Challenging situation of coronary artery anomaly associated with ischemia and/or risk of sudden death

Coronary artery anomaly is known as one of the causes of angina pectoris and sudden death and is an important clinical entity that cannot be overlooked.The incidence of coronary artery anomalies is as low as 1%-2%of the general population,even when the various types are combined.Coronary anomalies are practically challenging when the left and right coronary ostium are not found around their normal positions during coronary angiography with a catheter.If there is atherosclerotic stenosis of the coronary artery with an anomaly and percutaneous coronary intervention(PCI)is required,the suitability of the guiding catheter at the entrance and the adequate back up force of the guiding catheter are issues.The level of PCI risk itself should also be considered on a caseby-case basis.In this case,emission computed tomography in the R-1 subtype single coronary artery proved that ischemia occurred in an area where the coronary artery was not visible to the naked eye.Meticulous follow-up would be crucial,because sudden death may occur in single coronary arteries.To prevent atherosclerosis with full efforts is also important,as the authors indicated admirably.

A 63-Year-Old Male with D-Transposition of the Great Arteries Who Had an Early Form of the Arterial Switch Operation

We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the mid-1960s.We review the clinical and imaging data that support our conclusion.He had a diagnostic cardiac catheterization which demonstrated severe pulmonary hypertension responsive to epoprostenol and oxygen.Our case may represent one example of the experimental surgical work done prior to Dr.Adibe Jatene’s description of thefirst successful arterial switch performed in 1975.

Morphological Features of Complex Congenital Cardiovascular Anomalies in Fetuses:as Evaluated by Cast Models

Accurate prenatal diagnosis of complex congenital cardiovascular anomalies, vascular ones in particular, is still challenging. A fetal cardiovascular cast model can provide a copy of the cardiac chambers and great vessels with normal or pathological structures. This study was aimed to demonstrate three-dimensional anatomy of complex congenital cardiovascular anomalies in fetuses by means of corrosion casting. Twenty fetuses with prenatal-ultrasound-diagnosed complex cardiovascular anomalies were enrolled in this study（19 to 35 gestational weeks）. Fetal cardiovascular cast models were made by a corrosion casting technique. The specimens were injected with casting material via the umbilical vein, and then immersed in strong acid after casting fluid was solidified, to disclose the geometries of cardiovascular cavities. Nineteen cast models were successfully made from 20 specimens. The casts distinctly showed the morphological malformations and spatial relationship between cardiac chambers and great vessels. One hundred and eleven abnormalities were revealed by casting in the 19 specimens, including 34 abnormalities located in the cardiac chambers（3, 4 and 27 anomalies in the atria, atrioventricular valves and ventricles, respectively）, and 77 in the great vessels（28, 20, 24 and 5 anomalies in the aorta and its branches, the pulmonary artery, the ductus arteriosus and the major veins, respectively）. Corrosion casting can display three-dimensional anatomy of fetal complex cardiovascular anomalies. This improves our understanding of related pathomorphology and prenatal diagnosis.

Ellis-Van-Creveld Syndrome and Congenital Cardiac Anomaly: Common Atrium with Atrioventricular Canal Septal Defect

