左室训练术对矫正性大动脉转位合并三尖瓣关闭不全的影响

目的左室退化先天性矫正性大动脉转位患者常常合并不同程度的三尖瓣关闭不全,左室训练术可有效的提高形态学左心室的后负荷,改善形态学左心室的功能,本文进一步评价左室训练术对三尖瓣功能的可能影响。方法2005年5月至2011年5月,24例左室退化先天性矫正性大动脉转位患儿行左室训练术,男性13例,女性11例,年龄3．73±4．35岁（0．17-22岁）,体重15．71±10．95kg（5．1—61kg）,其中合并三尖瓣关闭不全23例（轻度11例,中度7例,重度5例）。术前经超声心动网,心血管造影或心导管检查确诊,形态学左室舒张末径21．56±6．60mm（8-32mm）,形态学左室后壁厚度4．29±1．52mm（2-7mm）,形态学左室与形态学右室压力比0．41±0．12（0．12-0．65）。手术均采用胸骨上端小切口或胸骨正中切口,在全麻下完成左室训练术。结果全组患者无住院死亡．出院时超声心动图检查：5例患者三尖瓣关闭不全消失,三尖瓣仍存在不同程度关闭不全18例（轻度12例,中度4例,重度2例）,其中：9例患者TR程度均较术前减轻,双心室结构和功能良好,室间隔位置较术前略向形态学右心室侧移位,形态学左室舒张末径较术前略增大。术后随访1月-35月,无远期死亡。所有患儿一般情况好,生命体征平稳,心功能I—II级,8例患者三尖瓣关闭不全消失,三尖瓣存在不同程度关闭不全14例（轻度8例,中度4例,重度2例）,其中：8例患者TR程度均较术前显著减轻,形态学左室舒张末径26．17±7．11mm（14—40mm）,形态学左心室后壁厚度4．95±1．44mm（4—9mm）。结论左室训练术可改善左室退化先天性矫正性大动脉转位患者三尖瓣关闭不全的程度,其原因可能与左室训练术增加形态学左心室压力负荷及增加形态学左心室后壁厚度,形态学左心室腔扩大,室间隔向形态学右心室侧移位,形态学右心室腔相对缩小,三尖瓣叶对合改善,三尖瓣反流减轻。     

Perventricular Closure of a Large Ventricular Septal Defect in Congenitally Corrected Transposition of the Great Arteries

We report the case of a 30 year-old male with congenitally corrected transposition of the great arteries, atrial, and ventricular septal defects (VSD), and pulmonary stenosis. He previously underwent three palliative surgical procedures before undergoing intracardiac repair at age 20 with a left ventricular to pulmonary artery (LV-PA) conduit, VSD closure, and replacement of the systemic atrioventricular valve. A residual VSD was noted postoperatively. He did well for approximately 10 years when he started becoming more breathless with daily activities and was noted to have a resting room air oxygen saturation of 85%. Despite increased diuretic therapy he continued to deteriorate and was ultimately admitted to the hospital in florid right and left heart failure with recurrent atrial fibrillation. Catheterization revealed pulmonary hypertension (pulmonary artery pressure = 80/17 mm Hg), moderate conduit stenosis, severe pulmonic regurgitation, and oxygen saturation of 75%. Calculated shunt fraction (Qp : Qs) was 1.3:1. He was referred for surgical intervention, specifically, LV-PA conduit replacement, oversewing of the pulmonic valve, VSD closure, and pacemaker placement. Intraoperatively, the VSD could not be closed despite multiple attempts through various approaches. Therefore, perventricular VSD closure using two Amplatzer septal occluders (AGA Medical, Golden Valley, MN) was performed in the operating room with the chest open off cardiopulmonary bypass. Following deployment, the residual shunt was small and the inferior vena cava-to-pulmonary artery saturation step-up was only 4%. The left ventricular systolic pressure decreased to one half systemic. This case highlights the utility and efficacy of a hybrid approach in the treatment of complex congenital heart disease.     

