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The Fontan Operation: Is Timing Everything?
EDITORIAL COMMENTARY
The Fontan Operation: Is Timing Everything? Harold M. Burkhart, MD, Jess L. Thompson, MD, and Sabrina D. Phillips, MD
Since the introduction of the Fontan operation over 4 decades ago, surgical and medical management strategies have evolved to improve outcomes and give hope to the patients with complex single ventricle anomalies. Unfortunately, along with these new treatment successes, another group of problems has become more evident stimulating us to rethink our current management with a new focus on long-term outcomes and quality of life. The primary detrimental physiologic conditions that Fontan operation imposes on the patient are central venous hypertension and reduced cardiac output.1 Hepatic dysfunction and protein losing enteropathy are felt to be related to the Fontan circulation with evidence that they may be a timedependent phenomena related to the time of Fontan operation.2 In addition, the ventricle is abnormal with evidence of diastolic dysfunction becoming apparent in most.3 With these sequelae in mind, there exists a controversy as to the best timing for completion Fontan. Some have adopted the belief that patients with total cavopulmonary connection have a limited amount of time before these issues become evident. Followers of the “ticking clock” theory would argue for delaying the Fontan operation until later in life in the hopes of postponing the long-term complications. Others argue that it may not only be time-dependent, and that performing an early Fontan operation may result in a better long-term single ventricle patient. Forsdick et al4 present the outcomes of 45 patients who underwent a Fontan operation from 1976-2006. The average age of the patient at time of Fontan was 18-year old. Importantly, this cohort of patients represents all the adolescent and adult patients who underwent total cavopulmonary configuration in Australia or New Zealand. Data were available for 87% of the patients with a mean follow-up time of 15.5 years. They reported an early mortality of 6% and late mortality of 18%. Although these numbers are not significantly different than other reported series of adult Fontan patients,5 they appear to be strikingly different than what has been reported by the same group for Fontan completion in a younger population.6 They conclude that the results of performing a Fontan operation in older patients are poor, and the operation, rather than being delayed, should be performed in childhood.
Division of Cardiovascular and Thoracic Surgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma
Although this manuscript provides some important insight into the late Fontan operation group, there are a number of weaknesses that should be pointed Harold Burkhart, MD out. Firstly, it should be noted Central Message that 13 centers were Given the long-term issues that accompany involved in the surFontan circulation, controversy exists with gical care of 45 regard to the best timing for total cavopulmoncomplex patients ary shunt completion. undergoing 1 of 3 Fontan configuraSee related article on pages 168–174. tions over a 30year period. With these low numbers, it is perhaps not surprising that the era of operation or type of Fontan did not influence late outcomes. Of note, only 22% of the patients underwent a bidirectional cavopulmonary shunt before Fontan completion, which is not reflective of contemporary surgical management. Crucial data is lacking that would better define what kind of a Fontan candidate we are dealing with. It would be helpful to know the systolic and diastolic ventricular function, ventricular end diastolic pressure and pulmonary artery hemodynamics before the Fontan operation. Perhaps most importantly, it is critical to know why the Fontan operation was delayed in this cohort. If a good portion of these patients were deemed poor Fontan candidates early in life, it is unlikely they became good candidates with time. In conclusion, poor outcomes in adult Fontan patients are probably not only due to timing, but more importantly related to patient selection. There are still going to be older patients that will benefit from a Fontan operation with the intent to relieve symptoms and improve quality of life for a limited time. As to the question of if an early Fontan operation will avoid the sequela associated with the configuration, the data are not yet available to answer that question. Although it would be a relief if timing were everything, we have to move forward in the form of drug therapy, mechanical assistance, and cellular regeneration therapies in hopes of improving the long-term outlook for these complex patients.
