Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation

Objective: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long‐term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure.
Design, Settings, and Patients: We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute contraindications. Intervention and Outcome Measures: Demographic and procedural data were cor‐ related with outcome measures. Outcomes analyzed included procedural success, reintervention rates, final circulation type, and functional class. Multivariate analysis and receiver operator curve were used to identify for parameters in predicting biven‐ tricular circulation.
Results: The procedural success rate was 92% (33 out of 36) in this group with mod‐ erate right ventricular hypoplasia (tricuspid valve z score −4.2 ± 3.0, 69.4% of pa‐ tients with z score <−2.5). Early reintervention rate was 39%, mostly being insertion of modified Blalock–Taussig shunt. Overall reintervention‐free survival was 53%, 30%, and 19% at 1, 6, and 12 months postintervention. Despite no significant catch‐ up right ventricular growth, majority of survivors (84%) enjoyed a biventricular circu‐ lation with good functional status. A tricuspid to mitral valve ratio >0.79 was a good predictor of biventricular outcome. (specificity of 100%, positive predictive value 100%).
Conclusion: Encouraging long‐term results with biventricular circulation and func‐ tional status were demonstrated with transcatheter pulmonary valve perforation in patients even with moderate hypoplastic right ventricle, which is comparable to that with mild right ventricular hypertrophy. The baseline tricuspid to mitral valve ratio was identified as a potentially useful tool in predicting biventricular circulation.     

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     

Does hypoplasia of one pulmonary artery preclude a definitive repair in pulmonary atresia,intact ventricular septum,and hypoplastic right ventricle?

Summary Twins with pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle associated with underdeveloped and discontinuous left pulmonary artery are described. Operations to connect the left pulmonary artery to the main pulmonary trunk, with subsequent biventricular repair assisted by bidirectional cavopulmonary anastomosis and atrial fenestration were performed. Follow-up catheterization proved spontaneous closure of the fenestration in one patient. Both patients have been in a satisfactory condition for 3 years since operation.     

非体外循环与体外循环下肺动脉瓣切开术治疗室间隔完整型肺动脉闭锁的疗效对比研究

目的 通过评价非体外循环和体外循环下肺动脉瓣切开术作为初期手术治疗室间隔完整型肺动脉闭锁(PA/IVS)的临床状况,比较两种方法的手术疗效和早中期预后效果.方法 回顾2015年1月至2020年12月,共45例PA/IVS患儿在我院接受非体外循环或体外循环下肺动脉瓣切开术,分为非体外循环组(28例)和体外循环组(17例)...     

单纯肺动脉瓣疏通术初期治疗危重婴幼儿室间隔完整型肺动脉闭锁

目的:探讨低龄危重婴幼儿室间隔完整型肺动脉闭锁,及危重肺动脉瓣狭窄的初期外科治疗方法。方法:2009年6月至2013年6月,我院共对10例室间隔完整型肺动脉闭锁及2例危重肺动脉瓣狭窄婴幼儿患者,施行经胸正中切口非体外循环下,单纯肺动脉瓣疏通术(改良Brock术)治疗,术中保留动脉导管未闭,三尖瓣反流未做处理。患儿年龄2~9个月,体质量4.3~10kg,平均6.4kg。所有患者均合并动脉导管未闭及房间隔缺损或者卵圆孔未闭,术前患者末梢血氧饱和度62%~73%,平均69%。结果:本组围术期死亡1例(8.3%),死亡原因为术后持续低氧血症,其余11例术后均缺氧状态改善,经皮血氧饱和度上升至84%~93%,平均89%。术后测得肺动脉瓣跨瓣压差为11~28mmHg(1mmHg=0.133kPa),平均15.6mmHg,术后随访11例,PDA均闭合,三尖瓣反流由术前大量转为少量或者消失。结论:作为危重婴幼儿的初期治疗,对于发绀严重,生长发育差,不能耐受体外循环的危重室间隔完整型肺动脉闭锁及危重肺动脉瓣狭窄患儿,Brock术可获得满意的治疗效果。     

Transcatheter coil occlusion of coronary artery to right ventricular fistulae in pulmonary atresia with intact ventricular septum

We describe a young adult with an underlying diagnosis of pulmonary atresia with intact ventricular septum and Fontan physiology who presented with extreme dyspnoea on exertion. Coronary artery to right ventricular fistulae and reversible myocardial ischaemia secondary to a right ventricular steal phenomenon were present. The right coronary artery to right ventricular fistula was completely occluded with coils, by a transcoronary approach, with amelioration of symptoms. However, one coil embolised and lodged uneventfully in the left atrium during the procedure. This case demonstrates that a transcatheter approach may be successfully employed not only in patients with isolated coronary artery fistulae, but also in patients with coexistent complex congenital heart disease.     

