Pulmonary Venous Pathway Research Articles

Abstract 12998: Preoperative Venous Variability Index Predicts the Need for Pulmonary Venous Reintervention in Total Anomalous Pulmonary Venous Return

Background: Postoperative obstruction in total anomalous pulmonary venous connection (TAPVC) is variable. Clinical risk factors are described but the role for preoperative echocardiographic markers in prognostication is unclear. Pulmonary venous variability index (PVVI), a novel metric developed at our center, is shown to better correlate to preoperative catheterization gradient and clinical markers of obstruction than standard echocardiographic measures. We hypothesized that preoperative PVVI is superior to maximum and mean velocity for prediction of outcome in TAPVC. Methods: We performed a retrospective review of TAPVC patients born 1/1/2006-9/30/2022 and repaired at our center. The medical record was reviewed for clinical and surgical risk factors. Preoperative echocardiograms were reviewed for clinical read and PVVI ((maximum velocity - minimum velocity) / mean velocity) was calculated from spectral Doppler of the pulmonary venous pathway. The outcome was time to surgical or catheter-based pulmonary vein reintervention or death. Results: In total, 162 patients were repaired. The outcome was met in 52 (32%). A univariate Cox proportional hazards model was performed for possible predictors (Table). Clinical predictors were single ventricle, heterotaxy, and a vertical vein not ligated during repair. Of echocardiographic metrics, PVVI and minimum velocity had strong associations with outcome (Figure). Conclusion: Preoperative PVVI predicts postoperative obstruction in TAPVC or death, while maximum and mean velocity and qualitative assessment of obstruction do not. Use of this quantitative metric may decrease practice variation and be valuable in prognosis.

Descending thoracic aortic homograft for superior vena caval translocation in the Warden procedure: Report of six cases.

Warden procedure has been described for repair of partial anomalous pulmonary venous connections inserted high into the superior vena cava. In the current report, we describe a new modification. A total of six patients (five females, 83%) with a mean age of 18.8 ± 10.8 years, underwent a modified Warden procedure with a descending thoracic aortic (DTA) homograft connected to the free wall of the right atrium, rather than to the right atrial appendage in the period between June 2021 and July 2022. All patients had evidence of enlarged right-sided cardiac chambers. Two patients had intact interatrial septum (33.3%), while three patients had sinus venosus (50%) and one had secundum atrial septal defects (16.7%). The procedure was performed via minimally invasive right thoracotomy in half of the patients. Patients were extubated in the operating room or soon after. No early mortality or reoperation. One patient required concomitant aortic root replacement and one needed repair of anomalous left upper pulmonary vein connection to the left innominate vein. Follow-up was complete in all patients with a mean of 6.2 ± 4.6 months. No late mortality or reoperation. All patients were discharged on antiplatelet therapy. Follow-up imaging studies showed satisfactory repair with widely patent superior vena caval and pulmonary venous pathways. The use of DTA homograft to facilitate superior vena caval translocation in the modified Warden procedure appears to be beneficial as it decreases tension on the anastomosis and avoids the need for anticoagulation.

Predictors of mortality and morbidity in total anomalous pulmonary venous connection with biventricular physiology: A 10-year Indian single centre experience of 492 patients.

