杂交技术治疗室间隔完整型肺动脉闭锁

目的 总结杂交技术经胸肺动脉瓣球囊扩张成形术治疗室间隔完整型肺动脉闭锁的即刻疗效及近、中期随访结果.方法 2005年3月至2010年3月,采用超声引导经胸肺动脉瓣球囊扩张成形术治疗室间隔完整型肺动脉膜性闭锁30例,年龄1天～48个月,平均(4.59±3.21)个月.胸骨正中切口,于右室流出道距离肺动脉瓣环下约2 cm缝荷包线,然后置入导丝.在超声引导下置入穿刺鞘管.确认穿刺针对准膜性闭锁的瓣膜后,在钢丝引导下放入球囊扩张管进行扩张,超声提示肺动脉瓣开放满意.＜3个月病婴行改良Blalock-Taussig(B-T)体肺分流术,并同期行动脉导管结扎术.＞3个月病婴行球囊扩张术后,如血氧饱和度改善明显,不常规行改良B-T分流术,并保留动脉导管开放,如血氧饱和度改善不明显,则考虑行改良B-T分流术,结扎或保留动脉导管.＞5个月病儿行球囊扩张后血氧饱和度改善不满意,且重度右心发育不良,则选择双向Glenn术.结果 30例行球囊扩张均取得成功,同期行动脉导管结扎术25例,改良B-T分流术8例,双向Glenn术2例.均未出现严重并发症.1例术后因低氧血症,术后第3天行动脉导管结扎术和改良B-T分流术;余者术后血流动力学稳定,顺利出院.术后随访1.5～62.0个月,平均(18.7±17.2)个月.血氧饱和度由术前0.73±0.08上升至0.94±0.04,心功能Ⅰ级.院外死亡5例,25例生长发育良好.结论 杂交技术经胸肺动脉瓣球囊扩张成形术是一种治疗新生儿及婴幼儿室间隔完整型肺动脉膜性闭锁的安全、有效的方法.

Abstract：

Objective In patients with pulmonary atresia and intact ventricular septum ( PAIVS) without right ventricular-dependent coronaries, catheter techniques including the use of a sniff wire, lasers, and radiofrequency have been the most widely used initial therapy. However, percutaneous perforation and balloon valvuloplasty were associated with higher rate of procedural failure and serious complications. Methods We report our experience with a hybrid approach for pulmonary atresia with intact ventricular septum, combining surgery and interventional catheterization techniques. Between March 2005 and March 2010, hybrid procedure was carried out successfully in 30 newboms and infants with favorable anatomy. The age ranged from 1 day to 48 months with a mean of (4.59 ±3.21) months. The heart was exposed through median sternotomy. A pursestring suture was placed in the right ventricular outflow tract 2 cm away from the pulmonary trunk. Then a 16-gauge intravenous catheter was punctured through the right ventrical and perforated the atretic PV with the guidance of echocardiography. A guide wire was then inserted into the sheath and used to guide the balloon across the PV. Sequential dilations were performed until a full opening of the PV with the guidance of epicardial echocardiography. In patients ＜ 3 months PDA ligation was performed followed by modified Blalock-Taussig (B-T) shunt. In patients ＞ 3 months PDA ligation was not performed. A modified B-T shunt was inserted if severe systemic oxygen desaturation occurred after PDA ligation. Bidirectional Glenn shunt was performed for severe hypoplasia. Hybrid procedure was achieved in all patients. The simultaneous procedures included 25 cases of PDA ligation. 6 newborns underwent modified B-T shunt placement (3.5 to 5 mm) after pulmonary valvuloplasty and PDA ligation, and 2 patients ＞ 1 month underwent modified B-T shunt. Another 2 patients were selected for univentricular palliative surgery because of a diminutive monopartite right ventricle and bidirectional Glenn procedure was performed. No pericardial effusion or cardiac tamponade was observed in all patients. Another case without PDA ligation underwent a modified B-T shunt because of hypoxemia three days after hybrid procedure, and the rest patients were discharged without any further surgical intervention.During the follow-up period of 1.5 to 62.0 months, 5 patients died. 25 (83.3%) survived and were all in New York Heart Association functional class 1. Peripheral oxygen saturation increased from 0.73 ± 0.08 to 0.94 ± 0.04 (P ＜ 0.05). One patient remains in a single-ventricle pathway, whereas 24 patients achieved a two-ventricle circulation. Results Conclusion Perventricular balloon pulmonary valvuloplasty using a hybrid approach is a safe and feasible procedure for patients with PAIVS.     

