Diagnosis Of Double Outlet Right Ventricle Research Articles

Follow-up of a Term Infant with a Prenatal Diagnosis of Double-Outlet Right Ventricle.

Follow-up of a Term Infant with a Prenatal Diagnosis of Double-Outlet Right Ventricle.

Is combined MDCT and echocardiography needed to guarantee accuracy in diagnosis and surgical planning of DORV and associated anomalies?

BackgroundThe 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.ResultsA 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.ConclusionsOur 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.

The independence of the infundibular building blocks in the setting of double-outlet right ventricle.

It has long been contentious as to whether the presence of bilateral infundibulums, or conuses, is a prerequisite for the diagnosis of double-outlet right ventricle. As the use of such a criterion would abrogate the so-called "morphological method", which correctly states that one variable entity should not be defined on the basis of another entity that is itself variable, it is now accepted that double outlet can exist in the setting of fibrous continuity between the leaflets of the atrioventricular and arterial valves. Although this debate has now been resolved, there are other contentious areas still requiring clarification in the setting of hearts unified because of the presence of this particular ventriculo-arterial connection - for example, it is questionable whether the channel between the ventricles should be described as a "ventricular septal defect", whereas it is equally arguable that the mere presence of fibrous continuity between the leaflets of the arterial valves does not necessarily place the channel in a doubly committed location. In this review, we describe a series of autopsied hearts in which the anatomical features serve to illuminate these various topics. We then discuss recent findings regarding cardiac development that point to the individuality of the building blocks of the ventricular outflow tracts, specifically the outlet septum, the inner heart curvature, or ventriculo-infundibular fold, and the septomarginal trabeculation, or septal band.

Three-Dimensional Reconstruction of Intracardiac Anatomy Using CTA and Surgical Planning for Double Outlet Right Ventricle: Early Experience at a Tertiary Care Congenital Heart Center.

Although transthoracic echocardiography (TTE) routinely establishes the diagnosis of double outlet right ventricle (DORV), it can be suboptimal for depicting exact ventricular septal defect (VSD) position, especially with respect to the outflow tracts. Advanced imaging with computed tomography angiography (CTA) can help visualize structures and relationships not easily seen by echo. Using computer-aided design, we have the ability to create three-dimensional (3D) models of the intracardiac anatomy, which can be helpful for better depicting the overall anatomy to assist surgical planning. Patients with a diagnosis of DORV were retrospectively reviewed at our institution from October 2013 to April 2015. Patients who preoperatively underwent both TTE and CTA with 3D reconstruction of the intracardiac anatomy were included. Computed tomography angiography findings with 3D intracardiac model creation were compared to the surgical findings. Twenty-five patients underwent surgical repair of DORV during the study period. Five patients had CTA with 3D reconstruction, in addition to the standard TTE images, and were included in the study. In all five cases, CTA with 3D reconstruction of the intracardiac anatomy accurately depicted the VSD position relative to important adjacent structures, including the outflow tracts. Three-dimensional reconstruction of the intracardiac anatomy using CTA data can provide accurate data for presurgical planning of DORV repair and has the potential for being especially useful in patients for whom intracardiac anatomy and VSD position cannot be well seen by TTE. A larger prospective analysis is warranted to help validate this approach.

Perioperative Management of DORV

The diagnosis of double-outlet right ventricle (DORV) characterizes a complex heterogeneous group of congenital cardiac malformations for which multiple classification schemes have been used. A clear understanding of the anatomy is critical to understanding the physiologic consequences of the specific type of DORV. Perioperative considerations include the medical management of the patient during the preoperative period, anesthetic and surgical management, and postoperative care. Both anesthetic and surgical management strategies are very different depending on the type of DORV. Key principles for anesthetic management include balancing the systemic and pulmonary circulations, optimizing systemic cardiac output, and closely monitoring for impaired oxygen delivery to the tissues. Depending on the specific anatomy the patient is usually placed on a 1- or 2-ventricle pathway, and initial palliation may involve placement of a systemic arterial to pulmonary artery shunt or pulmonary artery banding. In some cases the child may undergo a complete repair during the first few months of life. Surgical outcomes, both short and long-term, are dependent on the type of DORV and surgical procedure done. These patients require long-term follow up and may present for surgical or catheter-based interventions as adults.

