Outlet Of Right Ventricle Research Articles

Experience in the Treatment of Pentalogy of Cantrell with Artificial Materials in a Single Clinical Center.

To summarize experience in the treatment of pentalogy of Cantrell (POC) in our hospital and explore the effect of artificial materials in repairing sternal defects. A retrospective analysis was performed on treatment of five children with POC treated by using the Gore-Tex patch and titanium mesh in the Department of Cardio-Thoracic Surgery, Children's Hospital of Chongqing Medical University, from January 2010 to January 2019. The concurrent conditions included double outlet of right ventricle (n = 2), ventricular septal defect (VSD) and atrial septal defect (ASD) (n = 1), VSD and ASD and patent ductus arteriosus (n = 1), and VSD and left ventricular diverticulum (n = 1) in five cases with POC. Color Doppler echocardiography and computed tomography (CT) + three-dimensional (3D) reconstruction of the thorax and abdomen were performed preoperatively. The cardiac malformation was corrected according to color Doppler echocardiography, and a Gore-Tex patch was used to repair the pericardial defect. Titanium mesh was made according to CT 3D reconstruction with a 3D printing mold to repair sternal defects. All patients underwent a one-stage operation, all hearts were eventually repositioned, no deaths occurred after the operation, and follow-up was performed for 6 months to 2 years. The patients recovered well, and the exterior thorax was normal. The diagnosis of POC is not difficult. The priority of surgical treatment for POC is to obtain satisfactory corrections of cardiac malformation. The repair of the pericardial defect with the Gore-Tex patch and the sternal defect with the titanium mesh can make the heart return to the mediastinum, reduce the pressure on the heart, reduce the surgical trauma, reduce the difficulty of repairing the sternal defect, and optimally restore the exterior thorax.

Abstract 16895: Lrp1, an Endocytic Vesicle Trafficking Protein, is Associated With Congenital Heart Diseases - Through a "non-Autonomous" Action

Backgrounds: Congenital heart diseases affect nearly 1% of neonates per year in the United States and are the leading cause of neonatal death. Despite a strong indication of genetic contribution to congenital heart diseases, the developmental processes and genetic components are poorly understood. From the large-scale forward genetic screen using inbred mice chemically mutagenized with ethyl-nitrosourea (ENU), a novel finding of endocytic trafficking gene mutations, including Lrp1, were recovered as causing congenital heart disease phenotypes. Endocytosis plays roles in modulating cell signaling by regulating the trafficking of the internalized endocytic vesicles to different endosomal compartments, some destined for fusion with lysosomes and degradation while others are recycled to the surface by recycling endosomes. Mutations in these genes causes a spectrum of congenital heart disease comprising of outflow tract abnormalities and alignment defects. Methods and Results: The LRP1 [line 1554(MGI 96828)] mutant mouse line recovered from a forward genetic screen results from a missense (C4232R) mutation in the region encoding the epidermal growth factor (EGF) repeat domain of LRP1. The mutants exhibit a spectrum of septation and outflow tract defects including endocardial cushion defects, double outlet of right ventricle with normal related great arteries as well as DORV with D-malposition of great arteries (Taussig-Bing anomalies). Mouse embryonic fibroblasts (MEF) from LRP1 m/m mutants demonstrated decreased cell-surface expression and increased retention in the endosomes. Migration assay of MEF demonstrated decreased migration of LRP1 m/m compared with control with normal Golgi orientation. The decreasing migration LRP1 m/m also observed in the endocardial cushion tissue from E9.5-10.5 embryos. Different cell lineages crucial to cardiac development with tissue-specific genetic manipulations were used to define the roles of the LRP1 pathway in these three fields of cells during outflow tract development and septation. Cardiac neural crest cell (Wnt1-Cre) knockout of LRP1 recapitulate LRP1 m/m cardiac phenotypes as well as Nkx2.5/Tie2-cre double knockout of LRP1. RNA expression profile suggests Notch pathway and integrin pathway are associated with LRP1 during cardiac development. Conclusions: We provide the first evidence of endocytic vesicle trafficking pathway associated with the development of congenital heart diseases with malalignment/septation and outflow tract defects

