Exercise capacity in adult patients with congenitally corrected transposition of the great arteries

OBJECTIVE—To examine cardiopulmonary values, static lung function, and ejection fraction in adult patients with congenitally corrected transposition of the great arteries (CCTGA).
PATIENTS AND METHODS—41 patients who had undergone static lung function testing and cardiopulmonary exercise tests with measurements of ejection fraction were identified at the Toronto Congenital Cardiac Centre for Adults.
RESULTS—Aerobic capacity in patients with CCTGA was severely diminished, varying from 30-50% of the results achieved by healthy subjects. Normal values of right ventricular and left ventricular ejection fraction were found. However, the systemic right ventricular ejection fraction increased by 2% from rest to exercise, as opposed to the expected > 5% increase in a healthy population. The pulmonary left ventricular ejection fraction decreased by 2% at peak exercise.
CONCLUSION—Diminished values of heart rate, forced expiratory volume in one second (FEV₁), forced vital capacity, and systolic blood pressure compared to the predicted values may contribute to the reduced maximal oxygen uptake (VO₂max) found in patients with CCTGA. In addition, a limited increase in systolic right ventricular ejection fraction and a decrease in pulmonary left ventricle contractility suggest a dysfunction of both ventricles.


Keywords: aerobic capacity; ventricular dysfunction; lung function; congenitally corrected transposition of the great arteries     

Hypertrophic cardiomyopathy in the systemic right ventricle in a patient with congenitally corrected transposition of the great arteries: A case report

Congenitally corrected transposition of the great arteries is a rare clinical entity, which usually presents during adulthood with associated defects; atrioventricular block, heart failure, systemic valve failure, and arrhythmias usually complicate the clinical course. Even rarer is associated hypertrophic cardiomyopathy, which complicates the disease course and clinical decision-making. Herein, we present a patient with this condition who underwent heart transplantation, with adequate clinical resolution.     

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.     

Adverse impact of chronic subpulmonary left ventricular pacing on systemic right ventricular function in patients with congenitally corrected transposition of the great arteries

Background

Patients with congenitally corrected transposition of the great arteries (ccTGA) are at high risk of heart block requiring subpulmonary left ventricular (LV) pacing. Long-term right ventricular (RV) pacing in congenitally normal hearts is associated with LV dysfunction. We examined the effects of univentricular subpulmonary LV pacing on the systemic RV in a ccTGA cohort.

Methods

ccTGA patients with two echocardiographic studies at least 6 months apart were included. Records of 52 patients, 22 with pacing, were retrospectively reviewed. Seven patients with biventricular pacing were included for comparison.

Results

The LV-Paced Group experienced deterioration in the RV fractional area change (RVFAC) (28.7 ± 10.0 vs. 21.9 ± 9.1%; P = 0.003), systemic atrioventricular valve regurgitation (P = 0.019) and RV dilatation (end-diastolic area 32.7 ± 8.7 vs. 37.2 ± 9.0 cm²; P = 0.004). There was a corresponding deterioration in NYHA class (P = 0.013). Multivariate Cox regression analysis showed that pacing was an independent predictor of deteriorating RV function and RV dilation (hazard ratio 2.7(10–7.0) and 4.7(1.1–20.6) respectively). None of these parameters changed significantly in the Un-paced Group. The CRT Group showed improvement in RVFAC (22.0% to 30.7% (P = 0.030) and NYHA class (P = 0.030), despite having lower baseline RVFAC (22.0 ± 5.7 vs. 31 ± 9.7%; P = 0.025) and greater dyssynchrony (RV total isovolumic time 13.4 ± 2.1 vs. 9.3 ± 4.2 s/min; P = 0.016) when compared to the Un-Paced Group.

Conclusions

Univentricular subpulmonary LV pacing in patients with ccTGA predicted deterioration in RV function and RV dilatation over time associated with deteriorating NYHA class. Alternative primary pacing strategies such as biventricular pacing may need consideration in this vulnerable group already highly prone to mortality from systemic RV failure.     

From tricuspid to double orifice Morphology: Percutaneous tricuspid regurgitation repair with the MitraClip device in congenitally corrected‐transposition of great arteries

Edge to edge transcatheter mitral valve repair with MitraClip (Abbott Vascular, Menlo Park, CA) is increasing for high‐risk surgical patients with significant mitral regurgitation. Patients with congenitally corrected transposition of the great arteries (CCTGA) presenting with tricuspid valve regurgitation of a systemic right ventricle may represent particularly challenging candidates for MitraClip given their anatomy. We report the case of a 67‐year‐old gentleman with CCTGA and severe tricuspid regurgitation who was referred for MitraClip implantation after heart team consensus. Successful implantation of one clip was performed, achieving a significant reduction of the regurgitation. Similarly, favorable findings were confirmed at 6 months, 1 and 2 years follow‐up and the patient had no recurrent heart failure admissions after 2‐year follow‐up. We describe the technical considerations and the importance of 3D‐transoesophageal echocardiography for performing the MitraClip of a trileaflet systemic atrioventricular valve. © 2016 Wiley Periodicals, Inc.     