Double‐outlet right ventricle in a 10‐month‐old Friesian filly

A 10-month-old Friesian filly had a presentation that was consistent with chronic left- and right-sided congestive heart failure. Clinical pathology findings included abnormal haematological and biochemical variables, abnormal blood gas values and increased serum concentration of cardiac troponin I. Echocardiography revealed cardiac chamber dilation and dextropositioning of the aorta. Radiography revealed a generally enlarged heart and pulmonary interstitial infiltration. These findings were supported at necropsy and the diagnosis of double-outlet right ventricle was confirmed. The pathological changes and physiological responses subsequent to double-outlet right ventricle have not previously been described in detail in horses. Clinical progression closely resembles that seen in humans, in whom antemortem diagnosis relies on echocardiography. In horses, complex cardiac disease presents a diagnostic challenge to the clinician. Appropriate therapy must be based on an accurate diagnosis.

Thrombotic occlusion of Blalock-Taussig shunt in a patient with unnoticed protein C deficiency

A 6-month-old girl with a diagnosis of double-outlet right ventricle and pulmonary stenosis had a left modified Blalock-Taussig shunt. Chest computed tomography (CT) performed on postoperative day 11 showed good patency of the shunt. However, on postoperative day 16, oxygen saturation suddenly dropped below 40%, and chest CT showed thrombotic occlusion of the shunt. Urgent thrombectomy was performed successfully. Examination of coagulation factors revealed low levels of both the amount and activity of protein C (27% and 31%, respectively). Diagnosis of heterozygous hereditary protein C deficiency was made, and the patient was placed on warfarin. She is currently in good condition.

Diagnosis and Prognosis in Double-Outlet Right Ventricle

The aim of this study was to examine prenatal diagnosis of double-outlet right ventricle (DORV)-associated anomalies and prognosis of each case. Medical records were reviewed of fetuses with DORV who had fetal echocardiography at our institution from 2002 to 2006. Pre- and postnatal diagnosis and outcome were compared and evaluated. Twenty-one fetuses were diagnosed with DORV. The pregnancy was terminated in seven cases. Three cases had chromosomal abnormalities; three cases, hypoplastic left ventricle; and one case, encephalocele. Accurate prenatal diagnosis of the ventricular septal defect, outflow obstruction, and great artery relationship was achieved in 14 of 16 cases (87.5%). Only 2 of 13 live-born cases survived beyond 6 months. The overall prognosis for fetuses with DORV is poor. DORV is found in fetuses with a huge spectrum of associated cardiac and extracardiac anomalies. Careful assessment by fetal echocardiography can determine important anatomic details with adequate correctness for precise counseling.

OP21.12: Perinatal outcome after prenatal diagnosis of double outlet right ventricle

Objectives: To describe the accuracy of diagnosis, associated anomalies and perinatal survival of fetuses diagnosed with double outlet right ventricle (DORV). Methodolgy: During a four-year period, DORV was diagnosed in 15 fetuses. Their medical records were reviewed and analyzed. Reliability of diagnosis was assessed by postnatal examination and autopsy in cases of termination of pregnancy or perinatal death. Results: The median maternal age was 31 years-old (range 16–40) and the median gestational age at diagnosis 21 weeks (range 15–33). The indication for examination was an abnormal screening ultrasound in 13 cases and an increased nuchal translucency in the remaining 2 cases. Additional cardiac lesions detected were: mitral atresia with hypoplastic left ventricle (n = 5), pulmonary stenosis (n = 3), coarctation of the aorta (n = 4). Transposition were the most common arrangement of the great arteries (n = 12). Chromosomal abnormalities were found in 5 cases (33%): 1 trisomy 21, 2 trisomies 18 and 2 microdel 22q11-. All the diagnoses were confirmed at follow-up, 10 parents opted for termination (67%), 3 cases died either before or after surgery, and only 2 of the infants (13% of the whole group, 40% of the intented to treat) survived beyond 28 days. Conclusions: Prenatal diagnosis of DORV is highly accurate. The prognosis is extremely poor, not only related to the primary lesion but also depending on the associated chromosomal and intra-cardiac anomalies.

