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Published on: February 11, 2017
Management of pulmonary vascular disease associated with congenital left-to-right shunts: A single-center experience
Hythem Nawaytou1, Ramya Lakkaraju2, Leah Stevens3
1Department of Pediatrics, Cardiology, University of California, San Francisco, Calif.
Insights
Treating pulmonary arterial hypertension before left-to-right shunt repair in children may offer benefits without increasing risks. This approach shows sustained positive responses in pulmonary arterial hypertension management post-surgery.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Congenital Heart Disease
Background:
- Left-to-right shunts are common congenital heart defects.
- Pulmonary arterial hypertension (PAH) in children with these shunts presents complex management challenges.
- Current treatment strategies vary, necessitating research into optimal timing of interventions.
Purpose of the Study:
- To compare outcomes of two management strategies for pediatric patients with left-to-right shunts and PAH.
- To evaluate the efficacy of treating PAH before versus after shunt repair.
- To describe the long-term course and hemodynamic changes in these patients.
Main Methods:
- Retrospective single-center study (September 2015-September 2021).
- Inclusion criteria: Children (<18 years) with left-to-right shunts and PAH (indexed pulmonary vascular resistance ≥ 4 Wood units·m²), excluding Eisenmenger physiology.
- Data collected: Patient demographics, hemodynamics, PAH management, shunt repair, and outcomes.
Main Results:
- 51 children (6.8%) had left-to-right shunts with PAH.
- The 'Treat First' group (n=33) had a 6% mortality rate; the 'Repair First' group (n=18) had an 11% mortality rate.
- No significant change in indexed pulmonary vascular resistance was observed 2 years post-shunt closure in either group.
Conclusions:
- Pre-shunt repair PAH treatment in children appears safe and potentially beneficial.
- The positive effects of PAH-targeted therapy before shunt closure persist for 2-3 years post-repair.
- Findings provide insights into optimizing long-term management of pediatric PAH with left-to-right shunts.
Objective:
The study objective was to describe the course and outcomes of children under 18 years of age, with left-to-right shunts and pulmonary arterial hypertension undergoing 1 of 2 management approaches: pulmonary arterial hypertension treatment before left-to-right shunt repair (Treat First) and left-to-right shunt repair first with or without subsequent pulmonary arterial hypertension treatment (Repair First).
Methods:
We performed a retrospective single-center study, conducted from September 2015 to September 2021, of children with left-to-right shunts and pulmonary arterial hypertension (defined as indexed pulmonary vascular resistance ≥ 4 Wood units [WU]∗m2) but without Eisenmenger physiology. Patient characteristics, longitudinal hemodynamics data, pulmonary arterial hypertension management, left-to-right shunt repair, and outcomes were reviewed.
Results:
Of 768 patients evaluated for left-to-right shunt closure, 51 (6.8%) had left-to-right shunts associated with pulmonary arterial hypertension (median age 1.1 [0.37-5] years, median indexed pulmonary vascular resistance 6 [5.2-8.7] WU∗m2). In the "Treat First" group (n = 33, 65%), 27 patients (82%) underwent left-to-right shunt closure and 6 patients (18%) did not respond to pulmonary arterial hypertension therapy and did not undergo left-to-right shunt closure. In the "Repair First" group (n = 18, 35%), 12 patients (67%) received pulmonary arterial hypertension therapy and 6 patients (33%) did not. Mortality rates were 6% in the "Treat First" group and 11% in "Repair First" group with follow-ups of 3.4 and 2.5 years, respectively. After left-to-right shunt closure, there was no significant change in indexed pulmonary vascular resistance over a median follow-up of 2 years after surgery (P = .77).
Conclusions:
In children with left-to-right shunts and associated pulmonary arterial hypertension, treatment with pulmonary arterial hypertension-targeted therapy before defect repair does not appear to endanger the subjects and may have some benefit. The response to pulmonary arterial hypertension-targeted therapy before shunt closure persists 2 to 3 years postclosure, providing valuable insights into the long-term management of these patients.
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