Scanning suprasternal echocardiography.

Scanning suprasternal echocardiography was performed in 280 patients with a variety of cardiac anomalies. By using the special suprasternal transducer on the suprasternal notch, the aortic arch, right pulmonary artery, and left atrium were recorded superoinferiorly. From this reference position various scanning techniques were made to record the main pulmonary artery, pulmonary valve, ascending aorta, aortic valve, and distal aortic arch, wherever possible. These scans made it possible to assess (a) the integrity and size of right pulmonary artery and main pulmonary artery in pulmonary atresia, stenosis of origin of right pulmonary artery, pulmonary artery banding, kinked Waterston anastomosis, and aneurysm of pulmonary artery; (b) relative positions of aortic valve and pulmonary valve in malposition complexes; (c) the position and size of the aortic arch in tetralogy of Fallot and aortic coarctation. Measurements of the left atrium made by suprasternal echocardiography were consistently larger than those made by praecordial echocardiography. Changes in relative sizes of aortic arch, right pulmonary artery, main pulmonary artery, and left atrium were also documented in the various cardiac anomalies. The atrial baffle after Mustard repair for d-transposition of the great arteries and the atrial membrane in cor triatriatum were also demonstrated. The introduction of scanning techniques has thus widened the scope of suprasternal echocardiography.     

Aortic coarctation surgically treated in the 1st year of life. Results in 36 cases

Thirty-six infants under one year of age underwent surgical repair of coarctation of the aorta between 1968 and 1983 in our institution. Coarctation was isolated or associated to a patent ductus arteriosus in sixteen cases, while in twenty it was associated with significant intracardiac disease. Twenty-nine patients were operated on in the first three months of life and in twenty a severe heart failure was present before the operation. Nine patients (25%) died while in the hospital: all of them were less than three months of age and all but one were affected by major intracardiac anomalies and severe heart failure. Surgical repair was by subclavian flap aortoplasty in twenty-nine cases, resection with end-to-end anastomosis in three, patch aortoplasty in three and Blalock-Park anastomosis in one. Operative mortality was unaffected by the surgical technique. The surviving children were followed-up for 30 +/- 7 months; one late death occurred suddenly, two months after the repair of a ventricular septal defect. An arm/leg pressure gradient, indicative of recoarctation, was detected in five cases: only one had been repaired by the subclavian flap technique, while the others were the only survivors of the end-to-end anastomosis and patch aortoplasty group. Subclavian flap aortoplasty is suggested as the operation of choice for coarctation of the aorta in the first year of life.     

Surgical treatment of major intracardiac lesions associated with discordant atrioventricular connexion

Thirteen patients with a discordant atrioventricular connexion underwent repair of major associated intracardiac defects. Of the patients, 10 had a discordant ventriculoarterial connexion while 3 had double outlet from the morphologically right ventricle. A ventricular septal defect was the most frequently encountered lesion, present alone or in combination with other lesions in all patients. The other major lesions were pulmonary stenosis in 8, Ebstein's malformation of the left atrioventricular valve in 2, and calcific aortic valve disease in one. The operations performed were closure of the ventricular septal defect in 4 patients, closure of the ventricular septal defect with pulmonary valvotomy in 3 patients (one of whom subsequently underwent replacement of the left atrioventricular valve). Modified Fontan's procedure was performed in 6 patients, one of whom also had replacement of the aortic valve. There was no operative death, although there was one early death on the 40th postoperative day due to septicemia. There has been no late death after an average follow-up of 1.2 years. There was one case of surgically induced complete heart block. All other patients are in normal sinus rhythm in New York Heart Association functional class I or II. Elective repair of major intracardiac anomalies in association with a discordant atrioventricular connexion can now be accomplished safely. The modified Fontan's procedure is a viable alternative in certain cases to the placement of an external valved conduit for relief of pulmonary outflow tract obstruction.     

