© 2014 Wiley Periodicals, Inc.
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ORIGINAL ARTICLE _____________________________________________________________
Surgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair: A 22-Year Experience Massimo A. Padalino, M.D., Ph.D.,* Giacomo Cavalli, M.D.,* Marco De Franceschi, R.N.,* Daniela Mancuso, M.D., Ph.D.,y Nicola Maschietto, M.D., Ph.D.,z Vladimiro Vida, M.D., Ph.D.,* Ornella Milanesi, M.D.,z and Giovanni Stellin, M.D.* *Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy; yDivision of Cardiology, Public Health Hospital, Cittadella, Italy; and zPediatric Cardiology, Department of Women and Child’s Health, University of Padova Medical School, Padova, Italy ABSTRACT Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD), whose surgical repair is associated with high mortality and reoperation rates. We sought to identify predictors of early and late outcomes. Methods: Data from medical records of patients who underwent surgical repair for TAPVC from 1989 to 2012 were included. The patients were divided in two groups, according to absence or presence of associated major CHDs. Results: Forty-six patients were included (M/F: 26/20, median age 26 days, interquartile range 15 to 59, median weight 3.350 kg, interquartile range 1800 to 4470). Anatomic types of TAPVC were: supracardiac in 48%, intracardiac in 20%, infracardiac in 20%, and mixed in 12%; TAPVC was obstructive in 33%; TAPVC was isolated in 63%, complex in 37%. Single ventricle physiology was present in 11 patients, heterotaxy in eight patients. Overall operative mortality was 19.6% (9/46): 6.9% in isolated TAPVC, 41.2% in complex type (p-value: 0.002). It was associated with low weight at intervention (60 minutes (p = 0.097). At a median follow-up of 2.97 years (range 43 days to 22 years, 91% complete), there were nine late deaths (24.3%); 15 patients (40.5%) had major events (including late death). Multivariate analysis of event-free survival showed worse outcome in the complex group (p = 0.001). Conclusion: Surgical mortality and morbidity remain consistent in patients with TAPVC and associated major CHD, while the isolated type shows excellent outcomes. Pulmonary vein obstruction is a rare but highly lethal complication.
doi: 10.1111/jocs.12399 (J Card Surg 2014;29:678–685) Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD) occurring in 1% to 3% of neonates with CHD.1–3 Without surgical repair, TAPVC leads to greater than 78% mortality during the first year.3 Surgical outcomes of TAPVC repair have improved over the years, thanks to better diagnosis and surgical techniques.1–4 However, surgical repair does remain a challenge with consistent early mortality.1–4 In particular, TAPVC and
uni-ventricular heart (UVH) physiology or heterotaxy syndrome is associated with worse outcomes when compared to infants with isolated TAPVC with biventricular (BV) anatomy.1,4 We reviewed the institutional experience of more than two decades in order to assess our clinical early and late outcomes, and to outline the main predictors for surgical mortality and late outcomes.
MATERIAL AND METHODS Conflict of interest: The authors acknowledge no conflict of interest in the submission. Disclosures: none. Address for correspondence: Massimo A. Padalino, M.D., Ph.D., UOC Cardiochirurgia Pediatrica e Cardiopatie Congenite, Dipartimento di Scienze Cardiache, Toraciche e Vascolari Centro ‘‘Vincenzo Gallucci,’’ Via Giustiniani 2, 35128 Padova, Italy. Fax: þ39-049-8212409; e-mail: [email protected]
This is a retrospective surgical series including all consecutive patients undergoing TAPVC surgical repair between January 1st, 1989 and January 31st, 2012. Review of medical records was approved by our local hospital committee on clinical investigation. Individual patients were not identified, and the need for patient consent was waived. Clinical and echocardiographic
J CARD SURG 2014;29:678–685
PADALINO, ET AL. SURGERY FOR TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION
data were collected according to our institutional database. Patients were included when there was at least one-month follow-up echocardiogram available. For the purpose of data analysis, we arbitrarily divided all patients into two groups: Group 1, or isolated TAPVC, included those patients with no CHD other than a patent ductus arteriosus (PDA), a patent foramen ovale (PFO), or an atrial septal defect (ASD); Group 2, or Complex TAPVC, included all patients with other associated major CHD, such as common atrioventricular septal defect (AVSD), double outlet right ventricle (DORV), hypoplastic left heart syndrome (HLHS), functional UVH, Scimitar Syndrome (SS) associated with TAPVC, and heterotaxy syndrome. Various types of surgical repair were utilized, according to surgeons’ preference, as described elsewhere:1 direct anastomosis, anastomosis with right transatrial enlargement, modified ‘‘Van Praagh’’ procedure,5 and atrial reseptation with direct anastomosis (used in mixed type of TAPVC). Preoperative, intraoperative and postoperative variables, such as age, weight at operation