Surgical correction of total anomalous pulmonary venous connection (TAPVC) remains associated with significant mortality despite advances in intra-operative and postoperative management. We retrospectively analyzed 492 consecutive TAPVC patients with biventricular physiology, who were operated at our centre, with regard to predictors of mortality, morbidity, and intermediate-term outcomes. A total of 492 TAPVC patients with biventricular physiology were operated at our centre from August 2009 to November 2019. Their medical records were reviewed and were followed up during March-April 2020 for any symptoms of cardiac disease. Of 492, 302 (61.38%) were healthy at follow-up, 29 (5.89%) had postoperative mortality, 23 (4.67%) had mortality during the follow-up period, and 138 (28.05%) were lost to follow up. Age <1 month and weight <2.5 kg were associated with higher mortality with odds ratios (OR) of 6.37 and 5.56, respectively. There was no difference in mortality in different types of TAPVC. Obstructed TAPVC was associated with higher mortality with OR of 3.05. Acute kidney injury requiring peritoneal dialysis and sepsis were associated with higher mortality with ORs of 10.17 and 3.29, respectively. All follow-up mortality occurred in <1 year from the index operation. Anastomotic gradients were significantly higher in patients who died. Although peri-operative TAPVC mortality has reduced, mortality on follow-up continues to occur and is partly due to the obstruction of pulmonary venous pathway. Meticulous follow-up holds the key in further reducing the mortality. Larger studies are needed for the identification of risk factors for pulmonary venous obstruction and its preventive strategies.

Pulmonary venous pathway stenosis in patient with congenitally corrected transposition of great artery.

Pulmonary venous pathway stenosis in patient with congenitally corrected transposition of great artery.

Outcomes of double switch procedure in patients with congenitally corrected transposition of the great arteries with or without associated VSD

Anatomical repair by double switch procedure (DS) for patients with congenitally corrected transposition of the great arteries (ccTGA) remains under debate. The outcomes after DS for ccTGA without LVOTO, with specific focus on the left ventricle (LV) performance in patients with intact ventricular septum (IVS) were assessed. Since 1994, 39 patients underwent DS: 9 had an IVS, and 30 had an associated VSD. Palliative procedure consisted in pulmonary artery banding (PAB) for 31 patients (79%): 9 (twice in 5) for tricuspid regurgitation (TR) and 22 for hemodynamically significant associated VSD. Survival and LV performance in follow up were analyzed. PAB was efficient to diminish TR significantly in all 9. The median age at repair was 22 months (12 days–13 years). Associated VSD closure was performed in 76% of patients ( n = 30). Four patients required peri-operative pacemaker implantation. Hospital mortality occurred in 1 (2.5%: 70% CI 1–8%) neonate who underwent DS and aortic arch repair: he died because of low output syndrome. Two (5%) patients required early reoperation for pulmonary venous pathway stenosis release. Median follow up was 13.7 years (range: 3 months–26 years). Two late deaths occurred 16 and 84 months postoperatively because of refractory LV dysfunction: both patients had IVS and benefited from LV retraining. Six patients (16%) required 7 reoperations during follow up. At last visit, 5 patients (14%) presented with LV dysfunction (LV ejection fraction < 50%) requiring medical treatment while remaining 31 (84%) were asymptomatic. Actuarial survival curves are shown in Fig. 1 . Mortality was significantly higher in the IVS group compared to the VSD group (33% vs. 0%, P = 0.01). The DS appeared to be the procedure of choice for ccTGA associated with VSD. PAB was efficient for the management of TR in early life. Our Results suggest that the strategy of LV retraining by PAB to achieve DS at any price remains questionable.

Repair technique for a rare partial anomalous pulmonary venous return associated with retroaortic innominate vein

BackgroundRetroaortic innominate vein (RIV) is a rare vascular abnormality. Although RIV itself is asymptomatic, its presence in patients with partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) is surgically challenging because a simple Warden procedure is impossible.Case presentationA 16-year-old girl was diagnosed with tetralogy of Fallot, secundum, and sinus venosus atrial septal defect (ASD) at birth. She underwent total correction of tetralogy of Fallot and ASD closure at the age of 14-months. However, the diagnosis of PAPVR was missed. At the age of 16, she developed dyspnea on exercise. Echocardiography demonstrated severe pulmonary regurgitation, mild tricuspid regurgitation, and D-shaped left ventricle with paradoxical septal motion along with RIV and sinus venous ASD. Computed tomography confirmed RIV and PAPVR. Systemic and pulmonary venous blood pathways were separated by bovine pericardial patch, and pulmonary valve replacement was performed. Postoperative echocardiography demonstrated improvement of D-shaped left ventricle and laminar flow through the SVC and pulmonary veins. Postoperative computed tomography showed a well-reconstructed SVC and pulmonary venous pathway without stenosis. After an uneventful postoperative course, patient was discharged.ConclusionsPAPVR in patients with RIV may be surgically challenging to repair. We report the first case of successfully repaired PAPVR associated with RIV.

