Sinus Venosus Atrial Septal Defect Research Articles

Late diagnosis of incomplete Cantrell's syndrome on CT scan

MOTS CLES Syndrome de Cantrell ; Cardiopathie congenitale ; Scanner cardiaque A 45-year-old man with chronic myeloid leukaemia was referred for cardiac evaluation before allogeneic bone-marrow transplantation. His medical history included isolated right ventricle (RV) dilatation discovered 10 years earlier, with episodes of right heart failure induced by severe postchemotherapy anaemia. The electrocardiogram showed first-degree atrioventricular block, complete right bundle branch block and left atrial enlargement (Fig. 1). Chest radiographs showed cardiomegaly and large bowel images in the area anterior to the heart (Fig. 2). Transthoracic echocardiography showed isolated RV enlargement (53mm) with normal ejection fraction. Left ventricle (LV) dimension and ejection fraction were normal. The RV/LV diameter ratio was 1.2. No atrial or ventricular septal defects or pericardial abnormality was seen. Systolic pulmonary pressure estimation was 45mmHg. Pulmonary-to-systemic blood flow ratio (Qp/Qs) was 2.1 with no visible left-to-right shunt. Right heart catheterization showed normal pulmonary artery occlusion pressure (5mmHg), normal mean pulmonary pressure (18mmHg) and no sign of restrictive cardiomyopathy. Oximetric study demonstrated an oxygen saturation of 66% in the superior and inferior vena cava, 84% in the right atrium, 80% in the RV and 77% in the pulmonary artery. Qp/Qs was 1.6. A computed tomography scan showed the association of transdiaphragmatic hernia, partial apical agenesis of the pericardium, and sinus venosus atrial septal defect with abnormal pulmonary venous return (Fig. 3).

Diagnostic accuracy of multidetector spiral computed tomography for preoperative assessment of sinus venosus atrial septal defects in children

To prospectively assess the value of multidetector computed tomography (MDCT) for detecting partial anomalous pulmonary venous return (PAPVR) in children with suspected sinus venosus-atrial septal defect (SV-ASD). Forty-four children (mean age, 7.3years; range, nine months-16years) from whom transthoracic echocardiography (TTE) was inconclusive for the diagnosis underwent MDCT after contrast medium injection. Diagnosis was suspected on TTE by abnormal pulmonary venous return, no visualization of pulmonary venous ostia, or unexpected dilatation of right cavities. The first 11 children also underwent cardiac catheterization. Surgical findings constituted the diagnostic reference standard. Thirty-two (73%) children had SV-ASD with PAPVR. Of the first 11 patients, one had PAPVR by MDCT and 10 by conventional angiography; these 11 patients had PAPVR by surgery. Of the remaining 33 patients, 21 had SV-ASD and 12 had ostium secundum ASD, by both MDCT and surgery. MDCT had 100% sensitivity, 100% specificity, 100% positive predictive value, and 100% negative predictive value for diagnosing PAPVR in patients with suspected SV-ASD. Contrast-enhanced MDCT is a highly accurate, minimally-invasive technique for detecting PAPVR associated with SV-ASD. Contrast-enhanced MDCT may be used safely to replace conventional angiography for the definitive diagnosis and preoperative evaluation of children with suspected SV-ASD.

Sinus venosus atrial septal defect in a 31-year-old female patient: a case for surgical repair

A 31-yr-old female patient previously diagnosed with idiopathic pulmonary arterial hypertension (PAH) was referred to our centre for further evaluation. Cardiac magnetic resonance imaging (MRI) and echocardiography revealed a superior sinus venosus atrial septal defect (ASD) with partial anomalous pulmonary venous drainage. Following re-diagnosis, surgical repair was considered. Despite a disproportionately high mean pulmonary artery pressure (P(pa)) of 47 mmHg relative to the patient's age and defect, the decision to operate was based on the absence of oxygen desaturation (either at rest or during exercise), cyanosis or abnormally elevated haemoglobin. Other operability criteria included normal sinus rhythm at rest, vasoreactivity and a pulmonary to systemic blood flow ratio of 1.9 at rest. Surgical repair and continued advanced therapy with bosentan 125 mg b.i.d. and aspirin 75 mg o.d. proved successful, with post-operative improvements in exercise capacity and dyspnoea, and a reduction in P(pa) to 25 mmHg. Keen to start a family, the risks of pregnancy were discussed. This case illustrates the importance of secondary PAH in sinus venosus ASD and the need to exclude a sinus venosus ASD in unexplained right ventricular dilatation. Access to expertise in congenital heart disease and use of cardiac MRI can improve diagnosis and, in turn, treatment decisions.

