Characteristics and long-term outcome for congenital left main coronary artery atresia.

The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.

ACAOS (Anomalous Coronary Artery From the Opposite Sinus) as Part of a Chaos: 75 Years of Harmonious Occult Coexistence.

It is uncommon for the left anterior descending coronary artery to arise from the contralateral Valsalva sinus, and more of a rarity to see coexistent left main coronary artery anomaly. Our patient reported normal daily physical activity and denied further evaluation (stress test, scintigraphy) or cardiothoracic consultation. She continues to be symptom free at 1-month follow-up.

Reconstruction in left main coronary artery atresia with bypass graft obstruction.

Paediatric coronary artery bypass surgery with internal thoracic artery grafting is the optimal choice for left main coronary artery atresia; we report successful reconstruction in a patient with bypass graft obstruction. The pulmonary trunk was transected to expose the left main coronary stem, which was opened beyond the obstruction and the incision extended through the left main coronary artery to the circumflex bifurcation and left anterior descending artery. A funnel-shaped coronary ostium was created with a glutaraldehyde-treated autologous pericardium onlay patch. Surgical reconstruction is a good alternative for left main coronary artery atresia with graft obstruction and preserves the remaining internal thoracic artery for future use.

Profile and early outcomes of surgical reconstruction of coronary artery atresia in children.

OBJECTIVES: Coronary artery atresia is a rare coronary artery anomaly in children and has a high rate of misdiagnosis. We aimed to summarize the profile and early outcomes after the surgical reconstruction of coronary artery atresia in children. METHODS: A retrospective analysis was performed in 12 consecutive patients with coronary artery atresia who were admitted to the Department of Paediatric Cardiac Surgery of Fuwai Hospital between October 2016 and September 2020. Ten patients underwent surgical reconstruction of the coronary artery with the pulmonary artery anterior wall, and 8 patients underwent concomitant mitral valvuloplasty. RESULTS: There were 6 females and 6 males, with an age of 1.75 years [interquartile range (IQR), 1.0-3.5] and weight of 10.0 kg (IQR, 8.9-14.75). There were 10 cases of left coronary artery atresia and 2 cases of right coronary artery atresia. All the patients were initially misdiagnosed in the outpatient clinic, but further nonselective coronary angiography confirmed the diagnosis of coronary artery atresia. In all 10 patients with mitral regurgitation, echocardiogram of the mitral valve chordae and papillary muscle revealed ischaemic changes. The clamp time was 89.0 min (IQR, 75.0-101.0), the pump time was 126.0 min (IQR, 119.0-132.0) and the intensive care unit stay time was 1.5 days (IQR, 1.0-3.0). No perioperative deaths were noted. After 9.5 months (IQR, 5.5-13.5) of follow-up, 2 patients with fractional shortening that significantly decreased to 14.1% and 14.8% died and 1 patient had moderate pericardial effusion that improved after treatment with oral diuretics. Coronary artery ultrasound and enhanced computed tomography showed a patent main coronary artery in all the patients. CONCLUSIONS: Coronary artery atresia in children is often associated with mitral regurgitation, and mitral valve chordae and papillary muscle exhibit ischaemic changes. Coronary artery reconstruction is safe and effective in children with coronary artery atresia.

Coronary steal phenomenon following right ventricle decompression and revascularization of atretic left coronary ostium: case report.

BACKGROUND: Coronary steal phenomenon and myocardial ischemia is a complication following decompression of a hypertensive right ventricle in patients with left coronary-cameral fistulae. CASE PRESENTATION: We present a 12-year-old girl with a complex heart defect successfully operated on using a hybrid surgical-interventional approach to decompress the ventricle, embolize the fistula and reconstruct the atretic left coronary ostium. CONCLUSIONS: A novel hybrid strategy is the best solution for coronary-cameral fistulas reliant on high ventricular pressure at high risk for coronary steal phenomenon.

Surgical Repair of Congenital Atresia of the Left Coronary Ostium.

Congenital left main coronary artery atresia is an exceedingly rare condition with potentially fatal consequences if not diagnosed in a timely fashion. We present a case series in children and adolescents, including surgical repair and outcomes. We describe the presenting symptoms and subsequent management of each patient, including surgical repair and outcomes. (Level of Difficulty: Advanced.).

Congenital atresia of the left main coronary artery: management of a 5-year-old child in extremis.

Atresia of the left main coronary artery is a rare coronary anomaly. We describe the case of a 5-year-old child presenting in emergency in extremis. Clinical findings of haemodynamic collapse, malignant ventricular tachyarrhythmias and severe mitral regurgitation were indicative of a possible ischaemic aetiology. Surgical revascularization of the atretic left main coronary artery segment using an interposition autologous saphenous vein graft was successfully performed.

Sudden cardiac arrest of a 16-year-old boy with left main coronary artery atresia: a case report.

