Modified Fontan procedure for left atrial isomerism: Alternative technique

Anomalous systemic or pulmonary venous connections increase the risk and technical difficulty of the modified Fontan procedure. This report describes an alternative technique of total diversion of systemic venous return to the pulmonary artery in a child with left atrial isomerism, incorporating an extracardiac conduit between the hepatic veins and the right pulmonary artery.     

Right Ventricular Outflow Patch Reconstruction for Repair of Double-Outlet Left Ventricle

Double-outlet left ventricle (DOLV) is conventionally repaired with an extracardiac conduit when pulmonary stenosis is present. We report a case of surgical repair for DOLV with severe pulmonary stenosis where the right ventricular outflow tract was established using a vascular graft patch anteriorly after constructing the posterior wall with autologous tissue. This approach provides excellent hemodynamic repair without the use of an extracardiac conduit and has the major advantage of growth potential.     

Redirection of Hepatic Drainage for Treatment of Pulmonary Arteriovenous Malformations Following the Fontan Procedure

The development of unilateral pulmonary arteriovenous malformations in patients after total cavopulmonary connection with an extracardiac conduit has been reported. Unequal distribution of hepatic venous flow to the lung is theorized to be the causative factor. We report the surgical management of pulmonary arteriovenous malformations in a patient with heterotaxy syndrome, single ventricle, and interrupted inferior vena cava. The patient had previously undergone a total cavopulmonary connection with an extracardiac conduit draining hepatic venous flow to the right branch pulmonary artery. In the subsequent operation, we redirected the extracardiac conduit to the innominate vein. This operation provided the affected lung with hepatic venous blood without exposing the patient to the morbidity associated with cardiopulmonary bypass.     

全腔静脉-肺动脉连接术治疗儿童复杂先天性心脏病

目的探讨全腔静脉一肺动脉连接术治疗儿童复杂先心病的方法及疗效。方法年龄0．5～11．5岁的复杂先天性心脏病患儿(单心室伴其他畸形5例，右室双出口伴其他畸形3例，三尖瓣闭锁2例，三尖瓣下移畸形1例)11例施行了心外管道或房内侧壁隧道全腔静脉一肺动脉连接术，采用自体心包6例，采用Gore-Tex管道连接5例，11例中开窗5例。结果全组死亡1例，术后患儿血氧饱和度由65％～85％增加至92％～96％，运动耐力明显增强，心脏容量负荷减轻，血流动力学满意，近期疗效较好。结论全腔静脉一肺动脉连接术操作简捷，血流动力学满意，能有效提高患儿血氧饱和度及减轻心脏容量负荷。术后并发症和病死率较低。     

The value of early repair for total anomalous pulmonary venous drainage

Summary A total of 20 children with total anomalous pulmonary venous drainage (TAPVD) underwent complete repair within the past six years. The drainage was supracardiac in 11, infracardiac in seven, and into the coronary sinus in two. At repair mean age was nine weeks, and weight was 3.7 km. Operative technique in extracardiac types included complete mobilization of the common vein with division of anomalous channel when possible. The incision in the confluent vein was extended into the lobar veins when necessary to permit extensive incorporation of this structure into the posterior wall of the left atrium and resulted in a nonobstructing stellate-type anastomosis.Operative mortality was 10% (2 of 20). Deaths occurred only in the group of infants in whom severe obstruction to pulmonary venous return required emergency operation, and one of these patients has persistent neurologic deficit. Late cardiac catheterization has been performed in 11 of 18 survivors. Nine had no or only minor abnormalities and two required reoperation. There have been no late deaths with follow-up of 2–8 years (mean=4 years). Currently, all of the survivors are without cardiac symptoms and only one requires cardiac medication.Our experience identifies pulmonary venous obstruction with critical symptoms as the major operative risk factor in patients with TAPVD. With early operation prior to onset of critical symptoms, mortality is low and functional results are excellent.     

Echocardiographic and Three-Dimensional Computed Tomographic Diagnosis of Crossed Pulmonary Arteries: Report of Three Cases

Crossed pulmonary arteries are rare anomalies of the pulmonary trunk and its branches. In this anomaly, the ostium of the left pulmonary artery originates superiorly and to the right of the right pulmonary artery. This anomaly is usually associated with other congenital cardiac and extracardiac diseases. We report three neonates with congenital cardiac diseases who had this anomaly, which was detected first by echocardiography and then confirmed by cardiac three-dimensional computed tomography.     