Real Time Three-Dimensional Transesophageal Echocardiographic Evaluation of a Sinus Venosus Atrial Septal Defect

We present a case of a 65-year-old man who presented with atrial flutter and dilation of right heart was noted on transthoracic echocardiography. Transesophageal echocardiography revealed a large sinus venosus atrial septal defect close to superior vena cava and anomalous connection of right superior pulmonary vein. Additionally, real time three-dimensional transesophageal echocardiography provided superior spatial details and demonstrated the size, location of the defect and its spatial relationship to the surrounding structures. Patient underwent successful surgical repair.

A New Technique for Interrupted Aortic Arch Repair: The Neville Tube

We have developed a new technique for interrupted aortic arch repair in which the pulmonary artery anterior wall is cut off and tailored so as to re-establish aortic continuity with an autologous tube. We are describing this method herein, with an 8-year follow-up of the first patient.

Imaging Atrial Septal Defects by Real-Time Three-Dimensional Transesophageal Echocardiography: Step-by-Step Approach

There are currently no standardized three-dimensional (3D) transesophageal echocardiographic (TEE) views of the interatrial septum and atrial septal defects (ASDs). Without a standardized approach, it is difficult to ascertain the important anatomic relationships (such as the location of the aortic rim of an ASD), to perform relevant measurements (such as the size of an ASD or the size of its rims), or to guide the deployment of catheters and devices during atrial septal closure. Using a 3D TEE matrix-array transducer, 706 TEE studies were performed over a 14-month period. The purpose of the study was to develop a standardized protocol for anatomically correct orientation of 3D TEE images of the interatrial septum and ASDs. Among 706 TEE studies, there were 23 patients with ASDs, representing 3.3% of the study population. Eighteen patients had secundum ASDs, two had primum ASDs, and three had sinus venosus ASDs of the superior vena cava type. A protocol for properly orienting 3D TEE images of the interatrial septum and ASDs was developed. When the images are acquired at an angle of 0°, the septum is properly oriented by the tilt-up-then-left maneuver. The initial 3D TEE image in first tilted up to reveal the right atrial side of the septum. Then the image is tilted 180° around its vertical axis to reveal the left atrial side of the septum; the aortic rim is on the left, the superior vena cava on the top, and the right-sided pulmonary vein ostia on the right side of the screen. For acquisitions at a higher angle, the rotate-left-in-z-axis maneuver is used. The image is first tilted up to reveal the right atrial side of the septum, as in the tilt-up-then-left maneuver. The image is then rotated counterclockwise in the z axis until the superior vena cave is at 12 o'clock. Finally, the image is tilted 180° around its vertical axis to reveal the left atrial side of the septum. The use of standardized tilt-up-then-left and rotate-left-in-z-axis maneuvers enhances the diagnosis of ASDs, ascertains the important anatomic relationships of ASDs to surrounding structures, and facilitates communication between echocardiographers obtaining 3D TEE images and interventional cardiologists or cardiac surgeons performing ASD closures.

