Mixed total pulmonary venous drainage: Still a surgical challenge

The Annals of Thoracic Surgery, 1998

Background. Progressive stenosis of the pulmonary veins after repair of total anomalous pulmonary venous drainage is frequently refractory to surgical therapy.

The Cardiothoracic Surgeon

Background Surgical repair of total anomalous venous drainage (TAPVD) is lifesaving. The operative mortality is reported between 4 and 35%. Anatomical type, obstructed presentation, associated single ventricle, and heterotaxy syndromes are thought to influence short- and long-term outcomes. The effect of simple versus sutureless repair for primary surgery is unclear. This study reports the outcomes of the surgical repair and the effect of these variables in a contemporary setting. Results Between 2011 and 2019, all patients undergoing surgical repair for TAPVD were included. Operative mortality, length of hospital stay, and long-term survival were reported. The effect of anatomical type, surgical technique, obstruction, and associated lesions was assessed. Pearson’s test, Wilcoxson’s test, and generalized linear regression with Poisson distribution were used. Forty-nine patients from two centers underwent TAPVD repair. The operative mortality was 4%. Postoperative pulmonary vein ste...

Annals of Thoracic Surgery, 2008

pathway and effectively break up the previously mentioned vicious circle. We believe that this approach has never been reported in literature and should always be kept in mind in these complex vascular obstructions, mainly in low-weight patients. However, only a close follow-up will disclose us the long-term outcome of these vessels, as well as the need of further surgical or percutaneous interventions as the infant grows. Nevertheless, the use of stents potentially re-dilatable up to the adult pulmonary artery size, as was in this patient, should allow additional nonsurgical dilatations whenever the clinical conditions warranted them.

Annals of Surgery, 1969

Pediatric Cardiology, 1985

A total of 20 children with total anomalous pulmonary venous drainage (TAPVD) underwent complete repair within the past six years. The drainage was supracardiac in 11, infracardiac in seven, and into the coronary sinus in two. At repair mean age was nine weeks, and weight was 3 .7 km. Operative technique in extracardiac types included complete mobilization of the common vein with division of anomalous channel when possible. The incision in the confluent vein was extended into the lobar veins when necessary to permit extensive incorporation of this structure into the posterior wall of the left atrium and resulted in a nonobstructing stellate-type anastomosis. Operative mortality was 10% (2 of 20). Deaths occurred only in the group of infants in whom severe obstruction to pulmonary venous return required emergency operation, and one of these patients has persistent neurologic deficit. Late cardiac catheterization has been performed in 11 of 18 survivors. Nine had no or only minor abnormalities and two required reoperation. There have been no late deaths with follow-up of 2-8 years (mean = 4 years). Currently, all of the survivors are without cardiac symptoms and only one requires cardiac medication. Our experience identifies pulmonary venous obstruction with critical symptoms as the major operative risk factor in patients with TAPVD. With early operation prior to onset of critical symptoms, mortality is low and functional results are excellent .

Seminars in Thoracic and Cardiovascular Surgery, 2017

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon cardiac defect in children. The mixed subset accounts for 5%-10% of the TAPVD and is variable in its anatomy. The outcomes associated with this subset of patients are rarely reported. A retrospective review of all patients with mixed TAPVD undergoing repair at a single institution (1984-2014) was conducted. A descriptive analysis was performed. Twenty-four patients underwent repair for mixed TAPVD (6 univentricular physiology, 18 biventricular physiology). The mixed TAPVD anatomy included 8 patients in group I (2 + 2 veins), 11 patients in group II (3 + 1 veins), and 5 patients in group III (atypical). Preoperative pulmonary venous obstruction occurred in 8 patients (33%). The median age at repair was 2.2 months (range 2 days to 3 years) and median weight was 4.2 kg (range 1.9 to 12.5 kg). Operative mortality was 13% (3 of 24), 33.3% (2 of 6) for patients with univentricular physiology, and 5.6% (1 of 18) for patients with biventricular physiology. There have been no operative deaths in the biventricular group since 1997 (n = 11). Survival at 30-days was 83% ± 15% (95% confidence interval: 27%-97%) and 94% ± 5% (95% confidence interval: 67%-99%) for the univentricular and biventricular groups, respectively. Reoperation for recurrent pulmonary venous obstruction was required in 2 patients (8.3%) where the sutureless technique was used. The average followup after surgery was 9.3 ± 6.4 years (5 months to 21 years), and all surviving patients were asymptomatic. Mixed TAPVD can be repaired with good results in children, particularly in those undergoing biventricular repair.

Srpski arhiv za celokupno lekarstvo, 2014

Introduction. Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease and in some variants represents the only true surgical emergency in congenital heart surgery. Basic anatomical characteristic of this anomaly is an abnormal connection of pulmonary veins with systemic venous circulation. Although the results of TAPVC repair in infancy have been markedly improved in recent years, the recurrent pulmonary venous obstruction (RPVO) remains relatively frequent complication of surgical treatment. Objective. The aim of this study was a retrospective evaluation of TAPVC repair at a single institution, identifying the risk factors associated with the increased mortality and morbidity. Methods. Between January 2001 and January 2010, 43 consecutive patients underwent repair of TAPVC at the University Children?s Hospital, with median weight of 3.8 kg (1.8-13 kg). Median age at surgery varied from 5 days to 5 years. Distribution of TAPVC types was as follows: sup...

Journal of Cardiac Surgery, 2012

Background: This retrospective study evaluated factors associated with mortality and morbidity in patients having functionally biventricular heart (FBH) and functionally univentricular heart (FUH) undergoing total anomalous pulmonary venous connection (TAPVC) repair. Methods: We reviewed medical records retrospectively and identified 72 patients undergoing TAPVC repair between July 2002 and December 2010; and 62 patients had FBH and 10 patients had FUH. The median age and weight at repair was 9.1 months (range, 8 days-16 years) and 6.7 kg (range, 2.5-57 kg). The TAPVC anatomy was supracardiac in 41 (56.9%), cardiac in 18 (25%), infracardiac in eight (11.1%), and mixed in five patients (6.9%). Results: There were seven early deaths (2 FBH [25%] and 5 FUH [62.5%]) and one late death (FUH). Presence of FUH (p < 0.01), heterotaxy syndrome (p = 0.03), young age (p < 0.01), low weight (p < 0.01) and long CPB time (p = 0.04) at the time of surgery were statistically significant risk factors for mortality. Morbidity was related to long duration of postoperative mechanical ventilation, intensive care unit and hospital stay. Presence of FUH was the only significant risk factor for morbidity (p < 0.01). After a mean follow-up of 52.1 months (range, 3-74 months), 59 (92%) of 64 survivors were asymptomatic without any medication, and five have mild symptoms with regular physical activity. Conclusion: Operative results of TAPVC have dramatically improved in recent years. However, patients having FUH (especially with concomitant palliative surgery), heterotaxy syndrome, low weight, early age, and long CPB time still have an increased operative mortality.

Annals of Thoracic Surgery, 1995

Background. Congenital pulmonary vein stenosis is a rare cause of obstruction of pulmonary venous blood flow with a high mortality. Acquired pulmonary vein stenosis is an equally serious condition.

African Journal of Paediatric Surgery, 2013