Venous Flow Variation Predicts Preoperative Pulmonary Venous Obstruction in Children with Total Anomalous Pulmonary Venous Connection

Identifying preoperative pulmonary venous obstruction in total anomalous pulmonary venous connection is important to guide treatment planning and risk prognostication. No standardized echocardiographic definition of obstruction exists in the literature. Definitions based on absolute velocities are affected by technical limitations and variations in pulmonary venous return. The authors developed a metric to quantify pulmonary venous blood flow variation: pulmonary venous variability index (PVVI). The aim of this study was to demonstrate its accuracy in defining obstruction. All patients with total anomalous pulmonary venous connection at a single institution were identified. Echocardiograms were reviewed, and maximum (Vmax), mean (Vmean), and minimum (Vmin) velocities along the pulmonary venous pathway were measured. PVVI was defined as (Vmax-Vmin)/Vmean. These metrics were compared with pressures measured on cardiac catheterization. Echocardiographic measures were then compared between patients with and without clinical preoperative obstruction (defined as a need for preoperative intubation, catheter-based intervention, or surgery within 1day of diagnosis), as well as pulmonary edema by chest radiography and markers of lactic acidosis. One hundred thirty-seven patients were included, with 22 having catheterization pressure recordings. Vmax and Vmean were not different between patients with catheter gradients ≥ 4 and<4mm Hg, while PVVI was significantly lower and Vmin higher in those with gradients ≥ 4mm Hg. The composite outcome of preoperative obstruction occurred in 51 patients (37%). Absolute velocities were not different between patients with and without clinical obstruction, while PVVI was significantly lower in patients with obstruction. All metrics except Vmax were associated with pulmonary edema; none were associated with blood gas metrics. The authors developed a novel quantitative metric of pulmonary venous flow, which was superior to traditional echocardiographic metrics. Decreased PVVI was highly associated with elevated gradients measured by catheterization and clinical preoperative obstruction. These results should aid risk assessment and diagnosis preoperatively in patients with total anomalous pulmonary venous connection.

Repair of a mixed form of supracardiac total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection is a rare congenital heart defect. We report an infant with a mixed form of supracardiac TAPVC, in whom all pulmonary veins, except the right upper, entered a pulmonary venous confluence that is connected to a vertical vein and drained into the superior vena caval-right atrial junction. Several segmental right upper pulmonary veins entered the superior vena cava, superior to the entry of the vertical vein. Surgical repair consisted of the Warden procedure combined with direct anastomosis of the vertical vein to the left atrium. Separate pulmonary venous drainage pathways decreased the risk of post-operative pulmonary venous obstruction. Our patient had an uneventful post-operative course and encouraging 2-month follow-up echocardiography. Careful follow-up is warranted to detect post-operative complications, including obstruction of the pulmonary venous and cavoatrial anastomoses.

Long-Term Outcomes of the Double-Barrel Technique for Superior Sinus Venosus Defect With Partially Anomalous Pulmonary Venous Connection.

