Use of small stents for rehabilitation of hypoplastic pulmonary arteries in pulmonary atresia with ventricular septal defect.

Branch pulmonary artery stenosis frequently occurs in pulmonary atresia with ventricular septal defect (PA/VSD). Balloon dilation alone is often unsuccessful in patients with severely hypoplastic pulmonary arteries with residual stenoses after surgical repair. In an attempt to promote distal pulmonary artery growth, 17 stents were placed in 12 severely stenotic pulmonary artery lesions in 10 patients with PA/VSD. All had prior surgery, including pulmonary artery repair, right ventricle to pulmonary artery homograft, and, in 6 of 10, closure of VSD. Median age at stent placement was 16.8 months (range, 13.2-56). Stents were placed using 3.0, 3.5, or 4.0 mm balloons in all but one lesion, in which a 7 mm balloon was used. Following stent placement, there was an increase in the lesion diameter from 1.5 to 3.4 mm (P < 0.05) and an increase in flow to the affected lung from 27% to 34% (P < 0.05). Repeat catheterization 2 to 6 months after stenting in six patients revealed complete occlusion in two of eight lesions. In the other six vessels, there was an increase in distal vessel diameter from 2.96 to 3.94 mm (P < 0.05) even though four had severe restenosis requiring restenting. Two patients underwent surgical pulmonary artery reconstruction and stent removal because of adequate distal vessel growth. Stenting of hypoplastic pulmonary arteries in PA/VSD results in immediate improvement in vessel size and blood flow. Stent restenosis is common although distal vessel growth can be achieved. Stenting of these lesions should be reserved only for those patients unresponsive to other interventions.     

Stenting stenosed aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect

We present two cases of pulmonary atresia with ventricular septal defect who were not suitable for corrective surgery due to absent or hypoplastic native pulmonary arteries and were quite symptomatic following shunt surgery. We dilated and stented stenosed aortopulmonary collaterals as palliative procedure with improvement in oxygen saturation, and significant symptomatic relief.     

15例肺动脉发育不良的肺动脉闭锁合并室间隔缺损的临床预后分析

目的:探讨肺动脉发育不良的肺动脉闭锁合并室间隔缺损(PA-VSD)的手术策略,手术方法以及影响早期预后的因素。方法:回顾性分析15例肺动脉发育不良的肺动脉闭锁合并室间隔缺损患者资料,根据肺血主要来源分为两组,导管依赖组6例:男性2例,女性4例,中位年龄18(4~252)个月。非导管依赖组9例:男性4例,女性5例,中位年龄33(16~96)个月。全组行Sano分流13例,BT分流1例,中心分流1例。结果:全组15例患者全部接受分期手术,Sa O2由术前(62±16)%升至术后(89±10)%,术后患者均合并不同程度的肺部渗出,右心功能不全,早期出现呼吸机相关感染3例,经治疗后痊愈,死亡2例。随访11例,随访率85%(11/13)。全组随访期间无死亡。9例接受二期手术根治术,4例等待手术。结论:肺动脉发育不良的肺动脉闭锁合并室间隔缺损选择不同的手术方式,个体化治疗,可以取得较好的疗效。其中导管依赖型的围术期风险远低于非导管依赖型。     

Management of pulmonary atresia with ventricular septal defect

Opinion statement It has been nearly 40 years since Kirklin, at the Mayo Clinic in 1964, performed the first surgical repair of pulmonary atresia with ventricular septal defect using a nonvalved extracardiac conduit, which he created out of the patient’s pericardium, and this patient continues to do well. In the subsequent four decades, great advances have been made in the diagnosis, with regard to the often very complex anatomy of pulmonary blood supply that this extremely heterogeneous group of patients manifest, and their subsequent surgical management. Unifocalization procedures have permitted eventual complete correction in patients with nonconfluent pulmonary artery segments, and advances in infant surgery have enabled most patients to achieve correction in the early childhood years. Although the ideal extracardiac conduit has yet to be developed, necessitating periodic conduit replacement in corrected patients, the late results in this group of patients are generally excellent, with many now achieving survival well into the adulthood years with good quality of life. Continued advances in surgery and interventional catheterization techniques hold great promise for ever-improving outcomes in children being born with this complex congenital cardiovascular malformation.     

Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect.

A histologic study was performed on 22 specimens of pulmonary atresia with ventricular septal defect to 1) ascertain the existence of the main pulmonary artery; 2) distinguish the ductus arteriosus from the systemic collateral arteries (SCA); 3) establish the nutritive or functional nature of collateral circulation; and 4) evaluate the morphology of the distal pulmonary bed. Three cases had absent main pulmonary artery, one with and two without signs of infundibular septation. We suggest that absent main pulmonary artery may exist with both infundibular pulmonary atresia and persistent truncus arteriosus. SCAs have been found to have similar histological features as systemic muscular arteries of the same size--their medial muscular layer merges gradually into an elastic one at different depth inside the lungs. Injection of contrast material allowed us to demonstrate that these vessels are functional, since they inosculate into efficient pulmonary arteries ending in the respiratory units. When the distal pulmonary vascular bed is perfused by large SCAs, proliferative lesions like those found in large left-to-right shunts may occur. Early in infancy, banding of large, nonstenotic SCAs could protect the distal pulmonary vasculature. Moreover, total surgical repair should be associated with ligation of the SCA to avoid residual left-to-right shunt, if the pulmonary arteries can carry the full pulmonary blood flow.     

