Pulmonary atresia with intact ventricular septum

Neonates with pulmonary atresia with intact ventricular septum are ductus dependent from birth. The initial approach in these patients is to ensure continued adequate pulmonary blood flow in anticipation of imminent spontaneous closure of the ductus. Our experience and evolving approach in the management of 16 consecutive neonates with pulmonary atresia with intact ventricular septum from 1978 to 1984 is presented. On the basis of the revised classification by Bull and associates, we divided the patients into four different groups according to right ventricular morphology. Basically, three types of palliative surgical procedures were performed: systemic-pulmonary artery shunt; systemic-pulmonary artery shunt with pulmonary valvotomy; and pulmonary valvotomy alone. Systemic-pulmonary artery shunt is the most important part of neonatal palliation. Our present routine is to perform left subclavian-main pulmonary artery shunt with a polytetrafluoroethylene tube in all patients and, in addition, to perform an open transpulmonary valvotomy without cardiopulmonary bypass in patients with a patent infundibular portion of the right ventricle. Our technique and the advantages of this type of shunt procedure are discussed. Patients with right ventricular sinusoidal-coronary artery communications are a subgroup with an additional problem. In our series, patients with this anomaly are categorized into groups with major and minor connections and their surgical significance is discussed. Overall, there was one death among 16 patients after neonatal palliative procedures and one death among five patients after hemodynamic repair.     

Tricuspid valve avulsion or excision for right ventricular decompression in pulmonary atresia with intact ventricular septum

Retrograde right ventricular decompression through the tricuspid valve, by damaging or excising the valve, was attempted in five patients with pulmonary atresia, intact ventricular septum, and severely hypoplastic right ventricle. Two patients were neonates in critical condition, one of whom had received previous palliation with a shunt; the other neonate had received pulmonary valvotomy plus shunt followed by repeat valvotomy plus shunt ligation. Three patients were infants or children who had undergone placement of isolated neonatal shunts. One procedure was performed during cardiac catheterization. Three patients survived. In one, tricuspid avulsion and a Rashkind septostomy were done by percutaneous methods; in two patients, tricuspid excision plus bidirectional cavopulmonary anastomosis was done by open operation. At restudy, all showed subsystemic pressure in the right ventricle and diminution or disappearance of sinusoids. One also showed improvement of left ventricular function. Incorporation of a small right ventricle in the right atrium at the time of the two orthoterminal palliations seemed, at least, innocuous. Right ventricular decompression by tricuspid avulsion or excision could be suggested for all patients with pulmonary atresia, intact ventricular septum, hypersystemic hypoplastic right ventricle, and major sinusoids without right ventricular dependent coronary circulation in whom the outlet portion of the right ventricle is not functional.     

Early and late results in pulmonary atresia and intact ventricular septum.

We examined the early and late results of operations in 29 consecutive neonates with pulmonary atresia and intact ventricular septum treated from 1980 to 1988. Transventricular pulmonary valvotomy and central aorta-pulmonary artery shunting were performed in 19 of 22 infants who had a patent infundibulum. Pulmonary valvotomy alone was performed in 3 of the 22 infants with a patent infundibulum, but 2 of these required subsequent systemic-pulmonary artery shunts. Primary shunting was used to palliate 7 infants who had absent infundibular portions of the right ventricle and a very diminutive right ventricular cavity. Tricuspid valve excision and atrial septectomy were also performed in 5 of these 7 infants to decompress large fistulous communications between the right ventricule and coronary artery. Two early deaths (2/29, 6.9%) occurred overall. Both were in infants who had a very small right ventricle. Definitive operation has been accomplished in 16 patients; 13 have had closure of residual interatrial communications and shunt ligation with no deaths, and 3 have undergone modified Fontan repair with 1 death. Actuarial survival rate for the entire group, including operative deaths, is 86% at 5 years. The technique of transventricular pulmonary valvotomy and systemic-pulmonary artery shunting offers a reliable means of palliating neonates with pulmonary atresia and intact ventricular septum and obtains good late right ventricular growth. Systemic-pulmonary shunting, tricuspid valvectomy, and atrial septectomy may offer a means of reducing or obliterating right ventricular-coronary artery fistulas.     

