The Impact of Chronic Pulmonary Regurgitation on the Right Ventricle: Pulmonary Stenosis vs. Tetralogy of Fallot

2008 ◽  
Vol 17 ◽  
pp. S198
Author(s):  
Rajesh Puranik ◽  
Phillip Lurz ◽  
Wendy Norman ◽  
Alessandra Frigiola ◽  
Phillip Bonhoeffer ◽  
...  
2014 ◽  
Vol 36 (4) ◽  
pp. 796-801 ◽  
Author(s):  
Laura Mercer-Rosa ◽  
Eitan Ingall ◽  
Xuemei Zhang ◽  
Michael McBride ◽  
Stephen Kawut ◽  
...  

2020 ◽  
Vol 73 (11) ◽  
pp. 2364-2369
Author(s):  
Veronika M. Dudnyk ◽  
Olha O. Zborovska ◽  
Yuilia V. Vyzhga ◽  
Vladymyr P. Popov ◽  
Valentyn S. Bakhnivskyi

The aim: To improve efficacy of the right ventricle functional condition evaluation in children with tetralogy of Fallot after surgical correction by estimation of instrumental markers of myocardial dysfunction. Materials and methods: We completely examined 35 children with tetralogy of Fallot after their surgical correction at the age of 3 – 17 years. For all the patients was presented tissue doppler. We evaluated peak myocardial velocities of right ventrical in different phases of the heart cycle (S, E`, A`), tricuspid annular plane systolic excursion (TAPSE), diastolic myocardial velocities ratio (E/E`), peak myocardial velocity during isovolumic contraction (IVV), isovolumic relaxation time (IVRT). Results: All children of the study group had pulmonary insufficiency of different severity with main predominance of mild pulmonary regurgitation (20 patients, 57,14±8,36 %). Children with tetralogy of Fallot after surgical correction were admitted with: decreased TAPSE up to 1,39±0,28 cm, decreased S` up to 8,00±1,90 cm/s, and decreased IVV up to 5,69±0,95 cm/s that is significantly lower results of the healthy children. Severe pulmonary regurgitation usually followed by high chances of the right ventricle systolic dysfunction, exactly with: decresed TAPSE<1,5 cm (OR=0,500; 95% CI 0,323 – 0,775), S`<8,1 cm/s (OR=0,600; 95% CI 0,420 – 0,858) and IVV<5,9 cm/s (OR=0,250; 95% CI 0,117 – 0,534). As well we admitted significant decline of the velocities in earl and end diastole periods to compare with the results of the control group (E`= 12,11±1,22, A`= 4,56±0,92 cm/s (Р=0,009 and P=0.0002)), boost of the E/E` ratio – 7,96±2,33 (P=0.01) and decline of the RV IVRT up to 43,49±6,04 ms (P=0.017). Severe pulmonary regurgitation followed by high chances of the right ventricle systolic dysfunction development with TAPSE <1,5 cm (OR=0,500; 95% CI 0,323 – 0,775), S`<8,1 cm/s (OR=0,600; 95% CI 0,420 – 0,858) and IVV<5,9 cm/s (OR=0,250; 95% CI 0,117 – 0,534). As well we noticed high chances of the E/E`ratio > 6,0 in 1,5 times (95% CI 1,072 – 1,903) and decreased E` <12,2 cm/s (OR=0,200; 95% CI 0,083 – 0,481). Conclusions: Apart of clinical symptoms of the heart failure in children with tetralogy of Fallot after surgical correction markers of the right ventricle myocardial dysfunction are presented by indices of myocardial velocities, received during tissue doppler in different phases of the heart cycle.


2010 ◽  
Vol 20 (S3) ◽  
pp. 107-112 ◽  
Author(s):  
Leo Lopez ◽  
Meryl S. Cohen ◽  
Robert H. Anderson ◽  
Andrew N. Redington ◽  
David G. Nykanen ◽  
...  

AbstractThe long-term outcome of patients with congenitally malformed hearts involving abnormal right ventricular morphology and haemodynamics is variable. In most instances, the patients are at risk for right ventricular failure, in part due to morphological differences between the right and left ventricles and their response to chronic volume and pressure overload. In patients after repair of tetralogy of Fallot, and after balloon valvotomy for valvar pulmonary stenosis, pulmonary regurgitation is the most significant risk factor for right ventricular dysfunction. In patients with a dominant right ventricle after Fontan palliation, and in those with systemic right ventricles in association with surgically or congenitally corrected transposition, the right ventricle is not morphologically capable of dealing with chronic exposure to the high afterload of the systemic circulation. In patients with Ebstein’s malformation of the tricuspid valve, the degree of atrialisation of the right ventricle determines how well the right ventricle will function as the pump for the pulmonary vascular bed.


