Orifice Atresia Research Articles

Intraoperative diagnosis of coronary sinus orifice atresia associated with unroofed coronary sinus

Intraoperative diagnosis of coronary sinus orifice atresia associated with unroofed coronary sinus

Результати хірургічного лікування аноректальних вад розвитку у дітей

Мета. Оцінити віддалені результати хірургічного лікування аноректальних вад розвитку (АВР) у дітей з урахуванням анамнестичних даних.
 Матеріали і методи. Проаналізовано результати обстеження та хірургічного лікування 32 пацієнтів з АВР за останні 6 років.
 Результати. Оперативні втручання у дітей виконували в залежності від форми аноректальної вади. Післяопераційні ускладнення спостерігали у 9 (28,1%) із 32 пацієнтів, оперованих з приводу атрезії анального отвору та прямої кишки. Померли 2 (6,3%) недоношених дітей з супутніми вадами розвитку.
 Висновки. У дітей із вродженими АВР та ускладненим передопераційним періодом (недоношеність, супутні вади розвитку, внутрішньоутробна інфекція тощо) клінічний перебіг захворювання характеризувався великою кількістю різноманітних післяопераційних ускладнень у порівнянні з дітьми без обтяжливого анамнезу. Загальний стан дітей даної групи був значно тяжчим, вони потребували більш тривалої та інтенсивної передопераційної підготовки.

A Case of Unroofed Coronary Sinus with Coronary Sinus Orifice Atresia: Use of Multimodality Imaging.

A Case of Unroofed Coronary Sinus with Coronary Sinus Orifice Atresia: Use of Multimodality Imaging.

Clinical features and treatment for coronary sinus orifice atresia

Coronary sinus orifice atresia (CSOA) is a rare clinical condition, and usually does not require any treatment.

Clinical Features and Surgical Outcomes of Coronary Sinus Orifice Atresia.

This study aimed to review clinical features and surgical outcomes of coronary sinus orifice atresia (CSOA). From 2003 to 2015, 6 patients were diagnosed with CSOA. Median age at diagnosis was 2 years (range 0.2-73). CSOA was preoperatively diagnosed in 2 patients, intraoperatively in 3, and postoperatively in 1. Coronary venous blood flow drained into innominate vein through the left superior vena cava (LSVC) in 2, into the atrium through Thebesian veins in 2, and into both in 2. Three patients required surgical treatment. The first patient with a congenitally corrected transposition of great arteries had undergone ligation of the LSVC and a cutback of the proximal LSVC into the functional right atrium during the double-switch operation. The second patient with tricuspid atresia gradually developed hypoxia after the Fontan operation due to the increase in veno-venous shunt blood flow from the Fontan pathway to the right atrium through the LSVC and Thebesian veins, so that the LSVC was ligated and the coronary sinus was unroofed 6 years after Fontan operation. The other functional single ventricle patient had undergone ligation of the LSVC and a cutback of the proximal LSVC into the left atrium during the Fontan operation. There were no mortalities at the median follow-up of 9 years (1-11). CSOA should be suspected when the LSVC and usual-sized innominate vein were presented. Surgical treatment is required if the postoperative hemodynamics would affect coronary venous perfusion, like single ventricular palliation (244 words).

A Case of Concealed WPW Syndrome, Accompanied with Coronary Sinus Orifice Atresia and Persistent Left Superior Vena Cava

A 58-year old man was transferred because of repetitive attacks of tachyarrhythmia and crescendo angina pectoris. Electrocardiogram during attack showed paroxysmal supraventricular tachycardia with down slope ST depression. After emergent PCI, catheter ablation was performed. We could not insert the electrode catheter to coronary sinus, and delayed phase of CAG showed sinus orifice atresia and coronary venous flow to innominate vein via left superior vena cava. We successfully blocked left-sided concealed accessory pathway with trans-septal approach. Asynptomatic venous anomaly sometimes results in complication of cardiac surgery, device implantation and catheter ablation. The hemodynamic circuits should be precisely recognized before and during these procedures.

Coronary sinus orifice atresia with a persistent left superior vena cava and an aberrant communicating vein into the right atrium in an adult patient

Coronary sinus orifice atresia (CSOA) with a persistent left superior vena cava (PLSVC) is a rare cardiac anomaly.1,2 The gap between the blind end of the coronary sinus and the right atrium can be either a short-segment or a long-segment interruption.2 The anomalous PLSVCprovides themajor route for venous drainage from the coronary sinus; yet there can be small communicating veins between the coronary sinus and the right atrium.3 We report here on a rare case of an incidentally found CSOAwith PLSVC and there was an additional aberrant communicating vein into the right atrium inanadult patient. The patient was diagnosed using electrocardiogramgated 64-section multidetector row computed tomography (MDCT).

