double outlet right ventricle
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Tomofumi Mizuno ◽  
Nobuhiro Nishii ◽  
Hiroshi Morita ◽  
Hiroshi Ito

Abstract Background The frequency of arrhythmias increases after the Fontan operation over time; atrial tachycardia (AT) and sinus node dysfunction (SND) are frequently observed. Case summary Our patient was 63-year-old woman who underwent a lateral tunnel Fontan operation for double outlet right ventricle at age 36. She experienced paroxysmal AT for one year, and antiarrhythmic medication was not feasible due to symptomatic SND. Computed tomography revealed a 45 mm-sized thrombus in the high right atrium (RA). The patient had three coexisting conditions: paroxysmal AT, symptomatic SND and the RA thrombus, for which total cavopulmonary connection conversion and epicardial PMI would have been effective; however, given her age and comorbidities, surgical treatment was considered high-risk. Catheter ablation was avoided because of the RA thrombus. Finally, a transvenous pacemaker was implanted via the right femoral vein to avoid the RA thrombus and severe venous tortuosity from the left subclavian vein to the RA. After PMI, the patient was prescribed amiodarone and bisoprolol for AT suppression. AT occurred once in the third month after discharge. We increased the dose of amiodarone, and she has been tachycardia-free. Discussion Transvenous PMI must be considered in cases where open thoracic surgery or catheter ablation cannot be performed. This is the first report of transvenous PMI via the right femoral vein and successful AT and SND management in an elderly Fontan patient.

Samira Saraya ◽  
Yousra Mahmoud Ahmad ◽  
Ragab Hani Donkol ◽  
Hazem Hamed Soliman ◽  
Reem Ibrahim Ismail ◽  

Abstract Background The term of Double-outlet right ventricle (DORV) is used to describe a spectrum of complex congenital cardiac malformations where anomalies of the ventriculo-arterial alignment are noted where both great arteries (pulmonary artery and aorta) originate completely or predominantly from the right ventricle. The purpose of this study is to evaluate the diagnostic accuracy of electrocardiography (ECG) gated multidetector computed tomography (MDCT) in preoperative assessment of DORV and its associated malformations, providing key anatomic parameters that affect surgical planning. Results A total of 50 patients diagnosed by echocardiography with DORV referred from our institute between September 2018 and August 2020 were enrolled. The location of the ventricular septal defect (VSD) to the great vessels and the alignment of the great vessels to each other as well as other associated malformations that affect the surgical planning were assessed by MDCT. The patients were retrospectively classified into subgroups according to the relative positions of the great arteries, the relationship between the great arteries and the VSD, and the presence of associated malformations according to the classification of Society of Thoracic Surgeons database. The diagnostic value of MDCT and transthoracic echocardiography (TTE) in evaluation of associated cardiac malformations apart from double outlet right ventricle was compared in 20 patients, whom surgeries were performed and surgical results were obtained. Fallot type of DORV was the most common clinical type which represented 48% of the patients in which the VSD is committed to the aorta. The second most common clinical type was Taussig–Bing anomaly which represented 30% of the patients and the VSD was seen committed to the pulmonary artery. The atrio-ventricular septal defect (AVSD)/uni-ventricular type represented 16% of the patients in the current study, with pulmonary and systemic venous anomalies, heterotaxy syndrome and situs anomalies were commonly associated. A total of 53 malformations apart from DORV were found in 20 patients whom performed surgical interventions. MDCT detected different vascular and situs anomalies in those patients with 100% sensitivity and 100% positive predictive value compared to 71.4% and 94% respectively in TTE. On the other hand, in our study TTE was superior to MDCT for detection of intracardiac anomalies with 100% sensitivity and 100% accuracy compared to 87.5% and 95% respectively in MDCT. Conclusions Our study suggested that ECG gated MDCT serves as a rapid, noninvasive imaging modality with good spatial resolution and provides excellent diagnostic image quality and has satisfactory diagnostic accuracy in the preoperative assessment of DORV. It is superior to echocardiography in providing a comprehensive mapping of the anatomy of complex anomalies especially that of extra-cardiac structures such as aorta, systemic veins, pulmonary veins, and pulmonary arteries. However, it can miss tiny intracardiac malformations. So combining the results of MDCT and echocardiography would be beneficial to guarantee the accuracy of diagnosis of DORV.