BACKGROUND: In many cases, the cause of exercise-induced cardiopulmonary arrest in young persons is thought to be fatal arrhythmia, and one of the causes is ischaemic heart disease. Left main coronary artery atresia (LMCAA) is an extremely rare disease in which there is a congenital defect of the left main coronary artery, causing heart failure and exercise-induced angina attacks at a young age. Thus, it is disease that should be differentiated when examining young persons with chest pain. CASE SUMMARY: A 16-year-old boy experienced sudden cardiopulmonary arrest during soccer practice, was brought to our hospital for emergency treatment after return of spontaneous circulation. Elective coronary angiography revealed findings indicating an osmium defect in the left coronary artery (LCA) and blood flow via collateral circulation from the right coronary artery. Contrast-enhanced coronary computed tomography (CT) angiography showed a defect in the LCA ostium and LMCAA was diagnosed in the patient. After coronary artery bypass grafting was performed, but the patient was discharged in an ambulatory state with a wearable cardiac defibrillator. Postoperative course has been favourable. DISCUSSION: Left main coronary artery atresia is an extremely rare disease in which there is a congenital defect of the left main trunk of the coronary artery and should be differentiated when encountering cases of heart failure or exercise-induced angina/arrhythmia attacks in young persons who are not at risk for atherosclerosis. Exercise electrocardiogram may show a false negative result, and therefore coronary CT is useful for diagnosis.

Imaging diagnosis of congenital left main coronary artery atresia / 中国介入影像与治疗学

Objective: To explore the feasibility of imaging diagnosis of congenital left main coronary artery atresia (LMCAA). Methods: Data of 6 patients with LMCAA, including 3 males and 3 females, 5 infants and 1 adult were retrospectively analyzed. All 6 patients underwent echocardiography, multidetector CT (MDCT) and cardiovascular angiography. Results: Echocardiography showed small diameter of left coronary artery without clear ostium, could not exclude the abnormal origin of left coronary artery from pulmonary artery in 1 case, small caliber of proximal segment of the left main coronary artery, intramural left coronary artery or ostial stenosis of the left main coronary artery in another case, mitral valve prolapse with severe regurgitation without coronary arterial abnormality in the rest 4 cases. MDCT correctly diagnosed LMCAA in 5 cases, considered ostium atresia or severe stenosis of the left coronary artery in 1 case. All the 6 patients were definitely diagnosed as LMCAA using cardiovascular angiography in all 6 patients. Conclusion: LMCAA is extremely rare. Echocardiography could reveal this disease, while MDCT may be an useful method. Angiocardiography remains the gold standard for preoperative diagnosis of LMCAA.

Left coronary artery atresia in the young: long-term follow-up without exercise restriction.

An 11-year-old male was presented with exertional chest pain and was diagnosed with atresia of the left main coronary artery. A stress nuclear perfusion imaging was negative at initial presentation, and a vasodilator stress cardiac MRI was again negative 5 years later. The patient has fully participated in competitive sports for 6 years with no occurrence of cardiac events.

Right single coronary artery as an incidental finding in Takotsubo syndrome and acute heart failure: Case report and review of the literature.

INTRODUCTION: Single coronary artery (SCA) with no associated congenital heart disease is a rare congenital anomaly. Most cases are asymptomatic and incidental findings, but SCA can cause ischemia, congestive heart failure, and sudden cardiac death (SCD). CASE REPORT: A 44-year-old woman presented with Takotsubo cardiomyopathy and cardiogenic shock. Selective cannulation of the left coronary artery (LCA) was not possible on coronary angiography (CA); an SCA was revealed arising from the right sinus, continuing distally as the circumflex artery and thereafter as the left anterior descending artery. Coronary computed tomography angiography (CCTA) confirmed left main atresia and no coronary stenosis. Cardiac magnetic resonance imaging (MRI) showed diffuse myocardial edema and no perfusion defects. The patient's clinical course was favorable under conservative management. DISCUSSION: Our paper describes an incidental finding of right SCA. We report a Lipton type R-I, in which a dominant right SCA supplies the entire myocardium. It is the rarest SCA presentation, with an incidence of 0.0008%; only 15 cases have been reported in the literature, all of which were studied by CA. Of these 15, one had SCD, five angina, one ventricular arrhythmia and one complicated acute coronary syndrome. CCTA confirmed the diagnosis in seven patients, MRI in one and transesophageal echocardiography in another. Nine patients had coronary lesions. Two underwent coronary artery bypass grafting, one percutaneous intervention and 11 conservative treatment. CONCLUSION: Right SCA with congenital absence of the LCA is one of the rarest coronary artery anomalies. In a significant percentage of patients it is associated with ischemia and can be life-threatening. CCTA and MRI are the modalities of choice for diagnosis and risk stratification.

Large right ventricular sinusoids in an infant with aorta-left ventricular tunnel and proximal right coronary artery atresia.

We report a 1-month-old infant diagnosed with an aorta-left ventricular tunnel, ventricular septal defect, and right coronary atresia with right ventricular sinusoids. The patient's anatomy and physiology did not indicate right-ventricular-dependent coronary circulation, and therefore right ventricular decompression could be performed without compromising coronary perfusion during surgical correction. A detailed understanding of the coronary anatomy is critical in managing this defect when coronary anomalies are present.