Management of pulmonary atresia with intact ventricular septum

Infants with pulmonary atresia and intact ventricular septum pose a difficult clinical problem. Pulmonary valvotomy has been widely recommended for relief of the right ventricular obstruction, and most infants also have had an aortopulmonary shunt placed to ensure pulmonary blood flow. We have evolved a different approach that includes placement of a right ventricular outflow tract patch initially and continuation of prostaglandin E1 infusion postoperatively until the need for a shunt can be determined. We report here our management of 15 neonates with this diagnosis and suprasystemic right ventricular pressures. All of the infants were placed on a regimen of prostaglandin E1 before the operation to improve pulmonary blood flow, and all had an outflow patch placed early in life. Satisfactory postoperative right ventricular function, which would allow both outflow patching and ductus ligation, could be confidently predicted in only two of the 15 patients. For nine of the 15 an outflow patch was placed and prostaglandin was infused postoperatively to provide pulmonary blood flow until right ventricular function became adequate. Early in the series, three other infants were judged to need an aortopulmonary shunt in addition to decompression by an outflow patch, and one infant had only a shunt placed. Postoperatively, adequate pulmonary blood flow was present in all, and 11 of the 15 (73%) survived. Three of the deaths (average 2.8 days) after the outflow patch operation probably resulted from premature cessation of the prostaglandin infusion. One neonate with an outflow patch and a shunt died of myocardial ischemia because of coronary artery steal through right ventricular sinusoids. One late death occurred suddenly in the child with only a shunt, presumably because of an arrhythmia. The remaining survivors (10/15, 67%) are alive and have had complete repair. Study of these patients has also revealed that the definition of adequate right ventricular size needs to be more liberal. Five of the 10 surviving patients had a residual atrial septal defect with a right-to-left shunt at the ages of 1 to 3 years, but balloon occlusion of the atrial septal defect during cardiac catheterization revealed that the right ventricle in these patients was functionally adequate. These five children subsequently underwent closure of the atrial septal defect, and in two the aortopulmonary shunt was also taken down. In summary, correction was achieved in all survivors, in contrast to reported studies in which many patients are living with shunts.(ABSTRACT TRUNCATED AT 400 WORDS)     

室间隔完整型肺动脉闭锁的右心室减压策略

目的 总结室间隔完整型肺动脉闭锁(PA/IVS)右心室减压的经验与教训,反思右心室减压策略.方法 回顾性分析2015年3月至2019年12月于我院行右心室减压手术的12例PA/IVS患儿的临床资料,其中男10例、女2例,手术时中位年龄5(1～627)d.对减压后肺动脉瓣跨瓣压差与三尖瓣Z值变化进行相关性分析.结果 术后...     

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.     

Strategy for pulmonary atresia and intact ventricular septum

Pulmonary atresia with an intact ventricular septum is characterized by varying degrees of right ventricular cavity hypoplasia. This factor is critical in determining the most appropriate surgical approach for each patient. We describe a patient who underwent definitive biventricular surgical repair in early infancy. We used an atrial septal defect patch with a one-way valve and performed a right ventricular overhaul after a balloon valvotomy.     

Definitive repair in patients with pulmonary atresia and intact ventricular septum

Between 1977 and 1988, 22 patients underwent definitive repair for pulmonary atresia with intact ventricular septum. Fifteen underwent biventricular repair (mean age 24 months). All had mild to moderate right ventricular hypoplasia at the time of definitive repair. Repair consisted of closure of the atrial septal defect with enlargement of the right ventricular cavity and outflow tract with a patch in eight, insertion of a valved homograft in three, and superior vena cava-pulmonary artery connection in four. There was one operative death (7%). Seven patients had severe right ventricular hypoplasia at the time of definitive repair and underwent a Fontan procedure (mean age 46 months). In two a valved connection was made to the right ventricle and in five a right atrial-pulmonary artery nonvalved connection. There were two operative deaths (29%). Three patients had right ventricular-coronary sinusoids: Two of them underwent a Fontan procedure and one a biventricular repair. We conclude that with adequate early palliation a biventricular repair may be successfully performed for patients with mild or moderate right ventricular hypoplasia, and the Fontan procedure may be used for those with severe right ventricular hypoplasia.     

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.     

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.     

Right ventricular thrombosis in two patients with pulmonary atresia with intact ventricular septum

Thrombosis was detected in the right ventricle 7 days after a bidirectional Glenn operation in a 1-year-old boy with pulmonary atresia with intact ventricular septum (PAIVS) and immediately before pulmonary valvuloplasty in a 21-day-old boy with PAIVS. The risk of thrombosis and the blood clotting cascade should be assessed by blood tests, and surveillance echocardiography should be frequently applied in order to diagnose thrombosis early in such patients.     

The conduction system in hearts with pulmonary atresia and intact ventricular septum

BACKGROUND: Although a regular course of the conduction system is anticipated in hearts with pulmonary atresia and intact ventricular septum (PAIVS), it has never been demonstrated anatomically. In view of one of the surgical options in repair being debulking of the right ventricular wall, it is important to establish the location of the major conduction pathways. METHODS: Four hearts belonging to fetuses aged 18, 20, 22, and 29 weeks were examined. Entire hearts were serially sectioned and step sections were stained. The sinus node and atrioventricular conduction bundles were identified and their locations described. RESULTS: The conduction system in hearts with PAIVS is similar to that in normal hearts, although there was a minor variation in one of them. This was a heart affected by Ebstein malformation of the tricuspid valve. In this case, the right bundle branch terminated abruptly soon after its origin. CONCLUSIONS: Confirmation of the regular course of the cardiac conduction system is important to the development of strategies for surgical repair of PAIVS.