Diverticulum of the right ventricle associated with ventricular septal defect

A true diverticulum of the ventricle is a very rare congenital cardiac lesion which is characterized by a normal myocardial wall and active participation in the ventricular contractions. Diverticula of the right ventricle may originate from the apical or anterosuperior wall. We report three patients with an anterosuperior diverticulum of the right ventricle associated with a perimembranous ventricular septal defect, one of whom additionally had obstruction of the right ventricular outflow tract. These patients presented clinically with symptoms related only to their associated defects. The right ventricular diverticulum was diagnosed by angiography in all, but was also seen on cross-sectional echocardiography in one patient. The ventricular septal defect was repaired through an incision of the diverticulum, which was reduced in size at the same time. Complete resection appears necessary only if severe endocardial fibrosis is present within the diverticulum, because such a diverticulum does not contribute to right ventricular contraction, but rather is at risk of rupture.     

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。     

Echocardiographic Evaluation of Adult Postoperative Congenital Heart Disease

Diagnostic and surgical advances have resulted in a large and growing population of adult patients with congenital heart disease. Proper care of these individuals requires knowledge of the original defects as well as the multitude of variations on surgical intervention for those lesions. Most complex congenital cardiac malformations amenable to "biventricular repair" are cyanotic and include a mixture of septal defects, obstructive and regurgitant valvular, subvalvular, or supravalvular lesions, and malposition of the cardiac chambers and great arteries. Whether they reach adulthood unoperated largely depends upon the balance of pulmonary blood flow. Examples of anatomical substrates that are usually candidates for "biventricular repair" include conotruncal abnormalities such as tetralogy of Fallot, transposition complexes of the great arteries, double-outlet right or left ventricle, and truncus arteriosus. "Biventricular repair" results in two well formed functional ventricles with a subpulmonic ventricle and a subaortic ventricle that are able to provide a circulation in series without admixture of venous and systemic blood. A unifying theme includes patch closure of a ventricular septal defect and reconstitution of ventricular-to-pulmonary arterial blood flow.     

Left ventricular non‐compaction: Prevalence in congenital heart disease

Introduction

Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy, originally described as an isolated disease without other structural cardiac abnormalities. The aim of this study was to explore the prevalence of LVNC among adults with different types of congenital heart disease.

Methods

From our databases we identified adults with congenital heart disease who fulfilled diagnostic criteria for LVNC. We report frequencies of associated congenital cardiac defects and the prevalence of LVNC among patients with different congenital heart defects.

Results

From a total of 202 patients with LVNC, 24 patients (12%; mean age 32 ± 11 years, 19 males) had additional congenital cardiac defects. Associated defects were left ventricular outflow tract abnormalities in 11 patients (46%), including 7 uni- or bicuspid aortic valves; two aortic coarctations; one diffuse aortic hypoplasia and one subaortic stenosis, Ebstein anomaly in 6 patients (25%), tetralogy of Fallot in two (8%), and double outlet right ventricle in one patient (4%). In our cohort, the prevalence of LVNC was highest among patients with Ebstein anomaly (6/40, 15%), followed by aortic coarctation (2/60, 3%), tetralogy of Fallot (3/129, 2%) and uni- or bicuspid aortic valves (7/963, 1%).

Conclusion

In adults, various forms of congenital heart disease are associated with LVNC, particularly stenotic lesions of the left ventricular outflow tract, Ebstein anomaly, and tetralogy of Fallot. In the future, studying these patients in more depth may provide a better understanding of the interplay between genetic and hemodynamic factors that lead to the phenotype of LVNC.     

Diverticulum of the right ventricle

We report the case of a 38-year-old woman who underwent cardiac catheterization because of cyanosis from birth. This study revealed a right ventricular cavity hypoplasia, an atrial septal defect, and a diverticulum originated from the free right ventricular wall. Ventricular diverticuli, especially those in the right ventricle, are very rare malformations, usually associated with other cardiac defects. The right ventricular diverticulum is generally non-symptomatic, diagnosed by chance, and does not usually require surgical treatment.     

