Sinus Venosus Atrial Septal Defect Research Articles

Caval Division Technique for Sinus Venosus Atrial Septal Defect With Partial Anomalous Pulmonary Venous Connection

Repair of sinus venosus atrial septal defect (ASD) with high partial anomalous pulmonary venous connection (PAPVC) using an internal patch may be complicated by obstruction of the superior vena cava (SVC) or pulmonary veins, or both, and sinus node dysfunction. In cases in which the anomalous veins insert more than 2 cm above the cavoatrial junction, we have adopted the technique of caval division in which the SVC is divided and the proximal end is anastomosed to the right atrial appendage, and the distal SVC serves as a conduit for pulmonary venous drainage to the left atrium through the ASD. We retrospectively compare the results of the internal patch repair versus the Warden technique. Between 1991 and 2004, 54 patients diagnosed with sinus venosus ASD and PAPVC have undergone repair at our institution. Mean age was 13.4 years (range, 1.5 to 58). Thirteen patients (24%) had high insertion of anomalous veins and underwent the Warden technique. Follow-up averages 4.3 years (range, 1 to 13). There were no early or late deaths. All patients remain in normal sinus rhythm. Twelve of the 13 patients with Warden procedure have had postoperative echocardiograms, and 11 of these patients showed no evidence of SVC or pulmonary venous obstruction. In 1 patient, symptomatic pulmonary venous obstruction developed and required revision of a contracted intra-atrial pericardial baffle. Caval division for treatment of high PAPVC appears to be safe and is associated with low morbidity and mortality. The Warden procedure is an effective surgical option for patients undergoing correction of high PAPVC.

Hypertensive Pulmonary Vascular Disease in Adults with Secundum or Sinus Venosus Atrial Septal Defect

The development of hypertensive pulmonary vascular disease (HPVD) is considered a risk factor in the long-term course of patients with secundum atrial septal defects (ASD). The aim of this study was to assess the prevalence and histologic degree of HPVD and pulmonary hypertension in relation to preoperative clinical and hemodynamic data, intraoperative findings, and operative outcome in adults. Lung biopsies of 75 patients, mean age 44 +/- 14 years (18-71 years), with secundum ASD or sinus venosus defect including ten patients with partial anomalous pulmonary venous return were analyzed in accordance with preoperative and intraoperative findings as well as operative outcome. Lung biopsy was performed at the time of defect closure and was classified according to Heath and Edwards. Structural changes of the pulmonary vasculature were found in 59% of patients; grade 3 and higher changes were present in 19%. There were no statistically significant relations between histologic findings and preoperative clinical and hemodynamic data, intraoperative findings, and operative outcome. The prevalence of moderate (32-50 mm Hg) and severe (> 50 mm Hg) systolic pulmonary hypertension was 27% and 17%, respectively. Increased systolic pulmonary arterial pressure was associated with increased pulmonary vascular resistance (p < 0.000) and patients' age (p = 0.001). Patients with a lower functional capacity had a higher prevalence of pulmonary hypertension (p = 0.011). The prevalence of HPVD and pulmonary hypertension in adult patients with secundum ASD or sinus venosus defect is considerable. Preoperative hemodynamic data do not predict the degree of HPVD in lung biopsy. Closure is generally advised to prevent increasing pulmonary arterial pressure and decreasing functional capacity over time.

Sinus Venosus Atrial Septal Defect

Sinus venosus atrial septal defect (SVASD) differs from secundum atrial septal defect by its atrial septal location and its association with anomalous pulmonary venous connection (APVC). Data on long-term outcome after surgical repair are limited. We reviewed outcomes of 115 patients (mean age+/-SD 34+/-23 years) with SVASD who had repair from 1972 through 1996. APVC was present in 112 patients (97%). Early mortality was 0.9%. Complete follow-up was obtained for 108 patients (95%) at 144+/-99 months. Symptomatic improvement was noted in 83 patients (77%), and deterioration was noted in 17 patients (16%). At follow-up, 7 (6%) of 108 patients had sinus node dysfunction, a permanent pacemaker, or both, and 15 (14%) of 108 patients had atrial fibrillation. Older age at repair was predictive of postoperative atrial fibrillation (P=0.033). No reoperations were required during follow-up. Survival was not different from expected for an age- and sex-matched population. Clinical improvement was more common with older age at surgery (P=0.014). Older age at repair (P=0.008) and preoperative New York Heart Association class III or IV (P=0.038) were independent predictors of late mortality. Operation for SVASD is associated with low morbidity and mortality, and postoperative subjective clinical improvement occurs irrespective of age at surgery. Postoperative atrial fibrillation appears to be related to older age at operation. SVASD repair achieves survival similar to that of a matched population and should be considered whenever repair may impact survival or symptoms.

