Surgical treatment of pulmonary atresia with ventricular septal defect

Pulmonary atresia with ventricular septal defect (PAVSD) is a complex cardiopathy represented by a complete obstruction between the right ventricle outflow and the pulmonary trunk associated with a ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA). The goal of the unifocalization in the PAVSD is to prepare the pulmonary tree for the complete repair by connecting the MAPCAs to the central pulmonary arteries that should be enlarged. After that we can made the VSD or other intracardiac repair. This is a retrospective study on 31 patients. We report our results discussing the PAVSD classification and the strategy of the complete repair in comparison with other reported results.     

Pulmonary atresia,ventricular septal defect,and multiple aorta pulmonary collateral arteries

Pulmonary atresia with ventricular septal defect and major aorta pulmonary collaterals arteries is a rare and complex congenital cardiac defect. There is considerable variability in the anatomy, morphology, and geometry of the native pulmonary arteries and the collateral vessels. While the ultimate goal of therapy is a biventricular correction with complete unifocalization, establishment of right ventricular to pulmonary arterial continuity, and closure of all intracardiac defects, achieving this endpoint can be frustrating and difficult. A carefully considered approach for each individual patient is required. Patients with appropriate anatomy may undergo a definitive single-stage unifocalization and biventricular correction in early infancy. Patients with less favorable anatomy will require a more eclectic approach. While our knowledge of the genetics of this defect is rudimentary, further advances in genetic understanding and technology hold tremendous promise for the development of future therapies.     

Complete repair of pulmonary atresia, ventricular septal defect, and severe peripheral arborization abnormalities of the central pulmonary arteries. Experience with preliminary unifocalization procedures in 38 patients

From 1982 to 1987, 38 consecutive patients with pulmonary atresia and ventricular septal defect underwent staged unifocalization procedures in preparation for final intracardiac repair of the anomaly. Thirty-six patients had concordant visceroatrial, atrioventricular, and ventriculoarterial connections. A central pulmonary artery confluence was present in 19 patients and absent in the remaining group. All patients with central pulmonary arteries had inadequate peripheral pulmonary arterial arborization. Systemic collateral arteries were present in all 38 patients. The objectives of the unifocalization procedures were the interruption of extracardiac sources of pulmonary arterial blood flow, the restoration of segmental, lobar, and pulmonary arterial confluence, the replacement of missing central pulmonary arterial branches, and the creation of a central, accessible source of pulmonary arterial blood flow. A total of 54 unifocalization procedures were performed in the 38 patients. These procedures included 85 permanent connecting anastomoses, 15 temporary anastomoses to the ascending aorta, 13 angioplasty procedures, and 15 modified Blalock-Taussig shunts. Three patients died after unifocalization (two early and one late). By the end of the study, eight patients were still waiting for further unifocalization procedures or angiographic assessment. Four patients were rejected for further surgical treatment because of persistent, uncorrectable defects of the pulmonary arterial arborization pattern. Twenty-three patients underwent complete intracardiac repair with two deaths (one early and one late). Postrepair, intraoperative right ventricular/left ventricular systolic pressure ratio after complete surgical repair ranged from 0.4 to 1.0 (mean = 0.63, standard deviation = 0.14). At the end of follow-up, 21 survivors of complete repair were free of significant symptoms. Unifocalization procedures designed to improve the pulmonary arterial arborization pattern of patients with pulmonary atresia and ventricular septal defect with or without central pulmonary arteries can prepare a significant number of these patients for successful intracardiac repair of the anomaly.     

Major aorto-pulmonary collateral arteries of patients with pulmonary atresia and ventricular septal defect are dilated bronchial arteries.

OBJECTIVE: To test the hypothesis that major aorto-pulmonary collaterals (MAPCAs) have the same anatomy as bronchial arteries. METHODS: Two hundred and thirty-eight angiographies performed on 61 patients with pulmonary atresia, ventricular septal defect (VSD), and MAPCAs constituted the basis for this study. This represented all available angiographies performed on this patient group at our institution during the period 1972-2001. MAPCA anatomy was compared to bronchial artery anatomy as described in previous publications. RESULTS: Each patient had one to five MAPCAs (mean 3.2+/-0.94). A mean of 2.6+/-0.66 MAPCAs came from the descending aorta. MAPCAs with anatomy similar to right intercosto-bronchial arteries were found in 87% of the patients. Fifty percent of the patients had MAPCAs originating from the subclavian artery regions. These numbers were all similar to those previously described for bronchial arteries. All MAPCAs had anatomy similar to bronchial arteries. The distribution in different branching patterns of MAPCAs arising from the aorta was similar to the distribution of bronchial arteries described in previous angiographic studies (p=0.32 and p=0.24). CONCLUSIONS: In patients with pulmonary atresia and VSD, MAPCAs are likely to be dilated bronchial arteries. Bronchial arteries may have limited growth potential and their known vasoreactivity might preclude any long-term beneficial effects of unifocalization procedures.     

