Transcatheter double-blade valvotomy for the treatment of valvar pulmonary stenosis

Summary A transcatheter double-blade valvotome for cutting a stenotic pulmonary valve has been devised. The valvotome consists of a retractable rhomboid structure at its tip, with a blade on each side of its proximal half. By tugging the extended blades from the pulmonary artery to the right ventricle it is possible to tear the stenotic valve. After animal experiments proved the feasibility and safety of this method, it was used in three children with pulmonary stenosis. The results were encouraging. Editor's note. We have been informed by the authors that they developed the technique described here because commercially produced balloon catheters were not available to them.     

Anatomic features of congenital pulmonary valvar stenosis

Summary A total of 31 specimens of hearts with congenital pulmonary valvar stenosis were studied. To define the anatomy of the normal pulmonary valve, 210 specimens of hearts considered to have a normal pulmonary valve were also reviewed. On the basis of gross morphology of the valve leaflets and annulus, the stenotic valves were subgrouped into domed, unicommissural, bicuspid, tricuspid, hypoplastic annulus, and dysplastic. The valve leaflets in all subgroups were thickened. The thickness varied in degree, but involved the entire length of the leaflet. Microscopically, the thickness in most cases was due to an increase in myxomatous tissue. In a few cases, the elastic and collagen components of the leaflet were increased. The valve annulus was abnormal in most cases. The abnormalities included replacement of the fibrous backbone of the annulus by myxomatous tissue and partial or complete absence of the annulus. The impact on valvar anatomy by direct surgical valvotomy (14 patients) and closed Brock valvotomy (two patients) was reviewed. Precise knowledge of pulmonary valve anatomy is an aid to successful balloon pulmonary valvuloplasty.     

Left ventricular function and myocardial mass after aortic valvotomy in infancy

Summary Eighteen of 25 survivors of aortic valvotomy in infancy were reinvestigated by cross-sectional echocardiography a mean of 7.5 (2.3–13.4) years after surgery. They had been operated at a median age of 38 (5–330) days. At the follow-up examination the gradient across the aortic valve was 41±19 (15–85) mmHg and the ejection fraction was 0.73±0.10 (0.48–0.84). Left ventricular (LV) end-diastolic volume was 66±17 (33–191) ml/m². LV mass was 96±36 (44–204) g/m² and the LV mass volume index (LVMVI) (mass divided by end-diastolic volume) was 1.43±0.4 (0.9–2.28). Eleven of 18 patients had an abnormally high mass volume index compared with 95 age-matched controls with structurally normal hearts. The correlation between the residual pressure gradient across the aortic valve and mass volume index yielded anr value of 0.75 (p<0.0004). One patient had been reoperated and underwent resection of a subaortic stenosis 4 years after the initial operation. Four patients with a resting gradient of more than 50 mmHg and one with grade 4 aortic regurgitation are scheduled for further surgical treatment.We conclude that, although LV function was normal in most patients who underwent aortic valvotomy in infancy, LV mass remains elevated in a significant number of patients, who may remain at risk of developing subendocardial ischemia.     

瓣膜发育不良型肺动脉瓣狭窄经皮球囊扩张术治疗的探讨

为探讨瓣膜发育不的狭窄（简称ＰＳ）球囊扩张术（简称ＰＢＰＶ）效果,采用超大球囊法为３４例瓣膜发育不良型ＰＳ进行球囊扩张术。３４例中轻型２１例（平均年龄４．４０岁）,重型１３例（平均年龄５．１５岁）,最小年龄１１月。应用ＰＢＰＶ术球／瓣比值轻型１．３６～１．６４（平均１．１４）,重型１．２０～１．６４（平均１．５２）,术前后观察跨肺动脉瓣压差（△Ｐ）,出院后随访多普勒超专心动图以监测△Ｐ。结果：轻型     

