Absent Pulmonary Valve with Tricuspid Atresia or Severe Tricuspid Stenosis: Report of Three Cases and Review of the Literature

2000 ◽  
Vol 3 (4) ◽  
pp. 353-366 ◽  
Author(s):  
Silvio Litovsky ◽  
Michael Choy ◽  
Jeanny Park ◽  
Mark Parrish ◽  
Brenda Waters ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Pulmonary Valve ◽  
Left Ventricular ◽  
Tricuspid Atresia ◽  
Ventricular Free Wall ◽  
Ventricular Septum ◽  
Free Wall ◽  
Absent Pulmonary Valve ◽  
Right Ventricular Free Wall ◽  
The Right

Absence of the pulmonary valve occurs usually in association with tetralogy of Fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic.

Regional myocardial blood flow and oxygen delivery in fetal, newborn, and adult sheep

1982 ◽  
Vol 243 (5) ◽  
pp. H729-H731 ◽  
Author(s):  
D. J. Fisher ◽  
M. A. Heymann ◽  
A. M. Rudolph
Keyword(s):  
Blood Flow ◽  
Myocardial Blood Flow ◽  
Right Ventricular ◽  
Oxygen Delivery ◽  
Left Ventricular ◽  
Ventricular Free Wall ◽  
Regional Myocardial Blood Flow ◽  
Free Wall ◽  
Right Ventricular Free Wall ◽  
The Right

We measured and calculated their product, regional myocardial oxygen delivery, in unanesthetized, previously instrumented fetal, newborn, and adult sheep. In the fetus, blood flow and oxygen delivery were greater to the right ventricular free wall than to the left ventricular free wall. In the left ventricular free wall oxygen delivery increased significantly after birth and later decreased to a level in the adult that was similar to that of the fetus. There was a progressive decrease in oxygen delivery to the right ventricular free wall during the developmental period that we studied. Although the inner-to-outer blood flow ratio was significantly lower for the left and right ventricular free walls of the fetuses as compared with the newborns and adults, the ratio was greater than one in all three groups for both of the ventricular free walls. These data demonstrate that the changes that occur in the circulation after birth are associated with significant alterations in right and left ventricular myocardial blood flow and oxygen delivery, which most likely reflect changes in regional myocardial metabolic demands. In addition, there are further significant changes in regional myocardial blood flow during the transition from the newborn to adult hemodynamics.

Significant left ventricular contribution to right ventricular systolic function

1991 ◽  
Vol 261 (5) ◽  
pp. H1514-H1524 ◽  
Author(s):  
R. J. Damiano ◽  
P. La Follette ◽  
J. L. Cox ◽  
J. E. Lowe ◽  
W. P. Santamore
Keyword(s):  
Right Ventricular ◽  
Left Ventricular ◽  
Arterial Blood Flow ◽  
Ventricular Free Wall ◽  
Ventricular Pressure ◽  
Free Wall ◽  
Right Ventricular Free Wall ◽  
Arterial Blood ◽  
Pulmonary Arterial ◽  
The Right

To examine the importance of systolic ventricular interdependence on right ventricular function, we used a unique electrically isolated right ventricular free wall preparation. Double-peaked waveforms for right ventricular pressure and pulmonary arterial blood flow occurred over a wide range of pacing intervals between the left and right ventricles. One component of the waveforms could be directly related to right ventricular free wall contraction, whereas the other component was directly related to left ventricular and septal contraction. For left ventricular pressure, the left ventricular component was significantly larger than the right ventricular free wall component (92.7 +/- 3.2 vs. 7.3 +/- 3.2% peak-to-peak value, P less than 0.01). For right ventricular pressure, the left ventricular and septal component was significantly greater than the right ventricular component (63.5 +/- 10.9 vs. 36.5 +/- 10.9% peak-to-peak value, P less than 0.05). Similarly, for pulmonary arterial blood flow, the left ventricular component was significantly greater than the right ventricular component. When right ventricular free wall pacing stopped in diastole, 68 +/- 4% of right ventricular systolic pressure and 80 +/- 4% of pulmonary flow were obtained in the subsequent beat. The results of this study indicate that left ventricular contraction is very important for right ventricular developed pressure and volume outflow.

scholarly journals P1744 Motion and velocity of the different parts of the AV plane. Relation to site and wall length

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Stoylen ◽  
H E Molmen ◽  
H E Dalen
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Mitral Annulus ◽  
Left Ventricular ◽  
Individual Variability ◽  
Ventricular Free Wall ◽  
Free Wall ◽  
Right Ventricular Free Wall ◽  
Relative Variance ◽  
The Right