Ujjwal Chowdhury ◽  
Robert Anderson ◽  
Diane E. Spicer ◽  
Lakshmi Sankhyan ◽  
Niraj Pandey ◽  

Background and aim: On the basis of previously published accounts, coupled with our own experience, we have assessed the surgical approaches to patients with isomeric atrial appendages. Methods: We reviewed pertinent published studies on surgical treatment of individuals with isomeric atrial appendages, with the pertinent surgical details provided by most of the manuscripts. Results: Half of patients with right isomerism, and two-thirds of those with left isomerism have bilateral superior caval veins. Azygos extension of the inferior caval vein is reported in three-quarters of those with left isomerism. The coronary sinus is universally absent in right isomerism, along with totally anomalous pulmonary venous connection, and is absent in two-fifths of those with left isomerism.. Univentricular atrioventricular connections are expected in up to three-quarters of those with right isomerism. Atrioventricular septal defect is reported in up to four-fifths, more frequently in right isomerism, with such patients typically having discordant ventriculoatrial connections or double outlet right ventricle. Reported mortalities extend to 85% for those with right, and 50% for those with left isomerism. In right isomerism, mortality is up to 54% for systemic-to-pulmonary arterial shunting, up to 75% for univentricular repair, and up to 95% for repair of totally anomalous pulmonary venous connection itself. No more than one-quarter had undergone Fontan completion, with reported mortalities of 21%. Conclusion: Early surgical results are satisfactory in patients with left isomerism, but disappointing for those with right. Recent advances in cardiac and liver transplantation may offer improved survival.

2021 ◽  
Panthip Patrakunwiwat ◽  
Pirapat Makarapong ◽  
Thanarat Layangkool

Abstract Criss-cross heart (CCH) is a rare cardiac malformation which is characterized by crossing of the inflow streams of the two ventricles due to rotation of ventricular axis. The anomalies can be identified both atrioventricular concordance and discordance. The etiology of CCH is remaining unknown. Prominent symptom is cyanosis. The primary investigation is transthoracic echocardiography to identify this abnormality. Many patients need further investigation to review anatomy. Total correction is the aim for treatment but it is difficult for this complex anatomy. There were 5 patients who visited cardiology clinic at Queen Sirikit National Institute of Child Health (QSNICH) from 2002 to 2017. The objective is to review the treatment options that we performed in CCH. Most common associated anomalies were double outlet right ventricle and pulmonary stenosis. One patient died before surgical intervention; others were performed palliative surgery.

Arda Ozyuksel ◽  
Baran Simsek ◽  
Sener Demiroluk ◽  
Murat Saygi ◽  
Mehmet Bilal

Background: Intraextracardiac Fontan procedure aimed to combine the advantages of lateral tunnel and extracardiac conduit modifications of the original technique. Herein, we present our experience in our patients with intraextracardiac fenestrated Fontan Procedure. Methods: A retrospective analysis was performed in order to evaluate intraextracardiac fenestrated Fontan patients between 2014 and 2021. Seventeen patients were operated on with a mean age and body weight of 9.1 ± 5.5 years and 28.6 ± 14.6 kg. Results: Sixteen patients (94%) were palliated as univentricular physiology with hypoplasia of one of the ventricles. One patient (6%) with well-developed two ventricles with double outlet right ventricle and complete atrioventricular septal defect had straddling of the chordae prohibiting a biventricular repair. All of the patients had cavopulmonary anastomosis prior to Fontan completion, except one case. Fenestration was performed in all cases. Postoperative mean pulmonary artery pressures and arterial oxygen saturation levels at follow up were 10 ± 2.4 mmHg and 91.3 ± 2.7 %, respectively. Mean duration of pleural drainage was 5.4 ± 2.3 days. All of the fenestrations are patent at a mean follow up period of 4.8 ± 7.7 years, except one case. Any morbidity and mortality were not encountered. Conclusions: The mid-term results of intraextracardiac fenestrated Fontan procedure are encouraging. This procedure may improve the results in a patient population who should be palliated as univentricular physiology, especially in cases with complex cardiac anatomy.

2021 ◽  
Vol 18 (2) ◽  
pp. 57-60
Nirmal Panthee ◽  
Sidhartha Pradhan ◽  
Raamesh Koirala ◽  
Bishow Pokhrel ◽  
Deekshya Thapaliya ◽  

Double outlet right ventricle (DORV) with or without pulmonary atresia is a common indication for Rastelli operation. We very infrequently perform this surgery in our center. Here, we report a case of a ten-year-old girl who recently underwent Rastelli operation and patent ductus arteriosus (PDA) ligation for DORV, pulmonary atresia with ductal dependent pulmonary circulation by using custom-made valved conduit

2021 ◽  
Vol 12 (6) ◽  
pp. 778-782
Masahiro Nishibata ◽  
Masataka Kitano ◽  
Seiich Sato

Wire atrial septostomy, a new transcatheter technique, combines 2 atrial septal defects (ASDs) into a large one in a thickened atrial septum. Although the thin-wire-looped atrial septum between ASDs needs to be pulled deeply into a hard catheter to tear the septum, this action might harm the surrounding cardiac structures. We utilized radiofrequency wire energy to facilitate the tearing of the septum, making the procedure easier and safer. This technical article presents the modified procedure in detail; the procedure was performed in a 6-month-old boy with double outlet right ventricle and intact ventricular septum. Mini-abstract This technical article describes modified wire atrial septostomy for thickened atrial septum in detail. Radiofrequency energy facilitated a thin wire-loop in tearing the septum between 2 atrial septal defects to combine the defects into a large one. We believe that this transcatheter procedure is easier and safer than the original one not using a radiofrequency wire energy.

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