主动脉弓阻塞合并心脏畸形18例的一期矫治

目的总结一期矫治主动脉弓阻塞合并心脏畸形的诊治经验。方法2005年1月至2006年6月一期矫治18例主动脉弓阻塞合并心脏畸形。其中主动脉弓中断(IAA)6例,主动脉缩窄(COA)12例。手术均为正中开胸低温体外循环下一期畸形矫治术。结果手术采用降主动脉与主动脉弓端端吻合11例,端侧吻合6例,Gore-Tex片加宽弓成形1例。围术期死亡1例,总死亡率5.56%。喉返神经损伤2例,术后左主支气管受压1例。随访1～11个月,1例上下肢压差25mmHg,6例压差小于20mmHg,7例端侧吻合无压差。存活者无症状,生长发育良好。结论正中开胸一期矫治COA及IAA合并心脏畸形可获得满意的近期疗效。手术成功的关键是充分游离降主动脉,尽可能行端侧吻合术。     

New technique for single-staged repair of aortic coarctation and coexisting cardiac disorder

The management of adults with aortic coarctation and a coexisting cardiac disorder is still a surgical challenge. Single-staged procedures have lower postoperative morbidity and mortality rates than do 2-staged procedures. We present our experience with arch-to-descending aorta bypass grafting in combination with intracardiac or ascending aortic aneurysm repair.From October 2004 through April 2010, 5 patients (4 men, 1 woman; mean age, 45.8 ± 9.4 yr) underwent anatomic bypass grafting of the arch to the descending aorta through a median sternotomy and concomitant repair of an intracardiac disorder or an ascending aortic aneurysm. Operative indications included coarctation of the aorta in all cases, together with severe mitral insufficiency arising from damaged chordae tendineae in 2 patients, ascending aortic aneurysm with aortic regurgitation in 2 patients, and coronary artery disease in 1 patient. Data from early and midterm follow-up were reviewed.There was no early or late death. Follow-up was complete for all patients, and the mean follow-up period was 34.8 ± 18 months (range, 18 mo-5 yr). All grafts were patent. No late graft-related sequelae or reoperations were observed.For single-staged repair of aortic coarctation with a coexistent cardiac disorder, we propose arch-to-descending aorta bypass through a median sternotomy as an alternative for selected patients.     

One-stage repair of cardiac and arch anomalies without circulatory arrest

Between 1999 and 2002, 23 patients underwent single-stage complete repair of cardiac anomalies and aortic arch obstruction, without circulatory arrest. Median age was 1.2 years. Intracardiac defects included ventricular septal defect in 9, double-outlet right ventricle in 6, d-transposition of the great arteries and ventricular septal defect in 2, subaortic obstruction in 3, and atrial septal defect in 3. Fourteen patients had coarctation of the aorta, 6 had coarctation with hypoplastic aortic arch, and 3 had interrupted aortic arch. Simple techniques were employed such as cannulation of the ascending aorta near the innominate artery and maintaining cerebral and myocardial perfusion. After correction of arch obstruction, intracardiac repair was undertaken. The mean cardiopulmonary bypass time was 169 min, aortic crossclamp time was 51 min, and arch repair took 16 min. There was no operative mortality or neurological deficit. In follow-up of 1-43 months, no patient had residual coarctation. This simplified technique avoids additional procedures, reduces ischemic time, and prevents problems related to circulatory arrest.     

Correction of coarctation of aorta in adult patients--impact of corrective procedure on long-term recoarctation and systolic hypertension

BACKGROUND: Uncorrected coarctation of the aorta in adults predisposes to congestive failure, aortic rupture, stroke and endocarditis. Surgical correction of this condition is fraught with technical difficulties due to the complexity of the lesion, associated anomalies and extensive collaterals. The optimal surgical technique has not yet been well defined in adults. We describe our experience with adult coarctation and the long-term outcome with regard to recoarctation and systolic hypertension. MATERIALS AND METHODS: A consecutive series of 54 patients above the age of 12 years who underwent coarctation correction over a period of 8 years are included in this report. Eight patients underwent balloon angioplasty, one of whom also had stent placement. Forty-eight patients underwent surgical intervention. Twenty-seven patients underwent resection and anastomosis, 6 had patch angioplasty and 13 had a prosthetic graft repair. All patients were followed up at 3 months and then annually to look for recoarctation, regression of hypertension and aneurysm formation. RESULTS: There was no mortality in this series; major morbidities included reoperation for bleeding in 3 patients, pulmonary complications and paraplegia in 1 patient. Hypertension was well controlled in all patients at discharge. Follow-up is 100 % complete and mean follow-up was 4.6 years. There was one case of aortic dissection and one case of aneurysm formation in the balloon angioplasty group. There was a statistical trend towards increased rates of recoarctation in the balloon and resection and anastomosis groups. 32 % of patients were off antihypertensive medications at their last follow-up. CONCLUSIONS: Surgical correction of coarctation of aorta in adults can be achieved with an acceptable morbidity. A variety of options are available for the surgical management of coarctation of the aorta in adults. Prosthetic graft and patch repair are associated with good short and long-term results. Use of balloon angioplasty without stenting as the primary therapy requires further clarification. Hypertension is well controlled in most patients.     

