Sinus Venosus ASD Research Articles

MODIFIED DEEP TRANSGASTRIC TRANSESOPHAGEAL ECHOCARDIOGRAPHY IMAGING OF SUPERIOR VENA CAVA

<h3>Background</h3> Superior vena cava is usually interrogated intraoperatively by a standard bi-caval view as suggested by American Society of Echocardiography. A modified deep transgastric transesophageal echocardiography view is suggested that will superior interrogation of the superior vena cava intraoperatively. <h3>Objective</h3> Evaluation of a modified deep transgastric imaging of superior vena cava in patients undergoing cardiac surgery. <h3>Methods and material</h3> A modified deep transgastric imaging of superior vena cava was performed in children undergoing cardiac surgery. The deep transgastric interrogation with color Doppler blood flow map showed the venous return pattern. A pulse wave Doppler signal analysis was also feasible. The position of the tip of a central venous catheter tip that was inserted through the internal jugular vein could be seen. An agitated saline contrast injected into the left upper limb could exclude the presence of a left superior vena cava. In patients undergoing a undergoing a bi-directional Glenn shunt, considerable length of superior vena cava was visualized. In a child with a partial anomalous pulmonary venous drainage and a superior sinus venosus ASD, a point of the pulmonary vein joining the superior vena cava could be identified as it was possible to interrogate a considerable length of superior vena cava compared to the standard mid esophageal bi-caval view. The deep transgastric TEE interrogation of superior vena cava revealed a residual gradient in the superior vena cava following a sinus venosus atrial septal defect repair that was corrected at the same sitting. A color Doppler blood flow map across the superior vena cava following removal of a directly inserted venous cannula showed turbulence and a continuous-wave Doppler signal analysis revealed a residual gradient that was immediately corrected by the surgeon in another patient. <h3>Conclusion</h3> In conclusion, the deep transgastric TEE view of the superior vena cava is clinically useful. The doppler alignment is parallel to SVC flow (less than 20 degree) which is superior to bi caval view, where the doppler alignment is almost perpendicular to the flow. The probable difficulties that may be encountered that would interfere with proper visualization are: (i) poor contact of the probe with cephalad aspect of the stomach, (ii) air in the stomach, (iii) use of inadequate contact jelly, and (iv) lack of experience. There is a deep learning curve that can be a practical impediment but once honed, it could be a really useful adjunct to our routine trans-esophageal views.

Fusion between 3D transthoracic echocardiography and computed cardiac tomography: A novel diagnostic approach in congenital heart disease

Cardiac ultrasound has remained the primary tool for diagnostic and surgical decision-making for congenital heart disease (CHD). With recent developments in fusion imaging especially between CT and 3D echocardiography, previously described in percutaneous tricuspid annuloplasty and left atrial appendage closure, we obtain more information from combined images. Our aim is to study the feasibility and the added preoperative input and educational value of CT/3D-US fusion in prospective cohort of CHD patients more specifically in double outlet right ventricle, where a biventricular repair approach may need delicate preoperative analysis in order to achieve the best result. We prospectively included 25 patients (median age: 5 years) with mild to complex CHD, who underwent cardiac CT and 3D transthoracic echocardiography as part of their pre-operative or follow-up evaluation at our centre. We included three complete atrioventricular canal, three transposition of the great arteries, two congenitally corrected transposition of the great arteries with VSD and pulmonary stenosis, seven double outlet right ventricle, three truncus arteriosus, two tetralogy of Fallot, three Sinus Venosus ASD, one multiple VSD and one congenitally abnormal aortic valve. Time for completing fusion process and the quality of fusion between CT and 3D-US were noted. Educational value was evaluated by fellows. Fusion was successful in all patients with median fusion quality scored 4/5. The median time to complete the fusion process was 735 seconds [628–1163] regardless of the cardiopathy complexity. Landmarks were significantly modified in complex congenital heart disease. Educational value was scored by fellows (C: No-Little value; B: Intermediate value; A: Major value) ( Fig. 1 ). Using Fusion modatily is feasible for everyone with little knowledge. The simultaneous visualization of intracardiac structures with fusion helps better understand anatomical relations of atrioventricular valves with outlets and helps young fellows in pediatric cardiology better visualize intracardiac repair and familiarize with congenital heart disease. Fusion technique can be routinely applied to patients regardless of their age and weight.