21例合并气管狭窄肺动脉吊带患儿的治疗

目的：回顾性分析合并气管狭窄的肺动脉吊带患儿的治疗和预后,探讨单纯左肺动脉移植,避免气管成形术治疗策略的可行性。方法2009年4月至2015年11月,共有21例合并气管狭窄肺动脉吊带患儿在本单位接受手术治疗。其中6例患儿采用左肺动脉移植加气管干预手术策略。另外15例患儿采用左肺动脉移植、避免气管成型手术,术后采用早期拔管,无创CPAP过渡治疗策略。对所有患儿病例资料进行收集分析。结果21例合并气管狭窄患儿,男12例,女9例,年龄1个月~10岁,体重2.9~25.0 kg,术前均有中度至重度呼吸道症状,其中5例为重度,需要机械通气辅助呼吸。左肺动脉移植加气管干预手术患儿6例,存活出院1例,存活率16.7％;其中3例接受Slide气管成形术,2例气管吻合口肉芽组织增生,呼吸衰竭死亡;另外3例接受气管支架植入术,无存活。左肺动脉移植手术患儿共15例,采用经胸骨正中切口行左肺动脉移植术,存活出院13例,存活率86.7％;另外2例撤离呼吸机失败,体外膜肺辅助下行Slide气管成形术,术后气管吻合口愈合不良死亡。结论中、重度气管狭窄的肺动脉吊带患儿,采取左肺肺动脉移植,避免气管成形手术,术后早期拔除气管插管改无创CPAP过渡的治疗策略是可行的,预后也比较好,可以成为此类患儿的理想治疗策略。     

婴儿肺动脉瓣狭窄的介入治疗

目的　总结经皮球囊肺动脉瓣成形术（PBPV）治疗婴儿肺动脉瓣狭窄（PS）的经验,评价其必要性、安全性和有效性。方法　回顾性分析2009年1月至2018年12月青岛市妇女儿童医院心脏中心行PBPV治疗68例婴儿PS的临床资料。结果　患儿平均手术年龄为（6.34±3.6）个月,平均手术体重为（7.81±2.11） kg,术前体重/年龄Z值为-0.80±1.38,提示生长受限为22例（32.4%）,合并紫绀15例（22.1%）,呼吸急促23例（33.8%）。超声心动图评估右室长径/左室长径比值平均值为0.56±0.73,胸部正位片心胸比平均值为0.56±0.43,术前血氧饱和度平均值为94.74%±6.10%。术前右室/左室收缩压力比值平均值为0.68±0.26。球囊扩张后,肺动脉瓣平均压力梯度由（75.57±18.61） mmHg降至（22.37±5.21） mmHg,右心室收缩压平均值由（83.78±22.44） mmHg降至（54.16±20.81） mmHg。手术即刻成功率为100%。术中无严重心律失常、心腔穿孔、腱索断裂、血管损伤等并发症。术后早期患儿轻-中度肺动脉瓣反流比例为53.3%,无重度反流。随访3个月～10年间再狭窄率2.94%,32例随访5年以上患儿肺动脉瓣反流程度未进一步加重,无特殊处理。所有患者均未出现明显的右心室功能障碍。与接受PBPV治疗幼儿PS相比,婴儿PS临床症状明显,病情较重,术后早期及中远期随访手术效果满意。对本研究婴儿PS进行分组比较分析发现低体重、低龄儿可获得同样良好手术效果。结论　PBPV治疗婴儿肺动脉瓣狭窄十分必要,技术操作安全可行,随访结果持续有效。     

Aberrant left pulmonary artery with tracheal stenosis without vascular sling

Summary We report a case of abnormal origin of the left pulmoanry artery from the right pulmonary artery, associated with tracheal stenosis but with no vascular sling. This is the first such case, to our knowledge, without vascular sling.     

