Double Outlet Left Ventricle with Intact Ventricular Septum: A Rare Prenatally Diagnosed Case Report.

INTRODUCTION: Double outlet left ventricle (DOLV) is a rare congenital heart anomaly, and cases of DOLV with an intact ventricular septum are uncommon. To date, only four such cases have been reported in the medical literature. CASE PRESENTATION: This report presents a case of prenatally diagnosed DOLV. A fetal echocardiogram at 21 weeks of gestation demonstrated both great arteries, aorta and pulmonary artery, arising from the left ventricle with severely dysplastic tricuspid valve and severe hypoplasia of the right ventricle. Subsequent echocardiograms demonstrated no ventricular septal defect. The patient required balloon atrial septostomy in the first week of life, underwent pulmonary artery banding at 5 weeks of life, and is currently status post-bidirectional Glenn, and is awaiting final Fontan palliation. CONCLUSION: Prenatal diagnosis aided in predicting and guiding postnatal management.

Double-Outlet Left Ventricle: Case Series and Systematic Review of the Literature.

Double-outlet left ventricle (DOLV) is an abnormal ventriculo-arterial connection characterized by the origin of both great arteries from the morphological left ventricle. The aim of our paper is to describe the morphological and imaging features of DOLV and to assess the prevalence of the associated malformations and their surgical outcomes. METHODS From 2011 to 2022, we retrospectively reviewed the electronic case records of patients diagnosed with DOLV at the Bambino Gesu Children's Hospital. A systematic search was developed in MEDLINE, Web of Science, and EMBASE databases to identify reports assessing the morphology and outcomes of DOLV between 1975 and 2023. RESULTS: Over a median follow-up of 9.9 years (IQR 7.8-11.7 y), four cases of DOLV were identified at our institution. Two patients were diagnosed with (S,D,D) DOLV subaortic VSD and pulmonary stenosis (PS): one patient had (S,D,D) DOLV with doubly committed VSD and hypoplastic right ventricle, and another patient had (S,D,L) DOLV with subaortic VSD and PS (malposition type). Pulmonary stenosis was the most commonly associated lesion (75%). LITERATURE REVIEW: After systematic evaluation, a total of 12 reports fulfilled the eligibility criteria and were included in our analysis. PS or right ventricular outflow tract obstruction was the most commonly associated lesion (69%, 95% CI 62-76%). The most common locations of VSD were subaortic (pooled prevalence: 75%, 95% CI 68-81), subpulmonary (15%, 95% CI 10-21), and doubly committed (7%, 95% CI 4-12). The position of the great arteries showed that d-transposition of the aorta was present in 128 cases (59% 95% CI 42-74), and l-transposition was present in 77 cases (35%, 95% CI 29-43).

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.

Delving into the Molecular World of Single Ventricle Congenital Heart Disease.

PURPOSE OF REVIEW: Given a general lack of emphasis on the molecular underpinnings of single ventricle (SV) congenital heart diseases (CHD), our review highlights and summarizes recent advances in uncovering the genetic and molecular mechanisms in SV CHD etiology. RECENT FINDINGS: While common SV-associated genetic mutations were found in key cardiac transcription factors, other mutations were sporadic. With advances in genetic sequencing technologies and animal models, more disease-associated factors have been identified to act in critical cardiac signaling pathways such as NOTCH, Wnt, and TGF signaling. Recent studies have also revealed that different cardiac lineages play different roles in disease pathogenesis. SV defects are attributed to complex combinations of genetic mutations, indicating that sophisticated spatiotemporal regulation of gene transcription networks and functional cellular pathways govern disease progression. Future studies will warrant in-depth investigations into better understanding how different genetic factors converge to influence common downstream cellular pathways, resulting in SV abnormalities.

Role of sildenafil in a neonate with double outlet left ventricle and pulmonary hypertension.

Double outlet left ventricle (DOLV) is a rare congenital heart defect where the aorta and the pulmonary trunk arise predominantly from the morphologic left ventricle. The clinical manifestations depend upon the location of the ventricular septal defect in relation to the great arteries, degree of pulmonary and aortic outflow tract obstruction, and other associated cardiac defects. We describe a neonate with DOLV, sub-aortic ventricular septal defect, and side-by-side great vessels who continued to require oxygen for desaturation to 60% in the neonatal period. His clinical symptoms were suggestive of persistent pulmonary hypertension, with 5-8% higher saturations in the leg compared to arm. He was started on oral sildenafil and his oxygen requirement decreased from 2 L/min to 0.1 L/min within 24 hours of initiating sildenafil. .

