计算流体力学模拟在功能性单心室患儿虚拟Fontan手术中的应用

目的 对双侧、双向Glenn术后患儿进行虚拟Fontan手术,并对不同设计方案的血管吻合区域内血流进行计算流体力学(computational fluid dynamics,CFD)模拟.方法 利用Mimics 12.0(R) 进行三维解剖重建,通过虚拟手术改变双侧上腔静脉与肺动脉的吻合位置,并将下腔静脉连接到肺动脉的不同位置.建立数值模型,对4种虚拟手术方案在不同左、右肺动脉流量分配比(30:70、40:60、50:50、60:40、70:30)情况下的血流进行CFD模拟.结果 当左、右上腔静脉与肺动脉吻合口之间距离较大且下腔静脉与肺动脉吻合口位于中间时,在各种左、右肺动脉流量分配比情况下能量消耗均较其他3种设计方案低,且在左、右肺动脉流量分配比为50:50时能量消耗达到最低值23.60 mW.结论 对患有左侧上腔静脉残存的患儿施行Fontan手术时,将左、右上腔静脉分别吻合于同侧肺动脉并将下腔静脉与肺动脉吻合口置于左、右上腔静脉与肺动脉吻合口中间的设计方案能量消耗最低.     

心内隧道全腔静脉肺动脉连接术治疗复杂性先天性心脏病

目的总结心内隧道全腔静脉肺动脉连接术治疗小儿复杂先天性心脏病的效果。方法复杂性先天性心脏病患儿11例,均在全身中度低温、局部心脏低温、体外循环心脏停跳下手术;先充分游离上下腔静脉,以利插管建立体外循环,充分游离主肺动脉、左肺动脉及右肺动脉直达肺门,以利切断后吻合时减少吻合口张力。上腔静脉入右房处上方1 cm处横断,远心端与右肺动脉上缘端侧吻合,Gore-Tex人工血管壁的2/3与右房壁连接上下腔静脉口形成心内隧道,上腔静脉近心端与主肺动脉吻合;如果吻合口前壁有张力,采用自体心包片延长,存在左上腔静脉者与左肺动脉侧侧吻合。结果全组患儿无手术死亡,4例术后出现胸腔积液,经反复穿刺抽液及胸腔闭式引流治愈,末梢血氧饱和度96%～99%,术后心功能Ⅰ、Ⅱ级,无心律失常发生。结论心内隧道全腔静脉肺动脉连接术是治疗小儿复杂先天性心脏病切实可行的方法,优于双向Glenn手术,可避免患儿二次手术。     

单侧肺动脉起源于升主动脉的外科治疗

目的 探讨单侧肺动脉起源于升主动脉(AOPA)的外科治疗方法.方法 2006年5月-2008年8月共8例AOPA患儿接受外科治疗.男女各4例;年龄1～96个月;体质量4～24 kg.7例为右肺动脉起源于主动脉,同时并动脉导管未闭、肺动脉高压,其中4例并卵圆孔未闭.1例左肺动脉起源于主动脉,同时并法洛四联症型肺动脉闭锁.病例术前均经心血管增强CT确诊.其中7例右肺动脉起源于主动脉的病例,在体外循环心脏停跳下,游离并切断右肺动脉,将切断的右肺动脉直接吻合于主肺动脉.1例左肺动脉起源异常,由于并法洛四联症型肺动脉闭锁,肺动脉发育较差,因此游离左肺动脉后采用7 mm Goretex管道行中央分流.结果 本组患儿均成活,术后恢复顺利,手术效果良好.术后1个月超声复查右肺动脉与主肺动脉吻合口流速(1.71±0.24)m/s,压差(1.60±0.45)kPa,均未见明显狭窄.结论 AOPA肺动脉高压产生快,应早期诊断,早期手术治疗,近期手术效果良好.但仍需远期观察,以评估手术效果.     

