肝硬化患者肺功能状态观察

目的：了解肝硬化患者的肺功能状态。方法：测定40例肝硬化患者的肺功能及血气分析,并以健康人群作对照。结果：肝硬化患者肺功能指标（FVC、FEV1、FEV1／FVC、MVV、V25、V50、V75、DLco,PaO2)较健康对照组明显减低,P（A-a)O2明显升高,有显著差异（P＜0．01）,且肝硬化患者病情越严重肺功能损害越明显。结论：肝硬化患者的FVC、FEV1、FEV1／FVC、MVV、V25、V50、V75、DLco,PaO2较对照组明显降低,P（A－a)O2明显升高,表明肝硬化患者存在肺通气功能障碍和弥散功能障碍。腹水、肝性胸水是肺通气功能障碍的主要原因;而肺内血流异常（肺内动－静脉分流和肺血管扩张）是肺弥散功能障碍的主要原因。     

^（99）Tc^m-MAA全身显像在肝肺综合征诊断中的作用

目的 研究99Tcm-聚合白蛋白(99Tcm-MAA)全身显像在判断肝硬化患者肝肺综合征(HPS)中的作用.方法 74例受试者分为肝硬化组和正常对照组;肝硬化组分为HPS组和非HPS.全部受试者均行99Tcm-MAA全身显像,然后计算分流率.结果 ①肝硬化患者的分流率[(4.6±0.7)%]高于正常对照组的分流率[(2.7±0.8)%],差别有统计学意义.②HPS组中呼吸困难和杵状指的发生率高于非HPS组;HPS组与非HPS组相比,PaO2、血氧饱和度(SaO2)降低,PO2(A-a)增高.HPS组分流率[(7.1±0.9)%]与非HPS组分流率[(3.0±1.2)%]相比明显增高.③不同Child-pugh分级之间PaO2、SaO2、PO2(A-a)的差异有统计学意义;而不同Child-pugh分级之间分流率差异无统计学意义.结论 99Tcm-MAA全身显像是诊断HPS一种无创、准确检查方法.     

肝硬化患者肝肺综合征核素诊断方法的一项对比研究

目的:评价和比较2毫居(mci)全身显像法和2 mci部显像法诊断肝肺综合征(HPS)的优劣,探讨更为实用的诊断方法.方法:选择2004年住院的55例肝硬化患者和8例健康志愿者,利用~(99m)锝(Tc)标记的多聚白蛋白(MAA)灌注显像技术,采用2 mci全身显像法和2 mci局部显像法测定其肺内分流率并诊断HPS.结果:2 mci全身显像组与2 mci局部显像组的55例肝硬化患者分流率分别为3.16%±1.58%和3.18%±1.60%,其诊断HPS的检出率分别为7.27%和9.09%,两种方法检出率相比均无统计学差异.采用2 mci全身显像方法,测定对照组分流率均数为2.76%.HPS组为6.87%,非HPS组为2.86%.采用2 mci局部显像法,测定对照组分流率均数为2.52%,HPS组为6.85%,非HPS组为2.82%.两种方法健康对照组与非HPS组分流率均无统计学差异(P=0.811和P= 0.461,HPS组分流率高于健康对照和非HPS组(P<0.001).经计算Kappa值为0.88,说明两种检验方法检验结果一致性属于最强.结论:肝肺综合征的核素诊断方法应进一步统一和规范,2 mci全身显像法和2 mci局部显像法在肝肺综合征的诊断中均可靠、安全而实用,值得在国内推广.     

肝肺综合征的临床特点及诊断

目的探讨肝肺综合征(HPS)的临床特点及诊断方法,提高对其认识.方法回顾性分析我院1986年1月～2003年12月确诊的13例患者的临床资料.结果除1例为慢性酒精性肝损害基础上的急性药物性肝炎外,12例均为肝硬化患者,据Child-Pugh分级标准,肝功能B级和C级者11例(84.60%);平均动脉血氧分压(PaO2)为6.94±1.5 kPa,10例中9例直立位缺氧大于10%.临床主要表现为呼吸困难(100%)、紫绀(100%)、杵状指(84.6%)、肝掌(84.6%)、面部毛细血管扩张(84.6%)、蜘蛛痣(69.2%).9例行肺功能检查患者中8例为弥散功能障碍.所有患者均经99mTc标记的人血白蛋白聚合颗粒(99mTc-MAA)首次通过肺灌注显像确诊,测定肺内分流率为22%～57%.11例同时计算吸入100%氧气时动静脉分流占总血流量百分比(Qs/Qt)的结果为10.9%～27.4%,均小于前者所得.结论肝肺综合征的发生与肝功能不全和门脉高压症都有关系,同时有门静脉高压、紫绀、杵状指、蜘蛛痣或面部毛细血管扩张时应考虑该诊断.直立位缺氧(>10%)为典型表现,99mTc-MAA首次通过肺灌注显像是有效的诊断方法,所测定的肺内分流率大于Qs/Qt.     

