Case Report
Potts Shunt in a Child With End-Stage Pulmonary Hypertension After Late Repair of Ventricular Septal Defect
World Journal for Pediatric and Congenital Heart Surgery 4(3) 286-289 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113482739 pch.sagepub.com
Cecilie Petersen, BMedSc1, Morten Helvind, MD1, Tim Jensen, MD2 and Henrik Ørbæk Andersen, MD, DMSc1
Abstract We report on a 10-year-old boy with medically refractory pulmonary arterial hypertension (PAH) and end-stage right heart failure after closure of a ventricular septal defect. The boy was a candidate for lung transplantation (LTX), but an alternative option was to create an Eisenmenger physiology with right-to-left shunting. The shunt could be created either as an intracardiac or as an extracardiac shunt. We decided to create a Potts shunt, a direct anastomosis between the left pulmonary artery and the descending aorta. The Potts shunt functioned as a right-to-left shunt, thus reducing the afterload on the right ventricle. The boy’s clinical condition improved markedly, so he was discharged two weeks after the procedure. The ultimate therapeutic option for medically refractory PAH is LTX or heart–lung transplantation, but because of the short life span after LTX, time was bought by postponing the time of transplantation. Keywords congenital heart surgery, heart failure operations, palliation, pediatric, pulmonary vascular resistance/hypertension, shunts (Potts shunt), transplantation, heart–lung, transplantation, lung Submitted October 28, 2012; Accepted February 15, 2013.
Introduction Because of improvements in diagnosis and surgical interventions in patients with congenital heart defects, the number of patients with secondary pulmonary arterial hypertension (PAH) has decreased over the past decades. Patients with Eisenmenger physiology, that is, PAH with a right-to-left shunt, usually fare better than patients with idiopathic PAH,1-3 who have a dismal prognosis with mean survival of only a few years from diagnosis despite recent improvements in medical treatment with prostanoids, sildenafil, and endothelin antagonists.1 Patients with isolated, severe PAH and right heart failure will die of the disease if not treated with a lung transplantation (LTX) or heart–lung transplantation. However, the half-life of pediatric lung recipients is only 5.5 years.2 The superior prognosis in children with Eisenmenger physiology compared to that in children with idiopathic PAH has partly been explained by the differences in right ventricle (RV) function. In a patient with Eisenmenger, the unrestrictive right-to-left shunt precludes the development of suprasystemic RV pressure and maintains a reasonable RV function. The right-to-left shunt also allows the preservation of systemic cardiac output in the presence of RV failure.3 The Potts shunt is an anastomosis between the left pulmonary artery (LPA) and the descending aorta (Figure 1). This type
of shunt was first reported in 1946 in children with cyanotic congenital heart defects, working as a systemic-to-pulmonary shunt, that is, a left-to-right shunt.4 In this case report in a patient with PAH and a surgically closed large perimembranous ventricular septal defect (VSD), we describe the use of the Potts shunt, not as a left-to-right shunt but as a right-to-left shunt. The circulation changes from that of PAH physiology to an Eisenmenger physiology, like the one found in patients with persistent arterial duct who develop right-to-left shunting.
Case Report The boy was born prematurely on week 31 and had respiratory distress syndrome with subsequent development of bronc1 Department of Cardiothoracic Surgery, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark 2 Department of Pediatric Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
Corresponding Author: Cecilie Petersen, Department of Cardiothoracic Surgery 2152, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9, 2200 Copenhagen, Denmark. Email: [email protected]
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Abbreviations and Acronyms BPD LPA LTX LV NT-proBNP NYHA PAB PAH RV TTE VSD
bronchopulmonary dysplasia left pulmonary artery lung transplantation left ventricle N-terminal prohormone of brain natriuretic peptide New York Heart Association pulmonary artery banding pulmonary arterial hypertension right ventricle transthoracic Doppler echocardiography ventricular septal defect
Figure 1. Illustration of a Potts shunt.
