Echocardiographic assessment of neonates with pulmonary atresia and intact ventricular septum

In this prospective study, 27 consecutive neonates suspected to be suffering from pulmonary atresia and intact ventricular septum underwent detailed two-dimensional echocardiographic examination before cardiac catheterization. Of the 27 neonates 25 had pulmonary atresia and intact ventricular septum and the remaining 2 had "functional pulmonary atresia" secondary to severe Ebstein's anomaly of the tricuspid valve. In all 25 neonates with pulmonary atresia and intact ventricular septum, the diagnosis and right ventricular morphology based on the tripartite approach were correctly established by echocardiography. The associated Ebstein's anomaly in two babies with pulmonary atresia and intact ventricular septum was also correctly identified by echocardiography. Among the five babies who had a sinusoidal-coronary artery communication, echocardiography demonstrated the fistula in one and provided clues for its diagnosis in two others. In the 25 neonates with pulmonary atresia and intact ventricular septum, the echocardiographic dimensions of their tricuspid anulus, right ventricular infundibulum and main pulmonary artery correlated well with the angiocardiographic measurements (r greater than 0.8). The results of this study suggest that, in the management of neonates with pulmonary atresia and intact ventricular septum, preoperative evaluation by echocardiography is usually sufficient and cardiac catheterization should be reserved for selected cases.     

Laser-assisted tricuspid valve balloon dilation for acquired tricuspid valve atresia.

Acquired pulmonary valve atresia is a well-recognized but uncommon complication of surgical systemic-to-pulmonary artery shunts in patients with tetralogy of Fallot. Acquired atresia of the tricuspid valve, however, has not been reported previously. This complication developed in a 3-year-old girl, with pulmonary atresia and an intact ventricular septum, after a Blalock-Taussig shunt and right ventricular outflow tract reconstruction. Percutaneous transcatheter laser-assisted balloon dilation re-established antegrade flow across the tricuspid valve.     

Right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum: a case report

Pulmonary atresia with intact ventricular septum (PAIVS) is a morphologically heterogeneous lesion and accounts for 1-3% of critically ill infants with congenital heart disease. Numerous surgical approaches have been attempted with varying degrees of success, but the mortality rate is still high in most series. The optimal surgical procedure depends on the size and morphology of the tricuspid valve and right ventricle and the presence or absence of right ventricle-dependent coronary circulation. Therefore, it is pivotal to define the precise morphologic and hemodynamic characteristics, especially coronary artery anatomy. In this report, we describe a full-term female neonate with cyanosis soon after birth. Two-dimensional and color Doppler echocardiography corroborated the diagnosis of PAIVS and showed a small right ventricle. Cardiac catheterization indicated PAIVS and further revealed right ventricle-dependent coronary circulation. A systemic-to-pulmonary artery shunt was constructed with a positive immediate result.     

Management of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum with a small or hypoplastic right ventricle

Twenty-one neonates and infants less than 3 months old undergoing cardiac surgery for an obstructed right ventricular outflow tract, intact ventricular septum and a small or hypoplastic right ventricle were retrospectively analyzed, in order to assess the effects of a change in management protocol. Seven of the 8 patients with critical pulmonary stenosis survived surgery using a transannular outflow patch, whereas only 1 of the 8 patients with pulmonary atresia survived the same operation. Two patients in the latter group died 2 and 3 months after surgery but with complications arising from surgery. Of 5 patients with pulmonary atresia who had a modified Blalock Taussig shunt, 3 patients survived the surgery and were discharged home. These results significantly indicate that there is an unacceptably high mortality for the relief of pulmonary atresia (with intact septum) using a transannular outflow patch, and a Blalock Taussig shunt is the preferred operation. The transannular outflow patch is a safe operation for neonates with critical pulmonary stenosis, irrespective of the size of the right ventricle.     