肺灌注扫描在肝肺综合征诊断中的作用——附3例报告

肝肺综合征(Hepatopulmonary syndrome,HPS)是以肝功能不全,肺泡气-动脉血氧分压差上升和肺内血管扩张为三大主征的综合征。及早诊断HPS并采取相应治疗对改善其预后有很大的帮助。有报道认为99mTc-MAA肺灌注扫描是诊断肝肺综合征的一种方法,并利用99mTc-MAA肺灌注扫描显示肝肺综合征组肺外分流率明显高于肝硬化组和正常对     

对肝肺综合征诊治的76例临床分析

目的:探讨肝肺综合征(HPS)的临床特点及诊断方法,提高对其认识。方法:回顾性分析76例HPS患者的临床资料,并与无HPS的133例肝硬化患者在肺功能、血气分析等方面进行比较。结果:在HPS组中除4例为慢性酒精性肝损害基础上的急性药物性肝炎外,72例均为肝硬化患者。临床表现为呼吸困难、紫绀、杵状指及蜘蛛痣。据Child-Pugh分级标准,肝功能B级和C级者59例(77.6%),平均PaO2为(79.93±11.29)mm Hg(1 mm Hg=0.133 kPa),对照组为(92.48±13.53)mm Hg,2组比较有统计学意义(P0.01)。HPS组中76例中有48例直立性缺氧10%(63.1%)。不同门静脉内径、有无蜘蛛痣、不同肝功能分级的PaO2和SaO2值比较,均有统计学意义(P0.01)。影像学检查结果示肺内血管扩张。结论:肝肺综合征的发生与肝功能不全有明显的关系,该病患者常有呼吸困难、紫绀、杵状指、蜘蛛痣及直立性缺氧等典型表现,患者低氧血症发生率高,而肺功能存在弥散功能障碍,肺血管有明显的扩张。     

门脉性肝硬化与肺内真性分流的关系探讨

本文对29例门脉性肝硬化作动脉血氧分压(PaO_2)、肺泡动脉血氧分压差(P_A-aO_2)、肺内右→左分流占总肺血流的百分比(Qs/Qt)测定,从而探讨了肝硬化合并肺内真性分流的关系,阐述了发病机理,对治疗提出一点看法。     

高压氧治疗肝肺综合征大鼠的实验研究

目的 研究高压氧治疗对大鼠肝肺综合征(HPS)的作用.方法 采用胆总管结扎术(CBDL)制备HIS动物模型.44只雄性SD大鼠随机分为4组:假手术对照组(Sham组)8只、单纯造模组(CBDL5w组)12只、治疗1周组(HBOT1w组)12只、治疗2周组(HBOT2w组)12只.两治疗组在造模成功后送入高压氧舱治疗,分别治疗1周和2周.Sham组和CBDL5w组大鼠在术后5周,HBOT1w组和HBOT2w组在治疗结束后,进行血气分析、肝功能以及血清一氧化氮(NO)、内皮素-1(ET-1)浓度检测,肝、肺病理检查,并使用激光多普勒血流仪检测肝、肺微循环.结果 CBDL5w组大鼠肝功能检测中ALB降低,ALT、AST、ALP、γ-GT、TBIL及TBA均升高,血气分析中PaO2降低,PA-aO2增大,与Sham组相比均有显著差异(P＜0.01),且病理检查CBDL5w组大鼠的肝脏组织可见胆汁性肝纤维化表现,肺组织可见肺毛细血管扩张、充血,证实HPS大鼠模型制成;同时与Sham组相比,CBDL5w组大鼠血清NO及ET-1浓度均升高(P＜0.01),肝脏毛细血管血流量降低(P＜0.01),肺脏毛细血管血流量增大(P＜0.01).经高压氧治疗后,HBOT1w组大鼠与CBDL5w组相比,以上指标均好转(P＜0.01).HBOT2w组大鼠与CBDL5w组相比,除血清NO水平和肺脏毛细血管血流量无统计学差异(P＞0.05)外,其余指标的差异有显著性意义(P＜0.01).结论 高压氧治疗大鼠HPS可通过降低血清NO、ET-1浓度,提高组织氧含量及改善肝、肺微循环等,有效改善肝、肺功能及病理,且治疗前期效果更好.     