hopulmonary dysplasia (BPD). During his first year of life, he had repeated pulmonary infections and had a habitual low arterial oxygen saturation (SaO2) of 86% to 92% despite oxygen supplementation. The poor pulmonary condition of the boy was presumed to be caused by BPD alone, but at age 2 when his clinical condition started to deteriorate, it was decided to perform transthoracic Doppler echocardiography (TTE). The TTE revealed a large subaortic VSD with a right-to-left shunt, RV hypertrophy, dilatation, and pulmonary hypertension. A right heart catheterization showed a perimembraneous VSD with right-to-left shunting, systemic RV pressure, and PAH (Table 1). The findings were felt to preclude VSD closure. Treatment with endothelin receptor-selective antagonist (bosentan) was initiated but without any significant effect. After one month, a loose pulmonary artery banding (PAB) was performed with a decrease in pulmonary artery pressure from 80% of systemic pressure to 66% of systemic pressure, a drop in SaO2 from 92% to 75%, and decrease in hemoglobin from 9.5 to 8.8 mmol/L. The TTE showed a peak gradient of 55 mm Hg across the band, which had decreased to 45 mm Hg upon discharge one week later. Bosentan treatment was continued. Further tightening
of the PAB was not performed because of low SaO2 varying from 60% to 80%. After 10 months, the RV pressure had increased to a suprasystemic level, and the VSD was starting to become restrictive (Table 1). At this point, debanding was necessary to avoid RV failure. The pulmonary artery was debanded at age 3, one year after banding, and the VSD was closed with a flap valve patch. The flap valve patch was mde of 0.4-mm thick PTFE and was sutured onto one-third of the circumference of the VSD. A loose thread was sewed on to the unsecured part of the flap thus allowing an opening of approximately 4 mm. The boy was discharged with SaO2 of 80% to 90% on room air, with a combination therapy of sildenafil and bosentan. A lung biopsy, taken during the operation, showed no pathological changes. The function of the flap valve patch persisted only for nine months at which point no shunting through the VSD was observed on TTE. The clinical condition was stable in New York Heart Association (NYHA) class 2B during the following seven years. At 10 years of age, the boy experienced a rapid decline in his functional status with NYHA class decreasing from 2B to 3. TTE showed a dilated right atrium and RV with a decreased function and septum deviation compromising the left ventricle (LV), moderate tricuspid and pulmonary valve insufficiency, and completely closed VSD. Prostanoid (flolan) infusion and later treprostinil (remodulin) infusion were initiated with minimal improvement. During the next two months his functional status worsened, he became confined to his bed, and experienced frequent episodes of nausea and vomiting, interpreted as signs of cardiac failure. Repetitive TTE’s showed progressive RV failure and increasing pulmonary hypertension, with SaO2 of 82% to 85%. The clinical condition was complicated by pneumonia and sepsis, and the boy was admitted to the intensive care unit where he required tracheal intubation and mechanical ventilatory support. After successful treatment of the sepsis and extubation of the trachea, his RV function was unchanged, and the N-terminal prohormone brain natriuretic peptide (NT-proBNP) values were rising from 14.7 pmol/L to a peak value of 461 pmol/L. It was decided to create a Potts shunt to reduce the RV afterload. The shunt was made through a left thoracotomy in the fifth intercostal space. The descending aorta was exposed 10 cm distal to the subclavian artery, and the LPA was exposed from the bifurcation to a point distal to the first lobar branch. The LPA was clamped centrally, and the proximal descending aorta was isolated between two vascular clamps to make room for a large anastomosis (Figure 2). A 13- to 15-mm long opening was made in both the LPA and the descending aorta. The two vessels were anastomosed in side-to-side fashion with a running 4-0 polypropylene. The boy was weaned from mechanical ventilation 19 hours postoperatively, and the postoperative course was uneventful. The boy continued treatment with sildenafil, treprostinil, and bosentan and was discharged two weeks later. Vomiting and loss of appetite were present only during the first two months after the operation and disappeared when the hemoglobin reached levels of around 10 mmol/L. The clinical condition improved markedly, 13 months after the operation. The boy is stabile and is able to ride his bicycle to
