Corrected transposition of the great vessels with stenosis of the left atrioventricular valve in a 40-year-old woman

The authors reports the detection of a rare and remarkably well tolerated cardiac malformation in a 40 year old woman whose clinical presentation would have suggested an ordinary mitral stenosis, had there not been a number of very unusual signs. Cardiac catheterisation and angiography showed a corrected transposition of the great arteries with stenosis of the systemic atrioventricular valve. This case is used to illustrate the principal malformations observed in association with corrected transposition of the great arteries, especially the common and very variable abnormalities of the systemic atrioventricular valve. The unusual feature of the reported case was the stenotic nature of the valvular lesion and the fact that this was the only associated intracardiac abnormality, simulating, at first sight, a rheumatic mitral stenosis.     

Surgical treatment of cardiac abnormalities associated with corrected transposition of the great arteries (author's transl)]

Twelve patients with corrected transposition of the great arteries, who underwent surgical repair, are reported. In four cases a ventricular septal defect was present; in six cases, a ventricular septal defect and pulmonary stenosis and in two an incompetence of the systemic atrio-ventricular valve. The anatomic types of corrected transposition and the pertinent diagnostic characteristics were reviewed and discussed. The problems related to the surgical correction and the different approaches to the intracardiac lesions are considered and the difficulties of treating the obstruction of the venous ventricle successfully are emphasized.     

Electrocardiogram in corrected transposition of the great vessels of the bulbo-ventricular inversion type

Twenty cases of corrected transposition of the great vessels of the bulbo-ventricular inversion type, either lone or combined with other intracardiac anomalies, were analysed. Rhythm and/or atrio-ventricular conduction disturbances were common to all groups of cases. QRS pattern changes were found to be related both to ventricular inversion and to ventricular hypertrophy.Isolated corrected transposition and corrected transposition with systemic ventriculo-atrial regurgitation give rise to tracings suggestive of systemic ventricular hypertrophy.Corrected transposition of the great vessels with pulmonary stenosis or pulmonary artery hypertension is usually accompanied by the electrocardiographic signs of a venous-ventricular hypertrophy, with a characteristic inversion of the normal praecordial pattern.The conventional criteria of ventricular hypertrophy may be applied in corrected transposition of the great vessels but are less reliable than in cases without ventricular inversion.The so-called electrocardiographic pattern of ;ventricular inversion' in this anomaly is related not only to the inverted position of the ventricles but to a greater extent to the predominant, anatomically left, venous-ventricular hypertrophy which re-establishes the normal weight ratio between the anatomically right and anatomically left ventricles.     

Surgical repair of congenitally corrected transposition of the great arteries: Results and follow-up

Eighteen patients with congenitally corrected transposition of the great arteries had open heart repair for intracardiac associated defects. Fourteen patients (78%) are alive during the follow-up period (mean 4.5 years). Seventeen (94%) of the 18 patients had ventricular septal defect closure, and 12 (66%) insertion of a pulmonary artery conduit. Surgical repair of the tricuspid valve was required in 6 patients (33%) during the first operation and in 3 additional patients during a second operation (total 50%). When hemodynamic overload or cardiac compromise was detected after surgery it was directly related to identifiable residual defects such as atrioventricular valvular insufficiency, residual ventricular septal defect, or pulmonary conduit stenosis. Repeat open heart operation for residual defects was common during the follow-up period (8 of 18 patients, 44%). No patient showed primary systemic or pulmonary ventricular dysfunction during the follow-up period. None of the last 11 patients developed complete heart block. Postoperative intraventricular conduction defects were common and are presumably caused by surgical injury of the bundle branches.Our observations suggest that surgical repair of congenitally corrected transposition of the great arteries can be currently achieved with acceptable risk. Improved knowledge of the precise location of the specialized conduction system resulted in a marked decrease in the Incidence of atrioventricular (A-V) block in patients with congenitally corrected transposition of the great arteries undergoing intracardiac repair. In the absence of postoperative residual defects it can be expected that longevity and quality of life will improve considerably, but many of these patients may require a repeat operation.     

