Three-dimensional echocardiographic measurement of right ventricular volume in children with congenital heart disease validated by magnetic resonance imaging

Measurement of right ventricular volume and function by two-dimensional echocardiography is unreliable because of the asymmetric shape of the right ventricle. The purpose of this study was to validate the accuracy of transthoracic three-dimensional echocardiography in assessing right ventricular volumes in children with congenital heart disease after surgical repair of the defects, by comparison with those measured by magnetic resonance imaging. We examined 13 children after repair of tetralogy of Fallot (10), hypoplastic left heart syndrome (2), or atrial septal defect (1). Each underwent magnetic resonance imaging followed by three-dimensional echocardiography done with a transthoracic 5 MHz, prototype internally rotating omniplane transducer. In both methods, endocardial borders were manually traced and volumetric slices were summated. Close correlation was observed between the two methods (R2 0.91 for end-systolic volumes, 0.90 for end-diastolic volumes, 0.64 for ejection fraction, and 0.92 for interobserver variability). A limits-of-agreement analysis showed no adverse trend between the two methods under values of 100 ml and low variation around the mean values. We conclude that three-dimensional echocardiography measurement of right ventricular volumes correlates closely with magnetic resonance imaging in children with operated congenital heart disease and may allow accurate serial evaluation in these patients.     

Assessment of the role and reliability of sonographic post-prandial flow response in grading Crohn''s disease activity

CONTEXT: The incidence of infective endocarditis after surgical repair of congenital heart defects is unknown. OBJECTIVE: To determine the long-term incidence of endocarditis after repair of any of 12 congenital heart defects in childhood. DESIGN: Population-based registry started in 1982. SETTING: State of Oregon. PARTICIPANTS: All Oregon residents who underwent surgical repair for 1 of 12 major congenital defects at the age of 18 years or younger from 1958 to the present. MAIN OUTCOME MEASURE: Diagnosis of infective endocarditis confirmed by hospital or autopsy records. RESULTS: Follow-up data were obtained from 88% of this cohort of 3860 individuals through 1993. At 25 years after surgery, the cumulative incidence of infective endocarditis was 1.3% for tetralogy of Fallot, 2.7% for isolated ventricular septal defect, 3.5% for coarctation of the aorta, 13.3% for valvular aortic stenosis, and 2.8% for primum atrial septal defect. In the cohorts with shorter follow-up, at 20 years after surgery the cumulative incidence was 4.0% for dextrotransposition of the great arteries; at 10 years, the cumulative incidence was 1.1% for complete atrioventricular septal defect, 5.3% for pulmonary atresia with an intact ventricular septum, and 6.4% for pulmonary atresia with ventricular septal defect. No children with secundum atrial septal defect, patent ductus arteriosus, or pulmonic stenosis have had infective endocarditis after surgery. CONCLUSION: The continuing incidence of endocarditis after surgery for congenital heart defect, particularly valvular aortic stenosis, merits education about endocarditis prophylaxis for children and adults with repaired congenital heart defects.     

Decreased sensitivity to insulin during treatment with danazol in women with endometriosis

Reconstruction techniques for major vessels and intracardiac defect repair use synthetic grafts or autogenic pericardium. Here, autologous abdominal parietal peritoneum with the overlying posterior rectus sheath as a biologic membrane are evaluated. Twelve adult canines were used. Via a midline subumbilical incision, the parietal peritoneum and overlying posterior rectus sheath were harvested. In the first group of six, the membrane was used to repair the right ventricular infundibulum and perform pulmonary artery annuloplasty. In the second group of six, under cardiopulmonary bypass and moderate hypothermia, the right atrium was opened and a secundum type defect was created. Autopsies performed 90 days after surgery revealed mild intrapericardial adhesions and moderate pericardial reaction over the cardiotomy incisions. The right ventricular outflow tract patch was nonaneurysmal. The interatrial patch was intact without thrombi. Histologic examination revealed intact membrane morphology, fibroblasts, smooth muscle cells, and endothelialization. Proline C14 uptake and autoradiography detected cellular viability of implanted membranes. These findings suggest that the peritoneum with overlying sheath repaired vascular and intracardiac defects and substituted for pericardium. Future studies are needed before clinical use.     