Pattern of Vascular Anomalies Associated With Sinus Venosus Atrial Septal Defect.

ObjectiveTo evaluate children with sinus venosus atrial septal defect (SV-ASD) for associated vascular anomalies.MethodologyA total of 72 children with sinus venosus atrial septal defect with partial anomalous pulmonary venous return who presented to pediatric cardiology unit, Lady Reading Hospital Peshawar, from January 2019 till June 2021 were included in this cross-sectional study. Diagnosis of sinus venosus atrial septal defect was confirmed through two-dimensional (2D) and Doppler echocardiography performed by a pediatric cardiologist. Cardiac CT angiography was performed and assessed by a pediatric cardiac interventionist and radiologist. Patients were managed according to standard protocols and guidelines. The data were entered and analyzed with Statistical Package for the Social Sciences (SPSS) version 20. Percentages were used to express frequencies.ResultsMean age was 8.3 ± 2.7 years (interquartile range (IQR): two months to 18 years). There were 37 (51.4%) male and 35 (48.6%) female patients. Out of 72 patients, 64 (88.8%) patients had superior sinus venosus atrial septal defect, while inferior sinus venosus atrial septal defect was found in eight (11.1%) patients. In six (8.3%) patients, associated secundum atrial septal defect was identified. Bilateral superior vena cava was found in seven (9.7%) patients. Left aortic arch was seen in 70 (97.2%) patients, whereas two (2.7%) patients had right aortic arch.ConclusionSinus venosus ASD is a rare type of atrial septal defect which is also associated with both pulmonary and systemic vascular anomalies. Diagnosing these vascular anomalies is of paramount importance before any corrective intervention can be done. Recognizing the pattern of these anomalies should be known to every interventional cardiologist, radiologist, and cardiac surgeon. Echocardiography alone is not a good tool to assess these extracardiac structures. Imaging modalities like CT angiography and MRI have refined our preoperative workup which is essential for the ultimate outcome of the corrective intervention.

Electrocardiographic Patterns of Atrial Septal Defects

Objective: This study was designed to evaluate the electrocardiographic features of Atrial Septal Defect to establish an association with crochetage pattern and incomplete RBBB. Materials and methods: ASDs of Primum, Secundum and Sinus Venosus type were included in this study. From October 15, 2003 till January 15, 2004, a total of 104 consecutive patients of ASD were studied at Punjab Institute of Cardiology, Lahore. Patients were included in the study after confirmation of Atrial Septal Defect on transesophageal echocardiography. Results: The mean age of the study population was 26.28±11.40 years. Among these 56(53.8%) patients were females and 48(46.2%) patients were males. The study population consisted of 94(90.4%) Secundum ASD patients followed by Sinus Venosus ASD 8(7.7%) patients and Primum ASD in only 2(1.9%) patients. Only 59(56.7%) patients had crochetage pattern in any of the leads while it was absent in 45(43.3%) patients p=0.470. Incomplete RBBB was present in 92(88.4%) patients. It was observed in 2(1.9%) patients in ASD Primum, 82(78.8%) patients in ASD Secundum and 8(7.7%) patients in ASD Sinus Venosus type. P=0.483.Conclusion: Although we did not observe a statistically significant association between crochetage pattern or incomplete RBBB and ASD but the presence of any of these two considerably increases the specificity of the ECG for the diagnosis of ASD.

A Persistent left Superior Vena Cava: Case report

Background: Persistent left superior vena cava (PLSVC) is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress. It is most commonly observed in isolation but can be associated with other cardiovascular abnormalities including atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, and cortriatriatum. Objective: Aimof this case presentation was to describe a rare case of persistent left superior vena cava that is associated with atrial septal defect and partial anomalous pulmonary venous drainage. Also, how to suspect and then confirm the presence of PLSVC by echocardiographic examination, and to know the role of multi-slice CT and cardiac MRI for detection of other cardiac and extra-cardiac anomalies. Patients and methods: Male patient aged 32 year complaining of shortness of breath for about 1 year, echocardiographic examination revealed dilated coronary sinus and injection of agitated saline indicated the presence of persistent left superior vena cava. PLSVC may be associated with other anomalies so further evaluation by multi-slice CT was done to confirm the diagnosis of PLSVC as well as sinus venosus ASD and partial anomalous pulmonary venous drainage so surgical consultation was done for surgical correction. Conclusion: Diagnosis of PLSVC is important, as it is often associated with other anomalies as atrial septal defect and partial anomalous pulmonary venous drainage, which lead to pulmonary hypertension and need surgical correction before the occurrence of pulmonary hypertension.