Micropulmonary arteriovenous fistula causing cyanosis

This article presents a young girl who came to the Department of Pediatrics, AIIMS, with cyanosis and was diagnosed to be having multiple pulmonary arteriovenous fistulae. The cardiovascular evaluation was normal and so was the respiratory evaluation. The desaturation did not improve with oxygen. The chest X-ray was also normal. The echocardiogram did not reveal any abnormality but agitated saline contrast echocardiography suggested an extracardiac right to left shunt which was confirmed on pulmonary angiography.     

Noncardiac malformations in congenital heart disease: a retrospective analysis of 305 pediatric autopsies

Congenital heart disease (CHD) is one of the important groups of birth defects and contributes significantly to infant mortality. Extracardiac anomalies occur in 15-45% of cases with CHD. In this retrospective study, autopsies of cases born alive and diagnosed as CHD between 1977-2002 at Hacettepe University Ihsan Do?ramaci Children's Hospital, Pediatric Pathology Department, were investigated. In this period, a total of 3320 autopsies were performed and the incidence of CHD was 9.1%. The most commonly encountered CHD was ventricular septal defect (VSD) (15.3%). In 45.9% of cases, one or more extracardiac malformations were present. The most commonly encountered extracardiac malformation was craniofacial malformations. Less commonly seen were malformations of genitourinary, musculoskeletal, respiratory, gastrointestinal, central nervous systems and spleen anomalies. Ventricular septal defect, atrial septal defect (ASD), aortic coarctation, single ventricle, pulmonary stenosis, hypoplastic right heart syndrome, double outlet right ventricle, ASD+VSD, aortic arcus anomalies, and right and left atrial isomerism cases were often (>50%) accompanied by extracardiac malformations. No extracardiac malformations were detected accompanying pulmonary atresia with intact interventricular septum, Ebstein malformation, and mitral stenosis (MS). Spleen malformation was significantly high in cases with single ventricle (p<0.002). The anomalies of the gastrointestinal and genitourinary systems were found to be frequently associated with conotruncal heart defects (p<0.001). In the group with transposition of the great arteries, noncardiac malformations were present in only three cases (10%), differing from the rest of the material (p<0.001). In conclusion, when a heart malformation is detected in a patient, a detailed investigation should be done on extracardiac malformations or vice versa. Proper identification and treatment of CHD early in the prenatal period will save the family from the economic and emotional burden caused by having such a child with CHD.     

Isolated Left-sided Scimitar Vein Connecting All Left Pulmonary Veins to the Right Inferior Vena Cava

When the common pulmonary vein fails to develop, the embryonic connections of the pulmonary veins to one or more of the systemic veins almost always persist. Anomalous pulmonary venous connections to the inferior vena cava (IVC) are typically characterized by hypoplasia of the involved pulmonary veins and pulmonary artery, as well as abnormal parenchyma of the involved lung. Such cases have been described as “scimitar syndrome.” We report the case of a young female patient in whom all the left pulmonary veins converged into a common vessel that drained into the IVC but who had a normal left pulmonary artery and left lung. Surgical intervention was successful, and our patient is still alive.     