Repair of superior sinus venosus defect with high partially anomalous pulmonary venous connection (PAPVC) using an intracardiac baffle may be complicated by systemic or pulmonary venous pathway obstruction and sinus nodal dysfunction (SND). Our surgical strategy for repair of all types of superior sinus venosus defect has evolved chiefly to avoid the abovementioned complications and preserving the growth potential of the superior cavoatrial junction. Between 2007 and 2019, fifty consecutive patients aged 2 to 60 (mean, 17.6±16.7) years underwent repair of superior sinus venosus defect using the double-barrel technique as described. The anomalous pulmonary veins drained into the superior cavoatrial junction in 17 patients and more than 2 cm above the cavoatrial junction in 33 patients. There were no early or late deaths and no reoperations. At a mean follow-up of 103.9 (±50.2) months, all survived the operation, and actuarial freedom from SND was 97.9% (±standard error, 0.02%; 95% CI: 0.86-0.99). No patient had systemic or pulmonary venous pathway obstruction. A permanent pacemaker was required in one (2%) patient for sick sinus syndrome. The double-barrel method is an expedient, safe, and effective technique in superior sinus venosus defect. It provides dual drainage of superior vena cava preserving the superior cavoatrial junction without causing systemic or pulmonary venous pathway obstruction and can be utilized in all cases including those with high PAPVC. Preservation of the cavoatrial junction and use of autogenous atrial tissue for systemic venous pathway avoids SND and preserves growth potential.

Incidence and treatment of pulmonary vein stenosis after repair of total anomalous pulmonary venous connection.

In this study, we aimed to investigate the incidence and treatment of pulmonary vein stenosis after repair of total anomalous pulmonary venous connection. Between December 2010 and December 2016, a total of 40 patients (25 males, 15 females; mean age 45±41 days; range, 2 days to 6 months) who underwent total anomalous pulmonary venous connection repair were retrospectively analyzed. Eighteen (45%) of the patients were supracardiac, seven (17.5%) were cardiac, eight (20%) were infracardiac, and seven (17.5%) were mixed-type anomaly. Twelve patients (30%) had obstruction of the pulmonary venous pathways. Hospital mortality was seen in three patients (7.5%). All of non-survivors received emergent surgery and the causes of death were sepsis and multiple organ failure. A total of 23 complications were seen in 20 patients (50%) mainly delayed sternal closure (n=11; 27.5%) and prolonged mechanical ventilation (n=6; 15%). The mean follow-up was 12.2±9.6 (range, 1 to 36) months in all patients, except one. Pulmonary vein stenosis developed in three patients (8.3%) after repair. All of them underwent sutureless repair. Total anomalous pulmonary venous connection can be repaired with low mortality and morbidity rates and favorable mid-term outcomes. Close follow-up is necessary due to the risk of development of pulmonary vein stenosis. Sutureless repair may provide relief in such cases.

Impaired atrioventricular transport in patients with transposition of the great arteries palliated by atrial switch and preserved systolic right ventricular function: A magnetic resonance imaging study.

We aimed (1) determine if systemic right ventricle filling parameters influence systemic right ventricle stroke volume in adult patients with D-transposition of the great arteries (D-TGA) palliated by atrial switch, using cardiac magnetic resonance imaging and echocardiography, and (2) to study relationship of these diastolic parameters with exercise performance and BNP, in patients with preserved systolic systemic right ventricle function. Single-center, cross-sectional, prospective study. In patients with D-TGA palliated by atrial switch, diastolic dysfunction of the systemic right ventricle may precede systolic dysfunction. Forty-five patients with D-TGA and atrial switch and 45 age and sex-matched healthy subjects underwent cardiac magnetic resonance imaging and echocardiography. Filling flow-rates measured by phase-contrast cardiac magnetic resonance imaging were analyzed using customized software to estimate diastolic parameters and compared with exercise performance. In D-TGA, early filling of systemic right ventricle was impaired with a lower peak filling rate normalized by filling volume (Ef/FV measured by cardiac magnetic resonance imaging) and a higher early filling peak velocity normalized by early peak myocardial velocity (EUS /Ea measured by echocardiography) compared with controls (P ≤ .04). Stroke volume of systemic right ventricle showed a direct and significant association with pulmonary venous pathway size (respectively r = 0.50, P < .01). Systemic right atrial area and systemic right ventricle mass/volume index measured by cardiac magnetic resonance imaging, as well as Ef/FV were significantly correlated with exercise performances and BNP (P < .01). All correlations were independent of age, gender, body mass index and blood pressure. Systemic right ventricle pre-load and stroke volume depend mainly on intraatrial pathway function. Moreover, systemic right ventricle remodeling and right atrial dysfunction impair systemic right ventricle filling, leading to BNP increase and exercise limitation. Cardiac magnetic resonance imaging should assess systemic right ventricle filling abnormalities in D-TGA patients.

