Systemic hypertension in an infant with unrepaired tetralogy of Fallot: case report

2012 ◽  
Vol 23 (5) ◽  
pp. 746-748
Author(s):  
Michael Khoury ◽  
Michael Kallile ◽  
Joseph May ◽  
Rajesh Punn
Keyword(s):  
Blood Flow ◽  
Case Report ◽  
Tetralogy Of Fallot ◽  
Pulmonary Blood Flow ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Flow Dynamics ◽  
Systemic Hypertension ◽  
Pulmonary Valve Stenosis

AbstractPatients with severe right ventricular outflow tract obstruction in tetralogy of Fallot typically have right-to-left shunting, resulting in low pulmonary blood flow and cyanosis. Here we present the case of an infant with tetralogy of Fallot and severe pulmonary valve stenosis, complicated by systemic hypertension, the presence of which altered flow dynamics and possibly prevented cyanosis.

scholarly journals Tetralogy of Fallot with Absent Pulmonary Valve - A Case Report

1970 ◽  
Vol 6 (1) ◽  
pp. 48-50
Author(s):  
Md Mazibur Rahman
Keyword(s):  
Heart Failure ◽  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Absent Pulmonary Valve ◽  
Transannular Patch ◽  
Patch Closure ◽  
Successful Repair

A 25 years male patient of Tetralogy of Fallot (TOF) with congenital absent of pulmonary valve (APV) presented with symptoms of palpitation and exertional respiratory distress without congestive heart failure. He underwent successful repair of intracardiac defects. The procedures consisted of patch closure of ventricular septal defect and right. ventricular outflow tract reconstruction with a monocusp transannular patch. Resection or plication of dilated pulmonary artery was not required. The patient is doing well without any symptoms. Key words: Tetralogy of Fallot; Absent pulmonary valve DOI: 10.3329/uhj.v6i1.7197University Heart Journal Vol.6(1) 2010 pp.48-50

The current strategy of repair of tetralogy of Fallot in children and adults

2008 ◽  
Vol 18 (6) ◽  
pp. 608-614 ◽  
Author(s):  
Guo-Wei He ◽  
Xiao-Cheng Liu ◽  
Xiang-Rong Kong ◽  
Li-Xin Liu ◽  
Ying-Qun Yan ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Right Ventricular Function ◽  
Right Atrium ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Pulmonary Insufficiency ◽  
The Right

AbstractObjectivesThe strategies of repair of tetralogy of Fallot change with the age of patients. In children older than 4 years and adults, the optimal strategy may be to use different method of reconstruction of the right ventricular outflow tract from those followed in younger children, so as to avoid, or reduce, the pulmonary insufficiency that is increasingly known to compromise right ventricular function.MethodsFrom April, 2001, through May, 2008, we undertook complete repair in 312 patients, 180 male and 132 female, with a mean age of 11.3 years ±0.4 years, and a range from 4 to 48 years, with typical clinical and morphological features of tetralogy of Fallot, including 42 patients with the ventriculo-arterial connection of double outlet right ventricle. The operation was performed under moderate hypothermia using blood cardioplegia. The ventricular septal defect was closed with a Dacron patch. When it was considered necessary to resect the musculature within the right ventricular outflow tract, or perform pulmonary valvotomy, we sought to preserve the function of the pulmonary valve by protecting as far as possible the native leaflets, or creating a folded monocusp of autologous pericardium.ResultsThe repair was achieved completely through right atrium in 192, through the right ventricular outflow tract in 83, and through the right atrium, the outflow tract, and the pulmonary trunk in 36 patients. A transjunctional patch was inserted in 169 patients, non-valved in all but 9. There were no differences regarding the periods of aortic cross-clamping or cardiopulmonary bypass. Of the patients, 5 died (1.6%), with no influence noted for the transjunctional patch. Of those having a non-valved patch inserted, three-tenths had pulmonary regurgitation of various degree, while those having a valved patch had minimal pulmonary insufficiency and good right ventricular function postoperatively, this being maintained after follow-up of 8 to 24-months.ConclusionsBased on our experience, we suggest that the current strategy of repair of tetralogy of Fallot in older children and adults should be based on minimizing the insertion of transjunctional patches, this being indicated only in those with very small ventriculo-pulmonary junctions. If such a patch is necessary, then steps should be taken to preserve the function of the pulmonary valve.