Intravascular stents for management of pulmonary artery and right ventricular outflow obstruction

Summary This study was performed to determine the efficacy of balloon-expandable stents in the treatment of branch pulmonary artery-stenoses and conduit stenosis in children. A total of eight stainless steel stents were implanted in seven patients. Three patients had tetralogy of Fallot with pulmonary artery stenosis following total correction, one patient had conduit stenosis following correction of transposition of the great arteries, one patient had intra-cardiac conduit stenosis after septation for single left ventricle, and two patients had pulmonary artery stenosis after Fontan operation. Six stents were placed in the branch pulmonary arteries, one in the extracardiac conduit, and one in the intracardiac conduit. The mean age at implantation was 13 ± 3 years and the mean weight 37 ± 12kg. Follow-up time ranged from 0.3–2 years. The diameter of pulmonary arteries with stenoses increased from 5.6 ± 2.2 mm to 10.6 ± 1.8 mm (n = 7). The systolic pressure gradient decreased from 56 ± 26 mmHg to 22 ± 16 mmHg (n = 5). No embolization or thrombotic event has been noted. One stent placed in the intracardiac conduit was compressed and fractured. These data indicate that balloon-expandable stents are useful in the treatment of pulmonary artery branch stenoses and extracardiac conduit stenosis in children. The use of stents for intracardiac stenosis may result in stent fracture.     

Coarctation of the left pulmonary artery associated with congenitally corrected transposition.

We describe an infant with congenitally corrected transposition, ventricular septal defect and severe pulmonary stenosis. The heart occupied a midline position. Extension of ductal tissue had resulted in occlusion of the left pulmonary artery. As far as we are aware, this is the first report of an association of coarctation of the left pulmonary artery with corrected transposition.     

From academics to practice

In about 80 percent of the cases of corrected transposition of the great arteries reported in the literature, other primary lesions, mainly ventricular septal defect and/or pulmonic stenosis were present, and the isolated lesion is only rarely found [1–3]. We studied 10 cases of corrected transposition of the great arteries without shunt or obstructive lesions.     

Corrected transposition of the great arteries with previously unreported cardiac anomalies

The corrected transposition of the great arteries is a rare congenital cardiac anomaly characterised by atrio-ventricular and ventriculo-arterial discordance and is related to the largest incidence of cardiological complications. We report on a 40-year-old woman with congenitally corrected transposition of the great arteries, situs inversus, atrial septal defect, pulmonary stenosis, right arcus aorta and coronary artery anomalies.     

Electrocardiogram in corrected transposition of the great vessels of the bulbo-ventricular inversion type

Twenty cases of corrected transposition of the great vessels of the bulbo-ventricular inversion type, either lone or combined with other intracardiac anomalies, were analysed. Rhythm and/or atrio-ventricular conduction disturbances were common to all groups of cases. QRS pattern changes were found to be related both to ventricular inversion and to ventricular hypertrophy.     

合并心脏血管畸形的阵发性室上性心动过速的射频导管消融

对 8例心脏血管畸形合并室上性心动过速 (SVT)的患者进行了射频导管消融 (RFCA) ,其中 2例合并布 加氏综合征 ,1例肥厚型梗阻性心肌病 ,1例房间隔缺损 ,1例完全型矫正型大动脉转位 ,2例Ebstein畸形 ,1例二尖瓣重度狭窄。这 8例SVT心内电图与普通病例相比基本相同 ,但因心脏或大血管畸形 ,故操作过程有其特殊性。结果 :上述 8例患者全部消融成功 ,其中SVT合并完全型矫正型大动脉转位患者 2个月后心动过速复发 ,再次消融成功。对合并的心脏血管畸形除 1例Ebstein畸形和 1例完全型矫正型大动脉转位患者未接受进一步治疗外 ,其余均根据适应证进行了相应手术 ,2例布 加氏综合征进行了球囊扩张术 ,1例肥厚型梗阻性心肌病行室间隔化学消融术 ,1例房间隔缺损实施了Amplatzer伞闭术 ,1例Ebstein畸形行房化心室折叠术 ,1例二尖瓣重度狭窄患者行二尖瓣球囊成形术。结论 :合并心脏血管畸形的SVT进行RFCA安全、有效。     

