Abnormalities in myocardial perfusion after surgical correction of pulmonary atresia with intact ventricular septum

We describe the results of myocardial perfusion scintigraphy performed 4 to 15 years after surgery in 12 patients with pulmonary atresia and intact ventricular septum. We used single photon emission computed tomography after injection of technetium Tc-99m tetrofosmin at submaximal exercise test. The patients, 7 girls and 5 boys, with a mean age of 11 years, and a range from 6 to 19 years, had either undergone biventricular repair, in 5 cases, or univentricular palliation in the remaining 7. This second group included 4 patients with ventriculo-coronary arterial communications. Of the children, 3 with biventricular repair and 6 with univentricular palliation had perfusion defects. Children with biventricular repair had perfusion defects in the ventricular septum, while those having univentricular palliation also had defects located to the left ventricular free wall. All children with ventriculo-coronary arterial communications had perfusion defects both in the ventricular septum and in the left ventricular free wall.     

Pulmonary atresia with intact ventricular septum percutaneous radiofrequency-assisted valvotomy and balloon dilation versus surgical valvotomy and Blalock Taussig shunt

OBJECTIVE: We compared the result of radiofrequency (RF)-assisted valvotomy and balloon dilation with closed surgical valvotomy and Blalock Taussig (BT) shunt as primary treatment in selected patients with pulmonary atresia and intact ventricular septum (PA-IVS). BACKGROUND: Patients with PA-IVS who have mild to moderate hypoplasia of the right ventricle (RV) and patent infundibulum have the greatest potential for complete biventricular circulation. The use of RF or laser wires to perforate the atretic valve followed by balloon dilation provides an alternative to surgery. METHODS: Between May 1990 and March 1998, 33 selected patients underwent either percutaneous RF valvotomy and balloon dilation (group 1, n = 21; two crossed over to group 2) or surgical valvotomy with concomitant BT shunt (group 2, n = 14). Second RV decompression by balloon dilation or right ventricular outflow tract (RVOT) reconstruction were performed if necessary. Patients who remained cyanosed were subjected to transcatheter trial closure of the interatrial communication. Partial biventricular repair was offered to those with inadequate growth of the RV. RESULTS: The primary procedure was successful in 19 patients in group 1. There was one in-hospital death and two late deaths. Of the remaining 16 survivors, 12 achieved complete biventricular circulation, 7 of whom required no further interventions. Two patients required repeat balloon dilation, 1 RVOT reconstruction and 2 transcatheter closure of interatrial communication. Two patients underwent partial biventricular repair. In group 2, there were 3 in-hospital deaths after the primary procedure and 1 patient died four months later. All survivors (n = 10) required a second RV decompression, 8 by balloon dilation and 2 by RVOT reconstruction, after which, two patients died. Of the final 8 survivors, 7 achieved complete biventricular circulation, 5 after coil occlusion of the BT shunt and 2 after closure of interatrial communication. CONCLUSIONS: Radiofrequency valvotomy and balloon dilation is more efficacious and safe compared with closed pulmonary valvotomy and BT shunt in selected patients with PA-IVS.     

Management algorithm in pulmonary atresia with intact ventricular septum.

Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable morphologic variability, affecting not only the pulmonary valve but also the tricuspid valve, the RV cavity and coronary arteries. With advances in interventional techniques and congenital heart surgery, the management of PAIVS continues to evolve. This review is an attempt at providing a practical approach to the management of this disease. The basis of our approach is morphologic classification as derived from echocardiography and angiography. Group A, patients with good sized RV and membranous atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often it is the only procedure required in this group with the most favourable outcome. Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts with single ventricle physiology. The treatment at presentation is patent ductus arteriosus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6-12 months later followed by Fontan completion after a suitable interval. Patients in Group B, the intermediate group, are those with borderline RV size, usually with attenuated trabecular component but well developed infundibulum. The treatment at presentation is RF valvotomy and PDA stenting +/- balloon atrial septostomy. Surgical re-interventions are not uncommonly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (11/2 - ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar obstruction or small pulmonary annulus. Catheter based interventions viz. repeat balloon dilatation or device closure of patent foramen ovale (PFO) may also be required in some patients.     

