Tricuspid Valve Diameter Research Articles

Dimensions of Human Adult Tricuspid Valve Annulus in the Embalmed Cadavers

Introduction: In human heart, right atrio-ventricular orifice is guarded by tricuspid valve, which lies at the junction of right atrium and right ventricle of the heart controlling and directing unidirectional blood flow during ventricular diastole. Irreparable damage to tricuspid valve calls for its replacement using prosthetic valves. Prosthesis of these synthetic valves need detail knowledge of tricuspid orifice. In present study an attempt was made to study the dimensions of tricuspid orifice. Materials and Method: The present work was done by dissecting 42 formalin fixed adult human hearts. The sex of human was not considered. Tricuspid orifice was cut opened in these specimen and following measurements were taken (a) circumference of annulus of tricuspid valve (b) diameter of tricuspid valve in two vertically perpendicular plane. Results: The circumference of the tricuspid valve annulus ranged from 7.9 cm to 12cm, average was 9.7 cm±1.029 and median -9.5cm. The diameter measured in two separate perpendicular planes at maximally separated points ranged from 1.3 cm to 4.5cm. In one plane, mean diameter was 2.1±0.43cm and median was 2cm. In second plane, mean diameter was 3.03±0.54cm and median was 2.9cm. Conclusion: These data may be useful in designing the prosthetic valves.

Predictors of Tricuspid Valve Anulus Dilation in a Heart Recipient Population

BackgroundTricuspid valve regurgitation in reported in >20% of heart recipients. It severity has not only clinical impact, but it is also associated with increased mortality. Risk factors for developing tricuspid valve dysfunction include allograft rejection, donor/recipient pericardial cavity mismatch, preoperative transpulmonary gradient and vascular resistance, biatrial anastomosis technique, and biopsy-induced injury. Tricuspid valve annulus distention is reported to causative factor for most common type of tricuspid valve dysfunction after heart transplantation. The aim of the study was to estimate possible early predictors for tricuspid valve regurgitation after orthotopic heart transplantation performed with standard Lower-Shumway technique on magnetic resonance imaging studies. MethodsA total of 20 patients (18 men and 2 women) with a mean age of 45 ± 12 years were enrolled into the study. Echocardiographic evaluation followed by magnetic resonance studies were performed. The mean duration from time of transplantation was 34 ± 12 months. Magnetic resonance and echocardiographic imaging focused on tricuspid valve annulus diameter and atrium dimensions. ResultsThe was a progressive distension of tricuspid valve annulus observed during the follow-up period. Mean tricuspid valve diameter increased from 3.0 ± 0.3 to 3.34 ± 0.3 mm (P < .05). There was a positive correlation observed between recipient native right atrium and overall right atrium diameter and tricuspid valve diameter distension. ConclusionsOverall right atrium diameter and native recipient right atrium diameter were found to be a risk factor for tricuspid valve annulus distension.

The double orifice valve technique to treat tricuspid valve incompetence

A straightforward tricuspid valve (TV) repair technique was used to treat either moderate or severe functional (normal valve with dilated annulus) or for primary/organic (Ebstein's anomaly, leaflet retraction/tethering and chordal malposition/tethering, with annular dilatation) TV incompetence, and its long-term outcome assessed. A double-orifice valve technique was employed in 91 patients (mean age 52.6 ± 23.2 years; median age 56 years; range: 0.6-82 years) with severe tricuspid regurgitation. Among the patients, three had post-transplant iatrogenic chordal rupture, five had infective endocarditis, 11 had mitral valve insufficiency, 23 had Ebstein's anomaly, and 47 had isolated severe TV incompetence. The basic principle was to reduce the distance between the coapting leaflets, wherein the most mobile leaflet could coapt to the opposite leaflet, by creating two orifices, ensuring valve competence. The TV repair was performed through a median sternotomy or right anterior thoracotomy in the fifth intercostal space under cardiopulmonary bypass. The degree and extent of creating a double-valve orifice was determined by considering the minimal body surface area (BSA)-related acceptable TV diameter. Repair was accomplished by passing pledgeted mattress sutures from the middle of the true anterior annulus to a spot on the opposite septal annulus, located approximately two-thirds of the length of the septal annulus to avoid injury to the bundle of His. The annular apposition divides the TV into a larger anterior and a smaller posterior orifices, enabling valve closure, on both sides. In adults, the diameter of the anterior valve orifice should be 23-25 mm, and the posterior orifice 15-18 mm; thus, the total valve orifice area is 5-6 cm2. In children, the total valve orifice should be a standard deviation of 1.7 mm for a BSA of <1. 0m2, and 1.5 mm for a BSA of >1.0m2. During a mean follow up of 8.7 ± 1.34 years (median 10 years; range: 1.5-25.9 years) there have been no reoperations for TV insufficiency or stenosis. Reoperations on three patients (mean age 42.5 ± 8.7 years) were indicated for aortic valve replacement at 14 months postoperatively (n = 1) and for assist device implantation (n = 2) who eventually underwent heart transplant at 18 and 20 months after TV repair, respectively. The cumulative 12-year survival rate was 86.9%. This double-orifice technique is technically a straightforward repair to abolish TV incompetence with highly satisfactory results, particularly in patients with severe annular dilatation or with leaflet and chordal tethering. In the present series, the technique provided no pitfalls (if the location of the conduction system was borne in mind), requiring only a gentle placement of sutures. It also led to no residual regurgitation or reoperation during the follow up period.

