En Bloc Rotation of the Outflow Tracts - Double Root Translocation.

En bloc rotation of the outflow tracts or double root translocation offers an anatomic repair of transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction and closely related forms of double outlet right ventricle. The technical principle is to excise aortic and pulmonary root en bloc, rotate them as a whole by 180°, and reimplant them. The left ventricular outflow tract is enlarged with the patch closing the ventricular septal defect. In our experience, two thirds of the pulmonary valves could be preserved. Growth of the aortic and pulmonary root could be demonstrated in several studies performed by our group. It is still a complex and technically demanding procedure with long cardiopulmonary bypass periods and cross-clamp times. However, perioperative mortality and complications do not differ significantly from other forms of reconstruction. The reoperation rate is significantly lower. Presently, the best time to perform this operation seems to be after the newborn period within the first year of life.

Complex cyanotic congenital heart disease and cervical spine surgery: Ticking all the right boxes.

Although complex congenital heart disease (CHD) patients usually present in childhood, it is not rare to see adults well past middle age. These patients undergo continuous pathophysiological changes in their heart and blood vessels, making anesthetic management more challenging if surgery is required. Herein, we report a case in which understanding the anatomy and pathophysiology helped optimally manage a patient with a double outlet right ventricle (DORV) who underwent plating and fixation for a hangman's fracture in the prone position.

[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.

Fetal Diagnosis of Scimitar Syndrome in the Presence of Complex Congenital Heart Disease.

Fetal diagnosis of scimitar syndrome requires a high index of suspicion. We present two fetal cases of complex congenital heart disease associated with scimitar syndrome, one of them is diagnosed with scimitar syndrome in utero. We emphasize prenatal echocardiographic findings that may assist with arriving at the correct prenatal diagnosis. We also discuss potential challenges in suspecting the presence of scimitar syndrome in utero. The postnatal echocardiographic findings and course are described for both patients. We reviewed the available literature on prenatal diagnosis of scimitar syndrome in the presence of complex congenital heart disease. We describe a new association of VACTERL, imperforate anus, scimitar syndrome, and double-outlet right ventricle all on the same patient, as well as the first prenatal diagnosis of scimitar syndrome associated with hypoplastic left heart syndrome with restrictive atrial septum. Advanced imaging modalities such as a fetal lung Magnetic Resonance Imaging is suggested as a confirmatory test when scimitar syndrome is suspected in utero in the presence of complex congenital heart disease.

[A case of recurrent cerebral infarction in an adult patient with false Taussig-Bing anomaly].

The case was a 53-year-old woman. At birth, she was diagnosed with a false Taussig-Bing anomaly with pulmonary artery stenosis and a single ventricle. However, no cardiac surgery was performed, and conservative treatment was continued by a cardiovascular surgeon even after adulthood. Because of secondary polycythemia and a history of multiple cerebral infarctions, she took anti-platelet drugs and anti-coagulants. However, she was admitted with the diagnosis of cerebral infarction for the fourth time. It was considered that the patient was at high risk of paradoxical cerebral embolism due to cardiac malformation with cyanotic congenital heart disease accompanied by coagulation abnormalities. Considering the pathophysiology, we decided to use aspirin in combination with warfarin.

Combination of low-dose spinal anesthesia and epidural anesthesia as anesthetic management in patient with uncorrected Double Outlet Right Ventricle (DORV) underwent cesarean section.

Pregnant patients with uncorrected Double Outlet Right Ventricle (DORV) undergoing cesarean section are challenging for anesthesiologists. We present a case of a 24-year-old woman with a gestational age of 30-32 weeks with DORV, ventricular septal defect, pulmonary hypertension, and stage C functional class III heart failure who was successfully managed using a combination of low-dose spinal anesthesia bupivacaine 0.5% 7.5 mg with adjuvant fentanyl 50 mcg and epidural ropivacaine 0.2%, and fentanyl 50 mcg TV 10 cc given 30 minutes after the birth of her baby. Hemodynamics was stable after low-dose spinal anesthesia and until the end of the operation.

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.

Discordant Atrioventricular Connections in Situs Inversus, Dextrocardia with Double Outlet Right Ventricle, Pulmonary Stenosis, and Anomalous Coronary Artery: A Rare Case Report.

