Congenital right atrial diverticulum, atrial septal defect within the oval fossa, and complex pulmonary valvar obstruction in an infant with chromosome 8(p23.1) deletion

2005 ◽  
Vol 15 (3) ◽  
pp. 306-308 ◽  
Author(s):  
Aungkana Gengsakul ◽  
Suvro S. Sett ◽  
Martin C. K. Hosking
Keyword(s):  
Right Ventricular ◽  
Septal Defect ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Chromosome 8 ◽  
Right Atrial ◽  
Ventricular Compliance ◽  
The Right ◽  
Right Atrial Aneurysm

We describe an unusual right atrial aneurysm, occurring with progressive obstruction of the right ventricular outflow tract and increasing cyanosis, in a nine-month-old boy with chromosome 8(p23.1) deletion. Surgical resection of the diverticulum, and relief of the right ventricular obstruction, was successful, although impairment of right ventricular compliance persists after 19 months follow-up.

scholarly journals Surgical repair of tetralogy of Fallot using autologous right atrial appendages: short- to mid-term results

2020 ◽  
Author(s):  
Ahmadali Amirghofran ◽  
Fatemeh Edraki ◽  
Mohammadreza Edraki ◽  
Gholamhossein Ajami ◽  
Hamid Amoozgar ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Pulmonary Stenosis ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Right Atrial ◽  
The Mean ◽  
Mid Term Results ◽  
Tetralogy Of Fallot Repair

Abstract OBJECTIVES The prevention of pulmonary insufficiency (PI) is a crucial part of the tetralogy of Fallot repair. Many techniques have been introduced to construct valves from different materials for the right ventricular outflow tract, including the most commonly constructed monocusp valves. We are introducing a new bicuspid valve made intraoperatively using the autologous right atrial appendage (RAA) to prevent PI in these patients. METHODS The RAA valve was constructed and used in 21 patients with tetralogy of Fallot. The effective preservation of the native valve was impossible in all patients because of either a severe valve deformity or a small annulus. The RAA valve was created after ventricular septal defect closure and right ventricular outflow tract myectomy and was covered with a bovine transannular pericardial patch. The perioperative data were evaluated, and the echocardiography results were assessed immediately after operations and in follow-up with a median of 10.5 months. The data were retrospectively compared with 10 other patients with similar demographic data but with only transannular patches. RESULTS The mean age of the patients was 13.3 months. No mortality or related morbidity occurred after repair using the RAA valve. The PI severity early after the operation was trivial or no PI in 18 patients, and mild PI was observed in 3 patients, which progressed to moderate PI in one of them in the mean 12-month follow-up period. Fifteen patients had mild or no pulmonary stenosis, while moderate pulmonary stenosis was observed in 6 others. Compared with the other 10 patients with only transannular patches, the RAA valve patients had prolonged operative and clamping times, but no difference in postoperative course and shorter hospital stays. The degree of PI was, of course, significantly less in the RAA valve patients, but pulmonary stenosis was the same. CONCLUSIONS The RAA valve construction is a safe and effective technique to prevent PI after the tetralogy of Fallot repair, at least in terms of short- and mid-term results. A longer follow-up period is needed to confirm if this new valve can eliminate or significantly delay the need for pulmonary valve replacement in these patients.

scholarly journals COMPARISON OF TRANS-RIGHT ATRIAL VERSUS COMBINED (TRANS-RIGHT ATRIAL, TRANS-RIGHT VENTRICULAR) APPROACH FOR INTRA-CARDIAC REPAIR OF TETRALOGY OF FALLOT: SINGLE-CENTER EXPERIENCE

2022 ◽  
Vol 6 (1) ◽  
Author(s):  
Piyush Gupta ◽  
Manish Porwal
Keyword(s):  
Right Ventricular ◽  
Tetralogy Of Fallot ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Cardiac Repair ◽  
Right Atrial ◽  
Aortic Cross Clamp Time ◽  
Postoperative Intensive Care ◽  
Rv Function

