Ebstein’s malformation presenting with tricuspid stenosis: 1-year follow-up after surgical repair

2010 ◽  
Vol 20 (1) ◽  
pp. 80-85
Author(s):  
Rami N. Khouzam ◽  
Joseph A. Dearani ◽  
Paul R. Julsrud
Keyword(s):  
Right Ventricular ◽  
Thrombus Formation ◽  
Right Ventricular Outflow Tract ◽  
Rare Condition ◽  
Right Heart Failure ◽  
Ventricular Outflow Tract ◽  
Right Atrial ◽  
Cavopulmonary Anastomosis ◽  
Bidirectional Cavopulmonary Anastomosis ◽  
One Year

AbstractThis is a case of a young woman previously completely healthy, with two uneventful pregnancies and deliveries, who presented with bloating, shortness of breath, and signs of right heart failure. A thorough clinical evaluation, along with a work-up including an echocardiogram and a magnetic resonance imaging revealed the diagnosis of Ebstein’s anomaly malformation with tricuspid stenosis. A right ventricular outflow tract obstruction due to thrombus formation was thought to exacerbate her symptoms and lead to the diagnosis. Surgery in the form of right ventricular thrombectomy, right atrial reduction, porcine tricuspid valve replacement and bidirectional cavopulmonary anastomosis “bidirectional Glenn”, was successfully performed. The patient has been stable clinically more than one year after surgery. Discussion about this rare condition and operative details are provided.

Rheology of right ventricular outflow tract obstruction: sub-pulmonary membrane developing months after primary intervention to treat pulmonary atresia with intact interventricular septum

2021 ◽  
pp. 1-4
Author(s):  
Baher M. Hanna ◽  
Wesam E. El-Mozy ◽  
Sonia A. El-Saiedi
Keyword(s):  
Right Ventricular ◽  
Interventricular Septum ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Rare Condition ◽  
Surgical Excision ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Ventricular Outflow Tract Obstruction ◽  
The Right

Abstract Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.

scholarly journals Metastatic adenocarcinoma involving the right ventricle and pulmonary artery leading to right heart failure: case report

2017 ◽  
Vol 136 (3) ◽  
pp. 262-265 ◽  
Author(s):  
Turgut Karabag ◽  
Caner Arslan ◽  
Turab Yakisan ◽  
Aziz Vatan ◽  
Duygu Sak
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Right Ventricular Outflow Tract ◽  
Right Heart Failure ◽  
Ventricular Outflow Tract ◽  
Right Heart ◽  
The Right

ABSTRACT CONTEXT: Obstruction of the right ventricular outflow tract due to metastatic disease is rare. Clinical recognition of cardiac metastatic tumors is rare and continues to present a diagnostic and therapeutic challenge. CASE REPORT: We present the case of a patient who had severe respiratory insufficiency and whose clinical examinations revealed a giant tumor mass extending from the right ventricle to the pulmonary artery. We discuss the diagnostic and therapeutic options. CONCLUSION: In patients presenting with acute right heart failure, right ventricular masses should be kept in mind. Transthoracic echocardiography appears to be the most easily available, noninvasive, cost-effective and useful technique in making the differential diagnosis.

scholarly journals Successful balloon valvuloplasty of a subpulmonic membrane associated with cor triatriatum dexter: a case report

2019 ◽  
Vol 13 (1) ◽  
Author(s):  
Meryem Haboub ◽  
Abdenasser Drighil
Keyword(s):  
Pressure Gradient ◽  
Right Ventricular ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Balloon Valvuloplasty ◽  
Right Atrial ◽  
Cor Triatriatum ◽  
Ventricular Outflow Tract Obstruction ◽  
Cor Triatriatum Dexter

Abstract Background Subpulmonic membrane as a cause of right ventricular outflow tract obstruction in patients with concordant ventriculoarterial connection and intact ventricular septum is considered to be rare. Association with cor triatriatum dexter and success of subpulmonic balloon valvuloplasty have never been reported, at least to the best of our knowledge. Case presentation A 3-year-old Moroccan boy was referred to our tertiary care hospital with complaints of dyspnea on moderate exertion. A physical examination revealed parasternal lift, systolic thrill, and a 4/6 ejection systolic murmur, best heard over the left second intercostal space. His oxygen saturation was 99% on room air. Two-dimensional echocardiography showed a discrete circumferential membrane just below the pulmonic valve and a right atrial membrane. Continuous wave Doppler interrogation showed peak systolic pressure gradient of 85 mmHg across the subpulmonic membrane and no significant gradient across the right atrial membrane. Balloon dilation of the subpulmonic membrane was performed and the pressure gradient came down to 50 mmHg. During follow-up, he reported marked improvement in terms of exercise tolerance. Transthoracic echocardiography showed residual pressure gradient of approximately 40 mmHg across the membrane. Surgery resection of the two membranes was programmed, but he died after an extracardiac disease (appendicular peritonitis). Conclusions Subpulmonic membrane as an isolated cause of right ventricular outflow tract obstruction is rare. Its association with cor triatriatum dexter is even less common. The result of percutaneous balloon valvuloplasty of subpulmonic membrane is an interesting alternative while waiting for surgery. Surgery is currently the preferred modality of treatment with the resection of both right atrial and subpulmonic membranes.

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.

One-year outcome after radiofrequency catheter ablation of symptomatic ventricular arrhythmia from right ventricular outflow tract

2002 ◽  
Vol 89 (11) ◽  
pp. 1269-1274 ◽  
Author(s):  
Rungroj Krittayaphong ◽  
Charn Sriratanasathavorn ◽  
Kiertijai Bhuripanyo ◽  
Ongkarn Raungratanaamporn ◽  
Jaruprim Soongsawang ◽  
...  
Keyword(s):  
Catheter Ablation ◽  
Ventricular Arrhythmia ◽  
Right Ventricular ◽  
Radiofrequency Catheter Ablation ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
One Year

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.

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