Comparing levocardia and dextrocardia in fetuses with heterotaxy syndrome: prenatal features, clinical significance and outcomes

BackgroundTo investigate the differences in cardiovascular disease, extracardiac anomalies and outcomes between fetuses with levocardia and dextrocardia.MethodsClinical demographics, prenatal features, postnatal characteristics and the outcomes of fetuses with levocardia or dextrocardia were recorded and analyzed.ResultsSixty-five fetuses with dextrocardia and thirty-eight fetuses with levocardia were enrolled. Right ventricle outlet obstruction, atrioventricular septal defect and intestinal malrotation were common in both groups. Univentricular physiology, transposition of the great arteries and esophageal atresia were more frequent in fetuses with levocardia, whereas abnormal pulmonary venous connection, double outlet of right ventricle, left ventricle outlet obstruction and brain abnormalities were more frequent in the dextrocardia group. The accuracy of evaluating cardiac malformations was high, but the sensitivity in assessing extracardiac abnormalities was low.ConclusionsAlthough the disorders have certain overlapping features, there are several differences between fetuses with levocardia and dextrocardia. These findings might improve patient counseling and perinatal management.

The prenatal ultrasonic study of cerebral sulci and gyrus development in fetuses with conotruncal defects at the second and third trimester

Objective To evaluate the development of cerebral sulci and gyrus in fetuses with conotruncal defect(CTD) at the second and third trimester by ultrasound, and to improve clinician′s understanding of the cerebral cortical development in fetuses with CTD. Methods The study was conducted at Shenzhen Maternity and Child Healthcare Hospital Affiliated to Southern Medicial University from December 2015 to January 2017, enrolled 180 women with single fetus, among whom 60 fetuses with CTD and gestational age between 20 weeks to 33+ 6 weeks were diagnosed by echocardiographic examination. CTD included persistent truncus arteriosous, tetralogy of fallot, transpofition of the great arteries and double outlet of right ventricle. Evaluation indexes of cerebral sulci and gyrus included depth and angle of parietooccipital sulcus, width and depth of sylvian fissure, uncovered width of insular, uncovered insular ratio, depth of calcarine sulcus, head circumference; evaluation indexes of hemodynamics included umbilical artery resistance index, umbilical artery pulsation index, middle cerebral artery resistance index, middle cerebral artery pulsation index, the cerebral-to-placental resistance ratio and the cerebroplacental pulsation ratio. Above indexes were measured for statistical analysis. Results At the whole gestational age in the study, depth of parietooccipital sulcus and width of sylvian fissure in CTD fetuses were smaller than those in the control group, uncovered insular ratio was larger than that in the normal group(P<0.05); middle cerebral artery resistance index, middle cerebral artery pulsation index and the cerebroplacental pulsation ratio in CTD fetuses were lower than those of the control group (P<0.05). Conclusions The change of hemodynamics in fetuses with CTD may be one of the reasons that fetuses with CTD have cerebral cortical hypoevolutism. Key words: Ultrasonography, prenatal; Fetus; Conotruncal defects; Sulci and gyrus

Comorbidities with Congenital Heart Disease among Hospitalized Children in a Specialized Cardiac Hospital in Bangladesh

