Recent advances in multimodal imaging in tetralogy of fallot and double outlet right ventricle.

PURPOSE OF REVIEW: In the ever-evolving field of medical imaging, this review highlights significant advancements in preoperative and postoperative imaging for Tetralogy of Fallot (TOF) and double outlet right ventricle (DORV) over the past 18 months. RECENT FINDINGS: This review showcases innovations in echocardiography such as 3D speckle tracking echocardiography (3DSTE) for assessing right ventricle-pulmonary artery coupling (RVPAC) and Doppler velocity reconstruction (DoVeR) for intracardiac flow fields evaluation. Furthermore, advances in assessment of cardiovascular anatomy using computed tomography (CT) improve the integration of imaging in ablation procedures. Additionally, the inclusion of cardiac magnetic resonance (CMR) parameters as risk score predictors for morbidity, and mortality and for timing of pulmonary valve replacement (PVR) indicates its significance in clinical management. The utilization of 4D flow techniques for postoperative hemodynamic assessment promises new insights into pressure mapping. Lastly, emerging technologies such as 3D printing and 3D virtual reality are expected to improve image quality and surgical confidence in preoperative planning. SUMMARY: Developments in multimodality imaging in TOF and DORV are poised to shape the future of clinical practice in this field.

Navigating the spectrum of double-outlet right ventricle presentations: Outcomes from a contemporary cohort based on subtypes.

PURPOSE: Our aim in this study was to investigate the prenatal and postnatal prognosis of double outlet right ventricle (DORV) cases diagnosed prenatally by analyzing the outcomes based on the subtype. METHODS: This study is a retrospective chart review. Cases diagnosed with fetal DORV by prenatal ultrasound in the maternal-fetal medicine department of our hospital between 2014 and 2022 were included. Data on maternal characteristics, fetal echocardiographic features (type of DORV), pregnancy and neonatal outcomes (termination of pregnancy [TOP], intrauterine fetal death [IUD], neonatal death [NND], death in infancy (IND), survival) were collected and analyzed. RESULTS: Ninety-nine cases of prenatally diagnosed cases of DORV were included. The prenatal diagnosis was right in 97% of the liveborn fetuses. The cases were classified into subtypes, including transposition of great arteries (TGA), Fallot, ventricular septal defect (VSD), remote, and heterotaxy types. The cohort consisted of 32.3% TGA type, 19.1% fallot type, 11.1% VSD type, 2% remote type, and 35.3% heterotaxy type of DORV. An additional cardiac anomaly was observed in 87% and an extra-cardiac anomaly was observed in 54% of the cases. When we excluded the cases with heterotaxy type but without any chromosomal abnormality, additional genetic abnormalities were detected in 42% of the remaining cases. Outcome of pregnancy was livebirth in 68/99 (68.7%), IUFD in 5/99 (5.1%), and TOP in 26/99 (26.3%). Postnatal cardiac surgical repair was performed in 48 cases. Survival among livebirths was 39/68 (57.3%). Twenty-nine neonates or infants who had additional cardiac anomalies and/or genetic abnormalities died before any surgical intervention. The postoperative survival rate was 39/48 (81.2%). CONCLUSION: The prognosis in DORV depends on the anatomical subtype, the presence, and severity of associated anomalies. Survival increases in isolated cases without any additional structural or genetic anomalies.

Clinical Presentation and Therapy of Tetralogy of Fallot and Double-Outlet Right Ventricle.

Tetralogy of Fallot (TOF) is the most common cyanotic heart defect. TOF consists of the combination of four anomalies (Fig. 35.1): (1) a large malalignment ventricular septal defect, (2) an obstruction of the right ventricular outflow tract (usually infundibular and valvular pulmonary stenosis with a small pulmonary valve annulus and supravalvular stenosis, (3) an aorta that "overrides" the ventricular septal defect, and (4) right ventricular hypertrophy. TOF represents 4-8% of congenital heart defects. Specific variations of TOF include all forms of pulmonary atresia with VSD and absent pulmonary valve syndrome. In addition, the left and right main pulmonary arteries may be stenotic or hypoplastic. In these cases, there may be major aortopulmonary collateral arteries (MAPCAs) which are vessels arising from the aorta or the subclavian arteries that supply segments of the pulmonary arterial tree. Additional variations include an ASD (Pentalogy of Fallot), a right aortic arch, and coronary abnormalities.

[Arterial Switch Operation for the Criss-cross Heart Complicated by Muscular Ventricular Septal Defect:Report of a Case].