Downloaded from pch.sagepub.com at NORTHWESTERN UNIV LIBRARY on December 18, 2014
288
World Journal for Pediatric and Congenital Heart Surgery 4(3)
Table 1. Hemodynamic Characteristics Before and After Banding of the Pulmonary Artery. Age
Procedure
1 1 2 3
Cath 110/6 87/8 Banding of pulmonary artery Cath 97/7 107/7 Debanding of pulmonary artery and VSD closure with flap valve patch
year 10 months year 11 months years 9 months years 1 month
LV Pressure, mm Hg
RV Pressure, mm Hg
PA Pressure, mm Hg
PVR (Wood Units)
84/38
23
70/36
11
Abbreviations: LV, left ventricle; RV, right ventricle; PA, pulmonary artery; PVR, pulmonary vessel resistance; Cath, right heart catheterization; VSD, ventricular septal defect.
Figure 2. Illustration showing site of side-to-side anastomosis between the left pulmonary artery and the descending thoracic aorta.
and from school every day, but his lower body becomes cyanotic when engaging in more strenuous activities. His upper body SaO2 is 93% and lower body SaO2 is 73%, and there have been no symptoms of upper body cyanosis since two months after the operation. His NT-proBNP level has decreased to 10 pmol/L, and hemoglobin has increased from 7.5 to 10.2 mmol/L. Improvement was also noted on TTE, where the LV was less compressed by the RV (Figure 3).
Discussion This is the first case report that describes significant clinical improvement after performance of a Potts shunt in a child with end-stage PAH after late closure of a VSD. One earlier article reported similar improvement in two patients with transposition of the great arteries that had undergone arterial switch surgery,5 and two other reports had similar findings in 10 children with idiopathic pulmonary hypertension.6,7
Figure 3. Transthoracic echocardiogram showing parasternal longaxis view of the heart in end-diastolic phase. A, Before Potts shunt operation. B, One month after Potts shunt operation. LV, left ventricle; RV, right ventricle.
In our case, the boy had progressive RV failure with NTproBNP values increasing from 14.7 to 461 pmol/L through five months in spite of maximum pharmacological treatment. We considered two different treatments: decompression of the RV by providing an excess flow valve at intra- or extracardiac level that is converting the patient’s physiology into Eisenmenger physiology or transplantation. An atrial shunt is creation of an atrial septum defect that could present a risk of uncontrolled flow of deoxygenated blood from the pulmonary circulation to the systemic circulation causing serious hypoxemia. The same
Downloaded from pch.sagepub.com at NORTHWESTERN UNIV LIBRARY on December 18, 2014
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scenario could be expected if a shunt was made at ventricular level. However, creating an extracardiac shunt, such as the Potts shunt, between the systemic and the pulmonary circulation would exclusively shunt cyanotic blood to the lower body and maintain normal or near-normal SaO2 in the upper part of the body. Patients with Eisenmenger physiology are reported to live longer and to have less RV failure than patients with primary pulmonary hypertension.3,8 In our case, the improvement in RV function was demonstrated by both the TTE and the decrease in NT-proBNP values, after surgery. The final therapeutic option for pharmacological nonresponding pulmonary hypertension is LTX. The organ shortage and the limited life span, with a half-life of only 5.5 years, make this option a last resort, which should be postponed until there is no other alternative.2 In our case, the Potts shunt immediately reduced the afterload on the RV, thus avoiding RV failure, as shown by others.5-7 The subsequent option for transplantation is not prohibited, although there may be an increased risk compared to a standard transplantation, in view of the need to takedown the shunt at the time of transplantation. However, there are no reported experiences in the literature of LTX or heart–lung transplantation in patients with a Potts shunt. The survival rate for patients with Eisenmenger is 42% at 25 years and is therefore much more favorable than that of patients which is 50% at 5.5 years.2,3 In the present case, our patient benefited from the Potts shunt and experienced clinical improvement. There might be a future potential in treating patients with RV failure and severe PAH with the Potts shunt, thereby postponing the time of transplantation. Declaration of Conflicting Interests The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The authors received no financial support for the research, authorship, and/or publication of this article.