PEDIATRICS ◽  
1951 ◽  
Vol 7 (2) ◽  
pp. 230-239
Author(s):  
DANIEL F. DOWNING ◽  
CHARLES P. BAILEY ◽  
ROBERT P. GLOVER

The surgical relief of pulmonary stenosis has been both direct and indirect. The indirect procedures have inherent dangers; the direct approach of Brock appears to be without these dangers and the circulation is allowed a more normal course. This approach entails an incision into the right ventricular chamber and the cutting of the stenosed pulmonary valve or the removal of obstructing tissue in the outflow tract of the right ventricle. Cyanosis is usually relieved immediately. The patient's exercise tolerance is significantly increased. Eight cases are presented.


Author(s):  
Simone Ghiselli ◽  
Cristina Carro ◽  
Nicola Uricchio ◽  
Giuseppe Annoni ◽  
Stefano M Marianeschi

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.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Rodriguez Garcia ◽  
A Pijuan Domenech ◽  
J Perez Rodon ◽  
B Benito Villabriga ◽  
J Francisco Pascual ◽  
...  

Abstract Introduction Patients with repaired tetralogy of Fallot (rTF) and severe pulmonary regurgitation frequently progress to dilation and dysfunction of the right ventricle (RV). It has been documented in the literature that there is a correlation between the duration of the QRS in the surface electrocardiogram and the hemodynamic parameters of the RV of these patients, suggesting the presence of a mechanical-electrical interaction. Purpose To determine if there is an association between the contraction delay in certain areas of the RV measured in M-mode echocardiography and the delay in electrical activation measured in the electroanatomic map (EAM) of RV in patients with rTF. Methods Unicentric and observational study of all patients with rTF undergoing EAM, echocardiography with study of RV asynchrony and cardiac magnetic resonance imaging (MRI). Activation delay in the antero-basal area and in the RV outflow tract (RVOT) in the EAM were both analysed (Figure 1A). The shortening delay in the same areas in M-mode echocardiography was also evaluated (Figure 1B, C). MRI data regarding volume and ejection fraction was also collected. Results 64 patients were included (36.7±10.6 years, 65% men). The mean total activation time of the RV (RV-TAT) was 127.3±42.4 ms. Activation mapping showed a recurrent pattern with beginning in the interventricular septum and ending in RV antero-basal region and/or RVOT. A linear positive correlation was observed between RV-TAT and the activation delay in both regions analysed (ρ=0.60 and ρ=0.52, respectively; p&lt;0.001) and also between the electrical and mechanical delay in the anterior wall (ρ=0.41; p=0.001). On the other hand, it was observed a negative correlation between RV ejection fraction (RVEF), measured on MRI, and the RV-TAT (ρ=−0.41, p=0.002) and also between RVEF and the activation delay in the RV antero-basal region and in the RVOT (ρ=−0.32, p=0.016 and ρ=−0.36, p=0.007, respectively). Conclusions There is a mechanical-electrical interaction in the RV of patients with rTF, with a negative correlation between the activation delay and RVEF and between mechanical and electrical activation delay in specific anatomical regions (regional mechanical-electrical interaction). These results may guide future studies on resynchronization in this heart disease. Figure 1. EAM and echocardiographic measures Funding Acknowledgement Type of funding source: None


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Atsuko Kato ◽  
Christian Drolet ◽  
Shi-Joon Yoo ◽  
Andrew Redington ◽  
Lars Grosse-Wortmann

Introduction: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well known. We hypothesized that unilaterally increased pulmonary vascular resistance (PVR), resulting from lung compression by the enlarged and levorotated heart leads to greater PR in the LPA. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: This is a single-center retrospective analysis of 50 magnetic resonance studies in patients after TOF repair. Patients with more than mild discrete branch pulmonary artery stenosis were excluded. Blood flow was measured by phase-contrast velocity encoding within the branch pulmonary arteries. On the axial image with the largest total cardiac surface area, cardiac angle (α) between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured (Figure). Results: There was no difference in LPA and RPA diameters. The LPA showed significantly less total forward flow (p=0.04), smaller net forward flow (p=<0.001), and greater RF (p=0.001) than the RPA. Left lung area was smaller than the right (p<0.001). RVEDVi correlated with LPA RF (R=0.48, p<0.001), but not with RPA RF. Larger RVEDVi correlated with a larger α angle (R=0.46, p<0.001), i.e. a more leftward cardiac axis and with smaller left lung area (R=-0.58, p<0.001). LPA RF, but not RPA RF, correlated inversely with left lung area indexed to the left hemithorax area (R=-0.34, p=0.02). Conclusions: An enlarged and levorotated heart - as a result of PR - is associated with smaller left lung size, and augments diastolic flow reversal in the LPA, presumably via increased left PVR. By imposing a further volume load on the RV, LPA regurgitation may thus close a positive feed-back loop of PR and RV dilatation.


