Pulmonary-to-Systemic Arterial Shunt to Treat Children With Severe Pulmonary Hypertension.

BACKGROUND: The placement of a pulmonary-to-systemic arterial shunt in children with severe pulmonary hypertension (PH) has been demonstrated, in relatively small studies, to be an effective palliation for their disease. OBJECTIVES: The aim of this study was to expand upon these earlier findings using an international registry for children with PH who have undergone a shunt procedure. METHODS: Retrospective data were obtained from 110 children with PH who underwent a shunt procedure collected from 13 institutions in Europe and the United States. RESULTS: Seventeen children died in-hospital postprocedure (15%). Of the 93 children successfully discharged home, 18 subsequently died or underwent lung transplantation (20%); the mean follow-up was 3.1 years (range: 25 days to 17 years). The overall 1- and 5-year freedom from death or transplant rates were 77% and 58%, respectively, and 92% and 68% for those discharged home, respectively. Children discharged home had significantly improved World Health Organization functional class (P < 0.001), 6-minute walk distances (P = 0.047) and lower brain natriuretic peptide levels (P < 0.001). Postprocedure, 59% of children were weaned completely from their prostacyclin infusion (P < 0.001). Preprocedural risk factors for dying in-hospital postprocedure included intensive care unit admission (hazard ratio [HR]: 3.2; P = 0.02), mechanical ventilation (HR: 8.3; P < 0.001) and extracorporeal membrane oxygenation (HR: 10.7; P < 0.001). CONCLUSIONS: A pulmonary-to-systemic arterial shunt can provide a child with severe PH significant clinical improvement that is both durable and potentially free from continuous prostacyclin infusion. Five-year survival is comparable to children undergoing lung transplantation for PH. Children with severely decompensated disease requiring aggressive intensive care are not good candidates for the shunt procedure.

Outcomes of Atrioseptostomy with Stenting in Patients with Pulmonary Arterial Hypertension from a Large Single-Institution Cohort.

The aim of this study was to analyze results of stenting atrioseptostomy in patients with pulmonary arterial hypertension and a different level of risk for one-year mortality that is not well described. Patients that underwent atrioseptostomy with stenting were retrospectively divided in two groups: "intermediate" (n = 55) or "high" risk (n = 13), according to the 2015 ESC/ESR guideline. Results of atrioseptostomy were assessed during hospital period and at follow-up. Patients from "intermediate" risk group demonstrated lower mortality rate (10/55, vs. 6/13) during the course of the study period, as well as higher freedom from lung transplantation or Potts shunt. At discharge, patients of both groups presented improvement in functional class and mobility. Patients from "intermediate" risk group showed longer 6-min walking distance, and lower levels of brain natriuretic peptide. At the latest follow-up, stable position and full patency of stents with right-to-left or bidirectional shunt at atrial level and absence of syncope was confirmed in patients of both groups. Patients from the "intermediate" risk group demonstrated higher functional class, better performance of walking test, and lower levels of brain natriuretic peptide. Stenting atrioseptostomy reliably secured interatrial communication and improved clinical condition in patients with idiopathic pulmonary arterial hypertension. Mid-term results were better in "intermediate" risk group.

Potts shunt in children with pulmonary arterial hypertension: institutional experience.

OBJECTIVES: Since 2004, different techniques for the Potts shunt have been used in patients with incurable pulmonary arterial hypertension. However, the experience with these interventions in a single institution is limited. We present our series of Potts shunts in children with pulmonary arterial hypertension. METHODS: Eight patients with suprasystemic pulmonary arterial hypertension (pulmonary artery-to-aorta mean pressure ratio of 1.39 ± 0.30) had a Potts shunt at the median age of 13.5 months. Six patients were followed up for 2-32 (median 17) months after the operation. RESULTS: In all cases, the Potts shunt caused a decrease in arterial blood oxygen saturation at the lower extremities and was the genesis of the arterial blood oxygen saturation gradient between the upper and lower extremities (10.5 ± 1.8% at discharge). Postoperative echocardiography revealed flattening of the ventricular septum as well as a decrease in the right ventricle-to-left ventricle end-diastolic diameter ratio from 1.36 ± 0.14 preoperatively to 0.99 ± 0.22 at discharge (P = 0.010). The 2 patients with the highest preoperative pulmonary artery-to-aorta mean pressure ratio (1.79-1.86) died during the early postoperative period from repeated pulmonary hypertension crises and uncontrolled hypoxaemia. There were no deaths during the follow-up period. The paediatric functional class of the patients improved from IIIb-IV to I-II, syncopal episodes no longer persisted and the weight deficit decreased from 21.8 ± 7.5% preoperatively to 11.5 ± 5.9% at the latest follow-up examination (P = 0.004). The arterial blood oxygen saturation gradient between the upper and lower extremities and the right ventricle-to-left ventricle end-diastolic diameter ratio were the same as they were while the patients were in the hospital. CONCLUSIONS: Our limited experience demonstrates that the Potts shunt lowers suprasystemic pulmonary hypertension to the systemic level, limits manifestations of right-sided heart failure and improves the overall functional status of the patients.