Pediatric Liver Transplantation for Alagille Syndrome: Anesthetic Evaluation and Perioperative Management

13 October 2020 : Original article

Wen-He Yang^1ABF, Liang Zhang^1ABCDEF*, Fu-Shan Xue^1ADG, Azmat Riaz^2EF, Zhi-Jun Zhu^345ADG

DOI: 10.12659/AOT.924282

Ann Transplant 2020; 25:e924282

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Table 1 Baseline patient and liver graft characteristics.

Case	Age (mo)	Gender	Weight (kg)	Height (cm)	Z Score#	Genetic type	CTP Score	PELD Score	Echocardiogram Abnormities	NYHA Classification
1	12	Female	7.5	66	−3.60	JAG1	8	12	PFO, PAS	I
2	27.5	Male	10.5	82	−2.68	^*	6	8	Normal	I
3	12	Male	9	82	1.96	JAG1	8	13	VSD, PDA	I
4	149	Male	35.8	143	−1.03	JAG1	6	13	PS	I
5	16.5	Male	8.4	76	−1.31	JAG1	9	18	Normal	I
6	73	Male	15	102	−3.49	JAG1	5	−3	VSD	II
7	65.5	Male	15	97	−4.12	JAG1	12	31	PS	II
8	23.5	Female	8.5	80	−2.25	^*	9	21	BAVS	II
9	17	Male	7.9	71.5	−2.96	JAG1	8	12	PFO	I
10	12	Female	6.3	68	−2.80	JAG1	7	10	PS	I
11	114	Male	17	122	−2.69	JAG1	5	3	PS	I

1	Deceased			Whole		321		4.28	5	710
2	Living related			Left lateral lobe		226		2.15	2	83
3	Living related			Left lateral lobe		267		2.97	2	117
4	Deceased, split			Left lobe with MHV		525		1.47	3	570
5	Deceased, reduced-size			Left lateral lobe		264		3.14	3	640
6	Living related			Left lateral lobe		317		2.11	4	147
7	Living related			Left lobe with MHV		306		2.04	2	150
8	Living related			Left lateral lobe		214		2.52	2	90
9	Living related			Left lateral lobe		253		3.20	2	79
10	Living related			Left lateral lobe		200		3.18	1	67
11	Living related			Left lateral lobe		272		1.60	2	100
^* Diagnosis based on clinical diagnostic criteria. ^# Height for age Z score (Data form Chinese Center for Disease Control and Prevention). BAVS – bicuspid aortic valve stenosis; CIT – cold ischemia time; CTP – Child-Turcotte-Pugh; GRWR – graft-to-recipient weight ratio; MHV – middle hepatic vein; PAS – pulmonary artery sling; PDA – patent ductas arteriosus; PELD – Pediatric End-stage Liver Disease; PFO – patent foramen ovale; PS – pulmonary stenosis; VSD – ventricular septal defect; WIT - warm ischemia time.

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