不同手术时机对危重肺动脉瓣狭窄球囊扩张术疗效的影响研究

Perinatal integrative intervention for critical pulmonary artery valve stenosis

目的探讨不同手术时机对危重肺动脉瓣狭窄球囊扩张术疗效的影响。方法书面知情同意下纳入2007年4月至2011年12月四川大学华西第二医院胎儿超声心动图产前诊断和（或）经出生后超声心动图证实的危重肺动脉瓣狭窄患儿21例进行病例对照研究，实施介入手术时年龄≤60d，其中产前诊断者7例（产前组），院外转诊14例（分为产后A组和产后B组，产后A组：产后7～28d转诊到我院的危重型肺动脉瓣狭窄病例，共6例。产后B组：产后29—60d转诊到我院的危重型肺动脉瓣狭窄病例，共8例）。产前诊断者，经一体化干预小组共同会诊制定干预方案，进行宫内诊治、围产期监护、出生后急诊经皮肺动脉瓣球囊成形术（PBPV）；院外转诊者实施急诊PBPV。术前、术后1年内定期复查超声心动图评估右心室一肺动脉压差（PG）及右心室Tei指数。结果产前组出生后持续静脉滴注前列地尔状态下经皮氧饱和度（SpO：）82％～92％（86．57％±5．34％），无心功能不全患儿。出生后3～6d成功实施PBPV，术后Sp02（97．33±1．15）％，术前右心室肺动脉有创压差（86．34±11．77）mmHg（1mmHg=0．133kPa），术后右心室肺动脉有创压差（31．43±8．46）mmHg，术前RVTei指数0．68±0．05，术后迅速降低，术后1个月恢复正常。1例术后7个月因再狭窄再次实施PBPV。院外转诊14例，其中产前A组6例，术前心功能不全1例；产后B组8例，术前心功能不全3例。持续静脉滴注前列地尔状态下SpO：（83％～91％），手术时间为出生后10～57d，术后SpO，改善，术后心功能不全症状很快好转，甚至消失；术前右心室Tei指数明显增高，术后逐渐下降，产后A组术后12个月恢复正常，产后B组术后随访12个月，RVTel指数为0．51±0．06，仍然较产前组及产后A组高（P〈0．05）。1例术后9个月因再狭窄再次实施PBPV。产前组、产后A组、产后B组低氧暴露时间分别为（4．43±0．68）、（16．33±4．46）、（41．25±9．19）d，组问差异有统计学意义（P〈0．05）。结论产前明确诊断危重型肺动脉变狭窄、产后早期PBPV的患儿术前状况好、低氧暴露时间短、术后右心室功能恢复良好。“危重肺动脉瓣狭窄围生期一体化干预模式”能明显改善疾病预后及患儿生存质量。

Objective To investigate the effect of different operation time to percutaneous balloon pulmonic valvuloplasty （PBPV） to critical pulmonary valve stenosis （CPS）. Method Twenty-one infants （age〈60 days at operating day） suffered from CPS, diagnosed by fetal eehocardiogram and confirmed by echocardiography after birth, were enrolled in this case-control-study with written informed consent during April 2007 to December 2011. Of the 21 cases, 7 had prenatal diagnosis in our prenatal diagnosis center （prenatal group, Pre） and 14 were referred from other hospitals, who were divided into postpartum group A （Post A, referred within 28 days after birth） and postpartum group B （Post B, referred 29 to 60 days afterbirth）. To Pre-gToup, the integrative interventional protocol was cautiously made by the consultative specialists, including intrauterine diagnosis, perinatal care and urgent PBPV soon after birth. To Postgroup, emergency PBPV was preformed after the referral. Tel index of right ventricular and pressure-gradient （PG） between right ventricular and pulmonary artery were measured before and at different time points one year after PBPV. Result The values of SpO2 in Pre-group ranged from 82% - 92% （ 86. 57% ±5.34% ） under the state of continuous intravenous infusion of alprostadil. PBPV was successfully preformed within 3 - 6 days after birth. The values of SpO2 increased to 97.33% - 1.15% post procedure. The values of PG pre- and post- procedure were （ 86.34 ±11.77） mm Hg and （31.43 ± 8. 46） mm Hg respectively. Preoperative RV Tel-index was 0. 68 ±0. 05, it decreased rapidly after procedure, and recovered to normal one month after procedure. Only one case showed restenosis seven months after proeedure and repeated PBPV. Fourteen referral cases （6 cases in Post A group and 8 cases in Post B group, accompanied in 1 and 3 cases with heart failure） , the values of SpO2 ranged from 83% - 91% under state of continuous intravenous infusion of alprostadil. And the operating time was 10 -57 days after birth. The values of SpO2 recovered to normal post procedure, and heart failure alleviated. Increased preoperative RV pressure obviously decreased significantly post-procedure. And increased Tel-index declined gradually, at one-year follow-up, the value of Tel-index in Post A group recovered to normal, whereas that of Post B was （0. 51 ± 0. 06 ）, compared to Pre and Post A groups, the difference was significant（P 〈 0. 05 ）. One case showed restenosis nine months after procedure and repeated PBPV was performed. The hypoxic exposure durations were （4. 43±0. 68）, （ 16. 33 ±4. 46） , （41.25 ±9. 19 ） , respectively, and the difference among the three groups was significant （ P 〈 0. 05 ）. Conclusion To the fetuses with definite prenatal diagnosis of critical pulmonary valve stenosis, preoperative general condition can be adjusted to more suitable for emergency operation. Early PBPV can achieve shorter hypoxic exposure and better recovery of right ventricular function post procedure. Perinatal integrated intervention for CPS can significantly improve the prognosis and quality of life in this patient population.