Double Outlet Right Ventricle Infective Endocarditis: A Rare Combination and a Therapeutic Challenge

Infective endocarditis involving the right side of the heart occurs rarely and often involves the tricuspid valve. The isolated pulmonary valve infective endocarditis (IPVIE) is a less common condition that occurs in specific population. The double outlet right ventricle (DORV) is an unusual congenital heart disease. The association of DORV and IPVIE darkens the prognosis. We report two cases of the association of DORV and IPVIE. The transthoracic echocardiography (TTE) is the base to the diagnosis. Right sided infective endocarditis in the lack of a guided strategy remains a therapeutic challenge.

Structure Design and Finite Element Analysis on Patch in Intraventricular Tunnel / 医用生物力学

ObjectiveT o explore the influence of patch shape for intraventricular tunnel (IVT) construction on biomechanical performance of the double outlet right ventricle after correction. Methods Based on the idealized IVT model, a two-dimensional IVT patch was designed. Six groups of patch models with the rhombic long-to-short axis ratio of 1∶0.625, 1∶0.3, 1∶0.2, 1∶0.15, 1∶0.125, 1∶0.1 were established according to the difference between the long and short axis of the rhombus patch in the turning part, and finite element analysis method was used to numerically simulate the process of stitching, holding and propping up the patch into a three-dimensional (3D) IVT model. Results The maximum stresses on suture line of 6 patch models were mainly concentrated at acute-angle corners of the rhombus. As rhombic long-to-short axis ratio of the patch increased, the maximum stress of the IVT suture line first decreased and then increased, and the volume showed an increasing trend. The pressure difference between two ends of the tunnel first decreased and then increased. The patch with the long-to-short axis ratio of 1∶0.15 had a uniform surface stress distribution, and the maximum stress on the suture line was the smallest. Meanwhile the right ventricular volume was less encroached on, and the pressure difference at both ends of the tunnel was small. Conclusions The IVT shape can influence stresses of suture line, the right ventricle volume and the pressure difference of IVT with non-monotonic variations. The suture effect of the patch with the long-to-short axis ratio of 1∶0.15 is relatively better among the constructed models.

A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle"

Abstract Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

Dextrocardia con doble salida del ventrículo derecho con comunicación interventricular no relacionada asociada a malposición de grandes vasos / Right ventricular double outlet dextrocardia with unrelated ventricular septal defect associated with great vessel malposition

Resumen En el siguiente reporte se expone un caso complejo en el que coexisten malformaciones cardiacas congénitas de presentación atípica con un doble tracto de salida del ventrículo derecho, en un paciente con múltiples anomalías menores en la exploración física y un cariotipo normal. La atipia de cada una de estas y su coexistencia hacen pensar en posibles alteraciones genéticas que aún son desconocidas. Lo anterior supone un reto terapéutico con el fin de restaurar una fisiología cardiaca compatible con la vida, lo cual se logra en este caso mediante un cerclaje exitoso de la arteria pulmonar.

Abstract The following report presents a complex case in which congenital cardiac malformations of atypical presentation coexist with a double outlet right ventricle, in a patient with multiple minor abnormalities on the physical examination and a normal karyotype. The atypia of these and their coexistence suggest possible genetic alterations that are still unknown. Therefore, a therapeutic challenge in order to restore a cardiac physiology compatible with life is proposed, which is achieved in this case through a successful banding of the pulmonary artery.

Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect: 15-year experience of a single center / 中国胸心血管外科临床杂志

