Repair Of Total Anomalous Pulmonary Venous Drainage Research Articles

Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival

BackgroundMortality after repair of total anomalous pulmonary venous drainage (TAPVD) in neonates has remained high. Analysis of risk factors may help identify therapeutic targets to improve survival. MethodsRetrospective analysis of all neonates who underwent simple TAPVD repair. ResultsBetween 1973 and 2021, 175 neonates underwent TAPVD repair, at a median age of 6 days (interquartile range, 2-15 days) and a mean weight of 3.2 ± 0.6 kg. TAPVD was supracardiac in 42.3% of the patients (74 of 175), cardiac in 14.3% (25 of 175), infracardiac in 40% (70 of 175), and mixed type in 3.4% (6 of 175), with obstruction in 65.7% (115 of 175). Pulmonary hypertension (PHT) crisis occurred in 12% (21 of 175). Early mortality was 9.7% (17 of 175) and late mortality was 5.1% (8 of 158), with most deaths occurring within 1 year (75%; 6 of 8). Survival was 86.5% (95% CI, 80.3%-90.8%) at 1 year and 85.8% (95% CI, 79.6%-90.3%) at 5, 10, 15, and 20 years. Survival was lower in patients with obstructed TAPVD, patients with emergent surgery, and those with PHT crisis. PHT crisis (hazard ratio [HR], 4.93; 95% CI, 1.95-12.51; P = .001), urgency of surgery (HR, 2.51; 95% CI, 1.11-5.68; P = .027), and higher pulmonary artery pressure–to–systemic blood pressure percentage ratio (HR, 1.06; 95% CI, 1.01-1.11; P = .026) were identified as risk factors for mortality. Histopathological analysis of 17 patients (9.7%; 17 of 175) showed signs of pulmonary arterial hypertension with media hypertrophy in 58.8% (10 of 17). ConclusionsMortality after TAPVD repair occurred mainly within the first year of life. Urgency of surgery and persistent PHT appears to be risk factors for mortality. Lung biopsy might be useful for identifying patients at risk and guiding newer treatment modalities.

Long-term Outcomes of the Fontan Operation in Patients With Total Anomalous Pulmonary Venous Drainage

Few patients with total anomalous pulmonary venous drainage (TAPVD) and a univentricular circulation survive to Fontan completion. Hence, we sought to determine the long-term outcomes of the Fontan operation in patients with TAPVD. Patients with TAPVD who underwent the Fontan operation and survived to hospital discharge in Australia and New Zealand between 1985 to 2017 wereidentified (n= 54) from a binational Fontan registry. Thirty-two patients (60%) underwent repair of TAPVD at a median age of 0.8 (interquartile range: 0.3-1.6) years. Thirty-seven patients (69%) had heterotaxy. The median age at time of Fontan operation was 5.7 years. There were 4 late deaths and 3 patients required cardiac transplantation for a failing Fontan circulation. On univariate analysis, the concomitant diagnosis of pulmonary stenosis and right ventricular dominance was associated with late death or transplantation (P= .04). Freedom from late death or transplantation at 15 years after the Fontan operation was 88% ± 7% (95% confidence interval [CI], 67%-96%) for the repaired TAPVD group and 90% ± 6% (95% CI, 67%-98%) for the unrepaired TAPVD group (P= .47). Median follow-up after the Fontan procedure was 10.8 (interquartile range, 6.7-16.2) years. The majority of survivors (94%) were in New York Heart Association functional class I or II. The 15-year freedom from death or transplantation was similar for patients with TAPVD (89% ± 5%; 95% CI, 76%-95%) compared with patients without TAPVD in the Fontan registry (n= 1446; 92% ± 1%; 95% CI, 90%-93%) (P= .12). Long-term survival of patients with TAPVD who undergo the Fontan operation and survived to hospital discharge is comparable to Fontan survivors without TAPVD.