Sinus venosus ASD and PAPVC with constrictive pericarditis: a rare association.

This is a case of a young man who underwent preoperative computed tomography evaluation for sinus venosus type of atrial septal defect, and was found to have associated chronic constrictive pericarditis. Association of these two entities is rare and changes the management approach.

SINUS VENONUS ATRIAL SEPTAL DEFECT PRESENTING WITH EXERTIONAL SYNCOPE

Sinus Venosus ASD (SVSD) presents with dysphnea, exercise intolerance and atrial arrhythmias. We describe a case of large SVSD presenting with exertional syncope (ES). 26 year male presented with a witnessed syncopal episode while playing soccer. He had 2 other episodes of ES in recent past. ECG

P1729 Abnomral course of pulmonary vein causing right heart dilatation

Abstract Funding Acknowledgements None Introduction Partial anomalous pulmonary venous return is a rare congenital cardiac anomaly that usually involves the right pulmonary vein and an atrial septal defect. It is accounted for 0.7%. Isolated partial anomalous pulmonary venous return with an intact atrial septum is even rarer, and this condition is usually treated surgically in younger patients. We report a case of a young female, incidentally found to have a right upper pulmonary vein draining into the right atrium, confirmed by TOE and CT Venography pulmonary, with moderate right ventricular enlargement, without pulmonary hypertension, intra-cardiac shunt or accompanied congenital anomaly. A decision of surgical correction was made. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, even in absence of ASD. Case presentation A 26-year-old female, not known to have any chronic illness. She was referred because of poor appetite and low weight, to cardiology evaluation for accidental murmur finding during examination. She had no other symptoms. The TOE was done which showed left pulmonary veins are normal, the right lower pulmonary vein is normal, and there is high suspicion of aberrant right upper pulmonary vein draining in the right atrium, no evidence of intra-cardiac shunt. CT Venography pulmonary revealed anomalous (Giant) right superior pulmonary vein to the superior vena cava and draining to the right atrium. Figure 1. Patient was referred for surgical correction. Discussion Partial anomalous pulmonary venous return is a type of left to right shunt. It is a rare congenital abnormal cardiac defect in which the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Its accounted for 0.4 to 0.7%. However, as this rate is based upon autopsy data, the true prevalence of PAPVR may actually be higher. PAPVR is frequently associated with other congenital heart disease, most commonly ASD, in &amp;gt;75% of patients. It is estimated that 10% to 15% of patients with ASD and up to 85% of patients with sinus venosus ASD have PAPVC. Exclusive of ASD, other more complex cardiac malformations occur in ∼20% of patients. PAPVR can also be an isolated defect with intact atrial septum. TOE is more sensitive than TTE in detecting PAPVR. A potential limitation of echocardiography is the availability of acoustic windows. If all four PVs are not identified emptying into the left atrium on TEE, CCT and CMR may provide complementary data . CONCLUSION In conclusion, missed diagnosis always occurs in PAPVR, the debauched and blood flow of pulmonary vein should be over-viewed carefully during TEE examination. In a patient with unexplained RV enlargement PAPVC (one or more pulmonary veins) should be considered in the differential diagnosis. Multi-modality cardiac imaging using echocardiography, CT angiography and CMR may provide a comprehensive noninvasive evaluation of PAPVC. Abstract P1729 Figure. Fig1 TOE (Top),CT (Bottom) of RUPV

DILATED CARDIOMYOPATHY AND ATRIAL ARRHYTHMIA IN A PATIENT WITH A PRIOR REPAIRED ATRIAL SEPTAL DEFECT: DON'T LET THE HISTORY FOOL YOU

Unrepaired sinus venosus ASD is a rare but recognized congenital cardiac lesion. Modern multimodality imaging is required for comprehensive diagnosis and to direct surgical management. A 58 year old male with history of remote surgical ASD closure presented with dilated cardiomyopathy thought to be

Percutaneous repair of sinus venosus ASD: the end of congenital cardiac surgery?