1043-0679/$-see front matter ª 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1053/j.semtcvs.2015.09.003
175
THE FONTAN OPERATION 1. Rychik J: Forty years of the Fontan operation: A 3. Anderson PA, Sleeper LA, Mahony L, et al: 5. Burkhart HM, Dearani JA, Mair DD, et al: The failed strategy. Semin Thorac Cardiovasc Surg Contemporary outcomes after the Fontan promodified Fontan procedure: early and late results Pediatr Card Surg Annu 13(1):96-100, 2010 cedure: a Pediatric Heart Network multicenter in 132 adult patients. J Thorac Cardiovasc Surg 2. Friedrich-Rust M, Koch C, Rentzsch A, et al: study. J Am Coll Cardiol 52:85-98, 2008 125:1252-1259, 2003 Noninvasive assessment of liver fibrosis in 4. Forsdick V, Iyenjar A, Carins T, et al: Unsatisfac- 6. d‘Udekem Y, Iyengar AJ, Cochrane AD, et al: The patients with Fontan circulation using transient tory early and late outcomes after Fontan surgery Fontan procedure: Contemporary techniques elastography and biochemical fibrosis markers. delayed to adolescence and adulthood. Semin have improved long-term outcomes. Circulation J Thorac Cardiovasc Surg 135:560-567, 2008 Thorac Cardiovasc Surg 27:168-174, 2015 116:I157-I164, 2007
176
Seminars in Thoracic and Cardiovascular Surgery Volume 27, Number 2
The Fontan Operation: Is Timing Everything? Harold M. Burkhart, MD, Jess L. Thompson, MD, and Sabrina D. Phillips, MD
Since the introduction of the Fontan operation over 4 decades ago, surgical and medical management strategies have evolved to improve outcomes and give hope to the patients with complex single ventricle anomalies. Unfortunately, along with these new treatment successes, another group of problems has become more evident stimulating us to rethink our current management with a new focus on long-term outcomes and quality of life. The primary detrimental physiologic conditions that Fontan operation imposes on the patient are central venous hypertension and reduced cardiac output.1 Hepatic dysfunction and protein losing enteropathy are felt to be related to the Fontan circulation with evidence that they may be a timedependent phenomena related to the time of Fontan operation.2 In addition, the ventricle is abnormal with evidence of diastolic dysfunction becoming apparent in most.3 With these sequelae in mind, there exists a controversy as to the best timing for completion Fontan. Some have adopted the belief that patients with total cavopulmonary connection have a limited amount of time before these issues become evident. Followers of the “ticking clock” theory would argue for delaying the Fontan operation until later in life in the hopes of postponing the long-term complications. Others argue that it may not only be time-dependent, and that performing an early Fontan operation may result in a better long-term single ventricle patient. Forsdick et al4 present the outcomes of 45 patients who underwent a Fontan operation from 1976-2006. The average age of the patient at time of Fontan was 18-year old. Importantly, this cohort of patients represents all the adolescent and adult patients who underwent total cavopulmonary configuration in Australia or New Zealand. Data were available for 87% of the patients with a mean follow-up time of 15.5 years. They reported an early mortality of 6% and late mortality of 18%. Although these numbers are not significantly different than other reported series of adult Fontan patients,5 they appear to be strikingly different than what has been reported by the same group for Fontan completion in a younger population.6 They conclude that the results of performing a Fontan operation in older patients are poor, and the operation, rather than being delayed, should be performed in childhood.
Division of Cardiovascular and Thoracic Surgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma
Although this manuscript provides some important insight into the late Fontan operation group, there are a number of weaknesses that should be pointed Harold Burkhart, MD out. Firstly, it should be noted Central Message that 13 centers were Given the long-term issues that accompany involved in the surFontan circulation, controversy exists with gical care of 45 regard to the best timing for total cavopulmoncomplex patients ary shunt completion. undergoing 1 of 3 Fontan configuraSee related article on pages 168–174. tions over a 30year period. With these low numbers, it is perhaps not surprising that the era of operation or type of Fontan did not influence late outcomes. Of note, only 22% of the patients underwent a bidirectional cavopulmonary shunt before Fontan completion, which is not reflective of contemporary surgical management. Crucial data is lacking that would better define what kind of a Fontan candidate we are dealing with. It would be helpful to know the systolic and diastolic ventricular function, ventricular end diastolic pressure and pulmonary artery hemodynamics before the Fontan operation. Perhaps most importantly, it is critical to know why the Fontan operation was delayed in this cohort. If a good portion of these patients were deemed poor Fontan candidates early in life, it is unlikely they became good candidates with time. In conclusion, poor outcomes in adult Fontan patients are probably not only due to timing, but more importantly related to patient selection. There are still going to be older patients that will benefit from a Fontan operation with the intent to relieve symptoms and improve quality of life for a limited time. As to the question of if an early Fontan operation will avoid the sequela associated with the configuration, the data are not yet available to answer that question. Although it would be a relief if timing were everything, we have to move forward in the form of drug therapy, mechanical assistance, and cellular regeneration therapies in hopes of improving the long-term outlook for these complex patients.
1043-0679/$-see front matter ª 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1053/j.semtcvs.2015.09.003
175
THE FONTAN OPERATION 1. Rychik J: Forty years of the Fontan operation: A 3. Anderson PA, Sleeper LA, Mahony L, et al: 5. Burkhart HM, Dearani JA, Mair DD, et al: The failed strategy. Semin Thorac Cardiovasc Surg Contemporary outcomes after the Fontan promodified Fontan procedure: early and late results Pediatr Card Surg Annu 13(1):96-100, 2010 cedure: a Pediatric Heart Network multicenter in 132 adult patients. J Thorac Cardiovasc Surg 2. Friedrich-Rust M, Koch C, Rentzsch A, et al: study. J Am Coll Cardiol 52:85-98, 2008 125:1252-1259, 2003 Noninvasive assessment of liver fibrosis in 4. Forsdick V, Iyenjar A, Carins T, et al: Unsatisfac- 6. d‘Udekem Y, Iyengar AJ, Cochrane AD, et al: The patients with Fontan circulation using transient tory early and late outcomes after Fontan surgery Fontan procedure: Contemporary techniques elastography and biochemical fibrosis markers. delayed to adolescence and adulthood. Semin have improved long-term outcomes. Circulation J Thorac Cardiovasc Surg 135:560-567, 2008 Thorac Cardiovasc Surg 27:168-174, 2015 116:I157-I164, 2007
176
Seminars in Thoracic and Cardiovascular Surgery Volume 27, Number 2