Pulmonary atresia with intact ventricular septum: is neonatal repair advisable?

The optimal management of pulmonary atresia with an intact ventricular septum in the neonate remains controversial. The introduction of balloon septostomy and prostaglandin has significantly reduced early mortality but early surgical intervention is necessary to obtain a more adequate pulmonary blood flow. Fourteen neonates with pulmonary atresia and an intact ventricular septum were admitted to the Wessex Cardiothoracic Unit, Southampton from 1979 to 1986. Thirteen patients underwent cardiac catheterization. Cardiac catheterization data and right ventricular angiograms were reviewed retrospectively. Four patients with tripartite ventricles underwent total repair. The others received various palliative operations (valvotomy + modified Blalock-Taussig shunt or modified Blalock-Taussig shunt alone). Retrospective analysis of the angiograms indicated that right ventricular morphology alone is not a satisfactory criterion for surgical management. We have been able to demonstrate that there is a good correlation between the diameter of the tricuspid valve and the diameter of the infundibulum and that successful neonatal repair is possible when the tricuspid valve diameter is above 80% of the normal value for weight and when the tricuspid valve diameter to infundibular diameter ratio (TV/Inf ratio) is 2.2 or less. In patients with a tripartite ventricle but inadequate TV diameter and TV/Inf ratio, a closed pulmonary valvotomy with a modified Blalock-Taussig shunt remains the treatment of choice.     

Early and Late Results in Pulmonary Atresia

The early and long-term results following surgical treatment of 24 infants with pulmonary atresia with an intact ventricular septum were reviewed. Pulmonary valvotomy was the operation most often performed, and we came to realize that this was effective when the preoperative right ventricular angiogram had shown an open conus up to the atretic valve. By contrast, no infant without a patent conus survived pulmonary valvotomy. We suggest that this subgroup of patients (8 of the 24 in this series) should have a systemic-pulmonary shunt followed later by reconstruction of the right ventricular outflow tract.Diminutive right ventricles will grow and dilate if a reasonable passageway is formed from right ventricle to pulmonary artery. Thus the long-term results have been excellent when this has been carried out, with normal right ventricular pressures and appearance on angiography in 7 patients followed up to 13 years.     

Atriopulmonary shunt

A new alternative to cavopulmonary anastomosis or systemic pulmonary arterial shunt in the treatment of tricuspid valve atresia, pulmonary valve atresia, single ventricle, and underdeveloped right ventricle was studied experimentally in dogs. Complete diversion of all the systemic venous return from the right atrium directly to the pulmonary circulation was possible using a pulmonary valve homograft. The congenital lesions were simulated by surgical closure of the tricuspid valve. Four long-term survivors were studied by serial angiocardiography and cardiac catheterization, which demonstrated complete bypass of the right ventricle and normal hemodynamics. These experimental results support cautious clinical application of the operation provided pulmonary artery pressure is normal and flow through the left side of the heart is not obstructed.     

Balloon valvuloplasty after pulmonary valvotomy for babies with pulmonary atresia and intact ventricular septum.