Congenitally corrected transposition of the great arteries in an 80 year old woman

Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease characterised by atrioventricular as well as ventriculoarterial discordance. It is usually associated with a variety of severe intracardiac defects. Few patients with this abnormality survive past 50 years. An 80 year old woman was admitted to the hospital because of mild congestive heart failure. Cardiac examination revealed a 4/6 holosystolic and a 2/6 decrescendo diastolic murmur at the left sternal border. Radiography, echocardiography, and computed tomography confirmed newly diagnosed CCTGA without associated intracardiac defects.     

Corrected transposition of the great vessels in the adult. Presentation of a case simulating ischemic cardiopathy

We report the clinical and laboratory findings in a 58 years old woman with corrected transposition of the great arteries (CTGA), who that presented typical angina pectoris. The diagnosis of ischemic heart disease was supported by the history of a previous myocardial infarction. Other findings were a systolic murmur of mild mitral regurgitation, left bundle branch block and enlarged left ventricle on the chest X-ray. Cardiac catheterization showed a corrected transposition of the great arteries (L-malposition with situs solitus); left and right coronary arteries were free of luminal stenosis. We suggest therefore that anginal chest pain may be due to myocardial ischemia induced by discrepancy between myocardial oxygen consumption and coronary blood flow. This complication may occur in patients with corrected transposition of great arteries surviving in adulthood.     

Congenitally corrected transposition and degenerative severe aortic stenosis

Congenitally corrected transposition is a rare cardiac anomaly characterized by the combination of discordant atrioventricular and ventriculoarterial connections. Young patients with this lesion can present with congestive cardiac failure, usually secondary to a large ventricular septal defect or pulmonary stenosis. We report here our experience with a lady aged 79, admitted to our unit because of deterioration of her congestive cardiac failure as a consequence of uncorrected congenitally corrected transposition associated with degenerative severe aortic stenosis.     

同种带瓣管道在儿童先心病治疗中的应用

目的 :报告应用同种带瓣管道 (VHC)在儿童先心病治疗中的经验。方法 :全组 12例患儿男 7例 ,女 5例。平均年龄 7.5 (2 .2～ 13)岁 ,平均体重 2 1(12 .5～ 32 )kg。病种包括右室双出口 5例 ,共同动脉干 2例 ,矫正性大动脉转位合并室缺 2例 ,完全性大动脉转位 1例 ,法乐四联症合并肺动脉闭锁 1例 ,主动脉瓣关闭不全 1例。主动脉VHC应用 2例 ,肺动脉VHC应用 10例 ,均用于右室流出道重建。结果 :本组病例无手术死亡。平均随访 9.5 (3～ 19)个月 ,死亡 1例。存活患儿心功能明显改善 ,超声心动图检查显示吻合口无狭窄 ,VHC管腔通畅 ,瓣膜无明显返流。结论 :本组结果表明VHC是儿童先心病右室流出道重建的理想材料。     

Congenitally corrected transposition of the great arteries with coexisting stenosis of pulmonary trunk valve and ventricular septum defect in a 55-year-old woman--a case report

Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac malformation. This anomaly is characterised by atrioventricular as well as ventriculoarterial discordance. Isolated CCTGA may cause no symptoms until adult life. Most CCTGA cases with concomitant cardiac abnormalities are symptomatic and are therefore usually diagnosed in childhood. In the majority of patients, congestive heart failure secondary to right ventricular dysfunction occurring by the fifth or sixth decade enables diagnosis. We present an oligosymptomatic 55-year-old woman with corrected transposition of the great arteries and coexisting stenosis of pulmonary trunk valve and ventricular septum defect.     

