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

Abstract 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-o...

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Autores principales:	Arjun Agarwal, Durvesh Bhangale, Vinayak M. Sawardekar, Srikar Puvvada
Formato:	article
Lenguaje:	EN
Publicado:	SpringerOpen 2021
Materias:	Abernethy malformation Case report Congenital Portal vein Pulmonary hypertension Vascular anomaly Internal medicine RC31-1245
Acceso en línea:	https://doaj.org/article/20eccdeb59df48feb974943fc44e9daf
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spelling	oai:doaj.org-article:20eccdeb59df48feb974943fc44e9daf2021-11-28T12:03:28ZType II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature10.1186/s43162-021-00068-71110-77822090-9098https://doaj.org/article/20eccdeb59df48feb974943fc44e9daf2021-11-01T00:00:00Zhttps://doi.org/10.1186/s43162-021-00068-7https://doaj.org/toc/1110-7782https://doaj.org/toc/2090-9098Abstract 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.Arjun AgarwalDurvesh BhangaleVinayak M. SawardekarSrikar PuvvadaSpringerOpenarticleAbernethy malformationCase reportCongenitalPortal veinPulmonary hypertensionVascular anomalyInternal medicineRC31-1245ENThe Egyptian Journal of Internal Medicine, Vol 33, Iss 1, Pp 1-5 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Abernethy malformation Case report Congenital Portal vein Pulmonary hypertension Vascular anomaly Internal medicine RC31-1245
spellingShingle	Abernethy malformation Case report Congenital Portal vein Pulmonary hypertension Vascular anomaly Internal medicine RC31-1245 Arjun Agarwal Durvesh Bhangale Vinayak M. Sawardekar Srikar Puvvada Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature
description	Abstract 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.
format	article
author	Arjun Agarwal Durvesh Bhangale Vinayak M. Sawardekar Srikar Puvvada
author_facet	Arjun Agarwal Durvesh Bhangale Vinayak M. Sawardekar Srikar Puvvada
author_sort	Arjun Agarwal
title	Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature
title_short	Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature
title_full	Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature
title_fullStr	Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature
title_full_unstemmed	Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature
title_sort	type ii abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature
publisher	SpringerOpen
publishDate	2021
url	https://doaj.org/article/20eccdeb59df48feb974943fc44e9daf
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Type II Abernethy malformation in an adult male patient—a rare and reversible cause of pulmonary hypertension: a case report with review of literature

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