Echo- and angiocardiography in the diagnosis of critical valvular stenosis and atresia of the pulmonary artery with intact ventricular septum in infants]

Two-dimensional and Doppler echocardiographic findings were compared with those of coronary angiography and cardiac catheterization in 31 patients (11 with pulmonary atresia and 20 with critical pulmonary stenosis in the presence of ventricular septum). Two-dimensional echocardiography was found to accurately define the linear dimensions, volumes, and ejection fraction of the ventricles in patients with the disease. Continuous Doppler echocardiography is a valuable tool for measuring right ventricular systolic pressure and pressure gradient between the right ventricle and the pulmonary artery. Two-dimensional and Doppler echocardiographies enable surgical tactics to be chosen in most cases without interventions.     

Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? A 5th aortic arch with a systemic-to-pulmonary arterial connection

Major abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.     

动脉导管内支架置入术治疗新生儿室间隔完整型肺动脉闭锁的疗效

目的 评估动脉导管内支架置入术治疗新生儿室间隔完整型肺动脉闭锁的可行性和疗效.方法 选择2007年12月至2010年9月行动脉导管内支架置入术的室间隔完整型肺动脉闭锁新生儿11例.患儿年龄3～13(8.20±2.90)d,体重3.00～3.88(3.41±0.29)kg.根据血管造影结果选择冠状动脉支架和球囊,支架准确定位后充盈球囊扩张支架.术后观察患儿的血氧饱和度,检查超声心动图和正侧位胸片,记录支架的内径、位置,并且在支架术后1、3、6和12个月进行随访.结果 11例患儿均顺利置入支架.术前末梢血氧饱和度为(63.27±8.47)%,术前应用前列地尔后末梢血氧饱和度为(82.73±5.59)%,支架置入术后末梢血氧饱和度为(86.18±3.19)%,应用前列地尔后和支架置入术后末梢血氧饱和度均高于术前(P均<0.01),支架置入术后末梢血氧饱和度高于前列地尔应用后(P<0.05).术中测量动脉导管最窄内径为(1.69±0.37)mm,长度为(16.72±2.37)mm,置入支架内径均为4 mm,长度(20.18±3.40)mm.1例患儿在术后发生支架移位和血氧饱和度下降,并行B-T分流术;1例患儿随访期间在院外死亡,原因不明;1例患儿因术后4个月末梢血氧饱和度下降而行支架球囊扩张术;2例患儿分别在支架置入术后5和7个月行外科手术.结论 动脉导管内支架置入术治疗新生儿室间隔完整型肺动脉闭锁可行,且近期疗效良好,可作为室间隔完整型肺动脉闭锁一期手术的首选治疗方法.

Abstract：

Objective To evaluate the feasibility and efficacy of arterial duct stenting in neonates with pulmonary atresia and intact ventricular septum.Methods Eleven neonatal pulmonary atresia with intact ventricular septum patients received arterial duct stenting in our hospital from December 2007 to September 2010 were involved in this study. The average age was (8.20±2.90) days (ranged from 3 to 13 days). The average weight was (3.41±0.29) kg (ranged from 3.00 to 3.88 kg). The stents were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the balloon was inflated to expand the stent to desired diameter. Oxygen saturation was monitored, echocardiography was measured and stent diameter and location were observed by chest X-ray.Patients were followed up at 1, 3, 6 and 12 months post procedure. Results Stents were successfully implanted in all 11 patients. The preoperative peripheral oxygen saturation was (63.27±8.47)%, while increased to (82.73±5.59)% after alprostadil application and to (86.18±3.19)% after operation (all P<0.01).After the operation, the peripheral oxygen saturation was higher than alprostadil application (P<0.05).The intraoperative narrowest diameter of patent ductus arteriosus was (1.69±0.37)mm, the length was (16.72±2.37)mm. The internal diameter of implant stents was 4 mm, the length was (20.18±3.40)mm. After the operation, surgical B-T shunt operation was performed in one patient due to stent shift and pulse oxygen saturation decrease.One patient died post operation with unknown reason, another patient received stent balloon dilatation due to pulse oxygen saturation decrease at 4 months after the surgery. Pulmonary atresia with intact ventricular septum surgeries were performed in 2 patients at 5 and 7 months after stent implantation. Conclusion The neonatal pulmonary atresia with intact ventricular septum arterial stent implantation was a feasible and effective procedure and this method could be used as preferred treatment in pulmonary atresia and intact ventricular septum for neonates.     