Results of 100 Consecutive Extracardiac Conduit Fontan Operations

Throughout the years, the experience with Fontan's operation has increased and has opened the way to a much wider application of this principle. A number of major risk factors have been identified and managed by several modifications of the original Fontan procedure. In the past 15 years, operative and postoperative risks have been controlled to a major extent by the application of intermediate surgical procedures. Modifications to the original technique have been designed to simplify surgery and better protect the myocardium by avoiding, as much as possible, prolonged ischemic time. In 1988, we developed a new form of total extracardiac right heart bypass by means of associating an extracardiac conduit placed between the inferior vena cava (IVC) and the pulmonary artery with a bidirectional cavopulmonary anastomosis (BCPA)--the so-called total extracardiac cavopulmonary connection (TECC).Between November 1997 and October 2003, 100 patients with complex cardiac anomalies underwent a modified Fontan operation by TECC. In 88 patients, the repair was staged by preliminary BCPA that was bilateral in 15 patients and associated with a modified Damus-Kaye-Stansel procedure to bypass a subaortic obstruction in 24 patients. Early (in hospital) deaths occurred in 6% of patients and the extracardiac conduit was taken down in 2 additional patients for a total early failure rate of 8%. The cause of death was myocardial failure in 5 patients. Pulmonary artery distortion or hypoplasia appeared to be the cause of death in 1 patient and the cause of failure in 1 patient. Atrioventricular valve regurgitation was the cause of failure in 1 patient. Follow-up to December 2003 was available in all surviving patients. There were no late deaths. At follow-up, 87 patients (89%) were in New York Heart Association (NYHA) functional class I, 4 in class II, and 3 in class III due to moderate to severe atrioventricular valve regurgitation. Use of the following guidelines can result in the achievement of orthoterminal repair with complete separation of pulmonary and systemic circulation, with negligible early mortality and a long-term NYHA class I: (1) Not all patients with univentricular heart (UVH) should undergo the extracardiac Fontan procedure; (2) in UVH with favorable streaming, a Q (p)/Q (s) ratio of approximately 1 to 1, and mild cyanosis, natural history might be preferable to surgical history; (3) any form of fenestration is contradictory to orthodox application of Fontan's principle. Children in whom a planned fenestration seems necessary because of suboptimal conditions should instead undergo a combination of BCPA and associated forward pulmonary blood flow; and (4) negligible mortality should be considered mandatory in UVH, normal pulmonary arteries, and absent cardiomegaly after appropriated and correct staging.     

Radiological evaluation of pulmonary vein obstruction including two examinations by magnetic resonance imaging

Congenital obstruction of the pulmonary vein without anomalous drainage can cause long-standing pulmonary congestion and pulmonary arterial hypertension, and it may include stenosis of individual pulmonary veins and pulmonary vein atresia. We reviewed seven cases of pulmonary vein obstruction, five of which were accompanied by other cardiac anomalies. Right pulmonary veins were involved in all seven cases; one case was bilateral. Pulmonary veins were occluded totally in five and partially in three lungs. Diagnostic pulmonary catheterization and angiography were performed. Chest radiographs of total occlusion cases showed decreased lung volume, features of pulmonary edema, interstitial lesions, and pleural changes, which were quite specific, whereas pulmonary venous dilatation was the dominant finding in partial obstruction cases. Pulmonary perfusion scan (n=3) showed total perfusion defects in the cases with total occlusion of pulmonary veins. Magnetic resonance (MR) imaging (n=2) demonstrated totally occluded pulmonary veins at the venoatrial junction in two lungs and membranous focal obstruction in one lung. Two children underwent pneumonectomy and had the diagnosis histologically confirmed. Although catheterization and angiography are essential for the diagnosis of pulmonary vein obstruction, MR imaging is a useful adjunct.     

完全性肺静脉异位引流病理谱及个体化手术治疗

目的 描绘完全性肺静脉异位引流(TAPVC)患儿肺静脉走行"路线图"和形态"变异图",阐明肺静脉病理谱的变化规律,促进有效个体化手术.方法 2006年4月至2009年9月,139例TAPVC患儿进行手术,心上型61例,心内型55例,心下型6例和混合型17例.病理诊断依据超声心动图、核磁共振、计算机断层扫描或心导管和心血管造影检查以及术中解剖,根据患儿TAPVC类型和解剖特点选择个体化手术方法.结果 1.病理谱:①肺静脉走行连接"路线图":心上型按照垂直静脉(VV)走行方式分为4种,左行(47例)、右行(9例)、后行(2例)和双行(3例);心内型按照肺静脉回流部位分为3种,汇入冠状静脉窦(49例)、汇入右心房(5例)和同时汇人CS和右心房(1例),再依据肺静脉开口数目分为4个开口、2个开口和1个开口三种亚型;心下型按照VV汇入体静脉方式分为4种:汇入门静脉(1例)、肝静脉(3例)、同时汇入门静脉和肝静脉(1例)以及汇入下腔静脉(1例);混合型按照肺静脉回流双侧是否对称分为双侧对称连接的"2+2"型(5例)、双侧不对称连接的"3+1"型(10例)和"怪异型"(2例).②肺静脉形态"变异图":肺静脉入口狭窄、发育不良或多分支(11例)、共汇肺静脉发育不良或伴内膜增生(4例)、VV扭曲、短小和狭窄(9例).2.手术结果:本组早期死亡6例(4.3%),其中低心排1例,肺静脉梗阻5例.中期随访因肺静脉梗阻再手术6例,术后轻微梗阻2例.结论 TAPVC患儿肺静脉病理谱广,个体差异大;按照肺静脉走行"路线图"和形态"变异图"有利术中肺静脉解剖的探查和个体化手术设计.

