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. 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.
Highlights
Total cavopulmonary connection (TCPC) is the final palliative surgical procedure for children with single ventricle physiology
Children with single ventricle physiology are identified as a group who can suffer from clinically significant pulmonary hypertensive vascular disease even when their mean pulmonary arterial pressure is below 20 mmHg, the threshold of pulmonary hypertension [10,11,12,13]
All children < 18 years of age with single ventricle physiology who had been treated with pulmonary vasodilator therapy between 2004 and 2017 were selected for retrospective analysis
Summary
Total cavopulmonary connection (TCPC) is the final palliative surgical procedure for children with single ventricle physiology. Clinical signs and secondary effects of elevated pulmonary vascular resistance include cyanosis, reduced exercise tolerance, protein-losing enteropathy, and plastic bronchitis. Mechanisms such as passive non-pulsatile flow and cyanosis/hypoxemia caused by systemic-to-pulmonary venous collaterals have been suggested to contribute to vascular remodeling and increased resistance over time in a patient with single ventricle physiology [4,5,6]. Children with single ventricle physiology are identified as a group who can suffer from clinically significant pulmonary hypertensive vascular disease even when their mean pulmonary arterial pressure is below 20 mmHg, the threshold of pulmonary hypertension [10,11,12,13].
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
