一例室间隔完整的肺动脉闭锁朱鲜阳课件

一例室间隔完整的肺动脉闭锁 介入治疗的远期随访报告 沈阳军区总医院先心病内科 朱鲜阳 病史和体征 患儿女，9m，因活动后气促伴有口唇青紫 9m于2005-10-24入院。查体：身高 68cm，体重8.5kg，口唇紫绀，双肺呼吸 音清晰，心率114次/分，律规整，胸左第 2肋间闻及连续性机器样杂音，肺动脉瓣区 第二音增强。肝脾肋下未触及，可见杵状 指（趾） 辅助检查 l生化检查均正常 l心电图：窦性心律，右房、右室大 l心脏X线：肺血少，心胸比值0.75 l超声心动图：室间隔完整的肺动脉闭锁，PFO 2mm ，PDA 2.2mm，三尖瓣重度关闭不全，三尖瓣环直 径2.4cm，三尖瓣口面积176mm2，右室内径20mm ，LVEDD 26mm、LVEDV17ml、LVESV3ml、 LVEF0.82 l经皮血氧饱和度为77% 治疗经过 于2005-11-01在全麻下行右室、主动脉造影 ，肺动脉闭锁射频打孔、肺动脉瓣球囊成形术 ，术中测左室压80/2 mmHg 主动脉压80/50 mmHg 右室压69/10 mmHg 治疗经过 行肺动脉瓣射频打孔，将导引导管置于右室漏斗部 闭锁的肺动脉瓣下，以5瓦能量放电3秒，打孔成功 后用3.5*20mm冠脉球囊导管、10atm有效扩张肺 动脉瓣，更换12号Numed肺动脉瓣球囊扩张至凹 征消失，术后测量肺动脉压25/15 mmHg，右室漏 斗部压40/10 mmHg，右室压60/10 mmHg，跨 肺动脉瓣压差15 mmHg 术后经皮血氧饱和度维持在92%，继续给予多巴胺 、前列地尔静脉泵入 1次术后复查 l心脏超声：PFO 2mm，PDA 2.2mm， 三尖瓣轻度关闭不全，右室内径10mm， LVEDD 27mm、LVEDV20ml、 LVESV3ml、LVEF0.85 l心脏X线：肺纹理清晰，心胸比值0.75 l术后1周出院 l术后患儿生长发育良好，无气促、紫绀 术后1年半再次入院 2007-04-16再次入院 查体：身高93cm，体重14kg，无紫绀、 杵状指（趾），胸左2肋间有连续性杂音 股动脉血氧饱和度93% 再次心导管检查和介入治疗 2007-04-18在全麻下行右心导管、PDA封堵术 ，术中测右房压15/7mmHg、右室压58/5 mmHg、肺动脉压38/12mmHg，左室压 117/0mmHg、升主动脉压117/65 mmHg， 升主动脉造影测量动脉导管主动脉端12.0mm ，肺动脉端3.0mm，选择8/10号PDA封堵伞 进行封堵，术后重复测量肺动脉压30/10 mmHg，右室压40/5 mmHg，重复主动脉造 影未见残余分流 2次术后复查 l超声心动图：右室内径20mm、LVEDD 36mm、LVEDV41ml、LVESV11ml、 LVEF 0.77 l心脏X线：心胸比值0.70 l心电图：窦性心律、右心室肥厚 l术后第5天出院 射频打孔器材 射频打孔器材 射频打孔示意图 射频打孔前右室和主动脉造影射频打孔前右室和主动脉造影 F6右冠导管右室肺动脉瓣下定位，导入同轴导 管与射频打孔导管至肺动脉盲端，5W 3秒打孔成功 推送同轴导管至肺动脉更换冠脉导丝采推送同轴导管至肺动脉更换冠脉导丝采 用冠脉球囊和肺动脉瓣球囊扩张用冠脉球囊和肺动脉瓣球囊扩张 术后即刻与术后即刻与1 1年半后右室造影比较年半后右室造影比较 主动脉造影主动脉造影国产10/8号PDA封堵器封堵后吸氧Sat O2 96%，停吸氧30分SatO2不变 讨 论 概述 PA-IVS约占先心病的0.71%3.1% 如不治疗，85半年内死亡 外科手术，术后一年内死亡率52 病理解剖 闭锁瓣膜的形态学改变 型为三个增厚的瓣叶融合，联 合嵴线在中央 型为增厚的瓣膜完全闭锁，联 合嵴线在周围 病理解剖 三尖瓣结构异常分三型 型三尖瓣发育不良及三尖瓣环小 型严重三尖瓣发育不良 型右室发育良好 病理解剖 右心室发育不良分三型 型 右心室流入部、小梁部、流出 部均存在 但右室腔小（占53） 型 流出部缺如（占19） 型 仅有流入部（占28） 病理解剖 右室依赖性冠状血管循环 主动脉冠状动脉无连接，冠状动脉近 端闭锁、狭窄或离断 远端供血源于右心室，发生率约占20 血液动力学改变 房间交通伴右向左分流时左心容 量负荷增加，若房间交通过小或 过早关闭可引起体静脉淤血，死 于低排血量心衰 血液动力学改变 房间右向左分流，右室冠状动脉瘘 和右室高压使右室血液经窦状隙与冠 状动脉直接通道，使体循环和冠状动 脉血氧饱和度下降可引起缺血、缺氧 和代谢性酸中毒 血液动力学改变 PDA持续开放伴左向右分流是肺血流的唯 一来源，开放程度对婴儿存活起着关键作用 右室依赖性冠脉循环者，任何使右室收缩压 突然减低的原因都可能出现不可逆的心肌缺 血 肺动脉闭锁呈隔膜型，右心室发育近于正常心 腔由三部分结构组成 漏斗部开放呈长管形，发育好，肺动脉发育好 （Nakata 指数评估） 无右室依赖性冠状动脉循环（窦状隙开放） 适应证 禁忌证 右室腔很小 漏斗腔严重细小 存在冠状动脉异常 并发症 潜在的股动脉闭塞（13） 心脏穿孔（2044）多见于瓣膜打孔 后导引导丝穿破主肺动脉 严重主肺动脉瘤，重度三尖瓣反流和恶性心 律失常或心脏阻滞 本院于2005年4月起用 该技术在国内率先治疗 10例婴儿PAIVS 结 论 通过介入法实现双室循环的技术在国内刚刚起 步，但实施介入时患儿小且危重，易发生严重 并发症而使其应用受到一定的限制，操作规范 和及时处理可降低死亡率。