Two Diverticula of the Left Ventricular Outflow Tract Adjacent to the Commissures of a Bicuspid Aortic Valve

We report a rare combination of congenital cardiac malformations in an asymptomatic adult—a bicuspid aortic valve and double fibrous diverticula of the left ventricle. We describe the presentation and course of events followed by a brief discussion of ventricular diverticula and the management of this rare combination of anomalies.     

房室间隔缺损患儿血浆心钠素与心脏指数、钠排泄关系的探讨

对15例房间隔缺损,19例室间隔缺损儿童进行了血浆心钠素(ANP),心脏指数(CI)和钠排泄分数(FENa％)相关性的研究。结果表明:34例先天性心脏病患儿血浆ANP水平明显高于正常对照组(217.23+83.04 VS 17.51±6.55)P<0.01。CI则明显低于正常对照组(3.32±0.35 VS 4.06±0.33)P<0.05。FENa％与正常对照组比较无明显差别(0.37±0.06 VS 0.38±0.07)P>0.05;34例患儿血浆ANP水平与CI呈负相关(r=-0.75,P<0.05)。提示:房室间隔缺损患儿血浆ANP水平可用来估价该类患儿的心功能。本组患儿血浆ANP水平升高同时FENa％正常,这一现象可能与机体ANP受体的再调节有关。     

Multimodality detection of multiple left ventricular diverticula: A case report and brief review of the literature

Left ventricular diverticula (LVD) are rare congenital anomalies usually detected incidentally in the adult population. Most commonly, they are found as a single left ventricular diverticulum in association with other congenital abnormalities but multiple LVD are exceedingly rare. We are describing a patient who was found to have multiple LVD on multimodality imaging studies. He had presented with a sudden cardiac arrest attributed to a combination of alcohol intoxication and QT interval prolongation from hypokalemia and antidepressant medications. The patient was managed conservatively and discharged with an implantable loop recorder for detecting any occult arrhythmias.     

中国内地先天性室壁瘤和憩室临床特征文献的统计分析

目的 分析中国内地报道的先天性室壁瘤和憩室的临床特征。方法 通过检索万方数据库、中国知网全文数据库及PubMed数据库,获取2001年1月至2009年12月中国内地报道的先天性室壁瘤和憩室的文献资料,并对其临床特征进行分析。结果 共检索到符合要求的文献109篇,患者共116例,年龄l～80(33.5±21.3)岁,男性78例。先天性室壁瘤25例,其中包括一个家系的患者4例,男性13例;先天性憩室91例,男性65例。100例患者因体检或胸闷不适通过超声心动图检查出先天性室壁瘤和憩室。合并心内先天性缺陷34例,其中同时合并心外先天性缺陷4例。合并室性心律失常8例,血栓形成8例,心脏破裂2例,猝死4例（包括家系患者3例）。外科治疗46例,射频消融治疗3例,所有患者均未置人埋藏式心律转复除颤器。结论 先天性室壁瘤和憩室是一种罕见的先天性疾病,无症状患者可通过超声心动图检查发现。患者可能出现室性心律失常、血栓栓塞、室壁瘤或憩室破裂、猝死等病情,需要积极干预。     

Left ventricular diverticulosis: demonstration on cardiac CT

Cardiac computed tomography scanning is rapidly emerging as the noninvasive modality of choice for assessment of coronary artery disease. Secondary to the exquisite resolution of the modality, left ventricular diverticula can be well demonstrated. Left ventricular diverticula are rare, and in the past, the terms diverticula and aneurysm have been used interchangeably. The differentiation of diverticula and aneurysm is crucial for appropriate management. To the best of our knowledge, this is the first report of left ventricular diverticula seen on cardiac computed tomography examination.     

Congenital diverticulum of the right ventricle associated with coarctation of aorta, atrial and ventricular septal defect and ductus.

Congenital right ventricular muscular diverticula are extremely rare and are usually associated with other congenital cardiac anomalies, (in half of the cases tetralogy of Fallot). They functionally behave like an accessory ventricular chamber which contracts synchronously with the normal ventricles.Less than 30 patients with a right ventricular diverticulum have been reported in literature. An apical right ventricular diverticulum occurs in patients with thoraco-abdominal midline defects or abnormalities of the cardiac position([1]). However, an antero-superior diverticulum is usually associated with other congenital cardiac defects, such as a ventricular septal defect, tetralogy of Fallot, double outlet right ventricle and pulmonary stenosis([2--9]).We report an 11-year-old boy with an antero-superior diverticulum of the right ventricle associated with a coarctation of aorta, ductus arteriosus, and atrial and ventricular septum defects. To the best of our knowledge, such an association has not been reported before.     

Percutaneous Closure of Atrial Septal Defects in Patients with an Aberrant Retroaortic Coronary Artery: Is It Safe?

