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.     

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.     

The prevalence of coronary arterial abnormalities in pulmonary atresia with intact ventricular septum and their influence on surgical results

BACKGROUND: The relatively high mortality in patients with pulmonary atresia and intact ventricular septum may be related to the presence of significant coronary arterial anomalies. This retrospective review of cineangiocardiograms was undertaken to further elucidate the types and variety of such coronary arterial abnormalities, and to assess their effect on postoperative survival. MATERIAL AND RESULTS: Details regarding coronary arterial anatomy and abnormalities were assessed in 116 patients. We noted the site and severity of lesions, and the presence of fistulous communications from the right ventricle to the coronary arteries, assessing the proportion of left ventricular myocardium affected by coronary arterial interruptions or significant stenoses, in other words, the amount dependent on coronary circulation from the right ventricle. We also measured diameters of the tricuspid and mitral valves. Fistulas were found in 87 patients (75%), interruptions of major coronary arteries in 40 patients (34%), lack of connections between the coronary arteries and the aorta in 18 patients (16%), and single origin of a coronary artery, with the right coronary artery arising from the left, in 6 patients (5%). We found increased mortality in 47 patients (40%) who had a right ventricular-dependent coronary arterial circulation. The presence of fistulas in itself was not associated with higher mortality, but the presence of coronary arterial interruptions (p = 0.05), and a higher myocardial score (p = 0.0009), were. CONCLUSION: We encountered a higher prevalence of both coronary arterial abnormalities and right ventricular-dependent circulation than previously reported. Awareness of the severity of the coronary arterial abnormalities should assist in planning treatment.     

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.     

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.     

Guided transcatheter valvulotomy in pulmonary atresia with intact ventricular septum

Transcatheter valvulotomy in pulmonary atresia with an intact ventricular septum can be used as a first step to create biventricular circulation and to stimulate further development of the hypoplastic right ventricle. We describe our experience in a case of a neonate with this congenital cardiac defect who underwent successful transcatheter perforation of the atretic pulmonary valve. This report highlights the utility of a special technique based on the use of a gooseneck snare positioned just above the atretic valve to guide the advance of a coronary guidewire. Other therapeutic alternatives are considered.     

Echocardiographic Evaluation of Adult Postoperative Congenital Heart Disease

Diagnostic and surgical advances have resulted in a large and growing population of adult patients with congenital heart disease. Proper care of these individuals requires knowledge of the original defects as well as the multitude of variations on surgical intervention for those lesions. Most complex congenital cardiac malformations amenable to "biventricular repair" are cyanotic and include a mixture of septal defects, obstructive and regurgitant valvular, subvalvular, or supravalvular lesions, and malposition of the cardiac chambers and great arteries. Whether they reach adulthood unoperated largely depends upon the balance of pulmonary blood flow. Examples of anatomical substrates that are usually candidates for "biventricular repair" include conotruncal abnormalities such as tetralogy of Fallot, transposition complexes of the great arteries, double-outlet right or left ventricle, and truncus arteriosus. "Biventricular repair" results in two well formed functional ventricles with a subpulmonic ventricle and a subaortic ventricle that are able to provide a circulation in series without admixture of venous and systemic blood. A unifying theme includes patch closure of a ventricular septal defect and reconstitution of ventricular-to-pulmonary arterial blood flow.     

合并冠状动脉畸形法洛四联症的外科治疗

目的总结合并畸形冠状动脉的法洛四联症手术治疗经验,探讨有关技术的改进。方法自2002年1月至2007年12月共完成该类手术18例,其中经右房、肺动脉切口疏通右室流出道7例,改变右室切口4例,主肺动脉移位法2例,双流出道法2例,游离牵开左前降支（LAD）疏通加宽右室流出道（RVOT）2例,1个半心室矫治1例。结果无手术死亡,随访8～82个月,无晚期死亡及并发症发生。结论术中根据右室流出道狭窄程度和畸形冠状动脉走行争取个性化手术方式,疗效满意。     

The double switch procedure for anatomical repair of congenitally corrected transposition of the great arteries in infants and children