Long-Term Outcomes of Total Anomalous Pulmonary Venous Drainage Repair in Neonates and Infants

Outcomes of operations for total anomalous pulmonary venous drainage (TAPVD) have improved. However, operations in the neonatal period and the development of postoperative pulmonary venous obstruction are associated with a high mortality rate. A retrospective review was conducted for all neonates and infants (n= 214) who underwent operations for isolated TAPVD (1973 to 2014). Median age was 18 days (1 day to 1 year). There were 17 (7.9%) early deaths. Risk factors for early death were prolonged cardiopulmonary bypass time (p= 0.005) and neonatal age at the operation (p= 0.048). Early mortality was 2.5% for infants (n= 81) and 11% for neonates (n= 133; p= 0.021) during the entire study period. Hospital deaths for neonates remained unchanged during the four eras of 1973 to 1988, 1989 to 1998, 1999 to 2008, and 2009 to 2014. Survival at 10 and 20 years was 88% ± 2.2% (95% confidence interval, 82% to 91%). Reoperation for postoperative pulmonary venous obstruction was required in 22 patients (10%). Risk factors for reoperation were prolonged cardiopulmonary bypass time (p= 0.015), lower operative weight (p= 0.003), and an episode of postoperative pulmonary hypertensive crisis (p= 0.005). Freedom from reoperation at 20 years was 86% ± 3.2% (95% confidence interval, 78% to 91%). All survivors were asymptomatic at a mean of 13 ± 9 years (range, 1 month to 42 years) after the operation. Although isolated TAPVD repair in infants can be performed without death, the operation is associated with a high mortality rate in neonates that remained unchanged during the long study period. Survival beyond 1 year after the operation is associated with excellent long-term outcomes.

Repair Type Influences Mode of Pulmonary Vein Stenosis in Total Anomalous Pulmonary Venous Drainage

We hypothesized that primary sutureless (SL) repair of total anomalous pulmonary venous drainage (TAPVD) may have a lower incidence of postrepair pulmonary vein obstruction (PVO) and different modes of PVO compared with standard repair (SR). One hundred ninety-five patients who underwent TAPVD repair (1990 to 2012) with the exception of congenital pulmonary vein stenosis, isomerism, and single-ventricle anomalies were included. Survival, reintervention, incidence, degree of PVO were compared between groups. The mode of PVO was expressed as central or peripheral. The Mann-Whitney test, Kaplan-Meier analysis, and Cox regression were used. The SL group had more infracardiac or mixed TAPVD (p= 0.02) and preoperative PVO (p= 0.07). There were no differences between SR and SL groups in survival (5-year survival, 83.1% versus 82.5%, respectively; p= 0.73) and composite outcome (death, intervention, PVO, 5-year survival, 76.4% versus 80.7%, respectively; p= 0.225). The SL group had a lower incidence of PVO of moderate or greater degree (SR, 11.3% versus SL, 2.9%; p= 0.05) than the SR group, especially in the infracardiac and mixed TAPVD cohort (p= 0.011), with a lower pulmonary vein score (SR, 8 versus SL, 4; p= 0.01). The SL group had peripheral PVO exclusively (100%), whereas the SR group predominantly had central PVO (76.4%; p= 0.005). There was a trend toward less reoperation in the SL group (SR, 10.4% versus SL, 2.9%; p= 0.08). Survival after reoperation was comparable to primary TAPVD repair types as well as reoperation repair types. Primary SL appeared to be associated with a lower incidence and severity of PVO. The primary SL repair eliminated the risk of developing central PVO, although a relatively benign type of peripheral PVO could occur.

THE INCIDENCE AND NATURAL HISTORY OF MITRAL REGURGITATION FOLLOWING SURGICAL REPAIR OF TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE

Upon repair of total anomalous pulmonary venous drainage (TAPVD) in children, the left-sided structures face a sudden increase in preload that may alter mitral valve function. We sought to determine the natural history of mitral regurgitation (MR) following TAPVD repair. 97 patients who underwent

Unusual Causes of Late Right Heart Dilatation Long After Repair of Total Anomalous Pulmonary Venous Drainage

