Evaluation of pulmonary blood supply by multiplanar cine magnetic resonance imaging in patients with pulmonary atresia and severe pulmonary stenosis

The purpose of this study was to assess the capability of multiplanar cine magnetic resonance imaging (MRI) for evaluating pre- and post-operative pulmonary circulation in patients with pulmonary atresia and severe pulmonary stenosis. Seventy-three multiplanar cine MRIs were performed in 30 patients, aged 1 month to 7 years (mean age, 27 months). The morphology and size of the central pulmonary arteries (PA), source of the major aortopulmonary collateral arteries (MAPCA), patency of Blalock–Taussig (BT) shunt vessels, and the post-operative pulmonary circulation were assessed. The accuracy of cine MRI was compared with that of angiography in all patients. The PA was visualized to the first hilar branch in 21 patients, but not in 8 patients in whom the central PA was absent. On follow-up MRI, PA growth was measured, and the results showed excellent correlation with the results obtained by angiography. In 17 patients who had undergone 23 BT shunt operations, cine MRI correctly demonstrated all patient shunts and 5 of 6 stenotic lesions. Multiplanar cine MRI provided excellent detail of the peripheral PA in all patients, 7 of 8 peripheral pulmonary stenoses, 3 of 4 nonconfluent pulmonary arteries, and 2 of 3 PA obstructions. Although the sources of MAPCA were identified in 7 of 9 patients, the distal connection of the MAPCA was not detected in all patients. Seven patients were reexamined after pulmonary plasty; they exhibited normal pulmonary flow patterns. Multiplanar cine MRI provides high-resolution imaging of PA with dynamic visualization of flow and is an effective noninvasive technique for evaluating pre- and post-operative patients with pulmonary atresia and severe pulmonary stenosis.     

Right ventricular hypertrophy and diastolic dysfunction in arterial switch patients without pulmonary artery stenosis

Objective

To assess pulmonary flow dynamics and right ventricular (RV) function in patients without significant anatomical narrowing of the pulmonary arteries late after the arterial switch operation (ASO) by using magnetic resonance imaging (MRI).

Methods

17 patients (mean (SD), 16.5 (3.6) years after ASO) and 17 matched healthy subjects were included. MRI was used to assess flow across the pulmonary trunk, RV systolic and diastolic function, and RV mass.

Results

Increased peak flow velocity (>1.5 m/s) was found across the pulmonary trunk in 14 of 17 patients. Increased RV mass was found in ASO patients: 14.9 (3.4) vs 10.0 (2.6) g/m² in normal subjects (p<0.01). Delayed RV relaxation was found after ASO: mean tricuspid valve E/A peak flow velocity ratio = 1.60 (0.96) vs 1.92 (0.61) in normal subjects (p = 0.03), and E‐deceleration gradients = −1.69 (0.73) vs −2.66 (0.96) (p<0.01). After ASO, RV mass correlated with pulmonary trunk peak flow velocity (r = 0.49, p<0.01) and tricuspid valve E‐deceleration gradients (r = 0.35, p = 0.04). RV systolic function was well preserved in patients (ejection fraction = 53 (7)% vs 52 (8)% in normal subjects, p = 0.72).

Conclusions

Increased peak flow velocity in the pulmonary trunk was often observed late after ASO, even in the absence of significant pulmonary artery stenosis. Haemodynamic consequences were RV hypertrophy and RV relaxation abnormalities as early markers of disease, while systolic RV function was well preserved.     

MRI在合并大血管异常的小儿复杂型先天性心脏病诊断中的应用

目的 探讨MRI对复杂型先天性心脏病（CCHD）患儿大血管异常的诊断价值.方法 22例CCHD患儿接受心脏大血管MRI检查,所有患儿行手术治疗并证实诊断.结果 手术证实22例CCHD患儿中法洛四联症11例、内脏心房异位综合征4例、血管环2例、先天性主动脉缩窄5例.CCHD患儿合并的大血管异常在MRI上全部得到明确诊断.结论 MRI能够准确诊断CCHD患儿的大血管异常.     