During a 2 1/2-year period, staged procedures of transventricular closed pulmonary valvotomy followed by balloon valvuloplasty were attempted in 12 babies with pulmonary atresia and an intact ventricular septum. All babies immediately underwent valvotomy when echocardiography revealed a tripartite right ventricle with adequate inflow and outflow dimensions and without sinusoidal-coronary arterial fistulas. After valvotomy, the overall mortality rate was 25% (3/12), but the only surgical death (1/12, 8%) was due to failure to establish continuity between the right ventricular cavity and the pulmonary trunk. The other 2 babies died of neonatal complications after successful valvotomy. Angiocardiography performed 5 to 18 months after valvotomy documented substantial growth of the right ventricular inflow and outflow dimensions in the 9 survivors. Twelve balloon dilation procedures were then performed in 7 babies. All except 1 achieved a significant drop in the right ventricular to left ventricular peak systolic pressure ratio (0.96 +/- 0.40 to 0.56 +/- 0.28; p less than 0.01). Balloon valvuloplasty was not required in 1 baby and failed in the other, who then underwent successful right ventricular outflow tract reconstruction. After these staged procedures, follow-up at 1 month to 20 months (mean follow-up, 14.8 months) revealed resting cyanosis in 3 babies, which was related to severe residual infundibular stenosis (55 mm Hg) in 1 and a subnormal tricuspid valve annulus in 2. The remaining 5 babies (including 1 who required no valvuloplasty) were active and pink (saturation greater than 97%) and had a mean Doppler estimated gradient of 19 mm Hg (range, 8 to 36 mm Hg) across the pulmonary valve.(ABSTRACT TRUNCATED AT 250 WORDS)     

Valvotomy for pulmonary atresia with intact ventricular septum. A disciplined approach to achieve a functioning right ventricle

During the past 20 years, a consistent policy in applying early valvotomy has resulted in a unique opportunity to appraise the long-term results of this approach in pulmonary atresia with intact ventricular septum. Since 1964, 27 of 35 patients with pulmonary atresia with intact ventricular septum had type 1 or 2 right ventricle, 25 of these had early valvotomy, seven with and 18 without concomitant shunt. The remaining two patients with type 2 right ventricle and the eight patients with type 3 right ventricle received a shunt alone. Overall operative mortality was 34%; for those patients weighing more than 3 kg and those operated upon since 1977, it was 18% and 16%, respectively. There were 17 survivors of early valvotomy: 11 had valvotomy alone and six had valvotomy with shunt; 12 had type 1 right ventricle and five had type 2 right ventricle. Survival rates (+/- standard error) for these 17 patients were 85% +/- 10% and 68% +/- 17% at 5 and 10 years, respectively. The probability of reoperation was 100% by 6 years of age; outflow patch reconstruction was employed in all patients in whom reoperation has been performed. Aggressive follow-up and early recatheterization were essential features of management. Delayed reconstruction after shunt alone was unsuccessful in three patients. Primary valvotomy without shunt is the operation of choice for patients with pulmonary atresia with intact ventricular septum and type 1 right ventricle. Concomitant shunt may be required for some patients with type 1 and most with type 2 right ventricle, selected preoperatively by angiography or after valvotomy by clinical necessity. Delayed right ventricular reconstruction after shunt alone is not an acceptable approach when an outflow tract is present.     

Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum

BACKGROUND: Since 1991 we have performed a multistage palliative approach to biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum in infants with a detectable right ventricular infundibulum. METHODS: A total of 25 patients (19 pulmonary atresia and 6 critical pulmonary stenosis) underwent initial palliation consisting of a transarterial pulmonary valvotomy and a polytetrafluoroethylene shunt between the left subclavian artery and pulmonary trunk. Among the 23 survivors, 15 underwent balloon valvotomy. Six of these patients later required additional palliative surgery that consisted of repeat pulmonary valvotomy, adjustment of an atrial communication, and resection of the hypertrophied muscles in the right ventricle. RESULTS: Of the 25 patients, 23 (92%) survived. In all, 20 patients underwent definitive operations: 18 (90%) biventricular repair (12 pulmonary atresia, and 6 critical pulmonary stenosis), one bidirectional Glenn, and one Fontan procedure. The actuarial probability of achieving a biventricular repair at 36 months of age was 69%. In 18 patients right ventricular end-diastolic volume significantly increased but tricuspid valve diameter did not change. CONCLUSIONS: The multistage palliation procedure to promote right ventricular growth makes a definitive biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum possible in the majority of infants with a patent infundibulum.     