Stent Implantation of the Arterial Duct in Newborns with a Truly Duct‐Dependent Pulmonary Circulation: A Single‐Center Experience with Emphasis on Aspects of the Interventional Technique

Introduction: Ductal stenting for pulmonary blood supply in newborns with cyanotic congenital heart disease (CHD) might be a low risk and safe alternative to the surgical aorto‐to‐pulmonary artery (AP) shunt in dual‐source lung perfusion. Ductal stenting in truly duct‐dependent pulmonary circulation has not been evaluated. Methods: Prospective interventional and clinical follow‐up trial. Ductal stenting based on variable access sites, a 2‐wire technique when crossing a tortuous ductus, and use of premounted coronary stents. Primary outcome measures were procedural success and complication rates presented as early and mid‐term results. Results: From 2003–2009, 58 duct‐dependent newborns underwent ductal stenting; 27 of them were truly duct dependent, 20 had pulmonary atresia (PA)/ventricular septum defect or complex CHD, 4 had PA/intact ventricular septum, 2 had PA with Ebstein anomaly, and 1 had PA with tricuspid atresia. Ductal stenting was performed without procedure‐related mortality; 3 of 27 required an acute surgical AP‐shunt (stent migration in 1, acute duct obstruction in 2). During mid‐term follow‐up, 4 of 24 needed an AP‐shunt and two others stent redilation. Three patients died prior to follow‐up surgery (1 unexpectedly at home and 2 due to syndromatic disease). Fifteen patients received staged univentricular palliation, 8 had a biventricular repair, and 1 is awaiting follow‐up operation. Conclusion: Ductal stenting is a feasible, safe, and effective palliation in newborns with truly duct‐dependent pulmonary circulation irrespective of duct morphology. Vasucular access from various locations is important for technical success rate. Ductal stenting is a minimally invasive procedure to achieve adequate pulmonary artery growth for subsequent palliative or corrective surgery. (J Interven Cardiol 2010;23:581–588)     

Risk factors in the Glenn bidirectional shunt as an intermediate procedure before Fontan correction

OBJECTIVE: The bidirectional Glenn shunt is the most common palliation before the Fontan repair, especially in high-risk patients. We studied the influence of certain risk factors in bidirectional Glenn results, with and without an additional source of pulmonary blood flow. METHODS: Between 1993 and 1998 twenty patients (6-53 months of age) underwent a bidirectional Glenn shunt as the intermediate repair for the Fontan procedure. Diagnoses were: 7 cases of double inlet single ventricle, 4 of tricuspid atresia, 3 of unbalanced AV septal defect, 4 of mitral atresia, 1 hypoplastic left heart syndrome and 1 TGA with hypoplasia of the right ventricle. 17 patients had undergone previous operations. Mean preoperative arterial oxygen saturation was 78.5%. In 6 patients an auxiliary source of pulmonary blood flow was added. RESULTS: Hospital mortality was 15%. In 4 patients the bidirectional Glenn failed. By univariate analysis low weight, preoperative functional status and high pulmonary pressure were factors associated with early death. In bidirectional Glenn failure only the duration of ventilatory support was significant. By multivariate analysis, preoperative functional status and pulmonary pressure were significant. Mean postoperative arterial oxygen saturation at a mean follow-up of 10 months was 84%. CONCLUSIONS: Bidirectional Glenn shunt is an effective and low-risk palliation for patients with univentricular hearts. Only low weight and high pulmonary pressure were significant in hospital mortality, and we advise the association of an additional source of pulmonary blood flow in these patients at the start. Early extubation provides correct performance of the shunt.     

Transcatheter biventricular conversion in an adult patient with a 1.5 ventricle Glenn palliation and superior vena cava syndrome

Covered stents have a continually expanding spectrum of applications for patients with congenital heart disease. Here we report use of covered stents to successfully perform a first‐in‐human percutaneous biventricular conversion of a 1.5 ventricle Glenn palliation in an adult born with pulmonary atresia. This case demonstrates that in patients considered borderline for biventricular repair, surgery can potentially be modified to promote growth of underdeveloped structures and setup for transcatheter biventricular conversion.     

