Ebstein pulmonary hypoplasia

OBJECTIVE: To assess the safety and efficacy of transcatheter intervention for critical pulmonary stenosis (CPS) and pulmonary atresia with intact ventricular septum (PA/IVS) in neonates. METHOD: From June 2006 to September 2011, 27 neonates including CPS in 19 patients and PA/IVS in 8 patients underwent transcatheter intervention. All patients had membranous stenosis or atresia without severe Ebstein's anomaly and severe right ventricle and pulmonary valve hypoplasia, without right ventricle-dependent coronary circulation in PA/IVS...

Cor triatriatum dexter is a rare congenital anomaly, caused by the persistence of the right valve of the sinus venosus, which divides the right atrium into two chambers and its diagnosis is rather difficult, due to the features often mimicking Ebstein's anomaly. We describe a case followed from the foetal age for a relative right ventricular hypoplasia and suspicious pulmonary stenosis and diagnosed after birth to have cor triatriatum dexter with an obstructing membrane in the right atrium, needing surgical correction, done successfully...

OBJECTIVE: Patients with complex congenital heart disease, characterized by right ventricle hypoplasia, had a palliative surgical option with one and a half ventricular repair. METHODS: From July 2001 to March 2009, nine patients (mean age 5.2 years, range 3 to 9 years) with hypoplastic right ventricle, underwent correction with one and a half ventricle technique. Preoperative diagnoses included: pulmonary atresia with intact ventricular septum, in six and Ebstein's anomaly, in three cases...

Congenital tricuspid valve disease (Ebstein's anomaly, tricuspid valve dysplasia) with severe tricuspid regurgitation and cardiomegaly is associated with poor prognosis. Fetal echocardiography can accurately measure right atrial enlargement, which is associated with a poor prognosis in the fetus with tricuspid valve disease. Fetal lung volumetric assessments have been used in an attempt to predict viability of fetuses using ultrasonogram and prenatal MRI. We describe a fetus with tricuspid dysplasia, severe tricuspid regurgitation, right atrial enlargement and markedly reduced lung volumes...

In hypoplastic right ventricle the dysplasia of the tricuspid valve (TV) has repercussions on the development of the right ventricle. This paper documents the results of an anatomopathologic and morphometric studies of the tricuspid valve and the right ventricle in 25 hearts with this cardiopathy, which were morphologically analyzed using the segmentary sequential system. The following measurements were made: the annulus of the tricuspid valve, the thickness of the walls of the right ventricle, the ventricular septum and the distances from the atrioventricular-apex (AV-A) and from the apex-pulmonary valve (A-PV)...

OBJECTIVE: To report the experience of extracardiac conduit total cavopulmonary connection (ECTCPC) in surgical treatment of complex congenital heart diseases. METHODS: From 1998 to 2006, 68 patients underwent ECTCPC for complex congenital heart diseases. Among them, 45 had functional univentricle with transposition of the great artery (TGA) and pulmonary artery valve stenosis, 19 had tricuspid atresia with hypoplasia of right ventricle, 4 had Ebstein's anomaly with hypoplasia of right ventricle...

UNLABELLED: Ebstein's anomaly is an uncommon lesion of the tricuspid valve characterized by downward displacement of the tricuspid valve. The aim of the study was an analysis of the form, clinical outcome and treatment results in patients (pts) diagnosed with Ebstein anomaly. METHODS: 28 pts were analysed. Material has been divided into 3 groups, depending on clinical symptoms. The group A was formed with mild shape of the defect, group B all pts presenting significant clinical symptoms requiring treatment, and group C made of pts having severe symptoms requiring intensive treatment including surgical intervention...

The efficacy and safety of device closure of atrial defects in children with complex congenital heart disease and a hypoplastic right ventricle have not been detailed. The objective of this study was to determine the clinical impact and outcomes of a staged surgical catheter-based management strategy. A retrospective analysis of 17 children with a hypoplastic right ventricle who had undergone cardiac catheterization and attempted device occlusion of an atrial defect was undertaken. Clinical data, anatomical diagnoses, previous surgeries, and interventions were noted...

OBJECTIVE: One and one half ventricle repair is a surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. METHODS: From March 1994 to March 2001, eight patients (mean age 9.1 years, range 7 months to 35 years) with hypoplastic and/or dysplastic RV underwent correction of their intracardiac anomaly in association with a BCPS (one and one half ventricle repair). Preoperative diagnoses included: Ebstein's anomaly of tricuspid valve (TV) in two, inlet ventricular septal defect (VSD) in association with straddling/overriding TV in two patients, pulmonary atresia-intact ventricular septum in one, tertralogy of Fallot in association with complete atrioventricular canal defect in one, truncus arteriosus in one and heterotaxy syndrome with VSD and anomalous systemic venous return in one...

OBJECTIVE: One and one half ventricle repair is a surgical option for congenital cardiac anomalies characterised by right ventricle (RV) hypoplasia and/or dysplasia. METHODS: From March 1994 to March 2001, eight patients (mean age 9.1 years, range 7 months to 35 years) with hypoplastic and/or dysplastic RV underwent correction of their intracardiac anomaly in association with a BCPS (one and one half ventricle repair). Preoperative diagnoses included: Ebstein's anomaly of tricuspid valve (TV) in two, inlet ventricular septal defect (VSD) in association with straddling/overriding TV in two patients, pulmonary atresia-intact ventricular septum in one, tertralogy of Fallot in association with complete atrioventricular canal defect in one, truncus arteriosus in one and heterotaxy syndrome with VSD and anomalous systemic venous return in one...