Changes in tissue Doppler characteristics in a patient with pulmonary atresia and intact ventricular septum

Tissue Doppler measurements of the right and left lateral ventricular walls were made before and after perforation of the pulmonary valve using radiofrequency energy in a patient with pulmonary atresia and intact ventricular septum. The ratio of peak tissue velocity during rapid ventricular filling to atrial contraction increased for both atrioventricular valves after perforation of the pulmonary valve, and the patient was able to be weaned off prostaglandins without further intervention. Such measurements made using tissue Doppler may aid in the management of patients with pulmonary atresia and intact ventricular septum by predicting improvements in right ventricular relaxation.     

超声心动图在肺动脉闭锁合并室间隔缺损诊断及外科治疗中的应用价值

目的:探讨超声心动图在肺动脉闭锁合并室间隔缺损诊断及外科治疗中的应用价值。方法:回顾性选取云南省阜外心血管病医院2018年1月至2020年5月期间外科治疗的肺动脉闭锁合并室间隔缺损患者21例,分析其术前超声心动图特点、解剖分型,评估肺动脉发育及体肺侧支动脉情况,并与CT结果相对比。术中经食道超声心动图监测,术后随访超声心动图评估手术效果。结果:超声心动图对肺动脉闭锁的诊断分型、右肺动脉及McGoon比值的测量结果与CT测值差异均无统计学意义(P均>0.05),左肺动脉测值及Nakata指数较CT测值小(P均<0.05)。术中经食道超声心动图示3例室间隔开窗患儿室水平示右向左或双向分流。术后患者左右肺动脉内径及Nakata指数或新建肺动脉指数均较术前增长,差异均有统计学意义(P均<0.05)。结论:超声心动图可以对肺动脉闭锁合并室间隔缺损患儿的肺动脉发育进行初步评估,结合心血管造影检查制定个体化治疗方案,术中及术后可用于随访评价手术疗效,具有重要临床价值。     

Severe tricuspid stenosis presenting as tricuspid atresia. Echocargraphic diagnosis and surgical management.

Two cases of pulmonary atresia with intact ventricular septum and severe tricuspid stenosis are described in which the initial angiographic diagnosis was tricuspid atresia and pulmonary atresia. Two dimensional echocardiography showed the features of an imperforate tricuspid valve because in each case contrast echocardiography failed to show anterograde flow from the right atrium to right ventricle. Successful radical surgical repair was achieved in one patient with performing a tricuspid valvotomy and inserting an external valved conduit between the hypoplastic right ventricle and the main pulmonary artery. In the second case, an infant died four weeks after tricuspid valvotomy and right ventricular outflow tract reconstruction.     

Two-dimensional echocardiographic evaluation of right ventricular size and function in newborns with severe right ventricular outflow tract obstruction