Is a modified Senning with pericardial patch associated with less complications and better prognosis?

IntroductionThe Senning procedure continues to be indicated for complex cases of transposition of the great arteries (TGA) that are not suitable to the anatomical correction (arterial switch operation). Some modifications of the Senning procedure were proposed to augment both the atrial septal flab and/or pulmonary veins. In our study, autologous pericardium treated by saline and heparin was used to augment the right atrium (pulmonary venous) pathway. The aim was to avoid pulmonary venous pathway stenosis (PVPS) complications. Patients and methods74 patients who underwent a modified Senning procedure in Geneva University Hospital between 1989 and 2013 were analyzed retrospectively as well as prospectively during the time of the study. The follow up period extended up to 24 years, and any patient with follow up period less than 1 year was excluded. Results67 patients (90%) were discharged with sinus rhythm, and in the latest follow up, 34 patients (45%) maintained their sinus rhythm. 26 patients (35%) experienced at least a single episode of arrhythmia in the early post-operative period. This episode was either nodal rhythm (n = 10), complete AV block (n = 8), supraventricular tachycardia (SVT) (n = 6) and bradycardia (n = 2). Permanent pacemaker (PM) implanted in 7 patients (9.5%) before discharge. During the follow-up period, 22 patients (29.7%), presented with SVT (all referred for ablation), 1 presented with nodal rhythm and 1 presented in AV block (both had a PM implantation). These two cases raised the total of patients who had PM implantation at the latest follow-up to 9 patients (12%). We had 3 patients (4.1%) who had experienced pulmonary venous pathway stenosis (PVPS). One patient had early presentation on the 3rd day post-operatively requiring surgical revision and patch augmentation. 2 patients had the presentation during the follow-up period, 3 and 5 years respectively after surgery. Both had reoperation with smooth post-operative course. We assessed the survival of patients along the follow-up period, using Kaplan Mayer showing 86.1% survival at 24 years. At the latest follow-up, 9 patients out of 74 patients included in our study (12.2%) died during the follow-up period; 6 patients due to heart failure, 2 patients due to fatal arrhythmias, and one post tricuspid valve implantation. ConclusionThe modified Senning procedure using autologous pericardial patch augmentation, showed less incidence of late PVPS, and higher incidence of restoration of sinus rhythm at the time of discharge in comparison to the published results of the standard technique in the literature. It had a low mortality and comparatively better survival.

Anteroposterior Pulmonary Venous Anatomy: A Hitherto Undescribed Pulmonary Venous Arrangement With Implication for the Senning Operation and a Proposed Method of Dealing With It.

We have encountered a hitherto undescribed anatomical variant of pulmonary vein anatomy in hearts with d-transposition or congenitally corrected transposition of the great arteries. In this variant, the inferior right pulmonary vein lies posterior rather than inferior to the superior right pulmonary vein. This variant of pulmonary vein anatomy may predispose to the development of pulmonary venous pathway obstruction following a Senning operation, since it is difficult to open the pulmonary veins in a way that ensures a wide-open pulmonary venous pathway to the atrioventricular valve of the systemic ventricle. A method of neutralizing this effect is described, with medium-term results.

Congenital heart disease in the older adult: a scientific statement from the American Heart Association.