Background: Children presenting with physical features of chondro-ectodermal dysplasia (Ellis-Van Creveld syndrome) such as skeletal and joint abnormalities often have concomitant congenital cardiac anomalies. Presence of cardiorespiratory symptoms in children with Ellis-Van Craved syndrome warrants a thorough cardiologic evaluation to recognize and treat underlying congenital heart anomaly. Aim: A child with physical stigmata of Ellis-Van-Creveld syndrome is evaluated to detect an associated congenital cardiac anomaly and accomplish successful repair of the underlying cardiac lesion to reduce the cardiac related morbidity and improve the patient survival. Case Presentation: Ten years old boy with chondroectodermal dysplasia (dental anomalies, genu valgum and other skeletal abnormalities) presented with dyspnea and cyanosis. Cardiac evaluation by 2D echo revealed an atrioventricular (AV) canal septal defect with AV valve regurgitation and a common atrium. Angiocardiography showed a goose neck deformity of the left ventricular outflow tract. The Qp/Qs was 3.4: 1, with systemic arterial oxygen desaturation (SaO2 of 0.7) and O2 saturation in the common atrium was 0.7. The pulmonary venous connections to the common atrium were anomalous. Atriotomy on cardiopulmonary bypass and on a cardioplegic arrest discerned a partial AV canal septal defect with a common bridging leaflet, clefts in septal leaflets of tricuspid and mitral vlalves, an incompletely closed interventricular communication, and a common atrium with highly anomalous pulmonary venous insertions well anterior (8 cm) to vena caval orifices. Intracardiac repair was performed with two patches of Goertex to partition the common atrium into the pulmonary and systemic venous chambers after repair of the partial AV canal septal defect. Patient required only a temporary afterload reduction with enalapril;otherwise patient had an uneventful postoperative course. At a 2-year follow-up, the child was well without AV valve regurgitation and had normal biventricular function. Conclusion: A child with Ellis-Van-Creveld syndrome with skeletal abnormalities and dental anomalies had manifested with cardio-respiratory symptoms. Preoperative cardiac and intraoperative evaluation showed a common atrium with severely anomalous pulmonary venous connection and partial AV canal septal defect. Successful biventricular repair was accomplished by repairing the partial AV canal septal defect and partitioning the common atrium into left and right atrium by a complex atrial routing technique with two patches of Gore-Tex. On a follow-up at 2 years, the patient had adequate biventricular function without AV valve regurgitation. 114264

Isolated Type C Interrupted Aortic Arch in Adult:Extra-anatomic Repair Using Circulatory Arrest

INTERRUPTED aortic arch （IAA） is a rare congenital malformation that occurs in 5.8 per million live births. 1 IAA was thought to be incompatible with life once the ductus arteriosus closed. However, ifextensive collateral circulation is p possible. We report treated successfully an unusual resent at case of with extra-anatomic under deep hypothermia drculatory arrest birth survival is IAA, who was aortic repair

The Significance of Anatomical Variations of Hepatic Artery and Multiple Arterial Supplies in Embolization of Liver Tumors

A new therapeutic measure-step by step-embolization of every feeding vessel in dealing with liver cancer has been proposed on the base of the following criteria after reviewing the coeliac and superior mesenteric arteriographies in 100 cases of liver cancer.The emphasis is put on: 1.Anatomic variation of hepatic arteries. 2. Multiple feeding vessels, and 3. The importance of the presence of stenosis after embolization and the formation of collateral circulation.

Sixty-four-multi-detector computed tomography diagnosis of coronary artery anomalies in 66 patients

Background The abnormalities of coronary arteries, though rare and sometimes benign, may first present clinically as myocardial infarction or sudden death. Multi-detector computed tomography （MDCT） is a non-invasive test that is highly suitable for detecting these anomalies. The study aimed to review the 64-MDCT appearance of the coronary artery anomalies in 66 patients and to discuss the clinical importance of these anomalies.Methods In 6014 consecutive patients examined over 12 months by 64-MDCT for the study of coronary artery disease, 66 were diagnosed for coronary artery anomalies. All patients were symptomatic for one or more of the following diseases： chest pain, dyspnoea, palpitations, arrhythmia and myocardial infarction. Nine patients had undergone a coronary angiography. All the CT images were evaluated by two radiologists and one cardiologist. The right coronary artery （RCA） and the conus branch arising separately, myocardial bridging and duplication of arteries were not analysed in our study.Results The incidence of coronary artery anomalies found in our study group was 1.097%. In the selected patients, seven different types of coronary anomalies were found by 64-MDCT examination. The high takeoff, origin of the coronary artery from the opposite or noncoronary sinus with an anomalous course, and coronary artery fistula were the three common forms of anomalies （n=16, 18 and 16, respectively）. Compared with the results of the coronary angiography, the number of the drainage sites of two coronary artery fistula was less in MDCT images （3 small sites in total）. In all cases, coronary artery computed tomography angiography （CTA） technique was able to recognize the origin of the coronary artery, its three-dimensional course and its spatial relationship with the adjacent structures. Conventional coronary angiography in two cases, however, was unable to provide sufficient information for correct and complete diagnosis.Conclusions In conclusion, the study showed that 64-MDCT, especially the volume rendering technique （VRT）, may be useful for the assessment of complex variations, even if the conventional angiography may not be sufficient. It may be considered as the first-choice imaging modality when an anomalous coronary artery is suspected.