先天性心脏病肺动脉高压手术前后右心功能变化和治疗

目的 了解先天性心脏病左向右分流导致的重度肺动脉高压对右心功能的影响以及手术矫治后右心功能的恢复状态。方法 对室间隔缺损(室缺)合并重度肺动脉高压(全肺循环阻力增加)的40例患儿，用心导管的方法进行术前、术后5～7年右心功能、肺循环的血流动力学随访测定。结果 术前右心心搏指数、作功指数、心排指数显著高于术后；术前右房压、右室收缩压及舒张压、肺动脉压力和阻力均增高，缺损修补后右室舒张压恢复正常，收缩压的降低与肺动脉压力下降有关；大型室缺左向右分流重度肺动脉高压右心功能不全系继发性右室高排出量心力衰竭，且伴有舒张功能障碍。结论 治疗心力衰竭不宜首选正性心肌收缩药物；降低肺动脉压力、减少左向右分流、根治心内畸形是合理的选择；术后右室收缩压持续不能恢复至正常水平，提示继发性肺血管梗阻性病变存在。     

功能性单心室的外科治疗及近期疗效

目的 总结功能性单心室的外科治疗经验.方法 回顾性分析我科2003年12月至2010年7月59例功能性单心室患儿,对其手术时机,手术方式及手术效果进行分析,并用Logistic回归分析Glenn手术的高危因素.结果 死亡8例(病死率13.56%),其中4例为Glenn术后死于低心排和多器官功能衰竭,2例为Banding术后死于低心排,1例为TCPC术后死于多器官功能衰竭,1例为B-T分流术后死于低心排.Glenn术后的主要并发症为上腔静脉梗阻综合症和低心排,Fontan术后的主要并发症是房性心律失常和胸腔积液.Logistic回归分析得出房室瓣反流是Glenn术后患儿死亡的高危因素.结论 功能性单心室心内畸形复杂,需根据肺血流的情况及早进行姑息手术,选择合适的术式.改良Fontan是进行生理矫正的理想术式,保留肺动脉前向血流的Glenn手术是保证患儿顺利进行Fontan术的有效方法,但需根据肺动脉狭窄的程度进行处理,避免上腔静脉综合症的发生.

Abstract：

Objective To retrospectively analyze the outcome of surgical treatment of patients with functional single ventricle. Methods Betveen December 2003 and July 2010, 59 patients with functional single ventricle underwent corrective surgeries, and were followed up at this center. Their median age was 3. 0 months old at pulmonary artery band surgery (PAB), 13. 5 months at bidirectional Glenn procedure (BDG), and 11 years old at Fontan operation. The variables of the anatomical lesions with single ventricle physiology, combined abnormalities, age at surgery, and type of the surgical procedure were retrospectively analyzed. Logistic regression analysis was also performed to determine the risk factors associated with Glenn. Results Duration of mechanical ventilation was 30. 4 ± 19. 7 hours. Length of stay in ICU was 4. 3 ± 1.6 days. Length of hospital stay was 34. 8 ± 15. 9 d.SpC2 increased from 75 ± 6. 97% to 88 ± 6. 28% after operation. Exercise tolerance was also improved remarkably. Eight deaths were noted after surgery (13. 56%). The leading complications after Glenn surgery were superior vena cava syndrome and low cardiac output syndrome. Complications after Fontan surgery were atrial tachycardia and pleural effusions. Logistic regression analysis suggested atrioventricular valve regurgitation was an important risk factor for the death of the patients had Glenn surgery. Conclusions The clinical outcome of surgical treatment of patients with functional single ventricle is satisfied. The surgical strategy should be made according to anatomic abnormality of the functional single ventricle and pulmonary circulation. Modified Fontan operations can become an alternative therapy for patients who are not suitable for double ventricle correction. Glenn with antegrade pulmonary blood flow may be a temporary palliation prior to Fontan surgery, but the pulmonary blood flow should be controlled to avoid the occurrence of superior vena cava syndrome.     

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。     

儿童右肺动脉异常起源于主动脉的诊断与外科治疗

目的 评价儿童右肺动脉异常起源于主动脉(aortic origin of the right pulmonary artery,AORPA)的治疗方法及效果,并探讨AORPA的诊断与鉴别诊断方法.方法 回顾性分析1993年5月至2007年7月11例儿童AORPA术前诊断及手术治疗的围术期疗效及中长期随访结果.分别采用右肺动脉经人工血管(2例)或同种主动脉(2例)与主肺动脉吻合术、体外循环下右肺动脉与主肺动脉直接吻合术(3例)、体外循环下(主动脉环及肺动脉环片)双片法右肺动脉与主肺动脉吻合术(2例)、非体外循环下右肺动脉经人工血管与主肺动脉吻合术(2例).结果 11例中10例术前确诊,1例术前漏诊而在术中发现.5例通过右室造影及主动脉造影或多排CT/MRI检查确诊,6例单纯超声心动图检查患儿中确诊5例,1例漏诊.11例手术患儿围术期死亡2例.术后随访2.2～13年,1例于术后4年死于右心功能衰竭.生存8例患儿术后超声心动图复查射血分数(EF)值43%～52%,心功能I级5例,Ⅱ级3例.结论 右室造影及主动脉造影或多排CT/MRI检查可减少漏诊率.手术是治疗AORPA的有效方法,但应尽早手术以进一步提高手术疗效.应用自体组织加宽加长右肺动脉可减少吻合口狭窄的可能性.     