Arterial switch for double-outlet left ventricle - Diagnostic and surgical considerations.

Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly in which both the aorta and pulmonary artery arise completely or predominantly from the left ventricle. DOLV is a spectrum and can be classified depending on the position of the ventricular septal defect (VSD) relative to the great vessels, the relationship of the great vessels, and the presence or absence of pulmonary or aortic outflow obstruction. In the absence of tricuspid atresia or hypoplastic right ventricle, two ventricle repair is the preferred surgical treatment. We report a 31-day-old, 2.1 kg neonate with DOLV, subaortic VSD who underwent a successful arterial switch with VSD closure.

Role of Transesophageal and Epicardial Echocardiography to Assess Surgical Repair in Double-Outlet Left Ventricle.

A double-outlet left ventricle (DOLV) is a congenital cardiac anomaly that rarely is encountered. This case report demonstrates the echocardiographic features of DOLV in the form of the pulmonary artery arising completely from the left ventricle with D-looped ventricles, especially when evaluated by intraoperative transesophageal echocardiography (TEE), along with the correlation of characteristic features with cardiac computerized tomography. The features pertinent to the differentiation of DOLV from double-outlet right ventricle and congenitally corrected transposition of the great arteries by echocardiography have been described. To the authors' knowledge, this was the first report of intraoperative TEE in the case of DOLV. In addition, the use of intraoperative echocardiography to rule out coronary compression as a cause for post-repair ventricular tachycardia, by use of TEE to rule out ventricular dysfunction and regional wall motion abnormalities, as well as epicardial echocardiography to demonstrate normal coronary blood flow, has been reported.

Mechanism of ventricular tachycardia in a patient with double-outlet left ventricle.

We report the case of ventricular tachycardia (VT) ablation procedure in a patient with history of surgically repaired double-outlet left ventricle. The electrophysiology procedure revealed a re-entry pattern between the right-ventricle to main-pulmonary-artery conduit and the tricuspid annulus. The re-entrant mechanism was most likely promoted by a fibrous remodeling of this area, related to the surgical repair. This case is the first to describe a re-entry mechanism between fixed anatomical barriers in a repaired right ventricle of a double-outlet left ventricle. A pace mapping technique was used to highlight the VT isthmus.

Bi-ventricular repair of double outlet left ventricle: Experience and review of the literature.

BACKGROUND: Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly. The aorta and the main pulmonary arterial trunk arises predominantly from the left ventricle and is associated with a malaligned ventricular septal defect, various degrees of hypoplasia of the right ventricle, and presence, or absence of pulmonary stenosis. Bi-ventricular repair is the preferred treatment option whenever possible. Multiple techniques for bi-ventricular repair have been described. The best option for DOLV correction is by translocating the pulmonary root from the left ventricle to the right ventricle. In this series, we report four patients who underwent biventricular repair of DOLV with excellent outcomes. METHODS: This is a retrospective study of four patients who underwent surgical correction of DOLV between January 2014 and December 2018. We collected all patient details from the institute patient record system. Echocardiographic data were obtained from the records. Intraoperative charts were reviewed for further information on the surgical procedure and cardiopulmonary bypass. Postoperative data included survival, functional status, and followup echocardiography. RESULTS: Of the four children, three underwent pulmonary root translocation, and one child underwent a Reparation al etage Ventriculaire (REV) procedure. There was no mortality in our series, and all children are in a stable clinical condition in the recent follow-up, and no re-operations or interventions were required following primary surgical correction. CONCLUSION: DOLV is anatomically and surgically a challenging subset. Pulmonary root translocation in this anatomy is technically challenging but safe and superior option when compared to other alternative surgical procedures. Pulmonary root translocation can be performed with excellent results, even in infants.

A case of double-outlet left ventricle, malposition of the great arteries, and superior-inferior ventricular arrangement.

Superior-inferior ventricular arrangement with double-outlet left ventricle and malposition of the great arteries is an extremely rare congenital cardiac anomaly. The authors present the case of an infant who presented with cyanosis and respiratory distress. To the best of our knowledge, this is the first case reported.