Outcomes of transcatheter approach for initial treatment of pulmonary atresia with intact ventricular septum

To review the short and medium term outcome of transcatheter pulmonary valve perforation (PVP) in patients with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PA/IVS non-RVDCC). PVP in patients with PA/IVS non-RVDCC has become more common in the past two decades. However, data on outcomes with this strategy are mixed. Data were reviewed retrospectively for all patients with PA/IVS non-RVDCC treated from 1996- 2010 at our institution. Patients who had severe neonatal Ebstein malformation, or initial interventional management at another institution were excluded. PVP was attempted in 30 of 50 patients (60%); 26 (87%) of these had a successful procedure. Twenty-four patients (48%) had surgery without PVP. There were no deaths in the cohort. Complications of PVP included 5 (17%) myocardial perforations. Of those with successful PVP, 10 (38%) did not have surgery (PVP-NS) and 16 (62%) had surgery (PVP-S) prior to discharge. Tricuspid valve (TV) Z-score was larger in the PVP-NS than in PVP-S patients, with median TV diameter Z-scores of +0.7 (-0.9, 1.7) and -1.1 (-2.8, 2), respectively (P = 0.01). Time from PVP to either hospital discharge (PVP-NS group) or surgery (PVP-S group) was significantly different between groups: 15 (7, 22) and 8 days (0, 46), respectively (P = 0.01). There were no differences in the number of trials or lowest arterial PaO2 off prostaglandins between groups. All patients in the PVP-NS group had a biventricular circulation at a median follow-up of 4.3 years. The results of a collaborative approach to treating neonates with PA/IVS non-RVDCC are excellent. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy.

Aorto–Right Ventricular Tunnel Causing Functional Tricuspid Atresia

A girl with symmetrical intrauterine growth retardation and a birth weight of 2240 g was delivered at term by primary cesarean section to a 27-year-old primiparous woman. Fetal echocardiography had shown a nearly atretic tricuspid valve. Postnatally, the child stabilized rapidly but showed signs of heart failure with tachypnea and general weakness, as well as cyanosis (transcutaneous oxygen saturation, 85%). The presence of an aorto–right ventricular (RV) tunnel (ARVT) was confirmed by transthoracic echocardiography, which showed a 5 to 6×15–mm structure extending from the proximal ascending aorta to the outflow tract of the RV, with the left coronary artery originating from the aortic end of the tunnel. The ARVT caused a strong left-to-right shunt during diastole (Figure 1 and Movies I and II in the online-only Data Supplement). Figure 1. Echocardiographic views. A , Apical 4-chamber view. Arrow indicates the minimal tricuspid valve opening during diastole. B , Aorto–right ventricular tunnel (ARVT). C , Diastolic left-to-right shunt through the ARVT (arrow). RA …