Background: Congenital heart diseases (CHD) when associated with any comorbid condition may complicate the outcome of management. So to find out and treat comorbidities before definite treatment is essential for better outcome. Methods: This observational cross sectional study was conducted in the inpatient Department of Paediatric Cardiology at National Institute of Cardiovascular Diseases, Dhaka, during the period of January 2014 to January 2015 to identify the comorbid conditions of congenital heart disease.Results: A total of 794 children with different types of congenital heart disease were enrolled during the study period. Among them, 48.6% were simple CHD and 51.4% were complex CHD. Among simple CHD, ventricular septal defect (VSD) was commonest (27.2%), Tetralogy of Fallot (TOF) occupied the second position (20.5%). One child presented with Ectopia cardis. Among complex CHD most common anomalies were VSD with either atrial septal defect (ASD) or patent ductus arteriosus (PDA) or valvular lesion. Next common anomalies were TOF with PDA or ASD &amp;/or pulmonary valvular abnormalities. Double outlet of right ventricle (DORV) with shunt and transposition of great arteries (TGA) with shunt also occur frequently. AV canal defect with or without valvular lesion presented in significant number. Among them 616 (77.6%) had different types of comorbid conditions. Among comorbidities, respiratory and acquired cardiac comorbidities were common (37.9% &amp; 34.1% respectively). Other congenital or genetic comorbidities were in 11.2%. Multisystem involvement was in 9.8% cases.Conclusion: Pneumonia and heart failure were the most frequent comorbid condition among both simple and complex congenital heart disease. They were common among acyanotic heart disease. But cyanotic spell, acute stroke syndrome and brain abscess were common among cyanotic heart disease. Among genetic comorbidities Down’s syndrome occupied the major part.Cardiovasc. j. 2017; 9(2): 83-89

EP04.06: Prenatal diagnosis of absent pulmonary valve syndrome

To analyse the echocardiographic features of absent pulmonary valve syndrome (APVS). The echocardiographic findings were retrospectively analysed in seven fetuses with APVS and the echocardiographic features of APVS were summarised. Of all seven fetuses with APVS, all of which demonstrated cardiac enlargement, the axis of the heart was to the left and the main pulmonary artery or branches were significantly dilated. The pulmonary valve was absent or rudimentary. Colour Doppler flow imaging demonstrated severe pulmonary regurgitation. Spectral Doppler imaging showed stenosis of the pulmonary annulus. Four fetuses associated with tetralogy of Fallot, one case of which associated with small foci in the left ventricle and one case associated with single umbilical artery. Two fetuses associated with right aortic arch. Two fetuses associated with double outlet of right ventricle. One fetus associated with atresia of mitral valve, right-ventricle typed single ventricle. One fetus associated with double outlet of right ventricle. One fetus associated with ventricular septal defect. Three fetuses associated with absent ductus arteriosus. The fetal echocardiographic findings of the APVS are characteristic and the APVS should be considered when markedly dilated right ventricle and dilated pulmonary arteries in combine with the stenosis and severe regurgitation of the pulmonary annulus. Identifying whether or not the ductus arteriosus is present can help in evaluating the prognosis.

Heart Transplantation in a Patient with Left Isomerism

We report the case of a 37-year-old man who suffered from biventricular failure due to left isomerism, inferior vena cava interruption with azygos vein continuation, bilateral superior vena cava, double outlet of right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and isolated dextrocardia. Heart transplantation in patients with systemic venous anomalies often requires the correction and reconstruction of the upper & lower venous drainage. We present a case of heart transplantation in a patient with left isomerism, highlighting technical modifications to the procedure, including the unifocalization of the caval veins and reconstruction with patch augmentation.

Prevalence and spectrum of Nkx2.6 mutations in patients with congenital heart disease

Congenital heart disease (CHD) is the most common form of birth defect and is the most prevalent non-infectious cause of infant death. A growing body of evidence documents that genetic defects are involved in the pathogenesis of CHD. However, CHD is a genetically heterogeneous disease and the genetic basis underpinning CHD in an overwhelming majority of patients remain unclear. In this study, the coding exons and flanking introns of the Nkx2.6 gene, which codes for a homeodomain-containing transcription factor important for normal cardiovascular development, were sequenced in 320 unrelated patients with CHD, and two novel heterozygous Nkx2.6 mutations, p.V176M and p.K177X, were identified in two unrelated patients with CHD, respectively, including a patient with tetralogy of Fallot and a patient with double outlet of right ventricle and ventricular septal defect. The mutations were absent in 400 control chromosomes and the altered amino acids were completely conserved evolutionarily across species. Due to unknown transcriptional targets of Nkx2.6, the functional consequences of the identified mutations at transcriptional activity were evaluated by using Nkx2.5 as a surrogate. Alignment between human Nkx2.6 and Nkx2.5 proteins showed that V176M-mutant Nkx2.6 was equivalent to V182M-mutant Nkx2.5 and K177X-mutant Nkx2.6 was equal to K183X-mutant Nkx2.5, and introduction of V182M or K183X into Nkx2.5 significantly diminished its transcriptional activating function when compared with its wild-type counterpart. To our knowledge, this is the first report on the association of Nkx2.6 loss-of-function mutation with increased susceptibility to tetralogy of Fallot or double outlet of right ventricle and ventricular septal defect, providing novel insight into the molecular mechanism of CHD.