Percutaneous repair of sinus venosus ASD: the end of congenital cardiac surgery?

MY APPROACH to the adult patient with newly identified atrial septal defect

1016/j.tcm.2015.05.017 blished by Elsevier Inc. n PracticeUpdate on January 21, 2015. Republishe Secundum ASD is the most common type. Less common ASDs include sinus venosus, coronary sinus, and primum. There are other rare ASD subtypes, but these four are the ones seen most frequently. If you suspect that your patient has an ASD, the first test to establish the diagnosis is echocardiography, either transthoracic or transesophageal. The sensitivity of transthoracic echocardiography in detecting ASD varies by the size of the defect, and specific patient factors, such as body habitus, which may limit the ability to obtain diagnostic transthoracic views. After you identify the defect, you have to look for any associated congenital heart disease that can occur alongside an ASD; this is the second step. For example, sinus venosus ASD is commonly associated with a partial anomalous pulmonary venous connection. The latter can happen with secundum ASD as well, but not as commonly. So, whenever you are diagnosing someone with an ASD, you have to look for a partial anomalous pulmonary venous connection. Whenever you see a partial anomalous pulmonary vein, you have to look for an ASD. They often go together. The reason why it's important to define the anatomic type of ASD and the presence of an associated defect is that it can affect your management plan. For example, a secundum ASD is almost always treated with a device closure, but when a sinus venosus ASD, primum ASD, or coronary sinus ASD is present, device closure is not an option. If you go to surgery and the patient has an anomalous pulmonary venous connection plus an ASD, the surgery is different because you have to consider doing what is called a Warden procedure, whereby you channel the anomalous vein back into the left atrium through the atrial septum. Primum ASDs are commonly associated with cleft of the anterior mitral valve defects and mitral regurgitation, and they are commonly associated with left anterior hemiblock on EKG. Associated defects can usually be determined by EKGs or echocardiography. Occasionally, cardiac MR or CT is required to assess the pulmonary venous connections.

An Uncommon Presentation of a Rare Disorder - Ruptured Vein of Galen Malformation with Atrial Septal Defect

Vein of Galen aneurysmal malformations (VGAMs) are rare intracranial anomalies constituting 1% of all the cerebrovascular malformations. Its association with congenital heart disease has been described. Most reported cases are in the postnatal period associated with cardiac failure. Rupture of this malformation is uncommon. Here we describe a case of an otherwise healthy 25 year old presenting with sudden change in his mental status. Unenhanced CT brain showed intracranial hemorrhage secondary to rupture of the VGAM. Cerebral angiography showed stenosis of a persistent falcine sinus, which likely delayed his presentation. Endovascular embolization of the varix was performed. Echocardiogram showed a large sinus venosus ASD with anomalous pulmonary venous return. He sustained residual neurological deficits and is currently undergoing rehabilitation. This is a rare case of delayed presentation of this disease despite the underlying cardiac defect, with an uncommon complication of rupture and an unfortunate outcome.

Multiple intracranial abscesses: Heralding asymptomatic venosus ASD

A case of multiple intracranial abscesses in an immune-competent young girl is reported. She had chicken pox. Two weeks later, she presented with multiple intracranial abscesses. No significant cardiac abnormality was detected on transthoracic echocardiogram (TTE). The condition was treated medically. However, one of the abscesses adjacent to the CSF pathways enlarged on treatment and caused obstructive hydrocephalus that required stereotactic aspiration. Gram stain showed gram positive cocci in chain. Pus was sterile on culture. She was treated with broad spectrum IV antibiotics based on Gram staining report for 6 weeks followed by another 8 weeks of oral antibiotics. She made good recovery and had been leading a normal life. The abscess capsules took 30 months to resolve completely on MRI. A repeat TTE done in the follow up showed enlarged right heart chambers with a suggestion of a venosus ASD. A trans-esophageal echocardiogram (TEE) confirmed the presence of sinus venosus ASD from the SVC side with mainly left to right shunt. There was also partial anomalous drainage of the pulmonary veins. The patient underwent correction of the defect and has been doing well.

