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. 2022 Apr 28;74(1):34.
doi: 10.1186/s43044-022-00273-x.

Multiple congenital visceral abnormalities as a rare cause of pulmonary arterial hypertension

Parham Rabiee  1 , Sedigheh Saedi  2
Affiliations

Multiple congenital visceral abnormalities as a rare cause of pulmonary arterial hypertension

Parham Rabiee et al. Egypt Heart J. .

Abstract

Background: Pulmonary arterial hypertension (PAH) is a rare, progressive disorder. PAH is caused by a wide spectrum of pathologies but the cause remains undetermined on many occasions and patients are classified in the idiopathic group.

Case presentation: Here we report a young woman with rare congenital visceral abnormalities presenting with severe pulmonary hypertension.

Conclusions: Pulmonary hypertension is a complex disorder. Search for uncommon conditions that lead to pulmonary hypertension is necessary to determine the best management options.

Keywords: Abernethy malformation; Congenital heart disease; Mayer–Rokitansky–Küster–Hauser syndrome; Pulmonary hypertension.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Chest X-ray showing pulmonary artery dilation, right ventricular enlargement and scoliosis
Fig. 2
Fig. 2
Echocardiogram showing right ventricular enlargement (A), septal flattening in systole and diastole in favor of right ventricular pressure overload (B), and high tricuspid regurgitation gradients (C). RV right ventricle, LV left ventricle, IVS inter-ventricular septum
Fig. 3
Fig. 3
Contrast-enhanced axial CT scan in porto-venous phase demonstrating abnormal fistulous tract directly connecting splenic vein (SV) and superior mesenteric vein to inferior vena cava with absent main portal vein (A, B). Axial contrast CT image showing dilated pulmonary artery in favor of significant pulmonary hypertension (C)
Fig. 4
Fig. 4
Abdominal angiography showing absent portal vein and splenic and superior mesenteric veins draining directly to inferior vena cava (arrow). IVC inferior vena cava, ReV renal vein
See this image and copyright information in PMC

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