Criss-cross heart is an extreamly rare anomaly characterized by abnormal rotation of the heart on its long axis. Almost always there are associated cardiac anomalies such as pulmonary stenosis, ventricular septal defect (VSD) and ventriculoarterial connection discord, and most cases are candidates for Fontan procedure due to hypoplasia of right ventricle or straddling atrioventricular valve. We report a case of arterial switch operation for criss-cross heart with muscular ventricular septal defect. The patient was diagnosed with criss-cross heart, double outlet right ventricle, subpulmonary VSD, muscular VSD and patent ductus arteriosus (PDA). PDA ligation and pulmonary artery banding (PAB) was performed in the neonatal period, and an arterial switch operation (ASO) was planed at 6 months of age. Preoperative angiography showed nearly normal right ventricular volume and echocardiography showed normal subvalvular structures of atrioventricular valves. ASO, intraventricular rerouting and muscular VSD closure by sandwitch technique were successfully performed.

Rare association of absent pulmonary valve syndrome, complete atrioventricular canal defect, double outlet right ventricle, right aortic arch, and aberrant right subclavian artery in a fetal case.

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly. It is often associated with TOF, but may also be associated with an intact ventricular septum or, more rarely, with tricuspid atresia. It's combination with a complete atrioventricular septal defects and double outlet right ventricle has been reported extremely rare, even in the postnatal period. Herein, we report a 20-week-old fetus with a right aortic arch and an aberrant left subclavian artery with this rare combination. We report a case of a 20-week fetus diagnosed having this rare combination of right aortic arch and aberrant left subclavian artery. The APVS with complete atrioventricular septal defects may represent another type of APVS.

Delayed presentation of aorto-pulmonary window, double outlet right ventricle with pulmonary arterial hypertension: Illustration of uncommon association on dual-energy CT.

We present a case of a 26-year-old female with double outlet right ventricle and proximal aorto-pulmonary window (APW). The case highlights the role of computed tomography angiography in the diagnosis and characterization of APW defects, which are difficult to be diagnosed on transthoracic echocardiography.

Neonatal repair of atypical double outlet right ventricle.

BACKGROUND: A complex and rare form of double outlet right ventricle needs careful attention when choosing the optimal strategy for repair. AIM OF THE STUDY: To point out retrospectively what could have been done differently in our unique patient. METHODS: Primary repair was arranged in a neonate with double outlet right ventricle (of a non-committed ventricular septal defect type and lack of the outlet septum between the semilunar valves) with right aortic arch and dextro-malposition of great arteries. RESULTS: We managed to achieve intraventricular rerouting via a right ventricular incision concomitantly with the arterial switch maneuver. The patient is doing well with an excellent hemodynamic status. CONCLUSIONS: We considered that the radical approach we chose appeared to be sensible in this particular patient, although some other options could have been available.

Separate origin of left external and internal carotid arteries directly from aortic arch in a patient with double outlet right ventricle.

We report a case of a 1-year-old boy with double outlet right ventricle where computed tomography (CT) angiography incidentally demonstrated the separate origin of the left external and internal carotid arteries directly from the left-sided aortic arch with absence of a left common carotid artery. The case highlights the underlying embryological hypothesis as well as the importance of CT angiography in identifying anomalies of the aortic arch and arch vessels in the setting of complex congenital heart defects.

Absent pulmonary valve with double-outlet left ventricle: a case report.

Absent pulmonary valve syndrome and double-outlet left ventricle are rare congenital anomalies, with, to the best of our knowledge, no cases reported to date. We present the treatment course in a patient with an absent pulmonary valve, double-outlet left ventricle, dextrocardia, hypoplastic right ventricle, valvular aortic stenosis, and bronchomalacia.

Double outlet right ventricle in adults: Anatomic variability, surgical treatment, and late postoperative complications.

Double outlet right ventricle (DORV) is a highly complex congenital heart disease (CHD) entity, gaining increasing interest due to the rapid progress of cardiac surgery. The number of patients operated for this congenital defect has been growing since 1980s and over following decades with active transitioning of this cohort into the adult medicine. However, the diversity of initial anomaly and performed interventions makes challenging the management of these patients. This is particularly important in the regions where specialized adult CHD cardiology still remains underdeveloped. In this review, we observe the basic principles of DORV nomenclature, main types of the operations and possible late complications. The article focuses on adult patients and offers illustrations from clinical practice.

3D/4D spatiotemporal image correlation (STIC) fetal echocardiography provides incremental benefit over 2D fetal echocardiography in predicting postnatal surgical approach in double-outlet right ventricle.

