Additional mechanism for left ventricular dysfunction: chronic pulmonary regurgitation decreases left ventricular preload in patients with tetralogy of Fallot

2017 ◽  
Vol 28 (2) ◽  
pp. 208-213 ◽  
Author(s):  
Pekka Ylitalo ◽  
Eero Jokinen ◽  
Kirsi Lauerma ◽  
Miia Holmström ◽  
Olli M. Pitkänen-Argillander
Keyword(s):  
Left Ventricle ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Left Ventricular Dysfunction ◽  
Ventricular Dysfunction ◽  
Pulmonary Valve ◽  
Pulmonary Regurgitation ◽  
Left Ventricular ◽  
Valve Annulus ◽  
Left Ventricular Preload

AbstractBackgroundRight ventricular dysfunction in patients with tetralogy of Fallot and significant pulmonary regurgitation may lead to systolic dysfunction of the left ventricle due to altered ventricular interaction. We were interested in determining whether chronic pulmonary regurgitation affects the preload of the left ventricle. In addition, we wanted to study whether severe chronic pulmonary regurgitation would alter the preload of the left ventricle when compared with patients having preserved pulmonary valve annulus.MethodsThe study group comprised 38 patients with tetralogy of Fallot who underwent surgical repair between 1990 and 2003. Transannular patching was required in 21 patients to reconstruct the right ventricular outflow tract. Altogether, 48 age- and gender-matched healthy volunteers were recruited. Cardiac MRI was performed on all study patients to assess the atrial and ventricular volumes and function.ResultsSevere pulmonary regurgitation (>30 ml/m2) was present in 13 patients, of whom 11 had a transannular patch, but only two had a preserved pulmonary valve annulus. The ventricular preload volumes from both atria were significantly reduced in patients with severe pulmonary regurgitation, and left ventricular stroke volumes (44.1±4.7 versus 58.9±10.7 ml/m2; p<0.0001) were smaller compared with that in patients with pulmonary regurgitation <30 ml/m2or in controls.ConclusionsIn patients with tetralogy of Fallot, severe pulmonary regurgitation has a significant effect on volume flow through the left atrium. Reduction in left ventricular preload volume may be an additional factor contributing to left ventricular dysfunction.

scholarly journals Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis

2021 ◽  
Author(s):  
Anjali Chelliah ◽  
Anita J. Moon‐Grady ◽  
Shabnam Peyvandi ◽  
Joanne S. Chiu ◽  
James E. Bost ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Left Ventricular Dysfunction ◽  
Ventricular Dysfunction ◽  
Pulmonary Valve ◽  
Right Ventricular Dysfunction ◽  
Left Ventricular ◽  
Absent Pulmonary Valve ◽  
Intention To Treat ◽  
Overall Mortality

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10‐year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow‐up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P =0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1–30.0; P =0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.

scholarly journals Mid- to long-term follow-up of pulmonary valve replacement with BioIntegral injectable valve

2020 ◽  
Author(s):  
Simone Ghiselli ◽  
Cristina Carro ◽  
Nicola Uricchio ◽  
Giuseppe Annoni ◽  
Stefano M Marianeschi
Keyword(s):  
Cardiopulmonary Bypass ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Valve Replacement ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
Pulmonary Regurgitation ◽  
Pulmonary Valve Replacement