Double-outlet left ventricle: A rare case.

Double-outlet left ventricle (DOLV) is a rare cardiac malformation in which both great arteries originate from the morphological left ventricle. DOLV is associated with high mortality, generally due to heart failure, myocardial infarction, or aortic thrombosis. With surgery, the 5-year survival rate is estimated at 70%-75%. Most patients will continue to present with residual cardiac anomalies, such as aortic or mitral valve regurgitation, arrhythmias, or hypertension. Here, we report a 25-year-old male with DOLV with pulmonary stenosis and patent ductus arteriosus who presented to us with hemoptysis, which was due to respiratory tract infection. He improved with standard therapy.

Double-outlet left ventricle with a bicuspid pulmonary valve and aortic coarctation.

A double-outlet left ventricle (DOLV) is a rare congenital cardiac malformation. Here, we describe a case of DOLV with bicuspid pulmonary valve, aortic coarctation, and a subpulmonary ventricular septal defect in which both ventricles were well developed. The anatomic features were observed with echocardiography, and the diagnosis was confirmed at surgery. Many variations of DOLV have been described. The features of our case expand the spectrum of this entity and may provide new insight into its complex anatomy.

Three-dimensional printing of a complex CHD to plan surgical repair.

We used three-dimensional printing technology to create an anatomical three-dimensional model of a very rare and complex cyanotic CHD in a newborn, consisting of double-outlet left ventricle, ventricular septal defect, and pulmonary stenosis. This case demonstrates how this new innovative technology allows better understanding of the anatomy in complex CHDs and permits to better plan the surgical repair.

Brain infarction with a predilection for cerebellum in a patient with double-outlet left ventricle and vascular malformations.

Double-outlet left ventricle (DOLV) is a rare congenital heart disease characterized by the origin of the great arteries arising predominantly or completely from the left ventricle. In this report, we describe a case with brain infarction with a predilection for cerebellum in a patient with DOLV and vascular malformations. The cerebellar predilection of ischemic lesions appeared to have been caused by hemodynamic effects related to the specific anatomy of the brachiocephalic trunk. This is further supported by our observation that the mean flow velocity was significantly higher in the vertebral arteries than in the common carotid arteries.

Two cases of double-outlet left ventricle detected prenatally.

Double-outlet left ventricle is an exceedingly rare congenital heart defect. Its prenatal detection and precise anatomical definition are challenging for a variety of reasons and have never been previously reported. Here described are 2 cases of prenatally diagnosed double-outlet left ventricle. The technical limitations of prenatal diagnosis and its implications for the surgical management of patients affected by such a rare condition are discussed.

Pulmonary Trunk Translocation from LV to RV in Double-Outlet Left Ventricle: A case report / 대한흉부외과학회지

Double-outlet left ventricle(DOLV) is a rare congenital cardiac malformation, defined as the origin of both the aorta and the pulmonary artery being entirely or predominantly above the morphologically left ventricle, which is difficult to diagnose accurately. A 3-year old male was admitted for cyanosis and dyspnea. At the age of 2 months, he had undergone pulmonary artery banding and coarctoplasty. He was diagnosed as DOLV with subaortic ventricular septal defect(VSD). Biventricular repair was achieved by patch closure of VSD, primary closure of PFO, and pulmonary trunk translocation from left ventricle to right ventricle. The advantages of this procedure using native tissue for right ventricular outflow tract reconstruction are growth potential and preserved valve function, which contribute to a decreased likelihood of reoperation related to the right ventricular dysfunction related to pulmonary insufficiency.

Anatomical Repair of Double-Outlet Left Ventricle with Ventricular Septal Defect and Pulmonary Stenosis by Reight Ventricular Outflow Patch Reconstruction / 대한흉부외과학회지

Double-outlet left ventricle with ventricular septal defect and pulmonary stenosis was conventionally repaired with extracardiac conduit or pulmonary artery translocation. Here, we report an anatomically repaired double-outlet left ventricle without extracardiac conduit or pulmonary artery translocation in an 11 month old patient who had undergone palliative systemic-pulmonary shunt at a nonatal period. The location of ventricular septal defect, both great arteries and coronary arteries made it possible to reconstruct the right ventricular outflow tract using on-lay patch after incision and undercutting the tissue between the ventriculotomy and the pulmonary arteriotomy.