Value of Tissue Doppler Echocardiography in Children with Pulmonary Hypertension

The impact of pulmonary hypertension (PHT) on right ventricular and left ventricular (LV) function in children with PHT is unknown, and echocardiographic data combining conventional and Doppler tissue imaging (DTI) on PHT in children are sparse. Forty-one children (18 male; mean age, 7.9 ± 5.6 years) with PHT and structurally normal hearts (27 with idiopathic PHT, 14 with associated PHT) and 44 age-matched healthy controls were assessed using conventional echocardiography and DTI. Children with PHT had enlarged tricuspid valve diameters, right atrial areas, pulmonary artery dimensions, and LV eccentricity indices. In addition, pulmonary acceleration time and tricuspid annular plane systolic excursion were significantly reduced in patients compared with controls. DTI revealed that children with PHT had significantly lower systolic (S) and early diastolic (E) velocities at the tricuspid and septal levels. Despite preserved LV ejection fractions, left lateral free wall systolic velocities were significantly reduced in patients with PHT. Significantly reduced LV rapid filling velocities (E) suggested an underloaded left ventricle or LV diastolic dysfunction in children with PHT compared with controls. Pulmonary acceleration time and tricuspid annular plane systolic excursion correlated best with DTI systolic tricuspid and septal velocities. Despite not being evident on conventional two-dimensional echocardiography, LV systolic performance appears to be impaired in children with PHT. Quantitative DTI assessment of ventricular function and ventricular-ventricular interactions in this setting might provide further insights into the mechanisms leading to end-stage PHT and may guide clinicians to optimize antifailure treatment.

327: Prenatal tricuspid valve size as a predictor of postnatal outcome in patients with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum

Severe pulmonary stenosis and atresia (PS, PAIVS) are cardiac anomalies in which right ventricular (RV) outflow is obstructed. Depending on in utero RV growth in the face of this obstruction, severe PS and PA/IVS may exhibit a spectrum of severity, requiring postnatal interventions including balloon pulmonary valvuloplasty alone, surgical biventricular repair, or staged single-ventricle palliation. Tricuspid valve (TV) size at birth has been shown to predict outcome with a TV Z-score <-3 associated with single-ventricle outcome, but prenatal echocardiographic features that might predict postnatal TV size (and outcome) have not been as well studied.Our objective was to determine whether prenatal TV size or other echo-derived cardiac variables can predict favorable postnatal TV diameter in fetuses with PS and PA/IVS.

The potential of disproportionate growth of tricuspid valve after decompression of the right ventricle in patients with pulmonary atresia and intact ventricular septa

Tricuspid valve size is the major determinant of outcomes for patients with pulmonary atresia with intact ventricular septum. Lack of right ventricle-pulmonary artery continuity is associated with poor tricuspid valve growth (decrement in Z-value). However, most reports did not show evidence for disproportionate growth of the tricuspid valve after establishment of right ventricle-pulmonary artery continuity. We studied 40 patients with pulmonary atresia with intact ventricular septum who underwent initial right ventricular decompression for planned staged repair. The initial Z-value of the tricuspid valve diameter (Zt1) was obtained from the echocardiography-derived normal value. The late Z-value (Zt2) was measured before definitive repair or the last available Z-value, if definitive repair was not yet reached. The factors associated with the changes of Z-values (Zt2 - Zt1) were analyzed. The mean initial tricuspid Z-value (Zt1) was -6.2 +/- 3.5. After treatment (Zt2), the mean Z-value was -6.0 +/- 3.4 (n = 34). Overall, the tricuspid Z-values did not change. Individually, the change in Z-value (Zt2 - Zt1) was larger than +2 in 11 (32%) patients and smaller than -2 in 6 (18%) patients. Increases in Z-value (Zt2 - Zt1) were significantly associated with right ventricular pressure/left ventricular pressure ratio measured after initial palliation (r = -0.54; P = .001) and the initial tricuspid valve Z-value (Zt1) (r = -0.40; P = .02). Disproportional growth of the tricuspid valve can occur, especially in patients with small tricuspid valves and lower right ventricular pressures after decompression. The findings support the possibility of neonates with small tricuspid valves undergoing biventricular repair after right ventricular decompression surgery.