Surgical repair for anomalous drainage of left superior vena cava to left atrium

To investigate the characters and surgical treatment methods of anomalous drainage of left superior vena cava to left atrium. Clinical data of 8 cases were analyzed retrospectively.The patients aged from 6 months to 168 months with weight between 6 and 29 kg. The associated cardic lesions existed in all patients: 4 had partial atrioventricular septal defect (PAVSD),including 1 coronary sinus orifice atresia (CSOA), 1 ventricle septal defect (VSD), 1 ventricle septal defect associated with right ventricular outlet tract stenosis (VSD/RVOTS), and 1 atrial septal defect (ASD) and complete atrioventricular septal defect (CAVSD), respectively.No patient died. Preoperation diagnosis was in 1 case, intraoperation diagnosis in 5 cases,and postoperation diagnosis in 2 cases due to reoperation for hypoxemia.Three patients were repaired by intratrium tunnel, and the other 5 were managed by extracardic shunt. This lesion is easily missed in preoperation diagnosis,which can be avoided by awareness and careful inspection.The methods for management of anomalous drainage of left superior vena cava to left atrium depend on time of diagnosis and experience of surgeons.

Early Detection of Anomalous Origin of Left Coronary Artery from the Right Pulmonary Artery After Successful Repair of Critical Coarctation of the Aorta

Left ventricular (LV) function is impaired by increased afterload in neonates with severe coarctation of the aorta, which may result in endocardial fibroelastosis. Repair of the coarctation usually solves the problem, with LV function normalizing after a few weeks. This report describes a patient who underwent successful repair of critical coarctation with normalization of LV function despite signs of endocardial fibroelastosis but with persisting elevation of cardiac troponin T. Cardiac catheterization showed the rare coincidence of anomalous origin of left coronary artery from the right pulmonary artery (ALCAPA) and coronary sinus orifice atresia with left superior vena cava.

Light-guided surgery to repair coronary sinus orifice atresia with left superior vena cava

An 8-month-old male with coronary sinus orifice atresia, left superior vena cava, and single ventricle underwent light-guided coronary sinus unroofing concomitant with bidirectional cavopulmonary anastomosis to circumvent coronary sinus hypertension. During surgery, a 2.25 Fr angioscopic catheter was inserted into the coronary sinus via the left superior vena cava. The coronary sinus, lit by the illumination obtained from the catheter, was readily located from the left atrial interior, and unroofed. Light-guided coronary sinus unroofing is an easy, safe, and quick technique for the creation of unobstructed coronary sinus drainage in patients with coronary sinus orifice atresia and left superior vena cava.

Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia

A 6-month-old boy was diagnosed with coronary sinus orifice atresia, double-outlet right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and moderate common atrioventricular valve regurgitation associated with heterotaxy syndrome. Cardiac venous flow drained through a persistent left superior vena cava. We decided to perform coronary sinus orifice unroofing through the right atrium under a guide using a bougie. The persistent left superior vena cava was divided. Bidirectional Glenn anastomosis and edge-to-edge common atrioventricular valve repair were concomitantly performed. After a 1-year follow-up period, the patient is alive and well without any ischemic event.

Coronary Sinus Orifice Atresia With Left Superior Vena Cava in Patients With Univentricular Heart

Two patients with hypoplastic left heart syndrome had coronary sinus orifice atresia with persistent left superior vena cava. Both patients underwent successfully coronary sinus unroofing. One underwent surgery at the time of the bidirectional Glenn procedure and the other before creation of a total cavopulmonary connection. According to our population-based database, 10.3% of patients with univentricular heart have persistent left superior vena cava, and 2.3% have associated coronary sinus orifice atresia. Our cases highlight the importance of recognizing this anomaly in patients with univentricular heart to avoid high coronary venous pressure, which is potentially lethal.

Surgical repair of coronary sinus orifice atresia

We report a 10-month-old boy who underwent a bi-directional Glenn procedure and repair of coronary sinus orifice atresia. The left superior vena cava was the only vessel communicating with the coronary sinus in this case. The coronary sinus was allowed to communicate freely with the left atrium by creating a partially unroofed coronary sinus using a left superior vena cava flap.

Development of the coronary vasculature and its implications for coronary abnormalities in general and specifically in pulmonary atresia without ventricular septal defect

Coronary vascular anomalies are an important factor in congenital heart disease in the neonate. However, our knowledge of the pathomorphogenesis is still defective. (1) Study of coronary anomaly variations in congenital heart disease using specimens and (2) study of the role of epicardium-derived cells (EPDC) and neural crest cells in coronary vascular formation using quail-chicken chimeras. The clinical and pathological data revealed the existence of ventriculo-coronary arterial communications during fetal life before pulmonary atresia was established. This supported a primary coronary developmental anomaly as the origin of some cases of pulmonary atresia as opposed to other cases in which the pulmonary orifice atresia was the primary anomaly. Our experimental work showed the high relevance of the development of the epicardium and epicardium-derived cells for the formation of the coronary vasculature, and showed the coronary vascular ingrowth into the myocardium and subsequently into the aorta and the right atrium. The absence of epicardium-derived cells leads to embryonic death, while delayed outgrowth could result in the absence of the main coronary arteries to pinpoint orifice formation. In these cases, the circulation was maintained through ventriculo-coronary arterial communications. Neural crest cells were important for the patterning of the coronary vasculature. We have extended this knowledge to a number of other heart malformations. Coronary vascular anomalies are highly linked to the development of extracardiac contributors like the epicardium and the neural crest. A proper interaction between these cell types and the myocardium and aortic arterial wall are important for normal vascular development.

