Impact of Percutaneous Interventions for Pulmonary Artery Stenosis in Alagille Syndrome

Zussman, Matthew; Hirsch, Russel; Beekman, Robert H.; Goldstein, Bryan H.

doi:10.1111/chd.12219

Cited by 11 publications

(10 citation statements)

References 22 publications

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“…The retrieval strategy is shown in Figure 1 . As a result, a total of 38 articles were included and 19 articles were excluded [ 3 – 5 , 8 – 42 ].…”

Section: Methodsmentioning

confidence: 99%

Pulmonary artery pathologies in Alagille syndrome: a meta-analysis

Yuan¹

2022

pwki

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Alagille syndrome, caused by mutations in the gene encoding Jagged1 (JAG1), a ligand in the Notch signaling pathway, is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, face and skeleton. The aim of the present study is try to disclose the clinical features, management and outcomes of pulmonary artery stenosis associated with Alagille syndrome. By comprehensive literature retrieval, 38 articles involving 401 patients were recruited for this study. The pertinent variables closely related to pulmonary artery stenosis in patients with Alagille syndrome were comprehensively analyzed by following the PRISMA guidelines. The management of pulmonary artery pathologies, especially a severe type of pulmonary artery stenosis in Alagille syndrome, is a concerned matter. Publications of literature retrieval of recent 3 decades were the study material of this article. The pulmonary artery pathologies, especially the severe type of pulmonary artery stenosis in Alagille syndrome, warrant surgical or interventional treatments. After the procedures, the right ventricular to left ventricular pressure ratio was reduced by 25%. There were no intergroup differences in terms of recovery, reintervention and mortality rates between interventionally and surgically treated patients. Transcatheter treatment is preferable due to less trauma. Surgical treatment of pulmonary artery stenosis can be performed currently with intracardiac defect repair.

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“…The retrieval strategy is shown in Figure 1 . As a result, a total of 38 articles were included and 19 articles were excluded [ 3 – 5 , 8 – 42 ].…”

Section: Methodsmentioning

confidence: 99%

Pulmonary artery pathologies in Alagille syndrome: a meta-analysis

Yuan¹

2022

pwki

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“…This may be best accomplished by surgical reconstruction, particularly in younger patients (16). Alagille patients have experienced a bit more success with trans catheter intervention, although numbers and data remain sparse (19). The remainder of pulmonary artery stenosis tends to be post-operative or in association with other complex congenital heart disease.…”

Section: Pulmonary Artery Stenosismentioning

confidence: 99%

Congenital pulmonary vascular anomalies

Dimas¹,

Dillenbeck²,

Josephs³

2018

Cardiovasc. Diagn. Ther.

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Congenital pulmonary vascular anomalies are typically found in infancy or early childhood however, some may remain silent and present in adult patients. Anomalies may be separated into anatomic categories based on involvement of the pulmonary arteries, pulmonary veins or both with or without involvement of the lung parenchyma. Association with congenital heart disease and other syndromes is very common. Computed tomography (CT) and magnetic resonance imaging (MRI) are both invaluable at assessment of these anomalies allowing for both diagnosis and detailed treatment planning. This article will focus primarily on the use of CT, as the high resolution evaluation of the lung parenchyma is also important in many of these conditions. In young patients especially, rapid heart rate and concerns of radiation exposure are important considerations when performing CT. This article will discuss scan techniques as well as clinical diagnostic considerations and basic endovascular treatment of congenital pulmonary vascular anomalies.

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“…Although the natural history and morbidity of AGS have been related to liver and heart disease, AGS has an excellent prognosis due to improvements and innovations in liver transplantation and cardiac management . Therefore, vascular anomalies may be prognostic factors because the mortality of patients with AGS is caused by aneurysm rupture due to HT and chronic renal failure due to HT and renal anomalies.…”

Section: Introductionmentioning

confidence: 99%

“…due to improvements and innovations in liver transplantation and cardiac management. [9][10][11][12] Therefore, vascular anomalies may be prognostic factors because the mortality of patients with AGS is caused by aneurysm rupture due to HT and chronic renal failure due to HT and renal anomalies. Although intracranial and pulmonary vascular anomalies are well-known complications of and causes of mortality in AGS, [4][5][6][7][8][11][12][13][14] visceral artery anomalies including those of the abdominal aorta, CA, SMA, and RA are less commonly recognized.…”

mentioning

confidence: 99%

“…[9][10][11][12] Therefore, vascular anomalies may be prognostic factors because the mortality of patients with AGS is caused by aneurysm rupture due to HT and chronic renal failure due to HT and renal anomalies. Although intracranial and pulmonary vascular anomalies are well-known complications of and causes of mortality in AGS, [4][5][6][7][8][11][12][13][14] visceral artery anomalies including those of the abdominal aorta, CA, SMA, and RA are less commonly recognized. 6,[15][16][17][18][19][20][21][22][23][24][25] Herein, we present a retrospective analysis of our 17-year experience with pediatric LDLT with a focus on the current problems with and treatments for visceral artery anomalies in AGS after LDLT.…”

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confidence: 99%

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Visceral artery anomalies in patients with Alagille syndrome

Sanada

Naya

Katano

et al. 2019

Pediatric Transplantation

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Background Intracranial and pulmonary vascular anomalies are well‐known complications and causes of mortality in AGS; however, visceral artery anomalies are less commonly recognized. Herein, we present a retrospective analysis of our experience with pediatric LDLT that focuses on the current problems with and treatments for visceral artery anomalies in AGS after LDLT. Methods Between May 2001 and December 2017, 294 LDLTs were performed for 285 pediatric recipients. Of these, 13 LDLTs (4.4%) for 12 AGS patients were performed. We classified the visceral artery anomalies into aneurysms and stenosis. Results The overall incidence of visceral aneurysm was 2 of 12 recipients (16.7%) and included a SMA aneurysm in one patient and an IPDA aneurysm with a subsequent SPA aneurysm in one patient; the ages of the diagnosis of visceral aneurysm were 16.3, 21.1, and 21.7 y, respectively. An endovascular treatment was performed for a progressive IPDA saccular aneurysm (12.0 × 14.5 × 15.0 mm). The overall incidence of visceral artery stenosis was 7 of 12 recipients (58.3%) and the median age at the diagnosis of visceral artery stenosis was 15.5 y (range 1.7‐22.9 y). All 3 AGS patients with RA stenosis suffered from renal dysfunction (eGFR of 51, 78, and 51 mL/min/1.73m2). Conclusion The morbidity of visceral artery anomalies is not negligible. The performance of periodic imaging examinations is necessary, even for infants, because it is difficult to detect visceral vascular anomalies in the infant stage.

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Impact of Percutaneous Interventions for Pulmonary Artery Stenosis in Alagille Syndrome

Abstract: Transcatheter intervention for PAS in ALGS is generally safe and acutely effective. Although BMS implantation was associated with the greatest immediate improvement in MLD, HBA-treated vessels demonstrate interval growth, whereas BMS-treated lesions do not.

Cited by 11 publications

References 22 publications

Pulmonary artery pathologies in Alagille syndrome: a meta-analysis

Pulmonary artery pathologies in Alagille syndrome: a meta-analysis

Congenital pulmonary vascular anomalies

Visceral artery anomalies in patients with Alagille syndrome

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