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Severe Pulmonary Arterial Hypertension in Healthy Young Infants: Single Center Experience
Shrikiran Aroor1, Koushik Handattu2, Suneel C Mundkur1
1Department of Paediatrics, Kasturba Medical College (Manipal Academy of Higher Education), Manipal, Karnataka.
Insights
In infants with pulmonary arterial hypertension (PAH), supportive care and vasodilators are effective. Thiamine supplementation did not show additional benefits for recovery or mortality in this study.
Area of Science:
- Pediatrics
- Cardiology
- Neonatology
Background:
- Pulmonary arterial hypertension (PAH) can occur suddenly in healthy infants.
- Management strategies for infant PAH are crucial for improving outcomes.
Purpose of the Study:
- To investigate the clinical presentation and management of acute PAH in infants.
- To evaluate the efficacy of thiamine therapy as an adjunct treatment for infant PAH.
Main Methods:
- Retrospective review of hospital records for 56 infants under 6 months with diagnosed PAH.
- Comparison of outcomes between infants receiving standard care plus vasodilators and those also receiving thiamine supplementation.
Main Results:
- 80% of infants achieved complete recovery with supportive care and vasodilators.
- Infants who died had significantly worse acidosis, higher lactate, ventricular dysfunction, and shock.
- No significant differences in recovery rates or mortality were observed between infants who received thiamine and those who did not.
Conclusions:
- Supportive treatment and pulmonary vasodilator therapy are effective for infant PAH.
- Thiamine supplementation does not appear to provide additional benefits in managing acute PAH in this population.
Objective:
We studied the clinical presentation and management of acute pulmonary arterial hypertension (PAH) in healthy young infants, and the effect of thiamine therapy.
Methods:
Review of hospital records was conducted for 56 healthy infants (aged below 6 month) who developed sudden onset of pulmonary arterial hypertension as diagnosed on 2D echocardiography, and were admitted at our institution.
Results:
All patients received supportive care and pulmonary vasodilator therapy, whereas those admitted after Sep-tember, 2019 (n=28) received thiamine in addition, as per the institute's protocol. Overall, complete recovery was seen in 80% (n=45). Infants who died had significantly lower mean pH (7.05 vs 7.27; P=0.001) and serum bicarbonate (9.1 vs 14.9; P=0.007), higher arterial lactate (72.7 vs 61.5; P=0.92), ventricular dysfunction (16 vs 10; P=0.01) and shock (7 vs 9; P=0.008) when compared to those who survived. Baseline characteristics, severity of acidosis and pulmonary hypertension, time taken to recover from PAH, presence of ventricular dysfunction were comparable among those who received thiamine and those who did not receive it. Similarly, recovery (89% vs 71%; P=0.17) and mortality (11% vs 29%) were also comparable between the two groups.
Conclusions:
A significant proportion of infants with PAH improve with supportive treatment and pulmonary vasodilator therapy. Thiamine supplementation may not give any additional benefit in these patients.
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