We present the aims, methodology and initial results from the Italian Multicentric Study for the registration and follow-up of congenital heart disease. The general aims are to measure the prevalence of congenital heart disease in different geographic areas of Italy, and to assess the survival and outcome of affected babies. During the years 1992 and 1993, eighteen centers for Pediatric Cardiology spread all over the Country enrolled 1445 new babies with congenital cardiac malformations from a population of 341,647 surveyed livebirths...

Of the "five T's" of cyanotic congenital heart disease--tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)--the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot--right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis--are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation...

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 part of the repair. Diagnoses included pulmonary atresia with intact ventricular septum (n = 15), Ebstein anomaly (n = 5), levotransposition of the great arteries (n = 3), pulmonary stenosis with right ventricular hypoplasia (n = 2), tetralogy of Fallot (n = 3), dextrotransposition of the great arteries (n = l), and Uhl anomaly (n = l)...

Lung autopsies from four neonates with Ebstein's anomaly or tricuspid valve dysplasia and gross cardiomegaly were examined. The mean cardiothoracic ratio was 92 +/- 0.5%. The degrees of pulmonary hypoplasia and pulmonary artery muscularization were assessed and were compared with those in lungs from four controls and four patients with diaphragmatic hernias. Lung and body weight ratios and radial alveolar counts, which reflect pulmonary hypoplasia and immaturity, were significantly decreased only in patients with diaphragmatic hernia...

The procedure of bidirectional cavopulmonary anastomosis (DBCPA) is an effective method for the treatment of some complex congenital heart diseases. The clinical results of the procedure as a part of total cavopulmonary anastomosis were better than those of fontan operation in the treatment of single ventricle and tricuspid atresia. 6 cases were treated by using this procedure in our department in recent years. All of them receives total pulmonary anastomosis, but one this procedure plus ASD closure for the treatment of Ebstein anomaly with tricuspid valve stenosis and right heart hypoplasia...

The echocardiographic findings and clinical courses in 19 fetuses with marked cardiomegaly [heart length, width, area, cardiothoracic ratio, heart/thoracic area (CTA) ratio > 2 SD] were reviewed. An abnormal heart was the reason for referral in 16 cases of 19 (84.2%). The mean gestational age was 31.5 weeks at detection. Intracardiac hemodynamics were studied using pulsed, color, and M-Mode color Doppler. Despite the heterogeneous etiology of marked cardiomegaly (5 Ebstein's anomalies, 5 tricuspid valve dysplasias, 3 dilatative cardiomyopathies and 6 miscellaneous) a holosystolic tricuspid regurgitation with right atrial enlargement was found in 17 of the 19 cases (89...

The Fontan principle of redirecting systemic venous blood into the pulmonary arteries via a conduit has now gained a much wider application than initially intended. We have applied this type of operation in a series of 15 patients, five of whom had "classical" tricuspid atresia with concordant arterial connections. The other 10 patients showed a variety of congenital malformations, such as double-inlet univentricular heart with discordant arterial connections, double-outlet right ventricle with common atrioventricular canal...

The authors correlate echocardiographic and haemodynamic reports of a 12 year old patient with congestive heart failure and enlarged heart, secondary to fibrotic cardiomyopathy which effected the right ventricle (RV) and left ventricle (LV) apex. The echocardiographic M-mode examination showed: dilatation of the RV; ectasis and akinesis of the apex of the LV; the left auricle small for hypoafflux; tricuspid valve very noticeable with defective end-diastolic closure, delayed 0,80 msec as regards to the mitral valve, and in which it was not possible to identify the proto-diastolic opening; typical movement of the pulmonary valve, according to French's description, with synchronous opening to the atrial systole; paradoxical movement of septum, with the peculiarity of showing the maximum posterior movement relating to the atrial systole...

Correlative angiographic-anatomical studies in 19 cases of pulmonary atresia with infarct ventricular septum showed the following relations between the angiographic appearance of the pulmonary valve and the morphology of the right ventricle. (1) Doming of the pulmonary valve was associated with a nearly normal-sized right ventricle and a wide infundibulum patent to the level of the pulmonary valve. (2) A fixed valve was associated either with (a) pronounced hypoplasia of the ventricular changer and stenosis of the infundibulum or (b) less commonly, a massive right ventricle and Ebstein's malformation of the tricuspid valve...

Uhl's anomaly, or paper thin right ventricle is a rare congenital malformation which may present with variable degrees of hypoplasia. The most severe forms are encountered in infants and children, but a less serious form occurs in adolescents and adults which, clinically. There are a number of echocardiographic features common to both diseases but paper-thin right ventricle may be distinguished by the finding of premature opening of the pulmonary value. The increased atrial contraction in these patients with right ventricles of reduced compliance is directly transmitted to the pulmonary artery, leading to end diastolic opening of the pulmonary value before tricuspid closure...