全腔静脉-肺动脉连接术及双向格林分流术治疗复杂先天性心脏病的疗效

目的评价全腔静脉-肺动脉连接术(TCPC)和双向格林分流术(Glennshunt)治疗功能性单心室等复杂先天性心脏病的疗效。方法2002年1月至2004年5月,12例患者接受了TCPC及双向Glenn分流术。病种分别为三尖瓣闭锁(TA)3例,二尖瓣闭锁(MA)1例,右位心完全性大动脉错位(D-TGA)伴完全型房室共同通道(CAVSD)1例,右位心大动脉错位1例,左旋心单心室1例,单心室2例,D-TGA3例。12例中3例行双向Glenn分流术(TA、右位心D-TGA伴CAVSD和单心室各1例),其余9例行TCPC手术(TA2例、MA1例、右位心D-TGA及肺动脉狭窄1例、左旋心1例、单心室1例、D-TGA3例)。结果手术后近期效果:12例患者中11例存活,1例D-TGA患者术后19h死于急性肾功能衰竭,住院死亡率8.3%。手术中CPB时间78～155min,升主动脉阻断时间36～122min。机械辅助通气时间8～18h,ICU监护时间15～32h,住院时间16～58d。术后中心静脉压(即肺动脉压)8～18mmHg,脉搏血氧饱和度0.80～0.96。TCPC后,2例单心室和2例D-TGA患者发生乳糜胸,经置胸腔引流管和综合性治疗,分别于术后22d、33d、36d和48d痊愈出院。手术后中远期效果:术后随访2至28个月,1例右位心D-TGA伴CAVSD患者在双向Glenn分流术后1年因肺动静脉瘘(PAVF)引发大咯血死亡;其余10例存活,心功能～级,无恶性心律失常、无血栓形成和脑部并发症发生。结论TCPC和双向Glenn分流术是治疗功能性单心室等复杂先心病的生理性矫治方法,具有较好的手术安全性和近期、中远期的效果,手术中足够大的腔静脉和肺动脉吻合口和围手术期的积极处理是取得良好临床效果的关键。     

小儿复杂型先心病一期和分期全腔肺吻合术

1998年 3月至 2 0 0 2年 3月 ,我们共收治 15例复杂型心内畸形病儿 ,分别采用一期和分期全腔肺吻合术 (totalcavopulmonaryconnection ,TCPC)治疗 ,现报道如下。资料和方法　本组中 11例行一期手术 ,年龄 ( 5 7± 0 8)岁 ;单心室 6例 ,三尖瓣闭锁 5例。 4例行分期手术 ,年龄( 7 1± 0 5 )岁 ,单心室、三尖瓣闭锁各 2例 ,TCPC术前分别先做单侧或双侧双向腔肺分流术 (Glenn)术 ,行Glenn术时年龄( 1 8± 0 6 )岁 ,两次手术平均间隔 ( 5 7± 0 3)年。在进行双向腔肺吻合术前 ,分期手术组比一期手术组具有较多的高危因素 ,如年龄小、肺…     

心外管道全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的总结心外管道全腔静脉-肺动脉连接术（ECTCPC）治疗复杂先天性心脏病的临床经验,并就手术适应证、手术方法及手术效果进行讨论。方法1998年6月至2006年12月,68例先天性心脏复杂畸形的患者接受了ECTCPC。包括单心室伴有大动脉转位、肺动脉瓣狭窄45例：三尖瓣闭锁、右心室发育不良19例;三尖瓣下移畸形并右心室发育不良4例。其中合并永存左上腔静脉6例,双向Glenn术后行全腔静脉-肺动脉连接术18例（其中包括单心室、肺动脉闭锁、左肺动脉狭窄双向Glenn术后1例）,单心房、单心室、心上型完全性肺静脉异位引流、多发粗大体肺侧支1例。全组采用体外循环下手术共57例,其中8例患者因需要矫正心内畸形在主动脉阻断下手术外,其余49例均在全身麻醉并行体外循环心脏跳动下进行;非体外循环下手术11例。结果术后早期死亡2例,病死率为2．9％。其中1例死于术后反复肺内出血,1例死于上消化道反复大出血。66例痊愈出院,术后随访1个月至8年,无晚期死亡。所有患者症状消失,血氧饱和度90％～96％,恢复良好。结论ECTCPC方法简便易行,术后并发症较少,效果好,较其他术式有较大优点。     