Surgical strategy for pulmonary atresia with intact ventricular septum: initial management and definitive surgery

Pulmonary atresia with intact ventricular septum is difficult to manage, and the surgical procedures are associated with high mortality and morbidity. The lesion is morphologically heterogeneous, with varying degrees of right ventricular and tricuspid valve hypoplasia. Aberrations of the coronary circulation are common. A single approach is impractical because the wide spectrum of right heart morphology makes a uniform surgical approach impossible. Anatomical criteria, such as the degree of tricuspid valve or right ventricular hypoplasia or whether a right ventricular infundibulum is present, have been used to assign the infants to single-ventricular or biventricular repair tracks. If the patient is considered a candidate for biventricular repair, right ventricular decompression, either alone or combined with a systemic-pulmonary artery shunt, is performed, whereas only a systemic-pulmonary artery shunt is performed in a candidate for single-ventricular repair. Several authors reported that the Z-value of the tricuspid valve diameter could be a guide to the initial procedure; however, poor interobserver reproducibility and wide normal ranges are serious limitations. Definitive operations, including biventricular repair, one-and-a-half ventricular repair, or Fontan-type operation, are performed to eliminate cyanosis. The most important problem when planning a strategy for a definitive repair is the fact that right ventricular growth and its contribution to pulmonary flow is difficult to predict before the operation. The management protocol should be determined by the precisely estimated morphology of the right ventricle and tricuspid valve, and the adequacy of the protocol should be analyzed based on surgical outcomes.

Prediction of Therapeutic Strategy and Outcome for Antenatally Diagnosed Pulmonary Atresia/Stenosis With Intact Ventricular Septum

The therapeutic strategy for pulmonary atresia (PA) and severe pulmonary stenosis (PS) with intact ventricular septum is controversial. Recent improvements in prenatal detection necessitate the identification of predictors of outcome for appropriate counseling and prenatal management planning. Echocardiograms of 18 fetuses antenatally diagnosed with PA (n=14) and PS (n=4) were reviewed and the total cardiac dimension (TCD) and tricuspid valve diameter (TVD) were measured. The right ventricular end-diastolic volume (RVEDV) was calculated from the right ventriculogram of the neonatal period by the percentage of the predicted normal value (%RVEDV). There was a positive correlation between TVD/TCD and %RVEDV (p<0.001). As the initial treatment, balloon atrioseptostomy was performed in 13 cases of TVD/TCD<0.26. As the final treatment, patients with TVD/TCD<0.17 underwent or were planned for the Fontan procedure. Patients with TVD/TCD>0.21 underwent or were planned for biventricular repair. Patients whose TVD/TCD was between 0.17 and 0.21 underwent or were planned for 1.5 ventricular repairs. TVD/TCD is a useful index for selecting the postnatal initial treatment for PA/PS and to predict the final status of the fetus. Prenatal detection and prediction of the future status is helpful for family counseling. Furthermore, it will help to decide the postnatal management prenatally.

One-way valved patch repair in an adult with a hypoplastic right ventricle

Patients with congenital heart defects characterized by a hypoplastic right ventricle (RV) are at high risk for the development of postoperative RV failure. Although the usefulness of a 1-way valved patch 1 for biventricular repair of a hypoplastic RV 2 has been reported in pediatric patients, little is known about the effects of a 1-way valved patch in adult patients. Here we report biventricular repair in an adult patient by using a 1-way valved patch to reduce the risk of postoperative RV failure. Clinical Summary A 60-year-old man weighing 79 kg was given a diagnosis of a secundum atrial septal defect (ASD), severe pulmonary stenosis, and a hypoplastic RV. On admission, his oxygen saturation as measured by means of pulse oximetry was 80% at room air, and erythrocytosis (hematocrit level, 63%) was detected. Transesophageal echocardiography showed a secundum ASD of 15 mm with a right-to-left shunt and a tricuspid valve diameter of 33 mm (80% of the normal value). Angiocardiography revealed that the RV end-diastolic volume (index) was 91 mL (48.1 mL/m 2 ), and that the RV ejection fraction was 44%. The pulmonary valve diameter was 19 mm (87% of the normal value), with doming and calcification. The left ventricular end-diastolic volume (index) was 107 mL (57.2 mL/m 2 ), and the left ventricular ejection fraction was 65%. The cardiac index was 2.2 L · min 1 ·m 2 , and the Qp/Qs was 0.54. The left ventricular pressure was 171/9 mm Hg, and the

Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum

Background. Since 1991 we have performed a multistage palliative approach to biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum in infants with a detectable right ventricular infundibulum. Methods. A total of 25 patients (19 pulmonary atresia and 6 critical pulmonary stenosis) underwent initial palliation consisting of a transarterial pulmonary valvotomy and a polytetrafluoroethylene shunt between the left subclavian artery and pulmonary trunk. Among the 23 survivors, 15 underwent balloon valvotomy. Six of these patients later required additional palliative surgery that consisted of repeat pulmonary valvotomy, adjustment of an atrial communication, and resection of the hypertrophied muscles in the right ventricle. Results. Of the 25 patients, 23 (92%) survived. In all, 20 patients underwent definitive operations: 18 (90%) biventricular repair (12 pulmonary atresia, and 6 critical pulmonary stenosis), one bidirectional Glenn, and one Fontan procedure. The actuarial probability of achieving a biventricular repair at 36 months of age was 69%. In 18 patients right ventricular end–diastolic volume significantly increased but tricuspid valve diameter did not change. Conclusions. The multistage palliation procedure to promote right ventricular growth makes a definitive biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum possible in the majority of infants with a patent infundibulum.

Echocardiographic Determinants of Clinical Course in Infants With Critical and Severe Pulmonary Valve Stenosis

Objectives. The purposes of this study were to determine the growth pattern of the pulmonary valve (PV) annulus and right heart structures in patients with critical and severe pulmonary stenosis (PS) after balloon dilation, and to determine any morphometric or hemodynamic differences between cyanotic infants with critical PS and asymptomatic infants with severe PS that may account for their varied clinical presentations.Background. Growth of the PV annulus and right heart structures in patients with critical PS after balloon valvuloplasty has not clearly been defined. In addition, the anatomic and hemodynamic factors that determine whether an infant with severe PS will present with cyanosis or without symptoms are not well understood.Methods. Measurements of the PV annulus, tricuspid valve (TV) annulus and main, right and left pulmonary arteries were obtained from initial and follow-up echocardiograms, and Z values were calculated. Hemodynamic data and balloon pulmonary valvuloplasty techniques were reviewed. Right ventricular (RV) volumes were measured from angiograms.Results. Fourteen patients with critical PS (mean [±SD] age 0.21 ± 0.37 months) and 20 patients with severe PS (mean age 2.6 ± 2.9 months) were evaluated at presentation and at 32 ± 33 and 42 ± 32 months of follow-up, respectively. Balloon pulmonary valvuloplasty was successful in 64% of patients with critical PS and in 90% of patients with severe PS. The PV, TV and pulmonary arteries increased in size after balloon pulmonary valvuloplasty in both groups at a rate that paralleled or exceeded the rate of somatic growth. The initial TV diameter and RV volume were smaller in patients with critical PS than in those with severe PS (p < 0.05 and p < 0.0008, respectively).Conclusions. After balloon pulmonary valvuloplasty in infants with critical and severe PS, right heart structures increase in size at a rate that parallels or exceeds the rate of somatic growth. The primary morphometric differences between these groups are a smaller TV diameter and RV volume in infants with critical PS. This may contribute to increased right to left atrial shunting and account for the variations in clinical presentation.(J Am Coll Cardiol 1997;29:1095–101)© 1997 by the American College of Cardiology

New pediatric applications and techniques for balloon valvuloplasty: Tetralogy of Fallot, complex pulmonary stenosis/atresia, and pulmonary atresia with intact septum

Palliative pulmonary balloon valvuloplasty in Tetralogy of Fallot has been proposed to promote growth of the pulmonary valve so that it can be incorporated in a later repair without transannular patch to avoid the late complications from free pulmonary regurgitation. We have used the procedure in patients with severe hypoxemia less than 3 months old. Synopsis of 141 published cases and our own experience in 15 patients shows technical success in 93%, a complication rate of 5%, cyanotic spells in 9%, and a mortality of 0.6%. Arterial oxygen saturation instantly increased from 78 to 91%. Based on the intention to treat, emergent surgery was avoided in 65%. Despite a significant increase in pulmonary valve diameter, the incidence of transannular patch was reduced in only one study, partly reflecting the surgeon's preference. Palliative pulmonary balloon valvuloplasty can also promote pulmonary artery growth in other complex cyanotic lesions. In pulmonary valve atresia with intact ventricular septum without right ventricular dependent coronary circulation, catheter valvotomy and subsequent pulmonary balloon valvuloplasty can establish right ventricle to pulmonary artery continuity. Perforation of the atretic valve can be accomplished with a bare wire (rarely), with a hot tip laser wire or with a radiofrequency wire if available. We use a standard steerable 5 French electrode catheter to deliver radiofrequency pulses at 8–26 watt with subsequent dilatation using a 7–8 mm low profile balloon. If tricuspid valve diameter or right ventricular size are below normal, additional stenting of the ductus with a flexible stent expanded to 4–5 mm diameter should be performed. In patients with severe hypoplasia, stent placement across the outflow tract is necessary. With this strategy, overall outcome should be superior to the published data on 68 attempted catheter perforations: Out of the 74% technically successful procedures, only 47% remained without surgical intervention during early follow-up.

