A case report of definitive repair for pulmonary atresia and intact ventricular septum associated with aortic valve regurgitation

A successful definitive repair for a 10-year-old girl with pulmonary atresia and intact ventricular septum (PA.IVS) associated with aortic valve regurgitation is described. The Fontan type repair was not indicated in this case because of the left ventricular dysfunction due to aortic valve regurgitation and inadequate size of the pulmonary artery. Therefore, right ventricular outflow tract reconstruction, Glen shunt and aortic valve replacement were performed despite severe hypoplastic right ventricle (RVEDVI; 33% of normal) and restrictive tricuspid valve (TVD; 48% of normal). Postoperatively, good result was obtained. There is general agreement that biventricular repair could be safely performed using Glenn shunt, when RVEDVI is above 40% of normal and TVD is above 50% of normal in a patient with PA.IVS. Moreover recently including our case, several successful repairs for PA.IVS with more hypoplastic right ventricle and tricuspid valve have been reported. So it is suggested that the right ventricular outflow tract reconstruction and Glenn shunt can be reliably applied for PA.IVS with more hypoplastic right ventricle and more restrictive tricuspid valve. To our knowledge, this is the first successful report of definitive repair (right ventricular outflow tract reconstruction, Glenn shunt and AVR) for PA.IVS associated with AR.     

肺动脉发育不良型法洛四联症根治术前姑息手术和介入技术的应用

目的 总结姑息手术和介入技术治疗合并肺动脉发育不良的重症法洛四联症的临床经验.方法 2002年12月至2009年12月,1586例患儿行法洛四联症根治术中18例(男12例、女6例)在根治手术前进行了姑息手术和介入技术相结合的复合治疗.合并心血管畸形包括:房间隔缺损3例,左肺动脉缺如2例,左肺动脉起自主动脉1例,永存左上腔静脉1例,合并粗大体肺侧支血管7例.根治手术前行一次姑息手术者13例,两次者4例,三次者1例.手术术式包括改良Blalock-Taussig分流术14例次,Waterston分流术4例次,右室流出道重建术3例次,肺动脉瓣球囊扩张术3例次,肺动脉环缩1例,行侧支血管结扎6例(16支),侧支血管融合1例(2支),侧支血管介入封堵2例(6支).结果 全组无死亡,1例因人工血管堵塞在术后第1天再次行体肺分流术,患儿根治手术前Nakata指数和McGoon比值[(200±81)和(1.77±0.51)]均较姑息手术前[(84±40)和(1.14±0.33)]有明显增加(P＜0.001),末梢血氧饱和度和血红蛋白浓度[(0.71±0.09)和(175±46) g/L]均显著改善[(0.86±0.05)和(149±15) g/L,P＜0.05].所有18例患儿均完成了最终的根治手术.结论 采用姑息手术和介入技术相结合的复合治疗措施能有效改善肺动脉发育,为合并肺动脉发育不良的重症法洛四联症根治手术创造条件.     

Total cavopulmonary connection for grown diminutive pulmonary artery after staged Blalock-Taussig shunt

We report a two-year-old girl with asplenia, {A, L, L} DORV, pulmonary atresia, common AV valve, PDA, and TAPVC, who successfully underwent total cavo pulmonary connection (TCPC). Deep cyanosis was pointed out since birth. Cardiac catheterization performed on the sixth day after birth revealed a diminutive pulmonary artery tree of which PA index was 41 mm²/m². Left modified Blalock-Taussig shunt was created at 27 days of age. The PA index increased to 282 mm²/mm², but disparity in diameter between the left and the right pulmonary artery was yielded by PDA subsidence. Therefore additional contralateral B-T shunt was made at one year of age. Follow-up cardiac catheterization at 28 months of age showed well developed pulmonary artery; PA index of 460 mm²/m², right pulmonary resistance (Rp) of 3.49 units, left Rp of 2.33 units, and estimated total Rp was 1.39. According to study, bidirectional Glenn procedure or TCPC was indicated. Considering neccesity of urgent repair of common pulmonary vein obstruction, regurgitation of the common atrio-ventricular valve and pulmonary artery stenosis, TCPC was performed with concomitant repair of the associated lesions. Severe butterfly-figure stenosis of the central PA was augmented by anastomosing both the left SVC and the left-sided atrium. In conclusion, diminutive pulmonary artery could be adequately grown by phase-in Blalock-Taussig shunts. Pulmonary blood flow scintigraphy was thought to be useful for estimation of pulmonary resistance in such cases with different pulmonary resistance between right and left PA.     