肺动脉闭锁合并室间隔缺损及大的体肺动脉侧支患者肺循环的解剖分析和临床意义

目的 分析肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支的患者肺循环的病理解剖特点,并探讨其临床意义.方法 回顾性分析2002年4月至2010年6月33例肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支的患者,男性21例,女性12例,年龄11个月～29岁.根据1999年国际儿童心脏外科数据和命名会议的分型标准,B型22例,C型11例.29例(87.9%)存在自身肺动脉,其中发育较好的6例(18.2%),发育不良或左右肺动脉无汇合的23例(69.7%).4例(12.1%)自身肺动脉完全缺如.其中31例进行了33次手术治疗,包括主动脉-肺动脉分流手术8例,一期单源化手术2例,矫治手术23例.结果 分流手术和一期单源化手术的患者术后动脉氧饱和度83%～90%.矫治手术早期死亡1例,死亡原因为多脏器功能衰竭;低心排血量综合征4例,低氧血症(氧合指数<150 mmHg,1 mmHg=0.133 kPa)3例.16例矫治手术患者随访超过1年,右心室压41～99 mmHg;肺动脉瓣中度反流2例;射血分数>50%者14例,<50%者2例.结论 肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支应根据肺血管的解剖特点采取个体化治疗的方案,存在中央肺动脉、左右肺动脉有汇合、体肺动脉侧支与肺动脉之间存在肺内交通是决定一期单源化和心内矫治手术的关键.

Abstract：

Objectives To analyze the anatomy features of the pulmonary circuits in the patients with pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCA), and discuss the clinical significance. Methods From April 2002 to June 2010, the anatomy features of pulmonary circuits in 33 patients with PA/VSD/MAPCA were examined and analyzed. There were 21 male and 12 female patients. The age ranged from 11 months to 29 years. The anatomic types of PA/VSD included group B for 22 cases, group C for 11 cases. Thirty-one patients of them underwent 33 operative procedures. The operations included aorta-pulmonary shunt in 8 cases, one stage unifocalization with VSD open in 2 cases, complete repair in 23 cases. Results Twenty-nine (87.9%) patients had native pulmonary arteries, 6 of them were normal size and 23 were hypoplastic size. Four patients (12. 1%) had no native pulmonary arteries. The postoperative oxygen saturation of the patients undergone shunt and one stage unifocalization was increased to 83% to 90%. There was one early death after complete repair because of multiorgan function failure. There were 4 cases of severe low cardiac output and 3 cases of respiratory function failure. Sixteen patients after complete repair were followed up more than one year. The postoperative right ventricular pressure was 41 to 99 mmHg (1 mmHg =0. 133 kPa). The ejection fraction value was more than 50% in 14 patients and less than 50% in 2 patients. Two patients had medium pulmonary insufficiency. Conclusions An individualized approach based on the anatomy of the pulmonary circuits permits achievement in the patients with PA/VSD/MAPCA. The surgical strategy for PA/VSD/MAPCA mainly depends on the anatomy features of native pulmonary arteries, confluent pulmonary arteries and MAPCA.     

Use of azygos vein as interposition graft for surgical unifocalization of pulmonary blood supply

Hemodynamic repair in patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries with arborization defects, and major aortopulmonary collaterals necessitates prior unifocalization of pulmonary artery blood supply. When direct anastomosis between these collateral vessels and the central pulmonary arteries is not technically feasible, an interposition graft is required. Dacron, polytetrafluoroethylene, and pericardium have been used for this purpose. We describe our experience with the use of azygos vein as an interposition graft in 3 patients who underwent unifocalization of the pulmonary blood supply to the right lung. Our experience suggests that the azygos vein is a useful graft for use in this specific form of unifocalization procedure.     