Right atrial isolation for atrial fibrillation associated with atrial septal defect

Two patients with atrial fibrillation associated with an atrial septal defect underwent simultaneous surgical correction of the atrial septal defect and right atrial isolation. The right atrium was surgically isolated while the continuity with the sinoatrial node was preserved in the remainder of the heart. After the operation, the patients maintained normal sinus rhythm for 99 and 65 months. Thus, right atrial isolation offers an alternative to the current surgical treatment for atrial fibrillation associated with an atrial septal defect.     

Stress and pressure in the radiopharmacy: a cause for concern

Although papillary fibroelastoma is rare, it is the most common primary tumor of the heart valves. We describe herein the case of a 64-year-old woman scheduled to undergo atrial septal defect (ASD) repair, in whom a papillary fibroelastoma of the tricuspid valve was diagnosed by transesophageal echocardiography (TEE). Surgical resection of the papillary fibroelastoma at the time of ASD repair prevented the fatal embolization sometimes associated with this lesion. Thus, intraoperative TEE played an important role in identifying the location of the tumor and its anatomic attachment, and in assessing the adequacy of surgical treatment.     

Recurrent bronchogenic cyst with rupture into the pericardium. A case report

A case of tamponnade due to intrapericardial rupture of a recurrent bronchogenic cyst, presenting as pericarditis, is described. This case is unique because it demonstrates the possibility of rupture of a bronchogenic cyst into the pericardium and by the unusual mode of presentation. It also shows that bronchogenic cysts may recur many years after incomplete ablation. Bronchogenic cysts are benign dysembrioplasic formations characterised by their respiratory epithelial lining. The usual presentation in the adult is by haemorrhage or infection, but our case shows that recurrent pericarditis without an obvious cause may be due to bronchogenic cyst, which should be systematically excluded. The diagnosis suspected after medical imaging (chest X-ray, scanner, magnetic resonance imaging) is confirmed by histology. Total surgical ablation is the treatment of choice and may be curative.     

Cardiopulmonary physiology after surgical closure of asymptomatic secundum atrial septal defects in childhood. Exercise performance is unaffected by age at repair

AIMS: Most secundum atrial septal defects, once diagnosed, are corrected at a young age. The evidence to justify early vs delayed or even non-closure is equivocal and little is known regarding long-term effects of later closure. This is particularly pertinent to those patients awaiting transcatheter closure of their defect for whom a device is only just becoming available. We examined the exercise cardiorespiratory physiology of children surgically treated for an isolated secundum defect. METHODS AND RESULTS: One hundred and six healthy control children and 22 children more than 6 months after surgical repair for an isolated secundum atrial septal defect were studied. All were asymptomatic. Measurements of effective pulmonary blood flow, stroke volume, arteriovenous oxygen difference, minute ventilation, heart rate, oxygen consumption and carbon dioxide production were made using a quadrupole mass spectrometer during rest and graded exercise. Data from the normal children allowed calculation of z scores for the atrial septal defect group matched for age, sex, pubertal stage and surface area. Maximal exercise performance was equal between control and atrial septal defect groups, however, the atrial septal defect group had a significantly greater effective pulmonary blood flow and stroke volume but a lower heart rate than controls at a given exercise stage. Stroke volume abnormalities were most closely related to duration of follow-up (29% of the variance explained, P < 0.01) rather than age at surgery. CONCLUSIONS: We were unable to show a medium term benefit from early surgery for an asymptomatic secundum atrial septal defect during exercise. The clinical relevance of the haemodynamic differences that do exist remains unclear.     

Constrictive pericarditis after coronary artery bypass surgery as a cause of unexplained dyspnea: a report of five cases

Constrictive pericarditis after coronary artery bypass grafting (CABG) is rare and can present as unexplained dyspnea. We report five consecutive cases of post-CABG constrictive pericarditis seen within a period of 17 months at our institution. All patients presented with heart failure of unknown etiology within a period of 8-84 months after surgery. During the initial post-CABG period, two patients had developed postcardiotomy syndrome that was successfully treated with steroids. They were all assessed noninvasively and invasively. In all patients, the diagnosis of constriction was initially suspected clinically (symptoms, high jugular venous pressure with deep "X" and "Y" descents, pericardial knock). Echocardiography showed transmitral flow typical of constriction in all patients and hepatic venous flow in two. Two patients showed rapid left ventricular relaxation. In all patients, hemodynamic assessment showed diastolic equalization of pressures in all chambers, "W" shape waveform in right atrial pressure, and "dip and plateau" configuration in right and left ventricular pressure waveforms. Diagnosis was confirmed surgically in four patients who were subjected to pericardiectomy-pericardial stripping (three survived, one died). One patient refused surgery. We conclude that constrictive pericarditis, although rare, should be suspected in every case of unexplained dyspnea post CABG. It can appear early or late after surgery, and clinical examination plays an important role in its early recognition. It requires a full noninvasive and invasive assessment in case of clinical suspicion.     