Implantation of a Biatrial Pacemaker in a Patient with Sinus Venosus Atrial Septal Defect and Persistent Left Superior Vena Cava

Journal of Cardiovascular ElectrophysiologyVolume 17, Issue 1 p. 104-104 Implantation of a Biatrial Pacemaker in a Patient with Sinus Venosus Atrial Septal Defect and Persistent Left Superior Vena Cava NGAI-YIN CHAN M.D., NGAI-YIN CHAN M.D. Princess Margaret Hospital, Hong KongSearch for more papers by this authorCHUN LEUNG LAU M.D., CHUN LEUNG LAU M.D. Princess Margaret Hospital, Hong KongSearch for more papers by this author NGAI-YIN CHAN M.D., NGAI-YIN CHAN M.D. Princess Margaret Hospital, Hong KongSearch for more papers by this authorCHUN LEUNG LAU M.D., CHUN LEUNG LAU M.D. Princess Margaret Hospital, Hong KongSearch for more papers by this author First published: 13 January 2006 https://doi.org/10.1111/j.1540-8167.2005.00264.x Ngai Yin Chan, M.D., Princess Margaret Hospital, 2-10 Princess Margaret Hospital Road, Lai Chi Kok, Kowloon, Hong Kong. Fax: (852) 29903329; E-mail: ngaiyinchan@yahoo.com.hk J Cardiovasc Electrophysiol, Vol. 17, p. 1, January 2006. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume17, Issue1January 2006Pages 104-104 RelatedInformation

Intrapericardial Bronchogenic Cyst Overriding the Pulmonary Artery

We describe a case of a 3-year-old child with an intrapericardial bronchogenic cyst arising from the pericardium overlying the pulmonary artery found incidentally at the time of elective repair of a sinus venosus atrial septal defect with partial anomalous pulmonary venous return. A cystic mass overriding the right pulmonary artery was resected and diagnostic studies revealed multiple loculated cysts lined by respiratory mucosa with cartilage and smooth muscle in the wall consistent with a bronchogenic cyst. The cyst was completely resected and postoperative recovery was uneventful.

Spontaneous Left Atrial Dissection Presenting as Pulmonary Edema

Ahealthy 30-year-old man who had not had any prior medical or surgical history presented to the emergency department with substernal pain for 2 days that was sharp and exacerbated by inspiration and recumbency. Chest radiograph (Figure 1A) showed mild left atrial enlargement. The ECG showed normal findings. The next day, the patient complained of sudden onset of class 3 dyspnea and frothy blood-tinged sputum. Follow-up …

Síndrome de Wolff-Parkinson-White associada a comunicação interatrial tipo seio venoso

The Wolff-Parkinson-White syndrome (WPW) and sinus venosus atrial septal defect (ASD) association is very rare and not yet reported in the literature. It is the main basis for this case report.

Recombinant factor VIIa to control excessive bleeding following surgery for congenital heart disease in pediatric patients.

