Supracardiac Total Anomalous Pulmonary Venous Connection Research Articles

Supracardiac total anomalous pulmonary venous connection with cor triatriatum sinister: A rare diagnosis confirmed by saline contrast echocardiography.

In cor triatriatum sinister, the left atrium is divided by a membrane into a proximal and a distal chamber. Usually proximal chamber receives all the pulmonary veins and drains through an opening in the dividing membrane into distal chamber, which empties into left ventricle through the mitral valve. Rarely, the two chambers lack a communication and there is associated total anomalous pulmonary venous connection (TAPVC). We report a 1-month-old infant with cyanosis and heart failure, who had cor triatriatum sinister associated with supracardiac TAPVC. The case is reported for rarity of the association with a focus on contrast echocardiographic imaging.

Mid-term results of modified L-shaped incision technique for supracardiac total anomalous pulmonary venous connection.

Surgical outcomes of supracardiac total anomalous pulmonary venous connection (TAPVC) repair by the posterior technique (PT) remain unsatisfactory. This study aimed to compare the outcomes of the modified L-shaped incision technique with the PT for supracardiac TAPVC repair. From January 2009 to December 2019, 121 consecutive patients with supracardiac TAPVC undergoing surgical repair in our institution were included (L-group, n = 53; PT group, n = 68). A propensity score-matched analysis was performed. Patients with single-ventricle physiology or atrial isomerism were excluded. All clinical data were retrospectively analysed. In the unmatched cohort, the median follow-up duration was 33 months (interquartile range 26-65 months). There were 5 operative mortalities (4.1%) and 12 late mortalities (9.9%). Postoperative pulmonary venous obstruction (PVO) was documented in 21 patients. After matching (52 pairs), the overall survival rate in the L-group was 88.2% at both 3 and 5 years. For the propensity score-matched patients with preoperative PVO (n = 20), statistically significant differences (P = 0.002) were found by Kaplan-Meier curves with freedom from death and postoperative PVO at 1 and 3 years of 100% and 85.7% [standard deviation (SD): 13.2%] in the L-group and 90% (SD: 9.5%) and 22.9% (SD: 14.1%) in the PT group, respectively. Multivariable analysis revealed that the use of the PT was an independent risk factor for death and postoperative PVO (hazard ratio 4.12, 95% confidence interval 1.12-15.16; P = 0.03). The modified L-shaped incision technique provided an acceptable outcome for supracardiac TAPVC repair. Compared with PT, the modified L-shaped incision technique was significantly associated with decreased death and postoperative PVO in patients with obstructed supracardiac TAPVC.

Epiural anaesthesia: Superior modality of treatment in lower segment caesarean section in a patient with supracardiac TAPVC

Background: Total Anomalous Pulmonary Venous Connection (TAPVC) is congenital heart disease in which the pulmonary veins do not connect normally to left atrium. Instead they connect to right atrium, often by way of systemic vein. There may be associated cardiac malposition. Anaesthetic management requires detailed assessment of cardiac an extracardiac anomalies & its pathophysiological effects. Here we report a case of TAPVC posted for LSCS and its successful management. Case Presentation: 25yrs female known case of TAPVC at 34.5 wks of gestation admitted 3 weeks prior, Vitaly stable, we did this patient under plain epidural anaesthesia without haemodynamic instability. Conclusions: Epidural anaesthesia a better technique than general anaesthesia for supracardiac TAPVC posted for LSCS. Meticulous intraoperative monitoring is important. Cardiac Output must be maintained, avoid fall in Systemic Vascular Resistance (SVR) ensuring minimal change in shunt. Keywords: Supracardiac TAPVC, LSCS, Epidural Anaesthesia.

Numerical Simulation of Hemodynamics in Two Models for Total Anomalous Pulmonary Venous Connection Surgery.