合并室间隔缺损及粗大体肺侧支的肺动脉闭锁的选择性侧支融合术及粗大体肺侧支的再认识

目的探讨选择性侧支融合术或单源化治疗(unifocalization,UF)在室间隔缺损(VSD)及粗大体肺侧支(MAPCAs)的肺动脉闭锁(PA)患儿中的应用,并从组织病理学角度深入剖析不同解剖形态的MAPCAs。方法2017年6月至2018年12月,6例合并粗大体肺侧支及室间隔缺损的肺动脉闭锁(MAPCAs/VSD/PA)患儿实施选择性UF术加一期根治术,男4例,女2例;年龄6~96个月;体质量5.0~23.0 kg。术前均行心导管、选择性体肺侧支造影及心脏CTA检查,明确体肺侧支种类及解剖分布。对造影提示参与肺"气血交换"的粗大体肺侧支实行选择性UF,对不参与肺"血气交换"的功能性体肺侧支术中结扎或不处理。术中留取不同种类粗大体肺侧支的组织行病理检查,根据组织病理学结果明确侧支类型。结果围术期及随访期间无死亡。术前心导管、选择性侧支检查及术中组织病理学检查证实,参与肺"血气交换"的功能性MAPCAs具有肺动脉壁结构,属于弹性动脉;不参与肺"血气交换"的非功能性MAPCAs为小动脉壁结构,属于肌型动脉。结论术前可通过选择性MAPCAs造影检查明确参与肺"血气交换"功能性侧支,功能性侧支属于弹性动脉,具有肺动脉壁结构,术中对功能性侧支进行UF术可取得良好的手术效果。     

Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach

BACKGROUND: The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels. METHODS AND RESULTS: Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications. CONCLUSION: Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.     

Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect

BACKGROUND: The frequency, distribution, and surgical importance of coronary artery to pulmonary artery (CAPA) collaterals have not been established. The aim of this study was to establish prevalence, anatomical pattern, and significance of CAPA in patients with pulmonary atresia and ventricular septal defect (PA/VSD). METHODS: We reviewed cardiac catheterization and operative data of 87 consecutive, unselected patients who underwent one-stage complete unifocalization for PA/VSD and assessed major systemic to pulmonary collaterals from July 1992 to June 1998. RESULTS: CAPA collaterals were diagnosed in 9 of 87 patients (10%). The collaterals originated from the left coronary artery system in 7 patients and the right in 2. Collaterals from the left coronary system arose from the left main coronary artery in 3 patients and the circumflex in 4. All collaterals joined the central pulmonary artery, which bifurcated and supplied both lungs. One collateral from the right coronary system joined the stump of the main pulmonary artery and the other gave origin to a true left pulmonary artery, which was the sole supply to 75% of the left lung. Coronary artery enlargement was seen in 2 patients only. No patient had evidence of myocardial ischemia. Coronary collaterals comprised a dual source of pulmonary blood flow in all but 1 patient. During unifocalization, the CAPA collaterals were ligated at its origin in all cases, and the collateral from the right coronary to the left pulmonary artery was unifocalized. CONCLUSIONS: The prevalence of CAPA collaterals in patients with PA/VSD is approximately 10%. The diagnosis may be missed without appropriate angiograms. We recommend selective ascending aortogram or selective coronary angiogram in all patients.     

