© 2014 Wiley Periodicals, Inc.
836
LETTER TO THE EDITOR __________________________________________________________
Treat-and-Repair Approach to Eisenmenger Syndrome cile Tissot, M.D.,y and Maurice Beghetti, M.D., Ph.D.y Patrick O. Myers, M.D.,* Ce *Division of Cardiovascular Surgery, Geneva University Hospitals and School of Medicine, Geneva, Switzerland; and yUnit of Pediatric Cardiology, Children’s University Hospital Geneva, Geneva, Switzerland doi: 10.1111/jocs.12378 (J Card Surg 2014;29:836)
We read with great interest the report by Hu et al.1 on ventricular septal defect repair in Eisenmenger syndrome after pretreatment with Bosentan. The authors should be commended for their excellent clinical outcome. We would like to discuss this approach of ‘‘treat-and-repair’’ to Eisenmenger syndrome, which we reviewed recently.2 Eisenmenger syndrome has been synonymous with inoperability, as remodeling of the pulmonary vasculature is considered irreversible, placing these patients at high perioperative risk and high risk of postoperative right ventricular failure. With an improvement in pulmonary vasodilators and their more widespread use, it has been possible to reduce pulmonary vascular resistance and symptoms in a subset of patients with pulmonary arterial hypertension (PAH) and make these patients amenable to surgery,3 as reported in Hu et al.’s case. However, we do not know how many patients underwent similar ‘‘treat-and-repair’’ strategy and had a negative outcome, as they are seldom reported. Patients with Eisenmenger syndrome and simple cardiac defects overall have the best survival (93% at five years), much better than any other group of PAH patients and particularly better than those with PAH after surgical repair of congenital heart disease,4 even though their quality of life remains poor. Long-term outcomes are the key question. No data are available on the long-term response of the right ventricle to closure of intracardiac communications. Once the defect is closed, a pathophysiologic situation more similar to
Conflict of interest: Maurice Beghetti has served as consultant for Actelion Pharmaceuticals, Glaxo Smith Kline and EliLilly and received cile Tissot and Patrick lecture fees from Actelion Pharmaceuticals. Ce Myers have no disclosures related to this manuscript. Address for correspondence: Patrick O. Myers, M.D., Division of Cardiovascular Surgery, Geneva University Hospitals and School of Medicine, 4 rue Gabrielle-Perret-Gentil, 1211 Geneva, Switzerland. Fax: þ41 22 372 76 34; e-mail: [email protected]
idiopathic PAH is obtained, which is associated with much worse long-term outcomes and is never cured. Patients with Eisenmenger syndrome in adulthood seem to fare far better than patients with all other forms of PAH. The key to the relative longevity of these complex patients lies in the unique adaptation of the right ventricle.2 We commend the authors on their excellent outcome in a very challenging patient, who was doomed to a poor quality of life without their intervention. However, closure of intracardiac defects after a positive response to advanced PAH therapies cannot be generally recommended until evidence of its safety, efficacy, and durability becomes available. We would recommend that an international registry of patients with PAH managed with ‘‘treat-and-repair’’ and a ‘‘repair-andtreat’’ be created to better define this patient population and reliably report long-term outcomes. Defining subgroups of patients with high PVR who could benefit from surgical repair and/or targeted therapies would represent an important progress.
REFERENCES 1. Hu L, Tan L-H, Ye J: Repair of ventricular septal defect with Eisenmenger syndrome after Bosentan treatment. J Card Surg 2014;29(3):401–402. 2. Myers PO, Tissot C, Beghetti M: Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease. Circ J 2013;78: 4–11. 3. Beghetti M, Galie N, Bonnet D: Can ‘‘inoperable’’ congenital heart defects become operable in patients with pulmonary arterial hypertension? Dream or reality? Congenit Heart Dis 2012;7:3–11. 4. Manes A, Palazzini M, Leci E, et al: Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: A comparison between clinical subgroups. Eur Heart J 2014;35(11):716–724.