678
ORIGINAL ARTICLE _____________________________________________________________
Surgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair: A 22-Year Experience Massimo A. Padalino, M.D., Ph.D.,* Giacomo Cavalli, M.D.,* Marco De Franceschi, R.N.,* Daniela Mancuso, M.D., Ph.D.,y Nicola Maschietto, M.D., Ph.D.,z Vladimiro Vida, M.D., Ph.D.,* Ornella Milanesi, M.D.,z and Giovanni Stellin, M.D.* *Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova Medical School, Padova, Italy; yDivision of Cardiology, Public Health Hospital, Cittadella, Italy; and zPediatric Cardiology, Department of Women and Child’s Health, University of Padova Medical School, Padova, Italy ABSTRACT Background: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD), whose surgical repair is associated with high mortality and reoperation rates. We sought to identify predictors of early and late outcomes. Methods: Data from medical records of patients who underwent surgical repair for TAPVC from 1989 to 2012 were included. The patients were divided in two groups, according to absence or presence of associated major CHDs. Results: Forty-six patients were included (M/F: 26/20, median age 26 days, interquartile range 15 to 59, median weight 3.350 kg, interquartile range 1800 to 4470). Anatomic types of TAPVC were: supracardiac in 48%, intracardiac in 20%, infracardiac in 20%, and mixed in 12%; TAPVC was obstructive in 33%; TAPVC was isolated in 63%, complex in 37%. Single ventricle physiology was present in 11 patients, heterotaxy in eight patients. Overall operative mortality was 19.6% (9/46): 6.9% in isolated TAPVC, 41.2% in complex type (p-value: 0.002). It was associated with low weight at intervention (60 minutes (p = 0.097). At a median follow-up of 2.97 years (range 43 days to 22 years, 91% complete), there were nine late deaths (24.3%); 15 patients (40.5%) had major events (including late death). Multivariate analysis of event-free survival showed worse outcome in the complex group (p = 0.001). Conclusion: Surgical mortality and morbidity remain consistent in patients with TAPVC and associated major CHD, while the isolated type shows excellent outcomes. Pulmonary vein obstruction is a rare but highly lethal complication.
doi: 10.1111/jocs.12399 (J Card Surg 2014;29:678–685) Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease (CHD) occurring in 1% to 3% of neonates with CHD.1–3 Without surgical repair, TAPVC leads to greater than 78% mortality during the first year.3 Surgical outcomes of TAPVC repair have improved over the years, thanks to better diagnosis and surgical techniques.1–4 However, surgical repair does remain a challenge with consistent early mortality.1–4 In particular, TAPVC and
uni-ventricular heart (UVH) physiology or heterotaxy syndrome is associated with worse outcomes when compared to infants with isolated TAPVC with biventricular (BV) anatomy.1,4 We reviewed the institutional experience of more than two decades in order to assess our clinical early and late outcomes, and to outline the main predictors for surgical mortality and late outcomes.
MATERIAL AND METHODS Conflict of interest: The authors acknowledge no conflict of interest in the submission. Disclosures: none. Address for correspondence: Massimo A. Padalino, M.D., Ph.D., UOC Cardiochirurgia Pediatrica e Cardiopatie Congenite, Dipartimento di Scienze Cardiache, Toraciche e Vascolari Centro ‘‘Vincenzo Gallucci,’’ Via Giustiniani 2, 35128 Padova, Italy. Fax: þ39-049-8212409; e-mail: [email protected]
This is a retrospective surgical series including all consecutive patients undergoing TAPVC surgical repair between January 1st, 1989 and January 31st, 2012. Review of medical records was approved by our local hospital committee on clinical investigation. Individual patients were not identified, and the need for patient consent was waived. Clinical and echocardiographic
J CARD SURG 2014;29:678–685
PADALINO, ET AL. SURGERY FOR TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION
data were collected according to our institutional database. Patients were included when there was at least one-month follow-up echocardiogram available. For the purpose of data analysis, we arbitrarily divided all patients into two groups: Group 1, or isolated TAPVC, included those patients with no CHD other than a patent ductus arteriosus (PDA), a patent foramen ovale (PFO), or an atrial septal defect (ASD); Group 2, or Complex TAPVC, included all patients with other associated major CHD, such as common atrioventricular septal defect (AVSD), double outlet right ventricle (DORV), hypoplastic left heart syndrome (HLHS), functional UVH, Scimitar Syndrome (SS) associated with TAPVC, and heterotaxy syndrome. Various types of surgical repair were utilized, according to surgeons’ preference, as described elsewhere:1 direct anastomosis, anastomosis with right transatrial enlargement, modified ‘‘Van Praagh’’ procedure,5 and atrial reseptation with direct anastomosis (used in mixed type of TAPVC). Preoperative, intraoperative and postoperative variables, such as age, weight at operation
