Repair of “Simple” Total Anomalous Pulmonary Venous Connection: A Review From the Pediatric Cardiac Care Consortium

doi:10.1016/j.athoracsur.2012.03.006

The Annals of Thoracic Surgery

Volume 94, Issue 1, July 2012, Pages 133-138

Presented at the Fifty-eighth Annual Meeting of the Southern Thoracic Surgical Association, San Antonio, TX, Nov 9–12, 2011.

https://doi.org/10.1016/j.athoracsur.2012.03.006 Get rights and content

Background

Outcomes for repair of total anomalous pulmonary venous connection (TAPVC) from individual institutions suggest a significant improvement in mortality over the past several decades. The purpose of this study is to review the outcomes after repair of TAPVC from a large multiinstitutional registry.

Methods

A retrospective review of the multiinstitutional database, the Pediatric Cardiac Care Consortium (PCCC), was used to identify patients with the diagnosis of TAPVC who underwent complete correction between 1982 and 2007. Data reviewed included age, decade of primary operation, anatomic type, presentation, and in-hospital mortality.

Results

Of the 118,084 surgical procedures submitted to the PCCC, 2,191 (1.9%) consisted of primary surgical correction of TAPVC. Sixty-one percent of the cohort was male, with 6.8% reported as premature. Overall in-hospital surgical mortality for simple TAPVC was 13%. Mortality was 20% from 1982 to 1989, 16% from 1990 to 1999, and 8% from 2000 to 2007. Obstruction to the anomalous pulmonary venous connection occurred in 29%, with a mortality of 26%.

Conclusions

Surgical outcomes from repair of congenital cardiac anomalies have significantly improved over the past several decades. Multiinstitutional large databases are needed to confirm results published from single-institution experiences. Although improvements in surgical repair of TAPVC have occurred over the past three decades, specific subtypes still experience significant mortality.

Section snippets

Patients and Methods

Data used in this analysis were covered by the Data Use Agreement of the Pediatric Cardiac Care Consortium (PCCC) provided from individual member institutions. The study was approved by the Institutional Review Board at the University of Minnesota with an exception for obtaining individual patient consent.

Results

A total of 2,191 patients underwent primary surgical repair of TAPVC between 1982 and 2007. When evaluating patients with simple TAPVC (77%, n = 1,693), 91% (n = 1,543) were younger than 18 years, whereas 86% (n = 1,454) were younger than 1 year of life at the time of operation. This analysis will focus on those younger than 18 years of age at primary repair. Patient characteristics stratified by decade of primary repair are presented in Table 1. Sixty-two percent of this population was male.

Comment

Using a large multiinstitutional surgical registry, we analyzed changes in mortality and predictors of mortality in patients who underwent primary repair of TAPVC over an extended period. Several single-center retrospective studies have shown that survival in repair of TAPVC has generally improved over the past several decades [7, 8, 9]. Our goal was to confirm these findings using a large cohort of patients.

The overall mortality for patients with simple TAPVC undergoing primary repair was 13%.

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Cited by (58)