此类患儿实施介入 治疗，可避免或延迟新生儿和婴儿心肺转流手 术，减少死亡率，与外科手术对比中显示更安 全有效，部分病例可取代外科治疗 Long-term outcome of percutaneous intervention for pulmonary atresia and intact ventricular septum: a case report Xian-yang Zhu Department of Congenital heart disease, the General Hospital Shenyang Military Region Case presentation A 9 months female infant Admitted for exertional dyspnea with blue lips on Oct 24, 2005. Physical Exam: Height 68 cm，weight 8.5 kg Cyanosis and clubbed fingers and toes HR: 114bpm with regular rhythm An accentuated P2 and a continuous murmur over the left second intercostal space Lab test lLiver and renal functions: normal. lRCG：sinus rhythm with RA and RV hypertrophy. lX-ray：C/T ratio 0.75 with reduced pulmonary markings lUCG：Pulmonary atresia with intact ventricular septum， PFO 2mm，PDA 2.2mm，severe TI，diameter of the tricuspid annulus 2.4cm，tricuspid valve area 176mm2， inner diameter of RV 20mm，LVEDD 26mm, LVEDV 17ml, LVESV 3ml, LVEF 0.82. lPercutaneous oxygen saturation: 77%. Treatment Under general anesthesia, right ventricular angiography and aortography followed by radiofrequency perforation and balloon valvuloplasty were performed on Nov 1st, 2005. Pressure: 80/2 mmHg in LV 80/50 mmHg in aorta 69/10 mmHg in RV Procedure A guiding catheter was placed under the atresic PV. The radiofrequency energy was 5 watts for 3s. The valve was pre-dilated with a 3.5mm20mm balloon at 10 atm pressure and subsequently with 12mm30mm. Post-procedure pressure: 25/15 mmHg in PA，40/10 mmHg in RV infundibulum，60/10 mmHg in RV. Post-procedure pressure gradient across PV: 15 mmHg. Post-procedure percutaneous oxygen saturation: 92%. Dopamine and alprostadil were administered. Post-procedure examinations lUCG：PFO 2mm，PDA 2.2mm，mild TI，inner diameter of RV 10mm，LVEDD 27mm, LVEDV 20ml, LVESV 3ml, LVEF 0.85 lX-ray：C/T ratio 0.75 with normal pulmonary markings. lDischarged 1 week after procedure. lThe infant was well-developed with no cyanosis. Readmission 1.5 yrs after Re-admitted on April 4th , 2007 . PE： Height: 93cm，weight: 14kg. No cyanosis and clubbed figure and toe. A continuous murmur over the L2. Femoral artery oxygen saturation: 93% Re-catheterization On April 18, 2007 Pre-closure pressure: RA: 15/7mmHg, RV: 58/5 mmHg, PA: 38/12mmHg, LV: 117/0mmHg, aortic pressure: 117/65 mmHg. Diameters of PDA: 12.0mm at aorta side and 3.0mm at PA side. The PDA was completely closed by using an 8/10 mm ductal occluder. Post-closure pressure: PA: 30/10 mmHg, RV: 40/5 