Regression of Pulmonary Vascular Disease After Therapy of Abernethy Malformation in Visceral Heterotaxy

Raghuram, K; Sasidharan, Bijulal; Krishnamoorthy, Kavassery Mahadevan; Tharakan, Jaganmohan A.

doi:10.1007/s00246-012-0428-z

Cited by 11 publications

(10 citation statements)

References 8 publications

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“…Among them, 66 were female and 35 were male, with ages ranging from fetus to 61 years at the time of diagnosis. In total, 70 patients (69.30%) were<18 years of age, and <10% had type II malformations (45,58,75–77). …”

Section: Discussionmentioning

confidence: 99%

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review

Hao

Zhao

2014

Oncology Letters

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Abernethy malformation is a rare congenital malformation defined by an extrahepatic portosystemic shunt. The majority of affected patients are young (<18 years of age) and experience various symptoms, including vomiting, jaundice, dyspnea and coma. The current study presents a case of Abernethy malformation in an asymptomatic adult male patient. The patient exhibited congenital absence of the portal vein, congenital heart disease (postoperative ventricular septal defect status), and multiple liver lesions, confirmed to be focal nodular hyperplasia by biopsy. Ultrasonography and magnetic resonance imaging findings revealing the liver lesions, type II congenital absence of the portal vein and the portosystemic shunt are presented. In addition, the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder are investigated by reviewing 101 previously reported cases.

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Section: Discussionmentioning

confidence: 99%

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review

Hao

Zhao

2014

Oncology Letters

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“…Ersch et al [6] described a 20-month-old boy who died from right heart failure due to PAH caused by a CEPVS. Raghuram et al [7] described a one-year-old boy with a medical history of a corrected transposition of the great arteries and a moderate-sized ventricular septum defect. The patient presented with cyanosis and an RHC showed severe PAH which could not be explained by the intracardiac defect alone.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Arterial Hypertension Combined with a High Cardiac Output State: Three Remarkable Cases

2013

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A congenital extrahepatic portosystemic venous shunt (CEPVS), also known as an Abernethy malformation, is a rare cause of pulmonary arterial hypertension (PAH). In this case series, we describe three male patients of 30, 23, and 27 years of age with PAH due to a CEPVS. In all three patients, a right heart catheterization revealed a high cardiac output. The aim of this case series is to make pulmonary hypertension physicians aware of the possibility of a CEPVS when PAH is accompanied with a high cardiac output state.

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“…As the experience in patients with Abernethy malformation and hepatopulmonary syndrome is limited, treatment strategies must be tailored on a case-by-case basis (6). In general, patients with hepatopulmonary syndrome showed improvement of cyanosis when the hepatic function was restored or the portosystemic shunt was abolished (7). Adult patients with small shunts are amenable to interventional procedures such as device closure ( 8), while small children are usually selected for surgical ligation of the portosystemic shunt or orthotopic liver transplantation (7,9).…”

Section: Discussionmentioning

confidence: 99%

Successful percutaneous treatment with the Konar MFTM-VSD Occluder in an infant with Abernethy syndrome—case report

Loureiro

Georgiev

Ewert

et al. 2021

Cardiovasc Diagn Ther

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Cyanosis persisting after surgical repair of complex congenital heart disease (CHD) may be related to the underlying disease. However, extracardiac causes should be also considered. We report on a patient with heterotaxy syndrome and double outlet right ventricle, in whom postoperative cyanosis was associated with an Abernethy malformation type II causing a hepatopulmonary syndrome. Despite this complex anatomy, interventional closure of the portosystemic shunt was done with a Konar MF™-VSD Occluder. The patient recovered rapidly with relief of cyanosis within one month. This case highlights the importance of a careful diagnostic assessment in patients with complex CHD, who presents cyanoses after surgical repair. In addition, it shows the feasibility and safety of a percutaneous approach with complete closure of the vascular malformation in a patient with a complex anatomy.

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Regression of Pulmonary Vascular Disease After Therapy of Abernethy Malformation in Visceral Heterotaxy

Cited by 11 publications

References 8 publications

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review

Pulmonary Arterial Hypertension Combined with a High Cardiac Output State: Three Remarkable Cases

Successful percutaneous treatment with the Konar MFTM-VSD Occluder in an infant with Abernethy syndrome—case report

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