Total Anomalous Pulmonary Venous Connection: A 40 years' Experience Analysis
23 May 2016
15 July 2016
16 September 2016 (online)
Background Total anomalous pulmonary venous connection is a rare cardiac malformation associated with significant morbidity and mortality rates. We report a large surgical series study to evaluate mid-term and long-term results of conventional surgical techniques.
Methods and Results We performed a retrospective analytic study of all patients operated on for simple total anomalous pulmonary venous connection in the University Hospital of Lyon, France, between January 1973 and June 2014. A total of 180 patients were included (43% supracardiac, 27% intracardiac, 19% infracardiac, and 11% mixed types). Mean cardiopulmonary bypass and aortic cross clamp times were respectively 66 and 39 minutes. Overall mortality was 27.1%, including 38 early deaths (21.1%) and 12 late deaths (6.1%). The percentage of early death greatly decreased over the eras, from 42.1% in the seventies to 7.4% after 2010. Besides the earlier era of intervention (p < 0.0001), significant risk factors for death in multivariate analysis were preoperative pulmonary hypertension, acidosis, and cardiopulmonary bypass time. There were 24 reoperations, including 7 for pulmonary venous obstruction; 6 died. Factors directly and independently associated with late complications were the anatomic type (mixed forms, p = 0.0023), and length of aortic cross clamp time (p = 0.01). Long-term results for survivals are excellent. We report 84.7% of asymptomatic patients with a mean follow-up of 10.8 years.
Conclusions The overall prognosis of total anomalous pulmonary venous connection repair with conventional procedures has greatly improved over the years with excellent long-term results. A thorough evaluation of all preoperative characteristics is imperative to achieve the best outcome.
- 1 Hlavacek AM, Shirali GS, Anderson RH. Pulmonary Venous Abnormalities. Paediatric Cardiology. 3rd ed. Philadelphia, PA: Churchill Livingstone, Elsevier; 2010. 24: 497-522
- 2 Lewis FJ, Varco RL, Taufic M, Niazi SA. Direct vision repair of triatrial heart and total anomalous pulmonary venous drainage. Surg Gynecol Obstet 1956; 102 (6) 713-720
- 3 Craig JM, Darling RC, Rothney WB. Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies. Lab Invest 1957; 6 (1) 44-64
- 4 Hancock Friesen CL, Zurakowski D, Thiagarajan RR , et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg 2005; 79 (2) 596-606 , discussion 596–606
- 5 Seale AN, Uemura H, Webber SA , et al; British Congenital Cardiac Association. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation 2010; 122 (25) 2718-2726
- 6 Yong MS, d'Udekem Y, Robertson T , et al. Outcomes of surgery for simple total anomalous pulmonary venous drainage in neonates. Ann Thorac Surg 2011; 91 (6) 1921-1927
- 7 Karamlou T, Gurofsky R, Al Sukhni E , et al. Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection. Circulation 2007; 115 (12) 1591-1598
- 8 Kelle AM, Backer CL, Gossett JG, Kaushal S, Mavroudis C. Total anomalous pulmonary venous connection: results of surgical repair of 100 patients at a single institution. J Thorac Cardiovasc Surg 2010; 139 (6) 1387-1394.e3
- 9 Talwar S, Agarwala S, Mittal CM, Choudhary SK, Airan B. Diaphragmatic palsy after cardiac surgical procedures in patients with congenital heart. Ann Pediatr Cardiol 2010; 3 (1) 50-57
- 10 Zhang YB, Wang X, Li SJ, Yang KM, Sheng XD, Yan J. Postoperative diaphragmatic paralysis after cardiac surgery in children: incidence, diagnosis and surgical management. Chin Med J (Engl) 2013; 126 (21) 4083-4087
- 11 Akay TH, Ozkan S, Gultekin B , et al. Diaphragmatic paralysis after cardiac surgery in children: incidence, prognosis and surgical management. Pediatr Surg Int 2006; 22 (4) 341-346
- 12 Joho-Arreola AL, Bauersfeld U, Stauffer UG, Baenziger O, Bernet V. Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children. Eur J Cardiothorac Surg 2005; 27 (1) 53-57
- 13 Brunner N, de Jesus Perez VA, Richter A , et al. Perioperative pharmacological management of pulmonary hypertensive crisis during congenital heart surgery. Pulm Circ 2014; 4 (1) 10-24
- 14 Ando M, Takahashi Y, Kikuchi T. Total anomalous pulmonary venous connection with dysmorphic pulmonary vein: a risk for postoperative pulmonary venous obstruction. Interact Cardiovasc Thorac Surg 2004; 3 (4) 557-561
- 15 Hyde JA, Stümper O, Barth MJ , et al. Total anomalous pulmonary venous connection: outcome of surgical correction and management of recurrent venous obstruction. Eur J Cardiothorac Surg 1999; 15 (6) 735-740 , discussion 740–741
- 16 Whight CM, Barratt-Boyes BG, Calder AL, Neutze JM, Brandt PW. Total anomalous pulmonary venous connection. Long-term results following repair in infancy. J Thorac Cardiovasc Surg 1978; 75 (1) 52-63