Potts shunt in children with idiopathic pulmonary arterial hypertension: long-term results.
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.
Centre Chirurgical Marie Lannelongue, Département de Chirurgie cardiaque des cardiopathies congénitales, Le Plessis-Robinson, France. a.baruteau@ccml.fr
Alban-Elouen
AE
Baruteau
Alain
A
Serraf
Maryline
M
Lévy
Jérôme
J
Petit
Damien
D
Bonnet
Xavier
X
Jais
Pascal
P
Vouhé
Gérald
G
Simonneau
Emre
E
Belli
Marc
M
Humbert
D000367:Age Factors; D000714:Anastomosis, Surgical / Q000379:methods; D001013:Aorta, Thoracic / Q000601:surgery*; D058366:Blalock-Taussig Procedure / Q000379:methods; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016638:Critical Illness; D004452:Echocardiography / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D006976:Hypertension, Pulmonary / Q000151:congenital / Q000000981:diagnostic imaging / Q000401:mortality* / Q000601:surgery*; D007223:Infant; D008297:Male; D010166:Palliative Care / Q000379:methods*; D011651:Pulmonary Artery / Q000601:surgery*; D012189:Retrospective Studies; D018570:Risk Assessment; D012737:Sex Factors; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome