海德综合征与主动脉瓣置换术：已发表文献回顾

doi:10.24920/003881

摘要/Abstract

摘要：

目的探讨采用主动脉瓣置换术治疗的海德综合征患者的临床特点及结局。
方法使用检索词“Heyde syndrome”、“gastrointestinal bleeding”、“aortic stenosis”和“surgery”,检索 PubMed、Embase、Ovid、WANFANG、VIP和CNKI等数据库,提取纳入病例报道中的患者生物学特征、主动脉瓣狭窄程度、胃肠道出血部位、手术治疗和预后等临床资料。
结果纳入的46篇病例报道共包括55例患者,年龄46~87岁。主动脉瓣轻度狭窄1例,中度狭窄1例,重度狭窄42例（余未提及）。胃肠道出血部位分别为结肠（n=8）、空肠（n=6）、回肠（n=4）、盲肠（n=3）、十二指肠（n=3）和多个部位（n=8）;未发现特定出血部位的患者为23例。术前血红蛋白水平为43~117 g/L。16例患者接受高分子量von Willebrand 因子检测,结果显示 1例正常,其余病例均下降。在55例患者中,43例接受经胸主动脉瓣置换术,12例接受经导管主动脉瓣置换术;14例完成机械瓣置换,33例完成生物瓣置换。5例患者出现并发症：1例术后出现瓣周漏和消化道出血,并行二次主动脉瓣置换术;2例患者反复出现胃肠道出血。2例分别因急性硬脑膜下血肿和多器官衰竭死亡。在随访期内,其余患者状况良好。
结论海德综合征是一种以主动脉瓣狭窄和消化道出血为特征的综合征。主动脉瓣置换术是海德综合征的一种有效治疗方法。

关键词: 海德综合征, 胃肠道出血, 主动脉瓣狭窄

Abstract:

Objective To explore the characteristics and clinical outcomes of patients with Heyde syndrome (HS) who undergo aortic valve replacement (AVR).
Methods Electronic databases including PubMed, Embase, Ovid, WANFANG, VIP and CNKI were searched to identify all case reports of HS patients undergoing AVR surgery, using different combinations of search terms “Heyde syndrome”, “gastrointestinal bleeding”, “aortic stenosis”, and “surgery”. Three authors independently extracted the clinical data including the patients’ characteristics, aortic stenosis severity, gastrointestinal bleeding sites, surgical treatments and prognosis.
Results Finally, 46 case reports with 55 patients aging from 46 to 87 years, were determined eligible and included. Of them, 1 patient had mild aortic stenosis, 1 had moderate aortic stenosis, 42 had severe aortic stenosis, and 11 were not mentioned. Gastrointestinal bleeding was detected in colon (n=8), jejunum (n=6), ileum (n=4), cecum (n=3), duodenal (n=3) and multiple sites (n=8). No specific bleeding site was identified in 23 patients. Preoperative hemoglobin level ranged from 43 to 117 g/L. All but one of 16 patients showed decreased level of high molecule weight von Willebrand factor. Of the 55 patients, 43 underwent AVR, and 12 received transcatheter AVR. Aortic valves of 14 cases were replaced by mechanical valves, and 33 cases by biological valves. All patients recovered well during the follow-up, except 5 patients. One patient who had perivalvular leakage and gastrointestinal bleeding after AVR underwent the second AVR. Two patients had recurrent gastrointestinal bleeding. Two patients died of life-threatening acute subdural hematoma and multiple organ failure, respectively.
Conclusions HS is a rare syndrome characterized by aortic stenosis and gastrointestinal bleeding. AVR is an effective treatment for HS.

Key words: Heyde syndrome, gastrointestinal bleeding, aortic valve stenosis

Huili Li, Peishuang Lin, Yuntai Yao. Aortic Valve Replacement for Patients with Heyde Syndrome: A Literature Review[J].Chinese Medical Sciences Journal, 2021, 36(4): 307-315.