Anomalous Intramural Course of Coronary Arteries in Congenital Heart Disease—Three Case Reports and Review of the Literature

Anomalies of the coronary arteries are commonly associated with congenital heart disease (CHD). Anomalies include variations in number, shape, and location of the origin, as well as the course of the coronary artery. An intramural course of one of the coronary arteries is a rather rare condition. Most cases of anomalies are of no clinical relevance and are found incidentally during evaluation for other cardiac conditions. However, they become of importance when cardiac surgery/intervention, especially valve replacement, needs to be performed. We conducted a literature review for coronary anomalies with an intramural course in patients with CHD and include three cases from our own clinic. Note that transposition of the great arteries seems to be the condition most frequently associated with an intramural course of one of the coronary arteries. In case of a suspected anomaly, transesophageal echocardiography presents a valuable tool to visualize the origin and course of the coronary artery, as well as the relationship to important anatomical structures like the aortic or pulmonary valve. Alternatively, cardiac magnetic resonance imaging can be used. Although rare, both cardiologists and surgeons need to be aware of intramural courses of coronary arteries to prevent accidental trauma to them and thereby, reducing the risk for the patient.     

Dynamic Pulmonary Artery Obstruction Causing Right Ventricular Failure after Cardiac Transplantation in a Patient with Mustard–Senning Corrected d‐Transposition of the Great Arteries

Patients with congenital d‐transposition of the great arteries (d‐TGA) undergoing palliative atrial baffle surgery in infancy often develop systemic ventricular failure in adulthood. If they undergo cardiac transplantation, they are prone to morphologic right ventricular (RV) failure secondary to severe pulmonary hypertension as a result of systemic ventricular failure. We report a case of a patient with d‐TGA and biventricular ventricular failure requiring heart transplantation (HT) that developed RV failure postoperatively because of dynamic pulmonary artery (PA) obstruction at the anastomotic site of PA. Obstruction at the site of PA anastomosis due to torsion or redundancy of the donor or recipient PA is a rare but treatable cause of postoperative RV failure. In this case, rapid identification of the etiology of RV failure and implementation of corrective therapies before the development of end‐organ dysfunction, resulted in complete RV recovery and normal allograft function. This case represents the first known report of dynamic PA anastomoticobstruction resulting in RV failure after HT that was corrected with pulmonary arterioplasty, and RV assist device resulting in complete recovery.     

Two Challenging Translocation Procedures for Intramural Coronary Arteries in the Setting of Transposition of Great Arteries

Translocation of the coronary arteries remains a technical challenge in anatomic correction of transposition of great arteries. Myocardial ischemia related to the difficulties with coronary relocation is an important factor in perioperative and postoperative morbidity and mortality, particularly in the patients with complex coronary artery anatomy. Intramural coronary artery is a rare anatomic variety which may complicate the arterial switch operation in 2% to 4.6% of the reported cases. Even in the hands of experts, the mortality rate may be in twofold in this subset of patients compared with simple transposition of great artery procedures. In this report, 2 successful translocation techniques for intramural coronary arteries in the setting of arterial switch operation are described.     

Detection of a Coronary Artery Anomaly after a Sudden Cardiac Arrest in a 17 Year‐old with D‐transposition of the Great Arteries Status Post Arterial Switch Operation: A Case Report

Long‐term follow‐up and testing for patients who have undergone congenital heart surgery is an evolving field. We report the case of a 17‐year‐old patient who had an arterial switch operation as an infant for d‐transposition of the great vessels and suffered sudden cardiac arrest while participating in a cross‐country event. Previous evaluations, including a cardiac catheterization and stress testing, did not indicate any identifiable abnormalities. After the arrest, a computed tomography angiogram of his chest showed an abnormal takeoff of the reimplanted left coronary artery.     

Flail Tricuspid Valve in an Adult Patient with Congenitally Corrected Transposition of the Great Arteries

We describe a case of a 50-year-old woman with congenitally corrected transposition of the great vessels, in whom severe left-sided tricuspid (systemic atrioventricular) valve insufficiency was the only associated anomaly. The tricuspid valve was dysplastic and abnormally oriented toward the interventricular septum, without the downward displacement of Ebstein's anomaly. The mechanism of atrioventricular regurgitation was unusual in that it consisted of the rupture of chordae tendineae of both the anterior and septal leaflets. The left-sided tricuspid valve was replaced with a St. Jude prosthesis and the postoperative course was uneventful.     