Repair of tetralogy of Fallot with obstruction of right pulmonary artery; report of a case

Patients with tetralogy of Fallot showing unilateral obstruction of a pulmonary artery, especially the right pulmonary artery, are a high-risk group for pulmonary hypertension after repair. This case of tetralogy of Fallot with the obstruction of the right pulmonary artery received a Blalock-Taussig shunt at 7 months old, and the occluded right pulmonary artery caused empyema after surgery. At 2 years old, a cardiac catheter study showed a pulmonary artery index of 193.6 mm2/m2, so we undertook intracardiac repair. After the repair, she showed a relativery favorable clinical course. Systolic pulmonary artery pressure and right ventricular pressure were about 30 and 50 mmHg, respectively. We considered that tetralogy of Fallot with obstruction of right pulmonary artery could be repaired, as long as the pulmonary artery index was within the limits of indication and the left ventricle was well-developed.     

Surgical management of patients with pulmonary valve dysplasia

Pulmonary valve dysplasia is a distinct pathological entity consisting of markedly thickened, deformed, and largely immobile pulmonary valve leaflets. The clinical features and surgical management of 21 consecutive patients operated upon between 1975 and 1985 were reviewed. Mean age at operation was 26.7 months (range: 1 month to 9.5 years). Initial surgical intervention in 3 patients consisted of closed valvotomy or systemic pulmonary artery shunt. One of these patients subsequently had a partial pulmonary valvectomy; the other 2 had total valvectomy and pulmonary annuloplasty. Two patients had partial valvectomy as a primary procedure, and 1 has subsequently required total valvectomy and annuloplasty. Sixteen patients initially had total pulmonary valvectomy, 4 with a simultaneous transannular outflow tract patch. One patient subsequently required a transannular outflow tract patch. Therefore, definitive repair consisted of partial valvectomy in 2 patients, total valvectomy in 19, and a transannular outflow tract patch in 9 patients with a hypoplastic pulmonary annulus. There were no operative deaths, and all patients are asymptomatic at a mean follow-up of 37.9 months. Five patients who underwent total valvectomy were catheterized postoperatively. The mean residual gradient was 25 mm Hg. Pulmonary valvectomy appears to provide both effective relief of stenosis and long-term asymptomatic status in patients with pulmonary valve dysplasia. Total excision of all valve leaflets is recommended. Careful consideration should be given to a simultaneous transannular outflow tract patch if the annulus is hypoplastic.     

Evidence for palliative enlargement of the right ventricular outflow tract in severe tetralogy of Fallot

