scholarly journals Congenital heart disease in children and adults Comparison of the Mid-term Results of Pulmonary Valve-sparing Strategy and Transannular Patch Repair in Tetralogy of Fallot

2013 ◽  
Vol 4 ◽  
pp. 352-356
Author(s):  
Ozgur Arslan ◽  
Taylan Adademir ◽  
Ahmet Elibol ◽  
Oguz Ugur ◽  
Ebuzer Aydın ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Patch Repair ◽  
Transannular Patch ◽  
Valve Sparing ◽  
Mid Term Results

Variable expression of Alagille syndrome in a family with a new JAG1 gene mutation

2015 ◽  
Vol 26 (1) ◽  
pp. 164-167 ◽  
Author(s):  
Victoria C. Ziesenitz ◽  
Tsvetomir Loukanov ◽  
Christiane Gläser ◽  
Matthias Gorenflo
Keyword(s):  
Liver Transplantation ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Alagille Syndrome ◽  
Cardiac Repair ◽  
Absent Pulmonary Valve ◽  
Variable Expression

AbstractWe report the case of a patient with tetralogy of Fallot with absent pulmonary valve and familial Alagille syndrome who successfully underwent cardiac repair. The patient’s sister had liver and congenital heart disease. The father had undergone liver transplantation but showed no significant cardiac abnormalities. A yet-unknown mutation of the JAG1 gene was discovered in this family with variable expression of Alagille syndrome.

Applicability and Durability of Valve-Sparing Tetralogy of Fallot Repair

2021 ◽  
Vol 12 (5) ◽  
pp. 628-634
Author(s):  
Elizabeth H. Stephens ◽  
Brian L. Wolfe ◽  
Abhinav A. Talwar ◽  
Angira Patel ◽  
Joseph A. Camarda ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Demographic Data ◽  
Right Ventricular Outflow Tract ◽  
Patch Repair ◽  
Transannular Patch ◽  
Z Scores ◽  
Valve Sparing ◽  
Tetralogy Of Fallot Repair

Background: Although valve-sparing repair remains ideal for patients with tetralogy of Fallot, the durability of valve-sparing repair and which patients may have been better served with a transannular patch remain unclear. Methods: Retrospective review was performed of tetralogy of Fallot operations at our institution from January 2008 to December 2018. Standard demographic data were collected, including echocardiographic parameters, operative details, and clinical outcomes. Statistical analysis was performed comparing the transannular patch and valve-sparing repair groups. Results: Sixty-seven patients underwent tetralogy of Fallot repair with a median age of 4.5 (3.2-6.0) months and weight of 5.8 (5.2, 6.7) kg. Seventeen (25%) patients underwent transannular patch repair and 50 (75%) patients underwent valve-sparing repair. There was no difference in age or weight between patients who underwent a transannular patch repair and those who underwent a valve-sparing repair. At last follow-up (median 42 months), there was a trend of a higher peak pulmonary valve/right ventricular outflow tract gradient ( P = .06) in the valve-sparing group, but no difference in the pulmonary valve annulus z-scores. Additionally, the pulmonary valve z-scores in the valve-sparing group decreased from −2.3 ± 1.0 on predischarge echocardiogram of to −1.2 ± 1.6 on last follow-up, with the peak gradient on predischarge 23 (0-37) mm Hg remaining stable on last follow-up at 18 (0-29) mm Hg. There was one reoperation: pulmonary valve replacement six years after a transannular patch. Conclusions: Obtaining a postrepair pulmonary valve z-score of −2 yields satisfactory, stable valve-sparing repair with pulmonary valve growth, acceptable gradients, minimal regurgitation, and high freedom from reintervention during follow-up.

Congenital Heart Disease in Adults

2017 ◽  
Author(s):  
Susan E. Haynes ◽  
Heather L. Bartlett ◽  
David J Skorton ◽  
Luke J Lamers
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Vena Cava ◽  
Congenital Diseases ◽  
Septal Defects ◽  
Atrial Septal Defects ◽  
Repaired Tetralogy Of Fallot

With advances in medical and surgical care, an increasing number of children with congenital diseases of the heart and vasculature now survive to adulthood. The proportion of adults who are affected by congenital heart disease is expected to continue to increase. Thus, it is important for clinicians to be knowledgeable about the care of these patients. This review examines acyanotic disorders (shunts and valvular lesions), vascular anomalies, cyanotic disorders, and women’s health issues. Figures show the anatomy of atrial septal defects, transcatheter closure of atrial septal defects, an anatomic cross section showing the atrioventricular septum, the anatomic positions of ventricular septal defects, a computed tomography scan of aortic coarctation, angiogram of a persistent left superior vena cava draining into the right atrium, systemic artery-to-pulmonary artery shunts, magnetic resonance image of a patient with repaired tetralogy of Fallot and long-standing pulmonary valve insufficiency, treatment of pulmonary valve regurgitation with a transcatheter pulmonary valve, computed tomographic images of a patient with atrial switch palliation of transposition of the great arteries and multiple baffle obstructions, stages in the repair of functional single ventricles, and echocardiograms of a patient with Ebstein anomaly. Tables list recommendations for pulmonary valve replacement in repaired tetralogy of Fallot, conditions in which pregnancy is high risk, and cardiac indications for fetal echocardiography. This review contains 12 highly rendered figures, 4 tables, and 62 references.