Ventricular Assist Device Placement in an Adult with D-transposition of the Great Arteries with Prior Mustard Operation

Adults with congenital heart defects and congestive heart failure are a challenging population because of their complex anatomy, prior surgical palliation, and hemodynamic status. We report one of the first cases of a ventricular assist device placement in a patient with decompensated heart failure and a history of D-transposition of the great vessels who had a prior atrial switch operation.     

Unrepaired Tetralogy of Fallot with Major Aortopulmonary Collateral Arteries in an Adult Patient

Tetralogy of Fallot is characterized by a ventricular septal defect, a large, overriding aorta, subpulmonic stenosis, and right ventricular hypertrophy. These lesions can be associated with abnormal development of the pulmonary vasculature. This can include peripheral pulmonic stenosis, discontinuous pulmonary arteries, anomalous pulmonary venous return, and the development of aortopulmonary collateral vessels. Aortopulmonary collateral vessels develop to supply underperfused areas of the pulmonary bed and pose a unique and challenging problem at the time of surgical repair, which involves closure of the ventricular septal defect, relief of right ventricular outflow tract obstruction, maintenance of pulmonary valve competency when possible, and establishment of laminar pulmonary blood flow to all segments of the pulmonary bed. We describe a 36‐year‐old man with unrepaired tetralogy of Fallot with distinctive aortopulmonary collaterals, who underwent complete surgical repair with good outcome. Two‐dimensional echocardiogram, cardiac magnetic resonance imaging, and cardiac catheterization each provided vital details allowing a stepwise approach to defining his unique anatomy for surgical correction.     

儿童矫正性大动脉转位伴Ⅲ度房室阻滞的VDD生理性起搏(附一例报告)

一例１１岁矫正性大动脉转位（ＳＬＬ）的女性患儿有晕厥史１０年，超声心动图证实心内无分流，动态心电图及电生理检查均证实为Ⅲ度房室阻滞，窦房结功能正常。安置ＶＤＤ生理性起搏器，感知与起搏电极间距为１３ｃｍ，Ｐ波振幅２．２ｍＶ、心房感知０．７５ｍＶ、心室起搏阈值０．４Ｖ。术后观察心房感知率９８％。给心内无分流的心脏畸形患儿安置起搏器要充分考虑生理和生长发育的需要，如合理选择起搏器类型和电极长度，进行电生理检查测定心尖至高位右房的距离，同时熟悉复杂心脏畸形影像学的知识对保证安置术的成功十分必要。     

Long‐term Follow‐up of “Simple” Lesions—Atrial Septal Defect,Ventricular Septal Defect,and Coarctation of the Aorta

Surgery for congenital heart disease has advanced significantly in the past 50 years, such that repair of “simple” lesions, such as atrial septal defect, ventricular septal defect, and coarctation of the aorta carries minimal risk, with mortality risk much less than 1%. It was once thought successful repair of these lesions was definitively corrective. There is mounting evidence, however, that there are long‐term complications after these repairs, prompting the need for continued follow‐up. This review describes the current understanding of diagnosis, treatment, and long‐term outcomes for these patients, with the goal of advocating for lifelong surveillance. As the perioperative care of these repairs has evolved significantly over time, so must the way in which we study these patients in the long‐term.     

Congenital Left Ventricular Splint in an Adult Patient with Unrepaired Anomalous Left Coronary Artery from the Pulmonary Artery

A 24‐year‐old woman presented with a recent increase in dyspnea on exertion and development of presyncope. The patient stated that she has reproducible episodes of dizziness and near fainting when she climbs a flight of stairs and activity is limited to a slow gait.     