Concomitant valvotomy and subclavian-main pulmonary artery shunt in neonates with pulmonary atresia and intact ventricular septum

Our current approach to the management of neonates with pulmonary atresia and intact ventricular septum is to perform a transarterial pulmonary valvotomy through a left anterolateral thoracotomy followed by a polytetrafluoroethylene shunt between the left subclavian artery and the pulmonary trunk at the site of the pulmonary arteriotomy. From October, 1983, to December, 1985, 7 consecutive neonates with pulmonary atresia and intact ventricular septum were managed in this fashion. Mean age was 5.1 days (5 patients, less than 48 hours old), and mean weight was 3.3 kg (range, 2.5-4.3 kg). Right ventricular morphology was type I (tripartite) in 4 patients, type II (absent trabecular portion) in 2, and type III (absent trabecular and infundibular portions) in 1. The mean right ventricular to left ventricular peak systolic pressure ratio was 1.5. One patient who initially had valvotomy alone required a left subclavian-pulmonary trunk shunt the next day for hypoxemia. All other patients had a valvotomy and shunt during the same procedure. There were no operative or hospital deaths. Follow-up of 3.5 to 34 months (mean, 17.5 months) confirmed shunt patency in all patients. Three of 4 patients undergoing postoperative catheterization have shown good right ventricular growth; 2 have undergone successful repair at 10 and 23 months. There have been 3 late deaths at 3.5, 4, and 8 months. Two other patients are doing well and are awaiting postoperative catheterization. This procedure permits synchronous valvotomy and shunting without the need for cardiopulmonary bypass in these critically ill neonates.(ABSTRACT TRUNCATED AT 250 WORDS)     

Gore-Tex片作单瓣重建右心室流出道的早、中期疗效

目的评价0.1mm厚的Gore-Tex片作肺动脉单瓣重建右心室流出道(RVOT)的早中期临床效果。方法2002年6月至2006年7月,对48例合并肺动脉狭窄或闭锁的先天性心脏病患者施行矫治手术,术中采用0.1mm厚的Gore-Tex片作肺动脉单瓣的Dacron血管片重建RVOT,其中包括法洛四联症合并肺动脉狭窄33例、型肺动脉闭锁8例、肺动脉瓣缺如3例,右心室双出口合并肺动脉狭窄2例,永存动脉干1例,完全型大动脉错位、室间隔缺损、肺动脉狭窄1例。结果本组无手术死亡。术后血氧饱和度达1.00,右心室/左心室收缩压比值0.22~0.65,右心室与左、右肺动脉压差<10mm Hg。所有患者(100%)均随访3~48个月,无死亡和并发症。彩色多普勒超声心动图检查提示:无肺动脉狭窄,肺动脉瓣轻度反流13例,中度反流5例,Gore-Tex膜具有活动功能40例。结论采用0.1mm厚的Gore-Tex片作单瓣重建RVOT,其早中期临床结果显示有较满意的血流动力学效果。     

Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology

OBJECTIVES: Management strategy for pulmonary atresia with intact ventricular septum is controversial. We treated patients with this anomaly according to a management protocol based on our quantitative assessment of right ventricular morphology (index of right ventricular development and right ventricle-tricuspid valve index). In the present study, we reviewed a 20-year experience of the surgical treatment at our institution to define guidelines for selecting the appropriate type of surgical procedure. METHODS: Between April 1981 and June 2002, 45 consecutive patients with pulmonary atresia with intact ventricular septum underwent surgical treatment. Open transpulmonary valvotomy was performed in 27 patients and Blalock-Taussig shunt in 18 patients as the initial palliative procedure. Three patients who underwent a successful transpulmonary valvotomy alone in the neonatal period required no further operation. Definitive repair was performed in 32 patients. Biventricular repair was performed on 19 patients, one and a half ventricular repair in 3, and Fontan-type operation in 10. RESULTS: There were 1 early and 2 late deaths before the definitive operation in patients who underwent transpulmonary valvotomy. Two patients who had coronary artery interruption died 3 months and 13 years after the initial Blalock-Taussig shunt. There were 1 early and 2 late deaths after the definitive operation. Actuarial survival, including noncardiac death, was 91.1% at 5 years after the initial procedure and 81.5% at 10 years. CONCLUSIONS: We treated patients with pulmonary atresia with intact ventricular septum according to a management protocol based on our quantitative assessment of right ventricular morphology with good results.     