Diagnosis, classification and prognosis prediction of fetal double outlets of right ventricle by echocardiography

Objective To study the accuracy of diagnosis,anatomical classification and prognostic prediction of double outlet of right ventricle (DORV) by fetal echocardiography.Methods Fifty-nine fetuses detected and diagnosed with DORV by fetal echocardiography were investigated to analyze the anatomical characteristics,types and complicated malformations.Results Fifty-nine fetuses with DORV included 56 with atrioventricular accordance and 3 with atrioventricular discordance.Among them,there were 44 with dextro-transposition of great arteries,7 with levo-transposition,2 with normal position,6 with un-identified in early stage.SDD was the most common type in DORV for approximate 81％ (44/53).Complicated abnormalities included pulmonary artery stenosis in 21 fetuses,single atrium in 5,atrialventricular valvular stenosis in 3,and mild ascending aortic dysplasia in 2.Amniocentesis and chromosome were test in 7 fetuses including 5 fetuses with normal chromosomes,one with trisomy 21 and one with trisomy 18.Conclusions Fetal echocardiography shows high clinical value in diagnosis,classification and detecting complicated abnormalities of DORV.Careful examinations provide accurate evaluation in surgical procedure and prognosis. Key words: Ultrasonography, prenatal; Double outlet right ventricle

The Association between Epicardial Adipose Tissue and Coronary Artery Disease: an Echocardiographic Cut-off Point.

EAT is an independent factor in coronary artery disease (CAD). The objective of the current study was to define an echocardiographic cut-off point for EAT and to determine its diagnostic value in predicting the increase in CAD risk. Two hundred patients underwent coronary artery angiography for diagnosis of CAD and transthoracic echocardiography for measurement of EAT on the right ventricle (RV), RV apex and RV outlet tract. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of the EAT cut-off points in the three above-mentioned areas for predicting the severity of CAD were measured. The relation between the EAT and CAD risk factors was evaluated as well. EAT was independent from gender, height, hypertension, diabetes, HDL, total cholesterol, ejection fraction, acute coronary syndrome, and the location of the coronary artery stenosis in the coronary artery in all three anatomical areas. EAT on RV and RV apex had a significant relation with CAD (P ≤ 0.05). Overall, RV EAT≥ 10 mm and RV apex EAT ≥ 8 mm had sensitivity and PPV of more than 70% in predicting coronary stenosis ≥ 50% and acute coronary syndrome (ACS) and RVOT EAT ≥ 13 mm is of PPV=83.5% for predicting coronary stenosis ≥ 50%. EAT thickness has an acceptable diagnostic value for predicting severe coronary artery stenosis and ACS. Therefore, non-invasive EAT thickness measurement could be of great assistance to clinicians for detecting the patients at risk and helping them to undergo supplementary evaluations with invasive approaches.

Stress Transient Hypertrophic Cardiomyopathy and B-Type Natriuretic Peptide Role

This report describes a transient hypertrophic cardiomyopathy with right-ventricle outlet tract obstruction (RVOTO) induced by perinatal stress due to a major surgical procedure in a female newborn with congenital abnormalities. On day 10, she presented with heart failure, abnormal B-type natriuretic peptide (BNP), and an echocardiogram showing normal wall thickness. An in-hospital follow-up echocardiogram showed biventricular hypertrophy and RVOTO. At discharge, the infant was asymptomatic, with a normal echocardiogram and BNP. Transient RVOTO triggered by surgical stress and abnormal BNP have not been reported previously. Pathophysiology, the role of BNP, and clinical characteristics are discussed.