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     

Triplane tissue Doppler imaging to evaluate mechanical dyssynchrony before and after cardiac resynchronization in a patient with congenitally corrected transposition of the great arteries

We report the case of a 13-year-old girl with congenitally corrected transposition of the great arteries. Since the implantation of a conventional pacemaker for acquired complete atrioventricular block, the patient experienced increased heart failure symptoms. Using triplane tissue Doppler imaging, significant intraventricular dyssynchrony induced by unilateral pacing and associated with diminished exercise capacity was demonstrated. A biventricular pacemaker was successfully implanted transvenously, leading to synchronous activation of the systemic ventricle and improved exercise capacity.     

先天性矫正型大动脉转位行形态三尖瓣置换术

目的 探讨先天性矫正型大动脉转位（C CTGA）患者行形态三尖瓣置换的手术适应证及术后效果.方法 2007年至2012年河南省胸科医院收治CCTGA患者6例,男性5例,女性1例,年龄28～47（34.0±5.2）岁,术前心功能Ⅱ级1例、Ⅲ级5例（NYHA分级）,术前合并室间隔缺损1例、房颤1例、完全性左束支传导阻滞1例、完全性右束支传导阻滞1例、Ⅰ°房室传导阻滞2例.术前形态右心室射血分数（49.0±13.2）％.6例患者均施行形态三尖瓣置换术,术后随访患者心功能、形态右心室射血分数等指标.结果 全组无住院死亡.随访13～72个月,1例患者术后41个月因主动脉夹层死亡,余5例均存活.人工瓣膜功能正常,心功能较术前明显改善,术后1年时心功能Ⅰ级2例、Ⅱ级3例、Ⅲ级1例（NYHA分级）,与术前比较差异有统计学意义（P＜0.05）.术后1年时形态右心室射血分数（48.0±5.6）％,与术前比较差异无统计学意义（P＞0.05）.结论 CCTGA患者行形态三尖瓣置换手术效果满意,能够防止形态右心室功能的进一步损害.     

Deleterious effect of unintentional pacing from the middle cardiac vein in a patient with congenitally corrected transposition of the great arteries and ventricular septal defect. The beneficial effect of cardiac resynchronization therapy after heart surgery

This report presents a case of a 31-year-old male with congenitally corrected transposition of the great arteries, ventricular septal defect (VSD) and complete heart block who was admitted to our institution because of an exacerbation of heart failure after pacemaker implantation. The ECG and chest radiograph revealed that the ventricular lead was placed in the middle cardiac vein. After cardiosurgical procedure (VSD closure and atrioventricular valves replacement), the clinical symptoms of heart failure were still present. They diminished while the patient was on escape rhythm (40–70 bpm). Therefore, the previously implanted leads were removed and the transvenous, biventricular system was implanted, which resulted in a significant clinical improvement.     

Long term ventricular performance after intra-atrial correction of transposition: left ventricular filling is the major limitation

Objective—To establish the incidence of systolic and diastolic dysfunction of the right and left ventricle in a large cohort of patients after Mustard or Senning operations and to assess changes in the incidence on long term follow up.
Design—Postoperative case-control study using radionuclide ventriculography. Ejection fractions, peak filling rates, rapid filling periods and fractions, slow filling periods and fractions, and atrial contraction periods and fractions were studied.
Setting—Tertiary care centre, ambulatory and hospital inpatient care.
Patients—A convenience sample of 153 patients studied at median age of 6.9 years (median 4.4 years after surgery). In 99 cases another study was available at a median age of 15.3 years (median 13 years after surgery and 8.8 years after the first study).
Results—Respective incidences of dysfunction in the first and the second study were as follows: ejection fraction-right ventricle 7.8% and 8.1%, left ventricle 7.2% and 10.1%; peak filling rate-right ventricle 0% and 4.2%, left ventricle 14.3% and 29.5% (p < 0.05); rapid filling period- right ventricle 18.3% and 11.6%, left ventricle 30.2% and 30.5%; slow filling period—right ventricle 4.8% and 3.2%; left ventricle 11.9% and 23.2%; atrial contraction period-right ventricle 0.8% and 4.2%, left ventricle 15.1% and 26.3%; rapid filling fraction-right ventricle both 0%, left ventricle 82.5% and 79.0%; slow filling fraction-right ventricle 0.8% and 4.2%, left ventricle 37.3% and 30.5%; atrial contraction fraction-right ventricle both 0%, left ventricle 79.4% and 71.6%.
Conclusions—The incidence of systolic ventricular dysfunction is 8% (right ventricle) and 10% (left ventricle) 13 years after surgery, without a significant increase over the eight year follow up. Diastolic filling is abnormal in up to 80% of patients and left ventricular peak filling rate deteriorates with time.

Keywords: congenital heart defects; transposition of the great arteries; radionuclide ventriculography; ventricular function     

Severe subvalvular pulmonic stenosis due to a ball‐shaped accessory tissue in an asymptomatic woman with congenitally corrected transposition of great arteries and mesocardia: A rare case report

Congenitally corrected transposition of great arteries (CCTGA) is a rare anomaly described by atrioventricular and ventriculoarterial discordance. On the other hand, mesocardia that is extremely rare includes two relatively well‐defined apexes defined by each ventricle with the major axis of the heart lies in the midline. We describe a rare case of an asymptomatic 20‐year‐old woman with mesocardia, CCTGA, and severe subvalvular pulmonic stenosis due to a ball‐shaped accessory tissue in left ventricular outflow tract.