BACKGROUND: The right ventricle, when incorporated in the Fontan circulation, might enlarge and function as a pump to the pulmonary circulation. Experience has shown that over the long-term, this operation can be associated with major difficulties. METHODS: The late results, (13+/-6 years) after right atrioventricular connection as a Fontan modification, ...

OBJECTIVE: Late outcome of neonatal pulmonary atresia with intact ventricular septum remains poor in most reported series. We have followed a selective approach toward either single ventricle repair versus complete or partial biventricular repair based on the presence of right ventricle-dependent coronary circulation and growth of the right ventricle. METHODS: ...

BACKGROUND: Straddling tricuspid valve, despite extensive investigation, remains an incompletely understood form of complex congenital heart disease. METHODS: A morphometric study of 19 postmortem cases of straddling tricuspid valve was performed, and the results were compared with 32 normal control heart specimens. RESULTS: In straddling tricuspid valve, marked malalignment of ...

BACKGROUND: Prenatal echocardiography has shown evidence of prenatal development of congenital heart disease. Prenatal cardiac anatomy, chamber size and function change during gestation, so that the appearance of cardiac structure in abnormal hearts may be different from that which is usually seen postnatally. METHODS: Published prenatal echocardiographic studies were reviewed ...

Ebstein's malformation of the tricuspid valve is a complex and heterogeneous disorder that has diverse hemodynamic and arrhythmic manifestations. Hemodynamic dysfunction is due to valvar regurgitation and decreased pulmonary blood flow, varying in severity from mild tricuspid insufficiency to severe dysplasia of the right ventricle without forward flow. Atrioventricular reentrant ...

Left superior vena cava (LSVC) to the left atrium is a rare congenital cardiac complex, which may appear as an isolated anomaly, or as part of more complex cardiac anomalies. Traditionally, an intraatrial baffle was the preferred surgical technique. Although this technique has proved reliable and successful, acute ligation and ...

OBJECTIVE: To report our initial experience of the first 5 cases of partial left ventriculectomy (PLV or Batista operation), a new surgical therapy for treatment of end-stage dilated cardiomyopathy in China. METHODS: From April to August 1998, 5 patients with idiopathic dilated cardiomyopathy received PLV at Anzhen Hospital. The mean ...

The results of transcatheter valvotomy in pulmonary atresia with intact ventricular septum (PA-IVS) patients are presented with an attempt to identify the predictive factors for pulmonary valvotomy alone as definitive treatment. Between June 1995 and December 1997, 14 PA-IVS neonates with tripartite right ventricle underwent an attempted pulmonary valvotomy. For ...

Eight patients undergoing tricuspid valve replacement (TVR) for severe tricuspid regurgitation (TR) without stenosis were subjected to the study. Four patients had primary TR due to trauma, infective endocarditis, or Ebstein's anomaly, and the other 4 had secondary TR associated with mitral valve lesions. The right heart pressure as well ...

BACKGROUND: Tricuspid regurgitation (TR) may occur following orthotopic heart transplantation (OHT) and although a number of etiological factors have been suggested, the relative contribution of each of these remains to be elucidated. We aimed to assess the risk factors for TR in our 10-year experience of orthotopic heart transplantation (OHT). ...

Classic Fontan procedures, such as atriopulmonary connection or atrioventricular connection, are associated with right atrial dilatation and several late complications. Seven patients with univentricular heart who presented with exercise intolerance (n = 7), severely dilated right atrium (n = 7) with pulmonary vein compression (n = 3), atrial arrhythmias resistant ...

BACKGROUND AND AIMS OF THE STUDY: Patients with symptoms of right heart failure due to severe tricuspid regurgitation following a prior operation on left heart valves present a difficult problem. The outcome of tricuspid surgery in this setting is not well defined. We describe a single-center experience of isolated tricuspid ...