Effect of Prostaglandin Duration on Outcomes in Transposition of the Great Arteries with Intact Ventricular Septum

Objective. To study the effects of duration of preoperative prostaglandin E1 (PGE) exposure on perioperative outcomes of the arterial switch operation in patients with transposition of the great arteries with an intact ventricular septum. Design. Retrospective chart review. Setting. Pediatric cardiac intensive care unit in a tertiary care children's hospital. Patients. All patients with transposition of the great arteries with an intact ventricular septum from 1995 to 2008. Outcome Measures. Inotropic score was calculated for all patients in the first 5 postoperative days and maximum inotropic score was recorded. Length of postoperative mechanical ventilation, fluid balance, mechanical ventilation time, as well as intensive care unit and hospital stay were recorded for all patients. Results. Study population included 59 patients, 41 (69%) underwent balloon atrial septostomy. PGE was used in 52 patients, median exposure of 59 hours, range 0 to 272 hours. Longer preoperative PGE exposure was associated with longer preoperative mechanical ventilation (P < .001). There was no association between preoperative PGE duration and cardiopulmonary bypass time, cross‐clamp time, or total hospital stay. Patients with longer preoperative PGE exposure had a lower postoperative inotrope score (10 vs. 15 P = .02). Conclusion. Greater preoperative PGE exposure was associated with prolonged preoperative mechanical ventilation. Longer PGE exposure was associated with lower postoperative inotrope requirements. Aggressive efforts to avoid or shorten PGE infusion duration may not be warranted in this population.     

Cryoablation of the Slow Atrioventricular Nodal Pathway via a Transbaffle Approach in a Patient with the Mustard Procedure for d‐Transposition of the Great Arteries

The ablation of atrioventricular (AV) nodal reentrant tachycardia in patients with the Senning or Mustard procedure is quite challenging because these atrial baffle procedures isolate the AV node from systemic venous access. Cryoablation is commonly utilized for AV nodal slow pathway modification in patients with structurally normal hearts. The cryoablation technique offers the advantage of monitoring AV nodal conduction during lesions, with the ability to terminate a lesion prior to permanent injury to the AV node. This case describes the successful cryoablation of the slow AV nodal pathway via the transbaffle approach in a patient with d‐transposition of the great arteries status post the Mustard procedure.     

Atrial Baffle Problems Following the Mustard Operation in Children and Young Adults with Dextro‐Transposition of the Great Arteries: The Need for Improved Clinical Detection in the Current Era

Objective. Intraatrial Mustard baffle repair of dextro‐transposition of the great arteries (d‐TGA) is vulnerable to complications, typically obstruction and leaks. Because patients often require pacemakers or intracardiac electrophysiology studies (EPS)/ablation for arrhythmias, narrowed or obstructed baffles restrict cardiac access hindering intracardiac procedures. Current guidelines recommend clinical as well as comprehensive transthoracic echocardiographic/Doppler (TTE) studies to identify baffle problems. This study reviews the effectiveness of these guidelines in detection of baffle issues pre‐EPS catheterization and need for ancillary vascular interventions. Design. Data from all patients with repaired d‐TGA referred for hemodynamic catheterization or EPS between 1995 and 2009 at our institution were reviewed, including symptoms and TTE findings. Obstruction was defined as either a disturbed color Doppler flow or mean velocity >1 m/s above the mitral valve or directly measured pressure gradient >4 mm Hg or more than 50% baffle diameter narrowing by venography. Results. Of 59 patients (34 pacemaker, 9 ablation, 16 routine hemodynamic) ages 8–39 years (mean 22.8), only three (5%) had symptoms of obstruction. However, baffle complications were found in 33 patients (56%), some with more than one problem: superior vena cava (SVC) obstruction in 32, inferior VC in two and leak in four. Baffle stenting was required in 24 patients and leak closure in two. Precatheterization TTE was available in 51 patients and showed 34% sensitivity, 61% specificity, 63% negative predictive value, and only 37% positive predictive value in recognizing baffle complications when compared with the actual catheterization findings. Conclusion. This study reports that baffle complications in patients with d‐TGA following Mustard operation are more common than previously reported. However, comprehensive TTE and clinical symptoms are not effective enough to recognize these complications. Suspicion of and better noninvasive imaging prior to catheterization is required.     

Near‐fatal Outcome of Late Cardiac Perforation by a Pacemaker Lead in a Young Woman with Transposition of the Great Arteries

We report a case of ventricular perforation by an active‐fixation permanent pacemaker lead in a young woman with congenital heart defect and pacemaker because of atrioventricular block. The rarity of the case is based not only upon the late perforation (as opposed to the more common acute perforations) but also on the near‐fatal outcome. The patient experienced sharp chest pain and 1 week later passed out with cardiac arrest.