M. Rosic et al. / Interactive CardioVascular and Thoracic Surgery
Conflict of interest: none declared. References [1] Rosic M, Susak S, Redzek A, Velicki L. Closure of an atrial septal defect with a one-way flap patch in a patient with severe pulmonary hypertension. Interact CardioVasc Thorac Surg 2016;22:856–9. [2] Oztas DM, Canbay C, Beyaz MO, Ugurlucan M. eComment. Atrial septal defect closure with a composite check-valved patch in pulmonary hypertensive patients. Interact CardioVasc Thorac Surg 2016;22:858. [3] Li X, Guo Y, Ziegler KR, Model LS, Eghbalieh SD, Brenes RA et al. Current usage and future directions for the bovine pericardial patch. Ann Vasc Surg 2011;25:561–568. [4] Jiang WJ, Cui YC, Li JH, Zhang XH, Ding HH, Lai YQ et al. Is autologous or heterologous pericardium better for valvuloplasty? A comparative study of calcification propensity. Tex Heart Inst J 2015;42:202–208.
eComment: How to define operability in pulmonary hypertension secondary to congenital heart disease? Authors: Patrick O. Myers, Frederic Lador and Maurice Beghetti Pulmonary Hypertension Program, Cardiovascular Surgery, Pulmonology and Pediatric Cardiology, Geneva doi: 10.1093/icvts/ivw131 © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. Rosic et al. report the surgical management of a 54-year-old patient with an atrial septal defect (ASD) using a unidirectional fenestrated atrial septal patch, allowing for right-to-left shunting in the postoperative period. The patient had a favourable outcome, although the mean pulmonary artery pressure decreased from 30 mmHg preoperatively to 26 mmHg at follow-up. This patient thus still has pulmonary hypertension (PH), and requires treatment. This
begs the question: is it a success? In a retrospective study of patients with pulmonary artery hypertension (PAH) with follow-up to 20 years, patients with Eisenmenger syndrome and unoperated simple congenital cardiac defects overall have the best survival (87-86% at 20 years), much better than any other subgroup and particularly better than those with PAH after surgical repair of congenital heart disease (36%), even though their quality of life remains poor. Long-term outcomes are the key question, and unfortunately no long-term data are available. Once the defect is closed, a pathophysiological situation more similar to idiopathic PAH is obtained, which is associated with much worse long-term outcomes. This case illustrates several unsolved issues in patients with PAH-CHD: what are the criteria for operability? How should they be defined? Although novel biomarkers capable of defining the degree of pulmonary vascular disease (PVD), such as circulating endothelial cells or micro-RNAs, are currently being investigated, haemodynamic criteria based on right heart catheterization and vasoreactivity remain the gold standard, however flawed they may be. What are the long-term outcomes after surgical repair of CHD with PAH? Until what age should patients be referred for surgical repair, and in particular will a patient 50 years or older benefit from closing the hole? Furthermore, if right ventricular failure after closing the ASD is feared, how should the right ventricle be protected? Atrioseptostomy or a fenestrated atrial patch provides diastolic decompression of the RV providing a decrease in right atrial and end-diastolic right ventricular pressure, an increase in cardiac output at the expense of systemic oxygen desaturation, and an increase in left ventricular end-diastolic pressure that can overwhelm the left ventricle if the fenestration is oversized. If systolic failure due to PAH is suspected, a post-tricuspid shunt (Pott’s) seems to be a better solution that can provide systolic decompression and facilitate the difficult post-operative period, while maintaining adequate oxygen saturation to the upper body and coronary circulation. This sounds like a schizophrenic dilemma: we aim at closing the ASD when there is high pulmonary blood flow and PAH is present, but suggest to open an atrial communication in case of severe PAH. Finally, although Rosic et al. should be commended for the favourable early outcome, it should be noted that current guidelines recommend preoperative testing with iNO in patients with an indexed PVR of 6-9 WU/m2 (as opposed to nitroglycerin in this report), and that calcium channel blocker treatment for PAHCHD is contraindicated after surgical repair, and treatment with endothelin receptor antagonists, phosphodiesterase inhibitors or prostacyclin are recommended in case of persistent or recurrent PAH after shunt closure. Conflict of interest: Dr Beghetti has served as consultant, steering committee and/or advisory board member for Actelion Pharmaceuticals Ltd., Bayer-Healthcare, Eli Lilly, GlaxoSmithKline, and Pfizer. The other authors report no conflict.