Increasing experience with integrated approach to pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVES: To validate the preliminary surgical results of 'integrated approach' to pulmonary atresia, ventricular septal defect (VSD), and multiple aortopulmonary collateral arteries by retrospective analysis of our center experience. METHODS: Between 01/94 and 03/02, 37 patients aged 22 days to 13 years underwent surgery for pulmonary atresia, VSD, and multiple aortopulmonary collaterals. Case selection was based on preoperative calculation of total neopulmonary arterial index (TNPAI), pulmonary arterial index (PAI), and pulmonary arteries-to-collateral arteries lung segment perfusion ratio (S(pa):S(ca)). The decision for a possible VSD closure during one-stage procedures was based on an intraoperative pulmonary flow study. Twenty-five patients with a TNPAI equal to or greater than 150 mm(2)/m(2) underwent primary unifocalization, irrespective of PAI and S(pa):S(ca). Conversely, 12 patients with a TNPAI less than 150 mm(2)/m(2) and hypoplastic (PAI less than 100 mm(2)/m(2)) dominant (S(pa):S(ca) greater than 1) pulmonary arteries received a first-stage right ventricular outflow tract reconstruction, followed by unifocalization and repair (i.e. VSD closure) in nine cases. RESULTS: Among 34 patients who received total unifocalization, the overall repairability rate was 85% (first instance repairs: n=27; delayed VSD closure: n=2; 95% confidence interval, CI: 73-97%), with a survival rate at 7 years of 81%. Repaired survivors (n=26) are asymptomatic (n=22) or mildly symptomatic (n=4) at a follow-up interval of 43+/-28 months, with a 0.48+/-0.2 mean haemodynamic right ventricular/left ventricular pressure ratio, whereas palliated ones are waiting for either repair (n=3) or catheter study (n=2). Analysis of results has shown the following: (1) 100% (34/34 cases) feasibility of one-stage unifocalization in patients with a preoperative TNPAI equal to or greater than 150 mm(2)/m(2), whereas combined repairability rate was 79% only (95% CI: 65-93%); (2) 100% (12/12 cases) fulfillment of criteria for second-stage repairability (acquired TNPAI greater than 150 mm(2)/m(2)) in all patients treated with right ventricular outflow tract reconstruction; and (3) 93% (95% CI: 83-100%) overall accuracy of intraoperative flow study in predicting either postrepair mean pulmonary arterial pressure (VSD closed: n=23) or balanced pulmonary to systemic blood flow ratio (VSD left open: n=4). CONCLUSIONS: Increasing experience with 'integrated approach' to pulmonary atresia, VSD, and multiple aortopulmonaty collaterals has confirmed the preliminary results of our surgical series. The pulmonary flow study remains the most accurate intraoperative test for successful management of VSD during unifocalization procedures     

Surgical strategies for pulmonary atresia with ventricular septal defect associated with major aortopulmonary collateral arteries

Treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is challenging and controversial. Basically, the collateral arteries are treated surgically by unifocalization to integrate them and unify the blood flow in the pulmonary circulation. These major collateral arteries are anatomically similar to the bronchial arteries; however, they develop into different vessels because of different environmental exposures. Currently, treatment plans involving surgical intervention in early infancy to address the multiple variations of major aortopulmonary collateral arteries are being established to achieve definitive intracardiac repair. Historically, several surgical approaches have been proposed, including multi-stage unifocalization performed by lateral thoracotomy, rehabilitation of the central pulmonary artery followed by intracardiac repair, and single-stage unifocalization by a midline incision. Recently, single-stage unifocalization performed during infancy has become the preferred method for achieving low right ventricular pressure after closing the ventricular septal defect. Furthermore, for the maintenance of the lowest possible right ventricular pressure after definitive repair, combination therapy or hybrid therapy with catheter and surgical intervention is essential. The surgical treatment strategy for a single ventricle with major aortopulmonary collateral arteries is more difficult than that involving two ventricles.     

One-stage unifocalization and palliative right ventricular outflow tract reconstruction

We report a successful case of one-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction for pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries and central pulmonary arterial absence. Through a median sternotomy, one-stage unifocalization was accomplished using autologous pericardial conduit and no prosthetic material. To achieve an adequate pulmonary blood flow, the right ventricular outflow tract was reconstructed so that it would be 70% of the normal pulmonary annular diameter, while the ventricular septal defect was left open. One-stage unifocalization concomitant with palliative right ventricular outflow tract reconstruction is considered to be a good surgical option for selected patients with these diseases.     

Evolving surgical management for ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries

BACKGROUND: The purpose of this study was to evaluate the results of various surgical modalities that have been evolving for the treatment of ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: From 1993 to May 1997, 14 patients (group 1) were treated with staged unifocalization through thoracotomies and final repair by midsternotomy. From June 1997 to February 1998, 10 patients (group 2) were treated with midsternotomy, single-stage complete unifocalization, and repair. RESULTS: In group 1, 14 patients had 21 procedures (1.5 procedures per patient), of which 3 patients (21%) had final correction. There were two deaths (14%). One patient died of blocked shunt. Another patient who had aneurysmal dilation of homograft tubes that were used for unifocalization died after final repair because of low cardiac output. In group 2, 10 patients had ten surgical procedures for complete unifocalization and 9 of 10 (90%) of them achieved final correction. One patient with low cardiac output in whom we did not close the ventricular septal defect died (10%) of suprasystemic right ventricular pressure. CONCLUSION: In single-stage complete unifocalization, more patients had final correction. It reduces the number of operations and hospitalization and hence is more cost effective than multistaged procedures.     

