Potts Shunt in Children With Idiopathic Pulmonary Arterial Hypertension: Long-Term Results

doi:10.1016/j.athoracsur.2012.03.099

The Annals of Thoracic Surgery

Volume 94, Issue 3, September 2012, Pages 817-824

https://doi.org/10.1016/j.athoracsur.2012.03.099 Get rights and content

Background

Idiopathic pulmonary arterial hypertension (IPAH) remains a progressive fatal disease. Palliative Potts shunt has been proposed in children displaying suprasystemic IPAH.

Methods

A retrospective multicenter study was performed to evaluate Potts shunt in pediatric IPAH.

Results

Between 2003 and 2010, 8 children with suprasystemic IPAH and in World Health Organization functional class IV despite medical pulmonary arterial hypertension therapy underwent Potts shunt. Age at IPAH diagnosis ranged from 4 to 180 months (median age, 64 months). Surgical procedure was performed in a mean delay of 41.9 ± 54.3 months (range, 4 to 167 months; median delay, 20 months) after IPAH diagnosis. Mean size of the Potts shunt was 9.25 ± 3.30 mm. Two patients, whose medical pulmonary arterial hypertension therapy had been interrupted just after surgery, died at postoperative days 11 and 13 of acute pulmonary hypertensive crisis. After a mean follow-up of 63.7 ± 16.1 months, the 6 children who were discharged from the hospital were alive. Functional status improved markedly in the 6 survivors, with a World Health Organization functional class I (n = 4) or II (n = 2) at last follow-up, consistent with significant improvement of 6-minute-walk distance (302 ± 95 m [51% ± 20% of theoretical values] versus 456 ± 91 m [68% ± 10% of theoretical values]; p = 0.038) and decrease of brain natriuretic peptide levels (608 ± 109 pg/mL versus 76 ± 45 pg/mL; p = 0.035). No Potts shunt was found to be restrictive at last echocardiography.

Conclusions

Palliative Potts shunt constitutes a new alternative to lung transplantation in severely ill children with suprasystemic IPAH, carrying a prolonged survival and persistent improvement in functional capacities.

Section snippets

Patients and Methods

A retrospective multicenter study was performed to evaluate the operative risk and long-term results of the first consecutive children with IPAH undergoing Potts shunt in France. This program was approved by the ethics committee of each institution. An informed consent was obtained for each child or their parents.

Between 2003 and 2010, 8 children were referred to one of our tertiary institutions for refractory IPAH. Inclusion criteria in the Potts shunt program were children with IPAH with

Preoperative Considerations

Eight children with IPAH underwent a Potts shunt (5 girls and 3 boys). Their IPAH was diagnosed at a mean age of 68.4 ± 54.4 months (range, 4 to 180 months; median age, 64 months). Potts shunt was performed at a mean delay of 41.9 ± 54.3 months (range, 4 to 167 months; median delay, 20 months) after IPAH diagnosis.

When referred for Potts shunt, all children were in NYHA/WHO functional class IV. All had asthenia and growth retardation. The majority presented with repeated syncope (n = 6) and

Comment

Alternatives for children who worsen while receiving medical therapy for IPAH or those who experience repeated syncope or right heart failure are limited to lung or heart-lung transplantation and balloon atrial septostomy. Lung or heart-lung transplantation is the only surgical option in terminally ill children to date [24]. It carries several drawbacks and a limited median survival, with rates reported between 4.5 and 5.8 years [25, 26, 27, 28, 29, 30, 31]. Schaellibaum and colleagues [31]

Addendum

During the review process, a 32-month-old boy underwent the Potts procedure for refractory IPAH with a favorable early outcome.

References (38)

A. Fraisse et al.
Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France
Arch Cardiovasc Dis
(2010)
J. Sandoval et al.
Primary pulmonary hypertension in children: clinical characterization and survival
J Am Coll Cardiol
(1995)
R.L. van Loon et al.
Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies
Am J Cardiol
(2010)
W.E. Hopkins et al.
Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome
Am J Cardiol
(2002)
A.M. Keogh et al.
Interventional and surgical modalities of treatment in pulmonary hypertension
J Am Coll Cardiol
(2009)
P. Aurora et al.
Registry of the International Society for Heart and Lung Transplantation: Twelfth Official Pediatric Lung and Heart/Lung Transplantation Report-2009
J Heart Lung Transplant
(2009)
B.S. Goldstein et al.
Lung transplantation in children with idiopathic pulmonary arterial hypertension: an 18-year experience
J Heart Lung Transplant
(2011)
J. Sandoval et al.
Graded balloon dilation atrial septostomy in severe primary pulmonary hypertensionA therapeutic alternative for patients nonresponsive to vasodilator treatment
J Am Coll Cardiol
(1998)
R.J. Barst et al.
Updated evidence-based treatment algorithm in pulmonary arterial hypertension
J Am Coll Cardiol
(2009)
N. Galiè et al.
Guidelines for the diagnosis and treatment of pulmonary hypertension
Eur Respir J
(2009)