Abstract 17390: Pre-Operative Right Ventricular Outflow Tract and Pulmonary Artery Geometry Predicts Pulmonary Valve Replacement Outcomes in Patients With Tetralogy of Fallot

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Veronica Toro Arana ◽  
Frandics Chan ◽  
Nicole Shiavone ◽  
Doff McElhinney ◽  
Sushma Reddy ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Valve Replacement ◽  
Prosthetic Valve ◽  
Pulmonary Valve ◽  
Pulmonary Arteries ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Pulmonary Valve Replacement ◽  
Valve Dysfunction

Introduction: Patients with Tetralogy of Fallot who had pulmonary valve replacement (PVR) are at risk for prosthetic valve failure that requires repeated valve replacement. Hypothesis: We hypothesize that the pre-operative geometry of the right ventricular outflow tract (RVOT) and the central pulmonary arteries is a predictor of future prosthetic valve dysfunction. Methods: In a retrospective study, using pre-operative cardiac MRI, we measured morphologic parameters including bifurcation angles, length, major and minor diameters, area, and circumference in various locations along the RVOT, pulmonary trunk (PT) and branch pulmonary arteries (BPAs) in 48 patients with Tetralogy of Fallot before they underwent PVR. Physiologic data was collected from their imaging reports (age, weight, height, body surface area (BSA), ventricular volumes and ejection fractions, valvular regurgitant fractions). All measurements were normalized by the patients’ BSA. Post-operative pulmonary valve function was assessed using Echocardiograms performed at an average of 5.5 years after the surgery. Valve dysfunction was defined as pulmonary regurgitation and/or pulmonary stenosis of at least moderate intensity. All geometric and physiologic parameters were compared between the group of patients who developed pulmonary valve dysfunction and those who did not, using a two-tailed Student t-test. Results: Patients who developed valve dysfunction had (1) greater RVOT circumference (p=0.038), (2) a more acute bifurcation angle between the PT and the Left Pulmonary Artery (p=0.016), and (3) smaller cross-sectional area at the distal BPAs (p=0.031, p=0.026). Conclusions: A dilated RVOT leading to flow vortices may increase the shear stress experienced by the valve, a sharp bifurcation angle disrupts flow patterns, adding dynamic load to the valve, and stenosis in the distal BPAs lead to increased resistance and an increased volume load to the valve - all promoting valve degeneration. Our findings are consistent with physiologic expectations and will be further explored using computational fluid dynamic simulations to elucidate how the parameters identified impact the hemodynamics around the pulmonary valve. A deeper understanding of the hemodynamic implications may ultimately reduce the incidence of valve degeneration by helping surgeons identify patients who are at high risk for valve dysfunction and guiding them to reconstruct the RVOT in specific configurations.

Right ventricular outflow tract stenting in the management of tetralogy of Fallot with pulmonary artery hypoplasia and major aortopulmonary collaterals

2020 ◽  
pp. 41-56
Author(s):  
Gemma Penford ◽  
Oliver Stumper
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Fallot's Tetralogy ◽  
Operative Care ◽  
Key Points ◽  
Fallot’S Tetralogy ◽  
Surgical Options ◽  
Aortopulmonary Collaterals

Fallot’s tetralogy is the most common cyanotic congenital heart lesion. It is a term that encompasses a spectrum of morphologies, all emerging from the fundamental feature of anterior deviation of the outlet septum and associated abnormalities of pulmonary blood flow. This case follows the journey of a patient with severe Fallot’s tetralogy and multifocal pulmonary blood flow from the neonatal period through to his post-operative period. The case explores key points in the assessment of these patients, addressing the medical, interventional, and surgical options for neonatal cyanosis, and then goes on to discuss common issues and pitfalls surrounding peri-operative care.

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