References [1] Rosic M, Susak S, Redzek A, Velicki L. Closure of an atrial septal defect with a one-way flap patch in a patient with severe pulmonary hypertension. Interact CardioVasc Thorac Surg 2016;22:856–9. [2] Manes A, Palazzini M, Leci E, Bacchi Reggiani ML, Branzi A, Galie N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Hear J 2014;35:716–24. [3] Gatzoulis MA, Beghetti M, Landzberg MJ, Galiè N. Pulmonary arterial hypertension associated with congenital heart disease: Recent advances and future directions. Int J Cardiol 2014;177:340–347. [4] Myers PO, Tissot C, Beghetti M. Treat-and-repair approach to Eisenmenger syndrome. J Card Surg 2014;29:836–836. [5] 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.
CASE REPORT
evaluation of the impact of unidirectional patch valve orifice orientation on left atrial haemodynamics, comprehensive computational fluid dynamics analysis would be warranted. We believe that only such analysis would be able to provide us with data on atrial flow patterns, atrio-ventricular vortex patterns, velocity profiles and other important parameters. 3. Pulmonary hypertension treatment during the postoperative period. We were able to successfully manage pulmonary artery hypertension (PAH) during early postoperative course with sodium nitroprusside. Nitric oxide was reserved as a second line treatment strategy, although, as you’ve said, in some centers it is used routinely during postoperative recovery as a first line treatment. During hospitalization, the patient was prescribed and instructed to use sildenafil, but she refused to use this medication. For this reason, we had to gradually convert her to calcium channel blocker therapy (CCB). The patient was discharged with permanent CCB therapy. However, we do agree that oral sildenafil therapy should be prescribed as a first line therapy positively affecting long-term recovery and functional status of the patient. Sildenafil may also increase the overall survival rate in patients with PAH. 4. Concomitant tricuspid valve surgery. During surgery, tricuspid annuloplasty was also performed. Control transthoracic echocardiography performed one year after the surgery showed mild tricuspid regurgitation. We hope the above response addresses the concerns raised and wish to thank contributing readers for the questions.
859
Conflict of interest: none declared. References [1] Rosic M, Susak S, Redzek A, Velicki L. Closure of an atrial septal defect with a one-way flap patch in a patient with severe pulmonary hypertension. Interact CardioVasc Thorac Surg 2016;22:856–9. [2] Oztas DM, Canbay C, Beyaz MO, Ugurlucan M. eComment. Atrial septal defect closure with a composite check-valved patch in pulmonary hypertensive patients. Interact CardioVasc Thorac Surg 2016;22:858. [3] Li X, Guo Y, Ziegler KR, Model LS, Eghbalieh SD, Brenes RA et al. Current usage and future directions for the bovine pericardial patch. Ann Vasc Surg 2011;25:561–568. [4] Jiang WJ, Cui YC, Li JH, Zhang XH, Ding HH, Lai YQ et al. Is autologous or heterologous pericardium better for valvuloplasty? A comparative study of calcification propensity. Tex Heart Inst J 2015;42:202–208.