功能性单心室合并肺动脉高压的分期手术结果

目的分析功能性单心室合并肺动脉高压(FSV-PH)的分期手术结果,探讨初期手术时机及相关风险因素与治疗结果的关系。方法回顾性分析2008年4月至2015年12月我院收治功能性单心室合并肺动脉高压129例患者的临床资料,其中男81例、女48例,右心室双出口71例,三尖瓣闭锁17例,大动脉错位7例,单心室33例,完全性房室间隔缺损1例。初期进行肺动脉环缩术(PAB),之后根据肺血管状况,适时分期行Glenn手术和Fontan手术。结果 129例患者共行159例次PAB,术后死亡6例(4.7%),失访9例;87例(67.4%)患者行二期Glenn手术,死亡2例;43例(33.3%)患者行三期Fontan手术,术后死亡3例。32例合并主动脉缩窄、主动脉弓中断、心脾综合征、完全性肺静脉异位引流或房室瓣反流,分别有15例(46.9%)行Glenn手术和6例(18.8%)行Fontan手术。14例患者出现左室流出道狭窄。结论早期PAB有利于FSV-PH进行后期的Glenn和Fontan手术,合并复杂畸形是降低Glenn和Fontan完成率的危险因素。     

全腔肺动脉连接术在复杂先天性心脏病中的应用

许多复杂先天性心脏病（先心病），尤其是合并单心室发育不良、一组房室瓣等病儿往往只能接受Fontan类手术，目前应用最多的是双向Glenn术和全腔肺动脉连接术。全腔肺动脉连接术（TCPC）是完全性生理矫正，在我院占同期Fontan类手术的58．1％（25143例）。现总结报道2001年8月至2005年5月25例复杂先心病病儿在我院行全腔肺动脉连接术的治疗体会，报道如下。     

新生儿复杂先天性心脏病的外科治疗

目的总结29例新生儿复杂先天性心脏病的外科治疗经验。方法29例患先天性心脏病的新生儿,年龄3～28d,其中室间隔缺损3例,完全性大动脉错位10例,肺动脉闭锁1例,三尖瓣闭锁3例,单心室1例,法洛四联症6例,完全性房室管畸形4例,永存动脉干1例,所有患者合并房间隔缺损和动脉导管未闭。29例患者均在深低温低流量体外循环下行一期矫治术。结果术后死亡4例(13.8%),其中1例完全性大动脉错位和1例肺动脉闭锁患者死于低心排血量综合征,2例死于灌注肺、低氧血症,其余25例患者均治愈出院。随访19例,随访时间1～31个月,体重及各项发育指标与正常同龄儿基本相同。结论新生儿心脏手术麻醉、体外循环应平稳,手术操作要精细,手术成功的关键是畸形矫正满意及良好的心肌保护和肺保护。     

肺动脉环缩术治疗不同年龄伴肺动脉高压单心室

目的 总结肺动脉环缩术( PAB)在不同年龄段伴肺动脉高压先天性心脏病患儿行单心室修复术中的应用效果.方法 49例合并重度肺动脉高压仅能行单心室修复的复杂畸形患儿,男31例,女18例;年龄0.2～10岁,平均(7.8±3.8)岁;体重5～24 kg,平均(8.82 ±4.24) kg;经皮血氧饱和度( SPO2)0.85～1.00,平均(0.90±0.04);术前肺动脉平均压(mPAP) 52 ～91 mm Hg(1 mm Hg =0.133kPa),平均(54.6±16.8) mmHg.合并单心室13例,三尖瓣闭锁12例,室间隔缺损远离两大动脉伴左心室发育不良型右室双出口11例,三尖瓣重度狭窄5例,心室不均衡型完全型房室通道5例,十字交叉心3例.根据年龄分为3组:≤0.5岁17例、0.5 ～2.0岁17例、≥2.0岁15例.全组均先期行PAB,静脉吸入复合麻醉,吸入氧浓度40％时,SPO20.85,肺动脉平均压20 mm Hg.对比分析3组术后SPO2、mPAP、呼吸机使用时间、ICU停留时间以及手术死亡比率.结果 手术死亡1例系1岁6个月患儿,死因为肺部感染.全组术后肺动脉压明显下降.3组术后SPO2、mPAP、呼吸机使用时间、ICU停留时间均无明显区别.随访6 ～72个月,1例1岁2个月患儿术后2个月因误吸死亡;3例已完成双向格林或全腔肺动脉吻合术.结论PAB能有效降低不同年龄段伴肺动脉高压拟行单心室修复的先心病患儿的肺动脉压力,术后效果良好.     

