha-actin permitted microcirculatory morphometric analysis. Non-fasting total cholesterols were retrieved in 279 patients divided into four groups: Group A---143 cyanotic unoperated, Group B---47 rendered acyanotic by reparative surgery, Group C---41 acyanotic unoperated, Group D---48 acyanotic before and after operation. RESULTS: Extramural coronary arteries were mildly or moderately dilated to ectatic in 49/59 angiograms. Histologic examination disclosed loss of medial smooth muscle, increased medial collagen, and duplication of internal elastic lamina. Basal coronary flow was appreciably increased. Hyperemic flow was comparable to controls. Remodeling of the microcirculation was based upon coronary arteriolar length, volume and surface densities. Coronary atherosclerosis was absent in both the arteriograms and the necropsy specimens. CONCLUSIONS: Extramural coronary arteries in CCHD dilate in response to endothelial vasodilator substances supplemented by mural attenuation caused by medial abnormalities. Basal coronary flow was appreciably increased, but hyperemic flow was normal. Remodeling of the microcirculation was responsible for preservation of flow reserve. The coronaries were atheroma-free because of the salutory effects of hypocholesterolemia, hypoxemia, upregulated nitric oxide, low platelet counts, and hyperbilirubinrmia.

Transesophageal echocardiography, more than a diagnostic tool: use during surgical ligation of coronary artery fistulae - a case report.

Coronary artery fistulae (CAF) are an infrequent coronary abnormality. Herein, we describe the use of intraoperative transesophageal echocardiography (TEE) in the treatment of CAF. A 61 year-old woman presented with chest pain and symptoms consistent with unstable angina. Subsequent coronary angiography revealed the presence of 2 CAF, one extending from the left anterior descending artery to the pulmonary artery (PA) and the other extending from the proximal right coronary artery to the PA. Surgical ligation of the CAF without coronary bypass was arranged. Intraoperative TEE was successfully employed to localize the CAF, monitor fistula blood flow and heart wall motion, and confirm successful ligation. The patient recovered without complications. This case highlights the utility of intraoperative TEE during ligation of CAF.

Intermediate to long-term outcome following congenital coronary artery fistulae closure with focus on thrombus formation.

We sought to evaluate intermediate to long-term follow-up after coronary artery fistula (CAF) closure with emphasis on thrombotic complications. Transcatheter closure (TCC) or surgical closure (SC) is the current standard of treatment for significant CAF. Incidence and risk factors of coronary thrombosis after CAF closure have not been well described. Patients with CAF were identified from a departmental database and their medical records were retrospectively reviewed. CAFs were classified as proximal or distal based on origin and size as small, medium, or large. Of 16 patients, 12 underwent TCC and 4 SC. Median follow-up was 2.3 years (0.1 to 41.6). Myocardial infarction (MI) related to coronary thrombosis occurred in 3 patients; immediately, 0.9 year, and 42 years after closure. Ages at MI were 9.2, 57, and 49 years. All 3 had distal, large CAFs and underwent SC. Anticoagulation was used in 2 of 3 patients. In the remaining 13 patients, TCC was performed in 12 and SC in 1; mean age was 13.8 years (0.1 to 38.9). CAFs were proximal in 10 and distal in 3 and large in 10 and medium in 3. On follow-up, these patients were asymptomatic without MI. Anticoagulation was used in 9 of 13 after closure. In conclusion, patients with CAF are at risk for coronary artery thrombosis and MI after closure. Patients with distal type, large CAF, and older age at presentation may be at higher risk for coronary thrombosis. Close follow-up with anatomic and functional coronary evaluation is warranted in all patients after CAF closure.

Successful closure for a case of congenital coronary artery fistula using a patent ductus arteriosus occluder.

We describe a case of transcatheter occlusion of a large and tortuose coronary artery fistula in an 8-year-old girl. During 2.5 years' of follow-up, she had no complaints and developed normally. This method of reserving the guide wire in cardiac catheterisation is extremely useful. Through the application of a patent ductus arteriosus occluder, the transcatheter closure of coronary artery fistulas proves to be a safe, feasible, and cost-effective option to surgery.

Off-pump coronary artery bypass in a patient with congenital absence of the left pericardium and an extremely left-rotated heart.

Congenital anomalies of the pericardium are extremely rare and often difficult to diagnose. We describe a 66-year-old woman presenting with multivessel coronary artery disease and right heart insufficiency complicated by an extremely left-rotated heart due to a congenital absence of left pericardium. A multidetector row computerized tomographic study was performed to evaluate the torsion of the heart as well as the physical relationship between the left internal thoracic artery and the left coronary system. Off-pump coronary surgery was scheduled. Both the operative and postoperative courses were uneventful and the patient was discharged after 11 days.