Doppler estimation of pressure gradient in pulmonary stenosis: Maximal instantaneous vs peak-to-peak,vs mean catheter gradient

Summary We undertook a study to identify the hemodynamic significance of a Doppler-derived gradient across a stenotic pulmonary valve. Furthermore, we attempted to define the optimal plane for velocity data acquisition. A total of 17 children with valvar pulmonary stenosis were evaluated using Doppler echocardiography. Flow-velocity profiles were obtained from both the parasternal and subxiphoid windows. Ten of 17 patients were studied before and after balloon valvotomy. Therefore, 27 different transvalvar gradients were assessed by Doppler and these data were compared with the catheter-derived maximal instantaneous, peak-to-peak, and mean pressure gradients. The maximal Doppler gradient correlated well with the catheter-derived peak-to-peak pressure gradient (r=0.95) and catheter maximal instantaneous pressure gradient (r=0.95). Although these correlation coefficients were similar, the Doppler maximal gradient consistently overestimated the peak-to-peak catheter gradient by as much as 25%–40%. Such an overestimation was not observed when we compared the maximal Doppler gradient with the catheter-derived maximal instantaneous gradient. Moreover, the regression line of the latter comparison closely approximated the line of identity. The correlation coefficient between Doppler mean and catheter mean gradients was only 0.91. Doppler velocities were best derived when multiple transducer positions were employed to interrogate pulmonary artery velocity.     

Pulsed wave and color doppler findings in congenital pulmonary vein stenosis

Summary A premature infant presented at 8 weeks of age with respiratory failure and pulmonary hypertension. Two-dimensional echocardiography was not diagnostic but color flow imaging and pulsed Doppler examination revealed turbulent and continuous pulmonary venous flow. Differing patterns of disturbed flow suggested varying severity of obstruction in individual pulmonary veins. The Doppler findings are compared to the angiographic and autopsy data.     

Congenital valvar aortic stenosis and abnormal origin of the right coronary artery: Rare combination with important clinical implications

Congenital valvar aortic stenosis in association with an abnormal coronary artery origin and distribution has not been previously reported. The right coronary artery arose in the left ventricular (LV) outflow tract and supplied much of the LV myocardium. Aortic valvotomy with standard antegrade cardioplegia resulted in infarction of the left ventricle. Aggressive attempts to ascertain the origin of the coronary arteries in right dominant coronary systems must be performed before any intervention in this group of patients.     

儿童肺动脉瓣狭窄的诊治进展

肺动脉瓣狭窄是一种常见的先天性心脏病,其早期诊断与治疗对改善儿童生长发育及预后有重要意义.随着诊断技术及治疗方法的发展,经皮球囊肺动脉瓣成形术已取代手术,成为治疗单纯肺动脉瓣狭窄的首选.该文就近年关于肺动脉瓣狭窄的诊断与治疗进展作一综述.     

Hypoplastic left heart syndrome with dysplastic pulmonary valve with stenosis

Summary A dysplastic pulmonic valve in hypoplasia of aortic tract complex has not been dealt with in the literature. The recent attempted surgical correction for hypoplasia of aortic tract complex has given some hope of survival in this entity. The integrity of the tricuspid and pulmonic valves is important while considering total surgical correction. We describe three hearts of hypoplasia of aortic tract complex with dysplastic pulmonic valve. The echocardiographic recognition and the significance of the dysplastic nature of the pulmonary valve in the light of surgery are emphasized. Aided by grant HL 30558-02 from the National Heart Lung and Blood Institute of the National Institutes of Health, Bethesda, Maryland.     

Outcome of pulmonary and aortic stenosis in Williams-Beuren syndrome in an Asian cohort

AIMS: To define the cardiovascular anomalies and the long-term outcomes in an Asian cohort with Williams-Beuren syndrome (WBS). METHODS: Data were retrieved from a retrospective chart review of patients who had a definitive diagnosis of WBS by fluorescence in situ hybridization between 1995 and 2005. All patients underwent echocardiography every 3-9 months. Ten patients underwent cardiac catheterization. RESULTS: Twenty-one patients with a total follow-up of 134 patient-years (median: 72 months) were enrolled. Characteristic dysmorphic facial features were noted in 19 patients (n = 19, 90%). All except one had associated cardiac anomalies, accounting for 0.3% (20/6640) of the patients with congenital heart disease. The spectrum of cardiac anomalies included supravalvular aortic stenosis (SVAS) (n = 15, 71%), peripheral pulmonary stenosis (PPS) (n = 12, 57%), pulmonary valve stenosis (PS) (n = 10, 47%), mitral valve prolapse (MVP) (n = 9, 43%), coarcation of the aorta (n = 4, 19%), ventricular septal defect (n = 2, 10%) and atrial septal defect (n = 1, 5%). Concurrent SVAS and PS/PPS were found in 14 (70%) patients. Only one patient required balloon dilation of PS, which improved. Regression of the stenoses occurred with a probability of 31, 90 and 71% at the age of 10 years for SAVS, PS and PPS, respectively. CONCLUSIONS: Among our WBS patients, SVAS, PPS and PS were common, and were associated with probability of spontaneous regression, especially of right-sided lesions.     