Deep Sedation with Sevoflurane in Patients with Double Outlet of Right Ventricle

Deep Sedation with Sevoflurane in Patients with Double Outlet of Right Ventricle

Precise automated determination of the total and segmented right ventricular volumes for functional studies of the right ventricle using CMR

Background Cardiac magnetic resonance (CMR) is an acknowledged standard for quantification of left ventricular volumes, but the acceptance of CMR to measure the volume of the right ventricle (RV), including sub-units, such as inlet, outlet, and apical segment has been limited. In patients with Fallot (TOF), the size and function of the right ventricular outflow tract (RVOT) in the course of an RR-interval can serve as indication for re-operation in cases of pulmonary stenosis, regurgitation, or RVOT aneurysms. in addition to the results of conductance examinations. However, none of the available software applications provides a suitable systematic subdivison of the total volume. A novel algorithm was developed for this purpose and tested in this study. Methods We present an automated method for the determination of RV volumes based on short axis cine series from CMR examinations. The contour detection proceeds iteratively through slices and phases. Manual interaction is possible. For further functional analysis of the right ventricle, a subdivision into compartiments is applied. The subdivision is performed automatically from flat or curved layers as defined by landmarks identified by the investigator. The landmarks include the crista supraventricularis, the set in of trabeculary structures, and the apex. The conventional definitions of the compartiments are loose, therefore two definitions of the outlet are compared to each other: one robust and simple definition using flat separating layers, and a more complicated but flexible definition based on a curved layer. Results Depending on the quality of the images, this contour detection is successful (i. e. requires no further manual interaction) in up to about 95% of images in the midventricular region. Papillary structures in the apex as well as a weakly separable atrium in the basal region may require manual interactions. Volumes are provided for all phases in total and in specific regions. Stroke volume and ejection fraction arise naturally. The volumes obtained from the two outlet definitions turn out to agree typically within 10% of the total volume and thus do not lead to contradicting conclusions. Conclusion The application of this fast iterative contour detection method and volumetry may allow the evaluation of the chamber volumes for the full heart cycle in clinical routine. The automatic volume segmentation provides useful information about the function of a particular segment such as the RV outlet in TOF patients where it can complete the indications for a surgery.

Diagnosis of coronary arterial anatomy of patients with arterial switch operation using echocardiography

Objective To evaluate the accuracy of preoperative diagnosis on the anatomy of coronary artery of patients with arterial switch operation(ASO) using echocardiography, to conclude the diagnostic methods. Methods From March 2007 to March 2008,50 patients were performed ASO after the diagnosis with eehoeardiography. Prior to the operation, evaluation was conducted to all of anatomical pattern of the coronary artery,and the comparison was made to the observation in the operation. There were 23 patients with complete transposition of great artery(TGA), 20 patients with double outlet of right ventricle of abnormal great artery(DORV), 2 patients with corrected transposition of the great arteries(CTGA), 1 patients with double outlet of left ventricle of abnormal great artery (DOLV), 2 patients with TGA complicated with total anomalous pulmonary venous connection (TGA + TAPVC), 2 patients with TGA complicated with pulmonary valve stenosis(TGA + PS). Results Among 50 patents,there were 40 patients with normal coronary artery,and 10 patients with abnormal coronary arteries. Except for 2 patients failing to being successfully diagnosed due to bad condition of image,48 patients could be revealed the origin and the direction of the major coronary arteries, the visibility proportion was 96.0%. Compared to the observation in the operation,the total echocardiography accuracy for 48 patients was 95.8%. Among the 40 patients with normal coronary arteries,39 patients were diagnosed accurately except 1 patient failed to be showed clearly by echo. In the 10 patients with abnormal coronary arteries,4 patients with single abnormal coronary artery, 2 patients with 2 coronary artery originating from the same coronary sinus, 2 patients with left anterior descending coronary artery originating from right coronary artery, 2 patients with 2 coronary artery originating from two adjacent coronary sinus. One patient failed to be diagnosed by echo for the bad image,2 patients was misdiagnosed by echo, the accuracy of echo was 77.8%. Conclusions Through multi-section scanning,echocardiography can accurately diagnose anatomical pattern of coronary artery for most of ASO,thus providing comprehensive and accurate diagnosis information for ASO. Key words: Echocardiography; Transposition of great vessels; Coronary vessel anomalies