Outcome in infants less than 3 kilograms for placement of saphenous venous homografts as systemic-to-pulmonary arterial shunts

BACKGROUND: Establishing stable and adequate flow of blood to the lungs using a systemic-to-pulmonary arterial shunt in infants with low birth weight may involve significant morbidity and mortality. We reviewed our experience with this procedure in patients weighing less than 3 kilograms. METHODS: Between June, 2002, and June, 2007, we placed systemic-to-pulmonary arterial shunts in 32 infants weighing less than 3 kilograms, the range being 1.8 to 2.86 kg, with a median of 2.5 kg. The median age at placement of the shunt was 8 days, with a range from 2 to 70 days. In 17 patients (53%), the anatomic defects had produced a functionally univentricular heart, while 15 (47%) had defects which permitted staging to biventricular repair. Patients staged to univentricular palliation were much more likely to have a circulation dependent on the arterial duct as compared with those staged to biventricular palliation (p < 0.001). The latter patients tended to have smaller pulmonary arteries, significantly the left pulmonary artery, which has a median diameter of 3.6 versus 2.0 mm, p = 0.01. In all patients a saphenous venous homograft was used as the conduit, its size ranging in diameter from 2.5 to 4 mm, with a median of 3.0 mm. RESULTS: The overall hospital mortality rate for the entire cohort was 6.25%, with 2 patients dying. There was no significant difference between the two groups with regard to length of stay in intensive care or hospital. Follow-up has ranged from 3 months to 4.7 years, with a mean of 2.1 years). Of those with functionally univentricular hearts, 3 have subsequently died, along with 1 patient having a biventricular circulation (p = 0.3). All deaths occurred before takedown of the shunt. A trend toward longer survival was noted in those with biventricular as compared to functionally univentricular circulations (p = 0.06). CONCLUSION: Systemic-to-pulmonary arterial shunts can be constructed safely in infants with biventricular physiology born with low weight. Those having functionally univentricular circulations carry an increased rate of mortality for the period of shunting. Using the saphenous venous homograft permits use of smaller grafts, which does not significantly increase the risk for thrombosis or survival when compared to previous studies using polytetrafluoroethylene grafts.     

Formalin infiltration of ductus arteriosus in cyanotic congenital heart disease.

Formalin infiltration of the ductus arteriosus was performed in 13 neonates with pulmonary atresia (three with ventricular septal defect, two with tricuspid atresia, and eight with intact ventricular septum, one of whom had Ebstein's anomaly) in an attempt to maintain duct patency. Nine patients had an additional retrograde pulmonary valvotomy and one a Blalock-Taussig shunt. The mean preoperative systemic PO2 was 31 mmHg and rose significantly to 46 mmHg after operation. However, the PO2 rose in only two of the four patients who had formalin infiltration alone. There were five early deaths and three had clinical evidence of duct closure. Four patients needed further surgery (systemic-pulmonary artery shunt) within eight days. Seven of eight surviving patients were shown to have a closed duct at repeat cardiac catheterisation (two to 13 months after operation). The eighth died before restudy. There were four late deaths, one following late reoperation and the other three within five months of early reoperation. These results suggest that formalin infiltration cannot ensure long-term duct patency. As short-term patency can be maintained more reliably by prostaglandin administration, we have abandoned the operation.     

Determinants of exercise function following univentricular versus biventricular repair for pulmonary atresia/intact ventricular septum