Copyright of Journal of Cardiac Surgery is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
836
LETTER TO THE EDITOR __________________________________________________________
Treat-and-Repair Approach to Eisenmenger Syndrome cile Tissot, M.D.,y and Maurice Beghetti, M.D., Ph.D.y Patrick O. Myers, M.D.,* Ce *Division of Cardiovascular Surgery, Geneva University Hospitals and School of Medicine, Geneva, Switzerland; and yUnit of Pediatric Cardiology, Children’s University Hospital Geneva, Geneva, Switzerland doi: 10.1111/jocs.12378 (J Card Surg 2014;29:836)
We read with great interest the report by Hu et al.1 on ventricular septal defect repair in Eisenmenger syndrome after pretreatment with Bosentan. The authors should be commended for their excellent clinical outcome. We would like to discuss this approach of ‘‘treat-and-repair’’ to Eisenmenger syndrome, which we reviewed recently.2 Eisenmenger syndrome has been synonymous with inoperability, as remodeling of the pulmonary vasculature is considered irreversible, placing these patients at high perioperative risk and high risk of postoperative right ventricular failure. With an improvement in pulmonary vasodilators and their more widespread use, it has been possible to reduce pulmonary vascular resistance and symptoms in a subset of patients with pulmonary arterial hypertension (PAH) and make these patients amenable to surgery,3 as reported in Hu et al.’s case. However, we do not know how many patients underwent similar ‘‘treat-and-repair’’ strategy and had a negative outcome, as they are seldom reported. Patients with Eisenmenger syndrome and simple cardiac defects overall have the best survival (93% at five years), much better than any other group of PAH patients and particularly better than those with PAH after surgical repair of congenital heart disease,4 even though their quality of life remains poor. Long-term outcomes are the key question. No data are available on the long-term response of the right ventricle to closure of intracardiac communications. Once the defect is closed, a pathophysiologic situation more similar to
Conflict of interest: Maurice Beghetti has served as consultant for Actelion Pharmaceuticals, Glaxo Smith Kline and EliLilly and received cile Tissot and Patrick lecture fees from Actelion Pharmaceuticals. Ce Myers have no disclosures related to this manuscript. Address for correspondence: Patrick O. Myers, M.D., Division of Cardiovascular Surgery, Geneva University Hospitals and School of Medicine, 4 rue Gabrielle-Perret-Gentil, 1211 Geneva, Switzerland. Fax: þ41 22 372 76 34; e-mail: [email protected]
idiopathic PAH is obtained, which is associated with much worse long-term outcomes and is never cured. Patients with Eisenmenger syndrome in adulthood seem to fare far better than patients with all other forms of PAH. The key to the relative longevity of these complex patients lies in the unique adaptation of the right ventricle.2 We commend the authors on their excellent outcome in a very challenging patient, who was doomed to a poor quality of life without their intervention. However, closure of intracardiac defects after a positive response to advanced PAH therapies cannot be generally recommended until evidence of its safety, efficacy, and durability becomes available. We would recommend that an international registry of patients with PAH managed with ‘‘treat-and-repair’’ and a ‘‘repair-andtreat’’ be created to better define this patient population and reliably report long-term outcomes. Defining subgroups of patients with high PVR who could benefit from surgical repair and/or targeted therapies would represent an important progress.
REFERENCES 1. Hu L, Tan L-H, Ye J: Repair of ventricular septal defect with Eisenmenger syndrome after Bosentan treatment. J Card Surg 2014;29(3):401–402. 2. Myers PO, Tissot C, Beghetti M: Assessment of operability of patients with pulmonary arterial hypertension associated with congenital heart disease. Circ J 2013;78: 4–11. 3. Beghetti M, Galie N, Bonnet D: Can ‘‘inoperable’’ congenital heart defects become operable in patients with pulmonary arterial hypertension? Dream or reality? Congenit Heart Dis 2012;7:3–11. 4. Manes A, Palazzini M, Leci E, et al: Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: A comparison between clinical subgroups. Eur Heart J 2014;35(11):716–724.
Copyright of Journal of Cardiac Surgery is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