Pulmonary atresia with intact ventricular septum: Long-term results of “One and a Half Ventricular Repair”

Between 1982 and 1984, we successfully performed "one and a half ventricular repair" using a Glenn shunt for 3 patients with pulmonary atresia with intact ventricular septum. Here we review the 10-year follow-up results. In these patients, the preoperative Z scores of the tricuspid valve diameters ranged from -5.2 to -6.5. Right ventricular outflow tract reconstruction combined with a Glenn shunt were performed in all patients. Cardiac catheterization was done at least 10 years post-operatively. All 3 patients have maintained New York Heart Association functional class I status for more than 10 years. Angiography in 2 patients confirms sufficient left pulmonary artery pressure with pulsatile blood flow and good right ventricular contraction. A pulmonary arteriovenous fistula has developed in 1 patient. Although the lower limits of the tricuspid valve diameter for "one and a half ventricular repair" using a cavopulmonary shunt have not yet been determined, we successfully performed this procedure in 3 patients with severely hypoplastic right ventricles and tricuspid valve diameter Z scores of less than -5.0. The results up to 10 years postoperatively are acceptable.

Outcome measures for the neonatal management of pulmonary atresia with intact ventricular septum

The outcome in 135 patients with pulmonary atresia with intact ventricular septum was reviewed in terms of actuarial survival and "suitability" for eventual definitive repair (defined as having a tricuspid valve diameter larger than 2.4 standard deviations below the mean normal beyond the neonatal period). Of patients who underwent an initial closed valvotomy, 50% were dead, 22% were alive and suitable, and the remaining 28% were alive but unsuitable for definitive repair at 5 years. None of 66 patients who underwent primary shunt alone achieved suitability and 52% were dead at 5 years. No mode of neonatal closed valvotomy was consistently satisfactory: in only 10 of 26 survivors with serial measurements did the neonatal valvotomy alone achieve decompression to subsystemic pressures. Only half of the decompressed ventricles achieved growth of the tricuspid valve disproportionate to somatic growth as a result of the neonatal procedure. Strategies for the neonatal treatment of pulmonary atresia with intact ventricular septum should not rely on closed operations on the pulmonary valve to optimized right ventricular growth.

Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum.

Neonates with pulmonary atresia and intact ventricular septum (PA-IVS) are frequently born with hypoplastic right heart structures that must grow after right ventricular decompression (RVD) procedures for a complete two-ventricle physiology to be achieved. Previous authors have asserted that neonatal right heart size or morphology will predict right heart growth potential. Since 1983, our bias has favored early RVD regardless of initial right heart size. In 1986, we recognized a subset of patients with coronary artery abnormalities associated with poor outcome after RVD and have defined these patients as having a right ventricular-dependent coronary circulation (RVDCC). To assess the influence of right heart size on outcome independent of the presence of RVDCC, we measured echocardiographic right ventricular (RV) dimensions in 37 neonates with adequate studies presenting between 1983 and 1990. Coronary artery anatomy was adequately assessed by angiography in 36. RV volume and tricuspid valve (TV) diameter were significantly smaller in patients with RVDCC than in those without. However, there was no statistically significant association between RV volume or TV diameter and survival among patients with or without RVDCC: Among 29 patients without RVDCC, 23 of 24 (95.8%) who achieved RVD are alive compared with 1 of 5 (20%) who did not achieve RVD (P = .001). Twenty-one of the 23 survivors have a complete two-ventricle physiology with low right atrial pressure. Among 7 patients with RVDCC, 2 patients who underwent RVD died early of left ventricular failure, whereas 4 of 5 who did not undergo RVD have survived single ventricular palliation. Small right heart size is associated with RVDCC but is not associated with survival in PA-IVS. Patients without RVDCC have improved survival after RVD regardless of neonatal right heart size.