This study aimed to determine whether the exercise capacity of patients with pulmonary atresia/intact ventricular septum (PA/IVS) who have undergone biventricular repair is superior to that of patients with single ventricle repairs and to account for any differences. PA/IVS is generally treated with either biventricular (outflow tract reconstruction) or univentricular (Fontan) palliation. Although biventricular repair is believed to result in superior exercise function, this theory is untested. Symptom-limited programmed bicycle ergonometry with expiratory gas analysis was prospectively performed on all patients with PA/IVS >7 years old seen over 18 months. Nineteen biventricular and 10 Fontan patients (mean age 16.5 +/- 6.5 vs 12.7 +/- 5.0 years, p = 0.12) were enrolled. The exercise capacity of biventricular patients was not statistically superior to that of Fontan patients (predicted peak VO2 83.5 +/- 21% vs 76.0 +/- 17.5%, p = 0.34), although chronotropic function and ventilatory efficiency were significantly better in the former. The peak exercise capacity varied widely within each group, and there was considerable overlap between biventricular and Fontan patients. Within groups, imaging studies did not reliably predict exercise capacity. Most patients in each group had subnormal peak VO2, and there was a trend toward impaired performance with increasing age regardless of type of repair. In conclusion, biventricular repair may not guarantee superior exercise performance over single-ventricle palliation in PA/IVS. Regardless of repair type, aerobic capacity may deteriorate with age and is not reliably predicted by noninvasive imaging. These findings underscore the need for a quantitative, proactive approach to the assessment and preservation of exercise function.     

Muscular Infundibular Atresia Is Associated with Coronary Ostial Atresia in Pulmonary Atresia with Intact Ventricular Septum

Objective. To determine whether infundibular morphology is associated with coronary ostial atresia and/or right ventricle dependent coronary circulation in patients with pulmonary atresia with intact ventricular septum. Design. Neonatal echocardiograms of patients with pulmonary atresia with intact ventricular septum were evaluated for tricuspid valve size and infundibular anatomy (muscular pulmonary atresia vs. membranous pulmonary atresia). Right ventricle dependent coronary circulation and coronary ostial atresia were diagnosed angiographically. Medical record review determined patient outcome. Setting. Tertiary‐care pediatric hospital. Results. Of 72 patients, 24 had muscular pulmonary atresia including 23 with right ventricle dependent coronary circulation. Fourteen of these had unilateral or bilateral coronary ostial atresia. Of 46 patients with membranous pulmonary atresia, 5 five had right ventricle dependent coronary circulation and none had coronary ostial atresia. Muscular pulmonary atresia patients had smaller tricuspid valve z‐scores and were less likely to achieve a biventricular repair than those with membranous pulmonary atresia (P < .01). Muscular pulmonary atresia had 82% sensitivity, 98% specificity, and 96% positive predictive value for presence of right ventricle dependent coronary circulation. In the group with membranous pulmonary atresia, there were two deaths, no transplants, and 23 (48%) achieved a biventricular repair. In contrast, of the 24 with muscular pulmonary atresia, there were seven deaths, two transplants, and no biventricular repairs. Conclusions. In this cohort, muscular pulmonary atresia was strongly associated with right ventricle dependent coronary circulation and coronary ostial atresia, and appears to be a useful morphologic marker for poor outcome among pulmonary atresia with intact ventricular septum patients. This information may be useful during prenatal counseling and for presurgical evaluation.     

Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum

Objectives. This study reviewed our experience with percutaneous balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum and defined the anatomic and hemodynamic characteristics of infants in whom this procedure is successful and provides definitive therapy.Background. Unlike children with valvular pulmonary stenosis, the follow-up of infants with critical pulmonary stenosis undergoing percutaneous balloon valvotomy is limited.Methods. Between December 1987 and August 1992, percutaneous balloon valvotomy was attempted in 12 infants with critical pulmonary stenosis (n = 10) or pulmonary atresia with intact ventricular septum (n = 2). Two outcome groups were identified: Group A patients are acyanotic, have mild residual pulmonary stenosis and have not required operation; Group B patients have required operation.Results. Of the 12 infants, 11 had a successful balloon valvotomy procedure. Group A patients (n = 7) have a residual gradient of 22 ± 18.7 mm Hg (mean ± SD) at follow-up of 3.2 years (range 1.2 to 5.0). In Group B (n = 5), operation was required for inability to cross the pulmonary valve (n = 1) or persistent severe hypoxemia for ⪰2 weeks after valvotomy (n = 4). Significant differences (p ≤0.01) between the two groups (Group A vs. Group B) were identified in pulmonary valve annulus (Z value) 8.1 mm (−1.1) versus 5.5 mm (−3.4); tricuspid valve annulus (Z value) 14.0 mm (0.8) versus 8.8 mm (−1.8); right ventricular volume 65 versus 29 ml/m²; and Lewis index 10.9 versus 8.9.Conclusions. Percutaneous balloon valvotomy is effective and likely to provide definitive therapy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum who have a tricuspid valve annulus >11 mm, pulmonary valve annulus ⪰7 mm and right ventricular volume >30 ml/m².     