Coexistence of an ostium secundum type atrial septal defect and a coronary artery anomaly with an aberrant retroaortic course is a rare congenital anomaly that may potentially complicate percutaneous atrial septal defects (ASD) closure. If the anterosuperior rim of the defect is deficient, the abnormally located coronary artery may be compressed or distorted by the implanted device causing myocardial ischemia, arrhythmias, and eventually sudden cardiac death. Due to the potential occurrence of these fatal cardiac events, diagnosis of an aberrant coronary artery with a retroaortic course must be established before percutaneous ASD closure. In this report, two patients with this rare association are described in whom percutaneous closure of the defect was feasible and uneventful. The importance of a careful periprocedural, noninvasive echocardiographic coronary artery imaging is emphasized, and the rational for percutaneous atrial septal defect closure in this unusual anatomic arrangement is proposed.     

Long-Term Follow Up of Secundum Atrial Septal Defect Closure with the Amplatzer Septal Occluder

Background. During the past 15 years, closure of a secundum atrial septal defect (ASD) has moved from a surgical to a percutaneous transcatheter approach. Few long-term studies of the efficacy and safety of closure of an ASD by an Amplatzer septal occluder (ASO) exist. Methods. To examine the long-term results of secundum ASD closure using the ASO, data on 94 patients who underwent secundum ASD closure with the ASO between 1998 and 2002 were available and reviewed. Data regarding residual shunt, chest pain, palpitations, arrhythmias, headaches, transient ischemic attacks, cerebrovascular accidents, and mortality were collected. Results. Seven (7.4%) subjects had residual shunts immediately following ASO placement. During follow-up, 4 residual shunts closed for a complete closure rate of 97%. Eighteen (19%) patients reported chest pain during the follow-up period. Twenty-three patients (24%) reported palpitations during the follow up period, 7 were documented arrhythmias, including supraventricular tachycardia, atrial fibrillation, and premature ventricular beats. Migraine headaches were new-onset in 4 patients. Migraine cessation occurred in 2 patients after secundum ASD closure. One child died from a cerebral vascular event 18 months following device placement. Only 1 patient developed mild aortic insufficiency. Conclusion. These data indicate that for up to 120 months of patient follow-up, the ASO continues to be a safe device. Residual shunts and arrhythmias have low incidence post-ASO placement. Given the mortality in one high-risk patient, further investigation into anti-platelet therapy after device placement is warranted.     

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.     

先天性心室憩室的研究进展

先天性心室憩室是一种少见的心脏畸形。大部分为左室憩室,右室憩室较少,也可发生在两个心室。心室憩室常伴有其他的异常。先天性心室憩室多见于婴幼儿和儿童,但亦可见于各年龄组人群。现对先天性心室憩室的研究进展做一综述。     

Congenital Right Pulmonary Artery Agenesis with Atrial Septal Defect and Pulmonary Hypertension

Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7‐year‐old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.     

Atrial Septal Stent Implant: Atrial Septal Defect Creation in the Management of Complex Congenital Heart Defects in Infants

Objectives. Certain congenital heart defects require the creation of an unrestrictive atrial septal defect (ASD) to relieve atrial hypertension, to maintain systemic cardiac output, or to achieve adequate atrial mixing to improve systemic oxygen saturation. We describe a series of patients, ranging in age from 5 weeks to 17 months, in whom we implanted a stent across the atrial septum to create a lasting, unrestrictive interatrial communication. Methods. Five patients with left heart obstructive defects presented with severe left atrial hypertension. The patients weighed 3.5–10.1 kg. Patients 1 and 2 presented at 5 and 9 weeks of age, respectively. Patient 1 had a restrictive patent foramen ovale, and patient 2 had an intact atrial septum. Patients 3, 4, and 5 had previous cardiac surgery including atrial septectomy. At presentation, all patients were poor surgical candidates. Results. Using standard percutaneous technique, a premounted Palmaz–Genesis stent (Cordis Corporation) was implanted across the atrial septum in all 5 patients, using balloons ranging from 7 to 10 mm diameter. Each patient had a dramatic decrease in left atrial pressure and increased ASD diameter. All stented ASDs remained widely patent, confirmed by Doppler echocardiography, until elective surgical stent explant. Conclusion. Patients presenting beyond the neonatal period with left heart obstructive defects and intact atrial septum or restrictive ASD present a challenging problem. We report a safe and effective technique for relief of left atrial hypertension in infants using premounted intravascular stents. All patients had immediate marked hemodynamic improvement.     

Congenitally Corrected Transposition of the Great Arteries and Concomitant Coronary Artery and Valvular Disease in the Adult Patient

Congenitally corrected transposition of the great arteries (ccTGA) accounts for less that 1% of cardiac anomalies, and is defined as ventriculoarterial and atrioventricular (AV) discordance. The double discordant connection allows for survival with the right ventricle performing as the systemic ventricle, and the left ventricle as the pulmonary ventricle. We report a case of ccTGA in a 35-year-old male with situs inversus totalis status post repair of a ventricular septal defect (VSD) with a residual VSD, severe systemic AV valve regurgitation, and coronary artery disease who presented with chest pain. He subsequently underwent tricuspid valve replacement and VSD repair, followed by percutaneous coronary revascularization. This case highlights many important issues of adults with congenital cardiac disease, as well as the specific surgical management of anomalies associated with ccTGA. We review the literature and discuss the management of these complicated patients.     