Abstract Background Displacement (MAPSE) and peak systolic velocity (S") of the various parts of the mitral annulus differ. Also, both indices are higher in the right ventricular free wall. We aimed to investigate the relations between wall motion, wall lengths and sites in the left ventricle, as well as comparing to the right ventricle to assess the influence on systolic deformation of the AV-plane. Methods The HUNT study examined 1266 subjects without evidence of heart disease from a mixed urban/rural population of North Trøndelag county, S", linear diastolic left ventricular annulus to apex distance (WL) and MAPSE was measured in the septal, anterior, lateral and inferior points of the mitral annulus. Intra individual variance and relative variance (variance / mean) of S" and MAPSE was calculated and compared to the same variance of the values normalised for wall length (S"/WL and MAPSE/WL). Results Wall lengths, velocities and MAPSE are shown in figure 1. Wall lengths were associated with both MAPSE and S". Both wall lengths and motion were greatest in the free walls of the LV, but smallest in the septal parts of the AVplane. Variances are shown in table 1. Even if intra-individual variances are larger for S" than MAPSE, relative variance for both decreased by 80 - 90% when normalised for wall length. Conclusion There is an intra-individual variability of MAPSE and S" of the LV, corresponding to WL, when the values normalised for WL most of the variability disappears. Intra individual variability of strain and strain rate per wall is less than MAPSE and S" as shown earlier. As the apex is located centrally, walls corresponding to the free walls are longer than those closest to the septum, thus motion and wall length are both greatest corresponding to the LV free walls. Motion of the right ventricular free wall was higher than the LV free wall; TAPSE 2.8 cm, and S" 12.6 cm/s as shown previously. The relation of TAPSE and S" of the right ventricle to wall length will be different, as the afterload in the RV is far less. But as both lateral parts of the AV plane move more than the centre, givinga systolic bending of the AV-planealso as the central part of the AV-plane is centrally anchored to the large arteries as shown in fig. 1. Table 1 WL (cm) MAPSE (cm) MAPSE / WL (% points) S" (cm/s) S" / WL (/s) Mean of 4 walls 9.5 1.6 0.16 8.4 0.87 Variance 0.04 0.04 0.0004 1.2 0.01 Relative variance (%) 2.8 2.7 0.25 12.1 1.4 Wall lengths (WL), displacement(MAPSE), Systolic velocity (S") and mAPSE and S" normalised for WL. Means and intra individual variances. Abstract P1744 Figure. Fig. 1

Differential regional gene expression from cardiac dyssynchrony induced by chronic right ventricular free wall pacing in the mouse

2006 ◽  
Vol 26 (2) ◽  
pp. 109-115 ◽  
Author(s):  
Kenneth C. Bilchick ◽  
Sudip K. Saha ◽  
Ed Mikolajczyk ◽  
Leslie Cope ◽  
Will J. Ferguson ◽  
...  
Keyword(s):  
Gene Expression ◽  
Right Ventricular ◽  
Differential Expression ◽  
Stem Cell Differentiation ◽  
Left Ventricular ◽  
Ventricular Free Wall ◽  
Free Wall ◽  
Right Ventricular Free Wall ◽  
Cardiac Dyssynchrony ◽  
Clinical Prognosis

Routine clinical right ventricular pacing generates left ventricular dyssynchrony manifested by early septal shortening followed by late lateral contraction, which, in turn, reciprocally stretches the septum. Dyssynchrony is disadvantageous to cardiac mechanoenergetics and worsens clinical prognosis, yet little is known about its molecular consequences. Here, we report the influence of cardiac dyssynchrony on regional cardiac gene expression in mice. Mice were implanted with a custom-designed miniature cardiac pacemaker and subjected to 1-wk overdrive right ventricular free wall pacing (720 beats/min, baseline heart rate 520–620 beats/min) to generate dyssynchrony (pacemaker: 3-V lithium battery, rate programmable, 1.5 g, bipolar lead). Electrical capture was confirmed by pulsed-wave Doppler and dyssynchrony by echocardiography. Gene expression from the left ventricular septal and lateral wall myocardium was assessed by microarray (dual-dye method, Agilent) using oligonucleotide probes and dye swap. Identical analysis was applied to four synchronously contracting controls. Of the 22,000 genes surveyed, only 18 genes displayed significant ( P < 0.01) differential expression between septal/lateral walls >1.5 times that in synchronous controls. Gene changes were confirmed by quantitative PCR with excellent correlations. Most of the genes ( n = 16) showed greater septal expression. Of particular interest were seven genes coding proteins involved with stretch responses, matrix remodeling, stem cell differentiation to myocyte lineage, and Purkinje fiber differentiation. One week of iatrogenic cardiac dyssynchrony triggered regional differential expression in relatively few select genes. Such analysis using a murine implantable pacemaker should facilitate molecular studies of cardiac dyssynchrony and help elucidate novel mechanisms by which stress/stretch stimuli due to dyssynchrony impact the normal and failing heart.