ObjectiveTo numerically compare the prospective hemodynamic outcomes between a new window surgery and a traditional surgery in the treatment of supracardiac total anomalous pulmonary venous connection (S-TAPVC).MethodsA 3D geometry model, composed of pulmonary vein (PV) and left atrium (LA), was reconstructed based on summarized data with S-TAPVC. Two surgery models were established based on this model. One is the traditional surgery model, where an elliptical anastomosis was created by incising and stitching the LA and the common vein (CV) along the axis of the CV. The other is the new window surgery model, where the CV was incised with an H-shaped orifice, and LA was incised with a transposed H-shaped orifice, and then the orifice edges were stitched like a window. Two models with a relative cross sectional area (RCSA) of 300 mm2/m2 and 500 mm2/m2 were established, which correspond to traditional surgery and window surgery. Numerical simulation of hemodynamics was carried out. The velocity, left atrium and pulmonary vein pressure, the pressure difference of anastomosis and the energy conversion efficiency were analyzed to evaluate the prospective hemodynamic outcomes of these two operations.ResultsWindow surgery presented a lower blood flow velocity, pressure difference, and the WSS at the anastomosis, compared to traditional surgery. In terms of energy loss, the power conversion efficiency of window surgery was significantly higher than that of traditional surgery, with 66.8% and 53.5%, respectively.ConclusionThe new window surgery demonstrates a lower pressure difference of anastomosis and higher energy conversion efficiency, which may be a better choice compared with the traditional surgery for S-TAPVC patient.

Supra-Cardiac Total Anomalous Pulmonary Venous Connection via Rare Right-Sided Vertical Vein

A Total Anomalous Pulmonary Venous Connection (TAPVC) is an uncommon congenital anomaly which occurs as result of defective development or early atresia of common pulmonary vein. TAPVC is categorized into four with supracardiac variety occurring most frequently. Common pulmonary vein anomaly results in the retention of cardinal or umbilico-vitelline drainage systems. If cardinal venous system persists, it provides channels to the innominate or azygous vein, Superior Vena Cava (SVC). This case series reports two cases of supracardiac TAPVC draining into SVC via right-sided vertical vein. Careful evaluation before surgical repair is vital for such complex cardiac anomalies.

Supracardiac Total Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which there is developmental absence of connection of all four pulmonary veins with the left atrium. To report a rare case and share our experience in surgery and post-operative management for supracardiac TAPVC. Patient with supracardiac TAPVC with atrial septal defect (ASD) secundum variety with rudimentary patent ductus arteriosus (PDA) underwent rechanneling of pulmonary veins to left atrium (LA) with gluteryldehye treated autologous pericardial patch closure of ASD with ligation of ascending vertical vein and ligation of rudimentary PDA.Post operatively there were no events of pulmonary hypertensive crisis, low cardiac output syndrome, right heart failure or conduction defect were observed and echocardiogram showed adequate pulmonary venous drainage with no residual shunt across the interatrial septum. Marked development in surgical results of TAPVC has been observed in recent years with declining mortality rate from 65% in early sixties to 5% in current surgical scenerio.
 KYAMC Journal Vol. 10, No.-2, July 2019, Page 118-121

Late diagnosis of total anomalous connection of pulmonary veins during the puerperium

The total anomalous pulmonary venous connection (TAPVC) is a rare congenital anomaly. We present a case of a 36-year-old woman, in the postpartum period during the physical examination there was a heart murmur and beating in hands and feet. Cardiac magnetic resonance reported supracardiac TAPVC and large atrial septal defect. The survival of these patients is very uncommon in adults and even worse during pregnancy.

Vertical vein aneurysm in supracardiac total anomalous pulmonary venous connection.

Aneurysmal dilatation of the vertical vein in a case of supracardiac total anomalous pulmonary venous connection (TAPVC) is an extremely rare condition. It has been reported to occur secondary to severe compression of the vertical vein when it traverses between the left bronchus and the left pulmonary artery. We present a 14-year-old male with supracardiac TAPVC with a vertical vein aneurysm, probably secondary to stenosis caused by a thin membranous web just proximal to the aneurysm.

Total Anomalous Pulmonary Venous Connection in Children

Abstract Introduction: The aim of this study was to study the anatomical types of total anomalous pulmonary venous connection (TAPVC), the associated cardiac and extracardiac congenital malformations, clinical manifestations, and postoperative evolution. Materials and methods: Twenty-four patients with a mean age of 125 days, admitted to the Clinic of Pediatric Cardiology III between January 1, 2009 and December 31, 2015 and diagnosed with TAPVC were included in the study. The patients were evaluated clinically, electrocardiographically and echocardiographically, both pre- and postoperatively. Postoperative evolution was monitored at 1, 3, 6, 12 and 24 months. Results: The anatomical types of TAPVC were: supracardiac in 50% of cases (12 patients), cardiac in 37.5% (9 patients) and mixed type in 12.5% of cases (3 patients). The first clinical manifestation was cyanosis in 72.2% of cases. Surgical correction was performed at a mean age of 37 days in obstructive forms, and 254 days in non-obstructive forms. From the study lot, 8.4% of patients had associated extracardiac malformations (anorectal agenesis and Ivemark syndrome). Early postoperative complications included pulmonary hypertension crisis (60% of cases), supraventricular arrhythmias (35% of cases) and chylothorax (8.4% of cases). Late postoperative complications included: reintervention in 8% of patients with mixed type TAPVC. Conclusions: The most frequently encountered type was supracardiac TAPVC, which had a favorable postoperative evolution. Mixed type TAPVC had the highest rate of reintervention.