MRI for detection of anomalous pulmonary venous drainage in patients with sinus venosus atrial septal defects

Purpose of this survey was to estimate the value of MRI for the assessment of the anatomical and functional features of sinus venosus atrial septal defect (SVD). This prospective study included 13 surgically proven cases of SVD out of 81 subjects submitted to MRI due to inconclusive transthoracic echocardiography (TTE) or suspicion of high intracardiac and/or extracardiac shunt volumes based on echocardiographic findings. MRI examination included cine SSFP sequences, contrast-enhanced 3D gradient-echo (GE) sequences for MR angiography (MRA) and phase-contrast flow-measurements. MRI revealed nine patients with a superior and four with an inferior SVD. Anomalous pulmonary venous drainage (APVD) was observed only in subjects with a superior SVD, and it was right-sided in all cases. All MRI and MRA results for the SVD patients were confirmed intraoperatively. The Correlation coefficient between MR flow measurements and cardiac catheterisation was 0.94 (P<0.0001). According to MRI the rest of the subjects (n=68) presented a secundum ASD, whereas in 18% an APVD coexisted. The latter MR outcomes concurred with the cardiac catheterisation (n=56) and operative (n=12) results. MRI provides a reliable, non-invasive method for evaluation of SVDs, APVDs and shunt quantification.

Sinus Venosus Atrial Septal Defect in Adults: Utility of Cardiovascular MRI in resolving this Diagnostic Dilemma

Sinus venosus atrial septal defect (SV-ASD) can be a challenging diagnostic problem. It can present with non-specific signs and symptoms and, on imaging, can mimic other causes of right heart dilatation. Transthoracic echocardiography using standard scan planes may be limited, due to the defect and any associated anomalous pulmonary venous drainage lying outside the confines of the fossa ovalis. At our institution, we use cardiovascular magnetic resonance imaging (CMRI) as a problem-solving tool in these patients. In this article, we discuss the utility of CMRI in establishing the diagnosis by presenting our recent experience and illustrating it with sample cases. We wish to highlight the problems relating to making a diagnosis of SV-ASD in adult patients, and how CMRI has contributed to overcoming these in our practice.

Sinus venosus atrial septal defect: the importance of multimodality approach

A multidisciplinary approach to congenital heart diseases in adults is a primary element for modern medicine. The diagnosis of a sinus venosus atrial septal defect is a typical example of disease that requires the different skills of the cardiologist, cardiac surgeon and radiologist.

Long-term Noninvasive Arrhythmia Assessment after Surgical Repair of Sinus Venosus Atrial Septal Defect

The sinus venosus atrial septal defect (SVASD) has been successfully repaired with low risk for many years. Postoperative arrhythmias have not been comprehensively described. We sought to define the prevalence of late arrhythmias after surgical closure of the SVASD. We performed a cross-sectional noninvasive arrhythmia evaluation of patients > or =6 years of age, who had surgical repair of an isolated SVASD at a single institution from 1987 to 2004, to assess for sinus node dysfunction (SND), atrioventricular (AV) block, and atrial and ventricular arrhythmias. Evaluation included an electrocardiogram, echocardiogram, 24-hour Holter monitor, and exercise stress test. Thirty subjects were evaluated 10.1 +/- 4.7 years (range 3.3-20.4 years) after operative repair. Mean age at surgical repair was 6.4 +/- 8.1 years (range 1.3-45.7 years). Anatomic subtype was either superior vena cava- (SVC) (n = 24) or inferior vena cava- (IVC) type (n = 6). Type of surgical repair included: single patch (n = 13), double patch (n = 6), and Warden (n = 5) for SVC-type. All IVC-type had single patch repair. Overall, 50% (n = 15) of patients showed some evidence of SND, which is significantly associated with a longer duration of follow-up (P= .03). High-grade AV block and complex tachyarrhythmias were not seen. We conclude that postoperative SVASD patients have a significant prevalence of SND, which may increase with duration of follow-up. These patients warrant ongoing clinical surveillance for the potential development of signs and symptoms of SND.

Role of intraoperative echocardiography in surgical correction of the superior sinus venosus atrial septal defect

Superior type of sinus venosus atrial septal defect (SVASD) is invariably associated with the unroofing of right upper pulmonary vein (RUPV). Warden procedure and pericardial patch repair with rerouting of the RUPV are commonly performed operations for the superior SVASD. Both operations involve the risk of obstruction to the flow of superior vena cava or rerouted pulmonary vein in the postoperative period. The sinus venosus defects are well visualized on the transesophageal echocardiography (TEE) because of the proximity of the TEE probe to these structures. We are reporting two cases operated for the superior SVASD with unroofed RUPV, highlighting the intraoperative echocardiographic features before and after the surgery.