Balloon pulmonary valvotomy: palliation for cyanotic heart disease

Balloon pulmonary valvotomy was attempted in eight children with cyanotic congenital heart disease and pulmonic stenosis who were scheduled for a palliative procedure (shunt). In seven patients the balloon could be positioned across the pulmonary anulus, and valvotomy was performed. Five of the patients had tetralogy of Fallot with multiple levels of pulmonary outflow obstruction. For all patients in whom the balloon could be properly positioned the valvotomy was successful, as judged by arterial hemoglobin saturation, which increased from 72% +/- 5% to 83% +/- 5% (p less than .005). Valvotomy was followed by an immediate rise in mean pulmonary artery pressure (12.6 +/- 2.8 to 18.3 +/- 4.8 mm Hg, p less than 0.05) and a decline in hematocrit level at 2 months (54% +/- 5% to 47% +/- 4%, p less than 0.05). There was no mortality or complication from the valvotomy, and the need for a systemic to pulmonary artery shunt was eliminated in six of eight patients. Follow-up has ranged from 0.5 to 2.8 years. This trial indicates that balloon pulmonary valvotomy can be safely performed and is effective palliation in selected patients with cyanotic heart disease that is not suitable for primary repair. The increased pulmonary flow may improve oxygenation and growth of the pulmonary arteries without the need of a systemic to pulmonary artery shunt.     

Pulmonary valve atresia with intact interventricular septum. Mid-term results of the surgical treatment. Apropos of 45 cases]

Between 1980 and 1991, 45 patients with pulmonary atresia with intact interventricular septum (38 cases) or critical pulmonary stenosis (7 cases), underwent surgery. The right ventricle was tripartite in 23 children, bipartite in 12 and unipartite in 10. Sinusoid vessels were present in 20 cases. They reinjected a native coronary trunk in 10 cases (major sinusoids). During the initial operation performed in the neonatal period in 41 cases and later in 4 cases, 26 children had an isolated systemic-pulmonary shunt (3 deaths) and 19 had connection of the right ventricle and pulmonary artery (6 deaths). Perioperative mortality was 20% (9/45). Global actuarial survival at 5 years was 47%. Of the 22 patients referred later for univentricular repair, 8 (36%) died (6 perioperative and 2 sudden deaths). The actuarial survival for this group was 49% at 4 years. Of the 23 patients referred for biventricular repair, 11 (47%) died (9 perioperative and 2 sudden deaths) and 2 attained complete cure status. The actuarial survival is 43% at 4 years in this group. Children with intermediate forms (type II or tricuspid less than 8 mm) were orientated to univentricular repair, partly due to the high incidence of major sinusoids. None of the children with major sinusoids survived to over 3 years of age. When the anatomic form is favorable, the present strategy is to perform transpulmonary valvotomy under cardiopulmonary bypass associated with the implantation of a Gore-Tex tube between the innominate artery and right pulmonary artery during the neonatal period. The risk of secondary pulmonary regurgitation would seem to be less than the immediate risk of low pulmonary flow.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

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.     

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.     

A better approach for left ventricular training in transposition of the great arteries and intact interventricular septum: Bidirectional cavopulmonary anastomosis and pulmonary artery banding