2015 ◽  
Vol 46 (3) ◽  
pp. 832-842 ◽  
Author(s):  
Emmy Manders ◽  
Silvia Rain ◽  
Harm-Jan Bogaard ◽  
M. Louis Handoko ◽  
Ger J.M. Stienen ◽  
...  

Pulmonary arterial hypertension (PAH) is a fatal lung disease characterised by progressive remodelling of the small pulmonary vessels. The daily-life activities of patients with PAH are severely limited by exertional fatigue and dyspnoea. Typically, these symptoms have been explained by right heart failure. However, an increasing number of studies reveal that the impact of the PAH reaches further than the pulmonary circulation. Striated muscles other than the right ventricle are affected in PAH, such as the left ventricle, the diaphragm and peripheral skeletal muscles. Alterations in these striated muscles are associated with exercise intolerance and reduced quality of life. In this Back to Basics article on striated muscle function in PAH, we provide insight into the pathophysiological mechanisms causing muscle dysfunction in PAH and discuss potential new therapeutic strategies to restore muscle dysfunction.


2013 ◽  
Vol 4 (3) ◽  
Author(s):  
Alice I. Supit ◽  
Erling D. Kaunang

Abstract: Congenital heart disease is a structural defect due to the malformation of the heart, aorta, and or great blood vessels. It is the most frequent congenital malformation in newborn babies. Tetralogy of Fallot is one of the congenital heart diseases (CHD) with central cyanosis, and covers 5-10% of all CHD. We reported a boy of one year old with Tetralogy of Fallot and pulmonal atresia (ToF-PA), associated with bronchopneumonia. The diagnosis was based on anamnesis, physical examination, and other supporting examinations. The chest X-ray showed a normal sized heart (CTR 57%) with coer-en-sabot shape, and right and left parahilar infiltration, which resulted in bronchopneumonia and ToF. The electrocardiography showed a right deviation of axis and a hypertrophy of the right ventricle; the echocardiography showed a right ventricle hypertrophy, an over-riding aorta, a large malalignment of the ventricular septal defect, no visualization of pulmonar artery, and no visualization of patent ductus arteriosus (PDA). Conclusion: Based on all the tests performed, the diagnosis of this patient was Tetralogy of Fallot and pulmonal atresia (ToF-PA), associated with bronchopneumonia. The prognosis related to bronchopneumonia in this case was good due to the use of antibiotics. Keywords: tetralogy of Fallot, pulmona atresia, bronchopneumonia.  Abstrak: Penyakit jantung bawaan (PJB) ialah kelainan struktural akibat malformasi jantung, aorta dan atau pembuluh darah besar, dan merupakan kelainan kongenital tersering pada bayi baru lahir. Tetralogi Fallot merupakan salah satu PJB dengan sianosis sentral, dan mencakup 5-10% dari seluruh PJB. Kami melaporkan kasus seorang anak laki-laki berusia satu tahun dengan Tetralogi Fallot dan atresia pulmonal (ToF-PA) disertai bronkopneumonia. Diagnosis ditegakkan melalui anamnesis, pemeriksaan fisik, dan pemeriksaan penunjang. Hasil ekspertisi foto toraks AP memperlihatkan ukuran jantung normal (CTR 57%) berbentuk coer-en-sabot, dan pada paru-paru terlihat infiltrat parahilar kanan dan kiri serta corakan vaskular paru berkurang yang menunjukkan suspek bronkopneumonia dan ToF. Elektrokardiografi memperlihatkan deviasi aksis ke kanan dan hipertrofi ventrikel kanan, dan pada ekokardiografi tampak right ventricle hypertrophy, overriding aorta, VSD malalignment besar, tidak tampak visualisasi arteri pulmonal, dan tidak tampak patent ductus arteriosus (PDA) dengan hasil Tetralogi Fallot dan atresia pulmonal. Simpulan: Berdasarkan hasil pemeriksaan yang dilakukan, diagnosis pasien ini ialah Tetralogi Fallot dan atresia pulmonal (ToF-PA) disertai bronkopneumonia. Prognosis bronkopenumonia pada kasus ini baik yang dapat diatasi dengan antibiotika.Kata kunci: tetralogi Fallot, atresia pulmonal, bronkopneumonia.  


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