Critical pulmonary stenosis or atresia with intact ventricular septum represents a congenital cardiac lesion for which the long-term prognosis appears to depend partly on the size of the right ventricle. Thus, the capability of noninvasive assessment of right ventricular size to predict operative outcome was examined in 15 infants (aged 1 to 30 days, mean 5.6) with severe right ventricular outflow tract obstruction (either critical pulmonary stenosis [7 patients] or pulmonary atresia with intact ventricular septum [8 patients]). Using echocardiography in two orthogonal subxiphoid views, right ventricular volume, wall thickness, area change fraction, ejection fraction and tricuspid anulus dimension were measured. All patients with a normalized right ventricular enddiastolic volume of less than 5 ml/m2 and a normalized tricuspid anulus dimension of less than 1.0 cm/m2/3 required a shunt operation. Only one patient with a volume of more than 6 ml/m2 and a normalized tricuspid anulus dimension of more than 1.4 cm/m2/3 required more than relief of right ventricular outflow tract obstruction. In this patient, residual severe pulmonary stenosis necessitated the shunt procedure. One patient with a volume of more than 6 ml/m2 had an anulus diameter of less than 1.4 cm/m2/3 and one patient with an anulus diameter of more than 1.4 cm/m2/3 had a volume of less than 6 ml/m2; both required shunt procedures. It therefore appears that if either the ventricular volume or tricuspid anulus size is excessively small, a shunt procedure is necessary. Wall thickness, area change fraction and ejection fraction measurements were not significantly correlated with right ventricular volume or postoperative outcome.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cardiac Computed Tomography of Multiple Coronary Arteries to Right Ventricle Fistulas in a Newborn With Pulmonary Atresia and Intact Ventricular Septum

Right ventricle-dependent coronary circulation significantly alters the therapeutic options and prognosis in neonates with pulmonary atresia and intact ventricular septum. The contribution of cardiac computed tomography for the accurate assessment of multiple coronary arteries to right ventricle fistulas in a newborn infant with pulmonary atresia and intact ventricular septum is described.     

Transcatheter management of neonates with pulmonary atresia and intact ventricular septum

This report describes a 1-day-old infant with valvar pulmonary atresia with intact ventricular septum in whom we were successful in performing transcatheter guidewire perforation and balloon pulmonary valvuloplasty to establish right ventricle-to-pulmonary artery continuity and flow. Also described is implantation of a 4 mm coronary stent into ductus arteriosus in lieu of surgical aortopulmonary shunt to treat pulmonary oligemia and systemic arterial hypoxemia. Details of transcatheter guidewire perforation are presented and it is suggested that this method be used as an alternative to Laser/radio frequency wires, especially in the absence of approval of the latter wires by the regulatory agencies. Stenting of the ductus may be considered an alternative to surgical aortopulmonary shunt. Role of transcatheter technology in the management of selected patients with pulmonary atresia and intact ventricular septum is discussed. Cathet. Cardiovasc. Diagn. 42:395–402, 1997. © 1997 Wiley-Liss, Inc.     

Technique of percutaneous laser-assisted valve dilatation for valvar atresia in congenital heart disease.

OBJECTIVE--To investigate the efficacy and safety of transcatheter laser-assisted valve dilatation for atretic valves in children with congenital heart disease. DESIGN--Prospective clinical study. SETTING--Supraregional paediatric cardiology centre. SUBJECTS--Eleven children (aged 1 day-11 years; weight 2.1-35.7 kg) with atresia of pulmonary (10) or tricuspid (one) valve underwent attempted laser-assisted valve dilatation as part of the staged treatment of their cyanotic heart disease. INTERVENTION--After delineating the atretic valve by angiography and/or echocardiography a 0.018 inch "hot tip" laser wire was used to perforate the atretic valve. Subsequently the valve was dilated with conventional balloon dilatation catheters up to the valve annulus diameter. RESULTS--Laser-assisted valve dilatation was successfully accomplished in nine children. In two neonates with pulmonary valve atresia, intact ventricular septum, and coexistent infundibular atresia the procedure resulted in cardiac tamponade: one died immediately and one later at surgery. During a follow up of 1-17 months (mean 11) two infants with pulmonary valve atresia and intact ventricular septum died (one with congestive cardiac failure). The remainder are either well palliated and do not require further procedures (three), or are awaiting further transcatheter or surgical procedures because of associated defects (four). CONCLUSIONS--Laser-assisted valve dilatation is a promising adjunct to surgery in this high risk group of patients. It may avoid surgery in some patients, and may reduce the number of surgical procedures in those requiring staged operations.     