Levels of brain natriuretic peptide in children with right ventricular overload due to congenital cardiac disease

OBJECTIVE: To evaluate the role of the concentration of brain natriuretic peptide in the plasma, and its correlation with haemodynamic right ventricular parameters, in children with overload of the right ventricle due to congenital cardiac disease. METHODS: We studied 31 children, with a mean age of 4.8 years, with volume or pressure overload of the right ventricle caused by congenital cardiac disease. Of the patients, 19 had undergone surgical biventricular correction of tetralogy of Fallot, 11 with pulmonary stenosis and 8 with pulmonary atresia, and 12 patients were studied prior to operations, 7 with atrial septal defects and 5 with anomalous pulmonary venous connections. We measured brain natriuretic peptide using Triage(R), from Biosite, United States of America. We determined end-diastolic pressures of the right ventricle, and the peak ratio of right to left ventricular pressures, by cardiac catheterization and correlated them with concentrations of brain natriuretic peptide in the plasma. RESULTS: The mean concentrations of brain natriuretic peptide were 87.7, with a range from 5 to 316, picograms per millilitre. Mean end-diastolic pressure in the right ventricle was 5.6, with a range from 2 to 10, millimetres of mercury, and the mean ratio of right to left ventricular pressure was 0.56, with a range from 0.24 to 1.03. There was a positive correlation between the concentrations of brain natriuretic peptide and the ratio of right to left ventricular pressure (r equal to 0.7844, p less than 0.0001) in all patients. These positive correlations remained when the children with tetralogy of Fallot, and those with atrial septal defects or anomalous pulmonary venous connection, were analysed as separate groups. We also found a weak correlation was shown between end-diastolic right ventricular pressure and concentrations of brain natriuretic peptide in the plasma (r equal to 0.5947, p equal to 0.0004). CONCLUSION: There is a significant correlation between right ventricular haemodynamic parameters and concentrations of brain natriuretic peptide in the plasma of children with right ventricular overload due to different types of congenital cardiac disease. The monitoring of brain natriuretic peptide may provide a non-invasive and safe quantitative follow up of the right ventricular pressure and volume overload in these patients.     

新鲜自体心包在心脏手术中应用的经验

目的介绍新鲜自体心包在心脏手术中应用的经验。 方法我院在321例心脏手术中应用了未经戊二醛处理的新鲜自体心包作为修复材料,应用范围广泛,包括先天性心脏病、风湿性心脏病和心脏肿瘤。 结果术后早期死亡20例,手术死亡率6.1%。并发症1例法乐四联症根治术后室间隔缺损残余漏,经二次手术修补治愈出院。3例室间隔缺损修补术后少量残余漏,随诊观察。全组75.7%的病人获得随诊,无术后溶血、栓塞、感染性心内膜炎、补片钙化及心包片瘤样膨出等并发症。 结论新鲜自体心包是心脏手术的优良修复材料。     

Tachycardias of right ventricular origin.

Ventricular tachycardia arising from the right ventricle usually has a left bundle branch morphology and occurs in a variety of disorders. Uhl's anomaly and right ventricular dysplasia may represent a spectrum of one disorder and are a cause of right heart dilatation, failure, and premature sudden death due to ventricular arrhythmias. Familial forms of the disorder may account for focal clustering in some geographic areas. Management should involve aggressive stratification of arrhythmia risk and may include medical, surgical, or device therapy. In contrast, the syndrome of right ventricular outflow tract tachycardia, including nonischemic exercise-induced and repetitive monomorphic ventricular tachycardia, is a more benign entity. Management often involves beta- and calcium channel blocking drugs or type IC antiarrhythmic drugs. Catheter ablation of the arrhythmia focus in the right ventricular outflow tract has been used in selected patients. In this syndrome the right ventricle is normal, and noninvasive testing as well as electrophysiologic studies can be helpful in distinguishing it from the more malignant right ventricular dysplasia. Ventricular arrhythmias may also be seen after right ventricular incision, as in surgical repair of tetralogy of Fallot and ventricular septal defects. Significant ventricular ectopy associated with an abnormal right ventricle (enlarged or depressed systolic function) is associated with an increased risk for sustained arrhythmia and sudden cardiac death in this group. The optimal indicator(s) of highest risk in these patients remains under investigation but will likely include electrophysiologic testing. Bifascicular block occurs commonly after repair of tetralogy of Fallot, but is usually benign. Isolated right ventricular infarction is rare. Most right ventricular arrhythmias associated with ischemia occur in the setting of iatrogenic catheter manipulation for pacing or hemodynamic monitoring. In conclusion, right ventricular arrhythmias involve an unusual and interesting group of clinical entities and appear to span the spectrum of arrhythmias mechanisms. A macroreentrant activation ring around the ventriculotomy scar may account for the arrhythmias following repair of tetralogy of Fallot. Microreentry at sites of morphologic abnormalities results in the arrhythmias associated with right ventricular dysplasia and ischemia. Triggered activity related to DADs or, less likely, abnormal automaticity, produce repetitive monomorphic ventricular tachycardia and nonischemic exercise-induced ventricular tachycardia, both of which usually originate from the right ventricular outflow tract. Iatrogenic ventricular tachycardia associated with catheter manipulation is especially likely to occur in the presence of right ventricular ischemia and infarction. It is important to recognize these clinical entities because treatment is specific.(ABSTRACT TRUNCATED AT 400 WORDS)     