Median sternotomy single stage complete unifocalization for pulmonary atresia, major aorto-pulmonary collateral arteries and VSD-early experience.

OBJECTIVE: It is a prospective study to assess the results of median sternotomy, single stage complete unifocalization and repair for ventricular septal defect (VSD), pulmonary atresia and major aorto pulmonary collateral arteries (MAPCAs). METHODS: From June 97 to August 98, 20 patients were treated with single stage complete unifocalization and repair. Their ages ranged from 6 months to 11 years. Through median sternotomy, all MAPCAs were dissected and looped. On cardiopulmonary bypass, MAPCAs were anastomosed to native pulmonary arteries (PAs) or to MAPCAs. VSD was closed if possible and RV to PA continuity was established with a homograft conduit. If complete repair was not suitable, central shunt was done from ascending aorta to reconstructed PA with a polytetrafluroethylene graft. The patients were divided into three groups according to the arborization pattern in the lungs. Group 1 had well formed native PAs with MAPCAs, group 2 had hypoplastic PAs with MAPCAs and group 3 had only MAPCAs. RESULTS: Twenty patients had 21 procedures. All MAPCAs were unifocalized with tissue-to-tissue anastomosis for future growth, except one in whom polytetrafluroethylene tube graft was used to attain the confluence. In group 1, all seven patients had complete unifocalization and repair. In group 2, four patients had RV to PA conduit and two patients had central shunt. In group 3, three patients had complete repair, three patients had RV to PA conduit and one patient had central shunt. There were three deaths, two in group 2 and one in group 3. The first patient died due to a wrong decision to close the VSD, the second patient died due to missed large MAPCA in preoperative angio and the third patient was a 7-year-old boy who died with irreversible pulmonary vascular changes due to unprotected MAPCAs. CONCLUSIONS: To conclude, complete repair/RV-PA conduit/central shunt should be done according to the size of the total pulmonary vasculature in patients with group 1, 2 and 3 with protected PAs/MAPCAs and in hypoplastic or absent PAs with unprotected MAPCAs (less than 1 year) and protected MAPCAs. We are yet to determine the surgical procedure to be performed in hypoplastic/absent PAs with unprotected MAPCAs more than 1 year. It is very essential to delineate all the MAPCAs up to the level of the diaphragm preoperatively.     

Correction of pulmonary atresia with ventricular septal defect in the absence of the pulmonary trunk and the central pulmonary arteries (so-called truncus type IV)

A child with pulmonary atresia and ventricular septal defect and no extraparenchymal pulmonary arteries had all the bronchopulmonary arterial segments connected to naturally occurring systemic-pulmonary collaterals. A three-staged surgical correction was performed. At the first and second stages, the arteries of each hilus were interconnected with synthetic arteries. At the third stage, the ventricular septal defect was closed, and continuity between the right ventricle and the bilateral pulmonary circulation was established with a valved conduit giving rise to a side arm. Postoperative evolution was good, with acceptable postrepair per right ventricular--left ventricular pressure ratio.     

Postoperative coil embolization of residual MAPCAs greatly improved left heart failure in a patient after corrective surgery for pulmonary atresia, ventricular septal defect and MAPCAs

A male child, aged 1 year, with pulmonary atresia, ventricular septal defect and major aorto-pulmonary collateral arteries (PA, VSD, MAPCA) underwent corrective surgery including MAPCA ligation uneventfully. A few hours after admission to the ICU, severe heart failure, refractory to aggressive cardiac support including epinephrine infusion, became worse. Emergent cardiac catheterization on postoperative day 5 demonstrated the residual MAPCA and its occlusion by coil embolization dramatically resolved heart failure, indicating that the primary cause of this hemodynamic instability was likely excessive left-to-right shunt due to MAPCA. Residual LR shunt should be kept in mind to be a rare but significant cause of postoperative serious heart failure.     

Transluminal balloon angioplasty of pulmonary arteries after palliative reconstruction of the outflow tract from the right ventricle as a preparatory stage for radical surgery]

The authors describe their experience in the use of transluminal balloon angioplasty (TBA) of the pulmonary arteries (PA) carried out in stenosed segments in the late-term period after operation for palliative reconstruction of the right ventricular outflow tract without closure of a ventricular septal defect (VSD) in 7 patients: in 6 with Fallot's tetrad after previously created systemic-pulmonary anastomoses and in one with PA atresia and VSD. TBA was conducted in 11 stenosed segments of the pulmono-arterial tree. The TBA method is suggested as a procedure for preparing the PA for the final stage of operative treatment.     