Total anomalous pulmonary venous connection in a 9-year-old girl at Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease in which all the four pulmonary veins drain into the right atrium instead of the left. Without surgical intervention, 80% of the patients die before the age of 1 year. We report a 9-year-old girl with unrepaired supracardiac TAPVC complicated by severe pulmonary artery hypertension. The patient was managed conservatively including the use of pulmonary antihypertensive (sildenafil), with significant improvement. She is currently on follow-up at our pediatric cardiology clinic. TAPVC requires surgical intervention in early infancy to prevent the onset of pulmonary hypertension, which may contraindicate surgery. High index of suspicion and improved diagnostic skill will enhance early diagnosis and enable timely intervention.

Repair of a simple total anomalous pulmonary venous connection coexisting with a persistent left superior vena cava.

A simple total anomalous pulmonary venous connection (TAPVC) coexisting with a persistent left superior vena cava (PLSVC) is extremely rare. Connection of the PLSVC with the coronary sinus behind the left atrium induces coronary sinus dilatation. This reduces the free posterior wall space to which the common pulmonary vein is anastomosed for repairing the anomalous connection. Postoperative recurrent pulmonary venous obstruction (PVO) is the most important complication. To prevent PVO, sufficient tension-free anastomosis is necessary. When dilated, the coronary sinus becomes an obstacle for obtaining sufficient incision length in the left atrial cavity. We encountered two cases of a simple TAPVC with a PLSVC in infants weighing 1.8 and 2.9 kg, respectively. To obtain sufficient incision length, we extended the incision line to the right atrium for an atypical supracardiac TAPVC and incised from the left atrium to the coronary sinus via the right atrium for an infracardiac TAPVC. Moreover, we recreated the atrial septum with a rightward shift using a tanned pericardium in both cases. The postoperative courses were uneventful, without recurrent PVO.

Transcatheter closure of patent vertical vein after repair of total anomalous pulmonary venous connection.

Background:Vertical vein is left patent in some cases of supra-cardiac total anomalous pulmonary venous connection (TAPVC) when there is hemodynamic instability due to noncompliant left atrium and ventricle. After the peri-operative period, this results in features of pre-tricuspid shunt.Materials and Methods:Three cases with patent vertical vein following repair of supra-cardiac TAPVC presented with features of pre-tricuspid shunt on follow-up. Trans-catheter closure of patent vertical vein was performed using vascular plug in all three subjects.Results:The procedure was technically successful in all the patients. There was a complication related to catheter tip breakage in one of them, which was successfully managed. There was no impingement on pulmonary vein in any of the patients.Conclusion:Patent vertical vein following TAPVC repair results in features of pre-tricuspid shunt. Transcatheter closure of the patent vein is feasible.

The research on operation of obstructed total anomalous pulmonary venous connection in neonates.

Objectives. Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease. This study aimed to evaluate the outcomes of TAPVC repair in neonates, controlling for anatomic subtypes and surgical techniques. Methods. Between 1997 and 2013, 88 patients (median age: 16 days) underwent repair for supracardiac (31), cardiac (18), infracardiac (36), or mixed (3) TAPVC. All the patients underwent emergency operation due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included a side-to-side anastomosis between the pulmonary venous confluence and left atrium. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Results. The early mortality rate was 2.3% (2/88 patients). The echocardiogram showed no obstruction in the pulmonary vein anastomosis, and flow rate was 1.1–1.42 m/s in the 3-year follow-up period. Conclusions. The accurate preoperative diagnosis, improved protection of heart function, use of pulmonary vein tissue to anastomose and avoid damage of the pulmonary vein, and delayed sternum closure can reduce the risk of mortality. The preoperative severity of pulmonary vein obstruction, the timing of the emergency operation, and infracardiac or mixed-type TAPVC can affect prognosis. Using our surgical technique, the TAPVC mortality among our patients was gradually reduced with remarkable results. However, careful monitoring of the patient with pulmonary vein restenosis and the timing and method of reoperation should also be given importance.