Clinical analysis of surgical procedures and outcomes for corrected transposition of great arteries with heart anomaly

To determine the outcome of anatomically corrective repair and traditional repair of corrected transposition of great arteries (c-TGA) with heart anomaly. From April 2002 to December 2006, nineteen patients including fourteen male and five female with c-TGA, underwent operations, age ranged from 2 to 22 years old and weight ranged from 10 to 48 kg. Fifteen of them received anatomically corrective repair and the other four received traditional repair. Eighteen patients were referred to SLL (segmental anatomy) in situs solitus while fifteen of them with levocardia and three with dextrocardia. One patient was referred to IDD (segmental anatomy) in situs inversus with levocardia. Associated cardiac lesions included ventricular defect in eighteen patients, double outlet of right ventricle in one patient, pulmonary stenosis in seventeen patients and pulmonary hypertension in two patients. The operative procedures to anatomically correct atrioventricular discordance included an atrial switch plus a ventricle-arterial switch. The atrial switch was performed using the modified Senning procedure (n=13), Senning procedure (n=1) and Mustard procedure (n=1). The ventricle-arterial switch was performed using a Rastelli procedure (n=13) or an arterial switch (n=2). The patients underwent Mustard and Rastelli procedure had received bi-direct Gleen shunt due to postoperative high pressure of superior vena cava. Three patients underwent traditional cardiac repair because of small ventricular septal defect and one patient was reoperated to undergo traditional cardiac repair because of left ventricular failure after received anatomically corrective repair. In the patients received anatomically corrective repair, there was one early operative death received a modified Senning atrial switch and an arterial switch. The cause of death was acute myocardial failure due to imperfect coronary transfer. The postoperative complications included severe low cardiac output syndrome (n=1), temporary atrioventricular block (n=1) and thorax cavity fluidify (n=1). The survivors were followed up for 6 months to 4 years. All were sinus cardiac rhythm and in NYHA class I or II. There was no death in the patients received traditional repair. Four patients were followed up for 1 year. Three patients were in NYHA I or II class and one patient in class II. Anatomically corrective repair of c-TGA can be performed with good operative survival and intermediate-term outcome. The patients with good right ventricular function and well developed tricuspid valve who were difficult to undergo anatomically corrective repair might be fit to receive traditional repair.

Antenatal diagnosis of double outlet of right ventricle without extracardiac anomaly: A report of 4 cases

Four fetuses were diagnosed antenatally with double outlet of right ventricle (DORV) at 17, 20, 26, and 28 weeks' gestation, respectively, using 2-dimensional sonography. Chromosome study was normal in all 4 cases, and there were no extracardiac abnormalities. The sonographic diagnoses were based on the following findings: (1) arising of the 2 great vessels predominantly from the right ventricle; (2) the presence of bilateral coni; and (3) parallel direction of the 2 vessels rather than the normal perpendicular course. Two cases were terminated electively with postnatal autopsy confirmation; the other 2 cases were closely monitored throughout pregnancy and underwent normal vaginal delivery at term and subsequent surgical correction with good outcome. In conclusion, DORV can be readily diagnosed in utero by checking outflow tract even without 3-dimensional sonography and in the absence of obvious risk for congenital heart defects.