Successful repair of total anomalous pulmonary venous connection and incomplete endocardial cushion defect associated with left isomerism

Successful repair of a 8-month-old girl with polysplenia was reported. The cardiovascular anomalies were TAPVC (II b), incomplete ECD, interruption of inferior vena cava with hemiazygos continuation, bilateral superior vena cava, and left superior vena cava draining into the coronary sinus. Cardiopulmonary bypass was established with ascending aortic perfusion and caval cannulation. A left superior vena cava was directly cannulated after establishing partial bypass. In this case the left pulmonary vein drained into the right atrium near the orifice of the coronary sinus, so the atrial septal flap was made and sutured between the orifice of the left pulmonary vein and the coronary sinus in order to avoid late pulmonary vein obstruction. Then, atrium was separated by an intraatrial baffle which was sutured to the atrial septal flap. Recently, it becomes possible to surgical repair of polysplenia syndrome according to the advancements of the diagnostic methods, cardiopulmonary bypass, and the technique of the open heart surgery.     

Development of double-chambered right ventricle after surgical closure of a ventricular septal defect

The double-chambered right ventricle (DCRV) is a rare cardiac malformation, often associated with ventricular septal defect (VSD). CASE REPORT: The authors describe a case of DCRV in a child who underwent surgical repair of perimembranous VSD at the age of 7 months because of systemic pulmonary artery hypertension and growth retardation. At this time, no pressure gradient was measured within the right ventricle and no muscle proliferation was observed on echocardiography or right ventriculography. Postoperative follow-up revealed hypertrophy of the moderator band accompanied by progressive development of a right midventricular pressure gradient, which reached 60 mm Hg at the age of 3 1/2 years. The child was operated after invasive confirmation of the diagnosis of DCRV and the haemodynamic data. The child is now 6 years old and no restenosis has been observed. CONCLUSION: DCRV is usually associated with VSD, but the course of the two diseases can be dissociated. The diagnosis of DCRV must be considered in any child with a history of VSD, even after surgical or spontaneous closure, when clinical examination reveals a systolic ejection murmur.     

The management of severe subaortic stenosis, ventricular septal defect, and aortic arch obstruction in the neonate

Neonates with ventricular septal defect and aortic arch obstruction frequently have subaortic stenosis resulting from posterior deviation of the infundibular septum. Because the aortic anulus is often hypoplastic, making direct resection of the infundibular septum through the standard transaortic approach difficult, the optimal method of repair is uncertain. From September 1989 through November 1991, seven patients with ventricular septal defect, coarctation (n = 4), or interrupted aortic arch (n = 3) and severe subaortic stenosis underwent repair with use of a technique that included transatrial resection of the infundibular septum. Their ages ranged from 5 to 63 days (median 15 days) and weights from 1.3 to 5.4 kg (mean 3.1 kg). Only one patient was older than 1 month. The systolic and diastolic ratios of the diameter of the left ventricular outflow tract to that of the descending aorta were 0.53 +/- 0.09 mm (standard deviation) and 0.73 +/- 0.11, respectively. At operation, the posteriorly displaced infundibular septum was partially removed through a right atrial approach by resecting the superior margin of the ventricular septal defect up to the aortic anulus. The resulting enlarged ventricular septal defect was then closed with a patch to widen the subaortic area. In each patient the aortic arch was repaired by direct anastomosis. All patients survived operation; there was one late death from noncardiac causes 3 months after repair. The survivors remain well from 3 to 14 months after repair (mean 8 months). All are in sinus rhythm and none has a residual ventricular septal defect. One patient underwent successful balloon dilation of a residual aortic arch gradient late after repair. No patient has significant residual subaortic stenosis, although one has valvular aortic stenosis. This series suggests that in neonates with ventricular septal defect and severe subaortic stenosis resulting from posterior deviation of the infundibular septum, direct relief can be satisfactorily accomplished from a right atrial approach. This method provides effective widening of the left ventricular outflow tract and is superior to palliative techniques or conduit procedures.     