Abstract：

Objective To delineate the morphological spectrum of total anomalous pulmonary venous connection (TAPVC) by building the "road map" and the " variation chart" of pulmonary veins,and to advance individualized surgical treatment Methods Between April,2006 to June,2009, 139 consecutive patients with TAPVC underwent operations. There were 61 supracardiac,55 intracardiac,6 infracardiac and 17 mixed types. Pathological diagnosis was made by echocardiogram,magnetic resonance imaging, computerized tomography,or the cardiac catheterization and operative findings during operation. The option of procedure was determined by findings. Results (1) Pathological spectrum:①" the road map"of the pulmonary veins: the patients with supracardiac type were divided into 4 subtypes according to the course of vertical veins:left course (47 cases),right course (9 cases),posterior course(2 cases) and double courses (3 cases). The patients with intracardiac type were divided into 3 subtypes according to the draining site of pulmonary veins: to coronary sinus (49 cases) ,to right atrium (5 cases) or to coronary sinus and right atrium (1 case),and the openings of pulmonary veins may be 4,2 or 1 in each subtype. The patients with infracardiac type were divided into 4 subtypes according to the draining site of vertical vein:to portal vein (1 case),to hepatic vein(3 cases),to portal vein and hepatic vein (1 case) and to inferior vena cava (1 cases). The patients with mixed type were divided into 3 subtypes: bilateral and symmetrical connections "2 + 2" pulmonary venous drainage pattern; bilateral and asymmetrical connections"3 +1" pulmonary venous drainage pattern and bizarre anatomic variants. ② The morphological "variation chart" of pulmonary veins individual pulmonary vein stenosis or excessive tributary veins (11 cases) showed: hypoplastic confluence veins (4 cases) and vertical veins distortion or elongation or forming hemodynamic vise or common pulmonary vein that drained to coronary sinus or right atrium through a narrowed short vertical vein or a small window(9cases).(2)Surgical results：early death was encountered in 6 cases(4.3％).The causes included 10W cardiac output syndrome in 1 case,and pulmonary veins stenosis and associated complications in the other 5 patients.Six patients with pulmonary restenosis underwent reoperation.All survived.Two patients had mild residual obstruction during Intermediate-term follow-up.Conclusions The patients with TAPVC had a wide spectrum of pulmonary veins with high inter-individual variation.It was useful to delineate the anatomy of pulmonary veins and to plan personalized procedures during operations according to the"road map"and"variation chart"of pulmonary veins.     

肺静脉指数对肺血减少型先天性心脏病肺血管发育的评估

目的 探索能更准确反映肺血管发育及肺血流情况的指标,为外科手术方案的选择提供依据.方法 采用74例肺血减少型先天性心脏病心血管造影序列,测量左右肺动脉及4根肺静脉直径,分别计算Nakata指数,McGoon指数,肺静脉指数(PVI),分别与术后情况进行相关分析.结果 左、右侧肺静脉大小分别与左右肺动脉大小高度相关,左侧肺静脉与左肺动脉远端的相关性为0.73,左侧肺静脉与左肺动脉近端的相关性为0.72,右侧肺静脉与右肺动脉远端的相关性为0.67,右侧肺静脉与右肺动脉近端的相关性为0.71.PVI与术后监护时间,呼吸机维持时间,正性肌力药物用量的相关性分别为-0.51,-0.478和-0.693,均比Nakata指数,McGoon指数明显增高,能更准确的反映整个肺血管的发育情况.右室流出道重建术后无低心排组与低心排组的McGoon指数分别为1.97±0.58与1.36±0.51(t=2.347,P＜0.05),两组Nakata指数分别为(269±124)mm2/m2和(164±106)mm2/m2(t=2.218,P＜0.05),PVI分别为(273±125)mm2/m2和(152±77)mm2/m2(t=2.936,P＜0.01),低心排组肺血管值均明显小于无低心排组.当PVI小于180 mm2/m2时,术后易出现血流动力学不稳定,低心排,甚至死亡.结论 肺动脉、肺静脉发育彼此相关,PVI能更准确反映肺血管发育及肺血流情况的形态学指标,为外科手术方案的选择提供有效依据.     