Interrupted aortic arch. A series of 15 patients

Between 1983 and 1989, 15 children underwent surgical repair of interrupted aortic arch at 1 to 20 days of age. The anatomical form was a Celoria and Patton type B in all patients with an associated perimembranous ventricular septal defect in all but one who had multiple ventricular septal defects, and patent ductus arteriosus. Six children had a retro-esophageal right subclavian artery, two had subaortic stenosis and two had a right-sided descending thoracic aorta. In two children with severe hypoplasia of the ascending aorta the repair was performed in one stage with two deaths due to left ventricular failure. In the other B cases, a two-stage repair was carried out. The reconstruction of the aortic arch varied according to the individual case. All children had pulmonary artery banding. Seven children survived longer than 30 days. Six of them later underwent a complete repair. The only survivors were those patients in whom the neo-aortic arch grew harmoniously. The authors conclude that: a two-stage repair gave disappointing results in this series of consecutive patients, mainly because of the poor quality of the reconstruction of the aortic arch by thoracotomy.     

A new strategy for the surgical treatment of aortic coarctation associated with ventricular septal defect in infants using an absorbable pulmonary artery band.

OBJECTIVES: We propose a new strategy using coarctation repair together with a polidioxanone absorbable pulmonary artery banding to limit operative risk and to spare infants with aortic coarctation subsequent operations. BACKGROUND: The alternative for the surgical management of aortic coarctation associated with ventricular septal defect (VSD) is single-stage repair versus coarctation repair with or without banding of the pulmonary artery. METHODS: Eleven infants (mean weight 2,560 +/- 1,750 g, range 1,320 to 3,800 g) underwent a coarctation repair with a polydioxanone banding. Seven had a trabecular and four a perimembranous VSD. The mean size of the VSD was 5 +/- 0.7 mm (range 4 to 7 mm). The systolic pulmonary pressure was >80% of the aortic pressure in all. The pulmonary band was tightened until the systolic pulmonary pressure fell below 50% of the aortic pressure. RESULTS: There were no hospital deaths. The reabsorption of the banding was complete after 5.7 months in all patients (3 to 6.5 months). The VSD closed completely in four infants and partially in six, in whom the pulmonary artery pressure was normal without evidence for significant left-to-right shunt. One patient with a large trabecular VSD underwent surgical closure of his defect after four months. Finally, a subsequent open-heart surgery could be avoided in 91% (10/11) of patients. CONCLUSIONS: Provided the VSD belongs to types prone to close spontaneously, this policy may reduce the number of surgical procedures per infant as well as in-hospital mortality and morbidity rates. It should be proposed as an alternative to more complex procedures.     

Coarctation of the aorta and Fallot's tetralogy. Apropos of 2 cases

Two cases of the association of Fallot's tetralogy and coarctation of the aorta are reported. In both cases there was a right aortic arch. In addition, the second case had a cervical aorta and a left subclavian artery isolated from the aorta and vascularised by the left vertebral artery. The two children underwent complete repair: the first patient had a Waterston shunt followed by repair of the Fallot's tetralogy, followed by repair of the coarctation; the second patient first had a Gore-tex tube inserted from the ascending to the descending aorta and then underwent repair of the Fallot's tetralogy. The association of a coarctation with an obstructive lesion of the right heart is very rare; only two cases have previously been reported: one Fallot's tetralogy and one tricuspid atresia with pulmonary stenosis. Both cases also had a right aortic arch. This seems to go against the haemodynamic theory of coarctation: blood flow in the ascending aorta increases while flow in the pulmonary artery decreases. Coarctation could be caused by a partial involution of the terminal segment of the right primitive dorsal aorta as proposed in cases of coarctation with a right sided aortic arch. The second case also presented two uncommon features: a cervical aortic arch and a left subclavian artery isolated from the aorta.     