Objective: If the pulmonary artery (PA) tree in patients with Fallot's tetralogy (TOF) is extremely hypoplastic, a shunt procedure may be difficult and not desirable because of side-effects. Moreover, the failing catch-up growth of the pulmonary annulus is well known. In patients with a severe form of TOF, we performed palliative transannular patching of the right ventricular outflow tract. The early and long-term follow-up was evaluated. Methods: Eleven patients (93 days (10–245 days); 3.5±0.7 kg (2.5–4.3 kg)) had highly symptomatic TOF (Hb: 18±2 g/dl, SO₂: 68±11%); angiographic diameters: RPA: 4.1 mm (2.5–6.4 mm), LPA: 3.4 mm (1.6–7.0 mm), PA trunc: 4.4 mm (2.5–7.0 mm). All 11 underwent transannular enlargement of the right ventricular outflow tract without closure of the ventricular septum defect. A PA index (cross-sectional area of the pulmonary arteries to BSA) was used to compare pre- and postoperative data. For follow-up, the patients were repetitively examined clinically and echocardiographically. Results: Preoperative PA index was 87±40 mm²/m² (normal: 330±35 mm²/m²). Postpalliation angiograms (age: 10–14 months) demonstrated a significant catch-up growth in nine patients (PA index from 99±40 to 310±54 mm²/m²) and inadequate growth in two patients (PA index 63 and 115 mm²/m²). Perioperative mortality was zero. Ten patients (43 months; 6–105 months) underwent elective repair. Six patients received pulmonary homograft valves (6–15 years after repair) because of severe pulmonary valve insufficiency and severe RV dilation. Complications: One patient died 10 months postpalliation due to pneumonia, one patient received a pacemaker after repair and died (2 months post-repair) due to pacemaker failure, a 5-year-old patient died 1 month after repair due to sepsis. All eight long-term survivors (12–17years) are in excellent clinical condition. Echocardiography revealed good RV function and near normal diameters at peak systolic pressures between 25 and 50 mmHg. Only one patient developed brady-arrhythmia; a pacemaker was implanted 8 years after repair and 2 years after homograft implantation. Conclusions: In a very severe form of TOF, palliative right ventricular outflow tract construction may provide the potential for complete repair. In the presented high-risk patient group, mortality was not related to the hypoplastic pulmonary arteries. Obviously, all patients need pulmonary valve implantation in the long run.     

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.     

Pulmonary artery growth after Norwood and bidirectional Glenn procedure

A 19-day-old boy diagnosed with hypoplastic left heart syndrome underwent stage I bilateral pulmonary artery banding and main pulmonary artery-to-descending aorta shunt. A restrictive atrial septal defect existing before stage I recurred after balloon atrioseptostomy. After stage II Norwood and bidirectional Glenn procedure at age nine months, the Nakata index decreased to 73 mm(2)/m(2) (pulmonary artery mean pressure: 15 mmHg) and multiple systemic venous collaterals developed. Thus, we instituted oral sildenafil medication, and undertook surgical chest subcutaneous venous ligation and coil embolizations. Three years later, the Nakata index had increased to 117 mm(2)/m(2) (pulmonary artery mean pressure: 13 mmHg) and a Fontan procedure was successfully performed.     

A case of pulmonary artery rupture due to pulmonary artery catheter malposition after the weaning of cardiopulmonary bypass

An 80-year-old female with aortic regurgitation and angina was scheduled for aortic valve replacement and coronary artery bypass graft. After the induction of anesthesia, central venous catheter and pulmonary artery (PA) catheter were placed into the right internal jugular vein. At the weaning from cardiopulmonary bypass, we noticed pulmonary artery pressure (PAP) on the monitor indicating the PA catheter tip being wedged. Therefore, we pulled the PA catheter until the wedging PAP wave disappeared. However, immediately after repositioning of the PA catheter, massive fresh bleeding was found in the endotracheal tube. We estimated the cause of pulmonary bleeding was PA rupture due to malpositioned PA catheter because surgeons had not manipulated the PA during the operation. Soon after restarting the cardiopulmonary bypass, pulmonary bleeding was stopped. Examination with fiberoptic bronchoscope revealed no further bleeding and weaning from cardiopulmonary bypass was accomplished smoothly. Postoperative course was uneventful.     

Diagnosis of a left-sided superior vena cava during placement of a pulmonary artery catheter

We report a case of a left sided superior vena cava (SVC) that was diagnosed during placement of a pulmonary artery (PA) catheter. After entering the left internal jugular, the PA catheter passed into the left side of the heart, through the aortic valve, and into the aorta. This was an unusual cause of right-to-left shunting and persistent cyanosis in a patient who had undergone two open cardiac procedures, including repair of an atrial septal defect. Cardiac catherization and echocardiography also failed to reveal the abnormality.

The embryology and physiology of a left sided SVC is reviewed, including an historical perspective. A discussion of the variants of the syndrome is included, as is a review of aberrant placement of central venous catheters.     