左室收缩功能正常的左冠状动脉异常起源于肺动脉患儿的超声诊断13例病例系列报告

目的:探讨左室收缩功能正常的左冠状动脉异常起源于肺动脉(ALCAPA)患儿的超声心动图特点。方法:回顾性分析2010年1月至2019年12月广州市妇女儿童医疗中心收治并经手术证实的13例左心室射血分数（LVEF）≥55%的ALCAPA患儿的超声心动图特征和临床资料。结果:13例ALCAPA患儿均可见右冠状动脉增宽，其他超声征象包括左冠状动脉血流逆灌12例、左冠状动脉直接开口于肺动脉9例、肺动脉内逆灌血流7例、心肌侧枝血流8例、左室明显增大8例、二尖瓣功能不全7例、左室心内膜纤维化5例。<1岁ALCAPA患儿的心肌侧枝血流信号不明显，左心室大小正常或轻度增大且二尖瓣功能良好；≥1岁患儿可见丰富侧枝血管、左室明显扩大以及二尖瓣功能不全。4例误诊的ALCAPA左冠状动脉均起源于肺动脉右后方。结论:超声心动图可准确诊断左室收缩功能正常的ALCAPA，如果超声科医生在怀疑该诊断时能全面探查冠状动脉的起源和血流方向，可减少漏诊和误诊。     

心血管系统疾病

05 1 62 8　先天性心脏病肺动脉高压手术前后右心功能变化和治疗 /朱卫华…∥临床儿科杂志 .-2 0 0 4,2 2 (3 ) .-1 43～ 1 46方法 :对室间隔缺损 (室缺 )合并重度肺动脉高压 (全肺循环阻力增加 )的 40例患儿 ,用心导管的方法进行术前、术后 5～ 7年右心功能、肺循环的血流动力学随访测定。结果 :术前右心心搏指数、作功指数、心排指数显著高于术后 ;术前右房压、右室收缩压及舒张压、肺动脉压力和阻力均增高 ,缺损修补后右室舒张压恢复正常 ,收缩压的降低与肺动脉压力下降有关 ;大型室缺左向右分流重度肺动脉高压右心功能不全系继发性右室…     

70例小儿双向腔静脉-肺动脉分流手术后的临床处理

目的探讨双向腔静脉-肺动脉分流手术后的临床处理方法。方法1998年4月至2004年9月共有70例患儿接受双向腔静脉-肺动脉分流手术。结果术后早期存活66例,死亡4例。后期1例术后3个月出现吻合口阻塞,术后6个月死亡。术后并发症有:胸内出血、呼吸衰竭、急性肺水肿、右侧胸腔积液、乳糜胸、肺萎陷、气胸、败血症、脑部损害症状等。机械辅助呼吸时间(24.04±18.13)h,术后住院天数(11.26±5.10)d。术前上腔静脉压(8.33±1.92)mmHg,术后(19.64±3.47)mmHg(P<0.01);术前下腔静脉压(8.21±2.07)mmHg,术后(9.81±2.52)mmHg(P<0.01)。术前平均经皮血氧饱和度(79.70±5.76)%,术后(90.04±4.04)%(P<0.01)。术后随访1个月到5年。其中5例完成了Ⅱ期全腔静脉肺动脉连接术,1例完成了Ⅱ期法乐四联症根治术,3例因解剖原因,难以行Ⅱ期生理性矫治术外,其余存活病例仍在随访观察,等待Ⅱ期手术。结论双向Glenn手术对于功能性单心室的患儿来说是一种安全、有效的手术方式。根据手术的方式、术后血流动力学、手术前后病理生理的改变等特点,对患儿进行监护和处理,才能减少术后并发症的发生率,提高术后生存率。     

Comparison of Bilateral Pulmonary Arterial Level and Diameter in Transposition of the Great Arteries