M. Humbert et al.

Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era

Circulation

(2010)

M. Humbert et al.

Pulmonary arterial hypertension in France: results from a national registry

Am J Respir Crit Care Med

(2006)

V.V. McLaughlin et al.

ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association

Circulation

(2009)

L.J. Rubin

Primary pulmonary hypertension

N Engl J Med

(1997)

G.E. D'Alonzo et al.

Survival in patients with primary pulmonary hypertensionResults from a national prospective registry

Ann Intern Med

(1991)

M. Humbert et al.

Treatment of pulmonary arterial hypertension

N Engl J Med

(2004)

D. Yung et al.

Outcomes in children with idiopathic pulmonary arterial hypertension

Circulation

(2004)

S. Moledina et al.

Childhood idiopathic pulmonary arterial hypertension: a national cohort study

Heart

(2010)

O.G. Thilenius et al.

Primary pulmonary vascular obstruction in children

Pediatrics

(1965)

Cited by (110)

Thirty years of surgical management of pediatric pulmonary hypertension: Mid-term outcomes following reverse Potts shunt and transplantation
2024, Journal of Thoracic and Cardiovascular Surgery
Reverse Potts shunt (RPS) and lung or heart–lung transplantation are life-extending surgical interventions for pediatric patients with severe pulmonary arterial hypertension (PAH). Robust criteria for identifying patients who will benefit from these procedures remain elusive. Based on 30 years of experience, we sought to refine the surgical indications.
This single-center retrospective cohort study included 61 consecutive pediatric patients with PAH managed by RPS (2004-2020) or transplantation (1988-2020). Their mid-term outcomes were assessed.
Compared with the 20 patients managed by RPS, the 41 transplant waitlist patients, of whom 28 were transplanted, were older (14.9 vs 8.0 years, P = .0001), had worse right ventricular impairment (tricuspid annular plane systolic excursion, 12.5 mm vs 18.0 mm, P = .03), and were managed later in the evolution of the disease (6.0 vs 1.7 years, P = .002). After implementation of a high-priority allocation program in 2007, waitlist mortality decreased from 52.6% to 13.6% (P = .02) and 5-year survival increased from 57.1% to 74.7% after RPS and 55.6% to 77.2% after transplantation. At a median follow-up of 8.6 years after RPS and 5.9 years after transplantation, functional capacity had improved significantly, and PAH-specific drug requirements had diminished markedly in the RPS group. Two patients successfully underwent double-lung transplant 6 and 9 years after RPS.
In selected children with suprasystemic PAH, RPS is associated with functional capacity improvements and decreased pharmacotherapy needs over the midterm. RPS deserves consideration earlier in the course of pediatric PAH, with transplantation being performed in the event of refractory RV failure.
Treatment of pulmonary arterial hypertension: recent progress and a look to the future
2023, The Lancet Respiratory Medicine
Pulmonary arterial hypertension (PAH) is a severe but treatable form of pre-capillary pulmonary hypertension caused by pulmonary vascular remodelling. As a result of basic science discoveries, randomised controlled trials, studies of real-world data, and the development of clinical practice guidelines, considerable progress has been made in the treatment options and outcomes for patients with PAH, underscoring the importance of seamless translation of information from bench to bedside and, ultimately, to patients. However, PAH still carries a high mortality rate, which emphasises the urgent need for transformative innovations in the field. In this Series paper, written by a group of clinicians, researchers, and a patient with PAH, we review therapeutic approaches and treatment options for PAH. We summarise current knowledge of the cellular and molecular mechanisms of PAH, with an emphasis on emerging treatable pathways and optimisation of current management strategies. In considering future directions for the field, our ambition is to identify therapies with the potential to stall or reverse pulmonary vascular remodelling. We highlight novel therapeutic approaches, the important role of patients as partners in research, and innovative approaches to PAH clinical trials.
Transcatheter Valve Implantation in Reversed Potts Shunt in Pulmonary Arterial Hypertension: Keeping the Shunt Reversed
2022, JACC: Case Reports
Commentary: Keeping the reversed Potts shunt reversed: Insights from the fourth dimension
2022, Journal of Thoracic and Cardiovascular Surgery
Monitoring and evaluation of the surgical Potts shunt physiology using 4-dimensional flow magnetic resonance imaging
