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Left Lung Orthotopic Transplantation in a Juvenile Porcine Model for ESLP
Published on: February 14, 2022
Lung transplantation in children with idiopathic pulmonary arterial hypertension
Astrid E Lammers1, Michael Burch, Christian Benden
1Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK. astridlammers@gmx.de
Insights
Lung transplantation offers a promising outcome for children with idiopathic pulmonary arterial hypertension (IPAH) who meet listing criteria. Careful patient selection is crucial for successful outcomes in pediatric IPAH transplantation.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension
- Transplantation Medicine
Background:
- Idiopathic pulmonary arterial hypertension (IPAH) is a severe condition in children.
- Lung transplantation (Tx) is the only definitive treatment for end-stage pediatric pulmonary vascular disease.
Purpose of the Study:
- To evaluate the outcomes of lung transplantation in children with idiopathic pulmonary arterial hypertension (IPAH).
- To analyze the criteria for listing pediatric patients with IPAH for lung transplantation.
Main Methods:
- Retrospective review of clinical data for children with IPAH referred for transplantation assessment.
- Comparison of demographic, clinical, and functional parameters between listed and non-listed patients.
- Analysis of outcomes for transplanted patients and those awaiting transplantation.
Main Results:
- Seven of 14 assessed children with IPAH were listed for transplantation; five underwent lung or heart-lung Tx.
- Listed children had significantly worse functional class, lower exercise SpO(2), and reduced 6-minute walk distance compared to non-listed.
- Transplanted children showed good functional outcomes at 2.8 years post-Tx, while two died on the waiting list.
Conclusions:
- Lung transplantation outcomes in pediatric IPAH patients are encouraging.
- Establishing precise listing criteria for pediatric IPAH patients remains a significant challenge.
Background:
Despite improved medical therapy, transplantation (Tx) represents the only option for end-stage pulmonary vascular disease.
Methods:
Clinical data of children with idiopathic pulmonary arterial hypertension (IPAH) referred for Tx assessment between January 2002 and June 2007 were related to listing decision and outcome.
Results:
Seven of the 14 children assessed for Tx were listed. Five were transplanted (lung Tx, n = 3; heart-lung Tx, n = 2) and two died on the waiting list. Mean age at diagnosis was 3.7 (0.4-9.5) years. Time from diagnosis to listing was 3.6 years (range 1.4-9.3). Children listed were in a worse functional class (WHO 3.5 vs. 2.5; P = 0.0006), had a lower SpO(2) on exercise (76.5% vs. 89%; P = 0.0001) and a shorter 6-min walk distance (154 m vs. 330 m; P < 0.01) than those not listed. Right ventricular function was worse in those listed (P = 0.03), as was pulmonary vascular resistance index (PVRI) on vasodilator testing (34 U m(2) vs. 14.6 U m(2); P = 0.03). Age at diagnosis and at assessment, weight, height, mean pulmonary artery pressure, baseline PVRI, B-type natriuretic peptide, spirometry and resting-SO(2) did not differ between the two groups. For the five children transplanted, median waiting time was 81 days. Age at Tx was 5.4 years. After 2.8 years all transplanted children are alive with a good functional outcome. Two patients died on the active waiting list. All children considered too well for listing are still alive and stable on treatment.
Conclusions:
Outcome after transplantation in children with IPAH has been encouraging. Defining listing criteria for these patients remains a challenge.