肝肺综合征患者降钙素基因相关肽及内皮素含量检测

目的　肝肺综合征 (HPS)的发生与血循环或肺内扩张 /收缩血管物质失衡密切相关。检测HPS患者血浆中降钙素基因相关肽 (CGRP)和内皮素 (ET)含量 ,以探讨它们在HPS发病中的作用。方法　选择经肺灌注扫描及血气分析诊断的HPS患者 16例 ,无HPS的肝硬化患者 30例 ,用放射免疫分析法测定其血浆CGRP、ET含量 ,同时以 15名健康献血员作为对照。结果　HPS组血浆CGRP[(6 5± 15 )pg/ml]、ET[(78± 2 1) pg/ml]显著高于肝硬化组 [(5 1± 15 ) pg/ml,(6 0± 14 ) pg/ml,P <0 .0 1],也显著高于对照组 [(32± 12 ) pg/ml,(36± 11)pg/ml,P <0 .0 1];肝硬化组与正常对照组比较差异也有显著性(P <0 .0 1)。线性相关分析显示 ,HPS组患者血浆CGRP及ET含量与动脉血氧分压呈显著负相关 ,与肺内分流率大小无关。结论　HPS患者血浆CGRP、ET含量增高 ,其增高可能与HPS肺内血管扩张和动脉血氧分压下降有关。     

右旋美托咪啶复合异氟烷对单肺通气肺内分流及动脉氧合的影响

目的:观察右旋美托咪啶(Dex)持续静脉输注复合异氟醚(ISO)吸入用于单肺通气,肺内分流、动脉氧合以及血流动力学变化。方法:60例美国麻醉医师协会分级(ASA)Ⅰ～Ⅱ级择期开胸手术患者,随机分为静脉输注Dex复合ISO吸入组(DISO组)与静脉输注生理盐水复合ISO吸入组(NISO组)。2组患者麻醉诱导药物一致,麻醉维持均静脉持续输注瑞芬太尼0.1～0.2μg.kg-1.min-1,吸入ISO 1.0%～2.0%。DISO组持续静脉输注Dex 0.7μg.kg-1.h-1,NISO组持续静脉输注等容量0.9%氯化钠液。持续观察心率(HR)、平均动脉血压(MAP)、脉搏氧饱和度(SpO2),脑电双频指数(BIS),分别于单肺肺通气前、单肺通气10 min(OLV-10 min)、20 min(OLV-20 min)、30 min(OLV-30 min)、40 min(OLV-40 min)监测动脉血气、中心静脉血气及呼气末ISO浓度(EEIso)。通过BIS调整ISO吸入浓度。结果:45例患者完成研究,DISO组23例,NISO组22例;2组患者单肺通气时动脉氧分压、分流量与单肺通气前比较,PaO2明显下降(P<0.05),Qs/QT明显增加(P<0.05);单肺通气期间,与NISO组比较,DI-SO组PaO2明显增加(P<0.05),Qs/QT明显减少(P<0.05);DISO组EEIso明显减少(P<0.05),心率明显减少(P<0.05),平均动脉血压以及血管活性药物的使用比较,差异无统计学意义(P>0.05)。结论:静脉输注Dex复合ISO吸入用于单肺通气,能够降低动脉血氧分压下降程度、减少肺内分流及减少ISO需要的剂量。     

