Pulmonary Arterial Hypertension in a Patient with a Portosystemic Shunt: Diagnostic Challenge

Stepffer, Carolina; Marques, Adelia; Barbosa, Jesús Damsky; Ferrario, Sofía; Haag, Dora

doi:10.1016/j.case.2019.08.007

Cited by 3 publications

(1 citation statement)

References 10 publications

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“…Type I AM is usually observed in younger individuals with female predominance and is generally accompanied by abnormalities involving the heart, liver, gastrointestinal tract, and blood vessels, while Type II is rarer, frequently observed in adults, has male predominance, and is usually not associated with other abnormalities [7]. Patients with AM have a wide variety of symptoms ranging from those asymptomatic patients found incidentally in adulthood to those with congenital severe hepatic manifestations identified at birth.…”

Section: Discussionmentioning

confidence: 99%

Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature

et al. 2021

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Background Co-occurrence of Type II Abernethy malformation (AM) with Type 1 pulmonary hypertension (PH) is extremely rare. In these patients, management of AM leads to reversal of PH. Case presentation We report a case of 24-year-old male that presented with fever and dyspnea of insidious-onset. Initial X-ray chest revealed pulmonary edema, prominent pulmonary knuckle, and dilated right descending pulmonary artery. Two-dimensional echocardiography suggested raised pulmonary artery systolic pressure (145 mmHg) and pulmonary angiogram revealed findings suggestive of Type 1 PH. Treatment with Tab. Tadalafil-Bosentan (20/5 mg, thrice daily) was initiated, but no major relief was obtained. On day 10, dyspnea worsened. Routine ultrasound revealed splenomegaly. Further evaluation with Doppler of the portal vein was suggestive of portal hypertension, and arterial ammonia was found to be raised. Contrast-enhanced computed tomography of abdomen and pelvis demonstrated a single dilated (1.3 cm) and torturous venous shunt between right internal iliac vein and superior mesenteric vein. Thus, a definitive diagnosis of Type II AM was reached. The patient was stabilized and subjected to the shunt closure with ASD Cocoon stent graft and recovered well. Conclusion Our case demonstrates a rare but reversible cause of PH. Type II AM should be included in the differential diagnosis of a patient presenting with dyspnea and provisionally diagnosed as a case of PH.

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Section: Discussionmentioning

confidence: 99%

Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature

et al. 2021

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Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension

Deng

Zhou

et al. 2023

Eur J Med Res

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The etiology of pulmonary arterial hypertension (PAH) is complex, especially the investigation of rare pathogeny is difficult. Congenital portosystemic venous shunt (CPSS) is a rare congenital anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosystemic shunt, and drains directly into the inferior vena cava (IVC) (Howard and Davenport in J Pediatr Surg 32:494–497, 1997).CPSS is an uncommon cause of PAH (Christiane et al. in J Pediatr Gastroenterol Nutr 56:675–681, 2013), and often covered by other pathogenic factors. The clinical manifestations of CPSS-associated PAH are not specific, thus making it difficult to distinguish from PAH caused by other pathogenetic factors based on clinical presentations alone. This is a retrospective analysis of data from six patients with CPSS at a single center. Of these, five were diagnosed as PAH: four were also associated with other predisposing factors of pulmonary hypertension (PH). All patients had high serum bile concentration and high cardiac output. The aim of this retrospective study was to investigate the clinical recognition of PAH secondary to CPSS. The concentration of serum bile acid and cardiac output can be used as two important non-invasive indicators in clinical practice. Thus far, few studies have reported the clinical outcomes of CPSS-associated PAH specifically (Anna et al. in Hepatology 71:658–669, 2020;Franchi-Abella et al. in J Pediatr Gastroenterol Nutr 51:322–330, 2010;Uike et al. in Pediatr Pulmonol 53:505–511, 2018;). In the current study, such patients carried a poor prognosis if left untreated, or treated with pulmonary vasodilators alone.

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Congenital portosystemic shunt with multiple splenic artery aneurysms

Subramanian

Bharath

Barthur

et al. 2022

Annals of Pediatric Cardiology

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We report an unusual occurrence of multiple splenic artery aneurysms and splenomegaly in a young woman with severe pulmonary hypertension, secondary to a congenital portosystemic shunt (CPS). The splenic artery was occluded using an Amplatzer Duct Occluder-II device, and closure of the large intrahepatic CPS was achieved using a muscular ventricular septal defect occluder. There was resolution of splenomegaly with normal pulmonary artery pressures, a few months after the procedure.

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Pulmonary Arterial Hypertension in a Patient with a Portosystemic Shunt: Diagnostic Challenge

Cited by 3 publications

References 10 publications

Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature

Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature

Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension

Congenital portosystemic shunt with multiple splenic artery aneurysms

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