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Published on: November 18, 2018
Pulmonary Hypertension Associated With Vitamin C Deficiency Is Rapidly Reversible
Tomás Woodgate1, Jay Patel2, Thomas Day3
1National Paediatric Pulmonary Hypertension Service, Great Ormond Street Hospital, London.
Insights
Severe pulmonary hypertension in children can be caused by vitamin C deficiency. Prompt vitamin C replacement therapy rapidly reverses this condition, highlighting the importance of nutritional assessment in pediatric cardiovascular health.
Area of Science:
- Pediatric Cardiology
- Nutritional Science
- Radiology
Background:
- Severe pulmonary hypertension (PH) in children is a critical condition.
- Nutritional deficiencies, including vitamin C deficiency, can contribute to complex pediatric health issues.
- Scurvy, a manifestation of severe vitamin C deficiency, can present with subtle radiographic findings.
Purpose of the Study:
- To report a series of children with severe pulmonary hypertension linked to vitamin C deficiency.
- To highlight the diagnostic clues and treatment response in these cases.
- To emphasize the reversibility of PH associated with vitamin C deficiency.
Main Methods:
- Case series of 4 children (aged 3-9 years) with severe PH.
- Clinical presentation, echocardiography, N-terminal pro-B-type natriuretic peptide levels, and nutritional status assessment.
- Radiologic review for subtle signs of scurvy.
- Treatment with vitamin C replacement, with some receiving sildenafil concurrently.
Main Results:
- All 4 children presented with severe PH, right ventricular dysfunction, and elevated NT-proBNP.
- Musculoskeletal symptoms and peripheral edema were common presentations.
- Subtle radiographic features of scurvy were noted in 2 patients.
- Rapid clinical and echocardiographic improvement occurred within 3 months of vitamin C therapy.
- One patient showed no response to sildenafil alone, responding only after vitamin C supplementation.
Conclusions:
- Vitamin C deficiency is a potentially reversible cause of severe pulmonary hypertension in children.
- Early recognition of nutritional deficiencies, including scurvy, is crucial for diagnosing PH.
- Vitamin C replacement therapy is the primary treatment for PH associated with vitamin C deficiency.
Abstract:
We report 4 children (aged 3-9 years) with severe pulmonary hypertension (PH) associated with vitamin C deficiency. All presented with either musculoskeletal symptoms or cardiorespiratory deterioration with peripheral edema. Multiple nutritional deficiencies were identified in all children, and 2 had autistic spectrum disorder. Initial echocardiography results demonstrated severe PH with right ventricular dysfunction and significantly elevated N-terminal pro-B-type natriuretic peptide levels. In 2 patients presenting with musculoskeletal symptoms, specialist radiologic review revealed subtle features of scurvy that provided early diagnostic clues before laboratory confirmation. Vitamin C levels were undetectable where samples were processed prior to replacement, highlighting the importance of securing viable samples before treatment initiation for diagnostic certainty. All children received vitamin C replacement therapy, with 3 also receiving concurrent sildenafil. Remarkably, all demonstrated rapid clinical and echocardiographic improvement following vitamin C initiation, with complete resolution within 3 months. Importantly, 1 child receiving sildenafil alone showed no therapeutic response until vitamin C was added, indicating that improvement requires correction of the underlying deficiency rather than pulmonary vasodilation alone. This case series demonstrates rapid reversibility of vitamin C deficiency-associated PH and emphasizes the importance of considering this diagnosis in children with restricted diets and unexplained PH.
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