References 1. Haworth SG, Hislop AA. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart. 2009; 95(4): 312-317. 2. Benden C, Aurora P, Edwards LB, et al. The registry of the International Society for Heart and Lung Transplantation: fourteenth pediatric lung and heart-lung transplantation report—2011. J Heart Lung Transplant. 2011;30(10): 1123-1132. 3. Hopkins WE, Waggoner AD. Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome. Am J Cardiol. 2002;89(1): 34-38. 4. Potts WJ, Smith S, Gibson S. Anastomosis of the aorta to a pulmonary artery. J Am Med Assoc. 1946;132(11): 627-631. 5. Blanc J, Vouhe P, Bonnet D. Potts shunt in patients with pulmonary hypertension. New Eng J Med. 2004;350(6): 623. 6. Labombarda F, Maragnes P, Dupont-Chauvet P, Serraf A. Potts anastomosis for children with idiopathic pulmonary hypertension. Ped Cardiol. 2009;30(8): 1143-1145. 7. Baruteau AE, Serraf A, Levy M, et al. Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg. 2012;94(3): 817-824. 8. Hopkins WE, Ochoa LL, Richardson GW, Trulock EP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996;15(1 pt 1): 100-105.
Downloaded from pch.sagepub.com at NORTHWESTERN UNIV LIBRARY on December 18, 2014
Potts Shunt in a Child With End-Stage Pulmonary Hypertension After Late Repair of Ventricular Septal Defect
World Journal for Pediatric and Congenital Heart Surgery 4(3) 286-289 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113482739 pch.sagepub.com
Cecilie Petersen, BMedSc1, Morten Helvind, MD1, Tim Jensen, MD2 and Henrik Ørbæk Andersen, MD, DMSc1
Abstract We report on a 10-year-old boy with medically refractory pulmonary arterial hypertension (PAH) and end-stage right heart failure after closure of a ventricular septal defect. The boy was a candidate for lung transplantation (LTX), but an alternative option was to create an Eisenmenger physiology with right-to-left shunting. The shunt could be created either as an intracardiac or as an extracardiac shunt. We decided to create a Potts shunt, a direct anastomosis between the left pulmonary artery and the descending aorta. The Potts shunt functioned as a right-to-left shunt, thus reducing the afterload on the right ventricle. The boy’s clinical condition improved markedly, so he was discharged two weeks after the procedure. The ultimate therapeutic option for medically refractory PAH is LTX or heart–lung transplantation, but because of the short life span after LTX, time was bought by postponing the time of transplantation. Keywords congenital heart surgery, heart failure operations, palliation, pediatric, pulmonary vascular resistance/hypertension, shunts (Potts shunt), transplantation, heart–lung, transplantation, lung Submitted October 28, 2012; Accepted February 15, 2013.