Contrast-enhanced MR angiography of pulmonary venous abnormalities in children

BACKGROUND: Echocardiography and X-ray angiography have been considered as gold standards for evaluation of pulmonary venous abnormalities. However, each technique has its own limitations, such as limitation in visualization of the pulmonary veins within the lungs by echocardiography, and the invasive nature of and use of ionizing radiation in X-ray angiography. Contrast-enhanced MR angiography (MRA) is a fast noninvasive method of visualization of the vessels including the pulmonary arteries and veins. OBJECTIVES: To evaluate the utility of contrast-enhanced MRA in the evaluation of pulmonary venous abnormalities in pediatric patients and to compare its diagnostic accuracy with that of transthoracic echocardiography. MATERIALS AND METHODS: In 30 pediatric patients 31 contrast-enhanced MRA studies were performed for evaluation of pulmonary venous abnormalities. Each of 124 pulmonary veins was evaluated for site of connection, course within the lung, presence of obstruction, and topographic relationship with the adjacent structures. The findings of MRA were compared with echocardiographic findings for 116 veins in 29 studies in 28 patients. RESULTS: Contrast-enhanced MRA visualized 99% (123 of 124) of the pulmonary veins investigated, while echocardiography visualized 89% (103 of 116). Exact agreement was found between the two methods in 72% of the veins with a weighted kappa of 0.60 (0.47-0.73, 95% CI). Echocardiography failed to diagnose an abnormal connection in 2 of 15 pulmonary veins, a discrete stenosis in 2 of 19 veins, and diffuse hypoplasia in 10 of 14 veins. In 29% of patients, MRA made the uncertain echocardiographic findings clear. In another 29%, MRA provided a new diagnosis. CONCLUSIONS: Contrast-enhanced MRA is a powerful, safe, and accurate fast-imaging technique for the anatomical evaluation of pulmonary venous abnormalities. MRA may obviate the need for conventional X-ray angiography. Cardiac catheterization may be reserved for those patients in whom pulmonary vascular resistance needs to be determined.     

Doppler Echocardiography with Extended Transesophageal Atrial Pacing: Predicting the Efficacy of Permanent Atrial Pacing in the Patient with a Small Left Ventricle and Sinus Node Dysfunction

A 2100-g neonate underwent a two-ventricular surgical repair of a right ventricle-dominant unbalanced atrioventricular septal defect associated with the heterotaxy syndrome and sinus node dysfunction. Postoperative congestive heart failure persisted despite bradycardia management by temporary ventricular pacing. Spectral Doppler echocardiographic analysis of pulmonary venous inflow and aortic outflow patterns demonstrated significant improvement with transesophageal atrial pacing. Extended transesophageal pacing was performed for two days, resulting in dramatic clinical improvement. This is the first report of extended transesophageal atrial pacing complementing Doppler echocardiography predicting an improved outcome with permanent atrial pacing.     

Variation in prevalence of chromosome 22q11 deletion in subtypes of conotruncal defect in 254 children

Aim: To determine the frequency of chromosomal aberrations particularly 22q11 deletion in Indian children ≤2 years with different types of conotruncal malformations and their association with abnormal aortic arch. Additionally, extracardiac features were also studied. Methods: Conventional cytogenetic and fluorescence in situ hybridization analyses were performed in 254 patients with conotruncal defects. Multivariable logistic regression analysis was performed to ascertain extracardiac features helpful in identifying high‐risk patients with deletion. Results: Chromosomal abnormalities were identified in 52 (21%) children, of whom 49 (94%) showed 22q11 deletion and 3 (6%) had abnormalities of chromosome 6, 2 and X. None of the 11/254 children with tetralogy of Fallot with absent pulmonary valve showed deletion. The association of 22q11 deletion with right sidedness of the aortic arch varied with the type of conotruncal defect. The eight extracardiac features in combination showed 93.5% agreement with the presence of deletion. Conclusion: The extracardiac features along with specific type of conotruncal defect and associated cardiovascular anomaly should alert the clinician for 22q11 deletion testing. However, if deletion analysis is not possible, specific extracardiac features (six dysmorphic facial features, thin long fingers and hypocalcemia) can help to identify an increased risk of 22q11 deletion in patients with conotruncal defect.     