Echocardiographic findings of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum in utero

BACKGROUND: Neonates with right ventricular outflow obstruction and intact ventricular septum show serious hemodynamic problems, such as severe hypoxemia, congestive heart failure due to massive tricuspid regurgitation, respiratory distress related to huge pulmonary hypoplasia or ventricular dysfunction due to right ventricle-coronary communication. Recent advances in fetal diagnosis include many cases of in utero diagnosis of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum. METHOD: Among the fetuses examined from April 1994 to March 1998, five fetuses were found with pulmonary atresia (PA) or critical pulmonary stenosis (CPS). Fetal echocardiograms were reviewed to elucidate the accuracy of fetal information and the efficacy of fetal diagnosis in the perinatal management of patient with CPS or PA and intact ventricular septum. RESULTS: The five cases were divided into two groups: two with a very small right ventricle (group 1) and three with a tripartite right ventricle (group 2). Fetal cardiomegaly and right atrial dilatation were prominent in group 2, whereas cardiac sizes were normal in group 1. Serial fetal examination in one group 2 fetus revealed developing right ventricular hypertrophy in utero. All group 2 cases showed massive tricuspid regurgitation (TR). Estimated right ventricular pressures from TR always exceeded the systemic blood pressures of gestational age-matched neonates. Reversed flow through the ductus arteriosus was recorded in both groups and ductus-dependent pulmonary circulation after birth was anticipated. Patency of both tricuspid and pulmonary valves was difficult to recognize in utero, as was right ventricle-coronary artery communication. Four of the five cases were maternally transported and survived palliative and/or definitive intervention. One fetus with chromosomal abnormality was observed without intervention during infancy and received palliative surgery when she was two years old. CONCLUSION: Fetal hemodynamic information was useful for making decisions not only after birth, but also in utero, and may eventually result in improving the prognosis of babies with PA/CPS. Serial observation of the fetuses with PA/CPS may also suggest the possible pathogenesis of PA/CPS in utero.     

经皮支架植入治疗心脏术后肺动脉分支狭窄1例

目的 运用经皮支架植入术治疗复杂先天性心脏病二期外科矫治术后残留的双侧肺动脉分支狭窄。方法 5岁女孩因患有肺动脉闭锁合并室间隔缺损分别在3岁和4岁时接受外科Blalock–Taussig分流术以及心内畸形矫治术。术后双侧肺动脉残留显著狭窄,通过经皮支架植入术在患儿右肺动脉和左肺动脉内植入直径分别为10mm与7mm的Genesis 支架。 结果 支架植入术后患儿临床以及血液动力学状况改善显著。右肺动脉和左肺动脉狭窄处内径分别由4.90mm和2.89mm增加至8.55mm和7.35mm,同时狭窄两端收缩期压力阶差分别由19mmHg和32mmHg下降至0mmHg。结论 通过经皮支架植入术,置入易于输送和扩张的Genesis 支架,可有效而安全地治疗小年龄儿童的肺动脉分支狭窄。     

儿童肺动脉瓣狭窄介入治疗术并发症的防治

经皮球囊肺动脉瓣成形术已取代传统手术,成为肺动脉瓣狭窄首选治疗方法.虽然其临床疗效与安全性已得到公认,但仍存在不同程度的并发症,影响预后,甚至致死.该文就经皮球囊肺动脉瓣成形术术中与术后相关并发症的原因及防治措施进行综述.     