In normal anatomy, the left pulmonary artery (LPA) is usually situated higher than the right pulmonary artery (RPA); however, transposition of the great arteries (TGA), the LPA is not always situated higher than the RPA. This study was performed to clarify the relative position of the RPA and the LPA in transposition of the great arteries (TGA) as well as the implications. We reviewed 101 angiograms of patients with TGA (age 4.1 ± 1.2 months). The width of the RPA, the LPA, and the pulmonary trunk (PT) were measured just before their first branch in the frontal view. They were classified into four groups according to the ratio between the RPA and the PT (RPA/PT). The initial courses of the LPA and the RPA were compared and defined according to their height in the frontal view, and the preferential flow (or not) to the RPA was recorded. The equation of hydrodynamics was applied to evaluate the bifurcation angle. Both PAs were the same size in all cases. Forty-eight patients (47.5 %) had a RPA/PT diameter ratio < 0.49. The LPA coursed higher than the RPA in the majority of cases (81 [80.2 %]); in a minority of cases the LPA and RPA were at the same level (6 [5.9 %]); and in some cases the RPA coursed higher than the LPA (14 [13.9 %]). Patients with a high degree of PA hypoplasia tended to have both PAs at the same level or a higher-positioned RPA. Autopsy (1 of 3 cases) showed a posterior ridge against the bronchus in the higher RPA. Hydrodynamic calculation showed that the greater the angle between the RPA/PT, the greater the preferential flow. Preferential flow to the RPA in TGA did not necessarily result in LPA hypoplasia before its first branch. Higher RPA position relative to the LPA was associated with greater flow in it against the posterior bronchus. This situation was more prevalent in patients with severe PA hypoplasia.     

Modified Fontan operation for single ventricle with common atrium and abnormal systemic venous drainage: Usefulness of an additional superior vena cava to pulmonary artery anastomosis

Summary In two patients (aged 15 and nine years old) with single ventricle, common atrioventricular valve, common atrium, and bilateral abnormally draining superior vena cava (SVC), a modified Fontan procedure was performed together with a SVC to pulmonary artery (PA) anastomosis. This avoided complex intraatrial septation. A bilateral anastomosis was carried out in one patient and a unilateral one in the other, the PA not being divided in either patient. This procedure provided simple intraatrial septation while avoiding possible obstruction of either the pulmonary or systemic blood pathways if complete intraatrial septation had been carried out.     

CT findings in unilateral hepatopulmonary syndrome after the Fontan operation

Background  Patients with complex congenital heart defects palliated by connecting the systemic veins directly to the pulmonary circulation are known to develop hepatopulmonary syndrome (HPS). Although rare, HPS can develop following the Fontan operation. Objective  To present and analyse the CT findings of HPS in patients with a Fontan circulation. Materials and methods  From May to December 2005, six patients with HPS following the completion of a Fontan circulation were evaluated. CT findings were reviewed and were compared with angiographic findings. Results  All six patients showed unilateral involvement. All patients except one had inferior vena cava (IVC) interruption with azygos continuation. CT scans showed abnormal vascular dilatation in one lung, and properly demonstrated the anatomy causing the hepatic venous blood to flow preferentially into one lung. These CT findings correlated well with the angiography findings. Conclusion  HPS that develops after the Fontan procedure is typically unilateral and is often associated with IVC interruption and azygos or hemiazygos continuation. CT demonstrates dilatation of pulmonary vessels in the affected lung and may be able to demonstrate the underlying anatomical cause for the predilection of hepatic venous flow to the contralateral lung.     

Effects of Pulmonary Regurgitation on Distensibility and Flow of the Branch Pulmonary Arteries in Tetralogy of Fallot

Significant pulmonary regurgitation (PR) after repair of tetralogy of Fallot (TOF) may affect flow in the pulmonary artery (PA) side branches. We sought to assess flow changes and distensibility of the PA side branches in vivo and test correlation with the degree of PR and right-ventricular (RV) dilatation. Thirty patients after TOF repair and 16 controls underwent cardiovascular magnetic resonance for quantification of RV volumes and measurement of flow in the PA side branches. RV volumes and function, blood flow volumes, and cross-sectional area of the main, left (LPA), and right (RPA) PA were measured and regurgitant volumes and distensibility calculated. Results were compared between the LPA and the RPA and between patients and controls. Median regurgitation fraction of PR was 41 % (range 22–60 %). Regurgitant fraction was greater in the LPA (40 %) than in the RPA (29 %), resulting in lower net flow into the LPA (p < 0.001). LPA area was significantly greater than that of the RPA (303.9 vs. 232.7 mm²/m²) (p < 0.0001). The LPA showed lower distensibility than the RPA (39 vs. 44 %). PA side branch distensibility correlated with MPA regurgitant volume (p = 0.001), MPA regurgitant fraction (p = 0.001), and RV end-diastolic volume (p = 0.03). PA side branches have greater distensibility in patients with PR than in normal subjects. Significant PR leads to changes in flow profile and distensibility of the PA side branches. The LPA shows greater regurgitant volume and greater area but lower distensibility than the RPA.     