Urgent stenting via internal jugular vein for stabilization in critical neonates with total anomalous pulmonary venous connection and severe pulmonary venous obstruction

Reducing obstruction in the vertical vein is significant for stabilizing the condition in neonates with total anomalous pulmonary venous connection (TAPVC). Stent implantation into the vertical vein is an alternative treatment for rescue in critical patients with TAPVC and severe pulmonary congestion. We performed a stent implantation into the supra-cardiac vertical vein for rescue and stabilization of a critical newborn with TAPVC. The internal jugular vein was selected for sheath insertion under echo guidance in an urgent procedure, which took 11min from venous puncture to stent implantation. The internal jugular approach is optimal for stent implantation in patients with supra-cardiac TAPVC in a critical condition.<Learning objective: Learning alternative methods are valuable for rescue and treatment against critical conditions with congenital heart disease in neonates. This report can provide useful and optimal technique for rescuing a critical patient with total anomalous venous connection with severe pulmonary venous obstruction.>

Palliative stent placement in vertical vein in a 1.4 kg infant with obstructed supracardiac total anomalous pulmonary venous connection

Temporary relief of obstructed total anomalous pulmonary venous connection (TAPVC) may stabilize the preoperative clinical status. We report a 1.4 kg infant with obstructed supracardiac TAPVC to right sided superior vena cava, double outlet right ventricle, pulmonary atresia, major aortopulmonary collateral arteries, and atrioventricular septal defect. A palliative percutaneous stent was successfully placed and resulted in immediate clinical improvement. We performed a review of literature regarding interventional procedures for relief of obstructed TAPVC and found 17 cases including stent placement (13), balloon angioplasty (3), and Hybrid procedure (1). We conclude that temporary stent placement in obstructed TAPVC is feasible and efficacious in relieving pulmonary venous obstruction. The procedure can lead to subsequent surgical repair in a more favorable clinical status and has special significance in the low birth weight infant.

Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection

Obstructed total anomalous pulmonary venous connection (TAPVC) is frequently misdiagnosed as pulmonary disease and without operative correction early death is common. It is important to make a correct diagnosis before surgery. The purpose of this study was to describe the chest radiographic features of obstructed TAPVC and compare CT angiography with transthoracic echocardiography in the evaluation of obstructed TAPVC. Eighteen children with obstructed TAPVC were assessed. Their clinical and imaging data were retrospectively reviewed. The characteristic radiographic findings were analyzed and compared with surgical results, and the diagnostic accuracy of CT angiography and transthoracic echocardiography was evaluated in terms of pulmonary venous drainage and obstruction detection. The common radiographic features included pulmonary venous congestion or edema or both (16 of 18 cases, 89%), and absence of cardiomegaly (12 of 18 cases, 67%). CT angiography correctly diagnosed TAPVC and clearly revealed the draining sites in all children (five with supracardiac TAPVC, three with cardiac TAPVC, eight with infracardiac TAPVC and two with mixed TAPVC). The diagnostic agreement between CT angiography and surgery was 100%. Transthoracic echocardiography only correctly revealed the draining sites in 11 children (5 with supracardiac TAPVC, 2 with cardiac TAPVC and 4 with infracardiac TAPVC). The diagnostic agreement between transthoracic echocardiography and surgery was 61%. The diagnostic accuracy of CT angiography was higher than that of transthoracic echocardiography (P = 0.0156). Thirty-four sites of obstruction were correctly detected by CT angiography (11 in the mediastinum, 1 at the diaphragmatic level, 9 below the diaphragm and 13 stenotic individual pulmonary veins in the lung). The diagnostic agreement between CT angiography and surgery was 92%. Transthoracic echocardiography only correctly detected 15 sites of obstruction (11 in the mediastinum, 1 at the diaphragmatic level and 3 below the diaphragm). The diagnostic agreement between transthoracic echocardiography and surgery was 41%. The rate of detection for sites of obstruction with transthoracic echocardiography was much lower than that of CT angiography (P = 0.0002). In children with obstructed TAPVC, plain radiographs usually show a characteristic pattern of pulmonary venous congestion or edema, or both, and a normal cardiac silhouette. CT angiography is superior to transthoracic echocardiography in the evaluation of pulmonary venous drainage and obstruction, especially in children with infracardiac and mixed TAPVC.

