The value of 2-D Doppler echocardiography in the evaluation of asymptomatic patients with Mustard operation for transposition of the great arteries

2-D Doppler echocardiography was used to assess the occurrenceof haemodynamic abnormalities in 45 asymptomatic patients, aged4 to 16 years (median 7·4) after a Mustard operationfor transposition of the great arteries. The findings were comparedwith those derived from cardiac catheterization. Thirty-fivecardiac lesions were correctly diagnosed by 2-D Doppler echocardiographyin 23 patients, but on six occasions, minor abnormalities weremissed. 2-D Doppler echocardiography demonstrated systemic venouspathway obstruction of more than 3 mmHg at cardiac catheterizationin nine patients, and in five of the six patients with pulmonaryvenous channel obstruction. A left ventricular outflow tractobstruction (pressure difference > 15 mmHg) was diagnosedcorrectly by Doppler echocardiography in seven patients. Baffleleakage was found in two patients with a left to right shuntof 25% or more of pulmonary bloodflow, but was missed in fiveout of nine patients with small shunts. Tricuspid regurgitationwas well defined in eight patients, The absence of symptomsand a routine examination after a Mustard operation do not ruleout haemodynamic abnormalities. However, these, with the possibleexception of minor baffle leakage, can be detected by 2-D Dopplerechocardiography.     

The value of 2-D Doppler echocardiography in the evaluation of asymptomatic patients with Mustard operation for transposition of the great arteries.

2-D Doppler echocardiography was used to assess the occurrence of haemodynamic abnormalities in 45 asymptomatic patients, aged 4 to 16 years (median 7.4) after a Mustard operation for transposition of the great arteries. The findings were compared with those derived from cardiac catheterization. Thirty-five cardiac lesions were correctly diagnosed by 2-D Doppler echocardiography in 23 patients, but on six occasions, minor abnormalities were missed. 2-D Doppler echocardiography demonstrated systemic venous pathway obstruction of more than 3 mmHg at cardiac catheterization in nine patients, and in five of the six patients with pulmonary venous channel obstruction. A left ventricular outflow tract obstruction (pressure difference greater than 15 mmHg) was diagnosed correctly by Doppler echocardiography in seven patients. Baffle leakage was found in two patients with a left to right shunt of 25% or more of pulmonary blood flow, but was missed in five out of nine patients with small shunts. Tricuspid regurgitation was well defined in eight patients. The absence of symptoms and a routine examination after a Mustard operation do not rule out haemodynamic abnormalities. However, these, with the possible exception of minor baffle leakage, can be detected by 2-D Doppler echocardiography.     

Tissue Doppler derived isovolumic acceleration in patients after atrial repair for dextrotransposition of the great arteries

Background: Ventricular dysfunction represents one of the major problems in the long-term follow-up of patients after atrial repair for dextrotransposition of the great arteries. We aimed to study the role of tissue Doppler derived isovolumic acceleration (IVA) to detect early myocardial dysfunction in these patients. Methods: Twenty-four patients with dextrotransposition of the great arteries (D-TGA) that underwent atrial repair (Senning procedure: n = 12; Mustard procedure: n = 12) in infancy were examined at the age of 21 [12–33] years (median [range]) using tissue Doppler analysis of IVA and peak systolic myocardial velocity at rest and during exercise. 12 age-matched healthy subjects served as controls. Results: At rest, IVA and peak systolic myocardial velocity were reduced in the systemic ventricle (SV) of patients. IVA correlated with peak systolic myocardial velocity (r = 0.76, P < 0.001). During exercise, IVA, but not peak systolic myocardial velocity, increased significantly in the SV of patients (rest: 1.03 ± 0.44 cm/sec²; 1 W/kg: 1.80 ± 1.22 cm/sec²; 2 W/kg: 2.85 ± 1.26 cm/sec²). In the subpulmonary ventricle, IVA was significantly lower in patients compared to the controls (patients: 1.45 ± 0.49 cm/sec² vs. controls: 2.31 ± 0.43 cm/sec², P < 0.05). IVA but not peak systolic myocardial velocity was able to discriminate between patients and healthy subjects. Conclusions: IVA is superior to peak systolic myocardial velocity to assess a reduction in functional reserve of both ventricles in patients after atrial repair for D-TGA.     