Congenital left ventricular aneurysms and diverticula. Two case reports

The authors report the cases of two young adults (25 and 27 years) presenting with congenital left ventricular aneurysm or diverticulum with healthy coronary arteries. This saccular evagination of the ventricular wall is rare. The authors describe a classification distinguishing muscular (contractile) diverticula, composed of the three cardiac tunics, fibrous diverticula and finally aneurysms with a dyskinetic wall. Although the limits of this classification are sometimes poorly defined, it presents a prognostic value, because it appears logical to propose nonsurgical management in the context of muscular diverticula and it seems legitimate to operate on fibrous diverticula and aneurysms due to the risks of rupture, extension, thrombosis or arrhythmia.     

Morphologic,Mechanical, Conductive,and Hemodynamic Changes Following Transcatheter Closure of Atrial Septal Defect

Objective. The secundum type of atrial septal defect (ASD) is a common congenital cardiac anomaly. Transcatheter closure of the defect is the preferable therapeutic approach to avoid potential future complications. This work extends the data collected so far regarding the electrocardiographic, morphologic, hemodynamic, and mechanical changes following the procedure. Design. From April 2002 to October 2003, 42 consecutive patients underwent successful transcatheter closure of secundum type ASD. They were sampled for different parameters by transthoracic echocardiography before the procedure and at three different points over the next 4 years, and analyzed in two age groups: children and adults. Results. The dimensions of the chambers normalized throughout the study period. Abnormal movement of the interventricular septum normalized mostly during the first month, but its thickness increased gradually to normal levels during the 4 years of the study. High elevated pulmonary artery pressure values began declining to near normal levels from the second month following the procedure. The stroke volume and the right bundle branch block pattern improved throughout the study period. Conclusions. Transcatheter ASD closure is followed by morphologic, conductive, and hemodynamic changes at different time intervals during the first years and results in normalization or near normalization of the heart's structure and function.     

Atrioventricular Septal Defect Recently Diagnosed by Fetal Echocardiography: Echocardiographic Features,Associated Anomalies,and Outcomes

Objectives. We report our recent experience with atrioventricular septal defect (AVSD) diagnosed in utero. Methods. We reviewed fetal echocardiograms diagnosed with AVSD between November 2002 and November 2004, comparing fetuses with and without aneuploidy. We compared results with previous studies. Results. Twenty (1.8%) fetuses had AVSD. Mean maternal age was 33 years (range 19–43). Mean gestational age was 26 weeks (range 18–38). Indications for fetal echocardiography were: abnormal obstetrical ultrasound (75%), chromosomal anomaly (15%), undetermined (10%). AVSD was an isolated cardiac defect in 5 (25%), associated with double‐outlet right ventricle (9) or tetralogy of Fallot (3) in 12 (60%). Four had aortic arch anomalies. Atrioventricular valve regurgitation was mild in 7 (35%) and moderate in 4 (20%). Heart block existed in 2 (10%). Five (25%) with trisomy had Rastelli type A AVSD as a single lesion (odds ratio 24, P < .01). Extracardiac anomalies existed in 6, with and without aneuploidy. Pregnancy was terminated in 4 (20%), neonatal death in 4 (20%), and reparative surgery in 6 (30%), not ascertained in 6. Conclusion. Atrioventricular septal defect is usually an isolated cardiac lesion in fetuses with aneuploidy. In the absence of aneuploidy, fetal AVSD is often associated with conotruncal and aortic arch abnormalities, which are important in determining outcomes. Pregnancy termination and neonatal death continue to be prevalent.     

Large aneurysm of the left heart, an unusual cause of cardiomegaly in children

Idiopathic left ventricular aneurysms are rare in childhood as are the congenital epigastric muscular diverticula which form part of a complex congenital malformation. The aneurysms are usually symptomatic and present either with cardiac failure or another complication. The authors report a very rare case in which the aneurysm effectively realised a double chamber left ventricle. The aneurysm was larger than the true ventricle, muscular, lined by normal endocardium and covered by coronary vessels. Surgical ablation was indicated for cardiac failure. Two similar cases have been previously reported.     

超声心动图评价室间隔缺损封堵术后左心功能及形态的变化

目的 探讨超声心动图对室间隔缺损封堵术后左心功能及形态变化的评价作用.方法 60例接受Amplatzer封堵器封堵治疗的室间隔缺损患者,于术前1天、术后3天、3月及6月行经胸超声心动图检查,测量左心室舒张期末内径、左心室舒张期末容积、左心室收缩期末容积、左心房收缩期末内径、左心室射血分数、左心室侧壁基底部组织收缩期运动速度、舒张早期运动速度及舒张晚期运动速度.结果 室间隔缺损封堵术后3月、6月左心室舒张期末内径、左心室舒张期末容积、左心室收缩期末容积、左心房收缩期末内径、左心室射血分数、左心室侧壁基底部组织收缩期运动速度、舒张早期运动速度及舒张晚期运动速度均较术前1天及术后3天明显减少(P<0.05或0.01),但术后3天与术前1天比较差异无显著性(P>0.05).结论 室间隔缺损封堵术在消除异常分流的同时,可有效改善左心功能及几何形态.