BACKGROUND: Early treatment for the neonate with pulmonary atresia and intact ventricular septum (PA-IVS) is important if a high mortality rate is to be avoided. The treatment includes prostaglandin administration, balloon atrial septostomy, pulmonary valvotomy, a shunting procedure and patch repair for the right ventricular outflow tract (RVOT). This study ...

BACKGROUND: Analysis of right ventricular adaptation to tricuspid regurgitation was studied in 10 heart transplant recipients following inadvertent endomyocardial biopsy disruption of the tricuspid apparatus. METHODS AND RESULTS: Echocardiography demonstrated progressive diastolic right ventricular cavity enlargement (19.5+/-5.0 to 30.3+/-5.4 cm(2), P<0.0002), with disproportionate elongation along the midminor axis (3.5+/-0.6 to ...

OBJECTIVE: The aim of this study was to compare the medium-term results of right heart pressures, tricuspid valve dysfunction, overall cardiac performance, and survival between the bicaval and standard techniques. METHOD: Between 1991 and 1997, 201 heart transplantations were performed in our center. Right heart catheterization was performed up to ...

OBJECTIVE: Our goal was to establish the morphologic nature of the obstructive muscular lesions in double-chambered right ventricle. METHODS: We based our morphologic observations on 10 normal hearts and on the surgical findings in 26 patients, aged 0.5 to 24 years, with a mean of 6.9 years (SD 5.8 years). ...

OBJECTIVE: The conventional management of patients with atrioventricular discordance is directed at associated lesions, taking advantage of physiologic "correction"; however, the morphologic right ventricle and tricuspid valve support the systemic circulation. Questions surrounding survival using this approach led us to analyze our institutional results. METHODS: All patients with atrioventricular discordance ...

Traumatic lesions of the tricuspid valve complicating pacemaker lead extractions appear to be rare. We report two cases of partial rupture of the tricuspid valve, following apparently uneventful extraction of permanent ventricular leads, resulting in severe regurgitation and, in one case, chronic heart failure. TEE was useful to identify the ...

OBJECTIVE: To evaluate the feasibility of anatomical correction based on morphological and echocardiographic findings in patients and preserved hearts with discordant atrioventricular connections. DESIGN: A retrospective study with clinicomorphological correlations to assess potential contraindications for anatomical correction in the setting of discordant atrioventricular connections. SETTING: A tertiary referral centre for ...

BACKGROUND: Knowledge of the precise anatomy can be advantageous when striving to improve surgical results in patients with visceral heterotaxy. METHODS: We studied the ventricular mass, and its coronary arterial supply, in 125 specimens with isomeric right and 58 with isomeric left appendages. RESULTS: The situation in which each atrium ...

OBJECTIVE: This article presents a 10-year experience with one and a half ventricle repair for right ventricular hypoplasia or dysfunction. METHODS: From November 1986 to December 1996, 30 patients (mean age 6.7 +/- 8.5 years, range 4 months-40 years) with functionally abnormal right ventricles underwent a bidirectional Glenn shunt as ...

The aim of the study was to provide logical steps, alternative to the sequential chamber analysis, for diagnosing major congenital heart malformation prenatally. Between 1988 and 1995, of the 14,885 high-risk pregnancies screened at the Departments of Obstetrics and Paediatric Cardiology, University of Hong Kong, 92 fetuses with major congenital ...

Congenital cardiac lesions in the adult have characteristic roentgen patterns that should be recognized by the radiologist. In other instances, abnormalities in the aorta or the position of the organs can indicate the likelihood of associated cardiac anomalies. An increasing number of congenital cardiac patients are surviving into adult life ...

OBJECTIVES: The aim of this study was to characterize differences in the long-term effects of treatment for functional tricuspid regurgitation based on the primary cardiac lesion. METHODS: Ninety-six patients with valvular heart disease and 32 patients with atrial septal defects associated with tricuspid regurgitation were studied. The tricuspid annular diameter ...

OBJECTIVE: To compare the atrial and ventricular myoarchitecture in the normal heart and the heart with tricuspid atresia, and to investigate changes in the three dimensional arrangement of collagen fibrils. METHODS: Blunt dissection and cell maceration with scanning electron microscopy were used to study the architecture of the atrial and ...