The mechanism of apparent right bundle branch block after transatrial repair of tetralogy of Fallot.

The electrocardiographic pattern of right bundle branch block (RBBB) is routinely observed after transatrial repair of tetralogy of Fallot even though no ventriculotomy has been performed. The mechanism of this conduction disturbance was studied in 16 patients with tetralogy of Fallot and one patient with infundibular pulmonic stenosis. Preoperative ECGs and vectorcardiograms showed right ventricular hypertrophy and no RBBB. Epicardial activation maps were obtained before and after total surgical repair in all patients and after infundibular resection but before closure of ventricular septal defect (VSD) in four of these patients. After infundibular resection, RBBB appeared and activation was markedly delayed (greater than 30 msec) over the pulmonary outflow tract, but was unchanged over the body of the right ventricle. No further changes in ventricular activation occurred after closure of the VSD. This study shows that RBBB after transatrial repair of tetralogy of Fallot is usually produced by infundibular resection, but not by VSD closure, and is associated with delayed activation of the pulmonary outflow tract and base of the right ventricle which results from damage to portions of the right ventricular conduction system.     

Arrhythmien aus dem rechten Ventrikel

Right ventricular arrhythmias predominantly occur in young patients with rare cardiac diseases. Underlying cardiac conditions include idiopathic right ventricular outflow-tract tachycardia (RVOT-VT), arrhythmogenic right ventricular cardiomyopathy (ARVC), Brugada syndrome, and postoperative congenital heart disease (i.e. tetralogy of Fallot). According to the underlying cardiac disease, there are significant differences in the diagnostic and therapeutic management and prognosis which is mainly determined by life-threatening ventricular arrhythmia recurrences and sudden cardiac death. To provide optimal treatment for affected patients, a detailed diagnostic evaluation and risk stratification is mandatory. Tailored treatment strategies aim at the suppression or effective termination of recurrent ventricular tachyarrhythmias and prevention of sudden death by antiarrhythmic drug therapy, catheter ablation, and the implantation of cardioverter-defibrillators.This review summarizes the current knowledge on pathogenesis, diagnosis, treatment and prognosis of those conditions that are associated with arrhythmias originating from the right ventricle.     

Current role of radionuclide imaging in pediatric cardiology

Three main nuclear medicine methods are used in paediatric cardiology: sequential first-pass radionuclide imaging of the cardiac cavities, radionuclide equilibrium ventriculography and radionuclide myocardial imaging. Valuable functional information is obtained, and invasive explorations can be avoided in an ever increasing number of cases. Of particular interest is left-to-right shunt measurement which indicates that atrial septal defects must be surgically corrected when the pulmonary/systemic flows ratio (QP/QS) is above 2. This technique is also useful to evaluate the tightness of repairs in ventricular and atrial septal defects. Radionuclide studies of the right and left ventricles may detect dysfunction in one or the other cavity. The left ventricular ejection fraction is reduced in myocardiopathy an in aortic or mitral valve diseases seen at a late stage. The right ventricular function is often abnormal, notably during exercise, after repair of the tetralogy of Fallot and after atrial correction of complete transposition of the great arteries. An altered ejection fraction in patients with single ventricle is also a sign of deterioration. Right ventricular diastolic overload evaluated by radionuclide equilibrium ventriculography correlates with the QP/QS ratio value in atrial septal defects and with the inducibility of ventricular tachycardia by endocavitary pacing in repaired tetralogy of Fallot. Thallium 201 myocardial imaging provides information on myocardial ischaemia, notably that associated with congenital abnormalities of the coronary arteries. Its use had now been extended, albeit with some limitations, to the evaluation of right ventricular systolic overload. Other radionuclide techniques are being developed with new tracers: Kryton 81m for studies of the right ventricle, short-lived radionuclides for first-pass studies, Iodine 123-labelled fatty acids for myocardial imaging. More recently, some substrates, such as deoxyglucose, have been labelled with positron emitters permitting in vivo metabolic studies.     