成人先天性心脏病合并肺动脉高压患者的心脏磁共振评估

目的 探讨心脏磁共振（cardiac magnetic resonance,CMR）评价先天性心脏病合并肺动脉高压患者心室功能的临床价值.方法 对26例先天性心脏病合并肺动脉高压的患者行CMR检查,分别测量并计算右心室与左心室的短轴缩短率、舒张末期直径、舒张末期容积、收缩末期容积、每搏排血量、射血分数等心功能参数及主动脉、肺动脉直径,同时评价室间隔运动、心肌延时强化.采用配对样本t检验比较左、右心室功能参数,采用两个独立样本t检验比较室间隔运动正常组和异常组的右心功能情况,采用卡方检验比较室间隔运动异常与心肌延时强化的关联.结果 右心室舒张末期直径、舒张末期容积、收缩末期容积均显著高于左心室,差异有统计学意义（P＜0.05）;右心室短轴缩短率、射血分数均显著低于左心室,差异有统计学意义（P＜0.05）.26例患者中,14例室间隔运动异常,14例出现心肌延时强化.室间隔运动异常组心肌延时强化出现比例明显高于室间隔运动正常组,差异有统计学意义（P＜0.05）.同时,室间隔运动异常组的右心室舒张末期直径、舒张末期容积均显著高于正常组,差异有统计学意义（P＜0.05）;右心室短轴缩短率显著低于正常组,差异有统计学意义（P＜0.05）;射血分数低于正常组,但差异无统计学意义（P=0.08）.结论 合并肺动脉高压的成人先天性心脏病患者右心功能较左心功能差,室间隔运动异常患者的右心功能更差,室间隔运动异常患者出现心肌延时强化比例高.CMR能够提供先天性心脏病合并肺动脉高压患者的左、右心室功能及相关结构信息,对治疗和预后有重要价值.     

Stent implantation in the ductus arteriosus for pulmonary blood supply in congenital heart disease.

Indications for catheter-based interventions in patent ductus arteriosus (PDA)-dependent pulmonary blood flow have yet to be defined. The aim of this study was to assess the acute and midterm outcome after stent implantation in the PDA. Between 1996 and 2002, ductal stenting was performed in 21 neonates and infants (14 females/7 males) to maintain pulmonary blood supply in cyanotic congenital heart disease (CHD). Balloon-expandable stents were implanted in the PDA with a final diameter of 4-5 mm without procedural deaths. Stent patency was achieved for 8-1,130 days (median, 142). Reintervention was necessary in nine patients. Overall survival rate after 6 years was 86%. Two neonates died a few days after the procedure due to right heart failure not related to PDA stenting. Corrective surgery was possible in six patients. An additional aortopulmonary shunt was needed in three patients; Fontan type operations were performed in six. One patient died after bidirectional Glenn shunt, another five reached palliation by additional perforation of the atretic valve/balloon valvuloplasty, and two are awaiting surgery. We conclude that in many patients with cyanotic CHD, especially in those with ductal pulmonary perfusion and additional forward flow from the right ventricle, ductal stenting is an effective transcatheter approach. Morphology of the PDA predicts the risk of restenosis and necessity of reintervention. Growth of the pulmonary vascular bed allows corrective or palliative surgery, and some patients can be cured by the intervention alone.     

Congenital esophageal stenosis associated with esophageal atresia

Congenital esophageal stenosis (CES) is a rare clinical condition but is frequently associated with esophageal atresia (EA). The aim of this study is to report the diagnosis, management, and outcome of CES associated with EA. Medical charts of CES‐EA patients from Lille University Hospital, Sainte‐Justine Hospital, and Montreal Children's Hospital were retrospectively reviewed. Seventeen patients (13 boys) were included. The incidence of CES in patients with EA was 3.6%. Fifteen patients had a type C EA, one had a type A EA, and one had an isolated tracheoesophageal fistula. Seven patients had associated additional malformations. The mean age at diagnosis was 11.6 months. All but two patients had non‐specific symptoms such as regurgitations or dysphagia. One CES was diagnosed at the time of surgical repair of EA. In 12 patients, CES was suspected based on abnormal barium swallow. In the remaining four, the diagnostic was confirmed by esophagoscopy. Eleven patients were treated by dilation only (1–3 dilations/patient). Six patients underwent surgery (resection and anastomosis) because of failure of attempted dilations (1–7 dilations/patient). Esophageal perforation was encountered in three patients (18%). Three patients had histologically proven tracheobronchial remnants. CES associated with EA is frequent. A high index of suspicion for CES must remain in the presence of EA. Dilatation may be effective to treat some of them, but perforation is frequent. Surgery may be required, especially in CES secondary to ectopic tracheobronchial remnants.     