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目的探讨经皮球囊肺动脉瓣成形(PBPV)术治疗婴儿和新生儿肺动脉瓣狭窄(PS)及室间隔完整型肺动脉瓣闭锁(PA/IVS)的安全性及有效性。方法2006年1月至2009年4月,广东省心血管病研究所心儿科收治PS及PA/IVS婴儿和新生儿63例。其中危重新生儿20例(31.7%)。术前经超声心动图确诊为PS56例,PA/IVS7例。危重新生儿PS需先用直径2.5～4.0mm小球囊预扩张肺动脉瓣,PA/IVS患儿需先行射频瓣膜打孔术。PBPV术所选球囊直径为肺动脉瓣环径(PVAD)1.2～1.3倍(新生儿所选球囊直径为PVAD1.0～1.2倍)。结果全组63例(100%)PBPV术成功。球囊径/瓣环径:1.2±0.1。右室压力明显下降[术前(104.3±32.7)mmHg(1mmHg=0.133kPa),术后(52.0±10.5)mmHg,P<0.001],跨肺动脉瓣压力阶差明显下降[术前(99.2±23.5)mmHg,术后(27.7±12.4)mmHg,P<0.001]。平均手术时间(88.1±36.2)min,平均X线曝光时间(16.9±11.1)min。术中6例(9.5%)出现并发症,2例心包积液,2例低氧血...     

肺动脉瓣狭窄合并房间隔缺损的介入治疗效果分析

目的 探讨儿童先天性肺动脉瓣狭窄合并房间隔缺损的介入治疗方法和临床随访效果.方法 27例肺动脉瓣狭窄合并房间隔缺损的患儿,同时进行肺动脉瓣成形术和房间隔缺损封堵术,术后即刻、1周、3个月、6个月行超声心动图检查,随访其临床效果.结果 27例经皮球囊肺动脉瓣成形术均获得成功,术后即刻测量跨肺动脉瓣压力阶差由术前的(79±62)mmHg降至(49±36)mmHg,术后超声心动图随访过程中跨肺动脉瓣压力阶差持续下降.超声心动图测量房间隔缺损直径为3.4～24.6(11.7±6.9)mm,选择封堵器的直径为8～32(14.6±8.7)mm,27例中有24例成功进行封堵.结论 介入治疗肺动脉瓣狭窄并房间隔缺损是一种安全可靠的方法,严格掌握适应让,特别是房间隔缺损的适应证,可取得良好的临床效果.     

Physiological pulmonary branch stenosis in newborns: 2D-echocardiographic and Doppler characteristics and follow up

Transient systolic murmurs in neonates and premature infants due to mild left (LPA) and right (RPA) pulmonary branch stenosis is recognized but follow up studies are lacking. We studied echocardiographically 21 neonates with murmur and 10 controls. Diameters of the main pulmonary artery (MPA), LPA and RPA were smaller in patients with murmur. Colour-coded Doppler showed turbulent flow in LPA and RPA in 20/21 (95%) patients and flow velocities of both pulmonary branches were significantly higher than in controls. The follow up study at 3 months in 14/21 (67%) patients showed absent or decreased murmur in 9 (64%). Echographically, absolute and relative diameters of LPA and RPA increased whereas the ratio of MPA/aorta did not change suggesting accelerated growth or dilatation of the pulmonary branches. Flow velocities decreased significantly in the branches. Thus, transient systolic murmurs in neonates are associated with temporary relative hypoplasia of the pulmonary branches which showed increased growth leading to disappearance of the murmur in most cases within 3 months of life.     

Severe peripheral pulmonary artery stenosis is not a contraindication to liver transplantation in Alagille syndrome

We described a case of Alagille syndrome with severe peripheral pulmonary artery stenosis and very high right ventricular pressure that underwent successful living-related liver transplantation without any peri-operative and mid-term postoperative complication because of this cardiac malformation. The aim of this report is to point out that the severe pulmonary artery stenosis may be a risk factor but not a contraindication to liver transplantation in patients with Alagille syndrome.