The purpose of this article is to evaluate the efficacy of recombinant factor VII (rFVIIa) in the treatment of bleeding following cardiac surgery in a pediatric population. The study included a case series of postcardiac surgical patients with chest tube output of = 4 mL/kg/h for the initial 3 postoperative hours who received rFVIIa. Chest tube output for the 3 hours before and the 3 hours after rFVIIa was compared using a paired t test. In addition, chest tube output for the initial 3 postoperative hours and the 3 hours following rFVIIa was compared to 8 control patients who did not require rFVIIa. Recombinant factor VIIa was administered to 9 children (age = 9 +/- 4 years) following repair of tetralogy of Fallot (6), closure of ventricular septal defect (1), closure of sinus venosus atrial septal defect (1), and mitral valve repair (1). Chest tube output for the initial 3 postoperative hours prior to the administration of rFVIIa was 5.8 +/- 2.8 mL/kg/h and decreased to 2.0 +/- 1.3 mL/kg/h for the 3 hours following the administration of rFVIIa (P =.002). In the patients that did not receive rFVIIa, chest tube output for the first 3 postoperative hours was 1.6 +/- 0.9 mL/kg/h and 1.2 +/- 0.6 mL/kg/h for the next 3 hours (P = nonsignificant when compared to chest tube output for the 3 hours following rFVIIa in patients who received rFVIIa). No adverse effects were noted. Recombinant factor VIIa decreased chest tubing bleeding following cardiac surgery in children. Given its potential therapeutic impact, rFVIIa warrants further investigation in the pediatric cardiac population.

Modified cavoatriotomy for combined PAPVC repair and maze procedure

In patients with partial anomalous pulmonary venous connection (PAPVC) to the superior cavoatrial junction, the standard right-sided left atriotomy does not allow sufficient access to the mitral valve and the left atrium. And the injury and traction of the sinus node and sinus node artery should be avoided for prevention of the cardiac rhythm disturbance after operation. We herein report a useful approach to repair the sinus venosus atrial septal defect with PAPVC of the right pulmonary veins to the superior cavoatrial junction in patients also requiring mitral valve replacement and the maze procedure.

Noncontact and Electroanatomic Mapping of Atrial Flutter in Surgically Repaired Sinus Venosus Atrial Septal Defect and Rerouting of Anomalous Pulmonary Venous Drainage

Atypical atrial flutter with two prior failed ablations, complicating surgically repaired sinus venosus atrial septal defect and partial anomalous pulmonary venous connection, mapped by noncontact and electroanatomic mapping, is described. Electroanatomic and noncontact mapping clearly identified a narrow zone of normal voltage and activation which was targeted, with successful termination of the arrhythmia.

P3 Isolated right heart failure as a manifestation of a rare combination of sinus venosus atrial septal defect and Ebstein's anomaly

P3 Isolated right heart failure as a manifestation of a rare combination of sinus venosus atrial septal defect and Ebstein's anomaly

Secundum ASD closure using a right lateral minithoracotomy: Five-Year experience in 122 patients

Background Surgical closure of secundum atrial septal defect (ASD) is a standard procedure associated with very low mortality and morbidity. We evaluated outcomes in the era of catheter-based interventional closure and minimally invasive techniques. Methods From May 1996, February 2002, 177 patients with a body weight of more than 30 kg underwent surgical ASD closure. A right lateral minithoracotomy (LMT) was used in 122 patients and a conventional approach, in 55. Diagnoses included secundum ASD in 106 patients in the LMT group and 40 in the conventional group, sinus venosus ASD in 13 patients in each group, and status post interventional closure in 3 and 2 patients, respectively. Mean age was 37 ± 17 years in the LMT group and 43 ± 20 years, in the conventional group and mean body weight was 66 ± 17 kg and 70 ± 16 kg, respectively. In the LMT group, femoral cannulation was performed for cardiopulmonary bypass. Results Direct ASD closure was carried out in 67.2% of patients in the LMT group and 58.2% of those in the conventional group. The remaining patients had pericardial patch closure. There was one death: A patient in the conventional group who required explantation of an Amplatzer device because of infection died postoperatively. Average stay in the intensive care unit was 1.2 ± 0.5 days. Two patients required reoperation for residual ASD after direct closure; 1 sustained a temporary neurological deficit that resolved completely. On postoperative echocardiography, a minimal residual shunt was seen in only 3 patients. All patients were in good clinical condition with improved functional status at discharge from the hospital. Conclusions Secundum ASD closure by LMT has become as standard and safe an operation as the conventional technique and achieves good perioperative results and satisfactory long-term outcomes. Thus LMT is an attractive option for patients who are not suitable for closure using catheter-based devices.

Outcome following repair of sinus venosus atrial septal defects in children.