Impact of Warden's Procedure on the Sinus Rhythm: Our Experience

Our experience with the use of Warden's procedure for the repair of sinus venosus ASD with anomalous right upper pulmonary venous connection. Fifty-eight patients had Warden's procedure from September 2008 to May 2011. The demographic data, preoperative and postoperative ECG, aortic cross-clamp time, cardiopulmonary bypass time, Holter monitoring, complications length of ICU and hospital stay were analysed. The male to female ratio was 1:1. The median age was 10.9 years (range 2-48 years). Preoperatively all patients were in sinus rhythm. Twenty-six patients had associated left superior vena cava and two patients had also Tucker's procedure at the same time. The mean follow up was 1.2 years (range 1 months-2.8 years). Postoperative Holter monitoring showed sinus rhythm in all the patients. The mean CPB time was 83.6 min (range 54-163 min), mean aortic cross-clamp time was 48.0 min (range 22-112 min), mean ICU stay was 1.26 days (range 1-4 days) and length of hospital was 8.3 days (range 5-13 days) Postoperative echocardiography showed less than 1 mm Hg gradient across the SVC-RA appendage anastomotic site in all the patients. No mortality was recorded. This is the largest reported series of Warden's procedure in the literature till date. It is known from other studies that sinus node dysfunction and conversion to junctional nodal rhythm were quite a concern with the conventional techniques and Warden's procedure specifically avoids these problems.

Hybrid peratrial double device closure of a patent foramen ovale and sinus venosus defect in an infant with vein of galen malformation

Vein of Galen aneurysmal malformation is the most common cerebral arteriovenous malformation in children. Neurointerventional embolization has improved outcomes with this lesion. An association with cardiac defects has been recognized and can increase the risk of systemic embolic events during treatment. Cardiopulmonary bypass (CPB) to correct the cardiac defect before repair of the malformation may be associated with compromised cerebral perfusion and possible neurologic injury. We describe a successful hybrid peratrial closure of two intracardiac shunts (a patent foramen ovale and a sinus venosus ASD) before neurointervention, avoiding CPB, and eliminating the intracardiac embolic risk during treatment of the vein of Galen malformation.

Percutaneous mitral valvuloplasty following surgical repair of sinus venosus atrial septal defect

Mitral valvuloplasty performed 5 y after repair of a sinus venosus ASD was difficult because of a thickened septum, but resulted in improved mitral valve opening and did not lead to ASD. Thus, prior repair of a sinus venosus ASD may not be an absolute contraindication to mitral valvuloplasty.

Galvanic crystallization

Our experience with the use of Warden's procedure for the repair of sinus venosus ASD with anomalous right upper pulmonary venous connection.Fifty-eight patients had Warden's procedure from September 2008 to May 2011. The demographic data, preoperative and postoperative ECG, aortic cross-clamp time, cardiopulmonary bypass time, Holter monitoring, complications length of ICU and hospital stay were analysed.The male to female ratio was 1:1. The median age was 10.9 years (range 2–48 years). Preoperatively all patients were in sinus rhythm. Twenty-six patients had associated left superior vena cava and two patients had also Tuckers procedure at the same time. The mean follow up was 1.2 years (range 1 months–2.8 years). Postoperative Holter monitoring showed sinus rhythm in all the patients. The mean CPB time was 83.6 min (range 54–163 min), mean aortic cross-clamp time was 48.0 min (range 22–112 min), mean ICU stay was 1.26 days (range 1–4 days) and length of hospital was 8.3 days (range 5–13 days) Postoperative echocardiography showed less than 1 mmHg gradient across the SVC-RA appendage anastomotic site in all the patients. No mortality was recorded.This is the largest reported series of Warden's procedure in the literature till date. It is known from other studies that sinus node dysfunction and conversion to junctional nodal rhythm were quite a concern with the conventional techniques and Warden's procedure specifically avoids these problems.