Left-sided partial anomalous pulmonary venous return (PAPVR) with an intact atrial septum is a rare cardiovascular anomaly. This report deals with the case of a 22-year-old woman who was referred to our Institution because of resting palpitation and exertional dyspnea. Two-dimensional echocardiography revealed right heart volume overload and a dilated ...

OBJECTIVE: Our aim was to elucidate the morphologic spectrum of Ebstein's malformation of the tricuspid valve with regard to diagnosis and the feasibility of surgical repair, in the light of the currently favored reconstructive techniques. METHODS: We examined 23 autopsied hearts. Taking the displacement of the septal and mural leaflets ...

Pulmonary atresia with intact ventricular septum (PA/IVS) is a spectrum of diseases with varying severity of right ventricle hypoplasia and potential for biventricular, univentricular, or hybrid repairs. Pessimistic outcome measures for PA/IVS may give way to optimism with the refinement of early diagnosis and early intervention to encourage right ventricle ...

A case is described of a 54 year old woman who had acute pericarditis with large exudative effusion accompanied by severe right and left ventricular failure. The patient was finally diagnosed with carcinoid heart disease from an ovarian carcinoid teratoma. She was treated with octreotide--a somatostatin analogue--followed by radical surgical ...

OBJECTIVE:Pulmonary atresia with intact ventricular septum is an anatomically heterogeneous anomaly with a variety of surgical strategies possible. We sought to compare the outcome of patients with a single ventricle approach to those with a biventricular repair and to compare outcome of patients with coronary abnormalities to those with normal ...

Two-dimensional and color Doppler echocardiograms obtained in 117 patients during cardiac transplantation evaluation were reviewed. Right ventricular hypokinesia and dilation were more prevalent in patients with tricuspid regurgitation. In multivariate event-free survival analysis of 61 patients with complete clinical, echocardiographic, and cardiopulmonary exercise data, the absence of tricuspid regurgitation and ...

BACKGROUND: Moderate to severe Ebstein's malformation remains a surgical challenge. Although the various approaches that have been used are appropriate and successful in many patients, there are many for which these approaches are suboptimal. To improve the prognosis for patients across the full spectrum of Ebstein's malformation, alternative surgical approaches ...

Although combined mitral and tricuspid stenosis are rarely seen in patients with rheumatic heart disease, when both exist together, combined percutaneous balloon valvuloplasty can be an alternative to surgical treatment in suitable cases. We present the immediate and late follow up results of 12 patients with rheumatic tricuspid and mitral ...

Intramyocardial hematoma is an uncommon lesion, most often occurring after chest trauma, aortic valve disease, acute myocardial infarction, or coronary artery bypass surgery. We describe a 17-year-old patient who experienced cardiac tamponade after repair of Ebstein malformation. The malformation was caused by an enlarging intramyocardial hematoma that extended from the ...

OBJECTIVE: We sought to test the hypothesis that apoptosis is a method of remodeling in second-trimester fetal heart development. We also hypothesized that hearts from fetuses with Down syndrome would have different levels of apoptosis than would control hearts, associated with their abnormal heart development. STUDY DESIGN: We obtained hearts ...

Cardiac sequelae of stab chest wounds may be various and dramatic, and the right ventricle is the most commonly injured chamber. Correct diagnosis of cardiac damage may be done up to many years after the trauma. We describe a rare case of isolated, unexpected flail tricuspid valve detected by transthoracic ...

BACKGROUND: The concept of "one and a half ventricular repair" relates to situations where one ventricle is capable of pumping one half of the circulation while the other ventricle is deemed inadequate and requires off-loading by means of a shunt. The inadequate ventricle is usually assigned the role of pumping ...