图/表 3

Studies	Country	Age (yrs)	Sex	AS		AVA (cm²)	Gastrointestinal bleeding site^&	Hb (g/L)	vWF	Operation	Valve	Follow-up
Studies	Country	Age (yrs)	Sex	PPG (mm Hg)	MPG (mm Hg)	AVA (cm²)	Gastrointestinal bleeding site^&	Hb (g/L)	vWF	Operation	Valve	Prognosis	Time
Tsuchiya et al.^[5]	Japan	83	F	NR	81	0.5		NR	↓	TAVR	M	Well	2 years
Garcia et al.^[6]	Brazil	64	M	60	36.5	0.6	Jejunum	NR	NR	SAVR	M	Well
Mahboobi et al.^[7]	USA	56	F	NR	NR	NR		NR	NR	SAVR	NR	Well
Mirna et al.^[8]	Australia	73	NR	98	53	0.8	Ileum	65	NR	TAVR	M	Well
Alshuwaykh et al.^[9]	USA	56	F	NR	35	0.8		67	↓	TAVR	M	Well	6 months
Iijima et al.^[10]	Japan	77	M	88	NR	0.52		55	↓	SAVR	B	Well	20 months
Achamallah et al.^[11]	USA	73	F	NR	NR	0.75		NR	NR	SAVR	B	Well
Achamallah et al.^[11]	USA	84	F	NR	NR	0.94		NR	NR	TAVR	M	Well
Akutagawa et al.^[12]	Japan	67	F	NR	NR	0.8	Duodenum, jejunum	61	↓	SAVR	B	Well	6 months
Balbo et al.^[13]	Brazil	81	M	35	22	NR	colon	64	NR	TAVR	M	Well
Giovannini et al.^[14]	Italy	70	M	162	103	0.4	Jejunum	NR	NR	SAVR	B	Die	10 months
Iquille et al.^[15]	France	77	M	77	48	0.8		50	NR	SAVR	B	Well	1 year
Shibamoto et al.^[16]	Japan	87	M	NR	59	NR	Ileum	87	↓	SAVR	B	Well	20 months
Uchida et al.^[17]	Japan	77	F	100	NR	0.6	Colon	61	↓	SAVR	B	Die	31 days
Huang et al.^[18]	China	76	F	NR	NR	NR		65	↓	TAVR	M	Well
Hudzik et al.^[19]	Canada	82	M	NR	37	0.7		95	↓	TAVR	M	Well	12 months
Wang et al.^[20]	China	84	F	89	57	NR		69	NR	TAVR	M	Well	4 months
Perry et al.^[21]	USA	57	F	NR	NR	NR		NR	NR	SAVR	NR	Well
Benton et al.^[22]	USA	77	M	132	62	NR	Colon	NR	↓	TAVR	M	Well	10 months
İlkeli et al.^[23]	Turkey	46	F	144	86	NR	Duodenum, ileum, cecum, colon	90	NR	SAVR	B	Well	1 year
Kapila et al.^[24]	USA	70	M	NR	40	0.7	Duodenum, jejunum	NR	↓	SAVR	NR	Well	2 months
Dos Santos et al.^[25]	Brazil	71	M	NR	NR	NR	Cecum	NR	NR	SAVR	B	Well
Maor et al.^[26]	Israel	76	F	68	NR	0.8	Duodenum	70	NR	SAVR	B	Well	42 months
Negi et al.^[27]	USA	75	F	NR	NR	NR	Jejunum	NR	NR	TAVR	M	Well
Saad et al.^[28]	UK	76	F	72	45	0.9		50	NR	SAVR	B	Well	4 months
Godino et al.^[29]	Italy	83	F	104	58	0.7	Duodenum	61	NR	TAVR	M	Well	6 months
Gül et al.^[30]	Turkey	75	F	NR	52	0.6	Ileum, cecum	NR	NR	TAVR	M	Well	2 months
Michot et al.^[31]	France	79	M	140	75	0.6	Duodenum,ileum, cecum, colon	54	NR	SAVR	B	Well	9 years
Van et al.^[32]	Belgium	76	F	46	30	1		89	NR	SAVR	B	NR
Abi-Akar et al.^[33]	Lebanon	68	M	97	66	0.7	Duodenum, jejunum, colon	NR	NR	SAVR	B	Well	9 months
D’Souza et al.^[34]	Canada	79	M	NR	84	0.94	Jejunum	NR	↓	SAVR	B	Well
