A Case of Surgical Treatment of a Low-Birth-Weight Infant with Tetralogy of Fallot/Pulmonary Valve Absence, Cervical Arch, and Single Coronary Artery / 日本心臓血管外科学会雑誌

The case is an infant referred to the mother after a fetal ultrasound at 22 gestational weeks revealed a left renal cyst and congenital heart disease. He was born at 36 weeks and five days, weighing 2,282 g, with Apgar 8/8 points, and diagnosed with tetralogy of Fallot, absent pulmonary valve, pulmonary regurgitation, persistent left superior vena cava, right aortic arch, and cervical arch. He was placed on ventilatory management immediately after birth, but was extubated at age nine days. On approximately day 26, since the SpO2 markedly decreased and bronchoscopy showed only mild stenosis, we concluded that decreased pulmonary blood flow mainly caused cyanosis. Because of the low body weight and presence of a single coronary artery, we decided to proceed with a palliative surgery. However, since the patient had a cervical arch and the usual Blalock-Taussig shunt was anatomically difficult, we performed a central shunt＋main pulmonary artery ligation on day 49. Cyanosis improved and his respiratory condition was stable, but contrast-enhanced computed tomography showed a tendency for left pulmonary artery enlargement and left main bronchus compression. On day 87, the Rastelli procedure＋bilateral pulmonary artery plication was performed because worsening airway symptoms were anticipated. The patient’s respiratory condition stabilized postoperatively, and he was extubated 10 days postoperatively. On day 136, the patient was discharged with a home high-flow nasal cannula.

A study of simple congenital coronary artery anomalies based on a large-sample coronary CT angiography / 中华放射学杂志

Objective:To investigate the classification and prevalence of simple congenital coronary artery anomalies (CCAA) in Chinese in a large samples of coronary CT angiography (CCTA), and briefly summarize the CT characteristics of "malignant" anomalies.Methods:The data of patients who had undergone CCTA from July 2009 to January 2017 and were diagnosed as simple CCAA were analyzed retrospectively. CCAA were classified according to the origination, number of orifices and termination, and the prevalence of various CCAA was statistically analyzed. Anomalous origin of coronary artery included the origin of coronary artery from the opposite coronary sinus, the non-coronary sinus, the aorta or around the primary sinus, the pulmonary artery, and left circumflex artery originated from the diagonal branch. Abnormal number of orifices included single coronary artery (SCA), right coronary artery and conus branch arising separately, and left anterior descending branch and circumflex branch arising separately. Abnormal termination was coronary artery fistula (CAF). Anomalous origin of coronary artery from the opposite sinus, anomalous origin of the coronary artery from the pulmonary artery, SCA, and multiple or large CAFs were defined as"malignant"anomalies.Results:Among 165 133 patients, 2 148(1.301%) had coronary anomalies, including 1 302 (0.789%) of origin anomalies, 298 (0.181%) of abnormal number of orifices and 548 (0.332%) of abnormal termination. There were 700 cases (0.424%) with coronary artery rising from the opposite sinus, 179 of which had opening or proximal lumen stenosis due to compression, 7 of which had subendocardial myocardial ischemia or infarction on CCTA without coronary artery disease (CAD). The coronary arteries originated from posterior sinus, the aorta or around the primary sinus were found in 48 cases (0.029%), 531 cases(0.322%), respectively. Coronary artery originating from pulmonary artery was detected in 20 cases (0.012%), all of which were left main trunk originating from pulmonary artery, showed tortuous dilation of coronary arteries. Five cases showed obvious subendocardial myocardial ischemia or infarction without CAD. SCA, right coronary artery and conus branch arising separately, left anterior descending branch and circumflex artery arising separately were detected in 102 (0.062%), 53 (0.032%) and 143 (0.087%) cases respectively. Only 1 of 102 cases with SCA showed myocardial ischemia without CAD. A total of 548 cases (0.332%) were diagnosed as CAF, of which the coronary-pulmonary fistula was most common with a highest prevalence of 0.277% (458 cases). Coronary artery-atrial fistula and coronary artery-ventricular fistula were detected in 22 (0.013%) and 60 (0.036%) cases. There were 6 cases (0.004%) of coronary artery-coronary venous fistula and 2 cases (0.001%) of coronary artery-superior vena cava fistula.Conclusions:The occurrence of CCAA is not uncommon, among which anomalous origin of coronary artery is the most common, and special attention should be paid to "malignant"anomalies.

A Case of Surgical Treatment of Anomalous Origin of the Single Coronary Artery from the Pulmonary Artery / 日本心臓血管外科学会雑誌

A 2-month-old male infant was transferred to our hospital for suspected cardiomyopathy because he had livedo reticularis and peripheral coldness. An electrocardiogram showed ischemic change and an echocardiogram showed a dilated, poorly functioning left ventricle. Therefore, we performed urgent cardiac catheterization. The coronary artery was not visualized by aortography, but the single coronary artery arising from the pulmonary artery was depicted by pulmonary angiography. Therefore, we performed emergent surgery. The whole coronary arteries traveled directly inferiorly from the left side of the pulmonary trunk. A patent ductus arteriosus (PDA) was closed at the operation. We established cardiopulmonary bypass using two arterial cannulae through both ascending aortas and pulmonary trunk to maintain coronary blood flow. Direct implantation is difficult because the coronary ostium is far from the ascending aorta. Therefore, we chose to perform the Takeuchi procedure using an intrapulmonary artery tunnel. The patient's postoperative course was good, and he was discharged on postoperative day 22. A case of an anomalous origin of a single coronary artery from the pulmonary artery without any other heart disease is extremely rare. Management of cardiopulmonary bypass and myocardial protection in this abnormality is discussed.