Morbid anatomy in neonates with Ebstein's anomaly of the tricuspid valve: Pathophysiologic and clinical implications

The hearts of six neonates with Ebstein's anomaly of the tricuspid valve who died in the 1st month of life were compared with hearts of six age- and size-matched control neonates. All six hearts had morphologically severe disease with gross right atrial dilation and marked apical displacement of the tricuspid valve. All had a secundum atrial septal defect and four had additional cardiac lesions (pulmonary atresia in two, ventricular septal defect in two).There was significant thinning of the right ventricular free wall distal to the tricuspid valve (3 ± 0.2 mm vs. control 4.2 ± 0.2, p < 0.01) and right ventricular fiber diameter was reduced (7.2 ± 0) μm vs. control 11.4 ± 0.6, p < 0.001). The fibrous tissue content of both right and left ventricular free walls was increased (right, 29.3 ± 2.6% vs. control 8.7 ± 1.1, p < 0.091; left, 23.2 ± 1.5% vs. control 8.5 ± 0.7%, p < 0.001).Although the right ventricular abnormalities might be explained by hemodynamic stress in utero, abnormalities of the left ventricular free wall suggest that either genetic or nonhemodynamic environmental factors are involved in the morphogenesis of this condition. Increased right and left ventricular fibrosis may contribute to the poor early outcome in this group and may predispose to late complications, such as subnormal exercise performance, hemodynamic deterioration or late sudden death that may occur in patients with Ebstein's anomaly who survive the neonatal period.

Pulmonary atresia and intact ventricular septum: Definitive repair in the neonatal period

Pulmonary atresia with an intact ventricular septum and a small right ventricle is associated with high mortality and lacks a consensus surgical approach. The results of operations in eight of eleven patients with either pulmonary atresia and an intact ventricular septum or critical pulmonary stenosis, hypoplastic right ventricle, and intact ventricular septum, who were operated on between 1983 and 1989, are presented. Definitive correction was performed via a right ventricular transannular patch with prolonged postoperative prostaglandin E1 infusion. Limiting conditions in using this approach were (1) severely hypoplastic right ventricle, (2) massive tricuspid regurgitation, or (3) right ventricle-dependent coronary artery blood supply. Eleven neonates had the aforementioned diagnoses; eight underwent definitive repair, five successfully. Successful outcome, up to 5 postoperative years, was achieved if the tricuspid valve diameter was greater than or equal to 0.75 cm, or if the tricuspid/mitral valve ratio was greater than or equal to 0.70. Other significant predictors of success were a tripartite right ventricle (p less than 0.006), lack of sinusoids (p less than 0.05), the ratio of the right ventricular internal and external diameters greater than or equal to 0.73 (p less than 0.05), and some contractility (p less than 0.04). Thus we choose a right ventricular transannular patch with long-term prostaglandin E1 infusion for patients with pulmonary atresia and intact ventricular septum if (1) they have a tricuspid valve diameter of 0.75 cm or 70% of the mitral valve size, (2) they have a tripartite right ventricle, (3) they exhibit some right ventricular contractility, (4) they do not have marked tricuspid valve insufficiency, and (5) the coronary arteries do not fill primarily from sinusoids.

Pulmonary atresia with intact ventricular septum: is neonatal repair advisable?

The optimal management of pulmonary atresia with an intact ventricular septum in the neonate remains controversial. The introduction of balloon septostomy and prostaglandin has significantly reduced early mortality but early surgical intervention is necessary to obtain a more adequate pulmonary blood flow. Fourteen neonates with pulmonary atresia and an intact ventricular septum were admitted to the Wessex Cardiothoracic Unit, Southampton from 1979 to 1986. Thirteen patients underwent cardiac catheterization. Cardiac catheterization data and right ventricular angiograms were reviewed retrospectively. Four patients with tripartite ventricles underwent total repair. The others received various palliative operations (valvotomy + modified Blalock-Taussig shunt or modified Blalock-Taussig shunt alone). Retrospective analysis of the angiograms indicated that right ventricular morphology alone is not a satisfactory criterion for surgical management. We have been able to demonstrate that there is a good correlation between the diameter of the tricuspid valve and the diameter of the infundibulum and that successful neonatal repair is possible when the tricuspid valve diameter is above 80% of the normal value for weight and when the tricuspid valve diameter to infundibular diameter ratio (TV/Inf ratio) is 2.2 or less. In patients with a tripartite ventricle but inadequate TV diameter and TV/Inf ratio, a closed pulmonary valvotomy with a modified Blalock-Taussig shunt remains the treatment of choice.