Natural history of congenital pulmonary valvar stenosis: an echo and Doppler cardiographic study

1999 ◽  
Vol 9 (2) ◽  
pp. 129-135 ◽  
Author(s):  
Hanneke Gielen ◽  
Otto Daniëls ◽  
Henk van Lier
Keyword(s):  
Right Ventricle ◽  
Pressure Gradient ◽  
Pulmonary Valve ◽  
Anterior Wall ◽  
Jet Stream ◽  
Significant Relation ◽  
Severe Stenosis ◽  
History Of ◽  
The Right ◽  
Pulmonary Valvar Stenosis

AbstractDoppler echocardiography allows accurate serial assessment of pulmonary valvar stenosis by measuring the velocity of the jet stream through the pulmonary valve. Between 1979 and 1997, we saw 174 patients with isolated pulmonary valvar stenosis. At admission their ages ranged from 9 days to 22.5 years. We measured the velocity over the pulmonary valve, and the thickness of the anterior wall of the right ventricle, and made a study of their electrocardiograms. We found that rapid increases and decreases occurred in almost every age-group. For patients with a trivial, mild or moderate level of stenosis, severe stenosis developed in 3, 10 and 9%, respectively. In most of the patients, 122 (90%), in whom there was more than one examination, a change in pressure gradient between −12mmHg/year and +3mmHg/year was found. Only 7 patients had an increase of more than 10mmhg per year. In contrast with our patients having aortic stenosis, these with stenosis of the pulmonary valve showed no rapid increase in early childhood. Indeed, in 58% the severity of the stenosis decreased. No correlation was found when comparing the echocardiographic measurements of the thickness of the anterior wall of the right ventricle with the voltages on the electrocardiogram. A significant relation was found however, between an increasing pressure gradient and thickened valvar leaflets (p = 0.017).

Determinants for outcome of hypoplastic right ventricle with duct-dependent pulmonary blood flow presenting in the neonatal period

1992 ◽  
Vol 2 (4) ◽  
pp. 377-381 ◽  
Author(s):  
Michael L. Rigby ◽  
Micelia Salgado ◽  
Celia Silva
Keyword(s):  
Blood Flow ◽  
Right Ventricle ◽  
Pulmonary Blood Flow ◽  
Pulmonary Valve ◽  
Pulmonary Atresia ◽  
Cross Sectional ◽  
Mitral Valves ◽  
Significant Difference ◽  
The Right ◽  
Pulmonary Valvar Stenosis

SummaryA retrospective study was undertaken of patients with hypoplastic right ventricles, either with pulmonary atresia and intact ventricular septum or critical pulmonary valvar stenosis, and duct-dependent pulmonary blood flow who were investigated at the Royal Brompton Hospital between January 1976 and December 1990. The diagnosis was made on the basis of at least one diagnostic method (cross-sectional echocardiography, cardiac catheterization and angiography, or autopsy). Of the patients, 56 (82%) were found to have an imperforate pulmonary valve or infundibulum (pulmonary atresia), while 12(18%) had critical pulmonary valvar stenosis. The ratio of the diameters of the tricuspid and mitral valvar orifices was measured angiographically during diastole, and the right ventricle was analyzed according to the presence or overgrowth of the inlet, apical trabecular and outlet components. A correlation was made between the severity of the disease and the outcome. The overall mortality was 53% when those not undergoing any surgery were excluded, and significant differences were found between the group dying and those who survived. The incremental risk factors for death were a ratio between the diameter of the tricuspid and mitral valves of less than 0.6; the presence of fistulous communications with the coronary arteries; and obliteration of the apical trabecular component of the right ventricle. There was no significant difference between the group with pulmonary valvar atresia and the group with critical stenosis of the pulmonary valve.