Background and Objective: We compared trans-right atrial (t-RA) versus combined (trans-right-atrial and trans-ventricular (t-RA/RV) approaches for intra-cardiac repair of Tetralogy of Fallot (TOF) for the pre-operative and post-operative right ventricular (RV) function. The RV function was calculated using a tricuspid annular plane systolic excursion (TAPSE) using two-dimensional (2-D) echocardiography. Materials and Methods: This was a retrospective study. Fifty-three patients operated for the intra-cardiac repair of TOF between August 2019 and March 2021 were included in the study and divided into two groups based on the approach for repair as follows: t-RA or combined (t-RA/RV) approach. The first group (t-RA) had twenty-one patients, and the second group (combined t-RA/RV approach) had thirty-two patients. The assessment of pre-operative and post-operative RV function was done using TAPSE. Records of follow-up at 1 month and 3 months were evaluated. Results: Age, body surface area (BSA), preoperative saturation, cardiopulmonary bypass time, aortic cross?clamp time, postoperative intensive care unit (ICU) stay, and hospital stay were similar in both groups. However, t?RA/RV group had more pleural effusions (9 vs. 1 patients, P < 0.05), but had more improvements in Right Ventricular outflow tract (RVOT) gradients. There were no differences in arrhythmias in either group. Pre-operative TAPSE for both groups was similar (1.46 ± 0.27 vs. 1.61 ± 0.31, P > 0.05) and so was the post?operative TAPSE at discharge (1.54 ± 0.31 vs. 1.49 ± 0.33, P > 0.05), at 1 months (1.64 ± 0.25 vs. 1.48 ± 0.32, P > 0.05) and 3months (1.75 ± 0.19 vs. 1.7 ± 0.15, P > 0.05). Conclusion: Both approaches provide adequate palliation with effective improvements in RVOT gradients for patients with TOF. A limited right ventriculotomy does not adversely affect early RV function or increase the incidence of arrhythmias at the immediate post-operative period and early follow-up. More extensive studies with prospective randomized design and longer follow-ups are needed to address these issues further. Keywords: Tetralogy of Fallot, transatrial approach, intracardiac repair.

Quadruple Valve Replacement with Mechanical Valves: An 11-Year Follow-up Study

2012 ◽  
Vol 15 (3) ◽  
pp. 145 ◽  
Author(s):  
Yukun Cao ◽  
Chunhu Gu ◽  
Guocheng Sun ◽  
Shiqiang Yu ◽  
Hongbing Wang ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Valve Replacement ◽  
Heart Valves ◽  
Septal Defect ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Follow Up Study ◽  
Mechanical Valves ◽  
The Right

<p><b>Background:</b> We performed the first quadruple valve replacement with mechanical valves, combined with the correction of complex congenital heart disease on November 17, 1999. We report here the 11-year follow-up study.</p><p><b>Methods:</b> A 47-year-old man with subacute rheumatic endocarditis, a ventricular septal defect, and an obstruction of the right ventricular outflow tract required replacement of the aortic, mitral, tricuspid, and pulmonary valves; repair of the ventricular septal defect; and relief of the obstruction of the right ventricular outflow tract. The surgery was done on November 17, 1999, after careful systemic preparation of the patient. Warfarin therapy with a target international normalized ratio (INR) range of 1.5 to 2.0 was used. Follow-up included monitoring the INR, recording the incidences of thromboembolic and bleeding events, electrocardiography, radiography, and echocardiography evaluations.</p><p><b>Results:</b> The patient's INR was maintained between 1.5 and 2.0. All 4 mechanical prosthetic heart valves worked well. He is in generally good health without any thromboembolic or bleeding complications.</p><p><b>Conclusions:</b> Long-term management is challenging for patients who have experienced quadruple valve replacement with mechanical valves; however, promising results could mean that replacement of all 4 heart valves in 1 operation is feasible in patients with quadruple valve disease, and an INR of 1.5 to 2.0 could be appropriate for Chinese patients with undergoing valve replacement with mechanical valves.</p>

Initial clinical manifestations and mid- and long-term results after surgical repair of double-chambered right ventricle in children and adults

2008 ◽  
Vol 18 (3) ◽  
pp. 268-274 ◽  
Author(s):  
Ragiab Telagh ◽  
Vladimir Alexi-Meskishvili ◽  
Ronald Hetzer ◽  
Peter E. Lange ◽  
Felix Berger ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Septal Defect ◽  
Surgical Repair ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
The Right

AbstractObjectiveBy means of retrospective analysis of our institutional experience, we reviewed the clinical manifestation and outcomes of patients subsequent to surgical repair of double-chambered right ventricle.MethodsBetween 1988 and 2005, we performed surgical repair in 35 of 37 patients diagnosed with double-chambered right ventricle. The patients ranged in age from 4 to 69 years, with a mean of 21.3 years. Most presented in infancy, with initial manifestation of a short systolic murmur in 34 (92%) of all cases. Pressure gradients were measured invasively across the right ventricular outflow tract of between 30 and 140 mmHg, with a median of 60 mmHg. An associated ventricular septal defect was present in 26 patients (70%). Of the group, 4 patients were aged over 40 years, and 2 had previously undergone operative closure of a ventricular septal defect.ResultsThe operative interval ranged from 2 months to 41 years, with a median of 9 years. In all, we resected muscular bundles and enlarged the right ventricular outflow tract. There was no hospital or late death. Median follow-up subsequent to surgery was 7 years, with a range from 0.4 to 11 years. No patient required further surgery to relieve any obstruction of the right ventricular outflow tract, nor long term medical therapy or pacing because of cardiac arrhythmia.ConclusionsSurgical repair of a double-chambered right ventricle yields excellent haemodynamic and functional results over the mid to long term.

