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Case Reports
. 2023 Apr 3;16(4):e254730.
doi: 10.1136/bcr-2023-254730.

Scurvy-induced pulmonary arterial hypertension

Jehad Azar  1 , Mohammed Ayyad  2 , Yasmin Jaber  3 , Laith Azzam Ayasa  2
Affiliations
Case Reports

Scurvy-induced pulmonary arterial hypertension

Jehad Azar et al. BMJ Case Rep. .

Abstract

Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature that results in precapillary pulmonary hypertension. PAH is caused by a group of clinical conditions involving multiple organ systems. Several cases have been reported in the literature demonstrating an association between vitamin C deficiency and PAH. Low endothelial nitric oxide levels in the pulmonary vasculature, combined with the inappropriate activation of hypoxia-inducible transcription factors, seen in patients with ascorbic acid deficiency, are believed to be the main contributors to the pathogenesis of pulmonary vasculopathy and the exaggerated pulmonary vasoconstrictive response seen in patients with scurvy-induced PAH. Vitamin C supplementation is considered the definitive treatment.

Keywords: Malnutrition; Medical management; Pulmonary hypertension; Respiratory medicine; Vitamins and supplements.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
ECG: sinus tachycardia, right axis deviation, right ventricular hypertrophy and P pulmonale in lead II, suggesting right atrial dilatation.
Figure 2
Figure 2
TTE; parasternal long axis view: dilated right ventricle (red arrow), mild pericardial effusion (blue arrow). TTE, transthoracic echocardiogram.
Figure 3
Figure 3
TTE; apical four-chamber view: right atrial dilatation (red arrow) and right ventricular dilatation (blue arrow), the blue line shows the basal diameter is 4.3 cm (normal 3.29±0.47 cm). Flat interventricular septum. TTE, transthoracic echocardiogram.
Figure 4
Figure 4
TTE; parasternal short axis view with continuous wave Doppler interrogation across the tricuspid valve. The peak velocity is 3.22 m/s. The estimated pressure gradient between the right atrium and right ventricle is 41.5 mm Hg. Adding to this the right atrial pressure will give the estimated pulmonary artery systolic pressure of 56.5. TTE, transthoracic echocardiogram.
Figure 5
Figure 5
TTE; parasternal short axis view: dilated right ventricle (red arrow), with flattening of the interventricular septum during systole suggesting pressure overload (blue arrow), the positive ‘D shape’ sign. TTE, transthoracic echocardiogram.
Figure 6
Figure 6
Diagram illustrating the endothelial nitric oxide pathway. Endothelial nitric oxide synthase (eNOS) metabolises L-arginine into nitric oxide (NO), which in turn acts on guanylte cyclase (GC) to induce vascular smooth muscle relaxation (Laith A. Ayasa, Jehad Azar, et al. Scurvy-Induced Pulmonary Arterial Hypertension).
See this image and copyright information in PMC

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