The outcome of surgical repair of sinus venosus atrial septal defect was reviewed retrospectively. The operation was performed on 44 children aged 8 to 163 months, between April 1985 and November 1998. Median cardiopulmonary bypass and aortic crossclamp times were 58 minutes (range, 29 to 141 minutes) and 29 minutes (range, 4 to 67 minutes), respectively. Use of blood products decreased from 4.5 units per patient in the first period (1985 to 1989) to 0.6 units in the last period (1995 to 1998). Median intensive care and hospital stays were 2 days (range, 1 to 12 days) and 6 days (range, 4 to 16 days), respectively. There was 1 early death (2.3%). Complications included reexploration for bleeding in 2 patients (4.5%) and for superior vena cava obstruction in 1 (2.3%), and arrhythmias in 3 (6.8%), which required a pacemaker in one. During follow-up of 15 to 176 months, 83.8% of patients were in sinus rhythm. One required angioplasty for superior vena cava stenosis, hemodynamically insignificant residual shunt was found in 3, and mild superior vena cava stenosis in 3. Repair of sinus venosus atrial septal defect carries a low mortality and morbidity, but long-term follow-up is needed to monitor potential sinus node dysfunction and superior vena cava stenosis.

Residual sinus venosus atrial septal defect after surgical closure of atrial septal defect

We describe residual atrial septal defects in 3 patients who had previous surgical repair. The residual defects were the sinus venosus type near the orifice of the inferior vena cava. Preoperative and intraoperative transesophageal echocardiography may aid in the detection and facilitate the successful repair of these defects. (J Am Soc Echocardiogr 2001;14:738-41.)

The Correction of Congenital Heart Defects with Less Invasive Approaches

The minimally and less invasive approaches for the surgical treatment of different heart diseases are rapidly increasing because of their cosmetic and recovery-related advantages. Presented here are the results of less invasive surgery in 51 patients with congenital heart defects. From June 1996 to January 1999, we used less invasive techniques for the correction of congenital heart defects in 51 patients. In 32 patients, we performed right anterolateral thoracotomy (6-13 cm), and on the other 19 patients, we used the partial inferior sternotomy (4-7 cm). The ascending aorta and the caval veins were cannulated in all patients. The following congenital heart defects were corrected: ostium secundum atrial septal defect (n = 35), sinus venosus atrial septal defect with partial anomalous pulmonary venous connection (n = 7), ventricular septal defect (n = 7), tetralogy of Fallot (n = 1), and cor triatriatum sinistrum (n = 1). The average age of the patients was 15 years old ranging from 2 months to 48 years and the average weight 39.6 kg (range 3.8-86 kg). The patients were removed from artificial respiratory support on average 8 hours (range 1-48 hours) after surgery and left the hospital after 7 days (range 2-10 days). In 16 patients, blood transfusions were required, an average 5.7 ml/kg BW (range 1.45-19.75 ml/kg BW). The postoperative course was uneventful in all patients. Follow-up (range 3-33 months, mean 17.5 months) was complete with no late deaths or residual defects. The right anterolateral thoracotomy and the partial inferior sternotomy provide a safe approach for the correction of certain congenital heart defects. These techniques enable operative correction without any additional risks being incurred and can be performed with standard instruments and cannulation. Additional approaches for extracorporeal circulation are unnecessary.

Superior vena cava approach to repair of sinus venosus atrial septal defect

Superior vena cava approach to repair of sinus venosus atrial septal defect

Transesophageal Three-Dimensional Echo Assessment of Sinus Venosus Atrial Septal Defect.

The incremental value of three-dimensional echocardiography over transesophageal multiplane two-dimensional imaging in the assessment of sinus venosus atrial septal defect is demonstrated in the present study.

Intraoperative transesophageal echocardiography for pediatric patients with congenital heart disease.

Intraoperative transesophageal echocardiography for pediatric patients with congenital heart disease.

Congenital cardiac anomalies with vein of Galen malformations in infants

Published reports and personal experience are reviewed relating to patients under 1 year of age diagnosed with a vein of Galen malformation and congenital heart disease. Including five patients from...

Sinus Venosus Atrial Septal Defect?

Sinus Venosus Atrial Septal Defect?