Unifocalization of Major Aorto-Pulmonary Collateral Arteries in Pulmonary Atresia and Ventricular Septal Defect Using Antibiotic Sterilized Pulmonary Homograft: A Case Report

A unifocalization procedure in a 9-year-old girl with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (MAPCAs) was performed using an antibiotic sterilized pulmonary homograft. A tailored pulmonary homograft was used as an interposition graft to anastomose the pulmonary artery to the larger MAPCA after a side-to-side anastomosis had been done between the two MAPCAs. The pulmonary homograft was connected to the right subclavian artery with a 6-mm Gore-Tex shunt. Postoperative oxygen saturation (SaO₂) levels were satisfactory and a postoperative angiogram showed good unifocalization of the pulmonary vasculature. (J Card Surg 1994;9:115–119)     

Surgical creation of aortopulmonary window in selected patients with pulmonary atresia with poorly developed aortopulmonary collaterals and hypoplastic pulmonary arteries

OBJECTIVE: The morphologic characteristics of the pulmonary circulation vary widely in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. Although we favor single-stage unifocalization and complete repair as the procedure of choice, a subgroup of patients who meet specific criteria have been treated with initial surgical creation of an aortopulmonary window. METHODS: Eighteen patients who were considered unsuitable candidates for single-stage repair underwent surgical creation of an aortopulmonary window. Selection criteria included the following: (1) presence of centrally confluent true pulmonary arteries 1.0 to 2.5 mm in diameter, with a well-developed peripheral arborization pattern; (2) multiple small aortopulmonary collateral vessels, most of which communicated with the true pulmonary arterial system; and (3) the presence of marked cyanosis. RESULTS: There were no early deaths, and the 2 late deaths were both unrelated to the procedure. Follow-up angiography (n = 17) demonstrated good growth of true pulmonary arteries in 15 of 17 cases (88%). Mean pulmonary artery diameter increased from 1.76 mm to 3.45 mm. Subsequent operations have been performed in 15 of 18 cases (83%). Intracardiac repair with or without additional unifocalization was achieved in 8 of these 15 (53%). Seven patients (47%) have undergone staged unifocalization as the next procedure; of these, 3 were able to have intracardiac repair. Thus 11 of the 15 patients who have undergone second operations (73%) have had complete intracardiac repair. CONCLUSIONS: The initial surgical creation of an aortopulmonary window in carefully selected patients can increase the size of the true pulmonary arteries, making these patients better candidates for eventual intracardiac repair. The procedure should be avoided in patients with pulmonary overcirculation, a predominance of isolated supply collaterals, or true pulmonary arteries larger than 2.5 mm in diameter, and it is not applicable without a true pulmonary artery central confluence.     

Very small pulmonary arteries: central end-to-side shunt

Between 1980 and 1989, 28 patients with pulmonary atresia, ventricular septal defect, and very small pulmonary arteries with major aortopulmonary collateral arteries underwent direct central end-to-side shunts as staging procedures. Age range was 2 months to 32 years, with 19 patients less than 1 year of age. Pulmonary artery diameters ranged from 1 to 4 mm, with 24 less than 3 mm. Two patients (7%; 70% confidence limits, 2% to 16%) died after the shunt, and there were two further deaths after subsequent staging or correction. Acute shunt complications included congestive cardiac failure (mild to moderate, n = 8; severe, n = 3) and endocarditis (n = 1). Proximal right pulmonary artery stenoses have occurred in 75% of patients and left pulmonary artery stenoses, in 50%. Satisfactory pulmonary artery growth was achieved, however, in 16 of 24 hospital survivors investigated postoperatively. Twelve patients have proceeded through unifocalization to biventricular repair (in 2 the ventricular septal defect patch was subsequently fenestrated) with one death (8.3%; 70% confidence limits, 1% to 25%). Eight patients are still in staging, and 4 have been excluded from the program because of inadequate unifocalization. The direct central end-to-side shunt has proven satisfactory in attaining pulmonary artery growth in patients with very small central pulmonary arteries.