@#Objective    To explore the appropriate method of biventricular repair and analyze the risk factors for reoperation, by summarizing the 15-year treatment experience of biventricular repair for double outlet right ventricle with non-committed ventricular septal defect (DORVncVSD). Methods    Clinical data of 162 consecutive patients with DORVncVSD who had biventricular repair from 2005 to 2019 in our center were retrospectively analyzed. The children were divided into two groups according to the path of intracardiac tunnel: 110 patients with ventricular septal defect rerouted to the aorta were recruited into a group A (75 males and 35 females aged 3.6±3.2 years); 52 patients with ventricular septal defect rerouted to the pulmonary artery were into a group B (30 males and 22 females aged 2.8±2.7 years). In order to establish a smooth intracardiac tunnel, enlargement of VSD, the resection of conus muscle and the transfer of tricuspid tendon or papillary muscle, etc were performed at the same time. Results    In the patients with biventricular repair, there were 9 (5.6%) early deaths and 6 (3.7%) early intracardiac baffle obstructions. During the follow-up of 7.5±7.0 years, and 8 (4.9%) late deaths occurred. The 1-year, 5-year, 10-year and 15-year survival rates of the group A were 92.7%, 91.1%, 91.1%, 85.4%, respectively and those of the group B were 92.2%, 85.2%, 85.2%, 85.2%, respectively. The difference between the two groups was not statistically significant (P=0.560). The follow-up results showed that 10 (6.2%) patients had late-onset intracardiac tunnel obstruction, and 8 patients underwent reoperation. There were more late-onset intracardiac tunnel obstruction patients and overall intracardiac tunnel obstruction patients in the group A than those in the group B (9 patients vs. 1 patient, P=0.017; 15 patients vs. 1 patient, P=0.001). No significant difference of early mortality and late mortality was noted for the group A (P=0.386) and the group B (P=0.223). Also it was noted that performing tricuspid valve operation at the same time in the group A had a significant impact to reduce the occurrence rate of intracardiac obstruction (1/46 vs. 15/64, P=0.004), without any tricuspid regurgitation or stenosis. The reoperation rate of patients with Rastelli after right ventricular outflow tract lesions was significantly higher than that of REV surgery and double root replacement surgery (5/14 vs. 0/38, P<0.001). Conclusion    The effect of biventricular repair for DORVncVSD is satisfactory. And concomitant tricuspid procedures can help reduce the occurrence of intracardiac obstructions. Reconstruction of right ventricular outflow tract with biological valved conduit is a risk factor for reoperation.

Chinese expert consensus on surgical treatment of congenital heart disease (7): Double outlet right ventricle / 中国胸心血管外科临床杂志

@#Double outlet right ventricle (DORV) is a complex cardiac malformation with many anatomic variations and various approaches for surgical repair. DORV is mainly defined as the congenital heart disease with ventriculoarterial connection in which both pulmonary artery and aorta arising primarily (>50%) from the right ventricle, associated with continuity or discontinuity between the aorta and mitral valve. DORV can be subclassified by various ways. Now subclassification is usually performed according to the relationship between the ventricular septal defect (VSD) and the great arteries. Various approaches for surgical repair of DORV ranging from single ventricle palliation to biventricular repair are reported from many centers. However, the high-grade guideline of surgical management of DORV is still absent. Hence, we developed the Chinese expert consensus on DORV as the evidence for surgical strategies.

MDCT in diagnosis of double outlet right ventricle / 中国医学影像技术

Objective To observe the value of MDCT in diagnosis of double outlet right ventricle (DORV). Methods Clinical and imaging data of 36 patients with DORV confirmed with operation or cardioangiography were retrospectively analyzed. The preoperative results of MDCT and echocardiography (Echo) were compared with surgical findings. Results MDCT definitely diagnosed 34 patients with DORV, while 2 patients were misdiagnosed, and the accurate diagnostic rate was 94.44% (34/36).The accurate diagnostic rate of Echo was 75.00% (27/36), and the difference was statistically significant (P=0.022). Moreover, in the relationship of ventricular septal defect (VSD) with large artery and the special relationship of the large artery in DORV, the diagnostic accuracy of MDCT was 100% (36/36), of Echo was 80.56% (29/36), and the difference was statistically significant (P=0.017). For totally 119 intracardiac and extracardiac malformations in all 36 patients, no significant statistical difference of diagnostic accuracy for detecting intracardiac malformation was found between MDCT and Echo (P=0.474), whereas for extracardiac malformations, the diagnostic accuracy of MDCT (98.72% [77/78]) was higher than that of Echo (69.23% [54/78], P<0.001). Conclusion MDCT could diagnose DORV and combined malformation definitely, therefore providing important guidance for surgical planning.