非体外循环双向Glenn手术治疗小儿复杂先天性心脏病

目的总结非体外循环双向Glenn手术在小儿复杂先天性心脏病中的应用经验。方法 2005年4月至2008年8月,青岛市儿童医院收治22例先天性心脏病患者,男14例,女8例;年龄10个月～7岁,平均年龄3.0岁;体重6～18kg。功能性单心室合并肺动脉狭窄9例,室间隔完整的肺动脉闭锁4例,重度三尖瓣下移畸形3例,三尖瓣闭锁3例,矫正型大动脉转位合并右心室流出道狭窄2例,室间隔缺损合并重度三尖瓣狭窄1例。所有患者均在非体外循环下行双向Glenn手术,其中7例行双侧双向Glenn手术。结果无手术死亡。术后发生胸腔积液2例,经胸腔穿刺抽液后治愈。术后患者肺动脉平均压13.0±2.3mmHg,呼吸机辅助呼吸时间3.0±1.7h,住院时间9.0±2.3d,出院时脉搏血氧饱和度85%±8%,活动耐量明显改善。所有患者均获得随访,随访时间8个月～4年;超声心动图提示:所有患者吻合口通畅,均无扭曲及血栓形成;心功能Ⅰ级18例,Ⅱ级4例。结论非体外循环双向Glenn手术治疗小儿复杂先天性心脏病安全,可适当扩大手术适应证。     

一个半心室修补术在双心室修补术后急性右心功能不全治疗中的应用

目的探讨一个半心室修补术在双心室修补术后急性右心功能不全治疗中的应用。方法回顾性分析2007年2月至2012年6月青岛市妇女儿童医院5例先天性心脏病行双心室修补术后出现急性右心功能不全,急诊在非体外循环下加做双向Glenn手术(即转换手术方式为一个半心室修补术)患儿的临床资料,其中男4例,女1例;年龄7～18个月;体重6～13 kg。室间隔完整的肺动脉闭锁1例,室间隔缺损+房间隔缺损合并三尖瓣狭窄2例,法洛四联症合并三尖瓣狭窄2例。术后加强心肺功能维护,重点控制肺动脉压。结果急诊双向Glenn手术后72 h平均肺动脉压12～18 mm Hg。呼吸机辅助呼吸时间3～182 h,ICU滞留时间2～13 d。住院期间死于肺动脉高压危象1例。4例患者出院时静息不吸氧状态下经皮血氧饱和度为93%～99%,较术前有不同程度地提高。随访4例,随访时间6个月至4年,心功能(NYHA分级)Ⅰ级3例,Ⅱ级1例。心脏超声心动图提示:腔静脉肺动脉吻合口通畅,无血栓形成。结论一个半心室修补术可作为双心室修补术后发生急性右心功能不全的补救手术。     