OP21.13: National registry of fetal cardiac pathology in Poland (ORPKP)

Objectives: To describe the accuracy of diagnosis, associated anomalies and perinatal survival of fetuses diagnosed with double outlet right ventricle (DORV). Methodolgy: During a four-year period, DORV was diagnosed in 15 fetuses. Their medical records were reviewed and analyzed. Reliability of diagnosis was assessed by postnatal examination and autopsy in cases of termination of pregnancy or perinatal death. Results: The median maternal age was 31 years-old (range 16–40) and the median gestational age at diagnosis 21 weeks (range 15–33). The indication for examination was an abnormal screening ultrasound in 13 cases and an increased nuchal translucency in the remaining 2 cases. Additional cardiac lesions detected were: mitral atresia with hypoplastic left ventricle (n = 5), pulmonary stenosis (n = 3), coarctation of the aorta (n = 4). Transposition were the most common arrangement of the great arteries (n = 12). Chromosomal abnormalities were found in 5 cases (33%): 1 trisomy 21, 2 trisomies 18 and 2 microdel 22q11-. All the diagnoses were confirmed at follow-up, 10 parents opted for termination (67%), 3 cases died either before or after surgery, and only 2 of the infants (13% of the whole group, 40% of the intented to treat) survived beyond 28 days. Conclusions: Prenatal diagnosis of DORV is highly accurate. The prognosis is extremely poor, not only related to the primary lesion but also depending on the associated chromosomal and intra-cardiac anomalies.

Atypical Fallot's tetralogy with doubly committed subarterial ventricular septal defect: Diagnostic value of 2-dimensional echocardiography

Five patients with clinical features of tetralogy of Fallot had subarterial doubly committed ventricular septal defect (VSD) with absence or deficiency of the infundibular septum. Two-dimensional (2-D) echocardiography differentiated this special group from those with the usual Fallot's anatomy. In addition to the long-axis view of the left ventricle which showed the subaortic VSD and the aortic and mitral fibrous continuity, the short-axis view at the cardiac base clearly demonstrated the subpulmonary extension of the VSD in the atypical group. Two-dimensional echocardiography was found superior to biplane angiography, which in 3 patients suggested the erroneous diagnosis of double-outlet right ventricle with pulmonic valve stenosis. This variant form of Fallot's tetralogy poses special surgical problems.

Echocardiographic Diagnosis of Double-Outlet Right Ventricle

An echocardiographic technique (V scan) for confirming the diagnosis of double-outlet right ventricle is described. The technique demonstrates a frequent pathologic feature of double-outlet right ventricle, ie, origin in both great arteries side by side and anterior to the ventricular septum. The V scan offers greater diagnostic accuracy than the mere demonstration of mitral-semilunar valvular discontinuity.

Hemodynamic and anatomic correlation of electrocardiogram in double-outlet right ventricle.

Scalar electrocardiograms of 31 patients with double-outlet right ventricle (19 with and 12 without pulmonary stenosis) were correlated with the hemodynamic and anatomic findings. Atrioventricular conduction defects, intraventricular conduction delay, and counterclockwise frontal-plane loops were found less often than formerly reported. Counterclockwise frontal-plane loops were found with, as well as without, pulmonary stenosis. The presence or absence of left ventricular hypertrophy did not correlate with the severity of pulmonary vascular obstructive disease. Electrocardiographic evidence of left atrial enlargement was difficult to interpret. Additional intracardiac malformations could not be appreciated from the electrocardiographic findings. The predictive value of the electrocardiogram for the diagnosis of double-outlet right ventricle was considered to be low. In patients with double-outlet right ventricle without pulmonary stenosis, the electrocardiogram was not helpful in predicting the severity of pulmonary vascular obstructive disease.