32 year follow up of patients following atrial redirection surgery for transposition of the great arteries

Objective: A significant body of patients who have undergone Mustard or Senning procedure require lifelong follow up. In this retrospective review, we examined the cohort of such patients currently attending our center.
Design: Patients who had undergone either Mustard or Senning procedure were identified. We retrospectively reviewed medical records, recorded demographic in‐ formation and data regarding the clinical state, NHYA class, cardiopulmonary exer‐ cise testing, NT‐proBNP measurement, and recent cardiac MRI findings.
Results: Forty‐six patients were identified, the mean age was 32.2 years (± 6.1 years), 67.4% were male. Thirty‐two patients (69.6%) had undergone a Senning procedure. The median length of the follow‐up was 32 years. Thirty‐two patients (69.6%) were NHYA class 1. The mean VO_2max achieved was 24.2 ± 5.8 mL/min/kg. The mean NT‐ proBNP was 266.4 pg/mL (± 259.9 pg/mL). The mean right ventricular end‐diastolic volume (RVEDV) was 212.4 mL ± 73.1 mL (indexed 114.2 mL/m² ± 34.4 mL/m² ). The mean right ventricular ejection fraction (RVEF) was 53.7% ± 7.9%. The mean left ven‐ tricular end‐diastolic volume (LVEDV) was 161.5 mL ± 73.7 mL (indexed 87.8 mL/ m² ± 41.1 mL/m² ). The mean left ventricular ejection fraction (LVEF) was 59.8% ± 5.7%. There was a significant correlation between right ventricular (RV) size on MRI and NT‐proBNP level.
Conclusions: We present a relatively well cohort of patients with overall favorable long‐term outcome. The majority of patients are NHYA class 1 and the systemic right ventricular function appears to be well preserved as assessed by MRI. The exercise tolerance is reduced, with the majority of patients achieving around 60% of the esti‐ mated VO_2max. Regular specialist follow‐up and assessment with advanced imaging at regular intervals remain important for this group.     

Outcome in adult patients after arterial switch operation for transposition of the great arteries

Background

The arterial switch operation (ASO) is currently the treatment of choice in neonates with transposition of the great arteries (TGA). The outcome in childhood is encouraging but only limited data for long-term outcome into adulthood exist.

Methods and results

We studied 145 adult patients (age > 16, median 25 years) with ASO followed at our institution. Three patients died in adulthood (mortality 2.4/1000-patient-years). Most patients were asymptomatic and had normal left ventricular function. Coronary lesions requiring interventions were rare (3 patients) and in most patients related to previous surgery. There were no acute coronary syndromes. Aortic root dilatation was frequent (56% patients) but rarely significant (> 45 mm in 3 patients, maximal-diameter 49 mm) and appeared not to be progressive. There were no acute aortic events and no patient required elective aortic root surgery. Progressive neo-aortic-valve dysfunction was not observed in our cohort and only 1 patient required neo-aortic-valve replacement. Many patients (42.1%), however, had significant residual lesions or required reintervention in adulthood. Right ventricular outflow tract lesions or dysfunction of the neo-pulmonary-valve were frequent and 8 patients (6%) required neo-pulmonary-valve replacement. Cardiac interventions during childhood (OR 3.0, 95% CI 1.7–5.4, P < 0.0001) were strong predictors of outcome (cardiac intervention/significant residual lesion/death) in adulthood.

Conclusions

Adult patients with previous ASO remain free of acute coronary or aortic complications and have low mortality. However, a large proportion of patients require re-interventions or present with significant right sided lesions. Life-long cardiac follow-up is, therefore, warranted. Periodic noninvasive surveillance for coronary complications appears to be safe in adult ASO patients.     