Left subclavian-pulmonary trunk fistula with a vascular prosthesis as palliative treatment in Fallot conditions]

In this paper, our experience with a new systemic-to-pulmonary artery shunt: subclavian-pulmonary artery trunk shunt with PTFE (central shunt) is presented. Between November 1985 and March 1990 this central shunt was employed in 8 children with ages ranged between 4 days and 3 years, and weights between 2 and 12 kg. Diagnosis were Fallot's tetralogy in three; pulmonary atresia with intact septum in three; complete AV canal and Fallot's tetralogy in one, and univentricular heart and pulmonary atresia in 1 patient. There were no surgical deaths. Hospital mortality was present in 1 case (AV canal and Fallot? tetralogy) at the moment of reoperation 1 month later. Follow-up ranged between 2 and 46 months. One case, pulmonary atresia with intact septum, has undergone total correction. The remainder 6 cases are in good situation with O2 saturation above 70%. This central shunt has the advantages to provide a bidirectional blood flow to both pulmonary branches avoiding the risk of direct damage in the pulmonary arteries found with the conventional aorto-pulmonary shunts.     

新生儿及婴儿危重型肺动脉狭窄及肺动脉膜性闭锁的外科治疗

目的:总结我科1997年4月至2007年8月对新生儿及婴儿室间隔完整型肺动脉闭锁及危重型肺动脉瓣狭窄的手术治疗经验。方法:手术治疗共23例,年龄6d~11个月。其中室间隔完整型肺动脉膜性闭锁10例,危重型肺动脉瓣狭窄13例。19例经胸正中切口体外循环下心脏不停跳完成手术,4例应用左胸后外侧切口非体外循环方法。除早期1例同期行动脉导管未闭(PDA)结扎及卵圆孔未闭(PFO)缝合外,其余22例均采用保留PDA、单纯切开肺动脉瓣的方法。结果:围手术期死亡2例,分别死于低氧血症及急性肾功能衰竭。术后当日超声心动图测肺动脉跨瓣压差为37~132mmHg(1mmHg=0.133kPa),平均61mmHg。2周后复查示肺动脉跨瓣压差为26~77mmHg,平均43mmHg,较术后早期明显降低(P<0.05)。出院前不吸氧下测动脉血氧饱和度78%~92%,平均85%,较术前明显增高(P<0.05)。随访4个月至10年,平均5.8年。PDA均闭合,肺动脉血流通畅,三尖瓣返流消失或明显减轻。结论:保留动脉导管、单纯肺动脉瓣切开术对于治疗新生儿及婴儿室间隔完整型肺动脉膜性闭锁及危重型肺动脉瓣狭窄是一种安全有效的方法。     

Surgical treatment of pulmonary atresia with intact ventricular septum.

Fifty-nine children with pulmonary atresia and intact ventricular septum underwent various forms of surgical treatment at the Hospital for Sick Children, Toronto, during 1950 to mid 1975. Twenty-three patients had pulmonary valvotomy, 15 direct, 2 indirect, and 6 both direct valvotomy and infundibulectomy. All died, 19 early and 4 late. Of 13 patients who received a systemic-pulmonary artery shunt, 4 combined with surgical atrial septectomy, there are only 2 long-term survivors both of whom were children who had had a Waterston anastomosis. Recently we have been treating infants with small right ventricles with balloon atrial septostomy at cardiac catherterization followed by a Potts anastomosis and pulmonary valvotomy. If the Potts anastomosis appears satisfactory the persistent ductus arteriosus is ligated. This scheme was used in 23 infants, with 4 early deaths and 2 late deaths. Of 17 survivors, further shunts were required in 4 children. One child has had a formal repair, with insertion of valves in both tricuspid and pulmonary areas. We believe that this operative combination of Potts anastomosis and pulmonary valvotomy offers the infant with pulmonary atresia and a small right ventricle a relatively low initial mortality and the possibility of right ventricular enlargement and subsequent repair.     

Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years' experience.