The population of adults with congenital heart disease (ACHD) has increased dramatically over the past few decades, with many people who are now middle-aged and some in the geriatric age range. This improved longevity is leading to increased use of the medical system for both routine and episodic care, and caregivers need to be prepared to diagnose, follow up, and treat the older adult with congenital heart disease (CHD). The predictable natural progression of CHD entities and sequelae of previous interventions must now be treated in the setting of late complications, acquired cardiac disease, multiorgan effects of lifelong processes, and the unrelenting process of aging. Despite the advances in this field, death rates in the population from 20 to >70 years of age may be twice to 7 times higher for the ACHD population than for their peers.1 This American Heart Association (AHA) scientific statement will focus on the older adult (>40 years old) with CHD. It is meant to be complementary to the 2008 American College of Cardiology (ACC)/AHA guidelines for ACHD and orient the reader to the natural history, ramifications of childhood repair, and late initial diagnosis of CHD in the older adult. This population with CHD is unique and distinct from both the pediatric and young adult populations with CHD. Much of the information we provide is from scientific research combined with clinical experience from longitudinal care. We emphasize that this is the beginning of a discussion regarding this rapidly growing population, and continued research aimed at the progression of disease and complications reviewed here is necessary to advance the field of ACHD with the scientific rigor it deserves. ACHD encompass a broad range of presentations. There are people who are diagnosed for the first time in adulthood, as well as those with prior palliative repair …

Percutaneous Dilation of Pulmonary Venous Pathway Obstruction Post-Atrial Switch Operation

Percutaneous Dilation of Pulmonary Venous Pathway Obstruction Post-Atrial Switch Operation

Left-sided Pulmonary Venous Pathway Obstruction after Mustard Operation

Pulmonary venous pathway obstruction (PVPO) is a serious complication of the atrial switch procedure for transposition of the great arteries (TGA). Redistribution of pulmonary arterial blood flow may make conventional echocardiographic measures of unilateral PVPO difficult to interpret. Previous studies have demonstrated altered arterial flow patterns using phase-contrast magnetic resonance imaging (PC-MRI). The purpose of this study was to investigate arterial flow patterns in the setting of unilateral PVPO following the Mustard operation. We retrospectively reviewed the arterial flow patterns of 12 patients undergoing magnetic resonance imaging between 2006 and 2011 who had had the Mustard operation. Three patients had left PVPO and all showed >65% flow distributed to the right pulmonary artery (RPA). One had severe obstruction and was the only one to show significant continuous diastolic forward flow in the RPA or diastolic flow reversal in the left pulmonary artery. RPA/left pulmonary artery flow ratio appears to be a sensitive marker for PVPO. In the absence of pulmonary regurgitation, reversed diastolic flow in the ipsilateral and continuous in the contralateral pulmonary artery appears specific for severe obstruction. Branch pulmonary artery phase-contrast magnetic resonance imaging (PC-MRI) should be performed routinely for patients who have had an atrial switch for transposition of the great arteries.

Editorial Comment: Anatomic repair for congenitally corrected transposition of the great arteries: easier is better?