AIMS: To assess outcomes of anatomical repair (double switch procedure)in infants and children with congenitally corrected transpositionof the great arteries. METHODS AND RESULTS: Between September 1993 and August 1996, 17 patients with congenitallycorrected transposition underwent surgery at UCSF. Anatomicalrepair was performed in 11 of these patients, at ages rangingfrom 4·8 months to 7·8 years (median 3·2years). The remaining six patients did not undergo anatomicalrepair due to unfavourable anatomy (n=2), prior conduit repair(n=2), biventricular dysfunction (n=1), and isolated completeatrioventricular block (n=1). The 11 patients who underwentanatomical repair make up the study group for the present report.All 11 patients had a malalignment ventricular septal defect,while pulmonary outflow tract obstruction was present in ninepatients and significant tricuspid valve pathology or dysfunctionwas present in five. Anatomical repair was achieved with a Senning(n=7) or a Mustard (n=4) procedure combined with an arterialswitch operation plus ventricular septal defect closure (n=4),or a Rastelli procedure with left ventricle to aortic baffleand right ventricle to pulmonary artery conduit (n=7). Therewas one early death and no patients developed surgical completeatrioventricular block. At a median follow-up of 22 months,there were no late deaths. Two patients required a total ofthree late reoperations, and all patients were asymptomaticon no cardiac medication. Follow-up echocardiography revealednormal biventricular function in all patients. CONCLUSIONS: Anatomical repair of corrected transposition can be achievedwith low rates of early mortality and surgical heart block,and favourable mid-term results. Long-term follow-up will benecessary to determine if the double switch approach improvesthe natural history of corrected transposition when comparedto less aggressive surgical approaches that leave the rightventricle in the systemic circulation.     

Acquired ventricular septal aneurysm in a patient with pulmonary atresia with intact ventricular septum

Myocardial ischaemia and infarction in pulmonary atresia and intact ventricular septum with right ventricular-dependent coronary circulation is a well-established complication. We report an interesting case of an acquired aneurysm in the ventricular septum in a patient who underwent staged palliation.     

A most peculiar coronary circulation in a patient with pulmonary atresia and intact ventricular septum

A patient with pulmonary atresia and intact ventricular septum was found to have a right ventricular-dependent coronary circulation. In this infant both coronary arteries lacked their normal proximal connection with the aorta, perhaps the most egregious form of this prejudicial coronary circulation. Even more interesting was a direct collateral vessel originating from the descending thoracic aorta and connecting with the coronary circulation. This patient has undergone bilateral modified Blalock-Taussig shunts, and left ventricular function seems preserved.     

Double outlet right ventricle: Opinions regarding management

Optional statement In the current era of superb surgical results for congenital heart disease, several management options are available for patients born with double outlet right ventricle (DORV). The surgical repair of DORV is tailored to address the variety of abnormalities associated with this lesion. The treatment strategies are dependent upon the anatomy and relationship of the ventricular septal defect (VSD) and the great arteries. For patients with subaortic or doubly committed VSDs, without right ventricular outflow tract obstruction, the usual repair is an intraventricular tunnel from the VSD to the aorta. If right ventricular outflow tract obstruction exists, then augmentation of the right ventricular outflow tract or conduit placement from the right ventricle to the pulmonary artery is necessary. For the “Taussig-Bing anomaly” (subpulmonary VSD) an arterial switch operation is indicated with tunneling of the VSD to the neoaorta. For patients with a remote VSD, complex atrioventricular valve abnormalities, or unbalanced ventricles, a staged palliative approach is warranted. This approach will result in the patient having “single ventricle” physiology, and “Fontan anatomy” after the staging surgeries. Outcomes are determined by the surgery performed. Patients who require conduit placement from right ventricle to pulmonary artery will need subsequent surgery for replacement of the conduit. Complex intraventricular tunnels are at risk for developing subaortic obstruction. Patients who had arterial switch operations have thus far had excellent results. However, these patients may be at risk for development of neoaortic valve regurgitation requiring subsequent reoperation. Complex biventricular intracardiac repairs have been associated with a higher risk of reoperation. In the past 10 to 15 years, patients undergoing Fontan operations have had improved early and late survival. However, long-term problems after the Fontan operation include arrhythmia and development of protein-losing enteropathy.     