Follow-up study of pulmonary artery configuration in hypoplastic left heart syndrome

Objective. Pulmonary artery (PA) distortion significantly compromises the outcome of the staged approach to the Fontan operation in patients with hypoplastic left heart syndrome (HLHS). This retrospective study was designed to investigate the influence of the initial operation on postoperative PA anatomy. Methods. Forty-nine patients with HLHS and its variant were enrolled in this study. As an initial palliation, the Norwood operation with a modified Blalock-Taussig (BT) shunt was performed in 12, the Norwood operation with a right ventricle to pulmonary artery (RV-PA) shunt in 31, and bilateral PA banding in 6. The incidence and risk factors of postoperative central pulmonary artery stenosis (PS) were investigated, and the PA configuration was followed up until post-Fontan status. Results. Twenty-two patients (51.2%) had developed central PS after the Norwood operation (33.3% with a BT shunt vs. 58.1% with a RV-PA shunt). The RV-PA shunt with a polytetrafluoroethylene (PTFE) patch at the distal pulmonary stump significantly decreased the central PS (P = 0.035). The PA index after the Norwood operation was not statistically different between the BT and RV-PA shunt groups, although in the RV-PA group it was significantly higher in patients with a PTFE patch on the distal PA stump. PA plasty was performed in 16 patients in the second-stage palliation and in 15 with the Fontan completion. Freedom from PA plasty was significantly lower in the RV-PA shunt group than in the BT shunt group (63.5% vs. 31.1% at 5 years, P = 0.034). Six patients initially palliated with bilateral PA banding had no stenosis at the banding site in the Norwood + Glenn operation, and one patient required stent placement for left PS in the Fontan completion. Post-Fontan catheterization (n = 31) showed central venous pressure of 11.5 ± 2.6 mmHg, cardiac index of 3.6 ± 0.8 l/kg/min, and PA index of 194.0 ± 58.4 mm²/m²; there was no difference between the groups. Conclusion. The incidence of central PS after the Norwood operation was significant, and the shunt type and procedure for the distal PA stump influenced the postoperative configuration of the central PA. With an aggressive surgical approach to central PS, PA anatomy was satisfactory with good hemodynamic variables after Fontan completion. Bilateral PA banding did not cause later vascular deformity. Presented at the 59th Annual Scientific Meeting of the Japanese Association for Thoracic Surgery, held in Tokyo, Japan, October 1–4, 2006     

Swan-Ganz catheter-induced pseudoaneurysm of the pulmonary artery

Pseudoaneurysm of the pulmonary artery (PA) induced by Swan-Ganz catheter injury is an important complication with high mortality. We report a case of PA pseudoaneurysm treated by PA repair. A 52-year-old woman developing infiltrate in the right lung field in chest radiography after a second mitral valve replacement was diagnosed with PA pseudoaneurysm confirmed by contrast-enhanced computed tomography and pulmonary angiography. The cause was considered Swan-Ganz catheter injury. The patient was carefully observed because there was no evidence of bronchial hemorrhage. The perforation was closed and the cavity plicated under extracorporeal circulation to avoid delayed rupture of the pseudoaneurysm when she underwent a third mitral valve replacement.     

Double outlet right ventricle and pulmonary atresia with a birth weight of 1092 g

A premature infant with double outlet right ventricle and pulmonary atresia with a birth weight of 1092 g is reported. He underwent right modified Blalock-Taussig (RMBT) shunt with an expand-polytetrafluoroethylene (ePTFE) tube of 3.0 mm in diameter between the right subclavian artery and the right pulmonary artery through right thoracotomy. Eleven days later, he had to undergo central shunt between the innominate artery and the main pulmonary trunk due to poor pulmonary blood flow. Soon after the central shunt, severe heart failure occurred due to excessive pulmonary blood flow. RMBT division was performed immediately. He finally attained definitive repair at 17 months of age. Postoperative course was uneventful and he was discharged on the 17th postoperative day.     