双向Glenn分流术治疗儿童复杂紫绀型先天性心脏病

目的总结双向Glenn(BDG)分流术在治疗儿童复杂紫绀型先天性心脏病(先心病)的经验和体会。方法 1999年1月至2004年10月,对155例具有Fontan术高危因素的复杂先心病作BDG术。高危因素包括:年龄<2岁,左和(或)右肺动脉狭窄,平均肺动脉压升高,房室瓣反流及体静脉或(和)肺静脉异位回流等。术前诊断包括单心室(53例)、右室双出口(44例)、完全性大动脉转位(9例)、纠正性大动脉转位(4例)、三尖瓣闭锁(9例)、肺动脉闭锁伴室间隔完整(11例)、肺动脉闭锁伴室间隔缺损(17 例)、上下心室(7例)和法洛四联症伴完全性房室间隔缺损(1例)。其中56例为内脏异位综合征(HS) (36．1％)。130例和25例分别在体外循环和非体外循环下手术。手术类型包括单侧BDG术(84例),双侧BDG术(52例),下腔静脉-肺动脉外管道BDG术(4例),半Fontan术(13例),右半Fontan术十左BDG 术(2例)。其他手术包括完全性肺静脉(TAPVC)(8例)和部分性肺静脉(3例)异位回流纠治术,房室瓣修复术(12例),肺动脉重建术(26例)。结果术后早期平均动脉氧饱和度由术前0．75±0．07升高至 0．86±0．05。手术早期病死率1．9％(3/155例)。结论 BDG分流术可广泛应用于小儿复杂紫绀型先心病,尤适宜于无法行双室修复术,而又存在Fontan术高危因素病儿。HS手术时,术前诊断有无TAPVC十分重要,在作BDG术同时应行TAPVC纠治术。保留肺动脉前向性血流指征为BDG术后PaO2<30 mm Hg,SaO2<0．75,如SaO2>0．85则部分关闭肺动脉干。就医较晚的10岁以上病儿,行下腔静脉-肺动脉外管道BDG术不失为有效治疗方法。     

双向Glenn手术治疗儿童功能性单心室68例分析

目的总结1998年4月至2005年12月行双向Glenn手术治疗68例儿童功能性单心室的经验。方法本组男47例,女21例;年龄5个月～14岁（中位年龄3．7岁）;体重6．7～30．0kg（中位体重12．5kg）。右侧双向Glenn手术39例,左侧双向Glenn手术13例,双侧者16例。同期行肺动脉环束3例、肺动脉结扎1例、主动脉-肺动脉分流管道切断缝合术1例、动脉导管结扎6例、大侧支循环切断4例、全肺静脉异位引流矫治1例、部分肺静脉异位引流矫治2例、房室瓣整形4例。结果68例患者死亡3例,病死率4．4％。术后上腔静脉压力（15．9±2．4）mmHg（1mmHg=0．133kPa）,较术前的（8．3±1．8）mmHg显著上升（P〈0．01）。术后安静时经皮血氧饱和度（89．3±4．2）％,较术前的（78．4±6．0）％显著上升（P〈0．01）。结论双向Glenn手术治疗功能性单心室效果满意;双向Glenn手术宜保留肺动脉的搏动性血流。     

Left ventricular performance of pulmonary atresia with intact ventricular septum after right heart bypass surgery

OBJECTIVE: The left ventricular performance in patients with pulmonary atresia with intact ventricular septum who were Fontan candidates before and after the bidirectional Glenn procedure and a staged total cavopulmonary connection was compared with that in patients with tricuspid atresia. METHODS: Contractility (end-systolic elastance), afterload (effective arterial elastance), and ventricular efficiency (ventriculoarterial coupling, arterial elastance/end-systolic elastance ratio), and the ratio of stroke work and pressure-volume area were approximated on the basis of cardiac catheterization data before the bidirectional Glenn procedure, before and after staged total cavopulmonary connection, and approximately 1 year after the completion of total cavopulmonary connection in 20 patients with pulmonary atresia with intact ventricular septum and 21 patients with tricuspid atresia. RESULTS: The end-systolic elastance of the pulmonary atresia with intact ventricular septum group was significantly inferior to that of the tricuspid atresia group after bidirectional Glenn procedure and total cavopulmonary connection (1 year after total cavopulmonary connection 1.85 +/- 0.51 mm Hg . m 2 . mL -1 vs 2.84 +/- 0.96 mm Hg . m 2 . mL -1 , P < .01). The arterial elastance was not different between groups throughout the assessment period and tended to increase in a stepwise fashion after bidirectional Glenn procedure and total cavopulmonary connection. The arterial elastance/end-systolic elastance ratio and ratio of stroke work and pressure-volume area of the pulmonary atresia with intact ventricular septum group tended to worsen, whereas those of the tricuspid atresia group tended to improve. The difference reached statistical significance 1 year after total cavopulmonary connection (1.15 +/- 0.35 vs 0.82 +/- 0.23 and 64.2% +/- 6.7% vs 71.3% +/- 5.7%, respectively, P < .05 and P < .05, respectively). CONCLUSION: The contractility and ventricular efficiency of patients with pulmonary atresia with intact ventricular septum are inferior to those of patients with tricuspid atresia after bidirectional Glenn procedure and total cavopulmonary connection. A high-pressure residual right ventricle may impair the left ventricular performance of patients with pulmonary atresia with intact ventricular septum after bidirectional Glenn procedure and total cavopulmonary connection.     