Isolated congenital left ventricular diverticulum in adults.

Isolated congenital ventricular diverticulum or aneurysm is rare and usually arises from the left ventricle. The presentation of this condition is diverse. We report three cases of isolated congenital left ventricular diverticula. The age range was 17-30 years. Chest X-ray provided the earliest clinical suspicion in these three cases of a cardiac anomaly which was diagnosed by echocardiography and confirmed by angiocardiography. The location of the congenital left ventricular diverticulum was the left ventricular apex in two cases and basal in the other. We conclude that congenital left ventricular diverticulum is a disease of protean presentations. A high index of suspicion is necessary while interpreting chest X-rays and echocardiographs to diagnose congenital left ventricular diverticulum. A contractile accessory chamber of the left ventricle with a narrow neck with or without midline defects and an electrocardiogram without Q waves is consistent with the diagnosis of congenital left ventricular diverticulum.     

Arteriosclerotic heart disease following correction of tetralogy of Fallot

An increasing number of people who have undergone surgical repair of tetralogy of Fallot are living long lives. Several late sequelae of corrected tetralogy of Fallot have been found, including residual ventricular septal defect, restenosis of the pulmonary outflow tract, ventricular tachycardia, and right ventricular failure from pulmonary insufficiency. A long-term survivor of corrected tetralogy of Fallot is reported with acquired coronary artery disease unrelated to the congenital anomaly or its correction as an additional cause of late morbidity in this select but growing population of patients.     

Double outlet right ventricle: clinical spectrum and prognosis.

Patients with double outlet right ventricle can be separated into four distinct groups. In the largest (Group III), patients have a subaortic ventricular septal defect and pulmonary stenosis and clinically resemble patients with tetralogy of Fallot. In the next largest group (Group I), patients have a subpulmonry ventricular septal defect and no pulmonary stenosis and clinically resemble children with D-transposition of the great arteries and a ventricular septal defect. These patients have a high rate of coarctation of the aorta leading to early congestive heart failure, and their overall prognosis is poor. In the next largest group (Group II), patients have a subaortic ventricular septal defect and no pulmonary stenosis. Their presentation is similar to that of children with a large ventricular septal defect and pulmonary hypertension. In the smallest group (Group IV), the ventricular septal defect is uncommitted. Survivors in this group also clinically resemble children with a large ventricular septal defect and pulmonary hypertension. When present, coarctation of the aorta and severe mitral valve abnormalities greatly influence the prognosis in double outlet right ventricle. Although the prevalence of associated cardiac abnormalities is large, asplenia, polysplenia, chromosomal abnormalities and other congenital noncardiac abnormalities occur in only 12.5% of patients with double outlet right ventricle.     

Complete repair in total atrioventricular canal defect with cyanosis

Atrioventricular septal defects account for 4% of congenital cardiac malformations and over 50% of cardiac defects seen in Down syndrome. Clinical presentation is marked by congestive heart failure early in infancy. Cyanosis is rarely found in infants and suggests irreversible pulmonary hypertension or associated cardiac defects as tetralogy of Fallot, double outlet right ventricle, Ebstein anomaly, persistent left superior vena cava draining in the left atrium (Barbero Marcial, personal communication). Children with Down's syndrome is particularly difficult to assess because they often suffer from upper airways obstruction, which may contribute to the increased pulmonary vascular resistance determined at cardiac catheterization. This association of factors becomes a challenge for operability and, we will report one such case.     