Single coronary artery originating from the right sinus Valsalva and ability to work

We present a case of a 56-year-old male electrician who was admitted to the hospital with atrial fibrillation, atypical chest pain and dyspnea. He gave a history that on the morning he had working for almost 4 hours carrying out various activities with considerable physical effort. After cardioversion, conventional coronary angiography revealed a suspect of single coronary vessel (SCA) arising from the right sinus of Valsalva. The patient underwent multislice computed tomography that showed a SCA arising from the right sinus Valsalva and dividing in Right Coronary Artery (RCA) and Left Main coronary artery (LM). The finding of posterior course of the LM without atherosclerotic has proved crucial for the expression of an opinion of working capacity even with limitation.

Funtional significance of the intermediate lesion in a single coronary artery assessed by fractional flow reserve

No abstract available.

Truncus arteriosus: surviving at 46 years without intervention / Tronco arterioso: sobreviviendo a los 46 años sin intervención

The median survival of patients with truncus arteriosus is five weeks of age with rare cases reaching adulthood. The longest reported survivor with balanced ventricles, as the index case has, is 38 years of age. The index case is a 46-year old male with Type 3 truncus arteriosus. His case has never been documented in the English Literature as far as the author is aware.

La mediana de la supervivencia de pacientes con tronco arterioso es cinco semanas de edad, siendo raros lo casos que alcanzan la edad adulta. El caso más viejo reportado con ventrículos balanceados, como en el caso índice, es un sobreviviente de 38 años de edad. El caso índice es un hombre de 46 años con tronco arterioso de tipo 3. Su caso nunca se ha documentado en la literatura en inglés, hasta donde el autor tiene noticias.

Superdominant right coronary artery with absent left circumflex artery

Noninvasive imaging of coronary artery disease is rapidly replacing angiography as the first line of investigation. Multislice CT is the non-invasive modality of choice for imaging coronary artery disease and provides high speed with good spatial resolution. CT coronary angiography in addition to detecting and characterising atherosclerotic coronary artery disease is also a good imaging tool for evaluating anomalies of coronary arteries. Superdominant right coronary artery with absent left circumflex artery is one such rare coronary artery anomaly which is well evaluated with multislice CT angiography. The authors report one such case of superdominant right coronary artery with absent left circumflex artery imaged with 64-slice MDCT.

Unexpected Sudden Death of a 19-Year-Old Female with Congenital Single Coronary Artery Ostium during Exertion

Coronary artery anomalies are associated often with myocardial ischemia or sudden cardiac death. A 19-year-old woman who participated in an exertive game lost consciousness upon one such exertion. She was taken to a hospital where she died on the same day. An autopsy revealed that she had bifurcated coronary arteries, which arose from one coronary ostium in the left sinus of Valsalva. The right coronary artery arose from the left sinus and traveled between the aorta and the pulmonary trunk. The heart as well as the cardiac conduction system depended exclusively on the single coronary artery ostium for oxygenated blood supply, and the unbalanced blood distribution on her exertion probably led to sudden cardiac death. The case highlights the medicolegal importance of unexpected sudden cardiac death related to an anomalous origin of the coronary arteries.

Aortic Valve Replacement in a Case of Anomalous Origin of the Right Coronary Artery / 日本心臓血管外科学会雑誌

Congenital anomalies of the coronary artery are rare. However, they can cause sudden death because of arrhythmia. We present a case of a 62-year-old man with severe aortic valve regurgitation associated with an anomalous origin of a narrowed right coronary artery (IB₂ according to the Shirani Classification) detected on preoperative coronary three-dimensional computed tomography (CT) . The patient underwent both aortic valve replacement for aortic regurgitation, and coronary artery bypass. The postoperative course was uneventful.

A Case of Single Coronary Artery Originating from Left Aortic Sinus

An isolated single coronary artery from the left aortic sinus is one of rare congenital coronary anomalies. This anomaly was detected during elective coronary angiogram in a 44 year-old male patient with atypical chest pain. Treadmill exercise test demonstrated 1 mm horizontal depression at stage IV and the test was terminated at the V stage due to chest pain. No myocardial ischemia was demonstrated on myocardial SPECT using 99mTc-tetrofosmin. Coronary angiogram revealed single coronary artery originating from left aortic sinus without any stenotic lesions.

Stenting of an Anomalous Coronary Artery in Acute Myocardial Infarction

Single coronary artery is rare and the application of coronary angioplasty to these arteies presents unique technical challenge. As technical advances in the guiding catheter and balloon system occur, more challenging lesions including anomalous coronary artery are able to be treated. Correct guiding catheter selection is important to ensure adequate access to the anomalous vessel and provide support to cross the lesion. This report describes successful angioplasty with stent in right coronary artery of single ostium coronary anomaly in a patient presenting with an acute myocardial infaction.

A Case of Single Coronary Artery with Bicuspid Aortic Valve

Single coronary artery is one of coronary artery anomalies, which originates from single aortic ostium and distributes blood to whole myocardium and is reported to occur in about 0.02 percent of general population. Although this condition does not produce severe clinical complications, it is often combined with other congenital cardiac anomaly and may produce angina pectoris, arrythmia, and sudden cardiac death syndrome rarely. Bicuspid aortic valve is anomalous aortic valve which consists of two commisure and two cusps and is said to occur in about 2 percent of the population. In some cases, the valve may function normally for many decades, but in others, it may produce aortic stenosis and/or aortic regurgitation frequently. We report here a case of single coronary artery combined with bicuspid aortic valve.