Repair of secundum atrial septal defect: limiting the incision without sacrificing exposure

A simple and effective technique for repair of secundum atrial septal defect is described. The heart is exposed through a limited midline skin incision and partial sternotomy, and the atrial septal defect is closed through a right atriotomy with ascending aortic and dual venous cannulation. This approach achieves a cosmetically superior result with standard instrumentation and cardiopulmonary bypass techniques, without compromising exposure or using peripheral incisions.     

Right-sided maze procedure for right atrial arrhythmias in congenital heart disease

BACKGROUND: Atrial fibrillation and flutter, commonly associated with congenital heart anomalies that cause right atrial dilatation, may cause significant morbidity and reduction of quality of life, even after surgical repair of the anomalies. METHODS: In an effort to reduce the incidence of atrial tachyarrhythmias after repair of right-sided congenital heart disease, we performed a concomitant right-sided maze procedure. RESULTS: Eighteen patients with paroxysmal atrial fibrillation or flutter (n = 12) or chronic atrial fibrillation or flutter (n = 6) aged 10.9 to 68.4 years (mean 34.9 years) underwent a right-sided maze in association with repair of Ebstein's anomaly (n = 15), congenital tricuspid insufficiency (n = 2), and isolated atrial septal defect (n = 1). There were no early deaths, reoperations, or complete heart block. Discharge rhythm was sinus (n = 16) or junctional (n = 2). Follow-up was complete in all 18 patients and ranged from 3.1 to 17.2 months (mean 8.1 months); all are in New York Heart Association class I. Early postoperative arrhythmias developed in 3 patients (all were converted to sinus rhythm by antiarrhythmic drugs). There were no late deaths or reoperations. CONCLUSIONS: The inclusion of a right-sided maze procedure with cardiac repair in patients having congenital heart anomalies that cause right atrial dilatation and associated atrial tachyarrhythmias is effective in eliminating or reducing the incidence of those arrhythmias.     

Successful surgical repair for a patient with atrial septal defect and severe pulmonary hypertension--its surgical indication and prostaglandin E1 use for postoperative pulmonary hypertensive crisis

A 45-year-old woman with atrial septal defect and pulmonary hypertension was admitted for surgical repair. Cardiac catheterization data revealed pulmonary to systemic flow ratio (Qp/Qs) of 1.81, pulmonary artery pressure (PAP) of 82/30 mmHg and pulmonary vascular resistance (PVR) of 10.8 unit. Open lung biopsy was added to evaluate pulmonary vascular obstructive disease (PVOD) which was shown Heath-Edwards grade 3 PVOD. Following closure of the defect, PAP (systolic) exceeded momentarily systemic level after cardiopulmonary bypass. Prostaglandin E1 drip was remarkably effective to bring down PAP during early postoperative period. Although PAP has not been shown significant decrease on catheterization of one year after surgery, great symptomatic improvement has been achieved.     

New methods of treatment for cerebral aneurysms

We present a case of a 25-year-old male with a large secondum atrial septal defect (ASD) associated with a coronary artery fistula (CAF) between the right coronary artery and the right atrium. The ASD was diagnosed preoperatively by transesophageal echocardio-graphy (TEE). The fistula was found at surgery. The ASD and fistula were closed successfully without any complication. The case highlights the common presenting features of the two conditions as well as the low sensitivity of pre-operative TEE in diagnosing CAF in the setting of an ASD.     

Renovascular disease: the fifth frontier

BACKGROUND: Coronary sinus type atrial defect is the result of an incomplete formation of the atriovenous fold. This is a rare anomaly that in a very few cases took advantage of echocardiographic diagnosis before surgery. We report on a case of coronary sinus type atrial septal defect diagnosed by means of transthoracic and transesophageal echocardiography. PATIENT: A 65 year old woman who was admitted to hospital for evaluation of dyspnea and pre-syncope. A diagnosis of secundum type atrial septal defect had been achieved few months before. METHOD DESCRIPTION: Color Doppler transthoracic echocardiography demonstrated evidence of left-to-right shunt through the coronary sinus-left atrium common wall, while transesophageal echocardiography showed a defect in the coronary sinus roof in its terminal portion, proximal to the atrial septum. At that level the shunt flow was demonstrated by the presence of a negative contrast after contrast injection. Both transthoracic and transesophageal contrast echocardiographies demonstrated the persistence of a left superior vena cava draining into the enlarged coronary sinus: the existence of a right-to-left shunt at the coronary sinus level suggested by transthoracic echocardiographic examination was not confirmed by transesophageal echocardiography. CONCLUSIONS: This is one of the few reported cases of coronary sinus type atrial defect diagnosed noninvasively and the diagnostic usefulness of both transthoracic and transesophageal echocardiographic approaches is stressed.     