Persistent pulmonary hypertension of the newborn associated with pulmonary atresia and intact interventricular septum

Neonates with pulmonary atresia and intact interventricular septum (PAIVS) do not have pulmonary vascular disease secondary to their heart abnormality. Persistent pulmonary hypertension of the newborn has not been described in association with this condition. The case is reported of a female neonate born with PAIVS, who preoperatively had no clinical evidence or any risk factors for persistent pulmonary hypertension of the newborn, but whose postoperative course was highly suggestive of persistent pulmonary hypertension; necropsy confirmed the features of pulmonary vascular disease.


Keywords: persistent pulmonary hypertension; pulmonary atresia and intact ventricular septum; pulmonary vascular disease; surgery; congenital heart defects     

Endovascular stent implantation in a coronary artery to pulmonary artery fistula in a patient with pulmonary atresia with ventricular septal defect and severe cyanosis.

A 31-year-old male with pulmonary atresia, ventricular septal defect presented with exercise intolerance and severe cyanosis. A restrictive coronary-pulmonary artery fistula was identified as the main source of pulmonary blood flow. We report transcatheter stent implantation in the fistula to augment pulmonary flow as a palliative management option in the adult patient with complex congenital heart disease.     

Isolated atresia of the left pulmonary veins

Four cases of congenital isolated atresia of the left pulmonary veins were observed over a 12-year period. The diagnosis was established through radionuclide pulmonary perfusion studies together with cardiac catheterization and pulmonary arteriography. Both procedures showed an elevated pulmonary pressure wedge and decreased blood flow with late poor filling of the pulmonary capillary bed on the affected side. Arteriography revealed non-visualization of the left pulmonary veins. The possibility of congenital atresia of the pulmonary veins should be borne in mind when confronted by unexplained hemoptysis, recurrent lower respiratory infections with protracted infiltrates, or pulmonary arterial hypertension. Surgical treatment, though difficult, is feasible.     

The pulmonary circulation and its systemic arterial supply in pulmonary atresia

The pulmonary circulation and its systemic arterial supply in cases of pulmonary atresia were studied angiocardiographically. In 69% of the cases the pulmonary arteries were hypoplastic and supplied by a patent ductus arteriosus or a few large systemic arteries. A classification into four groups is suggested, and a developmental paradox represented by this classification is discussed.     

颈动脉狭窄和闭塞患者空间工作记忆的功能磁共振成像研究

目的探讨左侧颈内动脉、大脑中动脉狭窄和闭塞患者与正常人空间工作记忆的激活脑区差异。方法选择左侧颈动脉狭窄和闭塞患者10例(病例组),其中左侧颈内动脉狭窄4例,左侧颈内动脉闭塞2例,左侧大脑中动脉狭窄3例,左侧大脑中动脉闭塞1例,另选健康体检者20例(对照组),对病例组和对照组进行空间记忆任务的功能磁共振成像,采用AFNI软件进行分析。结果病例组空间位置记录正确率较对照组明显降低[(79.2±16.5)%vs(88.6±11.8)%,P<0.05]。对照组空间工作记忆的编码期、保持期、提取期脑激活明显强于病例组。结论左侧颈动脉狭窄和闭塞患者空间记忆任务的脑激活区存在损害,功能磁共振成像研究能为早期诊断认知障碍提供影像学依据。     