Arterial switch operation for transposition of the great arteries and double outlet of right ventricle with subpulmonary ventricular septum defect

To summarize the clinical experience on the arterial switch operation for complex congenital heart disease in recent 3 years in our hospital. Sixty-one patients with complex congenital heart disease received by arterial switch operation from Jan 2000 to May 2003. There were 26 patients with transposition of the great arteries and intact ventricular septum (TGA-IVS), 19 patients with transposition of the great arteries and ventricular septal defect (TGA-VSD) and 16 patients with double outlet of right ventricle with subpulmonary ventricular septum defect (Taussig-Bing). There were 2 death among TGA-IVS cases, 4 deaths among TGA-VSD cases and 4 deaths among Taussig-Bing cases. The total operative mortality was 16%. All patients were followed up 3 months to 3 years. In all patients, the cyanosis disappeared and the physical activities increased obviously. One patients with Taussig-Bing keep moderate mitral valve reflux unchanged, 2 patients with TGA had pulmonary and aortic supra-valve stenosis with 40 mmHg pressure gradient. One patient with subpulmonary stenosis and residual VSD was re-operated 3 months later. All survivor had good heart function. The effect of arterial switch operation on the treatment of TGA was well accepted in this study. The procedure used in treatment on Taussig-Bing can prevent pulmonary obstruction and avoid the complication of left ventricular outflow tract obstruction caused by intraventricular repair.

Modified Fontan Procedure with the Simultaneous Bidirectional Cavopulmonary Shunt

We reviewed our experience of 56 patients from 1989 to 1992 who underwent a modified Fontan procedure and a bidirectional cavopulmonary shunt simultaneously. There were 39 male and 17 female patients and their weight ranged from 6.54 to 29kg (mean weight 13.58 ± 3.96kg). Patient age ranged from 16 to 135 months (mean age 42.8 ± 3.7 months). Diagnoses included single ventricle in 29, tricuspid atresia in 11, double outlet of right ventricle in 10, hypoplastic left heart syndrome in 4, and pulmonary atresia with intact ventricular septum in 2 patients. The techniques of inferior vena cava to pulmonary artery (IVC-PA) connection were anastomosis of proximal superior vena cava (SVC) to pulmonary artery (PA) in 27 (group 1), direct atriopulmonary anastomosis with roof formation in 29 patients (group 2). There were significant differences in postoperative 1-hour right atrial (RA) pressure and period of chest tube drainage between group 1 and group 2. The early mortality was 12.5% (7/56), and 2 late deaths (4.1%) occurred with a mean follow-up period of 22.4 months. Risk factors for the late postoperative arrhythmia were immediate postoperative arrhythmia and prolonged pleuro-pericardial effusion. Direct connection of the remaining proximal SVC to PA with the bidirectional cavopulmonary shunt may have less pleuro-pericardial effusion and late arrhythmia than atriopulmonary anastomosis.

Arterial Switch for Repair of Simple and Complex Transposition of Great Arteries in a New Unit

Between January 1987 and October 1992, 11 neonates and 2 infants (12 males and 1 female) underwent arterial switch operation (ASO). There were 9 simple transpositions of great arteries (TGA), 3 TGA with ventricular septal defect (VSD), and 1 double outlet of right ventricle (Taussig-Bing) with TGA. There were 12 cases of patent ductus arteriosus (PDA) and 6 cases of atrial septal defect (ASD). Nine patients had Yacoub type A coronary pattern, 3 had type B, and 1 had type D. The neonates' mean age was 15.5 days (one 120 days old and one 22 months old). The average body weight of the neonates was 4.2 kg. The Lecompte maneuver was performed on all patients. The average bypass time was 187.8 min, and mean aortic cross-clamp time was 76.2 min, with a mean circulatory arrest time of 10 min in 6 cases. Early hospital mortality was 15.4% (2 deaths)—1 patient died during operation from myocardial infarction, and another died postoperatively from poor heart function. All survivors experienced early postoperative heart failure and needed temporary inotropic support. One patient was lost to follow-up. The other 10 patients were free from any cardiac symptoms at follow-up, with good left ventricular function. Mild to moderate aortic regurgitation and stenosis and the same in the pulmonary valve were seen on echocardiogram. Seventeen months after ASO, 1 patient required reoperation for severe postoperative pulmonary stenosis. We concluded that ASO can be performed with an acceptable mortality rate in a new unit with no previous experience of such surgery.