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.     

Repair of double outlet right ventricle with doubly-committed ventricular septal defect

OBJECTIVE: To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect. METHODS: We undertook repair in 8 patients with this particular feature. Of these, 2 patients had pulmonary stenosis, and another had interruption of the aortic arch. The subarterial defect was unequivocally related to both the aortic and the-pulmonary orifices in all, albeit slightly deviated towards the aortic orifice in one, and towards the pulmonary orifice in another. Intraventricular rerouting was carried out via incisions to the right atrium and the pulmonary trunk. To ensure reconstruction of an unobstructed pulmonary pathway, a limited right ventriculotomy was made in 5. RESULTS: All patients survived the procedure, and are currently doing well, with follow-up of 25 to 194 months, with a mean of 117+/-68 months. Catheterization carried out 16+/-6 months after repair demonstrated excellent ventricular parameters. Mean pulmonary arterial pressure was 16+/-7 mmHg, being higher than 20 mmHg in 2 patients. No significant obstruction was found between the right ventricle and the pulmonary arteries. A pressure gradient across the left ventricular outflow tract became significant in one patient in whom a small outlet septum was present, and a heart-shaped baffle had been used for intraventricular rerouting. Reoperation was eventually needed in this patient for treatment of the obstruction, which proved to be progressive. CONCLUSION: Precise recognition of the morphologic features is of paramount importance when choosing the optimal options for biventricular repair in patients with double outlet right ventricle and doubly-committed interventricular communication.     

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.     

Longevity of Neonatal Ductal Stenting for Congenital Heart Diseases with Duct‐dependent Pulmonary Circulation

Introduction. Ductal stent (DS) in duct‐dependent pulmonary circulation is less morbid than neonatal Blalock–Taussig shunt. However, there is concern if DS provides an adequately long palliation before definitive repair. Methods. This is a retrospective review of clinical follow‐up of all consecutive infants after successful DS performed by a single operator. They were divided into three anatomic groups. Group A neonates had balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum, who needed DS patency until the right ventricle was adequate to provide antegrade pulmonary flows. Group B patients with tetralogy of Fallot and pulmonary atresia suited for later biventricular repair needed ductal patency until conduit surgery was completed. Group C patients with functionally univentricular hearts needed DS patency until bidirectional Glenn shunt completion. Results. Among 22 infants, four Group A patients followed for 26–54 months after balloon pulmonary valvotomy had adequate oxygen saturation and needed only short‐term DS patency. In six out of nine Group B patients, corrective biventricular repair using conduits was performed after 5–14 months at a body weight of 5–7.5 kg. Bidirectional Glenn shunt and confluence repair were performed in seven of nine Group C patients weighing 6–8.5 kg after 8–15 months. The hilar pulmonary artery growth in B and C groups was adequate for surgical repair. No patient needed stent redilatations or additional shunts on follow‐up for hypoxia. Four patients had sudden death. Conclusions. The short‐term patency of DS was adequate after balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Duration of palliation by DS was also sufficient in univentricular hearts to allow adequate somatic growth before Glenn surgery. In patients with biventricular anatomy treated by DS, conduit repair had to be performed at a relatively early age. Interstage mortality was 18%.     

Subaortic obstruction after the use of an intracardiac baffle to tunnel the left ventricle to the aorta.

Postoperative hemodynamic studies in five patients document subaortic obstruction after surgical repair utilizing an intracardiac baffle to establish continuity between the left ventricle and the aorta. Four of the patients had a Rastelli procedure for D-transposition of the great arteries with a ventricular septal defect and pulmonary stenosis; one patient had repair of double outlet right ventricle with a ventricular septal defect and pulmonary stenosis. The left ventricular outflow was shown to be a long narrow tunnel by angiography in four of five patients and by echocardiography in one patient. Resting aortic peak systolic pressure gradient ranged from 10 to 42 mm Hg (mean 24). The obstruction was localized to the proximal end of the left ventricule to aorta tunnel (i.e., at the site of ventricular septal defect) in five patients. One patient with a gradient of 42 mm Hg has angina and decreased exercise tolerance. Subaortic obstruction is a newly described sequelae after the Rastelli procedure for transposition or repair of double outlet right ventricle. The obstruction may be hemodynamically significant and should be searched for at postoperative cardiac catheterization.     

