原发性肺动脉高压50例临床分析

CLINICALPRIMARYPULMONARYHYPERTENSION:ARETROSPECTIVESTUDYOF50CASES

为探讨原发性肺动脉高压（ＣＰＰＨ）早期的临床诊断，本研究回顾性分析了５０例临床诊断为ＣＰＰＨ患者的临床特征及随访结果。５０例患者中男２１例，女２９例，年龄２９±１２岁。其最常见的首发症状为劳力性呼吸困难，至确诊时平均病程４．０７±３．８４年；最常见的体征为肺动脉瓣听诊区可闻及第二心音亢进和收缩期杂音。窦性心律为其心电图特征；Ｘ线胸片、超声心动图及肺动脉造影表现以肺动脉主干增宽（扩张）和（或）右心增大为主。３０例患者行血液动力学检测，平均肺动脉压为８．５３±２．００ｋＰａ（１ｋＰａ＝７．５ｍｍＨｇ），≥６．６７ｋＰａ者占８４％；平均右房压为１．６０±０．８０ｋＰａ。心功能Ⅲ～Ⅳ级者中女性较男性多见（Ｐ＜０．０５）。对１７例患者随访（平均３．８年），右心衰竭死亡者１０例，猝死者３例。表明ＣＰＰＨ至确诊时肺动脉压多已呈中、重度升高，病情重，预后差，提示应注重其早期诊断。

Aretrospectiveclinicalstudywasmadein50patients(21male,29female,meanage29±12years)withclinicalprimarypulmonaryhypertension(CPPH).Themeanintervalfromsymptomonsettodiagnosiswas4.07±3.84years.Themostfrequentsymptomsweredyspneaandpalpitation.In-creasedpulmonicsecondheartsound(P2)andright-sidedthirdand/orfourthheartsoundswerefoundin90%and30%ofpatientsrespectively.Electrocardiographyshowedrightventricularhypertrophy(86%)andunderlyingsinusrhythm(100%).Echocardiographyshowedrightventricularenlargement(78%)andtricuspidregurgitation(44%).Chestfilmshowedprominentmainpulmonaryartery(90%)andrightheartenlargement(82%),butitwasnormalin5%ofthepatients.Hemodynamicstudy(n=30)showedthreefoldincreaseinmeanpulmonaryarterypressure(MPAP)(8.53±2.00kPa,1kPa=7.5mmHg),mildtomoderateelevationinmeanrightatrialpressure(MRAP)(1.60±0.80kPa).Patientswithfunctionalclass(NYHA)3～4hadrelativelyhigherMPAP(8.53±2.00vs7.73±3.20kPa)andMRAP(1.87±2.40vs0.93±0.93kPa)thanthosewithclass1～2.Patientswithclass3～4commoninfemale(71%)thaninmale(29%),P<0.05.Duringameanperiodof3.8yearsoffolow-upin17patients,10diedofrightventricularfailureand3ofsuddendeath;theMPAPsofthedeadwere6.13～9.60kPa(mean7.84kPa).Conclusion:CPPHpatientshadseveresymptomsandremarkablephysicalorlaboratoryabnormal-itieswhenthediseasewasdiagnosed.Thelongintervalfromappearanceofsymptomstoestablishmentofdiagnosisemphasizedtheimportanceofbeingconsciousofsuchacondition.