A Rare Case of Mitral Regurgitation with Congenital Left Main Coronary Artery Atresia / 日本心臓血管外科学会雑誌

A 65-year-old woman was admitted to our hospital complaining of chest pain in 2012. Coronary computed tomography angiogram revealed left main coronary artery atresia (LMCAA) with congenital absence of the left main trunk, and LAD received several collaterals perfusing from the RCA. She was observed because her symptoms were transient. However, her dyspnea on effort gradually became worse in 2016. Treadmill exercise electrocardiography showed ST depression in II・III・aVF and V4-6. Also, echocardiography showed severe MR due to the prolapse of the anterior leaflet and moderate TR. We performed mitral valve plasty and tricuspid valve plasty, and coronary artery bypass grafting using the left internal thoracic artery to the left descending artery in March, 2017. The postoperative course was uneventful. She was discharged in good condition 14 days after operation. LMCAA is a rare coronary anomaly. There are only 53 published case reports, and a few surgical reports in adult cases. This is a rare adult case of surgical treatment of LMCAA.

Expanding the cardiac spectrum of Noonan syndrome with RIT1 variant: Left main coronary artery atresia causing sudden death.

Noonan syndrome is a well-known genetic condition associating congenital heart defects, short stature, and distinctive facial features. Pulmonary valve stenosis and hypertrophic cardiomyopathy are the most frequent cardiac abnormalities, the latter being associated with a higher mortality. Here we report for the first time, a case of congenital left main coronary artery atresia in a Noonan syndrome associated with RIT1 variant, leading to unrescued sudden death. This case-report supports the already-suspected severity of the RIT1-related Noonan syndrome compared to average Noonan syndrome, and should encourage clinicians to be very cautious with these patients.

Congenital left main coronary artery atresia presenting as syncope and generalized seizure during exercise in a 13-year-old boy.

We report the case of a 13-year-old boy who, while running in a school gymnasium, experienced sudden syncope and seizure. CPR was started immediately, and an automated external defibrillator (AED) was attached, but shock was not induced. He was referred to our hospital for loss of consciousness and intermittent general tonic-clonic seizure. A 12-lead electrocardiogram showed normal sinus rhythm and no ST-T wave abnormalities. Echocardiography showed normal structural heart and normal cardiac function. On the second day of hospitalization, AED electrocardiogram showed complete atrioventricular (AV) block at syncope and seizure. After the patient recovered from this neurological state, we performed the treadmill exercise test, and it did not show ST-T wave abnormalities or AV block, and he did not complain of chest pain. Coronary angiography showed atresia of the left main trunk and the collateral vessel from the right coronary artery connected to the left coronary artery. He was diagnosed with congenital left main coronary artery atresia. We began administration of calcium antagonist and aspirin to prevent a coronary artery spasm and then performed a coronary artery bypass graft (CABG) to prevent sudden cardiac death. After CABG, he has had no syncope episodes at rest or during light exercise. .

Congenital atresia of the left main coronary artery in an adult: A rare anomaly with an unfavorable prognosis. Review of the literature.

Congenital atresia of the left main coronary artery (LMCA) is one of the rarest congenital anomalies, which may have an unfavorable prognosis leading to myocardial ischemia, ventricle dysfunction or even sudden cardiac death. There are 34 cases of LMCA in adults reported in the literature, most of them successfully treated with coronary revascularization. We report the case of an adult with LMCA who presented with terminal heart failure that required biventricular assistance and heart transplant.

Absent right coronary artery: A case of single coronary artery or congenital ostial atresia?

Atresia of the right coronary artery ostium is a rare anatomic variant of the coronary circulation. It is often difficult to differentiate from single coronary artery. Its presence unassociated with any other anomaly has never been described in an adult individual. We report this unusual anomaly and discuss its anatomical and pathophysiological significance and possible ways to differentiate from single coronary artery.

Right coronary artery atresia in Marfan's syndrome: A case report / 대한흉부외과학회지

A case of Marfan's syndrome with atresia of right coronary artery is reported. A 45-year-old woman, who was diagnosed as Marfan's syndrome 1 year ago, came to the hospital complaining of acute chest pain. The patient showed arachynodactyly, pectus carinatum, and long and slender extremities. In echocardiography there were severe aortic regurgitation measured grade IV and aortic dilatation of ascending aorta maximally 5.9 cm in diameter. Mitral regurgitation was mild, but there were also moderate left ventricular dilation and moderately decreased ejection fraction of left ventricle. At operation, atresia of right coronary artery was found. We performed Bentall type operation with SJM 27 mm valved conduit for left coronary artery, and Piehler's modification for right coronary artery bypass using 6 mm PTFE graft. The atretic portion of right coronary artery from the suspected right coronary ostium to distal coronary flow was about 4 cm in length. The combination of right coronary artery atresia and Marfan's syndrome is very rare. The author describes the rare case, which is treated with combined technique of Bentall and Piehler modification for reconstruction of coronary circulation.