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What's Old is New Again: Palliative Potts Shunt in Children with PAH


Stephanie Handler, MD
Children's Hospital of Wisconsin
Milwaukee, WI, USA
SHandler@chw.org



Despite advances in medical therapy and continued approval of new drugs to treat WHO Group 1 pulmonary arterial hypertension (PAH) in adults, 5-year survival for pediatric patients is still only 74% [1]. Even with initial symptomatic improvement in patients on prostacyclin therapy, many experience a decline in right ventricular systolic function associated with worsening functional class due to disease progression. In those patients, lung transplantation has been the only option.

The Potts shunt (direct anastomosis of left pulmonary artery to descending aorta) was first performed in 1946 at Children's Memorial hospital in Chicago by Dr Willis J Potts as a "blue baby" operation to provide pulmonary blood flow for cyanotic congenital heart disease. Palliative 'reverse' Potts shunt in which flow is directed from the pulmonary artery to the aorta was first described in 2 children with PAH in the New England Journal of Medicine in 2004 by Dr Blanc and colleagues [2].

The concept of converting a failing right ventricle into 'Eisenmenger physiology' with a 'pop-off' to the systemic circulation to maintain cardiac output is appealing given the improved survival of adult patients with Eisenmenger physiology compared to those with idiopathic PAH [3,4]. The same survival benefit was not seen in the REVEAL registry analysis of pediatric patients with Eisenmenger but included only 4- & 7-year follow up [5]. The idea is similar to creation of an atrial level shunt, but has the benefit of providing only lower body desaturation while the upper body continues to receive fully oxygenated blood with a lower risk of embolism to the brain.

Dr Baruteau and colleagues in Europe have published the largest series of palliative Potts shunts in pediatric patients- 24 patients from 2003-2014 underwent the procedure (19 surgical shunts- 1 unidirectional valved shunt for infrasystemic pulmonary artery pressure, 5 transcatheter PDA stents) with 3 early deaths (12.5%) and all 21 survivors experiencing improvement in functional class [6]. In the six patients on intravenous epoprostenol therapy, all were able to be weaned off.

Drs Grady and Eghtesady report the largest North American experience at Washington University with 5 patients undergoing surgical Potts shunt- 4 Group 1 PAH and 1 Group 2 with left heart disease [7]. The first two patients were placed on ECMO in the operating room prior to the procedure and were successfully decannulated after shunt placement. Overall, 4 of 5 patients had clinical improvement; the patient who did not benefit from the procedure had subsystemic PA pressures and died 3 months post-operatively.

Finally, there is a small series of transcatheter Potts shunt creation in the absence of a PDA out of Boston with technical success in 3 of 4 patients (1 died during the procedure as a result of a hemothorax) with short term survival of only 2 patients [8]. This technique consists of needle perforation of the descending aorta at the site of apposition the left pulmonary artery under fluoroscopic guidance to allow covered stent deployment between the vessels. At this time, it should probably be reserved for patients with drug refractory PAH and/or co-morbidities that are unfavorable for a surgical approach, and even then only in the hands of a very experienced pediatric interventional cardiologist.

Given the limited experience in pediatrics, Potts shunt may be considered as destination therapy or bridge to transplant based on the small aforementioned series in the European Paediatric Pulmonary Vascular Disease Network treatment consensus statement (Class IIb, Level of Evidence C - same class, evidence as atrial septostomy) [9]. Potts shunt is not included as a therapeutic option in the American Heart Association and American Thoracic Society guidelines recently published in Circulation although atrial septostomy is recommended for patients with RV failure, recurrent syncope, or crises despite optimized medical management (Class I, Level of Evidence B) [10].

As Dr Potts was quoted as saying in an October 27th, 1961 edition of the Chicago Daily Tribune describing his technique, "Progress in pediatric surgery has been so recent that a thorough realization of what can be done for children has not yet filtered through the medical profession to parents." This remains true today with new utilization of an old technique, one I'm sure Dr Potts would never have envisioned as he first thought of the procedure to relieve cyanosis. Although the goal remains the same- to improve the quality of life in pediatric patients with heart disease. ■

Disclosure Statement: The author has no conflicts of interest to disclose.


References:

  1. Barst RJ, McGoon MD, Elliott CG, Foreman AJ, Miller DP, Ivy DD. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation. 2012 Jan 3;125(1):113-22.
  2. Blanc J, Vouhé P, Bonnet D. Potts shunt in patients with pulmonary hypertension. N Engl J Med. 2004 Feb 5;350(6):623.
  3. W.E. Hopkins, L.L. Ochoa, G.W. Richardson, E.P. Trulock. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant, 15 (1 Pt 1) (1996), pp. 100-105.
  4. Diller GP, Dimopoulos K, Broberg CS, Kaya MG, Naghotra US, Uebing A, Harries C, Goktekin O, Gibbs JS, Gatzoulis MA. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur Heart J, 27 (2006), pp. 1737-1742
  5. Barst RJ, Ivy DD, Foreman AJ, McGoon MD, Rosenzweig EB. Four- and seven-year outcomes of patients with congenital heart disease associated pulmonary arterial hypertension (from the REVEAL Registry). Am J Cardiol. 2014 Jan 1;113(1):147-55.
  6. Baruteau AE, Belli E, Boudjemline Y, Laux D, Lévy M, Simonneau G, Carotti A, Humbert M, Bonnet D. Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterialhypertension: updated data from the first 24 patients. Eur J Cardiothorac Surg. 2015 Mar;47(3):e105-10.
  7. Grady RM, Eghtesady P. Potts Shunt and Pediatric Pulmonary Hypertension: What We Have Learned. Ann Thorac Surg. 2016 Apr;101(4):1539-43
  8. Esch JJ1, Shah PB, Cockrill BA, Farber HW, Landzberg MJ, Mehra MR, Mullen MP, Opotowsky AR, Waxman AB, Lock JE, Marshall AC. Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience. J Heart Lung Transplant. 2013 Apr;32(4):381-7.
  9. Hansmann G, Apitz C. Treatment of children with pulmonary hypertension. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016 May;102 Suppl 2:ii67-85
  10. Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, Hanna BD, Rosenzweig EB, Raj JU, Cornfield D, Stenmark KR, Steinhorn R, Thébaud B,Fineman JR, Kuehne T, Feinstein JA, Friedberg MK, Earing M, Barst RJ, Keller RL, Kinsella JP, Mullen M, Deterding R, Kulik T, Mallory G, Humpl T, Wessel DL. Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society. Circulation. 2015 Nov 24;132(21):2037-99.



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