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon congenital cardiac condition in which there is no connection between the pulmonary veins (PVs) and the left atrium (LA). The PVs form a confluence behind the LA that usually drains to a systemic vein such as the left innominate or right superior vena cava (supracardiac TAPVD), the portal venous system (infracardiac TAPVD), or the coronary sinus. TAPVD is treated surgically by creating an anastomosis between the pulmonary venous confluence and the LA, closing the (obligatory) atrial septal defect (ASD), and ligating any connection between the pulmonary and systemic veins. The most common reported long-term complication of TAPVD repair is pulmonary venous obstruction, which requires reoperation in 12% to 15% of cases. We present 2 patients in whom unusual causes of persistent right heart dilation were discovered many years after TAPVD repair. The first patient underwent neonatal repair of supracardiac TAPVD. At the operation, the pulmonary venous confluence was found to drain via a large vein into the superior vena cava. A routine transthoracic echocardiogram performed 14 years after repair showed dilatation of the right ventricle (RV). There was minimal tricuspid regurgitation, and it was not possible to reliably estimate pulmonary artery pressure. At transesophageal echocardiography, only the right upper and left upper PVs were identified draining into the pulmonary venous confluence, but no cause of RV dilatation was positively identified. At cardiac catheterization, drainage of the right lower PV to the portal system was suspected. A subsequent cardiac magnetic resonance imaging scan demonstrated that the superior right and left PVs drained to the LA, a vertical vein connected the pulmonary venous confluence to the portal vein, and the inferior right and left PVs drained to the vertical vein (Figure 1 and Movie I in the online-only Data Supplement). The RV end-diastolic volume was 128 mL/m 2 body surface area (upper limit of normal, 110 mL/m 2 ), 1 and the estimated ratio of pulmonary to systemic flow (Qp:Qs) was 1.3. In ≈10% of TAPVD cases, there are pulmonary venous connections to >1 site. In this case, the infracardiac connection, which originally coexisted with the supracardiac connection, had not been recognized at the original operation. Infracardiac connections through the portal system generally present higher resistance to flow than supracardiac connections, and it is likely that at the time of surgery there was relatively little infracardiac flow. Nevertheless, the persistent infracardiac connection provided a route for continued left-to-right shunting and consequent right heart dilatation. The RV dilatation resolved after device occlusion of the vertical vein (Movie II in the online-only Data Supplement). The second patient underwent repair of supracardiac TAPVD, in which the pulmonary venous confluence was connected to the innominate vein at 1 week of age. After a period of loss to follow-up and 2 uneventful pregnancies, routine echocardiography at 22 years of age showed RV dilatation, a possible residual ASD, and an apparent structure descending from the LA. There was mild tricuspid regurgitation, and the estimated pulmonary artery systolic pressure was 30 mmg Hg to 35 mm Hg. To clarify the anatomy, a cardiac magnetic resonance imaging scan was performed. This

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Invited Commentary

Hemodynamic Index for Risk Stratification After Neonatal Total Anomalous Pulmonary Venous Drainage Repair

Repair of total anomalous pulmonary venous drainage (TAPVD) in neonates remains a challenge. It is associated with a high mortality. We aimed at determining a method for risk stratification of this group of patients. From 1994 to 2008, 54 patients underwent simple TAPVD operations during the first month of life. Mean pulmonary arterial pressure (PAP), mean systemic arterial pressure (MAP), systolic blood pressure, diastolic blood pressure, central venous pressure, and left atrial pressure were recorded in 44 of the 54 patients for the first 36 hours postoperatively. The remaining 10 patients were excluded because data from invasive pressure monitoring were not available. There were overall 8 deaths (18.2%, 8/44), including 4 (9%, 4/44) early deaths, and 5 reoperations (11.4%, 5/44). The mean PAP was 23.1 ± 6.4 mm Hg, the mean MAP was 50.3 ± 5 mm Hg, and the PAP-to MAP-ratio (PAP/MAP) was 0.80 ± 0.36. By multivariable logistic analysis, the risk factors for mortality were a higher PAP/MAP (p = 0.037) and lower operative weight (p = 0.02). All deaths had either a PAP/MAP of greater than 0.80 or an operative weight of less than 2.5 kg. Hemodynamic index (PAP/MAP divided by operative weight) was predictive of mortality (p = 0.007). Furthermore, the hemodynamic index (p = 0.003) predicted prolonged length of stay in the intensive care unit by regression analysis. The hemodynamic index (PAP/MAP/weight) ≥0.25 in the first 36 hours after TAPVD repair in neonates is predictive of mortality. A higher index predicted longer stay in the intensive care unit. This hemodynamic index may be a useful adjunct for risk stratification in neonates undergoing TAPVD repair.