Upper Extremity Peripheral Venous Pressure Measurements Accurately Reflect Pulmonary Artery Pressures in Patients with Cavopulmonary or Fontan Connections

After the bidirectional cavopulmonary or Fontan operation, the physiologic consequence is passive flow of the systemic venous return to the pulmonary arteries. Knowledge of pulmonary artery pressure (PA) is valuable in the management of these patients, and obtaining this information without the need for a central line or cardiac catheterization would be advantageous. The aim of this study was to evaluate the correlation between upper extremity peripheral venous (PV) pressures and PA or superior vena cava (SVC) pressures in patients who have undergone cavopulmonary or Fontan connections. During cardiac catheterization, 19 patients with complex cyanotic heart disease who had undergone a cavopulmonary shunt or Fontan procedure were studied. Simultaneous pressure measurements were obtained from the peripheral intravenous line placed prior to the procedure and the SVC or PA. The mean pressures were compared. The mean PV pressure was 17.5 ± 5.6 mmHg. The mean SVC or PA pressure was 16.1 ± 5.4 mmHg. The mean difference was 1.5 ± 1.5 mmHg (p < 0.001). The correlation coefficient PV to SVC or PA pressure was 0.97 (p < 0.001). PV pressure measurements taken from an upper extremity accurately reflect PA pressures in patients who have undergone a cavopulmonary shunt or Fontan procedure.     

Reversal of protein-losing enteropathy in a child with Fontan circulation is correlated with central venous pressure after heart transplantation

We report on the reversal of protein-losing enteropathy (PLE) after heart transplantation (HTx) in a 10-yr-old boy with Fontan circulation, previously treated unsuccessfully with heparin for several months. The protein loss continued immediately after the Tx. During the following month, however, a gradual decrease in protein loss was observed, which correlated with a decrease in the inferior vena cava (IVC) pressure. The patient is doing well with a normal serum albumin level and a normal IVC pressure, 2 yr after Tx.     

Non‐invasive assessment of liver fibrosis in patients after the Fontan operation

Background: This study analyzed the change in liver fibrosis markers after the Fontan operation and investigated their clinical usefulness as an index of congestive liver fibrosis. Methods: The study enrolled 24 patients who underwent the Fontan operation between January 1994 and December 2008. We subdivided the postoperative period into four intervals and then compared the hepatological markers during each. Eighteen patients underwent postoperative cardiac catheterization and the correlation between hepatological markers and the inferior vena cava (IVC) pressure was analyzed. Results: The mean age of the patients was 138.6 months and the mean interval between the Fontan operation and the examination was 97.8 months. Type IV collagen was extremely high in every interval (I, 286 ± 93; II, 265 ± 93; III, 305 ± 143; IV, 206 ± 70), while none of the laboratory variables changed significantly in each interval. A significant positive correlation was observed between type IV collagen and the IVC pressure, but no significant correlation with any other hepatological marker was detected. Conclusions: No specific parameter that reflects the progress in liver fibrosis was identified in this study. The possibility exists that type IV collagen reflects the degree of hepatic congestion.     

Deep venous thrombosis in a child associated with an abnormal inferior vena cava

Congenital anomalies of the inferior vena cava (IVC), such as absence or atresia, although well documented, are uncommon and result from aberrant development during embryogenesis. Absence or atresia of the IVC is usually discovered accidentally. Patients are typically asymptomatic of the condition itself. Many concurrent cardiovascular-associated abnormalities have been described. We report a 10-y-old boy admitted to the emergency room with painful swelling of his right lower limb without previous trauma or surgery. After 3 d, swelling also involved the left lower limb. A Doppler ultrasound of the lower limbs revealed bilateral thrombosis of the vena iliaca communis, vena iliaca externa, femoral vein communis and superficial extending to the IVC. Magnetic resonance imaging (MRI) of the abdomen was performed. On MRI, we demonstrated a hypoplastic IVC. The results of blood coagulation studies, including levels of antiphospholipid antibodies, proteins C and S, and antithrombin III, were normal. The patient was treated with intravenous heparin for 8 d and discharged with oral warfarin therapy, which has been recommended for life.
Conclusion: Therapy against deep venous thrombosis must be focused on its prevention in the future. An abnormal inferior vena cava should be considered in young patients with deep venous thrombosis without apparent cause.