Cardiovascular findings, and clinical course, in patients with Williams syndrome

AIMS: We investigated the incidence and outcome of cardiac malformations in 53 patients with Williams syndrome. METHODS AND RESULTS: The mean age, and period of follow-up, were 3.6 and 5.3 years, with standard deviations of 4.0 and 5.6 years, respectively. Of the patients, 45 (85%) had cardiovascular anomalies, often combined. Males presented earlier than females, at the ages of 2.1 years, with standard deviation of 2.8 years, as opposed to 4.5 years, with standard deviation of 4.2 years (p < 0.01). Supravalvar aortic stenosis occurred in 32 patients (71%), pulmonary arterial stenosis in 17 (38%), and mitral valvar prolapse in 12 (27%), 9 of these having regurgitant valves. Pulmonary valvar stenosis, ventricular septal defect, coarctation of the aorta, persistent patency of the arterial duct, hypertrophic cardiomyopathy, and subaortic stenosis all occurred less frequently. In 21 patients (47%), 24 surgical or catheter interventions had been made, most often for repair of supravalvar aortic stenosis, undertaken on 16 occasions with just one recurrence, and in 4 along with surgery to the mitral valve. Other lesions requiring intervention were pulmonary valvar stenosis, pulmonary arterial stenosis, coarctation of the aorta, and subaortic stenosis. We lost 3 patients (7%), with severe supravalvar aortic stenosis and moderate or severe mitral regurgitation, 2 early and one late after surgery. CONCLUSION: The most frequent cardiovascular anomalies in Williams syndrome were supravalvar aortic stenosis, pulmonary arterial stenosis, and mitral valvar prolapse, which occurred more frequently in our patients than previously observed. Patients with left ventricular pressure and volume overload were at greater risk.     

Critical aortic coarctation: patch aortoplasty in infants less than age 3 months.

Twenty-three infants less than age 3 months (mean age 31 days) underwent patch aortoplasty for relief of coarctation of the aorta. All had intractable congestive heart failure, despite aggressive medical therapy. Each infant had other cardiac anomalies, including patent ductus arteriosus (83 percent) and ventricular septal defect (74 percent). All patients underwent closure of the ductus arteriosus and patch angioplasty of the aorta to produce a luminal diameter of at least 16 mm. In addition, 9 of the 17 patients (53 percent) with a large shunt ventricular septal defect underwent pulmonary arterial banding. There was one hospital death 42 days after operation secondary to bowel perforation and sepsis. Hospitalization beyond 21 days postoperatively was always due to other unrepaired cardiac lesions. The three late deaths at 3, 9 and 18 months after operation were associated with additional major anomalies. Fourteen patients have had postoperative catheterization. No gradient was found across the site of coarctation repair, but one patient had a gradient between the left carotid and left subclavian arteries. Surgical repair of critical coarctation of the aorta in infants can safely be offered despite the presence of other cardiac anomalies.     

Anatomical variants in aortic atresia. Potential candidates for ventriculoaortic reconstitution.

Aortic valve atresia is usually associated with severe hypoplasia of the left ventricle and mitral apparatus. Palliative procedures, including atrial septectomy, pulmonary artery-aorta anastomosis, and pulmonary artery banding, have been performed in some patients. However, because of the distinct underdevelopment of the left heart, the long-term prognosis must be guarded even in the survivors of these procedures. Rarely, aortic valve atresia is found with adequate left heart chambers. Because very few of these patients are described, the clinical, angiocardiographic, and the pathological findings in two patients are presented, with comments on palliation in the neonatal period, and the potential for ventriculoaortic reconstitution.     

Anatomical variants in aortic atresia. Potential candidates for ventriculoaortic reconstitution.

Aortic valve atresia is usually associated with severe hypoplasia of the left ventricle and mitral apparatus. Palliative procedures, including atrial septectomy, pulmonary artery-aorta anastomosis, and pulmonary artery banding, have been performed in some patients. However, because of the distinct underdevelopment of the left heart, the long-term prognosis must be guarded even in the survivors of these procedures. Rarely, aortic valve atresia is found with adequate left heart chambers. Because very few of these patients are described, the clinical, angiocardiographic, and the pathological findings in two patients are presented, with comments on palliation in the neonatal period, and the potential for ventriculoaortic reconstitution.     