Surgical treatment of coronary sinus orifice atresia with hypoplastic left heart syndrome after total cavo-pulmonary connection

Atresia of the coronary sinus orifice is rare. We describe the surgical treatment of coronary orifice atresia in an infant with a persistent left superior vena cava after total cavo-pulmonary connection for hypoplastic left heart syndrome. The diagnosis was made by cardiac catheterization after total cavo-pulmonary connection at 8 months of age. After surgery, cardiac performance deteriorated. At reoperation, the coronary sinus was fenestrated to the left atrium. The patient survived surgical treatment of coronary sinus ostial atresia unroofed to the left atrium, guiding the placement of the fenestration with a probe placed through the open cardiac end of left superior vena cava.

Surgical management of coronary sinus orifice atresia in a case with HLHS

Surgical management of coronary sinus orifice atresia in a case with HLHS

The surgical importance of coronary sinus orifice atresia

Coronary sinus orifice atresia is rare. We describe two cases, one with an atrioventricular septal defect and another with supracardiac totally anomalous pulmonary venous drainage. The association with the latter has not been described previously. The importance of diagnosing the defect is emphasized and surgical treatment is discussed.

Discordant atrioventricular and ventriculoarterial connections with partially unguarded left-sided atrioventricular orifice and aortic atresia

AbstractCongenitally unguarded morphologically tricuspid valvar orifice is a rare abnormality, usually associated with pulmonary atresia. We describe such a case found in association with discordant atrioventricular and ventriculoarterial connections in which the aortic valve was atretic. Presentation was with respiratory distress due to severe cardiac failure after birth. Echocardiography demonstrated a dilated systemic ventricle with poor function, left atrial enlargement, and aortic atresia. The clinical findings were confirmed at autopsy.

Unroofed coronary sinus and coronary sinus orifice atresia: Implications for management of complex congenital heart disease

The aim of this study was to assess the morphology of the coronary sinus, its drainage and associated cardiac malformations when there is either complete unroofing of the coronary sinus or atresia of its connection to the right atrium. As more children with complex cardiac anomalies are accepted for primary surgical repair or palliation with cavopulmonary anastomoses, a knowledge of coronary sinus and systemic venous anomalies is important if coronary venous return is to be preserved and residual shunts avoided. Twenty-six heart-lung specimens without a coronary sinus draining to the right atrium were identified from the Leiden collection of congenital heart malformations. These were classified into specimens with an unroofed coronary sinus and those with atresia of the coronary sinus orifice. Attention was paid to the associated cardiac malformations. In 14 (54%; confidence limits [CL] 35%, 73%) of 26 specimens, there was an unroofed coronary sinus, associated with persistence of the left superior caval vein. An inferoposterior location of an atrial septal defect was detected in 2 (14%; CL -4%, 33%) of 14. Atrial appendage anomalies were seen in 13 (93%; CL 79%, 106%) of 14 specimens, exemplified by both right and left isomerism. These were frequently associated with an atrioventricular septal defect (12 [86%; CL 67%, 104%] of 14). An atretic coronary sinus orifice was seen in 12 (46%; CL 27%, 65%) of 26. Atrial appendage anomalies (2 [17%; CL -4%, 38%] of 12) were rare in these cases. The drainage was then by way of a left superior caval vein or, in its absence, a coronary sinus to left atrial window. Ventricular hypoplasia was seen in both categories of coronary sinus abnormalities. Important ventricular hypoplasia was seen in 12 cases (46%; CL 27%, 65%). These findings emphasize the need to study coronary sinus drainage before procedures such as ligation or transcatheter coil embolization of a left superior caval vein, venous redirection or closure of a dorsal atrial septal defect are contemplated. These procedures might inadvertently lead to impairment of coronary venous return or persistence of an intracardiac shunt.

Coronary sinus orifice atresia and persistent left superior vena cava. A report of two cases, one associated with atypical coronary artery thrombosis.

Fourteen previously reported cases of atresia of the coronary sinus ostium, in which the coronary venous flow entered the right atrium by a persistent left superior vena cava, the innominate vein, and the right superior vena cava, are reviewed and two new cases reported. The first new case was in a 43 year old woman with atypical chest pains in whom investigations for suspected ischaemic heart disease, including coronary arteriography, yielded normal results. She died suddenly from massive myocardial infarction due to extensive old thrombotic occlusion of a major coronary artery without any appreciable underlying disease of the vessel wall. Exercise testing is considered to be advisable in symptomatic patients with normal coronary angiograms and attention to the venous phase might be informative. The second new case occurred in a child with an atrial septal defect and complete transposition of the great arteries, who died aged 1 month.