Congenital pulmonary vein stenosis as a rare cause of pulmonary hypertension]

We report on the history, the diagnostic, and the operative procedure in an infant with congenital stenosis of all pulmonary veins. First symptoms such as failure to thrive, tachydyspnea, tachycardia and hepatomegaly occurred in the eighth week of life. Electrocardiography, 2-dimensional echocardiography and radiography of the chest were unspecific. After recurrent episodes of pulmonary oedema cardiac catheterization was performed: bilaterally elevated pulmonary artery wedge pressure and a normal left atrial pressure proved pulmonary venous obstruction. Severe stenosis of all pulmonary veins was apparent cineangiographically only by selective injections into the right and left pulmonary artery branches in wedge position. The operation (excision of the stenotic area and reimplantation using autologous pericardium) was unsuccessful as in most cases described in the literature. Congenital stenosis of all pulmonary veins is a rapidly progressive malformation. Death occurs usually in the first year of life with and without operation.     

Total Anomalous Pulmonary Venous Return with the Circular Pulmonary Venous Connection: Outcome of Common Pulmonary Venous Agenesis

A rare case of total anomalous pulmonary venous return, in which the right and left peripheral pulmonary veins connected circularly and there was no central part of the pulmonary vein or the common pulmonary vein, is presented. To our knowledge, total anomalous pulmonary venous return with circular pulmonary venous connection has not been reported previously in the literature. It is thought that the complex connection between peripheral pulmonary veins with the absence of the central part of the pulmonary vein as well as the common pulmonary vein results from common pulmonary venous agenesis.     

Maturational changes in the pharmacological characteristics and actomyosin content of canine arterial and venous tissue

The purpose of this study was to compare the pharmacological characteristics and actomyosin content of arterial and venous tissue at different times during development. Rings of arteries (femoral, renal, carotid, pulmonary) and veins (saphenous, pulmonary, jugular) were obtained from 1 wk, 1 month, and adult dogs, mounted at their optimal length for force development and the contractile response to potassium chloride and phenylephrine determined. The strain at optimal length was less at all ages in pulmonary artery and pulmonary and jugular veins than in other vessels. All vessels exhibited an increase in maximum contractile response with development but the increase was greater for phenylephrine. In general, the magnitude of the maximum response of the jugular and pulmonary veins and pulmonary artery was less than other vessels at all ages. The sensitivity (half maximum response) either increased or was unchanged in arteries with development, while in the veins it either decreased or was unchanged. The relaxant effects of verapamil and isoproterenol were determined on potassium chloride contracted vessels. Arterial tissue was minimally responsive to isoproterenol at all ages while venous tissue either increased its responsiveness (saphenous, pulmonary) with development or remained highly responsive (jugular). Verapamil, unlike isoproterenol, was an effective relaxant of all vessels. The actomyosin content (mg/mm) of femoral and renal arteries and saphenous and jugular veins increased with development but this increase was accompanied by a parallel increase in total protein so that the ratio (actomyosin/total protein) was unchanged. In jugular veins from adult dogs this ratio was smaller than in arterial tissue.(ABSTRACT TRUNCATED AT 250 WORDS)     

Normalization of Pulmonary Vascular Resistance Long After Successful Primary Sutureless Repair for Congenital Pulmonary Vein Stenosis

A 3-month-old infant with perimembranous ventricular septal defect (VSD) and pulmonary hypertension presented at our center. Cardiac catheterization showed a systolic pulmonary-to-systemic arterial pressure ratio of 0.99 and a pulmonary vascular resistance of 9.32 μm². Pulmonary angiography showed focal orifice stenosis in the right lower and left lower and upper pulmonary veins, whereas the right upper pulmonary vein was atretic. Sutureless pericardial marsupialization concomitant with VSD patch closure was used to repair the three stenotic veins. Administration of home oxygen therapy and sildenafil citrate was continued after surgical repair. Postoperative catheterization 1.5 years after surgery showed patency of the three repaired veins and normalization of pulmonary vascular resistance.