Introduction Because of improvements in diagnosis and surgical interventions in patients with congenital heart defects, the number of patients with secondary pulmonary arterial hypertension (PAH) has decreased over the past decades. Patients with Eisenmenger physiology, that is, PAH with a right-to-left shunt, usually fare better than patients with idiopathic PAH,1-3 who have a dismal prognosis with mean survival of only a few years from diagnosis despite recent improvements in medical treatment with prostanoids, sildenafil, and endothelin antagonists.1 Patients with isolated, severe PAH and right heart failure will die of the disease if not treated with a lung transplantation (LTX) or heart–lung transplantation. However, the half-life of pediatric lung recipients is only 5.5 years.2 The superior prognosis in children with Eisenmenger physiology compared to that in children with idiopathic PAH has partly been explained by the differences in right ventricle (RV) function. In a patient with Eisenmenger, the unrestrictive right-to-left shunt precludes the development of suprasystemic RV pressure and maintains a reasonable RV function. The right-to-left shunt also allows the preservation of systemic cardiac output in the presence of RV failure.3 The Potts shunt is an anastomosis between the left pulmonary artery (LPA) and the descending aorta (Figure 1). This type
of shunt was first reported in 1946 in children with cyanotic congenital heart defects, working as a systemic-to-pulmonary shunt, that is, a left-to-right shunt.4 In this case report in a patient with PAH and a surgically closed large perimembranous ventricular septal defect (VSD), we describe the use of the Potts shunt, not as a left-to-right shunt but as a right-to-left shunt. The circulation changes from that of PAH physiology to an Eisenmenger physiology, like the one found in patients with persistent arterial duct who develop right-to-left shunting.
Case Report The boy was born prematurely on week 31 and had respiratory distress syndrome with subsequent development of bronc1 Department of Cardiothoracic Surgery, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark 2 Department of Pediatric Cardiology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
Corresponding Author: Cecilie Petersen, Department of Cardiothoracic Surgery 2152, Rigshospitalet, Copenhagen University Hospital, Blegdamsvej 9, 2200 Copenhagen, Denmark. Email: [email protected]
Downloaded from pch.sagepub.com at NORTHWESTERN UNIV LIBRARY on December 18, 2014
Petersen et al
287
Abbreviations and Acronyms BPD LPA LTX LV NT-proBNP NYHA PAB PAH RV TTE VSD
bronchopulmonary dysplasia left pulmonary artery lung transplantation left ventricle N-terminal prohormone of brain natriuretic peptide New York Heart Association pulmonary artery banding pulmonary arterial hypertension right ventricle transthoracic Doppler echocardiography ventricular septal defect
Figure 1. Illustration of a Potts shunt.
hopulmonary dysplasia (BPD). During his first year of life, he had repeated pulmonary infections and had a habitual low arterial oxygen saturation (SaO2) of 86% to 92% despite oxygen supplementation. The poor pulmonary condition of the boy was presumed to be caused by BPD alone, but at age 2 when his clinical condition started to deteriorate, it was decided to perform transthoracic Doppler echocardiography (TTE). The TTE revealed a large subaortic VSD with a right-to-left shunt, RV hypertrophy, dilatation, and pulmonary hypertension. A right heart catheterization showed a perimembraneous VSD with right-to-left shunting, systemic RV pressure, and PAH (Table 1). The findings were felt to preclude VSD closure. Treatment with endothelin receptor-selective antagonist (bosentan) was initiated but without any significant effect. After one month, a loose pulmonary artery banding (PAB) was performed with a decrease in pulmonary artery pressure from 80% of systemic pressure to 66% of systemic pressure, a drop in SaO2 from 92% to 75%, and decrease in hemoglobin from 9.5 to 8.8 mmol/L. The TTE showed a peak gradient of 55 mm Hg across the band, which had decreased to 45 mm Hg upon discharge one week later. Bosentan treatment was continued. Further tightening