Ventricular systolic interdependence: volume elastance model in isolated canine hearts

1987 ◽  
Vol 253 (6) ◽  
pp. H1381-H1390 ◽  
Author(s):  
W. L. Maughan ◽  
K. Sunagawa ◽  
K. Sagawa
Keyword(s):  
Left Ventricle ◽  
Right Ventricle ◽  
Cross Talk ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Ventricular Free Wall ◽  
Free Wall ◽  
Right Ventricular Free Wall ◽  
Systolic Volume ◽  
The Right

To analyze the interaction between the right and left ventricle, we developed a model that consists of three functional elastic compartments (left ventricular free wall, septal, and right ventricular free wall compartments). Using 10 isolated blood-perfused canine hearts, we determined the end-systolic volume elastance of each of these three compartments. The functional septum was by far stiffer for either direction [47.2 +/- 7.2 (SE) mmHg/ml when pushed from left ventricle and 44.6 +/- 6.8 when pushed from right ventricle] than ventricular free walls [6.8 +/- 0.9 mmHg/ml for left ventricle and 2.9 +/- 0.2 for right ventricle]. The model prediction that right-to-left ventricular interaction (GRL) would be about twice as large as left-to-right interaction (GLR) was tested by direct measurement of changes in isovolumic peak pressure in one ventricle while the systolic pressure of the contralateral ventricle was varied. GRL thus measured was about twice GLR (0.146 +/- 0.003 vs. 0.08 +/- 0.001). In a separate protocol the end-systolic pressure-volume relationship (ESPVR) of each ventricle was measured while the contralateral ventricle was alternatively empty and while systolic pressure was maintained at a fixed value. The cross-talk gain was derived by dividing the amount of upward shift of the ESPVR by the systolic pressure difference in the other ventricle. Again GRL measured about twice GLR (0.126 +/- 0.002 vs. 0.065 +/- 0.008). There was no statistical difference between the gains determined by each of the three methods (predicted from the compartment elastances, measured directly, or calculated from shifts in the ESPVR). We conclude that systolic cross-talk gain was twice as large from right to left as from left to right and that the three-compartment volume elastance model is a powerful concept in interpreting ventricular cross talk.

Association of epicardial fat thickness with left ventricular diastolic function parameters in a community population

2021 ◽  
Author(s):  
Wei Ma ◽  
Baowei Zhang ◽  
Ying Yang ◽  
Litong Qi ◽  
Jin Zhou ◽  
...  
Keyword(s):  
Confidence Interval ◽  
Right Ventricular ◽  
Diastolic Function ◽  
Ventricular Free Wall ◽  
Free Wall ◽  
Right Ventricular Free Wall ◽  
Community Population ◽  
The Mean ◽  
The Right ◽  
The Relationship

Abstract Purpose We examined the relationship between EFT measured by echocardiography and LV diastolic function parameters in a Beijing community population. Methods We included 1004 participants in this study. Echocardiographic parameters including E and A peak velocity, the early diastolic velocities (e’) of the septal and lateral of mitral annulus using tissue doppler imaging, E/e’, and EFT, were measured. EFT1 was measured perpendicularly on the right ventricular free wall at end-diastole in the extension line of the aortic root. EFT2 was the maximum thickness measured perpendicularly on the right ventricular free wall at end-diastole. Multivariate linear regression was used to analyze the relationship between EFT and the mean e’ and E/e’. Results The mean age of the participants was 63.91 ± 9.02 years old (51.4% men). EFT1 and EFT2 were negatively correlated with e’ lat, e’ sep, and e’ mean (p < 0.05), and positively correlated with E/e’ lat, E/e’ sep, and E/e’ mean. Multivariate regression analysis showed that EFT1 and EFT2 were independently and negatively correlated with e’ mean (EFT1: β = −0.089 [95% confidence interval = − 0.177, − 0.000, p = 0.050]; EFT2: β = −0.078 [95% confidence interval = − 0.143, − 0.012, p = 0.020]). There were no interactions between EFT and any covariates, including age or heart groups, sex, BMI, or presence of hypertension, diabetes, or coronary heart disease in relation to LV diastolic dysfunction. Conclusions EFT was negatively and independently correlated with e’ mean, suggesting that more attention to this type of adipose fat is required for cardiovascular disease therapy.

Progressive right ventricular outflow tract fibrosis after repair of tetralogy of Fallot

2020 ◽  
Vol 30 (9) ◽  
pp. 1366-1367
Author(s):  
Gauri R. Karur ◽  
Wadi Mawad ◽  
Lars Grosse-Wortmann
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
T1 Mapping ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Ventricular Free Wall ◽  
Free Wall ◽  
Right Ventricular Free Wall ◽  
The Right

AbstractObjectives:The objective of this study was to determine the evolution of fibrosis over time and its association with clinical status.Methods:Children with repaired tetralogy of Fallot who had undergone at least two cardiac magnetic resonance examinations including T1 mapping at least 1 year apart were included.Results:Thirty-seven patients (12.7 ± 2.6 years, 61% male) were included. Right ventricular free wall T1 increased (913 ± 208 versus 1023 ± 220 ms; p = 0.02). Baseline cardiac magnetic resonance parameters did not predict a change in imaging markers or exercise tolerance. The right ventricular free wall per cent change correlated with left ventricular T1% change (r = 0.51, p = 0.001) and right ventricular mass Z-score change (r = 0.51, p = 0.001). T1 in patients with late gadolinium enhancement did not differ from the rest.Conclusion:Increasing right ventricular free wall T1 indicates possible progressive fibrotic remodelling in the right ventricular outflow tract in this pilot study in children and adolescents with repaired tetralogy of Fallot. The value of T1 mapping both at baseline and during serial assessments will need to be investigated in larger cohorts with longer follow-up.

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