肺静脉内膜剥脱术治疗小儿先天性肺静脉狭窄

目的 分析先天性肺静脉狭窄患儿的临床特点,探讨肺静脉内膜剥脱术治疗小儿先天性肺静脉狭窄的疗效.方法 回顾性分析2008年1月至2009年12月收治的4例先天性肺静脉狭窄患儿的临床资料,其中男3例,女1例,年龄2个月～2岁11个月.均行胸片、心电图和超声心动图检查,其中2例行心脏多排计算机断层扫描检查,以期明确诊断.4例患者共有7支肺静脉狭窄,右上肺静脉3例,右下肺静脉1例,左上肺静脉1例,左下肺静脉2例.狭窄部位均在肺静脉开口处,均合并其他心血管畸形：室间隔缺损3例,房间隔缺损1例,动脉导管未闭2例,主动脉缩窄1例,术前均提示重度肺动脉高压.术前根据病情予强心、利尿、扩血管及抗感染等治疗.完善术前准备后在全身麻醉、胸骨正中切口、深低温、体外循环下行肺静脉内膜剥脱术.结果 术后患儿血流动力学稳定;机械通气时间3.7（2～4）d,重症监护时间4.2（3～5）d;围术期无一例死亡.随访18～26个月,患儿均存活,心功能（纽约心脏协会）Ⅰ级3例、Ⅱ级1例,生长发育均正常.结论 先天性肺静脉狭窄常合并各种心内畸形,超声心动图、心脏多排计算机断层扫描检查对诊断帮助较大.肺静脉内膜剥脱术治疗先天性肺静脉狭窄患儿安全、有效,手术成功率高,近期随访疗效较好,对于婴幼儿先天性肺静脉狭窄不失为一种治疗选择.     

Pregnancy outcome in patients with complex pulmonary atresia: case report and review of the literature

Pulmonary atresia, a rare and complex congenital heart disease, is characterized by the absence of the central pulmonary artery and by the presence of a ventricular septal defect and aortopulmonary collaterals. Pregnancy reports concerning maternal and offspring outcome after palliative operation or repaired pulmonary atresia are sparse. We report here on the outcome of pregnancy in a woman, aged 36, with complex pulmonary atresia in whom palliative operation had been performed at the age of 23. We review the medical literature on pregnancy course as well as maternal and foetal outcome in cases involving this maternal congenital heart disease.     

经皮球囊肺动脉瓣成形术治疗婴儿重度肺动脉瓣狭窄及室间隔完整的肺动脉瓣闭锁

目的:总结经皮球囊肺动脉瓣成形术(percutaneous balloon pulmonary valvuloplasty,PBPV)治疗婴儿重度肺动脉瓣狭窄(pulmonary stenosis,PS)及室间隔完整的肺动脉瓣闭锁(pulmonary atresiawith intact ventricular septum,PA/IVS)的经验,评价其疗效及安全性。方法:自2007年1月至2012年10月,采用PBPV治疗婴儿重度PS及PA/IVS患者共33例,男性24例,女性9例,手术年龄3～12(8.9±2.9)个月,体质量5～11.5(9.0±1.6)kg。术前完善心电图、X线片、超声心动图、右心导管检查及右心室造影,采用单球囊扩张完成PBPV。PA/IVS患儿需先行肺动脉瓣射频打孔术。结果:球囊扩张手术技术成功率为96.97%(32/33)。右心室收缩压(right ventricular systolic piessure,RVSP)由术前的95～205(130.8±28.2)mmHg(1 mmHg=0.133kPa)下降至28～135(73.2±27.4)mmHg(t=12.067,P<0.001);肺动脉瓣跨瓣压差由术前的81～180(110.3±26.3)mmHg下降至10～112(47.7±23.8)mmHg(t=12.958,P<0.001)。1例术中出现心脏压塞,转外科急诊手术,术后恢复良好。出院时10例患者复查超声心动图仍显示平均跨肺动脉瓣压差>50 mmHg;随访中值时间26个月,3例残余重度肺动脉瓣狭窄,2例接受二次PBPV后压差降至轻度。本组术后发生轻度以上肺动脉瓣关闭不全(pulmonary insufficiency,PI)22例。结论:随着介入技术的不断提高,经皮肺动脉瓣球囊扩张术,已成为救治婴儿危重先天性肺动脉瓣狭窄及肺动脉瓣闭锁的安全有效的重要方法。     

Dehiscence of a pulmonary bioprosthesis with a focal dissection of the pulmonary artery in a patient with congenital pulmonic stenosis

Pulmonary valve replacement (PVR) is the most common adult congenital cardiac operation performed. Valve degeneration leading to prosthetic stenosis and/or regurgitation is a long‐term risk in this population and may be associated with paravalvular leak (PVL). Complications involving the proximal pulmonary artery, including dissection, are less clearly defined. Herein, we report the case of a 30‐year‐old patient with a history of multiple pulmonary valve interventions secondary to congenital pulmonic stenosis, who developed dehiscence of a bioprosthetic PVR associated with significant paravalvular leak (PVL) and further complicated by a focal dissection of the proximal pulmonary artery.     