Growth and development of pulmonary circulation in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Twenty-four-hour electrocardiograms were recorded in the first 10 days of life on 134 healthy full-term infants with birthweights greater than 2.5 kg. The highest heart rate a minute, measured over nine beats, was 175 +/- 19 (SD). The lowest rates, measured over three, five, and nine beats were 82 +/- 12, 87 +/- 12, and 93 +/- 12, respectively. At their lowest rates 109 infants had sinus bradycardia and 25 had junctional escape rhythms. Thirty-three infants showed changes in P wave configuration with or without pronounced variation in PR interval. Atrial premature beats were present in 19 infants but only one had more than 12 per hour. In a randomly selected subgroup of 71 infants sinus pauses were found in 51 (72%). Five (7%) had electrocardiographic patterns and rhythm disturbances that could not be differentiated from those previously described as complete sinuatrial exit block or sinus arrest, eight (11%) had patterns indistinguishable from 2:1 sinuatrial block, and 23 (32%) had pattern indistinguishable from sinuatrial Wenckebach block. This study shows that normal infants have variations in heart rate and rhythm hitherto considered to be abnormal.     

Pulmonary atresia with hypoplastic right ventricle and large subaortic ventricular septal defect

A neonate is presented with a very rare association consisting of pulmonary atresia with large ventricular septal defect but additionally, combined with a hypoplastic right ventricle and well developed pulmonary arteries. The management strategy is described.     

Duplicate sources of pulmonary blood supply in pulmonary atresia with ventricular septal defect.

In pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, there is a tendency for each collateral artery to be the sole supply to a particular region of lung. On injection into a collateral artery, however, "wash-out" of contrast medium by non-opacified blood from a second source is sometimes seen. Alternatively, contrast medium may faintly "wash-in" to an adjacent lobar artery supplied from a second source. "Wash-in" and "wash-out" therefore show that a duplicate blood supply exists. To assess the importance of these phenomena, we reviewed the angiograms of 50 patients with this condition. Duplicate pulmonary blood supply not resulting from surgical shunts was found in 25 patients (50%), and in 24 of 37 patients (65%) who had selective collateral injections. In one patient two collaterals anastomosed with each other. Thirty-nine instances of duplicate supply occurred in the remaining 24. The duplicate connection was to a region of lung connected to a central pulmonary artery in 29 of 39 instances (74%). Determination of how much of the peripheral pulmonary vasculature is connected to central pulmonary arteries greatly affects decisions about palliative and corrective surgery. These results show that unless "wash-in" and "wash-out" are specifically looked for, the information obtained from angiography may be erroneous.     

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Effect of systemic pulmonary anastomosis.

In nine patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries, the effect of a systemic-pulmonary anastomosis on the lung was studied by comparison of pre- and postoperative cine angiograms. Selective injections into the collateral arteries were performed in all patients and the source of blood supply to each bronchopulmonary segment was sought. Central pulmonary arteries were present in six children and absent in three, as confirmed at thoracotomy. After insertion of a shunt, central pulmonary arteries increased in size, but the intrapulmonary vessels with which they connected remained abnormally small and were frequently stenosed. In addition, in each case the central pulmonary arteries increased flow to only five to 11 segments of lung. In the absence of central pulmonary arteries, shunting to a hilar or a lobar pulmonary artery increased perfusion to five to nine segments of lung per case. In all these three cases the pulmonary arteries at lobar level showed aneurysmal dilatation proximal to a severe stenosis. Sixty-eight per cent of collateral arteries were stenosed. The findings suggest that in this anomaly, when the central pulmonary arteries are hypoplastic, the intrapulmonary branches are also hypoplastic, emphasising the need for early surgical intervention to increase blood flow while the lung still has growth potential. Further, one collateral artery may connect with at least as many bronchopulmonary segments as does a central pulmonary artery, and the peripheral intrapulmonary arteries with which it connects may appear at least as normal angiographically as do vessels connected to central pulmonary arteries. These observations suggest that segments of lung connected to collaterals alone should, and sometimes can, be connected to central pulmonary arteries, the aim being to produce a unifocal blood supply as a prelude to total correction.     

Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries

INTRODUCTION: In order to establish the best strategy of treatment and predictors of outcome in infants with pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries, we reviewed our institutional experience concerning 47 infants. METHODS: Inclusion criteria included an angiographic diagnosis of pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries with or without central pulmonary arteries and a repair by the same surgeon. Thirty-one patients had confluent (type III) and 16 absent (type IV) central pulmonary arteries. Pulmonary arteries were considered to be adequate when they measured > or = 4 mm. RESULTS: There were 8 deaths (17%). Complete repair was performed in 24 patients (18 in group III and 6 in group IV) with 79.3% had a right/left ventricular pressure <0.5, 16; 5%<1 and 4.2%>1. Eleven patients are waiting for complete repair and 4 will be operated on pulmonary arteries. One stage complete repair was performed in 3 patients, two and third stage repair (after unifocalisation or right ventricle to pulmonary arteries conduit) was attained in 21 patients. Among patients having had a complete repair and showing a right/left ventricular pressure <0.5, 88.9% had a pulmonary atresia with ventricular septal defect type III and 50% a pulmonary atresia with ventricular septal defect type IV. Only 56% of type III patients with a right/left ventricular pressure <0.5 had adequate central pulmonary arteries. CONCLUSION: In our study, the small size and the absence of central pulmonary arteries do not prevent a positive outcome.     

Rehabilitation of hypoplastic pulmonary arteries and anatomic correction of pulmonary atresia with interventricular communication]

Conventional treatment of pulmonary atresia with ventricular septal defect (VSD), hypoplastic pulmonary arteries (PA) and major aorto-pulmonary collaterals (MAPCAs) is controversial: from symptomatic and palliative treatment for some authors to surgery with unifocalisation of collaterals for others. These treatments never use native pulmonary arteries as only source of pulmonary flow, but create "neo-pulmonary arteries". Nine cases of pulmonary atresia with VSD, hypoplastic PA and MAPCAs were treated by rehabilitation of native PA through a staged approach: 1) surgical neonatal connection between right ventricule (RV) and hypoplastic PA, 2) evaluation and interventionnal catheterism with angioplasty of PA stenosis and closure of collaterals, 3) complete surgical correction with reconstruction of right outflow track and PA and closure of VSD. After first surgical stage of RV-PA connection at the mean age of 4.8 months (+/- 5.6 months), 8 patients were alive and underwent 22 cardiac catheterisms (mean of 2.7 per patient), with angioplasty of PA, and occlusion of MAPCAs in 6 and 2 patients respectively. Seven patients underwent complete anatomical correction at the mean age of 28.8 months (+/- 17.7 months) with one late death. The 6 remaining patients had encouraging hemodynamic status (RV pressure/LV pressure ratio at 0.6 +/- 0.26; mean left and right distal pulmonary pressure at 15.2 mmHg (+/- 9.1 mmHg)), and good functionnal status (3 in NYHA functionnal class 1, and 3 in class 2), for a mean follow-up of 79.5 months (+/- 41.4 months). One patient had reoperation on right outflow track stenosis, 6 years after correction. This small series enhances the feasibility of a staged approach with rehabilitation of small PA, allowing complete surgical correction with the native PA with good hemodynamic and functional results in pulmonary atresia, with VSD, hypoplastic PA and MAPCAs.     

Coronary artery-pulmonary artery fistula in pulmonary atresia with ventricular septal defect

Summary This paper reports two patients presenting a combination of coronary artery-pulmonary artery fistula and pulmonary atresia with ventricular septal defect. A confluent central pulmonary artery arose from the proximal portion of the left coronary artery in both patients. The fistulous segment between the coronary artery and the central pulmonary artery was large, and no coronary arterial stenosis was found in either patient. Besides this fistulous origin of the pulmonary blood supply, large collateral vessels originated from the descending aorta in both patients. Neither patient has shown findings of myocardial ischemia, at least in consecutive rest and exercise electrocardiograms. Both patients were successfully operated, at the ages of 3 and 8 years, respectively. The clinical and embryological implications of this rare malformation are briefly reviewed.     

Unusual vascular ring in infant with pulmonary atresia and ventricular septal defect.

A 3-day-old infant investigated for cyanotic heart disease was found to have partial DiGeorge syndrome and pulmonary atresia with ventricular septal defect. The only source of pulmonary blood flow was a right-sided persistent ductus arteriosus which originated from an aberrant right subclavian artery. Such a vascular ring has not been described previously. Surgical treatment included a left-sided prosthetic shunt in infancy followed by resection of the ligamentum arteriosum and right subclavian artery during the second year of life.     

Coronary artery to pulmonary artery communications in pulmonary atresia with ventricular septal defect

Coronary artery to pulmonary artery communications (CAPAC) are an important source of pulmonary blood flow in approximately 10% of patients with pulmonary atresia and ventricular septal defect (PA-VSD). A diligent look for these abnormal communications is important to prevent perioperative complications and achieve a complete repair. We present two cases of PA-VSD with CAPAC, one in a 7-year-old child and the other in a 33-year-old adult. The method of occlusion of these communications could be either surgical or catheter based.