Arterial oxygenation associated with portopulmonary hypertension

STUDY OBJECTIVES: To characterize arterial oxygenation in patients referred to Mayo Clinic for liver transplantation with a diagnosis of portopulmonary hypertension (portoPH). DESIGN: Prospective study. SETTING: Liver transplantation program and pulmonary hypertension clinic in a tertiary referral center. PARTICIPANTS: Twenty consecutive patients with abnormal pulmonary hemodynamics documented by right-heart catheterization (mean pulmonary artery pressure [MPAP] > or = 25 mm Hg, pulmonary vascular resistance [PVR] > or = 120 dyne.s.cm(-5), and pulmonary capillary wedge pressure [PCWP] < or = 15 mm Hg). Liver transplant candidates with normal pulmonary hemodynamics via screening Doppler echocardiography (n = 40) served as control subjects. A subgroup of patients underwent postural and inspired 100% oxygen blood gas analysis, contrast echocardiography, and technetium-labeled macroaggregated albumin ((99m)TcMAA) lung/brain scanning to identify and quantitate the degree of intracardiac or intrapulmonary shunting. MEASUREMENTS AND RESULTS: portoPH was moderate to severe (MPAP > 35 mm Hg) in 18 of 20 patients (90%). Arterial-alveolar oxygen pressure gradient (P[A-a]O(2)) was abnormal (> or = 20 mm Hg) in 16 of 20 patients (80%). PaO(2) was abnormal (< or = 70 mm Hg) in 3 of 20 patients (15%). Pa0(2) was significantly less and P(A-a)O(2) was significantly greater compared to control subjects (p < 0.001). All patients had normal (99m)TcMAA brain uptake (< 6%) and negative transthoracic contrast echocardiographic findings. No significant correlations were found between oxygenation and hemodynamic variables (MPAP, PVR, PVR index, and transpulmonary gradient). CONCLUSIONS: Arterial oxygenation associated with portoPH was frequently abnormal and significantly worse when compared to patients with normal pulmonary hemodynamics by Doppler echocardiography. Hypoxemia, as measured by PaO(2) and P(A-a)O(2), was usually mild even in the setting of moderate-to-severe portoPH.     

Hepatopulmonary syndrome: a prospective study of relationships between severity of liver disease, PaO(2) response to 100% oxygen, and brain uptake after (99m)Tc MAA lung scanning

BACKGROUND: Because of the spectrum of intrapulmonary vascular dilation that characterizes hepatopulmonary syndrome (HPS), PaO(2) while breathing 100% oxygen varies. Abnormal extrapulmonary uptake of (99m)Tc macroaggregated albumin (MAA) after lung perfusion is common. GOAL: To describe relationships between (1) severity of liver disease measured by the Child-Pugh (CP) classification; (2) PaO(2) while breathing room air (RA) and 100% oxygen on 100% oxygen; and (3) extrapulmonary (brain) uptake of (99m)Tc MAA after lung scanning. Methods and patients: We prospectively measured PaO(2) on RA, PaO(2) on 100% oxygen, and brain uptake after lung perfusion of (99m)Tc MAA in 25 consecutive HPS patients. RESULTS: Mean PaO(2) on RA, PaO(2) on 100% oxygen, PaCO(2) on RA, and (99m)Tc MAA brain uptake were similar when categorized by CP classification. Brain uptake was abnormal (> or = 6%) in 24 patients (96%). Brain uptake was 29 +/- 20% (mean +/- SD) and correlated inversely with PaO(2) on RA (r = -0.57; p<0.05) and PaO(2) on 100% oxygen (r = -0.41; p<0.05). Seven patients (28%) had additional nonvascular pulmonary abnormalities and lower PaO(2) on 100% oxygen (215+/-133 mm Hg vs 391+/-137 mm Hg; p<0.007). Eight patients (32%) died. Mortality in patients without coexistent pulmonary abnormalities was associated with greater brain uptake of (99m)Tc MAA (48+/-18% vs 25+/-20%; p<0.04) and lower PaO(2) on RA (40+/-7 mm Hg vs 57+/-11 mm Hg; p<0.001). CONCLUSION: The degree of hypoxemia associated with HPS was not related to the CP severity of liver disease. HPS patients with additional nonvascular pulmonary abnormalities exhibited lower PaO(2) on 100% oxygen. Mortality was associated with lower PaO(2) on RA, and with greater brain uptake of (99m)Tc MAA.     