Double Outlet Right Ventricle with Dextrocardia and Situs Inversus in a Three-Year-Old Boy: A Case Report and Review of Literature

Aims: To highlight the rare occurrence of double outlet right ventricle (DORV) with Dextrocardia and Situs Inversus in a three-year-old boy viz-a-viz what has been reported in the literatures. Presentation of Case: N.A is a three year old boy who presented with easy fatiguebility, bluish discolouration of lips and tongue and occasional dyspnoea on exertion noticed since about 12 months of life. There was no associated history of cough, leg swelling or frequent hospitalization. Physical examination revealed a small for age, centrally cyanosed boy with conjuctival ejection, and grade 4 digital clubbing. Pulse rate was 100 beats /min regular and synchronous with other peripheral pulses. There was a praecordial bulge to the right with apex beat at the 4th right intercostal space mid-clavicular line. Heart sounds were 1st and 2nd with a grade 2 systolic murmur at the right upper sternal border. The liver was 4 cm palpable below the left costal margin firm non-tender. Pulse oximetry showed SPO2 value of 75-79% in ambient air. He had chest radiograph, electrocardiogram, abdominal ultrasound and two-dimensional echocardiography which confirmed a diagnosis of DORV with Situs totalis. Discussion and Conclusion: Situs Inversus totalis (Dextrocardia co-existing with Situs inversus) is associated with lower incidence of congenital heart defects as compared with Dextrocardia and Situs solitus. The defects commonly associated with it, are a transposition of great arteries (TGA) and ventricular septal defects (VSD) unlike in the index patient where DORV is being reported.

Application of three - dimensional printing technology in preoperative planning of double outlet right ventricle / 中华实用儿科临床杂志

Objective To assess the clinic value of three - dimensional(3D)printing models in operation scheme of double outlet right ventricle(DORV). Methods From September 2015 to December 2016,the imaging data of 29 patients (13 males and 16 females)with DORV were acquired using Dual Source CT. And then the cardiac models were generated using 3D printing technology. The cardiac models were used in diagnosing the type of DORV and guiding the surgery scheme. The 3D printed models were compared with two - dimensional imaging in diagnosis and sur-gical scheme of DORV patients. Results Both the two - dimensional imaging and 3D printed models were effective in the diagnosis and typing of DORV. According to 3D printing models,28 cases were consistent with the real operations, and 1 case was inconsistent. According to the two - dimensional imaging data,20 cases of surgical strategies were con-sistent with the real operations and 9 cases were inconsistent. For patients with DORV with non - committed ventricular septal defect (NC - VSD),3D printing models were more accurate in the designing of surgical strategies. Conclusions 3D printing models can display 3D anatomical structures and it is helpful in the diagnosis and making preoperative planning for DORV especially for DORV with NC - VSD,which provides a new method for the assessment of DORV.

Diagnosis of 320-row detector dynamic volume CT in complex congenital heart disease with double outlet right ventricle / 重庆医学

Objective To explore the diagnosis value of 320-row detector dynamic volume CT in complex congenital heart disease (CCHD)with double outlet right ventricle(DORV).Methods Seventy-eight patients who proveed DORV by surgery in Xinqiao Hospital of Third Military Medical University were reviewed.Thirty-six patients of group A performed 320-row detector dynamic volume CT by using segmental analysis,and were compared with the group B(42 Cases) performed conventional 64-slice CT respectively.Results In 36 cases of DORV confirmed by surgery in group A,MSCT provided accurate qualitative diagnosis in all cases.The accuracy rate of diagnosis of the group B was 90.7％.There was no significant differences compared with the group A (P＞0.05).There were ventricular septal defect in all the 78 cases,pulmonary stenosis in 56 cases,atrial septal defect in 34 cases,pulmonary hypertension in 21 cases,patent ductus arteriosus in 16 cases,coarctation of aorta in 9 cases.Conclusion The 320-row detector dynamic volume CT has important diagnostic value for DORV of the anatomical diagnosis.