Clinical Results of the Staged Fontan Procedure in High-Risk Patients

Background. For high-risk Fontan candidates, the introduction of a bidirectional Glenn shunt before total cavopulmonary connection (a two-staged strategy) may extend the indications for the Fontan procedure. The clinical results of the two-staged and one-staged Fontan procedure were thus reviewed and compared.

Methods. Between November 1991 and July 1996, the two-staged strategy was performed in 40 high-risk Fontan candidates with a mean interval of 17.2 months after introducing the bidirectional Glenn shunt (staged group). We considered a young age (<2 years), high mean pulmonary arterial pressure (≥20 mm Hg), high pulmonary vascular resistance (≥3 Wood units), small pulmonary artery (Nakata index <200 mm²/m²), atrioventricular valve incompetence (≥ moderate), distortion of pulmonary artery, anomalous pulmonary venous return, and poor ventricular function as risk factors for the successful completion of Fontan circulation. During the same pe-riod, 68 patients underwent the modified Fontan procedure in a one-step fashion (primary group).

Results. In the staged group after the bidirectional Glenn shunt, the mean pulmonary arterial pressure and ventricular end-diastolic pressure were both found to have decreased significantly to the same level as those in the primary group, whereas the pulmonary artery demonstrated a significantly smaller size than that in the primary group. Operative morbidity was similar in both groups. Operative mortality was also similar and low in both groups (1.5% in the primary group and 0% in the staged group).

Conclusions. A bidirectional Glenn shunt was found to be a useful interim palliation in high-risk Fontan candidates. This two-staged strategy may extend the operative indications for the Fontan procedure.     

Fontan type operation for complex lesions. Surgical considerations to improve survival

Twenty-five of 49 patients who underwent a Fontan type operation had complex lesions other than tricuspid atresia with ventriculoarterial concordance. Three patients had significant subaortic stenosis. Thirty-four palliative operations, including nine Glenn shunts, were performed before the Fontan operation. Direct atriopulmonary anastomosis was performed in 21 patients. In four, valved conduits were used. Twelve patients had right atrioventricular valve patch closure (three had running and nine had interrupted suture technique). On the basis of the presence of increased or decreased pulmonary blood flow before any surgical intervention, patients were divided into Group 1 (previous pulmonary artery banding, N = 8) and Group II (pulmonic stenosis, N = 17). Postoperatively, in Group I, 87% had significant effusions, mean right atrial pressure was higher (20.6 +/- 6.5 torr), and hospital stay longer (31 days). In Group II, 40% had significant effusions, mean right atrial pressure was lower (16.5 +/- 4.3 torr), and hospital stay shorter (15 days). Significant atrioventricular valve patch disruption occurred in three patients (two had running suture technique), and conduit occlusion occurred in two. Four patients (three with subaortic stenosis and pulmonary artery banding) without an established Glenn shunt required Fontan takedown for persistent low cardiac output, two of whom died (2/25 or 8%). There were three late deaths (3/23 or 13%). Nineteen of 20 surviving patients observed from 2 months to 6 years are doing well. We believe that early Fontan takedown in patients with persistent low cardiac output, interrupted suture technique for atrioventricular valve closure, avoidance of valved conduits, and a preliminary Glenn shunt in patients with pulmonary artery banding and/or subaortic stenosis can further improve the results with the Fontan operation for complex lesions.     

Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum

BACKGROUND: Since 1991 we have performed a multistage palliative approach to biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum in infants with a detectable right ventricular infundibulum. METHODS: A total of 25 patients (19 pulmonary atresia and 6 critical pulmonary stenosis) underwent initial palliation consisting of a transarterial pulmonary valvotomy and a polytetrafluoroethylene shunt between the left subclavian artery and pulmonary trunk. Among the 23 survivors, 15 underwent balloon valvotomy. Six of these patients later required additional palliative surgery that consisted of repeat pulmonary valvotomy, adjustment of an atrial communication, and resection of the hypertrophied muscles in the right ventricle. RESULTS: Of the 25 patients, 23 (92%) survived. In all, 20 patients underwent definitive operations: 18 (90%) biventricular repair (12 pulmonary atresia, and 6 critical pulmonary stenosis), one bidirectional Glenn, and one Fontan procedure. The actuarial probability of achieving a biventricular repair at 36 months of age was 69%. In 18 patients right ventricular end-diastolic volume significantly increased but tricuspid valve diameter did not change. CONCLUSIONS: The multistage palliation procedure to promote right ventricular growth makes a definitive biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum possible in the majority of infants with a patent infundibulum.     

Fontan operation for tricuspid atresia with dysplasia of the right ventricular myocardium and absence of pulmonary valve

We report a case of successful staged repair of tricuspid atresia with dysplasia of the right ventricular myocardium and absence of the pulmonary valve. The patient underwent the modified Blalock-Taussig shunt operation at 1 month the bidirectional Glenn procedure at 11 months, and the total cavopulmonary connection procedure using extracardiac graft at 2 years. It is important to prevent the left ventricular outflow tract obstruction (LVOTO) caused by the non-functioning right ventricle when the Fontan completion. We were safely able to disconnect the pulmonary trunk from the right ventricle using the transesophageal echocardiography to evaluate the LVOTO during operation.     

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.     

Results of total cavopulmonary connection in the treatment of patients with a functional single ventricle

Total cavopulmonary connection was proposed as a modification of the Fontan procedure that might have greater benefits than previous methods. To assess this procedure we reviewed case histories of 38 patients (aged 17 months to 30 years) who underwent Fontan procedures with cavopulmonary anastomoses between January 1987 and December 1989. The group included 32 patients with univentricular heart, 2 with pulmonary atresia and intact ventricular septum, 3 with tricuspid atresia, and 1 with hypoplastic left heart syndrome. One or more previous palliative procedures were performed in 34 patients, including 19 systemic-pulmonary shunts, 16 pulmonary artery bandings, 7 atrial septectomies/septostomies, 7 Glenn shunts, and 1 patent ductus arteriosus ligation. Preoperative hemodynamics showed a pulmonary artery pressure of 12 mm Hg (range 6 to 22 mm Hg), pulmonary-systemic flow ratio of 1.6 (range 0.37 to 3.0), left ventricular end-diastolic pressure 9 mm Hg (range 3 to 15 mm Hg), and systemic arterial oxygen saturation of 82% (range 67% to 94%). Concomitant with cavopulmonary connection, 13 patients underwent additional procedures, including 9 atrioventricular valve annuloplasties, 4 Damus-Stansel-Kaye procedures, and 2 resections of subaortic membranes. Modifying the Fontan procedure in this fashion was particularly useful in the management of 2 patients with pulmonary atresia and intact ventricular septum who had right ventricular-dependent coronary blood flow. Cavopulmonary anastomosis and atrial septectomy were performed in both patients, with resultant inflow of oxygenated blood to the right ventricle and coronary arteries. Excellent postoperative results were noted in each. Postextubation hemodynamics for the entire group included a mean right atrial pressure of 13 mm Hg (range 11 to 17 mm Hg), a mean left atrial pressure of 6 mm Hg (range 3 to 12 mm Hg), and a room air oxygen saturation of 96% (range 92% to 98%). Seven patients had pleural effusions, 3 required postoperative pacemaker placement, and 2 required reoperation for tamponade. A venous assist device was required in one patient on the second postoperative day, but the patient was weaned successfully within 24 hours. One early death (2.6%) occurred in a patient who had intractable ventricular fibrillation 2 days after operation. There was one late cardiac death (2.7%) caused by ventricular failure and one late noncardiac death. These results demonstrate that total cavopulmonary connection provides excellent early definitive treatment, with low morbidity and mortality, for a variety of complex congenital heart lesions.     

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.