Coronary artery anatomy in corrected transposition of the great arteries

Congenitally corrected transposition of the great arteries is an unusual cardiac malformation with discordant atrioventricular and ventriculoarterial alignments. Because knowledge of the coronary artery anatomy is a prerequisite for successful repair of this cardiac anomaly, selective coronary arteriography was performed in 13 children (4 male and 9 female; age range 18 months to 16 years) and 1 adult (aged 59 years) with congenitally corrected transposition of the great arteries and associated intracardiac defects. The typical coronary distribution of corrected transposition (that is, coronary artery-ventricular concordance) was found in 11 patients. In one patient, a single coronary ostium was observed; the right sinus of Valsalva gave rise to a short common branch that divided into three arteries: a left circumflex artery going to the right, a well developed left anterior descending artery running into the anterior interventricular groove and a third vessel that continued on the normal course of the right coronary artery directed posteriorly. In one patient, the left circumflex artery was particularly small. In another patient, with severe hypoplasia of the left anterior descending coronary artery, the anterior ventricular wall of the heart was supplied by three small branches that ended a short distance from their origins. The adult patient had a large anterior ventricular branch arising from the morphologic left coronary ventricular as well as a large acute marginal branch, with a wide distribution, from the morphologic right coronary artery. Presurgical coronary angiographic documentation is helpful because, in congenitally corrected transposition as well as in complex congenital heart disease, coronary anomalies (in origin, course and distribution) are occasionally present and knowledge of their presence can help determine the most appropriate surgical approach.     

完全性肺静脉异位引流的外科治疗

目的总结完全性肺静脉异位引流（total anomalous pulmonary venous drainage,TAPVD）的外科治疗经验。方法TAPVD27例（心上型15例,心内型12例）均在体外循环下手术。心上型6例采用传统双心房联合切口,9例采用心上法,后者经上腔静脉与升主动脉之间显露左心房与共同静脉干行-一侧吻合：心内型切开房间隔缺损与冠状静脉开口之间的残余房间隔壁,自体心包将肺静脉开口与冠状静脉窦隔入左心房。结果早期死亡2例,其他病人痊愈出院,术后随访3个月-4年,恢复良好,无吻合口狭窄发生,出院后无因心、肺衰竭死亡病人。结论TAPVD早期诊断极为重要,手术治疗的关键在于左心房与肺总静脉吻合口应足够大,避免狭窄的发生。     

Congenitally corrected transposition of the great arteries associated with hypoplasia of the morphological left ventricle in the setting of atrial situs inversus.

Hypoplasia of the morphological left ventricle associated with severe pulmonic stenosis and an intact ventricular septum in the setting of congenitally corrected transposition of the great arteries is a rare lesion. We report the clinical and echocardiographic findings of this lesion in a patient with atrial situs inversus.     

Congenitally corrected transposition of the great arteries: Morphologic study of 32 cases

The detailed anatomy of the heart is described in 32 autopsy cases of congenitally corrected transposition of the great arteries. This condition is defined as the combination of atrioventricular (A-V) discordance and transposition of the great arteries. Examples of primitive (single) ventricle with “inverted” (that is, left-sided in situs solitus) outlet chamber are excluded. Six hearts with A-V discordance and pulmonary atresia are described in an appendix. In 29 cases of corrected transposition the heart was in situs solitus; in 3 it was in situs inversus totalis. Only 5 of these 32 hearts had no potential for intracardiac shunting. Anomalies of the tricuspid valve (91 percent of cases), ventricular septal defect (78 percent) and pulmonary outflow tract obstruction (44 percent) occurred with sufficient frequency to be considered part of the basic malformation and are described in detail. The precise anatomy and disposition of the A-V valve tension apparatus, the coronary arteries and the conducting tissues are described with special reference to possible surgical approaches for repair of the anomalies. In two hearts with situs solitus the aortic valve was right-sided with respect to the pulmonary valve. This finding is important for both diagnosis and nomenclature.     

Corrected transposition of the great arteries. A study of twelve cases

Twelve cases of corrected transposition of the great arteries are reviewed, and the clinical and laboratory features that can be helpful in making this diagnosis are discussed. Conduction disturbances, ventricular septal defects, pulmonary stenosis, patent ductus arteriosus and left atrioventricular valve regurgitation were the most commonly associated defects.