Cardiac Herniation After Minimally Invasive Cardiac Surgery

A 37-year-old woman was referred to our institution for minimally invasive surgical repair of a partial anomalous pulmonary venous connection of the right upper pulmonary vein into the superior vena cava and a sinus venosus atrial septal defect. Diagnosis was refined on admission. The patient received routine preparation in the operating room by oral intubation, right radial arterial line, and a left jugular central venous pressure catheter. Peripheral superior vena cava cannulation (Medtronic Inc., Minneapolis, Minn) was used to reduce the length of the thoracotomy. Through a right posterolateral thoracotomy, cardiopulmonary bypass was performed, and the aorta was …

Diagnosis of Inferior Sinus Venosus Atrial Septal Defects Using Transthoracic Three-Dimensional Echocardiography

The authors report the cases of 2 patients with symptoms and signs related to severe pulmonary hypertension. Two-dimensional echocardiography demonstrated defects in the posterior portion of the atrial septum. Transthoracic three-dimensional echocardiography clearly showed inferior sinus venosus atrial septal defects and their relationships with the inferior vena cava, the pulmonary vein, and the muscular border of the fossa ovalis. These 2 cases not only elucidate the potential value of transthoracic three-dimensional echocardiography in delineating the structural characteristics of unusual interatrial shunting but also remind clinicians to be aware of this potentially treatable cardiac defect during workup for pulmonary hypertension.

MDCT of Partial Anomalous Pulmonary Venous Return (PAPVR) in Adults

The purpose of this study was to determine the lobar distribution and associated radiologic/clinical findings of partial anomalous pulmonary venous return (PAPVR) in the adult population using multidetector computed tomography (MDCT). The radiology information database was queried for patients with PAPVR diagnosed on chest computed tomography. Forty-seven cases of PAPVR were retrospectively identified from 45,538 contrast-enhanced chest computed tomography examinations performed over an 8-year period. Diagnostic findings were confirmed via consensus review by 2 cardiothoracic radiologists. Medical charts were evaluated for ancillary radiographic data, cardiopulmonary signs and symptoms, and subsequent surgical interventions. Calculated disease prevalence was 0.1%, with mean patient age of 58 years and a 58% female predominance. PAPVR was observed with 47% frequency in the left upper lobe, 38% right upper lobe (RUL), 13% right lower lobe, and 2% left lower lobe. Among cases of RUL PAPVR, 42% were associated with sinus venosus atrial septal defect (ASD). Other reported anomalies were right-sided volume overload (47%), isolated upper lobe PAPVR (29% left and 5% right), bilateral PAPVR (4%), scimitar syndrome (13%), persistent left superior vena cava (9%), and azygos continuation of the inferior vena cava (4%). Reported cardiopulmonary signs/symptoms and imaging modalities other than MDCT were neither sensitive nor specific for PAPVR. Surgical repair was performed in 21% of cases and included ASD patching, intracardiac baffle, anomalous vein anastomosis, systemic vein translocation, and Warden procedure. This represents the largest and only consecutive retrospective study of PAPVR in adults to date. Left upper lobe PAPVR was the most frequent location detected on MDCT, whereas RUL PAPVR was slightly less common and moderately associated with sinus venosus ASD. Utilization of contrast-enhanced studies and MDCT technology has enabled improved detection and characterization of PAPVR for early diagnosis and/or intervention.

Sinus venosus atrial septal defect and partial anomalous pulmonary venous connection with secondary hepatic cirrhosis and portal hypertension

A 60-year-old woman was admitted to the emergency department with a three-month history of exertional dyspnoea (NYHA class III) and reduced physical capacity. She had a short history of cryptogenic...

