A Very Uncommon Case of Pulmonary Arterial Hypertension

Jehad Azar¹, Ali Varasteh¹, Daniel Iltchev², Mona Soliman¹, Victor Baez¹ and Basel Altaqi^2,

¹Internal Medicine Resident, St. Vincent Charity Medical Center, Cleveland, USA

²Pulmonary and Critical Care Medicine, St. Vincent Charity Medical Center, Cleveland, USA

Cite this paper

Jehad Azar, Ali Varasteh, Daniel Iltchev, Mona Soliman, Victor Baez and Basel Altaqi. A Very Uncommon Case of Pulmonary Arterial Hypertension. American Journal of Medical Case Reports. 2019; 7(5):79-86. doi: 10.12691/AJMCR-7-5-2

Abstract

Pulmonary arterial hypertension (PAH) describes a rare, progressive disease of the pulmonary vasculature, involving a group of clinical conditions that result in precapillary pulmonary hypertension (PH). PAH is characterized by proliferative vasculopathy, and subsequent right heart failure. For the past several decades research has focused on identification of underlying molecular causes of this disease. Recently, there have been a number of reported cases of patients with scurvy developing pulmonary arterial hypertension. Thus, it has been hypothesized that vitamin C deficiency results in non-hypoxic activation of hypoxia inducible transcription factors (HIF) and low nitric oxide (NO) level in the pulmonary vasculature, leading to subsequent pulmonary vasculopathy and an exaggerated pulmonary vasoconstrictive response. Immediate supplementation of vitamin C is considered the definitive treatment, preventing an otherwise fatal outcome. In this paper, we describe a patient with a fatal case of scurvy related PAH, who was admitted with ecchymosis, oral ulceration, and dyspnea.

Keywords

pulmonary arterial hypertension, scurvy, right side heart failure, hypoxia inducible transcription factors, nitric oxide

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]	Peacock AJ, Murphy NF, McMurray JJ et al.., An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007; 30(1): 104.
[2]	Johnson JA, Hemnes AR, Perrien DS, et al. Cytoskeletal defects in Bmpr2-associated pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol. 2012; 302(5): L474-84. PubMed PMID: 22180660, Pubmed Central PMCID:PMC3311512.
[3]	Wood P. Pulmonary hypertension with special reference to the vasoconstrictive factor. Br Heart J. 1958; 20(4): 557-70. PubMed PMID: 13584643, Pubmed Central PMCID: 491807.
[4]	Palmer RM, Ferrige AG, Moncada S. Nitric oxide release accounts for the biological activity of endothelium-derived relaxing factor. Nature. 1987; 327(6122): 524-6. PubMed PMID: 3495737.
[5]	Farrukh Abbas, Le Dung Ha, et al. Reversible Right Heart Failure in Scurvy Rediscovery of an Old Observation. Circulation: Heart Failure. 2016; 9.
[6]	Taddei S, Virdis A, Ghiadoni L, et al. Vitamine C improves endothelium dependent vasodilatation by restoring nitric oxide activity in essential hypertension. Circulation. 1998; 97: 2222-2229.
[7]	Smith TG, Robbins PA, Ractliffe PJ. The human side of hypoxia- inducible factors. Br J Haematol. 2008; 141: 325-334.
[8]	Pullamsetti S, Kiss L, Ghofrani HA, Voswinckel R, Haredza P, Klepetko W, et al. Increased levels and reduced catabolism of asymmetric and symmetric dimethylarginines in pulmonary hypertension. FASEB J. 2005; 19(9): 1175-7. PubMed PMID: 15827267.
[9]	Duvall MG, Pikman Y, et al. Pulmonary Hypertension associated with scurvy and vitamin deficiencies in an autistic child.pediatrics. Nov 2013; 132: e1699-703.
[10]	Benza R, Biederman R, Murali S, Gupta H. Role of cardiac magnetic resonance imaging in the management of patients with pulmonary arterial hypertension. J Am Coll Cardiol. 2008; 52(21): 1683-92.
[11]	Yock PG, Popp RL. Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation. 1984; 70: 657-62.
[12]	Arkles JS, Opotowsky AR, Ojeda J, et al. Shape of the right ventricular Doppler envelope predicts hemodynamics and right heart function in pulmonary hypertension. Am J Respir Crit Care Med. 2011; 183: 268-76.
[13]	Forfia PR, Fisher MR, Mathai SC, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med. 2006; 174: 1034-41.
[14]	Tartulier MM, et al. Pulmonary arterial pressures in normal subjects. Effects of age and muscular exercise. Bull Physiopathol Respir (Nancy). 1972; 8(6): 1295-321.
[15]	Hoeper MM, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013; 62: D42-50.
[16]	Soichiro N, Avi N, et al. Pulmonary Hypertension secondary to vitamin C deficiency. Chest. 2009; 136(4-meeting abstracts): 4s.
[17]	Kupari et al. Chest. Reversible Pulmonary Hypertension Associated with Vitamin C Deficiency. Jul 2012; 142(1): 225-7.
[18]	Sarah G, Mauro P, et al. A Very Uncommon Case of Pulmonary Hypertension.
[19]	Singh I, Elizabeth G, A Sweet Remedy for Severe Pulmonary Arterial Hypertension, American Journal of Respiratory and Critical Care Medicine 2017; 195: A6187.
[20]	Elizabeth Petersen, Scurvy and Acute Pulmonary Hypertension in a Child with Autism, poster presentation, internal medicine and pediatric, Brigham and Women’s Hospital / Boston Children’s Hospital.
[21]	Terry D, Neeru K, et al. Cardiac arrest and pulmonary hypertension in scurvy: a case report.