Pulmonary atresia with intact ventricular septum. Sixteen-year experience.

Infants with pulmonary atresia and intact ventricular septum (PA-IVS) usually require urgent surgical intervention. Thirty patients with this anomaly, seen at the Columbia-Presbyterian Medical Center between 1962 and 1978, had palliative operations, 26 within the first 3 days of life. Six underwent a closed pulmonary valvotomy alone, with no survivors; six had only a systemic--pulmonary artery shunt, with three early survivors. Because of this experience, 17 had a combined procedure of valvotomy and shunt, with 14 early survivors. One patient recently underwent a definitive right ventricular outflow patch procedure with cardiopulmonary bypass. Eight patients subsequently have had corrective open-heart procedures, with five patients surviving from 2 to 10 years. A unicusp aortic homograft was used for repair in five and a Hancock valved conduit in three. Four patients are presently awaiting operation. We conclude that the initial surgical management of these critically ill infants must not only increase pulmonary blood flow but in addition provide an opportunity for right ventricular growth. Thus we continue to advocate the combined procedure of a valvotomy plus a shunt to provide adequate palliation. Repeat catheterization should be performed within a year to confirm the adequacy of the valvotomy, since this is essential to maximal right ventricular enlargement and to allow for definitive correction at a later date.     

Right ventricular growth potential in neonates with pulmonary atresia and intact ventricular septum

The cardiac catheterization data and angiograms of 30 infants with pulmonary atresia and intact ventricular septum were reviewed to evaluate the growth potential of the right ventricle after transventricular pulmonary valvotomy. An index of right ventricular size based upon the tricuspid valve anulus, right ventricular inlet, and right ventricular outlet dimensions was used. Fourteen infants (Group I) were treated with systemic-pulmonary arterial shunts only, whereas 16 infants (Group II) underwent pulmonary valvotomy and 14 had shunting as well. Follow-up studies demonstrated the lack of right ventricular growth in Group I (right ventricular index of 7.0 +/- 3.2 preoperatively versus 7.0 +/- 2.0 postoperatively) and persistence of severe right ventricular hypertension (systolic pressure of 121 +/- 31 versus 120 +/- 48 mm Hg). In contrast, the right ventricular cavity increased in nine of 11 Group II infants who underwent valvotomy. Right ventricular index increased from 7.7 +/- 1.6 to 11.0 +/- 3.1 (p less than 0.01) and systolic pressure fell from 132 +/- 31 to 83 +/- 50 mm Hg (p less than 0.1). Early and late mortality in Group I was 50% (7/14), whereas only three of 16 Group II infants died (p greater than 0.1). It is concluded that pulmonary valvotomy should be attempted in all neonates with pulmonary atresia and intact ventricular septum in whom an outflow tract is identified angiographically to maximize the potential for right ventricular growth and increase its functional contribution to normal circulation.     

Comprehensive management of pulmonary atresia with intact ventricular septum

The prognosis for patients with pulmonary atresia with intact ventricular septum is poor with or without conventional surgical intervention. Therefore, a comprehensive program of medical and surgical treatment is necessary to improve long-term outlook for these infants. Such a program consists of management of the neonate at initial presentation with prompt administration of prostaglandins and institution of a combination of surgical procedures (isolated pulmonary valvotomy, valvotomy plus modified Blalock-Taussig shunt, Blalock-Taussig shunt plus balloon atrial septostomy, or Blalock-Taussig shunt alone) depending on the results of morphological analysis of the right ventricle; this treatment regimen is designed to relieve hypoxemia, encourage right ventricular growth, and provide adequate egress of blood from the right atrium. Another important element of management is to perform follow-up hemodynamic and angiographic studies when the patient is between 6 and 12 months old to ensure that the objectives of the comprehensive program are being met. Finally, a definitive repair should be offered. This can be done by using or bypassing the right ventricle, depending on whether it can support the pulmonary circuit.     