Frequency of aberrant subclavian artery, arch laterality, and associated intracardiac anomalies detected by echocardiography

Tetralogy of Fallot is generally considered to be the most common congenital heart defect associated with an aberrant subclavian artery (ASA), but the prevalence of ASA in patients with other cardiac anomalies is not well described. The pediatric echocardiography database, with 15,871 initial echocardiograms, was searched for all patients with ASA. Arch laterality and associated intracardiac anomalies were documented for each patient. ASA was found in 226 patients, of whom 171 had a left aortic arch (LAA) and 55 had a right aortic arch (RAA). The occurrence of ASA was 1% in patients with LAA (171 of 15,650) and 25% in patients with RAA (55 of 221; p = 0.001). Intracardiac anatomy was normal in 32% of patients with ASA/LAA and 25% with ASA/RAA. Conotruncal anomalies occurred more frequently with ASA/RAA than ASA/LAA (36% vs 18%; p = 0.01). Atrioventricular canal defects accounted for 10% and left-sided cardiac obstructive lesions accounted for 11% of subjects with ASA/LAA. ASA was rarely associated with d-transposition of the great arteries (1 of 226) and double-outlet right ventricle (5 of 226). The prevalence of ASA was highest in patients with interrupted aortic arch (11 of 38; 29%). In patients with tetralogy of Fallot, the overall prevalence of ASA was 8% (34 of 447), but was higher with RAA (16 of 103; 16%). The highest prevalence of ASA occurred in the subgroup of patients with tetralogy of Fallot with pulmonary atresia and RAA (6 of 25; 24%). In conclusion, ASA was more common in patients with RAA, especially with conotruncal anomalies. In patients with LAA, hypoplastic left heart syndrome, aortic coarctation, and atrioventricular canal defects were commonly associated with ASA.     

Ectopia cordis and cardiac anomalies

Ectopia cordis is a rare disease that occurs in 5.5 to 7.9 per million live births. Only 267 cases had been reported as of 2001, most (95%) associated with other cardiac anomalies. We studied the cardiac malformations associated in 6 patients with ectopia cordis. Depending on where the defect was located, the cases of ectopia were classified into four groups: cervical, thoracic, thoraco-abdominal, and abdominal. All 6 patients died before the third day of life, 4 during delivery. Three of the patients were included in the thoracic group, whereas the other 3 belonged to the thoraco-abdominal group. All the patients had associated ventricular septal defects, 3 double-outlet right ventricle (50%) and the rest (50%) tetralogy of Fallot-pulmonary atresia. Two patients with double-outlet right ventricle presented mitral-valve pathology, a parachute valve and an atresic mitral valve. None of these cardiac anomalies have been reported to date.     

Acquired coronary artery fistula after open heart surgery for congenital heart disease

BACKGROUND: Most coronary artery fistulas were reported as congenital. Acquired coronary artery fistula occurring after cardiac surgery has rarely been reported. METHODS: From 1998 to 2003, 10 patients with coronary artery fistula detected by echocardiography after open heart surgery for congenital heart disease were included. Their ages ranged from 2 months to 41 years (median 4.2 years). The underlying heart disease was tetralogy of Fallot in five patients, ventricular septal defect in three, double chamber right ventricle in one, and transposition of the great arteries with ventricular septal defect in the remaining one. RESULTS: Of these 10 patients, the coronary artery fistula originated from the left coronary artery in four, right coronary artery in two, and unknown origin in the remaining four. The coronary artery fistula drained into the right ventricle in nine and into the left ventricle in the remaining one. The incidence of acquired coronary artery fistula after open heart surgery for congenital heart disease was 0.44% (8/1832). The identified risk factors for acquired coronary artery fistula were reoperation and right ventricular muscle resection in ventricular septal defect. After follow-up for 0.5-12 years (mean 4.1+/-3.3 years), the coronary artery fistula persisted, but neither symptoms nor significant left-to-right shunt was noted. CONCLUSIONS: Acquired coronary artery fistula is a rare complication after cardiac surgery. Reoperation and resection of right ventricular hypertrophic muscle increase the risk of this complication. Although shunt flow did not increase during follow-up, the significance of acquired coronary artery fistula needs further investigation.     

Left ventricular diverticulum in two adult patients

Left ventricular diverticulum is a rare congenital anomaly. In the adult population, the incidence was reported to be 0.26% in nonselected patients who underwent cardiac catheterization. Diverticula are usually localized near the apex and most often involve the inferior or anterior parietal walls of the left ventricle. In this report, two cases with congenital left ventricular diverticulum are presented, and the pathophysiologic, diagnostic, and therapeutic approaches of this cardiac malformation are discussed.