Primary sutureless repair for simple total anomalous pulmonary venous connection with a risk of postoperative pulmonary venous stenosis

Late mortality following surgical repair of total anomalous pulmonary venous connection (TAPVC) is often associated with pulmonary venous stenosis. We describe here two successful cases of primary sutureless repair for simple TAPVC in patients who had a potential risk of postoperative pulmonary venous stenosis. A 10-day-old neonate with mixed-type TAPVC and a 30-day-old infant with supracardiac TAPVC underwent primary sutureless repair with our modification. In the early follow-up, both patients are now doing well and have no signs of pulmonary venous stenosis. The sutureless repair can be applied as a primary surgical option to prevent postoperative pulmonary venous stenosis in selective patients with simple TAPVC.

Percutaneous Closure of a Large Unligated Vertical Vein Using the Amplatzer Vascular Plug II After Supracardiac Total Anomalous Pulmonary Venous Connection (TAPVC) Repair

It is well known that the vertical vein (VV) may have to be left open after repair of a total anomalous pulmonary venous connection (TAPVC) in children with preoperative obstruction, whose left heart chambers are small (Cope et al. in Ann Thorac Surg 64:23-29, 3). An unligated VV has been found to reduce pulmonary arterial pressure, decrease perioperative pulmonary hypertensive crisis, and provide better hemodynamics postoperatively (Chowdhry et al. in J Thorac Cardiovasc Surg 133:1286-1294, 2). Although these VVs are expected to close later, they may remain patent in about half of these children (Cheung et al. J Paediatr Child Health 41:361-364, 1).The patent VVs may be a cause for significant left-to-right shunting, and the children may be symptomatic. The case report describes a child who had a large patent VV after repair of supracardiac TAPVC and its closure using the Amplatzer Vascular Plug II device.

Modified Anastomosis for Repair of Supracardiac Total Anomalous Pulmonary Venous Connection in Infants

Recurrent pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) is usually restricted to the anastomosis between the pulmonary venous confluence and the left atrium. We describe a modified technique for repair of supracardiac TAPVC in infants. An L-shaped incision of left atrium is utilized and the right-sided anastomosis is enlarged by using autologous pericardium to create a large and tension-free anastomosis.

Vise Location of the Vertical Vein in a Neonate With a Supracardiac Total Anomalous Pulmonary Venous Connection

A newborn delivered at 37 weeks of gestation with a birth weight of 2,526 g was transferred to our hospital because of respiratory distress and cyanosis. The neonate had a heart rate of 140 beats/min and a respiratory rate of 50 breaths/min. The oxygen saturation was 75%. At auscultation, the pulmonary second heart sound was accentuated, and venous hum was noted in the second intercostal space of the left sternal border. Hepatomegaly also was noted. A chest X-ray showed a cardiothoracic ratio of 58%, with increased pulmonary congestion (Fig. 1). Echocardiography showed that the right heart was markedly enlarged and that all the pulmonary veins joined behind the left atrium connected to the innominate vein via the vertical vein, consistent with a supracardiac total anomalous pulmonary venous connection (TAPVC). Doppler imaging showed that the flow velocity was increased at the vertical vein. An atrial septal defect with right-to-left shunting and a large patent ductus arteriosus with a bidirectional shunt also were shown. Multidetector computed tomography (MDCT) (64 detectors) performed on the second day of age showed the horizontal common pulmonary vein draining to the left innominate vein via the vertical vein. The vertical vein ascended anteriorly to the left pulmonary artery and posteriorly to the ductus arteriosus and left bronchus, which was externally compressed by the ductus rather than the left bronchus (Figs. 2, 3). On the third day of age, we performed surgical correction using a cardiopulmonary bypass. The course of the vertical vein is vital with regard to obstruction in patients with supracardiac TAPVC. Usually, the vertical vein ascends anteriorly to the left pulmonary artery and the left bronchus, which is not obstructed. However, it may be obstructed when it passes between the left bronchus posteriorly and the left pulmonary artery anteriorly, the so-called ‘‘vise location’’ [1, 2]. Recent imaging methods such as MDCT and magnetic resonance angiography are useful for preoperative patients with TAPVC. Because echocardiography has a limited field of view for assessing peripheral pulmonary veins, we prefer MDCT for preoperative imaging, which requires less time for image acquisition [3]. In the current case, MDCT