Pulmonary Vasodilator Therapy in Children with Single Ventricle Physiology : Effects on Saturation and Pulmonary Arterial Pressure
(2020) In Pediatric Cardiology 41(8). p.1651-1659- Abstract
In children with single ventricle physiology, increased pulmonary vascular resistance may impede surgical progression or result in failing single ventricle physiology. The use of pulmonary vasodilators has been suggested as a potential therapy. However, knowledge on indication, dosage, and effect is limited. A retrospective case notes review of all (n = 36) children with single ventricle physiology, treated with pulmonary vasodilators by the UK Pulmonary Hypertension Service for Children 2004–2017. Therapy was initiated in Stage 1 (n = 12), Glenn (n = 8), or TCPC (n = 16). Treatment indications were high mean pulmonary arterial pressure, cyanosis, reduced exercise tolerance, protein-losing enteropathy, ascites, or plastic bronchitis.... (More)
In children with single ventricle physiology, increased pulmonary vascular resistance may impede surgical progression or result in failing single ventricle physiology. The use of pulmonary vasodilators has been suggested as a potential therapy. However, knowledge on indication, dosage, and effect is limited. A retrospective case notes review of all (n = 36) children with single ventricle physiology, treated with pulmonary vasodilators by the UK Pulmonary Hypertension Service for Children 2004–2017. Therapy was initiated in Stage 1 (n = 12), Glenn (n = 8), or TCPC (n = 16). Treatment indications were high mean pulmonary arterial pressure, cyanosis, reduced exercise tolerance, protein-losing enteropathy, ascites, or plastic bronchitis. Average dose of sildenafil was 2.0 mg/kg/day and bosentan was 3.3 mg/kg/day. 56% had combination therapy. Therapy was associated with a reduction of the mean pulmonary arterial pressure from 19 to 14 mmHg (n = 17, p < 0.01). Initial therapy with one or two vasodilators was associated with an increase in the mean saturation from 80 to 85%, (n = 16, p < 0.01). Adding a second vasodilator did not give significant additional effect. 5 of 12 patients progressed from Stage 1 to Glenn, Kawashima, or TCPC, and 2 of 8 from Glenn to TCPC during a mean follow-up time of 4.7 years (0–12.8). Bosentan was discontinued in 57% and sildenafil in 14% of treated patients and saturations remained stable. Pulmonary vasodilator therapy was well tolerated and associated with improvements in saturation and mean pulmonary arterial pressure in children with single ventricle physiology. It appears safe to discontinue when no clear benefit is observed.
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- author
- Jeremiasen, Ida LU ; Tran-Lundmark, Karin LU ; Idris, Nikmah ; Tran, Phan Kiet LU and Moledina, Shahin
- organization
- publishing date
- 2020-12
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Children, Pulmonary vascular resistance, Single ventricle physiology, Vasodilators
- in
- Pediatric Cardiology
- volume
- 41
- issue
- 8
- pages
- 9 pages
- publisher
- Springer
- external identifiers
-
- scopus:85088784164
- pmid:32734529
- ISSN
- 0172-0643
- DOI
- 10.1007/s00246-020-02424-w
- language
- English
- LU publication?
- yes
- id
- 0c32ec67-f114-473e-9e03-adf7b1bca8a7
- date added to LUP
- 2020-08-10 11:57:36
- date last changed
- 2024-04-03 11:19:46
@article{0c32ec67-f114-473e-9e03-adf7b1bca8a7,
abstract = {{<p>In children with single ventricle physiology, increased pulmonary vascular resistance may impede surgical progression or result in failing single ventricle physiology. The use of pulmonary vasodilators has been suggested as a potential therapy. However, knowledge on indication, dosage, and effect is limited. A retrospective case notes review of all (n = 36) children with single ventricle physiology, treated with pulmonary vasodilators by the UK Pulmonary Hypertension Service for Children 2004–2017. Therapy was initiated in Stage 1 (n = 12), Glenn (n = 8), or TCPC (n = 16). Treatment indications were high mean pulmonary arterial pressure, cyanosis, reduced exercise tolerance, protein-losing enteropathy, ascites, or plastic bronchitis. Average dose of sildenafil was 2.0 mg/kg/day and bosentan was 3.3 mg/kg/day. 56% had combination therapy. Therapy was associated with a reduction of the mean pulmonary arterial pressure from 19 to 14 mmHg (n = 17, p < 0.01). Initial therapy with one or two vasodilators was associated with an increase in the mean saturation from 80 to 85%, (n = 16, p < 0.01). Adding a second vasodilator did not give significant additional effect. 5 of 12 patients progressed from Stage 1 to Glenn, Kawashima, or TCPC, and 2 of 8 from Glenn to TCPC during a mean follow-up time of 4.7 years (0–12.8). Bosentan was discontinued in 57% and sildenafil in 14% of treated patients and saturations remained stable. Pulmonary vasodilator therapy was well tolerated and associated with improvements in saturation and mean pulmonary arterial pressure in children with single ventricle physiology. It appears safe to discontinue when no clear benefit is observed.</p>}},
author = {{Jeremiasen, Ida and Tran-Lundmark, Karin and Idris, Nikmah and Tran, Phan Kiet and Moledina, Shahin}},
issn = {{0172-0643}},
keywords = {{Children; Pulmonary vascular resistance; Single ventricle physiology; Vasodilators}},
language = {{eng}},
number = {{8}},
pages = {{1651--1659}},
publisher = {{Springer}},
series = {{Pediatric Cardiology}},
title = {{Pulmonary Vasodilator Therapy in Children with Single Ventricle Physiology : Effects on Saturation and Pulmonary Arterial Pressure}},
url = {{http://dx.doi.org/10.1007/s00246-020-02424-w}},
doi = {{10.1007/s00246-020-02424-w}},
volume = {{41}},
year = {{2020}},
}