We report a case of a 2-year-old female presenting with cyanosis since birth and having rare cardiac anatomy of visceral situs inversus, dextrocardia, discordant atrioventricular connections with double outlet right ventricle, atrial communication, subaortic interventricular communication, anteriorly leftward aorta with right aortic arch, single coronary artery with a branch crossing right ventricle outflow tract, pulmonary stenosis, and bilateral superior vena cava. Corrective repair with atrial switch (Senning technique), intracardiac baffle, and right ventricle outflow reconstruction was successfully performed with challenges from altered anatomical location and orientation of the heart and abnormal coronary artery.

Arterial Switch Operation in Patients With Taussig-Bing Anomaly and Aortic Arch Obstruction.

BACKGROUND: Arterial switch operation (ASO) in children with Taussig-Bing anomaly and associated aortic arch obstruction (AAO) is technically demanding. This study sought to determine outcomes of patients with Taussig-Bing anomaly and AAO who underwent ASO at a single institution. METHODS: From 1983 to 2015, 844 patients underwent an ASO for biventricular repair at The Royal Children's Hospital. Twenty-eight patients (3.3%) had Taussig-Bing anomaly and AAO. RESULTS: Of 28 patients, 21 (75%) underwent ASO and AAO repair as a single-stage procedure and 7 (25%) had arch repair before ASO. There were no early or late deaths. Follow-up after hospital discharge was available for all patients. Median follow-up was 17.5 years (interquartile range, 9.2-22.3; range, 4.0-31.5). All patients were in New York Heart Association class I at last follow-up. Freedom from reintervention was 61% (95% confidence interval, 40-76) and 56% (95% confidence interval, 36-73) at 5 and 15 years, respectively, after ASO. The most common reinterventions were repair of recurrent AAO, branch pulmonary artery stenosis, and neoaortic valve repair. Four of 28 patients (14%) required 5 reinterventions (3 reoperations and 2 catheter reintervention) for recurrent AAO at a median of 2.2 years after ASO (interquartile range, 2.1-2.3). CONCLUSIONS: Patients with Taussig-Bing anomaly and AAO can undergo ASO with excellent survival. Reintervention is equally common for both left- and right-sided obstruction.

Anesthetic Management of a Parturient With Double-Outlet Right Ventricle Obstruction and Pulmonary Stenosis Scheduled for Cesarean Delivery: Case Report and Review of Literature.

Double-outlet right ventricle (DORV) is a rare cardiac condition in which both the aorta and pulmonary artery arise from the right ventricle, resulting in parallel systemic and pulmonary circulations. Usually, DORV is present with ventricular septal defect; however, the location of the ventricular septal defect and presence of pulmonary stenosis (PS) result in various physiological features and subtypes of DORV. Because DORV without PS causes congestive cardiac failure and DORV with PS results in cyanotic heart disease, anesthesia management varies widely according to the resultant physiological characteristics. Reports of anesthesia management in a parturient with DORV undergoing cesarean delivery is scarce because of the low incidence of DORV and the discouragement of these patients to conceive. Only 8 known previous such cases are reported, and almost all these patients were administered regional anesthesia. Here we describe a parturient with DORV, to whom general anesthesia was administered because of incidental antiphospholipid syndrome with low platelets. To the best of our knowledge, this scenario has not been described previously.

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.

Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes.

OBJECTIVES: To document the clinical spectrum and outcomes of fetal double outlet right ventricle (DORV) without heterotaxy in a recent diagnostic era. METHODS: Prenatal cases of DORV consecutively diagnosed from 2007 to 2018 were retrospectively identified. Clinical records, including details regarding genetic testing and pre and postnatal imaging were reviewed. RESULTS: DORV was diagnosed in 99 fetuses without heterotaxy. The most common anatomic subtype was subaortic ventricular septal defect (VSD) and normally related great arteries with (n = 45, 45%) or without (n = 13, 13%) pulmonary stenosis. The remainder had a subpulmonic VSD with transposed great arteries (n = 15, 15%), atrioventricular valve atresia (n = 24, 24%), or remote VSD (n = 2, 2%). A genetic diagnosis was found in 32 (34%) of 93 tested. Major extracardiac anomalies were found in 40 (40%), including 17/24 (71%) with and 22/69 (32%) without an abnormal karyotype, with VACTERL association in 9. Genetic and/or extracardiac pathology was identified in 37/58 (64%) with a subaortic VSD, 5/15 (33%) with a subpulmonic VSD, 9/24 (38%) of those with AV valve atresia and 2/2 (100%) with a remote VSD. A genetic abnormality was a significant predictor of fetal demise (9/37 vs 1/62 p < 0.01) or pregnancy termination (12/35 vs 9/64 p = 0.03). CONCLUSIONS: Fetal DORV is associated with a high rate of genetic abnormalities and extracardiac pathology. The presence of genetic abnormalities impacts prenatal outcomes and parental decision-making.