Pulmonary dysfunction and hepatopulmonary syndrome in cirrhosis and portal hypertension

Background: Pulmonary dysfunction including the hepatopulmonary syndrome (HPS) is an important complication to cirrhosis and portal hypertension. However, the precise relation to liver dysfunction and the prevalence of HPS are unclear. Aims: We therefore aimed to assess (i) the prevalence of HPS in consecutive alcoholic cirrhotic patients, (ii) the degree of pulmonary dysfunction in relation to liver function and (iii) the response of a 100% oxygen test on cardiopulmonary and peripheral oxygenation. Methods: Fifty patients with cirrhosis and 12 matched healthy controls were entered in this study. All underwent haemodynamic and pulmonary investigations [lung diffusing capacity for carbon monoxide (DLCO), contrast‐enhanced echocardiography and detection of extrapulmonary shunt fraction]. A 100% oxygen test was performed with the assessment of arterial oxygen tension (PaO₂), the alveolar‐arterial oxygen gradient (AaPO₂) and peripheral transcutaneous oxygen tension (tcPO₂). Results: The prevalence of HPS was 10%. PaO₂ and DLCO were reduced in 32 and 72% and AaPO₂, was increased in 60% of the patients respectively. DLCO correlated with indicators of liver dysfunction (galactose elimination capacity, P<0.01, indocyanine green clearance, P<0.001), portal hypertension (post‐sinusoidal resistance, P<0.01) and central hypovolaemia (central and arterial blood volume, P<0.01). After 100% oxygen inhalation, the changes in PaO₂, AaPO₂, tcPO₂ and heart rate were abnormal in the patients compared with controls (P<0.02). Conclusions: Pulmonary dysfunction in alcoholic cirrhosis is common and relates to different aspects of liver dysfunction, whereas the prevalence of HPS is low. The haemodynamic response to oxygen inhalation is clearly impaired and HPS and pulmonary dysfunction seem to be caused by different pathophysiological mechanisms.     

Scintigraphic evaluation of intrapulmonary shunt in normoxemic cirrhotic patients and effects of terlipressin

Aim: The magnitude of intrapulmonary shunt (IPS) in cirrhotic patients without hypoxemia remains undefined. We evaluated the severity and clinical correlations of IPS in normoxemic cirrhotics, and possible IPS alterations after terlipressin treatment. Methods: Fifteen patients with alcoholic cirrhosis without hypoxemia were studied at baseline and after the administration of 2 mg of terlipressin. The IPS fraction was evaluated by lung perfusion scan after the i.v. injection of technetium‐99m‐labeled macroaggregated albumin (^99mTc‐MAA) and calculation of brain uptake (positive value ≥6%). Cardiac output (CO), pulmonary artery systolic pressure (PASP) and pulmonary vascular resistance (PVR) were evaluated by Doppler echocardiography. Mean arterial pressure (MAP) was measured and the ratio MAP/CO was calculated as an index of systemic vascular resistance (SVR). Portal vein velocity (PVV) and portal venous flow (PVF) were also assessed by Doppler ultrasonography. Results: Three patients (20%) had an IPS fraction of more than 6%. A significant inverse correlation with platelet count (P = 0.001) and a direct correlation with Child–Pugh score (P = 0.06), PVV (P = 0.07) and PVF (P = 0.07) were noted. IPS fractions decreased significantly after terlipressin administration (P = 0.00001); the IPS fraction fell below 6% in all three patients with positive baseline values. Terlipressin treatment induced a significant decrease in CO (P = 0.003) and significant increases in MAP (P = 0.0003), SVR (P = 0.0003), SPAP (P = 0.001) and PVR (P = 0.01). Conclusion: IPS fractions detected by ^99mTc‐MAA lung scan were inversely correlated with platelet count and directly with liver disease severity, and found abnormal in 20% of normoxemic cirrhotic patients. Terlipressin reduced significantly the magnitude of the shunt.     