Abstract OBJECTIVES Chronic pulmonary valve (PV) regurgitation is a common late sequela after repair of congenital heart diseases like tetralogy of Fallot or pulmonary stenosis, leading to right ventricular dilatation and failure and increased late morbidity and mortality. Timely reoperation may lead to a complete right ventricular recovery. An injectable PV allows pulmonary valve replacement, with or without cardiopulmonary bypass, under direct observation, thereby minimizing the impact of surgery on cardiac function. The aim of this study was to evaluate the feasibility and mid- to long-term clinical outcomes with this device. METHODS From April 2007 to October 2019, a total of 85 symptomatic patients with severe pulmonary regurgitation or pulmonary stenosis underwent pulmonary valve replacement with an injectable stented pulmonary prosthesis. Data were collected from the international proctoring registry. Mean patient age was 26.7 years. The underlying diagnosis was repaired tetralogy of Fallot in 69.4% patients; moderate or severe pulmonary regurgitation was present in 72.9%. All patients had echocardiographic scans before the operation and during the follow-up period. A total of 54.1% patients also had preoperative/postoperative cardiac magnetic resonance imaging (MRI) or catheterization; 25.9% had off-pump implants. In 53% patients, pulmonary valve replacement was associated with the repair of other cardiac defects. RESULTS Minor postoperative complications were observed in 10.8% patients. The overall mortality rate was 2.3%; mortality after valve replacement was linked to a severe cardiac insufficiency and it was not related to a prosthesis failure; 1 prosthesis was explanted from 1 patient because of endocarditis, and 6% of patients developed PV stenosis; minor complications occurred in 4.8%. The mean follow-up period was 4.8 years (2 months–12.7 years); 42% of the patients were followed for more than 5 years. Follow-up echocardiography and cardiac MRI showed a significant reduction in RV size and low gradients across the PV. CONCLUSIONS An injectable PV may be implanted without cardiopulmonary bypass and in a hybrid operating theatre with minimal surgical impact. The bioprosthesis, available up to large sizes, has a low profile, laminar flow and no risk of coronary artery compression. Incidence of endocarditis is rare. The lack of a suture ring permits the implant of a relatively larger prosthesis, thereby avoiding a right ventricular outflow tract obstruction. This device permits future percutaneous valve-in-valve procedures, if needed. Results concerning durability are encouraging, and mid- to long-term haemodynamic performance is excellent.

scholarly journals Altered biventricular hemodynamic forces in patients with repaired tetralogy of Fallot and right ventricular volume overload because of pulmonary regurgitation

2018 ◽  
Vol 315 (6) ◽  
pp. H1691-H1702 ◽  
Author(s):  
Pia Sjöberg ◽  
Johannes Töger ◽  
Erik Hedström ◽  
Per Arvidsson ◽  
Einar Heiberg ◽  
...  
Keyword(s):  
Blood Flow ◽  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Flow Direction ◽  
Pulmonary Regurgitation ◽  
Ventricular Volume ◽  
Left Ventricular ◽  
Hemodynamic Forces ◽  
Control Subjects ◽  
Repaired Tetralogy Of Fallot

Intracardiac hemodynamic forces have been proposed to influence remodeling and be a marker of ventricular dysfunction. We aimed to quantify the hemodynamic forces in patients with repaired tetralogy of Fallot (rToF) to further understand the pathophysiological mechanisms as this could be a potential marker for pulmonary valve replacement (PVR) in these patients. Patients with rToF and pulmonary regurgitation (PR) > 20% ( n = 18) and healthy control subjects ( n = 15) underwent MRI, including four-dimensional flow. A subset of patients ( n = 8) underwent PVR and MRI after surgery. Time-resolved hemodynamic forces were quantified using 4D-flow data and indexed to ventricular volume. Patients had higher systolic and diastolic left ventricular (LV) hemodynamic forces compared with control subjects in the lateral-septal/LV outflow tract ( P = 0.011 and P = 0.0031) and inferior-anterior ( P < 0.0001 and P < 0.0001) directions, which are forces not aligned with blood flow. Forces did not change after PVR. Patients had higher RV diastolic forces compared with control subjects in the diaphragm-right ventricular (RV) outflow tract (RVOT; P < 0.001) and apical-basal ( P = 0.0017) directions. After PVR, RV systolic forces in the diaphragm-RVOT direction decreased ( P = 0.039) to lower levels than in control subjects ( P = 0.0064). RV diastolic forces decreased in all directions ( P = 0.0078, P = 0.0078, and P = 0.039) but were still higher than in control subjects in the diaphragm-RVOT direction ( P = 0.046). In conclusion, patients with rToF and PR had LV hemodynamic forces less aligned with intraventricular blood flow compared with control subjects and higher diastolic RV forces along the regurgitant flow direction in the RVOT and that of tricuspid inflow. Remaining force differences in the LV and RV after PVR suggest that biventricular pumping does not normalize after surgery. NEW & NOTEWORTHY Biventricular hemodynamic forces in patients with repaired tetralogy of Fallot and pulmonary regurgitation were quantified for the first time. Left ventricular hemodynamic forces were less aligned to the main blood flow direction in patients compared with control subjects. Higher right ventricular forces were seen along the pulmonary regurgitant and tricuspid inflow directions. Differences in forces versus control subjects remain after pulmonary valve replacement, suggesting that altered biventricular pumping does not normalize after surgery.

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