Stress Doppler echocardiography in the evaluation of ischemic heart disease

Doppler echocardiography enables convenient, noninvasive evaluation of global, systolic performance at rest and during exercise. Early studies suggested that Doppler parameters of systolic function were sensitive to exercise-induced myocardial ischemia and could identify patients with severe coronary artery disease. Subsequent investigation, however, has identified several factors in addition to myocardial ischemia that can significantly influence exercise Doppler study results. Thus, in order to obtain reliable information, the many factors that can influence Doppler measurements of aortic flow velocity and acceleration must be accounted for. Further work in this area is likely to produce results that encourage greater application of this technique in experimental and clinical research. At present, the role of stress Doppler echocardiography in the evaluation of ischemic heart disease remains uncertain.     

Doppler color flow echocardiography: indispensable application to congenital heart disease

Doppler color flow imaging has become indispensable in the diagnosis and management of patients with congenital heart disease. Certain defects may not be possible, or may be very difficult to diagnose by two-dimensional echocardiography alone. Such examples include multiple ventricular septal defects, anomalous pulmonary venous connection, coronary artery malformations, and the hypertensive patent ductus arteriosus. Additionally, color flow Doppler echocardiography significantly provides additional information, and reduces the time for fetal and transesophageal echocardiographic studies. Doppler color flow imaging has become an essential part of the echocardiographic examination. Experience has broadened the use of this important technological advance, with anticipation of an ever expanding future for its clinical application.     

Outcomes of transposition of the great arteries in the era of atrial inflow correction

Objective—To examine long term morbidity and mortality following atrial inflow corrective procedures for transposition of the great arteries (TGA) and to investigate factors that influence morbidity and mortality.
Design—Retrospective cohort study from a single centre.
Setting—Cardiology and cardiothoracic surgical unit in a large tertiary referral centre.
Patients—All 130 patients who had TGA diagnosed between August 1972 and May 1988 and were considered suitable for atrial inflow correction; 109 of these underwent surgery (operative cohort: 84 Mustard operations and 25 Senning operations); 95 survived to hospital discharge (hospital surviving cohort).
Main outcome measures—Death and cardiac events.
Results—There were relatively good long term results from atrial inflow correction for TGA with 5, 10, and 15 year survivals of 77.3%, 75.9%, and 71.3%. However, there was an appreciable incidence of late cardiac death and events, with 5, 10, and 15 year cardiac event-free survivals of 74.5%, 67.1%, and 39.6%. Supraventricular tachycardia was the only significant risk factor for late cardiac death (relative risk 8.72, 95% confidence interval, 2.86 to 26.64). Senning patients had better event-free survival (p = 0.04).
Conclusions—Atrial inflow correction for TGA has a reasonably good 15 year survival (71.3%), but there is an appreciable incidence of late cardiac deaths and events (15 year event-free survival 39.6%). The Senning procedure is preferable to the Mustard procedure for cases unsuitable for arterial switching.

Keywords: transposition of the great arteries; congenital heart disease; Mustard operation; Senning operation     

Evaluation of total anomalous pulmonary venous drainage with cross-sectional colour-flow Doppler echocardiography

Three patients with total anomalous pulmonary venous drainage(TAPVD) were studied by real-time cross-sectional colour-flowDoppler echocardiography. Serial suprasternal, parasternal andsubcostal scans were obtained. In all cases surgical or angiocardiographicconfirmation was available. Two patients had supracardiac drainage(to the left vertical vein or to the right superior vena cava)and one patient had infracardiac drainage. An abnormal forwardflow in the left innominate vein and vertical vein was visualizedin those patients with supracardiac TAPVD. Abnormal venous flowwas also imaged in one patient with mixed drainage. In the patientwith infradiaphragmatic TAPVD characteristic flow signals wereidentified in the inferior vena cava (retrograde flow) and inthe descending aorta and anomalous pulmonary venous channel(forward flow). In all patients the patterns of pulmonary venousflow allowed us to distinguish TAPVD from contiguous structuresand to validate two-dimensional cross-sectional imaging.     