Occlusion with a Vascular Plug of a Congenital Circumflex Coronary Artery—Right Ventricle Fistula with Coronary Steal Phenomenon

Background: Coronary fistulas are abnormal communications with a worldwide prevalence of 0.002% in the general population. Aim: The aim of this study is to present a case of a congenital coronary artery fistula presented with coronary steal and describe this phenomenon. Methods: A systematic search was conducted to explore the relationship between the coronary steal phenomenon and coronary artery diseases using the PubMed database. Case Presentation: A seven-month-old female patient, was admitted to the hospital presenting diaphoresis on feeding and failure to thrive. Physical examination denotes continuous murmur in the fourth intercostal space left parasternal border (IV/VI) crescendo-decrescendo, wide and permanent unfolding of S2 (S1-A2-P2). An echocardiogram shows enlargement of right cavities, dilatation of the left coronary artery, circumflex artery connected to the right ventricle, and pulmonary artery systolic pressure of 45 mmHg. Tomography angiography demonstrated dilatation of the circumflex artery and a tortuous trajectory towards the posterior wall of the right ventricle, suggesting a coronary fistula. Results: The findings were corroborated by catheterization and a vascular plug (Amplatzer AVP II) was successfully placed. During follow-up complete obliteration was observed, with adequate weight gain and no associated symptoms. Conclusion: This case illustrates a distinctive clinical manifestation of congenital coronary artery fistula associated with the coronary steal phenomenon, establishing a new paradigm for the early diagnosis and management of this condition in the pediatric population.

Congenital coronary artery fistulas： dual-source CT findings from consecutive 6624 patients with suspected or confirmed coronary artery disease

Background Coronary artery fistulas （CAFs） are rare congenital abnormality often unintentionally found in patients with coronary artery disease. Clinical diagnosis of CAFs is difficult due to symptomless or lack of specific symptoms. Dual-source computed tomography （DSCT） might be a useful diagnostic tool for CAFs. The study aimed to retrospectively summarize the imaging features of CAFs delineated at DSCT in 48 CAF patients detected from consecutive 6624 patients with suspected or confirmed coronary artery disease in our institution. Methods Forty-eight patients underwent DSCT angiography by using retrospective electrocardiographic （ECG） gating after infusion of 70 ml of intravenous contrast material during breath hold. Maximum intensity projection （MIP）, curved planar reconstruction （CPR）, and volume rendering technique （VR） were obtained. Anomalous termination of coronary artery in each subject was evaluated by two radiologists （with more than 10 years experience with cardiovascular imaging）, and disagreement between diagnosis readers was settled by a consensus reading. Ten of 48 patients also underwent traditional coronary angiography （CAG） simultaneously. Results In each CAF case, DSCT angiography clearly demonstrated the origin, the termination, the size of abnormal vessel and its course in relation to surrounding great vessels. CAF arising from right coronary artery was the most common type, left circumflex was the least one involved among three coronaries, and pulmonary artery was the most common drainage site. Aneurismal fistulous tract, coronary atherosclerosis, myocardial bridging and anomalous origin of coronary artery were also detected in this group. The demonstration of drainage sites in CAG was consistent with DSCT angiography in 9 patients, and judgment on one anomalous connection in CAG was inconsistent with that in DSCT angiography. Conclusions DSCT angiography could provide accurate delineation of anomalous communications, size and numbers of fistulas in patients with CAFs. It suggested that DSCT is a useful tool for the assessment of CAFs and may be considered as the first-choice imaging modality, especially for patients with coronary artery disease.