mmHg. Post-closure examinations lUCG：inner diameter of RV 20mm, LVEDD 36mm, LVEDV 41ml, LVESV 11ml, LVEF 0.77 lX-ray：C/T ratio 0.70 lECG：sinus rhythm with RV hypertrophy. lDischarged 5 days after procedure. Devices for RF perforation Devices for RF perforation Schematic diagram of RF perforation Pre-perforation Pre-perforation Right Right ventriculogramventriculogram and and aortogramsaortograms RF perforation Balloon dilation Balloon dilation Right Right ventriculogramsventriculograms immediately and immediately and 1.5 yrs after perforation1.5 yrs after perforation AortogramsAortograms before and after PDA closure before and after PDA closure Oxygen Oxygen Sat: 96% Discussion Review PA-IVS accounts for 0.71%3.1% of congenital heart disease. 85 die within half a year after birth. 52 die within a year after surgery. Anatomy Morphology of atresic PV Type : three valves are thickened and fused with a joint ridge line in the middle of the valve Type : the valves is completely closed with a joint ridge line around the valve Anatomy Morphology of TV Type : TVs are poorly developed with a small tricuspid annulus Type : TVs are severe dysplastic Type : RV is well-developed Anatomy Morphology of RV Type : RV consists of three parts but the cavity is smaller than normal（53） Type: RV outflow tract is absent （19） Type : only inflow tract is left（28 ） Anatomy Right ventricle-dependent coronary circulation No connection is present between aorta and coronary artery and the proximal coronary artery is atresic or disrupted Coronary blood comes from the right ventricle (20). Haemodynamics Inter-atrial communication right-to-left shunt volume load of LV A small inter-atrial communication or premature closure of the communication will cause venous congestion and even death Haemodynamics Oxygen saturation in systemic and coronary arteries decreases due to right-to-left shunt through inter-atrial communication, RV- coronary artery fistulae and right ventricle- dependent coronary circulation, which causes ischemia, hypoxia and metabolic acidosis Haemodynamics A continuously opened ductus with left-to-right shunt is the only source of pulmonary blood flow. The size of ductus plays a key role in survival of infants. Sudden decrease of right ventricular systolic pressure will cause irreversible myocardial ischemia in patients with right ventricle-dependent coronary circulation . Membranous