Supracardiac total anomalous pulmonary venous connection type Ib: Morphology and outcomes
2023, Journal of Thoracic and Cardiovascular Surgery
Supracardiac total anomalous pulmonary venous connection is the most common subtype of total anomalous pulmonary venous connection. We aimed to describe the morphological spectrum of supracardiac total anomalous pulmonary venous connection and to identify risk factors for death and postoperative pulmonary venous obstruction.
From February 2009 to June 2019, 241 patients diagnosed with supracardiac-Ia (left-sided vertical vein, n = 185) or supracardiac-Ib (right-sided connection directly to superior vena cava, n = 56) total anomalous pulmonary venous connection underwent initial surgical repair at our institute. Cases with functionally univentricular circulations or atrial isomerism were excluded. Patients' postoperative survival was described by Kaplan–Meier curves. Cox proportional hazards models and competing risk regression models were used to identify clinical risk factors for death and postoperative pulmonary venous obstruction.
There were 8 early deaths and 4 late deaths. The overall survivals at 30 days, 1 year, and 10 years were 97.1%, 94.8%, and 94.8%, respectively, in the supracardiac-Ia group (2.7%, 5/185) (hazard ratio, 4.8; P = .003). Five patients required reoperation for pulmonary venous obstruction, including 2 patients who required reintervention for superior vena cava syndromes (all in the supracardiac-Ib group). One patient required superior vena cava balloon dilation for superior vena cava syndromes. Multivariable analysis showed that the supracardiac-Ib group (12.5%, 7/56) had a significantly higher mortality rate than the supracardiac-Ia group (adjusted hazard ratio, 8.5, P = .008). Surgical weight less than 2.5 kg (adjusted hazard ratio, 10.8, P = .023), longer duration of cardiopulmonary bypass (adjusted hazard ratio, 1.15 per 10 minutes, P = .012), and supracardiac-Ib subtype (adjusted hazard ratio, 4.7, P = .037) were independent risk factors associated with death. The supracardiac-Ib subtype (adjusted hazard ratio, 4.8, P = .003) was an incremental risk factor associated with postoperative pulmonary venous obstruction.
Morphological features of supracardiac total anomalous pulmonary venous connection, especially the supracardiac-Ib subtype, were risk factors associated with postoperative pulmonary venous obstruction and survival. Patients with unique anatomic subtypes might require more individualized surgical planning.
Cardiac-type total anomalous pulmonary venous return is not benign
2023, Journal of Thoracic and Cardiovascular Surgery
The objective of this study was to investigate the association between morphological variation and postsurgical pulmonary vein (PV) stenosis (PPVS) in patients with cardiac total anomalous pulmonary venous connection (TAPVC).
This single-center, retrospective study included 168 pediatric patients who underwent surgical repair of cardiac TAPVC from 2013 to 2019 (connection to the coronary sinus [CS], n = 136; connection directly to the right atrium [RA], n = 32). Three-dimensional computed tomography modeling and geometric analysis were performed to investigate the morphological features; their relevance to the PPVS was examined.
The connection type had no association with PPVS (CS type: 18% vs right atrial type: 19%; P = .89) but there was a higher incidence of PPVS in patients with a single PV orifice than > 1 orifice (P < .001). Confluence-to-total PV area ratio (hazard ratio, 4.78, 95% CI, 1.86-12.32; P = .001) and length of drainage route (hazard ratio, 1.22; 95% CI, 1.14-1.31; P < .001) had a 4- and 1-fold increase in the risk for PPVS in the CS type after adjustment for age and preoperative pulmonary venous obstruction. In the right atrial type, those with anomalous PV return to the RA roof were more likely to develop PPVS than to the posterior wall of the RA (P < .001).
The number of inter-junction PV orifice correlated with PPVS development in cardiac TAPVC. The confluence-to-total PV ratio, length of drainage route, and anomalous PV return to the RA roof are important predictors for PPVS. Morphological subcategorization in this clinical setting can potentially assist in surgical decision-making.
Early and Intermediate-Term Results for the Combined Superior Approach Correction of Supracardiac Total Anomalous Pulmonary Venous Connection in Neonatal Patients
2021, Heart Lung and Circulation
Citation Excerpt :
The purpose of any surgical technique to repair TAPVC is to achieve a wide-patency anastomosis between the PVC and left atrium so that pulmonary vein blood can be redirected to the left atrium without obstruction. Unfortunately, surgical correction of TAPVC is associated with high mortality and morbidity; the repair of TAPVC remains a particular challenge for neonatal patients [8,9]. There are certain risk factors for neonatal patients, including preoperative PVO, a very small LA, low body weight, and poor cardiac protection [2,10].
There are different surgical approaches used for repairing a supracardiac total anomalous pulmonary venous connection (TAPVC), with different results. This retrospective study evaluated the outcomes of surgical repair for supracardiac TAPVC through the combined superior approach in neonatal patients.
Medical records were retrospectively reviewed and 21 neonates who underwent supracardiac TAPVC repair with the combined superior approach between July 2014 and January 2020 were identified. There were 13 males and eight females.
The patients’ median age was 20.6±8.9 days (range, 3–27). The median weight was 3.1±0.39 kg (range, 2.5–3.7) The median aortic cross-clamp and cardiopulmonary bypass times were 49.3±19.5 minutes (range, 27–86) and 91.1±23.7 minutes (range, 57–146). They were two deaths during the intensive care unit stay. One (1) patient died 2 months after discharge, the other remaining patients had no pulmonary venous obstruction (PVO) at the 6-month and intermediate-term follow-ups.
The combined superior approach is a useful method for repair of neonatal critical supracardiac TAPVC. This technique may be more helpful in preventing early postoperative anastomotic stenosis and contribute to an improved patient outcome.
Venous Flow Variation Predicts Preoperative Pulmonary Venous Obstruction in Children with Total Anomalous Pulmonary Venous Connection
2021, Journal of the American Society of Echocardiography
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 (V_max), mean (V_mean), and minimum (V_min) velocities along the pulmonary venous pathway were measured. PVVI was defined as (V_max − V_min)/V_mean. 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 1 day 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.
V_max and V_mean were not different between patients with catheter gradients ≥ 4 and < 4 mm Hg, while PVVI was significantly lower and V_min higher in those with gradients ≥ 4 mm 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 V_max 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.
An unusual case of obstructed total anomalous pulmonary venous return with gastric varices
2021, Cardiology in the Young
Surgical prognosis for infracardiac total anomalous pulmonary venous connection:experience in a single institution
2024, Chinese Journal of Thoracic and Cardiovascular Surgery

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Original articlePediatric cardiacRepair of “Simple” Total Anomalous Pulmonary Venous Connection: A Review From the Pediatric Cardiac Care Consortium

Background

Methods

Results

Conclusions

Section snippets

Patients and Methods

Results

Comment

J Thorac Cardiovasc Surg

Ann Thorac Surg

Ann Thorac Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

Total anomalous pulmonary venous connection

Am Heart J

Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection

Circulation

Original article
Pediatric cardiac
Repair of “Simple” Total Anomalous Pulmonary Venous Connection: A Review From the Pediatric Cardiac Care Consortium