Concepts in assisted circulation

Assisted circulation by extracorporeal and extracardiac bypass techniques must be based on the requirements of the heart and of the total body, though these may differ. The cardiac problem in cardiogenic shock is more likely to be a biventricular problem demanding decompression of both sides. Extra pulmonary oxygenation should be avoided because of complexity in long-term use. Principles of assisted circulation may be applied in an extra-thoracic temporary manner or as an intracorporeal long-term device without removal of the heart. We have compared a number of extracorporeal mechanical bypass techniques (left ventricular bypass, left atrial bypass, right atrial plus left ventrical bypass, and right atrial plus left atrial bypass) in dogs at different flow rates with control groups (nitroprusside, metabolic substrates, and no therapy) for survival during and after 4-hour treatment periods in a standard severe myocardial infarction preparation with biventricular failure. The left ventricle was cannulated in a retrograde manner. Right atrial bypass was mixed with oxygenated left ventricular blood before return without oxygenation. Results in 50 dogs revealed that without treatment 20% survived; left ventricular plus right atrial bypass provided 100% survival during the 4-hour treatment, whereas left ventricular bypass and nitroprusside were intermediate with approximately 70% survival. Respiration and acid-base balance were not significantly altered by right atrial bypass, which provided adequate decompression of the right ventricle. Flow rates of 50% of control levels were adequate to provide normal or increased peripheral circulation as well as decompression of both ventricles and maintenance of sinus or nodal rhythm. Left atrial or left ventricular bypass alone was unable to support the heart or circulation in severe failure. Hemodynamics, including total body perfusion, atrial pressures, and dP/dt were improved significantly only by left ventricular plus right atrial bypass, even though reduced rates of flow were used. Similar findings were seen in lactic acid, CPK, and oxygen consumption. The addition of metabolic substrates currently does not improve survival though they appear to act as metabolic modulators. We conclude that the principle of a low flow left ventricular plus right atrial bypass without an oxygenator offers a technique for long- or short-term assistance.     

A Rare Case of Concordant Atrioventricular Connection to L-Looped Ventricles in Situs Solitus: 4-Dimensional Magnetic Resonance Imaging and 3D Printing

An infant male presented with the rare anatomy consisting of situs solitus, concordant atrioventricular connections to L-looped ventricles, double outlet right ventricle (DORV), and hypoplastic aortic arch. 6 months after neonatal aortic arch repair, the morphologic right ventricle function deteriorated, and surgical evaluation was undertaken to determine if either biventricular repair with a systemic morphologic left ventricle or right ventricular exclusion was possible. After initial echocardiography, magnetic resonance imaging (MRI) was used to create detailed axial and 4-dimensional (4D) images and 3-dimensional (3D) printed models. The detailed anatomy of this rare, complex case and its use in pre-surgical planning is presented.     

三尖瓣下移畸形的外科治疗(附24例报告)

手术纠治三尖瓣下移畸形24例，死亡4例（16．67％）。合并畸形或病变有房、室间隔缺损、预激综合征、心包炎。行房化心室折叠术18例，三尖瓣替换术6例。作者认为随着手术方法、心肌保护和体外循环技术不断改进，手术指征应较过去放宽。手术治疗的关键是房化心室的折叠和三尖瓣关闭不全的纠正。术中注意避免损伤传导束和冠状血管。对功能右心室极小的重型三尖瓣下移畸形，术后易发生难以纠正的右心衰竭，死亡率高，以选择改良Fontan手术或全腔静脉与肺动脉连接术为宜。行三尖瓣替换的人工瓣膜以选择生物辩为宜。