Radiofrequency perforation of the right ventricular outflow tract as a palliative strategy for pulmonary atresia with ventricular septal defect.

BACKGROUND: Radiofrequency perforation (RF) of the right ventricular outflow tract (RVOT), while an effective management strategy in children with an intact ventricular septum, has not been fully detailed in those presenting with a ventricular septal defect. OBJECTIVE: To determine whether transcatheter perforation of the atretic pulmonary valve is an acceptable management strategy prior to surgical repair. RESULTS: Valve perforation was attempted in eight children seen between May 2000 and March 2006, five being infants between 1 and 9 days of age. In five children, this was the first of two procedures, the second a planned surgical correction. The RF was successful in six children with one child requiring additional stenting of the RVOT. Of these children, three attained a biventricular repair within the next year without additional palliative surgical procedures. Of the remaining three patients, one is awaiting surgical correction, one did not require further surgery, and one had this procedure as the only planned palliation. The two children in whom RF was not possible were referred for surgical augmentation of pulmonary blood flow. CONCLUSION: A treatment strategy that includes pulmonary valve perforation as initial palliation to increase pulmonary blood flow may be effective. Additional experience to better define those children who would benefit from this treatment algorithm is required.     

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.     

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).

Transarterial pulmonary venous wedge arteriography in pulmonary atresia,ventricular septal defect,and intact atrial septum

Transarterial pulmonary venous wedge arteriography was performed in four cases of pulmonary atresia (PA), ventricular septal defect (VSD), and intact atrial septum. In two cases, only the left pulmonary artery was visualized by selective injection to the patent ductus arteriosus (PDA) or Blalock-Taussig anastomosis (B-T shunt), and the right pulmonary artery was opacified by transarterial retrograde pulmonary arteriography. In two other cases, with nonfunctioning B-T shunt, the pulmonary artery was not opacified by either aortography nor by injection into the B-T shunt. Transarterial pulmonary vein wedge arteriography visualized a confluent pulmonary artery. Transarterial pulmonary vein wedge arteriography was a useful and safe procedure for opacifying the pulmonary artery that could not be visualized antegradely in VSD, PA, and intact atrial septum.     

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.     

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..     

Transcatheter retrograde radio-frequency perforation of the pulmonic valve in pulmonary atresia with intact ventricular septum,using a 2 French catheter

A neonate with pulmonary atresia with intact ventricular septum (PA/IVS) underwent successful retrograde transcatheter perforation of the pulmonary valve, using a 2 Fr radio-frequency catheter with subsequent anterograde balloon dilation of the valve. Due to persistent hypoxemia, the neonate underwent placement of a 4-mm modified Blalock-Taussig shunt. Transcatheter retrograde transductal perforation of the pulmonic valve in PA/IVS is feasible and may be a potential alternative to anterograde perforation. Cathet. Cardiovasc. Diagn. 45:151–154, 1998. © 1998 Wiley-Liss, Inc.     

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.     

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.     

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     

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。     

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.     

Percutaneous pulmonary valve implantation in the native right ventricular outflow tract

Percutaneous pulmonary valve implantation into dysfunctional right ventricular (RV) to pulmonary artery conduits is being increasingly performed in many European and North American centers with satisfactory results and low‐complication rates. We report the first application of this elaborate technique in the native RV outflow tract of a young patient who developed severe supravalvular pulmonary stenosis following an arterial switch operation for transposition of the great arteries. The procedure may be used as an alternative to surgical repair for the treatment of selected patients with supravalvular pulmonary stenosis complicating congenital heart surgery. © 2011 Wiley Periodicals, Inc.     

Radiofrequency perforation for pulmonary atresia and intact ventricular septum

Surgical or interventional perforation of atretic pulmonary valve as initial palliative procedure has been advocated in patients with pulmonary atresia with an intact ventricular septum. We report two infants with pulmonary atresia and intact ventricular septum, who were treated by radiofrequency perforation and balloon dilatation.     

Transcatheter guidewire perforation of the pulmonary valve as a palliative procedure in pulmonary atresia with intact interventricular septum

We report two cases of pulmonary atresia with intact interventricular septum in whom we successfully perforated and performed balloon dilation of the atretic pulmonary valve using a simple guidewire technique. The technical challenges of performing this procedure in small infants are highlighted.     

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。     

Percutaneous pulmonary valve implantation for reconstruction of a patch‐repaired right ventricular outflow tract

Percutaneous pulmonary valve implantation (PPVI) is nowadays an accepted treatment option to repair post‐surgical conduit dysfunction of the right ventricular outflow tract (RVOT). In addition, many patients need a pulmonary valve to reconstruct a hemodynamically incompetent native or conduit free outflow tract. Based on our experience with percutaneous stent‐valve placement in a cohort of 125 patients, we report here transvenous reconstruction of a conduit‐free, patch repaired outflow tract by utilizing balloon‐expandable stent‐valves in 23 patients with a median age of 22 years (5‐60 years). In 20 patients, the step‐by‐step procedure was performed uneventful with the aimed success. Severe RVOT dysfunction in term of a clinical relevant regurgitation could be changed to mild, as it was confirmed by follow‐up color Doppler echocardiography. In a 5‐year‐old girl a Melody® valve was placed as a surgical‐interventional hybrid approach. In one patient, the procedure was complicated by stent embolization during preparation of the RVOT for stent‐valve implantation. Reposition of the embolized stent was nevertheless successful for finishing percutaneous valve‐implantation. In one patient, surgical approach became necessary because of the inability to advance the balloon‐mounted stent‐valve through a pre‐stented RVOT. Considering the current available balloon‐expandable stent‐valves, transvenous pulmonary valve implantation is feasible to treat even an incompetent conduit‐free RVOT. However, preparation of the RVOT by pre‐stenting, in most patients with more than two stents in telescope technique remains challenging. Reconstruction of RVOT by the current available valves is promising only for a carefully selected group of patients.