OBJECTIVE: To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum. DESIGN: Prospective study. SETTING: Tertiary cardiac unit. PATIENTS: All infants with pulmonary atresia and intact septum with no more than minor tricuspid valve hypoplasia referred between November 1990 and June 1995. Laser valvotomy was attempted in nine infants of median age 4-5 days and median weight 3.6 kg. INTERVENTION: The pulmonary valve was perforated using a 0.018 inch fibreoptic guidewire attached to a NdYag laser and introduced through a catheter positioned beneath the valve. After perforation the valve was dilated with progressively larger balloons. MAIN OUTCOME MEASURES: Successful laser valvotomy and balloon dilatation, complications, pulse oximetry, right ventricular outflow velocities, and need for surgical treatment. RESULTS: Valvotomy was successful in all but one case, the failure being due to laser breakdown. After perforation the valve was dilated to 6-8 mm diameter. Prostaglandin E was withdrawn immediately in six of the eight duct dependent infants, and 28 and 49 days later in two. No patient required an aortopulmonary shunt. Two patients had repeat valvoplasty at 20 days and three months of age, respectively; one required infundibular resection and closure of the atrial septum at age four and one is awaiting similar treatment. CONCLUSIONS: Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy.     

Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum

Children who die after operation for pulmonary atresia and intact ventricular septum may have myocardial ischemia. The relation between histologic evidence of myocardial ischemic injury and the presence of a right ventricle to coronary artery fistula, coronary artery dysplasia and operation in 17 autopsy specimens was assessed. Age at death ranged from 1 day to 16 years (median, 11 days). Of the 17 hearts, 6 (35%) had right ventricle to coronary artery fistulas, 5 of which had coronary artery dysplasia. In three cases, there was segmental or complete absence of a coronary artery. Ischemia was present in four of these six hearts, two of which had right ventricular outflow reconstruction. Six of the 11 hearts without right ventricle to coronary artery fistulas also had myocardial ischemia. Of these six cases, four had right ventricular outflow reconstruction and two had shunt operations. Death occurred from 1 to 8 days (mean 3) after operation. Hearts with pulmonary atresia and intact ventricular septum may have myocardial ischemia with or without either right ventricle to coronary artery fistulas or coronary artery dysplasia. Myocardial ischemia may occur after right ventricular outflow reconstruction or shunt operations. Thus, myocardial ischemia occurs commonly in patients with pulmonary atresia and intact ventricular septum and is not always related to coronary abnormalities or operation.     

Functional assessment of the Fontan operation: combined M-mode, two-dimensional and Doppler echocardiographic studies

Combined M-mode, two-dimensional and Doppler echocardiographic studies were used to assess the postoperative status of 33 patients who had undergone the modified Fontan procedure. Twenty-four patients had surgical repair with use of a simple direct right atrium to pulmonary artery anastomosis. The remaining patients had repair with use of a prosthesis or associated Glenn shunt. Twenty-seven patients were studied early in the postoperative period (2 months or less) and the remaining patients were studied up to 6 years postoperatively. A total of 36 examinations were performed. Of the 33 patients, 13 had tricuspid atresia, 12 had double inlet left ventricle with hypoplastic right ventricular outlet chamber and 8 had complex lesions with atrioventricular canal, double outlet right ventricle or a hypoplastic ventricle. Postoperative assessment by M-mode and two-dimensional echocardiography demonstrated normal or mildly reduced ventricular function (ejection fraction greater than 40%) in 22 patients. In 24 patients, a "normal" flow pattern was observed in the pulmonary artery by pulsed Doppler echocardiography, with predominant diastolic flow and accentuation by atrial systole somewhat similar to the venous flow pattern observed in the superior vena cava. "Abnormal" flow patterns (disorganized systolic flow, absence of atrial waves and little or no increase with inspiration) were observed in nine patients with reduced ventricular function or residual shunt. Continuous wave Doppler study also demonstrated mild dynamic subaortic obstruction in two patients. Combined pulsed and continuous wave studies showed atrioventricular valve insufficiency in 10 patients. Follow-up studies revealed a satisfactory clinical course in most patients. Three patients died approximately 4 to 8 months after their Fontan operation.