BACKGROUND: The concept of "one and a half" ventricular repair is to use "half" a ventricle to support the pulmonary circulation. The component makeup of any ventricle needs clarification for us to understand the nature of the so-called half ventricle. METHODS: The components of normal and abnormal ventricles are reviewed. ...

BACKGROUND: Ebstein anomaly is a rare tricuspid valve anomaly. Some fetuses with Ebstein's anomaly have concurrent severe cardiac function impairment, which results in hydrops fetalis. Most of these fetuses are inevitably terminally ill. No reports have demonstrated the potential prenatal therapy for fetuses under such conditions. CASE: Ebstein's anomaly and ...

BACKGROUND: Evaluation of hypoplastic ventricles with echocardiography requires an appreciation of the ultrasound methods used to assess ventricles of normal size. In this review, we present an overview of the most common techniques used to measure ventricular size, which may be analyzed as long- or short-axis dimension, area, volume, or ...

BACKGROUND: A variety of factors can influence the suitability of a congenitally malformed heart for biventricular repair, including size, morphology, function, and dimensions and function of the inflow and outflow, among others. Although certain features have been identified that may indicate a lower probability of successful biventricular repair, our ability ...

BACKGROUND: The surgical option of biventricular repair requires two ventricles, each fully capable of supporting the systemic or pulmonary circulation. The morphologic substrates that may preclude some hearts from biventricular repair need to be assessed. METHODS: Heart specimens were reviewed to assess the morphologic mechanisms that produce an unbalanced ventricular ...

BACKGROUND: Partial biventricular repair consists of bidirectional cavopulmonary anastomosis in the presence of separated systemic and pulmonary circulations, with antegrade flow of inferior caval return through an intact or reconstructed pulmonary outflow tract. This versatile procedure may be used as a definitive repair for patients with a functional right heart ...

OBJECTIVE: Modified Fontan procedures are now employed in several conditions unsuitable for bi-ventricular repair. Selection criteria have been relaxed. The procedure is palliative. Longterm outlook is unknown. This study evaluated factors associated with the development of a failing Fontan circulation and transplantation results. METHODS: Retrospective review of patients referred to ...

The origination of all three major coronary arteries from three separate ostia in the right sinus of Valsalva is an exceedingly rare coronary anomaly. Few radiographic and clinical details of this anomaly are available in the literature. We describe this anomaly in a patient with acute myocardial infarction who remained ...

To clarify the abnormal development of the cardiac conduction system in congenital heart anomalies, HNK-1 distribution of bis-diamine induced malformed hearts was studied in rat embryos. All the embryos showed not only conotruncal anomalies, such as persistent truncus arteriosus (PTA) and pulmonary hypoplasia with overriding aorta (comparable to tetralogy of ...

BACKGROUND: The echocardiographic criteria that have been used to evaluate severity of Ebstein's anomaly in utero are the same as those applied after birth. OBJECTIVE: The objective of this study was to establish prognostic criteria that take into account the peculiarities of the fetal hemodynamics. METHOD: The video recordings of ...

BACKGROUND: Seven patients with the diagnosis of Ebstein's malformation of the tricuspid valve were operated on. Mean age was 12 years (range, 7 to 16 years). All were cyanotic, with severe tricuspid regurgitation. Thromboembolism was not present. No associated cardiac malformations were present. METHODS: Surgical repair included tricuspid annuloplasty associated ...

To evaluate the effect of tricuspid annuloplasty (TAP) on right heart flow dynamics, we analyzed tricuspid inflow velocity pattern, jugular venous pulse and color Doppler flow signal of tricuspid regurgitation (TR) before and after surgery in 16 patients who underwent TAP (TAP group). Cardiac rhythm was atrial fibrillation in all ...

OBJECTIVES: Since 1990, transcatheter pulmonary valvotomy has become an alternative to surgical valvotomy in the management of neonates and infants with pulmonary atresia and intact ventricular septum. We sought to determine whether right ventricular growth after transcatheter pulmonary valvotomy is commensurate with body growth. METHODS: Laser or radiofrequency-assisted balloon valvotomy ...