Sojak et al .[ 1] reported their experience with bidirectional cavopulmonary anastomosis (BCPA) and the hemi-mustard procedure for an anatomic correction of a congenitally corrected transposition of the great arteries (ccTGA), a strategy that somewhat simplifies the classic atrial switch procedure as already reported by several groups [2]. In this recent small series (eight patients), the authors made the choice to use this approach in selected patients with atrio-apical discordance to technically simplify the atrial switch component of the surgery. For the functionally borderline right ventricle, and/or tricuspid valve dysfunction, a one-anda-half-type repair was chosen to unload the RV. Of the patients who benefited from this modified atrial switch, six had an associated Rastelli procedure and two had an arterial switch procedure. The early mortality was 12.5% (one patient); two patients were treated for supraventricular tachycardia and three patients developed pleural effusion but no superior vena cava syndrome was notably observed. Only one patient needed re-operation for a conduit replacement at 6 years, from a mean follow-up of 4.5 years. All except one patient were in NYHA class I at the last follow-up. Systemic or pulmonary venous obstruction, sinus node dysfunction or atrial baffle leak were not observed. Successful early outcomes with anatomic repair in ccTGA were first reported by several centres in the mid-1990s. Nevertheless, 20 years later, it remains a challenging surgical procedure and the anatomic variability observed poses technical difficulties. The excellent results published by most of the experienced centres in this field [3, 4] probably do not reflect the real morbidity and mortality observed worldwide for the anatomic repair of ccTGA. Therefore, a strategy that reduces the surgical complexity of the repair, whilst maintaining good early and long-term outcomes, has to be considered as an important alternative, particularly for patients presenting for surgery at lower volume centres. This one-and-a-half repair approach definitely reduces the technical challenge of the operation, especially when atrio-apical discordance is present (difficult surgical access to the atrial chambers and narrowed free wall of the right atrium) even though flipping the heart over into the left chest by opening the left pleura usually allows one to perform a Senning. Less complex surgery is associated with a shorter cross-clamp time, something that can be helpful in preserving the ventricular function in this subset of patients. This one-and-a-half strategy, perhaps offers the possibility that anatomic repair, even in the most complex forms of ccTGA, could be achieved by less-experienced surgeons. Another theoretical advantage of the procedure is the avoidance of a superior vena cava stenosis, though this complication has become rare, with almost no reported instances of re-operation in the most recent series [3, 4]. For the pulmonary venous pathway, one might expect a decrease in the rate of obstruction with this technique, although it appeared that, for all except one case in this latter series, the right atriotomy needed to be enlarged with a xenopericardial patch, something that might compromise the long-term growth of the channel. Reducing atrial suture lines did not avoid supraventricular tachycardia, but no sinus node dysfunction (associated with higher sudden death risk) was observed, a major advantage compared with the Senning procedure. Finally, the real consequences of the volume unloading of the right ventricle are difficult to appreciate, due to the lack of data concerning the pre- and postoperative function and dimension of the RV and importance of the TR in this series. Furthermore, the potential benefit of the unloading is highly dependent on the patient’s age, and this benefit is likely to decrease as the patient ages. Nevertheless, a staged approach, with initial BCPA alone, is an option for complex ccTGA with LVOTO that avoids iterative systemic-pulmonary shunts. This strategy allows a complete repair to be delayed at a time when construction of the intraventricular LV to aorta baffle would otherwise be hazardous and shortens the surgery at the time of a subsequent full repair. BCPA might also allow re-operation to be delayed in cases where the RV to pulmonary artery conduit becomes stenotic. However, the benefits (easier surgical procedure, sinus node rhythm, unloading of the RV) are counterbalanced by the numerous adverse effects of this technique. BCPA precludes any superior venous access for endovascular electrophysiological procedures, including pacing. Complete heart block is a frequent complication after the closure of the ventricular septal defect in ccTGA or during the natural history