一期大动脉调转术治疗完全性大动脉转位

目的:探讨一期大动脉调转术治疗完全性大动脉转位(TGA)的手术适应证及手术效果.方法:2006-05-2007-08对15例患儿[年龄0.5～14(2.51±0.76)个月,体重3～9(3.73±0.86)kg]实施一期大动脉调转手术.15例中室间隔完整TGA(TGA/IVS)13例,均并发房间隔缺损(ASD),同时并发动脉导管未闭(PDA)11例;TGA 并发室间隔缺损(TGA/VSD)2例,均同时并发PDA和ASD.12例并发中度肺动脉高压,3例并发重度肺动脉高压,术前均使用前列腺素E1.手术在全麻、中低温、低流量体外循环下完成.在2大动脉瓣上方横断,将左、右冠状动脉开口移植至肺动脉近端.经肺动脉分叉下方,吻合肺动脉近端和升主动脉远端.用自体心包修复主动脉近端缺失部分,并在新主动脉开放后吻合主动脉近端和肺动脉远端.结果:3例TGA/IVS患儿术后肺部感染,2例(分别为13个月、3个月龄)患儿术后12 h内突发心室颤动死亡,病死率为13.3%.未发生与冠状动脉移植相关并发症.术前肺动脉高压患儿,特别是重度肺动脉高压,术后肺动脉压力均明显下降.13例术后顺利恢复出院.随诊3～12个月,全部患儿心功能恢复良好,无远期并发症和死亡.结论:一期大动脉调转手术对于TGA有较好早期效果.对于年龄超过4个月部分患儿,即使并发肺动脉压,仍可取得满意治疗效果.     

Pregnancy after atrial repair for transposition of the great arteries

OBJECTIVE: To investigate the risk of pregnancy in patients with transposition of the great arteries (TGA) who have undergone atrial repair. DESIGN: Retrospective analysis (1962-94) of 342 TGA patients who underwent atrial repair. Of 231 known late survivors, 48 were women over 18 years old who were interviewed about possible reproductive plans and previous pregnancies. As a control, comparison was made with data of 57 500 women (mean age 26 years) obtained from the Swiss Statistical Bank in Bern. RESULTS: Mean follow up was 13.7 years; 66% remained asymptomatic, 29% had mild to moderate cardiac symptoms, and 5% suffered from severe cardiac symptoms (New York Heart Association grade III-IV). Thirty six of the 48 women wished to bear children and, to date, there have been 10 live births, two spontaneous first trimester abortions, and one induced abortion at 16 weeks. During pregnancy there was one case of cardiac deterioration and two cases of pneumonia. There was no evidence of congenital heart disease in the children. CONCLUSIONS: In this relatively small series the completion of pregnancy in women with TGA who had undergone atrial repair and who had normal functional cardiac status was uncomplicated     

成人心室双人口彩色多普勒超声心动图特征研究

目的 探寻成人心室双入口(double-inlet ventricle,DⅣ)彩色多普勒超声心动图(color Doppler echocardiography,CDE)特征.方法 应用CDE检查32例成人DIV患者,寻找其CDE特征,所有患者均有心血管造影对照,25例经手术证实.结果 根据CDE特征表现对30例做出正...     