Anomalous origin of one pulmonary artery from the ascending aorta

We describe the surgical repair in three infants presenting with one pulmonary artery arising from the ascending aorta, the other artery arising normally from the right ventricle via the pulmonary trunk. Repair consisted of reimplantation of the anomalous pulmonary artery to the pulmonary trunk, in association with repair of associated intracardiac malformations. All patients survived the surgical procedures, and were discharged in stable clinical condition. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and underwent reoperation. Although survival after operation is now expected for this malformation, reports of late results are lacking. Larger numbers of operations are needed before we can reach definitive conclusions. The origin of one branch pulmonary artery from the ascending aorta in the presence of a pulmonary valve and main pulmonary artery is a very rare congenital cardiac anomaly. PATIENTS AND METHODS: Between January 1995 and June 2003, 3 infant girls presented with the origin of one branch artery from the ascending aorta, while the other pulmonary artery originated from the pulmonary trunk which was in continuity with the right ventricular outflow tract. The pulmonary artery that arose from the right ventricle was left in 2 and right in 1 patient. RESULTS: At the age 13, 48 and 62 days respectively, the patients underwent surgical repair consisting with reimplantation of the anomalous pulmonary artery branch to the pulmonary trunk in association with repair of intracardiac malformations. There were no hospital deaths. Postoperative complications included: prolonged intubation in two patients (10 and 16 days), low output syndrome in 1 patient, cardiac tamponade in 1 patient and seizures in 1 patient. All patients were discharged in good clinical condition. There have been no late deaths. Subsequently, two of the three patients developed stenosis at the surgical anastomosis relatively early after the initial procedure, and after unsuccessful balloon dilation, underwent surgical reoperation. CONCLUSIONS: Although operative survival is now possible for this malformation, reports of late results are lacking. Two of the three patients developed stenosis at the surgical anastomosis relatively early after surgery. Larger numbers of operations are necessaries to reach definitive conclusions.     

High Long‐term Morbidity in Repaired Aortic Coarctation

Objective. The objective of this study was to assess late morbidity after repair of aortic coarctation and its association with residual aortic arch obstruction. Design and Setting. This is an observational cohort study of 133 patients who underwent surgical repair during 1965–1985. Echocardiography, bicycle exercise testing, 24‐hour ambulatory blood pressure monitoring, and magnetic resonance imaging/computerized tomography scan of the thoracic aorta were performed. The setting of this study was a tertiary referral center. Patients. Among 156 survivors, 133 (84 men) accepted study participation. Median age (range) was 10 (0.1–40) years at repair and 44 (26–74) years at follow‐up. Outcome Measures. Outcome measures used are prevalence of previous cardiovascular reinterventions, current cardiac and valvular function, exercise capacity, blood pressure levels at rest and during exercise, and presence of recurrent or residual aortic arch obstruction and/or aortic aneurysms. Results. Thirty‐five had undergone cardiovascular reinterventions. Sixteen had an aortic and three had a mitral valve prosthesis; 117 had a native aortic valve that was bicuspid in 63 and dysfunctional in 45. Ejection fraction was below 50% in 16. On exercise, performance was reduced in 37 and hypertension was induced in 47. Fifty‐eight had elevated blood pressures and further 17 received antihypertensives. The ascending aorta was aneurysmal in 28 and the distal arch in five. The presence of a bicuspid aortic valve was significantly associated with valve regurgitation and ascending aortic ectasia. Fifty‐eight of 121 patients had minimal aortic arch diameters between 46% and 79% of the diaphragmatic aortic diameter, indicating moderate/mild recoarctation. This was associated with elevated blood pressures and use of antihypertensive medication, but not with hypertension in unmedicated patients or with echocardiographic or exercise parameters. Only five patients had normal study findings, were normotensive, and without reinterventions after coarctation repair. Conclusions. Cure by repair of aortic coarctation is rare; heart diseases, aortopathy, and hypertension are common. Morbidity is only weakly associated with mild/moderate recoarctation.     