Historical Aspects of Transcatheter Treatment of Heart Disease in Children

The very first transcatheter intervention to treat congenital cardiac defects was reported by Rubio-Alvarez et al. [1] in 1953, when they performed pulmonary valvotomy using a modified ureteral catheter. A decade later Dotter, Rashkind, Porstmann and their associates described progressive dilatation of peripheral arterial stenotic lesions, balloon atrial septostomy and transcatheter occlusion of patent ductus arteriosus, respectively. The purpose of this review is to present these and other historical developments of catheter-based interventions in the treatment of heart disease in children. Historical aspects of 1. balloon angioplasty/valvuloplasty of valvar pulmonary stenosis, valvar aortic stenosis, fixed subaortic stenosis, native aortic coarctation, postsurgical aortic recoarctation, branch pulmonary artery stenosis, mitral stenosis, cyanotic heart defects with pulmonary oligemia, stenotic bioprosthetic valves, congenital tricuspid and mitral stenosis, truncal valve stenosis, subvalvar pulmonary stenosis, supravalvar pulmonary stenosis (congenital membranous or postoperative), stenosis of the aorta (Leriche syndrome, atherosclerotic and Takayasu’s arteritis), baffle obstruction following Mustard or Senning procedure (both systemic and pulmonary venous obstructions), superior and inferior vena caval obstructions, pulmonary vein stenosis, pulmonary veno‑occlusive disease, vertical vein stenosis in total anomalous pulmonary venous connection, pulmonary venous obstruction following repair of total anomalous pulmonary venous obstruction, specially designed pulmonary artery bands, cor triatriatum, cor triatriatum dexter, and coronary artery stenotic lesions that develop after Kawasaki disease; 2. stents to enlarge stenotic lesions of branch pulmonary arteries, systemic veins, systemic and pulmonary venous pathways after Mustard procedure, aorta, right ventricular outflow conduits, pulmonary veins and native right ventricular outflow tract or to keep the ductus arteriosus open in patients with pulmonary atresia and hypoplastic left heart syndrome and maintaining patency of stenosed aorto-pulmonary collateral vessels, surgically created but obstructed shunts or acutely thrombosed shunts as well as covered stents; 3. transcatheter occlusion of cardiac defects comprising of atrial septal defect, patent foramen ovale, patent ductus arteriosus, ventricular septal defect and aortopulmonary window and 4. catheter-based atrial septostomy such as Rashkind balloon atrial septostomy, Park’s blade atrial septostomy, balloon angioplasty of the atrial septum, trans-septal puncture and atrial septal stents were presented.

Partial anomalous pulmonary venous connection to the superior vena cava: the outcome after the Warden procedure

Partial anomalous pulmonary venous connection (PAPVC) draining into the superior vena cava (SVC) has been repaired with various techniques. We investigated the outcome of the Warden procedure for repair of this anomaly. From December 1994 to January 2011, 30 patients underwent a Warden procedure for repair of PAPVC to the SVC in our center. Their median age at the time of the operation was 4.9 years (range, 1 month to 55 years). Follow-up data were obtained through a review of medical records, correspondence with the patients' cardiologists, and direct telephone contact. The mean follow-up duration was 5.3 ± 5.1 years (range, 1 month to 16 years). One patient died of an underlying cardiac condition and cerebral complication unrelated to the Warden procedure. One patient had transient postoperative sinus node dysfunction. During follow-up, pulmonary venous pathway obstruction occurred in one patient, and systemic venous pathway obstruction occurred in three patients. Re-operation or re-intervention for systemic venous pathway obstruction was required in younger (<2 years) and smaller (<7 kg) patients within 1 year after the Warden procedure. All patients were in regular sinus rhythm in the latest electrocardiogram. The Warden procedure is a safe and effective surgical option for repair of PAPVC to the SVC in terms of preserving the sinus node function and non-obstructive pulmonary venous pathway. However, more attention must be paid to the reconstruction of non-obstructive systemic venous pathway, especially in younger and smaller children. Patch augmentation could be considered and effectively performed, if there is any doubt regarding tension-free anastomosis.

Partial Anomalous Pulmonary Venous Connection to the Superior Vena Cava

We describe the surgical technique of reimplantation of the right superior pulmonary vein into the left atrium in 2 patients with partial anomalous pulmonary venous connection to the superior vena cava without atrial septal defect. A right axillary minithoracotomy is done through the fourth intercostal space. The pulmonary vein is detached from its origin in the superior vena cava. This is sutured with 6-0 reabsorbable polydioxanone suture (Ethicon, Somerville, NJ). A lateral clamp is applied to the left atrium, and the pulmonary vein is reimplanted. The patient is extubated in the operating room. Neither cardiopulmonary bypass nor blood transfusion was required. It is simple, safe, and reproducible.