Arterial Switch Operation (Jatene Procedure) for Posterior TGA (Transposition of the Great Arteries) Type Double Outlet Right Ventricle / 日本心臓血管外科学会雑誌

We report a rare case of double outlet right ventricle (DORV) with sub-pulmonary type ventricular septal defect (VSD). The great arteries were almost side-by-side, and the ascending aorta was located slightly posterior to the right of the pulmonary artery. We performed complete repair at the age of 25 days. Intra-cardiac rerouting (VSD closure) was carried out through the tricuspid valve. Arterial switch procedure was performed without the Lecompte maneuver. His postoperative course was uneventful and he was discharged 19 days after the operation without any complications.

Complete Repair of Double Outlet Right Ventricle and Interrupted Aortic Arch (Arch Reconstruction+Arterial Switch Operation+Intra-ventricular Rerouting) after Bilateral Pulmonary Artery Banding / 日本心臓血管外科学会雑誌

We performed bilateral pulmonary artery banding (BPAB) through a median sternotomy on a four-day-old male infant with a double-outlet right ventricle (DORV) and interrupted aortic arch (IAA) who was delivered at 40 weeks of gestation. After urinary output improved, definitive repair was carried out 5 days later. Intra-ventricular rerouting was followed by arterial switch with the Lecompte maneuver. The aortic arch was reconstructed with direct anastomosis and the right ventricular outflow tract was augmented with a patch. The sternum was left open at the end of the procedure and the chest was closed on post-operative day (POD) 4. The patient was discharged from hospital on POD 78 after receiving treatment for pneumonia and chylothorax.

Doble salida de ventrículo derecho: Enfoque embriológico / Double outlet right ventricle: Embryological approach

Objetivo: Se presenta una explicación patogenética sobre la morfogénesis de la doble salida de ventrículo derecho que explica su origen. Método: Se describieron 35 corazones con doble salida de ventrículo derecho, 15 con grandes arterias ligeramente cruzadas, 10 con arterias lado a lado y 10 con aorta anterior y pulmonar posterior. Se comparó el plano de separación de las vías de salida y de las grandes arterias en los 3 tipos de esta cardiopatía, con el plano de la tabicación troncoconal normal del corazón embrionario. Se determinó el plano cefálico del tabique troncoconal y se comparó con la posición de su borde inferior, con lo cual se calculó el grado de torsión troncoconal en cada grupo de esta cardiopatía; esto permitió inferir el tipo de giro de ese tabique en las 3 formas anatómicas mencionadas. Resultados: En las grandes arterias ligeramente cruzadas el giro troncoconal fue de 135°,en las arterias lado a lado fue de 90°, en la aorta anterior derecha fue de 0° y en la aorta anterior izquierda fue de -90°. Conclusión: Embriológicamente esta cardiopatía se origina por persistencia de la continuidad entre el ventrículo derecho y el troncocono, que origina las vías de salida y las grandes arterias. Sus variantes anatómicas se originan por una detorsión progresiva del tabique troncoconal continuada con una torsión de -90°.

Objective: It is proposed a pathogenetic explanation that explains the morphogenesis of the anatomic variants of double outlet right ventricle. Method: An anatomic embryological correlation was made in which the plane separating the outlets and great arteries in the types of this cardiopathy was compared with the normal truncoconal septum in the embryonic heart. Thirty five hearts with double outlet right ventricle were described, fifteen with great arteries slightly crossed, ten with side by side great arteries and ten with anterior aorta and posterior pulmonary artery. The cephalic border of the truncoconal septum was compared with its inferior border in each group. With this procedure we calculated the type of torsion of the truncoconal septum. Results: In the slightly crossed great arteries the truncoconal twist was of 135° in side by side great arteries the twist was of 90° and in anterior right aorta the truncoconal septum was straight with 0° of rotation, and with left anterior aorta the rotation was of -90°. Conclusion: Embryologically double outlet right ventricle is originated by the persisting continuity between the right ventricle with the truncus and conus which form the great arteries and their outlets. The anatomic variations are the consequence of progressive detortion of the truncoconal septum followed by a torsion of -90°.