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.

Late Neo-Aortic Valve Regurgitation Long After Arterial Switch Operation.

BACKGROUND: The purpose of this study is to assess risk factors of neo-aortic regurgitation (AR) after an arterial switch operation and outcomes of neo-aortic valve surgery. METHODS: This is a retrospective study of 469 hospital survivors after arterial switch operation from 1982 to 2016. Preoperative diagnoses included 285 transpositions of the great arteries with intact septum, 140 transpositions with ventricular septal defect, and 44 double-outlet right ventricles. More than mild preoperative pulmonary regurgitation was found in 15 patients. Median age at the operation was 1.0 months (range, 0.1-81.5). Concomitant relief of left ventricular outflow tract obstruction was performed in 20 patients. Seventy-seven patients had more than mild neo-AR at hospital discharge. RESULTS: More than moderate neo-AR was found in 41 patients (8.6%) at a median follow-up of 19.0 years (range, 0.1-35.2). Multivariate analysis identified more than mild preoperative pulmonary regurgitation, concomitant relief of left ventricular outflow tract obstruction, and more than mild neo-AR at hospital discharge as risk factors for late neo-AR. Seventeen patients (3.6%) underwent neo-aortic valve surgeries, including 11 aortic valve replacements, 3 aortic valve plasties, 2 Konno procedures, and 1 aortic root replacement, with median age at the neo-aortic valve surgery of 16.5 years (range, 6.2-27.3). There were 2 late deaths and 5 reoperations to the neo-aortic valve in 4 patients. CONCLUSIONS: Concomitant relief of left ventricular outflow tract obstruction and preoperative more than mild pulmonary regurgitation increase the risk of neo-AR after the arterial switch operation. Reoperation for neo-AR can be done safely.

Fetuses with single ventricle congenital heart disease manifest impairment of regional brain growth.

OBJECTIVE: Anomalous neurological development associated with congenital heart disease (CHD) has been reported as early as third trimester of fetal development. While several studies have characterized variations in CHD neurodevelopmental outcomes in early childhood, these reports are often confounded by postnatal factors such as surgical outcome. Recent studies have focused on the comparing neurological variations between fetuses with CHD and normal controls. In this work, we present a comparison of in utero variations in brain development between fetuses with different types of CHD, by analyzing them under categories of single ventricle versus biventricular cardiac anatomy. METHODS: Using recent advances in fetal magnetic resonance imaging (MRI), we quantify the volumetric trajectories of various brain tissues (such as cortical plate, developing white matter, cerebrospinal fluid [CSF], and cerebellum). RESULTS: Our study is the first to differentiate between intraventricular and extra-axial CSF thereby allowing us to better identify variations in brain composition of the fetuses. CONCLUSIONS: Overall, our findings show that while total brain volume is similar between fetuses with single and biventricular anatomy, they exhibit statistically significant disparity in brain composition.

[Successful Staged Surgical Management for Double Outlet Right Ventricle with Ebstein's Anomaly and Aortic Coarctation;Report of a Case].

A combination of double outlet right ventricle(DORV) with Ebstein's anomaly and aortic coarctation (CoA) is vary rare. We report a successful staged surgical repair for DORV with Ebstein's anomaly associated with CoA. The patient had subaortic perimembronous ventricular septal defect (10×7.5 mm) and small atrial septal defect( 3 mm). Two great arteries were side by side position. The posterior leaflet of the tricuspid valve was 10 mm displaced to the right ventricle and tricuspid valve regurgitation was severe. The patients was unsuitable for primary biventricular repair because the size of the mitral valve was considered to be small. First, bilateral pulmonary artery banding was performed after balloon atrio-septostomy in the neonatal period. Three months after the 1st operation, cardiac catheteraization revealed that the left and right heart structure was suitable for biventricular repair. At 4-month-old-age, aortic arch reconstruction, intra-cardiac rerouting and tricuspid valve repair were performed. Post operative tricuspid valve regurgitation was mild. The patient was discharged on the 12th postoperative day with good hemodynamic condition.