膨体聚四氟乙烯片作单瓣重建右室流出道术后早期并发症及处理

目的：评价膨体聚四氟乙烯片（Gore-Tex片）作肺动脉单瓣重建右室流出道（RVOT）的近期疗效。方法：2002年6月-2011年11月对75例先天性心脏病合并肺动脉狭窄或闭锁的患者旋行矫治手术,术中采用厚度0．1mm的Gore—Tex片作肺动脉单瓣重建RVOT,其中男42倒,女33例。法洛四联症合并肺动脉狭窄57例,l型肺动脉闭锁10例．肺动脉瓣缺如4例,右心室双出口台并肺动脉狭窄2例．完全型大动脉转位1例,室间隔缺损1例,肺动脉狭窄1例。结果：术后血氧饱和度为96％～100％。动脉血氧分压82～207mmHg,右室／左室收缩压比值0．22～0．70,右室与左、右肺动脉间的压力阶差小于10mmHg。左室射血分数（LVEF）0．53～O．80,右室射血分数（RVEF）0．52～071,左室舒张末期容积指数（LVEDI）0,28～0．62ml／m2。术后早期并发症：心包积液7例,低氧血症（氧合指数〈150）6例。心律失常5例,低心排血量综合征4例．残余室间隔缺损4例。术后随访3～48个月,无死亡和并发症的发生。其中62~Gore-Tex片作肺动脉单瓣重建右心室流出道（RVOT）早期瓣膜活动功能正常,13例单瓣固定在开放状态：72倒均无肺动脉单瓣狭窄．3例单瓣轻度狭窄;21倒肺动脉瓣轻度返流．5例中度返流。结论：采用厚度0．Imm的Gote-Tex片作单瓣重建RVOT,可获得满意的lI缶床和血流动力学效果．近期效果良好。膨体聚四氟乙烯片作单瓣重建右心室流出道术后早期并发症应引起高度熏视,加强术后监护及综合治疗措施,及时纠正术后低心输出量综合征．积极防治术后心律失常等均为提高手术成功率的重要因素。     

分期全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的 总结分期全腔静脉.肺动脉连接术(TCPC)治疗复杂先天性心脏病的经验.方法 1998年6月至2008年3月,22例先天性心脏病复杂畸形患者接受分期TCPC.本组中单心室合并肺动脉狄窄9例,合并肺动脉闭锁3例;完全性大动脉转位,十字交叉心,肺动脉瓣狭窄1例;完全性房室通道,左心窒发育不良,肺动脉闭锁,房室瓣少-中量反流1例;完全性房室通道,动脉导管未闭,肺动脉狭窄,双向Glenn术后伴吻合口狭窄房室瓣大量反流1例;镜面右位心,功能性单心室,肺动脉闭锁,动脉导管未闭,体肺侧支,双侧双向Glenn术后2年,右下肺动-静脉瘘1例;三尖瓣闭锁并肺动脉狭窄4例;三尖瓣闭锁伴肺动脉闭锁,动脉导管未闭1例;镜面右位心,右心室双出口,窒间隔缺损,左心室发育小良,功能性单心房,肺动脉瓣狭窄,三尖瓣少量反流,粗大体肺侧支1例.第一期体肺分流术后,二期双向Glenn术5例,第一期单侧或双侧双向Glenn手术17例.行双向Glenn手术的年龄为(5.9±4.4)岁,Glenn术前肺动脉压为17～20 mm Hg(1 mm Hg=0.133 kPa);房室瓣膜关闭不全3例;一期手术前Nakata指数小于200 mm2/m2者4例.接受TCPC术平均年龄为(9.6±4.9)岁,与双向Glenn术间隔时间(3.7 ±1.2)年,术前经上腔静脉置管测得肺动脉压力均小于15 mm Hg.全组22例均采用心外管道TCPC,房窜瓣成形1例.结果 全组住院死亡1例,病死率为4.5%.死亡病例为单心室,肺动脉闭锁,第一期体肺分流术后,二期左肺动脉成形双向Glenn术后5年第三期行TCPC,术后左肺反复多次大出血死亡.余患者均顺利出院,术后中心静脉压12～18 mm Hg.出院前经皮测血氧饱和度为90%～96%,活动量明显增加,发绀及气促症状消失,心功能均为NYHA分级Ⅰ～Ⅱ级.结论 对于高危患者根据病情选择分期TCPC,可以使更多有高危因素不能行Fontan手术的患者得到救治的机会,扩大手术适应证,并取得满意疗效.     