彩色超声心动图诊断冠心病的临床价值

目的探讨和分析彩色超声心动图诊断冠心病的临床价值。方法选取我院经初步诊断为冠心病的患者160例,对所有患者进行彩色超声心动图及冠状动脉造影检查,将患者检查结果进行对比分析,并以冠状动脉造影检查的诊断结果作为评价标准,对彩色超声心动图诊断冠心病的临床价值进行评价。结果 160例初诊冠心病均采用彩色超声心动图检查,结果显示153例有不同程度的冠状动脉主干狭窄,再经冠状动脉造影检查后,确诊冠心病的患者有131例。结论对所有患者采用彩色超声心动图和冠状动脉造影检查后,将两种检查方法的诊断结果对比,充分证明彩色超声心动图诊断冠心病具有十分满意的效果且临床价值较高,值得临床推广及应用。     

Arrhythmias in transposition of the great arteries after the Mustard operation

Disorders of rhythm or conduction in patients with transposition of the great arteries (TGA) after the Mustard operation have been widely reported. This study provides a systematic evaluation of the electrophysiologic function of 87 survivors of the Mustard operation at a single institution. Surface electrocardiograms were reviewed in all 87 patients, Holter monitoring data in 26 patients, exercise electrocardiograms in 21 patients, and invasive electrophysiologic data in 61 patients. Surface electrocardiograms showed normal sinus rhythm in 52%, sinus node dysfunction in 27%, and atrioventricular block in 16%. Holter monitoring was obtained in an unselected subgroup of 26 patients who had a mean age of 12 years and a mean interval from operation of 9 years. Sinus node dysfunction was found in 58%, atrioventricular block in 27% ventricular ectopy in 50%, supraventricular ectopy in 27%, and no abnormalities in only 8%. Intracardiac electrophysiologic evaluation showed a high frequency of abnormal sinus node recovery times and suboptimal response of the atrioventricular-conduction system to rapid atrial pacing. When all modalities used in this study were considered, sinus node dysfunction occurred in 47%, ectopy in 34% and atrioventricular block in 23%. Although only 30% of patients had no evidence of arrhythmia, symptoms of rhythm or conduction disturbances were rare.     

Argument for a Doppler echocardiography during exercise in assessing asymptomatic patients with severe aortic stenosis

Aims: Exercise stress testing (EST) is recommended by guidelines torisk-stratify patients with asymptomatic valvular aortic stenosis(AS), though the role of quantitative exercise-Doppler echocardiographyhas rarely been studied. This prospective study sought to correlatestandard EST results with the haemodynamic measurements madeduring exercise by Doppler echocardiography. Methods and results: We performed rest and semi-supine exercise Doppler echocardiographyin 44 consecutive patients (mean age = 68 ± 12 years)with aortic valve areas 0.6 cm²/m². The effective aortic valvearea (EOA), cardiac output (CO), maximal transvalvular velocity,and pulmonary pressure were monitored over the test. No seriousadverse event was observed. EST was positive in 26 (Group 1)and negative in 18 (Group 2) patients. Baseline echocardiographicmeasurements were similar (EOA 0.77 ± 0.15 vs. 0.78 ±0.14 cm²; CO 5.5 ± 1.6 vs. 5.9 ± 2 L/min) in bothgroups. Exercise-induced changes in CO (+2.9 ± 2 vs.+4.3 ± 1.8 L/min, P = 0.04) and EOA (–0.04 ±0.18 vs. +0.15 ± 0.24 cm², P = 0.015) were significantlygreater in Group 2. A correlation between changes in EOA andchanges in CO during exercise was observed, but significantlyhigher in Group 2 (P = 0.04). Conclusion: In the presence of severe asymptomatic AS, exercise Dopplerechocardiography, assessing the mechanisms behind a positiveEST, appears very promising but further studies with prognosisassessment remain necessary.     

Doppler echocardiographic evaluation of ventricular septal defects in adults

Ventricular septal defects account for 10% to 15% of all congenital heart defects observed in adults. The type of defects observed tend to fall at the extreme ends of the spectrum: small restrictive defects with minimal shunts, and large nonrestrictive defects associated with severe occlusive pulmonary vascular disease. Doppler echocardiography has evolved into a powerful diagnostic modality that comprehensively evaluates the anatomical and pathophysiological consequences of a ventricular septal defect. The spectrum of findings caused by ventricular septal defects is the subject of this review.