肝肺综合征23例临床分析

目的提高对肝肺综合征(HPS)的临床认识,减少漏诊、误诊。方法回顾我院确诊的23例肝肺综合征病例,分析肝肺综合征的临床特点。结果肝肺综合征男、女发病比例为1.3∶1,平均年龄(42±21)岁,除1例急性重症肝炎外,其余22例均为肝硬化,门静脉高压发生率为82.6%。根据Child-Pugh分级标准,肝功能B、C级为78.2%。主要临床表现:呼吸困难发生率为91.3%,发绀为91.3%,肝掌为69.5%,杵状指(趾)为65.1%,面部血管扩张为56.5%,蜘蛛痣为56.5%。动脉血氧分压(PaO2)平均值为(50.8±14.1)mmHg(1mmHg=0.133kPa),直立性低氧血症发生率为85.7%(12/14),肺一氧化碳弥散量百分比(DLCO%)平均值为43.1%。39.1%(9/23)患者胸部X线检查表现为HPS而出现双下肺斑点状、小结节影或网状结节影。23例HPS均进行了99m锝-大颗粒聚合白蛋白(99mTc-MAA)核素首次通过肺灌注显像,平均分流率为36.3%。结论HPS主要发生于肝硬化Child-Pugh分级较重和(或)门静脉高压的患者。临床上对不能解释的低氧血症,同时有门静脉高压、蜘蛛痣(面部血管扩张)和(或)杵状指(趾)表现者应高度怀疑肝肺综合征。直立性低氧血症是HPS较特异性表现。HPS可有肺弥散功能降低、胸部影像学异常改变,但不具有特异性。99mTc-MAA核素首次通过肺灌注显像有助于确定肺血管扩张,明确HPS的诊断。     

Evaluation of intrapulmonary vascular dilatations with high-resolution computed thorax tomography in patients with hepatopulmonary syndrome

GOALS: We aimed to determine the role of thorax high-resolution computed tomography (HRCT) in demonstrating the pulmonary vasodilatation in patients with hepatopulmonary syndrome (HPS). BACKGROUND: Traditionally, the presence of intrapulmonary vascular dilatations can be detected by using one of the three diagnostic modalities: contrast-enhanced echocardiography, technetium 99 m-labeled macroaggregated albumin scan, and pulmonary angiography. STUDY: The study group included 10 patients with HPS (Group 1), 12 patients with normoxemic cirrhosis (Group 2), and 12 healthy controls (Group 3). All of the subjects underwent conventional and HRCT of thorax. The diameters of pulmonary trunk, main pulmonary arteries, and right lower lobe basal segmental arteries were measured. The ratios of right lower lobe basal segmental pulmonary artery to bronchus diameter were calculated. RESULTS: The mean diameters of the main pulmonary trunk, right and left main pulmonary arteries were not different between the groups. Mean diameters of right lower lobe basal segmental pulmonary arteries were significantly higher in Group 1 compared with Group 2 (P=0.01) and Group 3 (P=0.002). Mean right lower lobe basal segmental pulmonary artery to bronchus ratios were significantly higher in Group 1 compared with Group 2 (P=0.03) and Group 3 (P<0.001). Group 2 had significantly higher pulmonary artery to bronchus ratios than Group 3 (P<0.001). CONCLUSIONS: Thorax HRCT may be helpful in the diagnosis of HPS by demonstrating the dilated peripheral pulmonary vessels or increased pulmonary artery to bronchus ratios in patients with liver disease and hypoxemia.     

Single photon emission computed tomography in chronic thromboembolic pulmonary hypertension

Background and objective: The management of chronic thromboembolic pulmonary hypertension (CTEPH) is largely dependent on the extent of obstruction in the pulmonary arteries. Planar perfusion scans are commonly used to quantify perfusion defects in CTEPH patients. However, planar scans typically under‐represent the extent of vascular obstruction in CTEPH. We conducted this study to test the hypothesis that SPECT lung perfusion scans are more accurate than planar scans for determining the location and extent of perfusion defects in patients with CTEPH. Methods: Planar ventilation scans, planar and SPECT perfusion scans were performed preoperatively in patients undergoing pulmonary thromboendarterectomy for treatment of CTEPH. Two clinical experts independently documented the segmental anatomy of the vascular obstructions by reviewing clinical records, pulmonary and CT angiograms, and surgical specimens. A nuclear medicine expert documented the segmental anatomy of the perfusion defects observed by planar and SPECT scans independently. Results: Clinical/pathological evaluation disclosed 241 obstructed and 99 unobstructed lung segments in 17 patients. Sensitivity for detecting obstructed segments was significantly higher for SPECT than for planar scanning (63.5 ± 3.1% vs 42.7 ± 3.2%, respectively; P < 0.01). Specificities of SPECT and planar scanning were not significantly different (62.6 ± 4.8% vs 76.8 ± 4.2%, respectively; P = 0.092). Conclusions: The SPECT is more sensitive than planar perfusion scanning for identifying obstructed segments in CTEPH. However, even SPECT under‐represents the true extent of the vascular occlusions in CTEPH.     