Variability of the diameter of the pulmonary outflow tract and its relation to balloon valvoplasty for congenital pulmonary valvar stenosis

1993 ◽  
Vol 3 (2) ◽  
pp. 111-117
Author(s):  
Roxy N. S. Lo ◽  
Maurice P. Leung ◽  
T. C. Yung ◽  
C. H. Chan
Keyword(s):  
Right Ventricle ◽  
Pressure Gradient ◽  
Right Ventricular ◽  
Cardiac Cycle ◽  
Outflow Tract ◽  
Pulmonary Trunk ◽  
Initial Ratio ◽  
The Mean ◽  
The Right ◽  
Pulmonary Valvar Stenosis

SummaryThe diameter of the pulmonary outflow tract was measured from the right ventricular cineangiogram at different phases of the cardiac cycle prior to balloon valvoplasty in 54 patients with congenital pulmonary valvar stenosis. Measured at the level of the hinge point of the valvar leaflets, the pulmonary outflow tract was found to be largest at end-diastole and smallest at end-systole. The mean increase in diameter from systole to diastole was 25.3% (range 6–53%) for 47 patients with typical valvar stenosis, and 24.3% (range 14–41%) for seven patients with dysplastic valvar leaflets (p>0.5). Dilatation using balloons less than 1.2 times the size of the maximal end-diastolic diameter produced a mean reduction in the pressure gradient from right ventricle to pulmonary trunk of 71.1%, compared to 76.2% with bigger balloons (p>0.3). Two patients with an initial ratio between balloon and outflow tract of 0.9 required a second dilatation. The timing of measurement of the diameter of the pulmonary outflow tract should be standardized. Oversized balloons probably are not necessary for pulmonary valvoplasty.

scholarly journals Late follow-up of Persistent Truncus Arteriosus, after one-stage Repair, with Right Ventricular Remodeling.

2021 ◽  
Vol 4 (5) ◽  
pp. 01-04
Author(s):  
Miguel Maluf
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Ventricular Remodeling ◽  
Surgical Repair ◽  
Pulmonary Valve ◽  
Truncus Arteriosus ◽  
Persistent Truncus Arteriosus ◽  
The Right ◽  
Right Ventricular Remodeling

We report long-term outcome after one-stage, surgical repair, in a two months-old girl with persistent truncus arteriosus type I, II. The operation was carried out with the remodeling of the right ventricle, using a swine bicuspid pulmonary prosthesis. Twenty-six years later, the patient is in excellent clinical condition, CF I (NYHA), with normal peripheral oxygen saturation. Recent invasive and not invasive imaging show: absence of intracardiac shunt and growing of the right ventricle outlet tract and discrete double lesion of the pulmonary valve. The pulmonary flow directed uniformly for both lungs. In selected cases, the long-term prognosis of patients with persistent truncus arteriosus, undergoing early surgical repair, avoiding the use of valved conduit, makes for an excellent evolution, without new interventions. Endovascular procedures, now well standardized, for the implantation of a pulmonary valve stent, through a catheter, will allow an effective approach, in the presence of late obstructions, in patients who have undergone right ventricular remodeling, without the use of valved conduits.

scholarly journals The study of systemic hemodynamic disorders of patients with varicose and post-thrombotic diseases

2020 ◽  
Vol 19 (2) ◽  
pp. 32-37
Author(s):  
I. N. Shanaev
Keyword(s):  
Blood Flow ◽  
Right Ventricle ◽  
Right Atrium ◽  
Ultrasound Examination ◽  
Anterior Wall ◽  
Right Heart ◽  
Normal Limits ◽  
Flow Through ◽  
The Right ◽  
Restrictive Type