Biventricular refair for endocardial cushion defects with double outlet right ventricle / 中华胸心血管外科杂志

ObjectiveDouble-outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure.To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle.MethodsFrom July to November of 2009,6 patients (3 males and 3 females) aged from 7 to 24 (16.17±5.98) years and with endocardial cushion defects and double outlet right ventricle underwent operation of biventricular repair The duration of follow-up ranged from 10 days to 2 years(median,16 months).Endocardial cushion defects were repaired with a 2-patch technique.The artificial vascular patch was implanted to connect the ventricular septal defects and the aorta for draining the blood stream from the left ventricle to the aorta.The other patch was used to repair the ostium primum atrial septal defects.Right ventricular outflow tract obstructions was released and reconstructed by transplanting a bovine pericardium patch.If the size of pulmonary valve annulus was far more below the normal,a transannular pericardial patch was used.Rastelli procedure with a valved conduit between the right ventricle and the pulmonary artery would be performed if the obstruction in the right ventricular outfolw tract was severe.ResultsOne death occurred 2 days after the operation,resulting in a mortality rate of 16.6%.The case,a boy of 7 years old,had a mirror-image dextrocardia,complete endocardial cushion defect ( C type),anomalous pulmonary venous drainage and single atrium.In this case,the operation lasted for 8 hours,acute renal failure happened next day to the operation,the effect of CRRT was unsatisfied,and eventually cardiac arrest occurred as a result of hyperkalemia.The remaining cases had survived.Follow-up examinations showed that the systolic pressure gradients across the pulmonary valves decreased,with a range of 14 to 40 mm Hg,(23.9 ± 11.03) mm Hg.Mild mitral and tricuspid regurgiration were identified in 4 cases and moderate mitral regurgitation was identified in one case.The cardiac function in all patients was classified as NYHA class Ⅰ/Ⅱ,Conclusion Endocardial cushion defects with double outlet right ventricle can be corrected by means of biventricular repair procedure.The procedure was associated with a low mortality,The interim life quality of patients may be improved.The longterm outcomes should be further studied.

Cirugía de Rastelli: impacto adverso de la comunicación interventricular no relacionada con los grandes vasos en los resultados quirúrgicos / The Rastelli Procedure: Adverse Impact in Surgical Outcomes of a Ventricular Septal Defect Noncommitted to the Great Arteries

Introducción La evolución de los pacientes operados con técnica de Rastelli revela que se trata de un procedimiento quirúrgico complejo no exento de morbilidad y mortalidad a mediano y a largo plazos. Las complicaciones serían, aparentemente, más frecuentes cuando la comunicación interventricular (CIV) es anatómicamente remota o no relacionada con la aorta. Objetivo Evaluar el impacto de la localización anatómica de la CIV en la cirugía de Rastelli. Material y métodos Se incluyeron 47 pacientes con una media (X) de seguimiento posquirúrgico de 6 años (15 meses-14 años). Los pacientes se subdividieron en: grupo I, CIV relacionada con la aorta (n = 29) y grupo II, CIV remota o no relacionada (n = 18). Resultados En el posquirúrgico inmediato, las reoperaciones (p = 0,05) fueron más frecuentes en el grupo II, en el cual se observó también una tendencia a mayor frecuencia de arritmias (p = 0,06). Luego de una X = 3 años (1 día-13 años) posquirúrgicos se realizaron 27 reoperaciones en 22 pacientes. Se cerró CIV residual en 11 pacientes a una X = 1 mes. En 8 de ellos, la CIV era remota (p =0,007). Desarrollaron estenosis subaórtica que requirieron cirugía 6 pacientes a una X = 5 años. Se reemplazó el conducto ventrículo derecho-arteria pulmonar en 12 pacientes a una X = 5 años. La mortalidad en el período posquirúrgico inmediato fue del 6% (3 pacientes). La mortalidad global fue del 17,2% (n = 8); 7 pacientes tenían CIV no relacionada (p = 0,003). Conclusiones La CIV remota en la cirugía de Rastelli se asocia con: 1) mayor mortalidad, 2) mayor frecuencia de reoperaciones en el posquirúrgico inmediato y 3) tendencia a mayor frecuencia de arritmias en el posquirúrgico inmediato.