Abstract 14576: Durability of Valve-sparing Tetralogy of Fallot Repair

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Elizabeth H Stephens ◽  
Bryan L Wolfe ◽  
Abhinav A Talwar ◽  
Angira Patel ◽  
Joseph Camarda ◽  
...  
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Pulmonary Regurgitation ◽  
Pulmonary Valve Replacement ◽  
Patch Repair ◽  
Transannular Patch ◽  
Z Scores ◽  
Valve Sparing ◽  
Tetralogy Of Fallot Repair

Introduction: While valve-sparing repair is ideal for Tetralogy of Fallot (TOF), it’s durability and which patients may benefit from a transannular patch remains unclear. To this end, we reviewed our experience with valve-sparing TOF repair. Methods: Retrospective review was performed of all primary TOF operations at our institution from 1/2008 to 12/2018. Standard demographic, operative, and echo data were collected, along with clinical outcomes. Transannular patch and valve-sparing repair groups were then compared. Results: Sixty-eight patients underwent TOF repair with a mean age of 4.1±2.2 months and weight of 5.7±1.8 kg. There was no difference in age or weight between patients who underwent a transannular patch repair and valve-sparing repair (Table). There was also no difference in the frequency of hypercyanotic spells or beta-blocker use. As expected the pre-operative pulmonary valve size and z-score were significantly different between groups. Bypass times were longer in the transannular patch group (176±40 vs. 144±40 minutes, p=0.005). There were no differences in post-operative complications. At last follow-up (median 41.5 months) there was a trend of a higher peak pulmonary valve gradient (p=0.07) in the valve-sparing group, but no difference in pulmonary valve annulus z-scores. Additionally, the pulmonary valve z-scores in the valve-sparing group decreased from -2.3±1.0 on pre-discharge echocardiogram to -1.2±1.6 on last follow-up, with the peak gradient on pre-discharge 20 (33) mmHg stable on last follow-up at 18 (29) mmHg and degree of pulmonary regurgitation stable. There was one reoperation in the cohort: a pulmonary valve replacement in a patient who had undergone a transannular patch repair 6 years prior. Conclusions: Valve-sparing TOF patients demonstrated stable repairs with pulmonary valve growth, acceptable gradients, minimal regurgitation, and high freedom from re-intervention during follow-up.

Pulmonary Valve Endocarditis in a Corrected Tetralogy of Fallot

2021 ◽  
Author(s):  
Valentina Scheggi
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Surgical Intervention ◽  
Pulmonary Valve Replacement ◽  
Late Complications ◽  
Tract Infections

Tetralogy of Fallot is the most common cyanotic congenital heart disease. In the last decades, the number of adults with surgically corrected defects is increasing and we have to face with late complications. Echocardiography has a central role in the early diagnosis of these conditions. We report the case of a 31-year-old man, treated during childhood with surgical repair of the ventricular septal defect, ventricular right outflow plasty, and pulmonary valve commissurotomy. At the age of 30 years, the patient underwent a second surgical intervention (aortic root replacement and pulmonary bioprosthesis implantation). He was also affected by urethral stenosis and complained of relapsing urinary tract infections. At the admission to our department, he reported remittent fever ensued one month before. We performed a third surgical intervention (aortic and pulmonary valve replacement) after diagnosing Steptococcusagalactiae endocarditis involving the pulmonary prosthesis and the native aortic valve. In conclusion, echocardiographic follow-up and prevention of infection are the mainstays to improve the outcome of corrected congenital heart disease.

Congenital Heart Disease in Adults

2017 ◽  
Author(s):  
Susan E. Haynes ◽  
Heather L. Bartlett ◽  
David J Skorton ◽  
Luke J Lamers
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Tetralogy Of Fallot ◽  
Congenital Heart ◽  
Pulmonary Valve ◽  
Vena Cava ◽  
Congenital Diseases ◽  
Septal Defects ◽  
Atrial Septal Defects ◽  
Repaired Tetralogy Of Fallot

With advances in medical and surgical care, an increasing number of children with congenital diseases of the heart and vasculature now survive to adulthood. The proportion of adults who are affected by congenital heart disease is expected to continue to increase. Thus, it is important for clinicians to be knowledgeable about the care of these patients. This review examines acyanotic disorders (shunts and valvular lesions), vascular anomalies, cyanotic disorders, and women’s health issues. Figures show the anatomy of atrial septal defects, transcatheter closure of atrial septal defects, an anatomic cross section showing the atrioventricular septum, the anatomic positions of ventricular septal defects, a computed tomography scan of aortic coarctation, angiogram of a persistent left superior vena cava draining into the right atrium, systemic artery-to-pulmonary artery shunts, magnetic resonance image of a patient with repaired tetralogy of Fallot and long-standing pulmonary valve insufficiency, treatment of pulmonary valve regurgitation with a transcatheter pulmonary valve, computed tomographic images of a patient with atrial switch palliation of transposition of the great arteries and multiple baffle obstructions, stages in the repair of functional single ventricles, and echocardiograms of a patient with Ebstein anomaly. Tables list recommendations for pulmonary valve replacement in repaired tetralogy of Fallot, conditions in which pregnancy is high risk, and cardiac indications for fetal echocardiography. This review contains 12 highly rendered figures, 4 tables, and 62 references.

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