Regional pulmonary blood volume in patients with abnormal blood pressure or flow in the pulmonary circulation

We studied the effects of a chronic increase in flow and ofchronic hypertension on regional pulmonary blood volume andextravascular lung density (lung tissue and interstitial waterper unit thoracic volume) in one group of patients with intracardiac,left-to-right shunt and in another group with Eisenmenger'ssyndrome or primary pulmonary hypertension. We used positroncomputerized tomography to measure regional lung density (transmissionscans) and blood volume (labelling with ¹¹CO). The distribution of pulmonary blood volume was more uniformin patients with a chronic increase in pulmonary blood flowthan in normal subjects. There were also indications of an absoluteincrease in intrapulmonary blood volume. In patients with chronicpulmonary arterial hypertension, the regional distribution ofblood volume was abnormally uniform, but there were no indicationsof substantial abnormalities in overall intrapulmonary bloodvolume.     

Imaging of recurrent ventricular septal defect and supravalvular pulmonary stenosis eight years after assumed total surgical repair of tetralogy of Fallot

A 42-year-old man underwent assumed total surgical repair of an acyanotic tetralogy of Fallot: a perimembranous ventricular septal defect (VSD) was closed with a dacron patch and myectomy of the infundibulum of the right ventricular outflow tract was performed. Reexamination eight years later revealed a large recurrent VSD and by surprise a narrow supravalvular ridge above rudimentary pulmonary cusps in the pulmonary trunk, leading to a pressure drop of 70 mmHg across the supravalvular stenosis. The latter finding was not recognized during the operation eight years before. The diagnosis could noninvasively be established by means of magnetic resonance imaging. During revision surgery the VSD was closed and an aortic homograft was inserted as conduit between the right ventricle and the pulmonary artery.     

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study

Background: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echo‐ cardiographic parameters associated with 2V circulation, (2) identify echocardio‐ graphic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.
Methods: We performed a retrospective analysis of patients who underwent RV de‐ compression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn circulation).
Results: Eighty‐one patients were included. At last follow‐up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow-up echocardiograms were available in 43 (53%) patients. The ma‐ jority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left‐to‐right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z‐score (P = .017), ≥ moderate baseline TR (P = .045) and smaller baseline RV area (P < .001) were associated with larger increases in RV area. Baseline RV area ≥6 cm² /m² had 93% sensitivity and 80% specificity for identifying patients who ultimately achieved 2V circulation. All patients with RV area ≥8 cm² /m² at follow up achieved 2V circula‐ tion. This finding was confirmed in a validation cohort from a separate center (N = 25). Factors associated with achieving RV area ≥8 cm² /m² included larger TV z‐score (P = .004), ≥ moderate baseline TR (P = .031), and ≥ moderate postdecompression pulmonary regurgitation (P = .002).
Conclusions: Patients with PA/IVS and smaller TV annuli are at risk for poor RV growth. Volume‐loading conditions signal increased capacity for growth sufficient for 2V circulation.     

Ebstein's anomaly associated with severe valvar pulmonary stenosis: Successful palliation with balloon pulmonary valvuloplasty in an adult

We report a 52-year-old adult with Ebstein's anomaly associated with severe valvar pulmonary stenosis, secundum atrial septal defect, polycythemia, hypoxemia, and severe exertional limitations. Balloon pulmonary valvuloplasty relieved the valvar obstruction, reduced the degree of tricuspid regurgitation and polycythemia, and improved systemic oxygenation and exercise abilities. Use of this technique in patients with this combination of lesions can postpone the need for surgical intervention. Cathet. Cardiovasc. Intervent. 46:441–444, 1999. © 1999 Wiley-Liss, Inc.