Takahashi et al.^[35]	Japan	82	F	139	NR	0.6	Colon	78	↓	SAVR	B	Well	4 years
Undas et al.^[36]	Poland	74	F	98	68	0.6	Jejunum	NR	Normal	SAVR	B	Well	7 months
Morishima et al.^[37]	Japan	78	F	127	NR	NR	Jejunum, colon	93	↓	SAVR	B	Well
Singh et al.^[38]	USA	71	F	100	65	NR		NR	NR	SAVR	B	Well	1 year
Soran et al.^[39]	UK	80	M	100	NR	NR	Ileum	94	NR	SAVR	B	Well	1 year
Warkentin et al.^[40]	Canada	65	F	NR	NR	0.67	Jejunum	NR	↓	SAVR	B	Well	10 years
Warkentin et al.^[40]	Canada	70	F	NR	NR	0.5	Colon	NR	↓	SAVR	B	Well	10 years
Anderson et al.^[41]	Australia	62	F	NR	NR	0.5	Cecum	NR	NR	SAVR	NR	Well	6 months
Anderson et al.^[41]	Australia	77	F	NR	NR	0.5	Ileum	NR	NR	SAVR	NR	Well	6 months
Olearchyk et al.^[42]	USA	69	M	>50	NR	0.6	Cecum	NR	NR	SAVR	M	Well
Apostolakis et al.^[43]	Germany	67	NR	NR	NR	NR	Colon	117	NR	SAVR	B	Not Well^*
Apostolakis et al.^[43]	Germany	68	F	NR	NR	NR	Colon	60	NR	SAVR	B	Not Well^#
Casson et al.^[44]	Canada	68	F	NR	60	NR		100	NR	SAVR	NR	Well
Baciewicz et al.^[45]	USA	56	M	32	NR	0.8	Ileum, cecum, colon	43	NR	SAVR	B	Well	8 months
Cappell et al.^[46]	USA	66	F	120	NR	0.6	Duodenum	NR	NR	SAVR	B	Well	15 months
Cappell et al.^[46]	USA	69	F	110	NR	0.5	Colon	NR	NR	SAVR	B	Well	18 months
Scheffer et al.^[47]	USA	78	F	NR	65	NR		87	NR	SAVR	B	Well	9 months
Marcus et al.^[48]	RSA	66	F	NR	NR	NR		NR	NR	SAVR	B	Well	42 months
Marcus et al.^[48]	RSA	78	M	NR	NR	NR		NR	NR	SAVR	B	Well	42 months
Marcus et al.^[48]	RSA	72	F	NR	NR	NR		NR	NR	SAVR	NR	Well	12 months
Marcus et al.^[48]	RSA	66	F	NR	NR	NR		NR	NR	SAVR	NR	Well	12 months
Danilewitz et al.^[49]	USA	66	F	115	NR	NR		NR	NR	SAVR	B	Well	12 months
Love et al.^[50]	NR	72	F	100	NR	NR		NR	NR	SAVR	B	Well
Love et al.^[50]	NR	61	F	160	NR	NR		NR	NR	SAVR	B	Well	10 months

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Products	Mechanism of action	Others
Desmopressin	Selective V2 agonist; Releases endogenous VWF, FⅧ	Expensive; 0.3 μg/kg, iv, over 30 minutes
Alphanate/Fanhdi/Humate P	VWF/FⅧ concentrates	Expensive; Increased chance of thromboembolism; Need to check the vWF and FⅧ level
Vonvendi	Recombinant VWF	Expensive; Less thromboembolic risk as does not increase FⅧ level;
Tranexamic acid	Antifibrinolytic agents	Can be administered along with desmopressin and vWF/FⅧ concentrate
Platelet	Platelet activation and aggregation	Transmission of infection
Fresh frozen plasma	Contains VWF	Transmission of infection
Cryoprecipitate	Contains VWF	Transmission of infection

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