2022, Journal of Thoracic and Cardiovascular Surgery
The reversed Potts shunt is an increasingly applied mode of surgical palliation of severe pulmonary hypertension (PH). However, the long-term flow hemodynamic effect of the Potts shunt physiology and desirable long-term hemodynamic end points are not defined. The purpose of this descriptive study was to analyze a series of pediatric patients who underwent surgical Potts shunt as a part of end-stage PH palliation using 4-dimensional (4D)-flow magnetic resonance imaging (MRI) to (1) quantitate the flow through the anastomosis, (2) correlate the shunting pattern with phases of cardiac cycle and PH comorbidities, and (3) describe chronologic changes in shunting pattern.
This was a 2-center study evaluating 4 patients seen in the Pulmonary Hypertension Clinic at Children's Hospital Colorado who were evaluated and selected to undergo surgical reverse Potts shunt at Washington University School of Medicine and were serially followed using comprehensive imaging including cardiac MRI and 4D-flow MRI.
After the procedure, each child underwent 2 4D-flow MRI evaluations. Pulmonary pressure offload was evident in all patients, as demonstrated by positive systolic right-to-left flow across the Potts shunt. All patients experienced some degree of the flow reversal, which occurs primarily in diastole. Interventricular dyssynchrony further contributed to flow reversal across the Potts shunt. Lastly, systemic and pulmonary blood mixing in the descending aorta results in secondary helical flow persisting throughout the diastole.
4D-flow MRI demonstrates that children who have undergone a Potts shunt for severe PH can experience shunt flow reversal. Cumulatively, this left-to-right pulmonary shunt adds to right ventricular volume overload. We speculate that a valved conduit may decrease the left to right shunting and improve overall cardiac output.
Pediatric heart-lung transplantation: Technique and special considerations
2022, Journal of Heart and Lung Transplantation
Citation Excerpt :
This procedure mimics the physiology of Eisenmenger syndrome by creating an unrestrictive pulmonary-to-systemic shunt and decreasing the right ventricular afterload.28 Originally described in 1946 for the treatment of TOF, the Potts shunt has recently gained prominence as a surgical treatment for children with severe PH.29-32 Data regarding heart-lung transplantation after Potts shunt placement is scarce. In 1998, Fann et al performed a combined heart and single-lung transplant in a 33-year-old with tricuspid and pulmonary atresia who had undergone a Glenn and a Potts shunt during childhood, with no complications at 1-year follow-up.33
Heart-lung transplantation has historically been used as a definitive treatment for children with end-stage cardiopulmonary failure, although the number performed has steadily decreased over time. In this review, we discuss current indications, preoperative risk factors, outcomes, and heart-lung transplantation in unique patient subsets, including infants, children with single-ventricle physiology, tetralogy of Fallot/major aortopulmonary collateral arteries, and prior Potts shunt palliation. We also describe the different surgical techniques utilized in pediatric heart-lung transplantation.

View all citing articles on Scopus

View full text

Original articlePediatric cardiacPotts Shunt in Children With Idiopathic Pulmonary Arterial Hypertension: Long-Term Results

Background

Methods

Results

Conclusions

Section snippets

Patients and Methods

Preoperative Considerations

Comment

Addendum

Arch Cardiovasc Dis

J Am Coll Cardiol

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

J Heart Lung Transplant

J Heart Lung Transplant

J Am Coll Cardiol

J Am Coll Cardiol

Guidelines for the diagnosis and treatment of pulmonary hypertension

Eur Respir J

Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era

Circulation

Pulmonary arterial hypertension in France: results from a national registry

Am J Respir Crit Care Med

Circulation

Primary pulmonary hypertension

N Engl J Med

Survival in patients with primary pulmonary hypertensionResults from a national prospective registry

Ann Intern Med

Treatment of pulmonary arterial hypertension

N Engl J Med

Outcomes in children with idiopathic pulmonary arterial hypertension

Circulation

Childhood idiopathic pulmonary arterial hypertension: a national cohort study

Heart

Primary pulmonary vascular obstruction in children

Pediatrics

Original article
Pediatric cardiac
Potts Shunt in Children With Idiopathic Pulmonary Arterial Hypertension: Long-Term Results