Repair of anomalous origin of the left coronary artery in the infant and small child

Anomalous origin of the left coronary artery from the pulmonary artery is associated with myocardial infarction, left ventricular dysfunction, mitral valve dysfunction and, occasionally, intracardiac congenital abnormalities. A technique that utilizes a flap of the anterior wall of the pulmonary artery to serve as a neocoronary artery to direct aortic flow from a created aortopulmonary window to the pulmonary artery orifice of the anomalous left coronary artery was used in five patients aged 2.5 months to 4.75 years. Two patients were less than 4 months of age at operation. There was one death 2 days after operation and one late death. The two youngest patients required mitral valve replacement. Two of the three surviving patients are well at follow-up at 7 to 44 months. One patient has been lost to follow-up study. One patient had postoperative catheterization which showed an intact repair. The pulmonary artery neocoronary procedure is applicable to infants and small patients with anomalous origin of the left coronary artery from the pulmonary artery.     

Surgical treatment of pulmonary atresia with intact ventricular septum.

Fifty-nine children with pulmonary atresia and intact ventricular septum underwent various forms of surgical treatment at the Hospital for Sick Children, Toronto, during 1950 to mid 1975. Twenty-three patients had pulmonary valvotomy, 15 direct, 2 indirect, and 6 both direct valvotomy and infundibulectomy. All died, 19 early and 4 late. Of 13 patients who received a systemic-pulmonary artery shunt, 4 combined with surgical atrial septectomy, there are only 2 long-term survivors both of whom were children who had had a Waterston anastomosis. Recently we have been treating infants with small right ventricles with balloon atrial septostomy at cardiac catherterization followed by a Potts anastomosis and pulmonary valvotomy. If the Potts anastomosis appears satisfactory the persistent ductus arteriosus is ligated. This scheme was used in 23 infants, with 4 early deaths and 2 late deaths. Of 17 survivors, further shunts were required in 4 children. One child has had a formal repair, with insertion of valves in both tricuspid and pulmonary areas. We believe that this operative combination of Potts anastomosis and pulmonary valvotomy offers the infant with pulmonary atresia and a small right ventricle a relatively low initial mortality and the possibility of right ventricular enlargement and subsequent repair.     

Surgical repair of persistent truncus arteriosus in infancy

Fourteen patients younger than two years of age with persistent truncus arteriosus underwent primary repair. Twelve of them were less than 1 year and 4 less than 3 months of age. Intractable heart failure was the indication for surgery in all patients but one who had increased pulmonary vascular resistance. There were 5 hospital and 2 late deaths. Six out of the 7 survivors (median follow-up: 29 months) were symptom-free. The remaining infant who preoperatively had significant truncal valve regurgitation was doing fairly well 2 1/2 years after repair. Our experience suggests that, although the mortality remains high, primary repair for infants with persistent truncus arteriosus is feasible and offers better overall results than does pulmonary artery banding followed by later intracardiac repair. We advise primary repair for all infants with intractable heart failure or increasing pulmonary vascular resistance with or without truncal valve regurgitation. Elective repair is recommended before the age of 2 years to minimize the risk of pulmonary vascular disease.     

54例主动脉缩窄的外科治疗

我院 1984年 6月至 1998年 6月 ,共收治 54例主动脉缩窄病人。男性 2 7例 ,女性 2 7例 ,平均年龄 15.8岁 ( 5个月～ 39岁 )。其中婴儿型 18例 ,成人型 36例 ,合并动脉导管未闭 33例 ,室间隔缺损 2 0例 ,房间隔缺损 4例 ,主动脉瓣二瓣化畸形 4例 ,主动脉瓣下狭窄 3例 ,二尖瓣狭窄 2例 ,二尖瓣脱垂 1例。其中 39例进行狭窄处补片扩大成形术 ,5例进行狭窄处切除主动脉端端吻合术 ,3例进行了狭窄处切除人工血管移植术 ,4例进行了人工血管转流手术 ,3例用主肺动脉内隧道的方法 ,实现了升主动脉 降主动脉的连接 ,同时行室缺修补。手术死亡 1例 ( 1.85% )。远期随访死亡 3例 ( 5.6% ) ,其余效果良好