French experience in the closure of atrial septal defects of the ostium secundum type with the Sideris button occluder

From February 1992 to November 1995, four French teams used the Sideris button occluder to close 122 ostium secundum, foramen ovale or surgical fenestration atrial septal defects in 121 patients aged 2 to 79 years with body weights of 10 to 96 kg. a left-to-right shunt in 110 cases (average QP/QS = 2.09) or right-to-left shunt in 12 cases. The usual type of prosthesis was used in 115 cases, 8 centered on a guide wire, reverse type in 5 cases and the "centering-device" type in 2 patients. Nineteen implantation attempts were abandoned before releasing the prosthesis. The immediate results were: closure of the atrial septal defect in 116 patients: 59 were completely occluded, 43 had minimal residual shunts. Five patients were operated for non-buttoning or malposition of the prosthesis. In one other case, the device was removed by catheterisation. During follow-up ranging from 1 month to 3 years, 20 patients were operated for varying complications, the commonest of which was malposition of the prosthesis (17 cases) with a shunt of variable volume. In one other case, a second device was inserted. Seventy-seven patients were reviewed at 1 year, 28 a 2 years and 6 at 3 years. The residual shunts decreased with time but only completely disappeared in half the cases. Secondary fractures not requiring surgery were observed in 5 patients. Failures and complications were the result of various causes which are discussed. Successive technological improvements and the experience of the medical teams should reduce this incidence, but caution is required especially in the treatment of young children.     

Transcatheter closure of a residual postmyocardial infarction ventricular septal defect with the Amplatzer septal occluder

Acute ventricular septal rupture following myocardial infarction carries a high mortality. Early surgery improves survival but long term outcome depends on residual shunting and left ventricular function. Residual shunting is common despite apparently successful closure and may require reoperation. Transcatheter closure is an established method of treating selected congenital defects but clinical experience of transcatheter closure in postinfarction ventricular septal rupture is minimal. Transcatheter closure of a residual ventricular septal defect was successfully done using a new device, the Amplatzer septal occluder, in a 50 year old Indian man who had previously undergone emergency surgical repair for postinfarction acute ventricular septal rupture. The technique is described and its potential as a treatment in postinfarction ventricular septal rupture, its possible complications, and the important aspects of case selection and device design are discussed.     

Transcatheter closure of residual atrial septal defect following implantation of buttoned device

We report a case in which residual shunting after a buttoned device occlusion of atrial septal defect (ASD) was eliminated by transcatheter retrieval of a portion of the device, followed by implantation of a second device. This method may be helpful for those patients with residual ASDs who decline surgical device retrieval and defect closure.     

Usefulness of PKHs for studying cell proliferation

INTRODUCTION AND OBJECTIVES: The MAZE procedure was developed as a surgical approach to the management of patients with atrial fibrillation refractory to medical treatment. This study seeks to identify the risk and benefits of adding the MAZE procedure in patients with atrial fibrillation undergoing surgery for underlying organic cardiac disorders. MATERIAL AND METHODS: Since november 1993, we have performed 10 interventions with the MAZE procedure, for the treatment of refractory atrial fibrillation. The indication to perform the technique was systemic embolism in 5 patients, contraindication for the anticoagulant treatment in two cases and no response to antiarrhythmic treatment in 5 cases. Two patients had more than one indication. In all the cases another surgical procedure was performed, 5 replacements of mitral valve, a mitral repair, one tricuspid repair and tree repairs of an atrial septal defect. RESULTS: Soon after surgery 9 patients were in sinus rhythm, and one in atrial fibrillation. Four patients needed atrial pacing during the first days. One patient required a pacemaker due to symptomatic sinus bradycardia. During the first 3 months, 4 patients had episodes of paroxysmal atrial fibrillation and flutter. One patient died suddenly one month after surgery. Seven patients have completed two years of follow-up, and are in stable sinus rhythm, in functional class I and free of antiarrhythmic drugs. All of them have echocardiographic evidence of mechanical activity in both atria. Left atrium had been reduced from 5.3 +/- 0.7 cm to 4.5 +/- 0.7 cm (p < 0.05). No patient has presented new embolic events. CONCLUSIONS: The MAZE procedure is a good choice in selected patients with atrial fibrillation refractory to medical treatment, or a precedent of systemic embolism. However, several problems can complicate the patient's course.