Outcomes of Surgery for Simple Total Anomalous Pulmonary Venous Drainage in Neonates

Repair of total anomalous pulmonary venous drainage (TAPVD) in neonates remains a challenge as it is often associated with severe obstruction. We describe a large cohort of neonates who underwent TAPVD repair at a single institution. From 1973 to 2008, 112 patients underwent simple TAPVD surgery during the first month of life. Data collection occurred retrospectively. Preoperative pulmonary venous obstruction (PVO) occurred in 89 (79.5%) patients. There were 12 (10.7%) early deaths. Significant risk factors were bypass time greater than 65 minutes (p=0.014) and emergent surgery (p=0.005). Hospital mortality was unchanged throughout the 3 eras (1973 to 1988, 1989 to 1998, 1999 to 2008), despite an increase in patients with preoperative acidosis (p=0.004) and severe TAPVD obstruction (p=0.038) during the recent 10 years. There were 6 (6.25%) late deaths within 2 years of repair. Survival at 20 years was 83.4% (95% confidence interval 75 to 89). Risk factors for late death were operative weight 2.5 kg or less (p=0.004) and postoperative pulmonary hypertensive crisis (p=0.02). Reoperation for recurrent PVO was required in 13 patients (11.9%). Risk factors were operative weight 2.5 kg or less (p=0.035) and postoperative pulmonary hypertensive crisis (p=0.002). Follow-up was 96% complete and survivors (n=90) were asymptomatic at a median age of 11.7 years. Hospital mortality remained unchanged over the 36-year period. Survival beyond 2 years offers excellent outcome. Risk factors for mortality were the preoperative clinical status, prolonged bypass time, persisting micro-obstruction, and low operative weight. A reduction in mortality will likely require development of effective medical management for patients who have peripheral PVO not amenable to surgical repair.

Hybrid palliation for right atrial isomerism associated with obstructive total anomalous pulmonary venous drainage

A twenty-four-day-old girl, who was prematurely born at 36 weeks of gestation, and weighed 2.2 kg, and diagnosed with right atrial isomerism, functionally single ventricle, bilateral superior vena cava (SVC) and obstructive supracardiac total anomalous pulmonary venous drainage (TAPVD) draining to the junction between the right SVC and the right atrium, underwent a hybrid procedure in the operating room, which consisted of pulmonary artery banding, ductus ligation and stenting of the draining vein of TAPVD. Obstruction at the drainage site of TAPVD was initially relieved after stenting, but, one month after the procedure, the distal end of the stent became stenotic and she received bilateral sutureless repair of TAPVD. At postoperative seven months, she underwent bidirectional cavopulmonary shunt uneventfully, and she has been followed-up for two months in a stable state without any problem in the pulmonary venous pathway.

Early and long-term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants

To present our 17-year experience of surgical repair of total anomalous pulmonary venous drainage (TAPVD) in 71 consecutive neonates and infants, with particular emphasis on the role of preoperative pulmonary venous obstruction (PVO), the management of postoperative pulmonary hypertensive crises and the long-term results. From July 1977 to May 1994, 36 newborns and 35 infants, aged between 1 and 300 days, had repair TAPVD in our Department. Fifty-four patients had decompensated heart failure, whereas three were moribund. The anomalous drainage was supracardiac in 32 (45%), cardiac in 17 (24%), infracardiac in 17 (24%) and mixed in 5 infants (7%). Major associated cardiac anomalies coexisted in nine cases. Forty-five (63%) exhibited signs of PVO. The repair was performed under moderate hypothermia in 15 and deep hypothermic arrest in 56 patients. In case of postoperative pulmonary hypertensive crises, artificial hyperventilation with high oxygen concentration, sedation and relaxation, inotropic support and afterload reduction with phentolamine were applied selectively. The actual follow-up is based on functional symptomatology and cardiac echo-doppler studies. Six children (8.5%) died early: four perioperatively, two on the 2nd and 10th postoperative day, respectively. Poor preoperative condition, operation before 1987 and preoperative PVO were the main incremental risk factors for death. No death occurred among the last 38 cases. There were complications in 45 patients, in particular pulmonary hypertensive crises in 27. Two patients died late, after 80 and 118 days, respectively, of progressive intrapulmonary vein fibrosis: the second despite reoperation. The risk of late death approaches zero after 6 months. Among the 63 survivors, 56 (86%) are asymptomatic and without medication, 3 months-17 years after operation. Five have mild symptoms with ordinary physical activity. Two are lost to follow-up. Early repair of TAPVD with aggressive management of pulmonary hypertensive crises carries low operative mortality nowadays. Preoperative PVO as a risk factor has been neutralized since 1987. Long-term results are gratifying: no late death after 6 months, no reoperation and functional good results. Progressive pulmonary vein fibrosis remains an unpredictable rare cause of death within the 1st year after surgery.