OBJECTIVE: To analyze the incremental benefit of 3D/4D spatiotemporal image correlation (STIC) fetal echocardiography over 2D fetal echocardiography with respect to the accuracy of identification of anatomic details crucial for surgical decision-making and in predicting surgical approach in fetuses with double-outlet right ventricle (DORV). METHODS: This was a retrospective study of fetuses with DORV which had undergone both 2D echocardiography and 3D/4D STIC echocardiography and which underwent surgery postnatally in a tertiary pediatric cardiac center in Kerala between October 2015 and March 2019. All such cases with normal atrial arrangement, concordant atrioventricular connections and balanced ventricles were included. 2D and 3D/4D STIC fetal echocardiographic data were analyzed by two experienced observers blinded to the other dataset. Anatomic variables crucial for surgical decision-making, i.e. location and routability of the ventricular septal defect, relationship of the great arteries and presence of outflow obstruction, were compared between the two modalities with respect to agreement with postnatal echocardiography. The accuracy of prenatal prediction of the surgical pathway was compared between 2D and 3D/4D modalities with respect to the procedure undertaken. RESULTS: Included in the study were 22 fetuses with DORV which had undergone both 2D and 3D/4D imaging as well as postnatal surgery. Accuracy of prenatal interpretation of all four anatomic variables was significantly higher using 3D/4D STIC than using 2D fetal echocardiography (19/22 (86.4%) vs 8/22 (36.4%), P < 0.001). Surgical procedures included single-stage repair in 14 (63.5%) patients and a multistage approach in eight (36.4%). Prenatal prediction of the surgical pathway was significantly more accurate on 3D/4D STIC than on 2D echocardiography (20/22 (90.9%) vs 12/22 (54.5%), P = 0.021). Prenatal predictive accuracy of single-stage biventricular repair was significantly better for 3D/4D STIC than for 2D echocardiography (14/14 (100%) vs 8/14 (57.1%), P = 0.04). CONCLUSION: Addition of 3D/4D STIC to conventional 2D fetal echocardiography confers incremental benefit on the accuracy of identification of anatomic details crucial for surgical decision-making and the prediction of postnatal surgical approach in fetuses with DORV, thereby potentially aiding prenatal counseling. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Mixed-reality hologram for diagnosis and surgical planning of double outlet of the right ventricle: a pilot study.

AIM: To evaluate the mixed-reality (MR) hologram, a novel technology based on two-dimensional images, which simulates three-dimensional (3D) images and provides a dynamic and interactive alternative, for its usefulness in the diagnosis and surgical planning of double outlet of the right ventricle (DORV). MATERIALS AND METHODS: Thirty-four patients who were suspected of DORV based on ultrasound findings underwent cardiac computed tomography angiography (CTA). The patients were assigned randomly to the MR holographic guidance (MRHG) group or the control group. For the patients in the MRHG group, the CTA images were converted into Standard Template Library (STL) files after segmentation, 3D reconstruction, colourisation, and transparentisation, and then exported for MR holographic visualisation. The CTA images of the patients in the control group were analysed using routine 3D reconstruction only. Diagnostic accuracy and surgical planning were compared between the two groups based on visualisation at surgery. RESULTS: In the MRHG group, the 3D hologram observation was in concordance with the actual anatomical findings, and the DORV type was classified accurately in all patients. The diagnostic accuracy for the malformation was 95.5% in the MRHG group and 89.7% in the control group, but the difference was not significant (p=0.3). All the procedures were exactly the same as planned based on the 3D MR holographic model. The surgical planning time was shorter for the MRHG group (51.65 ± 11.11 min) than that for the control group (65.71 ± 18.07 min, p<0.05). CONCLUSION: MR 3D holograms may provide a clear and deeper anatomical perception of DORV and improve surgical planning.

Right aortic arch with brachio-bicephalic trunk mimicking an incomplete double aortic arch with distal left arch atresia.

We report a case of a 6-month-old girl with double outlet right ventricle where a right aortic arch with an abnormally posterior brachio-bicephalic trunk on the left side closely resembled an incomplete double-aortic-arch with distal left arch atresia on computed tomography (CT) angiography. The case highlights the role of preoperative CT angiography in demonstrating aortic arch and arch vessel anatomy in patients with complex congenital heart diseases.

Pulmonary ductal coarctation: An entity associated with congenital heart defects involving the right ventricle outflow tract.

We present a case of a prematurely born 2-month-old girl with a double outlet right ventricle with pulmonary atresia and a left pulmonary artery coarctation arising after ductal closure. The case highlights the importance of knowledge and identification of such an anomaly in patients with congenital heart malformations with severe pulmonary stenosis to atresia.

Prenatal diagnosis of double outlet right ventricle with intact ventricular septum in two foetuses.