eComment: How to define operability in pulmonary hypertension secondary to congenital heart disease? Authors: Patrick O. Myers, Frederic Lador and Maurice Beghetti Pulmonary Hypertension Program, Cardiovascular Surgery, Pulmonology and Pediatric Cardiology, Geneva doi: 10.1093/icvts/ivw131 © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. Rosic et al. report the surgical management of a 54-year-old patient with an atrial septal defect (ASD) using a unidirectional fenestrated atrial septal patch, allowing for right-to-left shunting in the postoperative period. The patient had a favourable outcome, although the mean pulmonary artery pressure decreased from 30 mmHg preoperatively to 26 mmHg at follow-up. This patient thus still has pulmonary hypertension (PH), and requires treatment. This
begs the question: is it a success? In a retrospective study of patients with pulmonary artery hypertension (PAH) with follow-up to 20 years, patients with Eisenmenger syndrome and unoperated simple congenital cardiac defects overall have the best survival (87-86% at 20 years), much better than any other subgroup and particularly better than those with PAH after surgical repair of congenital heart disease (36%), even though their quality of life remains poor. Long-term outcomes are the key question, and unfortunately no long-term data are available. Once the defect is closed, a pathophysiological situation more similar to idiopathic PAH is obtained, which is associated with much worse long-term outcomes. This case illustrates several unsolved issues in patients with PAH-CHD: what are the criteria for operability? How should they be defined? Although novel biomarkers capable of defining the degree of pulmonary vascular disease (PVD), such as circulating endothelial cells or micro-RNAs, are currently being investigated, haemodynamic criteria based on right heart catheterization and vasoreactivity remain the gold standard, however flawed they may be. What are the long-term outcomes after surgical repair of CHD with PAH? Until what age should patients be referred for surgical repair, and in particular will a patient 50 years or older benefit from closing the hole? Furthermore, if right ventricular failure after closing the ASD is feared, how should the right ventricle be protected? Atrioseptostomy or a fenestrated atrial patch provides diastolic decompression of the RV providing a decrease in right atrial and end-diastolic right ventricular pressure, an increase in cardiac output at the expense of systemic oxygen desaturation, and an increase in left ventricular end-diastolic pressure that can overwhelm the left ventricle if the fenestration is oversized. If systolic failure due to PAH is suspected, a post-tricuspid shunt (Pott’s) seems to be a better solution that can provide systolic decompression and facilitate the difficult post-operative period, while maintaining adequate oxygen saturation to the upper body and coronary circulation. This sounds like a schizophrenic dilemma: we aim at closing the ASD when there is high pulmonary blood flow and PAH is present, but suggest to open an atrial communication in case of severe PAH. Finally, although Rosic et al. should be commended for the favourable early outcome, it should be noted that current guidelines recommend preoperative testing with iNO in patients with an indexed PVR of 6-9 WU/m2 (as opposed to nitroglycerin in this report), and that calcium channel blocker treatment for PAHCHD is contraindicated after surgical repair, and treatment with endothelin receptor antagonists, phosphodiesterase inhibitors or prostacyclin are recommended in case of persistent or recurrent PAH after shunt closure. Conflict of interest: Dr Beghetti has served as consultant, steering committee and/or advisory board member for Actelion Pharmaceuticals Ltd., Bayer-Healthcare, Eli Lilly, GlaxoSmithKline, and Pfizer. The other authors report no conflict.
References [1] Rosic M, Susak S, Redzek A, Velicki L. Closure of an atrial septal defect with a one-way flap patch in a patient with severe pulmonary hypertension. Interact CardioVasc Thorac Surg 2016;22:856–9. [2] Manes A, Palazzini M, Leci E, Bacchi Reggiani ML, Branzi A, Galie N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Hear J 2014;35:716–24. [3] Gatzoulis MA, Beghetti M, Landzberg MJ, Galiè N. Pulmonary arterial hypertension associated with congenital heart disease: Recent advances and future directions. Int J Cardiol 2014;177:340–347. [4] Myers PO, Tissot C, Beghetti M. Treat-and-repair approach to Eisenmenger syndrome. J Card Surg 2014;29:836–836. [5] 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.
CASE REPORT
evaluation of the impact of unidirectional patch valve orifice orientation on left atrial haemodynamics, comprehensive computational fluid dynamics analysis would be warranted. We believe that only such analysis would be able to provide us with data on atrial flow patterns, atrio-ventricular vortex patterns, velocity profiles and other important parameters. 3. Pulmonary hypertension treatment during the postoperative period. We were able to successfully manage pulmonary artery hypertension (PAH) during early postoperative course with sodium nitroprusside. Nitric oxide was reserved as a second line treatment strategy, although, as you’ve said, in some centers it is used routinely during postoperative recovery as a first line treatment. During hospitalization, the patient was prescribed and instructed to use sildenafil, but she refused to use this medication. For this reason, we had to gradually convert her to calcium channel blocker therapy (CCB). The patient was discharged with permanent CCB therapy. However, we do agree that oral sildenafil therapy should be prescribed as a first line therapy positively affecting long-term recovery and functional status of the patient. Sildenafil may also increase the overall survival rate in patients with PAH. 4. Concomitant tricuspid valve surgery. During surgery, tricuspid annuloplasty was also performed. Control transthoracic echocardiography performed one year after the surgery showed mild tricuspid regurgitation. We hope the above response addresses the concerns raised and wish to thank contributing readers for the questions.
859