肺动脉融合术治疗肺动脉闭锁合并大主肺侧支动脉

目的 总结肺动脉融合术治疗肺动脉闭锁、室间隔缺损合并大主肺侧支动脉的初步经验.方法 1999年12月至2007年6月完成肺动脉闭锁,室间隔缺损合并大主肺侧支动脉(PA/VSD/NAPCAs)的肺动脉融合术17例,其中男7例,女10例;年龄0.8～18岁,平均(6.0±5.6)岁;体重6.5～55.0 kg,平均(20.0±14.9)kg.术前固有肺动脉指数(PAJ)为42.0～366.1mm2/m2,平均(133.7±87.8)mm2/m2.手术入路包括正中剖胸10例,正中+左后外侧剖胸5例,正中+右后外侧剖胸2例.一期肺动脉融合及心内畸形矫治术12例,肺动脉融合及姑息性体肺分流术4例,肺动脉融合及姑息性右室流出道扩大1例.全组病儿术前造影发现大主肺侧支动脉共44支;术中融合29支,结扎5支.肺动脉闭锁矫治术及肺动脉融合术前介入栓堵3个侧支1例;肺动脉融合及体肺分流术后介入栓堵2个残留侧支1例.结果 全组术中转流174.3min,主动脉阻断88.7min.术后呼吸机辅助7.4d,术后ICU 11.8d.生存11例,平均新建肺动脉指数(TNPAI)249mm2/m2.6例死亡,其中2例TNPAI<200mm2/m2,死于肺血管发育不良,术后发生低心排出量综合征(低心排)和突发室性心律失常;1例因低心排无法脱离体外循环,尸检发现肺小动脉明显肌型化,管腔高度狭窄,证实术前局部肺段已有严重的肺高压;3例>450 mm2/m2分别死于严重肺部感染、渗出、急性肾功能衰竭,反复气道大量出血和顽固性室性心律失常.其他主要并发症为大脑皮层盲、膈肌麻痹和阴沟杆菌性肺炎肺出血各1例.结论 完全矫治+肺动脉融合是治疗肺动脉闭锁/室间隔缺损合并大主肺侧支动脉最根本的治疗手段,但手术操作复杂.由于同一病儿肺动脉病变的多样性,常见MAPCAs高压及狭窄并存.适应证选择和手术技巧的提高仍需不断的探索.     

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.     

Surgical strategy for pulmonary atresia with ventricular septal defect associated with severely hypoplastic or absent central pulmonary artery

In patients of pulmonary atresia with ventricular septal defect associated with profound hypoplasia or complete absence of central pulmonary artery, surgical strategy depends on the morphology of native pulmonary artery and major aortopulmonary collateral arteries (MAPCAs). Type of surgery and timing of operation are determined by detailed analysis of angiography and 3-dimensional computed tomography (3D-CT) scan. When native central pulmonary artery exists, palliative procedure like systemicpulmonary shunt or palliative right ventricular outflow tract is recommended in order to promote growth of native pulmonary artery, followed by Rastelli-type definitive repair with/without uniforcalization of MAPCAs. In case of central pulmonary artery absence, staged reconstruction of bilateral pulmonary vascular bed by repeated uniforcalization or 1 stage uniforcalization and Rastelli operation is indicated. However, even after definitive repair, careful attention and scheduled cardiac catheterization with aggressive catheter intervention are required to maintain adequate pulmonary circulation.     

Intrathoracic pulmonary artery catheter allocation in the background of left atrial dilatation

