Correspondence

Potts Shunt in Patients with Pulmonary Hypertension

N Engl J Med 2004; 350:623February 5, 2004

Article

To the Editor:

Pulmonary hypertension is a rare complication of transposition of the great arteries.1 As in other forms of primary pulmonary hypertension, the prognosis is poor, particularly in children who have symptoms.2 Converting normal cardiac physiological features to features typical of Eisenmenger's syndrome by means of blade atrial septostomy has already been proposed for patients with this condition.3 But patients with severe right heart failure and markedly elevated pulmonary vascular resistance do not tolerate atrial septostomy, because massive right-to-left shunting may result in insufficient pulmonary blood flow and severe hypoxemia.

Therefore, in the cases of two boys who had suprasystemic pulmonary hypertension and right ventricular failure 4 years and 14 years after an arterial switch performed for transposition of the great arteries, we decided to create an anastomosis between the descending aorta and the left pulmonary artery (Potts shunt) (Figure 1Figure 1The Potts Shunt Procedure.). The ratio of pulmonary blood flow to systemic blood flow was 1, as there was no intracardiac shunt in either boy. The aim of this intervention was to decrease right ventricular afterload so as to improve right ventricular function and potentially prevent syncope and sudden death. The procedures were uneventful.

The patients' condition improved rapidly, and both are now in New York Heart Association functional class II. Right ventricular function was restored. Right-to-left shunting through the Potts shunt was responsible for cyanosis of the lower limbs, and consequently, polycythemia developed in both patients (the hematocrit values were 51 percent and 55 percent at the last follow-up). The patients did not have any further episodes of syncope after a follow-up of 18 and 6 months, respectively. Hemodynamic evaluation confirmed that the pulmonary-artery pressure and aortic pressure were equal. Left-heart output was preserved.

The advantages of the Potts procedure are the immediate decrease in the right ventricular afterload and the absence of cyanosis of the upper part of the body in the absence of intracardiac shunting. The chief concern remains the operative risk in patients with pulmonary hypertension. Without treatment, the mean survival of children is less than one year after diagnosis.2 For adults with pulmonary hypertension who are treated with prostacyclin analogues, the survival rate is estimated to be about 55 percent at five years,4 but comparable studies in children have not been conducted. Median survival in adults with Eisenmenger's syndrome is 53 years.5 Therefore, the Potts procedure could be an alternative for the treatment of children with severe pulmonary hypertension and right ventricular failure.

Julie Blanc, M.D.
Pascal Vouhé, M.D.
Damien Bonnet, M.D., Ph.D.
Hôpital Necker–Enfants Malades, 75015 Paris, France
damien.bonnet@nck.ap-hop-paris.fr

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Citing Articles (12)

Citing Articles

1. 1

   Cécile Tissot, Maurice Beghetti. 2012. Pediatric Pulmonary Hypertension. , 730-752.
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2. 2

   Minh Thanh Bui, Oswin Grollmus, Mohamedou Ly, Anca Mandache, Elie Fadel, Benoit Decante, Alain Serraf. (2011) Surgical palliation of primary pulmonary arterial hypertension by a unidirectional valved Potts anastomosis in an animal model. The Journal of Thoracic and Cardiovascular Surgery 142:5, 1223-1228
   CrossRef

3. 3

   Astrid E Lammers, Sheila G Haworth, Gerhard-Paul Diller. (2011) Atrial septostomy in patients with pulmonary hypertension: should it be recommended?. Expert Review of Respiratory Medicine 5:3, 363-376
   CrossRef

4. 4

   M.T.R. Roofthooft, R.L.E. van Loon, R.M.F. Berger. (2010) Management of Pulmonary Arterial Hypertension in Children. Paediatric Respiratory Reviews 11:4, 240-245
   CrossRef

5. 5

   Tilman Humpl, Ingram Schulze-Neick. 2010. Pulmonary Vascular Disease. , 1147-1161.
   CrossRef

6. 6

   Nazzareno Galiè, Marius M. Hoeper, Marc Humbert, Adam Torbicki, Jean-Luc Vachiery, Joan Albert Barberá, Maurice Beghetti, Paul Corris, Sean Gaine, J. Simon Gibbs, Miguel Ángel Gómez-Sánchez, Guillaume Jondeau, Walter Klepetko, Christian Opitz, Andrew Peacock, Lewis Rubin, Michael Zellweger, Gerald Simonneau. (2009) Guía de práctica clínica para el diagnóstico y tratamiento de la hipertensión pulmonar. Revista Española de Cardiología 62:12, 1464.e1-1464.e58
   CrossRef

7. 7

   Fabien Labombarda, Pascale Maragnes, Peggy Dupont-Chauvet, Alain Serraf. (2009) Potts Anastomosis for Children with Idiopathic Pulmonary Hypertension. Pediatric Cardiology 30:8, 1143-1145
   CrossRef

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   , N. Galie, M. M. Hoeper, M. Humbert, A. Torbicki, J.-L. Vachiery, J. A. Barbera, M. Beghetti, P. Corris, S. Gaine, J. S. Gibbs, M. A. Gomez-Sanchez, G. Jondeau, W. Klepetko, C. Opitz, A. Peacock, L. Rubin, M. Zellweger, G. Simonneau, , A. Vahanian, A. Auricchio, J. Bax, C. Ceconi, V. Dean, G. Filippatos, C. Funck-Brentano, R. Hobbs, P. Kearney, T. McDonagh, K. McGregor, B. A. Popescu, Z. Reiner, U. Sechtem, P. A. Sirnes, M. Tendera, P. Vardas, P. Widimsky, , U. Sechtem, N. Al Attar, F. Andreotti, M. Aschermann, R. Asteggiano, R. Benza, R. Berger, D. Bonnet, M. Delcroix, L. Howard, A. N. Kitsiou, I. Lang, A. Maggioni, J. E. Nielsen-Kudsk, M. Park, P. Perrone-Filardi, S. Price, M. T. S. Domenech, A. Vonk-Noordegraaf, J. L. Zamorano. (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). European Heart Journal 30:20, 2493-2537
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   Cecile Tissot, Maurice Beghetti. (2009) Advances in therapies for pediatric pulmonary arterial hypertension. Expert Review of Respiratory Medicine 3:3, 265-282
   CrossRef

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    Anne M. Keogh, Eckhard Mayer, Raymond L. Benza, Paul Corris, Philippe G. Dartevelle, Adaani E. Frost, Nick H. Kim, Irene M. Lang, Joanna Pepke-Zaba, Julio Sandoval. (2009) Interventional and Surgical Modalities of Treatment in Pulmonary Hypertension. Journal of the American College of Cardiology 54:1, S67-S77
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    Robyn J. Barst, Duncan Stewart. (2009) Conventional and targeted medical therapies. Cardiology in the Young 19:S1, 28
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    Emanuela Angeli, Olivier Raisky, Damien Bonnet, Daniel Sidi, Pascal R. Vouhé. (2008) Late reoperations after neonatal arterial switch operation for transposition of the great arteries. European Journal of Cardio-Thoracic Surgery 34:1, 32-36
    CrossRef