Objective: Management of the patients with transposition of the great arteries and intact ventricular septum may be challenging beyond the newborn period. Herein, we would like to present our alternative strategy for training the left ventricle in these patients.
Methods: Six patients with transposition of the great arteries and intact ventricular septum were evaluated in our clinic. Two of them were palliated with Glenn procedure and pulmonary banding as a definitive treatment strategy at other centers. Four patients were operated on and a bidirectional cavopulmonary anastomosis in combination with pulmonary artery banding was performed (stage‐1: palliation and ventricular training) in our center. In four out of these six patients, arterial switch operation was performed with takedown and direct re‐anastomosis of the superior vena cava to right atrium after an interstage period of 21‐30 months (stage‐2: anatomical repair).
Results: Any mortality was not encountered. The left ventricular mass indices increased from 18‐32 to 44‐74 g/m2 in patients undergoing the anatomical repair. All of the patients were uneventfully discharged following the second stage. The mean follow‐up period was 20 months (9‐32 months) following stage 2. All of the patients are doing well with trivial neoaortic regurgitation and normal biventricular function.
Conclusions: Bidirectional cavopulmonary anastomosis with pulmonary artery banding may be a promising left ventricle training approach in ventriculoarterial discordance when compared to the traditional pulmonary artery banding with concomitant systemic‐to‐pulmonary artery shunt procedures which still carry a significant interstage morbidity and mortality.     

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。     

Quality of life in children with pulmonary atresia and intact ventricular septum

OBJECTIVE: To measure quality of life in children with pulmonary atresia and intact ventricular septum, and make comparisons with a healthy group of children from the general Swedish population. An additional aim was to compare quality of life in two subgroups, children undergoing biventricular and univentricular repair, the latter by establishment of the Fontan circulation. METHODS: Quality of life was measured using a mailed questionnaire, taking into consideration the three spheres of personal, interpersonal, and external living conditions. In all, 52 children received the questionnaire, and the answers were compared with those of a random sample of 1856 healthy Swedish children. RESULTS: We received responses from 42 children and parents. The overall differences between groups were small, and no difference was found when comparing the children undergoing univentricular as opposed to biventricular surgical repair. In terms of personal quality of life, we found a higher level of psychosomatic complaints, and lower satisfaction with their own and parent-child activities, in those with the congenital anomaly than in their normal peers. In terms of the interpersonal sphere, more time was available for those with malformed hearts, but the experience of support from the relatives was significantly lower. CONCLUSIONS: Overall, quality of life was equal between children with pulmonary atresia and intact ventricular septum and the children in the reference group. The personal psychological conditions were less favourable for children with pulmonary atresia and intact ventricular septum. The perceived lack of support from relatives was also higher in these families. No difference was found when comparing the children undergoing univentricular and biventricular surgical repair.     

Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients

OBJECTIVES: We sought to determine trends, and outcomes, for a cohort of patients with pulmonary atresia with intact ventricular septum born between 1965 and 1998. BACKGROUND: Pulmonary atresia with intact ventricular septum is a complex lesion that remains a therapeutic challenge, particularly regarding the suitability for biventricular repair. METHODS: We identified 210 consecutive patients, and reviewed their medical records, initial angiograms, and echocardiograms, along with the relevant surgical and pathology reports. RESULTS: The mean initial Z-score for the diameter of the tricuspid valve was -0.99 +/- 1.95, with Ebstein's malformation in 8%. A right ventricular dependent coronary arterial circulation was found in 23%. The proportion of patients who received treatment increased over time, although placement of an arterial shunt was the predominant initial procedure throughout the experience. At the last follow-up, 107 patients had not reached the planned final stage of their repair, and 79% of these had died. Of the 103 reaching the final stage of planned repair, 58 had undergone attempted biventricular repair, with 34% dying; 14 had undergone attempted one and a half ventricular repair, with 7% dying, and 31 had undergone attempted functionally univentricular repair, with 10% dying. Overall, survival was 57% at the age of 1 year, 48% at 5 years, and 43% at 10 years. Survival improved over time, with survival of 75% at 1 year, and 67% at 5 years, for patients born between 1992 and 1998. An earlier date of birth, the presence of Ebstein's malformation, and prematurity were all significant independent factors associated with decreased survival. A greater severity of coronary arterial abnormalities was significantly associated with a greater likelihood of left ventricular dysfunction during follow-up. CONCLUSIONS: The outcomes for patients born with pulmonary atresia with intact ventricular septum have improved over time, albeit that careful initial management, and better selection, is still indicated for those planned to undergo biventricular repair.