Constrictive pericarditis in a patient with sinus venosus atrial septal defect and anomalous right upper pulmonary venous return

This is a report of a 49-year-old male, who presented with typical signs and symptoms of constrictive pericarditis. He was diagnosed with sinus venosus atrial septal defect (ASD) and anomalous right upper pulmonary venous return during his adolescence, which was elected not to be repaired. During the attempted repair of the ASD it was noted there was a thick fibrous material covering the heart, which had progressed over time leading to frank constrictive pericarditis. His ASD spontaneously closed over time. There have been less than 10 cases reported with constrictive pericarditis of nonsurgical etiology in a patient with ASD, and none with sinus venosus ASD.

Atrial septal defect combined with partial anomalous pulmonary venous return: complete anatomic and functional characterization by cardiac magnetic resonance

The presented case regards a 17-year-old male with new-onset right bundle branch block and significantly enlarged right-heart sections as the only pathologic finding on transthoracic echocardiography. Cardiac magnetic resonance (CMR) revealed the presence of a superior sinus venosus atrial septal defect associated with a partial anomalous pulmonary venous return, with the right upper lobe pulmonary vein draining into the superior vena cava. CMR has developed in recent years into an accurate modality for non-invasive evaluation of patients with congenital heart disease, especially through improvements in quality and speed of image acquisition. With echocardiography, sinus venosus defects and anomalous pulmonary vein drainage may be more easily detected by a transoesophageal approach because of the proximity of the transducer to the atrial septum. CMR may be specifically recommended as an alternative to transoesophageal echocardiography in any patient with an unexplained dilatation of the right ventricle.

Inferior Type Sinus Venosus Atrial Septal Defect

Atrial septal defects can occur at various levels of the interatrial septum. Sinus venosus atrial septal defect (SVASD) results from abnormal resorption of the embryologic sinus venosus, and may be of the superior or inferior type. In this paper, we describe a 46-year-old man with inferior-type SVASD who presented with arrhythmias and dyspnea. Cardiac magnetic resonance and cardiac catheterization were useful in evaluating the anatomic/functional characteristics of inferior-type SVASD and distinguishing it from unroofed coronary sinus.

Misleading Turbulent Flow Through Pulmonary Venous Anastomoses During Lung Transplantation

Misleading Turbulent Flow Through Pulmonary Venous Anastomoses During Lung Transplantation

Atypical atrial septal defects in children: noninvasive evaluation by cardiac MRI

Atypical left-to-right shunts at the level of the atrium in children such as sinus venosus atrial septal defects (ASDs) and partial anomalous pulmonary venous return (PAPVR) may be difficult to assess by transthoracic or transoesophageal echocardiography. Free-breathing cardiac MRI may be a powerful alternative. To assess the value of free-breathing cardiac MRI in the delineation of atypical ASDs in children. A total of 82 children (mean age 5.9 years, range 1.1-15.7 years) with suspected ASD and inconclusive transthoracic echocardiography underwent cardiac MRI under free-breathing, mostly sedated conditions. Phase-contrast MRI was used for defect visualization and shunt quantification, and multiphase inflow MR angiography for delineation of pulmonary/systemic venous connections. Of the 82 patients, 34 (41%) were diagnosed with atypical shunt lesions at the level of the atrium and 48 (59%) with simple secundum ASDs. No false-negative or false-positive findings were reported by MRI compared to cardiac catheterization and intraoperative findings. Superior sinus venosus ASD with partial anomalous PAPVR was present in 10 of the 82 children (12.2%), whereas 2 (2.4%) had a large posterior-inferior defect, 5 (6.1%) had isolated PAPVR, and 17 (20.7%) had multiple ASDs and/or associated vascular anomalies. Q(p)/Q(s) by phase-contrast MRI agreed well with oximetry values (mean difference 3%, limits of agreement +/-21-25%; Bland/Altman analysis). Free-breathing cardiac MRI under sedation allows reliable identification of atypical left-to-right shunt defects at the level of the atrium in children in whom transcatheter ASD closure is unsuitable, including delineation of pulmonary or systemic venous anomalies and shunt quantification.