Coronary artery fistula embolization in an infant with pulmonary atresia intact ventricular septum: a case report.

This report presents a case of pulmonary atresia/intact ventricular septum with right ventricular-dependent coronary circulation. At 7 months of age, the infant underwent coil embolization of the connection between the right ventricle and the coronary circulation. The child is currently well following surgical decompression of the right ventricle.     

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。     

Quantitation of the effective right ventricular output in patients with pulmonary atresia and intact ventricular septum after placement of a right ventricular outflow patch and an aorticopulmonary shunt

A mathematical derivation is presented of an equation that allows quantitation, in the cardiac catheterization laboratory, of the effective output of the right ventricle (Q_RV) in patients with pulmonary atresia and intact ventricular septum (PA/IVS) after palliation with a right ventricular outflow patch and an aorticopulmonary shunt. This derivation is based on the Fick principle and accounts for that portion of the right-to-left atrial shunt that flows through the aorticopulmonary shunt to the lungs and thus contributes to the effective pulmonary blood flow (Q_ep). This equation relates Q_RV to pulmonary blood flow (Q_p), systemic cardiac output (Q_s), and Q_ep by Q_RV = Q_p + Q_s - (Q_p)(Q_s)/Q_ep. By providing an assessment of right ventricular function, this equation may be helpful in determining the suitability of the right ventricle to support biventricular repair. © 1994 Wiley-Liss,Inc..     

室间隔完整型肺动脉闭锁及肺动脉瓣狭窄的手术治疗

目的探讨新生儿、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。方法19例患儿,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9例。15例经胸正中切口体外循环心脏不停跳下完成手术,4例经左胸后外侧切口非体外循环下完成手术。除早期1例同期行动脉导管未闭（PDA）结扎及卵圆孔未闭缝合术外,其余18例均保留PDA、单纯切开肺动脉瓣。结果本组围术期死亡2例,院外死亡1例。其余16例术后当日肺动脉跨瓣压差为37—132mmHg,平均61mmHg;2周后为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PDA均闭合,右心室较术前明显增大,三尖瓣反流消失或明显减轻,房水平分流消失。结论对室间隔完整型肺动脉闭锁及危重型肺动脉瓣狭窄实施单纯肺动脉瓣切开术治疗安全有效。     

Initial and late results after catheter intervention for neonatal critical pulmonary valve stenosis and atresia with intact ventricular septum: a technique in continual evolution.

Critical pulmonary valve stenosis or atresia with intact ventricular septum is a rare congenital cardiac defect that can be technically difficult to alleviate in the catheterization laboratory. Over the past 10 years, several techniques and modifications with variable results have been advocated to facilitate the valvuloplasty procedure. This report describes a single operator's experience using various techniques in 28 neonates with critical pulmonary stenosis or atresia who were considered candidates for transcatheter intervention. The first two patients underwent a gradational balloon valvuloplasty approach that resulted in prolonged fluoroscopy exposure. Thereafter, a "snare assisted" umbilical artery approach was developed which facilitated the valvuloplasty procedure and resulted in significantly fewer balloons used and shorter fluoroscopy times. Early in our experience, stiff guidewire perforation of atretic pulmonary valves was used, whereas in our last two patients, a simplified perforation technique with a new 0.9-mm excimer laser catheter was used. Late echocardiographic and clinical follow-up evaluation in 27 patients demonstrates persistent gradient relief, resolution of tricuspid valve insufficiency, and elimination of right to left shunting at the atrial level. Balloon valvuloplasty is the treatment of choice for critical pulmonary valve stenosis or atresia with intact ventricular septum. When necessary, the use of umbilical artery "snare assistance" facilitates the valvuloplasty technique and shortens procedure time while laser perforation is currently preferable for perforation of the atretic pulmonary valve.     

Transcatheter perforation of atretic pulmonary valve by the stiff end of a coronary wire in neonates with pulmonary atresia with intact ventricular septum: A solution in developing countries

Objectives

To evaluate the safety of using the stiff end of a coronary wire to perforate an atretic pulmonary valve (PV) in patients with pulmonary atresia with intact ventricular septum (PAIVS).

