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Reversible severe pulmonary hypertension related to scurvy in children
Marin Satawiriya1, Apichai Khongphatthanayothin2,3, Alisa Limsuwan4
1Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, 270 Rama 6 Rd, Rachathewi, Bangkok, 10400, Thailand.
Insights
Severe childhood pulmonary hypertension (PH) can be caused by vitamin C deficiency (scurvy). Prompt diagnosis and vitamin C supplementation rapidly reversed PH in two pediatric cases, highlighting the importance of nutritional assessment.
Area of Science:
- Pediatric Cardiology
- Nutritional Science
- Hematology
Background:
- Severe pulmonary hypertension (PH) in children is a rare but potentially life-threatening condition.
- Restrictive dietary habits can lead to nutritional deficiencies, including vitamin C deficiency (scurvy).
- Scurvy can present with diverse symptoms, sometimes masking underlying nutritional causes of serious conditions like PH.
Observation:
- Two pediatric cases presented with severe PH, initially managed with standard treatments.
- Case 1: A 2-year-old boy with recurrent hypotension and PH (PAP 67 mmHg) after resuscitation, later found to have low vitamin C levels.
- Case 2: A 6-year-old boy with dyspnea, fever, cyanosis, and PH (PAP 56 mmHg), diagnosed with scurvy due to undetectable vitamin C levels.
Findings:
- Both children with severe PH were diagnosed with scurvy secondary to restrictive diets.
- Vitamin C supplementation in both cases led to a rapid and complete reversal of pulmonary hypertension.
- Echocardiogram and cardiac catheterization confirmed significant pulmonary hypertension, which resolved with treatment.
Implications:
- Highlights the critical role of nutritional assessment in diagnosing pediatric PH.
- Emphasizes that PH associated with scurvy is reversible with timely diagnosis and treatment.
- Underscores the need to consider nutritional deficiencies as potential causes of severe pediatric conditions.
Background:
Severe pulmonary hypertension (PH) in childhood is rare and can manifest as a life-threatening episode. We present 2 children with restrictive dietary habits with severe pulmonary hypertension secondary to scurvy and iron deficiency anemia with treatment and outcome.
Case Presentation:
The first case is a 2-year-old boy who presented with vomiting, diarrhea, and fever. After rehydration, he had recurrent episodes of hypotension with intermittent abdominal pain. Fluid resuscitation and inotropic medication were given. Then he suddenly collapsed. After 4-min cardiopulmonary resuscitation, his hemodynamic was stabilized. Most of the medical workup was unremarkable except for PH from the echocardiogram with estimated systolic pulmonary artery pressure (PAP) at 67 mmHg. Transient PH was diagnosed, and milrinone was prescribed. Since he had restrictive dietary habits and sclerotic rim at epiphysis in chest films, his vitamin C level was tested and reported low-level result. The second case is a 6-year-old boy with acute dyspnea, a month of low-grade fever, mild cyanosis, and a swollen left knee. Echocardiogram indicated moderate TR with estimated systolic PAP at 56 mmHg (systolic blood pressure 90 mmHg). Milrinone was given. Right cardiac catheterization showed PAP 66/38 (mean 50) mmHg and PVRi 5.7 WU.m2. Other medical conditions causing PH were excluded. With a history of improper dietary intake and clinical suspicion of scurvy, vitamin C was tested and reported undetectable level. Administration of vitamin C in both cases rapidly reversed pulmonary hypertension.
Conclusion:
Pediatric PH related to vitamin C deficiency can manifest with a wide range of symptoms, varying from mild and nonspecific to severe life-threatening episodes characterized by pulmonary hypertensive crises. PH associated with scurvy is entirely reversible with appropriate investigation, diagnosis, and treatment. Our report highlights the importance of considering nutritional deficiencies as potential confounding factors in pediatric PH, emphasizing the need for comprehensive evaluation and management of these patients.
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