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.     

Pulmonary atresia with intact ventricular septum and systemic-pulmonary collateral arteries

Pulmonary atresia with intact ventricular septum and major systemic-pulmonary collateral arteries is a very rare congenital heart lesion with dismal natural history. Herein we report on a case of pulmonary atresia with intact ventricular septum with hypoplastic right ventricle, very small confluent pulmonary arteries, absent arterial duct, and pulmonary blood flow exclusively provided by bronchial-type systemic-pulmonary collateral arteries that was successfully treated at our institution.     

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: 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.     

Pulmonary atresia with intact ventricular septum showing Fontan-like circulation after biventricular repair

A 6-year-old girl was referred to our institute for cardiac evaluation. She had been diagnosed as pulmonary atresia with intact ventricular septum (PAIVS) at 16 days after birth and she had underwent balloon atrial septostomy and bilateral Blalock-Taussig shunts. A cardiac catheterization at 5 months showed that her right ventricular end diastolic volume was 58% of normal, the Z value (standard deviation units) of the diameter of the tricuspid valve was -3.3, and a biventricular repair was performed. After the operation, she suffered from severe congestive heart failure for 10 months. A cardiac catheterization at the age of 6 years demonstrated that the pulmonary blood flow was generated during the diastolic phase like Fontan circulation. Although biventricular repair had been performed at 5 months, the circulation may be less advantageous for long term survival than if the patient had undergone the staged Fontan procedure. Careful and continuous hemodynamic assessment is essential for surgical therapy of 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...     

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.     

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.     

Pulmonary atresia with intact ventricular septum, antegrade coronary-right ventricular sinusoidal communication, and Wolff-Parkinson-White syndrome.

A fenestrated Fontan operation was performed successfully in a patient with pulmonary atresia with intact ventricular septum, an antegrade sinusoidal communication, and Wolff-Parkinson-White syndrome. Unlike most cases, blood flow in the sinusoidal communication was antegrade, from the left anterior descending artery to the right ventricle. This is the first report of the combination of pulmonary atresia with intact ventricular septum and Wolff-Parkinson-White syndrome.