Prenatal diagnosis of right ventricular outflow tract obstruction with intact ventricular septum, and detection of ventriculocoronary connections

OBJECTIVES: To determine the accuracy of prenatal diagnosis of pulmonary atresia and intact ventricular septum (PAIVS), and pulmonary stenosis, including prenatal detection of ventriculocoronary connections, to evaluate heart size during the prenatal period, and to evaluate the outcome. DESIGN AND PATIENTS: Medical records of 20 cases with prenatally diagnosed PAIVS and pulmonary stenosis were reviewed retrospectively. Prenatal and postnatal echocardiography were also reviewed and dimensions of the ventricles and vessels were measured retrespectively. RESULTS: Of 20 prenatal diagnoses (15 PAIVS and five pulmonary stenosis), 16 were confirmed as correct. One critical pulmonary stenosis case had been diagnosed as PAIVS prenatally; three had no confirmation. Eight pregnancies were terminated, three had no active treatment, and nine were treated; all survived. Of 13 assessed with ventriculocoronary connections prenatally, seven were diagnosed correctly (four with, three without ventriculocoronary connections), but one was falsely positive; five had no confirmation. The more prominent hypoplasia of the main pulmonary artery and the tricuspid valve annulus, and the sigmoid shape of the ductus arteriosus, seemed to be associated with the presence of ventriculocoronary connections. CONCLUSIONS: Current prenatal echocardiography can accurately diagnose right ventricular outflow tract obstruction and ventriculocoronary connections. Prenatal detection of this constellation of abnormalities aids in family counselling and decisions on postnatal management.     

Transcatheter perforation of the right ventricular outflow tract as initial therapy for pulmonary valve atresia and intact ventricular septum in the newborn

The clinical impact of transcatheter perforation and dilatation of the right ventricular outflow tract in neonates with pulmonary atresia and intact ventricular septum was reviewed. Between April 1992 and December 1994, 8 neonates underwent transcatheter perforation of the right ventricular outflow tract. Radiofrequency energy was employed in 6 patients and wire perforation in 2 patients. Mean patient age at intervention was 1.9 ± 0.6 days and weight 3.4 ± 0.5 kg. Median tricuspid valve annulus was 10.9 mm (range: 4.0–13.0 mm) and Z-value −0.85 (range: −4.5–1.0). The mean right ventricular systolic pressure fell from 117 ± 16 to 55 ± 15 mm Hg (P < 0.0001), and the right ventricular to aortic pressure ratio decreased from 1.81 ± 0.33 to 0.82 ± 0.28 (P < 0.0001). The arterial duct was patent in all. No acute complications occurred. Aortopulmonary shunts were performed in 7 patients at a median 6 days (range: 3–23 days) following catheterization. One patient developed sepsis and died after surgical resection of infected tissue, while a second patient died of a blocked aortopulmonary shunt 17 months following discharge. Median follow-up for the 6 surviving patients was 8 months (range: 4–32 months). One patient has achieved and a second is awaiting biventricular repair. Transcatheter perforation appears to be a promising form of therapy in selected patients with pulmonary atresia, and potentially facilitates algorithms leading to a biventricular repair. Cathet. Cardiovasc. Diagn. 40:408–413, 1997. © 1997 Wiley-Liss, Inc.     

Role of Surgery on Growth of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum: Mid-Term Results of Modified Right-Ventricular Overhauling Procedure

Objectives: To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve (TV) growth in patients with pulmonary atresia with intact ventricular septum (PAIVS). Methods: We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling (mRVoh) between 2008 and 2019 at two institutions. Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle, peeling off fibrotic endocardial tissue in the right ventricle (RV) cavity, surgical pulmonary valvotomy, and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass. The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh. Results: No mortalities were observed during a median follow-up of 3 years (interquartile range: 1.3–4.7 years) of follow-up were noted. mRVoh was performed at a median age of 163.5 days (range: 21–560 days), including seven neonates and two infants (<60 days). During follow-up, the median TV annular z-score increased significantly from −2.24 to −1.15 before and after mRVoh (p = 0.004). In ten patients with a prior history of percutaneous interventions for RV outflow tract (RVOT) widening at least 6 months before mRVoh, the TV annular z-score significantly changed during the period after mRVoh (−2.03 to −1.61, p = 0.028) compared with the period before mRVoh (−2.51 → –2.03, p = 0.575) after percutaneous intervention only. Conclusions: mRVoh in PAIVS patients was positively associated with TV annular growth, and it was more effective than percutaneous RVOT widening interventions without mRVoh.