of the PAB was not performed because of low SaO2 varying from 60% to 80%. After 10 months, the RV pressure had increased to a suprasystemic level, and the VSD was starting to become restrictive (Table 1). At this point, debanding was necessary to avoid RV failure. The pulmonary artery was debanded at age 3, one year after banding, and the VSD was closed with a flap valve patch. The flap valve patch was mde of 0.4-mm thick PTFE and was sutured onto one-third of the circumference of the VSD. A loose thread was sewed on to the unsecured part of the flap thus allowing an opening of approximately 4 mm. The boy was discharged with SaO2 of 80% to 90% on room air, with a combination therapy of sildenafil and bosentan. A lung biopsy, taken during the operation, showed no pathological changes. The function of the flap valve patch persisted only for nine months at which point no shunting through the VSD was observed on TTE. The clinical condition was stable in New York Heart Association (NYHA) class 2B during the following seven years. At 10 years of age, the boy experienced a rapid decline in his functional status with NYHA class decreasing from 2B to 3. TTE showed a dilated right atrium and RV with a decreased function and septum deviation compromising the left ventricle (LV), moderate tricuspid and pulmonary valve insufficiency, and completely closed VSD. Prostanoid (flolan) infusion and later treprostinil (remodulin) infusion were initiated with minimal improvement. During the next two months his functional status worsened, he became confined to his bed, and experienced frequent episodes of nausea and vomiting, interpreted as signs of cardiac failure. Repetitive TTE’s showed progressive RV failure and increasing pulmonary hypertension, with SaO2 of 82% to 85%. The clinical condition was complicated by pneumonia and sepsis, and the boy was admitted to the intensive care unit where he required tracheal intubation and mechanical ventilatory support. After successful treatment of the sepsis and extubation of the trachea, his RV function was unchanged, and the N-terminal prohormone brain natriuretic peptide (NT-proBNP) values were rising from 14.7 pmol/L to a peak value of 461 pmol/L. It was decided to create a Potts shunt to reduce the RV afterload. The shunt was made through a left thoracotomy in the fifth intercostal space. The descending aorta was exposed 10 cm distal to the subclavian artery, and the LPA was exposed from the bifurcation to a point distal to the first lobar branch. The LPA was clamped centrally, and the proximal descending aorta was isolated between two vascular clamps to make room for a large anastomosis (Figure 2). A 13- to 15-mm long opening was made in both the LPA and the descending aorta. The two vessels were anastomosed in side-to-side fashion with a running 4-0 polypropylene. The boy was weaned from mechanical ventilation 19 hours postoperatively, and the postoperative course was uneventful. The boy continued treatment with sildenafil, treprostinil, and bosentan and was discharged two weeks later. Vomiting and loss of appetite were present only during the first two months after the operation and disappeared when the hemoglobin reached levels of around 10 mmol/L. The clinical condition improved markedly, 13 months after the operation. The boy is stabile and is able to ride his bicycle to
Downloaded from pch.sagepub.com at NORTHWESTERN UNIV LIBRARY on December 18, 2014
288
World Journal for Pediatric and Congenital Heart Surgery 4(3)
Table 1. Hemodynamic Characteristics Before and After Banding of the Pulmonary Artery. Age
Procedure
1 1 2 3
Cath 110/6 87/8 Banding of pulmonary artery Cath 97/7 107/7 Debanding of pulmonary artery and VSD closure with flap valve patch
year 10 months year 11 months years 9 months years 1 month
LV Pressure, mm Hg
RV Pressure, mm Hg
PA Pressure, mm Hg
PVR (Wood Units)
84/38
23
70/36
11
Abbreviations: LV, left ventricle; RV, right ventricle; PA, pulmonary artery; PVR, pulmonary vessel resistance; Cath, right heart catheterization; VSD, ventricular septal defect.
Figure 2. Illustration showing site of side-to-side anastomosis between the left pulmonary artery and the descending thoracic aorta.
and from school every day, but his lower body becomes cyanotic when engaging in more strenuous activities. His upper body SaO2 is 93% and lower body SaO2 is 73%, and there have been no symptoms of upper body cyanosis since two months after the operation. His NT-proBNP level has decreased to 10 pmol/L, and hemoglobin has increased from 7.5 to 10.2 mmol/L. Improvement was also noted on TTE, where the LV was less compressed by the RV (Figure 3).