Surgical management of hypoplastic right ventricle with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum

Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum includes 26 patients with pulmonary atresia and 4 with critical pulmonary stenosis. Group 1 consisted of 8 neonates managed initially by transventricular valvotomy; 6 later required a secondary procedure, with 100% survival. Group 2 had 11 neonates managed by aorta-pulmonary artery shunting without operative death. However, only 3 have survived over the long term and 1 has required an additional shunt procedure. Group 3 had 9 infants who underwent concomitant valvotomy and shunting. There were 4 operative deaths and 1 late death. Finally, Group 4 included 2 infants managed by primary repair at 3 days and 6 days old with prosthetic enlargement of the right ventricle; 1 required the addition of a shunt. Both are alive. Seven of the 15 patients in Groups 1, 2, and 3 who survived neonatal palliative procedures have undergone reparative operations. Two had no growth of the right ventricle and underwent repair after conversion to tricuspid atresia, by a Fontan procedure. Five had prosthetic enlargement of the right ventricle in childhood with 1 late death. Findings of this review were as follows: (1) effective palliation of pulmonary atresia and intact ventricular septum or critical pulmonary stenosis with cavitary hypoplasia of the right ventricle is rare unless transventricular flow can be established; (2) establishment of transventricular flow produces a high incidence of cavitary "growth," which permits later repair; (3) the Fontan operation is available for repair in patients who have no cavitary growth; and (4) when all three portions of the right ventricular cavity can be identified by angiography, a primary repair can be performed in the neonatal period with a good long-term prognosis.     

Surgical intervention in neonates with critical pulmonary stenosis.

The surgical experience with 18 consecutive neonates with critical pulmonary stenosis (PS) and intact ventricular septum was reviewed. All patients had cardiac catheterization with calculation of right ventricular volume. Group A patients (N = 8) had a small right ventricular end-diastolic volume (RVEDV less than 72% of predicted). Group B patients (N = 10) had a normal or enlarged RVEDV. All patients had a closed pulmonary valvotomy. Five Group A patients required a systemic-pulmonary shunt or prostaglandin (PGE1) after operation; one patient died. Nine Group B patients did well after valvotomy; one moribund patient died after valvotomy and shunt. Six of 16 survivors required reoperation: valvectomy in four patients and shunt takedown in two patients. Four of the six patients who had reoperation also had a transannular patch. There was one unrelated late death. All long-term survivors are asymptomatic. Recatheterization in four patients with a small right ventricle (RV) documented significant RV growth. In conclusion, most neonates with critical PS can be managed with closed valvotomy. Patients with a small RV may require PGE1 or a shunt after operation for persistent hypoxemia. Some patients with a small RV will have significant RV growth after valvotomy.     

Coronary artery air embolism following pulmonary valvotomy for pulmonary atresia: a note of caution

A case of coronary artery air embolism following open pulmonary valvotomy for pulmonary atresia with intact ventricular septum is reported. The presumptive source of this complication is a fistulous communication between the coronary artery and right ventricle, and may be preventable by certain modifications in technique.