Background The Rastelli procedure is complex surgical procedure with marked morbidity and mortality in the medium and long-term follow-up. These adverse outcomes seem to be more frequent when the ventricular septal defect (VSD) is anatomically remote or noncommitted to the aorta. Objective To evaluate the impact of the anatomical location of the VSD on the outcomes of the Rastelli procedure. Material and Methods A total of 47 patients were included with a mean follow-up of 6 years after surgery (15 months-14 years). Patients were divided into two groups: group I (committed VSD, n=29) and group II (remote or noncommitted VSD, n=18). Results During the immediate postoperative period, reoperations and arrhythmias were more frequent in group II (p=0.05 and p=0.06, respectively). After a mean follow-up of 3 years (1 day-13 years) following surgery, 22 patients underwent 27 reoperations. A residual VSD was closed in 11 patients at a mean of 1 month, 8 of which were remote VSD (p=0.007). Six patients developed subaortic stenosis requiring surgery at a mean of 5 years. The right ventricleto-pulmonary artery conduit was replaced in 12 patients at a mean of 5 years. Immediate postoperative mortality was 6% (3 patients). Global mortality was 17.2% (n=8); 7 patients had noncommitted VSD (p=0.003). Conclusions Remote VSD in patients undergoing the Rastelli procedure is associated with: 1) increased mortality, 2) greater incidence of reoperations in the immediate postoperative period, and, 3) a trend towards greater incidence of arrhythmias in the immediate postoperative period.

Characteristics of Double-Chambered Right Ventricle in Adult Patients

BACKGROUND/AIMS: This study evaluated the clinical features of double-chambered right ventricle (DCRV) in adults. Most cases of DCRV are diagnosed and treated during childhood. Consequently, very few reports include cases in which its clinical characteristics are evident in adults. METHODS: We reviewed the clinical data for 10 adult patients (age > or = 18 years) with DCRV. RESULTS: Electrocardiogram showed right ventricular hypertrophy in 3 DCRV patients. All cases were associated with ventricular septal defect (VSD; 7 for perimembranous, 2 for muscular outlet, and 1 for the subarterial type). Surgical correction was done for 7 DCRV patients all of whom survived operations. Their follow-up echocardiogram showed the pressure gradient in their right ventricle was significantly decreased from 69.4 +/- 17.2 mmHg preoperatively to 10.2 +/- 5.0 mmHg postoperatively (p < 0.05). In the short-term follow-up, there was no significant increase in the pressure gradient in the right ventricle. CONCLUSIONS: There are lots of cases of DCRV that are not diagnosed accurately in adults. In our experience, all DCRV cases had VSD and surgical correction of these cases showed excellent results. Therefore, accurate diagnosis of DCRV is necessary so that DCRV is not overlooked and operations are enabled within an appropriate time.

Surgical treatment of double-outlet right ventricle without pulmonary stenosis / 中华胸心血管外科杂志

Objective: To investigate the result of biventricular repair for double outlet right ventricle without pulmonary stenosis. Methods: From January 1975 to June 2003, 38 consecutive patients underwent biventricular repair for double outlet right ventricle without pulmonary stenosis. At surgery, the median age of this group was 5.8 years. Two types of repair techniques were applied: intraventricular baffle repair (n=36) and arterial switch operation with VSD to pulmonary artery baffle (n=2). Results: There were 5 hospital deaths, with mortality rate of 13 2%. Two of these patients died of pulmonary hypertension crisis and the other 3 died of low cardiac output syndrome. One case (2 6%) died of right heart failure on 95th day postoperatively. The follow up periods in 29 patients (76%) ranged from 1 month to 23 years. 19 cases (65.5%) of these patients were in NYHA class I, 9 (31.0%) in NYHA class II, and the 1 (3.5%) in NYHA class III. Conclusion: The biventricular repair might be an effective treatment for double outlet right ventricle without pulmonary stenosis is good. Operation should be performed as early as possible before the development of progressive pulmonary vascular disease.