We describe two cases of an unusual variant of double outlet right ventricle with intact ventricular septum diagnosed prenatally and confirmed by foetal autopsy in a case. The first case had mitral valve atresia, slit-like left ventricle, and normally related great arteries. The second case had mitral valve atresia, hypoplastic left ventricle, parallel outflows with an interrupted aortic arch.

Repeated pregnancies in uncorrected double outlet right ventricle: a rare occurrence.

Pregnancy in a woman with double outlet right ventricle, ventricular septal defect, and pulmonary stenosis is presented. Her previous two pregnancies ended in fetal losses. In the current pregnancy, she had thrombocytopenia, placenta previa, and severe fetal growth restriction. She underwent an emergency caesarean section followed by post-partum haemorrhage and COVID-19 infection. Literature review has been performed.

Prenatal diagnosis of double-outlet right ventricle with tricuspid valve atresia, anomalous pulmonary vein connection, persistent left superior vena cava, and right atrial isomerism.

Double-outlet right ventricle is a conotruncal cardiac disease in which both the aorta and the pulmonary artery predominantly or completely originate from the right ventricle. Here, we report a complex variant double-outlet right ventricle detected in utero and identified on the basis of a segmental approach.

[Ross-Konnno Procedure for the Patient of Intractable Subaortic Stenosis after Operation of Double Outlet Right Ventricle with Coarctation of the Aorta and Subaortic Stenosis:Report of a Case].

Recurrent left ventricular outflow obstruction (LVOTO) after intraventricular rerouting (IVR) with ventricular septal defect (VSD) enlargement and aortic arch repair for double-outlet right ventricle (DORV), non-committed VSD and coarctation of the aorta was successfully performed by a Ross-Konno procedure at 1 year 6 months as the second reoperation. Ross-Konno procedure could be an effective option in treatment of recurrent LVOTO after IVR for DORV.

Percutaneous edge-to-edge repair for common atrioventricular valve regurgitation in a patient with heterotaxy syndrome, single ventricle physiology, and unbalanced atrioventricular septal defect.

Congenital heart disease patients, specifically with unbalanced atrioventricular septal defects and common atrioventricular valves requiring single ventricle palliation, have substantial morbidity and mortality. Atrioventricular valve regurgitation (AVVR) is associated with poor outcomes in single ventricle patients, and many of them require surgical treatment of AVVR in their lifetimes. We describe a unique case of transcatheter edge-to-edge valve repair using the MitraClip system (Abbott, Chicago, IL) in a single ventricle patient with severe common AVVR.

Prediction of pulmonary pressure after Glenn shunts by computed tomography-based machine learning models.

OBJECTIVES: This study aimed to develop non-invasive machine learning classifiers for predicting post-Glenn shunt patients with low and high risks of a mean pulmonary arterial pressure (mPAP) > 15 mmHg based on preoperative cardiac computed tomography (CT). METHODS: This retrospective study included 96 patients with functional single ventricle who underwent a bidirectional Glenn procedure between November 1, 2009, and July, 31, 2017. All patients underwent post-procedure CT, followed by cardiac catheterization. Overall, 23 morphologic parameters were manually extracted from cardiac CT images for each patient. The Mann-Whitney U or chi-square test was applied to select the most significant predictors. Six machine learning algorithms including logistic regression, Naive Bayes, random forest (RF), linear discriminant analysis, support vector machine, and K-nearest neighbor were used for modeling. These algorithms were independently trained on 100 train-validation random splits with a 3:1 ratio. Their average performance was evaluated by area under the curve (AUC), accuracy, sensitivity, and specificity. RESULTS: Seven CT morphologic parameters were selected for modeling. RF obtained the best performance, with mean AUC of 0.840 (confidence interval [CI] 0.832-0.850) and 0.787 (95% CI 0.780-0.794); sensitivity of 0.815 (95% CI 0.797-0.833) and 0.778 (95% CI 0.767-0.788), specificity of 0.766 (95% CI 0.748-0.785) and 0.746 (95% CI 0.735-0.757); and accuracy of 0.782 (95% CI 0.771-0.793) and 0.756 (95% CI 0.748-0.764) in the training and validation cohorts, respectively. CONCLUSIONS: The CT-based RF model demonstrates a good performance in the prediction of mPAP, which may reduce the need for right heart catheterization in post-Glenn shunt patients with suspected mPAP > 15 mmHg. KEY POINTS: • Twenty-three candidate descriptors were manually extracted from cardiac computed tomography images, and seven of them were selected for subsequent modeling. • The random forest model presents the best predictive performance for pulmonary pressure among all methods. • The computed tomography-based machine learning model could predict post-Glenn shunt pulmonary pressure non-invasively.