Restrictive right ventricular performance assessed by cardiac magnetic resonance after balloon valvuloplasty of critical pulmonary valve stenosis

2015 ◽  
Vol 26 (3) ◽  
pp. 556-568 ◽  
Author(s):  
Ikram Massoud ◽  
Nader Botros ◽  
Atef Yehia ◽  
Hassan Abdelghafoor ◽  
Mohamed Donya ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Right Ventricular ◽  
Pulmonary Valve ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Balloon Valvuloplasty ◽  
Pulmonary Valve Stenosis ◽  
Gadolinium Enhancement ◽  
The Right

AbstractBackgroundLittle data are published about right ventricular diastolic performance in patients with critical pulmonary valve stenosis after balloon pulmonary valvuloplasty thus far.MethodsA total of 44 patients with isolated critical pulmonary valve stenosis who had undergone balloon valvuloplasty with haemodynamic recordings were enrolled to the study; 33 patients who came for follow-up underwent further imaging by echocardiography after 6 months and their right ventricular functional parameters were compared with 33 control patients of the same age and sex. Out of 33 patients, 21 underwent cardiac MRI with late gadolinium enhancement to assess the presence of right ventricular fibrosis.ResultsThe right ventricular systolic pressure (p<0.0001) and right ventricular outflow tract gradient (p<0.0001) decreased acutely (p<0.0001) after balloon valvuloplasty. During follow-up, M-mode left ventricular end diastolic dimension (p<0.001) and end systolic dimension increased (p<0.001), whereas right ventricular end diastolic dimension decreased (p<0.001). Compared with controls, patients (n=33) had significantly reduced tricuspid annular Ea and higher E/Ea (p<0.001). Right ventricular systolic dysfunction was also suggested by reduced tricuspid annular systolic velocity (p<0.001). Late gadolinium enhancement was demonstrated in 13 out of 21 patients with restrictive physiology, which involves the anterior right ventricular outflow tract, anterior wall, and inferior wall. The right ventricular late gadolinium enhancement score correlated positively with age (r=0.7, p<0.001) and right ventricular mass index (r=0.52, p<0.001).ConclusionThe persistence of right ventricular diastolic dysfunction after relief of chronic pressure overload of critical pulmonary valve stenosis suggests that a factor – other than increase in afterload – is involved in this physiology. Fibrosis is the most likely factor responsible for persistence of restrictive physiology as documented by late gadolinium enhancement.

scholarly journals Different conduits for the right ventricular outflow tract reconstruction in adults during the Ross procedure

2015 ◽  
Vol 17 (2) ◽  
pp. 23 ◽  
Author(s):  
A. M. Karaskov ◽  
I. I. Demin ◽  
R. M. Sharifulin ◽  
S. I. Zheleznev ◽  
A. V. Bogachev-prokofev ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Multivariable Analysis ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Ross Procedure ◽  
Acceptable Alternative ◽  
The Right ◽  
Pulmonary Homograft

We compared different conduits for the right ventricular outflow tract reconstruction (RVOT) in adults during the Ross procedure. Between 1998 and 2012, 586 consecutive adult patients underwent the Ross procedures. Mean age was 45,514,2 years. The RVOT was reconstructed with a diepoxy-treated xenografts in 372 and with glutaraldehyde-treated in 88 patients. A pulmonary homograft was used in 125 patients. Hospital mortality was 4,9%. Mean follow up was 43,216,9 months. At discharge systolic gradient was 8,1 3,7 mm.Hg for the pulmonary homograft, 11,44,7 mm.Hg for the diepoxytreated and 14,96,1 mm.Hg for the glutaraldehyde-treated xenopericardial conduits. Twenty eight patients underwent reoperation. The 3-year actuarial freedom from conduit explantation for pulmonary homograft was 100%, for diepoxy- and glutaraldehyde-treated xenopericardial conduits 99,20,7% and 84,74,7% respectively. Multivariable analysis identified the type of xenograft and age as independent factors for xenograft dysfunction. Results from this study show that the pulmonary homograft is the most preferred conduit for the RVOT reconstruction during the Ross procedure. The diepoxy-treated xenopericardial conduits are acceptable alternative to the homograft in patients older 45 years.

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