Aim. Study of heart function in the patients with CVD. Materials and methods. 46 patients with varicosity (VD) and 34 patients with post-thrombotic disease (PTD) were examined; the control group was represented by 15 healthy volunteers. The diagnosis was established using the CEAP basic classification. The study did not include patients with a diagnosed arterial hypertension, diabetes mellitus, chronic lung disease, significant hemodynamic heart defects, coronary heart disease. Ultrasound examination of the heart and veins of the lower extremities was performed on a Saote My Lab Alpha, Acuson Sequoia 512 apparatus. In addition to the standard protocol of heart ultrasound examination, the parameters of the right heart were calculated: sizes of the right ventricle (RV), right atrium, thickness of the anterior wall of the pancreas; to assess the ejection fraction (EF) of the pancreas the mobility of the lateral edge of the tricuspid ring was calculated, and the pressure on the tricuspid valve (TV) was measured. Diastolic ventricular function was studied by spectrograms of tricuspid and mitral blood flow. Results. Most of the indicators of cardiac activity in patients with VD were within normal limits, but a tendency to increase increasing of the right heart size was noted. In addition, the thickness of the interventricular septum and the right ventricle (RV) anterior wall was found to increase from 0.8 to 1.1 cm and from 0.3 to 0.5 cm, respectively, according clinical classes from C2 to C6 (CEAP). Eject fraction (EF) of both the RV and the left ventricle (LV) were also within normal limits, but with a tendency to decrease (67.8 % – C2, to 62 % – C6). The growth of the clinical class is followed by the increasing of percentage of non-restrictive blood flow through the tricuspid valve (TV). The restrictive type of blood flow in patients with VD had not been identified. Patients with PTD also showed a tendency to increase the right heart. However, whereas the size of the RV, as a rule, did not exceed 3.0 cm, the size of the right atrium was slightly higher than normal one in the clinical class C4 and C5.6. All the patients had EF of LV within normal limits, but it slightly decreased by the growth of class. Only patient classes C3 and C4 had EF of RV within the normal range. The 18 % of patient class C5.6 had EF lower than normal with value 48%. Diastolic dysfunction (DD) of the RV was detected in 73.3% of patients with class C3 and 100% with classes C4 and C5.6. Moreover, a restrictive type of blood flow through TV appeared from class C4 and the percentage increased up to 27.2% (class C5,6). Conclusions. DD of the RV was the main hemodynamic disorder.

CLINICAL CONFERENCE

PEDIATRICS ◽  
1957 ◽  
Vol 19 (6) ◽  
pp. 1139-1147
Author(s):  
Mary Allen Engle
Keyword(s):  
Blood Flow ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Pulmonary Blood Flow ◽  
Ventricular Hypertrophy ◽  
Pulmonary Valve ◽  
Muscular Tissue ◽  
Pulmonic Stenosis ◽  
Infundibular Stenosis ◽  
The Right

Dr. Engle: When pulmonic stenosis occurs as an isolated congenital malformation of the heart, it usually is due to fusion of the valve cusps into a dome with a small hole in the center. In Figure 1 the pulmonary artery has been laid open so that one can see the three leaflets of the pulmonary valve are completely fused, and that there is only a small, central, pinpoint opening which permits blood to leave the right ventricle and enter the pulmonary circulation. Valvular pulmonic stenosis is much more common than subvalvular or infundibular stenosis, where the obstruction to pulmonary blood flow lies within the substance of the right ventricle. There it may be due to a diaphragm of tissue which obstructs the outflow of the right ventricle, or to an elongated narrow tunnel lined with thickened endocardium, or to a ridge of fibrous or muscular tissue just beneath the pulmonary valve. The changes in the cardiovascular system which result from obstructed pulmonary blood flow are so characteristic that they permit the ready recognition of this condition. Proximal to the constriction, these changes manifest the burden placed on the right ventricle, which enlarges and hypertrophies. On physical examination this is demonstrated by the precordial bulge and tapping impulse just to the left of the sternum, where the rib cage overlies the anterior (right) ventricle. Radiographically, both by fluoroscopy and in roentgenograms in the frontal and both oblique views, right ventricular enlargement is seen. In the electrocardiogram, the precordial leads show a pattern of right ventricular hypertrophy.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle

2019 ◽  
Vol 30 (1) ◽  
pp. 126-128
Author(s):  
Cheul Lee ◽  
Kyung Min Kim ◽  
Jae Young Lee ◽  
Jihong Yoon
Keyword(s):  
Right Ventricle ◽  
Pulmonary Valve ◽  
Tricuspid Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Absent Pulmonary Valve ◽  
Cavopulmonary Anastomosis ◽  
Complete Exclusion ◽  
Bidirectional Cavopulmonary Anastomosis ◽  
The Right

AbstractTricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

scholarly journals Double-chambered Right Ventricle after Ventricular Septal Defect Operation Presenting as Syncope

2019 ◽  
Vol 21 (1) ◽  
pp. 65-66
Author(s):  
Sang-Hoon Seol
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Short Distance ◽  
Septal Defect ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Anomalous Muscle ◽  
Moderator Band ◽  
The Right

Double-chambered right ventricle (DCRV) is a cardiac disease of the right ventricular outflow tract obstruction characterized by anomalous muscle bundles that divide the right ventricle into two chambers. It may be also develop over time as an acquired lesion in patients with an abnormally short distance between the moderator band and the pulmonary valve. This report highlights the case of a man with double-chambered right ventricle after ventricular septal defect operation, who presented with syncope J MEDICINE JAN 2020; 21 (1) : 65-66

Echocardiographic features of the morphologically right ventriculo-arterial junction

2005 ◽  
Vol 15 (S1) ◽  
pp. 17-26 ◽  
Author(s):  
Richard M. Martinez ◽  
Robert H. Anderson
Keyword(s):  
Right Ventricle ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Pulmonary Trunk ◽  
Free Standing ◽  
Ventricular Mass ◽  
Echocardiographic Evaluation ◽  
The Right ◽  
Relationship Of

In the normal heart, the morphologically right ventricle supports the pulmonary trunk. The key to echocardiographic evaluation of the junction between these structures is to understand not only the arrangement of the pulmonary valve, but also the complete muscular infundibulum that supports the valvar leaflets, lifting the valvar complex away from the base of the ventricular mass. As explained in the previous review,1 it is the presence of this free-standing muscular infundibular sleeve that makes it possible for the surgeon to remove the pulmonary valve for use as an allograft in the Ross procedure.2 In this review, we will address the echocardiographic features of the junction, considering primarily the situation in which the morphologically right ventricle supports the pulmonary trunk, but making comparisons with the abnormal arrangements in which either the aorta, or both arterial trunks, arise from the right ventricle. As we will see, the basic arrangement of the free-standing complete muscular infundibulum, or conus, is preserved with these abnormal arrangements, but there can be variation depending on the precise arrangement of the inner heart curvature, or ventriculo-infundibular fold, and the morphology can be further changed depending on the relationship of the arterial trunks themselves. The key to analysis, therefore, and also to accurate description, is to analyse separately the way in which the arterial trunks are joined to the ventricular mass, the relationships of the trunks one to the other, and the precise structure of the supporting right ventricular outflow tract, or outflow tracts in the setting of double outlet connection. If each of these features is then described in its own right, avoiding the temptation to make inferences regarding one feature from knowledge of another, then it is possible to avoid many of the persisting controversies relating to nomenclature.

scholarly journals Fungal Pulmonary Valve Endocarditis Masquerading as a Pulmonary Embolism

2015 ◽  
Vol 2015 ◽  
pp. 1-2 ◽  
Author(s):  
Kevin B. Ricci ◽  
Peter H. U. Lee ◽  
Michael Essandoh ◽  
Ahmet Kilic
Keyword(s):  
Pulmonary Embolism ◽  
Right Ventricular ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Failure To Thrive ◽  
Ventricular Outflow Tract ◽  
Outflow Obstruction ◽  
Ventricular Outflow Obstruction ◽  
History Of ◽  
The Right

Septic pulmonary emboli (SPE) can be a difficult clinical entity to distinguish from thromboembolic pulmonary embolism (TPE) in a patient with history of IV drug abuse (IVDA). We present a case of a patient who presented with failure to thrive and presumed diagnosis of recurrent PE that ultimately was discovered to have fungal pulmonary valve endocarditis resulting in a right ventricular outflow obstruction. This required replacement of the pulmonary valve and repair of the right ventricular outflow tract. This case highlights difficulty in differentiating pulmonary valve endocarditis with septic emboli from chronic PE in a patient with a complex medical history.

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