Total anomalous pulmonary venous drainage in newborns with visceral heterotaxy

Children with visceral heterotaxy often present with total anomalous pulmonary venous drainage (TAPVD) associated with univentricular congenital heart disease. We reviewed our experience with the primary surgical management of this lesion under these circumstances. Over a recent 10-year span, 38 patients within the first 3 days of life were admitted to our institution and underwent primary palliation. Twenty-one of them had TAPVD, 18 to a systemic vein. Twelve (67%) of these 18 were seen with obstruction of the anomalous connection and underwent emergency operation. In 7 patients, repair of TAPVD was combined with a systemic-pulmonary artery shunt because of additional obstruction of the pulmonary blood supply, with two deaths. One patient had primary shunting and then repair of TAPVD. Four patients underwent repair of TAPVD alone. Two of them then showed signs of insufficient pulmonary blood flow, received a shunt in a second procedure, and subsequently died. Early mortality in the group with obstructed TAPVD was thus 4 (33%) of 12 patients. Statistical analysis of all 38 patients (univariate analysis, x 2 testing) showed that neither the presence of TAPVD ( p = 0.7) nor TAPVD repair alone ( p = 0.8) or with shunting ( p = 0.8) was a definite risk factor for early death. The performance of a shunt during the first operation, however, was associated with lower early mortality ( p = 0.03). Total anomalous pulmonary venous drainage is a common finding in newborns with visceral heterotaxy. Its presence and its subsequent early repair (requiring cardiopulmonary bypass) do not increase the mortality risk. The need of a concomitant shunt in obstructed TAPVD can initially be underestimated.

Surgical Management of Infants with Complex Cardiac Anomalies Associated with Reduced Pulmonary Blood Flow and Total Anomalous Pulmonary Venous Drainage

Eight infants with complex cardiac anomalies and pulmonary stenosis or atresia were noted to have obstructed total anomalous pulmonary venous drainage (TAPVD) either at the initial cardiac catheterization (Group 1; n = 2) or after creation of systemic-pulmonary artery shunts (Group 2; n = 6). The 2 patients in Group 1 underwent early repair of TAPVD (1 at 7 days, the other at 1½ months of age) before any subsequent operation and are now doing well at 18 months of age. The 6 patients in Group 2 underwent repeat cardiac catheterization because of persistent severe cyanosis with faint or absent continuous murmur and were found to have patent shunts and obstructed TAPVD (1 mild, 5 severe). One patient who underwent repair of TAPVD at 2½ months of age survived and is well at 2 years of age, whereas 4 patients who underwent repair at an average age of 6 months (age range, 3–16 months) subsequently died. The sixth patient, who did not undergo repair, remained severely cyanotic with hypoplastic pulmonary arteries in spite of repeated shunts. We feel that increased awareness of the possible association of TAPVD and reduced pulmonary blood flow in infants with complex cardiac defects, in combination with echocardiography, oxygen saturation studies, and angiography with prostaglandin E 1 challenge, should lead to early diagnosis, avoidance of unnecessary systemic-pulmonary artery shunts, and increased survival rates in these infants.