AIM: The aim of the study was to find out whether dilatation of the left atrium (LA) influences the intra-thoracic distribution of thermodilution pulmonary artery (TPA) catheter in either branch of pulmonary artery and compare the measured cardiac output. METHODS: In this prospective study of 132 consecutive patients in a university hospital setting, LA size and ejection fraction was assessed by echocardiography, in the preoperative period. In 66 patients posted for coronary artery bypass grafting (Group 1), a standard anaesthesia regimen was used and TPA catheter was floated through the right internal jugular. In another 66 patients of long-standing mitral stenosis for mitral valve repair/replacement (Group 2), TPA catheters were similarly floated. Intrathoracic placement of the tip of the TPA catheter into the right or left pulmonary artery (PA) was confirmed on chest X-ray. TPA catheter length to its wedging, intra-arterial pressure, heart rate, PA pressure, pulmonary artery wedge pressure (PAWP) and cardiac output by thermodilution technique were noted. RESULTS: Leftwards TPA catheter placement was significantly (p<0.001) more frequent (71%) in mitral stenosis patients (group 2) than the CABG (group 1), (18%). On regrouping the observations of rightwards placed TPA (Group R) and leftwards placed TPA (Group L), we observed that large LA (> or = 25 mm3/m2) body surface area (BSA) and high PAWP (> or = 20 mmHg) was associated with significantly (p<0.001) higher incidence of leftwards TPA catheters. Positive predictive value of both the factors in combination was significantly higher (96%) than individual factors large LA (81%) and high PAWP (88%). CONCLUSION: In long standing mitral stenosis, left atrium enlargement, with high PAWP and the hypokinesia of left atrium (atrial fibrillation) likely to influence the angulation of left PA with main PA and so the predominant entry of TPA catheter tip in left PA.     

左冠状动脉异常起源于肺动脉的诊断和外科治疗

目的总结先天性左冠状动脉异常起源于肺动脉的临床特点、诊断和外科治疗经验.方法1993年7月至2005年10月,外科治疗10例左冠状动脉异常起源于肺动脉病人,其中男5例,女5例;年龄13 d～40岁.术前均经超声心动图和心导管造影检查明确诊断.单纯左冠状动脉异常起源于肺动脉者3例,合并二尖瓣关闭不全6例,同时合并二尖瓣关闭不全及左心室心尖部室壁瘤1例.行异常冠状动脉结扎4例,在体外循环下行肺动脉内隧道成形术3例,升主动脉左冠状动脉开口植入术3例;同期行二尖瓣成形5例,室壁瘤切除1例.结果早期死亡1例.随访1个月～11年.1例因严重二尖瓣关闭不全而于第1次术后16个月再行二尖瓣置换术,余者无心肌缺血或梗死、残余分流和晚期死亡,心功能恢复到Ⅰ级.结论左冠状动脉异常起源于肺动脉者存在明显的血流动力学和心血管形态学改变,应尽早确诊、尽快手术治疗,适当的手术方法是取得良好外科手术疗效的关键.     

Palliative right ventricular outflow reconstruction in tetralogy of Fallot with pulmonary atresia and aberrant right subclavian artery; report of a case

A 1-month-old girl weighting 3.1 kg was diagnosed as tetralogy of Fallot (TOF) with pulmonary artery atresia (PA) and aberrant right subclavian artery. Before the operation, pulmonary blood flow from a ducus arteriosus was maintained by lipo prostaglandin E1 (PGE1). The patient underwent palliative right ventricular outflow tract reconstruction (pRVOTR) because the proximal aberrant right subclavian artery was stenotic and the ductus arteriosus and branch of the left pulmonary artery were so close. Postoperative course was uneventful and pulmonary artery showed good growth. The pRVOTR as 1st procedure is a useful method for hypoplastic pulmonary artery to get equal and good growth. Although there are controversies about the size of right ventricular outflow tract (RVOT), 5 or 6 mm diameter of RVOT is recommended for the operative repair of hypoplastic left heart syndrome. We concluded that the pRVOTR should be one of the options as 1st palliative procedure for TOF with PA and diminutive pulmonary artery.     

Palliative repair of aortic atresia associated with tricuspid atresia and transposition of the great arteries.

Successful palliative repair of aortic atresia and hypoplastic aortic arch associated with tricuspid atresia in a neonate is described. The repair consisted of reconstruction of the hypoplastic aortic arch with an equine pericardial patch, division of the patient ductus arteriosus, connection of the pulmonary artery to the aorta, implantation of the proximal part of the ascending aorta into the main pulmonary artery, and anastomosis of a polytetrafluoroethylene graft 5 mm in diameter between the right ventricular outflow tract and the central pulmonary artery, which was transferred anteriorly to the main pulmonary artery.