Hepatopulmonary syndrome can show spontaneous resolution: Possible mechanism of portopulmonary hypertension overlap?

Hepatopulmonary syndrome (HPS) (hypoxaemia due to intrapulmonary vasodilation and a right-to-left shunt associated with liver disease) resolves after liver transplantation. The authors describe a case of spontaneous resolution of HPS prior to liver transplantation. This patient was diagnosed with HPS associated with extra-hepatic biliary atresia when she was 10 years old. She exhibited digital clubbing, facial vascular dilation, cyanosis, and suffered from dyspnoea during exercise. The patient's PaO(2) at rest was 53.8 mm Hg in room air and a Technetium-99m macro-aggregated albumin lung perfusion scan demonstrated a right-to-left shunt. Although her symptoms and laboratory data supported a diagnosis of HPS, she nevertheless showed spontaneous resolution within 2 years. When she was 14 years old, pulmonary hypertension was evident upon examination of her echocardiogram. HPS may be improved or masked by an accidental overlap with pulmonary hypertension in the terminal stage of liver disease.     

Clinical features of hepatopulmonary syndrome in cirrhotic patients

AIM:To evaluate the frequency,clinical andparaclinical features of hepatopulmonary syndrome(HPS)and to determine their predictive values in di-agnosis of this syndrome in patients in Iran.METHODS:Fifty four cirrhotic patients underwentcontrast enhanced echocardiography to detect intra-pulmonary and intracardiac shunts by two cardiolo-gists.Arterial blood oxygen,O_2 gradient(A-a)andorthodoxy were measured by arterial blood gas(ABG)test.The patients positive for diagnostic criteria ofHPS were defined as clinical HPS cases and thosemanifesting the intrapulmonary arterial dilation but noother criteria(arterial blood hypoxemia)were definedas IHPS cases.HPS frequency,sensitivity,positive andnegative predictive values of clinical and paraclinicalfeatures were studied.RESULTS:Ten(18.5%)and seven(13%)cases hadclinical and subclinical HPS,respectively.The mostcommon etiology was hepatitis B.Dyspnea(100%)and cyanosis(90%)were the most prevalent clinicalfeatures.Dyspnea and clubbing were the most sen-sitive and specific clinical features respectively.Nosignificant relationship was found between HPS andsplenomegaly,ascites,edema,jaundice,oliguria,andcollateral veins.HPS was more prevalent in hepatitisB.PaO_2<70 and arterial-alveolar gradient had thehighest sensitivity in HPS patients.Orthodoxy speci- ficity was 100%CONCLUSION:Clubbing with positive predictive val-ue(PPV)of 75% and dyspnea with negative predic-tive value(NPV)of 75% are the best clinical factorsin diagnosis of HPS syndrome.PaO_2<70 and P(A-a)O_2>30 and their sum,are the most valuable negativeand positive predictive values in HPS patients.     

Per rectal portal scintigraphy as a useful tool for predicting esophageal variceal bleeding in cirrhotic patients

AIM： To investigate potential roles of per rectal portal scintigraphy in diagnosis of esophageal varices and predicting the risk of bleeding. METHODS： Fifteen normal subjects and fifty cirrhotic patients with endoscopically confirmed esophageal varices were included. Patients were categorized into bleeder and non-bleeder groups according to history of variceal bleeding. All had completed per rectal portal scintigraphy using ^99mTechnetium pertechnetate. The shunt index was calculated from the ratio of ^99mTechnetium pertechnetate in the heart and the liver. Data were analyzed using Student＇s t-test and receiver operating characteristics. RESULTS： Cirrhotic patients showed a higher shunt index than normal subjects （63.80 ± 25.21 vs 13.54 ± 6.46, P 〈 0.01）. Patients with variceal bleeding showed a higher shunt index than those without bleeding （78.45 ± 9.40 vs 49.35 ± 27.72, P 〈 0.01）. A shunt index of over 20% indicated the presence of varices and that of over 60% indicated the risk of variceal bleeding. CONCLUSION： In cirrhotic patients, per rectal portal scintigraphy is a clinically useful test for identifying esophageal varices and risk of variceal bleeding.