Discussion This is the first case report that describes significant clinical improvement after performance of a Potts shunt in a child with end-stage PAH after late closure of a VSD. One earlier article reported similar improvement in two patients with transposition of the great arteries that had undergone arterial switch surgery,5 and two other reports had similar findings in 10 children with idiopathic pulmonary hypertension.6,7
Figure 3. Transthoracic echocardiogram showing parasternal longaxis view of the heart in end-diastolic phase. A, Before Potts shunt operation. B, One month after Potts shunt operation. LV, left ventricle; RV, right ventricle.
In our case, the boy had progressive RV failure with NTproBNP values increasing from 14.7 to 461 pmol/L through five months in spite of maximum pharmacological treatment. We considered two different treatments: decompression of the RV by providing an excess flow valve at intra- or extracardiac level that is converting the patient’s physiology into Eisenmenger physiology or transplantation. An atrial shunt is creation of an atrial septum defect that could present a risk of uncontrolled flow of deoxygenated blood from the pulmonary circulation to the systemic circulation causing serious hypoxemia. The same
Downloaded from pch.sagepub.com at NORTHWESTERN UNIV LIBRARY on December 18, 2014
Petersen et al
289
scenario could be expected if a shunt was made at ventricular level. However, creating an extracardiac shunt, such as the Potts shunt, between the systemic and the pulmonary circulation would exclusively shunt cyanotic blood to the lower body and maintain normal or near-normal SaO2 in the upper part of the body. Patients with Eisenmenger physiology are reported to live longer and to have less RV failure than patients with primary pulmonary hypertension.3,8 In our case, the improvement in RV function was demonstrated by both the TTE and the decrease in NT-proBNP values, after surgery. The final therapeutic option for pharmacological nonresponding pulmonary hypertension is LTX. The organ shortage and the limited life span, with a half-life of only 5.5 years, make this option a last resort, which should be postponed until there is no other alternative.2 In our case, the Potts shunt immediately reduced the afterload on the RV, thus avoiding RV failure, as shown by others.5-7 The subsequent option for transplantation is not prohibited, although there may be an increased risk compared to a standard transplantation, in view of the need to takedown the shunt at the time of transplantation. However, there are no reported experiences in the literature of LTX or heart–lung transplantation in patients with a Potts shunt. The survival rate for patients with Eisenmenger is 42% at 25 years and is therefore much more favorable than that of patients which is 50% at 5.5 years.2,3 In the present case, our patient benefited from the Potts shunt and experienced clinical improvement. There might be a future potential in treating patients with RV failure and severe PAH with the Potts shunt, thereby postponing the time of transplantation. Declaration of Conflicting Interests The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The authors received no financial support for the research, authorship, and/or publication of this article.
References 1. Haworth SG, Hislop AA. Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart. 2009; 95(4): 312-317. 2. Benden C, Aurora P, Edwards LB, et al. The registry of the International Society for Heart and Lung Transplantation: fourteenth pediatric lung and heart-lung transplantation report—2011. J Heart Lung Transplant. 2011;30(10): 1123-1132. 3. Hopkins WE, Waggoner AD. Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome. Am J Cardiol. 2002;89(1): 34-38. 4. Potts WJ, Smith S, Gibson S. Anastomosis of the aorta to a pulmonary artery. J Am Med Assoc. 1946;132(11): 627-631. 5. Blanc J, Vouhe P, Bonnet D. Potts shunt in patients with pulmonary hypertension. New Eng J Med. 2004;350(6): 623. 6. Labombarda F, Maragnes P, Dupont-Chauvet P, Serraf A. Potts anastomosis for children with idiopathic pulmonary hypertension. Ped Cardiol. 2009;30(8): 1143-1145. 7. Baruteau AE, Serraf A, Levy M, et al. Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results. Ann Thorac Surg. 2012;94(3): 817-824. 8. Hopkins WE, Ochoa LL, Richardson GW, Trulock EP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996;15(1 pt 1): 100-105.
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